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Home > Help Desk > Health Concern > Health Concern C > Cystic Fibrosis (CF)

Cystic Fibrosis (CF)

Cystic fibrosis (CF) is an inherited disease of glands causing them to over-secrete. The digestive and respiratory systems are most affected. The most common manifestation of cystic fibrosis is frequent respiratory infection. Impaired digestion and malabsorption due to pancreatic insufficiency and blocked liver ducts is often seen as well. Management of this condition requires the help of a qualified doctor.

What are the symptoms of cystic fibrosis?
Symptoms include a persistent cough with thick and often greenish-colored mucus, failure to grow normally, recurrent sinus and bronchial infections, and frequent, bulky, foul-smelling stools. Infants may experience a set of acute symptoms, including a distended abdomen, failure to pass stool, and vomiting. Although the course of the disease is highly variable, largely dependent upon the severity and frequency of respiratory infections, CF inevitably leads to debility and death. Average survival is to age 31.

How is it treated?
Conventional treatment of CF typically includes a daily regimen of physical therapy that consists of pounding on the chest to loosen mucus. Usually, supplementation with fat-soluble vitamins, especially vitamin E and vitamin K, and oral pancreatic enzymes (to aid digestion) is required. In addition, doctors may prescribe antibiotics to reduce the chance of lung infection, enzymes (recombinant human DNAse) to thin the mucus in the airways, and oxygen therapy.

Dietary changes that may be helpful:
People with CF are usually unable to digest food completely and therefore need to consume more calories than a healthy person of similar size and weight. Current guidelines recommend calorie intakes 20 to 50% above the recommended daily allowance.

Children with CF lose a large amount of salt in their sweat and thus should be encouraged to salt their food liberally. In case of fever, an additional 2 to 4 grams (1/2 to 1 teaspoon) should be added to the daily diet.

Lifestyle changes that may be helpful:
Aerobic exercise appears to improve lung function in people with CF. In a three-year controlled trial, people with CF engaged in a home exercise program, during which they exercised for a minimum of 20 minutes, three times weekly, and attained a heart rate of approximately 150 beats per minute. A slower decline in lung function was observed in these people compared with non-exercisers. Those who exercised also tended to feel better about themselves, had more energy, and/or experienced less chest congestion.

Nutritional supplements that may be helpful:
People with CF tend to have insufficient pancreas function. Supplementation with pancreatic enzymes will often lead to improved digestion, especially of fats. The current recommendation for people with cystic fibrosis is to supplement with pancreatic enzymes at meals.

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