Basics About Thalassemia | Thalassemia | Blood Disorders | NCBDDD | CDC
Your doctor may also talk about thalassemia minor and thalassemia major, or Cooley's anemia. The minor type is less serious than the major one, and your type. In response, the Sickle Cell Disease Association of America was founded and later helped establish the Sickle Cell Anemia Control Act of , which allotted. Thalassemias can cause mild or severe anemia and other complications that Thalassemias occur more often among certain ethnicities, including people of.
Individuals born with alpha thalassemia major are treated with lifelong blood transfusions and other medical care.
Basics About Thalassemia
The parents brochure is here and the healthcare providers brochure is here. Beta Thalassemia People whose hemoglobin does not produce enough beta protein have beta thalassemia. There are three types of beta thalassemia that also range from mild to severe in their effect on the body.
Thalassemia Minor or Thalassemia Trait. In this condition, the lack of beta protein is not great enough to cause problems in the normal functioning of the hemoglobin.
Thalassemia - Symptoms and causes - Mayo Clinic
A person with this condition simply carries the genetic trait for thalassemia and will usually experience no health problems other than a possible mild anemia. As in mild alpha thalassemia, physicians often mistake the small red blood cells of the person with beta thalassemia minor as a sign of iron-deficiency anemia and incorrectly prescribe iron supplements.
In this condition the lack of beta protein in the hemoglobin is great enough to cause a moderately severe anemia and significant health problems, including bone deformities and enlargement of the spleen. However, there is a wide range in the clinical severity of this condition, and the borderline between thalassemia intermedia and the most severe form, thalassemia major, can be confusing. The deciding factor seems to be the amount of blood transfusions required by the patient.
The more dependent the patient is on blood transfusions, the more likely he or she is to be classified as thalassemia major. Generally speaking, patients with thalassemia intermedia need blood transfusions to improve their quality of life, but not in order to survive. This is the most severe form of beta thalassemia in which the complete lack of beta protein in the hemoglobin causes a life-threatening anemia that requires regular blood transfusions and extensive ongoing medical care.
People with thalassemia can get too much iron in their bodies, either from the disease or from frequent blood transfusions. Too much iron can result in damage to your heart, liver and endocrine system.
This system includes hormone-producing glands that regulate processes throughout your body. People with thalassemia have an increased risk of infection.
This is especially true if you've had your spleen removed. In cases of severe thalassemia, the following complications can occur: Thalassemia can make your bone marrow expand, which causes your bones to widen.
- American Society of Hematology
- About Thalassemia
- Alpha and Beta Thalassemia
This can result in abnormal bone structure, especially in your face and skull. Bone marrow expansion also makes bones thin and brittle, increasing the chance of broken bones. The spleen helps your body fight infection and filter unwanted material, such as old or damaged blood cells. Thalassemia is often accompanied by the destruction of a large number of red blood cells. This causes your spleen to enlarge and work harder than normal.
Splenomegaly can make anemia worse, and it can reduce the life of transfused red blood cells. If your spleen grows too big, your doctor may suggest surgery to remove it splenectomy. Anemia can cause a child's growth to slow. And thalassemia may cause a delay in puberty. Heart problems — such as congestive heart failure and abnormal heart rhythms arrhythmias — may be associated with severe thalassemia.
Sickle Cell Disease Click image to enlarge Sickle cell disease was first discovered in the early s, described as "peculiar, elongated sickle-shaped erythrocytes [red blood cells].
In a landmark study, Dr. Linus Pauling concluded that sickle cell disease is caused by abnormal hemoglobin, referred to as "hemoglobin S. Even though the underlying molecular cause of the disease was understood more than half a century ago, progress in translating this knowledge into improved patient care has been slow.
This partly reflects the intrinsic difficulty of treating the disease. However, it also results from the fact that, in the United States, sickle cell disease occurred in an underserved population for which health research and treatment were neglected. It was not until the civil rights movement of the early s that the poor treatment of these patients was recognized as a prime example of racial inequality in health-care.
In response, the Sickle Cell Disease Association of America was founded and later helped establish the Sickle Cell Anemia Control Act ofwhich allotted government health funds for screening, research, and treatment programs. As scientific progress and technology improved, new treatment regimens evolved for sickle cell disease patients. The Prophylactic Penicillin Study PROPS found that giving penicillin, an antibiotic, when patients were not sick could prevent death related to serious infections in sickle cell disease.
Later, the Multicenter Bone Marrow Transplant Study demonstrated that 84 percent of children with sickle cell disease who received a bone marrow transplant from a matched relative could be cured. Finally, in the mids, the U.
Food and Drug Administration approved a new therapy called hydroxyurea as a treatment to decrease complications of the disease.