Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening, multisystem disorder with an estimated incidence of 2-10 cases per million/year. This hematologic emergency affects all racial groups and both sexes, two- to three-fold higher among females.
TTP is characterized by five features (pentad) consisting of thrombocytopenia, microangiopathic hemolytic anemia (MAHA), fever, variable neurological abnormalities (mostly confusion or severe headache), and renal failure. – CancerAdvisorTherapy.com
In A Pandemic?!
The global corona pandemic had just begun officially in Feb 2020. The whole world was gripped in horror of the strange new disease “COVID 19”. Schools were shut down, malls were shut down, people lost their jobs, curfews were imposed, lockdowns were announced. Fear, panic and uncertainty enveloped everyone – including myself, a hopeful college student with big dreams. Could things get any worse?
Well, apparently, they could. This is my TTP story.
Hi, I’m Sara, a 23 year old female with previously no major health issues. I was just like any other college student, studying for my exams, except that I was getting increasingly more tired and fatigued. It reached a point where I was incredibly exhausted to even climb out of bed most days. But I thought nothing much of it.
Strange Occurrences
When I brushed my teeth, I saw my gums bleeding unusually but again, I thought maybe I was brushing too hard or something.
Then about two days before my period, I noticed crazy things happening, my urine looked pinkish, which I thought was from my period starting early. I also noticed small red dots appearing all over my thighs, along with a large bruise on my lower leg. “I must have bumped myself somewhere without knowing,” I shrugged. I did mention the weird red spots to my mother, who dismissed them as nothing serious.
A day later, I decided to google my symptoms on the internet and they matched an immune disorder called ITP (idiopathic Thrombocytopenic Purpura), where the blood doesn’t clot normally. Symptoms include red pinprick sized dots on the skin, bruising and abnormal bleeding. I was too sick and fatigued to even be terrified.
When I started my period, it was super heavy that I needed to change pads every two hours. The period cramps were on a whole other level of pain. I took ibuprofen to help with the pain. It didn’t work. I could only curl up into a fetal position on my bed, lying in agony, waiting for time to pass.
A Bloody Mess
Hours later, I got up from bed to use the bathroom and was alarmed to see my period bleeding had got so bad that it was running down my legs and gushing onto the floor. “This is getting out of control,” I gasped.
I texted my gynecologist and she told me to take tranexamic acid, a medication for heavy bleeding, which actually reduced the out of control bleeding immediately. I felt so relieved that this madness was over.
My period continued normally for 5 days, during which, I was still feeling extremely tired like never before and I had a severe headache. No matter what pain meds I took the headache was still there! I thought to myself, “This has got to be the worst period of my life.”
I even visited my gynecologist and requested her to take a blood test to check my CBC (Complete Blood Count) to find out my platelet level, since I read that ITP means low platelets. CBC results showed platelets of 65,000 and HB (haemoglobin) of 6.5.
The normal range for platelets is 150,000 to 450,000 platelets per microliter of blood. The normal range of HB for women is 12.0 to 15.5 grams per deciliter.
I went home thinking I just had to take iron tablets and eat iron-rich foods to get my blood count back to normal.
However, when my period ended, my headache got even worse, the worst migraine imaginable. I lost my appetite and was vomiting every time I tried to eat. I couldn’t stomach anything and I felt so weak. The tiny red spots on my skin kept persisting, and I got newer bruises too.
Visiting Another Doctor
I told my parents, “I’m really afraid, I think something is seriously wrong with my body. I need to go to a doctor.” We were in the middle of a lockdown and my parents weren’t very interested in taking me to the doctor once again. Hospitals were tending to COVID patients, doctors were overworked, patients suffering with illnesses other than COVID were struggling to get treatment.
Finally seeing my deteriorating condition, my parents took me to a clinic. In the clinic, they tested my urine and blood again. While waiting for the results, I begged to be given pain medication through IV because my head was killing me. I received the pain IV, while waiting for my blood report. When the results came out, the doctor rushed to us and told me I needed to get a platelet transfusion ASAP because my platelet count was too low. She also said I need blood transfusions because I had severe anemia.
My parents weren’t happy with the idea of a transfusion (due to religious reasons) and they found some pharmacist at the clinic who actually convinced them that there’s nothing to worry, I should take iron supplements and amp up my diet with lots of iron and I’ll be just fine. My parents were so soothed by the words of the pharmacist guy over the words of the doctor. I was so woozy and dazed that I just sat there hearing them chatting with no idea what was going on.
My parents drove me back home. I was given vomiting meds, pain meds and iron supplements. I somehow managed to fall asleep.
