a surgeon in prison, a broken health system: people with rare disorder struggle for care
people with eds have chosen euthanasia, including the b.c. woman in a controversial simons ad, because they can’t get care in canada
11 minute read
people with a connective tissue disorder known as eds have chosen euthanasia because they can’t get care in canada.
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it has been six years since mohammed shamji was arrested in the brutal death of a “beautiful soul.”
the slaying of his wife, dr. elana fric-shamji, shocked toronto in the fall of 2016. any horrific murder has echoes that reach far beyond the victim and their killer, but this harrowing story was also crushing for the patients for whom the murderous neurosurgeon had offered hope, people withering from a rare syndrome that weakens the connective tissues supporting the human body.
for those patients whose lives he also once held in his hands, shamji’s vicious murder of a well-loved family doctor and devoted mother left them horrified, and, for some, broken. they suffer from ehlers danlos syndrome, or eds, a group of genetically heritable connective tissues disorders that can cause lax and floppy ligaments, loose and painful joints, stretchy, fragile skin, gut problems, organ dysfunction and problems simply moving and thinking straight. a misunderstood and neglected disorder that has accounted for an alarming proportion of recently reported cases of people turning to, or being offered, euthanasia, including the b.c. woman featured in a simons ad that was criticized for romanticizing medically assisted death.
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before he was sentenced to life in prison and stripped of his licence to practice medicine, shamji was considered the go-to-surgeon for people living with eds, the ivy-league trained doctor and distance runner who took patients others turned away.
the toronto western hospital assured shamji’s patients, upon his incarceration, that they’d be re-assigned to other doctors. four surgeons worked through the jailed surgeon’s roster, and it was up to each of those surgeons, “working with the patient,” to determine whether surgery was indicated, a hospital spokesperson said.
according to patients’ stories, it was more often an exit interview, “checked off the list, hung out to dry.” advocates say the promised care never materialized, that people are waiting two to four years before getting their first consult with a neurosurgeon, that, once seen, many are being denied procedures that have shown benefit in other countries, and that people with eds are being abandoned by a system with a long history of shrugging off their symptoms as everything from growing pains to hysteria.
it’s not just an ontario problem. adults and children with eds across the country can’t get access to neurosurgical care, said sandy smeenk, founder and director of the ilc (improving the lives of children) foundation. there are some 300 neurosurgeons across canada. but toronto’s university health network is home to the largest neurosurgical centre in the country. “so, if the ontario spine program won’t take these patients on, none of the other neurosurgeons will take them on,” smeenk said.
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shamji’s position, in his area of expertise, was never replaced, she said.
her organization is appealing to the province to add legislation mandating hospitals and neurosurgeons provide care for those with eds. they want a dedicated surgeon “who can and will perform neurosurgeries” on them.
a spokesperson for the toronto western hospital said the hospital has the expertise and all surgical skills necessary to treat eds patients for whom surgery is recommended. eds patients are evaluated in a specialized clinic. “if that evaluation determines that the individual may be a candidate for spine surgery, they are referred to a spine surgeon, and that individual works with the patient to determine whether surgery will be of benefit and what the associated risks are from such a surgery,” said gillian howard, a spokesperson for the university health network, which includes toronto western hospital.
“sadly, at times, patients believe a surgical intervention is warranted, and clinical teams don’t see surgery as a clinically beneficial intervention,” howard said.
the ontario ministry of health says the hiring of spine and cranial surgeons isn’t “condition-or-disease specific,” fewer than five applications have been received for out-of-country surgical treatment of eds since 2016 and that the ministry is working with hospitals to ensure patients have access to “quality, evidence-based” care, “where and when they need it.”
