UNION CHRISTIAN COLLEGE

COLLEGE OF NURSING
Level III
San Fernando City, La Union

LECTURE NOTES
NCM 102

MEDICAL SURGICAL NURSING:

Cardiovascular Disorders
Respiratory Disorders
Gastrointestinal Disorders
Endocrine Disorders
Urological Disorders

PREPARED BY:

RODERICK C. SUMINTA, BSN - RN, PTRP

LEVEL III – CLINICAL INSTRUCTOR
 CARE OF CLIENTS WITH CARDIOVASCULAR DISORDERS

A. Alterations in Oxygenation
1. GAS TRANSPORT
(Cardiovascular System)
Anatomic and Physiologic Overview (The HEART)
 The heart is a hollow, muscular organ located in the center of the thorax, where it occupies the space
between the lungs (mediastinum) and rests on the diaphragm.
 It weighs approximately 300g (10.6 oz), although heart weight and size are influenced by age,
gender, body weight, extent of physical exercise and conditioning and heart disease.
 Heart pumps blood to the tissues, supplying them with oxygen and other nutrients.

 During systole (contraction of the muscle), the chambers of the heart become smaller as the blood is
ejected.

 During diastole (relaxation of the muscle), the heart chambers fill with blood in preparation for the
subsequent ejection.
 Normal resting adult heart beat is approximately 60 to 80 time per minutes.
 Each ventricles ejects approximately 70ml of blood per beat and has an output of approximately 5 L /
minute.

 The heart is composed of three layers. The inner layer, or endometrium, consists of endothelial
tissue, which lines the inside of the heart and valves.

 The middle layer, or myocardium, is made up of the muscle fibers and is responsible for the
pumping action.

 The exterior layer of the heart is called the epicardium.

 The heart is encased in a thin, fibrous sac called the pericardium, which is composed of two layers.
Adhering to the pericardium is the visceral pericardium. Enveloping the visceral pericardium is the
parietal pericardium, a tough fibrous tissue that attaches to the great vessels, diaphragm, sternum
and vertebral column and supports the heart in the mediastinum.
 The space between these two layers (pericardial space) is filled with about 30ml of fluid, which
lubricates the surface of the heart and reduces friction during systole.
 The heart chambers constitute the right-and left-sided pumping system.
 The right heart is made up of the right atrium and right ventricle, which distributes venous blood
(deoxygenated blood) to the lungs via the pulmonary artery (pulmonary circulation) for oxygenation.
 The right atrium receives blood returning from the superior vena cave (head, neck, and upper
extremities), inferior vena cava (trunk and lower extremities) and coronary sinus (coronary
circulation).
 The left atrium receives oxygenated blood from the pulmonary circulation via the pulmonary vein.
 Atria are thin-walled because blood returning to these chambers generates low pressures.
 Ventricular walls are thicker because they generate greater pressures during systole.
 The right ventricle has thinner walls than the left ventricle. The right ventricle contracts against low
pulmonary vascular pressure, whereas the left ventricle, with walls two-and-a-half times more
muscular than the right, contracts against high systemic pressure.

 Right ventricle lies anteriorly (just beneath the sternum) and the left ventricle is situated posteriorly.
The left ventricle is responsible for the apex beat or the point of maximal impulse (PMI), which is
palpable normally in the left midclavicular line of the chest wall at the fifth intercostals space.
MYOCARDIAL (CORONARY BLOOD SUPPLY)
1. LCA – located in the upper half of coronary sinus (sinus valsalva of the aorta)
Three branches:
• Left main coronary artery and two
bifurcation which are:
• Left circumflex – supplies left ventricle and
auricle or atrium
• Left Anterior descending artery – supplies
the anterior 2/3 of the septum and right bundle
branch.
2. RCA – supplies SA node, AV junction, posterior
bundle branch, posterior 1/3 of the septum, Right
ventricle and diaphragmatic surface of the Left
ventricle, Branch – posterior descending Artery.
3. Three systems of the Veins in the heart:
• thebesian vein
• anterior cardiac vein
• coronary sinus – primary means of draining from the left ventricle.

CONDUCTIVE SYSTEM
Sino-atrial note (Pace maker of the heart) 
atrioventricular node  Bundle of His  Right
and Left Bundle branch  Purkinje Fibers.
 Nervous system control – includes the
following:
a. Vagus nerve of the
parasympathetic – slows rate of impulse
formation in the SA node.
b. Sympathetic nerve – increase
the rate of the impulse formation,
conduction, and force of contraction.

PROPERTIES OF THE CARDIAC MUSCLE:

1. All-or-none Principle –
refers to the excitation of an entire
functional syncytium (multinucleate
mass of protoplasm resulting from
fusion of cell) rather than a single skeletal muscle fiber allowing synchronous pulsation of the entire
cardiac muscle as a whole unit.
2. Rhythmicity – refers to the regular formation and conduction of impulses.
3. Irritability – (excitability) – refers to the response stimuli.
4. Refractoriness – refers to delay in response to stimuli.
5. Conductivity – the ability of the cardiac muscles to transmit impulses throughout the
heart in a continuous rhythmic pattern.
6. Automaticity – the ability of the cardiac muscle to spontaneously initiate the impulses
without external control.
 7. Extensibility – the ability of the heart muscle to stretch or expand while the chamber
fill with blood between muscle contraction. This property is governed by the principle of Starling’s
Law which states that the greater the stretch of the cardiac muscle, the more forceful the contraction
of the heart.

OTHERS:
1. Cardiac Output (CO) – it is the volume of blood ejected per minute by each ventricle,
calculated by SV x HR.
2. Heart rate – refers to the actual number of heart beats occurring per minute.
3. Cardiac index – is C.O. divided by Body surface area (BSA); it is more accurate
measurement of cardiac output.
4. Stroke volume – refers to the amount of blood pumped from the ventricles during its
contraction of the heart (about 70ml of blood in an adult) and depends on:
i. Preload – resting force of the myocardium increased in venous return and depends
on two factors:
ii. Stretching potential of the Left ventricle (LV + diastolic pressure).
iii. The volume of blood remaining in the Left ventricle after diastole.
iv. Contractility –refers to the change in force of contraction without changing the resting
length.
v. Ejection Fraction – is the amount of blood ejected by the ventricle at each
contraction.
vi. Afterload – refers to the amount of ventricular tension during systole which is needed
to open the semi lunar valves and eject blood.
- Also refers to the resistance to the ventricular ejection offered by the
peripheral blood vessels minus total peripheral resistance(TPR).

BLOOD VESSELS (TRANSPORTATION NETWORK)

1. ARTERIES – composed of several layers and is elastic enough to accommodate the pressure of
blood.
Types of layers of arteries:
a. Intima – lining of the artery; smooth to propel blood but it has an affinity for certain lipids and
tends to acquire plaques during aging known as atherosclerosis.
b. Media – elastic to accommodate pulsatile forces. Calcium deposits in later life limits its
elasticity known as arteriosclerosis.
c. Adventitia – supports the artery. Hypertension, aneurysm, obstruction and inflammation are
common interferences.
2. MICROCIRCULATION (CAPILLARIES) – this is where actual exchange of O2 and CO2 normally
occurs. The exchange of substances across the capillary bed is affected by Hydrostatic pressure on
both sides of the capillary membrane and osmotic pressures both inside and outside the capillary
bed.
3. VEINS – it is less elastic and depends largely on muscular action to provide a forward flow of
circulatory blood sometimes called the capacitance vessels because they can distended and
accommodate 75% of blood volume, stasis lead to varicosities.

Flow Regulation: Two concepts involve in flow regulation:

a. Pressure Gradient – blood will flow from an area of higher pressure (the heart) to an area of
lower pressure (the blood vessels).
 b. Flow resistance – this primarily depends on the vessels diameter and the viscosity of the
fluid. Arterial BP is a reflection of C.O. and the resistance.

 Blood and Lymphatics –

 The blood is the transport media of O2, CO2 and metabolites.

COMPOSITION AND FUNCTION OF BLOOD:

1. Red blood cells (Erythrocytes) – Normal value is 4.5 – 5 million / cubic mm. for women; 4.8 – 5.4
million in men.
• Life span is between 115 – 130 days.
• Normal erythropoesis depends in adequate amounts of iron, B12, pyridoxine, folic acid,
protein, copper, cobalt.
• B12 absorption depends on the presence of the intrinsic factor secreted by gastric mucosa.
• Contains hemoglobin – which transports O2.
Hemoglobin:
i. Most of the oxygen in the blood is contained within the red blood cells, where it is chemically
bonded to Hemoglobin. Each hemoglobin molecule consists of (1) a protein globin part,
composed of four polypeptide chains and (2) four nitrogen-containing, disc-shaped organic
pigment molecules called hemes. Each of the four globin groups plus one heme group
consist of one iron molecule which can combined with one oxygen molecule.
ii. One hemoglobin molecule can combine with four molecules of oxygen.
iii. Each Red blood cell composed of approximately280 million hemoglobin molecules and over
a billion molecules of oxygen per red blood cells.
iv. In response to tissue hypoxia, the kidneys secrete hormone erythropoietin.
2. Erythropoietin – hormone produced primarily by the kidneys which is responsible for the production
of hemoglobin and RBC in the bone marrow. The production of erythropoietin and RBC is stimulated
when the amount of Oxygen is lower than normal. RBC production is also promoted by androgens
which explain why the hemoglobin in men averages 1 – 2 g per 100ml higher than in women.
a. Normal value: 14.5 – 16 g/100ml in male.
b. 13.0 – 15.5 g/100ml. in female.
c. Hematocrit: 42 – 54% in male; 38 – 46% in female.
Functions of RBC:
a.Transport hemoglobin (with O2 and other nutrients from the lungs to the body tissues and vital
organs.
b.Removes CO2 from the tissues and return to the lungs.
c.Hemoglobin with RBC acts as an acid-base buffer accounting 70% buffering.
3. White Blood Cells (Leukocytes) Normal value: 5,000 – 10,000 /mm3
4. Types of Leukocytes:
a. Granulocytes (Polymorphonuclear) is formed in the bone marrow, and has 3 formed of
mature types
i. Neutrophils (60 - 70%) – increased in acute infections and severe tissue damage.
ii. Eosinophils (0 – 5%) – increased in allergic reaction and parasitic infection.
iii. Basophils () – 3%) – releases small quantities of heparin.
b. Agranular cells – originate in bone marrow and lymphatic tissue.
i. Lymphocytes (30 - 40%) – wall off chronic infections and produce antibodies.
1. T – Lymphocytes – derived from thymus and responsible for cellular
immunity.
2. H – Lymphocytes – derived from bone marrow and responsible for humoral
immunity
ii. Monocytes () - 5%) – becomes macrophages and engulf large particles.
5. Platelets (Thrombocytes) Normal value 150,00 – 500,000/mm3
Function: (Blood coagulation)
6. Plasma – 90% water and contains blood proteins (albumin which is responsible for plasma
colloidal osmotic pressure) globulin; fibrinogen; prothrombin, anticoagulants clotting factors and
antibodies.
 7. Blood has a major role in homeostasis; transport nutrients and electrolyte to various tissue
and distribute hormones and enzymes that regulate body activities. Normal amount of blood – 5 –
6 liters of blood.
8. The lymphatic circulation can be considered as an adjunct to the delivery and the regulatory
role of the cardio-vascular system although its primary function is the production of immune
antibodies.

ASSESSMENT
1. Nursing History
i. Health History
A. Non-modifiable risk factors:
1. Age – atherosclerosis is disease of middle age (40 - 50). Cardiovascular disease is
greater in men until 65 when the incidence equalizes.
2. Gender – some theories attributes it to the higher estrogen levels of pre-menopausal
women because there maybe a relationship between increase in estrogen level to high
density lipoproteins which are inversely related to atherosclerosis and ischemic heart disease
(IHD)
3. Race - - IHD is higher in whites; HPN is twice greater in blacks.
4. Genetic History – family history appears to be a significant risk factor for
predisposition to heart disease.
B. Modifiable Risk Factors:
1. Hyperlipidemia – plasma lipids (cholesterol), triglycerides, phospholipids and fatty
acids) are produced from endogenous synthesis in the liver and from the dietary intake of
fats.
Types of Hyperlipidemia:
i. Primary hyperlipidemia – caused by inborn error of lipid metabolism.
ii. Secondary Hyperlipidemia – related to such conditions as DM or hypothyroidism.
iii. For lipids to be used and transported by the body they need to become soluble in
blood by combining with protein to form macromolecules called lipoproteins.
Lipotprotein are vehicle for fat mobilization and transport.
2. Types of lipoproteins:
i. HDLs – contain more protein by weight and less lipid han any other lipoprotein. They
carry lipids away from arteries and to the liver for metabolism. Therefore, high serum
LDL are desirable.
ii. LDLs – contain more cholesterol than any of the other lipoproteins and have an
affinity for arterial walls. Elevated LDL correlate most closely with an increased
incidence of atherosclerosis.
iii. VLDLs – contain most triglycerides. High VLDL concentration may increase the risk
of premature atherosclerosis when associated with other factors such as diabetes,
hypertension and cigarette smoking.
4. Hypertension – known to be a precursor of atherosclerosis.
Prevention: control sodium and caloric intake.
Management: Diuretics; antihypertensive
 Sodium restricted diet
 Calorie controlled diet
 Therapy must be continued for lifetime and compliance on this program is
imperative.
 5. Smoking – (Hazard) – the hazard of tobacco smoking are produced by the inhalation
of nicotine, which is a vasoconstrictor and carbon monoxide which reduces the oxygen
carrying capacity of the blood.

 Elevated serum lipids, hypertension and cigarette smoking are the (3)
most significant modifiable risk significant.
6. Sedentary lifestyle –
 Prevention: Engage in regular exercise; this will promote muscle strength,
flexibility, endurance.

7. Obesity – it is defined as 20% over the ideal weight.

 Risk Factors: Hypertension and hyperlipidemia.
 Prevention: Dietary and exercise habits should begin during prenatal
development and early years of life.
 Management: Weight reducing program. Latest management is the bariatric
surgery.
8. Stress – a positive relationship exist between psychological stress and
cardiovascular disease.
Management:
 Stress management – includes behavior modification.
 Temporary use of sedatives and tranquilizers.
9. Glucose intolerance – serum glucose levels greater than 120mg/100ml are twice the
risk for heart disease.
 Prevention: maintaining weight at normal level
 Management: Plan as to the prevention of hyperglycemia and glucosuria.
10. Alcohol abuse
11. Caffeine – known to cause cardiac dysrhythmias. Avoid coffee, tea, some soft drinks
or chocolate.
12. Environmental risk – Environmental pollution
ii. Developmental History
iii. Social History
iv. Psychological History

2. Physical assessment of Heart:

a. Inspection and palpation:
1. Aortic area – 2nd ICS to the right of the sternum.
2. Pulmonic area – 2nd ICS to the left of the sternum
3. Right Ventricular area – a circle around the 5th cartilage to the patient’s
left of MSL (midsternal line)
4. Apical or left ventricular area – 5th ICS at the MCL (miclavicular line)
5. Observe for cardiac movement at the PMI located at MCL at the 5th
ICS (intercostals space). Thrills are vibration that occur as blood flow
through a marrow or damaged valves.
b. Percussion – CAD (cardiac area of dullness)
c. Auscultation: rate and rhythm
Heart Sounds
a. S1 – first heart sound due to closure of atria-
ventricular valves; loudest at the apex.
b. S2 – second heart sound due to closure of
semilunar valves; loudest at the base.
c. S3 – “ventricular gallop” – 3rd heart sound
normal in children and young adult.
 d. S4 – “Atrial gallop” fourth heart sound normal in children and young adult but in adult it is
associated with systemic or pulmonary hypertension M.I. and other cardiac diseases.
ii. Murmurs – sounds produced by vibrations within the heart and great vessels caused by
turbulence of flow.
iii. Rubs – sounds produced by the interlacing of parietal and visceral surfaces of the pericardium
iv. Auscultatory areas:
a. Aortic area
b. Pulmonic area
c. Third left ICS or Erb’s point
d. Tricuspid area – 5th (L) ICS to the sternum
e. Mitral (Apical area) – 5th (L) ICS first medial to the MCL
3. Physical Assessment of Blood Vessels:
f. Inspection
i. Color – inadequate circulation may produce pallor, rubor, cyanosis.
Cyanosis is best visualized with good lighting. Vascular nailbeds offers best visualization.
ii. Circulation of extremities
9. Hair growth – absence means inadequate circulation
10. Clubbing
11. Capillary refill – prolong filling time is indicative of inadequate circulation.
g. Palpation
i. Edema – assess over a bony prominence such as the medial malleolus, anterior
tibia, sacrum. Press for 5 seconds and measure for pitting.
ii. Pulse – carotid, dorsales pedis, poplitial, posterior tibial.
h. Auscultative – normal arteries do not produce sounds. Bruit are blowing sounds heard in
conditions like A-V fistula.

4. Manifestations of heart Diseases:

1. Dyspnea – discomfort associated with breathing:
Types of Dyspnea:
i. Exertional dyspnea
ii. Orthopnea – shortness of breath when lying down.
iii. Paroxysmal nocturnal dyspnea (PND) sudden dyspnea at night while lying down.
iv. Cheyne-stokes periodic breathing characterized by gradual increase in depth of respiration
followed by a decrease in respiration resulting in apnea.
2. Chest pain
3. Edema – abnormal accumulation of serous fluid in the connective tissues.
Types of Edema:
i. Ascites – excessive fluid in peritoneal cavity.
ii. Hydrothorax – excessive fluid in the pleural cavity.
iii. Anasarca – gross generalized edema.
4. Palpitation – a rapid, forceful or irregular heartbeat felt by the patient.
5. Hemoptysis – is coughing out of blood.
a. Small quantities of dark-clotted blood – indicates mitral stenosis.
b. Admixture of blood and pus – indicates pulmonary suppuration.
c. Pink, frothy sputum – in acute pulmonary edema.
d. Blood-streaked sputum – in acute pulmonary congestion.
e. Frank hemoptysis – due to lung pathology.
6. Fatigue
7. Syncope and fainting – may be caused by anoxemia or reduced cardiac output resulting
inadequate circulation.
8. Cyanosis – bluish discoloration of the skin and mucus membranes.
9. Abdominal pain or discomfort.
10. Clubbing of fingers – angle of the nail is 180o due to chronic hypoxia.
11. Jaundice – yellowish discoloration of skin and sclerae.
 DIAGNOSTIC ASSESSMENT:
1. DIAGNOSTIC TESTS RELATED TO CARDIOVASCULAR FUNCTION
A. LABORATORY TESTS
A. COMPLETE BLOOD COUNT (CBC)
 For evaluation of general health status
 Elevated RBCs suggest inadequate tissue oxygenation.
 Hypoxia stimulates the secretion of erythropoietin causing the bone
marrow to increase RBC production (polycythemia)
 Elevated WBC may indicate infectious heart diseases and MI
 ERYTHROCYTE SEDIMENTATION RATE (ESR)
 Is a measurement of the rate at which RBC ‘settle out’ of anticoagulated
blood in an hour.
 Is elevated in infectious heart disorders or MI
 Normal range is as follows:
- Males : 15 – 20 mm/hr
- Females : 20 – 30 mm/hr
B. BLOOD COAGULATION TESTS
 PROTHROMBIN TIME (PT, PRO – TIME)
 Measures the time required for clotting to occur after thromboplastin and
calcium are added to decalcified plasma
 Is valuable in evaluating the effectiveness of Coumadin
 Normal range: 11 – 16 seconds
 Therapeutic range: 1.5 to 2
times the normal
 PARTIAL THROMBOPLASTIN TIME (PTT)
 Measures the time required for clotting to occur after a ‘partial
thromboplastin reagent’ has been added to blood plasma
 Is the best single screening test for disorders of coagulation
 Is commonly evaluated to monitor the effectiveness of heparin
 Normal range: 60 – 70 seconds
 Therapeutic range: 1.5 to 2 times the normal
 ACTIVATED PARTIAL THROMBOPLASTIN TIME (APTT)
 Has the same as PTT
 Is most specific to evaluate the effectiveness of heparin
 Normal range: 30 – 45 seconds
 Therapeutic range: 2 to 2.5 times the normal
C. BLOOD UREA NITROGEN (BUN)
 Is an indicator of renal function
 Decreased cardiac output leads to low renal tissue perfusion and reduction in
glomerular filtration rate, hence, BUN level becomes elevated
  Normal range: 10 – 20 mg/dl
D. BLOOD LIPIDS
 CHOLESTEROL
 The client should be on NPO for 10 to 12 hours
 Normal range: 150 – 250 mg/dl
 TRIGLYCERIDES
 The client should observe fasting for 10 – 12 hours
 Normal range: 140 – 220 mg/dl
 BLOOD CULTURES
 To assist in the diagnosis if infectious diseases of the heart
 Caution is taken to prevent contamination of the specimen

E. ENZYME STUDIES
 ASPARTATE AMINOTRANSFERASE (AST)
 Formerly SGOT
 Elevated level indicates tissue necrosis
 Normal range is 7 – 40 mu/ml
 Range with Myocardial Infarction
o Initial elevation: 4 – 6 hours
o Peaks: 24 – 36 hours
o Returns to normal: 4 – 7 days
 CREATINE PHOSPHOKINASE (CPK)
 Is the most cardiac specific enzymes
 Is an accurate indicator of myocardial damage
 Normal range:
- Males : 50 – 325 mu/ml
- Females: 50 – 250 mu/ml
 Range with Myocardial Infarction
- Onset: 3 – 6 hours
- Peaks: 12 – 18 hours
- Returns to normal: 3 – 4 days
 LACTATE DEHYDROGENASE (DLH)
 Among the five isoenzymes
 Is the most sensitive indicator of myocardial damage
 In MI, LDH1 is elevated and its level exceeds LDH2 making LDH1 /LDH2
ratio “flipped”
 Range with Myocardial Infarction
- Onset: 12 hours
- Peaks: 48 hours
- Returns to normal: 10 – 14 days
 HYDROXYBUTYRATE DEHYDROGENASE (HBD)
 Its elevation always accompany elevation of LDH1
 Is valuable in detecting “Silent MI” because it remains elevated for a long
period of time, even after the other enzymes have returned to normal
 The HBD/LDH ratio may be increased in MI
 Range with Myocardial Infarction
- Onset: 10 – 12 hours
- Peaks: 48 – 72 hours
- Returns to normal: 12 – 13 days
 F. URINALYSIS
 Is usually performed to assess the effects of cardiovascular disease on renal
function and the existence of concurrent renal or systemic diseases
 Albuminuria is detected in clients with malignant hypertension & CHF
 Myoglobinuria supports diagnosis of MI
G. BLOOD URIC ACID
 Reflects adequacy of renal tissue perfusion thereby glomerular filtration of
metabolites.
 CVD result to decreased renal tissue perfusion
 This will cause impairment of the ability of the kidneys to clear the plasma of
end products of metabolism like uric acid
 Normal range: 2.5 to 8 mg /dl
H. SEROLOGIC TESTS
 VDRL helps indicate presence of syphilis
 This involves development of aortic disorders
I. SERUM ELECTROLYTES
 Electrolytes affect cardiac contractility, specially Na, K, Ca
 Normal range:
- Na - 135 – 145 mEq/L
- K - 3.5 – 5 mEq / L
- Ca - 4.5 – 5.5 mEq /L
B. ELECTROCARDIOGRAPHY (ECG, EKG)
 Is the graphical recording of the
electrical activities of the heart
 Indicates alterations in myocardial
oxygenation
 Is the first test done when CVD is
suspected
WAVES, COMPLEXES AND INTERVALS
 P wave
 Depolarization of atria
 Duration is 0.04 to 0.11 seconds
 PR interval
 Time of impulse transmission from SA node to the AV node
 Duration is 0.12 to 0.20 seconds
 QRS complex
 Depolarization of the ventricles
 Duration is 0.05 to 0.10
 ST segment
 Represents the plateau phase of action potential
 T wave
 Ventricular repolarization
 Should not exceed 5 mm amplitude

NURSING RESPONSIBILITIES
 Inform the client that the procedure is painless and that he will not
experience electrocution or a shock

COMMON ECG CHANGES

  Hypokalemia:
wave
 Hyperkalemia:
 Myocardial Infarction:
C. HOLSTER MONITORING
 It is continuous ECG monitoring
U wave, Depressed ST segment, Short T
Prolonged QRS complex, Elevated ST segment,
Peaked T wave
Elevated ST segment (first to occur in MI), Inverted
T wave, Pathologic Q wave

 The portable monitoring system is called telemetry

atrium.
 This attempts to assess the activities which
precipitate dysrhythmias, and the time of the day
when the client experiences dysrthymias
 The nurse should log / record the activities of the
client, and any unusual sensations experienced.

D. INVASIVE HEMODYNAMIC MONITORING

CENTRAL VENOUS PRESSURE

 Monitor the pressures within the right atrium
 Monitors blood volume, adequacy of venous return to the heart, pump function of
the right side of the heart.

 The 0 level of the manometer is placed at the right, mid – axillary, 4th ICS, the
approximate level of the right atrium when in supine position.
 Place the client in supine
position or in the same
position as during the initial
reading.

 Practice strict asepsis.

Cleanse catheter insertion
site and change sterile
dressings daily
 Normal readings:

 Superior Vena Cava:

0 – 12 cm H2O

 Right atrium: 5 – 12 cm H2O

 Use other parameters to validate CVP reading – BP, urine output,

pulse

2. PULMONARY ARTERY PRESSURE (PAP) &

PULMONARY CAPILLARY WEDGE PRESSURE (PCWP)

 Swan – Ganz Catheter is inserted via antecubital vein into the right side of the
heart and is floated into the pulmonary artery.
 It reflects pressure in the left heart

 Swan – Ganz Catheter is a flow – directed, balloon – tipped, 4 lumen catheter.

