EDS sufferer Steph Aston describes how being accused of faking her symptoms has ruined her health and her life. Video by Mark Mitchell
Stephanie Aston was told the debilitating illness she experienced existed in her head. She rejects that and says the diagnosis has adversely affected the care she gets, writes Natalie Akoorie.
Stephanie Aston had never met 'Doctor A' before he suggested her debilitating illness was in her head.
At 25 she was flatting in Mt Albert, Auckland when the effects of Ehlers-Danlos syndrome [EDS] began to take hold in October 2015.
Like many adults with EDS, Aston did not know she had the inherited condition and each illness or injury over the years was treated by individual specialists.
She suffered severe migraines, joint dislocations, easy bruising, abdominal pain, multiple injuries, iron deficiency, fainting and tachycardia.
At one of her frequent referrals to Auckland Hospital for severe anaemia, gastrointestinal and heart problems, skin allergies and low potassium, Aston encountered the doctor, who the Herald has agreed to call Doctor A.
She says he entered the room and told her support people to leave.
When they wouldn't, Aston and her two friends were taken to a private room where she says Doctor A suggested she was not seriously unwell and indicated she was causing her illnesses.
"There was no [in person] evaluation prior to this, no psych consultation, nothing."
When Aston complained to Auckland District Health Board, director of adult medicine Dr Barry Snow said Doctor A assessed her at the request of general medicine and it was normal practice for clinicians to speak to patients alone in case of confidential issues.
In a complaint to the Health and Disability Commissioner [HDC] in November 2016, Aston said she strongly disagreed with a factitious disorder diagnosis and believed it continued to have a detrimental effect on the care she received and the way she was treated.
This included rectal examinations, being on a psychiatric watch, accusations of eating disorders and self-harm, suggestions her mother was harming her and that she was faking fainting spells, coughing fits and fevers.
"I feel like I have had my dignity stripped and my rights seriously breached."
Her other concern was that because it was suggested she was self-harming she had been denied a permanent intravenous port to administer blood transfusions for severe anaemia.
"Due to my extremely low haemoglobin levels, my specialist feels I am at serious risk of dying."
Doctor A's response to the HDC said a diagnosis of factitious or somatic symptom disorder was "recognition of a pattern of behaviour that a patient is engaging in, similar to self-harm, that can have deadly or disabling consequences if it is not recognised".
He said factitious disorder behaviour was more prevalent among health care workers, which Aston was, and that Ehlers-Danlos did not explain her pattern of symptoms.
Factitious behaviour included high rates of health care use, unusual and rare symptoms that did not correspond to a diagnosis, intense symptoms disproportionate to physical findings, inexplicable lab results, opposition to psychiatry, and eagerly accepted risky tests and procedures.
"I believe she has real distress and that she does have comorbid medical problems but that her presentations are not explained by Ehlers-Danlos."
Aston, who has family members with diagnosed EDS, said she was not opposed to consulting psychiatry and never declined a referral for psychiatric evaluations, and that she did not eagerly accept risky tests but instead declined some procedures when told they would have little benefit.
Because Aston had low potassium, doctors ordered tests to see if she was taking diuretics to flush out evidence of drugs, or laxatives to maintain an eating disorder.
The tests were negative.
"They've never had anything to back up their story and they just get to make all these false allegations," Aston said.
HDC complaints resolution deputy commissioner Meenal Duggal ruled in May last year that it was not the role of the office to either overturn or confirm a disputed diagnosis, or limit the involvement of mental health services.
Duggal said it was appropriate for clinicians to share information with other providers and that a clinician cannot be compelled to provide treatment if in their opinion it is not warranted. The HDC took no further action.
Steph Aston is so unwell she must live with her parents, needs a wheelchair, cannot travel or work, and has no escape from this life without proper medical care. Photo / Supplied
In June 2016 Aston saw rheumatologist Dr Fraser Burling, New Zealand's only specialist in EDS, who diagnosed her with classical EDS, possibly the vascular type.
Vascular EDS is the most severe type and common symptoms include thin, translucent skin, easy bruising, and fragile arteries, muscles and internal organs.
EDS was later confirmed by two New Zealand geneticists, Dr Patrick Yap of Auckland Medical Specialists and Dr Juliet Taylor of Genetic Health Service New Zealand.
But Aston said Auckland DHB took no notice of Burling, Yap and Taylor's diagnoses.
"They're not willing to back down or budge when they get it wrong."
Despite an unblemished midwifery career, Aston's ability to work had been questioned two months earlier by Auckland DHB general medicine clinical director Dr Robyn Toomath.
Toomath said Aston had undergone a large number of sophisticated investigations in the public and private sectors "without clear abnormalities being demonstrated".