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toronto philanthropist susan hawkins and her husband have spent hundreds of thousands of dollars for out-of-country spine surgeries for their daughter, erin somers. hawkins is prepared to support the establishment of a surgical program for eds patients in ontario. her charity foundation has $180 million in funds, she has an annual disbursement quota and she’s waiting for a hospital “to tell me what they would like money for.”
hawkins said american surgeons who have treated canadian patients have been dismissed by some of their canadian counterparts as charlatans just out for the money. her daughter has had two surgeries to fuse her spine, and a third to untether her spinal cord. risky operations, but “we’ve never regretted it,” hawkins said. erin has the hypermobility form of eds and a related circulation disorder. pre-surgery, she would black out up to 40 times a day, basically whenever she stood up. her knees and finger joints would randomly pop out and her gi problems made her so sick and nauseous, at her lowest she weighed barely 44 kilograms. her symptoms started when she was 12 but eds wasn’t diagnosed until she was 25, after a pain specialist saw her tall and skinny frame curled up in a ball in his waiting room. “you look really flexible,” the doctor told erin. could her thumb be pushed back to touch her forearm? her pinkie lifted upwards at the knuckle, to go back beyond 90 degrees? “i think you might have eds,” he told her. a geneticist confirmed it.
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after her first surgery in 2013 at a maryland hospital to fuse the first and second vertebrae in her neck, “it was shocking to just feel so great after,” erin, 35, said. she’s now the mother of a one-and-a-half-year-old toddler and pregnant with her second child, due in february. “i’ve been the best i’ve felt in my adult life,” she said. “i don’t know what my life would be if we couldn’t afford these surgeries.”
susan hawkins, right, and her daughter erin somers, who has eds with cranio-cervical instability. hawkins is offering financial support to establish eds surgery in ontario.
hawkins family
in canada, “nobody is taking an interest,” hawkins said. having eds on a diagnosis chart “is just like a red flag for nobody to pay attention to you.” she’s fully aware she could send her daughter anywhere in the world for care. “it’s not me who i’m concerned about. i just feel so badly for these other patients who are like my daughter.”
the true prevalence of eds is hard to know because it often goes unrecognized. estimates range from as few as one in 5,000 people, to as many as one in 300. it disproportionately impacts women. it often begins in childhood. babies can be born extra floppy. as kids, they’re clumsy and bruise easily. their joints are hypermobile. kids have had their shoulders dislocated when their parents playfully swung them from their arms. symptoms vary. not everyone suffers the bone-deep pain or stomach issues or other disabilities. but many describe living with eds like falling down a rabbit hole, spending years navigating the system in search of an accurate diagnosis and proper care. people have been left to feel “invalidated and defeated,” doctors and researchers with the uhn’s specialized eds clinic wrote last year. it’s hard to believe so much can be so wrong in one body: cranial cervical instability, a condition caused by lax ligaments from the skull to the spine. chiari malformation, where brain tissue extends into the spinal cord. cerebral spinal fluid leak. people have multiple “co-morbidities,” and when there’s chronic pain but little care, they become desperate.
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b.c.’s jennyfer hatch, the central figure in a three-minute film by canadian clothing retailer simons on the “hard beauty” of assisted death, chose to have a doctor end her life after struggling for years to find treatment for eds, telling ctv, “it is far easier to let go than keep fighting.”
in toronto, a 32-year-old woman, who is on a waitlist for subsidized housing and whose application for out-of-country care for a condition related to eds was rejected, is in the final stages of the maid process. she uses a wheelchair, wears a rigid neck brace, is bedbound for approximately 15 hours a day and lives in “abject poverty” on government disability support. she’s had two assessments for maid and was told she qualifies. “there is one outstanding document that dying with dignity is helping me get to the second doctor,” she wrote in an email to the national post. “after that i will be in a 90-day waiting period, where afterwards i can book my date.”
“it’s so disheartening,” said a 49-year-old ottawa woman, a former french immersion teacher living with eds. “i want health care, not maid.” in her 20s, she was hiking the mountains of peru. today, the headaches and numbness and dizziness from the lax ligaments in her neck make it feel as if she’s living at 6,000 metres above sea level, “like i’m suffering altitude sickness, without the giddiness.”
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she hasn’t been able to work in more than four years. a trip to the grocery store can put her out for the entire day. “my kids know me as sick. it’s heart-breaking to live that way.” like most others interviewed, she requested anonymity, for fear she would be labelled a “difficult” patient, and the consequence would be no care. she isn’t willing to bankrupt the family by seeking surgery in the u.s. her disability payments are roughly a quarter of what she earned as a teacher. she hates that she can no longer teach. she can’t do much of anything, really. “i don’t think i’ll ever be normal again. but i think my quality of life could be improved with the right surgery.”
like many with eds, she has cranio-cervical instability. the ligaments holding her head onto her neck are weak and loose because her body is producing faulty or insufficient amounts of collagen, the cellular glue that runs through the connective tissue that supports joints, bones, blood vessels and internal organs. it can feel as if her head is having trouble staying on her body, like a bobble head. the more she moves her head and neck, the more she feels “very bad,” so she moves stiffly. “it’s like trying to walk not using the ball of your foot.”
