“Sickle Cell disease a cause for concern in tribal India belt” says Dr Nirad Vengsarkar, Renowned Orthopaedic Surgeon, Lilavati Hospital &Jaslok Hospital

“Sickle Cell disease a cause for concern in tribal India belt” says Dr Nirad Vengsarkar, Renowned Orthopaedic Surgeon, Lilavati Hospital &Jaslok Hospital

Doctor Helps Abhijeet Gunwant, A Sickle Cell Patient To Stand On His Own Feet & Get Back To Normal Life

Sickle-cell disease (SCD) or sickle-cell anaemia (SCA)is a hereditary blood disorder characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickling decreases the cells' flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the haemoglobin gene. According to the World Health Organisation, SCD is the biggest killer disease after malaria for children under five. If the disease takes place then it gets vital to treat the same. And, if the disease remains untreated then the child can even die. Early diagnosis of sickle cell in newborns as well as comprehensive care can effectively improve the situation and reduce mortality rate by 70 percent in some countries.

Says Dr Nirad Vengsarkar, reputed joint replacement surgeon associated with several hospitals like Bhatia General Hospital, Lilavati Hospital, Saifee Hospitaland Jaslok Hospital, 

“other than hereditary one of the biggest causes of SCD is poverty and the poor knowledge about the diseasein rural India. Especially in the interiors of  Maharashtra and Gujarat where this disease is more prevalent. Other state like Chhattisgadh, Madhya Pradesh, Orissa, Andhra Pradesh, Tamil Nadu also has a problem of Sickle Cell Anemia in Tribal area in variable proportions.”

India has also very huge populations of tribal community about 18 crores and expected to have 1.80 crores sickle cell trait and 14 lakhs of sickle cell disease. These show the big burden on the public health of India. Although SCD has been described in India in numerous ethnic groups, it is most prevalent among indigenous ethnic groups, classified as scheduled tribes, who have a high prevalence of socio-economic disadvantage and are frequently medically underserved. In Maharashtra, Nagpur is a belt endemic for SCD.

Citing a recent case of a 27 year SCD patient in the interior of Nagpur district, Dr Vengsarkar says, the patient was diagnosed withSCD at the age of 1.50 years and he had been getting severe pain in the hip.So basically he was operated in 2010 for his left hip and then last month for his right hip replacement.” However because of this condition the blood supply to the head of the femur gets affected, causing a condition called avascular necrosis of the head of femur. This then causes the head to collapse and loose its smooth round shape and become irregular and fragmented which in turn causes arthritis.

Adds Dr Vengsarkar, “the treatment for hip arthritis is hip replacement which in these young children is cause for concern. The problem in doing hip replacements in these young patients is that they would most likely need a revision hip replacement in the future since they have a long active life after surgery, that is they may outlive the life of the hip replacement and need a revision after 20-25 years.”

The common sign and symptoms of SCD are Pallor, Frequent jaundice, Bone & Body ache, Enlarged Spleen, Retarded Growth, Frequent Infections and Dactylitis (Hand-foot Syndrome)

On the treatment of SCD, Dr Vengsarkar says, “treatment involves preventing sickling phenomenon. Keeping the patients hydrated and with good oxygen levels. Taking medicines that reduce fragility of the red blood cells and regular antibiotic prophylaxis to prevent infection.” It is important to maintain hygiene and have an access to clean water.

SCA has no widely available cure. However, treatments can help relieve symptoms and treat complications. The goals of treating SCA are to relieve pain; prevent infections, organ damage, and strokes; and control complications. Blood and marrow stem cell transplants may offer a cure for a small number of people who have SCA. Infants who have been diagnosed with SCA through newborn screening are treated with antibiotics to prevent infections and receive needed vaccinations. Their parents are educated about the disease and how to manage it. Researchers continue to look for new treatments for the disease.