PATHOPHYSIOLOGY RELATED TO CLIENT PROBLEM : CF is caused by mutation in CF transmembrane conductance regulator (CFTR) gene. the CFTR proteins produced by this gene regulates the movement of chloride and sodium ions across epithelial cell membranes.
HEALTH PROMOTION AND DISEASE PREVENTION : Cystic fibrosis can't be prevented. However, testing should be performed to the couples who have this disease or relatives with this disease. Maintain proper hygienes and disinfection.
RISK FACTORS : the only risk factor for getting CF gene is having two parents who carry abnormal CF gene and pass the gene to their child.
EXPECTED FINDINGS : the thick and sticky mucus associated with CF clogs the tube carrying air in and out of the lungs.
LABORATORY TESTS : blood test ( IRT ) immunoreactive trypsinogen
pulmonary function test
chest X ray
sputum and stool evaluations
DIAGNOSTIC PROCEDURES : the sweat chloride test is the standard diagnostic procedure
genetic testing
prenatal testing
COMPLICATIONS : Bowel problems
coughing up blood
chronic respiratory failure
diabetes
infertility
liver diseases
malnutrition
nasal polyps and sinusitis
SAFETY CONSIDERATIONS : infection control
oxygen therepy
physiotherepy
vaccination
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