She said no unifying diagnosis had been made and the cause of significant problems such as the severe anaemia had not been found.
"It is my belief that the most coherent diagnosis is that of self-harm presenting as factitious illness."
Independent psychiatrist Dr Kjell Granrud was engaged by the Midwifery Council to assess Aston, who had been working as a midwife at Auckland DHB for five years.
He conducted personality testing that was "unremarkable" and did not show signs of personality disorder.
"It appears to me by going through Stephanie's complete hospital file and by having interviewed her extensively and made a psychiatric evaluation of Stephanie Aston that she fails to meet the crown symptom of factitious disorder which is proving that she intentionally is producing or feigning physical or psychological signs or symptoms."
Granrud wrote a nine-page report in October 2016 concluding Aston did not fulfil criteria for any psychiatric diagnosis, that the factitious disorder diagnosis should be put to rest, and found her fit to practice midwifery.
He told the Herald there was no evidence in Aston's extensive medical notes of self-harming.
"I think factitious disorder is a diagnosis that we as professionals should be extremely careful when using," Granrud said.
He believed doctors should be prepared to present absolute proof that symptoms have been self-inflicted and be "absolutely 100 per cent sure" the diagnosis is correct.
"Especially when you're dealing with a diagnosis such as factitious disorder where the fallout from having the wrong diagnosis is so detrimental to the treatment, because the minute she walks into an emergency room now, people look at her as a fake patient.
"The risk of her dying waiting to be seen is there because she's not actually taken seriously."
Steph Aston is barely alive. She can't access specialist medical treatment for Ehlers-Danlos syndrome symptoms following a diagnosis of factitious disorder. Photo / Michael Craig
By mid-2016 Aston's health deteriorated and she moved home to live with her school teacher parents near Albany, taking her out of the care of Auckland DHB and into that of Waitematā District Health Board.
Aston, now on a long-term sickness benefit, began receiving weekly blood transfusions to address the severe anaemia but they stopped suddenly.
She believes Waitematā DHB was informed she was self-harming.
"I was told because I had been a midwife I must be stealing needles from the hospital and bleeding myself through my vagina, my bowel or my throat."
The accusations led to humiliating internal examinations and unannounced strip searches.
"Sometimes without my consent they would pull my pants down. It's terrifying. I feel like I'm living in a nightmare and I can't wake up."
Burling said it was beyond his understanding "how a patient can cause bleeding from their own bowel when the colonoscopy showed no rectal/sigmoid damage".
He said the greatest suffering in EDS patients came from being misunderstood and undiagnosed.
"Patients are being denied care that they need. Where a nasojejunal (nose-to-bowel) feeding tube or intravenous feeding would stabilise them, some are being denied even this."
The Herald has learned of two suspected suicides in EDS patients this year, after they were allegedly denied appropriate therapy. The patients did not have a factitious disorder diagnosis.
"The risk for suicide, when not being believed, or being denied appropriate treatment, is understandably higher."
The American Journal of Medical Genetics reported in March last year that approximately 70 per cent of EDS patients were affected with psychiatric issues, but most of these were secondary to EDS.
In September 2016 Aston was escorted to hospital by police after her GP believed she would die without treatment.
Three months later when she was readmitted to North Shore Hospital for exploratory tests, acute and emergency medicine division head Dr Gerard de Jong led a case review which discounted her EDS diagnosis.
After Aston refused further treatment by de Jong, police and paramedics swooped on her home with her GP and forced her back to hospital.
Aston's haemoglobin blood count by then was extraordinarily low, in the 30s - normal levels for women range between 120 and 160 grams per litre of blood.
"I was told that if I didn't go in willingly they were going to drug me, against my will and take me in [to hospital]. I didn't want to go in because they don't actually treat me."
Aston was sectioned under the Mental Health Act that day in January last year, meaning she could not leave hospital and was admitted to a medical ward with a 24-hour psychiatric watch.
"I wasn't even allowed to go to the toilet or bathroom by myself."
Aston received an iron infusion despite a previous severe allergic reaction but claims a blood transfusion never happened.
She remained sectioned for almost three weeks.
Steph Aston suffers from life-threatening blood loss but says she can't easily access blood transfusions because doctors believe she is self-harming. Photo / Facebook
The situation has taken a toll on Aston's parents David and Pam who wrote a letter to Waitematā DHB in their daughter's defence.
"It has become apparent that there are serious disagreements between highly qualified medical professionals and a significant power control base which has complicated and resulted in a serious deterioration in Stephanie's health," they said.
"When the diagnosis of factitious disorder was made hospital staff appeared to uniformly exclude any other medical condition.
