NEW DELHI: With an estimated 1.8 million cases, India came second behind Nigeria in the list of countries with the most sickle cell disease (SCD). The worst affected is our impoverished tribal population which reported 1.4 million cases.
An inherited blood disorder, SCD is characterised by repeated episodes of debilitating pain. It can lead to serious health complications including pneumonia, bloodstream infections, stroke, and acute and chronic pain.
A survey by ‘The Economist’, has revealed that 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.
SCD is more prevalent in Chhattisgarh, Bihar, Uttar Pradesh, Maharashtra, Madhya Pradesh, Jharkhand, and Rajasthan which is referred to as the sickle cell belt.
With the social stigma attached to it, tribals often try their best to hide it fearing isolation from their community. Stigma only worsens the case as people often avoid treatment. And by the time they seek help, it is already too late.
Though the tribal communities suffer more, the recent findings have shown that cases of sickle cell diseases are now appearing in city folks too.
Dr Amita Mahajan, senior 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,” Dr Mahajan said.
Minimal or no understanding of the disease and lack of access to healthcare facilities for people in rural regions also hampers diagnosis and timely treatment.
Gautam Dongre, secretary, National Alliance of Sickle Cell Organizations (NASCO), said, “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,” Dongre said.
“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,” he adds.
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 haemophilia and thalassemia.
A national policy on hemoglobinopathies will be instrumental and once it is implemented at the national level, effective adoption will have to take place at the state level.
“In India, we have been able to build meaningful partnerships with the Ministry of Tribal Affairs (MoTA), National Health Mission (NHM), relevant State Governments, and National Alliance of Sickle Cell Organisations (NASCO) in past few years for providing a comprehensive SCD management ecosystem to our patients,” said Vaishali Iyer, Country Head-Communications, Engagement & CSR, Novartis in India.
“Awareness and stigma around the condition are two critical gaps that we are trying to address,” Iyer added.