thrombotic thrombocytopenic purpura: what it feels like
5 minute read
bruce morton has ttp, a blood disorder caused by a faulty enzyme called adamts13. supplied
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when it comes to the rare — and often fatal — blood disorder thrombotic thrombocytopenic purpura (ttp), bruce morton is a pro. for a disorder that is only treated in about four people per one million and only reoccurs in 20 to 50 per cent of patients after treatment, morton has been treated for the condition five times.
ttp is a blood disorder caused by a faulty enzyme called adamts13, according to the national heart, lung and blood institute. the result is blood clotting in the body, which can lead to blocked blood flow to critical areas, like the brain or lungs. excessive clotting also means that there aren’t enough platelets in the body to stop other sources of bleeding, which can cause easy bruising, difficulty dressing wounds and internal bleeding.
morton sat down with healthing to talk about the early warning signs for ttp, what it takes to filter litres of your blood and a new treatment that might change everything.
what led to your diagnosis?
i was really sick in january of 2012 and the symptoms just kept on coming — headache, stomach issues and dark urine. if you looked at any one thing you would think it’s nothing, but eventually the items were building up. at the end, i was starting to lose words — i ended up with a speech impediments.
what was the diagnosis process like?
it was actually quite quick. i went to the emergency department, where they took my blood. they said it was ttp — they could see through the microscope that the blood was damaged. the hospital i was at did not have a plasmapheresis (blood plasma therapy) centre and so i was sent to the ottawa general hospital.
what went through your mind?
i don’t think a lot was going through my head at the time — i was pretty down and out. i don’t think i ever really thought about until later, but we didn’t know anything about ttp. all we had was the acronym — we didn’t even know what the letters stood for.
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what is the treatment for ttp?
ttp is an autoimmune disease. i also have rheumatoid arthritis to my left knee and i also have ulcerative colitis which has been dormant since 2007. i don’t know if these things pile up or not, but they piled up on me. to stop the autoimmune diseases, i get a corticosteroid. [ttp is treated by exchanging plasma (plasmapheresis) to remove the antibodies that inhibit the adamts13 enzyme and causes clotting of the platelets and replace it with normal adamts13.]
how is the plasma replaced?
it’s done a couple of ways. you can have two needles in your arm, one for inflow and one for outflow. basically, all your blood is going into a machine and it’s separating out the plasma for waste, and you’re getting new plasma added back into your body. or, they can put in a line in your neck into your jugular vein — it can take three to six hours.
[the jugular] is probably the more comfortable way. i mean, they have to stick a lot of holes in your arm. if you think of the size of a person [and] how much blood you have — half that blood is plasma. it’s litres of plasma that you need to replace, so there’s a lot of flow needed in order to do that. if you’re using the arms, the holes are small, and it takes a long time. but when you have the line in your neck, it’s much more efficient.
it can be risky though — the thing about not having platelets is that you bleed, so putting a line in someone’s neck when their platelets are low is something to think about.
ttp is treated by exchanging plasma (plasmapheresis) to remove the antibodies that inhibit the adamts13 enzyme and causes clotting of the platelets and replaces it with normal adamts13. supplied
how often do you have that done?
i’ve had occurrences of ttp five times. there’s this flurry of having your plasma exchanged, and then when everything gets back to normal you go back to your normal life. you really don’t do anything to prevent it after that. the only thing i do is get my blood tested once every month. but i’m not taking any drugs or maintenance to prevent ttp, we’re just monitoring.
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are there ways to prevent ttp from happening?
we take a steroid to try to stop it, but there are two drugs — one called rituximab [that] tries to prevent the adamts13 enzyme activity from going low. typically, you either get that while you are being treated for ttp or right after. mine has always been after i had ttp. if my adamts13 levels are dropping, i might get rituximab to try to stop it.
another drug which is very new — it’s been approved by health canada but it hasn’t been approved to be paid for — is called caplacizumab. it’s a very expensive drug that basically stops the platelets from clotting.
how does ttp impact your life?
ttp patients can become depressed or have anxiety. typically, ttp patients have issues that stay with them forever. mine is word recollection or mixing up two words. the first time i had a seizure, i could never remember the word ‘seizure.’ i’d have to ask someone what i had.
are there any special considerations that you have to take in your daily life?
no. if you ask any doctor, ‘what can i do? how should i change my diet?’ there’s nothing. i did my own research and ended up dropping meat and dairy. i’ve no idea if this is impacting my immune diseases, but i don’t get the inflammation in my left knee anymore. i used to get cortisone every six months, i don’t anymore. and i have lost 15 pounds. i don’t know if [changing my diet] helped out with the autoimmune disease, but it had other benefits.
you’ve done a lot of work with answering ttp — what are some of the things the group is trying to accomplish?
the first time you notice symptoms of ttp is the worst time because you don’t know what the problem is, and it might take a long time to diagnose. [also, you don’t always respond to treatment.]
we had a 19-year-old in ottawa and she wasn’t responding. she had, i think, three strokes. there wasn’t really any familiarity with caplacizumab, but her mother called the company that manufactured it and got it through a compassionate access program. a few days later, the patient was starting to get back to normal.
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caplacizumab is the drug that we’re working on so that people who don’t respond to treatment get more support.
readers interested in learning more can check out answering ttp and the national organization for rare disorders.
what it feels like is a regular series that explores the experience of being diagnosed, treated and living with a health challenge. have a story to tell? email info@healthing.ca.
emma jones is a multimedia editor with healthing. you can reach her at emjones@postmedia.com or on twitter @jonesyjourn.
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