 The catheter allows continuous monitoring of the following:

  Right and left ventricular function
 Pulmonary artery pressures (PAP & PCWP)
 Cardiac output
 Arterial – venous oxygen difference
 Normal range:
 PAP: 4 – 12 mmHg
 PCWP: 4 – 12 mmHg
 PCWP reading above 25 mmHg suggests impending
pulmonary edema

NURSING RESPONSIBILITIES
 Inflate balloon only for PCWP readings, deflate between
readings
 Observe catheter insertion site, culture site every 48 hours
 Assess extremity for color, temperature, capillary filling and
sensation

E. SONIC STUDIES
1. ECHOCARDIOGRAPHY
 Uses ultrasound to assess cardiac structure and mobility
 No special preparation is required
 It is painless and takes approximately 30 – 60 minutes to complete
 The client has to remain still, in supine position slightly turned to the left side, and
with HOB elevated 15 – 20 degrees.

2. TRANSESOPHAGEAL ECHOCARDIOGRAPHY (TEE)

 Allows ultrasonic imaging of the cardiac structures and great
vessels via esophagus
NURSING RESPONSIBILITIES BEFORE TEE
1. Ascertain history of esophageal surgery, malignancy, or allergy to
anesthetics or sedatives
2. NPO for 4 – 6 hours before the procedure
3. Encourage to void before the procedure
4. Remove dentures and other oral prosthetics
5. Administer sedative as ordered
6. Keep suction and resuscitation equipment readily available
7. Cardiac monitoring is done during the entire procedure
8. Topical spray anesthetic is administered to depress gag reflex
9. Place the patient in chin – to – chest position to facilitate passage of
endoscope

NURSING RESPONSIBILITIES AFTER TEE

1. NPO until gag reflex returns
2. Place in lateral or Semi – Fowler’s position
3. Encourage to cough
4. Throat lozenges or rinses may be used to relieve throat soreness
5. Observe for signs and symptoms of complication ( Pharyngeal bleeding,
cardiac dysrhythmias, vasovagal reaction & transient hypoxemia)
F. STRESS TESTING OR EXERCISE TESTING
 ECG is monitored during exercise
on a treadmill or a bicycle – like
device.
 The purpose of stress test are as
follows:
• Identify
ischemic heart disease
• Evaluate
patients with chest pain
• Evaluate
effectiveness of therapy
• Develop
individual fitness program

Nursing Interventions: Treadmill Test

 Get adequate sleep the night before the test
 Avoid tea, coffee and alcohol on the day of the test
 Avoid smoking and taking nitroglycerine, 2 hours before
the test
 Wear comfortable, loose – fitting clothes
 Eat a light breakfast / lunch at least 2 hours before the
test
 Wear low – heeled, rubber – soled pair of shoes
 Inform the physician if any unusual sensations develop
during the test
 Rest after the test

G. RADIOLOGIC TESTS

1. CHEST ROENTGENOGRAMS (X – RAYS)

 To determine overall size and configuration of the heart and size of the
cardiac chambers
2. CARDIAC FLUOROSCOPY
 Facilitates observation of the heart from varying views while it is in
motion
3. CARDIAC CATHETERIZATION
 The purpose of the test are as
follows:
 Assess: oxygen levels, pulmonary
blood flow, cardiac output, heart
structures
 Coronary artery visualization
 Right – sided catheterization is
done by insertion of a catheter via a
cutdown into a large vein, eg. Medial
antecubital or brachial vein
 Left – sided catheterization is done
by passing a catheter into the aorta via the
brachial or femoral artery

NURSING INTERVENTIONS: CARDIAC CATHETERIZATION

  BEFORE THE PROCEDURE:
 Provide psychosocial support
 Assess for allergy to iodine / seafoods
 Obtain baseline VS
 Withhold meals before the procedure
 Have the client void
 Administer sedatives as ordered
 Mark distal pulses
 Do cardiac monitoring
 Done under local anesthesia
 May experience warm or flushing sensation as the contrast medium is
injected
 “Fluttering” sensation is felt, as the catheter enters the chambers of the
heart

 AFTER THE PROCEDURE

 Bed rest: If the catheter insertion site is an upper extremity, until VS
are stable, while if it is a lower extremity, for 24 hours
 Monitor VS, especially peripheral pulses
 Monitor ECG, note for dysrhythmias
 Apply pressure dressing and a small sand bag or ice over the puncture
site to prevent bleeding
 Immobilize affected extremity in extension to promote adequate
circulation
 Do not elevate HOB more than 30 degrees if femoral site was used
 Monitor extremities for color, temperature and tingling

4. ANGIOGRAPHY
 Involves introduction of contrast medium into the vascular system to outline the
heart and blood vessels
 It may be done during cardiac catheterization
 Nursing interventions are similar to that of cardiac catheterization
 Observe for hypotension after the procedure because the contrast medium may
cause profound diuretic effect

5. MAGNETIC RESONANCE IMAGING (MRI)

 Strong magnetic field and radiowaves are used to detect and define differences
between healthy and diseased tissues
 It can actually show the heart beating and blood flowing in any direction
 It can image over three spatial dimension and over time
 It is used for the examination of the aorta, detection of tumors, cardiomyopathies
and pericardial disease
NURSING INTERVENTIONS: MRI
 Secure written consent
 Inform the client that the procedure lasts 45 to 60 minutes
 Assess for claustrophobia.
 Remove all metal items
 Instruct the patient to remain still during the procedure
 Inform the client that MRI unit makes a loud, knocking noise
  Caution: client pacemakers, prosthetic valves or recently implanted clips or wires
are not eligible for MRI scans
6. MYOCARDIAL SCINTIGRAPHY
 It involves intravenous injection of a radioisotope via a catheter
 Myocardial function, motion and perfusion are studied through the use of an
external gamma camera
 Techniques used are as follows:
 Thallium 201 scintigraphy
 Dipyridamole – thallium – 201 test
 Technetium 99m ventriculography
 First pass cardiac study
NURSING INTERVENTIONS: MYOCARDIAL SCINTIGRAPHY
 Inform the client that ECG or treadmill test may be done during the procedure
 Assess for pregnancy because the test involves radiation exposure
 Instruct the client to take a light meal, to prevent nausea and stomach cramping
during exercise and for better uptake of radioisotope
 Omit the usual dose of prescribes beta – blockers, calcium – channel blockers and
xanthines before the procedure
 Instruct the client to report any chest pain experienced during the procedure
H. NON – INVASIVE HEMODYNAMIC MONITORING: INTRAARTERIAL PRESSURE
MONITORING
 This provides continuous detection of arterial BP via an indwelling intra – arterial
catheter
 It is valuable in monitoring the BP of clients with low cardiac output, fluctuating
hemodynamic status and excessive peripheral vasoconstriction and in whom BP
cuff measurements are undetectable
 Intra – arterial readings are at least 10 mmHg higher than cuff BP readings
 The intra – arterial BP line can be used for obtaining blood samples for ABG and
blood studies
 Heparinize the catheter to maintain patency
 Check catheter insertion site for hemorrhage, hematoma, redness or signs of
infection
 Do neurovascular check distal to catheter insertion site – color, temperature,
capillary refill and sensation

NURSING GOALS IN THE TREATMENT OF ANGINA PECTORIS:

a. Increasing Oxygen to the myocardium and relief of acute attacks.
MANAGEMENT:
A. Antiplatelet aggregation therapy – this is the first line of pharmacological intervention in the treatment of
angina pectoris. Aspirin is the drug of choice. Dipyridamole (persantine)
B. Nitrates – Maybe:
Rapid Acting Nitrates
a. Nitroglycerine is the drug of choice and acts to relieve the pain in about 3 minutes and has a
duration of approximately 45 minutes by producing dilation of coronary blood vessels. Usual dose is .
3mg (1/200gr); takes sublingually; retain saliva before swallowing.
b. Nitrostat – a stabilized form of nitroglycerine. Has uniform potency and can be carried without
fear of decomposition.
 c. Amyl Nitrate – given in form of pearls or ampules which are crushed and inhaled.
Long-Acting Nitrites:
- act to maintain coronary artery vasodilatation, thereby promoting a
greater flow of blood and oxygen to heart muscle.
 Nitroglycerine ointment / Nitrol IV; Tridil
C. Beta-adrenergic blocking agents – acts to decrease O2 requirement by decreasing heart rate and
redistributing blood flow to non-ischemic portion of the heart.
D. Calcium-blocking agents
- Systemic vasodilation with decrease systemic vascular resistance
(SVR); decrease myocardial contractility; coronary vasodilatation.
- potentiates the action of digoxin by increasing serum digoxin levels
during early part (first week) of therapy. Therefore serum digoxin levels should be closely
monitored upon institution of this therapy and the client should be taught the sign and symptoms
of digoxin toxicity.
- Example of drugs are:
Nifedipine (procardia)
Verapamil (Calan, Isoptin)
Diltiazem (Cardizem)
Nicardipine (Cardene)
E. Whisky or brandy (30 – 60 ml) acts to promote the dilatation of blood vessels and general relaxation. It
also help to synthesize lipids in the blood.
• Reducing the demand for oxygen
• Helping client prevent future episodes of angina
F. Common Drugs used for the Reduction of Sodium absorption and fluid retention:
a. Diuretics – suppress the reabsorption of salt and water by the kidneys; may lead to
potassium depletion because it also blocks the reabsorption of Chloride, Na and K ion in
the proximal tubules.
b. Examples:
i. Thiazides – Chlorothiazide (Diuril); Chlorthalidone (Hygroton
ii. Loop diuretics –very potent – Furosemide (lasix); Ethacrynic acid (Edecrin);
Bumetanide (Bumex)
iii. Potassium-sparing – spironolactone (aldactone); Trianterone (Dyrenium);
iv. Combination agents – Aldactazide (spironolactone and hydrochlorothiazide)
G. Drugs commonly used to reduced BP or BP reducing pharmacological agents:
c. Sympathetic depressants – Reserpine (serpasil)
d. Selective sympathetic depressants – Guanethidine (Ismelin); Methyldopa
(Aldomet); Hydralazine hydrochloride (Apresoline)
e. Amine-oxidase inhibitors – Eutonyl
f. Beta-adrenergic blocking agents – Propanolol (Inderal)
g. Anti-renin – Clonidine (catapres); Suggest chew gum or hard candy to relieve dry
mouth.
h. Angiotensin – converting Enzyme inhibitors (ACE inhibitors) – Captopril (Capoten)
use aspirin can decrease drugs effectiveness.
i. Calcium antagonists – Nifedipine (Procardia)
Precautions in taking these drugs:
i. Decrease BP cause dizziness, drowsiness, light headedness, lethargy and orthostatic
hypotension;
ii. Avoid long periods of standing – blood may tend to pool in the peripheral vessel.
iii. Rest reduces vasodilation, which combined, with antihypertensive drug may cause the
client to faint.
iv. Avoid hot baths or being overheated during summer.
 v. Certain foods increases BP – chocolate, chicken liver. Beer, wine, strong or aged cheese
vi. Nasal decongestants generally have vasoconstrictor actions that tend to increase BP.
vii. Avoid cheese, beer or wine when taking butonyl – a severe reaction with possibility of
several hemorrhage.
Indigenous Preparation for Heart problems or Hypertension:
1. Garlic – can help to reduce blood pressure.
2. Banaba decoction can also be use as diuretics.

Hematologic System:
 The hematologic system consists of the blood and the sites where blood is produced, including
the bone marrow and the reticuloendothelial system (RES).

 Blood is a specialized organ that differs from other organs in that it exists in a fluid state. Blood is
composed of plasma and various types of cells.

 Plasma is a fluid portion of blood; it contains various proteins, such as albumin, globulin,
fibrinogen, and other factors necessary for the clotting as well as electrolytes, waste products
and nutrients.

 About 55% of blood volume is plasma.

 45% of which is the cellular components of blood which are the Leukocytes (white blood cells,
WBC), Erythrocytes (red blood cells, RBCs), and thrombocytes (platelets).

 Hematopoiesis is a continuous process of replenishing the dead blood cells, since most of the
blood cells have short life span.

 The primary site for hematopoiesis is the bone marrow. The liver and spleen may also be
involved.

 Under normal conditions, the adult bone marrow produces about 175 billion RBCs, 70 million
neotrophils (mature form of white cells), and 175 million thrombocytes (platelets) each day.

 The volume of blood is 7% – 10% of the normal body weight and amounts to 5 to 6 liters.

 Hemostasis is the balance between two systems which is the clot formation and clot dissolution
or fibrinolysis.

 The bone marrow, is the site of hematopoiesis or blood cell formation. In a child, all skeletal
bones are involved, but as he person ages, marrow activity decreases. By adulthood, marrow
activity is usually limited to the pelvis, ribs, and sternum.

 Marrow is one of the largest organs of the body, making up 4% to 5% of total body weight. It
consists of islands of cellular components (red marrow) separated by fat (yellow marrow).

 In cases of adult disease causing marrow destruction, fibrosis, or scarring, - the liver and spleen
can also resume production of blood cells by a process as extramedullary hematopoiesis.

 The marrow is highly vascular. Within it are primitive cells called stem cells.

 These stem cells have the ability to self-replicate, thereby ensuring a continuous supply of stem
cells throughout the life cycle.

 When stimulated to do so, stem cells can begin a process of differentiation into either myeloid or
lymphoid stem cells.

 These stem cells are committed to produce specific types of blood cells.

 Lymphoid stem cells produce either T or B Lymphocytes.

  Myeloid stem cells differentiate into three broad cell types: erythrocytes, leukocytes, and
platelets.

 Thus, with the exception of lymphocytes, all blood cells are derived from the myeloid stem cell,
and thus it is easy to appreciate why a defect in the myeloid stem cell can cause problems not
only with white cell production, but also with red cell and platelet production.

 Differentiation of the primitive myeloid stem cell of the marrow into an erythroblast is stimulated
erythropoietin, a hormone produced primarily by the kidney. If the kidney detects low levels of
oxygen, the release of erythropoietin is increased

 The increased erythropoietin then stimulates the marrow to increased production of RBCs. The
entire process typically takes 5 days.

 The normal erythrocyte production, the bone marrow also requires iron, vitamin B12, folic acid,
pyridoxine (vitamin B6), and other factors. A deficiency of these factors during erythropoiesis can
result in decreased RBC production and thus anemia.

 Normally 0.5 to 1 mg of ingested iron is absorbed from the small intestine.

 The rate of iron absorption is regulated by the amount of iron already stored in the body and by
the rate of red cell production.

 Additional amounts of iron, up to 2mg daily, must be absorbed by the adult female to replace
blood lost during menstruation.

 Total body iron content in the average adult is approximately 3mg, most of which is present in
hemoglobin or in one of its breakdown products.

 Iron is stored in the small intestine as ferritin and in reticuloendothelial cells.

 When required, the iron is released into the plasma, binds to transferrin, and is transported into
the membranes of the normoblasts (erythrocyte precursor) within the marrow, where it is
incorporated into the hemoglobin. All these process takes only 6 to 8 minutes.

 Iron is lost in the feces, either in bile, blood or mucosal cells from the intestine.

 Normally concentration of iron in blood is about 75 to 175µg/dL (13 – 31 µmol/L) for men and 65
to 165µg/dL (11 - 29µmol/L) for women.

 With iron deficiency, bone marrow iron stores are rapidly depleted; thus, hemoglobin synthesis is
depressed, and the red cells produced by the marrow are small and low in hemoglobin.

 Vitamin B12 and folic acid are required for synthesis of deoxyribonucleic acid (DNA) in many
tissues, but deficiencies of either of these vitamins have the greatest effect on erythropoiesis.

 Both B12 and folic acid are derived from the diet.

 Folic acid is absorbed in the proximal small intestine, but only small amounts are stored within the
body

 Vitamin B12 found only in foods of animal origin, strict vegetarians may ingest little B12. B12
combines with intrinsic factor complex is absorbed in the stomach. The vitamin B12 – intrinsic
factor complex is absorbed in the distal ileum

PLANNING / IMPLEMENTATION FOR HEALTH RESTORATION & MAINTENANCE for CLIENTS WITH
CARDIOVASCULAR PROBLEMS
 A. Neonate
1. CONGENITAL HEART DEFECTS
i. are structural or functional anomalies in the heart that occur during fetal development
and are present at birth.
PATHOPHYSIOLOGY

Left – to – Right Shunts

 Atrial Septal Defects (ASD)
 Ventricular Septal Defects (VSD)
 Patent Ductus Arteriosus (PDA)
 Atrioventricular Canal Defect
 are considered to be acyanotic heart defects, some mixing of oxygenated blood with non –
oxygenated blood occurs due to blood being shunted from areas of higher pressure to
areas of lower pressure.
 In ASD, blood is shunted from the left to the right atria, which increases the amount of
blood in the right atria. This produces pressure in the systemic system that feeds the right
atria and decreased venous return and systemic congestion.
 VSD, blood is shunted from the left to the right ventricle, which increases the amount of
blood in the right ventricle and increases the workload of the right ventricle. This eventually
results in the right ventricular hypertrophy and heart failure.
 PDA, oxygenated blood is shunted from the higher pressure aorta to the lower pressure
pulmonary artery carrying non- oxygenated blood to the lungs to be oxygenated.
 This increases blood flow into the pulmonary system causes pulmonary congestion
and creating back pressure in the right ventricle as it attempts to continue to pump
blood into the pulmonary artery.
 Back pressure in the right ventricle results in increased workload and right ventricular
hypertrophy.

Right – to – Left Shunts

 Tetralogy of Fallot
 Transposition of great arteries
 Truncus arteriosus
 Pulmonary atresia
 Tricuspid atresia
 Hypoplastic Left Heart Syndrome
 Are generally considered cyanotic heart defects and involve the pumping of non –
oxygenated blood into the systemic system.
 TOF, the presence of an overriding aorta over a VSD and pulmonary stenosis allow non-
oxygenated blood to be shunted into the aorta.
 This occurs because pulmonary stenosis creates higher pressure due to the
decreased vessel lumen and the aorta becomes the vessel of lesser pressure.
 Consequently, non – oxygenated blood is pumped out of the aorta into the systemic
circulation.
 Truncus Arteriosus, a single large vessel takes the place of the aorta, pulmonary and
coronary circulation.
 Because the aorta and the pulmonary artery are the same vessel and a VSD is
commonly present, the blood enters both ventricles, is mixed in the one common
vessel, and desaturation occurs.
 This blood is pumped into the systemic circulation.
 Pulmonary Atresia, the pulmonary valve is absent.
 There is no blood flow from the right ventricle into the pulmonary artery.
 The blood entering the right atria is unable to enter the ventricle and travels into the
left atria via a patent foramen ovale.
 This non- oxygenated blood is then pumped into the left ventricle and out into the
systemic circulation via the aorta.
 Tricuspid atresia, the tricuspid valve does not develop, which causes a lack of
communication between the right atria and the right ventricle.
 The pressure forces the development of ASD or patent foramen ovale to the left atria
through the mitral valve into the left ventricle.
 This blood is then shunted into the right ventricle via a VSD and into pulmonary
circulation.
 The mixing of oxygenated and non – oxygenated blood causes desaturation and
varying amounts of pulmonary congestion due to pressure back up.
 Hypoplastic Left Heart Syndrome, there is essentially no left ventricle.
 The small to absent left ventricle and co – existing valvular stenosis of the aortic and
mitral valves creates inadequate systemic perfusion.
  Most blood in the left atrium flows across the patent foramen ovale, into the right
atrium, the right ventricle, and the pulmonary artery, and then still non- oxygenated, it
is shunted through the PDA into the systemic circulation.

STENOTIC LESIONS
 Pulmonary Stenosis
 Aortic Stenosis
 Coartation of the Aorta
 There is decreased blood flow through the major vessels existing the heart – the pulmonary
artery or the aorta. The chamber from which the stenotic vessel exits determines whether
right or left ventricular hypertrophy occurs.
 Pulmonary Stenosis
 there is obstruction of blood flow into the pulmonary artery due to the thickening and
fusing of the leaflets of the pulmonary artery.
 This causes increased pressure into the right ventricle as its workload increases to
try to pump blood against the obstruction.
 This leads to right ventricular hypertrophy and back up into the systemic circulation
attempting to return blood to the right atrium.
 Aortic Stenosis and Coarctation of the Aorta
 obstruction is present in the aorta either into the aortic valve or in the aortic arch.
 This causes increased pressure in the left ventricle as it attempts to pump blood into
the systemic circulation against the obstruction.
 The resultant increased left – sided pressure causes back pressure in the pulmonary
circulation and pulmonary congestion.
COMPLICATIONS
 Congestive Heart Failure
 Failure to Thrive
 Decreased Cardiac Output
 Pulmonary Edema
 Hypoxia
 Complications associated with surgical repair of defects

CLINICAL MANIFESTATIONS
Left – to – Right Shunts
 Murmur
 Dyspnea on exertion
 Growth Retardation
 Failure to Thrive
 Diaphoresis
 Right Ventricular Hypertrophy
 Right sided CHF

Right – to – Left Shunts

 Murmur
 Cyanosis
 Failure to Thrive
 Left Ventricular Hypertrophy
 Left sided CHF
 Pulmonary Edema
 Tachypnea

Stenotic Lesions
 Murmur
 Dyspnea in exertion
 Right or left sided heart failure and consequent systemic or pulmonary signs and symptoms.

Diagnostic Tests
 Cardiac Auscultation
 Blood Pressure
 Chest X- ray
 ECG
 Echocardiogram
 Cardiac Catheterization
 MRI

Laboratory (Preoperative)
 CBC
 ABG
  Electrolyte levels
 Coagulation Studies
 Cardiac Enzymes
 Urinalysis

Therapeutic Management
Medical Management
 Digoxin
 Furosemide
 Propanolol
 Enalapril
 Captopril

Surgical Management
 Open Chest Surgery and Cardiopulmonary Bypass

Nursing Intervention
 Promote activity tolerance by minimizing effects of CHF.
 Provide information to parents.
 Promote parental relaxation and reduce anxiety.
 Promote family processes.
 Promote normal growth and development.
 Provide for adequate tissue perfusion.
 Maintain adequate gas exchange.
 Maintain adequate cardiac output.
 Restore fluid balance and prevent future overload.
 Assist in return of body temperature to WNL.
 Prevent cardiac dysrhythmias.
 Promote adequate gas exchange and patent airway.
 Prevent infection.
 Promote effective breathing pattern and maintain pain control.
 Prevent thrombus formation.
 Promote rest and sleep.
 Promote health maintenance.
Discharge Teaching
 Provide instructions on importance of regular follow – up visits to screen for residual
cardiovascular complications.
 Provide instructions on medication administration including dosages, schedule of
administration and adverse effects to report to physician.
 Provide information on normal growth and development and importance of normalcy for child.
 Provide written instructions for home care after cardiac surgery.

ACUTE LYMPHOCYTIC LEUKEMIA

 proliferation of immature WBC’s
 ALL is the most common pediatric cancer; it also strikes adults of all ages. Malignant transformation and
uncontrolled proliferation of an abnormally differentiated, long-lived hematopoietic progenitor cell results in a
high circulating number of blasts, replacement of normal marrow by malignant cells, and the potential for
leukemic infiltration of the CNS and abdominal organs. Symptoms include fatigue, pallor, infection, and easy
bruising and bleeding. Examination of peripheral smear and bone marrow is usually diagnostic. Treatment
typically includes combination chemotherapy to achieve remission, intrathecal chemotherapy for CNS
prophylaxis and/or cerebral irradiation for intracerebral leukemic infiltration, consolidation chemotherapy with
or without stem cell transplantation, and maintenance chemotherapy for 1 to 3 yr to avoid relapse.
 Two thirds of all ALL cases occur in children, with a peak incidence at age 2 to 5 yr; ALL is the most common
cancer in children and the 2nd most common cause of death in children < age 15. A 2nd rise in incidence
occurs after age 45.
CM:
Signs and symptoms related to anemia like easy fatigability.
weight loss, fever, frequent infections
weakness, fatigability
abnormal bruising and lymphadenopathy
bone and joint pain, headache, splenomegaly, Hepatomegaly, neurologic dysfunction
Lab. Data:
Increased WBC count: Hyperleukocytosis
 hemoglobin, hematocrit
 platelet
Abnormal PT and PTT
BMA
Lumbar puncture
Prognosis
Prognostic factors help determine treatment protocol and intensity.

Favorable prognostic factors are

• Age 3 to 7 yr

• WBC count < 25,000/μL

• French-American-British (FAB) L1 morphology

• Leukemic cell karyotype with > 50 chromosomes and t(12;21)

• No CNS disease at diagnosis

Unfavorable factors are

• A leukemic cell karyotype with chromosomes that are normal in number but abnormal in morphology (pseudodiploid)

• Presence of the Philadelphia (Ph) chromosome t(9;22)

• Increased age in adults

• B-cell immunophenotype with surface or cytoplasmic immunoglobulin

Treatment
• Chemotherapy

• Sometimes stem cell transplantation or radiation therapy

The 4 general phases of chemotherapy for ALL include

• Remission induction

• CNS prophylaxis

• Postremission consolidation or intensification

• Maintenance

Treatment
• Chemotherapy

• Sometimes stem cell transplantation or radiation therapy

The 4 general phases of chemotherapy for ALL include

• Remission induction

• CNS prophylaxis

• Postremission consolidation or intensification

• Maintenance

Induction therapy:

The goal is to induce remission. Several regimens emphasize early introduction of an intensive multidrug regimen. Remission
can be induced with daily oral prednisone (deltasone)
and weekly IV vincristine (Oncovin) with the addition of an anthracycline or asparaginase (Espar)
. Other drugs and combinations that may be introduced early in treatment are cytarabine(Cytosar)
and etoposide (Etopophos)
as well as cyclophosphamide (Cytoxan)

In some regimens, intermediate-dose or high-dose IV methotrexate (Rheumatrix) is given with leucovorin rescue. The
combinations and their dosages are modified according to the presence of risk factors. Imatinib (Gleevec)
can be added to the drug regimen in patients with Ph chromosome–positive ALL.
CNS prophylaxis: An important site of leukemic infiltration is the meninges; prophylaxis and treatment may include high-dose
intrathecal methotrexate (Rheumatrix), cytosine arabinoside, and corticosteroids. Cranial nerve or whole-brain irradiation may be
necessary and is often used for patients at high risk of CNS disease (eg, high WBC count, high serum LDH, B-cell phenotype)
but has been used less often in recent years.

