Kiwis suffering from a "very misunderstood" and debilitating condition are crying out for change after another person was forced to travel overseas to get help.
Taylor Doyle has vascular compressions, which is a rare condition that causes excruciating pain during eating and drinking.
She had medical professionals here dismiss her and the horrific pain she was in, so she travelled overseas twice this year for surgery.
But she isn't even the first New Zealander this year who's travelled to the specialist in Germany for the expensive surgery, prompting calls from her family for better care in New Zealand.
The Ministry of Health admits there is a need for a stronger system response for rare disorders in New Zealand - so what do Kiwis with the rare condition say?
'Not as rare as previously thought'
Taylor, 14, has suffered from vascular compressions and chronic illnesses her whole life.
Mid-last year, the Canterbury teen had a sudden onset of extreme upper abdominal pain and nausea and found out she had median arcuate ligament syndrome (MALS). Because eating and drinking would cause her severe pain, she became malnourished and dehydrated.
While she could receive care for her conditions in New Zealand, surgery is the only cure and it isn't available in New Zealand. So she and her family started looking internationally to find an experienced and knowledgeable specialist.
Following extensive research, she made a trip to Sydney in March this year to treat her MALS and flew to Germany in May to get surgery for her other compressions. The surgery was a huge success but her journey isn't over yet.
And the lack of help available in New Zealand is a big concern for her mother, Zalie Doyle, who thinks the condition might be more of an issue than most think.
"One thing we've discovered along the way from people we've met is I don't think they are as rare as people previously thought," Zalie told Newshub.
"Twenty years ago, hardly anyone was a celiac and hardly anyone had a gluten allergy. But now we know it's far more prevalent and that's purely because there's been time and effort and research that's gone into it. I think it's the same with vascular compression syndromes, and more than that, the underlying connective tissue disorder Ehlers-Danlos syndrome."
Along with MALS, Taylor has high-grade May-Thurner syndrome, severe pelvic congestion syndrome, nutcracker syndrome, severe superior Mesenteric artery syndrome, nephroptosis, and severe visceroptosis in her liver and kidneys. She also has hypermobile Ehlers-Danlos syndrome which is believed to be the underlying condition causing her problems.
Taylor spent an estimated 150 nights in hospital last year alone. However, nothing doctors tried made a difference to her pain and nausea, nor her tolerance for eating and drinking.
Taylor is by no means the first person in New Zealand to travel to Germany for this specialist procedure. Earlier this year, Newshub spoke to Sarah Wills who spent nearly a decade trying to get a diagnosis and help for Ehlers-Danlos syndrome, MALS, and other compressions, and later travelled to Europe at great cost for surgery.
At the time, Wills believed people who had compressions were "everywhere" and it wasn't "as rare as people think it is". She said patients with the condition are "ignored" and "left to die or suffer".
Taylor and Zalie also spoke of other Kiwis who have made the trip to Germany and said they've received messages from other New Zealanders considering overseas surgery.
Before her surgeries, Taylor spent countless nights in New Zealand hospitals receiving care but felt she wasn't listened to.
"When I was admitted to hospital, every second morning they'd come in and talk to me… and every time I'd tell them the same thing over and over and over and it felt like they weren't listening," Taylor told Newshub.
"They wouldn't say these exact words, but it felt like they said, 'That's nice', and move on."
For Zalie, seeing her daughter's pain not being taken seriously was difficult.
"Taylor used to say things to me like, 'What's the point in even telling them how I'm feeling because they don't listen to me anyway'.
"They would ask her how she's feeling and she'd say, 'I feel like shit, nothing's improved', and then that was it, they'd just go 'Okay, keep trying'."
On one occasion after Taylor had a nasogastric (NG) tube inserted, she told doctors she was in severe pain because the formula was being pumped into her stomach. Her doctor told Zalie it would take her four to five days for her stomach to get used to the feedings, but after 23 long days of trying, Taylor told her mum she'd "rather be dead" than in pain.
Another time, Taylor believed she had an NG tube explode in her stomach, but doctors told her that wasn't possible. She had an X-ray and, sure enough, it had exploded and half of the tube had to be surgically removed.
