On World Sickle Cell Day, Experts Highlight the Need for Better Management of Sickle Cell Disease as a Public Health Priority
· Sickle Cell Disease, an inherited blood disorder, poses a major health burden in India. Yet, it remains neglected, leading to low screening and ineffective management
· Approximately 10% of Gujarat’s tribal population, which ranges between 90 lakh and 1 crore people, are sickle cell trait carriers
· There is an urgent need for increased screening, especially amongst newborns, in pre-marital and genetic counselling, as well as holistic treatment policies and management protocols
Ahmedabad, June 19, 2021: World Sickle Cell Day, observed annually on June 19th, aims to raise awareness on Sickle Cell Disease (SCD) at a national and global level. SCD, 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.
Dr. Anil Khatri, Member of Managing Committee, Indian Red Cross Society and Member of Thalassemia Jagruti Organisation, Ahmedabad said, “Sickle Cell Disease prevalence is significant in Gujarat, skewed towards the tribal populations. Roughly 10% of Gujarat’s tribal population, which ranges between 90 lakh and 1 crore people, are estimated to be sickle cell trait carriers. Raising awareness and enabling patient education are necessary to facilitate timely diagnosis & treatment and also better management of severe pain episodes or crises. With efforts by the government and NGOs, the social stigma surrounding the disease can also be overcome.”
Dr. Anil Khatri continued, “Through a combination of lifestyle modifications, such as hydration and avoiding strenuous activities for prolonged durations, and treatment including pain relieving medications and oxygen (if needed), the condition can be better managed. At the same time, the Government should also extend support to make advanced therapies in the field more accessible. Failure to treat conditions in a timely manner can lead to severe, chronic complications, including Acute Chest Syndrome which can be life threatening. Research and innovation in treatment have been slow, however this deficit can be bridged by having multi-state Centres of Excellence for the treatment and mass education of patient populations and collection of data.”
He also outlined the vital role of the state and central government in disease management, saying, “With the launch of guidelines for managing and preventing hemoglobinopathies (2018) by the government and an anticipated national policy to follow, we are starting to move forward in disease management. It is also incumbent on the state to drive local multi-stakeholder interventions including policy makers, medical experts, the pharmaceutical industry and NGOs towards this common goal. The Gujarat government has been sensitive to this cause, undertaking various initiatives to enable the betterment of patients and improve their quality of life through well-defined treatment protocols, and ensure prevention where possible.”
“Capacity building of primary care physicians in tribal areas is crucial for implementing
comprehensive Sickle Cell Disease (SCD) management in tribal areas. By raising awareness among the general population, we ensure that SCD Warriors have a better quality of life,” said Dr. Dexter Patel, CEO, Sickle Cell Awareness Foundation, Gujarat.
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.