My name is Ernie Kowalski. My wife and I divide our year between our 2 homes. From November-April we have a home in Lakeland, Florida. May-October we have a home in Cochrane, Alberta, Canada which we consider our primary residence. My wife and I are Canadian citizens.
I’ve been married to my wife, Lorna for 30 years. We have no children. We both retired 7 years ago after working for the same company almost our entire careers.
I’ve been the victim of TTP a total of 4 times over the last 22 years.
My early days in the workforce, I worked as a heavy equipment operator. I was offered a welding apprenticeship which I did for about 10 years, then I was promoted to a supervisor position which I did for about 10 years before retiring at the age of 55. I was exposed to welding fumes, silica sand and diesel fumes on a daily basis.
1997 was my first experience with TTP, I was 43 years old. I had never heard of this disease up until this point in my life. At first I thought I had the flu, just a general unwell feeling, lethargic, tired, headaches, vision seemed to be getting blurry. We were celebrating our tenth wedding anniversary so I didn’t want to alarm my wife, I ignored the way I was feeling for a number of days. Finally after about a week of feeling this way, I awoke in the middle of the night. My lower back was aching and I had a feeling of impending doom. I woke my wife and told her I needed to see a doctor immediately or I felt I was going to die. We were staying on a remote island off the coast of British Columbia; there was an emergency clinic she took me to. Blood work was taken, the results came back, and looking at the doctor, we knew something was seriously wrong. He told me my platelet count was at 8 which meant nothing to me at the time. He told me there was nothing they could do for me, I needed to get to a hospital equipped to help me. I was taken by ambulance to Victoria, British Columbia. Blood was drawn, my platelet count was down to 3, and I was immediately taken by helicopter to Vancouver General hospital.
Upon arrival, I went to the operating room and a port was installed in my groin and I received my first plasma exchange. I was barely able to comprehend what was happening, I had a severe headache which they were giving me morphine to control. My condition was considered critical, my wife called my family, over the next few days all my close relatives came to see me, I was delusional from all the pain medication they were giving me so thinking back on it now, it’s hard to recollect exact details. It was at this time I was given the official diagnosis of TTP, I had never heard of this. I was told it was a very rare blood disorder and the plasma exchange (plex) was all that was keeping me alive. I received daily plex for about 10 days after which the line in my groin became infected, a new line was installed in my neck, and this line stayed trouble free for the remainder of my treatment for this first experience with TTP. I spent the next 3 weeks in Vancouver hospital with daily plex, I think I was getting 22, 500ml bags each exchange, my platelet numbers would go up one day and down the next.
My wife was stressed to the max, we were living in Fort McMurray at the time, and thank goodness the company we worked for supported us 100%. Once my platelets had stabilized over 100, the decision was made to medivac me to Edmonton University Hospital so we were closer to home. The doctor’s tried many different drugs trying to get the TTP under control; I spent the next 4 weeks getting daily plex. The platelet count started to consistently stay in the normal range so I did outpatient plex for another 4 weeks, 2 times a week. I was finally declared in remission and returned home in December, we had left home for our 10th anniversary in August, and I finally returned home in mid December, 4 ½ months from the time we had left home to celebrate our 10th wedding anniversary.
My wife had to go back to work during my time in the Edmonton hospital. She would come to see me on her days off, it’s a 5 hour drive for her, the company we worked for allowed her to use the corporate jet on many occasions (this was a great help), and they were nothing short of fantastic throughout all of this. The hematologists I dealt with were great; they were knowledgeable and answered all our questions on my condition. I had access to the internet so I started my own research on TTP; this is when I realized how rare this condition was. I tried to determine why this happened to me, was it something I ate, something I drank? I could come up with only one thing I did that I could say that was the cause. I’d broken my nose years ago playing hockey, as a result I always felt congested. I decided to try a nasal spray called Otrivin. I used this for a few years prior to our 10th anniversary trip constantly; I always had a bottle in my pocket. Even to this day I’ve never really checked what’s exactly in it. Whether this was the reason I got TTP for the first time, I can’t without a shadow of a doubt say for sure. I never touched this nasal spray again.
