Splenic sequestration in children with sickle cell disease

What causes the spleen to become larger?

When sickled cells are trapped in blood vessels leading out of the spleen ins tea of flowing through it. This is called sequestration. When this happens, the spleen is very large and it is easy to feel. Sometimes sequestation is painful. Medication can be given to ease the discomfort.

What does the spleen do?

The spleen is normally a small organ located under the rib cage on the left side of the body. It functions as part of the body’s defense against infection by removing bacteria (germs) from the bloodstream. In the child with sickle cell anemia, the spleen is not able to remove bacteria, so bacteria can grow in the bloodstream and cause an infection of the blood called septicemia.

Is splenic sequestration a serious problem?

If the spleen enlarges suddenly, the red blood cell count may drop causing severe anemia. This is a serious and potentially life-threatening problem. When the spleen gradually gets larger over several weeks, the blood count does not change as much, so it is not as serious. Any enlargement of the spleen is of concern and must be watched for changes. Parents should check their child’s spleen every day , particularly when the child is sick. They should know how their child’s spleen usually feels so that whenever the child seems sick they can check the spleen to see if it’s bigger. If the spleen suddenly becomes larger, a doctor should check the child as soon as possible. If the child also has symptoms of a low blood count, then the situation becomes an emergency.

What are the symptoms of splenic sequestration?

  • Irritability
  • Pain on the left side of the abdomen
  • Weakness
  • Fast heart rate
  • Big spleen
  • Unusual Sleepiness
  • Looks pale

What are the symptoms of low blood count?

A child with a low blood count is likely to be irritable, unusually sleepy, and has a rapid heart beat. If the lips and fingernails do not have any pink color even when the fingers are warm, the child is pale. A child can have a seriously low blood count without many symptoms. Sometimes the only symptom is that the child is less active.

At what age do problems with the spleen usually occur?

Babies and young children are at greatest risk of splenic sequestration and septicemia. Complications can develop as young as 2 months of age, but usually occur between the ages of 6 months and 5 years. As children get older, the spleen becomes smaller. Eventually, it may not enlarge anymore.

Can splenic sequestration happen more than once?

Yes. A child who has one episode of splenic sequestration is likely to have other episodes.

What is the treatment for splenic sequestration?

If the blood count is dangerously low, then red blood cell transfusions is the treatment. If a child has several episodes, surgery to remove the spleen, called a splenectomy, may be recommended.

What can be done to help children with sickle cell disease fight infection since the spleen does not work?

Prevention and early treatment of infection is the best defense against serious complication. The child who is sick should be carefully watched for symptoms of serious infection. A fever of 101 degrees or higher should always be considered a symptom of possible septicemia or bacteria in the blood. Penicillin may be given twice daily to prevent infection. Pneumococcal vaccines, given in the form of a shot, are an option that may be given to some patients to boost immunity against harmful infections.