Progeria patients surviving for existence

Seeing 22-year-old Ikramul Khan and his brother Ali Hussein, a 11-year-old child, with their almost translucent skin, emaciated bodies, thin hair, weird looking fingers and a pair of alien-like eyes with a big smile revealing unaligned teeth … is not a very pleasant sight. They are like Auro in the film Paa starring Amitabh Bachchan, who plays the role of a child suffering from progeria, a genetic disorder in which the child ages at an unbelievably fast rate. It is a debilitating, rare illness with just 45 odd cases in the world. The disease, which infects one in four lakh people, is present in India's Bisul Khan and Razia Khatun's family, where more than one member suffers from progeria. Bisul Khan (45) and his wife Razia (40) had seven children in all. Five of them were born with progeria, which is characterized by premature ageing. Out of the five, three daughters are dead. Their daughter Sanjita (21) and youngest child Gulab are healthy. Ikramul and Ali Hussein are still alive but their medical ages are 85 and 66 respectively. Rehana was 23 when she died a natural death last year. Her medical age was 90 years. Though they are basically from Dumri village in Saran district of Bihar, they were forced by the villagers to shift their base out of the village in 2003. Villagers believed that the children of Razia and Bisul Khan were cursed. "We could not forget those dreadful days. My children could not come out of the house during daytime.

They were considered bad omens; they were called 'ghosts' and the villagers abused my wife as a witch. We were forced to live at the periphery of the village," said Bisul, who works as a security guard in Kolkata. Their mother Razia too could not stop herself and says, "The villagers made our life hell. The children could not wear stitched clothes, as they would not fit. They had to wrap blankets around their body. Whenever they came out with blankets around them, people laughed at them. So, they used to sit locked up in the house all day." Tired of being taunted and ostracized, Razia and Bisul Khan even planned a family suicide. Until they reached Kolkata about six years ago, they had no clue about this medical condition. Bisul became sure that his children were abnormal, as they could not bend their legs while sitting for toilet. Their legs were stiff even at tender ages of two and three. "We did everything for our children. We took them to child specialist, skin specialist, homeopathy and even ayurvedic practitioners. But no one could diagnose the disease. Had we been explained the problem earlier, we would not have gone for so many children," said Bisul. It was a chance meeting with doctors like Apurba Ghosh, director of the Institute of Children Health, Kolkata and Dr Chandan Chatterjee from Switzerland, who were the first to diagnose this disorder. In fact, when Rubena died of pneumonia, it was the first time the medical team was convinced that the kids were affected with progeria. Rubena's death proved that progeria was actually an inherited and recessive condition. "There are, in all, 45 known cases worldwide, all isolated and seemingly random.

A progeric child's ageing process remains normal up to the age of seven. After that they develop striking characteristic syndromes like failure to thrive, premature baldness, heart disease, thinning bones and arthritis and several other symptoms that people grapple with in their old age. But progeria does not hamper the average IQ level of the children," says Shekhar Chatterjee, general secretary of S B Devi Charity Home, Kolkata which takes care of progeria family's needs, financial problems and medical requirements. Dr Chatterjee has been working in collaboration with the University of Basel, Switzerland, where a team led by him has been constantly monitoring the progress of the family. Progeria is caused by a single tiny defect in the child's genetic code, but it has devastating and life-changing consequences. Traditionally, progeria victims do not live beyond their early 20s. But as compared to other victims, Ikramul who is now 22 is still alive. "We know there is no cure for the condition, but over the years we have seen that healthy food and lifestyle changes can enhance the life expectancy. All the credit goes to proper medication, healthy diet and regular psychological sessions that Ali and Ikramul have been able to survive for so long," says Dr Ajay Mukherjee, the senior researcher with the project. Ali is vivacious among them. He sounds more like an enthusiastic 11-year-old despite the 66-year-old body where he has been trapped. "I'm a die hard Salman Khan fan and could dance like him six months back. I danced well in a Rotary Club function. They had given us Rs 2,000 to get a set-top box." Ikramul loves Shahrukh Khan. But his frail body cannot retain this enthusiasm. He takes long breaths in between. His slow movement from one place to another inside the house proves that his brittle bones and stiff joints do not permit him to move freely. Both the boys know about the film Paa and want to meet Amitabh and Abhishek Bachchan. "I'll be very happy to meet them. At least they thought it important to work in a film based on our life," said Ikramul. For Ali also meeting Amitabh will be a memorable day of his life." The Khans look upon Dr Chatterjee as a family member. "He looks after us, bears all our expenses, provides us accommodation. Due to his kind cooperation we are now able to stay at our village home.

Our relatives and neighbours frequent our home to see Ikramul (23), the only progeric person to have survived this long. He is father figure for our children," said Bisul Khan, father of the boys. But in spite of all endeavours, life is far from normal for the boys. According to Dr Chatterjee, both of them suffer from astro-arthritis and cannot bend their legs or sit properly. Apart from weak bodies, they have underdeveloped livers and hearts. "They face difficulty in maintaining their balance when a fan is in full speed. They even need help for simple activities like changing clothes," says their mother. Ikramul, who speaks less and is depressed ever since his elder sister Rehana passed away, knows the pitiable condition of his disease. "I feel bad that I cannot run and play like other children. My ailments are like those of old people. I feel so sorry for myself," he laments. In spite of knowing that death is imminent for them, they enjoy their lives and have fun in their own way. Ali and Ikramul spend most of their time watching TV or downloading songs on the mobile phone, which Dr Chatterjee has gifted them and manage to find reasons to smile.link