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Management of Cystic Fibrosis

Management of the Gallbladder and Liver

Gallbladder

Defects in CFTR result in thick “white bile” production in the gallbladder that clogs the cystic ducts, forms gallstones, and eventually causes sclerosing cholangitis.

Bile acid metabolism is disrupted in patients who have pancreatic insufficiency, so enzyme supplementation, as well as fat-soluble vitamin supplementation, is needed for management. Patients are also treated with ursodeoxycholic acid to de-concentrate bile and improve flow. Eventually, patients may need laparoscopic surgery for removal of the gallbladder. During the surgery, a liver biopsy is usually conducted as well.^18,28,39

Source: National Digestive Diseases Information Clearinghouse (NDDIC). Gallstones: What are gallstones? Assessed February 2009¹⁰

Liver

Steatosis (fatty liver) and liver enlargement occur as a result of pancreatic insufficiency and the absence of bile. Pancreatic enzyme replacement is administered to prevent the progression of cirrhosis.

When esophageal varices develop as a result of portal hypertension, beta blockers may be prescribed to prevent the onset of variceal bleeding. In patients with large varices who do not tolerate beta blockers, tying off the varices endoscopically is an option.  Patients at high risk often require surgical placement of a portal vein shunt.¹⁸

Prophylactic Therapy

It is recommended that all patients with CF liver disease receive prophylactic therapy, such as:

• Immunization for hepatitis A and hepatitis B, or documented evidence of previous infection with the viruses
• Advice about the risks of drinking alcohol
• Advice about the potential adverse effects on the liver of certain medications and herbal therapies¹⁸

References:
10. National Digestive Diseases Information Clearinghouse (NDDIC). Gallstones: What are gallstones? Assessed February 2009
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.