Hello, I'm Stephen Smith, and, you know, sometimes it feels like living with a rare disease should come with a degree in project management. It's not for weaklings. It takes a lot of strength to manage a rare disease. Now, that might sound doubly strange coming from someone whose rare disease impacts their muscles. But throughout a tough year with MG, telling my story over and over to doctors, I've been reminded of how important management skills are in navigating a chronic illness.
For example, I wrote the original article that became this podcast episode from a neuro ICU bed. It was the start of a challenging spring and summer. But more on that in a moment. Let's talk about management.
My first job in management. I was 16 years old. I started as a cook at Kentucky Fried Chicken. I must have shown some initiative because five months later I was cross-trained on the front and promoted to assistant manager on the night shift. I learned a lot about managing people, working processes and systems, and dealing with customers. I'm sure I was awful at it, but it taught me a lot.
Fast forward to New Year's Day 1996, when I left a newspaper career to launch a marketing and communications agency with my wife. It was just us. But by the end of the year, we'd hired our first employee. Fast forward again to 2020 and we sold the company to a strategic partner, having grown it to 30 employees serving clients throughout the eastern half of the US and landing on the Inc 5000 list in the process.
I give all that as background to establish the fact that I have many years of management experience, and I've put much of it to use in managing myasthenia gravis. A perfect example is the series of events that led me to the ICU bed earlier this year. In 2021. I was doing well with a course of treatment that consisted of oral meds and IVIg infusions every four weeks. For the most part, everything was under control. Then in January of 2022, I was switched to a different brand of IVIg. I had a reaction and was hospitalized. After five days, I came home to resume my IVIg infusion but at a lower dose — and I never fully regained control of my symptoms. Vyvgart, a new medication from argenx, was the hot topic in the myasthenia gravis world. Results sounded promising. Working with my neurologists, we pursued approval to try it. It took months, but I finally started the new infusion. Results were good but didn't last long. And then there were the dreaded respiratory infections. Known side effects of the drug. Some cycles weren't too bad, but the final cycle, in early 2023, was awful. The infection exacerbated my symptoms and I started declining. My neurologist and I decided to return to IVIg. By the time we could jump through all the hoops with insurance and the like, it was too late. I completely lost my swallow and couldn't take my meds. I had to go to the emergency department. They admitted me, did two more days of IVIg to see if that would get me over the hump. It did not.
The doctors began a series of plasma exchange. I did better and then I got worse. And it was just like being on a roller coaster. I finally had ports placed in my chest to do monthly outpatient plasma exchanges, followed by in-home IVIg. I continue on that course and it's helping.
The details of this story involve spreadsheets to track treatment dates and outcomes, charting symptoms, communicating with insurance and prior authorization contractors. Scheduling with infusion companies. Presenting quality data to my neurologist to help him make informed decisions. Advocating for myself when I do not agree with certain actions by various parties. An example of that would be when I arrived at the emergency department. On one occasion this summer, a resident was all but ready to intubate me. It wasn't time for that, and I knew it. We would have been skipping an important step. You really have to advocate for yourself.
Those living with a rare disease have much more to deal with than the disease itself. I've worked with some fine project managers in my career, and I'm sure they would be impressed with the skills needed to manage the rare disease world. What is your experience been like managing a rare disease? Email me at hello@liveliferare.com and visit liveliferare.com and subscribe to the newsletter that is the companion to this website. I'll be talking with others who are walking similar paths and sharing more about my story. Thanks for coming along for the journey as we work together to discover and pursue what a rare life is really all about.
