By CHADI NABHAN, MD, MBA, FACP
Have you thought about your own mortality?
Who hasn’t, given the frequency of seeing death and grief depicted in the media or through real life encounters with friends, relatives, neighbors, or patients? These incidents trigger uncomfortable and sometimes uneasy thoughts of how we might personally deal with potential illness and disease. The same thoughts are soon displaced by the busyness of living.
Despite dealing with the death of his mother from a brain tumor, we learn David Fajgenbaum was healthy, living life to its fullest, and a future doctor in the making. He may have thought about his own mortality as he grieved the death of his mother, but likely never imagined anything dire would happen to him. Fajgenbaum was pushing forward on several fronts, including leading a non-for-profit organization for grieving college students, symbolically named “Actively Moving Forward” or “AMF” after his mother’s initials, all while first playing college football and then attending medical school. By all accounts, this was a vigorous young man, meticulous about his diet and physicality. When he became ill, it was a blunt reminder that life is unpredictable.
In his book “Chasing my Cure”, Dr. Fajgenbaum takes us back to the time when he first got ill. He vividly describes his physical symptoms and various scans which detected his enlarged nodes. Interestingly, we learn how long he was in denial of these symptoms, thereby delaying medical attention in favor of studying. This neglect of self-care highlights part of his personality, but also represents the pressure and expectations placed upon a majority of medical students.
Fajgenbaum was hospitalized and his symptoms puzzled even the best of doctors. He describes how doctors thought he might have had lymphoma, infections, an autoimmune disease, or some esoteric illness; for unknown reasons, a lymph node biopsy was not done. He did have many other tests such as a liver biopsy, bone marrow biopsies, PET-scans, and renal arteriograms; however, none of these tests revealed his underlying disease. He spent 4 weeks at the University of Pennsylvania hospital, receiving visits from friends who came to say their goodbyes as death appeared imminent. The reader gets a firsthand account of David’s prolonged and agonizing hospital stays, including being airlifted to a hospital in Raleigh in a desperate move by his father who worked in the area as an orthopedic surgeon. David was started on high-dose steroids in an attempt to alleviate his mystery illness. He eventually stabilized and was discharged home after 7-weeks of inpatient stay between two hospitals, but without a true diagnosis. In the absence of knowing what caused his illness, he relied on prayers and hopes that whatever he had would not return.
Steroids worked for a brief period, but within a few weeks David started getting ill again and was rehospitalized. This time however, a lymph node biopsy was done. If I were to get sick, I would want my doctor to share the news with me in a manner that is much better than the way it was communicated to David. When the results were available, his oncologist was not present. Instead, the nurse practitioner walked into David’s room, and in a giddy mood declared the good news that the biopsy did not show lymphoma, but rather “HHV-8 negative idiopathic multicentric Castleman Disease iMCD”. No further explanation was offered.
This news represented a turning point in David’s quest for answers. David, who was not familiar with this disease, took on the books, the internet, and the literature, finding himself astonished at how little information were available, and that some of the published information were inaccurate. He discovered that Frits Van Rhee in Arkansas is the world authority in Castleman Disease. David traveled to see him and found himself hospitalized in Little Rock when the blood tests performed showed so many abnormalities that it was clear treatment was needed immediately. Van Rhee called David, who was at the airport, and asked him to return to be hospitalized and to be started on treatment.
The ability to secure compassionate use of siltuximab, an antibody against IL-6, appeared seamless and David was granted the drug. However, siltuximab was not effective initially, and David was switched to chemotherapy. Eventually, he stabilized, only to relapse again. After a couple of more relapses, he experimented by treating himself with sirolimus, based on research he personally performed. Sirolimus worked, and he remains in remission as of this writing.
When his disease was not active, David was relentless in finding ways to organize forces to better understand Castleman disease; he was instrumental in designing a checklist to facilitate assigning the proper diagnosis. Along with a few others, he successfully established the Castleman Disease Collaborative Network (CDCN), which started to gather during the annual American Society of Hematology meeting. The CDCN was tasked with establishing a framework on how to diagnose, treat, and research the disease. These efforts led to several high-profile publications that helped physicians diagnose Castleman much faster and more easily than David’s doctors did. These efforts continue to this day. The reader can sense the impact of the work of CDCN when the author cites several examples of other patients diagnosed with the same illness, and how he bonded with them sharing his human journey along with his scientific expertise.
