I still hope to do all of those things, but unfortunately those dreams, and many others, have been put on hold.
I have a genetic disease called Ehlers Danlos Syndrome. You’ve probably never heard of it. I certainly hadn’t. And a fair number of my doctors haven’t either.
Ehlers Danlos Syndrome is a connective tissue disorder. Connective tissue is basically the glue that holds our bodies together, which means that EDS can affect pretty much every part of you.
It’s classified as a rare disease and as a result, not much time is devoted to understanding it in mainstream medicine. Currently there is no cure, and most treatment options consist of a wheelchair, bracing, pain medication and a life of progressive disability.
It’s so under the radar, especially in Canada, that I spent years getting steadily more ill, having to drop out of school and move back in with my parents, unable to look after myself. I was given no diagnosis and was recommended to ‘stop looking for answers and move on with my life’, as I lost the ability to walk or hold my head up independently.
It was only after my own desperate research that I paid to travel to see a Geneticist in the States who specializes in connective tissue disorders. I was immediately diagnosed with Ehlers Danlos Syndrome, and rushed to a Neurosurgeon.
While I experience joint, vascular, hormonal and gastro-intenstinal issues due to EDS, my primary disability comes from neurological symptoms - headaches, difficulty balancing, weakness, fatigue, confusion, memory loss, blurred vision, slurred speech, drooping facial muscles, an inability to hold my own head up, and a host of other fun stuff including episodes of being completely uncommunicative, unable to speak or move, struggling to breathe.
The neuro-surgeon ordered upright MRIs with flexion and extension - technology that is not used diagnostically in Canada. These weight bearing MRIs showed what previous studies hadn’t - that when I’m in an upright position my whole spine is extremely unstable, causing my skull to move and crowd my brain.
A month and a half ago I had my first surgery in the States - a fusion of my C4-C5 vertebrae. It’s a common enough procedure for the average population, but when you factor in faulty connective tissue the surgery becomes much more dangerous, requiring a completely different approach and experience that does not exist in Canada.
That procedure was a definite success, but it wasn’t the end of the road.
My latest scans have shown that my cerebellar tonsils are protruding through the base of my skull, interfering with the flow my Cerebral Spinal Fluid. It’s called a Chiari Malformation.
The scans also show that my skull has become even more unstable, shifting further every time I move.
Both of these things can lead to stroke, paralysis, and a whole other host of scary stuff if left untreated - you can learn more about them and their relationship to EDS through this article or this video.
As a result I am scheduled for a decompression surgery and cranio-cervical fusion on October 26th 2015 to make room for my brain and hold everything in place- an essential procedure both to improve my quality of life and to avoid the life theatening risks of such a precarious postion.
This procedure can only be done safely on an EDS Patient by a very small number of Neurosurgeons, under 5 in the world- none of whom are covered by Ontario Health Care, despite Canada’s complete lack of options for addressing the condition and its various complications.
Luckily, the surgeon who will be operating on me is a world renowned expert in this field. 90% of his patients that have undergone this particular surgery have experienced a substantial increase in their quality of life.
So now, I’m asking for help. Getting steadily worse and becoming completely bed bound is not an option. I have too many hopes, dreams and plans for that. I am so lucky that my family has supported me in every way through my previous surgery and are prepared to do everything they can to help me through this one as well, but the financial cost is simply too great to handle on our own this time.
I’m looking to cover the costs of this surgery as well as a portion of the travel and hotel expenses. Any amount helps, nothing is too small.
And if you want to help in other ways, please, please, please do! I’m taking all the help I can get right now.
Please share this page as far and wide as you can, and if you’re interested in asking people to sponsor you to complete a particular challenge, run a certain distance, knit a certain amount of scarves, eat a banana upside down, share some artwork, music or anything else that pops to mind in an effort to help meet this goal then please go for it! And if you'd like, use the hashtag #MakeRoomForOphirasBrain!
Thank you all so much. This may not be a cure and it may not be the end of my medical expenses, but it is a major procedure that should have a major impact. I can’t wait to recover from it and see what all I’ll be able to do. I can’t wait to get started on my dreams.
- Ophira, xo
Fantastic news, Ophira. Continue on the road to health! Very happy for you. As an aside, do you know Gabrielle is getting married Feb 14?! We're very excited!
Ophira, this is wonderful news! Congratulations on the success of your operation, your courage in going through all that you've been through, and the renewal of living that you're now enjoying. You are such an inspiration! Hope we'll see you back in BC someday (assuming you're at home in Ontario?).
Wanted to thank all of the generous folk on here. You all inspire me. Love you Ophira!!!!
Best wishes from a fellow zebra - I was diagnosed by CHEO genetics last year. Tell your Mom to give me a call if she wants to talk. Eddy Roue has my number.
Wishing you all the best I have posted and have asked others to hopefully we can get the funding that you will need.