Management of Cystic Fibrosis
Management of the Pancreas
Overview
Some patients will require management of pancreatic insufficiency as well as management of diabetes. The primary pancreatic feature in CF is failure of the exocrine pancreas. There is pancreatic enzyme deficiency that leads to malabsorption of protein, fat, and fat-soluble vitamins.
While the islets of Langerhans which produce insulin are not initially involved, destruction of the pancreas leads to endocrine dysfunction known as cystic fibrosi-related diabetes, or CFRD, in approximately 7% of patients.3
Pancreatic Insufficiency
Patients with pancreatic insufficiency are given enzyme replacement in the form of capsules that are usually enteric coated to prevent inactivation of the enzymes by the acidic environment in the stomach. The capsules are given with meals and snacks, and dosing may be calculated based on fat content of the meal. More practical dosing is often based on body weight.
Patients with CF can have various individual characteristics that may affect the success of enzyme replacement, for example, inadequate bicarbonate secretion or rapid GI transit. In patients who continue to have symptoms of steatorrhea in spite of enzyme replacement, an attempt is made to identify these characteristics and adapt enzyme administration. An acid blocker is often added to help decrease acid in the gut and increase effectiveness of the enzymes. Patients who still continue to exhibit signs of steatorrhea are evaluated for other conditions, such as gastroenteritis, celiac disease, Crohn disease, or intestinal tumors, and treatment for those disorders is conducted as indicated.18
Cystic Fibrosis-related Diabetes (CFRD)
Treatment for patients with CFRD differs from therapy for type 1 or type 2 diabetes patients. Patients with CFRD are treated with insulin only, nor oral medication to lower blood glucose. CFRD patients follow a cystic fibrosis diet, not a diabetes diet. More attention is paid to energy requirements, the timing of meals, and avoiding concentrated carbohydrates.18,28
References:
3. Welsh M. Cystic Fibrosis. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 22nd ed. Philadelphia: WB Saunders; 2004. p. 1-22.
18. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004 January;125(1 Suppl):1S-39S.
28. Smyth RL. Diagnosis and management of cystic fibrosis. Arch Dis Child Ed Pract 2005;90:1-6.