Oliver Floodsmith-Ryan's joints have been dislocated six times in two days.





Oliver Floodsmith-Ryan dislocated his shoulder while reading a book on Christmas Day.

DAVID UNWIN/FAIRFAX NZ Tracey Jourdain says she lives in constant pain, but it has become an everyday feeling.

It wasn't the first time. A month earlier, it had come loose while he was playing cricket. Another time, he was out to dinner when he felt as if his jaw was falling out of place.

The 18-year-old who was once an aspiring sportsman has Ehlers-Danlos syndrome.

The condition is genetic, and could affect about one in every 5000 people in New Zealand. But it is often misdiagnosed — if at all — and district health boards have no protocol in place to treat patients with the syndrome.

DAVID ROWLAND Rachel Palmer is unable to walk and suffers severe pain.

The syndrome causes abnormal collagen synthesis, which means that although the body produces it, the collagen is weaker and causes the joints and its connective tissue to become loose.

Auckland-based Dr Fraser Burling, an expert in chronic soft tissue injuries, said he was currently treating about 40 patients with the syndrome.

Burling said one of the most common signs of Ehlers-Danlos was hyper mobility - great flexibility - and stretchy skin, and some patients may bleed easily.

If a patient's joints were treated roughly, it could cause them to become dislocated or cause an extreme amount of pain.

"Some physiotheraphy can injure patients. I know of patients injured from physio therapy," said Burling.

Like other Ehlers-Danlos patients, Floodsmith-Ryan said he had been injured before and is in constant pain but tries to go for the odd jog or walk.

"I've had to give it up basically," he said. "It's painful, I'm left quivering on the ground."

Now, he's lost a lot of muscle around his shoulder. He tries not to use it too often because of the pain. But his condition is also hard for his friends to understand, and he has to opt out when they play sport. He's has no visual symptoms, or casts or bandages.

"They don't get it in the slightest, they might take the piss sometimes."

Floodsmith-Ryan said it also affected his work and he had to give up his landscaping job. Although, now he's been able to start part-time at a local cafe — washing dishes.

His mother, Kerry Ryan, said they have struggled to find the right care, if any, for her son.

"If we were in America, or Australia we would have been under a team of specialists," she said.

"We were told by the shoulder specialist there was nothing he could do. It's not like cancer, when you go to see an oncologist."

Although her son has a GP who understands this condition, there was simply no provision in New Zealand's health system for caring for people with Ehlers-Danlos, said Ryan.

And, she said, when they try to bring the condition up with other doctors, she said their knowledge, or lack of, was concerning.

"I had to do my own research. I feel like I know more than they do.

"You get a diagnosis and then you're on your own. I think in New Zealand, it shouldn't be like that."

Floodsmith-Ryan is not the only person in this country to live with the mystery illness that carries with it a world of constant pain.

Palmerston-North woman Tracey Jourdain was born with her feet pointing in the wrong direction. It was the first sign of many that has been overlooked.

She has suffered from Ehler-Danlos her entire life, having had surgery more than 20 times.

Her jaw joints have been replaced, her shoulder joints too. The pain is only bearable because it's become an "everyday feeling".

A qualified electrician, Jourdain said she thought the pain was normal.

"I thought all the pain that I was in was because I was a woman in the trade. I convinced myself it was all my fault."

But that was never the case. She was suffering immense pain in her nerves, joints and just about everywhere else. Even her earlobes throb in pain.

Treatment should be easy, it should be cost-effective, and it could help up to 2000 people countrywide. But, because of her condition, ACC refuses to help when she and other Ehler-Danlos sufferers are injured.

After a car crash in 2014, Jourdain said she was refused help by ACC, and DHBs have been largely unhelpful.

She said part of the problem is if they go untreated, their conditions get worse and more complicated. Another problem was having people understand their conditions.

"The biggest thing is that family, friends don't believe you. They don't believe you and think of you being a psychological case. I can no longer garden, no longer clean the house as much as I could I can no longer cycle," Jourdain said.

"It would save a lot of suffering and a lot of costs for the DHBs if they treated it."

Gabrielle Scott, executive director at Allied Health said there were no "established care pathways or protocols in New Zealand DHBs" to treat Ehlers-Danlos, and it was primarily self-managed.

