Sara Waits: Ten Lives and Counting
Treatments #1 & 2:
“I don’t know what’s wrong with me,” I choked out to my primary care physician during a routine physical. I was in tears. The surprised look on his face reflected his true concern. He had never seen me this distressed, as I generally presented myself as a cheerful, energetic patient. “I know I’m 45, but it seems everything requires too much physical effort,” I complained. “Bike rides are exhausting. I can barely make it through my hikes, I’m always out of breath and my legs feel heavy, as if I’m wearing concrete socks and shoes. I’ve tried to increase my workout routines for fear I’m fast getting out of shape, but nothing seems to help. I’m exhausted!”
It was December 2008. The prior year I’d gotten routine bloodwork and all seemed fairly normal, although a few things had begun to happen. My CRP was insanely high, but no one knew what would be causing the inflammation. At 39, I went through early menopause, and we were trying to adjust my hormones. This just seemed like another mid-life health annoyance. I suspect my unusual blood numbers prompted my doctor to dig a little deeper. That same day, he sent me for a complete lab workup. By late afternoon, I got a call from his office asking me to meet with him ASAP. It was at that follow-up visit that I heard these words, “It looks like you have leukemia. I’m referring you to an oncologist.” The feeling of panic and terror that I experienced at hearing those words is indescribable. It is an adrenaline rush of the worst kind. Immediately, I was ushered to the hospital for two units of blood and my first lesson in anemia. That week, my husband Kreg, and I went to see my newly assigned oncologist.
“The good news is, you have “Waldenstrom’s macroglobulinemia,” described my oncologist. “It’s an incurable but manageable disease. I know of a couple people who’ve had it twelve years and are still doing fine.” This comment was meant to be reassuring, but at 45, with my new granddaughter at just one year old, twelve years seemed a short time. “Let’s start you out on prednisone and chlorambucil (Leukeran) tablets. The goal is to bring your IgM down from 6000 to something under 2000.” It was December 22nd. Over the next few months, my IgM slowly dropped. However, in July, when my IgM rose to 4683, we added rituximab treatments as a second line. By September, my IgM had dropped back to 1240, my nosebleeds stopped, and I felt like myself again.
During this time, I began some research on the internet to see what else I could learn. I was horrified to see that the life expectancy for WMers published at the time was only 10-12 years. It seemed the more I researched, the less I could sleep at night. At the time, I did not want to join support groups. I was fearful that hearing others’ sad stories, in addition to my own terror, would serve only to put me on a path to panic attacks and depression. It wasn’t until much later that I discovered the IWMF. The wealth of information I found there would eventually empower me. I decided to be pragmatic about my treatments when it became time to address them. The rest of the time, I would simply live and not let cancer define me. I refused to follow in its shadow, preferring to think of it as something that would follow ME. I would be in charge of my life and manage this unwelcome interruption whenever it appeared. So, I led my life as I had before. My career as Project Manager and Service Executive at a major telecommunications and technology company kept me working long hours. I was supporting some of the corporation’s largest customers and working my way up the corporate ladder. Always rolling my laptop and briefcase bags into the Cancer Center, I never missed work. During chemotherapy, I often hosted project calls (with a one-hour time-out for my Benadryl-induced naps). I was grateful that I tolerated treatments well. There were occasional days of fatigue, but my management and co-workers were incredibly supportive. So, this went on for several years. My husband and I traveled, mostly going to Colorado or Utah for camping and four-wheeling our Jeep in the desert or mountains. Our kids, all three of them grown, were also supportive.
In December 2010, I lost 13 pounds for no reason. My hemoglobin was down to 7.7 and my IgM was back to 4800. It was time to consider a third line of treatment. This time it would be dexamethasone and prednisone, and right after Christmas I would start fludarabine. This regimen left me feeling shaken and anxious with a constant headache. I pretty much just wanted to sit on the couch and stare at the TV. Treatments were for three consecutive days, each month, January 2011 through June 2011. By September, my IgM was down to 926. I’d still not had a bone marrow biopsy. In 2012, a quarterly rituximab maintenance plan kept everything in check.