The next day, I woke up with my heart beating at an abnormally fast rate and the catheter area where I was given pain med IV had developed a large crimson bruise. Other than that, I spent the day relatively normally, the vomiting meds helped me eat properly after days and I felt better. “Whew, at last, I’m getting better,” I thought.
A Stroke
But things took a turn for the worse in the evening when I felt my vision suddenly go hazy. That’s when the right side of my face started tingling and began to droop. I felt my right arm turning into jelly and my leg soon followed. I was losing sensation. I couldn’t feel. This would be my first ischemic stroke, as my doctor would later inform me. It is one of the worst things to ever go through. The pain is indescribable.
I somehow managed to call for my parents while trying to wave my arm. It felt like it was boneless. Another creepy thing is I also noticed a blue bruise being formed in my right arm as I was looking at it, and it was getting darker by the minute. It was so surreal like I was watching blue dye being injected into my arm.
My parents came into my room and I tried to speak but I began slurring and soon just couldn’t say a word. Did I mention how my head was killing me?!
I kept slurring and blabbing to my dad to take me to the hospital but he kept telling me it’s COVID curfew and we could only go in the morning. I thought that was crazy but hey, we were in the beginnings of complete lockdown so I believed him.
My dad thought I was experiencing severe migraines and had actually gone and bought me some heavy-duty meds targeted at helping migraine sufferers. They didn’t do anything for me.
I spent the whole night vomiting and vomiting and vomiting.
Admitted At Last
The next day, my parents drove me to a hospital and I was admitted. I was severely anemic and my platelets were down to 10,000, below which is danger zone.
I spent the next two weeks getting blood transfusions for my anemia. But to no avail. I would receive two units of blood and lose them the next day (blood in urine). If my HB was 8.5 and two units of blood were given to me to make it 10.5, the very next day, the CBC test results would show my HB reduced to 8 or less. At this time, my eyes became yellow and I was jaundiced. I’m still thankful for all the people who donated blood, and plasma that helped me survive today.
At this hospital, they kept drawing my blood daily for various tests, but still, the doctors didn’t know what was wrong with me. I had to suffer through two more days without knowing my diagnosis. I was being given nightly Vitamin K injections, in addition to blood transfusions. I would develop huge bruises at the injection sites and transfusion sites.
My primary care doctor was frustrated and finally told us to consult a hematologist. The hematologist came in after 2 days and examined my upper body checking for lymph node swellings. I guess to rule out cancer?
Finally, after hearing my story and symptoms as well as going through my test results, he told me I may have a rare autoimmune disorder called ITP. I thought to myself, “This is what I suspected 2 weeks ago after googling my symptoms.” But still, it was devastating to hear such news but I felt relieved that I finally had a diagnosis. I was started on the treatment – high dose steroids.
Despite starting treatment, I still felt unwell. I still had a fast heartbeat and high blood pressure. Luckily, my migraine headache and non-stop vomiting was gone. But I still felt like I was dying and felt terrible.
The catheters put in to give me transfusions were intensely painful, I felt like my arm was being cut into pieces. Every day, blood was drawn and the places the needle pierced turned into large purple and green bruises. I started getting huge new red dots on my arms.
Moon Faced
My eyes were very yellow. My face swelled up. I thought I looked like an alien from another planet. I remember feeling particular distressed by my yellow eyes. I felt like my eyes would never become white again.
The steroids made my whole body blow up. My cheeks became huge in a weird way, a condition called “moon face” in steroid patients. I recall taking a selfie on my phone and feeling horrified by the changes in my face.
My parents supported me in hospital but stuff kept getting worse. My vision got worse. I couldn’t see anything at a far distance. I was seeing double when I looked at objects far away. I was in disbelief when this happened. I felt very numb and dissociated. I kept my doctor informed about all my symptoms.
After another week of madness, the hematologist came back and told me that I did not have ITP. Instead, I may have TTP or HUS. By this time I was too fatigued to even care. He also said I should be transferred to another bigger hospital to receive another type of treatment involving replacing plasma.
Transferred to a Better Hospital
My parents then drove me to the bigger hospital and I had to go through every test all over again. I also had to get a COVID test, which thankfully was negative.
I received some plasma transfusions that night. The next day, I was scheduled for a jugular vein central line insertion.
What should have been a simple procedure turned into a nightmare. The young doctor doing the procedure messed up big time. It’s as if he didn’t know what he was doing. The anesthesia used didn’t work, I felt extreme pain when the cut was made and the catheter was pushed through. I felt my mind screaming. I threw up everything I ate. I couldn’t breathe. The doctor was unprofessional. He threw a towel over my face. This made me gasp for air. I felt like I was suffocating. My vision had black spots and I completely lost my hearing. I gasped, “I can’t hear! I can’t hear! I can’t breathe!” It seemed no one was listening. I thought, “This is it. This is how I die.”