"We believe (Doctor A's) incorrect diagnosis of factitious disorder has caused significant damage to Stephanie's life and her ability to access safe, both physical and psychological, care in the medical system."
The pair told the Herald they felt doctors wielded power over patients.
"We've both grown up respecting doctors and believing them but it just seems in this situation that if they don't know what's wrong, she must be lying. It's appalling."
Aston's support person, a mental health consumer advocate, said she would not have believed the treatment of Aston by New Zealand's health care system possible had she not witnessed it.
"Stephanie is exceptionally traumatised by the health care she has received over the past several years."
The advocate, who did not want to be named, said Aston had been treated as a "clinical case" rather than a suffering human being.
She recommended an individual plan for Aston under a different doctor to access weekly blood transfusions.
"I am understandably very worried about her not surviving long enough for this to happen if it depends on the organisational cogs turning at the usual pace."
In March Waitematā DHB associate chief medical officer Dr Jonathan Christiansen wrote to Aston's Member of Parliament Erica Stanford, noting 56 "contacts" including admissions and specialist outpatient reviews of Aston over six specialities, and an "unknown number of consultations in the private sector" since 2011.
Christiansen blamed the breakdown in treatment on Aston, saying she declined further transfusions on December 23, 2016.
He said the Mental Health Act was activated because of Aston's life-threatening anaemia and concern that she "did not have insight into the significance of that".
"During the subsequent period of 1:1 supervision no source of blood loss was seen, and the haemoglobin rose from 34g/l to 66g/l without transfusion," Christiansen wrote.
The implication was that Aston was unable to self-harm while she was being monitored, but Aston says her haemoglobin rose because of the iron infusion given the day she was sectioned.
She self-discharged against medical advice on February 9, 2017 because doctors allegedly said they would not treat her medically, but instead keep her on an indefinite psych watch.
In a letter to the DHB in May last year, Burling pointed out Aston's haemoglobin dropped during a prolonged stay in hospital and while she was under observation, in December 2016.
It was the same month de Jong and his team undertook an exhaustive review of Aston's medical history after he became involved in her care.
De Jong noted: "We could not confirm the [EDS] diagnoses postulated (by Dr Burling) to play a role in Ms Aston's medical history."
Christiansen said Aston's repeated refusal to engage with Waitematā DHB meant it had not provided any outpatient care for her since March 1, 2017 and no admissions since June the same year.
An emergency plan remained in place for Aston, he said.
"Waitematā DHB retains grave concerns about Ms Aston's wellbeing and future health, and will continue to explore all avenues to ensure she receives the care we believe she needs."
Aston argued that de Jong's review omitted previous test results, making the outcome biased, and maintained she had only ever declined care from de Jong, not the DHB.
In November last year a mediation between Aston and the DHB, chaired by former Health and Disability Commissioner Ron Patterson, did not resolve the stand-off.
Stephanie Aston was sectioned under the Mental Health Act for refusing treatment. She says she only refused care from a certain doctor. Photo / Supplied
That same month Auckland forensic psychiatrist Dr Caleb Armstrong assessed Aston as having "no mental health diagnosis" and noted she had been "poorly served by the DHBs".
"Should Stephanie be able to prove the refusal to provide treatment to her for a medical condition was based on her having a co-existing psychiatric disorder (whether or not this diagnosis is an accurate reflection of reality), then it would be possible for her to claim that she has been discriminated against on the basis of a suspected psychiatric illness, and she would have recourse to the Human Rights Act via the Human Rights Commission."
Armstrong told the Herald there was enough medical evidence of EDS that the diagnosis should be taken seriously.
"She's now been assessed by four psychiatrists, and there's only one who has said he thinks that she has factitious disorder."
Armstrong, Granrud and Dr Anthony Asteriadis all contradict Doctor A , saying Aston does not have a mental health condition.
Armstrong said he believed there was no reason why Doctor A's diagnosis should not be removed from her medical file.
"It's her life at stake. Currently they don't transfuse her. She has a low haemoglobin because they accuse her of doing all sorts of things to herself and they've got no evidence of that."
In a letter to Waitematā DHB chief medical officer Dr Andrew Brant in September, Armstrong said in his view factitious disorder could not be substantiated.
"I believe that this is impacting clinical decision-making in a negative way that adversely places Stephanie at risk of not being treated when she needs it, including in situations that would generally be recognised as a medical emergency.
"Therefore, this diagnosis is at best uncertain and worst significantly undermining her care."
Armstrong called it an "extraordinary situation" that he believed represented a "grave risk of disservice and perhaps danger to the patient".
In late October, more than two months after Aston emailed Brant asking for "safe care", Brant responded, assuring her she could access emergency care at North Shore Hospital without the "anxiety" of de Jong's involvement following a change in requirements.
