Father's sacrifice in vain

Aiden Roux who died of a rare liver disease, Alagille Syndrome, was a happy, energetic child. Photo / Supplied

A toddler died of a rare genetic condition, two days after his father donated part of his liver in a last-ditch bid to keep the boy alive.

Aiden Roux was born with Alagille Syndrome, a liver condition which affects only one in 100,000 people.

The 2-year-old was put on the waiting list for a transplant in January and his father Paul, who shared Aiden's blood type, was tested to see if he could be a living donor.

Paul got the go-ahead in April and Aiden's nine-hour surgery went ahead on Tuesday.

But when he came out his liver wasn't working as well as doctors expected.

"He got really sick and they listed him as priority in Australasia for another liver - meaning the next one that came," said his mother Sharon.

Aiden never got it. In the early hours of Wednesday his parents were told to say their goodbyes.

"He had another surgery and they saw part of his bowel had died," said Sharon. "They cut it out and expected him to get better but he didn't."

Aiden had a third operation and doctors found his entire bowel had died.

"We said goodbye for a second time ... everyone was praying for a miracle," said Sharon. "It was so hard, my husband was in a hospital bed and was told not to move. They moved his bed beside Aiden's but he couldn't hold him while he was dying."

On Thursday the Hamilton couple turned off Aiden's life support and told his 5-year-old sister Hayley the sad news.

"She kept saying she wanted to see him. She stroked his hair and held his hand and when they pulled the blanket over his face she said, 'mummy, he can't breathe anymore'.

"I said he didn't need to breathe anymore because he's in heaven."

Aiden's funeral was held on Friday. Sharon said an independent assessment was being carried out to establish why his bowel died but it appears the blood supply was affected.

However, she and Paul, who is still recovering, knew their son's medical team had done everything possible.

"They all worked so hard to save his life ... the transplant surgeon was devastated."

Aiden was diagnosed aged 6 months. There was no history of the condition in the family.

Sharon said alarm bells rang when his jaundice did not clear up after his birth.

Medics thought he had biliary atresia, a rare condition in babies where the bile duct between the liver and the small intestine is blocked or absent. But several months later he was referred to Starship Hospital where specialists diagnosed AGS.

"He had a mutation his specialists had never seen before."

Aiden's jaundice continued and he developed severe itching as a result. Sharon and Paul barely slept for 10 months, tending to him "every half hour".

"But the lack of sleep didn't faze him. He was never a sickly child, he was always running around laughing and smiling ... a typical little boy," Sharon said. "He was priceless to us. He was cheeky, boyish and energetic. He was very busy - not at all like a sick child."

Sharon and Paul received huge support from family and friends. They also wanted to thank Aiden's GP, Dr Helen Evans, and nurse Karen Sanson at Starship, Ronald McDonald House, True Colours and the Medically Fragile Children's Care Programme in Hamilton.

Genetic disorder

* Alagille Syndrome (AGS) is a genetic disorder restricting the liver's ability to produce and distribute bile.

* In a healthy liver, bile moves through ducts into the small intestines where it helps digest fat.

* In AGS sufferers, bile builds up in the liver, causing scarring and preventing it from eliminating waste.

* AGS can also affect the heart, kidneys, pancreas, eyes, face, skeleton and central nervous system.It is estimated to affect one in 100,000 babies, but some children show no symptoms.

* There is no cure and more than 10 per cent of sufferers die, mostly from heart or liver disease.

* Most sufferers with severe liver or heart problems associated with the disease are diagnosed in infancy.