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This story is from the Anamnesis episode called I Quit and starts at 25:32 on the podcast. It's by David Fajgenbaum, MD, MBA, MSc, FCPP, assistant professor of medicine in the Division of Translational Medicine & Human Genetics at the University of Pennsylvania; associate director, patient impact at the Penn Orphan Disease Center; founding director of the Castleman Disease Center at Penn; and co-founder & executive director of the Castleman Disease Collaborative Network. He is also the author of
Once you've mastered the basics of CPR technique, hand placement, head tilt, and timing, and after you've accepted the inevitable feeling of shattering ribs beneath the heels of your hands, the hardest thing about performing it is knowing when to stop.
What if one more pump could do it or one more after that, when no matter how hard you push, how hard you hope and pray, that pulse just will not return? What comes next is entirely up to you. The life has already been lost, but hope hasn't been. How long do you try to bring someone back?
Hope is a Force, Not a Superpower
Eventually, you will remove your hands from the body. Eventually, you'll have to, but eventually isn't a number. You won't see it in a CPR diagram. When you eventually stop, you stop because there's no more hope. That's what makes the decision so difficult.
Your effort allows you to hope that life is possible, and your hope inspires you to push even harder. The three of those things: hope, life, and effort chase one another, keep one another moving around a track.
I've performed CPR twice in my life. Both times the patients were nearly dead when I began my relentless chest compressions and prayers and both ended up dying. I didn't want to stop.
Hope can be a force, but it isn't a superpower. Neither is any part of medicine, much as we'd like it to be. I learned this the hard way.
In 2010, I was 25 years old in my third year of medical school, where I was pursuing my dream of becoming an oncologist in memory of my mom. Then insatiable fatigue, weight loss, abdominal pain, and fevers hit me out of nowhere.
Over the course of a few days, I went from treating patients to taking myself down the hall to the ER. "Your liver, kidneys, and bone marrow are shutting down," they said. "We need to hospitalize you right away."
A retinal hemorrhage made me blind in my left eye. I gained 70 pounds of fluid in around every organ, drifted in and out of consciousness, and was transferred to the ICU.
Still no diagnosis.
Giving Up on Life
This inexplicable attack caught me flat-footed and unprepared, and in those first days and weeks, at times, I was ready to give up. Those words -- give up -- don't quite translate across the barrier between the ailing and the well.
Even now, I've lost some touch with what it once meant in the moment mentally to give in to death, but I remember doing so. Death seemed to promise me peace and an end to my suffering. That was alluring at a time when every breath was painful.
The harder I pushed to breath sufficiently deeply, the more knife-like pain I felt, so I slowed my breathing and fought less for each breath. But it was my family, with whatever special sensitivity only families can have, who must have realized I was slipping away. It was their urging that brought me back. "Just breathe," I remember hearing. That was enough.
I snapped out of my trance and began fighting for each breath again. However unlikely it may have been, I dreamed that I could survive and have a family of my own with my girlfriend Caitlin that also kept me fighting.
My illness progressed, but I still had no diagnosis. After 11 weeks, my doctors encouraged my family to say their goodbyes and a priest read me my last rites.
A diagnosis was also finally pronounced: idiopathic multicentric Castleman disease or IMCD, an enigmatic disease at the intersection of lymphoma and autoimmunity. The immune system becomes hyperactivated and shuts down the body's vital organs for no known reason.
Overtime
I started chemotherapy in a last-ditch effort to stay alive. I've considered that day to be the start of my overtime. Fortunately, the chemotherapy saved my life. Unfortunately, I would go on to have several more life-threatening relapses.
Though we now had a name for my disease, we didn't have much else. There were no FDA-approved treatments, no diagnostic criteria, no treatment guidelines, and no coordinated research at the time. As a result, a third of patients died within five years of diagnosis, and another third died within ten, but there was one reason to be hopeful.
A new drug was undergoing clinical trials and the FDA granted me compassionate use. I was in remission for a year before I relapsed again and nearly died for the fourth time.
My doctor gave me a combination of seven chemotherapies in another hail Mary attempt to keep me alive. While the chemo was infusing into my veins, he informed me there were no more drugs in development, and there were no more promising leads.
Nothing. No reason to be hopeful whatsoever. I wanted to quit again.
My sisters, girlfriend Caitlin, and dad were seated around the bed and had listened to my doctors every word. Their presence and knowing there were so many others with my disease who had received similar news inspired me to not quit.
I interrupted the silence by saying something I knew was my only choice. "If I survive this, I'm going to dedicate the rest of my life, however long that may be, to curing this disease." I knew it was unlikely that I'd make progress in time to save my own life and that I likely wouldn't get to marry to Caitlin and have the family that I was dreaming of, but I wanted to go out swinging.
Out of Luck
I returned to medical school on a mission, partnering with my mentor, Arthur Rubenstein, MD, and my doctor, Frits van Rhee. We founded the or CDCN, to spearhead a novel approach for advancing research.
Rather than raising money and hoping that the right research idea would be proposed by the right applicant with the right skill set at the right time, we built a community of over 400 physicians, researchers, and patients. We invited them to crowdsource the most important ideas, then recruited the best researchers inside and outside of our community.
In parallel, I began conducting laboratory and clinical research into IMCD, mostly working on the samples that were easiest to access: my own. It felt good to fight back.
I even got engaged to Caitlin and graduated from medical school during this time. I so badly wanted to make it to our wedding date of May 24th, 2014. Then I relapsed again.
Despite the weekly chemotherapy, I was back in the hospital with multiple organ failure and dying for the fifth time. Fortunately, chemotherapy saved my life again, but I was running out of luck. I needed to identify a drug that could prevent IMCD from coming back. I pored over thousands of pages of my medical records and data from lab experiments I performed on my own samples to identify a signal or target for a drug, something, anything.
The Importance of 0.12 Months
I discovered that a key cellular communication line called mTOR was highly active. The best part about learning that mTOR was activated is that there's a safe and potent inhibitor of it called sirolimus, originally developed for kidney transplantation 30 years ago. It had never been used before for IMCD, and there were no guarantees that it would work, but my doctors and I decided to try it.
Thanks to this drug I made it to our wedding day and Caitlin and I even had a daughter together one year ago. Just a few months ago, I shared my story of not quitting in a memoir that I wrote called "Chasing My Cure."
Today marks 70.12 months since my last relapse, about nine times longer than my average remissions before starting sirolimus. The .12 is important.
I learned that I can't round up. I may relapse tomorrow, but I also refuse to round down because my team and I have worked so hard for every day of remission for myself and other patients with IMCD.
The CDCN has spearheaded incredible progress, including developing the first-ever diagnostic criteria and treatment guidelines and generating insights into IMCD pathogenesis and treatment. I recently launched a clinical trial of sirolimus to investigate its efficacy in IMCD patients.
Though it has helped some, it is not helping all these patients and we don't have any more options for them, so I can't and I won't quit fighting to advance research and treatment for these patients who are waiting and hoping. Knowing that I'm doing everything humanly possible to unlock the mysteries of this disease to help as many patients as possible in the time before my next relapse helps me to worry less about when and -- dare I say -- if it will come.
I won't have any regrets. I will have fought with everything I've got and I will have enjoyed every moment of this journey chasing hope and life around the track.
Other stories in the I Quit episode include My MD, My Baby, My Nightmare and Why I Fled the Country.
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