Finding the Treasure: A journey of a Myeloproliferative Neoplasm patient from diagnosis to a Stem Cell Transplant
This is a story about a regular joe who happened to be diagnosed with a Myeloproliferative Disorder. This story is dedicated to all those who have traveled on a similar MPN journey that came before me, to those who traveled with me on my journey and those of you who are just starting the journey.
At the age of 38, I went to my primary care physician because I had been experiencing a chronic cough. I didn’t really feel sick nor did I think I had a cold or flu; just a cough.
My physician did the usual physical exams and ordered routine blood work. When the blood tests came back, it showed everything normal with the exception of my hematocrit. The lab report showed that I had a HCT count of 72%. When my physician reviewed those results, he was unconcerned as this HAD to be a lab error. No one has a HCT that high! So we had another complete blood count done. That report came back with a HCT of 73%.
So off I went to a Hematologist, and underwent all of the confirming tests. I was officially diagnosed with Polycythemia Rubra Vera. He did tell me, “If you have to have something, this is not too bad.” Which at first really put me off, but in the end, he was right.
At that point, I started doing research on the internet to understand what this disease was all about. What I read about PV life expectancy and the prognosis was not all that encouraging. Frankly, it scared me. But I went on with my active life with a full time career, and remained engaged in athletics as a weekend warrior with both water skiing and snow skiing as a Ski Patroller. My treatment plan consisted of therapeutic phlebotomies to try and reach a target below the 45% level.
Approximately, six months after my original diagnosis, I was enjoying a beautiful day of pristine water skiing until I was climbing aboard the boat. I felt a very sharp pain in my left chest. Hmmm, I thought, I must have overdone it waterskiing. But that pain did not go away, and I knew from my anatomy that there was only one thing in my body that the pain was coming from… my heart! I went to the emergency room and after being examined by the ER physician, it was determined that I had had an myocardial infarction. There I was, at the age of 38, in a cardiac ward.
After many more tests and consultations, it was probable that my heart attack was caused by my thick blood from Polycythemia Vera. It was at that point, I decided it was time to take this disease much more seriously than I had previously. I sought out an expert in MPN treatments and we began Hydroxyurea as the first line treatment.
During the course of my disease my treatment with Hydroxyurea worked well to keep my HCT counts lower. I still required monthly therapeutic phlebotomies to keep my counts near normal. I did try a course of regular interferon for about nine months to see if it would be a better treatment. The interferon performed as well as Hydroxyurea, but the side effects of interferon were much worse than Hydroxyurea and made me very weak.
In the tenth year of my disease, we began to see significant changes. My spleen which was typically about 5 cm below the costal margin began to increase in size. The spleen eventually moved into my pelvis and across my midline. I was also experiencing a significant amount of bone pain.
It was decided that I should undergo a splenectomy. I had the surgery at City of Hope in Southern California. The recovery from the splenectomy was relatively smooth for such a major operation. For those patients who have had a splenectomy, it is not uncommon to have a “normal” platelet count of up to 1 million.
However, my blood counts took a major turn for the worse. My platelet count reached a peak of just under 3 million. My WBC reached levels of almost 50 and I still had an elevated HCT level. My treatment at that point was to use high doses of Hydroxyurea, Anagrelide and Pegasys concurrently to try and keep my blood counts under control. Even with all of the prescription medications, we had a need to perform plateletpheresis to assist in reducing the platelet count. That was not a fun time in my journey.
It was determined that my best course of treatment was to undergo a reduced intensity allogeneic stem cell transplant (SCT) at City of Hope. I was blessed to have an exact, HLA sibling match with one of my brothers. One only has a 25% chance of having a sibling match, which I am so thankful for.
I have to share with you that the decision to undergo the SCT treatment was filled with anxiety and fear. As a husband and father of three young children it was a gut-wrenching mental challenge to determine if the risks involved, including death, outweighed the benefit.
I am now two years post transplant and I can happily share that I am doing very well. My doctor has given me the greatest complement in that my blood counts are “Unremarkably boring”, which means, the blood counts are all in the normal ranges. There are no signs of any a MPN disease.
I could write volumes on what it is like to undergo a transplant, the critical role of your caretaker and the extraordinary amounts of ups and downs that I experienced during the SCT procedure and recovery, perhaps, another time.
One amusing story to share with you happened after I returned home after transplant. The SCT procedure does make you lose all of your hair. One day, my seven year old daughter remarked to me, “Daddy, your hair is coming back.” Really, I thought as I excitedly ran over to the mirror to see. But after careful checking again and again, I did not see any hair. So I asked my daughter again, are you sure you saw hair growing? She replied, “Of course Daddy, look in your nose.” Sure enough, and as a joke from above, there was my new hair. I learned from that point, to always look up !
I did want to share a couple of lessons that I learned through this journey. The first and foremost is that your journey with an MPN will be uniquely yours. While you may share similar experiences with other patients, your success and failures with treatments will be your own.
Secondly, take this life event and find the treasure in it. My treasure has been meeting you. I am blessed to be in the company of such amazing group of compassionate patients and caregivers. If you look around, you will find the treasure. For example, ever notice that myeloproLIFErative has LIFE in the middle of it? Don’t let this disease hold you back for what you were made for in this world. Keep living and all the best to you!