In 2014, my IgM was only 921, but my other counts were low. I had my first bone marrow biopsy. It showed my bone marrow was not rebuilding. It was markedly hypercellular (95%) and showed 60-70% involvement and consistent with WM. I went back on rituximab monthly, March through May. To check for lymph node or spleen enlargement as well as internal bleeding, I was sent for a CT scan. All else was normal. It appeared that there was no explanation for the low hemoglobin. My iron stores were normal. When rituximab had no effect, we started the fourth line of treatment in September, consisting of rituximab and bortezomib (Velcade). The side effects for this included a rash on my back and stomach for a week, along with stabbing pain in my groin/abdomen. For a while, the treatment seemed to put WM behind me. Once again, I sped along with life as I started a pet photography business, did pet shelter fundraisers and opened a retail home decor shop with friends. I wrote articles for the Indiana State Beekeepers Association and for “Off-Road Adventure” magazine. This was in addition to my full-time job. My energy and enthusiasm still knew no bounds.
As my veins were shot, and we could no longer even draw blood for labs without a lot of work and stress for everyone, I finally agreed to have surgery to install a central line port in May 2016. I also met with doctors at the Indiana Bone Marrow and Transplant Center. They talked me through some of the process suggesting I harvest cells for an autologous transplant. I declined, feeling as though I had other options available and was mostly terrified at the thought. To me, that sounded so FINAL. But always the unwelcome visitor, WM, made itself known again, by dropping hemoglobin levels into the 7’s and 8’s. My IgM levels were in the 300’s – a little high but unconcerning. This time we would try bendamustine (Treanda) plus rituximab from May through June. Again, it seemed to work, but by December the biopsies were showing uncommon mature B cells, pancytopenia and >90% involvement. We began looking at other treatment options.
Treatment #6: ibrutnib (Imbruvica), Jan 2017 – Jul 2018
In January, I needed weekly transfusions of blood and started ibrutinib. In March, I needed only one blood transfusion! This sustained me the rest of the year. By April 2018, my hemoglobin and platelet counts started decreasing again, though IgM was still a non-issue. In fact, it was sometimes even low. In October another BMB showed 95% of my marrow was still involved. My oncologist thought it seemed to be a more aggressive acute lymphoma. He wanted me to go back to the Transplant Center for another discussion.
By November, I’d become transfusion dependent for both blood and platelets. My white counts were low. I had to start shots of Neupogen (a white blood cell stimulator). I was advised to avoid being around sick people and to do nothing that would damage soft tissues such as careful brushing, no suppositories, no sex, and was cautioned against driving in case of a brain hemorrhage. This new lifestyle that had been suddenly thrust upon me was surreal. I often teased my nurses that I need to get my transfusion of blood and platelets so I could go horseback riding, zip-lining, and four-wheeling, and my Neupogen shots so I could clean my chicken coop. Sometimes I was teasing, and sometimes I wasn’t!
I began having what I learned were “neutropenic fevers”. This necessitated blood cultures, being put on prophylactic medications, random admissions to ER, and guest appearances in the critical care unit several times in the next couple of months. The cultures always came back negative and the fevers remained a mystery. For fear of potential bacteria because my white count was 0.4, all of my beloved fresh vegetables were pulled. However, I could have all the cheeseburgers and pizza I wanted. There’s always a silver lining!
The transfusions continued into December. I had to finally acknowledge I needed some time away from work. The fatigue was starting to catch up with me, and to say my attention span was short was an understatement. I couldn’t focus on anything for more than a few minutes! After my oncologist consulted with Dr. Steven Treon over Thanksgiving, we agreed to make arrangements to go to Dana-Farber Cancer Institute to meet with Dr. Jorge Castillo. The bone marrow biopsy results found my marrow to be extensively involved by a B-cell lymphoma. Given my extensive history of prior therapies, the options were limited. After Christmas, Dr. Castillo consulted with my oncologist. They decided to start me on carfilzomib, dexamethasone, and Cytoxan. Carfilzomib is a second generation equivalent of Velcade. Treatment would be for one week. Plan B would be venetoclax, which wasn’t yet approved for WM, so we got started working on insurance approvals right away. Despite all the transfusions and hospitalizations, I continued my version of normalcy. I readied myself each day as if I were going to work instead of chemo. I loved visiting with friends, family, and those at the Cancer Center. My spirits and sanity depended on it. Whenever my spirits needed a little extra boost, it was music that lit me up. The year prior, the band The Goo Goo Dolls released a song titled, “So Alive”. The music and lyrics resonated with me to the core and left me feeling energized, always ready to face the next health challenge with vitality.
