Cystic Fibrosis

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 weight¹ or 500 to 1,000 lipase units per gram of dietary fat consumed,² 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.³Because pancreatin is rapidly emptied from the stomach during digestion, people taking these enzymes may obtain better results by spreading supplementation throughout the meal.⁴

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.⁵ 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,⁶ and taking pancreatic enzymes helps prevent B12 deficiencies.⁷

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

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.¹¹ This deficiency may be reversed by supplementation with corn oil (1 gram per 2.2 pounds body weight per day),¹²safflower oil (1 gram per 2.2 pounds body weight per day),¹³ linoleic acid (7.7 grams per day),¹¹ and eicosapentaenoic acid (EPA from fish oil) (2.7 grams per day).¹⁵ 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.¹⁵

Taurine is an amino acid and a component of bile acids, which are important for proper fat digestion. Some,^15,16 but not all,¹⁷ 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.¹⁵

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 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.²¹ 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,²² and taking pancreatic enzymes helps prevent B12 deficiencies.²³

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.²⁴ 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.²⁵