Six weeks later she is still waiting to be admitted.
In a written response to Herald questions the DHB said it had gone to considerable lengths to give Aston the highest quality care.
"We have plans in place to ensure she can continue to access timely and appropriate care."
An Auckland DHB spokeswoman said for ethical and privacy reasons it could not comment on individual patients' treatment.
However she said the DHB was fortunate to have high calibre specialists who worked hard to provide patients with treatment that was appropriate for their individual needs.
"In line with the principles of the Medical Council of New Zealand, Auckland DHB doctors strive to provide effective treatments based on the best available evidence and that are in our patients' best interests.
"We avoid inappropriate clinical interventions that risk causing unnecessary harm. We encourage patients to talk to us directly if they have concerns about any aspects of their care."
The Herald also asked the Auckland DHB for an interview with Doctor A. The spokeswoman said he was unable to talk about individual patients' treatment for ethical and privacy reasons, despite privacy waivers signed by Aston and the other three women.
The spokeswoman said Doctor A was unable to talk about factitious disorder even in general terms as this could be mistaken for comments on an individual patient. However on Friday Doctor A provided names via the DHB of two international experts in factitious disorder who could be interviewed instead.
The Herald spoke to clinical professor of psychiatry at the University of California at Davis, Dr Glen Xiong, an expert in factitious disorder and also an internist, who said it was possible Doctor A's diagnoses were wrong.
However Xiong, who trained with Doctor A and has co-authored papers with him, said it was statistically more likely that patients who needed excessive treatment for conditions that should usually be manageable were presenting with some other kind of distress.
"Those are the red flags. There's some kind of foul play there."
Earlier American doctor Heidi Collins, an Indiana rehabilitation specialist who has EDS, said it was the most maligned condition in medical history with many cases of patients being diagnosed with a psychiatric disorder when they actually had EDS.
"I think the key is when you take these people and you diagnose them with factitious disorder or whatever it might be, and you go ahead and treat according to the medical established standards of care for those disorders, they don't get better."
Collins said female EDS patients were more often misdiagnosed.
"They are looked at as crazy women. As if they're histrionic and suck the time away from the doctor because they're so complicated, is what the doctor perceives.
"I believe this tendency to just come away with a psychiatric diagnosis is because it's easier for the doctor to not delve into the complexity of knowledge that they think this disorder requires."
A typical EDS patient had dozens if not hundreds of contact with doctors and hospitals in their life which Collins described as "baggage" that some clinicians can't see past.
"The patient has been so rocked by this experience that they're just exhausted and they become exhausting to the medical professional."
Ehlers-Danlos Advocacy Organisation lead coordinator Tracey Jourdain said EDS patients notoriously struggled to get acknowledgement of their condition and this was traumatising.
"You're basically giving a disabled person who's already struggling less resilience and less ability to cope and we all know where that leads."
Burling told the Herald Aston's haemoglobin levels were unsurvivable for most adults.
"She's what I call my patient whose life is most threatened."
Aston hasn't had a blood transfusion since June last year and credits her survival to Burling who started her on a nasal spray that clots her blood.
Steph Aston wants a factitious disorder diagnosis removed from her medical file and for her care to be transferred to a different DHB. Photo / Michael Craig
She ingests two hot drinks and one Up and Go smoothie a day, and says she has been told by Burling her case will probably end up at a coroner's inquest.
"I am so sick that I am fighting for my life."
Since publication, Auckland District Health Board has responded to the Herald's series about EDS and factitious disorder published this week. An Auckland DHB spokeswoman said the stories seemed to have singled out Doctor A, which was not a fair representation of the diagnostic process.
"Although we can't comment on the specifics of individual cases, these sorts of diagnoses are not determined by an individual clinician in isolation but form part of a diagnostic process and comprehensive medical assessment that includes input from a number of specialist clinicians."
Medical glossary
Somatic symptom disorder An extreme focus on physical symptoms such as pain or fatigue causing major emotional distress and problems functioning.
Differential diagnosis In medicine, a differential diagnosis is the distinguishing of a particular disease or condition from others that present similar clinical features.
Haemoglobin The protein found in red blood cells that helps carry oxygen from the lungs to the rest of the body.
Anaemia When there is not enough haemoglobin or functional red blood cells leading to a lack of oxygen flow to the body's organs.
Tachycardia A heart rate that exceeds the normal resting heart rate.
Sigmoid colon The S-shaped last part of the large intestine, leading into the rectum.
Sectioned When the Mental Health (Compulsory Assessment and Treatment) Act is used to give compulsory treatment to a patient including keeping them in hospital.
