Cystic Fibrosis
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 [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 [14].
• 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. The goals of nutritional supplementation include the following:
Correct Deficiencies. To help these problems, there must be aggressive steps taken. Most, if not all, of the above deficiencies can treated and corrected with simple supplementation. Supplementation should focus on a program that includes vitamins E, A (or beta carotene), K, zinc and magnesium. Most of these vitamins are readily available individually or as part of a multiple vitamin. These vitamins help prevent many chronic problems. For example, vitamin K is needed for healthy bones and vitamin A is needed to prevent infections and good eyesight. Magnesium is needed for all muscles, including the chest wall and also to utilize essential fatty acids [16]. Zinc is needed for the immune system and for tissue integrity and healing. Further suggestions would include vitamin D and CoQ10. Vitamin D is necessary for bone development and to help boost the immune system, both desperately needed in CF patients.
Control Inflammation. By controlling inflammation in the lungs, the body is much less prone to infection. Fish oils can help decrease inflammation [13,15]. Essential fatty acids are needed by basically every cell in the body, but fats will be poorly digested in CF patients. Essentially fatty acids can be supplied by fish and flax oils, as well as coconut and evening primrose oils to meet the needs of the body.
Prevention Infection and Colonization by the Bad Bacteria. The lungs are the organs that stand to get hit the hardest with more and more infections. These infections must be prevented so that antibiotic resistance does not develop and lung function can be maintained. N–acetyl cysteine (NAC) has been used to help loosen secretions and decrease inflammation [17]. Probiotics have been able to help prevent pseudomonas infection in animals and may help keep the bowels healthy. Lower levels of CoQ10 were associated with higher rates of pseudomonas infection, making this nutrient even that much more important [18].
The diet of CF patients is even more important than the average person since the cystic fibrosis will limit how many vitamins and nutrients are absorbed. Large quantities of fruits, vegetables, proteins and healthy fats are encouraged not only for daily growth and repair, but also for maintenance of all organs and to keep the immune system strong. A diet high in junk food and refined sugar is asking for more infection in a body already predisposed to problems.
There are two other exciting treatments available now for cystic fibrosis patients that may dramatically improve the condition and function of the lungs, potentially having a significant impact on life expectancy. Animals studies have found that curcurmin (found in the spice curry) has actually been able to help bypass the defect in cystic fibrosis and dramatically improved survival [19,20]. Human studies of curcumin are now being pursued. This is fantastic news! Curcumin has a very high range of safety and is also being clinically used in cancer treatments, joint pain and other chronic illnesses. It is a regular part of Asian cuisine where it has been used for centuries. Even if the effect is not quite as good in humans, it is still something to strongly consider because of its many benefits and its very broad safety range. Dosing may start at 50mg for infants working up to 1000mg or more for adults. The dosage can be titrated up to help with the symptoms experienced by the patient.
Glutathione is a second treatment that provides significant benefit to the lungs. The body actually makes glutathione as one of its major treatments critical for repair and detoxification. CF patients have been shown to be low in glutathione, particularly in the lining of the lungs [21]. In CF, lower levels of glutathione have correlated with poorer lung function [22]. As a treatment, intravenous glutathione has been used to treat many conditions including Parkinson’s disease and fibromyalgia. For CF patients, glutathione has actually been used in an inhaled form that has been shown to improve lung function [23,24]. There are now oral forms of glutathione that utilize special forms of fatty acids called liposomes that improve the absorption of glutathione (since it is usually broken down in the stomach). Glutathione can be made by compounding pharmacies from special orders by a physician. An Internet search for compounding pharmacies who supply glutathione can put families in touch with pharmacies knowledgeable in this area.
Many gains have been made in the treatment of CF. Patients are experiencing significantly longer life expectancies which will improve even more once we have mastered the treatment of the many nutritional deficiencies that come with CF and taken advantage of other promising yet simple treatments like curcumin and glutathione. We may soon see the day where CF is much less a disease than a milestone in the treatment of chronic illness.
References:
1. Excessive Fecal Loss of Vitamin A (Retinol) in Cystic Fibrosis. Ahmed, F., et al, Archives of Disease in Children, 1990;65:589-593.
2. Essential Fatty Acid Status and Fluidity of Plasma Phospholipids in Cystic Fibrosis Infants. Lloyd-Still, John D., et al, American Journal of Clinical Nutrition, 1991;54:1029-35.
