Sunday, November 11, 2012


TORONTO—For American families struggling to cope with diagnosis and treatment of Ehlers-Danlos, our fragmented health care system can be a nightmare. From primary care physicians who know little about the disease—and even less understanding of how to treat it—to insurance companies who withhold approval for expensive diagnostic tests, specialist appointments, and treatments, the idea of a single-payer national health care system seems idyllic. Yet, every time Americans who are fed up with an increasingly complex, expensive, and often infuriating for-profit, employer-based system, the county-by-county vagaries of Medicaid and other government-sponsored health insurance—or, the financial devastation of being uninsured—Canada or the UK produces a horror story that makes use question the "national health care" model.

For Canadian Erika Crawford, the pain of Ehlers-Danlos was exacerbated by both Chiari Malformation—and a seemingly uncaring Ontario health care bureaucracy, reports the Toronto Sun:
TORONTO - Erika Crawford is a beautiful young woman with big dreams — and a terrible disease. The Brantford teen’s dreams include being able to play soccer.
The nightmare of her illness is that any small physical contact could cause her a catastrophic injury. Erika, 17, has Ehlers–Danlos Syndrome (EDS), an inherited connective tissue disorder that among other things is causing her spine to press on her brain stem and the main artery in her neck. 
Brantford Expositor reporter Susan Gamble first broke this story. “I was told there was absolutely no cure or treatment available,” Erika told Gamble.  
If she twists her neck too quickly it can cause her to have a stroke or cause damage to her brain stem that could paralyze her. Erika saw scores of doctors before she finally got the devastating diagnosis in April.
EDS can differ in severity from patient to patient. For Erika it could be deadly unless she gets life-saving surgery in the U.S. with a doctor who specializes in EDS. No doctors in this province are experts in the disease. The operation will cost about $60,000. On Thursday the family got the devastating news from heartless OHIP bureaucrats that they’ve turned down her appeal. They won’t pay for her surgery. Erika can’t wait. She’s living in the shadow of imminent death. The surgery is booked for Oct. 9 and the U.S. hospital needs $30,000 on deposit before they can go ahead. It’s disgusting that a government that spent $230 million to cancel two power plants to save the jobs of a couple of Liberal MPPs, that squandered hundreds of millions more on Ornge, won’t spend a nickel to save a child.

Schools and churches are rallying with fundraisers.

While health ministry officials insist the surgery can be done here, those familiar with the complex nature of EDS say that’s just not true. The problem is identifying an Ontario interdisciplinary medical team and neurosurgeon that’s trained to perform the urgent, life-threatening and complex surgery that’s required on a 17-year-old girl, says a spokesman for the charity Improving the Life of Children.
“The family would gladly stay here. This family is facing spending hundreds of thousands of dollars,” said ILC’s Sandy Smeenk. The U.S. doctor has offered to come here to perform the surgery and train doctors in this province. Health Minister Deb Matthews insists the surgery can be performed here and says about 97% of applications for out of country care get approved. She said 35 such surgeries have been performed here. “Given that the application was not accepted, I can tell you that there would be a reason for that and I can tell you that if a child or anyone needs a procedure, no matter how complicated that procedure is, and there’s someone here, or a team here who can do that then, of course, we don’t pay for someone to go out of province when that procedure can be performed in Ontario,” Matthews said.
That’s no comfort to Erika and her family. On top of the stress of dealing with a desperately ill child, her mom, Michelle, is frustrated that they must also fight an uncaring health bureaucracy. She says doctors here aren’t EDS specialists, while 90% of the U.S. doctor’s patients suffer from the condition. “EDS children are totally different,” said Michelle. Erika has complex underlying complications, such as an underlying blood disorder and connective tissue problems.
Shame on this government for letting a child like Erika down. They should hang their heads in shame. If you want help, you can donate to Erika at any CIBC branch using the Transit No. 029528490783.
Luckily for Erika—with classmates and individuals contributing to the cost—the surgery was not only successful, but the experience brought the community together.

Unfortunately, while millions of Americans find themselves in similar circumstances when dealing with countless private insurances companies, Medicare, Medicaid, the Veterans Administration, and many other public insurance plans, stories such as Erika's become the default response to pleas for a single-payer national health care system. 

Clearly, Canada—and the UK—need to modernize their national healthcare systems to account for the complexity and expense of 21st century medicine. Concurrently, those of us who advocate for a single-payer system in the US need to be aware of the challenges facing patients under systems in the UK and Canada and to advocate for reforms that would make single payer national health care in the US a model for the world.
WASHINGTON, DC—It's nice of the Washington Times to provide some coverage to the suffering caused by Ehlers-Danlos—and good supportive commentary for caregivers:

Five Considerations for Supporting People with EDS 

1. People with EDS may seem preoccupied or withdrawn even when they appear to be healthy. The introspection might result from fearful anticipation about what the future holds, from managing pain, or having to be careful about all their movements. They may be exhausted and recovering from exertion. 
2. As do most of us when our activity is limited, people with EDS experience anger. Since EDS is not curable, the anger is something they must learn to manage. The least the rest of us can do is avoid annoying them further by refraining from telling them what they need or how they might cure themselves.
Those who live daily with EDS symptoms know what they need and what to avoid. For example, getting out of the house and sitting in a park might seem like a great idea to you, but the effort can exacerbate EDS symptoms and bring your friend to a point of exhaustion. When having a bad day they will know enough to stay home.
3. Nonetheless, those with EDS like being invited to go out with their friends. Even if they are not up to accepting the invitation it is important to feel included. If an activity is one they cannot participate in having an option of going along to watch will be appreciated.

4. People with EDS can look healthy even when fatigued or in pain, and their stamina changes from one minute to the next. Activities enjoyed last week can be impossible this week, and sometimes they must cancel plans at the last minute. 

5. No one likes being told how they should feel, or that someone else knows how they feel. That is annoying no matter what your health status, but extra grating when you are ill and hear it frequently. Support and understanding are what is welcome. 


Unfortunately, the article fails to fully discuss the potential for cardiovascular risks and complications caused by the disorder. To this, we would add the following further warnings:

1. Ehlers-Danlos Syndrome carries a high risk for stroke, aneurysm, hypotension, mitral valve prolapse, and Chiari Malformation. EDS patients should be evaluated and monitored cardiovascular complications on a regular basis. This may include annual abdominal ultrasounds to rule out the potential for abdominal aortic aneurysms. Extreme fatigue may be a sign of hypotension, which can be treated with relatively inexpensive medication.

2.  Extreme hypermobility is a blessing and a curse. Young dancers, gymnasts, and others with extreme flexibility should be evaluated for EDS, especially if there is a family history of extreme joint pain, disability, stroke, migraines, and aneurysm. Dance can be helpful therapy to strengthen the musculature of EDS patients, thereby reducing the risk of joint dislocation, however, understanding the underlying reason for hypermobility may also help to avoid injury and cardiovascular complications.

3. If diagnosed with EDS, patients and their families should ensure that all medical professionals that care for them understand their condition. EDS can seriously affect surgical procedures and outcomes. Prepared physicians can adjust for this, if they understand the underlying condition in advance.