I’m Back (for now anyway)

Somebody asked me why I stopped posting. My last post was a little over  year ago. Most of the reason was that I contracted an extremely rare type of bone marrow cancer called mastocytosis. Basically, it was like getting hit in the jaw with a Mike Tyson punch.

The disease is insidious. It replaces red blood cells in the bone marrow with mast cells. It produces a march toward death that is sort of like a snowball starting down a hill. At first the snowball is small and of little concern, but eventually it picks up power and smashes anything in its way. Red blood cells disappear and mast cells fill in. Your organs fail. Not a great death by any means.

Everybody has mast cells. They release histamine and other substances during inflammatory and allergic reactions. Hives are a good example of your mast cells going to work.

Healthy people have less than 1% mast cells in their bone marrow. I had 70%. The red blood cells and the oxygen they carry were being demolished by the mast cells. If you’re wondering how this happened, one of my genes, the one that controls mast cells, was corrupted. Instead of keeping me at the same levels as other people, the culprit gene was destroying me from the inside out. It had moved to the blood organs – spleen and liver – and eventually would have caused all my organs to fail.

I realized something was seriously wrong at the end of the 2017 basketball season. I was dragging myself up and down the court hoping nobody noticed. After I finished the season I went to the facility considered the best hospital in the world for respiratory problems – National Jewish Hospital. Trust me. You go inside, you see the ugliness of lung diseases. The oxygen tanks, people shuffling across the carpet with the little strength they could muster, small babies and their anxious parents.

I had ballooned in weight to about 230 pounds (I’m 5’10” tall). A lot of it was retaining excess water. My stomach was pushed out as the spleen and liver grew in size to almost double the normal size. I was an average size person hiding in what looked like a fat man.

I had a baseline physical and then they started testing. I had over 20 different tests. Poking and prodding and holding my breath. They asked me if I wanted to check in to the hospital. That way they could wheel me to another test at a moment’s notice. I’ve got a thing about sleeping in my own bed. I turned down the offer. Turns out they really wanted to keep me because they thought I might kick the bucket.

They moved me around to see a couple of other doctors. The doctor most familiar with auto-immune diseases posited that I might have a mast cell problem and decided to send me to the University of Colorado Cancer Center. Luckily, National Jewish didn’t have anyone on staff that could do a bone marrow biopsy.If I thought the waiting room at National Jewish was depressing, the cancer center had it beat by a city block. They drew blood – every cancer ward draws blood like the society of Nosferatu.

The result was grim. The doctor I was seeing at National Jewish had me come in for an appointment. He pulled no punches. My bone marrow mast cell level was 70% instead of the normal 1%. Without treatment he didn’t expect me to live more than four more months.

As far as the doctor knew, the only treatment available was the same as for leukemia.  A bone marrow transplant and chemo. Even so, the doctor warned me it wouldn’t give me more than two years. Better than nothing, but still too much time to worry.

Believe me. There were a number of times in my life something happened that should have killed me. I won’t list them, but cats were jealous of how many lives I had.

I went home and tried to figure out how I was going to tell everyone I had a terminal illness. Would it be best to be upbeat or dismal, casual or morose? As I thought about it the phone rang. It was a doctor from the University of Colorado. He said that there was one space open in a drug trial for a chemo medication called BLU-285, developed specifically for what I had.

Mast cell leukemia is highly uncommon. Maybe no more than a tenth of a percent of the world’s population had it. Out of that an even smaller number had it anywhere near as bad as I did. The second highest patient only had 20% mast cells compared to my 70%. Turns out most people with the disease don’t get to 70% because they die first.

They University of Colorado hospital retested me (I’ve had seven bone marrow biopsies to this point), confirmed the initial findings, and handed me 50 pages of paper that laid out my commitments to the study drug (like my family wouldn’t sue them if I died).

What the hell. I signed up. In May 2017.  I went to the hospital and took my first dose.

