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A patient with BPDCN finds her champion.
Image credit: C Davids/peopleimages.com | stock.adobe.com
Joseph A. Kalis, PharmD, BCOP, is an ambulatory oncology pharmacist at UCHealth in Colorado Springs, Colorado. Kalis writes from the Front Range of Colorado and enjoys climbing rocks and frozen water. He rages therapeutically at concerts of all sorts.
"Hi, I’m Fred. I’m a handful.” With those words began one of my most treasured friendships. Fred wasn’t her real name but a nom de plume she liked to use when introducing herself to see how people would react. “I know you all know my name from the chart, but you don’t know me. I can tell if I’m going to like someone or not based on how they respond to me throwing them a curveball,” Fred explained.
Chuckling, I responded that my name was Joe but that she could call me whatever she liked. When we met in the infusion center procedure room at Flagstaff Medical Center (FMC) in Arizona, Fred was a vivaciously independent 76-year-old woman. Slender, with gray hair and Western-style attire, she single-handedly lit up a room. The complexity of Fred’s diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) belied the inherent simplicity of how she lived her life. BPDCN is a rare subtype of leukemia affecting the body’s natural killer (NK) cells. Think of the NK cells as the body’s SEAL Team 6, able to quickly respond and annihilate a threat without asking too many questions. If you’re thinking that SEAL Team 6 going rogue would be bad, you’ve got an accurate idea of BPDCN.
Fred came to us by way of the University of Arizona Cancer Center in Tucson. Before you start wondering, yes, Tucson is a long way from Flagstaff. It’s even farther away from Fred’s off-grid ranch somewhere near Seligman, Arizona. Off-grid is how Fred liked things: She didn’t like depending on anyone besides herself, a trait consistent with her generation’s flinty resolve. Fred loved telling the story of how she always carried a trusty 9-mm pistol with her whenever she was at home “in case a rattler needed shooting.” Coincidentally or not, her favorite stories were those of Zane Grey, Roy Rogers, and John Wayne: icons of the literary and silver screen cowboy tradition.
Although these raconteurs and many of their tales are fictional, Fred’s philosophy of life was not. She had her opinions, respected those of others, and would help whomever she could. She preferred to fly under the radar and not take up any space she didn’t need to, despite saying “there’s plenty of space here out West.” An apt description for Fred would be a female version of writer and enfant terrible Edward Abbey. But I tell you what: If Fred called and invited me on a family houseboat vacation to Glen Canyon Dam à la The Monkey Wrench Gang, a novel by Edward Abbey, the only question I’d ask is, “Whose car are we taking?” She was that kind of lady—the kind you couldn’t help but believe in.
Fred’s unexpected diagnosis led to her initially being treated at the University of Arizona Cancer Center. However, with a 9-hour round trip from her ranch to Tucson, routinely traveling back and forth simply wasn’t feasible for Fred. “My dogs need to eat and to run outside, and I miss them,” she told me during that first conversation. Fred was subsequently referred to the oncology practice in Flagstaff, from which an associate reached out to me to assist in coordinating her chemotherapy.
Treatment for BPDCN often uses a complex regimen called the CALGB 8811 protocol, after the trial that established its use. However, the CALGB 8811 protocol is like Schrödinger’s cat; it’s 2 things at the same time: straightforward and complex. The CALGB 8811 protocol is straightforward in its organization of certain drugs at certain doses on certain days according to a road map, yet complex in the amount of coordination, organization, and traveling needed to pull it off. Taking a broad view, the complexity of the CALGB 8811 protocol acutely reflects the complexity of BPDCN.
Fred was at FMC to receive injections of a drug called asparaginase erwinia chrysanthemi (recombinant)-rywn (Rylaze; Jazz Pharmaceuticals) to treat her cancer. Asparaginase erwinia chrysanthemi is unique in how it works: It’s an enzyme that breaks down the amino acid asparagine. Normal healthy cells can make their own asparagine, but leukemia cells cannot, allowing asparaginase erwinia chrysanthemi to essentially starve the leukemia to death by depriving it of a crucial component. It’s not a common drug or one that FMC was easily able to access. Of course, Fred didn’t know that detail, nor did she need to. That’s why I was there.
A few phone calls and several hours of meetings later, we were in business with a contract to purchase the drug outside our usual distribution channels. But my pharmacy director was not immediately enthused with the plan. “Why do the things you need always torpedo my plans for the day and sink my budget projections?” the director asked me semiseriously.
