I will never forget the phone call that changed our lives. It was in the autumn of 1998 when my sister and I received a call from my mother. She was extremely upset. Our father had just received a call from his doctor who reported his lab work showed a very low hematocrit value of 17. The doctor advised my father to report to the hospital right away. Once at the hospital, we were told my father had one of two conditions, either leukemia or Waldenstrom’s. The prognosis was grim, maybe 3 – 5 years. While my father had more tests, I recall going to the car with my sister and mother to gather our thoughts. We sat there in disbelief. With tears streaming down my face, I belted out, “Why is this happening to him?” Just a few months prior, a close family friend had passed away from breast cancer at the young age of 36. The pain of losing someone so young and so close to us was still extremely raw. I recall wondering if my dad sat during the funeral service worrying about his own mortality, and the symptoms of exhaustion that he must have been feeling for several months prior to the diagnosis. Being headstrong and determined, my father pushed through his symptoms for months, maybe years, before his bloodwork conclusively unveiled what he could no longer ignore. I say “conclusively” because before his panic-value hematocrit level led to his hospitalization, routine lab work in the preceding years had regularly shown an elevated ESR (erythrocyte sedimentary rate). His doctor had brushed off the elevated ESR as non-specific of anything concerning, but something to continue to monitor.
This new cancer diagnosis was not our father’s first rodeo with cancer. In 1993, he was diagnosed with cancer of the parotid gland. However, after successful surgery and radiation treatment my dad had been cancer-free. We thought cancer was behind us, but here we stood.
My dad’s tests revealed he had Waldenstrom’s macroglobulinemia (WM). With a concrete diagnosis, our next step was finding a physician who knew about this rare disease. My sister, Gina, turned to the internet, which was still quite new at the time, to research the disease. In her research, she stumbled across a support group on Waldenstrom’s that had been founded by Arnold Smokler. Gina contacted the organization and was able to speak directly to Mr. Smokler at length about our father’s recent diagnosis and this rare disease. Like us, Arnold Smokler was residing in the Washington, DC area. Mr. Smokler advised that he would send a package of information that the organization had compiled for the newly diagnosed. He was a wealth of information and our first source of positive news about the condition. At the time, Mr. Smokler had been successfully battling WM for several years. He discussed all the new treatments and dispelled outdated life expectancy information that was still in circulation. By the end of my sister’s phone conversation, she had the name of a physician in the Washington, DC area who specialized in treating WM patients and who was also Mr. Smokler’s treating hematologist/oncologist. Mr. Smokler’s positivity, his care package of information and access to the IWMF organization’s online forum provided a newfound well of hope. In the years that followed, my father’s hematologist/oncologist would become our other saving grace, guiding my father through multiple relapses and monitoring him throughout his remissions.
As if cancer of the parotid gland and WM were not enough, in 1999 my father was informed that he had prostate cancer. Because of the WM, surgery was not the best option, so we went with traditional radiation. In 2000, while attending a wedding in St. Thomas, my mother shared the bad news that the prostate cancer had returned. Due to his WM diagnosis, my father was still not a candidate for traditional prostate surgery, and further radiation was not considered appropriate, having been ineffective as a first line of treatment and given the extensive radiation my father already had received for his parotid gland cancer.
After researching treatment options on the internet, my sister saved the day yet again, this time discovering a new procedure that was less invasive called cryosurgery. After consultation, it was determined that our dad was a good candidate. So, as always, the family banded together and we made our way to New York City for the surgery at NY Presbyterian Columbia Medical Center. The procedure was a success, and my dad was prostate cancer-free.
We all went back to living normal lives after my dad recovered. When my father was not experiencing a WM relapse, he loved living the life of an active retiree. He built a tool shed, a fence for his ¼-acre backyard, did family research and traveled extensively. It was on one of those trips that his symptoms returned aggressively. As he tells in his story of hope, September 11, 2001 was scary for more than just the obvious reasons. My parents had left for Europe on vacation the night of September 10th. My dad had begun experiencing symptoms prior to going but was really looking forward to the trip. So, to help sustain him, he got a blood transfusion and antibiotics before leaving. When we got a hold of our parents to make them aware of the horror that was going on in the United States, we learned that he had been experiencing symptoms such as night sweats and a fever. This was always a telltale sign for us that treatment was imminent. Fevers, night sweats, napping, lack of appetite, and a cough that my dad always described as a “tick” in his throat were consistently his indicators that treatment for a relapse was right around the corner. My father soldiered on in Europe, saw all the tourist sites he wanted to see and enjoyed his trip as much as he could. But this was my father – courageous, strong-willed and determined. Upon return, he was hospitalized for a blood infection and recovered. But over the next twelve years, he suffered several more relapses, and required treatment including for transformation of his WM into large B-cell lymphoma affecting his lungs. His fifth relapse came in 2013 and then they became more frequent: one at the end of 2016, and a second at end of 2017. Along with every relapse also came our reliance on the IWMF and its support groups for hope, resources and answers.
By the time he had his relapse in 2017, a new drug had been introduced called ibrutinib (Imbruvica). My father was full of hope after genetic testing determined he qualified for this drug. He was pleased to think that he might be able to live the rest of his years by simply taking a daily pill to keep WM symptoms at bay. I recall hearing a patient discuss his issues with ibrutinib a year earlier at a Washington DC-area IWMF support group meeting. So, my sister turned to IWMF’s Facebook page to get some recent feedback on what other WM patients had experienced with this drug. It was clear that some had issues, but for the most part, the feedback was positive.
