My name is Holly Wiencek and I live in Powell, a suburb of Columbus, Ohio. I was a nanny for six years, but now I am 100% focused on school as a full time student at Franklin University. Here is my TTP story:
In 2004, as a student at the University of Dayton (age 19), I started to feel very weak, out of breath, and had a racing heart rate. I had been diagnosed with Lupus (and Mixed Connective Tissue Disease) 10 months earlier, and I thought that maybe these symptoms had something to do with that. I almost passed out on my way to my dorm and decided to drive myself back home to Columbus to visit my local hospital. Ten hours after I arrived in the ER, I was diagnosed with TTP. This was a terrifying diagnosis, as I had never heard of TTP. I bounced back very quickly and was back to school the following week for finals. This was the first time that I noticed memory problems. I noticed issues with word association and numbers. For example, I would say things like “It’s a pencil, but you click it like a pen. I can’t remember what it’s called” (Mechanical pencil). Another example is when I was trying to prepare for my final exams. I tried to make a schedule of the reading. I had X number of pages to read in Y number of days and I wanted to figure out how many pages I needed to read per day. I knew that I should easily be able to use division to figure this out, but for the life of me I could not remember how to do it. Luckily, these issues seemed to resolve themselves fairly quickly and my memory felt restored.
As I learned more about TTP, I thought that I would be one of the patients who only experienced it once. Unfortunately, 7 months later I relapsed and was back in the hospital. I knew exactly what was happening before the ER gave me the lab results. All the symptoms were back. My heart rate was rapid and my urine was very dark. Since being diagnosed with Lupus, I learned how to listen to my body and this helped me recognize the TTP symptoms. This TTP relapse enabled me to start on a trial for a drug called Cyclosporine, a drug that is known to keep the ADAMTS13 level from dropping. It took me longer to bounce back this time and I ended up being in the hospital for about 3 weeks.
On both of my admissions to the hospital, my platelet count was 8 (normal range 150-400), LDH levels were elevated around 700 and I was very anemic with a hemoglobin level between 4 and 6 (normal 12-15).
My third hospitalization was the worst one. I caught the onset of it before my numbers were too low because I was experiencing significant memory loss. I was taking Algebra at The Ohio State University and I had to get a tutor. I remember being in a tutoring session and thinking, “This guy must think I’m an idiot. I’m having trouble with simple division!” Then it hit me! I left the session and paged my doctor. I explained my symptoms, went straight to the lab, and was directly admitted to the hospital. My platelet count was 56 and my LD was not very elevated. Unfortunately, my levels got worse in the hospital and I struggled with torsades de pointes, a form of ventricular tachycardia, due to an electrolyte imbalance from the many transfusions and apheresis treatments. The scariest part was when I passed out and woke up with a team running in with a crash cart. Needless to say, this was my longest stay in the hospital.
Each time I experienced a TTP flare, I never felt sick. I was tired and pale, but I felt fine. That is something that was hard to get used to and is why it’s necessary to listen to what your body is saying! I also struggled with allergic reactions during plasma exchange and transfusions. We figured out a good cocktail of Benadryl and steroids before treatments which greatly cut down on the hives and other side effects. During the third hospitalization, I received a synthetic more processed plasma which cut down on allergic reactions.
I did not realize how much my illnesses had impacted my life until a few years later. I struggled a lot with school, which was unlike me, and I hid it from everyone. I was always tired, sick, and had memory problems, but I was ashamed to admit anything because I thought it was my fault. I came to realize how much my life was affected by Lupus and TTP. Luckily, each year since my last TTP relapse, I have noticed an improvement in my memory. A funny story: The winter of 2007, I was on the way to a cabin for the weekend with some friends. It was beautiful outside and I exclaimed, “Look at all of those frozen drips!!! It’s so pretty!” The car erupted with laughter and my friend said, “Ummm, those are what we call icicles…” It’s important to be able to find the humor in the situation. Knowing that you used to be smarter, is the hardest thing to deal with. I would say that I’m 95% better after 9 years. I still struggle with keeping my train of thought when giving detailed explanations, however, it seems to be more of an insecurity than an impairment.
I used to be nervous about moving forward with my life because of the relapses. I felt like every time that I went back to school, or started a new job, my TTP or Lupus would flare up. It took a few years without a relapse for me to feel more at ease that another TTP crisis wasn’t hiding around the corner. Now, I don’t fear a TTP relapse at all and am proud to say that I’ve been back in school for 2 years without any major health issues. If I do have a relapse, I know that I can deal with it! My main concern is keeping the Lupus stable! I have had a laundry list of issues related to Lupus in the past 11 years and this is my main concern. It has been determined that my TTP is a secondary condition to Lupus. If my Lupus is in a flare, the likelihood of a TTP relapse is greater. I was diagnosed with Lupus in July 2003 and it took about 7 years to get the Lupus under control. I had 3 TTP episodes within a year and a half (April 2004, October 2004 and July 2005).
I feel so lucky to have been treated at The Ohio State Wexner Medical Center by a doctor who is a TTP expert. I felt comfort and at ease, even in the scariest moments, because of the amazing nurses and doctors who treated me. I finally feel like my diseases are under control and I have been able to move on with my life.
If I could offer one piece of advice to new TTP patients, or refractory TTP patients, it would be to participate in research, (registries, clinical trials, etc.) if you can! It is research that will make a difference for us all!