My name is Lorraine and my TTP story and diagnosis is a little long and complicated. I am 60 years old, I am married, I have 3 adult children and 2 grand babies and live in Sherwood Park, AB, Canada. I wish I could say that the only life-threatening disease I have was TTP. Unfortunately, in 2016 I was diagnosed with 3 different life-threatening illnesses, of which TTP is one and currently the deadliest. My health journey started in October 2015 when I found a lump on right side of my neck. My worst fear used to be getting cancer. Little did I know that would be the least of my worries. As it turned out, the lump in my neck was a rare form of parotid gland cancer. I had the tumor removed in March of 2016. Though the tumor was cancerous and there was no evidence of spread, it was recommended that I have radiation therapy to ensure there were no cancer cells left behind.
I started my 30 rounds of radiation in May and finished on June 21, 2016. Since the beginning of 2016 I was experiencing some other symptoms, including numbness and pain in my hands. I originally thought I had carpal tunnel syndrome. My hands also swelled to the point I had to get a ring cut off. I also had these sores on the ends of my fingers, which I later found out were digital ulcers. I went to my doctor who recommended me to a rheumatologist with an appointment scheduled months away. I was also experiencing nausea during and after my radiation therapy as well as shortness of breath going up the stairs. I assumed everything I was experiencing was related to my cancer, stress, radiation treatments and being out of shape. I not only worked full time for the government, but I was also a part time fitness instructor.
I had just finished my radiation treatments then I had some very strange vision issues where there were small fields of vision missing in my eye sight. I had also noticed multiple small bruises on my body. My first thought again, was that it must be a result of the radiation to my head and neck. I contacted my oncologist who said this was not because of the radiation and referred me to my optometrist, who then found hemorrhages in my eyes. My Optometrist sent me to my GP for bloodwork to find the cause of the bleeding.
My GP ran some basic bloodwork which came back that my platelets were extremely low (69). He then had me in within the week to see a Rheumatologist. The date was now June 30 and the rheumatologist ran a ton of blood work. She called me that evening at home and told me then that I needed to report to the University of Alberta hospital the next day to the hematology outpatient clinic, sooner rather than later. I was admitted to hospital on July 1, 2016 and at that time they told me they thought I was in renal crisis. They may have mentioned TTP, but I don’t really remember. They treated me with blood pressure meds though my blood pressure seemed fine and were amazed at how healthy I seemed, stating people in my condition were usually a lot sicker.
They continued to run a battery of tests and 4 days later 7 doctors and residents including specialties of hematogy, rheumatology, nephrology, general and internal medicine walked in my room and told me I had TTP. I was by myself and knew it must be pretty serious given the number of white coats standing in front of me. I asked if this disease was potentially fatal and they said yes and suggested that I not google it. I got very upset and remembered that I started hyperventilating and crying.
They explained about the need for the IJ port and that it would need to be inserted and that I would start plasmapheresis the next day. I think they also mentioned another disease they thought I may have called scleroderma, but they were waiting for some more specialized tests to come back to confirm this.
I spent 25 days in the hospital for the TTP and went through 9 rounds of plasmapheresis. When I wasn’t responding as they hoped I would they then started rituximab treatments which I would end up completing as an outpatient. During my stay they confirmed I also had another rare life-threatening autoimmune disease called Scleroderma or Systemic Sclerosis. This is an autoimmune disease where your body creates too much collagen and creates scar tissue in your major organs heart, lungs, kidneys and skin and can potentially lead to major health complications and death as well. This explained a lot of the symptoms I had been experiencing since the beginning of 2016. As I was in the initial flair of this disease, it created contraindications for the treatment of my TTP. The doctors researched how to manage the scleroderma and TTP together and told me they could only find 9 documented cases of patients like myself who had both TTP and Scleroderma. I like to be special, but not this special.
I left the hospital doing blood work 2-3 times a week and had to go back in early August for 4 days when my platelets dropped to 104. It turned out I had something viral and did not need more plasma and my platelets came up on their own and I was discharged. I continued to do bloodwork weekly and then monthly to monitor my platelets. My hematologist told me that he thought this TTP episode was likely a one-time thing and unlikely to come back. He gave me standing orders for blood and told me to get my blood work done if should get sick and/or notice unusual bruising. I basically put TTP out of my mind as I now had bigger fish to fry as my Scleroderma was progressing and that disease took over as the priority.
