When A Doctor Chases His Cure. Dr. David Fajgenbaum And Castleman Disease

Journey of a Disease: Dr. David Fajgenbaum was diagnosed with a rare disease – Castleman disease

During the years as a talk show host discussing medical research, I have had the opportunity to meet some awesome people and hear stories that inspire and motivate. Dr. David Fajgenbaum’s is one of the most compelling ones that I have heard.

David has written a book – ‘Chasing My Cure: A Doctor’s Race to turn Hope into Action – documenting his crazy decade-long battle with his disease and more recently developing a drug that he is using himself. With his book out in September, I cannot but reminisce about our chat.  

“When I started medical school at the University of Pennsylvania the first two and a half years of medical school was fine. I never had any medical issues and I was planning to become a clinical oncologist. I had lost my mom to cancer a few years before and I knew I wanted to be a doctor to treat cancer patients”, started off David. 

However, things suddenly changed for David. He went from being this totally healthy third year medical student to being sick in the intensive care unit with night sweats weight loss, and abdominal pain. His liver and kidneys began to shut down and bone marrow stopped functioning properly. And he was hospitalized in the same intensive care unit at the University of Pennsylvania that he had previously worked in as a medical student. 

He got sicker and sicker by the day with no diagnosis!

“It was absolutely terrifying to really lose grips on life, really without knowing what it was that was making me so sick.”

Fortunately, over the course of several weeks, David’s condition improved without any explanation. He regained consciousness and some of his organs began to work again, but still no diagnosis.

The relief was short lived and David found himself back in the ICU with liver failure, kidney failure, bone marrow failure, in and out of consciousness. In fact, he was so sick the second time around that his physicians encouraged his family to say their goodbyes and a priest came in to administer his last rights. 

But right before he was administered his last rites, an important diagnostic test was done – a lymph node biopsy. A review of his lymph nodes indicated that he had a disease called idiopathic multicentric Castleman disease. This is a rare and deadly inflammatory immune system disorder where your immune system gets out of control and begins to attack your healthy vital organs. 

Castleman disease had been known since the 1950s but has remained largely a mystery. A hallmark of the condition is enlarged lymph nodes, and most people with the disease have a form that affects just one part of the body and can usually be cured through surgery.

The form ravaging David’s body — multicentric Castleman disease — is even more rare and deadly. Only about 1,200 to 1,500 people are discovered to have it every year in the United States. It defied classification, occupying a no man’s land between cancer and immune disease. Doctors weren’t sure of the cause in patients like him: Some believed it was a type of cancer, while others thought it was an inherited genetic disorder, or was triggered by a virus.

One thing was clear: The disease was deadly.

Armed with this diagnosis, David’s doctors decided to start him on a form of chemotherapy to help turn around the disease.

The first dose of chemotherapy kept him alive but relapses began to happen needing more intensive regimens including seven agent combination chemotherapy. Finally, after 6 months in and out of hospital, David returned home and began to improve. He returned to medical school and back to his mission to treat cancer patients. 

David with his dad

And then everything came to a halt again!

About one year later, David had another relapse of all of his previous symptoms, all of his previous organ failure and was back in the hospital. He was once again administered seven different chemotherapies. During this hospitalization, he also learned that that there were no other drugs that were in development for Castleman.

This time when he returned to medical school, he wasn’t back on the same track he was on before. This time he returned on a mission to take on Castleman disease. 

He began conducting laboratory research in the lab at Penn. And decided to create a foundation called the Castleman Disease Collaborative Network (CDCN) to try to bring together researchers from around the world to push forward Castleman disease research.

“Research that really makes a difference, needs to work across institutions, it needs to work across countries and needs to be highly strategic. We needed to all work together. You need to think really critically about what’s the most important next step. How do you utilize the resources available to you, whether they’re financial or tissue samples or data? And a lot of those sort of decisions and ways to think about things are really not taught to you in either medical school or in PhD programs, those sort of operational decisions and strategic decisions or are really kind of the bread and butter of the business world. And so, I felt that it would make sense to focus some time and do an MBA to try to pick up some of those skills. And I really do think that that’s had an important impact on the approach to the CDCN has taken to research. We spend a lot of time thinking really critically about how to perform our research studies and how to make sure we’re making the most of every dollar that we raise for research,” explains David on the need to have founded CDCN and attended Wharton Business School. 

David takes over

Since he received the diagnosis, David, had been collecting weekly blood samples and keeping track of his immune system, tabulating results.  This time when the disease returned, he persuaded his doctors to remove a piece of a lymph node, test it and save it for future research.

After a round of chemotherapy, he improved enough to be discharged and started looking into what the tests might reveal. He observed that his immune system seemed to have started gearing up for a fight even though there was no apparent threat. His T cells (one of the key weapons in the body’s immune system) had started activating and he had started producing a protein call VEGF in excess. This protein instructs the body to make more blood vessels. 

