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: December 18, 2020 05:07 PM
Dr. Athena Pefkarou discusses the four types of sickle cell disease in this edition of Children's Health Chats.
Patient Success Stories
While her high school senior peers were trying on prom dresses and applying to college, Sydnie Romero was grappling with a decision that would be tough for anyone, let alone a chronically ill 17-year-old. Sydnie was born with sickle cell disease, a genetic disorder in which bone marrow produces defective hemoglobin that can block blood supply to parts of the body, resulting in “pain crises,” which are episodes of severe pain that can last for hours to days.
Learn more about
Thalassemia is a blood disorder in which the body produces hemoglobin that doesn’t function properly.
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.
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.
Exchange Transfusion Therapy
Exchange transfusion therapy is a form of blood transfusion in which the body’s blood is removed and replaced.