I don’t remember what happened afterwards but my mom showed me a picture that she had taken while I was completely knocked out. I had so many bandages piled up around my neck. The white bandages were completely red as I was bleeding from my neck. I looked like I had just survived a gunshot to the neck. The pain in my neck was horrible. I couldn’t move my head. When I finally woke up at night, I couldn’t stop throwing up. I kept throwing up to the point where I couldn’t even breathe properly. It was horrible. They gave me meds for vomiting and pain through IV.
The next day, they took an X-ray to see if the central line was in the right place and guess what? It wasn’t. I asked them to please take it off because it was so painful but the nurse told me my platelets were very low and if they took it out I could bleed out and die.
A head nurse arrived and successfully inserted another catheter in my femoral vein, near my groin region. Through this catheter, I was given plasmapheresis, a procedure where a machine is used to separate the liquid part of the blood, or plasma, from the blood cells, replaced with new donor plasma, and returned back to the body. Plasmapherisis can have life threatening complications such as shock, drop in arterial pressure and sepsis.
Plasmapherisis
There was only 1 plasmapheresis machine in the hospital and I had to wait until the night to get my turn. As they reeled in the machine, I was fearful that I would get electrocuted from it. I know that sounds silly but that’s how I felt. Indeed when the procedure began, the current from the machine made me feel like electricity was pulsing through my entire body. I felt very cold, shivery and extremely itchy. I was given Benadryl to help with the itchiness. It was gruesome. But it was my only hope of survival. I struggled through it.
The next day, blood tests revealed that my platelets had not improved. Because it was lockdown due to the corona pandemic, plasma was very hard to find. Crowds were not allowed and it was hard to gather plasma donors. My next plasmapheresis was given a day later.
My CBC the day after the procedure was HB: 8.1 and platelets: 45,000. However, a day later they decreased to 19,000. My HB also decreased to 7.6. My liver test showed my LDH (liver enzymes) were high. Kidney tests showed elevated creatinine levels which means the kidneys are not functioning properly. I was still passing blood in my urine.
Throughout my hospital stay, I had plasmapheris for a total of 4 times. For some people, plasmapheresis gives them an immediate improvement. But for me my platelets were still low. It wasn’t working for me..
A New Treatment
Because I was showing no improvement, my doctor decided to introduce a new treatment – a drug called Rituximab which I read about on the internet as one of the first line treatments for TTP patients who don’t respond immediately to plasmapheresis. When given Rituximab, my platelets rose to 30,000. I was elated. I thought Rituximab was my miracle drug and I was going to be finally okay.
Alas, every time my hopes raised high, they came crashing back down again. My platelets decreased all the way back to 10,000 even with Rituximab. All the while, I continued being transfused with a total 5 packets of plasma. But guess what?
As the days passed, my platelets actually went down to 8,000 and even 6,000! Dangerous internal bleeding can occur when your platelet count falls below 10,000 platelets per microliter. This is scary information. Even my doctor was worried crazy. The third plasmapheresis also didn’t do anything.
A Month Later…
My HB was still 7. My period decided to show up. I was so scared I would actually bleed out and die during my period. My doctor told me not to worry and also not to take Tranexamic acid tablets which I was planning on taking to reduce heavy bleeding.
Thankfully, my period was normal – I even had a lighter flow than normal. However, I was still losing blood through urine, my mouth had dark bruises and the steroids were giving me extreme hot flashes throughout the day and night. Everyone around me was feeling cold but I had to keep the fan on at all times, which was weird because I had anemia and anemic patients are supposed to feel cold, right? I believe the hot flashes brought on from the steroids kept me warm – unbearably warm, that is. I was sweating round the clock.
A week later, I was given Rituximab again and this time it hurt my stomach so badly. I suffered from severe abdominal pain, diarrhea and other IBS symptoms. I had also developed a puffy moon face, had edema on my ankles and legs and put on a lot of weight.
My whole body was weak and I felt incredibly tired. My eyes were blurry and my mouth was full of oral thrush – all side effects from high dose steroids I was told.
Would a Cancer-Treating Drug Be My Final Hope?
My condition wasn’t getting any better. I developed new huge bruises on my foot and central line incision. I was starting to lose hope because the standard treatment for TTP – plasmapheresis, steroids and Rituximab were not helping raise my platelets.
