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Management of Cystic Fibrosis

Management of the Digestive System

Uncomplicated Meconium Ileus

Meconium ileus (MI) is managed in the newborn as an intestinal obstruction. The goal of management is to evacuate meconium from the intestine while preserving as much intestinal length as possible.

1. The patient is stabilized, receives intravenous hydration, gastric decompression, empiric antibiotic therapy, and surgical evaluation.
2. Diagnostic contrast enema and radiography must have excluded complications of MI. With uncomplicated MI, clearance is first attempted with Gastrografin^® enema. Deconcentrating agents may be used to make the meconium more liquid. The reported success rate if 63% to 83%.
3. Following successful clearance, deconcentrating agents may be continued, and feeding is resumed, with vitamins and supplemental pancreatic enzymes as needed, usually within 48 hours.³⁹
4. When clearance is unsuccessful and/or there is clinical deterioration, surgical exploration is indicated.³⁹

Complicated Meconium Ileus

Complicated MI always requires surgery, usually involving resection and placement of a temporary stoma.

1. Following surgery, deconcentrating agents can be continued through the stoma.
2. Continuous enteral feeding or total parenteral nutrition (TPN) is required.
3. Once oral feeding begins, it is usually with predigested formulas. Vitamin and pancreatic enzyme supplementation is added as needed³⁹

DIOS and Fibrosing Colonopathy

Abdominal pain is a frequent complain in patients with CF. Although there are many causes of abdominal pain, DIOS and fibrosing colonopathy are unique to CF.

DIOS is given a suitable trial of medical management to promote clearance of the obstruction and passage of stool. Mild symptoms are usually treated with adequate hydration and oral osmotic laxatives.²⁸ Radiography is often used to document clearance. If the patient has complete obstruction or evidence of peritonitis, surgery is necessary.³⁹

Initial management of fibrosing colonopathy is to reduce pancreatic enzyme dosage, and to provide adequate nutritional supplementation. If the patient continues to deteriorate, surgery may be required. The affected bowel is resected and a primary anastomosis is made. If the rectum is involved, the patient will require an ostomy. The patient is regularly monitored for any signs of deterioration or recurrence.³⁹

Other digestive Disorders

Some causes of abdominal pain that occur in the population at large may occur with greater frequency in patients with CF.

GERD

Gastroesophageal reflux (GERD) can exacerbate respiratory dysfunction. It is treated with medications to reduce acidity, alternatives to head down positioning of physiotherapy to clear mucus, and in some cases, surgery to tighten the lower esophageal sphincter (LES). The LES is the ring of muscle fibers between the esophagus and stomach that maintains tone at times other than swallowing.^18,39 Additionally, GERD medication may increase the effectiveness of pancreatic enzymes by reducing acid in the gut.

Rectal Prolapse

Rectal prolapse occurs in approximately 20% of patients, most commonly those aged 1 to 3 years, and can be recurrent. Initial management involves manually reducing the prolapse and maximizing fat absorption by adjusting the dose of pancreatic enzymes. Recurrent episodes may be treated with rectal submucosal injection of 5% phenol in almond oil or hypertonic sodium chloride solution (30%). When all conservative options are exhausted, surgical intervention may be considered, although results or surgical procedures are often unsatisfactory.³⁹

Cancer

There is a higher risk of digestive tract cancers in patients with CF. Patients are monitored for signs of developing tumors in various organs of the GI tract.³⁹

References:
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.
39. Irish MS. Surgical Aspects of Cystic Fibrosis and Meconium Ileus. eMedicine com 2006 July 31;1-25. Accessed March 6, 2007.