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EO 1.1: Terms
Haemophilia
X-linked, autosomal recessive bleeding disorder occurs as result of deficiencies in coagulation proteins
Also called haemophilia type A in which there is a deficiency in coagulation factor VIII (Antihemophilic factorAHF) produced in liver
EO 1.1: Terms
Christmas Disease
Also called haemophilia type B Characterized by deficiency in coagulation factor IX (Plasma thromboplastin componentPTC)
Hemoarthrosis
Bleeding into the joints Mainly knee, hip & elbow disabling
EO 1.1: Terms
Hydrops fetalis
Accumulation of fluid in two or more body areas in the fetus Serious condition
Occurs breast fed babies @ 5-7dys old Products (fatty acids, b- glucuronidase & pregnanediol) in breast milk inhibit bilirubin conjugation
EO 1.1: Terms
Icterus neonatorum (Physiologic jaundice)
Yellowing of the skin in the newborn due to increased bilirubin in blood Why?
Newborns immature liver cannot excrete bile @ same rate destruction RBCs occurs.
Kernicterus Neurologic condition in which high levels of bilirubin result in the deposition of bilirubin into the brain & spinal cord. Produces convulsions in infants.
Deficiency in factor VIII inability to from thromboplastin during phase I of blood-clotting process = prolonged bleeding anywhere from or in the body. Bleeding into the mouth, neck or thorax serious cause can lead to obstruction
1. SQ & IM hemorrhages 2. Hemoarthrosis s/s: stiffness, tingling, achy joint 3. Bony changes as result repeated bleeding episodes 4. Prolonged bleeding 5. Spontaneous hematuria 6. Epistaxis-nose bleed 7. Ecchymoses & SQ hematomas
a)Factor VII & IX assay (deficiency) b)Clotting times c)PT=N, aPTT prolonged, INR=N
EO-1.2:Describe w/ rationale, each of the following for the child w/ HAEMOPHILIA 2. Corticosteroids tx hematuria, Hemoarthrosis, synovitis 3. NSAIDS for painibuprofen...NO ASPIRIN increases bleeding 4. E-aminocaproic acid (EAC, Amicar) to prevent clot destruction 5. Exercise & physical therapy active ROM so pt can detect own level of painNO CONCTACT SPORTS 6. Teach family: venipuncture if <8yr
Factor VIII concentrates & Cryoprecipitate AHF (antihaemophilic factor) (not for VII deficiency though, )-for acute bleeding when factor concentrates not available ex. Aafact (Alphonate)from human plasma, Advaterecombinant antihaemophilic factor synthetic) DDAVP (1-deamino-8arginine vasopressin)synthetic vasopressin, results 3-4 fold increase factor VIII
2.
3. 4. 5.
1. 2. 3. 4.
Bleeding, Risk for Impaired mobility Acute pain Deficient fluid vole r/t loss blood 5. Ineffective health maintenance 6. Fear r/t high risk HIV 7. Fatigue r/t loss blood volume
1.Goal = Prevent or treat bleeding Outcome: maintain stable VS w/ minimal blood loss 2. Goal= prevent contractures &joint deformities Outcome/intervention: maintain mobility/RICE, bed rest, ambulation, AROM, positioning 3. Goal = treat pain Outcome/interventions: be free of pain/admin analgesics
EO 1.6 Describe the classification, actions, & NSG implications of the following:
1. Antihemophilic factor (AHF)( Alphonate, - from pooled human plasma DDAVP synthetic vasopressin & cryoprecipitate AHF-) prevent & control bleeding those w/ Haemophilia A by increasing factor VIII levels in blood 2. Anti-inhibitor coagulant complex- (Amicar, EACA, Autoplex T)- prevents clot destruction (fibrinolysis), used if mouth trauma, surgery (dose factor concentrate must be given first)
3. Factor IX complex(Alphanine SD, Proplex, Proplex Tfrom pooled human plasma)-to prevent & control bleeding in those w/ haemophilia B by increasing factor IX levels in blood NSG implications: from plasma increased risk transmission of viruses & allergic rxs
Blood contains agglutinogens which produce immune response. In ABO & Rh (recessive) blood group, antigens occur naturally.
2. Increased serum Unconjugated bilirubin 3. Anemia-from hemolysis lg amts RBCs 4. Hyperbilirubinemia-cause liver unable conjugate & excrete excess bilirubin 5. Hepatomegaly & varying degrees Hydrops 6. Anemia 7. Hypovolemic shock 8. Hypoglycemiadue to pancreatic cell hyperplasia
3. Ultrasonography- adjunctivecheck for alterations placenta, umbilical cord, amniotic fluid volume & hydrops 4. Timing & appearance jaundice
Confirm w/ Direct Combs test or Direct antiglobulin test
Goal therapeutic management is PREVENTION Involves phototherapy, exchange transfusion, pericardial & pleural fluid aspiration, mechanical ventilation & inotrope therapy
Signs blood exchange transfusion rx: Tachycardia or bradycardia Respiratory distress Dramatic change blood pressure Temperature instability rash
Goal= early detection & implementation phototherapy for reduction Hyperbilirubinemia IVIG transfusions are used in combination w/ phototherapy in some centers.
1.Risk for injury 2.Neonatal jaundice r/t Rh or ABO incompatibility 3.Deficient knowledge r/t tx regime 4.anxiety