7-8 years ago I was experiencing some weird physiological symptoms. At night I would experience night sweats, it happened most nights sometimes light but most times I would soak my pillow, what was really strange was that it only happened from the shoulders up, the rest of my body was normal. I would get nose bleeds for no reason, my nose would just start bleeding not super heavy and they would stop after 3-5 min. Headaches were the same way but usually required aspirin to make them go away. Cramps in my legs were common place, sometimes at night they would be so severe that they would launch me out of bed, and the more I drank the worse the cramps were. The worst part of this whole experience was my skin would itch, after a shower my skin would start itching; this would start about 3-5 min after the shower and last for about 3-5 min, it was very aggravating, actually horrible. I tried everything, oils, lotions, changing my diet, went to all natural fibers, short showers, cold showers, anti-histamines, nothing worked.
I had spoken to our unit Dr several times about my symptoms, finally in Jan 2009 he decided to do a complete blood count (CBC). When the lab conducted the CBC they freaked out!!! They could not get in touch with my Dr so they called me and asked me if I knew where he was, they would not tell me what was going, just that I needed to locate my Dr and come with him back down to the lab immediately. I found the Doc and off we went, the lab Director said that they needed to redo the CBC, that there was a mistake and they just needed a new sample. Well, the results were not a mistake, I had 3 times too many platelets in my blood (normal is 200,000-300,000 mine was 1.1 million) and it was causing all of the issues.
2 Days later I was in Landstuhl Regional Medical Center Oncology clinic learning all about a condition called Essential Thrombocythemia (ET) and not to be alarmed by being in the Oncology clinic, anything with bone marrow falls into Oncology’s realm. Over the next 3 days I saw numerous specialists and had more labs, x-rays and ultra sounds conducted than I really cared for. The ultra sound revealed that my spleen was/is 20% bigger than normal the Dr said it was because it was trying to filter out the excess platelets. Oh and let’s not forget the bone marrow biopsy, this needed to be done to confirm the diagnosis, it was not as bad as I was expecting, the Dr let everything numb up and then numbed some more. Now when they actually take out the core it is uncomfortable but not enough to make me cry or cus. For the next year I was in the clinic every month so that they could monitor me. I was put on a drug called Hydroxyurea, it limits the bone marrows ability to produce platelets as well as red blood cells (RBC) and white blood cells (WBC). It reduced/eliminated the headaches, nose bleeds, cramps and night sweats. It only partially reduced the itching but partially was better than nothing. I was told that ET could turn into Myleofibrosis but it was only about a 15% chance.
In 2011 I was sent to Italy to support a NATO event, I had 4 hours in the middle of the day that I had nothing to do, so I would run, go to the gym and swim after a couple of days at the pool in the sun I quit itching. Apparently some people with ET who have itching issues find relief from sun exposure. This is all great while in Italy, Belgium, well not so much so since 2011 I have maintained a slight tan year around and I do not itch.
I continued along, every 6 months going to the Oncology clinic for follow ups and every couple of years I would get a new Dr as they rotated out, as happens with the military. The good part about it was that every couple of years I got a second opinion. I expected to live the rest of my life with this ET and somewhere past 80 I would follow my ancestors.
Now we come to recent events, let’s say the last 9 months Jul 15 until now. Last summer I was spending a lot of time on my bike, it was glorious!! I was training for the “Leadville 100” Mountain Bike Race, I had a really great plan, I was consistent with training, I was eating well, and everything was coming together. In Jul I felt fatigued, this was coupled with a weekend of 100 degree weather so I took 4 days off, it was good, I needed the rest. I was definitely getting stronger but I was not really getting faster, I was able to justify this with the fact that what I really wanted was endurance for the 100 miles in the mountains. I thought it strange that people would sometimes comment that I looked pale but I wore sunscreen so maybe I looked a little paler. My recoveries were taking a little longer but as people are fond of saying “you’re getting old”, I chocked up the recoveries to pushing my body and maybe I was a bit older…………….
I went and did the race, it was a great time, lots of fun even though I was pulled out at the 40 mi check point for failing to meet the cut off time. On the climbs my heart rate was through the roof and I was having a very difficult time catching my breath, it was more than the altitude but I did not know it then. My only thoughts were that I would come out two weeks early in 2016 so that I could acclimatize better.
