MEDICAL: Local teen risks all to seek relief from rare disorder
Caroline Smith, 16, of Princeton suffers from Ehlers-Danlos syndrome
Sixteen-year-old Caroline Smith knows the surgery might kill her and cost her dad every cent he has.
But those are risks the Oxford County teen will take in two weeks to escape a life where friends shun her, doctors dismiss her and Ontario health ministers have left her and others to suffer with a rare disorder that can cause bones to dislocate at the slightest touch.
“I can’t have (any) physical contact . . . I can’t risk my neck being injured in any way. If I pick up something wrong, I could dislocate my wrist or my thumb,” she said this week.
Smith, from Princeton, near Woodstock, has a condition that weakens connective tissue like ligaments that bind bones: Ehlers-Danlos syndrome affects one in 5,000, only some of whom suffer the worst symptoms.
The toll on the teen has been immense: horrendous pain, limbs that pop out of their sockets, bowels she can no longer control, blood pressure that drops so she passes out and a spine so fragile a small blow to the neck might leave her a paraplegic.
Last year alone, Smith went about 200 times to emergency rooms and doctors, but it’s her next appointment that’s critical and expensive: A Maryland surgeon and facility will charge $100,000 to fuse a bone to her neck Aug. 20 to support a spinal cord that no longer safely supports her head.
Smith’s father Bruce, who’s looked after her as a single parent the past three years, isn’t wealthy: He doesn’t own a home, and while he works in a scrap yard, he’s seeking help from friends and strangers because the Health Ministry hasn’t agreed to pay for surgery outside Ontario.
“It hurts to watch her, how much pain she’s in. . . . We came in to the emerg one day . . . with her crying, saying ‘I’m not crazy,’ and the doctor, calling a psychiatrist, saying she is. This is basically how she’s been treated the last three of four years,” Smith said.
She’s not alone in misery.
About 100 Ontarians have Ehlers-Danlos, with about 20 with symptoms so severe their lives are consumed seeking help from doctors who don’t know what to do, said Darren Crawford, whose 19-year-old daughter Erika has undergone two American surgeries at a cost of $185,000.
“(Some) neurosurgeons here have such big egos, they say, ‘If I don’t know about it, it doesn’t exist’, ” Crawford said.
Sixteen-year-old Caroline Smith of Brantford suffers from Ehlers-Danlos Syndrome, with her father Bruce. (DEREK RUTTAN, The London Free Press)
For Caroline Smith, though, one doctor has been a godsend — London pediatrician Tony D’Souza, who started a practice here after forming and leading the pediatric emergency department at McMaster University Children’s Hospital.
In the face of several obstacles, D’Souza has been fighting for a teenager he calls determined and vibrant. Each time he sees her, she gets worse and worse.
“The neck issue is pressing,” he said. “Could she die? I don’t know.”
It was D’Souza who reached out on Smith’s behalf to surgeon Fraser Henderson, who’s done about 2,000 operations on those with Ehlers-Danlos, including on Erika Crawford, in a facility in suburban Washington, D.C.
Henderson responded in two weeks and said he'd re-arrange his busy schedule so he could see and treat her.
“He’s not doing this because he wants the business,” D’Souza said.
Not a single Ontario surgeon has stepped forward to say he or she could do the surgery, he said.
As for Bruce Smith, there’s no way he’d allow an Ontario surgeon to cut open his daughter without having extensive experience doing spinal surgery on patients with Ehlers-Danlos.
“(I’m not going) to trust someone from here (to) use her as a guinea pig,” Smith said.
Caroline Smith leaves for Maryland Aug. 18 and will have surgery two days later — Henderson required an initial payment of just $20,000 so the operation wouldn’t be delayed.
While there is speedy treatment south of the border, governing Liberals in Ontario won’t pay.
“These cases are heartbreaking,” then Ontario health minister Deb Matthews said in September 2012.
Three years later, the Health Ministry is still fighting the Crawfords from getting reimbursed for the $190,000 spent on Erika’s surgeries, and Matthews’ successor, Eric Hoskins, is dodging questions and calls for help.
Seventeen days ago, in the face of criticism from families and media, Hoskins issued a statement, writing his ministry had identified specialists who would decide the best course of care here or outside the province.
“I have asked my ministry officials to connect families to these physicians to ensure that patients are receiving the care they need.”
But more than two weeks later, no one from the ministry had contacted the Smiths or the Crawfords. When The Free Press pressed Hoskins to provide the list of specialists, he refused, writing in an e-mail that doctors’ names must be kept confidential.
Asked how many spinal surgeries had been done in Ontario on patients with Ehlers-Danlos, and what the complication and mortality rates had been, Hoskins ignored the question.
“We would encourage (patients) to contact the ministry for assistance at 613-536-3123 and ministry staff will connect them with a specialist who can determine the appropriate course of care for them,” Hoskins wrote.
But when the Smiths and Crawfords each phoned that number, bureaucrat Bev Lyman refused to disclose doctors’ names.
It was only after The Free Press pushed Hoskins for answers that Lyman sent by courier letters to families. Only referring doctors, and not families, will get a list of specialists, she wrote.
“It’s a run-around,” Erika’s mother Michelle Crawford said Thursday.
Many people have done much to help Caroline Smith, her father says.
But those efforts aren’t enough and Caroline Smith is left to imagine a health-care system in Ontario where patients like her would be helped with care and compassion.
“Children should never have to go through being (called) crazy,” she said. “They (shouldn’t) have to randomly become a millionaire to afford (treatment).”
DEADLY SYNDROME, LACK OF CARE
- Ehlers-Danlos Syndrome damages connective tissues that support the skin, bones, blood vessels, and other organs. Symptoms vary from mild to life-threatening.
- A leading pediatrician can’t find any surgeon in Ontario who will operate on those who need potentially life-saving neck surgery.
- An American surgeon has done 2,000 operations on those with the syndrome but Ontario’s Health Ministry refuses to pay.
- Ontario Health Minister Eric Hoskins promised action more than two weeks ago but families of patients say he’s giving them the run-around.
HOW TO HELP
- To help Caroline Smith: http://www.gofundme.com/caroline-smith
- To sign online petition asking Ontario to pay for care out of province: https://goo.gl/O3FAzL
London pediatrician Tony D’Souza has tried to find Caroline Smith help in Ontario, an effort that has led instead to dead-ends:
- Neurosurgeons know too little about Ehlers-Danlos.
- Many doctors dismiss her problems as something else.
- A specialist says the teen must wait nine months just to consult him.
- Ontario’s Health Ministry won’t consider paying for surgery outside Ontario unless a specialist requests it.
- Hospitals don’t know how to do MRI scans on those with Ehlers-Danlos and have refused to learn.