I was honoured to be asked to write a few words for the TTP Network pages about me and my TTP journey. I thought this would be really easy, after all it’s me that has been ill. It’s amazing how hard I have found this though. The time before knowing what was wrong was easy but trying to describe my time in hospital and then my recovery has proven to be really hard. In one respect this is because I actually don’t remember much about it due to being so ill and on another level it has proven to be really upsetting. I had obviously compartmentalised some of my TTP journey and locked it away in a cupboard in my brain where it couldn’t hurt me. Going back into that cupboard and looking around opened up a whole lot of emotions I was trying to forget. I wrote my story then couldn’t edit it for over a month just because I was so exhausted by having to deal with everything again.
What can I tell you about me, I’m Joanne. I’m married to Richard and we have two wonderful children and a dog called Sammy. All in all I’m a pretty average late 40’s woman who has had an incredible 18 months. It’s only now trying to write about my TTP story that I realise I was so ill, then when I started to get better I was so tired for months that my days were all the same. Re-visiting my TTP journey and remembering what it was like at its worst is hard, my thoughts and support go out to anyone currently going through this. Hopefully my story here will show you there can be light at the end of the tunnel, it can just be a long tunnel!
It all started last March 2019. I was tired and felt like I might be coming down with something. Nothing specific just really tired. Saturday night when getting ready for bed I noticed some tiny spots, a bit like blood blisters on both feet. I had been wearing my trainers all day and just thought I must have tied the laces far too tight, thought nothing of it and went to sleep. Sunday came and went, I was still tired so just took things easy. By 8pm I was good for nothing so decided to go up to bed. Getting into my pyjamas I noticed my ‘foot spots’ had now become foot and lower leg spots. I hadn’t noticed them all day and they were not itchy, or raised but definitely there were more of them. I called Richard and asked him to take a look. They were really tiny but all over the lower part of both legs up to about mid shin. It looked like I’d been drawn on with a fine tipped biro. We knew about the ‘press a glass to the rash’ test so looked around and found an aftershave bottle. Richard then was rolling this bottle over the spots trying to make them go, but they didn’t. At this point I knew I shouldn’t ignore it so I phoned the out of hours service and got an appointment that night. When I got there I had to wait for a while to be seen so I was very relaxed. Well, if it was anything serious they would have told me to go to A&E wouldn’t they? The doctor was lovely, he examined my rash and admitted he didn’t know what it was. He asked me more questions about my general state of health and I told him about my tiredness. I was told I needed blood tests at my GP the next day. OK I thought, that’s fine, they are not worried and I can go home and get some sleep.
Monday morning I booked my blood tests for the afternoon as the doctor also wanted to check me over. I phoned work and explained I would have to work from home as I had to go to the doctors. I work in Cambridge so leave home at 7am and don’t get back until almost 7pm. I absolutely love my job as a Research Governance Manager and felt guilty at just not turning up. However, after my lack of sleep I would not have been fit to drive, even if I didn’t have the GP appointment.
By the time I saw the GP the ‘rash’ was now up to almost my knees. I didn’t have a temperature, I had no itches, my blood pressure was OK, no sore throat or any other symptoms. She was stumped. She arranged a follow up appointment to discuss the results in 1 week. I was relieved there was nothing ‘serious’ going on. I went home and actually fell asleep for the afternoon.
That evening 10pm my phone rang. I didn’t recognise the number but answered in case it was my children who were at guides and scouts. It was a doctor from the out of hours clinic. He knew I was going for blood tests today and had looked up my test results. He asked if I was with anyone, it was this point that I thought something might be up. I reassured him I had my family here. At this point he said ‘Your platelet count is 7, I’ve arranged for an ambulance to take you in to hospital, it’s on its way’. WHAT? It was April 1st, but this was a dreadful April fools joke. What was going on?
