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 help you manage this inherited disease:
- 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
- Keep up the weight
- Consume enough calories to compensate for poor food digestion
associated with cystic fibrosis
- Work in a workout
- Start a regular program of aerobic exercise to slow the decline in
lung function
These recommendations are not comprehensive and are not intended to replace
the advice of your doctor or pharmacist. Continue reading the full cystic fibrosis article for
more in-depth, fully-referenced information on medicines, vitamins, herbs, and dietary and
lifestyle changes that may be helpful.
About cystic fibrosis
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.
Product ratings for cystic
fibrosis
What are the 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.
Medical options
Prescription strength pancreatic enzymes
(Pancrease MT, Lipram, Viokase) are used to aid digestion. Healthcare practitioners might also
prescribe antibiotics to prevent possible lung
infections, enzymes (recombinant human DNAse) to thin the mucus in the airways, and oxygen therapy.
Treatment typically includes a daily regimen of physical therapy that consists of pounding
on the chest to loosen mucus.
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.
Vitamins 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].