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SICKLE CELL DISEASE (UPSC-RAS) Current Affairs

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  • Sickle Cell Disease (SCD) is a genetic disorder causing crescent-shaped, rigid red blood cells that block blood flow. It leads to complications like severe pain, swelling, anemia, infections, and organ damage (heart, liver, kidneys). The lifespan of affected individuals is significantly shortened, averaging 40 years.

Feature

Normal RBCs

Sickle Cells

Shape.     

Biconcave disc (round, flexible)

Crescent/sickle-shaped (rigid)

Lifespan

120 days

10–20 days

Function

Flow freely; carry oxygen efficiently

Block small blood vessels

Result

Normal oxygen delivery

Pain crisis, organ damage, anaemia

Symptoms & Complications -

Acute (immediate) complications:

  1.  Vaso-occlusive crisis (pain crisis) - Sudden intense pain in bones, chest, back, arms, legs  most common symptom.
  2. Acute Chest Syndrome - Life-threatening fever, chest pain, breathing difficulty.
  3. Splenic sequestration - Blood pools in spleen cause severe anaemia.
  4. Stroke - Blocked blood vessels in brain.

 Chronic (long-term) complications-

  1. Chronic anaemia: leading to fatigue, weakness, and paleness. Painful episodes (sickle cell crisis) in bones, chest, back, arms, and legs. 
  2. Progressive organ damage heart, liver, kidneys, eyes.
  3. Pulmonary hypertension.
  4. Leg ulcers, Avascular necrosis (bone death), Immune deficiency frequent infections

India Burden -

♦ More than 1 crore people have sickle cell trait (carriers) in India.

Over 1 million people in India are affected by SCD.

♦ Predominantly affects Scheduled Tribe populations highest prevalence in tribal belts.

♦ High-burden states - Odisha, Chhattisgarh, Madhya Pradesh, Maharashtra, and Gujarat together account for an estimated 95% of all confirmed cases nationally. 

♦ Other affected states- Rajasthan, Gujarat, Telangana, Karnataka, Uttarakhand, Andhra Pradesh, Tamil Nadu, Kerala, Assam.

♦ Tribal communities most affected - Gond, Bhil, Munda, Oraon, Kondh, Saura, and other indigenous communities across central and eastern India.

Treatment Options

  • Blood Transfusions- Can help relieve anaemia and reduce the risk of pain crises.
  • Hydroxyurea - A medication that can help reduce the frequency of painful episodes and prevent some of the long-term complications of the disease. It can also be treated by bone marrow or stem cell transplantation.

Treatment

Nature

Access

Hydroxyurea

Oral drug reduces pain crises frequency, increases HbF (foetal haemoglobin)

Most accessible; recommended first-line

Blood transfusion

Regular transfusions reduce anaemia

Available at secondary/tertiary care

Bone marrow transplant

Potentially curative  replaces defective stem cells

Expensive; limited availability; requires matched donor

Stem cell therapy

Emerging curative approach

Research stage in India

Gene therapy / CRISPR

Cutting-edge  corrects the defective gene

Clinical trials; not yet widely available

Voxelotor

New drug (FDA approved 2019)  prevents sickling

Very expensive; limited access in India

L-glutamine

Reduces acute complications

Approved in some countries

National Sickle Cell Anaemia Elimination Mission (NSCAEM) –

Vision -To eliminate sickle cell disease as a public health problem in India before 2047.

Vision -To eliminate sickle cell disease as a public health problem in India before 2047.

Key features of the Mission

  • Universal screening of tribal population (0-40 years).
  • Pre-marital and pre-conception counselling to prevent birth of affected children.
  • Digital health cards for screened individuals.
  • Real-time monitoring through National Sickle Cell Portal .
  • Large-scale screening has improved early identification of patients and carriers, while awareness campaigns and counselling initiatives have increased community-level awareness about genetic disorders.
  • The mission has also strengthened data collection and coordination through digital health cards and real-time monitoring systems.

Conclusion -

  • Achieving the 2047 goal requires a multi-pronged approach involving diagnosis, treatment, awareness, and research, with active participation from all stakeholders. Specifically: expand hydroxyurea access at sub-district level; strengthen pre-marital genetic counselling in tribal communities; develop affordable gene therapy pathways; leverage CRISPR research partnerships (India-US iCET framework); integrate SCD screening with PM-WANI and Ayushman Bharat Arogya Mandirs; build community health worker (ASHA/CHO) capacity in tribal areas for sustained follow-up.