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Search Health Information    Cystic Fibrosis (Holistic)

Cystic Fibrosis (Holistic)

About This Condition

Get support for cystic fibrosis by focusing on overall fitness and your unique nutritional needs. According to research or other evidence, the following self-care steps may be helpful.
  • Fill up on fat-soluble supplements

    Help avoid deficiencies of fat-soluble vitamins by taking daily amounts of vitamin A (5,000 IU), vitamin D (1,000 IU), and vitamin E (100 IU); also take 5 mg of vitamin K every three days

  • Fight back with fatty acids

    Work with a nutritionist to determine how much vegetable oil or fish oil should be included in your diet to prevent essential fatty acid deficiency

  • Shake on the salt

    Get plenty of salt in your diet to replace the large amounts lost through sweating

About

About This Condition

Cystic fibrosis (CF) is an inherited disease that results in impaired transport of chloride into and out of cells. 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.

Symptoms

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.

Healthy Lifestyle Tips

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.1 Those who exercised also tended to feel better about themselves, had more energy, and/or experienced less chest congestion.

Eating Right

The right diet is the key to managing many diseases and to improving general quality of life. For this condition, scientific research has found benefit in the following healthy eating tips.

Recommendation Why
Get enough calories
People with cystic fibrosis are usually unable to digest food completely and therefore should get 20 to 50% more calories than the recommended daily allowance.

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.2

Shake on the salt
Children with cystic fibrosis lose a large amount of salt in their sweat and thus should be encouraged to salt their food liberally.

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.3

Supplements

What Are Star Ratings?

Our proprietary “Star-Rating” system was developed to help you easily understand the amount of scientific support behind each supplement in relation to a specific health condition. While there is no way to predict whether a vitamin, mineral, or herb will successfully treat or prevent associated health conditions, our unique ratings tell you how well these supplements are understood by the medical community, and whether studies have found them to be effective for other people.

For over a decade, our team has combed through thousands of research articles published in reputable journals. To help you make educated decisions, and to better understand controversial or confusing supplements, our medical experts have digested the science into these three easy-to-follow ratings. We hope this provides you with a helpful resource to make informed decisions towards your health and well-being.

3 Stars Reliable and relatively consistent scientific data showing a substantial health benefit.

2 Stars Contradictory, insufficient, or preliminary studies suggesting a health benefit or minimal health benefit.

1 Star For an herb, supported by traditional use but minimal or no scientific evidence. For a supplement, little scientific support.

Supplement Why
3 Stars
Lipase
Up to 10,000 IU daily per 2.2 lbs (1 kg) body weight or 500 to 1,000 lipase units per gram of fat consumed in the diet
People with cystic fibrosis tend to have insufficient pancreas function. Supplementing with pancreatic enzymes will often lead to improved digestion, especially of fats.

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. Amounts should not exceed 10,000 IU of lipase per day per 2.2 pounds body weight4 or 500 to 1,000 lipase units per gram of dietary fat consumed,5 as larger amounts may damage the large intestine. A double-blind trial found enteric-coated microsphere enzyme preparations to be superior to enteric-coated capsules for reduction of abdominal pain and improvement of digestion.6Because pancreatin is rapidly emptied from the stomach during digestion, people taking these enzymes may obtain better results by spreading supplementation throughout the meal.7

3 Stars
Vitamin A
5,000 to 10,000 IU daily
The fat malabsorption associated with cystic fibrosis often leads to a deficiency of fat-soluble vitamins, such as vitamin A. Supplementing with this vitamin can help counteract the deficiency.

The fat malabsorption associated with CF often leads to a deficiency of fat-soluble vitamins. Oral supplementation of these nutrients is considered crucial to maintaining good nutritional status.8 Current recommendations for supplementation are as follows: vitamin A , 5,000 to 10,000 IU/day; vitamin D , 1,000 to 2,000 IU/day; vitamin E , 100 to 300 IU/day; and vitamin K , 5 mg every three days. Of the water-soluble vitamins, only vitamin B12 is poorly absorbed in cystic fibrosis,9 and taking pancreatic enzymes helps prevent B12 deficiencies.10

3 Stars
Vitamin D
1,000 to 2,000 IU daily
The fat malabsorption associated with cystic fibrosis often leads to a deficiency of fat-soluble vitamins, such as vitamin D. Supplementation can help counteract the deficiency.