Consolidation therapy: The goal of consolidation is to prevent leukemic regrowth. Consolidation therapy usually lasts a few
months and combines drugs that have different mechanisms of action than drugs used in induction regimens. Allogeneic stem
cell transplantation is recommended as consolidation of Ph chromosome–positive ALL or in 2nd or later relapses or remissions.

Maintenance therapy: Most regimens include maintenance therapy with methotrexate (Rheumatrix)
and mercaptopurine (Purinethol). Therapy duration is usually 2½ to 3 yr but may be shorter with regimens that are more intensive
in earlier phases and for B-cell (L3) cases. For patients in continuous complete remission for 2½ yr, the risk of relapse after
therapy cessation is about 20%, usually within 1 yr. Thus, when therapy can be stopped, most patients are cured.

Relapse: Leukemic cells may reappear in the bone marrow, the CNS, or the testes. Bone marrow relapse is particularly
ominous. Although a new round of chemotherapy may induce a 2nd remission in 80 to 90% of children (30 to 40% of adults),
subsequent remissions tend to be brief. Only a few patients with late bone marrow relapses achieve long disease-free 2nd
remissions or cure.

If an HLA-matched sibling is available, stem cell transplantation offers the greatest hope of long-term remission or cure (see
Transplantation: Hematopoietic Stem Cell Transplantation). Cells from other relatives or matched, unrelated donors are
sometimes used. Transplantation is rarely used for patients > 65 yr, because it is much less likely to be successful and adverse
effects are much more likely to be fatal.

When relapse involves the CNS, treatment includes intrathecal methotrexate (with or without cytarabine or corticosteroids) twice
weekly until all signs disappear. Most regimens include systemic reinduction chemotherapy because of the likelihood of systemic
spread of blast cells. The role of continued intrathecal drug use or CNS irradiation is unclear.

Testicular relapse may be evidenced clinically by painless firm swelling of the testis or may be identified on biopsy. If unilateral
testicular involvement is clinically evident, the apparently uninvolved testis should undergo biopsy. Treatment is by irradiation of
the involved testis and administration of systemic reinduction therapy as for isolated CNS relapse.

Nsg. Dx: Risk for infection

Interventions:
 Monitor VS every 4 hours
 Monitor for fever and signs of infections
 Assess respiratory functions
 Assess for changes in mental status
 Monitor platelet count on a regular basis
 Monitor urine, stool and emesis for and occult blood
 Use meticulous handwashing
 Instruct the patient to avoid crowded places.
 Avoid venipunctures.
 Watch for signs of bleeding and infection.
 Allopurinol is given to the child undergoing Chemotherapy to decrease uric acid production.

YOUNG
1. HODGKIN’S DISEASE
• a malignant condition characterized by proliferation of abnormal giant,
multinucleated cells, called Reed-Sternberg cells, which are located in lymph
nodes.
• Although the cause of Hodgkin’s lymphoma remains unknown, the main
interacting factors include infection with Epstein-Barr virus, genetic
predisposition, and exposure to occupational toxins.
• The incidence of Hodgkin’s lymphoma is increased in incidence among
human immunodeficiency virus infected patients.
 • The nursing care for Hodgkin’s lymphoma is largely based on managing
problems related to the disease (e.g., pain due to tumor), pancytopenia, and
other side effects of therapy.
• Malignancy in the lymphoid system.

IM: Painless enlargement of lymph nodes.

S/sx:
 painless swelling of lymph nodes
 persistent fever
 night sweats
 weight loss
 fatigue/malaise
 edema of face & neck
 jaundice

Dx:
 Lymph node biopsy
 CXR
 CT scan
 Lymphangiography
Tx:
 Chemo/radiation therapy
 Bone marrow transplant
 Blood sternal transfusions
 Immunotherapy

Lab. Data: Lymph node biopsy reveals REED – STERNBERG cells.

Nsg. Dx:
 Potential for infection

Interventions:
 Instruct the patient to report any signs of infection.
 Prepare the patient for chemotherapy and radiation therapy.
 Relaxation techniques
 Administer meds as ordered
 Provide emotional support.
 Advise patient taking Prednisone against sudden withdrawal of drug & not to change dosage or
discontinue drug without consent of physician.
 Good nutrition
 Use soft toothbrush
 Avoid crowds & any person with known infection

2. INFECTIOUS MONONUCLEOSIS (KISSING’S DISEASE)

 acute infectious disease of the lymphatic system caused by the Epstein barr virus
Incubation Period: 4-7 wks
MOT: contact with saliva
CM: Sore throat
S/sx:
 Periorbital edema
 headache
 sore throat
 cervical lympadenopathy
 petechiae
 splenomegaly
Lab. Data: Heterophil antibody agglutination test reveals increase in Titer
Epstein - Barr virus specific anti-body test
Nsg. Dx: Pain
Interventions:
 Treatment is symptomatic and supportive.
 Avoid heavy lifting, strenuous exercise and contact sports until recovery is complete.
 Observe for left upper quadrant abdominal pain radiating to the left scapula, this
indicates splenic rupture.
 The disease is transmitted by saliva and through intimate physical contact like kissing
or sharing of utensils.
 Medicines:
 Analgesic
 Corticosteroid

3. KAWASAKI’S DISEASE
 o Multi – system vasculitis primarily affecting the cardiovascular system.
CM: High spiking fever, 5 or more days
Criteria for Dx:
 Fever lasting for more than 5 days
 Bilateral conjunctivitis
 Changes of lips & oral cavity
S/Sx
 dry, red fissure lips
 strawberry tongue
 diffuse erythema of mucous membrane
 Changes of peripheral extremities
 erythema of the palms & soles
 indurative edema of the hands & feet
 membranous desquamation from fingertips
 Polymorphous rash (primarily on trunk)
 Acute non-purulent swelling of cervical lymph node to > 1.5 cm in diameter
Lab. Data: Elevated ESR during the acute stage
Nsg. Dx: Pain
Interventions:
 Administer ASPIRIN as ordred
 Monitor VS
 Offer clear liquid diet like popsicles
 Teach the parents to perform CPR

4. PERICARDITIS
 is an inflammation of the pericardium.

CAUSES:
 bacterial, fungal, or viral infection
 Neoplasm
 Autoimmune disease
 Postcardiac injury
 Drugs such as procainamide or Hydralazine
S/Sx:
• pain in the anterior chest
• associated symptoms of dyspnea, fever, sweating and chills
• pericardial friction rub
Dx
• CRX
• Echocardiogram
• ECG
• WBC and differential count
• Tuberculin testing
• Pericardiocentesis
Mx:
• Monitor HR, rhythm, BP and RR
• Assess for cardiac tamponade
• Assess for CHF
• Institute continuous cardiac monitoring
• Encourage bed rest
• Help the patient to a position of comfort
• Provide relief to anxiety

ACUTE PERICARDITIS
 sharp, offensive, sudden pain usually starts over the sternum and radiates to the neck,
shoulder, back and arms.

CHRONIC PERICARDITIS
 gradual increase in systemic venous pressure and produces symptoms similar to those of
chronic right heart failure.
Dx:
 Normal or elevated WBC count
 elevated ESR
 slightly elevated cardiac enzyme levels
 culture of pericardial fluid obtained by open surgical drainage or cardiocentesis
 ECG – elevation of ST segment
Interventions:
 Provide complete CBR
 Assess pain in relation to respiration and body position.
 Place the patient in an upright position to relieve dyspnea and chest pain.
  Explain tests and treatments to the patient.
 Instruct on DBE and coughing exercises.

5. MYOCARDITIS - is focal or diffuse inflammation of the cardiac muscle.

a. is a focal or diffuse inflammation of the myocardium caused by viruses,
bacteria, fungi, radiation therapy, and pharmacologic and chemical factors.
b. is frequently associated with acute pericarditis, particularly when it is caused
by coxsackievirus B strains.
c. results in cardiac dysfunction and has been linked to the development of
dilated cardiomyopathy.
CAUSES:
 Viral infections – Coxsackievirus A & B strains
 Bacterial Infections
 Hypersensitive Immune Reactions
 Radiation Therapy
 Chemical poisons
 Parasitic Infections
 Helminthic Infections


Clinical manifestations:
 Fever, fatigue, malaise, myalgias, pharyngitis, dyspnea, lymphadenopathy, and
nausea and vomiting are early systemic manifestations of the viral illness.
 Early cardiac manifestations appear 7 to 10 days after viral infection and include
pleuritic chest pain with a pericardial friction rub and effusion.
 Late cardiac signs relate to the development of HF and may include an S3 heart
sound, crackles, jugular venous distention, syncope, peripheral edema, and angina.
 fatigue, dyspnea, palpitation, feveCollaborative care includes the following:
Managing associated cardiac decompensation with:
a. Digoxin (Lanoxin) to treat ventricular failure
b. Diuretics to reduce fluid volume and decrease preload
c. Nitroprusside (Nitropress), inamrinone (Inocor), and milrinone (Primacor) to
reduce afterload and improve cardiac output
d. The use of anticoagulation therapy may be considered in patients with a low
ejection fraction who are at risk for thrombus formation from blood stasis in the
cardiac chambers.
e. Immunosuppressive therapy to reduce myocardial inflammation and to prevent
irreversible myocardial damage.
f. Oxygen therapy, bed rest, and restricted activity.
g. Intraaortic balloon pump therapy and ventricular assist devices.
Dx:
 ECG typically shows diffuse ST segment and T wave abnormalities, conduction defects
(prolonged PR interval), and supraventricular arrhythmias.
 Stool and throat cultures, endomyocardial biopsy
 Cardiac enzyme levels, WBC count and ESR are increased.
 Antibody titers are elevated.
Tx:
 Antibiotics
 modified bed rest
 activity restriction
 sodium restriction
 Diuretics
 digitalis glycosides
Interventions:
 Nursing interventions focus on assessment for the signs and symptoms of HF and
include assessing the level of anxiety, instituting measures to decrease anxiety, and
keeping the patient and family informed about therapeutic measures.
 Most patients with myocarditis recover spontaneously, although some may develop
dilated cardiomyopathy. If severe HF occurs, the patient may require heart
transplantation.
 Observe for signs of digitalis toxicity (anorexia, nausea, vomiting, blurred vision, cardiac arrythmias)
 Stress the importance of bed rest.
 Resume normal activities gradually and avoid competitive sports.

6. ENDOCARDITIS
  Infection of the inner lining of the heart caused by direct invasion of bacteria leading to
deformity of the valve leaflets
 Endocarditis usually refers to infection of the endocardium (ie, infective endocarditis). The
term can also include noninfective endocarditis, in which sterile platelet and fibrin thrombi
form on cardiac valves and adjacent endocardium. Noninfective endocarditis sometimes
leads to infective endocarditis. Both can result in embolization and impaired cardiac
function.
 Endocarditis can occur at any age. Men are affected about twice as often. IV drug
abusers and immunocompromised patients are at highest risk.

Pathophysiology and Etiology

 The normal heart is relatively resistant to infection. Bacteria and fungi do not easily
adhere to the endocardial surface, and constant blood flow helps prevent them from
settling on endocardial structures. Thus, 2 factors are generally required for
endocarditis: a predisposing abnormality of the endocardium and microorganisms in
the bloodstream (bacteremia). Rarely, massive bacteremia or particularly virulent
microorganisms cause endocarditis on normal valves.
Consequences: Endocarditis has local and systemic consequences.

Local consequences:
• formation of myocardial abscesses with tissue destruction and sometimes
conduction system abnormalities
• Severe valvular regurgitation
• Aortitis
• Prosthetic valve infections
Systemic consequences (primarily due to embolization)
• septic pulmonary emboli
• Mycotic aneurysms
• Cutaneous and retinal emboli

Symptoms and Signs

• low-grade fever (< 39° C)
• night sweats
• fatigability
• malaise
• weight loss
• Chills and arthralgias
• Symptoms and signs of valvular insufficiency
• Retinal emboli
• Cutaneous manifestations (petechiae on the upper trunk, conjunctivae,
mucous membranes, and distal extremities)
• painful erythematous subcutaneous nodules on the tips of digits (Osler's
nodes)
• splinter hemorrhages under the nail
• splenomegaly
• Clubbing of fingers and toes.
Nsg. Dx: Altered Cardiac Output
Diagnosis
3 blood cultures
Echocardiography
TEE
no specific laboratory findings.
normocytic-normochromic anemia
elevated WBC count
increased ESR
increased Igs
circulating immune complexes, and rheumatoid factor
Prognosis
1. infective endocarditis is always fatal

2. prognosis is also poorer for people with aortic or multiple valve

involvement

Treatment
Treatment consists of a prolonged course of antimicrobial therapy.
Surgery may be needed for mechanical complications or resistant organisms.
Antibiotic regimens:

Ampicillin
Nafcillin
gentamicin
vancomycin
rifampin

Cardiac valve surgery:

1. Surgery (debridement, valve repair or replacement) is frequently required for

abscess, persistent infection despite antimicrobial therapy (ie, persistent positive
blood cultures or recurrent emboli), or severe valvular regurgitation.

Prevention
Antimicrobial prophylaxis is recommended for patients at high to moderate risk of infective
endocarditis before procedures associated with bacteremias and subsequent infective
endocarditis

Nursing Interventions:
 Record daily weight
 Evaluate JVD – this signifies the development of CHF.
 Instruct the patient to take antibiotics before dental procedures that can cuse
bleeding.
 Avoid sharing of needles.
 Teach the women in child bearing years the risks of using IUDs, or birth control
(source of infection).

a. Adult
1. ATHEROSCLEROSIS
 Atherosclerosis is patchy intimal plaques (atheromas) in medium-sized and large arteries
 The plaques contain lipids, inflammatory cells, smooth muscle cells, and connective tissue.
 Risk factors include dyslipidemia, diabetes, cigarette smoking, family history, sedentary
lifestyle, obesity, and hypertension.
  Atherosclerosis can affect all large and medium-sized arteries, including the coronary,
carotid, and cerebral arteries, the aorta, its branches, and major arteries of the extremities.
 It is the leading cause of morbidity and mortality in the US and in most developed countries.
 Atherosclerosis is rapidly increasing in prevalence in developing countries, and as people in
developed countries live longer, incidence will increase. By 2020, atherosclerosis is
expected to be the leading cause of death worldwide.
Pathophysiology
 The hallmark of atherosclerosis is the atherosclerotic plaque, which contains lipids
(intracellular and extracellular cholesterol and phospholipids), inflammatory cells (eg,
macrophages, T cells), smooth muscle cells, connective tissue (eg, collagen,
glycosaminoglycans, elastic fibers), thrombi, and Ca deposits.
 Endothelial injury is thought to have a primary role.
 Atherosclerosis preferentially affects certain areas of the arterial tree.
 Macrophages elaborate proinflammatory cytokines that recruit smooth muscle cell migration
from the media and that further attract and stimulate growth of macrophages.
 The result is a subendothelial fibrous plaque with a fibrous cap, made of intimal smooth
muscle cells surrounded by connective tissue and intracellular and extracellular lipids.
 Atherosclerotic plaques may be stable or unstable.
 The strength of the fibrous cap and its resistance to rupture depend on the relative balance
of collagen deposition and degradation. Plaque rupture involves secretion of
metalloproteinases, cathepsins, and collagenases by activated macrophages in the plaque.
These enzymes digest the fibrous cap, particularly at the edges, causing the cap to thin and
ultimately rupture. T cells in the plaque contribute by secreting cytokines. Cytokines inhibit
smooth muscle cells from synthesizing and depositing collagen, which normally reinforces
the plaque.
 Once the plaque ruptures, plaque contents are exposed to circulating blood, triggering
thrombosis; macrophages also stimulate thrombosis because they contain tissue factor,
which promotes thrombin generation in vivo. One of 5 outcomes may occur:
o The resultant thrombus may organize and be incorporated into the plaque, changing
the plaque's shape and causing its rapid growth.
o The thrombus may rapidly occlude the vascular lumen and precipitate an acute
ischemic event.
o The thrombus may embolize.
o The plaque may fill with blood, balloon out, and immediately occlude the artery.
o Plaque contents (rather than thrombus) may embolize, occluding vessels
downstream.

Risk Factors
Nonmodifiable
1. Age
2. Family history of premature atherosclerosis*
3. Male sex
 4. Modifiable, established
5. Certain dyslipidemias (high total or LDL level, low HDL level, increased total-
to-HDL cholesterol ratio)
6. Cigarette smoking
7. Diabetes mellitus
8. Hypertension
Modifiable
1. Alcohol intake (other than moderate)
2. Chlamydia pneumoniae infection
3. High CRP level
4. High level of small, dense LDL
5. High lipoprotein(a) level
6. Hyperhomocysteinemia
7. Hyperinsulinemia
8. Hypertriglyceridemia
9. 5-Lipoxygenase polymorphisms
10. Low intake of fruits and vegetables
11. Obesity or metabolic syndrome
12. Prothrombotic states (eg, hyperfibrinogenemia, high plasminogen activator
inhibitor level)
13. Psychosocial factors (eg, type A personality, depression, anxiety, work
characteristics, socioeconomic status)
14. Renal insufficiency
15. Sedentary lifestyle†
16. CRP = C-reactive protein, HDL = high density lipoprotein, LDL = low density
lipoprotein.
17. *Disease in a first-degree relative before age 55 for men and before age 65
for women.

Symptoms and Signs

Atherosclerosis is initially asymptomatic, often for decades.
Symptoms and signs develop when lesions impede blood flow.
Transient ischemic symptoms
stable exertional angina
transient ischemic attacks
intermittent claudication
Symptoms of unstable angina or infarction, ischemic stroke
Rest pain in the limbs
Chest pain
Dyspnea
Tachycardia
Palpitations
Diaphoresis
Sudden death
Diagnosis
Approach depends on the presence or absence of symptoms.

Treatment
1. Diet: Several changes are beneficial:

• Less saturated fat

• No trans fats
• More fruits and vegetables
• More fiber
• Moderate (if any) alcohol
2. Physical activity:
• Regular physical activity (eg, 30 to 45 min of walking, running, swimming, or
cycling 3 to 5 times/wk) reduces incidence of some risk factors (hypertension,
dyslipidemia, diabetes), CAD (eg, MI), and death attributable to
atherosclerosis in patients with and without previous ischemic events.
3. Antiplatelet drugs: Oral antiplatelet drugs are essential because most
complications result from plaque fissure or rupture with platelet activation and
thrombosis. The following are used:
• Aspirin
• Clopidogrel (Plavix)
• Ticlopidine (Ticlid) (no longer used due to its neutropenic effect)
4. Other drugs:
• ACE inhibitors
• angiotensin II receptor blockers
• Statins
• Thiazolidinediones

Tx:
 P – ercutaneous
 T – ransluminal
 C – oronary
 A –ngioplasty

Objective:
 To vascularize the myo cardium
 To prevent angina
 Increase survival rate
 C – oronary
 A – rterial
 B – ypass
 G – raft surgery

2. CORONARY HEART DISEASE

 loss of oxygen and nutrients to myocardial tissue because of diminished coronary blood
flow.
 Occurs more often in men than in women, in whites and in middle aged and elderly
people.
CAUSES:
 Atherosclerosis
 Risk factors:
  family history, hypertension, obesity, smoking, DM, stress, sedentary lifestyle, high serum
cholesterol and triglyceride levels.
S/Sx:
 angina
Dx:
 ECG
 Treadmill or bicycle exercise test
 Coronary angiography
 Myocardial perfusion imaging
Tx:
 reduce myocardial oxygen demand or increase oxygen supply
 nitrates, isosorbide dinitrate, beta – adrenergic blockers, calcium channel blockers.
 Angioplasty
 Prevention:
 dietary restriction of salts, fats, and cholesterol
 stress reduction and abstention of smoking
Intervention:
 During AP, monitor BP and HR.
 Keep nitroglycerin available for immediate use.
 Before cardiac catheterization, explain the procedure to the patient.
 After catheterization, review the expected course of treatment with the patient and family.
 After surgery, monitor BP,I & O, breath sounds, chest tube drainage and ECG.
 Before discharge, stress the need to follow the prescribed drug regimen, exercise program, and
diet. Encourage regular, moderate exercise.

3. ANGINA PECTORIS
 Angina pectoris is a clinical syndrome of precordial discomfort or
pressure due to transient myocardial ischemia without infarction. It is typically
precipitated by exertion or psychologic stress and relieved by rest or sublingual
nitroglycerin.
Etiology and Pathophysiology
1. Atherosclerosis, coronary artery spasm, coronary artery embolism
2. Acute coronary thrombosis
3. angina if obstruction is partial or transient
4. MI.
 Because myocardial O2 demand is determined mainly by heart rate,
systolic wall tension, and contractility, narrowing of a coronary artery typically
results in angina that occurs during exertion and is relieved by rest.
 In addition to exertion, cardiac workload can be increased by disorders
such as hypertension, aortic stenosis, aortic regurgitation, or hypertrophic
cardiomyopathy. In such cases, angina can result whether atherosclerosis is
present or not. These disorders can also decrease relative myocardial perfusion
because myocardial mass is increased (causing decreased diastolic flow).
 A decreased O2 supply, as in severe anemia or hypoxia, can
precipitate or aggravate angina.

Symptoms and Signs

1. troublesome ache to severe, intense precordial crushing sensation.
2. Discomfort may radiate to the left shoulder and down the inside of the left
arm, even to the fingers; straight through to the back; into the throat,
jaws, and teeth; and, occasionally, down the inside of the right arm. It
may also be felt in the upper abdomen. The discomfort of angina is never
above the ears or below the umbilicus.
3. Some patients have atypical angina (eg, bloating, gas, abdominal
distress), often ascribing symptoms to indigestion; belching may even
seem to relieve the symptoms. Others have dyspnea due to the sharp,
 reversible increase in LV filling pressure that often accompanies
ischemia. Frequently, the patient's description is imprecise, and whether
the problem is angina, dyspnea, or both may be difficult to determine.
Because ischemic symptoms require a minute or more to resolve, brief,
fleeting sensations rarely represent angina.
4. During the attack:
heart rate may increase modestly
BP is often elevated
heart sounds become more distant
apical impulse is more diffuse
2nd heart sound may become paradoxical because LV ejection is more prolonged during
an ischemic attack
4th heart sound is common
3rd heart sound may develop
5. Symptom severity is often classified by the degree of exertion resulting in
angina.

Canadian Cardiovascular Classification System of Angina Pectoris

Class Activities Triggering Chest Pain
1 Strenuous, rapid, or prolonged exertion
Not usual physical activities (eg, walking, climbing stairs)

2 Walking rapidly
Walking uphill
Climbing stairs rapidly
Walking or climbing stairs after meals
Cold
Wind
Emotional stress

3 Walking, even 1 or 2 blocks at usual pace and on level ground

Climbing stairs, even 1 flight

4 Any physical activity

Sometimes occurring at rest

Unstable angina: Because angina characteristics are usually predictable for a given
patient, any changes (ie, rest angina, new-onset angina, increasing angina) should
be considered serious. Such changes are termed unstable angina and require
prompt evaluation and treatment.

Coronary Artery Disease: Braunwald Classification of Unstable Angina*

 Braunwald Classification of Unstable Angina*
Classification Description Designation
Severity

I New onset of severe angina or —

increasing† angina
No angina during rest

II Angina during rest within past month Subacute angina at rest

but not within preceding 48 h

III‡ Angina during rest within 48 h Acute angina at rest

Clinical situation

A Develops secondary to an Secondary UA

extracardiac condition that worsens
myocardial ischemia

B‡ Develops when no contributory Primary UA

extracardiac condition is present

C Develops within 2 wk of acute MI Post-MI UA

Diagnosis
1. Typical symptoms
 Diagnosis is suspected if chest discomfort is typical and is precipitated by exertion

and relieved by rest.

 Patients whose chest discomfort lasts > 20 min or occurs during rest or who have

syncope or heart failure are evaluated for an acute coronary syndrome.

 Chest discomfort may also be caused by GI disorders (eg, reflux, esophageal

spasm, indigestion, cholelithiasis), costochondritis, anxiety, panic attacks,

hyperventilation, and other cardiac disorders (eg, pericarditis, mitral valve prolapse,
supraventricular tachycardia, atrial fibrillation), even when coronary blood flow is not
compromised (see Approach to the Cardiac Patient: Chest Pain).
2. ECG: If typical exertional symptoms are present, ECG is indicated. Because angina
resolves quickly with rest, ECG rarely can be done during an attack except during stress
testing. If done during an attack, ECG is likely to show reversible ischemic changes: T wave
discordant to the QRS vector, ST-segment depression (typically), ST-segment elevation,
decreased R-wave height, intraventricular or bundle branch conduction disturbances, and
arrhythmia (usually ventricular extrasystoles). Between attacks, the ECG (and usually LV
function) at rest is normal in about 30% of patients with a typical history of angina pectoris,
even those with extensive 3-vessel disease. In the remaining 70%, the ECG shows
evidence of previous infarction, hypertrophy, or nonspecific ST-segment and T-wave (ST-T)
abnormalities. An abnormal resting ECG alone does not establish or refute the diagnosis.

3. Stress testing with ECG or imaging (echocardiographic or nuclear)

Stress testing: More specific tests include stress testing with ECG or with myocardial
imaging (eg, echocardiography, radionuclide imaging) and coronary angiography. Further
testing is needed to confirm the diagnosis, evaluate disease severity, determine appropriate
exercise levels for the patient, and help predict prognosis.
 If a patient has a normal resting ECG and can exercise, exercise stress testing with ECG is
done. In men with chest discomfort suggesting angina, stress ECG testing has a specificity
of 70%; sensitivity is 90%. Sensitivity is similar in women, but specificity is lower, particularly
in women < 55 (< 70%). However, women are more likely than men to have an abnormal
resting ECG when CAD is present (32% vs 23%). Although sensitivity is reasonably high,
exercise ECG can miss severe CAD (even left main or 3-vessel disease). In patients with
atypical symptoms, a negative stress ECG usually rules out angina pectoris and CAD; a
positive result may or may not represent coronary ischemia and indicates need for further
testing.

