February 1, 2024
Christine' story was initially written for another publication and she has decided to share it here too:
In 2013 I complained to my family that I felt very unwell. I thought I had the flu and my head throbbed. I had flashing lights, like a migraine, I took paracetamol and went to lay down. After a few days I began to feel a little better but when I got up to shower, we noticed that I had extensive bruising. I looked like I had been in an accident, the bruises were huge. My husband phoned our GP and thankfully he saw me the same day. He made me an appointment for early the next morning for blood tests, which went off to Musgrove Park Hospital that same day. At around 5pm I received a phone call from the GP, my platelets were down to 5 (the normal range is 150 to 400 approx) he said to go straight to Musgrove Park, and to take an overnight bag with me. Everything seemed unreal. Little did I know back then.
At Musgrove Park, more tests were carried out and I was admitted to the Haematology Ward. The next morning I was told they suspected I had TTP, a condition I had never heard of, and knew nothing about. I was told to call my family and was transferred to the BRI by ambulance with paramedics in attendance. I remember they wouldn’t allow me to walk from my room, and I was wheeled on a trolley. My confusion deepened.
Once at the BRI, more blood tests were undertaken. I had a Hickman line inserted and a Plasmapheresis nurse bought a machine into my room, high dose steroids and plasma exchange began. I had never heard of plasma exchange and the continuous beeps and blockages of the tubes only increased my anxiety. TTP was confirmed and explained to me, it’s caused by low Adamts 13, an Enzyme that is present in Plasma. The Adamts13 enzyme does not function as it should, leading to small blood clots in the blood vessels, low platelet counts and destruction of red blood cells. The meds and speed of these events muddled my brain. How could I have become so ill, so quickly? I can remember being taken for an MRI and Cat scan, being inside the tube. I had 29 daily Plasmapheresis sessions, in all, with staff coming from the Blood Donation Unit, each session lasting for hours and using bag upon bag of plasma. More steroids! Wait two days after 5 sessions to see what happened and then begin again when my platelets fell repeatedly. The disappointment each time was overwhelming.
A new drug, at that time, Rituximab, was introduced, and finally after 6 infusions my platelet level began to stay up on its own. In all, I spent 6 weeks on the Haematology Ward at the BRI. My kidneys were damaged but I had survived. Staff were amazing, steering me through the complexities of the treatment, the disappointments when the treatment seemed to fail and had to be started again. Endlessly patient with my panic attacks and tears. High dose steroids continued.
After discharge, I continued to have outpatient appointments every other day to begin with and my husband drove me to Bristol without complaint, but much concern. Even in outpatient appointments I had panic attacks, until gradually the steroids were withdrawn and my body and mind began to heal. Eventually I was passed back to Musgrove Park, and gradually over time the appointments changed to every 3 months. The relief of being told each time that the results were good was enormous. After counselling, I returned to work on a phased return. Our lives began to settle down.
In 2017 I cried again the day the Haematologist at Musgrove told me that my Adamts 13 had fallen again to 22, (normal range is 64 - 132 approx). However, this time the fall had been picked up early, 4 doses of Rituximab, once a week over a month, performed their magic and my levels rose quickly. I was able to holiday abroad and barely had a day off work. I thought I had put TTP in its place.
In 2019, my Adamts13 level fell yet again, this time to 2, alarmingly fast. I was admitted to the Haematology Ward at Musgrove Park for an infusion of Rituximab,but did not complete the first infusion. I had a florid relapse, I fell of the cliff. I lost my speech, collapsed, and was put on a breathing machine. I was transferred to the BRI again by ambulance with blue flashing lights. There is no doubt again that the Haematology Consultants saved my life. I came round in the ICU after 5 days, having been intubated, received Plasmapheresis, steroids, put on a kidney machine and other meds, while under sedation. Tubes were everywhere. I had been cared for by amazing staff. My first memory was being asked who the Prime Minister was.
After more daily Plasmapheresis and infusions of Rituximab I had scans, x rays and ultrasounds. My kidneys were damaged again and I had steroid induced diabetes. Eventually my platelet level becoming more stable, Caplacizumab, another new drug, was added to the treatment. After 3 weeks on ICU I was allowed home. Caplacizumab, along with steroids, was continued at home, my husband injecting me as I couldn’t hold the syringe steady. I began another round of outpatient appointments with blood tests, every other day to begin with, lengthening to 6 weekly. Again my Adamts13 levels rose and seemed to remain steady. Relief was huge. I had survived TTP again.
When Covid 19 came upon us, we drove to Musgrove with the appointment letters in my hand. It was so odd not seeing anyone on the road at all. Blood samples were taken in the Bumble Bus, in the car park, patients admitted one at a time. Consultations re results were taken over the phone. I was listed as extremely vulnerable. In between blood tests I was confined to the house, food delivered to the door and family and friends seen only on Skype. I thought I would go mad and I knew I needed a project. With the Consultant’s permission, and as some rules were relaxed for exercise, I began a virtual walk, from Lands End to John O’Groats. My husband and I took our daily steps around the block and then on Exmoor when allowed. It restored my sanity and we still walk on Exmoor regularly.
In 2022, my Adamts13 slipped yet again. I was admitted to the Haematology Ward, but with the frequent blood tests, TTP was picked up early and treated with more steroids and Retuximab. I didn’t return to work this time. TTP has left me fatigued and I find I need to have occasional rest days, but I’m alive and I try to maintain a positive outlook on life. I am a survivor!
There is no cure for TTP. I am in remission at present but relapses every 2 or 3 years seems to be my norm. Nothing can be taken for granted. Some patients have TTP once in a lifetime, others relapse frequently. Amazing staff at the BRI and Musgrove Park, watch over me and are available at any time. I am calmer, more accepting. I understand what TTP is and how it’s treated now and research is advancing bringing better outcomes. I continue to have regular appointments and blood tests, and I’m so grateful for that reassurance. Huge thanks to all NHS staff, my family, friends, and my long suffering husband. I wish everyone could be as fortunate as I am.
I decided to tell my story because TTP is so rare. Emergency treatment is essential. Few people have heard of it and education and early diagnosis saves lives. The Haematology Consultants treat so many complex conditions. TTP is not cancer as so many assume it to be, but an autoimmune disorder. I am one lucky lady!
