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World sickle cell day 2017

Sickle cell disease is endemic among some populations in India. As a genetically inherited condition some populations are disproportionately affected. In India. There are many poorer, rural areas in which the disease is prevalent, as well as within a number of tribal populations. Marking June 19th as World sickle cell day, charities asked that the government step in to do more to tackle the condition.

Copyright: rob3000 / 123RF Stock PhotoNagpur needs a National Sickle Cell Treatment, Research and Training Institute in the city, according to the Sickle Cell Society of India, Nagpur, has spoken to Union Social Justice Minister Thavarchand Gehlot requesting him to make efforts towards the setting up of a. This would be a significant advancement in aiding those afflicted with the condition, as Nagpur city is a central location within what is considered the sickle cell belt of India.

There are 14 lakh patients with sickle cell disease across 13 states and one union territory but  70 percent reside in an area close to the Nagpur region according to the President of the Sickle Cell Society of India, Sampat Ramteke. This would make the city a strategically important location for any attempts to deal with the disease.

There has long been criticism that not enough is being done to tackle the disease. As previously reported on Health Issues India, the Sickle Cell Institute was set up in 2013 to combat it, though of the 180 positions on offer at the institute, 154 remain vacant. Critically, of the positions that remain vacant, most are roles established for medical personnel. Of the few positions that have found an employee, most are administrative.

In the communities affected with the disease, up to 35 percent of people can be affected. When an inherited disease is as frequently occurring as this, it is inevitable that a large number of children born in the area will also have the disease. This necessitates the presence of specialised healthcare units in accessible areas as the illness will recur across generations and require lifetime treatment.

The genes related to the disease code for an abnormal haemoglobin protein, haemoglobin S. If genes from both parents code for haemoglobin S, the most severe form of the disease is inherited. This condition, haemoglobin SS or sickle cell anaemia, can be fatal.

The abnormal protein inherited in sickle cell anaemia forms a sickle shape, instead of the standard disk shape. Disk shaped blood cells are flexible, capable of navigating the varying diameters of blood vessels without issue. Sickle cells lack this flexibility. This causes an increased chance of blood vessel clogging which can kill.

A severe symptom is known as sickle cell crisis. This occurs when blood flow to a region of the body is deprived due to sickle shape blood cells clogging a blood vessel. This can cause severe pain which may last an hour, or up to several weeks. Severe pain and aching are common features, as well as a susceptibility to infection resulting in fever.

Though treatments have been suggested and trialled for stem cell and bone marrow transplants, the procedure is risky, and may result in immune system rejection by the recipient. Currently the most utilised treatment method is consistent blood transplants across the patient’s lifetime. This again highlights the importance of the calls from the Sickle Cell Society of India that treatment should be available locally for those in highly affected areas.


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