Fading teen desperate for surgery
She searches her phone for the list of favourite foods she can’t wait to enjoy again.
Maia de Wit is 17, so naturally pizza rates highly. Talking about toppings is one of the few times she tries for a smile.
She’s not quite sure what her favourites are any more. There are many flavours her taste buds have simply forgotten.
The Ashhurst teenager has not eaten a meal since 2022.
She is sustained by a feeding tube. The occasional spoonful of jelly is purely functional. It puts her abdominal muscles to work, and then she is overcome by pain, nausea and vomiting.
It has been this way since she was 14. Maia suffers from hypermobile Ehlers-Danlos syndrome (EDS), a genetic connective tissue disorder that affects collagen production in the body. It comes with myriad symptoms including joint pain as well as problems with digestion and internal organs.
This has been compounded by a number of rare compressions of veins or arteries, contributing to her pain and gastrointestinal symptoms.
It has left her merely existing – with little to no energy, out of school, and short on hope.
What she has left of the latter is pinned to life-changing surgery in Düsseldorf, Germany, performed by vascular surgeon Wilhelm Sandmann.
But it comes at a huge expense, and the one avenue – a high-cost treatment pool for one-off treatments overseas not funded by New Zealand’s public health system – paved by Health New Zealand Te Whatu Ora has been closed to her.
A review of vascular compression surgeries in December determined the procedures were experimental and therefore no longer eligible for funding.
Six-figure targets
Maia’s illnesses are rare, but her situation is hardly unique.
Six months won’t go by without a New Zealand news agency reporting on a young person with the same afflictions, which flare up at puberty and are either misdiagnosed or misunderstood by New Zealand doctors.
Instead of treatment plans, the patients have Givealittle fundraising pages with six-figure targets.
There is Olivia Adie in Dunedin, and Taylor Doyle and Oscar Parker in Canterbury.
Sarah Wills, in Palmerston North, fundraised her way to Germany in 2022, while Waikato teen Jemima Thompson is believed to be the only person suffering from vascular compressions to successfully access the high-cost treatment pool.
However, she was already in Germany awaiting surgery, thanks to fundraising and the family mortgage, by the time it was approved. Her sister had already died from dehydration after experiencing similar symptoms.
Both Sarah and Jemima’s surgeries were successful, allowing them to eat and drink again without pain, but Te Whatu Ora wouldn’t acknowledge this had treated their conditions.
In its December ruling that surgical management of vascular compression syndromes was experimental, the health agency’s northern clinical practice committee cited a lack of data, clinical trials and published studies.
It made a number of recommendations for how such surgeries should be carried out, though nobody in New Zealand performs them.
And there was no guidance for patients, such as Maia, on how else their illness could be treated.
‘It’s like there’s a brick wall’
It’s this lack of an alternative that has left Maia and her mother, Rebecca Lester, devastated and desperate, and tasked with raising $170,000.
“It’s like there’s a brick wall and they haven’t provided us with a ladder,” Maia said.
“Nutrition shouldn’t be conditional. It’s a right, not a privilege, but they act as though it is, and it ends up killing people.
“No nutrition and dehydration can kill you.
‘‘There are people who have died from it in the past year with these conditions, and you would think that would be enough to make a change, but it doesn’t change anything.”
Te Whatu Ora did not respond to the Manawatū Standard’s questions about what remedial treatment options were available to patients with EDS and vascular compressions, or whether it accepted Maia’s claim that the only support offered to her at Palmerston North Hospital was psychological services and physiotherapy.
In previous news coverage of similar cases, similar questions were unanswered but with an acknowledgement that very rare conditions might require treatment overseas and that the high-cost treatment pool managed this.
Maia said her general practitioner, Tregon Singh, had been incredible, “a champion” who advocated for her and never doubted her.
“Every time we were turned away from the hospital, he would make another referral for us.”
But each time she has been to the emergency department suffering dehydration and rapid weight loss, she has been met with scepticism and stubbornness.
“Is this the girl who starved herself?” is how one health professional introduced themselves, while an ED doctor encouraged her to practise “mindfulness” in the belief that her severe abdominal pain was caused by anxiety.
Lester said her daughter was in acidosis – when there is too much acid in the blood – and suffering starvation because of severe dehydration.
“The doctor said, ‘You know, the body does strange things when you’re stressed,’ and sent us home.”
Maia had tried to keep up with classes at Palmerston North Girls’ High School, but her attendance waned as her condition got worse in early 2023 and she was forced to drop out. “It will take longer than most,” she said. “I’m already having to repeat a year – I just hope I can achieve it this year.
“But I also can’t push myself. I don’t have the energy to push myself.”
It’s the simple, normal things she missed most. Sleepovers, swimming, bike rides, eating snacks – being a teenager.
Social media was a lifeline to the outside world, but it was a double-edged sword – a window to the life experiences passing her by. “It is very hard. It’s not to say I don’t want [friends] living their lives, because I’m very happy for them, but I wish it was me too.”
Lester was a social worker. She said it was extremely difficult to be in that role helping others but knowing there was nothing she could do to help her own child.
“I’m probably a bit of a wreck on the inside, but when you’re a single parent there’s no option to fall apart ... [when] trying to juggle three kids, a job and a full-time sick child. I’m not going to drop any of those balls.”
She said they were grateful to not be among the first families dealing with the illness, and those who were had provided “a bit of a road map” and a lot of support.
Call for rare disorders strategy
Chris Higgins, the chief executive of Rare Disorders New Zealand, said there was very poor awareness and understanding of rare disorders among health professionals in New Zealand.
“We often hear anecdotally of patients being told it is all in their head, which can be quite traumatising for patients who are desperately seeking help and answers.”
He said having health professionals who were willing to walk alongside their patient to get answers made all the difference for a patient’s mental wellbeing and for the likelihood of reaching a desired health outcome.
Rare Disorders NZ is calling on the Government to develop a centre of expertise for rare and undiagnosed disorders to identify gaps in the delivery of service and support in the health system.
“For those without a definitive diagnosis such as Maia, the centre would be available as a referral centre to any health or other professional with clients or patients presenting with unusual co-presenting symptoms, with or without a suspected diagnosis.”
Higgins said it was the group’s hope that a rare disorders strategy may in time inform what funding pools could be available for overseas surgical options.
Focused on the future
A hot rod and car display was held at Tokomaru School last night to raise money for Maia’s surgery in Germany.
Her Givealitte page has so far raised $5900 and she is chronicling her battle on a Facebook page called Maia’s Body’s Broken.
The family has also made a pact: no dinners out until Maia is well enough to join them and enjoy the meal. “She has a deal with my youngest,” Lester said, “that once she comes back [from Germany] they’re going to get a big pizza, and we’re all just going to eat this massive pizza.”