India drafted a national-level policy in 2018 that provided comprehensive guidelines on the prevention and control of hemoglobinopathies that included sickle cell disease along with hemophilia and thalassemia
Sickle cell disease (SCD), an inherited blood disorder, continues to remain a public health challenge in India. Characterized by repeated episodes of debilitating pain, it can lead to serious health complications including pneumonia, bloodstream infections, stroke, and acute & chronic pain. India is estimated to have the second-highest burden of SCD after Nigeria[i]– it is being estimated that there may be 18 million SCT and 1.4 million SCD patients among the tribal population[ii]. Moreover, before reaching the age of two, 20 per cent of tribal children with sickle cell disease die and 30 per cent of children die before reaching adulthood. Given this, experts have pointed out the urgent need for a comprehensive care model for SCD.
The disease is reportedly 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.
People with SCD often suffer from social stigma. This not only adds to the growing disease burden but also presents challenges with respect to screening, diagnosis, and treatment. As Dr Amita Mahajan, Sr Consultant, Paediatric Haematology and Oncology, Indraprastha Apollo Hospitals points out, “Due to the social stigma, people are reluctant to undergo pre-marital screening, and hence, we fail to identify sickle cell carriers. It needs to be understood that timely diagnosis is imperative for appropriate management of the condition. Hydroxyurea is an effective drug that reduces the frequency of pain episodes and is an important component of care. Other therapies include the prescription of antibiotics to combat infections and vitamin supplements to help generate red blood cells.”
In addition to social stigma, minimal or no understanding of the disease, and lack of access to healthcare facilities for people in rural regions also impede diagnosis and timely treatment.
Gautam Dongre, Secretary, National Alliance of Sickle Cell Organizations, NASCO said, “People in urban settings have a poor understanding of the disease and hence, are unaware of the need for screening. Furthermore, in these areas, there is a minimal focus on screening for sickle cell disease. On the other hand, patients in rural areas do not have access to proper healthcare infrastructure that leads to delayed diagnosis due to multiple referrals to numerous HCPs and institutions. “Initiatives such as intensive prenatal and newborn screening must be made mandatory along with providing Pneumococcal vaccination to adult SCD patients under doctor’s guidance. Managing complications like avascular necrosis (AVN), acute chest syndrome, and stroke becomes critical at a later phase hence proactive steps must be taken to create awareness regarding SCD, its screening, and treatment options.”
He further elaborates, “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 per cent enhanced life quality for patients. Timely, comprehensive, and appropriate treatment is of utmost importance. For this, we need to ensure last-mile connectivity and delivery of healthcare models in the remotest parts of the country and actively establish centres of excellence to meet advanced care requirements. While the Ministry of Health and Ministry of Tribal Affairs has taken active measures at a central and state level, emphasis needs to be put on skilling of healthcare professionals and other medical personnel so that they can identify SCD and accordingly recommend treatment.”
Vaishali Iyer, Country Head – Communications, Engagement & CSR, Novartis in India said, “Novartis has a long-standing commitment towards improving and extending lives of those affected by the sickle cell disease. In India, over the last few years, we have been able to build meaningful partnerships with Ministry of Tribal Affairs (MoTA), National Health Mission (NHM), relevant State Governments, and National Alliance of Sickle Cell Organisations (NASCO) and joined our forces towards providing a comprehensive SCD management ecosystem to our patients. Awareness and stigma around the condition are two critical gaps that we are trying to address amidst people living in the regions where the incidence is high.”
India drafted a national-level policy in 2018 that provided comprehensive guidelines on the prevention and control of hemoglobinopathies that included sickle cell disease along with hemophilia and thalassemia. While India has made much progress in its response to sickle cell disease, the condition continues to impact patients. Therefore, the national policy on hemoglobinopathies becomes important and once it is implemented at the national level, effective adoption will have to take place at the state level.
References:
[i] Hockham C, Bhatt S, Colah R, Mukherjee MB, Penman BS, Gupta S, et al. The spatial epidemiology of sickle-cell
anaemia in India. Scientific reports. 2018;8(1):17685.
[ii] https://www.ijcm.org.in/article.asp?issn=0970-0218;year=2017;volume=42;issue=4;spage=218;epage=221;aulast=Saxena