Normal Function
The liver, a large organ found in the lower right side of the abdomen, has the job of filtering blood and removing harmful toxins from the body. The liver also produces bile, a fluid needed to digest fats. The gallbladder, a small, pouch-like structure underneath the liver, stores the bile that is made by the liver, and releases it when it is needed in the small intestine.
| Click on the two buttons above to compare normal bile flow to bile flow with CF. Normal: Bile flow of the liver into the small intestine aids digestion. With CF: Thick bile in CF patients blocks the bile ducts in the liver.
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Abnormal Function in Cystic Fibrosis
In CF patients, the bile, which helps break down fats we eat, tends to be drier and more acidic than normal bile. Also, the liver produces less of it. And because it cannot balance out the acidic contents of the small intestine the way it is supposed to, the lining of the small intestine can be damaged. Also, bile can collect in the gallbladder and produce gallstones, which can cause pain, fever, nausea, and vomiting. If this happens, the gallbladder may need to be removed. Thicker bile may also obstruct bile ducts in the liver. Since the liver has trouble digesting fat, the result can be hepatic steatosis, or “fatty liver.” Found in up to 60 percent of CF patients, it causes more liver problems.
Cirrhosis and Portal Hypertension
Cirrhosis of the liver is a serious condition that kills liver cells. How does this happen? Often, liver bile ducts will be blocked by dried-out mucus and bile, inflaming the liver and damaging its overall function.
Fibrosis, or scarring, of the liver ducts affects blood flow to the liver, it sends blood to other vessels. These vessels are unable to handle the extra blood, and they swell and can bleed. The result might be liver failure, a potentially fatal condition that allows poisonous substances normally removed by the liver to remain in the body. Your doctor will check your liver and spleen during each clinic visit. Each year, they'll do special tests to check for liver-enzyme levels, and for toxins and infections. Radiology tests can identify a blocked liver duct or gallbladder, changes of the liver that show cirrhosis, and changes that show steatosis, or fatty liver. Good nutrition and taking care of yourself can help prevent liver disease. | | Severe CF liver disease can lead to cirrhosis or scarring in the liver. The liver enlarges and there is increased blood pressure in the liver that causes the spleen to enlarge as well. |
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