Sickle beta zero thalassemia (ST0) is similar to sickle cell anemia; this means the red blood cells contain an abnormal hemoglobin called hemoglobin S or sickle hemoglobin. The red blood cells also have a defect called thalassemia, which results in cells that are small in size and more pale than usual. The cells are also not round or donut shaped, but may appear sickled or banana shaped. Click here to see the difference between a normal and sickled red blood cell.
How Sickle Beta Zero Thalassemia is Inherited
Possibilities with each pregnancy. There is a 25% chance of having a baby with sickle beta zero thalassemia with each pregnancy.
Problems Seen in Children with Sickle Beta Zero Thalassemia
Infection. Like sickle cell anemia, your child has an increased risk of getting infections, such as pneumonia. Because these children are more susceptible to infections, they are frequently put on penicillin to help prevent these infections from occurring. It is important that your child take penicillin as directed. Any fever of 100.7 Fahrenheit should be taken seriously and your child taken to your family doctor or clinic immediately!
Pain. The red blood cells of ST0 disease are stiff and don't move easily. Sometimes they clog up the small vessels in the bones and other parts of the body. If the oxygen carried by the red cells can't get to these small vessels, it can cause pain. Usually this pain occurs in the arms, legs, back and stomach. Sometimes the pain lasts only for a few hours and sometimes it lasts for as long as a week. The pain is not the same every time. Some children have no pain at all. Your doctor can give you pain medicine guidelines to help your child through these pain crises.
Spleen. The spleen is a small organ located on the upper left side of the abdomen up under the rib cage. It helps the body to fight infection. Children with this disease may have a bigger spleen than other children, but it usually does not cause any problems.
What You Can Expect
A child with sickle beta zero thalassemia can have a normal lifestyle and life span. These children should not be considered "sick" and can be treated normally. Be sure to receive regular checkups and vaccinations. Your child will also need to make several visits a year to a hematologist or blood specialist.
