Vexas: Dan's Story

Dan Davison 

Star Prairie, Wisconsin    
Vexas
Transplanted in 2021  

Many thanks to Mayo Clinic and the American Society for Transplantation and Cellular Therapy for helping us share Dan's story. 

You are among a small number of people successfully treated for VEXAS, a rare and relatively newly identified disease. First off, let’s begin with a definition of VEXAS, which many people may be unfamiliar with.  

VEXAS syndrome (Vacuoles in bone marrow cells, E1 enzyme defect, X-linked, Autoinflammatory, and Somatic) is a severe, adult-onset inflammatory and blood disorder, caused by a genetic mutation that triggers the immune system to attack healthy tissues. First identified in 2020, the name is an acronym of its defining features.  

Given how unusual your symptoms were, what was the process of diagnosis like?  

Well, 2013 was a very tough year. My wife passed away in April from breast cancer and by November, I started feeling off. I had chills, bloodshot eyes, and a rash. At first, I didn't know if I felt so crummy because of grief, or if I was just getting older. I went to my local doctor, who shipped me up to a hospital in St. Paul.

What did the doctors in St. Paul find?

I was in the hospital for four days, and they tested me for everything from AIDS to Lyme disease. My blood counts were off, but they couldn't give me a diagnosis. On the third day, my nose and ears started swelling up—I looked like Dumbo, the elephant. An older rheumatologist evaluated me and suspected relapsing polychondritis. He started me on high doses of prednisone, which cleared up my ears, nose, and eyes. But the one anomaly was my low blood counts; the steroids didn't touch those.  

So, you’d stumped the doctors at the local care level. What happened from there?  

My hematologist in St. Paul agreed it was polychondritis, but insisted something else was going on, so he referred me to Mayo Clinic. At Mayo, they re-ran the tests, confirmed polychondritis, but still suspected another underlying issue. For three or four years, I shuttled between hematology and rheumatology at Mayo. Each specialty thought the other should be able to figure it out, but they didn't collaborate as much as I had hoped. When I said to a Mayo hematologist that I was a bit of a Zebra, he laughed and replied, “Dan, you’re an albino Zebra."

That’s when I took matters into my own hands and joined a Facebook group for relapsing polychondritis. There was a doctor in that group, Dr. Ferrada from the NIH—who had polychondritis herself and, alongside Dr. David Beck, was trying to identify other patients with complicating issues. I filled out a questionnaire explaining that my polychondritis responded to steroids, but my blood issues never went away.

Almost immediately, Dr. Ferrada responded. She called me on a Friday night while I was out to dinner with my partner, Sue. Dr. Ferrada said they had just discovered a new disease and asked if I’d send a blood sample. They FedExed me a vial, I sent it back, and she called to say I was patient number 25 in their cohort for this newly identified condition: VEXAS syndrome.

In early spring 2020, Sue and I flew to NIH in Washington D.C. for extensive testing. They confirmed I was clinically akin to the rest of their emerging cohort. My timing was truly fortunate because this was the exact moment that COVID-19 was hitting. The day we left, they locked the doors of NIH behind us. Eventually Drs. Ferrada and Beck published their research paper in the New England Journal of Medicine, which helped other doctors start to identify VEXAS patients worldwide.

What was your reaction when you received the diagnosis?

It was a mix of relief and fear. Hearing that out of the 25 patients in the initial study, only seven of us were still alive was hard. Dr. Beck reassured me that one of the patients had lived to 95 and died of other causes. So, VEXAS wasn't a death sentence, but it was scary because there were so few proven treatments, much less a cure, available.

During those years when your disease could not be consistently treated, how did you manage daily life and work?

For 40 years, I worked at a small community bank, which I loved. And I continued to work there for five years, after my symptoms began, before retiring in 2018. During that period, I didn't feel terrible, but I was going in for two units of blood every 10 days or so. When I’d feel tired, draggy, and out of breath, I knew it was time for a transfusion.

How did you first hear about stem cell transplant as a potential cure for VEXAS?

NIH and Mayo kept telling me that a bone marrow transplant was the only real option; at the same time, they didn't have a standard protocol or approved drugs for VEXAS in transplant. A hematologist at Mayo took on my case, working diligently with NIH to establish a protocol. I didn't really want to be a guinea pig, but I had no other choice or option for a cure. Miraculously, my private insurance approved the procedure in just seven days.

What was the stem cell transplant experience like for you and your family?

