Cystic fibrosis a genetic condition that involves decreased secretion from the lungs and the lower digestive tract. About 1 in 25 people has one copy of the gene that causes cystic fibrosis, and they actually benefit from it.
Having just one copy of the gene that reduces secretion makes it easier to resist the flu. It reduces the risk of catching food-borne infections, and it stops asthma and allergies. Having just one copy of the gene that causes cystic fibrosis reduces secretion in ways that don't interfere with normal body function and which fight disease.
However, having two copies of this gene, one from each parent, is a threat to life itself. Cells in the lungs and pancreas do not secrete, that is, they don't let go of, the chloride from sodium chloride (salt). Sodium and chloride are nearly perfectly balanced in the body, so they swell with fluid inside but become dry on the outside. The mucus that normally surrounds cells in the lungs and pancreas dries out and becomes stiff. And these cells also make lots of inflammatory chemicals because of the way they use the omega-3 essential fatty acid known as docosahexaenoic acid, or DHA.
The cells in the lungs and pancreas that are affected by the cystic fibrosis gene not only take up DHA from the bloodstream faster than normal cells, they also use DHA very quickly. They use DHA to stop a process that creates another fatty acid, arachidonic acid, also known as AA, which becomes inflammatory hormones that constrict air passages and which interfere with the release of pancreatic enzymes.
If this is all a little hard to follow-I have trouble following all the enzyme and second messenger reactions myself-let me explain it this way.
In cystic fibrosis, cells get inflamed and swollen outside. They become inflamed and desiccated outside. The combination of effects makes it very difficult for affected tissues to do their jobs in the body. Providing DHA to the cell relieves the inflammation inside.
There are over 500 different biochemical reactions affected by this one gene, and even experts in the field do not understand them all. But here are the basics of a nutritional program for supporting optimal health possible with cystic fibrosis:
- Provide at least 900 mg of DHA every day. Since people who have cystic fibrosis take lots of medications and lots of supplements, the best way to do this is with the Xtend Life Fish Oil, which contains 300 mg of DHA per capsule. Microalgae supplements are also high in DHA, but they are more expensive and usually require taking more capsules.
- When possible, use a brand of fish oil that is both high in DHA and that contains CoQ10. Coenzyme Q10 is especially important during infections, since it gives cells the ability to operate with less oxygen and it also helps increase lung capacity. Xtend-Life Omega-3 QH Ultra is a product that contains both supplements.
- It also helps to take N-acetyl-cysteine (NAC), 200 mg a day for children and 400 mg a day for adults. This product makes mucus less "sticky".
- The amino acid taurine helps bile salts "catch" fat-soluble vitamins and plant nutrients such as A, D, E, and K, and beta-carotene. Taking taurine may help prevent vitamin deficiencies.
Nutritional intervention alone, unfortunately, is never adequate for controlling cystic fibrosis. It can only assist the healing process as modern medicine manages the inevitable infections and respiratory crises caused by the disease.
There are some nutritional supplements which should not be used by people who have cystic fibrosis. Some supplements claim to "stimulate growth hormone" with gamma-oryzanol, which is derived from rice bran oil. Gamma-oryzanol may actually stimulate growth of muscles in the diaphragm, but it decreases production of growth hormone, and it is impossible to predict the combination of these two effects.
Also, even though people with cystic fibrosis have problems related to pancreatic enzymes, it is usually not a good idea to take supplemental enzymes. While pancreatic enzyme replacement may indeed relieve abdominal pain and bloating and stop smelly bowel movements, it can also cause irreversible narrowing of the colon.
It is hard to find good answers to the questions about nutrition to support the best possible health during cystic fibrosis. Questions, comments, and your experiences in managing cystic fibrosis are welcome in the Comments Box.