Sickle Beta 0 Thalassemia
Also known as: sickle beta thalassemia, sickle cell beta thalassemia.
What is sickle beta 0 thalassemia?
Sickle beta 0 thalassemia is an inherited disease. It causes the red blood cells to have abnormal hemoglobin. This is called hemoglobin S (sickle hemoglobin) with another problem called thalassemia. It causes the body’s hemoglobin, or red blood cells, to take on a sickle shape and not flow through the blood vessels as smoothly. This can cause a number of complications. The “zero” indicates that the blood has no normal hemoglobin. This differs from sickle beta + thalassemia, in which a person has a lower than normal amount of normal hemoglobin.
What causes sickle beta 0 thalassemia?
Sickle beta 0 thalassemia is a genetic disorder, Hemoglobin S is inherited from one parent and Thalassemia is inherited from the other parent.
What are the symptoms of sickle beta 0 thalassemia?
Sickle beta 0 thalassemia often causes anemia, tiredness or weakness. Occasionally, pain and stiffness in the arms, legs or back can occur as blood clumps up in the vessels. Others problems can include an increased risk of infection, an enlarged spleen, eye problems and more.
What are sickle beta 0 thalassemia care options?
Treatments typically involve preventing potential complications and treating them as they arise. People with the disorder receive continuous care to prevent and manage the complications of the disease. Hydroxyurea is the main treatment for reducing complications. Education is essential to provide supportive and preventive care.
The only cure for Sickle Cell disease is bone marrow transplantation; gene therapy will be a curative therapy and will be coming available soon.
Reviewed by: Athena Pefkarou, MD
This page was last updated on: July 05, 2022 02:45 PM
Dr. Athena Pefkarou discusses the four types of sickle cell disease in this edition of Children's Health Chats.