At the hospital

He hypothesized that probably the problem was with one of the body’s communication lines, triggering overproduction of VEGF and T cell activation. If he could get his body to shut down that communication line (known as the mTOR pathway) he might be able to stop his immune system from overreacting and prevent a relapse.

He and his doctors explored existing drugs that were known to shut down the mTOR pathway. They discovered Sirolimus, also known as Rapamune, commonly given to kidney transplant patients to prevent their bodies from rejecting the organ. The drug had been on the market for years and was known to have few serious side effects, but had NEVER been used for Castleman disease. 

David chose to become his own test subject.  

“I’d recently graduated from medical school and was certainly very early in my career. But I made what was probably the most important decision of my young life to try this drug that had never been used before to treat Castleman disease,”. 

Dr. Fajgenbaum, an assistant professor in Penn Medicine’s Translational Medicine and Human Genetics division, continues to lead research efforts of his own disease. And hopes to help raise awareness about Castleman disease through his work. 

David, recently celebrated 5 years of remission. During this time, he got married. “And then my wife, Caitlin and I had our first child, Amelia, who has brought us so much happiness in these first six months. And it was something that certainly when I was on my death, but I never thought that I would see. And, it’s been the most amazing experience of my life to be a father.”

INTERVIEW : Caitlin Fajgenbaum, Wife Of Dr. David Fajgenbaum

David & Caitlin

Know Very Well That This Fight Is Not Over

What is the one quality of David that you admire in him?

Caitlin : One quality that I admire about David is his drive and passion for the work that he does. When David decided that he was going to make it his life’s mission to find a cure for Castleman Disease it was never a question, he put his head down and ploughed through the obstacles that were standing in his way.

Were you part of his decision-making process – I mean when he decided to try new drugs on himself, go to business school?

Caitlin: When he decided to try a new drug on himself it was a game-time decision. All his research thus far had led him to make the most educated decision. It was pretty terrifying for the both of us that there were no other patients on this drug for his illness, but I was supportive if he and his doctors thought this was the right path to take. I was also part of his decision to attend business school. He had realized that while he was sick it wasn’t only the doctors that make an impact on your life, but also the role that the drugs play. He felt that attending business school would bring his medical background to a new light and help him in finding his cure.

What was your thought process during his relapses? Were you terrified?

Caitlin: I handled each of his relapses a little differently. One in particular I can remember feeling like we were heading into war. He had been through this before and could get through it again with no problem. I felt confident and knew he was going to come out of this. The last relapse 5.5 years ago was difficult. We were engaged at that point and things felt a bit more “real”. He wasn’t bouncing back as quickly as he had in the past and I knew something wasn’t right. Thankfully he pulled through, but it was a bumpy road.

You were with him throughout and so you have seen him go through quite a bit, how did you get through those years, what was your support system?

Caitlin: I would not have been able to get through this without my family and friends. My parents were a constant support system for me. My mom came out to Little Rock, AR to be with me and to support Dave. My close friends called/texted me constantly to make sure I was doing ok, and I was so thankful for that.

Now that the book is being released soon, would be great to hear your thoughts on the book and the process of writing it.

Caitlin: I am very excited for the book to be released and for Dave’s story to be heard by more than just our family and friends. It is an inspiring journey and one that I hope will inspire readers to reflect on their hopes/prayers/wishes and then turn their hopes/prayers/wishes into action, like David did with identifying a treatment for himself. The process of writing the book was a whirl of emotions for me. I had to dig back into my memories that I had tried so hard to tuck away. With David being in remission I try to stay positive and look forward to the future, knowing very well that this fight is not over and there is still lots of work that needs to be done.

Book Excerpts

Dying, With Hope

The truth is that no one knows everything, but that’s not really the problem. The problem is that, for some things, no one knows anything, nothing is being done to change that, and sometimes medicine can be frankly wrong.

I still believe in the power of science and medicine. And I still believe in the importance of hard work and kindness. And I am still hopeful. And I still pray. But my adventures as both a doctor and a patient have taught me volumes about the often unfair disconnect between the best that science can offer and our fragile longevity between thoughts and prayers and health and well-being.

This is a story about how I found out that Santa’s proxies in medicine didn’t exist, they weren’t working on my gift, and they wouldn’t be delivering me a cure. It’s also a story about how I came to understand that hope cannot be a passive concept. It’s a choice and a force; hoping for something takes more than casting out a wish to the universe and waiting for it to occur. Hope should inspire action. And when it does inspire action in medicine and science, that hope can become a reality, beyond your wildest dreams.

In essence, this is a story about dying, from which I hope you can learn about living.

– Excerpted from Chasing My Cure by David Fajgenbaum – Copyright © 2019 David Fajgenbaum

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