Sickle Beta+ Thalassemia

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WHAT IS SICKLE B+ THALASSEMIA?

Sickle B+ Thalassemia (Sickle BA-ta Plus Thal-a-SEE-me-a) is a "mild" form of sickle cell disease. Your child's red blood cells contain an abnormal hemoglobin called hemoglobin S or sickle hemoglobin in addition to a small amount of the normal hemoglobin called hemoglobin A. The red blood cells have a defect called beta plus thalassemia, which results in cells which are small in size and more pale than usual.

Instead of appearing round or like donuts, your child's red blood cells are somewhat small, pale and misshapen. Because Sickle Beta Plus Thalassemia (sickle-thal) is inherited, it is a lifelong disorder. There is no treatment or cure for the disorder. Your child will always have a mild anemia or a slightly low blood count. This may cause occasional tiredness and weakness.

body with arrows to areas the pain can be present

PROBLEMS SEEN IN CHILDREN WITH SICKLE B+ THALASSEMIA

Painful episodes can occur with sickle-thalassemia. The sickled red blood cells in sickle-thalassemia, somewhat like those in sickle cell anemia, are rigid and stiff and may sometimes cause "log jams" in the small blood vessels in the bones, organs, and other parts of the body. Since oxygen (which is carried by the red blood cells) cannot get past the "log jams" into the bones and organs, this can cause pain. These painful episodes occur most commonly in the back, stomach, arms, and legs. They can last for a few hours to as much as a week or more. Occasionally, the pain is accompanied by swelling. Painful episodes can vary in severity of the pain and the duration. There are medicines to help ease the pain and make your child more comfortable.

Your child has an increased risk of getting infections, especially pneumonia. The abnormal sickled red blood cells "clog up" the lungs and thus increase the risk of infection there.

Sometimes children with Sickle Beta Plus Thalassemia have a slightly enlarged spleen, but this usually does not cause any problems.

IT IS IMPORTANT TO INFORM ANY PHYSICIAN THAT SEES YOUR CHILD THAT HE OR SHE HAS SICKLE BETA PLUS THALASSEMIA!

WHAT YOU CAN EXPECT

A child with Sickle B+ Thalassemia has the likelihood of a normal life-style and life span. Your child should not be considered "sick", and you should treat him or her normally. He/She will need to be seen regularly by your family doctor for regular check-ups and vaccinations. Your child will also need to be seen each year by a hematologist or blood specialist.

For more information about the Newborn Screening Program please contact:
Texas Department of State Health Services
Newborn Screening Unit M-555
1100 West 49th Street
Austin, Texas 78756
MC 1918
Phone: 1-800-252-8023, ext. 3957  


 

Last updated December 07, 2010