When the resting ECG is abnormal, false-positive ST-segment shifts are common on the
stress ECG, so patients should have stress testing with myocardial imaging. Exercise or
pharmacologic stress (eg, with dobutamine (Dobutrex)
or dipyridamole (Persantine) infusion may be used. The choice of imaging technique
depends on institutional availability and expertise. Imaging tests can help assess LV
function and response to stress; identify areas of ischemia, infarction, and viable tissue; and
determine the site and extent of myocardium at risk. Stress echocardiography can also
detect ischemia-induced mitral regurgitation.

4. Coronary angiography for significant symptoms or positive stress test

Angiography: Coronary angiography is the standard for diagnosing CAD but is not always
necessary to confirm the diagnosis. It is indicated primarily to locate and assess severity of
coronary artery lesions when revascularization (percutaneous intervention [PCI] or coronary
artery bypass grafting [CABG]) is being considered. Angiography may also be indicated
when knowledge of coronary anatomy is necessary to advise about work or lifestyle needs
(eg, discontinuing job or sports activities). Obstruction is assumed to be physiologically
significant when the luminal diameter is reduced > 70%. This reduction correlates well with
the presence of angina pectoris unless spasm or thrombosis is superimposed.

Intravascular ultrasonography provides images of coronary artery structure. This test can
provide more information about coronary anatomy than other tests; it is indicated when the
nature of lesions is unclear or when apparent disease severity does not match symptom
severity. Used with angioplasty, it can help ensure optimal placement of stents.

Imaging: Electron beam CT can detect the amount of Ca present in coronary artery plaque.
The Ca score (from 1 to 100) is roughly proportional to the risk of subsequent coronary
events. However, because Ca may be present in the absence of significant stenosis, the
score does not correlate well with the need for angioplasty or CABG. Current indications for
cardiac MRI include evaluation of cardiac structure and function, assessment of myocardial
viability, and possibly diagnosis and risk assessment of patients with either known or
suspected CAD.

Prognosis
 The main adverse outcomes are unstable angina, MI, and sudden death due to
arrhythmias.
 Annual mortality rate is about 1.4% in patients with angina, no history of MI, a
normal resting ECG, and normal BP. Women with CAD tend to have a worse
prognosis.
  Mortality rate is about 7.5% when systolic hypertension is present, 8.4% when the
ECG is abnormal, and 12% when both are present. Type 2 diabetes about doubles
the mortality rate for each scenario.
 Prognosis worsens with increasing age, increasingly severe anginal symptoms,
presence of anatomic lesions, and poor ventricular function.
 Lesions in the left main coronary artery or proximal left anterior descending artery
indicate particularly high risk.
 Although prognosis correlates with number and severity of coronary arteries affected,
prognosis is surprisingly good for patients with stable angina, even those with 3-
vessel disease, if ventricular function is normal.

Treatment
1. Modification of risk factors (smoking, BP, lipids)

2. Antiplatelet drugs ( aspirin plus clopidogrel)

3. β-Blockers
4. Nitroglycerin (Nitro – bid, Nitro – dur, Nitrol, Nitroquick)
5. Ca channel blockers for symptom control
6. Revascularization if symptoms persist despite medical therapy
7. ACE inhibitors and statins
Drugs:

• The main goals are to relieve acute symptoms, prevent or reduce ischemia and
prevent future ischemic events. For an acute attack, sublingual nitroglycerin is the
most effective drug.
• To prevent ischemia, all patients diagnosed with CAD or at high risk of developing
CAD should take an antiplatelet drug daily. β-Blockers, unless contraindicated or not
tolerated, are given to most patients. For some patients, prevention of symptoms
requires Ca channel blockers or long-acting nitrates.

. Antiplatelet drugs inhibit platelet aggregation.

• Aspirin binds irreversibly to platelets and inhibits cyclooxygenase and platelet

aggregation.
• Clopidogrel blocks adenosine diphosphate–induced platelet aggregation. Either drug
can reduce risk of ischemic events (MI, sudden death), but the drugs are most
effective when given together. Patients unable to tolerate one should receive the
other drug alone.

B. β-Blockers limit symptoms and prevent infarction and sudden death better than
other drugs.

• β-Blockers block sympathetic stimulation of the heart and reduce systolic BP, heart
rate, contractility, and cardiac output, thus decreasing myocardial O2 demand and
increasing exercise tolerance.
• They also increase the threshold for ventricular fibrillation. Most patients tolerate
these drugs well.
 • Many β-blockers are available and effective. Dose is titrated upward as needed until
limited by bradycardia or adverse effects.
• Patients who cannot tolerate β-blockers are given a Ca channel blocker with
negative chronotropic effects (eg, diltiazem – cardizem, verapamil (Calan, Isoptin).
• Those at risk for β-blocker intolerance (eg, those with asthma) may be tried on a
cardioselective β-blocker (eg, carvedilol – COREG) perhaps with pulmonary function
testing before and after drug administration to detect drug-induced bronchospasm.

. Nitroglycerin

• is a potent smooth-muscle relaxant and vasodilator.

• Its main sites of action are in the peripheral vascular tree, especially in the venous or
capacitance system, and in coronary blood vessels.
• Even severely atherosclerotic vessels may dilate in areas without atheroma.
• Nitroglycerin lowers systolic BP and dilates systemic veins, thus reducing myocardial
wall tension, a major determinant of myocardial O2 need.
• Sublingual nitroglycerin is given for an acute attack or for prevention before exertion.
• Dramatic relief usually occurs within 1.5 to 3 min, is complete by about 5 min, and
lasts up to 30 min.
• The dose may be repeated q 4 to 5 min up to 3 times if relief is incomplete.
• Patients should always carry nitroglycerin tablets or aerosol spray to use promptly at
the onset of an angina attack. Patients should store tablets in a tightly sealed, light-
resistant glass container, so that potency is not lost. Because the drug deteriorates
quickly, small amounts should be obtained frequently.
• Long-acting nitrates (oral or transdermal) are used if symptoms persist after the β-
blocker dose is maximized. If angina occurs at predictable times, a nitrate is given to cover those
times. Oral nitrates include isosorbide dinitrate and mononitrate (the active metabolite of the
dinitrate). They are effective within 1 to 2 h; their effect lasts 4 to 6 h. Sustained-release
formulations of isosorbide mononitrate appear to be effective throughout the day. For transdermal
use, cutaneous nitroglycerin patches have largely replaced nitroglycerin ointments primarily
because ointments are inconvenient and messy. Patches slowly release the drug for a prolonged
effect; exercise capacity improves 4 h after patch application and wanes in 18 to 24 h. Nitrate
tolerance may occur, especially when plasma concentrations are kept constant. Because MI risk is
highest in early morning, an afternoon or early evening respite period from nitrates is reasonable
unless a patient commonly has angina at that time. For nitroglycerin , an 8- to 10-h respite period
seems sufficient. Isosorbide may require a 12-h respite period. If given once/day, sustained-release
isosorbide mononitrate does not appear to elicit tolerance.

D. Ca channel blockers may be used if symptoms persist despite use of nitrates or if

nitrates are not tolerated. Ca channel blockers are particularly useful if hypertension
or coronary spasm is also present. Different types of Ca channel blockers have
different effects. Dihydropyridines (eg, nifedipine – Adalat, amlodipine – Norvasc,
felodipine – Plendil) have no chronotropic effects and vary substantially in their
negative inotropic effects. Diltiazem - Cardizem and verapamil, other types of Ca
channel blockers, have negative chronotropic and inotropic effects. They can be
 used alone in patients with β-blocker intolerance or asthma and normal LV systolic
function but may increase cardiovascular mortality in patients with LV systolic
dysfunction.
E. Revascularization: Revascularization, either with PCI (eg, angioplasty, stenting) or
CABG should be considered if angina persists despite drug therapy and worsens
quality of life or if anatomic lesions (noted during angiography) put a patient at high
risk of mortality. The choice between PCI and CABG depends on extent and location
of anatomic lesions, the experience of the surgeon and medical center, and, to some
extent, patient preference.

PCI is usually preferred for 1- or 2-vessel disease with suitable anatomic lesions.
Lesions that are long or near bifurcation points are often not amenable to PCI.
However, as stent technology improves, PCI is being used for more complicated
cases.

CABG is very effective in selected patients with angina. The ideal candidate has
severe angina pectoris and localized disease, or diabetes mellitus. About 85% of
patients have complete or dramatic symptom relief. Exercise stress testing shows
positive correlation between graft patency and improved exercise tolerance, but
exercise tolerance sometimes remains improved despite graft closure.

CABG improves survival for patients with left main disease, those with 3-vessel
disease and poor LV function, and some patients with 2-vessel disease. However,
for patients with mild or moderate angina (class I or II) or 3-vessel disease and good
ventricular function, CABG appears to only marginally improve survival. For patients
with 1-vessel disease, outcomes with drug therapy, PCI, and CABG are similar;
exceptions are left main disease and proximal left anterior descending disease, for
which revascularization appears advantageous.

Nursing Interventions:
 Administer nitroglycerine sublingually to relieve the pain.
 Teach the patient that a burning sensation under the tongue after
nitroglycerine administration indicates that the drug is potent.
 The drug may also cause facial flushing and headache.
 Prepare the patient for PTCA by informing the patient that a balloon tipped
catheter will be introduced through a guide wire into a coronary vessel.
VARIANT ANGINA
Variant angina is angina pectoris secondary to epicardial coronary artery spasm
(Prinzmetal's angina).

Most patients with variant angina have significant fixed proximal obstruction of at
least one major coronary artery. Spasm usually occurs within 1 cm of the obstruction
(often accompanied by ventricular arrhythmia).
 Symptoms are anginal discomfort occurring mainly during rest, often at night, and
only rarely and inconsistently during exertion (unless significant coronary artery
obstruction is also present). Attacks tend to occur regularly at certain times of day.

Diagnosis is suspected if ST-segment elevation occurs during the attack. Between

anginal attacks, the ECG may be normal or show a stable abnormal pattern.
Confirmation is by provocative testing with ergonovine or acetylcholine, which may
precipitate coronary artery spasm, identified by significant ST-elevation or by
observation of a reversible spasm during cardiac catheterization. Testing is done
most commonly in a cardiac catheterization laboratory and occasionally in a
coronary care unit.

Average survival at 5 yr is 89 to 97%, but mortality risk is greater for patients with
both variant angina and atherosclerotic coronary artery obstruction. Usually,
sublingual nitroglycerin promptly relieves variant angina. Ca channel blockers may
effectively prevent symptoms. Theoretically, β-blockers may exacerbate spasm by
allowing unopposed α-adrenergic vasoconstriction, but this effect has not been
proved clinically. Oral drugs most commonly used are sustained-release diltiazem
120 to 540 mg once/day, sustained-release verapamil 120 to 480 mg once/day
(dose must be reduced in patients with renal or hepatic dysfunction), or amlodipine
15 to 20 mg once/day (dose must be reduced in elderly patients and patients with
hepatic dysfunction). In refractory cases, amiodarone may be useful. Although these
drugs relieve symptoms, they do not appear to alter prognosis.

SYNDROME X
Syndrome X is cardiac microvascular dysfunction or constriction causing angina
(microvascular angina).

Some patients with typical angina that is relieved by rest or nitroglycerin have normal
coronary arteriograms (eg, no atherosclerosis, embolism, or inducible arterial
spasm). Some of these patients have ischemia detected during stress testing; others
do not. In some patients, the cause of ischemia seems to be reflex intramyocardial
coronary constriction and reduced coronary flow reserve. Other patients have
microvascular dysfunction within the myocardium: The abnormal vessels do not
dilate in response to exercise or other cardiovascular stressors; sensitivity to cardiac
pain may also be increased. Prognosis is good, although symptoms of ischemia may
recur for years. In many patients, β-blockers relieve symptoms. This disorder should
not be confused with variant angina due to epicardial coronary spasm or with
another disorder called syndrome X, which refers to the metabolic syndrome.

SILENT ISCHEMIA
Patients with CAD (particularly diabetics) may have ischemia without symptoms.
Ischemia is evidenced by transient asymptomatic ST-T abnormalities seen during
24-h Holter monitoring. Radionuclide studies can sometimes document
asymptomatic myocardial ischemia during physical or mental stress (eg, mental
arithmetic). Silent ischemia and angina pectoris

5. MYOCARDIAL INFARCTION
 destruction of cardiac tissue due to reduced coronary blood flow.
IM:
 Lower sternal pain not relieved by rest and nitroglycerine, characterized as
crushing or excruciating.
 S/Sx:
 Chest pain
 dyspnea
 hyperthermia
 initial  in BP
 mild restlessness & apprehensions
 cool, moist, ashen skin
 occasional findings
Dx:
 Cardiac Enzymes (CPK-MB, LDH, SGPT, SGOT, Troponin test)
 ECG tracing (widening of QRS complexes)
 Serum cholesterol & uric acid
 CBC
Lab. Data:
 Elevated creatinine phosphokinase, and TROPONIN
 ECG reveals ST segment elevation or depression and T wave inversion.
Nursing Dx:
 Pain related to decreased tissue oxygenation.
Interventions:
 Narcotic analgesics : Morphine SO4
 Administer O2 inhalation
 Enforce CBR without BRP
 Avoid valsalva manuever
 Semi-Fowler
 Provide a general liquid diet that is ↓ in
 Monitor V/S, I&O, & ECG tracings
 Take 20-30 ml/wk
 Assist in surgery: CABG
 Provide health teaching
 Avoid modifiable risk fx
 Prevent complications
 Arrhythmias
 Shock
 Thrombophlebitis
 CHF
 Dressler’s syndrome
 Diet - low fat, low cholesterol, low sodium diet.
 Teach importance of follow up care
 Resume ADL
 Strict compliance to meds
 Vasodilators
 Anti-arrhythmic
 Beta blockers
 ACE inhibitors
 Ca antagonist
 Thrombolytics/fibrinolytics
S/E: (Monitor for bleeding)
♦ Sreptokinase
♦ Urokinase
♦ Tissue plasminogen activating factor
Anti-coagulants
Heparin
↓
PTT
↓
If prolonged bleeding
↓
Antidote: Protamine SO4

Coumadin (Warfarin)
↓
PT
↓
prolonged bleeding
↓
Antidote: Vit K

6. CONGESTIVE HEART FAILURE

 Inability of heart to pump blood towards systemic circulation.
LEFT SIDED HEART FAILURE
Predisposing Fx:
 90% mitral valve stenosis
Dx:
 ASO Titer (Anti-Streptolysin O)
 CXR
 PAP – Pulmonary Arterial Pressure
 PCWP – Pulmonary Capillary Wedge Pressure
 Swan-Ganz catheterization
 CVP – central Venous Pressure
 ↑ CVP > 10 : hypervolemia
 ↓ CVP < 4 : hypovolemia
 Echocardiography
 Arterial Blood Gas: ↑ PCO2, ↓ PO2 = hypoxemia =
 Liver Enzymes - ↑ SGPT (ALT) & SGOT (AST)

 Drugs given:
 Steroids
 Penicillin
 Aspirin
Complications:
 RS-CHF
 Aging
 Ischemic Heart Disease
 HPN, MI, Aortic Stenosis
S/sx:
 Pulmonary congestion/Edema
 dyspnea
 orthopnea
 paroxysmal nocturnal dyspnea
 productive cough with blood
 frothy saliva
 cyanosis
 rales / crackles
 bronchial wheezing
 point of maximal impulse displaced laterally
 pulsus alterans
 anorexia & general body malaise
 S3
Nsg Mgt:
 M – orphine SO4
 A – minophyline
 D – igitalis
 D – iuretics
 O – xygen
 G – ases

RIGHT SIDED HEART FAILURE

Predisposing Fx:
 90 % tricuspid stenosis
 COPD
 Pulmonary embolism
 Pulmonic stenosis
 Left sided heart failure
S/sx:
 Venous congestion:
 neck or jugular vein distention
 pitting edema
 ascites
 weight gain
 hepatomegaly / splenomegaly
 jaundice
 pruritis
 esophageal varices
 anorexia & general body malaise
Dx:
 CXR
 CVP
 Normal:
 ↑ CVP > 10 : hypervolemia
 ↓ CVP < 4 : hypovolemia
 Position of patient when giving CVP -Trendelenburg –
 Echocardiography
  Liver Enzymes - ↑ SGPT (ALT) & SGOT (AST)
Nsg Mgt: ↑ force of myocardial infarction = ↑ cardiac output
 Administer meds:
 Cardiac Glycosides
 Digoxin (Lanoxin)
 Antidote: Digibind
 Loop Diuretics
 Lasix
 Bronchodilators
 Aminophyline (Theophyline)
 Narcotic analgesic
 Morphine SO4
 Vasodilators
 Anti-arrythmics
 Lidocaine
 Ethacrynic acid – Thiazide diuretics
 Administer O2 inhalation
 High Fowler’s
 Restrict Na
 Provide meticulous skin care
 Weigh patient daily. Assess for pitting edema. Measure abdominal girth daily & notify MD.
 Monitor V/S, I&O, breath sounds, ECG readings
 Institute bloodless phlebotomy. Rotating torniquet or BP cuff rotated clockwise q 15 min =
promote venous return
 Diet: ↓ salt, fats & caffeine
 Health teaching
 complications:
 Shock
 Arrhythmia
 Thrombophlebitis
 MI
 Cor pulmonale – Right ventricular hypertrophy

7. HYPERTENSION
• is defined as a persistent systolic blood pressure (SBP) greater than or equal to 140
mm Hg, diastolic blood pressure (DBP) greater than or equal to 90 mm Hg, or
current use of antihypertensive medication. There is a direct relationship between
hypertension and cardiovascular disease (CVD).

• Contributing factors to the development of hypertension include cardiovascular risk

factors combined with socioeconomic conditions and ethnic differences.

• Hypertension is generally an asymptomatic condition. Individuals who remain

undiagnosed and untreated for hypertension present the greatest challenge and
opportunity for health care providers.

REGULATION OF BLOOD PRESSURE

• BP is the force exerted by the blood against the walls of the blood vessel. It must be
adequate to maintain tissue perfusion during activity and rest.

• Regulation of BP involves nervous, cardiovascular, endothelial, renal, and endocrine

functions.
o Sympathetic nervous system (SNS) activation increases heart rate (HR) and
cardiac contractility, produces widespread vasoconstriction in the peripheral
arterioles, and promotes the release of renin from the kidneys.
o Baroreceptors, located in the carotid artery and the arch of the aorta, sense
changes in BP. When BP is increased, these receptors send inhibitory
impulses to the sympathetic vasomotor center in the brainstem resulting in
decreased HR, decreased force of contraction, and vasodilation in peripheral
arterioles.
o A decrease in BP leads to activation of the SNS resulting in constriction of the
peripheral arterioles, increased HR, and increased contractility of the heart.
o In the presence of long-standing hypertension, the baroreceptors become
adjusted to elevated levels of BP and recognize this level as “normal.”
o Norepinephrine (NE), released from SNS nerve endings, activates receptors
 located in the sinoatrial node, myocardium, and vascular smooth muscle.
o Vascular endothelium produces vasoactive substances and growth factors.
 Nitric oxide, an endothelium-derived relaxing factor (EDRF), helps
maintain low arterial tone at rest, inhibits growth of the smooth muscle
layer, and inhibits platelet aggregation.
 Endothelin (ET), produced by the endothelial cells, is an extremely
potent vasoconstrictor.
o Kidneys contribute to BP regulation by controlling sodium excretion and
extracellular fluid (ECF) volume.
 Sodium retention results in water retention, which causes an increased
ECF volume. This increases the venous return to the heart, increasing
the stroke volume, which elevates the BP through an increase in CO.
o Endocrine system:
 The adrenal medulla releases epinephrine in response to SNS
stimulation. Epinephrine activates β 2-adrenergic receptors causing
vasodilation. In peripheral arterioles with only α 1-adrenergic receptors
(skin and kidneys), epinephrine causes vasoconstriction.
 The adrenal cortex is stimulated by A-II to release aldosterone.
Aldosterone stimulates the kidneys to retain sodium and water. This
increases BP by increasing CO.
 ADH is released from the posterior pituitary gland in response to an
increased blood sodium and osmolarity level. ADH increases the ECF
volume by promoting the reabsorption of water in the distal and
collecting tubules of the kidneys resulting in an increase in blood
volume and BP.

CLASSIFICATION OF HYPERTENSION
• Hypertension is classified as follows:
o Prehypertension: BP 120 to 139 / 80 to 89 mm Hg
o Hypertension, Stage 1: BP 140 to 159 / 90 to 99 mm Hg
o Hypertension, Stage 2: systolic BP greater than or equal to 160 or diastolic
BP greater than or equal to 100 mm Hg.

• Subtypes of hypertension:
o Isolated systolic hypertension (ISH): average SBP greater than or equal to
140 mm Hg coupled with an average DBP less than 90 mm Hg. ISH is more
common in older adults. Control of ISH decreases the incidence of stroke,
heart failure, cardiovascular mortality, and total mortality.
o Pseudohypertension (false hypertension) occurs with advanced
arteriosclerosis. Pseudohypertension is suspected if arteries feel rigid or
when few retinal or cardiac signs are found relative to the pressures obtained
by cuff.

ETIOLOGY OF HYPERTENSION
• Primary (essential or idiopathic) hypertension: elevated BP without an identified
cause; accounts for 90% to 95% of all cases of hypertension.

• Secondary hypertension: elevated BP with a specific cause; accounts for 5% to

10% of hypertension in adults.

PATHOPHYSIOLOGY OF PRIMARY HYPERTENSION

• The hemodynamic hallmark of hypertension is persistently increased SVR.

• Water and sodium retention:

o A high-sodium intake may activate a number of pressor mechanisms and
cause water retention.

• Altered renin-angiotensin mechanism:

 o High plasma renin activity (PRA) results in the increased conversion of
angiotensinogen to angiotensin I causing arteriolar constriction, vascular
hypertrophy, and aldosterone secretion.

• Stress and increased SNS activity:

o Arterial pressure is influenced by factors such as anger, fear, and pain.
o Physiologic responses to stress, which are normally protective, may persist to
a pathologic degree, resulting in prolonged increase in SNS activity.
o Increased SNS stimulation produces increased vasoconstriction, increased
HR, and increased renin release.

• Insulin resistance and hyperinsulinemia:

o Abnormalities of glucose, insulin, and lipoprotein metabolism are common in
primary hypertension.
o High insulin concentration in the blood stimulates SNS activity and impairs
nitric oxide–mediated vasodilation.
o Additional pressor effects of insulin include vascular hypertrophy and
increased renal sodium reabsorption.

• Endothelial cell dysfunction:

o Some hypertensive people have a reduced vasodilator response to nitric
oxide.
o Endothelin produces pronounced and prolonged vasoconstriction.

CLINICAL MANIFESTATIONS OF HYPERTENSION

• Often called the “silent killer” because it is frequently asymptomatic until it becomes
severe and target organ disease occurs.

• Target organ diseases occur in the heart (hypertensive heart disease), brain
(cerebrovascular disease), peripheral vasculature (peripheral vascular disease),
kidney (nephrosclerosis), and eyes (retinal damage).

• Hypertension is a major risk factor for coronary artery disease (CAD).

• Sustained high BP increases the cardiac workload and produces left ventricular
hypertrophy (LVH). Progressive LVH, especially in association with CAD, is
associated with the development of heart failure.

• Hypertension is a major risk factor for cerebral atherosclerosis and stroke.

• Hypertension speeds up the process of atherosclerosis in the peripheral blood

vessels, leading to the development of peripheral vascular disease, aortic aneurysm,
and aortic dissection.

• Intermittent claudication (ischemic muscle pain precipitated by activity and relieved

with rest) is a classic symptom of peripheral vascular disease involving the arteries.

• Hypertension is one of the leading causes of end-stage renal disease, especially

among African Americans. The earliest manifestation of renal dysfunction is usually
nocturia.

• The retina provides important information about the severity and duration of
hypertension. Damage to retinal vessels provides an indication of concurrent vessel
damage in the heart, brain, and kidney. Manifestations of severe retinal damage
include blurring of vision, retinal hemorrhage, and loss of vision.

DIAGNOSTIC STUDIES
• Basic laboratory studies are performed to (1) identify or rule out causes of secondary
 hypertension, (2) evaluate target organ disease, (3) determine overall cardiovascular
risk, or (4) establish baseline levels before initiating therapy.

• Routine urinalysis, BUN, serum creatinine, and creatinine clearance levels are used
to screen for renal involvement and to provide baseline information about kidney
function.

• Measurement of serum electrolytes, especially potassium levels, is done to detect

hyperaldosteronism, a cause of secondary hypertension.

• Blood glucose levels assist in the diagnosis of diabetes mellitus.

• Lipid profile provides information about additional risk factors that predispose to
atherosclerosis and cardiovascular disease.

• Uric acid levels are determined to establish a baseline, because the levels often rise
with diuretic therapy.

• ECG and echocardiography provide information about the cardiac status.

• Ambulatory blood pressure monitoring (ABPM) is a noninvasive, fully automated

system that measures BP at preset intervals over a 24-hour period.
o Some patients with hypertension do not show a normal, nocturnal dip in BP
and are referred to as “nondippers.”
o The absence of diurnal variability has been associated with more target organ
damage and an increased risk for cardiovascular events. The presence or
absence of diurnal variability can be determined by ABPM.