Treatment #7: Carfilzomib (Kyprolis) (Jan 23/2019)
Beginning January 23, 2019, we did 4 treatments of carfilzomib. We knew if it were going to work, we would see improvements right away. Unfortunately, my condition continued to deteriorate.
Treatment #8: Venetoclax (Venclexta) (Feb-Apr 2019)
On February 4, the decision was made to stop the carfilzomib and transition to venetoclax in hopes of a beneficial result. Insurance initially denied our request, but my oncologist appealed to their staff doctor and the decision was overturned.
My oncologist had been able to talk to Dr. Treon at a conference about my lack of response. He suggested we titrate venetoclax up to 800 mg and see what happens in 2-3 months. By this time, I was really feeling like a guinea pig. In May, we increased dosing to 1200 mg, then at Dr Castillo’s prompting, it was decreased to 800 mg, but with supplementation of rituximab. We got some marginal improvement on white counts, but hemoglobin not so much.
I suffered other unusual symptoms as well. I began to sarcastically call them “fun with cancer.” The left side of my mouth would become red and swollen, and the jaw bone would hurt. I was given some “Mary’s Magic” mouthwash that would help for a short time. Random mouth sores and hematoma on my tongue plagued me. I got a salivary parotid gland disorder, gingivitis and ringworm. At one point, excruciating pain in my back immobilized me. No amount of Tylenol, rubs, or heating pads would resolve this pain. An MRI and chest x-ray showed no expansion of WM into the vertebrae. In fact, I was told my bones look like a 30-year old’s! I was treated for internal shingles with three shots of morphine, which made me feel silly in the head but didn’t do a lot for the pain. Then there were the hematomas on my legs and arms. Many of these left bruises that I still have two years later.
Finally, in June, I accepted the seriousness of my disease and took an early retirement. I got my affairs in order, as they say, and had some very difficult discussions with my husband of thirty years. Until now, I’d been lighthearted about the process, not dwelling on the negatives beyond my control. But now, I faced some serious decisions and potential outcomes for which I was not prepared. Planning and talking about it, as hard as it was, became the only way I could feel in control of anything. The rest I gave to the universe and moved forward with a positive outlook.
Treatment #9: CHOP (Summer 2019)
In an effort to sustain me, I was given solo rituximab for the month of June. It did little. With treatment options rapidly running out, and deteriorating blood counts, the last option in our arsenal at the Cancer Center was CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone). This was where I’d lose my hair. For years, I had been dreading it. I faced it and made the best of it. Friends offered to help. I let them. This included my hair stylist who graciously donated a beautiful trim when the hair first started falling out. Another friend would comb out loose pieces for me. Finally, I let my husband shave my head completely. Friends donated scarves and wigs, making me feel so cared for and loved. On social media, I shared monthly health updates. This included some of the funny things that happen such as a wind test in my convertible to see how fast I could drive without my wig flying off!
The outpouring of support gave me a feeling I’d never before experienced to this depth. It was a gift. To show my appreciation for all those who cared for me, I was determined to be the best person I could be through all of this. That circle of love was a buoy for my spirit in the ocean of fear I knew was ahead of me. My doctor and nurses contributed more to my spirit than they will ever know. To show a token of my appreciation, I nominated the entire Oncology Department for a Daisy Award and was invited to the ceremony. It was a morning of happy tears for us all.
