Sickle Beta + Thalassemia
Also known as: hemoglobin sickle beta thalassemia, Hb beta-thalassemia.
What is sickle beta + thalassemia?
Sickle beta + thalassemia is a disease that cause a mild form of sickle cell anemia. This 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 “plus” indicates that the blood has a lower than normal amount of normal hemoglobin. This differs from sickle beta 0 thalassemia, in which a person has no normal hemoglobin.
What causes sickle beta + thalassemia?
Sickle beta + thalassemia is a genetic disorder that is passed along from parents to their children. It’s a recessive genetic disorder, which means that both parents must pass along different genes in order for the child to develop the disorder.
What are the symptoms of sickle beta + thalassemia?
Sickle beta + thalassemia often causes mild 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 + 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. Education is essential to provide supportive and preventive care. May require Hydroxyurea if major complications occur. 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: September 30, 2020 05:33 PM
Dr. Athena Pefkarou discusses the approaches to treating sickle cell disease in this edition of Children's Health Chats.
Patient Success Stories
At a young age, Bi’Yanie was diagnosed with sickle cell, a blood disorder that causes the cells to take on a crescent or sickle shape and can lead to very painful episodes called crises. Today, Bi’Yanie is getting stronger by the day, thanks to the bone marrow transplant she received at Nicklaus Children’s Hospital.
Learn more about
Sickle Beta 0 Thalassemia
Sickle cell causes the body’s hemoglobin, or red blood cells, to take on a sickle shape and not flow through the blood vessels as smoothly. The “zero” indicates that the blood has no normal hemoglobin.
Blood and Marrow Transplantation
The transplantation of blood or bone marrow is the procedure of transferring and replacing the hematopietic stem cells.
Hydroxyurea is a medicine that can help children and adults with sickle cell disease.