Gall Bladder Issues in Cystic Fibrosis

The gall bladder is a small, pear-shaped organ that connects to the liver by a series of ducts. As part of the digestive system, the gallbladder’s role is to store bile (a bitter liquid produced by the liver) that will be used to digest food, particularly fat, on its way to the small intestine.

Nearly every cystic fibrosis (CF) patient has or will develop gallbladder problems at some point during his or her lifetime. In people with CF, the gallbladder is abnormally small and fails to function properly due to blockage by thick bile. In many cases, the gallbladder becomes atrophied because of non-use. Gall stones may form when the gall bladder fails to empty properly for long periods of time. In cases where the gall bladder becomes blocked because of gall stones, the risk of pancreatitis (swollen pancreas) increases.

Symptoms that may indicate gallbladder irregularities include:

-abdominal pain in the pit of the stomach

- pain and discomfort when eating

-difficulty taking deep breaths

-vomiting

-pale stools

-dark urine

-abnormal or unusual bloating with indigestion

In order to determine if the gall bladder is functioning properly, or if gall stones are creating a problem, your doctor may order imaging tests, such as an ultrasound, abdominal CT scan, or x-rays. One of the most effective ways to see how well the gall bladder is working is by performing a CT scan with contrast. In this type of scan, a special type of dye is injected into the patient’s arm. The CT scanner will then take a series of images as the dye moves from the liver to the bile duct, gall bladder, and ultimately, small intestine.

Occasionally the gall bladder will need to be surgically removed. This is often done via laparoscopy, a procedure in which only a few small incisions are made in the abdomen. This type of surgery is relatively simple, typically performed on and outpatient basis. Removal of the gall bladder will not impair digestion. The only difference is that your body will no longer be able to store bile between meals. Once the gall bladder is removed, bile will move directly from the liver to the bile duct and into the small intestine.

According to Dr. Brian K. Weinstein, "Up to 27% of patients with CF have gallstones, but only about 4% are symptomatic. Biliary colic is the pain associated with stones as they pass. This pain usually passes, but can recur. When the gallbladder is swollen, with pain, this can be cholecystitis, an inflammation and infection of the gallbladder. This can initially be treated with antibiotics, but usually is an indication for surgery."

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Supporting links

Gall Bladder Disease in Cystic Fibrosis

The Gall Bladder and Biliary Tract in Cystic Fibrosis

Bile Acid Secretion in CF: Evidence for a Defect Unrelated to Fat Malabsorption