A LEADING liver doctor has paid tribute to the bravery of a little York girl who needs a transplant for a life-threatening disease.

Yesterday we reported how Lydia Mellen, six, from Shirley Avenue, York, was battling a rare illness called PFIC (Progressive Familial Intraheptic Cholestasis).

Lydia inherited the disease from her parents, Richard and Sam, as a baby because they happened to have a particular mutated gene which has the wrong instructions for bile formation and excretion.

Neither of her parents knew they had the abnormal gene until Lydia was born in 2000.

Today, the bubbly six-year-old has to take daily medication and parents say she now needs a transplant in order to survive.

Dr Sanjay Rajwal, a consultant hepatologist (liver specialist) at St James's Hospital, Leeds, said little Lydia was a credit to the care of her mum and dad.

He said: "Her parents are brilliant. They manage her very well and that's the reason she's doing quite well at the moment. She's a credit to both of them."

Previously, Lydia would have had to rely on getting a liver donation from a dead person to get a transplant. There are usually about 50 people on the waiting list for a liver transplant at St James' Hospital.

The average person has to wait for up to a year for a suitable organ, and one in five die waiting.

But last month history was made at St James's when surgeons performed the first live liver transplant there.

Dr Rajwal said the operation gave patients like Lydia a real new hope.

He said: "This condition is unusual. She might need a liver transplant, unfortunately we can't predict when she will need one.

"There is a more than 90 per cent chance she'll do well if we can do a transplant.

"We can do a cadaverous liver transplant (from a dead donor) or we can do a live one as well - and that does give her hope."

St James's Hospital is one of only three specialist liver hospitals in the country. It has more than 20 children on its books with PFIC, who have come to be cared for there from across the country.

Transplants from live donors have been carried out in other countries for more than 15 years, but have only just arrived in Britain.

To perform the operation, surgeons take out part of the healthy liver from a living person rather than waiting for a suitable match from a dead donor.

The organ re-grows to almost its normal size in the donor within weeks.


What is PFIC?

* PFIC refers to a group of conditions, all inherited, which cause "cholestasis", or reduced bile flow, in children.

* Bile is a yellow/green liquid made in the liver which plays an important part in breaking down the fat in food into very small particles so it can be digested, its energy used and its vitamins absorbed.

* Research is still being carried out into PFIC, which can go undiagnosed for months or years.

* In most cases, if a couple has a child with PFIC, any other children they have together will have:

* A 25 per cent chance of having PFIC * A 50 per cent of being a "carrier" of the disease * A 25 per cent chance of being completely clear from it.