NURSING AND COLLABORATIVE MANAGEMENT

• Treatment goals are to lower BP to less than 140 mm Hg systolic and less than 90
mm Hg diastolic for most persons with hypertension (less than 130 mm Hg systolic
and less than 80 mm Hg diastolic for those with diabetes mellitus and chronic kidney
disease).

• Lifestyle modifications are indicated for all patients with prehypertension and
hypertension and include the following:
o Weight reduction. A weight loss of 10 kg (22 lb) may decrease SBP by
approximately 5 to 20 mm Hg.
o Dietary Approaches to Stop Hypertension (DASH) eating plan. Involves
eating several servings of fish each week, eating plenty of fruits and
vegetables, increasing fiber intake, and drinking a lot of water. The DASH diet
significantly lowers BP.
o Restriction of dietary sodium to less than 6 g of salt (NaCl) or less than 2.4 g
of sodium per day.
o This involves avoiding foods known to be high in sodium (e.g., canned soups)
and not adding salt in the preparation of foods or at meals.
o There is evidence that greater levels of dietary potassium, calcium, vitamin D,
and omega-3 fatty acids are associated with lower BP in those with
hypertension.
o Restriction of alcohol to no more than two drinks per day for men and no
more than one drink per day for women
o Regular aerobic physical activity (e.g., brisk walking) at least 30 minutes a
day most days of the week. Moderately intense activity such as brisk walking,
jogging, and swimming can lower BP, promote relaxation, and decrease or
control body weight.
o It is strongly recommended that tobacco use be avoided.
o Stress can raise BP on a short-term basis and has been implicated in the
development of hypertension. Relaxation therapy, guided imagery, and
biofeedback may be useful in helping patients manage stress, thus
 decreasing BP.

Drug Therapy
• Drug therapy is not recommended for those persons with prehypertension unless it
is required by another condition, such as diabetes mellitus or chronic kidney
disease.

• The overall goals for the patient with hypertension include (1) achievement and
maintenance of the goal BP; (2) acceptance and implementation of the therapeutic
plan; (3) minimal or no unpleasant side effects of therapy; and (4) ability to manage
and cope with illness.

• Drugs currently available for treating hypertension work by (1) decreasing the
volume of circulating blood, and/or (2) reducing SVR.
o Diuretics promote sodium and water excretion, reduce plasma volume,
decrease sodium in the arteriolar walls, and reduce the vascular response to
catecholamines.
o Adrenergic-inhibiting agents act by diminishing the SNS effects that increase
BP. Adrenergic inhibitors include drugs that act centrally on the vasomotor
center and peripherally to inhibit norepinephrine release or to block the
adrenergic receptors on blood vessels.
o Direct vasodilators decrease the BP by relaxing vascular smooth muscle and
reducing SVR.
o Calcium channel blockers increase sodium excretion and cause arteriolar
vasodilation by preventing the movement of extracellular calcium into cells.
o Angiotensin-converting enzyme (ACE) inhibitors prevent the conversion of
angiotensin I to angiotensin II and reduce angiotensin II (A-II)–mediated
vasoconstriction and sodium and water retention.
o A-II receptor blockers (ARBs) prevent angiotensin II from binding to its
receptors in the walls of the blood vessels.
o Thiazide-type diuretics are used as initial therapy for most patients with
hypertension, either alone or in combination with one of the other classes.
o When BP is more than 20/10 mm Hg above SBP and DBP goals, a second
drug should be considered. Most patients who are hypertensive will require
two or more antihypertensive medications to achieve their BP goals.
o Side effects and adverse effects of antihypertensive drugs may be so severe
or undesirable that the patient does not comply with therapy.
 Hyperuricemia, hyperglycemia, and hypokalemia are common side
effects with both thiazide and loop diuretics.
 ACE inhibitors lead to high levels of bradykinin, which can cause
coughing. An individual who develops a cough with the use of ACE
inhibitors may be switched to an ARB.
 Hyperkalemia can be a serious side effect of the potassium-sparing
diuretics and ACE inhibitors.
 Sexual dysfunction may occur with some of the diuretics.
 Orthostatic hypotension and sexual dysfunction are two undesirable
effects of adrenergic-inhibiting agents.
 Tachycardia and orthostatic hypotension are potential adverse effects
of both vasodilators and angiotensin inhibitors.
 Patient and family teaching related to drug therapy is needed to
identify and minimize side effects and to cope with therapeutic effects.
Side effects may be an initial response to a drug and may decrease
with continued use of the drug.

• Resistant hypertension is the failure to reach goal BP in patients who are adhering to
full doses of an appropriate three-drug therapy regimen that includes a diuretic.

Blood Pressure Monitoring

• The majority of cases of hypertension are identified through routine screening
 procedures such as insurance, preemployment, and military physical examinations.

• The auscultatory method of BP measurement is recommended. Initially, the BP is

taken at least twice, at least 1 minute apart, with the average pressure recorded as
the value for that visit. Size and placement of BP cuff are important for accurate
measurement. The forearm is supported at heart level and Korotkoff sounds are
auscultated over the radial artery.

• BP measurements of both arms should be performed initially to detect any

differences between arms. The arm with the higher reading should be used for all
subsequent BP measurements.

• Orthostatic (or postural) changes in BP and pulse should be measured in older

adults, in people taking antihypertensive drugs, and in patients who report symptoms
consistent with reduced BP upon standing (e.g., light-headedness, dizziness,
syncope).

• Orthostatic hypotension is defined as a decrease of 20 mm Hg or more in SBP, a

decrease of 10 mm Hg or more in DBP, and/or an increase of 20 beats/minute or
more in pulse from supine to standing.

• BP monitoring should focus on controlling BP in the person already identified as

having hypertension; identifying and controlling BP in at-risk groups such as African
Americans, obese people, and blood relatives of people with hypertension; and
screening those with limited access to the health care system.

NURSING MANAGEMENT
• The primary nursing responsibilities for long-term management of hypertension are
to assist the patient in reducing BP and complying with the treatment plan. Nursing
actions include patient and family teaching, detection and reporting of adverse
treatment effects, compliance assessment and enhancement, and evaluation of
therapeutic effectiveness.

• Patient and family teaching includes the following: (1) nutritional therapy, (2) drug
therapy, (3) physical activity, (4) home monitoring of BP (if appropriate), and (5)
tobacco cessation (if applicable).
o Home monitoring of BP should include daily BP readings when treatment is
initiated or medications are adjusted and weekly once the BP has stabilized.
A log of the BP measurements should be maintained by the patient. Devices
that have memory or printouts of the readings are recommended to facilitate
accurate reporting.
o A major problem in the long-term management of the patient with
hypertension is poor compliance with the prescribed treatment plan. The
reasons include inadequate patient teaching, unpleasant side effects of
drugs, return of BP to normal range while on medication, lack of motivation,
high cost of drugs, lack of insurance, and lack of a trusting relationship
between the patient and the health care provider.

GERONTOLOGIC CONSIDERATIONS
• The prevalence of hypertension increases with age. The lifetime risk of developing
hypertension is approximately 90% for middle-aged (age 55 to 65) and older (age
>65) normotensive men and women.

• A number of age-related physical changes contribute to the pathophysiology of

hypertension in the older adult.

• In some older people, there is a wide gap between the first Korotkoff sound and
subsequent beats (auscultatory gap). Failure to inflate the cuff high enough may
result in underestimating the SBP.
 • Older adults are sensitive to BP changes. Reducing SBP to less than 120 mm Hg in
a person with long-standing hypertension could lead to inadequate cerebral blood
flow.

• Older adults produce less renin and are more resistant to the effects of ACE
inhibitors and angiotensin II receptor blockers.

• Orthostatic hypotension occurs often in older adults because of impaired

baroreceptor reflex mechanisms.

• Orthostatic hypotension in older adults is often associated with volume depletion or

chronic disease states, such as decreased renal and hepatic function or electrolyte
imbalance.

• To reduce the likelihood of orthostatic hypotension, antihypertensive drugs should

be started at low doses and increased cautiously.

HYPERTENSIVE CRISIS
• Hypertensive crisis is a severe and abrupt elevation in BP, arbitrarily defined as a
DBP more than 140 mm Hg.
o Hypertensive crisis occurs most often in patients with a history of
hypertension who have failed to comply with their prescribed medications or
who have been undermedicated.
o Hypertensive crisis related to cocaine or crack use is becoming a more
frequent problem. Other drugs such as amphetamines, phencyclidine (PCP),
and lysergic acid diethylamide (LSD) may also precipitate hypertensive crisis
that may be complicated by drug-induced seizures, stroke, MI, or
encephalopathy.

• Hypertensive emergency develops over hours to days and is defined as BP that is

severely elevated (more than 180/120 mm Hg) with evidence of acute target organ
damage.
o Hypertensive emergencies can precipitate encephalopathy, intracranial or
subarachnoid hemorrhage, acute left ventricular failure with pulmonary
edema, MI, renal failure, dissecting aortic aneurysm, and retinopathy.
o Hypertensive emergencies require hospitalization, intravenous (IV)
administration of antihypertensive drugs, and intensive care monitoring.

• Antihypertensive drugs include vasodilators, adrenergic inhibitors, and the ACE

inhibitor enalaprilat. Sodium nitroprusside is the most effective IV drug for the
treatment of hypertensive emergencies.

• Mean arterial pressure (MAP) is generally used instead of systolic and diastolic
readings to guide therapy. MAP is calculated as follows: MAP = (SBP + 2 DBP) ÷ 3.

• The use of an intraarterial line or an automated, noninvasive BP machine to monitor

the MAP and BP is required. The rate of drug administration is titrated according to
the level of MAP or BP.

• The initial treatment goal is to decrease MAP by no more than 25% within minutes to
1 hour. If the patient is stable, the target goal for BP is 160/100 to 110 mm Hg over
the next 2 to 6 hours.

• Lowering BP excessively may decrease cerebral, coronary, or renal perfusion and

could precipitate a stroke, acute MI, or renal failure.

• Additional gradual reductions toward a normal BP should be implemented over the

next 24 to 48 hours if the patient is clinically stable.
• Regular, ongoing assessment (e.g., ECG monitoring, vital signs, urinary output, level
of consciousness, visual changes) is essential to evaluate the patient with severe
hypertension.

• Hypertensive urgency develops over days to weeks and is defined as a BP that is

severely elevated but with no clinical evidence of target organ damage.
o Hypertensive urgencies usually do not require IV medications but can be
managed with oral agents.
o If a patient with hypertensive urgency is not hospitalized, outpatient follow-up
should be arranged within 24 hours.

8. BUERGER’S DISEASE (THROMBOANGITIS OBLITERANS)

 Vasculitis of the small and medium size veins and arteries usually in the lower extremities. It
is more common in men.
 Pain is an outstanding symptom. Intermittent claudication is the common problem.
 is inflammatory thrombosis of small and medium-sized arteries and some superficial
veins, causing arterial ischemia in distal extremities and superficial thrombophlebitis.
 Tobacco use is the primary risk factor.
 Symptoms and signs include claudication, nonhealing foot ulcers, rest pain, and
gangrene. Diagnosis is by clinical findings, noninvasive vascular testing,
angiography, and exclusion of other causes. Treatment is cessation of tobacco use.
Prognosis is excellent when tobacco use is stopped, but when it is not, the disorder
inevitably progresses, often requiring amputation.
 Thromboangiitis obliterans occurs almost exclusively in tobacco users (nearly all of
them smokers) and predominantly affects men aged 20 to 40; it rarely occurs in
women. It occurs more commonly in people with HLA-A9 and HLA-B5 genotypes.
Prevalence is highest in Asia and the Far and Middle East.
 Thromboangiitis obliterans produces segmental inflammation in small and medium-
sized arteries and, frequently, in superficial veins of the extremities. In acute
thromboangiitis obliterans, occlusive thrombi accompany neutrophilic and
lymphocytic infiltration of the intima; endothelial cells proliferate, but the internal
elastic lamina remains intact. In an intermediate phase, thrombi organize and
recanalize incompletely; the media is preserved but may be infiltrated with
fibroblasts. In older lesions, periarterial fibrosis may occur, sometimes affecting the
adjacent vein and nerve.
 The cause is unknown, although cigarette smoking is a primary risk factor. The
mechanism may involve delayed hypersensitivity or toxic angiitis. According to
another theory, thromboangiitis obliterans may be an autoimmune disorder caused
by cell-mediated sensitivity to types I and III human collagen, which are constituents
of blood vessels.

Symptoms and Signs

1. Onset is gradual, starting in the most distal vessels of the upper and lower
extremities with coldness, numbness, tingling, or burning.

2. These symptoms may develop before objective evidence of disease.

3. Raynaud's syndrome is common. Intermittent claudication occurs in the

affected extremity (usually in the arch of the foot or in the leg; rarely in the hand, arm,
or thigh) and may progress to rest pain.

4. Frequently, if pain is severe and persistent, the affected leg feels cold, sweats
excessively, and becomes cyanotic, probably because of sympathetic nerve
overactivity.

5. Later, ischemic ulcers develop in most patients and may progress to

gangrene.
 6. Pulses are impaired or absent in one or more pedal arteries and often at the
wrist.

7. In young men who smoke and have extremity ulcers, a positive Allen's test
(the hand remains pale after the examiner simultaneously compresses the radial and
ulnar arteries, then alternately releases them) suggests the disorder.

8. Pallor with elevation and rubor with dependency frequently occur in affected
hands, feet, or digits. Ischemic ulceration and gangrene, usually of one or more
digits, may occur early in the disorder but not acutely.

9. Noninvasive tests show greatly decreased blood flow and pressure in the
affected toes, feet, and fingers.

Diagnosis
1. (Other causes of ischemia excluded by testing)
2. Angiography
3. History and physical examination
4. Echocardiography
5. Leg arteriography
6. Allen's test
Treatment
1. Smoking cessation
2. Local measures
3. Sometimes drug therapy
• iloprost (Ventavis) 0.5 to 3 ng/kg/min IV infusion over 6 h may help prevent
amputation.
• Pentoxifylline (Trental)
• Ca channel blockers
• Thromboxane inhibitors

Ns. Dx: Altered peripheral tissue perfiusion

Interventions:
 Instruct the patient to stop smoking.
 Administer calcium channel blockers as ordered.

OTHERS:

a. Across the life-span

e.1. Anemias
e.2. Leukemia
e.3. DIC

Part 2
Alterations of Hematologic Functions
1. Anemias are a laboratory definition that implies a low red cell count and hemoglobin or
hematocrit level that is below normal. Physiologically, anemia exists when there is an
insufficient amount of hemoglobin to deliver oxygen to the tissues.
Altered Physiology
i. The appearance of anemia may reflect (1)-decreased production of red cells, (2) red
cell loss, or (3) increased destruction.
ii. Marrow failure may occur as a result of nutritional deficiency, toxic exposure, tumor
invasion, or unknown causes.
iii. Red cells may be lost through hemorrhage or hemolysis (increased destruction).
• This problem may be rooted in an intrinsic red cell defect that is incompatible with
normal red cell survival or is explainable on the basis of some factor extrinsic to the red
cell that promotes red-cell destruction.
• Red cell lysis occurs mainly within the phogocytic cells of the reticuloendothelial system,
notably within the liver and spleen.
• As a by-product of this process, bilirubin, formed by the metabolism of hemoglobin
within the phagocyte, enters the blood stream, and an increase in hemolysis in promptly
reflected by an increase in total plasma bilirubin.
• General Considerations:
• 1. Red cells and hemoglobin are normally formed at the same rate at which they are
destroyed.
• 2. Whenever formation of red cells or hemoglobin is decreased or their destruction is
increased, anemia results.
• 3. The ability of red blood cells to carry hemoglobin is decreased.
• 4. The ability of the hemoglobin to oxygenate the tissues and remove carbon dioxide for
excretion by the lungs is also decreased.
• 5. Less hemoglobin is available to act as a buffer in regulating the pH of the blood.
Clinical manifestations:
(Most manifestations are attributable to a decrease in the oxygen-carrying capacity of the
blood, and are the same regardless of the cause of anemia.)
i. The more rapidly the anemia develops, the more severe its symptoms:
• Pallor
• Susceptibility to fatigue
• Shortness of breath
• Headache; disturbed cerebration; dizziness
• Development of angina pectoris or congestive heart failure in susceptible individuals.
ii. Severity of symptoms dependent on:
• The speed with which and the degree to which the anemia has developed.
• Its prior duration (i.e., its chronicity)
• The metabolic requirements of the particular patient.
• Any other disorders currently afflicting the patient, particularly cardiac conditions, etc.
• Special complications or concomitant features of the condition producing the anemia.
a. Iron Deficiency Anemias are conditions in which the total body iron content is decreased below normal.
Etiology:
Iron deficiency develops when the body’s need for iron exceeds the supply.
 Chronic blood loss – gastrointestinal (GI) bleeding (secondary to gastritis, peptic ulcer, hemorrhoids,
malignancy), excessive menstrual bleeding, multiple pregnancies, hookworm infestation.
 Impaired GI absorption of iron – small bowel disease, certain gastric resections.
 Insufficient intake.
 Increased iron requirements – during pregnancy, periods of rapid growth, menstruation (average of
20 mg. Of iron lost per menstrual cycle.)
 Excessive ingestion of aspirin (leading to occult GI blood loss).
Iron Balance and Stores:
 Approximately 6mg. Of iron is ingested per 1000Kcal. or 16-20 mg / day.
 The amount of iron ingested is related to the number of calories consumed. Normally about 5% - 10%
of ingested iron is absorbed by the GI mucosa, bound to transferrin (main iron-binding protein in
plasma), and carried through the blood stream to the bone marrow. In the marrow, iron is transported
to red cells and reticuloendothelial cells.
 Normal adult male has iron stores of 900mg.; adult female has 300 mg.

 Adult male loses about 1mg. Iron per day; premenopausal woman loses about 1.5-mg iron per day.
Management:
i. Oral Iron Therapy
 Allows patient to regenerate hemoglobin. (Hematologic values should return to normal in 4 – 8
weeks) Therapy is continued approximately 6 months following normalization of blood values to
restore to hemoglobin and iron stores. It is continued longer in-patients with continued blood loss.
 Choice of iron depends on (1) patient tolerance, (2) GI absorption, and (3) dosage according to
estimate of hemoglobin deficiency.
a. Oral iron preparations
a. Ferrous sulfate (preferred and least expensive)
b. Ferrous gluconate
ii. Parenteral Iron Therapy
 Parenteral iron therapy is given only when:
 (1) the patient is unable to tolerate iron preparations orally,
 (2) the patient has severe GI disorders,
 (3) there is continuing negative iron balance while patient is taking maximum oral dose tolerated, or
 (4) There is nonadherence by patient.
 Parenteral iron preparations:
 (1) Iron dextran (Imferon)
 (2) Iron sorbitex (Jectofer) – may cause patient’s urine to turn black on standing, since about 50% of
iron is excreted in the urine within 24 hours.
Nursing Alert: Extravasation of iron medication results in painful local induration. An anaphylactic reaction
may occur following either intramuscular or intravenous injection of iron dextran.
Clinical Manifestations:
Reduction in hemoglobin concentration decreases the capacity of the blood to transport and deliver oxygen to
the tissues.
1. Easy fatigability
2. Headache, dizziness, tinnitus
3. Palpitations and dyspnea on exertion
4. Pallor of skin and mucous membranes
5. Smooth, sore tongue associated with burning sensation.
6. Cheilosis (lesions at corners of mouth)
7. Pica (Craving to eat unusual substances)
8. Koilonychia (spoon-shaped fingernails)
Patient Problems / Nursing Diagnoses
1. Fatigue related to anemia
2. Discomfort (headache, etc.) related to anemia
3. Activity intolerance related to reduced energy.
4. Inadequate nutritional intake related to dyspepsia
5. Ineffective individual coping related to related lifestyle resulting from activity limitations.
6. Diarrhea or constipation related to iron ingestion
b. Megaloblastic Anemias
1. A Megaloblast is a nucleated red cell with delayed and abnormal nuclear maturation.
2. B12 deficiency (pernicious Anemia) and / or Folic acid deficiency cause the most common
megalobastic anemias.
3. Anemias due to deficiencies of Vitamin B12 and folic acid show identical bone marrow and peripheral
blood changes. This is because both vitamins are essential for normal DNA synthesis.
* Folic Acid Deficiency is necessary for normal red blood cell production. Folate depletion results in
progressive anemia.
Causes:
1. Inadequate dietary intake
a. Common in alcoholics
b. Elderly individuals who live on “tea and toast”
c. Patients on hyperalimentation or chronic hemodialysis.
2. Impaired absorption – most absorption of folic acid takes place in upper jejunum
3. Increased requirements – chronic hemolytic anemias; pregnancy, etc.
4. Impaired utilization – from folic antagonists (methotrexate)
Clinical Manifestations:
Symptoms of anemia – fatigue, weakness, pallor, sore tongue, cracked lips.

Diagnostic Evaluation:
Assay of serum folate
Management:
1. Give oral folic acid replacement as prescribed.
2. Inform the patient that a proper diet will prevent most instances of folate deficiency: green vegetables
(asparagus, broccoli, and spinach); yeast, liver, organ meats, some fresh fruits.
Megaloblastic Anemias has two types, the Pernicious Anemia and Folic acid deficiency anemia. Folic
acid and vitamin B12 are necessary for the synthesis of nucleoproteins, which are essential for maturation of
red blood cells. So, deficiencies or disturbances in their normal metabolism of these two substances will
interfere with the synthesis of nucleoproteins. As a result, RBC is immature and larger than normal at every
stage of their development and number of circulating RBC is decreased.
* Pernicious Anemias are caused by lack of intrinsic factor, which normally is produced by gastric mucosal
parietal cells. B12 deficiency is also seen in diseases of the small intestine – i.e., malabsorption, blind
loop syndrome, following gastrectomy.)
Altered Physiology:
Pernicious anemia is a type of macrocytic anemia caused by failure of absorption of Vitamin B 12 (cobalamin).
Cobalamin is released form its protein-bound state by an acidic gastric environment. Lack of gastric acid may
lead to pernicious anemia, however; the most common cause is lack of intrinsic factor, a glucoprotein
produced by the parietal cells of the gastric lining. Vit B12 is absorbed in the terminal ileum, so if that part of
the bowel is surgically resected, absorption cannot occur. The cause of pernicious anemia may also be from
an autoimmune response. Without Vitamin B12, deoxyribonucleic acid (DNA) synthesis and cell replication
are impaired. RBC precursors do not divide normally, and large, poorly functioning RBCs are created..
Erythropoiesis (from impaired folic acid from the lack of vitamin B12 directly) is greatly affected.
1. Symptoms due to anemia
a. Pallor
b. Dyspnea, tachycardia, easy fatigability
c. Angina pectoris, arrythmias, CHF
d. Edema of the legs
2. Symptoms due to physiologic changes in gastrointestinal tract
a. Sore mouth with smooth, red, “beefy” tongue
b. Loss of appetite
c. Indigestion and epigastric discomfort
d. Recurring diarrhea or constipation
e. Weight loss
3. Symptoms due to neurologic changes (neuropathy occurs in high percentage of untreated patients)
a. Tingling and numbness or burning pain (paresthesia) involving hands and feet.
b. Loss of position sense, leading to disturbances of gait
c. Disturbances of bladder and bowel function
d. Psychiatric symptoms – from cerebral dysfunction
Diagnostic Evaluation:
1. Blood smear – reveals marked variation in size and shape of cells and a variable number of unusually
large cells containing a normal concentration of hemoglobin.
2. Tests for serum and red cell folate levels and serum Vitamin B12 level
3. Gastric analysis – the gastric juice lacks free hydrochloric acid (achlorhydria).
4. Schilling Test – a test for vitamin B12 absorption.
Purpose:
To prove that the patient cannot absorb oral vitamin B12 unless intrinsic factor is added.
i. The patient is given a small dose of radioactive B12 in water to drink followed by a nonradiocative
intramuscular dose.
ii. When the oral vitamin is absorbed, it will be excreted in the urine; the IM dose helps to flush it into
urine.
iii. A 24-hours urine specimen is collected and measured for radioactivity. All urine must be
collected. Patients with renal disease may require longer periods of collection.
iv. If very little has been excreted, the test is repeated several days later (the “second stage”) with a
capsule of oral intrinsic factor added to the oral Vitamin B12.
v. If the patient has pernicious anemia, this time much more radioactivity will be found in the 24-
hours urine specimen.