Last fall I was entering a bunch of 10k races and 45k Mountain Bike races and rides and the problems persisted. During the 10ks I would need to walk, especially if they had any type of hill, the same on the bike. I would do decent on the flats but when the trail got steep or muddy I would run out of air and my heart rate would start to spike. Slightly disconcerting. I also noticed that climbing a flight of stairs would cause me to breathe hard. I should have had a follow up in Oct but I was traveling and assigned to a planning effort so I put it off until the end of Dec. At my routine follow up appointment on 28 Dec 2015 the Dr informed me that I was anemic, so since I was on a drug that reduced my bone marrows ability to produce RBCs the Dr took me off of it and said come back in 30 days, sooner if things change.
28 Jan 2016 I went back to the Dr and I was even more anemic. This did not surprise me, I could feel it, the Dr scheduled me for a Bone marrow biopsy and to receive 2 units of blood on 29 Jan. The biopsy went OK, felt just like the first one. The transfusion was unique, it takes 2-2.5 hours to put 1 pint of blood in you. They do this slowly to limit any reaction to the blood product. I never really felt any better, I knew that I had more blood but I was still lethargic. The Dr said that I could get a energy bump or I might not even notice it, it all depended on how good the product was. So apparently I got a just ok product.
On 19 Feb 2016 the Dr called me and informed me that I had Myleofibrosis.
“I will stop and wait while you Google Myleofibrosis, the Mayo site is pretty good, reads well, lots of information presented in a digestible manner.”
Your back, that was fast. I know what the site says, things are not so bad. The anemia kicks my butt but I can still function, I drink less alcohol, and tend to be ready for bed by 10pm. My working out has been very limited, I just need to be moderate in physical activities.
Back to the 19 Feb telephone call. We talked about the anemia, how I was feeling and that I should give thought to maybe having a Bone marrow transplant (Bone Marrow transplant and Stem Cell transplant are both used but from now on I will use only “Stem Cell Transplant”). So I did some research on the internet to find out about Myleofibrosis and Stem Cell transplant. I also made a 14 Mar 2016 appointment with him to have a follow up and to get bunches of questions answered.
14 Mar 2016, follow up appointment, 1.5 hours, like I said, lots of questions. Another CBC and as I already knew, I was anemic again, worse than in Jan but I was not feeling too terrible, I could function all day but I just did not have interest in doing anything extra. I chose not to get a blood transfusion at that time, well, one week later on 22 Mar I was back to Landstuhl sitting in a chair getting 2 more units of blood. I had started to feel just different, fuzzy, eyes itching, a couple periods of light headedness. This time the product was much better. I actually feel normal.
Since 19 Feb I and the Dr have been working to start the process for a Stem Cell transplant. I have a consult in Seattle on 8 Apr 2016, this is an initial consult for a second opinion. I chose the Fred Hutchinson cancer research center in Seattle, WA so that I have family close by, Ryan being in Nebraska is the farthest one, most everyone else lives within 5 hours. I mentioned family above but I also have some friends in the area that I feel confident will come and visit during the recovery.
So let’s talk a bit about Stem Cell transplant. We will find a donor, siblings are the best chance for a match. When the donor is found they connect the donor to a machine, take their blood, filter it through the machine, the machine filters out the stem cells and then they put the blood back into the donor. Now it is my turn, they give me some high dose chemotherapy, they kill all my existing bone marrow and the fibers and the chemo kills a lot more. When my body is ready they will give me those stem cells and let my bone marrow rebuild itself. The hospital stay will be 6 weeks and then I will be able to leave the hospital but will need to stay close to the hospital for another 6 weeks and then if everything goes well I will be released back to the world and 12 months after the transplant I will be back to what has been called “my new norm”.
There is no timeline for any of this, first the consult and then establish the way forward.
I have returned from Seattle and my consult with Dr Rachel Salit of the Fred Hutch cancer research center. It was a productive visit, I spoke with the Dr for 2 hrs reference me and my body.
She is doing a medical trial using a drug called “Jakafi” prior to stem cell transplants. Jakafi shrinks the spleen, why is this important you ask, well it is like this. It is important that the Stem Cells given to me go to the correct place, in this instance my bones that need to rebuild the bone marrow. An enlarged spleen is a “greedy” spleen, when this greedy spleen sees these nice new, extra stem cells the greedy spleen will try to absorb as many as possible, the more it absorbs the less that go directly to my bones, so by shrinking it we will make it less “greedy”. I will take Jakafi for a minimum of 2 months to get the most benefit from the drug shrinking my spleen. Even if a donor is found on day one of the search, nothing will happen until I am on the Jakafi for 2 months.
By the way, I believe “Greedy” is a very technical medical term.