I cancelled the ambulance as all I could think was I didn’t want my children concerned that there is anything wrong. I didn’t have a clue what a platelet count of 7 meant but I felt OK so it couldn’t be anything bad could it? I got my husband to phone my mum to come round to stay with the children for a couple of hours whilst we got everything sorted at the hospital and we jumped in the car.
We arrived at the hospital A&E and told the reception desk the story of the phone call, the platelet count and being told to come in. It was 11pm on a Monday night, and the department was full of people with bandages, blood, and vocal opinions. When I suddenly queue jumped straight through to the doctors I got lots of looks, after all there didn’t look to be anything wrong with me. The doctors took more bloods and sent them off for testing. My husband and I sat in very uncomfortable chairs wondering what all the fuss was about. After an hour or so I was moved into the acute admission unit and given a trolley to rest on whilst they waited for a bed. A doctor came to see me saying my platelet count was now 3 and they were waiting for the haematology on-call team to come and see me. By now my rash was above my knees on both legs.
Time passed with lots of people coming to see me, lots of blood test being done on me, a cannula, inserted, saline drips set up, cups of tea for me and my husband. Then in the early hours of the morning the on call haematologist said ‘We think you’ve got something called TTP, we are not certain’. I had never heard of TTP but they explained it was a rare autoimmune disease and answered our questions, though to be honest we were in shock and didn’t know what to ask. In my head I was thinking, OK so I’ve got TTP, its just an autoimmune disease, I’ve already got Rheumatoid Arthritis, how bad can this be? I remember turning to my husband and saying ‘they have this all under control, they know what’s wrong and now they are treating me. Its after 3am and the children will be awake at 7, please go home so you can be there when they waken up and tell them I’m fine’. We kissed and he left.
I was so incredibly tired I just wanted to sleep however I was being constantly monitored, drips were changed, saline became plasma, bloods were drawn, another cannula was inserted, I drifted in and out of sleep. I was assigned my own nurse who kept changing the drip bags and phoning the haematologist. I remember at one point she looked like she was going to cry and kept apologising that she needed to keep phoning other people about my treatment. Then she said something like ‘I need to keep checking in with the consultant because we are really worried you are going to die’. WHAT?! She couldn’t be talking about me, I’d just sent my husband home. After that I fell asleep, barely waking when blood pressures were taken, drips changed, drugs given.
The next morning my husband and children came to see me. I was delighted even though I hurt all over and had bruises all over my body where I had been lying on the bed. Months later they told me I had only been awake for a few minutes at a time and they were all terrified as I looked incredibly ill. I was moved to the haematology ward where I saw the kidney team, the haematology team, the acute admission team, it was like Piccadilly Circus beside my bed. Once on the haematology ward my memory gets fuzzy. Days passed. I now had a central line in, was on steroids and other medications and I started plasma exchange therapy. Honestly I couldn’t tell you if this was the next day or a couple of days. I drifted in and out of sleep. The first plasma exchange was an education. The plasma they took out of me set like a huge ball of honey in the bag. It didn’t look unpleasant but certainly didn’t look correct. I remember if I had a conversation whist on exchange it would cause my jugular to move and my blood to clot and they would have to start all over again. I became incredibly proficient at lying still not speaking. It was school Easter holidays. My family developed a new routine, my son used to cycle to the hospital, buy a coffee from the shop and bring it to my bedside whilst I went on plasma exchange. He would sit holding my hand, telling me things about his day, sipping his coffee and having some quiet time with mum. He was 14 at the time and this will always be very precious memories for me. I couldn’t chat back but felt so much love from him. He didn’t like coming when anyone else was around, he wanted me all to myself. In the evenings my husband and daughter would come to visit. She would climb into the bed with me and cuddle me. It wasn’t allowed to have others in your bed with you but all the nurses used to say they ‘couldn’t see anything’ and we decided that some rules are made to be broken.
On the 12th April my platelets had risen to 161 so I was allowed home for the weekend with strict instructions and carrier bags of drugs. I was self-injecting with Caplacizumab and swallowing lots and lots of pills. It felt amazing to be allowed out of hospital even if it was only for 48 hours. I was home. I had Sunday roast with my family and it had never tasted so good. I was so very tired but also very happy. I think all I did that weekend was eat and sleep.
