Hello all,

My last entry on this site was posted  August 15, 2020.  Shorty after posting that entry, Marty said when he was feeling well  he would write the next one.  

The following journal entry was written by Marty:

I'm still here. Nearly four years after being diagnosed with myelofibrosis (MF) I am forging ahead with my life as it now exists. Throughout this excruciatingly long update I will often refer to me or my, but know that I am blessed with a remarkable support group and I couldn't have gone through this without them, most especially my wife, Karen. It has been a shared journey. Engaged couples pay particular heed to the vow, "in sickness and in health". Quite a commitment.

A primer. MF is Greek/Latin-speak for a buildup of scar tissue in the bone marrow that affects the formation of healthy blood cells. The disorder occurs when mutations develop in certain genes in the DNA. It is rare. About 1.5 per 100,000 people, on average. Less than 50 people in the state of Iowa will contract it with one mutation. Even rarer is developing two mutations. Guess who drew both jokers from the deck? This guy.

MF is considered a cancer in that it prevents the bone marrow from producing an adequate number of healthy blood cells, it continues to produce immature red blood cells (reticulocytes, or blast cells) however, which are released into the blood. The blast cells eventually crowd out the healthy cells and jump the shark to leukemia. Many can survive with MF without treatment depending on age, relative health, disease aggression, etc.

I was diagnosed with MF following a routine X-ray of my knee that had been bothering me for a while. The X-ray showed nothing unusual about the knee itself but something else caught the radiologist's eye. The hollows of the large bones should appear black-ish. Mine appeared more grey indicating some sort of build up. Soon I was set up with an appointment at Gundersen clinic in La Crosse. My heart sank when I saw it was at the hematology/oncology department.

My doctor there was a soft-spoken younger man from Nepal. Dr. Uprety. He made a very complicated disease more understandable and took a real interest in my care. About the time I started my regimen in Rochester he also started a fellowship in the oncology department there. On occasions when I relapsed and was readmitted to the hospital he would come and visit. He even stayed with me for a while during the worst of Covid when no one else was allowed to visit. On one visit he gave us his cell phone number and said we could call each other now since I was no longer his patient but a friend. A friend, indeed.

MF's rarity and cunning makes it hard to diagnose. So, how do you find it? By eliminating every other ailment known to mankind, of course. For weeks I was subjected to a battery of diagnostic tests that explored every square inch of my body inside and out. The final determination was a bone marrow biopsy (ouch!) which sealed my fate.

Then followed:

Drs.: There is little that can be done to treat it.

Me: F**k

Drs.: But wait, there is a cure.

Me: Well, let's get after it.

Drs: Not so fast.

Me: Why not?

Drs.: Well, there is a high percentage of an "undesirable outcome" and 1 in 3 chance of mortality.

Me: Well, f**k me!

Thus, I was introduced to the Bone Marrow Stem Cell Transplant (BMT) - to be performed as a last resort.

Gundersen does not perform BMT's but UW-Madison, U of Iowa and the Mayo Clinic do. No offense to the UW or U of I,  but, come on, it's the Mayo Clinic. My first appointment was scheduled with a particular doc in BMT but when we arrived we were assigned a different doc. Dr. Litzow. The head of BMT at Mayo! CRAP! Don't get me wrong, who wouldn't want the best, but what did he see that he wanted to manage my case? My condition must have been unique enough that he chose me. As it turned out, I could not have been more fortunate.

My condition worsened to the point that we finally scheduled a BMT for early October, 2019. Most transplants are "autogenic", meaning your own stem cells are extracted, fortified and transplanted back into your own body as a form of immunotherapy. Because my stem cells were junk, I had to have an "allogenic" transplant, or someone else's stem cells. With an allogenic transplant rejection is much more likely. The aim is to find a donor who is the best match on the "human leukocyte antigen" (HLA) scale. Fortunately my sister Janet and I were a 10/10 match. I can never thank her enough for her willingness to donate some of her stem cells to me. HLA's are the little buggers that attack and destroy foreign objects in your body. A useful attribute unless you want to intentionally introduce a foreign object like someone else's stem cells.

So, just prior to transplant my immune system had to be subdued. This was done by carpet bombing me with various chemotherapy concoctions until my immune system was reduced to "near zero". I was told I would lose my hair but it would come back jet black. They lied.

Shortly after transplant I was "released" from the hospital but needed to check in twice daily for 100 days. We stayed those days at a transplant house close to the clinic called the Gift of Life. A far cry from home but a fantastic place where we met and became friends with some wonderful people. Sadly, some are no longer with us

The goal now was to allow my implanted stem cells to fully imbed and replace my own stem cells. Keep in mind, mine was unlike other transplants. Usually the host (the body) attacks the graft (transplanted object) and the host needs to be subdued to allow the graft to acclimate. In my case my graft (transplanted stem cells) wanted to attack the host (my body). Referred to as graft vs. host disease or GVHD. With constant monitoring and cocktails of some very potent drugs, I have been the embodiment of a life-or-death balancing act.

My implanted stem cells are driven. For some time, perhaps my lifetime, I will have to deal with minor skirmishes of GVHD. Hopefully I am beyond the battles that have accorded me ground ambulance rides to Gundersen Hospital in La Crosse to be stabilized then airlifted to Mayo's ICU to spend several days in the hospital for sepsis. Might I suggest the helicopter rides at the fair, if that's your thing.

At this point, test results show that my donor cells are 100% grafted. But I can't let my guard down. Like the disinfectants that claim to kill 99.99% of germs, it might be a rounding error that lets that 0.01% rear its ugly head and wreak havoc.

Being fully engrafted means that I share an identical DNA with my sister, Janet. In fact, I have her chromosomal makeup, too, essentially making me an involuntary transgender. But some of you suspected that already.

My doctors - Thomes in hematology at Gundersen and Litzow at Mayo - are pleased with my progress. I  need only see them every three months now and get blood tests every three weeks unless circumstances require more frequency. A far cry from testing once or twice a week and getting frequent infusions of red blood cells and platelets. Most of my blood counts are now near normal with the exception of platelets. They are increasing slowly but steadily. I am starting to rebuild myself, too. If the disease doesn't destroy you the cure will. I had my teeth extracted in May and will be fitted for dentures soon. I had the cataracts removed in both eyes and can see clearly now. I have received about half of my re-immunizations. I have not had my covid vaccination yet, though, because my doctors fear it will negatively interact with my compromised immune system. I'll be the guy you see wearing his mask. Please get vaccinated for those of us who cannot.

I won't even get into what a challenging year 2020 was. Best be forgotten.

Mere words cannot convey my gratitude to my wife, Karen, my children, Devin & Paige, Alison & Mark and Colin & Maria, family, friends and the medical professionals who continue to care for me and whom I am glad to say are my friends. I thank you for keeping me in your thoughts and prayers these last few years. You gave me the strength to persevere, especially during the very dark days when my faith was tested.

I'm not ready to wave the checkered flag yet but I can see the finish line from here. I'll see you, soon, in the winner's circle.

Marty