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The Purpose of Blood Tests in Sickle Cell Disease

by Lewis Hsu, M.D., Ph.D.
reviewed by Laura Jana, M.D., F.A.A.P.
Sickle cell is a disease of the red blood cells. It has many potential complications--not all directly related to the abnormal sickle shape of the red blood cell. Blood tests can prove useful both for identifying the particular cause of an existing problem and for their potential to help prevent complications.

Measuring for comparison purposes
Healthcare providers try to measure base-line blood counts during regular checkups, when children generally are feeling well. The blood counts vary not only from child to child, but over time from one age to the next. Established base-line blood counts help determine whether a child's blood cell counts are higher or lower than usual, which can signal complications.

What are doctors looking for?
Sickle cell tests examine red blood cells for two things in particular: how much pigment is in the blood (hemoglobin) and the proportion of red blood cells in the blood (hematocrit). When the hemoglobin and hematocrit levels drop below base line, it suggests the occurrence of a complication. Results that measure higher than base line typically are caused by dehydration or an excessive blood transfusion and can signal that the blood is too viscous (thick) and that the child needs extra fluids or some other kind of treatment.

The reticulocyte count measures the body's production of red blood cells. Lower-than-normal reticulocyte counts, or a reticulocyte count of zero, suggests that the body has slowed down--or shut off--production of red blood cells (called an aplastic crisis). This usually is a temporary problem caused by a viral infection or a medication; nonetheless, it is very important that your child's doctor keeps a close eye on it. Some infections, medications, or other treatments also can cause red blood cells to break down faster than usual (hemolytic crisis), which also lowers blood counts.

The white blood cell (WBC) count measures cells that fight infection. A higher-than-base-line WBC count can help determine whether to treat a fever as a major, rather than a minor, infection. This is particularly important in children with sickle cell disease, since they are more susceptible than other children to certain types of infections.

The platelet count measures cells that aid blood clotting. A lower-than-base-line platelet count can indicate that the spleen has become enlarged and trapped blood cells (splenic sequestration). It also might be a sign of an infection that can trap blood cells in the lungs (acute chest syndrome).

Evaluating fever
If your child has a fever, blood tests are an important way to judge the severity of the situation. In general, it's good news if the blood counts remain stable. If the counts are not at base line, however, they may indicate a complication of sickle cell disease and call for a different type of treatment than those typically used to remedy a fever. The potentially greater risks associated with infection in children with sickle cell disease means that your doctor is likely to check your child's blood counts if you bring her in for a fever.

 RELATED INFORMATION
*  General Medical Care for Children With Sickle Cell Disease
*  Infections In Sickle Cell Disease: Overview
*  Sickle Cell Disease


Created February 09, 2001
Reviewed February 11, 2001
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