I am sixty years old and I live in Hagerstown, MD with my wife, daughter, older brother, two dogs, and a cat. Another daughter lives about an hour away in Baltimore, MD.
In July 2005, shortly after my 53rd birthday, we returned from several days camping at Assateague Island National Park. I am fairly light-skinned, so I tend to sunburn easily. My arms were covered in small itchy blisters that became ugly bruises when I scratched them. I felt extremely tired and very thirsty, so I did little more then relax on the couch and drink lots of ice water that weekend before returning to work. I assumed that I might have gotten sun poisoning.
On Monday morning, when I used the toilet before taking a shower, I noticed my urine was rust colored. My wife took me to the Emergency Room where, luckily for me, one of the nurses on duty remembered a patient with similar symptoms from a few weeks earlier. She told the ER Doctor of her previous patient, who had been transported to the University of Maryland Medical Center (UMMC) in Baltimore, the closest hospital with an Apheresis unit, with a diagnosis of T.T.P. Once my blood was tested and showed platelets at 12,000, I was taken by ambulance to UMMC.
While going through the triage process, I became confused and aphasic, unable to think of or say coherent sentences. Once I was admitted, my Hematologist began plasma pheresis therapy, exchanging my damaged platelets with donated platelets. She also began chemotherapy with a Retuximab drip combined with steroids. My platelet count responded quickly, and after a few days, I went home.
A week later, my wife noticed that I was acting strangely and called my Hematologist for advice. We began to drive to Baltimore, 75 miles away, but my ability to speak was rapidly deteriorating and we headed instead to the local hospital. My platelets had dropped to 8,000, so I was medevaced to UMMC. I had also been diagnosed with Cold Aglutenin Disease, which made getting an accurate type and cross match difficult, thus delaying plasmapheresis treatment for nearly 15 hours. I was told that my Hematologist had never heard of anyone presenting with T.T.P. and Cold Aglutenin Disease at the same time. After more plasmapheresis treatments, chemotherapy and steroids, my platelets finally reached normal levels and stabilized. However, either the delay in receiving treatment or the Cold Aglutenin levels, caused several strokes permanently affecting my balance and ability to walk.
Because I had responded so quickly to treatment and had plunged even more quickly, I was told that I had Chronic Idiopathic T.T.P. and might expect future relapses. For the next year, I had periodic blood tests to monitor my platelet count. I returned to work full time in Customer Service at a men’s clothing factory and then at a call center after the factory went out of business.
Five years later, in December 2010, I came out of remission and once again ended up at UMMC. I received more plasma exchanges and another round of Retuximab, but I was unable to maintain normal platelet levels. Another chemotherapy drug, Fludarabine, was tried but was also ineffective. Depending on what my platelet count was each morning, I had either one or two sessions of pheresis daily. Next, Vincristine was tried without success, then IVIG without improvement. In February 2011, after two months in the hospital, an older remedy was tried. I had a splenectomy, after which I ended up in the Medical Intensive Care Unit (MICU) incoherent and unresponsive, as I was having total organ failure. After more pheresis and whole blood drips, I slowly returned to being able to speak and was brought back from the MICU.
I then began Cyclosporine A, an immuno-suppressant drug normally given to organ transplant recipients to prevent organ rejection. My platelet counts have returned to the normal range even though my ADAMTS13 enzyme level is still severely deficient. I was released from the hospital on April 18th, 2011 but returned twice weekly for pheresis. My last session was on the Friday prior to Memorial Day 2011. In the period from December 3rd until Memorial Day, I received 146 pheresis treatments, averaging 15 donated units of plasma per treatment, which equals approximately 2,200 units of plasma.
Two years later, I am still taking 200 mg of Cyclosporine A daily and routinely have blood tests to monitor platelet and toxicity levels. Due to the strokes, I walk with a cane or a walker; my gait is unsteady as I fail to pick up my feet and so I trip and fall often. My Neurologist says the spasticity in my legs is caused by the message from my brain telling my legs to relax not getting delivered. I have returned to work on a part time basis as I tire easily.
The long hospital stay was hard on my wife and family because I was 75 miles away from home and even a quick visit was a three hour round trip drive on top of work schedules, school, and normal household chores. We are continually watching for any indications that I might be having a relapse. In addition to being forever thankful for the support of my family, friends, and hospital staff, I am truly very thankful that the 2,200 units of plasma were donated so that I could be here today writing this.
Keep your spirits and platelets high!