Visual impairment is a public health problem of global proportions. More than two hundred and eighty-five million people worldwide have some form of visual impairment. Thirty-nine million of them are blind. Ninety percent of those affected by blindness and severe visual impairment live in the poorest countries in the world. Fourteen million live in India alone. Yet a good proportion of worldwide blindness, including 75% of corneal disease, is curable. Blindness is heavily impacted by poverty. Between six and seven million of the twelve million people worldwide who are currently waiting for Cornea transplants live in India. Whilst the average age of a patient needing a corneal transplant in Canada is seventy-five, in India the average age is just sixteen years old, which severely impacts life prospects and outcomes.
Siblings Ameer (14) and Sadiya (12) Hamza live in Jeedimetla, a poor urban community on the outskirts of Hyderabad in India. They are in many ways typical of most young teenagers. They have many friends. Ameer loves cricket and playing with smartphones. He is an extrovert who avoids doing household chores and dreams of one day becoming a pilot. Younger sister Sadiya is quiet and sensitive and prefers to stay inside to play with her dolls and practice reading.
The family lives in a two-bedroom flat with their mother and father, as well as their elder brother Irshad and elder sister Shazia. Both Ameer and Sadiya suffer from Xeroderma Pigmentosum (XP) a rare genetic condition characterised by extreme sensitivity to sunlight.
The local urban community in Jeedimetla is mostly comprised of families from poorer backgrounds who are working and aspiring towards the lower middle classes of India. A typical day for the Hamza family starts early, especially during Ramadan, when the family rises early for Sehri before fasting through the day until Iftar and then dinner at around 18:45. After dinner the family might typically be found huddled around their television.
Life is not easy for a family with two children living with XP. Paying for treatments and buying medicines is expensive. A few years ago their father had to sell all of their mother’s jewellery to pay for treatment for Ameer and Sadiya. A false ceiling contractor by trade, work is not regular for their father, and so the family has little reliable income and security. It was not until they discovered the Tej Kohli Cornea Institute that Ameer and Sadiya were handed a lifeline: the opportunity for life-changing cornea transplant operations funded by the Tej Kohli Foundation.
Despite both living with challenging symptoms of XP and deteriorating eyesight , Ameer and Sadiya have a relationship that is typical of any teenage brothers and sisters. They fight a lot, but also have a strong bond, and Ameer is very protective of Sadiya. Due to their condition, they are mostly unable to leave the house, so they often play together inside.
Sadiya is the sensible one: she follows all instructions precisely and is very careful to ensure that her skin isn’t affected by the sun and that her eyes don’t suffer. Ameer by contrast is cheeky and naughty, and often ignores his parents’ warnings and goes outside unprotected.
Sadiya feels great sadness about her XP. She wants to be ‘normal’, but knows that she is not like most other young girls. Even Ameer questions if he will ever get better, and although he is active, there are moments when he questions why he too is not like other boys his age.
Not being able to play in the sun also creates a lot of restrictions, compounded by the prejudice that Ameer and Sadiya face as a consequence of their physical appearance. They used to enjoy going to school, where their classmates were extremely supportive, but they stopped attending school because their deteriorated eyesight made it impossible to learn in a classroom.
There are only a few studies into the prevalence of XP and very few recent ones. Japan has a high incidence of XP of one in every 20,000 of population. In the UK there are just 200 patients in the entire population. Tej Kohli Cornea Institute data suggests a prevalence in India of between 1 and 27 people in every 10,000 of population, but there is underreporting because of a lack of awareness. There is also a social stigma attached to skin problems in India, and being a sunny tropical country, the high degree of sun exposure makes the visual symptoms of XP more pervasive and aggravates the degradation of ocular problems that lead to blindness. Many still believe the superstitions that skin conditions are a curse and don’t seek medical help.
At birth Ameer did not show any symptoms. But over time and through exposure to sunlight, his parents realised that something was wrong. At just 18 months old his parents first discovered strange spots under Ameer’s eyes. They first assumed it was an allergy, and so for some time treated the spots with balms and oils, but the damage had already been done to his cornea, which became opaque.
As the spots kept growing, Ameer’s father took him to Mumbai and then Lucknow to see skin specialists – a costly trip far beyond the financial means of the family. Initially Ameer received treatment for his skin, but by the age of eight, his eyes started turning red and then white, and his vision started becoming seriously impaired. Doctors were unable to come up with a solution, and eventually referred Ameer to the specialists at the Tej Kohli Cornea Institute.
XP affects every part of the body, but loss of eyesight is particularly pervasive. Scarring of the cornea over time makes vision opaque and foggy. With more severe scarring, the cornea becomes completely opaque, which then leads to tumours. The cornea can become devoid of blood vessels, reducing moisture in the eye and culminating in a loss of vision that can only be corrected by a complete corneal transplant. Ameer and Sadiya had developed severe vascularisation in their corneas, and full thickness corneal scarring meant that they each needed corneal transplants for both eyes.
The Tej Kohli Cornea Institute is funded by the philanthropic Tej Kohli Foundation. The vast majority of patients of the Tej Kohli Cornea Institute do not pay for their treatments. Ameer received his first cornea transplant for one eye in October 2018 and his second transplant for the other eye took place in May 2019. Soon his vision will be fully restored, and he hopes to soon start going back to school again. If Ameer and Sadiya had not received surgery before their cornea damage became permanent, they would have irreversibly lost their vision forever.
The technologists of the Tej Kohli Foundation are developing solutions to major global health challenges whilst also making direct interventions that transform individual lives around the world. A primary activity of the Foundation is the Tej Kohli Cornea Institute in Hyderabad, which is a globally eminent institution for corneal health and expertise. The Tej Kohli Cornea Institute is a global leader in research and development, preventative medicine and Corneal transplants. The majority of patients of the Tej Kohli Cornea Institute receive completely free treatment.