In October, we did another BMB. The involvement now showed 20% while I continued to be hypocellular. At the Indiana Blood and Marrow Transplantation Center, we had a conversation about CAR-T cell therapy. Due to my poor blood counts, the physician said I was not a candidate. I never got accustomed to hearing that I was too sick to be treated. So, stem cell transplant it was to be, and the time to act was now before the marrow involvement climbed once more.
Treatment #10: November 2019
In preparation for my allogeneic transplant, this was a busy month. Because my own marrow was in such bad shape, a donor had to be found. I was fortunate in that my donor HLA match was a perfect 10/10 and was also the same blood type. Because the existing port in my chest would not be sufficient for all the IV lines needed, a PICC line was placed in my arm. As it interfered with showering, this may have been my least favorite treatment device. This simple pleasure became a hassle. The day prior, and to destroy my remaining stem cells, I was to be given total body radiation and a High dose of Cytoxan, fludarabine and rituximab. These drugs would prepare me for my donor’s cells. Despite knowing my own cells were no longer functioning properly in my current bone marrow environment, destroying all I had left made me feel extremely vulnerable. My team and I agreed that I would attempt an outpatient transplant procedure. This is not done frequently. However, due to my overall commitment to the process, my involvement with the team, and my display of competency in coordinating my medications and schedule, the doctors thought me an excellent candidate. Aside from having non-functioning blood, I was a healthy and positive 56 year-old. Within two weeks, I was prepped and ready for the big day.
December 10, 2019, Day 0
Stem cells have a short shelf life, so unlike blood that is stored, the donor must be activated and prepped as well, during the two weeks that I was being prepped. The cells are harvested, put in a cooler, and given to a courier whose job it is to not let that cooler out of their sight and get it to the Transplant Center, ASAP. In my case, it came from overseas, most likely Germany. My husband and I waited at home for a call from a nurse, who had said once it arrived, I’d need to come right away. The call came at 11 pm. We made the 30-minute journey to get started. With all the lead-up to the event, the transplant itself was a non-event. It was like any other transfusion I’d had. We were there for just a few hours and then sent home, only to return again at 7 am. We would do this every day for the next 30 days. This was the trade-off for doing an outpatient transplant. By Day +9, one of my doctors remarked that one of the reasons I was doing remarkably well, was that I had a “high emotional IQ”. By this, he meant I was able to understand and manage my emotions in a positive way in order to relieve stress, communicate effectively, and to overcome challenges.
I continued to do very well until January 13, 2020, when I was admitted for a fever. Like before, it seemed to be a neutropenic fever. I was given the standard IV antibiotics. Out of an abundance of caution, they kept me for a week. While there, I got up and made my bed every day just like at home. I went to the work-out room for an hour daily, read my books, and played my guitar. I looked forward to nurses’ visits. My husband faithfully visited every day, bringing in carry-out dinner so we could eat together.
Eventually, our lives returned to a sort of normal. In January, 2020, I was no longer getting transfusions of any kind. My labs normalized for the first time in two years! By February 3rd, my marrow was 100% donor cells! My PICC line was removed. I was still taking the most insane program of medications I’d ever seen, which amounted to more than twenty pills per day. Some were prophylactics, some were designed to prevent GvHD (graft versus host disease), some were to counteract symptoms caused by other required medications. Along the way, high blood pressure, edema of the arms and legs, rashes, low grade fevers, foot neuropathy, and a new random nausea related to GERD appeared. My digestive system didn’t rebound as fast as everything else, so I would periodically vomit, if I wasn’t careful about what I ate.
But as time went on, these things subsided or minimized. By March, I was back out on the golf course, training for a 50-mile bicycle event, hiking with my granddaughter, and spending time with family and friends. My husband and I even made a dream trip to Yellowstone National Park where we camped for two weeks! COVID slowed our pace some, but the life we had led gave us plenty of practice in protecting ourselves.
My bone marrow biopsies continue to show 100% donor cells, but a 90% tumor load of WM cells. However, my blood labs are perfect so I continue to be both a star patient in terms of recovery, yet a medical mystery of sorts. Who knows where or when this WM journey will take me next? But for now, travel plans have been made for 2021 and living life to the fullest will continue.