Apart from signs of decreased mobility such as walking slower and hunching over, there were no outward signs of side effects to us while my father was taking ibrutinib. My dad was a fast walker and always stood tall. So, these issues were noticeable and did stick with me. I also noticed his balance changed a little after he began ibrutinib therapy. When going up stairs he would lean into the rail. I did not know if this was natural aging or if something else was going on. But then again, I never considered my dad to be the stereotypical octogenarian. After all, he still walked his yard daily to do chores like raking leaves, and even to our chagrin, completed his own roof work. So, I shared with him an online app that provided online balance and stretch exercises hoping that these would help.
The first sign of a relapse in 2018 came during one of my father’s daily morning temperature recordings. His temperature was up and continued to trend that way for about a week before he finally mentioned it to my mother. I am sure he was hoping that the low-grade temperatures were just a fluke or related to allergies or something, but they weren’t. It was the beginning of what would be a downward spiral. From this point on, my mother took over his usual routine and she took and recorded his temperatures and pulse, twice a day. His WM symptoms and the symptoms associated with treatment came with a vengeance we had never before encountered. Sure, we had been told before that his condition was grave – specifically back in 2001 – but he recovered quickly on that occasion. This time was different. We would later learn, after restaging tests, that my father’s WM infiltration of his bone marrow had increased significantly from 15% to 50%. Initially, my father’s doctor had administered weekly rituximab in an effort to preserve treatment with ibrutinib. Despite weekly rituximab therapy, his symptoms persisted. So, the next course of treatment attempted was bortezomib (Velcade). However, this resulted in extreme diarrhea, muscle weakness and eventually he landed in the hospital.
While in the hospital, treatment was switched again to bendamustine/rituximab, a combination that had successfully worked in the past. After three courses, he started making progress – walking better and his numbers were moving in the right direction. I had read on IWMF’s Facebook page that this treatment was just as successful at 4 as it was at 6 courses. The doctor agreed, after we shared with her the studies and research that had been published. But it turned out, my dad was unable to make it past 3. He ended up contracting pneumonia and sepsis ironically soon after an upbeat visit with his hematologist/oncologist. And, he ended up not contracting sepsis once but twice. I later learned at the 2019 IWMF Educational Forum, that the likelihood of sepsis is very high during bendamustine/rituximab treatment. After the second bout with sepsis/pneumonia, a swallow test diagnosed that he had been aspirating and required a honey-consistent diet plan. It was suspected that my father’s extensive radiation of the neck to treat his parotid gland cancer had contributed to these issues.
My mother had usually borne the brunt of the caregiving duties, but this time my father needed greater support. So, my sister and I provided additional support by helping with vitals and meal preparation. We also never left him alone overnight in the hospital. One or both of us stayed with him every night while friends and my mom were on duty during the day. My sister and I are grateful that we were able to be there to help my father and to support my mother as the main caregiver. One evening when I was trying to help my dad with his short walk from the wheelchair to the bed, my father was so weak that I told him to put his feet on top of mine so I could support him. My mother said “your father used to do that with you when you were a baby and here you are doing that with him.” Life truly comes full circle. Throughout his 25 years of dealing with various cancers my dad fought, and he fought hard. But he could no longer fight. The weakness from lack of appetite and nutrients caused him to lose even more weight. After landing in the hospital for a fourth time, he passed away in August, 2019.
Throughout this experience, our strong foundation of love and support helped us endure. We stuck to that script every time a relapse or another illness occurred. Our mantra was that we were in this together. When my dad had a relapse, we hunkered down and followed a routine that included getting online and doing research after an initial consultation with the doctor. This often included checking IWMF’s many resources. In addition, my mother would take vitals several times a day during treatment. And except for trips to the doctor, my father stayed out of social settings due to his compromised immune system.
Here are a few recommendations and take-aways that have helped us as the family and caregiver of a WM patient:
- Caregiver self-care is crucial. It is a 24-hour job. Even when you are not present, you are still thinking about your loved one. My mother tried to release some stress by attending line-dancing classes when she could.
- Rely on friends and family to help provide support. We are so grateful for our core of friends that helped out in a variety of ways, including visiting with my father when my mother needed to run an errand or taking my mom out to lunch for a quick break from caregiver duties.
- Your loved one should keep extensive records of treatments received over the years, side effects, symptoms, etc. After my father’s first hematologist/oncologist stopped seeing patients to focus on research, he had two other doctors. My father kept a huge binder of all his health history and treatment information. When evaluating your health history and deciding among treatment options, a new doctor may not be as all-seeing as one would think. Health history can be extremely important, and we are compelled to be partners in our own healthcare and active members of the healthcare team. Many of the symptoms and side effects my father experienced this last time during treatment, he had experienced before, and it would have been helpful to be more conscious of them during this last course of treatment. Obviously, he was much younger and able to endure more in those earlier years of treatment. His age, weight loss, muscle breakdown, neuropathy and other harsh effects of chemotherapy that he experienced during his last relapse added to his inability to ever fully recover.
- Always be an advocate for your loved one. Be a second set of eyes and ears during hospital stays, doctors’ visits and chemotherapy treatments.
- Help your loved one conduct research on treatment options and advancements; and do not be apprehensive about getting a second opinion. It was the research that led us to Arnold Smokler and the outstanding resources provided by the IWMF. It was the hematologist/oncologist that provided a second opinion of hope resulting in 22+ more years of life.
We will be forever thankful for the encouragement and support that the IWMF provides to its members. The organization is a beacon of light to WM patients and their families. We thank the staff and volunteers and we will continue to value and support this organization in years to come.
Dominique Blandine Smith
Washington, DC – Metro Area