Watch and Wait MDS Doesn’t Hold Me Back
My story begins after a routine, annual check-up with my doctor. I was 49 years young. Overall, my exam was uneventful, but a few days after my examination I received a call from the doctor’s office asking me to return for a second blood draw.
I really wasn’t concerned until my doctor said he couldn’t explain why my counts, particularly for my platelets and white blood cells, were so low. The anxiety began to climb when he referred me to a hematologist/oncologist. Healthy people don’t need to go there, do they?
Search for Answers
During that first trip to the hematologist/oncologist in December of 2013, a bone marrow biopsy was performed and was found to be normal. Unfortunately, it did not shed any additional light on my condition and my doctor was unable to pin down a diagnosis. I went through a battery of tests. I tried a few different local doctors and spent those years receiving generic diagnoses of pancytopenia, thrombocytopenia, neutropenia, anemia and even copper deficiency. The cause for my lowered blood counts eluded my doctors and frustrated me.
Although I was frustrated, I didn’t let my condition slow me down. I still ran 6-8 half marathons and 10K running events per year, joined a kickboxing gym, started biking and generally became more active than before. This wasn’t particularly easy. I was slow and never in contention to win a race, but always had just enough energy to finish. I still didn’t know what was wrong with my blood, but at this point in my life I felt relatively healthy and was in best shape of my life.
Fast Forward: In the fall of 2016, my neutrophil counts really started to decline and my hematologist wanted to start me on the steroid prednisone. He was worried that my counts (ANC 0.2 X 10*3) were so low that I was at serious risk of getting potentially life-threatening infections. As much as I was concerned about infections, I was less interested in being on a steroid just to see if it worked. Consequently, my wife and I packed up and flew to Moffitt Cancer Center in Tampa, Florida to get a second opinion.
A few blood tests and another bone marrow biopsy later, my new doctor called with the results and a new diagnosis – de novo, hypocellular myelodysplastic syndrome (MDS). The subtype is refractory anemia with excess blasts, also known as RAEB. He then told me my Revised International Prognostic Scoring System (IPSS-R) risk classification was Intermediate. This risk is associated with the chance of MDS progressing to acute myeloid leukemia (AML).
The hypocellular variant of my disease mimics aspects often associated with another disease affecting bone marrow, aplastic anemia. This similarity is rare but happens in 5-10 % of MDS patients. The difference in my case was the presence of 5% abnormal blast cells in my bone marrow and dysplasia (the abnormal shape and size of my blood cells).
My doctor chose to initially treat my MDS using an immunosuppressive therapy of horse anti-thymocyte globulin (h-ATG) as would be done if I had aplastic anemia. He saw a 30% chance that using h-ATG might “reset” my marrow and hopefully see my blood counts return to normal – at least for a while. We thought 30% was good enough and decided to initiate treatment.
In December of 2016, just over one month since diagnosis, I became an inpatient at Moffitt Cancer Center and received the h-ATG treatment. Except for a few incidents of unnerving fever/chills and the necessity for a few platelet infusions, the treatment went well. Fortunately, my wife could stay in the room and was able to sleep on a large chair that converted to a flat bed. Having the support of family is as important to the healing process as the knowledge and skill my doctors and nurses.
I was discharged with a list of prescriptions that included antibiotics, anti-viral and anti-fungal drugs, steroids and the immunosuppressive drug, cyclosporine. Over time, I tapered off the steroids, followed later by the anti-bacterial, viral and fungal medications. Eventually, the only drug that remained was the cyclosporine. My blood was monitored weekly and adjustments were frequently made to the amount of cyclosporine I needed.
After 5 months, it was clear that the treatment did not have the effect on my bone marrow that we hoped for. My white blood cell and neutrophil counts fell to levels that I had before the h-ATG was administered. Additionally, my anemia worsened. As my hemoglobin dropped, so did my energy. Before the treatment I had completed some challenging half marathons, I now found myself getting winded walking up one flight of stairs.
In May of 2017, I had a conversation with my doctor — it was clear the treatment was ineffective and we decided that I should stop taking the cyclosporine.
Watch and Wait
As many struggling with this disease know, “watch and wait” is the treatment plan for many low to intermediate risk patients. I am now in that watch and wait category.
What does watch and wait mean for me? Right now, it calls for semi-annual trips to Moffitt to see my doctor and bi-monthly blood draws for routine CBC w/diff and a metabolic panel. No medications, over-the-counter or otherwise are required, and azacitidine (Vidaza®) is not yet needed. There aren’t any currently recruiting clinical trials that apply to my situation.
My Moffitt transplant doctor agrees with my current treatment plan. Although he and his expert transplantation team stand ready to perform an allogeneic stem cell transplant, the only known treatment to cure MDS, he does not think now is the right time. He worries that despite my excellent health and the advantage of my relatively young age, the risks associated with stem cell transplantation outweigh the benefits. The game now is to simply monitor my consistently substandard blood test results and live my life.
Life Moves Ahead
My watch and wait status let me continue to do things like participate in MDS Walks in Boston and 5K March for Marrow runs in Washington DC. It lets me continue to work, travel and more fully participate in the lives of my family.
But watch and wait is frustrating because I’d like this to be… done. I’d prefer not to be anemic and have more energy. I’d like to not worry about excessive bleeding or bruising. I also don’t want to worry about contracting a potentially deadly virus, fungus or bacteria whenever I leave my home as even shaking hands with a friend can have devastating consequences.
In time, I I know that a stem cell transplant might cure my MDS and return my life to a relative normality but it can potentially introduce new equally life-threatening medical issues, like graft versus host disease where my donor transplant’s immune cells attack my own organs and tissues.
I also decided to get another opinion from the team at Dana Farber Cancer Institute in Boston, MA. There, my doctor did even more genetic testing and looked at the length of my chromones for something called short telomeres. Having shorter telomeres at the end of chromosomes is not a good thing from a prognosis perspective. After the tests came back, I was fortunate to discover that my telomer length was normal. With no additional discovery of genetic or chromosomal abnormalities, I was returned to my Watch and Wait status.
Deciding to take more control of my future, I decided to start up a support group for patients with MDS in the Washington DC area. It has been a wonderful experience to both share my story and listen to those of the participants. I also journal my thoughts to keep my mind focused and sharp. I have learned to lean on the support of family and friends and do so without reservation or hesitation. This is too big for one person to handle alone.
For now, learning to live with the uncertainty that things can get worse at any time is hard, but with the proper support from family and medical professionals, it can be done. I keep a cautious eye on my labs, continue to work with my support group and keep a positive attitude. Before I learned of the significant risks, a transplant was what I wanted. Now, I simply hope to live as long as I can in this watch and wait state with a good quality of life before a transplant becomes necessary. It’s the best plan that I could have and I’m happy to have it!