1. 2. Assessment:
      1. Newborn: meconium ileus.
      2. Frequent, recurrent pulmonary infections: bronchitis, bronchopneumonia, pneumonia, and ultimately chronic obstructive pulmonary disease (COPD) due to mechanical obstruction of respiratory tract caused by thick, tenacious mucous gland secretions.
      3. Malabsorption syndrome: failure to gain weight, distended abdomen, thin arms and legs, lack of subcutaneous fat due to disturbed absorption of nutrients that results from the inability of pancreatic enzymes to reach intestinal tract.
      4. Steatorrhea: bulky, foul-smelling, frothy, fatty stools in increased amounts and frequency (predisposed to rectal prolapse).
      5. Parents may note that child “tastes salty” when kissed, due to excessive loss of sodium and chloride in sweat.
      6. Sweat test reveals high sodium and chloride levels in child’s sweat, unique to children with cystic fibrosis.
      7. Sexual development:
         1. Male: sterile (due to aspermia).
         2. Female: difficulty conceiving and bearing children (due to increased viscosity of cervical mucus, which acts as a plug in the cervical os and mechanically blocks the entry of sperm).
   3. Analysis/nursing diagnosis:
      1. Ineffective breathing patterns related to thick, viscid secretions.
      2. Altered nutrition, less than body requirements, related to diarrhea and poor intestinal absorption of nutrients.
      3. Decreased cardiac output related to COPD and decreased compliance of lungs.
      4. Activity intolerance related to respiratory compromise.
      5. Self-esteem disturbance related to body image changes.
      6. Knowledge deficit related to disease process, treatments, medications, genetics.
      7. Noncompliance (potential) related to complicated and prolonged treatment regimen.
   4. Nursing care plan/implementation:
      1. Goal: assist child to expectorate sputum.
         1. a. Perform postural drainage as prescribed: first thing in morning, between meals, before bedtime, not after meals to prevent aspiration.
         2. Administer nebulizer treatments, expectorants, mucolytics, bronchodilators. Avoid or limit use of medications that suppress cough mechanism.
         3. Provide for exercises that promote position changes and keep sputum moving up and out.
         4. Encourage high fluid intake to keep secretions liquefied.
         5. Suction, administer oxygen pm.
      2. Goal: prevent infection.
         1. Standard precautions to prevent infection.
         2. Evaluate carefully, check continually for potential infection (especially respiratory); report to physician promptly.
         3. Limit contact with staff or visitors (especially children) with infection.
         4. Administer antibiotics as ordered, to treat respiratory infections and prevent overwhelming sepsis.
         5. May be placed on prophylactic antibiotic therapy between episodes of infection.
         6. Teach importance of prevention of infection at home: adequate nutrition, frequent medical checkups, stay away from known sources of infection.
      3. Goal: maintain adequate nutrition.
         1. Diet: well balanced, high calorie and protein to prevent malnutrition.
         2. Administer pancreatic enzyme (viokase, pancreatin) immediately before every meal and every snack to enhance the absorption of vital nutrients, especially fats.
         3. If child is unable to swallow tablets, mix pancreatic enzyme powder with cold applesauce.
         4. Administer water-miscible preparations of fiat-soluble vitamins (A, D, E, K), multivitamins, and iron.
         5. Encourage extra salt intake to compensate for excessive sodium losses in sweat (unless CHF is present).
         6. Encourage extra fluid intake (e.g., Gatorade) to prevent dehydration/electrolyte imbalance.
         7. Daily I&O and weights to monitor nutritional and hydration status.
         8. Encourage child to assume gradually increasing responsibility for choosing own foods within dietary restrictions.
      4. Goal: teach child and family about cystic fibrosis.
         1. Discuss diagnostic procedures: sweat test, stool specimens.
         2. Review multiple medications: use, effects, side/toxic effects.
         3. Stress need to care for pulmonary systems (major cause of mortality/morbidity).