Liver Disease in Cystic Fibrosis
Why is liver disease a concern to people with cystic fibrosis (CF?)
About 5-7% of people with CF have liver disease. There are two reasons why this problem has not received much notice until recent years. First, liver disease occurs silently over time. Therefore, people do not notice or have symptoms for many years. Also, the symptoms most people focus on are respiratory (lung) and digestive (pancreatic) problems. In recent years, there have been many advances in CF care and research. People with CF can now expect to live longer lives. This makes early detection of liver problems even more important.
What are some of the complications of liver disease?
Due to the bile being sticky in people with CF, bile ducts can become blocked. This blockage (cholestasis) can prevent a normal flow of bile through the duct. Fibrosis of the liver tissue can occur over time, and may lead to scarring (cirrhosis). If the liver becomes scarred, the blood cannot get through the liver at the normal pace. This can create a higher than normal pressure on the main vein feeding the liver (portal vein). This is known as portal hypertension.
What are the symptoms of liver disease?
People with CF who have liver disease may have some of these symptoms:
- A change in blood tests called liver function tests (LFTs)
- Enlarged liver or spleen
- Bruising or bleeding
- Fluid pooling in the abdomen
- Nausea or belly pain
- Weight loss or poor weight gain
- Change in the color of your stool and how often you have a bowel movement
What does a change in liver function tests (LFTs) mean?
A change in LFTs is only one finding that may suggest liver problems. These tests will be repeated over time. Your health care provider may also order a scan that looks at your liver and spleen. LFTs that remain high, and knowing whether your liver is enlarged, will help your health care provider figure out the source of the problem and decide on the best treatment.
What is the treatment for liver problems?
There are many sources of liver problems in people with CF. Once the source has been figured out, your health care provider may prescribe an oral medicine or a special diet. One of the most common drugs used is Ursodiol (pronounced er–so-DI-all). This pill is taken one to three times a day to help increase bile flow which improves liver function. Side effects, which are rare, consist of itching and diarrhea. In a very small number of people with CF who have severe liver disease, a liver transplant may be needed.
Checking for liver disease before symptoms occur is the best practice. The CF Clinical Care Guidelines state that LFTs should be checked yearly. Once LFTs start rising, early treatment to preserve healthy liver function is crucial.
For questions or concerns, call the CF Center at 608-263-6420.
If you live out of the area, call 1-800-323-8942 and ask for the CF Center.
The information provided should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Any duplication or distribution of the information contained herein is strictly prohibited.
Last Updated: 08/23/2011
Copyright © 08/23/2011 University of Wisconsin Hospitals and Clinics Authority. All rights reserved. Produced by the Department of Nursing. HF#6186
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