3. Low Plasma Zinc Concentrations in Young Infants With Cystic Fibrosis. Krebs NF, et al, J Pediatr, December, 1998;133(6):761-764.
4. Low Concentrations of Sodium and Magnesium in Erythrocytes From Cystic Fibrosis Heterozygotes. Foucard, T., et al, ACTA Pediatrica Scand., 1991;80:57-61.
5. Carnitine Metabolites in Infants With Cystic Fibrosis: A Prospective Study. Lloyd-Still, John D., et al, Acta Pediatrica, 1993;82:145-9.
6. Decreased coenzyme Q10 concentration in plasma of children with cystic fibrosis. Oudshoorn JH, Lecluse AL, et al, J Pediatr Gastroenterol Nutr, 2006; 43(5): 646-50.
7. Oxygen Free Radicals and Antioxidants in Cystic Fibrosis: The Concept of an Oxidant-Antioxidant Imbalance. Winklhofer-Roob, Brigitte, M., et al, ACTA Pediatrica, Suppl. 1994;395:49-57.
8. Antioxidant Deficiency in Cystic Fibrosis: When Is the Right Time to Take Action? Back EI, Frindt C, et al, Am J Clin Nutr, 2004;80:374-384.
9. Vitamin A Deficiency and Nocturnal Vision in Teenagers With Cystic Fibrosis," Huet, F., et al, European Journal of Pediatrics, 1997;156:949-951.
10. The Occurrence and Effects of Human Vitamin E Deficiency: A Study in Patients With Cystic Fibrosis. Farrell PM, Bieri JG, Fratantoni JF, et al, J Clin Invest, July 1977;60:233-241.
11. Vitamin D Levels in Children With Cystic Fibrosis. Henderson, Richard C., M.D., Ph.D. and Lester, Gayle, Ph.D., Southern Medical Journal, April, 1997;90(4):378-383.
12. Efficacy of high dose phylloquinone in correcting vitamin K deficiency in cystic fibrosis. Drury D, Lands LC, et al, J Cyst Fibros, 2008 May 27.
13. Eicosapentaenoic Acid in Cystic Fibrosis: Evidence of a Pathogenetic Role for Leukotriene B4. Lawrence, Richard and Sorrell, Tania, The Lancet, August 21, 1993;342:465-469.
14. Leukotriene B4 Markedly Elevated in Epithelial Lining Fluid of Patients With Cystic Fibrosis. Konstan, Michael W., et al, American Review of Respiratory Diseases, 1993;148:896-901.
15. Omega-3 Fatty Acids Suppress the Enhanced Production of 5-Lipoxygenase Products From Polymorph Neutrophil Granulocytes in Cystic Fibrosis. Keicher, U., et al, European Journal of Clinical Investigation, 1995;25:915-919.
16. The Role of Magnesium in Cystic Fibrosis - A Hypothesis. Horn, Bernard, M.D., Journal of Nutritional Medicine, 1991;2:203-207.
17. High-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis. Tirouvanziam R, Conrad CK, et al, Proc Natl Acad Sci U S A, 2006; 103(12): 4628-33.
18. Decreased Total Serum Coenzyme-Q(10) Concentrations: A Longitudinal Study in Children with Cystic Fibrosis. Laguna TA, Sontag MK, et al, J Pediatr, 2008 May 29.
19. Egan ME, Pearson M, et al. Curcumin, a major constituent of tumeric, corrects cystic fibrosis defects. Science, 2004 Apr 23;304(5670):600-2.
20. Can Curcumin Cure Cystic Fibrosis? Zeitlin P, N Engl J Med, August 5, 2004;351(6):606-608.
21. Glutathione: A Radical Treatment for Cystic Fibrosis Lung Disease? Day BJ, Chest, January 2005;127(1):12-14.
22. Erythrocyte Glutathione in Cystic Fibrosis: A Possible Marker of Pulmonary Function. Mangione, Salvatore, M.D., et al, Chest, May 1994;105:5:1470-1473.
23. Rethinking Cystic Fibrosis Pathology: The Critical Role of Abnormal Reduced Glutathione (GSH) Transport Caused by CFTR Mutation. Hudson VM, Free Radic Biol Med, 2001;30(12):1440-1461.
24. A Pilot Study of the Effect of Inhaled Buffered Reduced Glutathione on the Clinical Status of Patients With Cystic Fibrosis. Bishop C, Hudson VM, et al, Chest, January 2005;127(1):308-317.
