Depending on the severity, Thalassemia begins to show at different times. Most patients carrying the gene α or β thalassemia show no signs. Signs of hemolytic anemia (e.g. pale skin, hepatosplenomegaly) at birth (in α thalassemia) or a few months after birth (in β thalassemia) indicate that this is a severe form of the disease, especially if there is a lack of small red blood cells.
COMPLICATIONS OF ANAEMIA
This is one of the major causes of death in patients with thalassemia
When red blood cells containing abnormal hemoglobin of the patient are destroyed, or red blood cells of transfused blood get older, large amounts of iron are released and gradually stagnant in organs such as the liver or the heart, which causes fibrosis and organ failure.
Patients are bleeding easily and sensitive to infection.
Secondary endocrine disorders in patients with multiple blood transfusions, expressed by
- Decreased height
This risk is particularly high in patients whose spleens are removed. This is due to the importance role of the spleen in immunegenesis.
Deformation of bone
In patients with severe thalassemia, red blood cells are destroyed, leading to erythrocyte proliferation in bone marrow to compensate for the deficiency. Hyperplasticity of the bone marrow causes the bone to become deformed and turned into brittle and fragile. Flat bones like the face skull are very distorted. Children with severe thalassemia have forehead, tipped nose, jawbone on the protruding face.
Enlarged Spleen (splenomegaly)
There are important physiological functions of the spleen which are listed below:
- Eliminates abnormal cells such as old red blood cells
- Production of antibodies, involved in immune response
- Storage of Red blood cells, white blood cells, and platelets. They are ready to be released into the circulatory system when needed.
In thalassemia, the spleen has to work excessively to destroy abnormal red blood cells that are enlarged and dysfunctived. The spleen causes anemia to become severe due to the reduction of the life expectancy of red blood cells received through blood transfusion. Thus, this makes the risk of infection increase. In case of splenomegaly, patients need splenectomy (surgical remove of the spleen).
Cardiac complications are one of the major causes of death in patients in severe conditions. It is caused by excessive iron deposition in the heart muscle which lead to congestive heart failure and arrhythmias.
It depends on the type of thalassemia and the severity of the disease.
Signs and symptoms are usually mildly less likely to be treated. Sometimes special blood transfusions may be required after surgery, after childbirth or when there is an infection.
Moderate or Severe Thalassemia
In dangerous situation, blood transfusion is needed at least 8 times a year. After about 10 to 20 blood transfusions, the body will be overloaded with iron that damages the heart, liver and other organs. To avoid iron overload, iron chelation therapy, intravenous, subcutaneous or oral medications should be used.
Some severe cases may require stem cell transplants, but this method requires appropriate stem cell transplantation, which is extremely complex and risky.
Lifestyle and home remedies
Lifestyle changes or home remedies are recommended to be daily routine of patients with Thalassemia.
- Avoid overloading iron: Try not take iron medications yourself
- Healthy Diet: A balanced diet rich in nutrients can keep your body healthy. Folic acid may be used to increase red blood cell production, but must be prescribed by a physician. Try to keep the bones filled with calciumand vitamin D absorbtion. Consult with your doctor to see when to take the medicine.
- Avoid infections: Wash your hands more often and avoid contact with sick people. Especially if you have splenectomy, patients with thalassemia should be vaccinated against flu, encephalitis, pneumonia, hepatitis B. If you have fever or infection symptoms, you should see a doctor for treatment in time.
Hello Health Group does not provide medical advice, diagnosis or treatment.
Review Date: June 11, 2017 | Last Modified: June 10, 2017
Borgna-Pignatti C, Gamberini MR., Complications of thalassemia major and their treatment., https://www.ncbi.nlm.nih.gov/pubmed/21668399. Accessed on May 29, 2017
Centers for Disease Control and Prevention, Thalassemia Complications and Treatment, https://www.cdc.gov/ncbddd/thalassemia/treatment.html. Accessed on May 29, 2017