There is so much to learn here of how these scientific advances require harmonized collaborative efforts between all stakeholders involved. David petitioned pharma to fund the largest patient-driven registry known for this disease. Collaboration between pharma and academia is critical to advance the field if the goal is to help patients in need. While some academicians seem to always attack the pharmaceutical industry, this book shows that collaboration did benefit patients and their families. We learn about the importance of mentors when David meets Arthur Rubenstein and becomes involved in establishing the Orphan Disease Center at the University of Pennsylvania. Every reader, young or old, would want to have an Arthur Rubenstein in his/her life. I know I would.
David shares a very surreal human element in which he refused to see his ex-girlfriend (now his wife Caitlin) on two occasions while hospitalized. He simply did not want her to see him in such a condition, and preferred that she thought of him as the healthy vibrant ex-football player and weight lifter. As I read through these paragraphs, I asked myself who did I want visiting me if I were to be lying helpless in a hospital bed? I can’t make up my mind, but I think I’d want to see more people to say my goodbyes, not fewer.
We learn about the deep love that David has for Caitlin and we cheer for both as their wedding is scheduled and takes place in a late spring. As I was getting emotional reading about the wedding, David managed to make me smile when he took a few shots at his dad’s ability to play guitar at the wedding. In the weeks preceding the wedding, we learn about the ups and downs of David’s mental state, and his concern whether the wedding is fair to Caitlin given the unpredictability of his illness. It reminded me of a patient of mine who invited me to her wedding and how that invitation affected me deeply.
Throughout the book, there are uses of old metaphors of “battle” against cancer and “winning the war”. Some have criticized the use of these metaphors, suggesting that they adversely affect patients. I have argued against that criticism from the position that every patient should use whatever metaphor they wish to use if it helps them move forward. Who are we to judge what metaphors patients use? I embrace David’s use of these words, which made me feel as though I was standing alongside him in his courageous battle.
But there were some elements that I struggled with a bit. The author did not discuss some of the limitations of how orphan diseases are defined. Current definition would essentially lend every cancer as an orphan, and in fact, as most cancers are being subcategorized into various subtypes, the definition might need to be revisited. I did not see a critical discussion of the topic.
There was almost not much talk on the cost of care except when David describes the travel cost between Pennsylvania and Raleigh to receive siltuximab. Given that the author was addressing challenges of orphan diseases, research needs, funding, and other items, elaboration on the cost of care was in order.
I sensed from reading the book that the author was very much in favor of the off-label use of drugs. This might have been biased by his own experience and the fact that he has benefited from the off-label use of sirolimus. In fact, in one paragraph he writes that we need to mitigate the systemic barriers to off-label use. But most patients don’t benefit, and I would have wanted to read more words of caution as opposed to a strong endorsement for such a strategy.
These were minor drawbacks to a book that is well-written by the patient who suffered from the disease, and who happens to be the researcher that made a significant impact on this disease. We live David’s journey and he artfully brings us into his home and into his hospital room.
One of my favorite statements in the book was when David was severely ill, and he thinks to himself: “Greatest regrets were things that I had thought of doing but never did”. He goes on, “It’s the absence of memories that you wish you had made and the realization that you’ll never get the chance to make that coalesce into a horribly vivid pit of regret in your stomach.” These made me pause and think. David is so right.
I highly recommend this book; there are numerous lessons to learn and not easy to summarize in one column. One of the best books I have read in 2019.
Tune into this week’s Outspoken Oncology podcast for more on Chasing My Cure as Chadi interviews author David Fajgenbaum.
Chadi Nabhan is an oncologist in Chicago whose interests include strategy and business of healthcare. He’s a prolific speaker and occasional tweeter. He can be reached @chadinabhan.