Chamarajanagar: Unusual incidence of Xeroderma Pigmentosum

Xeroderma Pigmentosum is a genetic disorder that impairs the DNA’s ability to repair damage that may be caused by exposure to ultraviolet rays for instance. Symptoms include severe sunburns after even short term exposure to the sun, dry skin and hearing loss. Patients are at risk of skin cancer.

 It is an autosomal recessive disorder. What this means is that if 2 parents who do not have the disease but are still carriers of the gene have an offspring, there is a 25% chance that the offspring would also be affected by the disease. Why this becomes relevant in the case of Chamarajanagar is because the prevalence of the disease is higher where the prevalence of consanguineous marriages are common. Most of the affected children are from the Banajiga Shetty community. If genetic testing is carried out before marriage, it is possible to identify carriers and prevent matches that are detrimental. 

But there is not enough acceptance among the villagers. They justify the continuing practice in the name of tradition. Doctors have been stressing on the need to increase awareness and improve genetic testing facilities. 

Doctors are also pressing the government to take cognizance of this pressing issue. “The government must ensure that the entire community undergoes genetic testing. The government should provide the tests at subsidised rates,” Dr Bhat said while speaking to The South First.