Splenectomy and Sickle Cell Disease

About 1 in 4 young children with sickle cell disease (SCD) experience a complication called splenic sequestration crisis. This happens when the spleen enlarges because of trapped red blood cells. It causes potentially life-threatening anemia.

Treatment of splenic sequestration crisis usually involves blood transfusions to release the trapped red blood cells. However, your doctor may recommend that the spleen be removed by a surgical procedure called splenectomy. This prevents further splenic sequestration episodes.

The major risk of having the spleen removed is an increased risk of severe infection. Your doctor may recommend long-term antibiotics and additional vaccinations to reduce the risk of infection.

What is a splenectomy?

Splenectomy is a surgical procedure to remove the spleen. The spleen is an organ on the upper left side of your abdomen. It is an important part of the immune system that helps fight certain infections and filters out waste from your blood.1

Other organs perform many of the spleen’s functions. This means it is possible to live a normal healthy life without a spleen. Surgical removal of the spleen is often done to remove a spleen that has ruptured following an injury. It is also performed to treat an enlarged spleen, some blood disorders, and some cancers.1

Doctors usually perform a splenectomy with a tiny video camera and special surgical tools. After the surgical removal of the spleen you may be able to leave the hospital the same day and fully recover within a few weeks.1

When is surgery performed?

Doctors sometimes perform a splenectomy after a life-threatening complication of SCD called splenic sequestration crisis. This happens when sickle cells get trapped in the spleen and make it grow too large. Fewer red blood cells are then available to circulate in the blood, causing severe anemia.2,3

The first treatment for a splenic sequestration episode is usually a blood transfusion. This helps the body release sickle cells that are trapped in the spleen and improves the anemia. However, the risk of having a splenic sequestration episode increases after the first episode, so your doctor may recommend splenectomy to prevent further episodes.4,5

We do not yet know when and at what age the procedure should be performed. In a study of children who experienced a splenic sequestration crisis, about 1 in 3 had their spleen surgically removed. In nearly all cases, splenectomy was performed after the second episode.

Most children who have their spleen removed first receive chronic blood transfusions. Sometimes, blood transfusions can delay the procedure until an age when the risk of infection is lower.4

How should I prepare for the procedure?

Your doctor will explain what you need to do before the procedure. You may need to avoid eating or drinking for some amount of time. You may also need to stop taking certain medicines. Your doctor may give you blood transfusions before the procedure to make sure you have enough red blood cells.1

Before the procedure, you will be given anesthesia, and doctors will monitor your heart rate and blood pressure. During the procedure, your doctor will make small incisions in the abdomen to place a small video camera and surgical tools. After removing the spleen, they will close the incisions. This is called a “laparoscopic splenectomy.” If the spleen is larger, your doctor may instead perform an “open splenectomy” with a larger incision, generally under the left side of the rib cage.1

What are the risks?

Splenectomy is usually a safe procedure. However, there are potential risks, especially for people with SCD. Some of these risks include:1

  • Bleeding
  • Blood clots
  • Infection
  • Injury to nearby organs

Since people with SCD are already more vulnerable to severe infections, your doctor may take extra precautions to prevent infections. You may continue to take prophylactic (preventive) penicillin through adulthood. You may also need to get additional vaccinations, if you have not already.3

Get emergency help and talk to your doctor if symptoms of an infection appear, including:1

  • Fever
  • Redness or tender spots
  • Sore throat
  • Chills

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Written by: Matthew Zajac | Last reviewed: January 2021