Hereditary spherocytosis (sfir-oh-sye-TOE-sis) is a blood condition that runs in families. It happens because red blood cells (RBCs) are round like a sphere instead of disk-shaped. These red blood cells (called spherocytes) are fragile and get stuck in the spleen. This leads to anemia, an enlarged spleen, and other medical problems. Treatment can help your child lead a healthy, active life.

• Give your child any vitamin supplements or prescribed medicine as directed by the health care provider.
• Anemia can make your child tire easily. Let your child rest when needed.
• An enlarged spleen is easily injured. Talk to the health care provider about which activities your child should avoid.
• Tell your child's teachers and other caregivers about the condition, as well as symptoms they should watch for or precautions to take with activities.
• Be sure to follow up with your child's health care provider and go to all checkups. 
• Follow any other instructions your health care provider gave you.

Your child has:

• new, worse, or lasting fatigue (tiredness)
• a yellow tinge to the eyes or skin (jaundice)
• belly pain, or belly pain that also can be felt in the back
• loss of appetite
• fever
• headaches or dizziness

Your child:

• has trouble breathing, an irregular heartbeat, or chest pain
• is extremely tired or pale
• can't be awakened
• has red, brown, or tea-colored pee
• has severe belly pain

Why can the spleen get enlarged in hereditary spherocytosis? The spleen is an organ near the ribcage that clears worn-out RBCs and other foreign bodies (such as germs) from the blood. When many spherocytes collect in the spleen, it becomes enlarged.

Why do some kids with hereditary spherocytosis get jaundice? When RBCs break down, they release a colored substance called bilirubin. Many RBCs break down in hereditary spherocytosis, so there's more bilirubin in the body than normal. The higher level of bilirubin causes the jaundice and can also cause gallstones. 

How is hereditary spherocytosis treated? Treatment depends on a child's symptoms. Some kids don't need any treatment. Newborn babies with jaundice may get light therapy (phototherapy) where the light changes the bilirubin to a form that can easily pass out of the body. In kids with anemia, the health care provider may prescribe the vitamin folic acid to help the body make more blood cells. Sometimes other medicines or blood transfusions are used to treat anemia.

Sometimes, surgery to remove all or part of the spleen (a splenectomy) can help. Most symptoms then go away because the spleen is no longer destroying RBCs. Often, surgeons wait to do a splenectomy until a child is older, though, because the spleen plays an important role in fighting infection in the first few years of life. To help prevent infections, a child might get some extra vaccines before the splenectomy and take daily antibiotics after it.