"In general patients would be cared for in the community under the care of their general practitioner," she said.

"For patients referred to MidCentral DHB, we are focused on patient-centred care - that is ensuring that the care provided is specific to the individual and their needs."

Scott said a GP would co-ordinate the patient's care, and would seek specialist services as required.

"These services would typically include occupational therapy, physiotherapy, psychologist support and pain management."

Treatment could include symptom management, muscle strengthening exercises and education to self-mange their condition.

"Muscle strengthening usually focuses initially on trunk core strengthening and progresses to include strengthening muscles around the other affected joints."

But Burling, who is currently treating up to 40 patients with Ehlers-Danlos said New Zealand was lagging behind in implementing a protocol for patients.

"A lot is just not known about it. Part of it needs to be general education because EDS is much more common that we first thought," said Burling.

"Certainly, a protocol needs to be set up."

Burling said a person with Ehlers-Danlos will always have the syndrome.

"You can't change genetics but you can treat the various aspects of the complications of EDS. The more we are diagnosing people, the more we can come up with treatments. And as more diagnoses become common, more information about it will become available."

Burling said a protocol for caring for people with Ehlers-Danlos should include gentler physiotherapy.

"Patients take longer to heal so they need a specialised approach to improve and injuries are more common.

"Ehlers-Danlos is not the cause of the injuries, but it makes it harder for people to heal if they are injured."

But the pain caused by the hard-to-heal injuries has become too much for Jourdain, and the others. Jourdain has appealed for help from MPs, the Ministry of Health and DHBs. But she said her pleas have gone largely unheard.

As a result, Jourdain received a letter from Minister of Health Jonathan Coleman, which stated that she and other Ehler-Danlos sufferers would be able to receive the care they needed under the health system.

But, the letter also stated: "The Ministry of Health and district health boards do no usually endorse classification or diagnostic criteria ... except for conditions that have a major health impact, such as diabetes."

But, as all Ehler-Danlos sufferers know, the condition has a major impact on their health and in many cases it gets so bad they're unable to walk, or are completely bedridden.

Dr Rachael Palmer suffered from stomachs, fainting and clumsiness. She never thought much about it.

When she turned 11, her legs became bandy and she struggled to walk up stairs. But doctors could find nothing wrong with her.

As a teenager, she developed endometriosis and started developing stress fractures in her bones.

"I ended up with about 40 or 50 injuries with ACC. It was getting to the point where it was quite weird."

Palmer had three hip surgeries, but she said she kept getting injured before she could fully heal. She was eventually diagnosed with Ehlor-Danlos when she turned 39. But doctors couldn't explain her 16kg weight loss, or her severe gastrointestinal problems.

In 2014, she was referred to a hospice after doctors told her the pain was all in her head.

Palmer, a criminologist, was devastated the people would not take her seriously. One specialist suggested she go to Australia where a simple stool test revealed she lacked an enzyme which allows the body to absorb fat. An enzyme treatment helped her put the weight back on, but when she returned to New Zealand, doctors dismissed the enzyme theory.

"There's a huge problem I think in New Zealand with this disorder in that there's not awareness of it," she said.

Palmer's quality of life is not the best. She needs a wheelchair to get around, but the one she's been given is inappropriate because the joints in her arms are too flexible. The pain clinics have told her Ehler-Danlos is not painful, but she is the one that feels it.

"This condition is one of the most painful conditions there is. Were are on some of the most heavy duty painkillers there are. We spend our lives trapped in our homes."

Palmer said rehab has been mentioned and noted in her file since March 2015, but it never happens.

"Since being in hospital last year, I have spent most of my time bed-bound. I've had so many injuries and accidents, I've become so debilitated and de-conditioned. Nobody has been able to help me."

She said she has also been injured while she was in hospital. Caregivers and orderlies don't understand her condition, and have left her to fall on many occasions.

"I'm quite frankly terrified of going into public hospital."

She said ACC withdrew her home-help care ad childcare for her daughter just before Christmas 2015.

"There are a lot of people who are a lot worse off than us. I have friends who are suffering terribly who can't get any support or don't know how to access support."