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| |    Sickle Cell Disease and Elective Splenectomy by Lewis Hsu, M.D., Ph.D. and Laura Jana, M.D., F.A.A.P. reviewed by Allan Platt, PA-C It is not uncommon for a doctor to recommend that a child with sickle cell disease have her spleen removed, a procedure known as elective splenectomy. If you find yourself in this situation, here are a few things to consider. The effects of sickle cell disease on the spleen It's an unfortunate fact that the spleen loses function very early in life in children with the HbSS type of sickle cell disease (the type in which the child has received a gene for sickle cell from both her parents). Studies from about 15 years ago show that 20 percent of children with HbSS lose spleen function by one year of age, and over 40 percent lose spleen function by two years of age. For young patients with enlarged spleens and splenic sequestration, nearly 100 percent had lost spleen function. Infection risks Parents often fear that removing the spleen will put their children at greater risk for infection. A couple of years ago, the Jamaican Sickle Cell Center showed that children who had surgical removal of the spleen did not have more infections than sickle cell children without spleen surgery. Keep in mind, however that all children with sickle cell disease-spleen or no spleen-need to be watched carefully for infection because they are more vulnerable than children without the disease. What are the risks of splenectomy? While there are risks associated with having the spleen surgically removed, they are not as significant as the risks of a procedure such as heart surgery. Potential complications include sickle cell pain, breathing problems leading to sickle acute chest syndrome, surgical infection, bleeding, and difficulty recovering bowel function. The key factors in reducing the risks of splenectomy are:
The traditional approach is through an incision in the left upper abdomen. A new approach gaining in favor is laparoscopic splenectomy, which involves much smaller incisions that allow a periscope-like surgical tool access to the spleen. Using this special tool, the surgeon is able to tie off the blood vessels, put a plastic bag around the spleen, and carefully remove it in pieces small enough to fit through the small incisions. What happens after splenectomy? Your child will recover in the hospital from his surgical wounds, receive intravenous fluids until he is able to drink well, and receive postoperative pain medications. Most doctors also keep children on long-term oral antibiotics to reduce the chances of a serious bacterial infection in the future. What happens if the spleen is not removed in a child with sickle cell? Some children suffer episodes of splenic sequestration and require chronic blood transfusions--a preventive measure associated with its own potential risks and complications. In some children, the spleen may never cause splenic sequestration, but it becomes scarred and useless, its red blood cell filtering capability overwhelmed, and its immunologic function destroyed.
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