Management During Acute Stage:

• Hydroxocobalamin or cyanocobalamin (vitamin B12 IM) as directed (daily for 7 days, weekly for 10 weeks,
and then monthly for rest of life; many protocols for administration of B12 are in use).
• a. Parenteral vitamin therapy is necessary, since most of these patients are unable to absorb the vitamin
by mouth.
• b. Reticulocytes begin to increase on 4th day after therapy is started; normal hemoglobin values are
obtained in approximately 6 weeks.
• c. Patient begins to improve in general well being and mental status in a few days.
• d. Recent neurologic changes will usually be reversed.
• Give transfusion of packed cells; administer very slowly.
• a. Transfusion is given only to patients whose anemia is life threatening (symptoms of hypoxia to heart or
brain).
• b. Place the patient in a sitting position in bed.
• Too rapid administration of transfusion to a patient with anemia may produce acute pulmonary or cerebral
edema. These patients are particularly susceptible to volume overload because they have an
expanded plasma volume.
Health Education:
i. Impress upon the patient that vitamin B12 must be continued for his lifetime.
 Maintenance dose schedule – vitamin B12 IM every 4 weeks.
 Teach patient and family or have community health nurse give maintenance therapy.
 Untreated pernicious anemia is fatal
ii. Instruct the patient to report for follow-up examinations every 6 months – for hematocrit and physical
examination.
 Patient may develop hematologic or neurologic relapse if therapy is inadequate.
 Patients with pernicious anemia have a higher incidence of gastric cancer and thyroid problems;
therefore, periodic stool examination for occult blood and gastric cytology, along with thyroid function
tests, should be made.
iii. Following total gastrectomy (and occasional subtotal gastrectomy), patient should receive
maintenance dose of vitaminB12 as often as indicated – removal of gastric fundus deprives the patient
 of all intrinsic factor; may take as long as 10 years for clinical symptoms to appear, because of the
small amount of daily vitamin B12 required and the large body stores available for use.
• Folic Acid deficiency (Folic acid is necessary for normal red blood cell production. Folate depletion
results in progressive anemia). Folic acid is necessary for DNA synthesis. Serum folate levels are less
than 4 ng/ml in cases positive with folic acid deficiency. (normal reading is 7 to 20 ng/ml)
• Causes:
• 1. Inadequate dietary intake
• a. Common in alcoholics
• b. Elderly individuals who live on “tea and toast”.
• c. Patients on hyperalimentation or chronic hemodialysis.
• 2. Impaired absorption – most absorption of folic acid takes place in upper jejunum.
• 3. Increased requirements – chronic hemolytic anemias; pregnancy, etc.
• 4. Impaired utilization – from folic acid antagonists (methotrexate).
• Clinical manifestations:
• Symptoms of anemia – fatigue, weakness, pallor. Sore tongue, cracked lips.
• Diagnostic evaluation
• Assay of serum folate
• Management:
• 1. Give oral folic acid replacement as prescribed.
• 2. Inform the patient that a proper diet will prevent most instances of folate deficiency: green vegetables
(asparagus, broccoli, and spinach); yeast, liver, organ meats, some fresh fruits.
• Hypoplastic anemia
• The bone marrow is unable to manufacture new RBC and hemoglobin at a rate necessary to maintain a
normal concentration of these substances in the circulating blood.
• Anemia of infection
• 1. Life span of the RBC is moderately decreased.
• 2. The ability of the bone marrow to produce RBC is significantly decreased. (This is the principal factor in
determining the degree of anemia).

• Hemolytic anemias
• 1. The RBC is destroyed at abnormally high rates.
• 2. The activity of the bone marrow increases to compensate for the shortened survival time of the RBC.
• 3. Products of red cell breakdown increase with hemolysis.
• 4. Jaundice results when the liver is unable to clear the blood of the pigment resulting from breakdown of
hemoglobin from destroyed red cells.
• 5. Bone marrow hypertrophies and occupies a larger than normal share of the inner structure of bones.
• Sickle Cell Anemia
• Sickle cell disease is a severe, chronic, hemolytic anemia occurring in persons who are homozygous for
the sickle gene. The clinical course is characterized by episodes of pain due to the occlusion of small
blood vessels by sickled blood cells. Persons heterozygous for the sickling gene are said to possess
sickle cell trait, which is associated with a benign clinical course.
• Etiology:
• 1. Genetically determined, inherited disease
• 2. Each person inherits 1 gene from each parent, which governs the synthesis of hemoglobin.
• Transmission of Sickle Cell Disease
Probability of abnormal Hemoglobin in Offspring
Genotype Parents Normal Trait Disease
1 Parents with trait 50% 50% 0
Both parents with trait, 25% 50% 25%
1parent with trait; 1 parent with
disease 0 50% 50%
Both parents with disease 0 0 100%
• Incidence:
• 1. Found almost entirely in American Blacks and persons of Spanish-American Ancestry
• 2. Approximately 8% of Black Americans have sickle cell trait.
• 3. Approximately 1 of every 600 Black infants born in the US has sickle cell anemia.
• Altered Physiology
• 1. Each hemoglobin molecule consists of 4 molecules of heme folded into 1 molecule of globin.
• 2. Each globin molecule consists of 2 alpha chains and 2 beta chains.
• 3. The amino acid sequence on the beta chain is altered in sickle cell hemoglobin. Valine is substituted for
glutamic acid in the 6th position.
• 4. Sickle cell hemoglobin aggregates into elongated crystals under conditions of low oxygen
concentration.
• 5. This distorts the membrane of the RBC causing it to assume a crescent or sickle shape. The cells
easily become entangled and enmeshed leading to increase blood viscosity.
• 6. Sickled red cells are fragile and are rapidly destroyed in the circulation.
• 7. Anemia results when the rate of destruction of red cells is greater than the rate of production.
• Prognosis:
• 1. Variable; improving with new forms of treatment.
• 2. Greatest risk of death is in children under 5 years of age.
• 3. Crises usually become less frequent and severe, as child becomes older.
• Preventive measures
• 1. Every black child admitted to the hospital should be tested for sickle cell anemia.
• 2. Parents at risk should be counseled regarding the genetic aspects of sickle cell anemia.
• 3. All siblings of any child who admitted to the hospital with sickle cell anemia should be tested for the
disease.
• Clinical manifestations:
• A. Symptoms
• 1. Children are rarely symptomatic until late in the first year of life
• 2. Clinical manifestation is sporadic.
• a. The child may be asymptomatic for several months.
• b. Periods of crisis occur at variable intervals.
• c. Precipitating factors of crisis include:
• (1) Dehydration
• (2) Infection
• (3) Trauma
• (4) Strenuous physical exertion
• (5) Extreme fatigue
• (6) Cold exposure
• (7) Hypoxia
• 3. Signs of crisis
• a. Loss of appetite
• b. Paleness
• c. Weakness
• d. Fever
• e. Pain in abdomen, back, joints, and/or extremities
• f. Swelling of joints, hands, or feet (“hand-foot syndrome”)
• g. Irritability
• Jaundice
• B. Thrombocytic Crisis (Most common form of crisis)
• 1. Small blood vessels are occluded by the sickle-shaped cells, causing distal ischemia and infarction.
• 2. Extremities
• 2a. Bony destruction
• 2b.Periosteal reaction
• 2c. Ulcers
• 3. Spleen – abdominal pain
• 4. Cerebral occlusion
• 4a. Strokes
• 4b. Hemiplegia
• 4c. Blindness
• 5. Pulmonary infarction, thromboses
• 6. Cardiac decompensation
• 7. Impaired liver function
• 8. Convulsions, cerebral infarction
• 9. Retinal damage, blindness
• C. Sequestrian Crisis
• 1. Large amounts of blood become pooled in the liver and spleen.
• 2. Spleen becomes massively enlarged.
• 3. Signs of circulatory collapse develop rapidly.
• 4. Frequent cause of death in infants with sickle cell disease.
• D. Aplastic Crisis – Bone marrow ceases production of red blood cells
• E. Chronic Symptoms
• 1. Jaundice
• 2. Gallstones
• 3. Progressive impairment of kidney function.
• 4. Fibrotic spleen
• 5. High susceptibility to salmonella, osteomyelitis, and pneumococcal septicemia.
• 6. Growth failure
• 7. Delayed puberty
• 8. Decreased life span.
• β -Thalassemia
• Beta-thalassemia or Thalassemia Major or Cooley’s refers to an inherited group of blood disorders
characterized by a reduction or absence of the beta globulin chain in hemoglobin synthesis. Homozygous
β-thalassemia is the most severe of the β-thalssemia syndromes and is also known as thalassemia major
or Cooley’s anemia.
• Etiology:
• 1. Genetically determined, inherited disease.
• 2. Autosomal-recessive pattern of interitance. Two types of β-thalassemia genes produce different
severities of the disease.
• Altered Physiology
• 1. Insufficient beta globulin synthesis allows large amount of unstable alpha chains to accumulate.
• 2. The precipitates of alpha chains that form cause red cells to be rigid and easily destroyed, leading to
severe anemia and resultant chronic hypoxia.
• 3. Erythroid activity is markedly increased in an attempt to overcome the increased rate of destruction,
resulting in enormous expansion of bone marrow.
• 4. Rapid destruction of defective RBC, decreased production of hemoglobin, and increased absorption of
dietary iron due to the body’s response to anemia result in an excess supply of available iron.
• 5. In response to the low level of adult hemoglobin, large concentration of fetal hemoglobin, which does
not contain beta chains, are produced.
• Prognosis
• 1. No known cure
• 2. Unable to predict which severely afflicted children will follow a more favorable course.
• 3. Often fatal in late childhood or early adolescence.

• Preventive Measures:
• 1. Parents of a child with thalassemia should be tested for the trait, and referred for genetic counseling.
• 2. Prenatal diagnosis is possible through fetal blood sampling early enough to allow the opportunity to
terminate the pregnancy.
• Management:
• 1. Frequent and regular blood transfusions to maintain hemoglobin levels above 9-10 gm./dl
• 1a. Washed, packed red cells are usually used to minimize the possibility of transfusion
reactions.
• 1b. The frequency and amount of transfusions depend on the size of the child, with older
children often reaching a peak requirement of 500ml of packed cells every 2-3 weeks.
• 2. Iron chelation therapy to reduce the toxic effects of excess iron.
• 3. Supportive management of complications.
• Clinical Manifestations:
• 1. Onset is usually insidious, with symptoms noted toward the end of the first year of life.
• 2. Symptoms are primarily related to the progressive anemia, expansion of the marrow cavities of the
bone and the development of hemesiderosis (excess iron storage in various body tissues).
• 3. Early symptoms often include progressive pallor, poor feeding, and protuberant abdomen due to
hepatospleenomegaly.
• 4. Further signs of progressive anemia include headache, bone pain, exercise intolerance, and
listlessness.
• Complications:
• 1. Spleenomegaly
• 2. Growth and endocrine Complications
• 3. Skeletal complications
• 4. Cardiac complications
• 5. Liver enlargement
• 6. Gallbladder disease
• 7. Megaloblastic anemia
• 8. Skin and leg ulcers
• Diagnostic Evaluation:
• 1. Hemoglobin level – decreased
• 2. Red cells indices – microcytocis and hypochromia
• 3. Peripheral blood smear –
• 4. Reticulocyte count – low, usually less than 10%
• 5. Hemoglobin electrophoresis
• Aplastic Anemia
• Anemia from bone marrow aplasia (failure) is characterized by deficiency in all the cellular elements of the
blood.
• Aplastic Anemia is a stem cell disorder characterized by bone marrow failure with pancytopenia
(deficiency in all cellular elements of the blood).
• Causes:
• 1. Idiopathic (50% of all cases).
• 2. Chemical toxins
• 3. Idiosyncratic response to drugs.
• 4. Virus, especially hepatitis.
• 5. Congenital (Fanconi’s anemia)
• Note: Secondary bone marrow aplasia is a predictable marrow suppression induced by antineoplastic
agents and radiation therapy or bone marrow transplantation.
• Etiology and Risk factors:
• Acquired Aplastic anemia may result from either an autoimmune mechanism or a direct injury by
myelotoxins. Three groups of myelotoxins are:
• 1. Agents that always cause marrow damage when received in insufficiently large doses, such as radiant
energy (x-rays, radium, and radioactives, alkylating agents, and antimetabolites used to treat malignant
tumors. Radiation causes the bone marrow to stop producing blood cells by inhibiting mitosis, or cell
division, and antimetabolites used in cancer therapy block the synthesis of purines or nucleic acids.
• 2. Agents that occasionally cause marrow failure, such as chloramphenicol (Chloromycetin, the drug most
commonly linked with aplastic anemia), sulfonamides, quinacrine, phenylbutazone, the anticonvulsants
phenytoin and mephenytoin, and gold compounds.
• 3. Agents that have been linked with aplastic anemia in only a few cases, such as streptomycin,
tripelennamine, DDT, meprobamate, hair and aniline dyes, and carbon tetrachloride.
Clinical Course:
• 1. The clinical course is variable, and the overall mortality rate is high; patients with severe pancytopenia
with totally aplastic marrow have a poor prognosis.
• 2. Patients with aplastic anemia are at serious risk of infection, hemorrhage, and other complications of
chronic anemia.
• Treatment:
• 1. Search for cause / prevention of toxic exposure
• 2. Bone marrow transplant (treatment of choice for patients with severe aplastic anemia who have
matched donors.
• 3. Androgens (oxymetholone or testosterone enanthate) – controversial.
• 4. Immunosupressive treatment (splenectomy; cyclophosphamide).
• 5. Immunologic therapy (antithymocyte globulin [ATG])
• Clinical Manifestations:
• 1. Abnormal bleeding – resulting from thrombocytopenia
• a. Bleeding from gums, nose, gastrointestinal and genitourinary tracts.
• b. Purpora; petechia; ecchymoses.
• 2. Anemia – resulting from depression of hemoglobin; symptoms pronounced because of rapidity of blood
cell change.
• a. Pallor; weakness
• b. Exertional dyspnea, palpitations
• 3. Infections with high fever – resulting from granulocytopenia.
• a. Pharyngitis and oropharyngeal mucositis.
• b. Sepsis via gastrointestinal tract or genitourinary tract.
Diagnostic Evaluation:
• 1. Peripheral blood smear shows pancytopenia.
• 2. Potential for infection related to lack of circulating granulocytes.
• 3. Potential for hemorrhge related to thrombocytopenia.
2. Clotting Disorders
a. Vascular Purpuras:
• The term purpura refers to extravasation (escape) of blood into the skin and mucous membranes.
Purpuric lesions may occur spontaneously as an isolated phenomenon or as an accompaniment of
obvious disease.
• Types of Purpura
• 1. Petechiae – small pinpoint hemorrhages under the skin.
• 2. Ecchymoses – escape of blood into tissues; producing a large bruise.
• 3. Petechiae and echymosses may occur as the result of vascular rupture, permitting the leakage of blood
into the subcutaneous tissue of the mucous membranes.
• 4. Symptomatic or secondary purpura – certain types of blood stream infections (e.g., meningococcemia
and infective endocarditis) exhibit this phenomenon because of damage to the vascular walls by the
infectious agent or by immune complexes.
• 5. Severe arterial hypertension – may cause the patient to bruise easily; valsalva maneuver may cause
petechiae.
• 6. Anaphylactoid purpura – generally regarded as an allergic disorder in which there are various skin
lesions (purpuric and otherwise) and episodes of arthritis, abdominal pain, hematuria, gastrointestinal
hemorrhage, and fever.
• A. Attacks last several weeks and recur for years.
• B. Steroid therapy is often effective.
• 7. Familial hemorrhagic telangiectasia – a hereditary disorder manifested by an abnormal tendency to
bleed and bruise.
• A. Precise nature of defect is obscure.
• B. Condition does not respond to any proved method of treatment.
• 8. Toxic Purpura – a condition observed after exposure to certain drugs and poisons.
• 9. Vitamin C deficiency – a vascular purpura.
• 10. Senile purpura.
• 11. Rheumatic and collagen – vascualr diseases – associated with palpable purpura caused deposition of
immune complexes in blood vessels of the skin.
• 12. Steroid purpura – associated with loss of capillary integrity.
b. Hemophilia
Hemophilia is an inherited, congenital blood dyscrasia, which is characterized by a disturbance of blood
clotting factors. It appears in males but is transmitted by females.
Three major types of Hemophilia:
1. Hemophilia A (Classic hemophilia)
2. Hemophilia B (Christmas disease)
3. von Willebrand’s disease
• Etiology:
• 1. Hereditary (about 80% of patients)
• Sex-linked, recessive trait

• a. Caused by a gene carried on the X chromosome, one of the sex chromosomes.

• b. Transmitted by asymptomatic females who carry the hemophilic gene on 1 of there X
chromosomes.
• c. Appears in males who have the hemophilic gene on there only X chromosomes.
• d. Affected males may carry a latent form of the disease to female offspring.
• e. May appear in females if a female carrier mates with a male hemophiliac.
• 2. Spontaneous mutations may cause the condition when the family history is negative for the disease
(about 20% of patients).
• Transmission of hemophilia:
Probability of Abnormality in Offspring
Female Female
Genotype of Parents Normal Carrier Hemophiliac Normal Hemophiliac
Female carrier / normal male 50% 50% 0 50% 50%
Noncarrier female/ hemophiliac male 0 100% 0 100% 0
Female carrier/hemophiliac male 0 50% 50% 50% 50%

• Altered Physiology:
• 1. Hemophilia results from the absence or malfunction of any one of the blood clotting factors from the
plasma.
• 2. These blood clooting factors are necessary for the formation of prothrombin activator, which acts as a
catalyst in the conversion of prothrombin to thrombin.
• A. The rate of formation of thrombin from prothrombin is almost directly proportional to the
amount of prothrombin activator available.
• B. The rapidity of the clotting process is proportional to the amount of thrombin formed.
• 3. The most common types of hemophilia and the clotting factors involved in the chart below:
Type of Hemophilia Clotting Factor
Hemiphilia A (Classic Hemophilia) Factor VIII (antihemophilic globulin)
Hemophilia B (Christmas disease) Factor IX (Plasma thromboplastin component)
Hemophiliac C Factor XI (Plasma thromboplastin antecedent)
Clinical (Manifestations) Signs and Symptoms:
1. Easily bruised which results to slow persistent bleeding
2. Prolonged bleeding from the mucous membranes of the nose and mouth or from lacerations.
3. Spontaneous soft-tissue hematomas
4. Hemorrhages into the joints – especially elbows, knees, and ankles (Hemarthrosis).
a. Causes pain, swelling, limitation of movement.
b. Repeated hemorrhages may produce degenerative changes with osteoporosis and muscle athrophy.
5. Spontaneous hematuria
6. Gastrointestinal bleeding
Complications:
• 1. Airway obstruction due to hemorrhage into the neck and pharynx
• 2. Intestinal obstruction due to bleeding into intestinal walls or peritoneum.
• 3. Compression of nerves with paralysis due to hemorrhaging into deep tissues.
• 4. Intracranial bleeding
• 5. Secondary complications associated with therapy – liver disease, immunologic problems, thrombotic
complications.
• Diagnostic Evaluation:
• 1. Routine bleeding and clotting tests – often normal
• 2. Partial thromboplastin time (PTT) – prolonged
• 3. Prothrombin consumption – decreased
• 4. Thromboplastin generation – increased
• 5. Specific assays for clotting factors – abnormal
c. Immune Thrombocytopenic Purpura. Is a group of bleeding disorders due to immune destruction of
platelets. Antiplatelet antibodies are produced for unknown reasons, so that the platelet life span is
 markedly shortend. Antibody-coated platelets are removed from the circulation by reticuloendothelial cells
of the spleen and liver.
• Clinical Manifestations:
• 1. History of Easy bruising
• 2. Bleeding – mild to severe (Thrombocytopenia not usually accompanied by bleeding unless the platelet
count falls accompanied by bleeding unless the platelet count falls below 20,000/mm3)
• a. Skin lesions – small red hemorrhages; do not blanch on pressure.
• b. Purpuric lesions may occur in vital organ (brain).
• c. Bleeding may occur from nose, mouth, genitourinary tract.
• Laboratory Manifestations:
• 1. Platelets may be absent or only slightly decreased in number; abnormalities may be seen in platelet
size or morphologic appearance.
• 2. Increased levels of immunoglobulins (IgG) or complement components on the platelet surface.
• 3. Bone marrow examination – bone marrow megakaryocytes are increased in numbers.
d. Idiopathic Thrombocytopenic Purpura (ITP) is an autoimmune bleeding disorder characterized by the
development of autoimmune bodies to one’s own platelets, the binding of autoantibodies to antigens, and
the destruction of platelets in the spleen and, to lesser extent, in the liver.
 Normally, Platelets survive 8 t0 10 days within the circulation.
 In ITP, platelet survival is as brief as 1 to 3 days or less.
• Clinical manifestations:
• 1. Petechia
• 2. Ecchymosis
• 3. Epistaxis
• 4. Bleeding from the gums
• 5. Easy bruising
• 6. In women may have extremely heavy menses or bleeding between periods.
• Diagnostic Findings that confirm the presence of ITP include:
• 1. A platelet count below 100,000/mm3
• 2. Prolonged bleeding time with normal coagulation time (all coagulation factors are present and normal)
• 3. Increased capillary fragility, as demonstrated by the tourniquet test.
• 4. Positive platelet antibody screening
• 5. Bone marrow aspirate containing normal or increased numbers of megakaryocytes.
• Complications of ITP include:
• 1. Cerebral hemorrhage, which proves fatal in 1% to 5% of clients with ITP,
• 2. Severe hemorrhages from the nose, GI tract, and urinary system,
• 3. Bleeding into the diaphragm, which can result in pulmonary complications; and
• 4. Nerve pain, extremity anesthesia, or paralysis resulting from the pressure of hematomas on nerves or
brain tissues.
• Medical management:
• Treatment of choice is steroids to inhibit the macrophage ingestion of the antibody-coated platelets.
Plasmapheresis is sometimes used as short-term therapy until the steroid therapy takes effect. If the
client is actively bleeding or requires surgery, IV gamma globulin can be used to increase the platelet
count.
• Spleenectomy is also recommended if the client has not have sustained remission.
• The effectiveness of spleenectomy is believed to be related to the removal of the site of premature
destruction of the antibody-sensitized platelets.
• Danazol (Danocrine) has been also used with success in some clients.
• Immunosuppressive therapy used in refractory cases includes vincristine, vinblastine (Velban),
azathioprine (Imuran), and cyclophosphamide.
e. Thrombocytopenia is a decrease in the circulating platelet count, which may result in bleeding or
hemorrhage.
• Altered Physiology and Causes:
• 1. Decreased platelet production (infiltrative diseases of bone marrow, leukemia, myelosuppressive
therapy, other tumors, myelofibrosis, radiation therapy, drug effect, aplastic anemia, etc).
• 2. Increased platelet destruction (infection, immune thrombocytopenic purpura, disseminated intrvascular
coagulation, drug-induced, etc).
• 3. Abnormal distributions or sequestration – hypersplenism.
• 4. Loss of platelets from body, (extracorporeal circulation, dilution due to blood loss and multiple blood
transfusions).
• Clinical manifestations:
• When the platelet count drops below 20,000/mm3:
• 1. Petechiae occur spontaneously.
• 2. Ecchymoses occurs at the sites of minor trauma (venipuncture, bruises).
• 3. Hematomas occur at sites of more significant trauma (surgical wounds).
• 4. Bleeding from mucosal surfaces, nose, GI, and genitourinary (GU) tracts, respiratory tract, and CNS
may occur.
• 5. Menorrhagia is common (excessive menstrual bleeding).
• 6. Excessive dental after dental extractions is seen.

• Treatment and Nursing Management

• 1. Treat the underlying disease (e.g., treat leukemia, discontinue offending drugs, etc.).
• 2. Assist with administration of platelet transfusions if platelet production is impaired; if excessive
destruction of platelets is the problem, transfused platelets will also be destroyed and will not raise the
count. Steroids are helpful in autoimmune thrombocytopenia.
• 3. Hormonal control of menstrual periods is usually carried out.
• 4. Teach the patient to avoid any maneuver that increases intracranial pressure when platelet count is
<20,000/mm3.
f. DIC (Disseminated Intravascular Coagulation) is an acquired hemorrhagic syndrome in which there is
widespread clotting in small vessels of the body, leading to consumption of the clotting factors and
platelets so the bleeding and thrombosis are occurring simultaneously. It is seen as a complication of a
variety of disorders. (Lippincott)
Disseminated Intravascular Coagulation (DIC) is a complex and important coagulation disorder
characterized by two apparently conflicting manifestations: (1) diffuse fibrin deposition within arterioles
and capillaries, with resultant widespread clotting, and (2) hemorrhage from the kidneys, brain, adrenal
glands, heart, and other organs occurs following activation of clotting factors and fibrinolytic enzymes
throughout arterioles and capillaries. DIC is often called a “Consumptive Coagulopathy” because of the
depletion of platelets.
• Etiology and Risk Factors:
• The causes of DIC are many, and there is considerable overlap among the syndromes that precede its
occurrence. Sepsis is the leading cause; up to 73% of clients with sepsis develop DIC. FOUR
CATEGORIES of causative factors are:
• 1. Introduction of tissue coagulation factors into the circulation.
• 2. Damage to vascular endothelium.
• 3. Stagnant blood flow
• 4. Infection
• Pathophysiology:
• Two pathways can initiate DIC. An extrinsic pathway of massive tissue damage due to burns or trauma or
an intrinsic pathway due to damage to the endothelium release thromboplastic substances that result in
activation of thrombin, which in turn activates fibrinogen and results in deposition of fibrin results from
increased generation of thrombin, suppression of anticoagulation mechanisms, and delayed removal of
fibrin due to impaired fibrinolysis.
• Platelet aggregation or adhesiveness is increased; this enables fibrin clots and microthrombi to form in
the brain, kidneys, heart, and other organs, causing microinfarcts and tissue necrosis. RBCs become
trapped in the fibrin strands and are destroyed (hemolysis). The resultant sluggish circulation of blood
reduces the flow of nutrients and oxygen to the cells. Platelets, prothrombin, and other clotting factors are
consumed in the process, which compromises coagulation and predisposes to bleeding.
• Excessive clotting activates the fibrinolytic mechanism, which causes the production of fibrin degradation
products. Fibrin degradation products act to inhibit platelet clotting functions, which causes further
bleeding, Ultimately, with lysis of clots and depletion of clotting factors, the blood losses its ability to clot.
• Conditions which precipitate DIC:
• Shock
• Cirrhosis
• Purpura fulminans
• Glomerulonephritis
• Acute fulminant hepatitis
• Acute bacterial and viral infections
Conditions that may cause the release of platelet factor III:
• 1. Fat embolism
• 2. Snake bite
Hemolytic process caused by:
• 1. Infection
• 2. Transfusion Reaction
• 3. Immunologic Disorders
Tissue damage caused by:
• 1. Trauma
• 2. Heat stroke
• 3. Extensive Burns
• 4. Transplant rejections
• 5. Surgery – particularly if extracorporeal circulation was used.
Conditions that may cause the release of thromboplastin from tissues:

