Father Donating Liver to Son

A Nelson twin with a rare genetic disorder will soon be undergoing the first liver transplant of its kind in New Zealand and Australia – with his father as the live donor.

Nine-month-old Louie Charles was diagnosed at birth with a rare genetic disorder, Propionic Acidemia, which prevents his body breaking down certain amino acids. This can result in a buildup of dangerous acids and toxins which can damage internal organs, such as the liver, heart and brain.

The disorder affects about one in 250,000 people, and there are thought to be only two children in the country with the condition.

He and his twin brother, Kees, were born four weeks premature last August, and are Deon and Nic Charles' first children.

Louie will have a liver transplant at Starship Hospital within the next six weeks, with his father, Deon, as the live donor.

This is the first live liver transplant to be done on someone with this condition in New Zealand or Australia.

The son and father will be operated on at the same time, and the family will live in Auckland at Ronald McDonald house for three months following the surgery.

Mr Charles said Louie's liver would be removed, and the left lobe of Mr Charles's liver – almost a quarter – would be transplanted into Louie.

"That part of my liver won't grow back but the cells regenerate so what's left gets bigger and grows back to the original full size in six to eight weeks. Louie's will grow to however big his body requires. It's pretty amazing."

Starship Hospital metabolic paediatrician and rare disease specialist Callum Wilson presented Louie's case at a conference in Australia earlier this year where colleagues recommended a liver transplant as the best treatment option, Mr Charles said.

"It was a shock. We knew it was always a possibility but he had been so healthy for the first six months we almost thought it wasn't necessary."

About a week later the family were in Auckland while Louie and Deon underwent a week of testing: blood tests, ECG tests, heart tests, chest x-rays, liver scans and ultrasounds.

Mr Charles also had an MRI, cat scan and psychiatric testing.

"At that stage didn't know whether I would be a match or not but I decided early on I'd like to be a live donor.

"With organ donation rates being so bad in New Zealand there was a possibility he may never have the opportunity to have a deceased liver donor. And it just makes sense – I have got something he needs."

Mr Charles received the news he was a match last week. The first thing that came to mind was relief, rather than any fear for himself.

"I'm mainly nervous about Louie. I don't think when you have kids you give too much thought about yourself. I'm also concerned about Nic and Kees.

"I'm sure Louie is not going to look in too good shape when he comes out of surgery, and the fact I'm not going to be there to support Nic is a big worry for me.

"Sure I've got to have surgery but Nic's got a lot to contend with looking after three people."

Mrs Charles said she was happy at first but the reality of having both her husband and her child in the operating room soon hit.

"Sometimes I'm quite strong, and I say it's fine and other times, I have my moments. It's always in the back of my mind.

"But I know everything's going to be fine. Our mums will both be there and we've got a good support network so that helps ease my mind a bit.

"And you have to be strong – we've still got Kees to look after and I can't be different around him."

She said the staff at Nelson Hospital including paediatrician Nick Baker were providing excellent support and care.

Mrs Charles said the other worry was how the twins would cope without each other.

"The first thing they do when they wake up is look at each other. When Louie is not there Kees wakes up all grizzly."

Mr Charles said he just wanted to try to raise awareness about organ donors.

"I just hope at least one person reads this and changes their mind about being a donor or talking to their family about their wishes. If they do, they can save lives – one donor can save up to 10 people."

WHAT IS THE DISORDER?

Propionic Acidemia (PA) is a rare genetic disorder where the body cannot break down certain amino acids – parts of protein – from foods eaten because of a non-functioning enzyme called PCC.

This causes a buildup of dangerous acids and toxins, which can cause damage to the person's organs, such as the brain, liver and heart, cause seizures, and delays to normal development like walking and talking.

Episodes of illness can be triggered by illness, stressful events, eating large amounts of protein, or going too long without food.

PA is inherited from both parents. They rarely have the disorder themselves, but are "carriers" of a single non-working gene for PA.

By Sarah Young - The Nelson Mail