I ended up being off work for a total of 10 months due to the combination of post radiation fatigue, TTP fatigue and major symptoms with the Scleroderma. I eventually got back to a new normal resumed working fulltime and starting teaching fitness classes again occasionally until April 2018 when I relapsed. I had been sick less than a month earlier and my platelets were fine. Ironically another marker that my rheumatologist was testing was not good and she wanted me to redo my bloodwork before my appointment with her at the end of April. The week prior to this, my husband noticed a small bruise on my back and I had a couple of small bruises on my legs. I knew I was doing a CBC with my rheumatogist’s requisition so I would get my platelets done but not expecting anything. It was a Saturday when I had my bloodwork done and received the results online that night. My platelets were at 27. I knew I was relapsing and so we went to the hospital. Once again, very minimal symptoms.
This time I spent a month in hospital and had 17 plasmapheresis exchanges and another 4 rounds of rituximab to get stable. While in hospital I met another TTP patient who was relapsing and she is the one who got me on the Facebook TTP websites. Believe it or not, it was now that I finally googled TTP and found a lot of information including this website and realized how serious this disease was and that relapses can happen anytime.
This time my hematologist started testing my ADAMTS13 monthly and for six months it remained less than 3%, though my platelets, LD and haptoglobin were normal. As he was worried about me relapsing, he chose to do another 4 rounds of rituximab in January 2019. After that my ADAMTS13 went up very slowing and we were only testing every 6 months.
Fast forward to January 2021. I ended up contracting Covid19 from my end of life dying father who was comatose and had tested negative days before his admittance to the hospital. It is a long story and I have kept a health blog since 2016 chronicling my journey with all my illnesses. If you are interested, anyone is welcome to check more information at https://www.caringbridge.org/visit/lorrainewigston
I was worried the Covid 19 would trigger another relapse and I was not wrong. I was very fortunate that I had a very mild case of Covid 19, due to my numerous underlying health conditions. I went to my local community hospital as I had a low-grade fever, was coughing and the health unit said I needed to be checked out for pneumonia. Of course, they did bloodwork which I wanted and my platelets had dropped 50 points since my regular draw 24 days earlier. It took another 5 days of daily bloodwork for my platelets to drop below 140 to 78 and I was finally admitted. I was still considered a Covid patient and potentially infectious and put on the Covid unit. I received three rounds of plasmapheresis and one round of rituximab. My platelets remained in the 200’s for 3 days without treatment and so they released me as there was no reason to keep me. The next day I got my bloodwork done and platelets dropped to 95. Back to the hospital.
They infused me with plasma overnight, but my platelets continued to drop and were at 25 after 3 units of plasma. I now was getting very worried and scared that maybe I would not dodge the bullet this time. They did plex that day and then the pharmacist walked in the room with a piece of paper. He told me that I had been approved for compassionate coverage of a new FDA approved drug called caplacizumab that costs $180,000.00 for a 30-day supply of treatment. I almost had a heart attack!
I had heard about this drug and the clinical trials during my relapse in 2018, but now I needed it and was more than happy to receive it, especially without any cost to myself. The drug arrived the next day and I started the injections. I remained in hospital for a total of 2 weeks this time and had an additional 6 rounds of plex and continued my rituximab treatments.
I will admit this TPP relapse really kicked my butt. I went on anti-psychotic drugs to counteract the effects of the prednisone, which were much worse than I had ever experienced. The side effects were awful and I have just finished weaning off of them as well as the prednisone. I have gained at least 20-25 lbs this time and I am more exhausted/ fatigued than I remember before. It is hard to know if I feel this way because of post TTP or post Covid 19.
I am grateful to be alive, though I still worry about catching Covid 19 again, because even though I have had it, the rituximab could have nullified any protection I may have had. This will also affect when I am able to get the Covid 19 vaccine. I think the caplacizumab likely reduced my hospital stay and need for plasma and I am also so very grateful to have received this very expensive drug. My recent ADAMTS13 test was 87! The highest it has ever been since we started testing it. All I can do it take it one day at a time. I take nothing for granted, I hope for the best, and continue to live the best of my life, not simply the rest of my life.