Professional Documents
Culture Documents
(MedlinePlus, 2006)
Presentation of Disease
Pancreas
• neonatal screening
Sputum cultures
• Sweat test
Pulmonary
• Blood tests
function tests
• Metabolic alkalosis
FEV1
• Chest x-rays Stool evaluations
Pancreatic
function tests
Screening
. Screening of all newborn infants for CF is
routinely performed throughout the UK.
Immunoreactive trypsin (IRT) is raised in CF
patients.
Those samples are then screened for
common CF gene mutations.
Infants with two mutations have a sweat
test to confirm the diagnosis.
.
Screening
• 40-60 mEq/L ?
The Sweat Test
• False positives • False negatives
– adrenal insufficiency – severe malnutrition
– nephrogenic DI with edema
– hypothyroidism – too little sweat
– mucopolysaccharidosis – inexperienced tester
– G6P deficiency
– hypoproteinemia
– anemia from poor
nutrition
MANAGEMENT
Cystic Fibrosis: A Disease for a
Multidisciplinary Team
• Clinic coordinator
• Social worker
• Respiratory therapist
• Nurse
• Registered dietitian
Management of Lung Disease
• The aim is:
1. Clear secretions
2. Control infection
3.PHYSIOTHERAPY and physical activity
Chest physiotherapy
Postural drainage
Positive expiratory pressure
Agents to promote airway secretion clearance
-Hypertonic saline
-DNase I (dornase alfa)
-N-acetylcysteine
Management of Lung Disease…
ANTIBIOTIC THERAPY
1. IV Antibiotics
2. Aerosolized Antibiotics
3. Oral Antibiotics
Antibiotics
• Often unable to eradicate the organism
• Determining optimal delivery mode for a
drug is difficult
• CF patients require higher doses
– Altered volume of distribution
– Rapid clearance of drugs
IV Antibiotics
Indications:
1.Severe exacerbations
2.Bacterial resistance to all orally
administered antibiotics
3.Failure of oral antibiotic therapy to resolve
symptoms
G.I.T. Management…
• Pancreatic enzyme replacement (enteric
coated granules)
– Lipase, Amylase, Protease
– Not more than 2500 lipase units/kg/meal