July 24, 2024
In June 2023, I was nearing the end of my first pregnancy with my son and while I’d experienced the usual aching hips, back pain and morning sickness that accompanies many pregnancies, I’d had a fairly uncomplicated experience. That was until the 13th June. I attended my routine midwife appointment and as I stood in the car park, my eyes were filled with flashing white light and I realized that I couldn’t remember walking from my car to the doctors surgery. Tired from pregnancy insomnia, I assumed I needed sleep. However, upon returning home, again, I seemed to teleport from making my lunch to sitting on the coach, confused by how I’d got to the sofa, where I’d been and how I’d lost 20 minutes of time. Little did I know this was the first of several transient ischemic strokes I’d experience in June. I was dismissed from A and E as “heat stroke”. 2 weeks later, I woke at 4am and shuffled downstairs. Wide awake on the sofa, I felt like my entire body was involuntarily being pulled to the left. I tried to move. I couldn't. Eventually, I recovered some autonomy in my bodily function and, confused, I literally dragged myself up the stairs and climbed into bed. I was lucky that I woke up again. As soon as I opened my eyes, the dragging sensation continued so I called for an ambulance. I was told it was a 4 hour wait and to attend A and E myself. After an 8 hour wait, visits from confused obstetricians and blood tests, a general consultant appeared and told me I was “just anemic”. At this stage, nobody had spotted the alarming rate at which my platelets were trending downwards. 360,000… 220,000… 131,000… 100,000. TTP was in full affect by this stage and my symptoms suggested as much but, pregnancy bias from medical staff had pigeonholed me as, and I quote, “an overcautious first timer”. During each visit with medics, Dylan and I tried desperately to advocate for ourselves and communicate that something was wrong but, we were repeatedly told “it’s just pregnancy”.
My waters broke at 11pm on 17th July. I’d been waiting to become a Mum since my early 20’s and after years of infertility, my waters breaking didn’t scare me, I was cool, calm, collected and ready. Or so I thought. Arriving at the maternity ward, I had meconium in my waters so was quickly sent up to the delivery suite to assess next steps.That meconium potentially saved my life as if my waters had been clear, I’m told I would have been sent home to labour until things intensified. “We need to take some bloods, so do you want this one needle or shall I set a cannula up now incase we need to use it again?” asked the midwife. I brushed off the need for the cannula, not knowing just how many cannulas, needles and ports I’d become far too familiar with over the next 9 days. As my blood was drawn, it was fairly evident something was going awry. “Gosh, you’re a bleeder aren’t you!” the midwife exclaimed as the hole in my arm painted the floor scarlet red. I laughed but, immediately, my gut told me something was off. I’ve had countless blood tests over the years and that’s never happened before. Combined with the weeks of dismissed symptoms in the lead up to this moment, my brain quickly connected the dots that something wasn’t as it should be. 30 minutes after my blood results returned, a flurry of new faces appeared. Lead consultants, senior midwives, anesthesiologists… they tried, with varying degrees of confidence, to explain that my platelet count was dangerously low and my baby needed to arrive as soon as possible if either of us were to survive. The sentence is merely a summary of the carnage that ensued following the medics realization that something was seriously wrong, but, I’m still notable to articulate the conversations we had during that moment. The trauma is too raw. I remember signing waiver forms that accepted death may be the outcome of an emergency c-section and pleading with them to do everything they could to save my baby and I. “My partner doesn’t deserve this” I cried as I let go of Dylan’s hand and they wheeled me and my unborn son down to theatre.
In the days that followed our survival, we were blessed to be allocated a phenomenal care team including a consultant called Dr Rutter. Dr Rutter heard about my case and on her days off, spent hours looking at my labs, consulting with colleagues and contacting specialist centres. “You don’t fit any of the usual boxes for any of the usual diagnosis but, your platelets just aren’t staying up. But, we’re not giving up”. My labs indicated I was seriously unwell but, I was still conscious, cognizant and capable of most daily living skills despite surgery. This presentation was confusing the consultants.I had 3 platelet transfusions as it was suspected I had HELLP syndrome but as the days ticked by, my platelets continued to crash, I was beginning to turn yellow and a midwife noticed rash like bruising across my chest and back.
Following an all clear liver scan on the morning of the 22nd of July, 10 medical staff arrived and stood at the bottom of my bed. Their faces painted the picture and the words they spoke filled the gaps. The Director of Nursing sat on the edge of the bed and gently said, “Sophie, we need to get you to Birmingham. We think you have something called TTP and we need to get you there now.” Confused, I asked “Are Arlo and Dylan coming with me?”. The medics looked at each other. “No, you’re going in an ambulance now, Sophie. This is very serious.” I began to sob. Iasked them if I’d caused this. I asked them what I’d done wrong during my pregnancy as I’d been so careful. I listed off all the vitamins I took. All ofthe measures I had in place to keep me and Arlo well. Meanwhile, a whirlwind of activity was taking place around me and I was wheeled into the back of an ambulance, feeling emotionally numb. I’ll never forget racing down the M6 with Cher’s “Believe” blasting on the radio, it felt like a weird sitcom come to life. The paramedics were doing their best to maintain a positive attitude,cracking jokes, asking me “would your rather?” questions but, having worked in health and social care for well over a decade, I knew what they were doing.They knew how desperately unwell I was and were doing their best to keep me from dropping unconscious and needing intubation in the back of the ambulance.
