When daylight kills: India's XP children




  • In Science
  • 2019-06-26 08:15:20Z
  • By The Telegraph
 

As the sun sets on the poor Muslim neighbourhood of Jeedimetla in the city of Hyderabad, life begins anew.

The faithful wander out to answer the call to prayer and so too do those cursed with Xeroderma pigmentosum (XP), a rare genetic condition that makes the sun's rays lethal.

For Ameer Hamza it is the best and worst of times. The cricket mad 14-year-old must stay indoors during the day but, as the sun sets, he dashes down from his family's apartment to a dusty playground to join dozens of other local children at a makeshift wicket. It's his only real freedom but few of the other children understand.

"What the hell are you doing here?" one jeers, trying to wrestle the cricket bat from his grasp. Visibly shaken, Ameer says bullying is part and parcel of life. XP marks him out as visibly different and many of the other children, wrongly believe the condition is infectious.

"This is why we don't play here too often and I stay indoors," Ameer explains. Only one other boy in the entire neighbourhood will talk to Ameer, 12-year-old Junaid.

Together they leave the playground and trudge back to his home and away from their tormentors. It is estimated that up to one in 370 Indians suffers from XP - one of the highest incidences of the condition anywhere in the world.

Contact with UV rays causes a sufferer's skin to pigment, burn and take on a blistering, scaly appearance. Despite its prevalence in the country there is still enormous stigma with sufferers shunned as people believe they are cursed.

Fear around the disease is compounded by the unknown; sufferers usually stay indoors in order to avoid UV ray exposure.

Without treatment, half of those with the condition contract skin cancer by the time they turn 10.

And around a third of those with XP also suffer from a range of debilitating nervous system conditions including difficulty walking, decreased intellectual function and seizures. Without treatment, life expectancy is little over 30.

"XP is a rare, genetic condition where the body is not able to repair damage to the DNA in the skin, caused by UV rays present during daylight hours," explains Dr Hiva Fassihi, the clinical lead of the National XP Service at Guy's and St Thomas' NHS Trust in London.

"Because of this XP sufferers develop abnormal freckles at exposed sites and are at significantly increased risk of developing skin cancer from a young age.

"The management is all about prevention and education, as soon as you see a child with freckling on the skin - which is not common in India - they must protect their skin from UV rays."

Blindness is also endemic among those with XP as the front covering of the eye, the cornea, becomes increasingly damaged with exposure to the sun.

Few Indian children with the condition finish school because of their deteriorating eyesight deteriorating. Without qualifications, unable to venture out into the sunlight and at the mercy of their communities they typically spend their days hidden away in family homes.

Ameer's sister Sadiya, 13, also suffers from the condition. Their father Mazhar Ahmed, 47, is wracked with worry about who will support his children when he is no longer able to work. Significantly, Ameer's parents are cousins.

Although few reports into the condition have been carried out it is believed to be more common in India due to genetic mutation caused by the high prevalence in consanguine marriages - those between second cousins or closer relatives.

A 2013 study in the International Journal of Sociology and Social Policy found over a quarter of unions in the states of Tamil Nadu, Maharashtra, Andhra Pradesh and Karnataka were between close family members.

In-family marriages are generally rare among Hindus but much more common among India's 195-million strong Muslim population - more than 25 per cent marry consanguine.

"As XP is a recessive condition there is much higher risk within closed communities and where we are seeing consanguineous marriages, cousins marrying cousins, for example," said Dr Fassihi.

"Within these communities, marriage within the family has significant social and financial importance so as medical professionals, it is important not to judge but to educate the families with affected children about the risks in future pregnancies."

The Indian government has attempted to raise awareness of the dangers of in-family unions, but it has limited campaigning for fear of being seen to target the religious minority. However, sufferers accuse the government of abandoning them and describe years of trawling different hospitals looking for treatment, without state financial support.

Sultana Shaheen, 23, also has XP. She is house bound and lives with her parents - who are first cousins - and fourteen siblings in the Hyderabad neighbourhood of Warasiguda. Two older sisters died in their early twenties from heart complications thought to be related to XP while another brother Anwar, 24, also has the condition.

She is currently even unable to walk around her house after fracturing her ankle in a recent fall. A limited time spent in the sun means that many XP sufferers suffer from a Vitamin D deficiency, with brittle bones and a depleted immune system.

After leaving school at the age of 10 when she lost her vision, she says the highlight of her day is now washing her family's clothes. "I couldn't see clearly and so I was asked to leave school by the teachers," Sultana said. "I was extremely disappointed as I wanted to study further but school wouldn't give me any help."

To fund the search for treatment her father Khaja, 65, sold his business and her older brothers also left school as teenagers to find employment. "I just want my children to have a normal life, to go out and get married or have a job," said Sultana's mother Rehmat, 54, in between sobs. "That's my dream but what is the point of having hope for something that won't happen."

Despite her situation, Sultana counts herself as lucky. In 2016, she underwent corneal replacement surgery at the Tej Kohli Corneal Institute (TKCI) in Hyderabad. Since 2016 it has carried out 15,748 cornea replacement surgeries, largely for free.

This is vital in India where an estimated 55 million people fall into poverty annually due to out-of-pocket expenditure on healthcare and would otherwise have to pay for the surgery. "It is a very neglected condition," said Dr Sumita Chaurasia, a consultant at the TKCI. "But, it is a very important issue because as a skin disease it has so much stigma and patients are inhibited and restricted to the home."

Sai Medha Bondagiri, 19, is recovering at the TKCI after having cornea replacement surgery the day before she meets the Telegraph. Despite the after effects of surgery she is in spirited form, believing that the operation will transform her life. "I used to cry for myself when I was at home and the other kids were playing outside," Sai Medha says. "Then I realised I was always going to be like this so I had to get used to being on my own and so I have started thinking positively."

Unlike many sufferers of XP she was able to complete school after only beginning to lose her sight over the previous years. The surgery and restoration of her sight will now allow her to pursue her dream of assisting those who are partially sighted in opening a bank account.

Stories like Sai Medha's have led doctors at the TKCI to call on the Indian government to make free cornea replacement surgery available across the country and ensure expert dermatologists are available in local hospitals.

Still, sufferers in India have a long way to go both in terms of battling the serious health implications and crushing stigma. "My mother always cries when she sees me with the other girls," says Sai Medha. "She says they look so normal and she begins to cry. She says I am so different from the other girls."

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