Before I had the transplant at Mayo in July 2021, I did my research. So, the procedure itself didn't scare me. The only scary element was that it had never been done for my exact disease. I was patient number one for VEXAS transplant.  

Mayo did a good job telling me in advance what to expect as far as how I would feel, my appetite, energy level, etc.  Looking back, those were the things that were most difficult. I’m a rather large guy at 6’4” and normally have an appetite that goes along with it. During my worst symptoms, all I could eat was bacon and pears. Even drinking liquids was difficult.

But the hardest part came post-transplant in the hospital. I developed severe confusion and don't remember 95% of those five weeks. I didn't know who or where I was, or even who the president was. My son was in the room when the doctor asked where I was from, and I could only blurt out "Sheboygan" because the US Golf Open was being held there. When I finally came to, I thought a week had passed, but they told me I’d been out of it for five weeks. My doctors still aren't entirely sure what caused it. And it’s not at all a typical reaction to SCT.

Beyond that, the logistics were tough on my family. Mayo required me to stay 100 days with a full-time caregiver, within 10 minutes of the hospital. My son, Patrick, came to help from Idaho, and my daughter, Lauren, took time off work to come. Sue filled in on weekends and other times around her new job, and several other family members helped too. I hated imposing on them, but I needed their help.

How are you feeling today?

I feel really good! My stem cell doctor still calls me a "poster child" for transplant because I never developed Graft-versus-Host Disease (GvHD). All my follow-up tests show no signs of disease. I'm 68 now, I walk, ride electric bikes, and play pickleball. Physically, I can do pretty much everything I did before.

You now spend time peer-counseling newly diagnosed VEXAS patients. How did you get into this, and what is your approach when talking with patients?

Since I was the first person in the world to be treated with a stem cell transplant for a known case of VEXAS, I asked Mayo Clinic to share my story and history with patients who are concerned about getting a stem cell transplant or have questions. I have spoken with many, an tried to relate my experience and hint at what they might be facing.  

When I talk to people I focus on listening. First, you have to hear what’s on their mind and draw out their concerns. Then, I will try to understand if they want details about my experience or just reassurance. Having been so confused and scared myself back when there was nobody to talk to, I really enjoy helping others navigate this process. I’ve given Mayo a blanket "yes" to share my phone number with prospective patients.  

What does fun look like for you and your family these days?  

Well, Sue and I bought a winter home in Florida in April of 2023 and love it as an escape from Wisconsin winters. We love boating in both Wisconsin and Florida and spending time outdoors. For me, an ideal day is spending time with Sue and all our extended family including both our “kids” and my granddaughter, Maeve (who was 3 at the time I had my transplant!) and my one- month-old granddaughter, Charley. They are both an absolute joy and of course, the best grandchildren in the world.  

We especially love to spend time on or near the water, cooking out and relaxing around a campfire. Very low-key and with family is my idea of a great day.

Finally, looking back, what are the biggest lessons or "pearls of wisdom" you’ve taken from the experience of dealing with a disease as rare as VEXAS, and in recovering so fully?  

Two things. First, your baseline health going into a major medical event is super important, get and stay as healthy as you can! I’m not a health nut, I eat too much red meat and too much candy, but I try.  

Second, be your own advocate. If I hadn't joined that Facebook group, I would be dead. Often, we default into complacency, people think, "Well, the doctor said this, so that’s what we’re doing." Caring for my wife when she got sick taught me that you can't always take what you're told at face value. If you have a unique disease or unusual symptoms, keep pursuing a true diagnosis and make sure your care team isn't ignoring you. You deserve their best thinking and attention!

Mayo Clinic is one of the largest providers of bone marrow transplants in the United States. It has performed more than 10,000 stem cell transplants at its campuses in Arizona, Florida, and Minnesota. Since 1963, Mayo Clinic has been at the leading edge of transplant research worldwide, enabling patients to be among the first to benefit from innovative therapies and clinical trials. Learn more about Mayo’s collaborative care and excellent results at mayoclinic.org.  

The American Society for Transplantation and Cellular Therapy (ASTCT), formerly known as the American Society for Blood and Marrow Transplantation, is a professional society of more than 2,200 healthcare professionals and scientists from over 45 countries who are dedicated to improving the application and success of blood and marrow transplantation and related cellular therapies. ASTCT strives to be the leading organization promoting research, education, and clinical practice to deliver the best, comprehensive patient care.