Why in news?
Recent reports highlight serious safety lapses in blood transfusions for thalassemia patients. In Madhya Pradesh's Satna district, four children aged 12-15 tested HIV positive after routine transfusions at a district hospital blood bank around four months ago, prompting an official probe into contaminated blood or needles.
What is Thalassemia?
- Definition: A genetic blood disorder where the body produces fewer healthy hemoglobin proteins, resulting in fewer red blood cells (RBCs) and anemia.
- Cause: Mutations in the genes responsible for hemoglobin production.
- Impact: Reduced oxygen transport in the body, causing fatigue, weakness, and developmental issues.
Types of Thalassemia
- Alpha Thalassemia:
- Caused by defective alpha-globin genes inherited from both parents.
- Severity depends on the number of defective genes.
- Beta Thalassemia:
- Caused by defects in beta-globin genes.
- More common in India and the Mediterranean region.
Symptoms
- Mild cases: Growth issues, delayed puberty, bone abnormalities.
- Severe cases: Poor appetite, jaundice, dark urine, facial bone irregularities, enlarged spleen.
- General: Chronic fatigue, weakness due to anemia.
Treatment
- Blood transfusions: Regular transfusions to maintain hemoglobin levels.
- Iron chelation therapy: To remove excess iron from the body (e.g., deferoxamine, oral chelators).
- Bone marrow transplant: Potential cure in severe cases.
- Emerging therapies: Gene-editing methods like adenine base editing are being tested.
Government Initiatives
- National Mission to Eradicate Thalassemia: Union government pledged to eliminate thalassemia over the next 25 years.
- Screening programs: Mandatory premarital and prenatal screening in some states.
- Awareness campaigns: Conducted to reduce incidence through genetic counseling.
- Drug availability issues: Reports of shortages of deferoxamine in Delhi hospitals highlight challenges.
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