Fair care? Years of anxiety as doctors struggle to diagnose disorders
Advocate says EDS sufferers face discrimination in the health system because of a lack of funding and understanding of condition more common than autism.
New Zealanders with a condition more common than autism are regularly discriminated against by the country’s health system, an advocate says.
And it’s because of a lack of funding and awareness of Ehlers-Danlos syndrome, the Ehlers-Danlos Advocacy Organisation lead coordinator Tracey Jourdain said.
Ehlers-Danlos syndrome (EDS) is a hereditary, overlapping group of connective tissue disorders that leads to widespread and seemingly unrelated symptoms.
It can take years for sufferers to be correctly diagnosed because it is not widely understood and even with a diagnosis many are disbelieved and misdiagnosed with a mental illness, despite early intervention being key to avoiding injuries.
The Herald has this week highlighted four cases where women diagnosed with EDS were labelled with either factitious disorder or traces of personality disorders and, they said, effectively left with minimal or no care for their lifethreatening illnesses.
There is worldwide momentum to increase awareness of factitious disorder in patients to stop them causing harm to themselves by undergoing myriad tests, and to save time and money on wasted procedures.
There are about 1800 known EDS sufferers in New Zealand, although it’s thought that figure could be higher, ranging from minor hypermobility to major life-threatening conditions such as spontaneous bleeding and severe bowel dysfunction.
“Refusing to believe a disabled person is in pain, or is suffering dysfunction and/or accusing them of mental illness because their pain and dysfunction is caused by disability rather than disease is discriminatory,” Jourdain said. “Similarly, failing to provide treatments when we have greater clinical need and benefit because we are in the minority is also discrimination.”
She wants national clinical guidelines and a specialised hypermobility hub to keep pace with other developed countries and said EDS patients were being unnecessarily injured because of the lack of knowledge.
“ACC tends to discriminate by saying EDS caused the injuries, which is not true, it only makes us more prone to injury and slower to heal.”
Jourdain, who has EDS, said it was on the back of this destructive pathway that she and husband Nigel Jourdain wrote to previous Health Minister Dr Jonathan Coleman two
years ago about EDS treatment protocols.
Coleman initially told Jourdain’s husband in a letter dated October 13, 2016, that people living with one of the 13 types of EDS can receive publicly funded care.
He also said the Ministry of Health and district health boards did not usually endorse guidelines for managing specific diseases, except for conditions that had a major public health impact such as diabetes.
However, in another letter Coleman said it was inappropriate as Health Minister for him to direct clinicians or DHBs to offer particular treatments to individual patients, and that DHBs were responsible for deter- mining the health needs of their populations.
And later he wrote: “As DHBs have to prioritise their spending, some services may not be provided free.”
In response, Jourdain pointed out EDS sufferers were considered the minority.
“When a person with diabetes and a person with EDS have a need for physiotherapy, if the EDS patient has a greater need and benefit, they would still be denied in favour of the diabetes patient even if that particular patient has a lower need and benefit because there is not funding for EDS as it is not a ‘high impact’ condition.
“I’m glad diabetes patients get good
Half the time they’re letting us go over the cliff and they’re refusing to send the ambulance to the bottom. Tracey Jourdain, Ehlers-Danlos Advocacy Organisation lead co-ordinator
support, I’m just asking that we get the same level of support.”
Jourdain said not spending enough money to manage EDS patients was akin to “putting the ambulance at the bottom of the cliff”.
“But half the time they’re letting us go over the cliff and they’re refusing to send the ambulance to the bottom.
“They just let us suffer terribly or even die at the bottom. After witnessing this distressing situation over and over again, that’s what I believe is happening.”
Three of the four women featured in the Herald special investigation were at one point denied a liquid food known as total parenteral nutrition (TPN) either for years or indefinitely after they attracted a mental health diagnosis.
At between $250 and $500 per bag, one patient using TPN fulltime — seven bags a week — could cost up to $182,000 per year, or as much as $546,000 for the three patients, not to mention surgical insertion of feeding lines and any complications that require hospital treatment.
Forensic psychiatrist Dr Caleb Armstrong, who did not believe EDS patient Stephanie Aston had factitious disorder, said the motivation for monetary savings would be out of kilter with what was at stake.
“It’s her life at stake. I just don’t understand what’s in it for them other than some people don’t like being wrong. It’s not their money.”
Jourdain said while she had not been labelled with a mental disorder, a medical doctor suggested it and included it in her file.
“I decided to get a full psychological evaluation and this proved I was psychologically healthy. I felt I had to do this because a doctor hinted at psychological causes, even though it was outside of his scope.”
The New Zealand Organisation for Rare Disorders (NZORD), of which EDS is one, lists the main challenges for patients as:
Getting a diagnosis, being misdiagnosed, being told it’s all in their head, being disbelieved, and getting doctors to listen.
Doctors and specialists not having the knowledge to treat EDS.
Pain and fatigue and a lack of understanding in the wider community.
NZORD chief executive Dr Collette Bromhead said it was a common experience for people with rare disorders to be diagnosed as “hypervigilant” or with some form of mental illness causing them to misconstrue the situation.
“When doctors can’t figure out what’s going on, this is you don’t fit a usual pattern, then they can jump to that resort that there’s something mental health going on.”
She cited one case in Wellington where a child died earlier this year from an extremely rare disorder but during the first doctor’s visit he was diagnosed as being hypersensitive and his mother as being overly worried.
Bromhead said she would be concerned if there was a pattern of factitious disorder diagnoses in EDS patients and suggested further training by doctors in rare disorders.
She said NZORD hoped to produce universal clinical guidelines on EDS early next year.
New Zealand’s only EDS specialist, rheumatologist Dr Fraser Burling, said he was very concerned that four of his patients had been diagnosed with or suggested as having a psychiatric disorder, as he believed it was harming their prospects of necessary treatment.
“As soon as people get this label then the hospitals have withdrawn care from them, and this is where it’s dangerous.
“Not only that but people like Steph, we’ve tried to get her care in private but because this label’s gone with her, they’re washing their hands of her and won’t treat her.”
Ehlers-Danlos Society international executive director Lara Bloom claimed similar situations existed internationally, where EDS patients had been misdiagnosed with a psychological disorder.
“Unfortunately, it is something that we do see in our community, usually down to the years people have spent seeking a [medical] diagnosis.”
A spokesman for Minister of Health David Clark said the matter was not one for him to comment on.
A spokesman for the Ministry of Health said complaints about health services should go to the Health and Disability Commissioner’s office, which had already considered these cases.