I was re-admitted on the Monday morning and continued my treatments. The amount of plasma I was getting through was ridiculous, between 18 and 25 bags a day! By the 16th April my platelets had stabilised enough that I was allowed to be discharged to finish my treatment as an outpatient. I’d seen the last of plasma exchange and felt capable self-injecting my drugs. My platelets were 184 and I felt like I’d got this TTP lark sussed. I would have to go back in every week or so for some IV Ristuximab but that was OK, it was just a few hours not overnights.
The 2 weeks I was in hospital were an absolute roller coaster but I had progressed from being in a wheelchair and covered in bruises to going home. I had loads of cards around my bed and it took me ages to ensure I had collected them all up. I’d had an endless stream of visitors and was frantically messaging everyone to tell them, don’t visit, I’m going home. I wanted to thank each and every person individually for helping me through my ordeal. Thankfully it was over. It felt incredible. My husband collected me and off we went. I had to go back to the hospital each day for blood tests and checks but I was home. Each day took on a similar routine, I was mostly asleep, always incredibly tired but happy just sitting with my family or reading a book.
Then on the 20th April it all changed again. I couldn’t breathe. It was horrible and made me panic which made my breathing worse. We went back into the haematology department and they confirmed a lung clot. I was re-admitted overnight, taken off Caplacizumab, given different anticoagulant injections morning and night. After a night in hospital I was much better and again allowed home with a carrier bag of different drugs and strict instructions should my breathing get worse I needed to come straight back. I was booked outpatient appointments for blood tests and monitoring. But again I was home. I was so very excited. I’d had enough of the TTP roller coaster and was ready for the ride to stop.
On the 23rd my blood tests showed my platelets had dropped through the floor and I was immediately re-admitted. The next day my platelets had dropped even further. I was devastated. I had another central line inserted and again started on plasma exchange. By the 25th my platelets were even lower, it was a Thursday and I was informed I’d be in until after the weekend. It felt like such a backward step. My poor family were exhausted with the ins and outs of hospital. By the 26th April I’d used 244 bags of plasma during my therapy so far and knew I’d need more. The TTP roller coaster doesn’t run smooth and I got an infection. Temperatures of 40C, vomiting and violent shivers were added to my repertoire of symptoms but at least it meant I was upgraded to a single room, with en-suite no less! IV antibiotics were added to my routine cocktails of drugs. Thankfully I responded well to treatment and there was no obvious cause of my infection.
On the 28th April two of my brothers and my sister in law were running the London marathon. I was supposed to have gone to London to cheer them on but instead I was lying in a hospital bed having plasma exchange and tracking them on an app. I felt really low and sorry for myself. I think this was the first day I had a proper cry. By 262 bags of plasma my platelets were still only at 40, the next day they were 62. At 298 bags of plasma my platelets were 91. I knew I wasn’t going to be allowed out of hospital again until my platelets were 150+ and stable, but things were looking up, they were rising.
I’d started feeling better in myself, I didn’t look grey or yellow anymore and took photos of myself showing off my big puffy face from the steroids and my ‘neck jewellery’ (my central line), sharing them with my friends and family on Facebook. Plasma exchanges continued and my platelets continued to rise. By the 2nd May they were 126!!! I clotted the plasma exchange machine twice that day and on the 3rd May I was given a day off from exchange. I even got a lie in as they didn’t do my first obs till 6.15am. Living the dream!
The rest day did me good and my platelets went to 191! I felt awesome. I had a shower, blew dry my hair, covered myself in Coco Mademoiselle perfume and looked the best I ever had since the 1st April. I was allowed out for a couple of hours that afternoon with my husband. We went to the pub and had lunch. It felt incredibly naughty but really good.
