Pancreatic Enzymes
Pancreatic enzyme supplements help overcome CF pancreatic dysfunction. The enzymes contain amylase, lipase, and protease, which help to digest starches, fats, and proteins. Enteric-coated enzyme capsules help prevent inactivation in the acidic stomach environment. Patients usually need 500 to 4000 units of lipase per gram of fat per day. Dosages are also based on body weight, with approximately 2000 units of lipase needed per kilogram of body weight for every meal (and half of that for snacks). This may be adjusted based on symptoms and results of a 72-hour fecal fat collection. Substitution with generic brands is not recommended because the amount of enzymes and preparations may vary. If a correct dosage is ineffective, this may be due to the acidity of the GI environment. Additional medication to neutralize stomach acid can be helpful to improve the effectiveness of the enzymes. Vitamin Supplements
CF patients are prone to malabsorption, and require replacement of fat-soluble vitamins, including vitamins A, D, E and K. Vitamin Dosages per Day | Vitamin A | 10,000 IU | Vitamin D | 400 to 800 IU and adequate sunlight exposure | Vitamin E | 200 to 400 IU | Vitamin K | 2.5 to 5 mg/week |
Patients taking antibiotics regularly or those with significant liver disease may require an additional 2.5 to 5 mg of vitamin K each week. Annual blood tests to check levels of vitamins A, E and D should be conducted and dosages should be adjusted accordingly.
High Fat, High Calorie Diet
Good nutrition is important to long-term survival of CF patients. A diet consisting of 35-40 percent fat is recommended. Monitoring nutritional status and body weight is required to avoid malnutrition. If malnutrition is a particular concern, consultation with a nutritionist can identify how calorie-dense foods can be added to the diet. Nutritional Supplements
Malnourished patients, or those losing weight, should have a medical evaluation and nutritional consultation. Commercial nutritional supplements can be used to provide calories and vitamins, and should serve to enhance meals. In some cases, a feeding tube can be placed for supplemental feedings, usually during sleep, to raise caloric intake. Gastrointestinal Motility Agents
CF patients often experience constipation, a side effect of malabsorption. Significant constipation may lead to a problem know as distal intestinal obstruction, or DIOS. Obstruction of the bowel by stool causes pain and cramping and, in some cases, vomiting. Laxatives such as Miralax® are the mainstay therapy for DIOS. In some cases, enemas may also be helpful. In more severe cases, a large volume of an electrolyte solution like GoLYTELY® is needed. This therapy may require hospitalization. A Gastrografin® enema can also be performed by a radiologist to help alleviate the obstruction syndrome. CF patients who are prone to intestinal obstruction and have experienced repeat episodes of DIOS may require preventive therapy. In these cases, Miralax®, N-acetylcysteine, mineral oil, prokinetic agents (metoclopramide), and electrolyte solutions (magnesium citrate, GoLYTELY®) may be used on a routine basis. Patients should take an adequate dose of supplemental enzymes and avoid getting dehydrated. Blood Glucose and Insulin Therapy
Patients with pancreatic insufficiency have an increased risk of developing high blood sugar or CF-related diabetes (CFRD). Monitoring usually requires blood testing several times a day. A blood test measuring glycosylated hemoglobin (Hgb A1C) can be performed periodically to monitor CFRD. Insulin, the hormone that controls the balance of sugar between the blood and tissues, is the best therapy for CFRD. Treatment usually consists of short-acting insulin injected under the skin before meals. A long-acting form of insulin is available when needed. Insulin therapy helps maintain normal blood glucose levels and helps patients gain weight. Patients with poorly controlled blood sugars tend to have a faster deterioration of lung function, more frequent pulmonary infections and other complications.
 |