"So many times, Taylor would tell them something was happening and they would dismiss her but she would be right," Zalie said.
That same dismissal happened when Zalie suggested to doctors her daughter may have vascular compression syndromes.
"I said, 'Look, I've come across this condition called MALS. I know it's rare, but is it possible', because as far as I could see, it lined up. And he just said to me that it's too rare," she said.
"Just because it's rare doesn't mean it's not real. Rare is real and they do have it. She had about six vascular compression syndromes. Even the report from the surgeon in Sydney after her MALS surgery, our health system still said MALS wasn't causing her discomfort. Even with images of her insides, even with beneficial relief from the surgery, they still denied it was possible."
Zalie said she thinks New Zealand doesn't have the right tools to treat rare conditions because it's such a small country.
"It doesn't mean you can't get it, it just means you have to find the right people, and sometimes that means going overseas and looking internationally and sometimes that means finding someone that's prepared to look outside the box," she said.
"We've been lucky. We've come home with confirmation of a diagnosis… We've got some pretty hefty knowledge behind us and we've been lucky to come back to specialists who are accepting and acknowledging of Taylor's diagnosis and the treatment that she's had, but that certainly hasn't always been the case."
The $120,000 price tag for surgery
For Zalie and Taylor, finding international specialists wasn't the hard part, nor was it difficult setting up treatment. What was challenging was funding the surgery and all the associated costs.
All up, travelling to Germany for the procedure cost about $120,000, including the $85,000 surgery. There was also an additional scan to pay for, as well as a month of accommodation and flights.
Te Whatu Ora and the Ministry of Health respond
A spokesperson for Te Whatu Ora told Newshub they are saddened to hear about Taylor's situation.
They said while there are a range of vascular services available across New Zealand, they recognise some patients choose to go overseas for further treatment.
There is funding available for New Zealanders requiring "high-cost treatment", which is available from the High Cost Treatment Pool set aside by the Ministry of Health for one-off 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 currently available outside the public health system, for example in private hospitals," they said. "Only people who are New Zealand citizens or permanent residents may be considered for treatment funded under the High Cost Treatment Pool."
Applications to the High Cost Treatment Pool are only accepted from regional specialists and applications are assessed by a panel of Te Whatu Ora chief medical officers from across the country.
Other eligibility criteria include the treatment being well-established and not experimental, it would lead to reasonable prospects of survival and improved quality of life, and failure to receive the treatment would result in serious irreversible deterioration in the patient's condition.
"There are certain eligibility criteria that are used as a guide for assessing applications, however each case is also considered for exceptional circumstances beyond that criteria," the spokesperson added.
In response to this, Zalie said they weren't told about or offered any support in terms of funding for Taylor's treatment
"We specifically asked for them to provide us with a treatment plan and options for the conditions she had and their response was that she only requires treatment in the form of 'chronic pain management', but then advised there was not a chronic pain team for paediatrics in Christchurch," Zalie said.
It was only after Taylor returned from Germany and doctors in New Zealand were provided with the German surgical report and imaging that they "accepted" Taylor's diagnosis, Zalie said.
However, the Ministry of Health has acknowledged New Zealand needs a more robust response for people who have rare conditions.
"Manatū Hauora is aware there is a need for a stronger system response to rare disorders in Aotearoa, and so we are working on the development of a Rare Disorders Strategy," a spokesperson said.
"This strategy will lead to better, more timely services, and more equitable support and outcomes."
Minister of Health Andrew Little was unavailable to answer questions directly related to Taylor's situation.
But earlier this year, following the Pharmac review in which it was recommended rare disorders and services to treat them be reassessed, he said he had directed the Ministry of Health to lead development of a strategy for this.
"The Government agrees with the independent review panel that Pharmac must put much more emphasis on equity across communities, including for those who have rare diseases and their families," he said at the time.
"The [rare disorders] strategy will include Pharmac, but has a broader remit to encompass the entire health sector. It will require Pharmac to collaborate with the Ministry of Health and the new health entities Health New Zealand and Māori Health Authority."
Little added Pharmac assured him it was already working to include the perspectives and experiences of people with rare disorders in their work and on how it provides information to the public, including people with rare disorders and disabilities and their families.
