Jaundice and Sickle Cell Disease

Jaundice is a common early symptom of sickle cell disease (SCD). It happens when sickled red blood cells die faster than the liver can filter them out. Free hemoglobin, a protein in red blood cells that carries oxygen throughout the body, gets converted to a pigment that collects and turns the skin and eyes yellow.¹

Jaundice may be treated with drugs that reduce the sickling of red blood cells. It may also be treated with blood transfusions that increase the number of healthy red blood cells.

What is jaundice?

Jaundice is a condition where the skin and whites of the eyes turn yellow. The yellow color is caused by a high level of bilirubin, which is a yellow-colored pigment. Bilirubin is normally made as the body breaks down hemoglobin, and is excreted in bile and urine. It is also what causes the yellow color of bruises.²

A high level of bilirubin is usually a sign of an underlying disease. Diseases that affect bilirubin metabolism, liver function, or gallbladder function can cause jaundice. This includes sickle cell disease, hepatitis, cirrhosis, pancreatic cancer, and many other conditions.³

How does sickle cell disease cause jaundice?

Normal red blood cells live for about 120 days. They then burst and spill their contents, including hemoglobin, into the bloodstream. Special white blood cells then clean up hemoglobin and turn it into a mixture called bilirubin. Bilirubin then travels to the liver, where it gets turned into a form that is sent to the gallbladder. There, it gets turned into other mixtures that give feces and urine their typical colors.⁴

People with SCD have abnormal red blood cells called sickle cells. Sickle cells do not survive as long as normal red blood cells because they are rigid and break apart easily. When sickle cells die faster than the liver can filter them out, bilirubin builds up. It then deposits around the skin, whites of the eyes, and mucous membranes, causing a yellowish appearance.^4,5

Liver disease is also common in people with SCD. Liver dysfunction can be caused by blocked blood flow to the liver or by complications of the disease, such as gallstones. This can also lead to high levels of bilirubin and jaundice.⁵

For many people with SCD, jaundice may be the most visible aspect of the disease. It can negatively impact their quality of life, especially self-esteem and social wellbeing.⁶

How is it treated?

Treatment depends on the underlying disease. People with SCD who experience jaundice may be treated with hydroxyurea or blood transfusions.⁶

However, multiple blood transfusions can increase iron levels in the blood. Too much iron can damage the liver, heart, pancreas, and other organs. This can cause jaundice, as well as other health problems. Regular blood transfusions should be given with iron chelation therapy to reduce excess iron.⁷

Some people with SCD and chronic jaundice have also been treated with voxelotor. This is a drug that prevents red blood cells from sickling. It may reduce the destruction of red blood cells, which will lower the amount of bilirubin in your body.⁸

You can also help reduce jaundice by maintaining liver function. Eating a balanced diet, exercising regularly, and reducing alcohol consumption will keep your liver healthy. Talk to your doctor for specific advice in treating jaundice. They may advise lifestyle or medication changes depending on your situation.