To rephrase the title of John Green's book about teen cancer patients, I have a 'Fault in my stars'. Of course, I'm not a teen and by stars I mean my cells.
MPNs weren't always classified as cancers. When I was first diagnosed in 2002 with Thrombocythemia (aka Essential Thrombocytosis or ET for short), I was told I had a Myeloproliferative Disorder. And for about 6 years, I went through the acceptance that I had a yet to be determined gene 'disorder' that made me produce platelets at 4 times a normal human's. My closest friends said I was like, the Wolverine from Marvel comics with the ability to heal easily. I wish. Too many platelets can have the opposite effect. There's a chance the bleeding won't stop.
Anyway, my first diagnosis, following a trip to Hong Kong, showed that I had 1,200,000 platelets, where in normal peeps (people) it should have been around 300,000 platelets. My Hong Kong doctor, at the time, literally chased me out of the country, he arranged my flight home, which abruptly ended a fun vacation with close Hong Kongese frieds. Bummer.
My return to the US, put me in front of my American doctor in Sunnyvale, CA. This was the much better choice. His group immediately jumped on board and started testing me. That group of physicians measured my blood and found that they couldn't pinpoint the cause. This began a series of CT scans, Ultrasounds, X-rays, Philadelphia Chromosome test, late night calls asking me to go the hospital immediately, and finally a quite painful Bone Marrow Biopsy. The scans showed nothing, but the biopsy and the genetic testing that followed showed my cells were growing out of control.
They started me that week on a drug called Anagrelide, aka Agrylin. This drug sucked! I had to take it once a day at the same exact time as I had the day before. If I missed it by a minute either before or after, I would get intense migraines. My medical support remained awesome, but the drug, which I turned to calling Agrolin, because of it's aggressive side-efficts, didn't change. It got worse to the point that I wanted to quit, but change was on its way,
In 2005, I moved to San Diego for work and started visiting a new set of doctors at Sharp Hospital's Cancer Clinic. My doctor swapped me out of Agrylin and prescribed Hydroxy Urea, aka Hydrea. This medical group was good, but also realistic. They told me Hydrea had a useful life of 10 years before it cause the disease to convert. This would prove to be ultra important. He also expected that I would have a long and healthy life. What he didn't say was that the scientific knowledge about the disease was growing at a fast rate. What I didn't know was how much my life would grow otherwise.
I went about my life. Each day I committed to living life to it's fullest. I hiked, climbed mountains, camped with friends in a through hike of the Grand Canyon. Planned to try rock climbing when I had the money to afford it and so on. And I have kept my promise.
Three years under the care of my Sharp Hospital doctors, I learned that my MPD was now an MPN. My perception of the disease remained the same. Okay, I had cancer. Big deal.
My disease changed again in 2015 into a cancer, but by that point I had ended a three year stint of joblessness by earning a master's degree in Educational Technology from San Diego State University. This led to me getting job at 2-1-1 San Diego for a year and led again to working for a high-tech aerospace company where I've been employed for 11 years as of this writing.
I mentioned 2015 above because that year while under the great care with Sharp Medical Cancer Center, I learned that my underlying genetics for my disease. I had the newest identified mutation called the Calreticulin (CALR) gene at exon 9. Previously there were two other gene mutations on the books, which explained 70% of the cases. Thanks to science, they could now account for about 30% of the total MPN cases. This meant that I was part of three well known causes of the cancer.
But what is it that I have? The confusing part of MPNs is they are driven by genetics. These mutated genes express or trigger without cause or warning. Usually they're seen in older populations. In my case, having been at the start of the 'Young People with Cancer' epidemic, I got sick. My only explanation for me is that I'm an ACES, short for Adverse Childhood Event(s) Survivor. My ACES story deserves a blog entry all its own. Suffice to say that ACES have two things in common: high achievement and early disease expression. I had my first eye cataract removed in 2001 at 36 years of age, a year later I was diagnosed with cancer.
From 2002 to 2025 or so my body made lots of platelets. My treatments kept them low. In between years of dosing with Hydrea, my hematologist and I decided I try an injectable drug called, Pegasys (peginterferon alfa-2a). This drug gave me hope. The research said it could promise drug free remission and stop disease progression. I injected it for two years and went into remission. What I didn't know is that the second of Pegasys's promise was true if I kept using it, which presented problems of it's own for long term users. I took my remission and ran.
Then my disease returned.
I went back on Hydrea in 2022 after a short clinical trial of Bomedemstat, while living in Texas. This trial required my second bone marrow biopsy, which still hurt like hell. Bomedemstat didn't work for me. I moved from Texas to Colorado, met Suzanne Pelletier, my girlfriend and got back into adventuring in the outdoors and discovering my new state.
Fast forward to 2025, I started losing energy. I'd been taking Hydrea for 17 years (recall it's useful life of 10 years). Hiking, rock climbing, snow-shoeing, and cycling became breathless and muscularly painful chores. I experienced a progressively worsening anemia. Finally, in November while rock climbing at Indian Creek in Utah, I decided to reach out to my Colorado hematologist.
This disrupted my world again.
The anemia worsened and forced my hematologist to request my third bone marrow biopsy ever. I went about my hard-to-catch-air life. The results of the biopsy made me drop to my knees. My harmless little cancer had progressed (altered or mutated) into what's called Myelofibrosis converted from Essential Thrombocythemia, also known as Post Secondary Myelofibrosis. My doctor and a second opinion from another hematologist gave me mixed news. Both said I was terminally ill. I had reached the end stage of my cancer. Both doctors disagreed as to how long I had to live. The first said eight years. The second said I had three. Eight versus three. An eleven on a casino craps table. Not good.
By now I've asked myself to stop burying the lead. What is Post Secondary Myelofibrosis or MF? It's still an MPN. The difference between it and ET is having reached this point, the MF had worn out my bone marrow. This mutated form of my cancer turned my bone marrow into scar tissue. My body needed new ways to make red blood cells, hemoglobin, platelets, and white blood cells. My spleen and liver took over the role of producing my blood cells. Organs can do this, but they can't for long. Soon they would break down too and the disease would eat up my organs on top of my bone marrow.
I was dying and dying faster than I imagined.
I had to take matters into my own hands, so I did...
Wow i never realized what you have been going through before we became friends
ReplyDelete