Sickle cell anemia is a genetic disorder that cannot be cured but only be treated. Proper interventions, modern treatments and therapy of tribal communities in the Nilgiris, Tamil Nadu, have been found to lessen the disease burden over a period of time.
Sickle cell anemia, a genetic disorder, cannot be cured but only be treated. All photos: By arrangement
The United Nations recognises June 19 as World Sickle Cell Day to promote awareness on sickle cell anemia and other sickle cell disorders, which are rare diseases affecting people across the world.
Closer home, in India, an estimated three per cent of the tribal population suffers from sickle cell anemia and another 23 per cent carries and transmits the sickle cell gene to their children. Nearly all tribal communities living in forested terrains across the country, have cases of sickle cell anemia.
Sickle cell anemia is a genetic disorder that cannot be cured but only be treated. However, with appropriate state-of-the-art diagnostics and intervention strategies, the disease burden at the population level can be reduced.
Two days ago, on June 17, the Union minister of health and family welfare, Harsh Vardhan tweeted about a National Digital Portal established by his ministry to help children affected by rare diseases including sickle cell anemia.
Sickle cell anemia (also called sickle cell disease) is an inherited change in the blood. The protein called hemoglobin in the blood transports oxygen from the lungs to the tissues.
In sickle cell disease the hemoglobin is chemically different (sickle hemoglobin) and though still capable of carrying oxygen, it tends to crystallize when the oxygen is removed causing change in shape of the red blood cells and rendering them sickle-shaped or a half-moon shape.
If the sickle cell is inherited from one parent it is called sickle cell trait or sickle cell carrier. If inherited from both the parents it is called sickle cell disease or sickle cell anemia (homozygous sickle cell). There is no other way one gets sickle hemoglobin except from the parents.
The normal lifespan of a sickle cell anemia patient is approximately 40 years. Proper interventions and modern treatments and therapy have been found to lessen the burden of the disease over a period of time.
The tribal communities of the Nilgiris, Tamil Nadu, have a prevalence of sickle cell anemia. In fact, it was in the course of his work with the tribal hamlets in remote corners of the Nilgirs in the late 1950s and the early 1960s, that the founder of the non-profit Nilgiri Adivasi Welfare Association (NAWA), S Narasimhan, identified the prevalence of sickle cell anemia among the forest dwelling communities. He was awarded Padma Shri and Dr. B.C Roy Award (the highest Indian award in the medical category) in recognition of his dedicated tribal welfare and medical relief work.
Ever since, NAWA has relentlessly carried out medical research and intervention amongst the tribal communities with a focus on sickle cell anemia. Regular studies, screenings and testing have been carried out over the years, identifying communities with great incidences of sickle cell anemia and organising interventions for it.
NAWA has so far screened 26,609 individuals, including 5,008 non-tribals, in the Nilgiris and parts of Coimbatore. Of these, 107 are diseased and 2,359 are carriers.
A monthly package treatment consisting of HydroxyUrea, folic acids, multivitamins, proteins, food packs and minerals are provided to improve their nutrition. The aim of these services is to lessen the pain crisis, and extend lifespan.
GKD Charity Trust, a unit of Coimbatore-based Lakshmi Machine Works, under its CSR funds, supports NAWA for sickle cell screening and intervention in the predominantly tribal areas of Anaikatti for the past two years.
A dedicated team of doctors, nurses, lab technicians and support staff has kept the sickle cell anaemia research and interventions going in Tamil Nadu. The central and state governments, non profits, dedicated social workers and generous people in the region have kept NAWA relevant to the community needs for the past 63 years enabling it to do meaningful work in the area of sickle cell anaemia.
In 1995, the Indian Council of Medical Research (ICMR) formed a national network to study Nutritional Anemia and Haemoglobinopathies among some tribal populations and NAWA became involved in the work.
A sickle cell screening and intervention programme was started in 1998 and continued till 2004. In the period, approximately 4,000 individuals from tribal communities in the Nilgiris were screened and suitable interventions were made.
Since 2009, NAWA has been also associated with the National Health Mission in Tamil Nadu and interventions are being carried out with government support in Coimbatore and the Nilgiris. Antenatal patients, whether tribal or non tribal, are referred to NAWA for screening by all Primary Health Centres in the area.
In 2019, the ICMR appointed NAWA as a task force member in the assessment of neonatal screening approaches for sickle cell disease and the effect of early interventions in the management of the disease in tribal populations.
NAWA has a laboratory to conduct advanced tests with state-of-the-art testing equipment. New born babies are also being screened for sickle cell in Tamil Nadu at present.
Thus, in the course of more than five decades of work in the field, NAWA has acquired an in-depth knowledge in the management of sickle cell disease.
For better outreach and impact, the need of the hour is to establish a specialty hospital with modern equipment to screen and treat sickle cell patients. This hospital should also be equipped to provide community outreach, genetic counseling and appropriate intervention measures.
An exclusive hospital for the inherited blood disorder, staffed with well-trained, full time doctors and hematologists will go a long way to minimize the incidence of sickle cell.
The following points need to be kept in mind while responding to sickle cell anemia:
K.Vijayakumar is director, education, Nilgiris Adivasi Welfare Association. He has been associated with the non-profit since 1977 and overseen many health related initiatives.
Views are personal.