CCMB to coordinate project on Sickle Cell Anemia

The Centre for Cellular and Molecular Biology (CCMB) a CSIR institution here will coordinate a Mission Mode Project on diagnosis, prevention and management of Sickle Cell Anaemia (SCA).

Sickle Cell Anaemia (SCA) is one of the most common blood-related disorder in India. Every year approximately 5, 00,000 children are born with SCA worldwide with India accounting for nearly 50% of the cases.

According to a statement, the mission mode project will be led by Dr Giriraj Chandak of CCMB in close association with Sickle Cell Institute of Chhattisgarh, Raipur (SCIC), with whom CCMB has entered into a MoU last year.

Five other CSIR labs, Indian Institute of Chemical Biology, Kolkata, Institute of Genomics and Integrative Biology, New Delhi, Indian Institute of Integrative Medicine, Jammu (CSIR-IIIM), Institute of Microbial Technology, Chandigarh and National Chemical Laboratory, Pune will work together in achieving the objectives of this project.

The disease is mainly concentrated in the scheduled tribe, scheduled caste and other backward caste populations of Chhattisgarh, Madhya Pradesh, Orissa, Jharkhand, Gujrat, Andhra Pradesh, where carrier frequencies range between 5-40% or more.

SCA is a genetic disease caused by a genetic defect in the beta-globin gene resulting in the replacement of glutamic acid by valine, which under deoxygenation state gives rise to a type of haemoglobin named as sickle haemoglobin (HbS).

The sickle haemoglobin sticks to each other and becomes long, rod-like structures that cause red blood cells (RBCs) to become stiff and develop a sickle shape. Due to these changes, RBCs pile up and their oxygen-carrying capacity is severely compromised.

Typically, SCA patients show symptoms such as anaemia, breathlessness, body pain, jaundice, repeated infection etc.

Their lifespan is usually reduced to 42-48 years with 50% of children with SCA dying before the age of 5. The disease management is usually symptomatic and hydroxyurea is the major therapeutic agent that is available for its treatment.

Hence early and affordable detection, treatment as well as preventive measures are important in managing this disease.
 
Under a state-sponsored programme, close to 15 lakh children (9-15 years) from 11 districts of Chhattisgarh have been screened. Overall, the project proposal is expected to reduce the burden of SCA and alleviate the suffering of SCA patients, in the state of Chhattisgarh.

Based on the success of the programme, the protocol may be implemented in other states where sickle cell disease is quite prevalent.