At a glance
Sickle cell disease (SCD) is a group of inherited blood disorders associated with severe pain and complications that can affect the entire body. This page provides information on SCD and liver problems.
Overview
The liver is an organ that helps the body digest food and remove toxins. Sickled cells in the liver can cause damage to the liver, leading to liver disease. Additionally, some persons with SCD receive repeated blood transfusions, which can result in excess iron in the body, known as iron overload. Iron overload can also damage the liver. Sickle hepatopathy is a term used to describe the different causes of liver disease in people with SCD.
Health problems
A few health problems that can occur include acute sickle hepatic crisis, intrahepatic cholestasis, and cholelithiasis.
Acute sickle hepatic crisis is when sickled cells in the blood vessels cause a pain crisis occurring in the liver.
Intrahepatic cholestasis occurs when sickled cells block blood flow in the liver. The blockage prevents oxygen from reaching the liver, damaging the liver.
Cholelithiasis is when gallstones (hard, rock-like "stones") form in the gallbladder (an organ behind the liver that stores and releases bile to help break down fatty foods). When red blood cells break down, they release bilirubin. Sickled red blood cells break down faster than healthy red blood cells, producing excess amounts of bilirubin, which can lead to the formation of gallstones. Bilirubin is a yellowish substance that is made during the body's normal process of breaking down red blood cells. A healthy liver will mostly remove bilirubin from the body.
Symptoms
Symptoms can vary by the liver condition, but common symptoms of liver problems can include:
- Pain in the upper right side of the abdomen (belly)
- Nausea
- Vomiting
- Yellowing of the eyes and skin (jaundice)
Resources
CDC resources
5 Steps to Safer Blood Transfusions if You Have Sickle Cell Disease
Other resources
Blood Transfusions: What You Need to Know and Do (Video Series), Episode 5: All About Iron
Liver Disease | National Institutes of Diabetes and Digestive and Kidney Diseases
Liver Disease | National Library of Medicine, MedlinePlus