*The below story is Sydney's updated story, as she presented it at the 2014 Chance for Change Game Night.
On September 12, 2008, a three word medical diagnosis that I could not pronounce changed the course of my life –Thrombotic Thrombocytopenic Purpura (TTP). I was no longer an indestructible 28 year-old newlywed. I was suddenly a patient faced with a life threatening autoimmune blood disorder that was creating blood clots throughout my body. My eyes had already been affected and irreversible kidney, heart or brain damage from stroke could have been next. Thanks to the medical staff at St. Michael’s Hospital (SMH) in Toronto Canada, I was diagnosed with TTP and received the best of care.
My TTP symptoms had started about a week earlier when I removed my swim goggles and a dark hole in the center of my visual field appeared. I was not terribly worried because I had been suffering from strange visual migraines for the last 8 months and I thought this was just a new twist to the psychedelic migraine aura that usually lasted about 20 minutes. So there I sat, waiting in the ladies locker room for my vision to come back so I could open my locker. But after the usual 20 minutes elapsed, there was no change. I gave up waiting and somehow I was able to dial my cell phone to call my husband, Alec, to come get me.
After waiting at home for about 3 hours for the migraine aura to clear, Alec and I nervously gave up and went to a major downtown Toronto emergency room only to be turned out after 5 hours with nothing but a migraine diagnosis. I felt silly for troubling the ER with a migraine.
The next day my eyes were still not right, but I was still feeling foolish about the trip to the ER. My mom was nervous, so she contacted my regular doctor whose nurse called me to inform me that migraines can last 3 weeks. “Don’t worry”.
Luckily my mom didn’t quit! The next day I went for a third opinion at a private clinic in downtown Toronto. In addition to getting me an appointment to see a retina specialist, the doctor noticed some bruising and took some routine bloodwork, something that was not done at the ER 2 days earlier.
The next day, before 9 am, the doctor called me at home to tell me to go to SMH ER straight away. My platelet level was only 23 (normal platelet levels are over 150). The risk of low platelets is that your body can’t stop bleeding. I thought that it was a lab error. I put on my favorite jeans and when Alec dropped me at the ER I told him “don’t worry I’ll meet you for lunch.”
We didn’t go for lunch that day. Instead, I spent the day in the ER with specialists running in and out, and trips to the imaging floor. Sure, I was a bit tired and my vision was still strange, but other than that I felt ok.
That night, I was admitted. It was the first of many nights in the hospital but little did I know at the time. I actually felt guilty for taking up a bed.
TTP is difficult to diagnose. First, my doctor started me on Prednisone, an immune suppressant medication, to see if the drug on its own would help increase my platelets. When it didn’t, she told me that she was only about 50% certain I could have a rare blood disorder called TTP. She wanted to treat me quickly because complications of TTP include heart attack, stroke and kidney failure from blood clotting. But she warned me that the treatment was not easy and did come with its own set of risks including blood clots, blood product exposure, and the potential for severe allergic reactions.
By that evening, I was in bed with a central line coming out of my neck that was hooked up to an ancient looking machine. I was receiving my first Plasmapheresis treatment. These blood treatments involve the replacement of my blood plasma with that of 10 donors during each 3 to 4 hour treatment. I have had 68 Plasmapheresis treatments to battle TTP over 6 relapses so far. This translates to almost 700 gifts from blood donors. Everyone in this room would have to give blood 3 times just for me to survive since 2008.
Plasmapheresis treatments are tough. It feels as if your whole body is humming. Given my engineering background, I respect the technology used to separate my blood into components and replace my plasma with that of strangers. But it isn’t something I really like to think about when it is actually happening to me. When I get a bag/a donor that I am more sensitive to, I might develop hives and get hot. As soon as I feel this, I would tell my nurse so she could give me another shot of Benadryl to counteract the reaction from the blood product. With every shot, I felt the rush of the Benadryl and I would try to give in and sleep. But mostly only my eyes close – the rest of my body is anxiously awake. I try not to think about the probability of a more serious reaction. Thanks in part to the advocacy work of the Answering TTP Foundation, the blood product I received this year is cleaned and treated to reduce the probability of contracting a blood born disease or suffering allergic reactions. This makes plasmapheresis safer and easier.
About 60% of TTP patients will go through this treatment process once and never experience a relapse. For the others, like me, TTP continues to reoccur. Plasmapheresis blood treatments are not a cure for relapsing TTP patients like me. But the 700 gifts of blood from strangers has provided me more time and, therefore, a chance to try new treatments aimed to extend my remission time.
My latest relapse landed me in hospital on March 26, 2014. I still have my central line for plasmapheresis treatment, but I’m hoping that it will be out in the next week or so. So does my daughter, Heather Anne. She doesn’t understand why mommy keeps shielding her right side from baby attack. The hardest part about this relapse was the 36 hours I had to go without seeing my 9.5 month old baby girl. This was the longest I’d been away from her smiling face and she was just transitioning into crawling and I was missing it!
More devastating for the long-term was the reality that this relapse signaled that the immune suppression therapy I had started only 18 months earlier had stopped working. Not only did I have to battle this relapse into remission with chemotherapy, plasmapheresis and immune suppressants, but we needed a new plan to keep me healthy. Thanks to the work of the Answering TTP Foundation, we had a world-renowned doctor, Dr. Tsai, come to last year’s TTP treaters conference in Winnipeg. He spoke of using a chemotherapy drug to maintain remission. This is the newest development and I will be on it. There is only 1 other patient in the world on this chemotherapy maintenance for TTP every 3 months.
Over the past few years, we have learned the hope of new treatments to extend remission time. Over the next few years, I hope we will come up with the miracle cure. But so far we still don’t know what triggers TTP, why TTP happens in the first place, what may trigger a relapse from remission, how to ease treatment or how to ultimately cure T.T.P.
TTP is an orphan disease that afflicts only 3 in 1 million people. It is too rare to make it economical for pharmaceutical companies to specialize research to find a cure for TTP. Answering TTP Foundation is successfully funding half a million dollars in TTP research thru 2016, thanks mostly due to the annual Chance for Change fundraiser that started in my basement 5 years ago. This research will help us understand what triggers TTP, what other medications may work to extend remission and how to improve the long-term outcome for TTP patients.