Journal
NUTRITION IN CLINICAL PRACTICE
Volume 30, Issue 4, Pages 488-500Publisher
WILEY
DOI: 10.1177/0884533615591604
Keywords
cystic fibrosis; pancreas; exocrine pancreas insufficiency; lung diseases; diabetes mellitus; nutrition therapy
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Despite significant advancements made in life expectancy over the past century, cystic fibrosis remains a life-threatening genetic disease that affects the gastrointestinal tract, and it has significant impact on the nutrition status of those with the disease. Nutrition management includes a high-calorie/high-fat diet, pancreatic enzyme replacement therapy, vitamin and mineral replacement, and enteral support as needed. As patients are living longer, clinicians may encounter patients with cystic fibrosis in obstetrician offices, endocrine clinics, or hospital settings, owing to lung transplantation or for treatment for distal intestinal obstruction syndrome.
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