Cystic Fibrosis Mechanisms
- Autosomal Recessive
- CFTR Chromosome 7
- Cl- channel Defect
- Decreased Chloride Secretion
- Increased Na and Water Reabsorption
- Increased Na and Cl in Sweat
- Dehydration of Mucous Layers
Cystic Fibrosis Symptoms and Complications
- Recurrent Pulmonary Infections
- Chronic Bronchitis
- Nasal Polyps
- Pancreatic Insufficiency
- Intestinal Obstruction
- Malabsorption and Diarrhea
- Vitamin Deficiencies
- Chronic Hepatic Disease
- Infertility in Males
Cystic Fibrosis Assessment
- Thickened Mucus
- Recurrent Pulmonary Infections
- Pulmonary Congestion
- Pancreatic Insufficiency
- Steatorrhea
- Fat-Soluble Vitamin Deficiencies
- Salty Taste to Skin
- Meconium Ileus
- Delayed Growth and Poor Weight Gain
Cystic Fibrosis Interventions
- Bronchodilators
- N-Acetylcysteine (Mucolytics)
- Inhaled Hypertonic Saline
- Early Antibiotics
- Dornase Alfa (Mucolytic Agent)
- Postural Drainage with Percussion
- Fat Soluble Vitamin Supplements
- Pancreatic Enzymes with Every Meal
- High Protein, High Calorie Diet
- Added Dietary Salt
- Glucose Monitoring
Cystic Fibrosis Diagnosis and Treatment
- Sweat Chloride Test > 60 mmol/L
- Meconium Ileus
- N-acetylcysteine
- Antibiotic Prophylaxis
- Pulmonary Maintenance
- Lung Transplant
- Vitamin Replacement