Rare genetic disease highlights ills of marriage tradition.
Byline: Kamila Hyat
Quetta The discovery in the Bolan and Kaachi areas of the south-western Balochistan province of seven children suffering a genetic photosensitivity skin disorder that endangers their lives has focused attention on the widespread practice of marriages between close relatives.
Dr Fazeela Abbasi, adviser to the ministry of health, told the media in Islamabad that the children were suffering from xeroderma pigmentosum, an extremely rare autosomal recessive genetic disorder of DNA repair in which the ability to repair damage caused by ultraviolet light was deficient. The disease involves photosensitivity, pigmentary changes, premature skin ageing and malignant tumour development.
People in the affected area say hundreds of children have died due to the disease in the past. "I lost two nephews to the disease a few years ago. Dozens others have died. No one has helped us," Imdad Baloch told said from Bolan.
A team from the Balochistan Health Department, headed by Dr Eisa Khan Jogezai, has visited the village where the seven children suffering the disease are based. According to media reports, the team has stated "no successful treatment" was offered to afflicted children in the past.
Marriages between close relatives, especially cousins, are extremely common in Pakistan. Research from the 1990s indicated 46.5 per cent of marriages in the eastern Punjab province may involve such unions, usually arranged by family elders.
"We need far more research on this," Dr Qazi Amjad, a Quetta-based paediatrician held.
According to some experts, 44 per cent of thalassaemia cases are a result of marriages between cousins. There are an estimated 50,000 to 100,000 thalassaemia sufferers in the country while 5,000 babies suffering the blood disorder which reduces levels of haemoglobin in the blood are born each year.
A 2007 study shows parents of children affected by thalassaemia strongly favour genetic screening before marriage. However, Dr Amjad said, "In provinces such as Balochistan, there is almost total ignorance about such matters."
According to official figures, Balochistan has a literacy rate of only 34 per cent, while the national literacy figure is 52 per cent. Diseases in children, attributed to genetic factors, have also been reported from other parts of the country.
The widespread media attention for the children from Balochistan has generated new attention to the issue. "I had no idea such serious health problems could be caused by marriage within families," Zarina Shaukat, the mother of three teenage daughters, said.
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