On the 3rd May some of my tap dancing friends came to see me in hospital. They brought fruit and non-alcoholic cocktails and olives. We sat in my bedroom and chatted and laughed ate and drank. It felt like we were having a party and felt wonderfully normal. By the 4th May I’d used 320 bags of plasma, my platelets were 250 and again I was allowed out for the afternoon. On the 5th they were 307. If they continued like this I was getting out the next day. On the 6th it happened, I was discharged.
Thankfully this time the drugs kept working and physically I was doing really well. Emotionally I wasn’t so good. I was happy but exhausted and often an emotional wreck. My stamina was around zero. I still had carrier bags full of drugs that I needed to take. By the 8th May they started to reduce some of my steroids and I changed anticoagulants from injections to tablets. This was a very nervous time as, if this wasn’t a real remission, I could end up back in hospital on treatment. I found a message on my phone that I had sent to work that day saying ‘sorry I’m very tired still so I’m probably not going to be back before June.’ I actually love how naive and optimistic I was.
On the 10th May it was our wedding anniversary. Being an old fashioned romantic I went weak at the knees and collapsed onto the floor a few times. Our anniversary plans were changed to a sandwich at the hospital whilst I underwent lots of tests for bleeding on the brain, apparently a side effect of some of my meds. On the plus side my ADAMST13 results showed my levels were 100% so this was unlikely to be TTP related. Thankfully it wasn’t a brain bleed, I had positional vertigo and was sent from the hospital to a GPs surgery for the Epley manoeuvre, it worked and I stopped collapsing.
I was out of hospital but very anaemic and couldn’t actually manage a flight of stairs without becoming breathless and needing to sit down. Before all this I used to run regularly now I could only manage very short walks round the garden. Would I ever be me again? By the 15th May I decided to stay up late, 8.30pm, as I really wanted to see my daughter after her high jump training. It felt like another small step towards normality.
On the 22nd May I got great news. I was able to stop all drugs except the blood thinners. Yippee, no more puffy steroid face. Again I messaged work and said I thought I’d be back by mid June. May ended and I was doing well. I was still asleep by 7-8pm and not awake until about 9 or 10 am each day and having afternoon snoozes but I hadn’t relapsed. I was determined to get back to work so I booked an Occupational Health appointment at work on the 1st July. On the 21st June I realised that this was not very realistic. I tried driving and made it to the supermarket (a couple of miles) but was so exhausted by the level of concentration needed that I couldn’t drive home again and was asleep by 6.30 pm. There was no way I was going to be driving 70 miles to work in a week and a half. My GP talked some sense into me and signed me off until the 9th July and I cancelled Occupational Health. My GP wanted to sign me off for longer and said I needed to ‘stop being optimistic and start being realistic’. That was a really sad day. Was my life now just an endless round of constant fatigue? Would I ever get better?
On the 18th July I got tonsillitis. It totally knocked me for six and I felt like my energy levels were back to absolute zero. I felt really low. The GP actually made me smile at the appointment though. He discussed my medical history and said I’d make a ‘good study case’ and he’d ‘love to see if there was a genetic link to my autoimmune conditions’. As a molecular biology scientific researcher for over 20 years, and now working at a genomic research institute, I totally understood his enthusiasm.
Although I was TTP free, my fatigue continued and I was still sleeping for 14+ hours a night at the end of July. I certainly wasn’t going back to work any time soon. Life at this time was actually really lonely. I’d awaken after my family had gone to work and school and be asleep immediately after the evening meal This is where the worst bits of TTP were for me. Looking at me I looked well, too well actually as the steroids and inactivity had led to some impressive weight gain. However, I was exhausted. Speaking to people wore me out. I didn’t have the concentration to do anything. I couldn’t see I was ever going to get back to the job I loved, or even just having enough concentration and energy to live a normal life. I felt like I was really old before my time. I felt like life was over. Before TTP I used to go walking with the dog, running with my children and friends, hold down an important job that I loved. All of that felt way beyond my reach. Life as I knew it was over and I was quite depressed.