It was the summer of 1999, I experienced my second episode with TTP. I had what was called spontaneous pneumothorax (collapsed lung). While in the hospital, routine blood work was done, my wife noticed my platelet count was fewer than 100. She talked to the doctors about my history with TTP. They said they weren’t concerned and would monitor my blood, the next blood work taken my count had dropped to 60, she basically begged them to get me back to Edmonton. I was medevac’d to Edmonton. There, they installed the line in my neck and plex began for 7 days. I responded quickly this time and was released after a week of treatment.
The next 16 years of my life returned to normal, it took about a year to regain my strength. We continued to work, go on vacations and plan our future. TTP was forgotten, other than a work related problem with arthritis in my neck and a diagnosis of a heart murmur (aortic stenosis) which was being monitored by my cardiologist, I was healthy. I was told sometime in the future I would need this valve replaced. I had no symptoms and all the tests showed it was deteriorating at a slow rate. The prediction was possible intervention in my mid to late 60’s. However the arthritis in my neck became intolerable, I was prescribed Tylenol 4 to manage the pain, when I discussed the side effects of using so much of this drug the decision was made to change to Oxy Neo 10mg twice a day. I’m still taking this now. The decision was made to schedule surgery to fuse C4-C6. We arranged for this surgery when we returned from our time in Florida for April of 2016.
In February, 2016 I wasn’t feeling well, not suspecting anything serious I thought I had contacted some kind of virus and it would pass on its own accord. After about a week of feeling this way I decided it was time to seek medical attention. I went to a local clinic in Lakeland, Florida. I presented with chest pressure and elevated blood pressure. The doctors suspected a heart attack until blood results returned. My platelet count was at 10 and other numbers were alarming as well. It was then I told them I had TTP many years ago. More blood work was done and a diagnosis of relapsed TTP was confirmed. Due to the condition of my aortic valve which seemed to have deteriorated they were concerned about inserting the plex line. I was first given a bag of plasma, and then moved to the operating room to install the line in my neck.
During the procedure, my lungs started filling with fluid, an emergency requiring the insertion of a breathing tube had to be installed. I awoke later in ICU with a breathing tube in me and I was strapped to the bed, hands and feet. After regaining my composure and breathing without assistance the tube was removed.
I began daily plex for the next 7 days; my platelet count began to respond. I then did outpatient plex for the next 3 weeks twice a week. When I was released we immediately made arrangements to return to Canada. We were informed by our travel insurance company that they had determined I had not declared a pre-existing condition (aortic stenosis) therefore my insurance was null and void; they would not be responsible for any of the cost of my emergency in Florida. To say we were devastated is an understatement. We were forced to hire a lawyer and eventually worked out a settlement with the hospital. I won’t go into any more detail on this stressful experience other than to say it was a very stressful and expensive ordeal; something that we have to put behind us after almost a year of negotiations.
After returning to Canada I had seen my cardiologist, an echo was done and the results showed my aortic valve had deteriorated to the point I needed surgery soon. Surgery was performed on May 31/2016. All went well, I spent the summer recovering.
October, 2016, after getting the all clear from my doctors, we made plans to leave for Florida. We left our home in Cochrane, Alberta, Nov 6/2016. We were happy to be back in Florida, I was feeling good. Started working out at the gym, golfing and generally enjoying life.
It was shortly after Christmas I had been noticing a drop in my weight and a general feeling of not feeling well. This persisted until on January 1/2017 we decided to see a doctor in Lakeland, Florida. He recommended blood work which we did the next day. February 1st we went and got the results, we were in shock when he told us my platelet count was at 49 (normal range is 150-400)! I phoned my hematologist in Canada, he urged me to go directly to the hospital. We talked about my experience last year with the insurance company and I asked if he thought I was well enough to travel. He urged me to get home as soon as possible. I booked a direct flight and left the next afternoon. I went straight to the Foothills Hospital in Calgary, Alberta.
I waited until 4:00am before getting results from blood work, my platelet count was at 8. Things started happening fast, I was moved to the operating room and a line was installed in my next and my first plex (plasma exchange) was done. The plex continued for the next 2 weeks daily, my platelet count started to respond slowly. I was finally released from the hospital on February 24. I’m now at home recovering. I’m on a high dose of prednisone; I started a tapering off process over the next 4 weeks. I’m also taking medication for my heart as well. During my stay in the hospital I was given a drug called Rituximab, once a week for 4 weeks, it was explained to me by the hematologists to either prolong the time between relapses or possibly eliminate TTP from ever coming back again. We hope and pray this is the case. I never want to go through this again.