Early diagnosis is critical to containing the growing number of Sickle cell disease cases in India

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Did you know the prevalence of Sickle Cell Anemia in India is the second largest worldwide? On the occasion of World Sickle Cell Awareness Day, we give you an insight into this hereditary disorder that is a growing concern for the country.


  • Rahul, a six year old, from a remote area in Chattisgarh, often misses school.+
  • Vikram a young, 22 year old boy from Gujarat recently grabbed a lucrative offer at one of the MNCs. However, he had to soon quit. +
Rahul and Vikram are both related, not by blood, but by a blood disorder - Sickle Cell Disease. A group of inherited disorders affecting the hemoglobin, sickle cell disease, can cause severe and life-threatening consequences, such as anemia, infections, stroke, tissue damage, organ failure, intense painful episodes and premature death.
A chronic disorder of global health importance, ironically, sickle cell disease, is often neglected. India is estimated to have the second highest burden of the disease in the world, a fact not to be proud of! In India, it is mainly the scheduled population belonging to the most socio-economically disadvantaged communities, particularly in the belt stretching across Gujarat, Maharashtra, Chattisgarh and Odisha that are most affected by the disease.

Crescented moon in the blood

Sickle Cell Anemia is the most common form of Sickle Cell Disease (SCD) and affects millions worldwide. It is caused by a mutation in the gene that helps make hemoglobin and causes the RBC’s to form a crescented moon shape, like a sickle. The sickle shaped cells contain abnormal hemoglobin (HbS). Because of their shape, sickled RBCs cannot pass through small blood vessels. Instead they stack up and cause blockages (vaso-occlusion) that deprive organs and tissues of oxygen carrying blood. This ultimately results in damaging the tissues and vital organs, leading to serious medical problems. Normal RBCs live about 120 days in the bloodstream, but sickled red cells die after about 10 to 20 days. Because they cannot be replaced fast enough, the blood is chronically short of RBCs, leading to anemia.

Taking over India and the world


  • According to a 2019 report, SCD occurs in approximately 3,00,000 births annually, globally.* 
  • By 2050, the number of people with SCD is expected to grow to 30%.** 
  • In India, the trait is prevalent in 40% of the population.** 
  • India has the highest number of sickle cell gene carriers (>50%) in the world.
  • According to the Indian Council of Medical Research (ICMR), 20% of the children with SCD don’t survive beyond 2 years of age in India.***

Why sickle cell disease is prevalent in malaria endemic areas like India?


Here is an interesting fact, carriers of SCD, don’t exhibit any symptoms and have very less difference in their blood counts when compared to a normal individual, however, they exhibit a 30% reduction in susceptibility to malaria.


RBCs can become home to malarial parasites that grow, replicate and then burst from the cells during an infection. These damaged cells are removed from circulation, and reduction causes anemia.

Over generations, majority of the population in the malaria endemic areas are carriers of the HbS gene. Their RBCs become susceptible to changing to a rigid sickle cell shape, once infected with the malarial parasite. Due to the sickle shape, there is a reduction in the oxygen, making it difficult for the malarial parasite to survive and multiply. With the death of the RBC, the parasite also dies.

Early diagnosis as a preventive step


There are many tests available that can aid in detection of sickle cell anemia at an early stage and prevent complications. Making these tests available in the remotest village, which forms the hub of the disease, is the biggest challenge. Further, there is also a need to combine the diagnostic tests with awareness programs. Simple blood tests, such as carrier screening, can be done either before marriage or before planning a baby. It helps to diagnose if you or your partner have sickle cell trait and the chances that you may pass it on to your baby. Similarly, new born screening tests, can aid in early management of disease. This will reduce the morbidity and mortality associated with it. If undetected, it can lead to life threatening complications and early death.

Government’s role in tackling the disease


Thankfully, the Government recognizes the need to address the issue of Sickle Cell Disease, and the Ministry of Health and Family Welfare has taken keen interest in working with various State Health Departments and Ministry of Tribal Affairs for conducting awareness programs, screening and counselling sessions for management of SCD. The state governments of Gujarat, Maharashtra and Chattisgarh are working with the National Rural Health Mission (NRHM) on the same. Infact, Gujarat was the first state to initiate the Sickle Cell Anemia Control program in 2006 with the aim of reducing sickle cell births, early diagnosis, awareness generation and offering counseling. The need of the hour is for the other states to follow suit, and for the government to formulate a comprehensive policy or framework to tackle the disease.

India’s Leading IVD Company offers solutions


Transasia’s latest hematology range of three and five part differential analyzers aid in the detection of the disease. A comprehensive suit of tests which includes CBC and differential WBC analysis, help in accurately detecting parameters that can indicate the presence of the disease. These hematology analyzers have been developed at Transasia’s global subsidiary, Erba Europe. Backed by the largest sales and application support, these analyzers are reaching out to the remotest areas in India, and aiding in affordable and early diagnosis of a host of blood related disorder, Sickle Cell Anemia being one of them.

Sources:


  1. **https://www.ncbi.nlm.nih.gov/books/NBK482384/
  2. **http://www.scdcoalition.org/pdfs/ASH%20State%20of%20Sickle%20Cell%20Disease%202016%20Report.pdf
  3. ***https://www.biospectrumindia.com/features/17/10498/world-rare-disease-day-prevention-of-sickle-cell-disease-early-screening-and-awareness-a-must.html
  4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3499995/
  5. https://medicalxpress.com/news/2019-06-sickle-cell-disease-attention.html
  6. https://www.biospectrumindia.com/features/17/10498/world-rare-disease-day-prevention-of-sickle-cell-disease-early-screening-and-awareness-a-must.html
  7. http://www.bloodjournal.org/content/116/21/1633?sso-checked=true
  8. https://www.sciencedaily.com/releases/2011/04/110428123931.htm
  9. https://qz.com/africa/1450731/to-beat-malaria-red-blood-cells-keep-evolving-like-sickle-cell/
+ All names are fictitious


Authored by:

Dr. Avi Nahar,
Business Unit Head - Hematology,
Transasia Bio-Medicals Ltd.

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