Nutrition Management of Cystic Fibrosis in the 21st Century

Nutr Clin Pract. 2015 Aug;30(4):488-500. doi: 10.1177/0884533615591604. Epub 2015 Jun 25.

Abstract

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.

Keywords: cystic fibrosis; diabetes mellitus; exocrine pancreas insufficiency; lung diseases; nutrition therapy; pancreas.

Publication types

  • Review

MeSH terms

  • Cystic Fibrosis / complications
  • Cystic Fibrosis / diet therapy*
  • Cystic Fibrosis / surgery
  • Diabetes Mellitus / etiology
  • Diet, High-Fat
  • Energy Intake
  • Enteral Nutrition / methods
  • Exocrine Pancreatic Insufficiency / drug therapy
  • Exocrine Pancreatic Insufficiency / etiology
  • Female
  • Gastrointestinal Agents / therapeutic use
  • Humans
  • Intestinal Obstruction / etiology
  • Lung Transplantation
  • Male
  • Malnutrition / diet therapy*
  • Malnutrition / etiology
  • Nutritional Status*
  • Pancreatin / therapeutic use
  • Pregnancy
  • Pregnancy Complications / etiology
  • Vitamins / therapeutic use

Substances

  • Gastrointestinal Agents
  • Vitamins
  • Pancreatin