Cystic Fibrosis

1. Pathophysiology

1.1. All secretions of exocrine glands of the respiratory, gastrointestinal and reproductive tracts become thick and obstruct normal flow.

1.2. Sweat glands do not reabsorb sodium, so salt depletion in sweat can occur.

2. Complications

2.1. -Obstruction of pancreatic exocrine function.

2.2. Chronic pancreatitis and secondary Type 1 DM.

2.3. Potentially fatal recurrent respiratory episodes of infections and atelectasis

2.4. Thorax may become barrel-shaped due to respiratory effort.

2.5. Steatorrheic stools, malnutrition, hypovitaminosis and bowel obstruction

2.6. Sterility

3. Medical Care

3.1. Lung Transplant

3.2. Genetic counseling

3.3. Pancreatic enzyme therapy

3.4. Antibiotics for respiratory episodes

3.5. Chest percussion

3.6. Supplemental Oxygen.

4. Assessment and Diagnostic

4.1. -Testing of sweat electrolytes,

4.2. -pancreatic enzyme testing for infants who present with a meconium ileus and children with repeated or severe pulmonary disease.

4.3. -Genotype testing of the parents and child.

5. Additional

5.1. Chest percussion and pancreatic enzyme replacement must be continued.

5.2. Vaccination for influenza and pneumonia are recommended for high-risk individuals

5.3. Monitor Lung sounds and O2 saturation every 4 hours

5.4. Monitor stools, assess for small bowel obstruction.

5.5. assess labs for hypovitaminosis and low total protein.