Sickle Cell Centre

Sickle cell disease (SCD), an autosomal recessive disorder of the red blood cell, is the commonest monogenic disease worldwide with more than 300,000 affected births annually, mostly in low and middle-income countries. The deformed red cells in SCD lead to recurrent acute painful vasoocclusive episodes called crisis, hemolysis,  anemia, and organ damage resulting in impaired quality of life, and premature death.

SCD was first described in India in 1952 among the Toda and Irula tribes living in the Nilgiri Hills to the southeast of the Gudalur Valley. It is now known to be present among the Adivasis of the Deccan plateau of Central India, Orissa, Wyanad district Kerala, Gujarat, and areas where malaria is endemic. SCD is an evolutionary and protective mechanism against malaria.

In 2010 three countries, Nigeria, the Democratic Republic of the Congo, and India accounted for more than half the births with SCD globally with approximately 44,400 babies born in India. This is more than 20 times the number born in the US.

The Gudalur Adivasi Hospital (GAH) has played an important role in addressing SCD in the Gudalur Valley. The SCD programme was started in 1997 with the establishment of the haemoglobin electrophoresis diagnostic facility, using the equipment and training provided by Dr. Ronald Nagel of Albert Einstein College of Medicine, New York, USA.  In 2004 the programme was greatly enhanced with the establishment of the SCD Centre at GAH with the support of Mr. Hari Prabhakar of Tribal India Health Foundation, the American Tamil Medical Association, and the  Government of Tamil Nadu. Screening for SCD was started in the villages, Area Centres, and GAH as a standard of care, using the sodium metabisulfite sickle cell preparation test. Positive samples underwent cellulose acetate electrophoresis to be differentiated into HbAS, Sickle Cell Trait (SCT), and HbSS, SCD. In Dec 2011, neonatal screening for SCD by High Performance Liquid Chromatography (HPLC) performed by an accredited laboratory (Neogens Lab, Bangalore) was initiated at GAH.

In the SCD patients, a standardized bundle of care termed “comprehensive care” is delivered, inclusive of counselling, healthcare review either at the Area Centres or GAH, multivitamins, iron, and analgesics. From 2005, prophylactic penicillin until 5 years of age was offered; symptomatic patients and those with a history of admissions in crisis were started on 5 hydroxyurea (5HU). In 2011 the polysaccharide pneumococcal vaccine was introduced, as this was not part of the National Immunization Program, and administered at the age of 2 years and penicillin discontinued.

The Centre currently takes care of more than 300 patients with SCD. It is supported by the Govt. of Tamilnadu and mentored by Dr. Lakshmanan Krishnamurthi of Emory University, Atlanta, USA