Sickle Cell Disease

• The states are receiving assistance from the Indian government in their efforts to treat and prevent Sickle Cell Disease through the National Health Mission.
• The government declared a goal to end Sickle Cell Anaemia by 2047 in the Union Budget 2023–24.

What is SCD, or Sickle Cell Disease?

• The devastating systemic illness caused by SCD, a chronic single gene defect, includes chronic anaemia, acute painful episodes, organ infarction, chronic organ damage, and a significantly shortened life expectancy.
• It is an inherited genetic disease where a point mutation in haemoglobin makes it abnormal and prone to structural change. This causes the red blood cells to take an abnormal “sickle” shape, which obstructs blood flow.
• Common symptoms are anaemia, jaundice, and liver and spleen enlargement. In severe cases, patients have debilitating orthopaedic conditions called avascular necrosis of the femur. The disease can be very severe and reduces the quality of life. Patients have very painful conditions called “crisis.” There is no complete cure. The only way we can help the patient is by providing symptomatic treatment and pain management.

Symptoms:

• Sickle Cell Disease symptoms might vary; however, some typical ones are as follows:
• The symptoms of Chronic Anemia include weakness, paleness, and exhaustion.
• Painful episodes, commonly referred to as sickle cell crisis, can cause jolting, excruciating pain in the arms, legs, chest, and back.
• Delayed puberty and growth.

Treatment:

• Blood transfusions: These can treat anaemia and lower your chance of experiencing pain crises.
• Hydroxyurea is a drug that can help lessen the number of painful episodes and stop some of the disease's long-term effects.
• Additionally, bone marrow or stem cell transplantation may be used to treat it.

India’s Disease Burden:

• The disease burden from Sickle Cell Anaemia in India is prevalent in tribal populations, especially in Maharashtra. The disease burden figures may exceed 14 lahks across India, but with intensified screening, the numbers are likely to increase. Tribes like Pawara, Bhil, Madia, Gond, and Pardhan from Maharashtra have a very high prevalence.
• Sickle Cell Anaemia is most prevalent in the central India belt covering states like Gujarat, Maharashtra, Chhattisgarh, Orissa, and parts of Bengal. There are pockets in the south, Tamil Nadu, Kerala, and parts of Telangana.

Issues :

• There is a lack of screening awareness and inadequate screening centres/facilities in tribal and rural areas.
• Also, failure to access primary healthcare is a concern.
• Sickle Cell anaemia is a genetic disease, and the only prevention lies in a timely diagnosis through cost-effective and large-scale screening camps along with marriage counseling. Prenatal diagnosis can also play an important role.

Initiatives by the Government to Combat SCD:

• In 2016, the Government published technical operational recommendations for the prevention and control of hemoglobinopathies, including Sickle Cell Anaemia.
• In 22 tribal districts, integrated clinics have also been set up for treatment and diagnosis.
• To address the difficulties in screening and treating the condition, Madhya Pradesh developed the State Haemoglobinopathy Mission.