Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease of glands causing them to over-secrete. 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.

Checklist for Cystic Fibrosis

Rating Nutritional Supplements Herbs
3Stars Lipase (pancreatic)
Vitamin A
Vitamin D		  
2Stars Fish oil (EPA)
Taurine
Vitamin B12
Vitamin K		  
1Star Vitamin E
Zinc		  
3Stars Reliable and relatively consistent scientific data showing a substantial health benefit.
2Stars Contradictory, insufficient, or preliminary studies suggesting a health benefit or minimal health benefit.
1Star An herb is primarily supported by traditional use, or the herb or supplement has little scientific support and/or minimal health benefit.

What are the symptoms of cystic fibrosis? 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.

How is it treated? Conventional treatment of CF typically includes a daily regimen of physical therapy that consists of pounding on the chest to loosen mucus. Usually, supplementation with fat-soluble vitamins, especially vitamin E and vitamin K, and oral pancreatic enzymes (to aid digestion) is required. In addition, doctors may prescribe antibiotics to reduce the chance of lung infection, enzymes (recombinant human DNAse) to thin the mucus in the airways, and oxygen therapy.

Dietary changes that may be helpful: 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.1

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.2

Lifestyle changes that may be helpful: 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.3 Those who exercised also tended to feel better about themselves, had more energy, and/or experienced less chest congestion.

Nutritional supplements that may be helpful: 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.6 Because pancreatin is rapidly emptied from the stomach during digestion, people taking these enzymes may obtain better results by spreading supplementation throughout the meal.7

Taurine is an amino acid and a component of bile acids, which are important for proper fat digestion. Some,8 9 but not all,10 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.11

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.12 This deficiency may be reversed by supplementation with corn oil (1 gram per 2.2 pounds body weight per day),13 safflower oil (1 gram per 2.2 pounds body weight per day),14 linoleic acid (7.7 grams per day),15 and eicosapentaenoic acid (EPA from fish oil) (2.7 grams per day).16 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.17

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.18 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,19 and taking pancreatic enzymes helps prevent B12 deficiencies.20

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.21 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).22

Children with slowed growth associated with CF were found, in a preliminary study, to have overgrowth of bacteria in the small intestine compared to healthy children.23 There is as yet no evidence that elimination of this overgrowth will lead to improvement of CF symptoms.

Are there any side effects or interactions? Refer to the individual supplement for information about any side effects or interactions.

References:

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

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

3. 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.

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

9. 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.

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

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

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

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

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

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

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

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

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

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

20. 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.

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

22. Hansen RC, Lemen R, Revsin B. Cystic fibrosis manifesting with acrodermatitis enteropathica-like eruption. Arch Dermatol 1983;119:51–5.

23. Berezin S, Dhole A, Mascia A, Newman LJ. Small intestinal bacterial overgrowth may impair the nutritional stature of children with cystic fibrosis. Fed Proc 1985;44:1858 [abstract].