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Monday, November 3, 2008

3-year-old's rare liver disorder cured

October 22, 2008

NEW DELHI: Fatima (name changed) wasn't old enough to be at work for long hours. The three-year-old child from the Middle East did not even have time to play or go on vacations since her birth. She wasn't even free on the weekends.
Wondering why? Fatima was suffering from Crigler Najjar Syndrome (CNS) a rare yet fatal liver disease. And the only way she could stay alive was to be under constant phototherapy lay still without clothes, exposed to specific wavelengths of light, for 10 to 16 hours a day.

But now, for the first time in her life, Fatima is free of such captivity, thanks to a team of Indian doctors from Indraprastha Apollo Hospital in Delhi.

To eliminate the need for phototherapy, Dr Subhash Gupta (liver transplant expert) and Dr Anupam Sibal (paediatrician) have successfully transplanted part of Fatima's father's liver into her.

The bilirubin level in Fatima's body is now normal. Cured of CNS, Fatima has now returned to her country to lead a normal life.

CNS is a rare disorder in which bilirubin (a substance made by the liver) cannot be changed into its water-soluble form, bilirubin glucuronide. The defect is a deficiency of a liver enzyme with carries out the conversion. Children born with Type 1 CNS develop severe jaundice soon after birth. If not treated immediately, these infants suffer from brain damage which is usually fatal.

The disease affects 0.6-1 per million live births. Liver transplantation is the most effective way to provide the missing enzyme.

"Since the defect was with the liver, we decided to transplant part of the healthy liver of Fatima's father into her. She will not require any more phototherapy. She will only have to be on medicines all her life, the dosages of which will lessen as she ages," Dr Gupta told Times City.

Dr Sibal said CNS typically manifests in the first few days of life and the jaundice, instead of disappearing in the first two weeks, persists and increases over the next few days to weeks.

"Very high levels of bilirubin can cause permanent brain damage," Dr Sibal said. "The main therapy for patients with this disease is phototherapy. Greater the surface area of the body that is exposed to light and greater the intensity of light falling on the skin, higher is the efficacy of phototherapy," he added.

"Fatima was living in agony, spending 10 to 14 hours every day under phototherapy since birth. This was severely restricting her lifestyle. Even travelling and vacations were a great problem."

Phototherapy also has many side effects which include increased water loss, diarrhoea and tanning of the skin. Phototherapy becomes less effective with age as the skin thickness.

"The requirement to be without clothes during phototherapy caused Fatima tremendous embarrassment and also caused problems with maintenance of body temperature. Both Fatima and her father are doing well. Her eyes and skin are no more yellow in colour," Dr Sibal said.

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