Experts Highlight the Need for Better Management of Sickle Cell Disease as a Public Health Priority

Sickle Cell Disease (SCD) is, an inherited blood disorder, poses a major health burden in India. However, it remains neglected, with the ongoing pandemic and increasing burden on healthcare infrastructure in the country. This contributes to low levels of awareness, reduced screening coverage and a paucity of data which hinders research and treatment innovations. In order to advance SCD as a public health priority and enable timely diagnosis and effective disease management, there is an urgent need to scale screening of newborns and pregnant women as a first step of a holistic strategy to better support SCD patients and limit their complications. 

India is estimated to have the second highest national burden of sickle cell disease in the world. SCD prevalence varies significantly across the country, with particular prominence in tribal groups. The condition is reported to be most prevalent in central India, across the “sickle belt”, stretching from Gujarat to Odisha, with the states of Chhattisgarh, Bihar and Uttar Pradesh estimated to have the highest prevalence. Recently, its prevalence has been observed across non-tribal populations as well. This raises the urgent need to recognise sickle cell disease as a public health priority in the country. 

Discussing SCD prevalence in Maharashtra, Dr. Dipty Jain, Head of Department – Paediatrics, Government Medical College, Nagpur said, “Across India, the prevalence of sickle cell disease varies across different communities and can range from 7% to as high as 30% in communities with high prevalence. Moreover, the severity of cases also differs across regions, with Nagpur and Chhattisgarh reporting highly severe cases. There is a lack of well documented data on the disease prevalence, and a planned epidemiological survey could offer a better understanding of the local disease prevalence and burden.” 

Mr. Saumil Mody, General Manager, Novartis Oncology India and Member of the National SCD Council said, “At Novartis, we are committed to driving awareness and partnering with patient communities, to ensure SCD warriors are not alone in their fight. Through innovative and digitally-enabled solutions, we hope to meet our dual objectives of empowering our patients through improved SCD screening and access to advanced therapies across the country. Through various targeted initiatives like health system strengthening at the primary level, Novartis is committed to building a holistic and effective ecosystem for management of Sickle Cell Disease (SCD) in partnership with relevant stakeholders.”

Dr. Dipty Jain also highlighted gaps in SCD management, outlining the key needs of the hour. “Universal screening is ideal, but with limited funds at our disposal, we are targeting priority groups to maximize screening benefits. Three core stages of intervention aiding timely diagnosis and treatment include Primary Prevention (pre-marital counselling and screening of the reproductive age group), Secondary Prevention (screening of women in the first two months of pregnancy for prenatal diagnosis) and Neonatal Screening (ensuring early diagnosis so children can be swiftly placed in comprehensive health programs to enhance quality of life). Increased government funding can support this. In Maharashtra, the government offers funding for sickle cell warriors, spanning free medication, investigations and travel concessions. Further support by the government in terms of including advanced therapies under this funding can go a long way in making such treatment solutions more accessible.”

She continued, “Another key aspect to address is the need for India-specific, standardized protocols. Today, we are extrapolating protocols developed for sickle cell populations in Africa and other countries, across treatment offerings and antibiotic policies. There is an urgent need to undertake robust studies on Indian sickle cells and develop our own policy, culture organism and sensitivity pattern so we can better cater to the population with evidence-based solutions.”  

Mr. Gautam Dongre, Member Secretary of National Alliance of Sickle Cell Organisations (NASCO), is the father of 2 sickle cell patients. According to him, “Inclusion of patient’s and caregiver’s voice is essential to frame and implement any policy for the Sickle Cell Disease (SCD) community across India. Our mission is to ensure every Sickle Cell Disease warrior across India lives a pain-free life and gets timely access to comprehensive quality care & treatment at all healthcare facilities.”

SCD refers to a group of inherited red blood cell disorders. It is characterised by repeated episodes of debilitating pain, also known as vaso-occlusive crises (VOCs), and fever. VOC episodes among sickle cell patients are also associated with increased hospitalizations and morbidity. Organ damage, chronic kidney disease and functional disability are a few of its longer-term complications, with SCD patients also more susceptible to infections, stroke, acute chest syndrome, fatigue and leg ulcers. Delay in diagnosis and treatment of the disease can amplify these problems. 

Owing to SCD being historically neglected, progress in terms of diagnosis, treatment and the adoption of innovation has been slow. To be recognised as a public health priority, SCD requires a national programme, a comprehensive care plan and establishment of standard India-specific treatment protocols, supported by capacity building at the grassroots level, so that states can deliver localised plans catering to their demographic’s specific needs. 

By shaping policies, scaling the availability of better treatment facilities and advanced therapies, patients’ abilities to manage the disease and their quality of life can be significantly improved.