1. Neoplastic growth
• a. Acute leukemias
• b. Prostatic cancer
 • c. Bronchogenic cancer
• d. Giant cavernous hemangioma
• 2. Obstetric conditions
• a. Abruptio placenta
• b. Retained dead fetus
• c. Amniotic fluid embolism
• Clinical Manifestations:
• The onset of DIC is usually acute and develops within days to hours after an initial assault to the body
system such as shock. Subacute DIC may not be apparent initially but may become fulminant as the
clinical course progresses. Chronic cases of DIC characteristically develop in clients with cancer or in
women carrying a dead fetus.
• Manifestations may be mild or extremely severe. They include
• 1. Purpura, petechia, and ecchymosis on the skin, mucous membranes, heart lining and
lungs;
• 2. Prlonged bleeding from venipuncture;
• 3. Severe, uncontrolled hemorrhage during surgery or childbirth;
• 4. Oliguria and acute renal failure;
• 5. Ischimia of the peripheral tissue leads to coolness and mottling of the extremities.
• 6. Convulsion and coma.
• The prognosis for clients with DIC varies.
• The condition may be self-limiting.
• On the other hand, hemorrhage, organ damage (especially acute respiratory distress syndrome), or even
death may occur within a few days or even a few hours if associated with gram-negative sepsis.
• In severe cases, the mortality rate reaches to 80%.
• Diagnostic Findings:
• Prolonged prothrombin time and activated partial thromboplastin time.
• Very low (and failing) platelet count (<100,000/mm3),
• Low plasma levels of coagulation inhibitors.
• Prolonged clotting time
• Medical management:
• To treat DIC successfully, clinicians must:
• 1. Correct the basic problem (such as infection, delivery of a fetus, surgery, or irradiation for cancer),
• 2. Reverse the pathologic clotting,
• 3. Control bleeding and shock
• 4. Detect occult bleeding
• 5. Measure blood loss,
• 6. Administer blood products and medication as prescribed, and
• 7. Observe for and report transfusion reactions and medication side effects.
• Manifestations of thrombosis are treated with IV heparin, which interrupts the clotting cascade by blocking
thrombi.
• The use of heparin is controversial and is reserved for clients with thrombosis seen in acute renal failure
to skin ischemia.
• Low dose doses are given, such as 300 to 500 units per hour by continuous infusion.
• Washed packed RBCs are administered to replace blood volume lost through hemorrhage without
administering anticoagulant substances.
• Cryoprecipitate is given for depletion of factors V and VIII.
• Administration of Antithrombin III (in fresh frozen plasma) shortens the course of the disorder and
reduces the complications of DIC.
• When bleeding cannot be controlled with heparin, aminocaproic acid (Amicar) is given.
• Nursing Management:
• Assess all body systems for the effects of DIC, including:
• 1. Integumentary bleeding or oozing of blood from venipuncture sites or mucosal surfaces and wounds,
pallor, petechiae.
• 2. Respiratory tachypnea, hemoptysis, orthopnea, and basilar rales,
• 3. Cardiovascular tachycardia and hypotension
• 4. GI abdominal distention, guaiac-positive stools or gastric contents;
• 5. Genitourinary hematuria and oliguria
• 6. Neurologic vision changes, dizziness, headache, changes in mental status, and irritability.
3. Bone marrow Transplantation (BMT) since 1970s, BMT has progressed from a treatment of last resort
to a viable therapeutic modality for a variety of hematologic, malignant, and nonmalignant disorders.
• Indications (Bone marrow transplant may be considered as a treatment for clients with the following:
• 1. Aplastic anemia;
• 2. Malignant disorders, specifically myelodysplastic syndromes, leukemia, lymphoma, multiple myeloma,
neuroblastoma, and selected solid tumors.
• 3. Nonmalignant hematologic disorders, such as Fanconi’s anemia, thalassemia, and sickle cell anemia.
• 4. Immunodeficiency disorders, such as severe combined immunodeficiency disease and Wiskott-Aldrich
syndrome.
• Sources of Bone Marrow:
• There are three types of Bone marrow donors:
• 1. Allogeneic bone marrow is obtained from a relative or unrelated donor having a close HLA (Human
Leukocyte antigen) type. This was the most common type of marrow transplant but it carried the highest
rate of morbidity and mortality because of complications of incompatibility such as GVHD (graft-versus-
host disease).
• 2. Syngeneic Bone Marrow is donated by an identical twin. Although syngeneic marrow is perfect HLA
match, which eliminates the risks of marrow rejection, the incidence of leukemic relapse is higher than
when an allogeneic donor is used because GVDH is considered to have an antileukemic effect.
• 3. Autologous Bone marrow is removed from the intended recipient during the remission phase to allow
another course of ablative therapy to be given if a relapse occurs. This relapse may be due to
contamination of the harvested bone marrow by malignant cells or to failure of pretransplant
chemotherapy to eradicate completely the tumor cells from the body.
• Siblings have a 1 in 4 chance of having identical sets of HLA antigens.
• Nonrelated clients have less than a 1 in 5000 chance of having identical HLA types.
Allogeneic donor preparation: an extensive work-up is performed for ensuring compatibility and the mental
and physical well being of the prospective donors. Evaluation includes:
• 1. Histocompatibility testing
• 2. Medical history
• 3. Physical examination
• 4. Chest film
• 5. ECG
• 6. Laboratory evaluation (CBC, chemistry profile, viral testing, rapid plasma regain test, ABO and Rh
blood typing, coagulation studies),
• 7. Psychological testing (may include psychiatric consultation).
• Bone marrow harvest, needs an informed consent, including potential donor complications, (pain, fever,
hematoma). Because of the potential for significant blood loss during the harvesting process, syngeneic
and allogeneic donors are required to donate autologous blood for reinfusion before the procedure.
• Newborns currently are being used as potential donors through the use of their cord blood, which is rich
in stem cells. Newborns cord blood are being frozen for future use.
• Marrow collection:
• When collecting marrow, the client or donor is given general or spinal anesthesia in the operating room.
The marrow is obtained in 2- to – 5 ml aliquots from the marrow spaces of the posterior and, occasionally,
anterior iliac crest and sternum. Numerous skin punctures may be required; the aspiration needle is
redirected to various marrow spaces without being withdrawn. A total of 400 to 800 ml of marrow usually
is obtained. The blood is placed in heparinized tissue culture media and filtered for removal of fat and
bone particles. Marrow can be infused immediately or frozen in a solution containing dimethyl sulfoxide
(DMSO), which preserves stem cells in the frozen state.
• Peripheral Stem Cell Collection
• Peripheral stem (progenitor) cells are harvested by Apheresis or Leukapheresis, a process that
removes blood through a large-bore catheter and runs it through a machine that removes the stem cells
before running the blood to the client. Because stem cells concentration is much lower in peripheral blood
compared with bone marrow, a process to increase the concentration in the peripheral blood must be
initiated first. To increase the number of circulating stem cells, a stimulus, such as a Colony Stimulating
Factor (CSF),interleukins (ILs), fusion molecules (made from a combination of a CSF and IL-3), or some
chemotherapeutic agents, may be given to the donor before the stem cell harvest. As mentioned earlier,
the umbilical cord of newborns also is rich in stem cells.
• Once the stem cells are harvested, they are preserved in the manner as bone marrow. The engraftment
of stem cells occurs at approximately the same rate as or slightly fastere than with marrow
transplantation.

ALLOGENEIC TRANSPLANT:
• Recipient Preparation:
• The physical and psychological evaluation of the recipient is similar to that of the donor. Additional testing
may be required to stage existing disease accurately. The recipient must undergo a preparative regimen
before transplantation. Such a regimen serves three purposes:
• 1. Malignant cells are destroyed.
• 2. The immune system is inactivated, which reduces the risk of GVHD in allogeneic transplant clients.
• 3. The marrow cavities are emptied to provide space for implantation of the transfused stem cells.
• Common protocols combine total body irradiation and high doses of a single chemotherapeutic agent
(cyclophosphamide is one of the most common agents used) or fractionated/high doses of multiple
agents. A multilumen central venous catheter is inserted to provide suitable access for marrow infusion as
well as for antibiotics, blood products, hyperalimentation, and frequent blood sampling.
Bone Marrow Infusion:
• The infusion of the marrow is commonly anticlimactic after the client has undergone the rigorous
preparatory chemotherapy and radiation therapy (often referred to as the Conditioning regimen). The
marrow usually is administered immediately after the conditioning regimen is complete. Marrow is
 administered from a large blood infusion bag by a multilumen catheter, using an infusion pump, or small
volumes may be prefiltered and given by IV push by a physician.
• The BMT client remains pancytopenic until the transplanted stem cells make their way to the medullary
cavities, where subsequent growth and reconstitution of the marrow are confirmed. Indications of
successful engraftment are an increase in platelets and RBCs in peripheral blood count. This change may
occur 14 days after marrow infusion. Each day that recovery is delayed places the client at added risk.
Graft rejection is evident if the bone marrow fails to produce peripheral blood cells after several weeks.
• Nursing Mangement of BMT clients follows the plan of care for any completely immunosuppressed
client. Clients receiving allogeneic transplant must be observed closely for manifestations of GVHD.
Potential immediate adverse reactions are allergic (urticaria, chills, fever), volume overload, and
pulmonary complications secondary to fat emboli. Renal damage may occur from too many erythrocytes.
The period immediately after transplant is crucial. Multisystem failure related to ablative therapy is
common, as are immune reactions caused by the transplanted cells.
• The most common and disastrous complication of allogeneic BMT is GVHD, which may occur 7 to
30 days after infusion of viable lymphocytes. The donor T lymphocytes form an immunologic reaction
against the host cells. The clients at highest risk for development of GVHD are those who have had
allogeneic BMT. But moderate risk is noted to clients with HLA-identical donors.

ANEMIA, APLASTIC
 bone marrow hypoplasia or aplasia resulting in pancytopenia (decreased WBC, RBC and
platelets)
 Aplastic anemia is a normocytic-normochromic anemia that results from a loss of blood cell precursors,
causing hypoplasia of bone marrow, RBCs, WBCs, and platelets. Symptoms result from severe anemia,
thrombocytopenia (petechiae, bleeding), or leukopenia (infections). Diagnosis requires demonstration of
peripheral pancytopenia and the absence of cell precursors in bone marrow. Treatment is equine
antithymocyte globulin and cyclosporine. Erythropoietin, granulocyte-macrophage colony-stimulating factor,
and bone marrow transplantation may also be useful.
 The term aplastic anemia commonly implies a panhypoplasia of the marrow
with associated leukopenia and thrombocytopenia. In contrast, pure RBC
aplasia is restricted to the erythroid cell line. Although both disorders are
uncommon, aplastic anemia is more common.

Etiology
True aplastic anemia (most common in adolescents and young adults) is idiopathic in about ½ of cases. Recognized causes are
chemicals (eg, benzene, inorganic arsenic), radiation, and drugs (eg, antineoplastic drugs, antibiotics, NSAIDs, anticonvulsants,
acetazolamide (Diamox), , gold salts, penicillamine (Cuprimine), , quinacrine) . The mechanism is unknown, but selective
(perhaps genetic) hypersensitivity appears to be the basis.
Fanconi's anemia is a very rare, familial form of aplastic anemia with bone abnormalities, microcephaly, hypogonadism, and
brown pigmentation of skin. It occurs in children with abnormal chromosomes. Fanconi's anemia is often inapparent until some
illness (especially an acute infection or inflammatory disorder) supervenes, causing peripheral cytopenias. With clearing of the
supervening illness, peripheral values return to normal despite reduced marrow mass.

Pure RBC aplasia may be acute and reversible. Acute erythroblastopenia is a brief disappearance of RBC precursors from the
marrow during various acute viral illnesses (particularly human parvovirus infection), especially in children. The anemia lasts
longer than the acute infection. Chronic pure RBC aplasia has been associated with hemolytic disorders, thymomas, and
autoimmune mechanisms and, less often, with drugs (eg, tranquilizers, anticonvulsants), toxins (organic phosphates), riboflavin
deficiency, and chronic lymphocytic leukemia. A rare congenital form, Diamond-Blackfan anemia, usually occurs during infancy
but has also been reported in adulthood. Diamond-Blackfan anemia is associated with bony abnormalities of the thumbs or digits
and short stature.

Symptoms and Signs

Although onset of aplastic anemia usually is insidious, often occurring over weeks or months after exposure to a toxin,
occasionally it is acute. Signs vary with the severity of the pancytopenia. Symptoms and signs of anemia (eg, pallor) usually are
severe.

Severe thrombocytopenia may cause petechiae, ecchymosis, and bleeding from the gums, into the conjunctivae, or other
tissues. Agranulocytosis commonly causes life-threatening infections. Splenomegaly is absent unless induced by transfusion
hemosiderosis. Symptoms of pure RBC aplasia are generally milder and relate to the degree of the anemia or to the underlying
disorder.

Diagnosis
• CBC

• Bone marrow examination

Aplastic anemia is suspected in patients, particularly young patients, with pancytopenia (eg, WBC < 1500/μL, platelets <
50,000/μL). Pure RBC aplasia (including Diamond-Blackfan anemia) is suspected in patients with bony abnormalities and
normocytic anemia but normal WBC and platelet counts. If either diagnosis is suspected, bone marrow examination is done.

In aplastic anemia, RBCs are normochromic-normocytic (sometimes marginally macrocytic). The WBC count reduction occurs
chiefly in the granulocytes. Platelets are often far below 50,000/μL. Reticulocytes are decreased or absent. Serum iron is
elevated. The bone marrow is acellular. In pure RBC aplasia, normocytic anemia, reticulocytopenia, and elevated serum iron are
present, but with normal WBC and platelet counts. Bone marrow cellularity and maturation may be normal except for absence of
erythroid precursors.

Treatment
• Equine antithymocyte globulin, corticosteroids, and cyclosporine (Sandimmune)

• Sometimes hematopoietic cell transplantation

• Sometimes cytokines

• Sometimes surgery in thymoma-associated RBC aplasia

In aplastic anemia, treatment of choice is equine antithymocyte globulin (ATG) 10 to 20 mg/kg diluted in 500 mL saline and
infused IV over 4 to 6 h once/day for 10 consecutive days. Shorter regimens are also used. About 60% of patients respond to
ATG. Allergic reactions and serum sickness may occur; some experts advocate skin testing (to identify allergy to horse serum)
and concomitant corticosteroids ( prednisone), (Delatasone), 40 mg/m2 po once/day beginning on day 7 for 10 days or until
symptoms subside). Cyclosporine(5 to 10 mg/kg po once/day) is as effective as ATG and yields responses in about 50% of ATG
failures, suggesting that its mechanism of action is different. Combined ATG and cyclosporine is also effective. If aplastic anemia
is very severe or fails to respond to ATG and cyclosporine
, bone marrow transplantation or treatment with cytokines (erythropoietin, granulocyte or granulocyte-macrophage colony-
stimulating factor) may be effective (Deltasone), cyclosporine, or cyclophosphamide (Cytoxan).

Hematopoietic cell transplantation may help younger patients (particularly patients < 30) but requires an identical twin or an HLA-
compatible sibling. At diagnosis, siblings are evaluated for HLA compatibility. Because transfusions pose a risk to subsequent
transplantation, blood products are used only when essential.

Pure RBC aplasia has been successfully managed with immunosuppressants ( prednisone), or cyclophosphamide
(Cytoxan).especially when an autoimmune mechanism is suspected. Because patients with thymoma-associated pure RBC
aplasia improve after thymectomy, CT is used to seek the presence of such a lesion, and surgery is considered.

IM: Abnormal bleeding

Lab. Data: Decreased RBC, WBC and platelet count
Nsg. Dx: Risk for infection, Risk for injury
Interventions:
 Prepare the patient for bone marrow transplant.
 Assess for signs and symptoms of bleeding.

ANEMIA, FOLIC ACID DEFICIENCY

 depletion of folate, which results to progressive anemia.
Folic acid is known by a number of names (see Folacin). More commonly today, it is called
folacin; but, as far as anemia is concerned, the two most popular names still used are “folic
acid deficiency anemia” and “folate deficiency anemia.”
This type is a common, slowly progressive, megaloblastic anemia characterized by red blood cells that
are larger than normal and hence the term macrocytic when referring to this type. The red blood cells
are also deformed, and both their rate of production and their lifespan are diminished. Folic acid anemia
occurs most often in infants, adolescents, pregnant and lactating females, alcoholics, the elderly, and in
those with malignant or intestinal diseases.
Folic acid is needed for the orderly production of deoxyribonucleic acid (DNA) in all tissue cells and is a
component of three of the four DNA bases -- thymine, adenine, and guanine -- (the fourth is cytosine). In
bone marrow, it is required for the normal production of the red blood cells and for RNA synthesis. Folic
acid circulates through and is stored in the liver and a deficiency is almost always because of insufficient
amounts in the diet.
Absorption of folic acid occurs primarily in the upper small intestine and does not depend on intrinsic
factor as vitamin B12 does. A deficiency of folic acid is more common than a cobalamin (B12)
deficiency. Folic acid stores are also depleted more rapidly than cobalamin stores and, without proper
dietary intake, a megaloblastic anemia will develop.
Clinical manifestations of folic acid anemia are similar to those of pernicious anemia except for the lack
of neurologic symptoms common in a B12 deficiency. Evaluation is based on blood tests, measurement
of serum folate levels, and signs and symptoms. Diagnosis is made following the Schilling test and a
therapeutic trial of vitamin B12 injections to distinguish between folic acid deficiency anemia and
pernicious anemia. Significant blood test findings include macrocytosis, decreased reticulocyte count,
abnormal platelets, and a serum folate less than 4 mg/ml.
The elderly are particularly at risk for developing this type of anemia as their diets often wane for one
reason or another, including a lack of interest in food, poverty, immobility, and/or ill-fitting dentures.
Interestingly, when folate supplements are given to the elderly, good medical supervision must be
undertaken as folate supplements can mask the megaloblastic anemia of B12 deficieny. Since the
elderly are already at risk for B12 deficiency, giving folate supplementation by mistake, or by design, can
aggravate an additional problem of B12 deficiency.
Alcohol abuse also contributes to this type of anemia since alcohol interferes with folate metabolism in
the liver, resulting in a profound depletion of folate stores. Patients with neoplastic diseases and such
skin diseases as chronic exfoliative dermatitis are also are at risk for folic acid anemia.
Folic acid deficiency anemia is common during pregnancy. Both folate and iron are essential for red cell
production and during pregnancy there is an increased need to supply both the mother and the
developing infant(s). This type of anemia is common in newborns because of the increasing survival
rates of premature infants. Not only can it be a danger to the mother, but also contributes to fetal
malformations. The most common birth defect resulting from a deficiency of this vitamin is spina bifida.
During the 1980s, a considerable body of evidence accumulated stating that spina bifida and other
neural tube defects were associated with folate deficiency. It is now widely recognized that folate
supplements are necessary and best started before pregnancy occurs since closure of the neural tube
occurs by day 28 of pregnancy. This is generally long before the woman knows she is pregnant. It was
also established that receiving enough folate from fortified foods was nearly impossible and supplements
were highly recommended. Even though the studies successfully used the folate monoglutamate form of
folic acid, it is pteroylmonoglutamic acid form that is used in vitamin supplements and fortified foods.
Most naturally occurring folates are pteroylpolyglutamates.
According to one source, adding folate to foods may even be dangerous if vitamin B12 is not also
included (see http://www.victorherbert.com/cv812.htm). Folate supplements will mask the megaloblastic
anemia of vitamin B12 deficiency and may hasten the development of irreversible nerve damage. This is
especially a problem of the elderly, who may already suffer from an impaired absorption of vitamin B12.
The addition of relatively large amounts of B12 to foods, as well as folate, would permit passive
absorption of adequate amounts of vitamin B12 to prevent deficiency from developing.
Folic acid deficiency is also common in tropical areas where poverty results in a poor diet. In North
America and other regions of the world where access to food is rarely a problem, folic acid deficiency
still occurs because dietary needs are not met, especially during the growth of children and adolescents
and during pregnancy. These age groups are more prone to folic acid deficiency anemia because of
their heavy use of folate-deficient cow's milk, which also inhibits the absorption of iron, causing an
additional risk of iron-deficiency anemia as well.
Causes of folic acid deficiency anemia include:

• alcohol abuse (alcohol prevents absorption of several nutrients especially the B

vitamins)
 • poor diets (common in alcoholics, the elderly, those living alone or in poverty, and
infants, especially those with infections or diarrhea)
• impaired absorption because of intestinal dysfunction from such disorders as celiac
disease, tropical sprue, regional jejunitis, Crohn's disease, or bowel resection
• bacteria competing for available folic acid
• overcooking of food, destroying valuable water-soluble nutrients, including a high
percentage of folic acid
• limited storage capacity in infants
• prolonged drug therapy, especially from anticonvulsants and estrogens
• not addressing increased folic acid needs of certain age groups, as well as in
patients with neoplastic diseases and some skin disorders (eg. chronic exfoliative
dermatitis).

Signs and symptoms of folic acid deficiency anemia gradually produces clinical features
similar to other megaloblastic anemias, but without neurologic manifistations of B12
deficiency. Symptoms include the following:

• progressive fatigue
• shortness of breath
• heart palpitations
• weakness
• glossitis (inflammation of the tongue)
• nausea
• anorexia
• headache
• fainting
• irritability
• forgetfulness
• pallor
• slight jaundice

Conventional treatment consists primarily of folic acid supplements (about 400 mg. three times daily)
and, more importantly, the elimination of contributing causes. Oral administrations of folate preparations
are 1 to 5 mg daily. Prophylactic doses are given in pregnancy or those considering getting pregnant.
Parenteral administration of folic acid can relieve acute symptoms within 48 hours. Blood transfusions
are given to treat severe cardiac or respiratory distress as a result of severe deficiency.

IM; Fatigue
Lab. Data: Decreased folate levels
Nsg. Dx: Activity intolerance
Interventions:
 Teach the patient to increase sources of folic acid in the diet like: green vegetables (asparagus,
broccoli and spinach), yeast, liver, organ meats and fresh fruits.
 Avoid overcooking of vegetables.
 Teach the patient regarding oral folic acid replacement.

ANEMIA, IRON DEFICIENCY(Anemia of Chronic Blood Loss; Chlorosis)

 decreased oxygen carrying capacity of the blood. The condition is usually associated with
nutritional deficiency of iron.

Iron deficiency is the most common cause of anemia and usually results from blood loss. Symptoms are usually nonspecific.
RBCs tend to be microcytic and hypochromic, and iron stores are low as shown by low serum ferritin and low serum iron levels
with high serum total iron binding capacity. If the diagnosis is made, occult blood loss is suspected. Treatment involves iron
replacement and treatment of the cause of blood loss.

Pathophysiology
Iron is distributed in active metabolic and storage pools. Total body iron is about 3.5 g in healthy men and 2.5 g in women; the
difference relates to women's smaller body size, lower androgen levels, and dearth of stored iron because of iron loss with
menses and pregnancy. The distribution of body iron in an average man is Hb, 2100 mg; ferritin, 700 mg (in cells and plasma);
hemosiderin, 300 mg (in cells); myoglobin, 200 mg; tissue (heme and nonheme) enzymes, 150 mg; and transport-iron
compartment, 3 mg.

Iron absorption: Iron is absorbed in the duodenum and upper jejunum. Absorption of iron is determined by the type of iron
molecule and by what other substances are ingested. Iron absorption is best when food contains heme iron (meat). Dietary
nonheme iron must be reduced to the ferrous state and released from food binders by gastric secretions. Nonheme iron
absorption is reduced by other food items (eg, vegetable fiber phytates and polyphenols; tea tannates, including
phosphoproteins; bran) and certain antibiotics (eg, tetracycline SOME TRADE NAMES
ACHROMYCIN V
TETRACYN
TETREX
Click for Drug Monograph
). Ascorbic acid is the only common food element known to increase nonheme iron absorption.

The average American diet, which contains 6 mg of elemental iron/kcal of food, is adequate for iron homeostasis. Of about 15
mg/day of dietary iron, adults absorb only 1 mg, which is the approximate amount lost daily by cell desquamation from the skin
and intestines. In iron depletion, absorption increases, although the exact signaling mechanism is not known; however,
absorption rarely increases to > 6 mg/day unless supplemental iron is added. Children have a greater need for iron and appear to
absorb more to meet this need.

Iron transport and usage: Iron from intestinal mucosal cells is transferred to transferrin, an iron-transport protein synthesized in
the liver; transferrin can transport iron from cells (intestinal, macrophages) to specific receptors on erythroblasts, placental cells,
and liver cells. For heme synthesis, transferrin transports iron to the erythroblast mitochondria, which insert the iron into
protoporphyrin for it to become heme. Transferrin (plasma half-life, 8 days) is extruded for reutilization. Synthesis of transferrin
increases with iron deficiency but decreases with any type of chronic disease.

Iron storage and recycling: Iron not used for erythropoiesis is transferred by transferrin, an iron transporting protein, to the
storage pool which has 2 forms, ferritin and hemosiderin.The most important is ferritin (a heterogeneous group of proteins
surrounding an iron core), which is a soluble and active storage fraction located in the liver (in hepatocytes), bone marrow, and
spleen (in macrophages); in RBCs; and in serum. Iron stored in ferritin is readily available for any body requirement. Circulating
(serum) ferritin level parallels the size of the body stores (1 ng/mL=8 mg of iron in the storage pool).The 2nd storage pool of iron
is in hemosiderin, which is relatively insoluble and is stored primarily in the liver (in Kupffer cells) and in the marrow (in
macrophages).

Because iron absorption is so limited, the body recycles and conserves iron. Transferrin grasps and recycles available iron from
aging RBCs undergoing phagocytosis by mononuclear phagocytes. This mechanism provides about 97% of the daily iron needed
(about 25 mg of iron). With aging, iron stores tend to increase because iron elimination is slow.

Iron deficiency: Deficiency develops in stages. In the first stage, iron requirement exceeds intake, causing progressive depletion
of bone marrow iron stores. As stores decrease, absorption of dietary iron increases in compensation. During later stages,
deficiency impairs RBC synthesis, ultimately causing anemia.

Severe and prolonged iron deficiency also may cause dysfunction of iron-containing cellular enzymes.

Etiology
Because iron is poorly absorbed, dietary iron barely meets the daily requirement for most people. Even so, people who eat a
typical Western diet are unlikely to become iron deficient solely as a result of dietary deficiency. However, even modest losses,
increased requirements, or decreased intake readily produces iron deficiency.

