In the summer of 2014, I became slightly short of breath during a hike up a granite dome mountain called “The Thumb” at Sequoia National Park. This was highly unusual for me. I have been a high-energy person my entire life, working in the construction industry for 40 years and now on my small hazelnut tree farm.
“How can I be tired?” I asked myself on the hike during a family trip to national parks in Arizona and California. “I am not out of shape.”
I eventually visited my primary care doctor, and blood work revealed that I was anemic. The doctor prescribed an iron supplement. Despite this supplementation, a blood test six months later showed that the anemia persisted. The doctor then ordered gastrointestinal X-rays, the results of which found no evidence of internal bleeding.
The straw that broke the camel’s back came on a day I was unloading the dishwasher. While I was bending over to retrieve the clean dishes, a flow of blood poured out of my nose onto the kitchen floor. To say the least, I knew then that something was not right with my body. Panic set in and I was full of fear.
I told my doctor I wanted to see a hematologist, only to wait three months for an appointment. The hematologist performed a bone marrow biopsy, which found that my bone mass was a 9 on a scale of 1 to 10. This was comforting to me, as the high degree of bone mass proved that I am no couch potato.
The hematologist sent the sample to MD Anderson Cancer Center in Texas, where a molecular DNA analysis was performed. In July 2017, I was diagnosed with Waldenstrom’s macroglobulinemia (WM). When I read about WM following my diagnosis, I learned that nose bleeds, like what I had experienced previously, are common with WM patients.
My hematologist wanted me to start ibrutinib therapy. I obtained my first 30-day supply of the pills, along with a lot of literature about the drug, from a specialized pharmacy. I was told it was a wonder drug that would spare me from chemotherapy.
But after reading about the drug – which cost $13,000 for just 30 pills – I learned that patients must take it for life. This scared me. So I did not take the medication, despite having paid such a hefty price for it.
I searched the internet and realized I should not limit myself to one physician’s opinion. I found the website for the International Waldenstrom’s Macroglobulinemia Foundation (IWMF), which lists a number of physicians who specialize in WM. One of those doctors was located near my home on Long Island. I quickly contacted the doctor’s office but was told I would have to wait two months for an appointment.
Meanwhile, my disease went into overdrive, sapping my energy. I followed up with the hematologist’s office and explained I might not be alive by the time of my appointment. They told me to drop by for a consult. My blood was drawn, and I received an iron infusion during that initial appointment.
I now know that the mutant B cells in my bone marrow were producing an inordinate amount of IgM protein. This causes hyperviscosity of the blood, reducing the delivery of oxygen throughout the body. The mutant cells also release a substance that blocks the ferritin enzymes that permit the body to absorb digestive iron. Without iron, red blood cell production is diminished. In addition, the mutant cells take up or compete for receptor sites in the bone marrow, further decreasing red cell production. In other words, the cancer cells were taking over my body.
I think I should note that my mother’s sister also had WM. My mother had lymphoma, and I had a brother who died from leukemia at age 5. All these family members were exposed to high degrees of chemical carcinogens and radiation. I believe these environmental exposures, coupled with a genetic predisposition, contributed to the cancers.
At the time of my first chemotherapy treatment, my IgM blood level had risen to 4,200 mg/dL. My blood had the consistency of maple syrup. I received six cycles of bendamustine chemotherapy, in combination with a monoclonal antibody called rituximab. I continued periodic rituximab treatments as “maintenance therapy” for two years following chemo.
The summer before my treatments, I could not do much at all. I feared I would have to throw in the towel on my hazelnut tree orchard. I had spent the past 13 years establishing the orchard, but the future seemed to hold no hope. The outlook had become so unpromising that I had an attorney prepare a last will and testament.
But in the months following chemotherapy treatments, I started to feel like my old self again. And today, my IgM count is down to 214 mg/dL, which is in the normal range. I now have much more optimism about the future, and have resumed work on the hazelnut tree farm. And better still, I welcomed a granddaughter into the world on November 5, 2018.
At the time of my diagnosis, I could barely walk up the flight of stairs in my house. Now I can once again prune and plant hazelnut trees, digging holes in the earth with a large auger that is maneuvered by hand. The work is strenuous, but it gives me peace.
When I am alone in the field planting, the only other sound is from the songbirds.
I know in my heart that God is not done with me. Evidently, he has other plans.
Garden City, New York