Why you should leave your niece alone

The pressure cooker whistle goes off suddenly shattering the peace Your old mother starts But your first child now around seven months old doesnrsquot even blink She continues cooing and gurgling Soon enough you go to the paediatrician worried something may be wrong More tests are done and you learn with profound dismay that your baby was born deafA year or so later another child comes along He too is deaf You now have two deaf childrenDeaf children do not learn language or speech since they cannot hear and are often cruelly called deaf and dumb In their bleak silent world nothing makes sense Their clumsy sometimes incongruous responses lead their parents to conclude wrongly that the child is mentally retarded or god forbid possessed by a demonIt can take a long time sometimes forever to realise that your adorable child is deafIt can take even longer to understand that you the parents may be the cause of that deafnessThe father in the story is real he is Nagaraj Reddy wife Parvathy When they talk about their two children their eyes brim over and their voices break with pain But herersquos the thingmdashParvathy is the daughter of Nagarajrsquos elder sister They were under pressure from their families to marry even though neither particularly wanted toldquoIf we had known then what we know today we would never have marriedrdquo he saidNagaraj is one of countless Indians who agreed to a consanguineous marriage often under family pressure They married a first or second cousin Or an uncle married a nieceAlways neither understood the dreadful horror their marriage was going to unleash on the children they would siremdashnot just deafness but any of a host of birth defects from malnutrition deformation to cognitive impairmentYesterday I spoke with a frail 89yearold woman a lifelong paediatrician who for 33 years has been singlemindedly chipping away at the problem finding ways to tell people about consanguineous marriages and preventable deafness Her name is Dr Najoo VarkeyThe word wizened describes her perfectly Blinking behind glasses she is alert and attentive missing nothing around her memories past and present intact Over 33 years ago as a practising paediatrician she began offering free services to examine deaf children at Bengalurursquos Sheila Kothawala Institute for the Deaf It was not easy most children had no birth or family details Each child took about a year to document in detailBut then she began to notice an odd thingmdashabout half the deaf children she examined came from parents who had been closely related to each other before marriage Consanguineous marriagesDriven by nothing more than a feeling that something was dreadfully wrong and needed to be fixed she began to gather information to understand the problem Najoo Varkey is driven by empathy and compassion not donor funds and her fuel is her keen scientific intellect Nothing could stop herFirst question Why does marriage between close relatives even cause deafnessAn embryo receives genes from both parents strung out along strings called chromosomes Each parent contributes 23 chromosomes half of their own set which join to become the blueprint of the humantobe When the parents are unrelated their genes will be diverse In consanguineous marriages many of the parentsrsquo genes or chromosomes will be identical There may be multiple copies of defective diseasecausing genesSuch as the one that causes NonSyndromic Recessive DeafnessThe specific gene that causes deafness when relatives marry is Connexin26 snug inside chromosome 13 It was found to be behind deafness in about 20 per cent of the children in two studies by the AIIMS in Delhi and the Jawaharlal Nehru Centre for Advanced Scientific Research in BengaluruLeading the list were the four south Indian states of Kerala Tamil Nadu Karnataka and Andhra Pradesh Nearly half the marriages there are consanguineal specially among impoverished rural communitiesAbout 52 per cent of deafness in India today is caused by consanguineous marriages That means about 85 lakh of 84 lakh deaf in India are that way because their parents should never have marriedWhatrsquos the solution The law is an ass It will just ban such marriages and just drive an ageold cultural practice underground But everything Dr Varkey has done in three decades shows that any young person who understands the tragic future that awaits a consanguineous marriage will run from it Without hesitationYou can do something though Send an email to pervinvarmayahoocom and say yoursquod like to help Theyrsquoll send you a cute free little book in which all the results of Dr Varkeyrsquos formidable labour of love have been put together by her granddaughter and other family members so beautifully that you could give a TEDx talk on it or a discussion at the next anganwadi meetingAsk for it Yoursquoll love the bookrsquos name Donrsquot Marry Your NieceHere viewed from there C Y Gopinath in Bangkok throws unique light and shadows on Mumbai the city that raised him You can reach him at cygopigmailcomSend your feedback to mailbagmiddaycomThe views expressed in this column are the individualrsquos and donrsquot represent those of the paper