Select from the menu above to compare the normal bile flow to the 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.

Abnormal Function in Cystic Fibrosis
The effects of cystic fibrosis on the liver and gallbladder result in problems with bile production. This bile is dehydrated and more acidic than normal bile, and its flow into the small intestine is reduced. Because the acidic contents of the small intestine can not be neutralized, the lining of the small intestine may be damaged. Dehydrated bile can collect in the gallbladder and produce gallstones, which can cause inflammation of the gallbladder and symptoms such as pain, fevers, nausea, and vomiting. If this occurs, the gallbladder may need to be removed.

The liver is also affected by dehydrated bile if mucus obstructs the bile ducts in the liver. Since the liver has trouble digesting fat, the result can be hepatic steatosis, or “fatty liver,” which occurs when fat collects in the liver. Found in up to 60 percent of CF patients, this further worsens liver function. 

Cirrhosis and Portal Hypertension
Cirrhosis of the liver is a serious complication that causes the destruction of liver cells.  Often, liver bile ducts will be blocked by dehydrated mucus and bile, leading to inflammation and damage to overall liver function. Eventually, several areas of the liver may be affected. 

Fibrosis, or scarring, of the ducts affects blood flow to the liver. Blood is redirected to other blood vessels, but because these vessels are unable to accommodate the increased volume, they swell and are prone to bleeding. The result might be liver failure, a potentially fatal condition that allows toxic substances normally removed by the liver to remain in the bloodstream.

The liver and spleen should be examined each clinic visit. Yearly liver tests should check liver enzyme levels, and for toxins and infections. Radiographic tests can identify a blocked liver duct or gallbladder, changes of the liver that show cirrhosis, and fatty changes that show steatosis. Good nutrition and managing complications 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, too.