Nutrition in Cystic Fibrosis

Cystic fibrosis is a genetic disorder that affects the body's exocrine glands, including the various mucus and sweat glands found throughout the body. Since cystic fibrosis is an inherited disease, most people with the disease are usually diagnosed by their first birthday.

 

The most common problems due to cystic fibrosis are related to the lungs and the digestive system. In the lungs, the mucus produced by the various exocrine glands is abnormally thick, creating obstructions in the breathing passageways and, eventually, damage to the lungs. Furthermore, the thick mucus creates an ideal breeding ground for various bacteria and other organisms, which results in frequent lung infections, damage to the lungs and, ultimately, respiratory failure.

Cystic fibrosis also affects the pancreas. The pancreas is responsible for producing various enzymes to aid in digestion, but in cystic fibrosis the pancreas is unable to excrete sufficient amounts of pancreatic enzymes to help digest food. In people with cystic fibrosis the passageway from the pancreas to the small intestine is usually lined with thick sticky mucus, often resulting in blocked ducts and damage to the pancreas. With the inability to properly digest the food, malabsorption and failure to thrive become major problems. The insulin-producing cells in the pancreas may also be damaged as the disease progresses, resulting in cystic fibrosis related diabetes.

 

Cystic fibrosis patients exhibit various symptoms, mainly concerning the lungs and the digestive system. Some of the symptoms related to pancreatic insufficiency include:

In people with cystic fibrosis, the pancreas is usually unable to supply the small intestine with digestive enzymes because the passage between the two is blocked with thick, sticky mucus. This leads to incomplete digestion of food and malabsorption, especially of fats.

To solve this problem, pancreatic enzyme replacements are prescribed: Over 90 percent of people with cystic fibrosis take pancreatic enzyme replacements. The pancreatic enzyme replacements are in the form of small beads contained in a capsule. The capsule is usually taken with liquid before each meal. Once the capsule enters the small intestine, it dissolves, releasing the pancreatic enzymes to help in digestion.

Patients with cystic fibrosis are also encouraged to increase their fat and calorie intake. Insufficient digestion leads to malabsorption, so an increased intake of calories increases the chances of absorbing enough nutrients to maintain good health. The body also has to remain healthy in order to fight lung infections, which are commonplace in people with cystic fibrosis.

 

 

Dietary supplements may be recommended for people with nutritional deficiencies as a result of malabsorption or dietary habits.

Resources

Cleveland Clinic Health System. (updated 2004). Cystic fibrosis.

Maguiness, K., Casey, S., Fulton, J., Luder, E., McKenna, A. & Hazle, L. (2002). Nutrition: Pancreatic enzyme replacement in people with cystic fibrosis. Cystic Fibrosis Foundation.

McKenna, A., Casey, S., Fulton, J., Luder, E., Maguiness, K & Hazle, L.. (2002). Nutrition: Cystic fibrosis: Changes through life. Cystic Fibrosis Foundation.

National Library of Medicine. (updated 2004). Cystic fibrosis. MedlinePlus Medical Encyclopedia.