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there isn’t one form, but 13 sub-types, and people spend, on average, four to 16 years getting a diagnosis, according to researchers at the goodhope eds clinic at toronto general hospital. the first of its kind in canada, the clinic opened in 2017, largely due to the advocacy efforts of people living with eds, to provide more timely diagnosis and coordination of care. the current wait time for an initial assessment is up to 24 months. a second clinic, at sickkids, has a typical wait time of six to eight months for children with less urgent concerns. kids with more serious symptoms are seen faster.
it has been estimated that about one per cent of people with hypermobile eds require cervical spine fusion or another neurosurgery. surgery isn’t a panacea. fusion at the craniovertebral junction takes a great deal of judgement when deciding what level of the spine to fuse, and when, say american surgeons who do this. complications can include wound infections — that stretchy skin can be difficult to close — tears on the dura, the tough, outer layer of tissue that covers the brain and spinal cord and, in rare cases, stroke. people with eds don’t heal as fast as others and take longer to recover. it’s tricky to manage their pain, because they sometimes don’t absorb pain meds well. but eds patients say they aren’t looking for a miracle. they just want to feel more normal. when successful, surgery can improve fainting, headaches, neck pain, vertigo, balance problems, bladder problems. the problems don’t disappear, but they’re better. but doctors don’t want to take on people with eds, advocates say, because they’re seen as difficult. their symptoms can cause high levels of anxiety and depression, which makes the pain worse, and when pain becomes unrelenting, hope fades.
jennyfer hatch, 37, was the central figure of all is beauty, a three-minute film produced by simons that celebrated hatch’s last days before seeking medically assisted death.
la maison simons/youtube
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one woman, a former business analyst for telus communications, said she has started to consider applying for maid, because “i just can’t imagine my life continuing indefinitely in this amount of pain.”
she has cranio-cervical instability and myelopathy, an injury to the spinal cord caused by severe compression. she had a cervical spine fusion in 2015, but several further surgeries planned have never happened. she’s had more than 20 referrals to neurosurgeons, even from emergency doctors, since 2017. “none of them will take me.” she can’t get access to a pain clinic, let alone surgery. “you can’t physiotherapy or meditate away bone vertebrae slipping into your cord.” a bone surgeon recently told her he can bolt her spine together, like a construction worker, but he can’t fix the electrical part. he can’t strip the scar tissue and decompress the nerves, meaning she would still be left with chronic pain.
a neurologist has advised her to go to an emergency room when she can no longer walk, stand, or have control of her bladder, in the hope she’ll be given emergency surgery, which was the case in 2015, when she was rushed to hospital after having a massive seizure during a physiotherapy session.
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an urgent call went out that day for any available neurosurgeon as nearby as possible. “i was ridiculously lucky that at the time, they had a neurosurgeon who specialized in ehlers danlos.” it was shamji. he told her that her spine was compressed in three separate places, like kinks in a garden hose, ready to burst.
the surgery was wildly successful. in 2016, shamji operated again. she got out of her wheelchair. she started hiking and kayaking and going to baseball games with her son again. “i was great for a while, but it’s known that when you fuse one level, eventually the levels above and below will go,” she said. “i’m a pretty bendy ehlers-danlos patient. (shamji) told me right from the start we would be doing surgeries for life.” two more vertebrae are slipping into her spine, “and i’ve blown the discs.” because it’s not being treated, “my spine is trying to fuse itself, so now i’m growing bone.” she has a large osteophyte, a bony lump, compressing her spinal cord.
“do i want to have a massive stroke and end up in long-term care and not know who my son is? i’m 49. i should be looking forward to him going off to university and enjoying his adult years. and instead, i’m in bed.”
she felt safe with shamji. “i told people what a wonderful doctor he was,” she said. “i could not put that man together in my brain with a man who could do what he did. i couldn’t reconcile those two people.”
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“i felt like a loved one died when i found out who he really was.”
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