Upon my arrival to the QE Hospital in Birmingham, I was met at the reception by a consultant who immediately transported me to theatre. I had a vascular catheter inserted into my groin and I remember taking comfort in the fact that the surgeon was from the same town as me. That sounds somewhat silly, but, arriving to the QE was like crash landing on an unknown planet, so that sense of familiarity was significant in that moment. I was eventually placed in a side room, greeted by my Dad. I was never so grateful to have a parent by my side and I knew that my Mum was by my partners side, helping him navigate sudden solo parenting as a first time Dad.A wonderful nurse called Claire appeared and within minutes, she explained what plasma exchange was and hooked me up to the machine that ultimately saved my life. As the plasma exchange machine swapped my swamp shade plasma for new,Claire tried to explain TTP to us. At that stage, flight or fight mode hadtruly activated in my mind and I was almost oblivious to how seriously ill I was. I just wanted to see my son and drink a bottle of Lucozade! After weeks of being dismissed by medical professionals, I was also finding it incredibly difficult to trust the people around me who were trying to help. However, from the moment I met Claire, it was the first time in weeks I’d felt safe. Again,she was from the same town as me and in between the formality of changing plasma bags, we chatted about how much we love a bargain from the middle aisle at Aldi. The entire experience felt like a fever dream.
Eventually, my consultant, Dr Percy arrived and introduced himself. I now know that without his intervention and discussion with Telford hospital, I wouldn’t be alive today. I owe my life to his diligent efforts and will forever be grateful to him. He told us that while TTP is rare, it’s not rare for it to be triggered by pregnancy in women my age. He explained that the next 48 hours would determine steps in my treatment plan and reassured me I’d be in safe hands from here on out. He wasn’t wrong. Over the next 5 days, the team at the QE including Dr Percy, Claire, Holly and Lida re-stored my faith in the healthcare system and have been nothing short of phenomenal since my diagnosis 12 months ago. I had 4 days of plasma exchange as an inpatient and was incredibly lucky that by day 3, my platelets had gone from 20,000 to 170,000. I received the gold standard of care in the shape of steroids, Rituximab and the miracle drug that is Caplacizuzimab. Upon discharge from hospital, my ADAMTS13 levels were still undetectable but, I was assured we were on the right track.
Navigating 4 weeks of rituximab outpatient treatment as a freshly postpartum, first time Mum is the hardest thing I’ve ever done. My mental health took a nose dive and there was a moment where I was quite literally picked up off the floor by a nurse. I lived between the mental limbo of wanting so desperately to be at home with my son but,whenever I left the hospital, I was convinced I would die. My ADAMTS13 levels slowly began to creep up from 0 to 1 to 7 to 56, finally landing at 99%. This confirmed that I had immune mediated TTP, not congenital TTP as first suspected. I’ve now been in remission for 12 months and whilst I’m still adjusting to my new-found identity as a TTP patient, it doesn’t define me in the way I anticipated it would when I was newly diagnosed. Another TTP survivor told me that TTP simply makes us a different version of ourselves, the 2.0 edition. And they’re right. Do I hate the 3 monthly blood tests? Yes. Do I fear relapse? Absolutely. Am I hypervigilant to bruises and other ‘benign’ bodily experiences? To a fault. But, I’m also living life. I’m a Mum, newly engaged to Dylan, back at work as a psychology practitioner, moving house, as well as building new friendships with other TTP survivors. If you’re reading this as someone who is newly diagnosed, there is life after your acute episode. Life certainly looks a little different but, getting a second chance at it is powerful.
Since my diagnosis, I’ve dedicated myself to living. Not just existing, but, finding joy in the tiny, in the still, in the ‘mundane’. You’ll no doubt read other accounts from people like me, who have danced the dangerous line between being here and not and it’s safe to say that many of us would agree, facing your own mortality is a powerful teacher. The fact we even exist is a miracle in itself,we’re literally made of luck and stardust! TTP taught me that that dying, is easy. Living on the other hand, and I mean, really, REALLY, living takes effort. But, it’s worth every. single. second.
As the 22nd July 2024 rolls around, I’ll be reclaiming the date of my diagnosis by leading a charity fundraising trek to raise funds for the TTPNetwork. The support and advocacy they have offered to me and my family since diagnosis has been monumental in empowering me to live as fully as I can and I hope I can continue to turn my pain into purpose through raising funds and awareness of TTP. I truly believe raising awareness of TTP can save lives and improve future outcomes for all rare patient groups. I am forever grateful to the love and support from Dylan, Arlo and my family as well as the expertise and kindness from my care team at the Birmingham TTP specialist centre and the TTPNetwork. I’d also like to express a huge amount of gratitude to the other TTP survivors who helped me make sense of my diagnosis when I was first diagnosed as well as the fellow patients I now proudly call my friends.
