Vyron Smith sacrificed his promising rugby career to save his father's life, but now his own life is in danger.
Just a year after he donated his left kidney to his father – former high school teacher Evan who had end-stage kidney disease – Smith started to pass blood before collapsing in pain.
He was flown to Waikato hospital from his Bay of Plenty home on the "verge of death".
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"When I saw blood in my urine I was scared, because I knew something was really wrong," he said.
"I had pain in my lower back on my right side – where my remaining kidney was – so at first I thought I had an infection and just needed antibiotics, but it turned out I needed much more."
Hospital doctors battled to save his life as his condition rapidly deteriorated.
His remaining kidney was failing, and his blood count was dangerously low.
"My blood platelet count was so low I was barely alive," Smith said.
"My skin turned yellow, and I couldn't move.
"It got so bad that I was really close to needing a kidney transplant myself.
"I had to have constant blood plasma infusions and be on a drip for ten days. I was feeling so afraid."
Smith's initial thought was that it must be something to do with the kidney transplant, but doctors discovered that it was unrelated.
"Even though I was in a serious condition, that came as good news to me," he said.
"Dad had been tormenting himself, worrying that it was somehow his fault, so it was a relief to everyone that it had nothing to do with the transplant."
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Smith's diagnosis, however, was not good news.
Tests revealed that he had developed an extremely rare, life-threatening and progressive autoimmune disease called Atypical Hemolytic Uremic Syndrome or aHUS.
The disease, which only affects a handful of people per million, impacts the blood system and kidneys.
This can lead to reduced or lost function of the kidneys and other organs, low blood counts (low platelets and anaemia), tiredness and difficulty functioning.
It may also damage the heart, brain, lungs and other systems.
Without treatment, it can cause blood clots, stroke, kidney failure and death.
"The doctors told me I would need a plasma transfusion every two weeks," Smith said.
"There are drugs which could treat the disease without the need of ongoing transfusions, but they are not funded in Aotearoa and would cost $670,000 a year to buy them from the United States."
That drug eculizumab is marketed under the brand name Soliris.
It has been approved in New Zealand for limited other conditions, but it's not publicly funded for aHUS.
Rare Disorders NZ chief executive Michelle Arrowsmith told Stuff the government is failing New Zealanders like Smith who develop rare illnesses.
"New Zealand continues to sit at the bottom of the OECD when it comes to modern medicine access," she said.
"The current Pharmac model disproportionately fails the rare disorder community in New Zealand, due to its excessive focus on cost-saving by bulk-buying high demand medicines, and failing to enable alternative pathways for low volume treatments.
"While some rare disorder medicines may be expensive, the costs of not funding these treatments tend to be much higher."
A recent survey conducted by Rare Disorders NZ found that while 85 per cent of people in New Zealand with rare conditions took medication for their disorder, most of the publicly-funded medicines being used were to relieve pain and inflammation due to the lack of accessible, effective modern medicines.
Pharmac chief executive Sarah Fitt said they had not received an application to fund eculizumab for people with atypical haemolytic uremic syndrome, but this was a therapeutic area which it is monitoring following feedback from the sector.
"We are aware that there are a number of medicines available internationally which could be used to treat this condition," Fitt said.
"And we will be seeking advice from our Haematology Specialist Advisory Committee soon to gain a better understanding of the health needs and therapeutic options available for people with this condition.
"Depending on the advice that we receive, Pharmac may request that the relevant pharmaceutical suppliers submit a proposal with supporting evidence for funding consideration."
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After leaving hospital, Smith moved from his Tauranga home to be with his older brother Jermaine in Melbourne, and even dyed his hair bright orange and gold to raise awareness about donating plasma.
"People know about donating blood," Smith said.
"But plasma is really needed too, and they don't have enough.
"There's a shortage in New Zealand"
Smith is applying to see if he will be eligible to get the drugs he needs in Australia, where they are subsidised by Medicare.
Paying himself is not an option – he had had to give up his rugby career after the earlier kidney transplant as no contact sport is allowed.
Before the transplant he'd had a stint playing for Melbourne Rebels before moving to Sydney to play in the Shute Shield – a semi-professional club competition – with the aim of eventually making it with the New South Wales Waratahs.
"It was an easy choice to make – giving my dad 20 more years of life, or rugby," Smith said.
"I don't regret it ever, even though it was tough going through it.
"Now it is just unbelievable that I have this disease, and some freak coincidence it is in my kidney."
Before his diagnosis, Smith was a personal trainer, and helped his dad learn how to eat healthier.
Three years ago he also spent time in Australia with Jermaine, who at that time was seriously obese and had three heart attacks at only 36 years old.
Smith helped his brother lose more than 100kg.
"He not only saved dad's life, but mine too," Jermaine said.
"He is so focussed on hauora (health). It was heartbreaking for the family to see him in a hospital bed on the edge of death and despair."
"Speaking to him online was emotional, seeing his health deteriorate and his mana barely intact. It was hard to witness.
"Even though he was going through all that he was asking about dad and asking how my health and fitness are going."
Jermaine has set up a crowdfunding page to raise funds for the treatment and his brother's loss of income.
Although it has already raised $20,000, it will not be enough for the medication Smith desperately needs.
There are also links to encourage people to donate blood or plasma in Aotearoa and Australia.
Despite the challenges ahead of him, Smith remains determined to battle the disease and get the medication that could save him.
"I really need those drugs long term, as that will help me live a normal-ish life," he said.
"The medication has only been around for four years and was only just funded in Australia, so I am trying to stay optimistic that I can get it in Australia and that it could become subsidised in New Zealand in the future.
"But there are no guarantees."
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For the time being, although he is stable, without the drugs he won't be able to do high intensity exercise, running or surfing.
"I love venturing, exploring and just being outdoors," he said.
"Some days I am still too sick to even walk, but I am trying to manage it, and hope I can get the medication."
This originally appeared on Stuff and has been reproduced with permission.
