Cystic Fibrosis

Cystic Fibrosis is a genetic disorder which involves poor utilization of the chloride ion. Chloride is one of the electrolytes (sodium, potassium, and chloride). The poor utilization of chloride results in the flawed metabolism of the fat soluble vitamins, A, E, D, K.

The flawed metabolism of vitamin A results in lung damage. The lungs require vitamin A for normal, healthy, development. The flawed development results in the creation of mucus in the lungs and difficulty breathing. For many years physicians felt that it was important to give these children with CF a great deal of vitamin A for their lungs. But, due to the flawed metabolism of A, the vitamin simply made the condition worse, and these children had little or no vitamin A in their systems. The treatment was later changed to giving children beta carotene. Beta carotene is the precursor for vitamin A. The liver converts beta carotene into vitamin A. (Beta carotene sources are fruits and vegetables, carrots having the greatest vitamin A content. Vitamin A food sources are foods in which animals have already converted beta carotene into vitamin A: liver, cheese, milk, eggs.) Both vitamin A and beta carotene are metabolized in the liver. But beta carotene requires a double process. It must first be converted into A, and them metabolized as A. It appears that both processes require chloride. Beta carotene therefore results in less vitamin A reaching the system, because the liver in cystic fibrosis uses all of its chloride in converting beta carotene into A, and then cannot metabolize as much vitamin A. Or not as much A is available for metabolism because not all of the beta carotene cannot be converted to A due to lack of chloride. In any case, children do better with beta carotene supplements than with vitamin A supplements, because they actually receive less vitamin A.

The point, as I see it, is that no supplements of vitamin A or of beta carotene should be given to children or adults with cystic fibrosis, because it is the vitamin A that causes the lung damage. Individuals with cystic fibrosis should take no supplements of vitamin A or of beta carotene. They should restrict their diets in vitamin A and beta carotene. Of course, we need vitamin A to live and to develop healthy lungs. However, these individuals walk the razor's edge between having just enough A for life and lung development and not more A than can be properly metabolized. Supplements of betaine hydrochloride with meals help to provide chloride and make it available for the metabolism of A. There are not many other sources of chloride, just salt (sodium chloride) and potassium chloride supplements.

The flawed metabolism of vitamin E seems to cause depression. No supplements of vitamin E should be given. Magnesium may be important in the proper metabolism of vitamin E. It should be given with vitamin E, if E is used.

The flawed metabolism of vitamin K causes bleeding. (K is important for blood clotting; however, when the metabolism is flawed, the result is bleeding). No supplements of K should be given.

The flawed metabolism of vitamin D causes peripheral neuropathy. Vitamin D is an important nutrient. There are pre-metabolized forms of D available. Magnesium is critical for the metabolism of vitamin D. It should always be taken with D.

The above is my recommendation for the treatment of cystic fibrosis.