Cystic fibrosis (CF) may be one of the most common genetic diseases to go unnoticed by many. It is actually quite common because nearly 1 in 31 Americans are carriers for the defect that leads to this disease. The diagnosis used to mean that the child would not live to see elementary school. Now the life expectancy is significantly longer. Even better is that there are several nutritional factors that can be utilized to make the bodies of the cystic fibrosis patients stronger and healthier.

Cystic fibrosis is disease that significantly impacts the lungs and the digestive tract. Because of genetic mutations, secretions become very thick leading to damage of the lungs, along with chronic inflammation and chronic infections. The gastrointestinal tract is also affected because the digestive enzymes of the pancreas are not effectively released in to the intestines. Lack of digestive enzymes means that food is not properly broken down and digested. This leads to MANY nutritional deficiencies. Cystic fibrosis patients are commonly small for their size due to lack of nutrition. Nutritional deficiencies will also compromise just about every organ, including the lungs. Early deaths from CF historically come from ongoing and worsening pulmonary function and infections. Treatments have included digestive enzyme replacement, aggressive lung therapy including percussions to loosen the thick secretions in the lungs and aggressive antibiotic therapy.

There is now so much more known about CF and nutrition in general. We can combine this knowledge to help prevent nutritional deficiencies, strengthen the body and help prevent infections. The problems for the CF patient include:

  • Lack of digestive power leading to many nutritional deficiencies including: vitamins A, D, E, K, beta carotene, essential fatty acids, zinc, magnesium and CoQ10 to name a few [Source: 1-12]. As lung capacity decreases, more vitamins will be used at an even faster rate.
  • The lungs are more prone to inflammation and thus more infections [Source: Keitcher].
  • Infections are often harder to clear.
  • Bowel issues: poorly digested food, constipation, diarrhea, odorous stools.
  • Colonization of bad bacteria such as pseudomonas, a bug that is often very hard to treat and very hard to rid the body of.

Dosing of various vitamins will be determined by the extent of the disease and by the age and weight of the patient. Please utilize a team approach in discussing dosages including a physician and nutritionist.

On the next page, learn about the goals of nutritional supplementation.