What is Sickle Cell Disease?

Context

The union budget of FY 2023-24 announced a mission to eliminate sickle cell anaemia by 2047. 

• The mission entails focus on awareness creation, universal screening of approximately seven crore people in the 0-40 years age group in affected tribal areas and counselling through collaborative efforts of central ministries and state governments.

Probable Question

The Government's target of eliminating sickle cell anaemia in the nation by 2047 is a very positive step as it will also aid in combating the endemic haematological illness like anaemia that plague the nations indigenous communities. Explain. (150 words, 10 marks).

Related Data

• With around 18 million people with sickle cell traits and 1.4 million patients with sickle cell disease, India is expected to have the second-highest numbers in the world. 
• A few states in India have a significantly higher SCD prevalence: Chhattisgarh, West Bengal, Uttar Pradesh, Maharashtra, Madhya Pradesh, Jharkhand, Gujarat, Odisha, Kerala and Rajasthan. Collectively, these are referred to as the sickle cell belt. 
• Although more common among tribal communities, the disease is gradually finding its way across the country due to migration and intermarriages.

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What is Sickle Cell Disease? 

• Sickle cell disease (SCD) is an inherited group of blood disorders that is genetic in nature. 
• It is usually transferred from the parents to the child during birth i.e. both parents can be carriers of SCDs. 
• The disease affects haemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body.
• People with this disease have atypical haemoglobin molecules called haemoglobin S, which can distort red blood cells into a sickle, or crescent shape which slows down or blocks the blood flow in smaller blood vessels.

Symptoms of Sickle Cell Disease

• SCD is a chronic single gene disorder causing a debilitating systemic syndrome characterised by chronic anaemia, acute painful episodes, organ infarction and chronic organ damage and by a significant reduction in life expectancy. 
• Episodes of pain known as sickle cell crises are one of the most common and distressing symptoms of sickle cell disease.
• Babies with sickle cell anaemia may not exhibit symptoms for several months after birth.
• People can experience swelling of the hands and feet since sickle cells damage the spleen, increasing vulnerability to infections. Due to a shortage of RBCs, babies and children could experience slow or delayed growth and puberty.
• People with sickle cell anaemia may have various and increasingly significant medical problems as they age, which occur when organ tissues do not receive enough oxygen.

Procedure for diagnosing Sickle Cell Disease (SCD)

• A blood test can determine whether you have SCD or sickle cell trait.
  • All states now check babies as part of their screening programmes, allowing treatment to begin as soon as possible.
• SCD can also be diagnosed before a baby is born. A sample of amniotic fluid (the fluids in the sac around the foetus) or placental tissue is used in this test (the organ that brings oxygen and nutrients to the baby).

Treatment therapies available for Sickle Cell Disease (SCD)

• SCD can only be cured by bone marrow or stem cell transplantation.
• These transplants are normally reserved for children with severe SCD since they are hazardous and can have substantial adverse effects.
  • The bone marrow must be a close match for the transplant to succeed. 
  • A brother or sister is usually the ideal donor.

Government’s Initiatives to tackle Sickle Cell Anaemia

• Under the National Health Mission, the Government of India supports the states for prevention and management of sickle cell disease as per their annual PIP proposals. 
• The Ministry of Health and Family Welfare has released technical operational guidelines for prevention and control of hemoglobinopathies in 2016 including sickle cell anaemia.  
• Support is given under the National Health Mission for capsule hydroxyurea, free blood transfusion for all Sickle cell patients (men & women) as per the State’s proposal.
• The Ministry of Tribal Affairs (MoTA) has launched the Sickle Cell Disease Support Corner to bridge the gap between patients and health care services in tribal areas.
  • The Portal provides a web-based patient powered registration system which collates all information related to SCD among tribal people in India.
• The Unmukt Project is being implemented by the Central Government to strengthen screening and timely management of Sickle Cell Anaemia.
• The Tribal Affairs Ministry established the National Council on Sickle Cell Disease and Tribal Health Cell, which would coordinate with the Ministry of Health and State Governments.
• State Haemoglobinopathy Mission has been established in Madhya Pradesh to tackle the challenges in screening and management of sickle cell disease. 

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Way Forward

• SCD requires a comprehensive model of care that includes timely and accurate treatment along with intense programmes for pre-marital, prenatal, and new-born screening besides administering Pneumococcal vaccination to adult patients.
• Extended family screening is recommended for people who are carriers, who have a family history of sickle cell diseases.
• There is a need to screen all populations for sickle cell trait especially high risk communities. 

Frequently Asked Questions (FAQs)

News Source: Indian Express and PIB

https://indianexpress.com/article/lifestyle/health/union-budget-2023-24-sickle-cell-anaemia-elimination-2047-possible-experts-talk-8417533/

https://pib.gov.in/PressReleasePage.aspx?PRID=1896039

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