“Gotta spend money to make money,” I said with a chuckle. I also mentioned my ace in the hole of the anticipated Health Resources and Services Administration 340B Drug Pricing Program reimbursement. Then, with the pharmacy director’s allegiance temporarily secured, it was time to move to the next phase of Operation Fred.
A key component of the CALGB 8811 protocol is intrathecal chemotherapy. Delivered directly to the central nervous system via the spinal cord or ventricles within the brain, intrathecal chemotherapy bypasses the body’s physical layers of defense to treat any cancer cells holed up beyond the reach of standard treatments. Prior to my time there, FMC hadn’t administered intrathecal chemotherapy. Those patients had historically been referred to larger centers in the Phoenix, Arizona, area. But to an authority-impaired, divergent-thinking enthusiast such as myself, history wasn’t a barrier. A call to interventional radiology revealed a physician who felt similarly. If I could provide the chemotherapy and acquire consent from the patient, they would handle the procedure. Simple enough, right? A few training sessions for the intravenous room and chemotherapy compounding teams, a discussion with the pharmacy buyers on purchasing preservative-free methotrexate, training for my colleagues on verifying chemotherapy orders, and voilà—pharmacy was ready to go as well.
Fast-forward a few months, and Fred had completed intrathecal chemotherapy and received several doses of asparaginase erwinia chrysanthemi prior to developing a hypersensitivity reaction. Somehow with all this, she’d found time to sew a Roy Rogers–themed quilt as a gift when my son was born. As fate would have it, Fred’s leukemia was as feisty as she was. Despite intensive treatment, the leukemia advanced rapidly and Fred was admitted with febrile neutropenia.
Shortly thereafter, the palliative care and hospice team (of which I was also a part) was consulted: Fred was ready to go home. It was a heavy walk from my office to Fred’s room on the third floor of FMC. The hallways seemed darker, closing in around me. The skywalk bridge from one building to another offered no respite despite the bright Arizona sun. I knew I was walking to say goodbye.
As a young practitioner at the time, I hadn’t professionally encountered death or navigated its effects on my clinical practice. When I saw Fred, we both knew it was the last time we’d see each other. It was an odd sense of finality walking into her hospital room as a hospice nurse prepared her for discharge. We were both emotional, and yet there was peace. As we parted, Fred summed it all up via Roy Rogers’ closing jingle: “Happy trails to you, until we meet again.” There was closure, sure, but also realization. I see Fred in every patient I help now, in every small battle, in every clash of principle.
You might be asking at this juncture, “Why did you help Fred? Why did you, a new practitioner freshly planted in your first big boy job, stick your neck out with the pharmacy director, health system pharmacy and therapeutics committees, clinical manager, and other departments on behalf of one person? Why are you even telling this story? To gratify your fragile ego and feel better about the compromises you’ve made in your career?” No—and who told you about that last part?—it all comes down to one idea: Either we all matter, or none of us do. In today’s health care industrial complex, the waters of who matters and who doesn’t are murky and opaque. The Talmud states that “anybody who preserves a single life is counted as if he preserved an entire world,” meaning for that person, you saved their world. Working with and for Fred showed me this in a way nothing else could.
Why did I do what I did? On one level, to paraphrase climber Scott Backes, “Because I could and others could not.” Every patient we can help care for counts. Every interaction counts. Every moment counts. We are in the right place at the right time with the right skill set to do what we can for the patients we are able to. Stoic philosophy emphasizes the concept of sympatheia, an idea of mutual interdependence expressed by Marcus Aurelius as, “All things are mutually woven together and therefore have an affinity for each other.” Understanding how we are all connected and dependent on each other spurs us not only to be good but to do good for each other. Charles Dickens stated that “no one is useless in this world who lightens the burdens of another.” In health care, isn’t that our mission? To lighten the burdens of another? I think so, even if it means taking on more burdens myself. Perhaps you do too.
As time marches on through our careers, the concepts of humility and hubris can help us to lighten the burdens of others. Although humility and hubris appear diametrically opposed at first, I see them as opposite sides of the same coin. Both are needed to maintain balance. We need therapeutic humility to remain open-minded and to be receptive to new ideas, new information, and new paradigms in oncology. But if we only have humility, we will never do anything except be humble. We need humility to learn and hubris to execute. Hubris is the excessive self-confidence that pushes us forth to enact change in the face of potential defeat. It’s a heady mixture for sure, but one that requires constant upkeep. I encourage each of you to remain vigilant and to hone your awareness, for we never know when we’ll be called upon to lighten a burden that no one else can.
