How to mend a broken heart

Chase Porter needs a heart transplant, but doesn’t qualify for one here and the health service won’t pay for his life-saving operation overseas. Jo Lines-MacKenzie talks to a family forced to take it day by day.

His smile is wide and his giggles loud. As Chase Porter rides his electric balance bike in his family’s driveway what isn’t immediately obvious is that this 5-year-old needs a new heart.

The Hamilton boy has been diagnosed with the rare Barth Syndrome and is living with heart failure – which requires a heart transplant.

He’s been denied funding to go overseas for one – so his family are living day-by-day until he’s able to go on the transplant list.

Two weeks after Chase was born in 2017, mum Kelsi Porter noticed the first signs something wasn’t right.

‘‘I fell asleep on the couch. And Andy [Chase’s father] woke me up and said, ‘Chase is making funny noises’, and I was like, leave it, babies make funny noises. And he’s like, ‘no, I just have a bad feeling’.’’

It resulted in a trip to Waikato Hospital’s emergency department. Porter, a former pharmaceutical rep, said she knew it was serious when she saw them intubating Chase.

‘‘They sent a chaplain and that’s when I was like, things are really bad... Three hours later a doctor said there’s something wrong with your baby’s heart, but we don’t know what it is.’’

Transferred to Starship, a cardiologist said Chase has dilated cardiomyopathy and left ventricle non-compaction.

But Porter had a nagging feeling there had to be more to Chase’s illness.

She learnt her grandmother had two sons that died as babies in the 1920s. Some digging in death records found it was from Barth Syndrome, so she asked doctors for Chase to be tested.

‘‘They [said] no, no-one has it. It’s so rare that it won’t be that.’’

Not one to give up, Porter found a simple urine test could diagnose him. Chase’s paediatrician was surprised to report it returned a positive result.

‘‘He can’t convert energy to make muscles. It means that he can’t make white blood cells to fight infections.’’

Aged two Chase suffered a respiratory infection.His heart couldn’t pump the fluid around his body, which damaged his mitral valve.

Porter was told Chase’s ventricle was already weak from cardiomyopathy; they wouldn’t be able to get him off bypass if they attempted a surgery.

Chase has only 28% heart function and is living with heart failure, meaning he takes five different cardiac medications.

‘‘So the only thing they could do was a heart transplant.’’

This was to be done in Australia, because New Zealand doesn’t do organ donations in children.

But Chase was denied funding through the Ministry of Health’s high-cost treatment fund, for oneoff treatments not otherwise funded by the public health system.

Treatment that may be considered includes medical treatment that is only available outside New Zealand or treatment that is only available outside the public health system.

‘‘So we’ve just kind of been winging it. Starship keeps a close eye on Chase’s heart. We go up there for echoes really frequently and heart clinic days. In 2019, we had 16 hospital admissions in April. And that’s admissions, not just the visits.’’

Chase is being cared for under Rainbow Place, New Zealand’s only specialist children’s and young person’s hospice service which he was discharged to from

‘‘He’s actually the only boy with Barth Syndrome that can ride a pedal bike... I think it’s because we’ve encouraged him to live his life.’’

Kelsi Porter

hospital, with specialists saying there was nothing more they could do for him.

Porter says she was told ‘‘to just go home, and enjoy him. So he’s kind of like a little boy who just keeps on living’’.

There are no visible signs that Chase is sick, Porter said you can see it when he’s around other children because he doesn’t have the energy they do.

‘‘He makes lots of noise. But when they go to jump or run, he kind of waddles, he’s just like a little old man.

‘‘But his personality definitely makes up for the strength he doesn’t have.’’

The main priority is keeping Chase away from bugs including Covid-19, respiratory and stomach viruses.

The plan is, when Chase is big enough for an adult heart transplant, to get him on the inactive transplant list.

‘‘They also wouldn’t transplant until he was sick, because transplant hearts only last about 20 years, so they want to get the most out of Chase’s heart.’’

Apart from attempting to keep him safe from bugs Porter said they make sure Chase leads a regular life. Porter admits for the time being they’re getting by on a lot of luck.

‘‘He’s actually the only boy with Barth Syndrome that can ride a pedal bike.

‘‘He can’t ride it far because he gets tired. But I think it’s because we’ve encouraged him to live his life.

‘‘The doctors have always said to us just take each day as it comes, right from the start. Don’t look ahead and don’t look back.’’

A Givealittle page has been started to help the family with treatment costs and when it comes time for Chase to have a transplant.

The Ministry of Health said since 2001 there have been seven approved applications to the High-Cost Treatment Pool for paediatric heart transplants.

Because of the low number of paediatric donors, paediatric heart transplants are rare.