Stem Cell donor. It will take the Fred Hutch lab approximately 2 weeks to do an “antibody screening”, once this is completed they will start the search for a donor. If you would like to know more, google “HLA screening” for details. In the family a full sibling is the best chance for a match (25%), after that the percentages go down (less than 12%) for ½ siblings. LeAnn is not a candidate due to her own ongoing medical condition (for those of you who do not know my sister had an aneurism a little over two years ago, she is improving all of the time but it takes her off the list). If you are already in the registry then great, if not and you want to, stand by, I will find details to assist you in joining the registry. (If you want to try to locate a drive in your area you can try this is link to “Be the match” https://bethematch.org/
When the Stem Cell Transplant happens every one will know, not only because you will all hear me “whining” but many of you will be part of my “Care Plan”. I will not be allowed to check in to the hospital without having a care plan in place, that means that I will need to have a person designated for every day of the Stem Cell process. This period will actually start 3-5 days prior to the Chemo therapy, during this period I will be given medication to prepare my body for the chemo, I would love to be able to tell you what they are called and what they do but I can’t it is a secret……….. Ok, maybe not a secret but they were not interesting enough for me to write them down, I was stuck on the fact that my Dr was going to give me drugs that could make me sick before trying to poison me with chemo therapy. Care plan, I am not asking anyone to drop their lives and sit with me, although this is allowed. What I will need is people to volunteer time to come to Seattle and hang out with me, maybe take me to appointments, make me food, take me for walks (when allowed), run errands and well kind of be at my beck and call. My Mother, Linda, made the mistake of “bragging” that she was retired and could do whatever she wanted, so she will be the base of my care plan (Mom, thank you). We will take time later, closer to the transplant date to set up a plan that you can volunteer for dates or multiple dates (have I told you lately how awesome you are).
Dr Salit called me last night (Monday, 11 APR) to inform me that I was anemic (6.4 gm/dL), (during my consult with her she told me that she did not want me to go below 7gm/dL), guess I messed that one up. I was in Seattle on Saturday, 9 APR, and I told Nate (Brother) or Brandon (Oldest Son) that I was starting to feel a bit anemic, apparently I was lower than I thought.
The hemoglobin level is expressed as the amount of hemoglobin in grams (gm) per deciliter (dL) of whole blood, a deciliter being 100 milliliters.
The normal ranges are:
- Newborns: 17 to 22 gm/dL
- One (1) week of age: 15 to 20 gm/dL
- One (1) month of age: 11 to 15 gm/dL
- Children: 11 to 13 gm/dL
- Adult males: 14 to 18 gm/dL
- Adult women: 12 to 16 gm/dL
As you can see from the chart above, normal for me is 14-18 gm/dL, today as I said I am at 6.4 gm/dL, so I will drive down to Landstuhl and get a blood transfusion on Thursday. I do not feel that bad but the numbers say differently. The medical rule is to give blood transfusions to anyone below 7 gm/dL, I have been down to 6.1 gm/dL at my lowest, I feel pretty crappy at 6.1, 6.4 does not really feel too bad for me.
I think that is all of the new information. I am doing well, I feel good, I feel so good that last night I decided that today after work that I would go for a bike ride, that is now, not an option. As my Dr and friend Mike Henry has so eloquently explained to me, hemoglobin are like a train box cars that carry oxygen, less box cars, the less ability to carry oxygen, and if they are busy carrying oxygen to the muscles then they can’t carry oxygen to the brain and other important organs. I know what many of you are thinking, just how much oxygen could Pat’s brain possibly need………….
Thank you for sticking around and reading it all. Feel free to ask questions, what I do not know I will make up.
13 Apr 2016 (Wednesday) I drove to Landstuhl, Germany to the Landstuhl Regional Medical Center, this is the medical facility that I go to for the Oncology appointments. It is slightly inconvenient, the drive is 3.5-4 hrs, but on the plus side, it is a US facility, I am seen as a US service member so no insurance paperwork for the visits and any pharmacy products are just given to me. Kind of makes me feel special. I had to be there in the afternoon so they could type and cross match me for a blood transfusion 14 Apr 2016 (Thursday morning).
14 Apr 2016 (Thursday morning), the Oncology clinic did a Complete Blood Count (CBC), I was now at 5.9, wahoo, and this is the lowest I have been. I did not feel bad, my thighs were sore but no more than they have been in the recent past. Sore thighs have been indicators to me that I am getting anemic. So apparently I went through the last batch of blood (22 Mar 2016) faster than the Dr thought I would. Thursday afternoon, after the transfusions I was at 7.7, good but not great. Let’s see how fast I go through this.