The fat malabsorption associated with CF often leads to a deficiency of fat-soluble vitamins. Oral supplementation of these nutrients is considered crucial to maintaining good nutritional status.11 Current recommendations for supplementation are as follows: vitamin A , 5,000 to 10,000 IU/day; vitamin D , 1,000 to 2,000 IU/day; vitamin E , 100 to 300 IU/day; and vitamin K , 5 mg every three days. Of the water-soluble vitamins, only vitamin B12 is poorly absorbed in cystic fibrosis,12 and taking pancreatic enzymes helps prevent B12 deficiencies.13

2 Stars
Fish Oil
2.7 grams of EPA daily
The impaired fat digestion in people with cystic fibrosis often leads to a deficiency of essential fatty acids, which may lead to increased risk of respiratory infection. This deficiency may be reversed by fish oil supplementation.

The impaired digestion of fats in people with CF often leads to a deficiency of essential fatty acids. This deficiency may in turn lead to lowered immune function , which makes people with CF more susceptible to respiratory infection.14 This deficiency may be reversed by supplementation with corn oil (1 gram per 2.2 pounds body weight per day),15safflower oil (1 gram per 2.2 pounds body weight per day),16 linoleic acid (7.7 grams per day),17 and eicosapentaenoic acid (EPA from fish oil ) (2.7 grams per day).18 EPA supplementation was particularly effective. In a double-blind trial, six weeks of supplementation with 2.7 grams of EPA per day led to a reduction in sputum and improvement in lung function in children with chronic respiratory infection due to CF.19

2 Stars
Taurine
30 mg per 2.2 lbs (1 kg) of body weight daily
Taurine is an amino acid and a component of bile acids, which are important for proper fat digestion. Supplementing with taurine may help improve fat digestion.

Taurine is an amino acid and a component of bile acids, which are important for proper fat digestion. Some,20 , 21 but not all,22 investigators have reported improvement in fat digestion among people with CF when they supplemented with 30 mg taurine per 2.2 pounds of body weight daily. Greater improvement was seen in people with the worst maldigestion.23

2 Stars
Vitamin K
5 mg every three days
The fat malabsorption associated with cystic fibrosis often leads to a deficiency of fat-soluble vitamins, such as vitamin K. Supplementation can help counteract the deficiency.

The fat malabsorption associated with CF often leads to a deficiency of fat-soluble vitamins. Oral supplementation of these nutrients is considered crucial to maintaining good nutritional status.24 Current recommendations for supplementation are as follows: vitamin A , 5,000 to 10,000 IU/day; vitamin D , 1,000 to 2,000 IU/day; vitamin E , 100 to 300 IU/day; and vitamin K , 5 mg every three days. Of the water-soluble vitamins, only vitamin B12 is poorly absorbed in cystic fibrosis,25 and taking pancreatic enzymes helps prevent B12 deficiencies.26

1 Star
Vitamin E
Refer to label instructions
The fat malabsorption associated with cystic fibrosis often leads to a deficiency of fat-soluble vitamins, such as vitamin E. Supplementation can help counteract the deficiency.

The fat malabsorption associated with CF often leads to a deficiency of fat-soluble vitamins. Oral supplementation of these nutrients is considered crucial to maintaining good nutritional status.27 Current recommendations for supplementation are as follows: vitamin A , 5,000 to 10,000 IU/day; vitamin D , 1,000 to 2,000 IU/day; vitamin E , 100 to 300 IU/day; and vitamin K , 5 mg every three days. Of the water-soluble vitamins, only vitamin B12 is poorly absorbed in cystic fibrosis,28 and taking pancreatic enzymes helps prevent B12 deficiencies.29

1 Star
Zinc
Refer to label instructions
The malabsorption produced by cystic fibrosis may adversely affect zinc absorption. Supplementing with zinc can help counteract this deficiency.