Blood loss is almost always the cause. In men, the most frequent cause is chronic occult bleeding, usually from the GI tract.In
premenopausal women, cumulative menstrual blood loss (mean, 0.5 mg iron/day) is a common cause. Another possible cause of
blood loss in men and women is chronic intravascular hemolysis (see Anemias Caused by Hemolysis) if the amount of iron
released during hemolysis exceeds the haptoglobin-binding capacity.Vitamin C deficiency can contribute to iron deficiency
anemia by producing capillary fragility, hemolysis, and bleeding.

Increased iron requirement may contribute to iron deficiency. From birth to age 2 and during adolescence, when rapid growth
requires a large iron intake, dietary iron often is inadequate. During pregnancy, the fetal iron requirement increases the maternal
iron requirement (mean, 0.5 to 0.8 mg/day—see Pregnancy Complicated by Disease: Anemia in Pregnancy) despite the absence
of menses. Lactation also increases the iron requirement (mean, 0.4 mg/day).

Decreased iron absorption can result from gastrectomy and upper small-bowel malabsorption syndromes. Rarely, absorption is
decreased by dietary deprivation from undernutrition.

Symptoms and Signs

Most symptoms of iron deficiency are due to anemia. Such symptoms include fatigue, loss of stamina, shortness of breath,
weakness, dizziness, and pallor. Fatigue also may result from dysfunction of iron-containing cellular enzymes.

In addition to the usual manifestations of anemia, some uncommon symptoms occur in severe iron deficiency. Patients may have
pica, an abnormal craving to eat substances (eg, ice, dirt, paint). Other symptoms of severe deficiency include glossitis, cheilosis,
concave nails (koilonychia), and, rarely, dysphagia caused by a postcricoid esophageal web (Plummer-Vinson syndrome).

Diagnosis
• CBC, serum iron, iron-binding capacity, and serum ferritin
• Rarely, bone marrow examination

Iron deficiency anemia is suspected in patients with chronic blood loss or microcytic anemia, particularly if pica is present. In such
patients, CBC, serum iron and iron-binding capacity, and serum ferritin are obtained.

Iron and iron-binding capacity (or transferrin) are usually both measured, because their relationship is important. Various tests
exist; the range of normal values relates to the test used.In general, normal serum iron is 75 to 150 μg/dL (13 to 27 μmol/L) for
men and 60 to 140 μg/dL (11 to 25 μmol/L) for women; total iron-binding capacity is 250 to 450 μg/dL (45 to 81 μmol/L). Serum
iron level is low in iron deficiency and in many chronic diseases and is elevated in hemolytic disorders and in iron-overload
syndromes (see Iron Overload). Patients taking oral iron may have normal serum iron despite a deficiency; in such
circumstances, a valid test requires cessation of iron therapy for 24 to 48 h. The iron-binding capacity increases in iron
deficiency. Serum transferrin receptor levels reflect the amount of RBC precursors available for active proliferation; levels are
sensitive and specific. The range of normal is 3.0 to 8.5 μg/mL. Levels increase in early iron deficiency and with increased
erythropoiesis.

Serum ferritin levels closely correlate with total body iron stores. The range of normal in most laboratories is 30 to 300 ng/mL,
and the mean is 88 in men and 49 in women. Low levels (< 12 ng/mL) are specific for iron deficiency. However, ferritin is an
acute-phase reactant, and levels increase in inflammatory and neoplastic disorders, so ferritin may also be elevated in cases of
liver injury (eg, hepatitis) and in some tumors (especially acute leukemia, Hodgkin lymphoma, and GI tract tumors). The most
sensitive and specific criterion for iron-deficient erythropoiesis, however, is absent marrow stores of iron, although a bone marrow
examination is rarely needed.

Stages of iron deficiency: Laboratory test results help stage iron deficiency anemia.

Stage 1 is characterized by decreased bone marrow iron stores; Hb and serum iron remain normal, but serum ferritin level falls to
< 20 ng/mL. The compensatory increase in iron absorption causes an increase in iron-binding capacity (transferrin level).

During stage 2, erythropoiesis is impaired. Although the transferrin level is increased, the serum iron level decreases; transferrin
saturation decreases. Erythropoiesis is impaired when serum iron falls to < 50 μg/dL (< 9 μmol/L) and transferrin saturation to <
16%. The serum ferritin receptor level rises (> 8.5 mg/L).
During stage 3, anemia with normal-appearing RBCs and indices develops.

During stage 4, microcytosis and then hypochromia develop.

During stage 5, iron deficiency affects tissues, resulting in symptoms and signs.

Diagnosis of iron deficiency anemia prompts consideration of its cause, usually bleeding. Patients with obvious blood loss (eg,
women with menorrhagia) may require no further testing. Men and postmenopausal women without obvious blood loss should
undergo evaluation of the GI tract, because anemia may be the only indication of an occult GI cancer. Rarely, chronic epistaxis or
GU bleeding is underestimated by the patient and requires evaluation in patients with normal GI study results.

Other microcytic anemias: Iron deficiency anemia must be differentiated from other microcytic anemias (see Table 1: Anemias
Caused by Deficient Erythropoiesis: Differential Diagnosis of Microcytic Anemia Due to Decreased RBC Production ). If tests
exclude iron deficiency in patients with microcytic anemia, then anemia of chronic disease, structural Hb abnormalities (eg,
hemoglobinopathies), and congenital RBC membrane abnormalities are considered. Clinical features, Hb studies (eg, Hb
electrophoresis and HbA2), and genetic testing (eg, α-thalassemia) may help distinguish these entities.

Treatment
• Oral supplemental iron
• Rarely, parenteral iron

Iron therapy without pursuit of the cause is poor practice; the bleeding site should be sought even in cases of mild anemia.

Iron can be provided by various iron salts (eg, ferrous sulfate SOME TRADE NAMES
FEOSOL
FER-GEN-SOL
FER-IN-SOL
Click for Drug Monograph
, gluconate, fumarate) or saccharated iron po 30 min before meals (food or antacids may reduce absorption). A typical initial dose
is 60 mg of elemental iron (eg, as 325 mg of ferrous sulfate SOME TRADE NAMES
FEOSOL
FER-GEN-SOL
FER-IN-SOL
Click for Drug Monograph
) given 1 or 2 times/day. Larger doses are largely unabsorbed but increase adverse effects, especially constipation. Ascorbic acid
either as a pill (500 mg) or as orange juice when taken with iron enhances iron absorption without increasing gastric distress.
Parenteral iron causes the same therapeutic response as oral iron but can cause adverse effects, such as anaphylactoid
reactions, serum sickness, thrombophlebitis, and pain. It is reserved for patients who do not tolerate or who will not take oral iron
or for patients who steadily lose large amounts of blood because of capillary or vascular disorders (eg, hereditary hemorrhagic
telangiectasia). The dose of parenteral iron is determined by a hematologist. Oral or parenteral iron therapy should continue for ≥
6 mo after correction of Hb levels to replenish tissue stores.

The response to treatment is assessed by serial Hb measurements until normal RBC values are achieved. Hb rises little for 2 wk
but then rises 0.7 to 1 g/wk until near normal, at which time rate of increase tapers. Anemia should be corrected within 2 mo. A
subnormal response suggests continued hemorrhage, underlying infection or cancer, insufficient iron intake, or, very rarely,
malabsorption of oral iron.

IM: Easy fatigability; poor sucking (infants) chubby but pale babies (milk babies)
Lab. Data: Decreased Hgb and Hct; microcytic, hypochromic RBC’s.
Nsg. Dx: Activity Intolerance
Interventions:
 Instruct the patient to have frequent rest periods.
 Increase iron in the diet (organ meat, egg yolk)
 Milk is a poor source of iron. Administer oral iron supplement as ordered.

ANEMIA, PERNICIOUS

Other names:
Macrocytic achylic anemia; Congenital pernicious anemia; Juvenile pernicious anemia; Vitamin B12
deficiency (malabsorption)
Definition Return to top
Pernicious anemia is a decrease in red blood cells that occurs when the body cannot properly absorb vitamin
B12 from the gastrointestinal tract. Vitamin B12 is necessary for the formation of red blood cells.
Pernicious anemia is a type of megaloblastic anemia.
See also: Anemia
Causes Return to top
Pernicious anemia is caused by a lack of intrinsic factor. Intrinsic factor is a protein produced by the stomach
that helps the body absorb vitamin B12. When stomach does not have enough intrinsic factor, it cannot
properly absorb the vitamin. Nerve and blood cells need vitamin B12 to function properly.
Very rarely, infants and children are born without the ability to produce enough intrinsic factor. Congenital
(born with) pernicious anemia is inherited as an autosomal recessive disorder (you need the defective gene
from each parent to get it). Most often, however, pernicious anemia and other forms of megaloblastic anemia
in children result from other causes of vitamin B12 deficiency or other vitamin deficiencies.
Other causes of low levels of intrinsic factor (and thus of pernicious anemia) include atrophic gastric mucosa,
autoimmunity against gastric parietal cells, and autoimmunity against intrinsic factor.
The onset of the disease is slow and may take decades. Although the congenital form occurs in children,
pernicious anemia usually does not appear before age 30. The average age at diagnosis is 60.
Risk factors include:

• Family history of the disease

• Scandinavian or Northern European descent
• History of autoimmune endocrine disorders, including type 1 diabetes, hypoparathyroidism, Addison's
disease, hypopituitarism, testicular dysfunction, Graves disease, chronic thyroiditis, myasthenia
gravis, secondary amenorrhea, and vitiligo

In addition to pernicious anemia, other causes of vitamin B12 deficiency include:

• Certain drugs, including colchicine, neomycin, and para amino salicylic acid used for tuberculosis
treatment
• Gastrointestinal disease (stomach removal surgery, celiac disease, Crohn's disease)
• Infection (intestinal parasites, bacterial overgrowth)
• Metabolic disorders (methylmalonic aciduria, homocystinuria)
• Nutritional problems (strict vegetarians without B12 supplementation, poor diet in infant, or poor
nutrition during pregnancy)

Symptoms Return to top

Too little vitamin B12 gradually causes neurological problems. It is important to know that the neurological
effects may be seen before anemia is diagnosed.
Symptoms may include:

• Shortness of breath
• Fatigue
• Pallor
• Rapid heart rate
• Loss of appetite
• Diarrhea
• Tingling and numbness of hands and feet
• Sore mouth
• Unsteady gait, especially in the dark
• Tongue problems
• Impaired sense of smell
• Bleeding gums
• Positive Babinski's reflex
• Loss of deep tendon reflexes
• Personality changes, "megaloblastic madness"
Exams and Tests Return to top
Tests that may used to diagnose or monitor pernicious anemia include:

• Complete blood count

• Bone marrow examination (only needed if diagnosis is unclear)
• Measurement of serum holotranscobalamin II
• Measurement of methylmalonic acid (MMA)
• Reticulocyte count
• Serum LDH
• Schilling test
• Vitamin B12 level

This disease may also alter the results of the following tests:

• Bilirubin
• Cholesterol test
• Gastrin
• Leukocyte alkaline phosphatase
• Peripheral smear
• TIBC

Vitamin B12 deficiency affects the appearance of all epithelial cells, therefore an untreated woman may have
a false positive pap smear.
Treatment Return to top
Monthly vitamin B12 injections are prescribed to correct the vitamin B12 deficiency. This therapy corrects the
anemia and may correct the neurological complications if taken soon enough.
Some doctors recommend that elderly patients with gastric atrophy take vitamin B12 supplements by mouth
in addition to monthly injections.
There is also a preparation of vitamin B12 that may be given through the nose).
A well-balanced diet is essential to provide other elements for healthy blood cell development, such as folic
acid, iron, and vitamin C.
Outlook (Prognosis) Return to top
The outcome is usually excellent with treatment.
Possible Complications Return to top
People with pernicious anemia may have gastric polyps and get gastric cancer and gastric carcinoid tumors
twice as often than the normal population.
Persistent neurological defects may be present if treatment is delayed.

 reduced Vitamin B12 absorption due to the absence of the intrinsic factor, usually related to
gastrectomy and atrophy of the gastric mucosa.

IM: Fatigue; beefy red tongue or glossitis is a common sign.

Lab. Data: Schilling’s Test reveals more than 40% urinary excretion of ingested Vitamin B12.
Nsg. Dx: Activity Intolerance
Interventions:
 Teach the patient that monthly IM Vitamin B12 replacement therapy is lifelong.
 Inform the patient to report tingling sensation in the lower extremities as this indicates a complication
(Peripheral Neuropathy)

DISSEMINATED INTRAVASCULAR COAGULATION (Consumption Coagulopathy; Defibrination

Syndrome)
Disseminated intravascular coagulation (DIC) involves abnormal, excessive generation of thrombin and fibrin in the circulating blood. During the process,

increased platelet aggregation and coagulation factor consumption occur. DIC that evolves slowly (over weeks or months) causes primarily venous

thrombotic and embolic manifestations; DIC that evolves rapidly (over hours or days) causes primarily bleeding. Severe, rapidly evolving DIC is diagnosed

by demonstrating thrombocytopenia, an elevated PTT and PT, increased levels of serum fibrin degradation products, and a decreasing plasma fibrinogen

level. Treatment includes correction of the underlying cause and replacement of platelets, coagulation factors (in fresh frozen plasma), and fibrinogen (in

cryoprecipitate) to control severe bleeding. Heparin is used as therapy (or prophylaxis) in patients with slowly evolving DIC who have (or are at risk for)

venous thromboembolism.
Etiology and Pathophysiology
DIC usually results from exposure of tissue factor to blood, initiating the coagulation cascade (see Fig. 2: Hemostasis: Fibrinolytic pathway. ). DIC occurs

in the following clinical circumstances:

• Complications of obstetrics—eg, abruptio placentae, saline-induced therapeutic abortion, retained dead fetus or products of conception, or
amniotic fluid embolism. Placental tissue with tissue factor activity enters or is exposed to the maternal circulation.
• Infection, particularly with gram-negative organisms. Gram-negative endotoxin causes generation of tissue factor activity in phagocytic,
endothelial, and tissue cells.
• Malignancy, particularly mucin-secreting adenocarcinomas of the pancreas and prostate and acute promyelocytic leukemia, in which tumor cells
expose or release tissue factor activity.
• Shock from any cause that produces ischemic tissue injury and tissue factor exposure.

Less common causes of DIC include severe tissue damage from head trauma, burns, frostbite, or gunshot wounds; complications of prostate surgery that

allow prostatic material with tissue factor activity (along with plasminogen activators) to enter the circulation; venomous snake bites in which enzymes enter

the circulation that activate one or several coagulation factors and generate thrombin or directly convert fibrinogen to fibrin; profound intravascular hemolysis;

and aortic aneurysms or cavernous hemangiomas (Kasabach-Merritt syndrome) associated with vessel wall damage and areas of blood stasis.

Slowly evolving DIC primarily causes venous thromboembolic manifestations (eg, deep venous thrombosis, pulmonary embolism), although occasionally

cardiac valve vegetations occur; abnormal bleeding is uncommon. In contrast, in severe, rapidly evolving DIC, thrombocytopenia and depletion of plasma

clotting factors and fibrinogen cause bleeding. Bleeding into organs, along with microvascular thromboses, may cause hemorrhagic tissue necrosis in

multiple organs.

Symptoms and Signs

In slowly evolving DIC, symptoms of venous thrombosis and pulmonary embolism may be present.

In severe, rapidly evolving DIC, skin puncture sites (eg, IV or arterial punctures) bleed persistently, ecchymoses form at sites of parenteral injections, and

serious GI bleeding may occur. Delayed dissolution of fibrin polymers by fibrinolysis may result in the mechanical disruption of RBCs and mild intravascular

hemolysis (see Thrombocytopenia and Platelet Dysfunction: Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic-Uremic Syndrome (HUS)).

Occasionally, microvascular thrombosis and hemorrhagic necrosis produce dysfunction and failure in multiple organs.

Diagnosis
DIC is suspected in patients with unexplained bleeding or venous thromboembolism, especially if a predisposing condition exists. If DIC is suspected, platelet

count, PT, PTT, plasma fibrinogen level, and plasma D-dimer (an indication of in vivo fibrin deposition and degradation) are obtained.

Slowly evolving DIC produces mild thrombocytopenia, a normal to minimally prolonged PT (results are typically reported as INR) and PTT, a normal or

moderately reduced fibrinogen level, and an increased plasma D-dimer level. Because a variety of illnesses stimulate increased synthesis of fibrinogen as an

acute-phase reactant, a declining fibrinogen level on 2 consecutive measurements can help make the diagnosis of DIC.

Severe, rapidly evolving DIC results in more severe thrombocytopenia, more prolonged PT and PTT, rapidly declining plasma fibrinogen concentrations, and

a high plasma D-dimer level.

A factor VIII level can sometimes be helpful if severe, acute DIC must be differentiated from massive hepatic necrosis, which can produce similar

abnormalities in coagulation studies. The factor VIII level is elevated in hepatic necrosis, because factor VIII is made in hepatocytes and released as they are

destroyed; factor VIII is reduced in DIC because of the thrombin-induced generation of activated protein C, which proteolyses factor VIII.

Treatment
Immediate correction of the underlying cause is the priority (eg, broad-spectrum antibiotic treatment of suspected gram-negative sepsis, evacuation of the

uterus in abruptio placentae). If treatment is effective, DIC should subside quickly. If bleeding is severe, adjunctive replacement therapy is indicated,

consisting of platelet concentrates to correct thrombocytopenia; cryoprecipitate to replace fibrinogen and factor VIII; and fresh frozen plasma to increase

levels of other clotting factors and natural anticoagulants (antithrombin, proteins C and S). The effects of infusion of concentrates of antithrombin or activated

protein C in severe, rapidly evolving DIC are under study.

Heparin SOME TRADE NAMES

HEPFLUSH-10
Click for Drug Monograph

usually is not indicated in DIC. An exception is in women with a retained dead fetus and evolving DIC with a progressive decrease in platelets, fibrinogen,

and coagulation factors. In this circumstance, heparin SOME TRADE NAMES

HEPFLUSH-10
Click for Drug Monograph

is administered for several days to control DIC, increase fibrinogen and platelets, and decrease excessive coagulation factor consumption before uterine

evacuation.

 widespread coagulation all over the body resulting to subsequent depletion of clotting factors.
CM: Petechiae and ecchymosis on the skin, mucous membrane, heart, lungs and other organs.
Lab. Data: Prolonged PT and PTT
Nsg. Dx: Risk for injury
Interventions:
 Monitor for signs of bleeding
 Administer heparin as ordered.
 Heparin inhibits thrombin thus preventing further clot formation and allowing coagulation factors to
accumulate.
 Administer blood transfusion as ordered.
 Instruct the patient to avoid aspirin or aspirin containing compounds.

CM: Fever
Lab. Data: Elevated ESR
Nsg. Dx: Altered Cardiac Output
Interventions:
 Record daily weight
 Evaluate JVD – this signifies the development of CHF.
 Instruct the patient to take antibiotics before dental procedures that can cuse bleeding.
 Avoid sharing of needles.
 Teach the women in child bearing years the risks of using IUDs, or birth control (source of
infection).

RHEUMATIC HEART DISEASE

RHEUMATIC FEVER AND HEART DISEASE

• Rheumatic fever is an inflammatory disease of the heart potentially involving all
layers of the heart.

• Rheumatic heart disease is a chronic condition resulting from rheumatic fever that
is characterized by scarring and deformity of the heart valves.

• Acute rheumatic fever (ARF) is a complication that occurs as a delayed sequela of

a group A streptococcal pharyngitis and affects the heart, joints, central nervous
system (CNS), and skin.

• About 40% of ARF episodes are marked by carditis, meaning that all layers of the
heart are involved, and this is referred to as rheumatic pancarditis.
o Rheumatic endocarditis is found primarily in the valves. Vegetation forms and
valve leaflets may fuse and become thickened or even calcified, resulting in
 stenosis or regurgitation.
o Myocardial involvement is characterized by Aschoff’s bodies.
o Rheumatic pericarditis affects the pericardium, which becomes thickened and
covered with a fibrinous exudate, and often involves pericardial effusion.
o The lesions of rheumatic fever are systemic, especially involving the
connective tissue, as well as the joints, skin, and CNS.

• Clinical manifestations of ARF include the following:

o The presence of two major criteria or one major and two minor criteria plus
evidence of a preceding group A streptococcal infection.
 Major criteria:
• Carditis results in three signs: (1) murmurs of mitral or aortic
regurgitation, or mitral stenosis; (2) cardiac enlargement and HF;
(3) pericarditis.
• Mono- or polyarthritis causes swelling, heat, redness, tenderness,
and limitation of motion.
• Chorea (Sydenham’s chorea) involves involuntary movements,
especially of the face and limbs, muscle weakness, and
disturbances of speech and gait.
• Erythema marginatum lesions are bright pink, nonpruritic, maplike
macular lesions that occur mainly on the trunk and proximal
extremities.
• Subcutaneous nodules are firm, small, hard, painless swellings
located over extensor surfaces of the joints.
 Minor criteria:
• Clinical findings: fever, polyarthralgia
• Laboratory findings: elevated ESR, elevated WBC, elevated
CRP

• Complications of ARF include chronic rheumatic carditis.

• Skin should be assessed for subcutaneous nodules and erythema marginatum.

• The overall goals for a patient with rheumatic fever include (1) normal or baseline
heart function, (2) resumption of daily activities without joint pain, and (3)
verbalization of the ability to manage the disease.

• Health promotion emphasizes prevention of rheumatic fever by early detection and

treatment of group A streptococcal pharyngitis with antibiotics, specifically penicillin.
o The success of treatment requires strict adherence to the full course of
antibiotic therapy.
o The primary goals of managing a patient with ARF are to control and
eradicate the infecting organism; prevent cardiac complications; and relieve
joint pain, fever, and other symptoms with antibiotics; optimal rest; and
antipyretics, NSAIDs, and corticosteroids.
o Secondary prevention aims at preventing the recurrence of rheumatic fever
with monthly injections of long-acting penicillin. Additional prophylaxis is
necessary if a patient with known rheumatic heart disease has dental or
surgical procedures involving the upper respiratory, GI (e.g., endoscopy), or
GU tract.

• The expected outcomes for the patient with rheumatic fever and heart disease
include (1) ability to perform ADLs with minimal fatigue and pain, (2) adherence to
treatment regimen, and (3) expression of confidence in managing disease.
 damage to the heart valves resulting to regurgitation, obstruction, narrowing or stenosis.

IM: Sore throat usually evident 2 – 4 weeks before diagnosis.

Tachycardia at rest is a common sign.
Lab. Data: Elevated ASO titer
Nsg. Dx: Altered cardiac output
Interventions:
 Limit physical activity during acute phase.
 Administer penicillin as prescribed.
 Monitor the heart rate.

SICKLE CELL ANEMIA

 defective hemoglobin molecule ( Hemoglobin S) that causes RBC to become sickle shaped.
 Severe chronic, hemolytic anemia characterized by episodes of pain due to the occlusion of
small blood vessels by sickled RBC’s.
IM: Growth retardation
S/sx
 chronic fatigue
 unexplained dyspnea
 joint swelling
 chest pain
 jaundice
 Hallmark
 Sickle Cell / Pain crisis → severe abdominal, thoracic, muscle, bone pain, possibly
jaundice, dark urine, fever
Dx:
 Blood Smear
 Umbilical Cord Blood - electrophoresis
 CXR “Lincoln log” deformity
 Ophthalmoscopic exam – corkscrew, comma shaped vessels
 (+) Sickle cell Hgb.
Nsg. Dx: Altered tissue perfusion
Tx:
 Vaccines – Haemophilus influenzae B vaccine
 Anti-infective - penicillin
 Analgesic
Intervention:
 Priority is hydration.
 Report s/s of vasoocclusion, MI or CVA
 Refer the parents to a geneticist
Pattern of transmission:
 If both parents have the trait: 25% of the children will have the disease.
 If one parent has the trait and the other has the disease:50% of the children will have the disease.
 If both parents have the disease: 100% of the children will have the disease.

HEMOPHILIA AND VON WILLEBRAND DISEASE

• Hemophilia is a sex-linked recessive genetic disorder caused by defective or
deficient coagulation factor. The two major forms of hemophilia, which can occur in
mild to severe forms, are hemophilia A and hemophilia B.

• Von Willebrand disease is a related disorder involving a deficiency of the von

Willebrand coagulation protein.

• Replacement of deficient clotting factors is the primary means of supporting a patient

with hemophilia. In addition to treating acute crises, replacement therapy may be
given before surgery and before dental care as a prophylactic measure.

• Home management is a primary consideration for the patient with hemophilia

because the disease follows a progressive, chronic course.

• The patient with hemophilia must be taught to recognize disease-related problems

and to learn which problems can be resolved at home and which require
hospitalization.
 Deficiency of clotting factors. Sex linked recessive trait (type A & B). More common in males
but transmitted by females.
 VON WILLEBRANDS DISEASE
 is transmitted to both male and female offspring of a carrier.

CM:
 Hemarthrosis, usually in the elbows, ankles and knees.
  Increased pain means bleeding continues.
Lab. Data:
 Prolonged PTT
 Normal PT
Nsg. Dx:
 Altered Tissue Perfusion
 Fluid Volume Deficit
 Pain
Interventions:
 Avoid ASPIRIN
 Control bleeding by utilizing RICE Principle.
 Avoid contact sports and rectal temperature monitoring.
 Administer missing coagulation factors as ordered. Stop transfusion if hives, headache, tingling,
chills, flushing or fever develops.
 Administer short course of corticosteroids to relieve inflammation.
 Refer to PT to prevent contractures and orthotics to prevent joint injury. Gentle, passive exercise 48
hours after the acute phase can be implemented for less severe hemarthrosis.
 Airway obstruction may occur due to bleeding in the neck and pharynx.