Sickle cell disease (SCD) is an inherited blood disorder characterized by the presence of abnormally shaped red blood cells. These sickle–shaped cells are rigid and can block blood flow by getting stuck in blood vessels, leading to severe pain, organ damage, and increased mortality—especially among children.
The disease is inherited when a child receives a copy of the sickle cell gene from each parent. First documented in Western medicine over a century ago, SCD is more common among individuals of African, Hispanic, Middle Eastern, Asian, Indian, and Mediterranean descent.
SCD predominantly affects low and middle–income countries, with India bearing the third–highest global burden in the number of SCD births, following Nigeria and Democratic Republic of the Congo. The disease predominantly affects the tribal populations in central India, with 80 percent of this demographic residing in ten states. In central India, approximately 1 in 86 births are affected by SCD, with around 20 percent of these children dying before their second birthday.
A national mission to eliminate sickle cell disease
In 2023, the government introduced the National Sickle Cell Anemia Elimination Mission (NSCAEM), expanding its previous efforts to address the disease burden, with the ambitious goal of eliminating SCD as a public health issue by the year 2047.
The mission aims to provide affordable, accessible, and quality care to all SCD patients while reducing disease prevalence. It combines screening and awareness strategies to ensure early detection and treatment while promoting awareness and education so that people do not unintentionally pass it on to their children.
PATH, in collaboration with the public health department of Maharashtra, is providing technical assistance to accelerate the roll–out of NSCAEM. The primary goal of this work is to enhance the availability, accessibility, and quality of SCD–related services. Our work also aims to demonstrate a scalable district approach, offering intensive support to Nandurbar and Gadchiroli districts.
PATH’s model to provide technical assistance to the state government in Maharashtra to improve SCD care.
Integrating sickle cell disease management in primary health care
PATH is advocating for decentralizing SCD management by integrating it into the primary care framework. This involves developing customized training materials and building the capacity of health care staff.
Capacity–building activities are organized at both district and state levels to ensure comprehensive training for various health workforce cadres on screening, diagnosis, primary management, appropriate referral to higher facilities, counseling, and linking with social supports.
The focus is on ensuring appropriate care provision through centers closer to the people, and establishing a continuum of care for complex conditions.
‘’Sickle cell disease poses a critical health challenge in Gadchiroli, a primarily tribal district where awareness about the disease and health-seeking behaviors are hindered by deep-rooted cultural traditions. Recently, the Public Health Department, with support from experts and PATH, has provided essential training to front-line workers. This training aims to enhance their ability to deliver Hydroxyurea-based care to SCD patients and to successfully implement the SCD program in the district. By ensuring accurate medication dosages, regular monitoring, ongoing support, and counseling, these efforts seek to significantly improve the quality of life for individuals affected by SCD", says Dr. Prafull Hulake, District Reproductive & Child Health Officer, Gadchiroli and Nodal officer For Sickle Cell Program.
“By ensuring accurate medication dosages, regular monitoring, ongoing support, and counseling, these efforts seek to significantly improve the quality of life for individuals affected by SCD.”— Dr. Prafull Hulake, District Reproductive & Child Health Officer
Bringing in systemic improvements
Screening is a critical first step in identifying individuals with SCD. The NSCAEM recommends universal screening for individuals up to 40 years old in high– prevalence districts.
PATH is focusing on strengthening screening activities—particularly for newborns, pregnant women and unscreened populations—to ensure early detection and intervention including rolling out point–of–care diagnostics and increased treatment adherence.
A screening camp organized at Shirve Govt Aashram School in Taloda block, Nadurbar district, Maharashtra, on World Sickle Day 2024. Photo: PATH
A streamlined supply chain is crucial for effective SCD management. While some medications overlap with treatments for other illnesses, specific products are necessary for SCD diagnostics and management.
PATH is strengthening supply chains of these products such as point–of–care test kits, hydroxyurea, etc. We are accelerating this through improved forecasting, monitoring use, and the implementation of the supply chain information system to ensure that hydroxyurea is available at all facilities, including primary health level facilities termed as Ayushman Arogya Mandir.
“Mapping and convening potential manufacturers of screening and diagnostic kits can be a gamechanger. Strengthening supply chain linkages can go a long way in bolstering the larger continuum–of–care model we are trying to accomplish.”— Satish Tajne, State Lead Maharashtra
Leveraging community platforms for awareness
Community engagement efforts are not only crucial for creating more awareness about SCD, but to also counter stigma and discrimination against it. To deeply engage with communities, we are collaborating with community health platforms like Jan Arogya Samitis, local non–governmental organizations and village council members, and including them in the program activities to reduce stigma.
Facilitating the formation of patient support groups is another key strategy, which complements the ongoing efforts to improve compliance with care protocols, thereby enhancing the quality of life for SCD patients.
Driving progress towards a SCD–free India
The government’s program implemented in 17 high–focus states across the country, is advancing large–scale screening, prevention, and awareness, and improving the care and prospects of all SCD patients.
Inter–sectoral convergence will be vital for coordinated and targeted action against SCD. PATH supports the establishment and operationalization of various forums at the state and district levels. The convergence involves departments like Health, Tribal Development, Education, and Social Justice. With this, we ensure cohesive efforts towards improving awareness, resource pooling, social benefits, and improving health outcomes for all.