The malabsorption produced by CF may adversely affect mineral absorption as well. Blood concentrations of zinc were low in a group of children with CF.30 One child with CF was reported to have a severe generalized dermatitis that resolved upon correction of zinc and fatty acid deficiencies by using a formula containing zinc (about 3 mg per day) and medium chain triglycerides (amount not reported).[REF] In a double-blind trial, supplementation with 30 mg of zinc per day for one year significantly decreased the number of days that children with CF needed antibiotics to treat respiratory infections. The beneficial effect of zinc was more pronounced in children who had low or low–normal plasma zinc levels than in those who had higher levels.31

References

1. Schneiderman-Walker J, Pollock SL, Corey M, et al. A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J Pediatr 2000;136:304–10.

2. Wilson DC, Pencharz PB. Nutrition and cystic fibrosis. Nutrition 1998;14:792–5 [review].

3. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805–22 [review].

4. Littlewood JM, Wolfe SP. Control of malabsorption in cystic fibrosis. Paediatr Drugs 2000;2:205–22.

5. Borowitz DS, Grand RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus committee. J Pediatr 1995;127:681–4.

6. Vyas H, Matthew DJ, Milla PJ. A comparison of enteric coated microspheres with enteric coated tablet pancreatic enzyme preparations in cystic fibrosis. A controlled study. Eur J Pediatr 1990;149:241–3.

7. Taylor CJ, Hillel PG, Ghosal S, et al. Gastric emptying and intestinal transit of pancreatic enzyme supplements in cystic fibrosis. Arch Dis Child 1999;80:149–52.

8. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805–22 [review].

9. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537–40.

10. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33–41.

11. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805–22 [review].

12. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537–40.

13. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33–41.

14. Chase HP, Dupont J. Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis. Lancet 1978;2:236–8.

15. Rosenlund ML, Selekman JA, Kim HK, Kritchevsky D. Dietary essential fatty acids in cystic fibrosis. Pediatrics 1977;59:428–32.

16. Lloyd-Still JD, Simon SH, Wessel HU, Gibson LE. Intravenous linoleic acid supplementation in children with cystic fibrosis. Pediatrics 1979;64:50–2.

17. Chase HP, Dupont J. Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis. Lancet 1978;2:236–8.

18. Lawrence R, Sorrell T. Eicosapentaenoic acid in cystic fibrosis: evidence of a pathogenic role for leukotriene B4. Lancet 1993;342:465–9.

19. Lawrence R, Sorrell T. Eicosapentaenoic acid in cystic fibrosis: evidence of a pathogenic role for leukotriene B4. Lancet 1993;342:465–9.

20. Darling PB, Lepage G, Leroy C, et al. Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatr Res 1985;19:578–82.

21. Belli DC, Levy E, Darling PB, et al. Taurine improves the absorption of a fat meal in patients with cystic fibrosis. Pediatrics 1987;80:517–23.

22. Thompson GN, Robb TA, Davidson GP. Taurine supplementation, fat absorption, and growth in cystic fibrosis. J Pediatr 1987;111:501–6.

23. Darling PB, Lepage G, Leroy C, et al. Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatr Res 1985;19:578–82.

24. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805–22 [review].

25. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537–40.

26. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33–41.

27. Turck D, Michaud L. Cystic fibrosis: nutritional consequences and management. Baillieres Clin Gastroenterol 1998;12:805–22 [review].

28. Lindemans J, Neijens HJ, Kerrebijn KF, Abels J. Vitamin B12 absorption in cystic fibrosis. Acta Paediatr Scand 1984;73:537–40.

29. Gueant JL, Vidailhet M, Pasquet C, et al. Effect of pancreatic extracts on the faecal excretion and on the serum concentration of cobalamin and cobalamin analogues in cystic fibrosis. Clin Chim Acta 1984;137:33–41.

30. Krebs NF, Sontag M, Accurso FJ, Hambidge KM. Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr 1998;133:761–4.

31. Abdulhamid I, Beck FWJ, Millard S, et al. Effect of zinc supplementation on respiratory tract infections in children with cystic fibrosis. Pediatr Pulmonol 2008;43:281–7.

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