I managed to drive to work late July to see all my colleagues one day. It took me and hour and 15 minutes to get there. I went up in the lift to the office, walked in and burst into tears. I had no energy left. My boss took me for a cup of tea and some cake, I rested for an hour or two then came home. I know I wasn’t ready to go back. Although discharged and all my blood tests showed I was well, I was still tired and struggling with my memory. The summer months passed with me spending lots of time in the garden. By this time I had been off work for 5 months. I didn’t have any more paid sick leave. I needed to get back to work. I did have some holiday so took August off as annual leave and made a plan with Occupational Health for a phased return in September. I think I started back on 2 days a week, for a few hours each day. It was exhausting but felt wonderful to do something normal. I was probably 80% better but got tired so easily and my brain seemed to have been replaced with mush. I couldn’t remember things, even people’s names even though I’d know them for years.
Over weeks and months I slowly increased the amount of time I was at work and started to add in some working from home. Occupational Health were amazing, as was my wonderful manager and all my colleagues. However, even though my hours were increasing, my brain was still on a go slow. I wondered if I would even be able to do my job again. I seriously thought I should quit and find something else. When I discussed it with my husband he used to remind me what I had achieved this month as compared to last and I could see there was improvement, it was just slow. I took lots of annual leave over Christmas and had almost a month off. I spent the time resting, eating, reading and being with my family.
By January I had made huge progress and went back to full time hours at work. I could feel that finally my brain was beginning to work. I finally believed there would be a light at the end of the tunnel. In February I took back responsibility for my team as manager. This terrified me. What if they could see I still wasn’t fit enough mentally to do my job? I was still struggling but my memory and stamina was improving a little every day, and I had to keep reminding myself of that. Getting through each day took all the energy I had, but I was getting through them and proud of myself.
March came and COVID struck. Lockdown! Just as I was thinking I could get my normal life back, the whole world changed. I was now working from home full time. I’d been told to shield. I had my family beside me and was no longer feeling chronically tired every day. It had taken me almost a year but the old me was re-emerging and I could see life was returning to normal, well as normal as COVID lockdown could be. Spring was coming and my body was showing the signs of no exercise and lots of cake for over a year. I didn’t like it but knew I am not good at dieting, I like food too much! So I decided exercise would be the best way to get back into my clothes. I would try and run again, just round the garden. Our garden is a good size and one lap was about 50 m. Previously I used to run 10km for fun, surely I would be able to do this. I could barely make it once round the garden at a snails paced jog. However I was determined. I decided I was going to circuit my garden for 20-30 min, three times a week. I didn’t care if I walked, ran, crawled. I needed to do something.
The most amazing thing started to happen. My family started to join me on my running laps in the garden. I started to run for longer before walking and most importantly I felt good. Each day I ran round the garden I felt so very happy at my achievement. It was marvellous. I managed to run 3km one day and I felt like I had achieved it all. By this time there was a worn circle of grass in the garden. Details on shielding were changing and I started to go out round the streets at 7am with my family for my runs. Then on the 4th June I did it. I completed a virtual 5km run. It was slow, it involved as much walking as jogging but I did it, my first post-TTP 5km run in 46 minutes.
I’ve kept up my running and have increased my 5km speed by over 6 minutes. I’m never going to break any speed records but considering I was in a wheelchair unable to walk in April 2019 due to TTP and thinking my life as I knew it was over, I’m happy with slow. I am so very lucky to have the family, friends, NHS and colleagues that have helped me through this time. I still look for rashes every day, try and remember if I bashed myself when I notice a bruise and I don’t think that will ever go away. I really don’t want to have another episode of TTP. For now though, I’m going to enjoy every minute I can.
Hopefully some of my story will resonate with others and help those, like me, who discovered this illness the hard way, by actually getting it! I wouldn’t wish TTP on anyone but also know that I’m not going to let TTP define me, who I am or what I do. However, I will always respect it, never ignore it and always do whatever I need to help overcome it. But, whilst it’s gone away, I’m going to live and enjoy every minute.