The definition of sickle beta thalassemia is a disease that causes a mild form of sickle cell anemia. This induces the body’s hemoglobin, or red blood cells, to take on a sickle shape and not flow through the blood vessels as placidly. This can create a number of major complications. The “plus” indicates that the blood has a lower than normal amount of normal hemoglobin. This varies from sickle beta 0 thalassemia, in which a person has no normal hemoglobin.
People who have this form of SCD inherit one sickle cell gene (“S”) from one parent and one gene for beta thalassemia, another type of anemia, from the other parent. There are mainly two types of beta thalassemia: “0” and “+”. Those with HbS beta 0-thalassemia usually have a severe form of SCD. People with HbS beta +-thalassemia tend to have a milder form of SCD.
What is Sickle Cell Trait
People who have SCT derive one sickle cell gene (“S”) from one parent and one normal gene (“A”) from the other parent. This is called sickle cell trait (SCT). People with SCT usually do not have any of the signs of the disease and live a normal life, but they can pass the trait on to their children. Additionally, there are a few, uncommon health problems that may potentially be related to sickle cell trait.
What creates sickle beta + thalassemia?
We can say sickle beta thalassemia is a genetic disorder that is passed along from parents to their children. It’s a receding genetic disorder, which means that both parents must pass along different genes in order for the child to develop the ataxia.
What are the symptoms of sickle beta + thalassemia?
The major symptom of sickle beta thalassemia often causes mild anemia, tiredness or sometimes weakness. Periodically, pain and stiffness in the arms, legs or back can occur as blood chunks up in the vessels. Sometimes other problems can include an increased risk of infection, an expanded anger, eye problems and many more.
What are the key points of sickle beta + thalassemia care options?
For sickle beta thalassemia major treatments are typically involved preventing potential complications and treating them as they occur. Many people with the disorder receive continuous care to prevent and manage the complications of the disease. Mainly education is essential to provide supportive and precautionary care. If major complications occur sometime it may require Hydroxyurea. The only cure for Sickle Cell disease is bone marrow transplantation.
Bone marrow is a soft, fatty tissue inside the center of the bones where blood cells are made. A bone marrow or stem cell transplant is a procedure that takes healthy cells that form blood from one person—the donor—and puts them into someone whose bone marrow is not working properly.
Bone marrow or stem cell transplants are very risky, and can have very serious side effects, including fatal. So, for the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister of his/her family. Bone marrow or stem cell transplants are used only in cases of severe SCD for children who have minimal organ damage from the disease.
SCD is a genetic condition that is present at birth. It is inherited when a child receives two sickle cell genes—one from each parent.
Diagnosis of SDC
As you know SCD is diagnosed with a simple blood test. It most often is found at birth during routine newborn screening tests at the hospital itself. In addition, SCD can be diagnosed before birth also.
Because children with SCD are at an increased risk of infection and other health problems, early diagnosis and treatment are important for the child to get cure as early as possible.
You can call your local sickle cell organization to find out how to get tested.