Pathologic Conditions
- Definition: An inherited disease of the exocrine glands.
- Pathophysiology: Increased viscosity of mucous gland secretions. Elevated sweat electrolytes and salivary enzymes and abnormalities of the autonomic nervous system result in frequent and severe respiratory infections, progressive lung dysfunction, loss of sodium chloride, and decreased absorption of nutrients.
- Etiology: Disease is an inherited autosomal recessive disorder. Both parents must be carriers. About 1 in 20 whites carry the gene.
- Manifestations: Meconium ileus in up to 15% of newborns, failure to thrive, dyspnea, cough, frequent respiratory infection, excessive sodium loss in sweat, bulky, foul-smelling stools containing undigested food, abdominal distention, thin extremities, and rectal prolapse.
- Med Tx: Daily percussion and postural drainage of lungs (chest physiotherapy, or CPT), physical exercise, pancreatic enzymes administered with food, multivitamins, increased salt intake, calorie intake increased to 150% of RDA, and aggressive antibiotic therapy during respiratory infections.
- Nsg Dx: Ineffective airway clearance; potential for infection; altered nutrition, less than body requirements; knowledge deficit; fear; diversional activity deficit; sleep pattern disturbance.
- Nsg Care: Adhere to strict handwashing regimen. Assure compatibility of IV antibiotics. Encourage frequent fluid intake. Offer at least 6 feedings per day. Coordinate activities so that meds and CPT do not interfere with appetite or sleep and rest. Provide teaching and emotional support.
- Prognosis: Median survival is 20 to 27 years. Males generally survive longer than females.