Social stigma and ignorance around the Sickle Cell Disease (SCD) continue to remain a public health challenge in India which has the second-highest burden of genetic blood disorder after Nigeria. Moreover, even as various states have undertaken screening programmes, still there is no central database for sickle cell carriers and diseased. Tribal populations are the worst hit with this debilitating and painful disease with children being at the receiving end: Before reaching the age of two, 20% of tribal children with sickle cell disease die, and 30% of children die before reaching adulthood.
As per doctors, there may be 18 million Sickle Cell Traits (SCT) and 1.4 million SCD patients among the tribal population. “There is a need to have a comprehensive care model for SCD,” said Dr. Rahul Bhargava, Principal Director, Hematologist Department, Fortis Hospital in Gurugram, Haryana.
In this regard, the Union Tribal Ministry has been taking steps to take action on the prevention and elimination of sickle cell disease in a mission mode. Anil Kumar Jha, Secretary of Tribal Affairs stressed on the need for data-based management and elimination plans for the disease.
He also informed that the Ministry would support the initiative of IGIB on research on CRISPER -technology involving gene-editing after consultation with the Department of Science and Technology, ICMR, and Union Health Ministry.
According to officials, the Ministry of Tribal Affairs is also working on a road map for the management, control and eradication of Sickle Cell disease. The action plan will be implemented in coordination with the Ministry of Health and Family Welfare, State Governments, Organizations working on SCD and Non-Governmental Organizations (NGO), he said.
Characterized by repeated episodes of debilitating pain, SCD can lead to serious health complications including pneumonia, bloodstream infections, stroke, and acute & chronic pain.
The disease is said to have a higher prevalence in Chhattisgarh, Bihar, Uttar Pradesh, Maharashtra, Madhya Pradesh, Jharkhand, and Rajasthan which is referred to as the sickle cell belt.
This condition is most common in tribal communities in India but is gradually finding its way throughout because of migration into the cities, said Dr Tulika Seth, Professor of Hematology at All India Institute of Medical Sciences (AIIMS), Delhi.
She also felt that due to social stigma, people are reluctant to undergo pre-marital counseling so that it is understood and timely diagnosis is undertaken for appropriate management conditions. Hydroxyurea is an effective drug that reduces important component care. Other therapies include prescription antibiotics to combat infections and vitamin supplements to help generate red blood cells. "Initiatives such as intensive prenatal and newborn screening must be mandatory along with providing Pneumococcal vaccination to adult patients under doctor's guidance.
Managing complications like avascular necrosis (AVN), acute chest syndrome, stroke becomes a critical phase, proactive steps must be taken to awareness screening, and treatment options", Gautam Dongre, Secretary, National Alliance Sickle Cell Organizations, NASCO told a news agency.
He added, "We need to re-think the model of care for SCD. In my experience of dealing with patients, proper and timely treatment has led to normal life expectancy and almost 70-80% enhanced life quality for patients. Timely, comprehensive, and appropriate treatment is of utmost importance.”
According to health experts, bone marrow transplantation (BMT) is an excellent start to finding its cure. The newer gene therapy studies in pre-clinical and clinical trials have shown favorable outcomes in patients with sickle cell. Although bone marrow transplantation is not yet perfect because of the complications involved, it still serves as a hope for patients with severe sickle cell disease as the benefit outweighs the risks, they said.