That’s when my doctor prescribed another drug Bortezomib, which is actually a chemotherapy drug. I had read about how patients who weren’t responding to Rituximab were given this drug and it finally put them into remission. I prayed I would be one of those. By this time, I was used to being in hospital and I had accepted whatever outcome.
The next day, my platelets had risen all the way to 172,000! I was within the normal range! The day after, my platelets were 171,000 and HB was 8.6.
Bye, Bye, Hospital!
The doctor came asking me if I was ready to go home and I was in shock. I had spent 7 weeks in hospital. I thought I’d be in hospital for a few more days to make sure my condition was steady. But the doctor, who was on friendly terms with me after all my days of admission, laughed and said, “Get out of here. I don’t want to see you here. Go home!” Oh, yes. I was more than ready to!
My central line was removed at last and I was wheeled out of the hospital. Because of the central line, I actually had a limp and had to walk slowly because it hurt.
Back Home
At home, everything felt surreal. Almost all my bruises had disappeared. I continued having a fast heartbeat for many days. I was scared to do any activity that may cause me to bleed. I was worried that my platelets may fall back again. But I continued to take my steroids.
I also received Bortezomib three more times as an outpatient, one dose per week. Bortezomib came with its own side effects such as blurry vision, burning fever, chills and hives.
The hives were unbearable and I was given at least two different creams including hydrocortisone cream which worked temporarily but I still itched.
The only thing that seemed to give me relief was me applying a mixture I made out of aloe vera gel and a few drops of peppermint and tea tree essential oils.
A Clot in My Eye
I still had a disturbance in my vision. So I went to an ophthalmologist and discovered both my eyes had internal vitreous bleeding in them. So this was the internal bleeding that happened when my platelets were stuck on 10,000 and below. My hematologist told me that the bleeding must have been much worse when I was experiencing double vision.
The ophthalmologist actually recommended various treatments including injecting an expensive drug via needle into my eyes to remove the bleeding. A consultant at the clinic even scared me saying the blood needs to be removed or else it will scar and cause permanent vision problems.
I was so exhausted by this point I wasn’t in the mood for any more needles, leave alone needles in my eye! I felt like my vision was getting better with time anyway, so soon it would clear it up on its own. I couldn’t fathom myself having to experience the horror of a syringe piercing into both my eyes! I decided not to go ahead with the eye treatments to remove the vitreous hemorrhage.
Recovery
It’s now been almost three months since I was discharged. I have trouble recalling things sometimes, my energy levels fluctuate, I have anemia and my skin randomly itches all over my body. I was told these are some of the side effects of the drugs I took and the after-effects of TTP. I was already depressed before TTP and after TTP my depression got worse. Don’t get me wrong, I’m grateful I survived, but most days I feel low and tired.
I randomly check my skin to see if I’m suddenly developing red dots (petechiae) or bruises (purpura). I’m scared of bad headaches because I think it may be a stroke.
Life changed a lot. I put a hold onto my post grad education and my life choices have changed greatly. Many TTP suffering women get a relapse when they get pregnant and some die in childbirth. This has made me to reevaluate pregnancy and childbirth.
The pandemic also made it harder than ever. It is very difficult for TTP patients to get a proper diagnosis and treatment in time. Especially in a pandemic situation when medical necessities for TTP patients like plasma and blood are not easily available. I’m grateful that I was able to get a diagnosis and a drug that worked before I experienced irreversible organ damage.
What Caused My TTP?
I personally believe the cause of my TTP was stress and trauma. I went through a lot in the last two years before TTP struck me. I won’t go into detail but I was devastated by family-related betrayals and drama. I also had toxic friends.
One of the most important things I did when I came out of TTP was cutting out all of my so-called ‘friends’ because they added to my stress and depression, which I believe triggered my TTP.
The thing is, TTP itself was stressful and traumatizing for me. So at the end of the day, what matters is our ability to deal with the stress and trauma life throws at us in a peaceful, proactive way.
Final Thoughts
Life is fickle. We come to this world alone and go out alone. I think it is time for everyone to let go of things that make their minds and lives toxic including negative relationships and friendships. This kind of stress may trigger all sorts of autoimmune conditions in our bodies.
It’s not entirely possible to cut out stress from our lives. But we have to learn how to manage the stress without letting it mess us up. I’ll be talking about some ways to tackle stress, along with good relaxation techniques in my blog. Update: How to Lower Cortisol (Stress) Levels Naturally
I wanted to share my TTP story and general health journey so it could be useful to someone. That’s why I started my blog, July Blossom.
Thanks for reading, and I hope this was helpful to you. I wish you all the best.
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