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LWB's Thalassemia video

FACTS:

• Thalassemias  (thal-a-SE-me-ahs) are genetic disorders that involve the decreased and defective production of hemoglobin.  The disorder results in a patient's body producing blood cells that are defective and die prematurely. 
• It is an inherited disease that is not contagious.  The only way it can be passed on is from parents to their children through genes. 
• People who have thalassemias can have mild to severe anemia. 
• In the past 20 years, new therapies have greatly improved the quality of life and life expectancy in kids who have these diseases.  Treatments for thalassemias have improved greatly in the past few years. People who have moderate and severe thalassemias are now living longer and have better quality of life than before.
• However, there are still complications from thalassemias and severe forms require frequent treatment.  People who have moderate or severe thalassemias must closely follow their treatment plans. They need to take care of themselves to remain as healthy as possible.
• Hemoglobin is made of two proteins: Alpha globin and beta globin. Thalassemia occurs when there is a defect in a gene that helps control production of these proteins.
  1. Alpha thalassemia occurs when a gene or genes related to the alpha globin protein are missing or changed (mutated).
  2. Beta thalassemia occurs when similar gene defects affect production of the beta globin protein.
• The two types of thalassemia are: alpha-thalassemia and beta-thalassemia. Their names describe which part of the hemoglobin molecule that is effected, the alpha or the beta chain. Hemoglobin contains two different kinds of protein chains named alpha and beta chains. Any deficiency in these chains causes abnormalities in the formation, size, and shape of red blood cells. 
• Each type of Thalassemia has several severities.  Some known as trait, intermedia and major.  Persons with the minor forms of alpha and beta thalassemia have small red blood cells, but no symptoms.
• Kids who have with different forms of thalassemia have different kinds of health problems. Some only have mild anemia with little or no effects, while others require frequent medical treatment.
  

TREATMENT:

• Children with Thalassemia should not be given vitamins with iron.  Some doctors suggest limiting the intake of iron rich foods as well.
  
  
• Persons who receive significant numbers of blood transfusions need a treatment called chelation therapy to remove iron from the body.  This can be done with an oral medication.  If the patient does not tolerate the oral form of a chelation, a sub-clavious (under the skin) method.
  
  
• A bone marrow transplant may help treat the disease in some patients, especially children.

PROGNOSIS:

• If left untreated, severe thalassemia can cause early death due to heart failure, usually between ages 20 and 30.  Diligence is needed to follow all treatment plansFrequent blood transfusions with therapy to remove excess iron, improved chelation techniques and continued research has greatly improved the prognosis and daily living of Thalassemia patients.  Less severe forms of thalassemia usually do not result in a shorter life span.
  
• Untreated, thalassemia major leads to heart failure and liver problems, and makes a person more likely to develop infections.
  

RESOURCES:

LWB's Thalassemia video