My name is Bruce Morton. I am a 5-time, 10-year TTP veteran. My first episode was in 2012, second in 2014, third in 2017 and twice in 2019. I am not afraid of TTP. It is part of me - my body and mind have found a way to survive.
I feel fortunate as I have had minimal long-term problems. I am not depressed and not really concerned about getting TTP again. Over the years, I have dealt with some loss of word recollection, which may also make my responses slower as I try to recall the right word before I talk. Most people, even the people closest to me, never get used to this issue.
I was probably lucky in 2012. I assume most TTP survivors are lucky when they first get TTP. The reason is that TTP is rare, and most doctors never get a chance to make the diagnosis. Many patients are sent home and then may suffer dire consequences. For me, it took me a couple of weeks to finally go to the hospital. I was sick with headaches, dark urine, sniffles, blood in one nostril, petechia, bruising, shortness of breath, and finally loss of speech. Once in the hospital I was diagnosed quite quickly, but the hospital did not have an Apheresis clinic, so I was transferred. In the hospital I had a stroke, a seizure and discovered I have anaphylaxis (an allergy) to fresh frozen plasma transfusions – not good for a TTP patient. I was in ICU twice. I may have been the first patient to receive solvent detergent plasma, which was only approved for use in Canada after I had already received it. I had plasmapheresis 40 times. With all the issues, I was well and back to work in just over two months. Due the seizure, I lost my driver’s license, and sadly it took me almost 7 months to get it back.
In 2014, I was in the hospital for only 2 weeks, plasmapheresis 10 times, and back to work in about 6 weeks. I did develop deep vein thrombosis in my left leg, so had to have those blood thinner needles injected in my lower abdomen. Some fun, and lots of bruises.
We were on vacation in July 2017 when we saw the bruising. We were at the hospital 4 hours later. I had plasmapheresis in my arms for 9 days straight. With all the holes and bruising, I must have looked like a serious addict. The good new was that my platelets were over 200, so we took Friday through the weekend off. The bad news is I developed retinal detachment and my plasma dropped to 39. We had to start all over again, but this time with the line in my neck.
In the Apheresis clinic, I saw a hashtag on the window which said #stustrong. This was posted by a local radio broadcaster which had been treated for leukemia. I thought about this and came up with @BruceFightsTTP. I had Bruce Fights TTP t-shirts created and decided to raise funds on International TTP Day. I held a walk where all participants received a t-shirt. The Bruce Fights TTP fundraisers led the pack and raised the most funds that year. We have continued raise funds in the following years with much success. In 2020, we continued to raise funds during the COVID-19 pandemic, where most fundraisers sent me pictures of themselves in their t-shirts.
So, what have I learned during my TTP tenure? First, you can’t forecast TTP by checking your platelets. The measurement of my platelets has either told be I don’t have TTP, or I do have TTP. The best method to forecast TTP is to measure your ADAMTS-13 activity. This is an enzyme that must be active, or your platelets will start to clot which is TTP. The change in your ADAMTS-13 is much slower than your change in platelets, but it is the change which needs to be monitored. ADAMTS-13 activity of less than 10% of the normal level is a symptom of a TTP patient.
Second, although rituximab is a drug for cancer it is also a drug for TTP patients. The recommendation is that TTP patients should have rituximab when their ADAMTS-13 activity is less than 20% of the normal level. When a patient does have TTP, then rituximab is recommended to prevent TTP from occurring in the future. We need to help ensure TTP patients have easy access to rituximab.
Finally, there is a new TTP drug called caplacizumab. This drug will not prevent TTP, nor will it stop TTP; however, caplacizumab is a drug for which prevents platelets from clotting. This helps the patient from suffering from consequences which I have suffered from such as headaches, strokes, seizures, deep leg thrombosis, retinal detachment, and kidney problems. This also gives much needed time for corticosteroids, plasmapheresis, and rituximab to work and increase both the ADAMTS-13 activity and the platelets level. The good news is caplacizumab is approved by Health Canada, but the bad news is that Canadian Agency for Drugs and Technologies in Health (CADTH) issued a negative recommendation for caplacizumab. This means payment for this expensive but impactful drug is not readily available for Canadians. Hopefully this will change in the near future to help the next suffering TTP patient.
That’s my TTP story. I am so lucky I have a wonderful wife and an awesome family. They took care of me when I needed it most.
Oh, if you have a chance, please give blood, donate, and give thanks.
Bruce Morton
@BruceFightsTTP