They started on 400 mg of the chemo drug every day. It seemed like I was in and out of the hospital every other day for a month. The chemo drug was powerful. I had daily nausea and threw up a few times. I didn’t want to throw up because if it happened too often I’d have to drop out of the study.

Sometimes the nausea was embarrassing. I went out to breakfast once. I ordered pancakes. The waitress put the plate in front of me, and I immediately bolted for the door and puked in the parking lot. Somebody came out and asked me if I was ok. I nodded and when my stomach settled I went back in and ate one pancake. I was happy about getting the pancake down, mainly because I had no appetite and a lot of food I loved to eat made me want to throw up. In five months I lost 40 pounds.

In October I went to New York. I love New York. I go a few times a year. I walked around and looked at many of my favorite places. I sat on a bench at Bryant Park and watched people play bocce ball. It was like I was a kid watching the adults throw bocce balls and argue. I finally decided the cure was far worse than the disease.

I got back to my room and picked up the phone to call my doctor and let him know I couldn’t tolerate 400 mg every morning. In addition to the physical effects, it was also affecting my mental acuity. It was like having Alzheimers. Imagine your brain was filled with filling cabinets, and when someone asked you a question you’d go to the right cabinet and pull out the answer. With me all the cabinets were locked and I couldn’t get into them. It certainly affected my ability to handicap. I couldn’t concentrate on a horse much less a race. I’d ask what day it was. Whether or not I ate lunch. Often I couldn’t sleep. Needless to say I couldn’t write either.

The doctor listened to all that I had on my mind and said, “Your last bone marrow test and MRI came in. Your mast cell count is back to normal and your liver and spleen have shrunk back to their normal size.” He allowed me to drop my dose to 200 mg a day and convinced the insurance company to pay for a daily anti-nausea drug.

I made up my mind that I was going to live a normal life. It was hard. I had done very little exercise, and I had spent a lot of time indoors mostly in bed or on the couch. One of the other horrible side effects of the drug is that I can’t go in the sun for 10 minutes without turning red. I would even get a sunburn driving the car on a sunny day. So in 2018 I wore long sleeves, gloves on my hands and a wide brim straw hat and went golfing. My arms and legs were like jello. It was going to take a long time to get into shape and I still have a way to go.

All the hair on my body turned white, at least the hair that didn’t fall out. I can still grow facial hair (but I’m clean shaven now) and enough of the hair on my head stayed so that I don’t have to wear a bandana.

I’m now considered to be in remission, but I still have to take chemo daily. 200 mg. I asked when I could get off the drug. The answer was one I didn’t want to hear. It seems the drug didn’t kill the corrupt gene. In simple terms, it keeps the gene from doing whatever it does to take you on a journey toward death. As long as I take the drug I’m protected. If I stop I only have a short time to live. Easy choice, but I try not to spend too much time thinking about living under the BLU regime or dying without it. I learned a lot about myself. It isn’t death that scares me. It’s dying leading up to death that does.

How did I get the disease? The doctors assured me it is not something that was programmed from birth. I was most likely exposed to something environmental that corrupted the gene. The doctors assured me my kids didn’t have a genetic predisposition to the same cancer I have.

I thought a lot of things we were all exposed to 50 years ago. Bad water, bad air, asbestos – who knows how I was targeted. But as I said, I’ve had plenty of experience dodging death. Maybe sometime I’ll write about all my escapes.

I’ve adjusted the best I can. I golf, I ride horses, I do my volunteer work with autistic kids, I walk the dog to give me an incentive to rebuild my muscles, I ice skate indoors, although I don’t play hockey. I ride my bike and I’m lifting light weight. I also do other things I’m too discreet to mention.

How long will I live?  Who knows. Still, given my test results I have reason for optimism.

One last thing. If not for Twitter and Pace Advantage I might have gone nuts. It was great that I could talk to people from my living room. I also realized how great my friends were. It all helped me to get through the worst of it.

I have two more blogs I want to write but not today. I’m not done spreading my opinions yet.

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