Gallstones and Sickle Cell Disease

People with sickle cell disease (SCD) often experience gallstones. These are solid, rock-like structures that form in the gallbladder. The condition is also called cholelithiasis.

Gallstones form in SCD when a compound called bilirubin builds up in the gallbladder. This happens when too many sickle cells burst and release their contents into the bloodstream.

Gallstones can lead to gallbladder inflammation and other serious health problems. If you have abdominal pain, jaundice, or other symptoms, talk to your doctor immediately. They can perform tests to determine if you have gallstones and decide on the best treatment.

What are gallstones?

Gallstones are hard deposits that form in the gallbladder. The gallbladder is an organ that stores digestive fluid made by the liver and releases it into the intestines. Gallstones can range in size from a grain of salt to a golf ball.¹

Different materials in the body can accumulate and form gallstones. In people without SCD, most gallstones are made up of cholesterol. In people with SCD, most gallstones are formed from bilirubin. About 25 percent of people with SCD develop gallstones.^1,2

Gallstones often do not cause any harm. People can live their entire life with gallstones and never have a problem. However, gallstones can lead to serious health complications, such as cholecystitis. This is gallbladder inflammation, and it often requires surgical removal of the gallbladder. Gallstones can block the duct from the liver to the intestines and cause severe pain, nausea, and vomiting. Complications of gallstones can also lead to an acute sickle cell crisis.^2,3

Why does sickle cell disease cause gallstones?

Normal red blood cells circulate in the blood for about 120 days. When they burst, they release hemoglobin, which gets broken down into a substance called bilirubin. This is an orange-yellow pigment responsible for the color of bruises and skin color in jaundice.⁴

The sickle cells of people with SCD are unstable and only circulate for about 20 days. When too many sickle cells burst, a large quantity of bilirubin can form stones that are known as calcium bilirubinate.^2,5

Gallstones are most common in people between 11 and 29 years old. Men and women are equally likely to get them. People with sickle cell anemia (HbSS) are more likely to have gallstones than people with sickle hemoglobin C (HbSC).^2,3

Symptoms

Gallstones may cause discomfort when they collect in the gallbladder. They can also cause inflammation of the gallbladder, or cholecystitis. If left untreated, this can lead to life-threatening complications, such as gallbladder rupture. Some symptoms of gallstones and gallbladder inflammation include:^1,6

• Pain in the abdomen
• Fever
• Nausea and vomiting
• Yellow skin and eyes (jaundice)
• Dark urine
• Clay-colored stool
• Severe pain in the upper abdomen that moves to the shoulders and back

If you experience any of these symptoms, call your doctor. They can perform blood tests and imaging of your abdomen to determine if you have gallstones.

What are treatment and prevention options?

We do not yet have any medications that prevent gallstones. There is no evidence that hydroxyurea reduces the incidence of gallstones. The best way to decreases chances of developing gallstones is through lifestyle changes, such as:^1,2

• Losing weight
• Eating a healthy diet low in fat and high in fiber
• Exercising
• Avoiding smoking and alcohol
• Drinking plenty of water

If you have gallstones, treatment will usually include:¹

• IV fluids to keep you hydrated
• Medications to relieve nausea, vomiting, or pain
• Antibiotics if you develop a fever
• Surgical removal of the gallbladder

Many people who have gallstones have their gallbladder removed. This surgery is usually successful and causes few complications. However, it is usually only done after symptoms appear and other treatments do not work.²