Life Life Rare Stephen V. Smith

Wes Michael is founder and president of Rare Patient Voice. He joins host Stephen V. Smith to discuss how Rare Patient Voice connects patients and caregivers with the opportunity to voice their opinions through surveys and interviews to improve medical products and services.

Hello, I'm Stephen Smith and today we're going to talk about being present. Our one and only grandson turned two years old recently. He had a small party and he was blessed with many gifts from his family, mostly toy tractors, dump trucks, cars, and things like that. And while the things we bought for him were special, it was, it was really the week leading up to his birthday that we will always remember. Highlights of his birthday week included rides with Mawmaw and Pawpaw, meals at his favorite restaurants, a visit to the fabulous Tennessee Aquarium. Seeing prize chickens and live music at the county fair, and a hiking trip at our local state park. By Saturday night, we were exhausted, but full of love and gratitude for this little boy and all the light and love that he's brought into our lives.

You know, I've read that around age two is about the youngest point at which humans form lasting memories. Now, whether he grows up retaining this birthday week or not, we don't know. But one thing we do know for sure this boy knows that he's surrounded by family members who love him. That weekend, his parents said that he would be playing and then just randomly start saying, "Mawmaw and Pawpaw" to himself. And you know, that tells me all I need to know: That we were on his mind and in his little heart.

The years I spent running our small business, a marketing agency, enforced in me a loop-closing mentality. Success consists of projects that are done right. Made up of steps that need to be checked off in order to accomplish what's necessary to achieve your goals. I have to consciously work at setting that mentality aside when it comes to the business of life. You see, that part of my brain would have said, "Buy some toys? Check. Attend a party? Check. Sing the happy birthday song? Check. Success." If I had allowed that mentality to rule, I would have missed so many blessings that weekend. You'd think that a few years of wrestling with a rare disease would have taught me to slow down, to focus on the truly important things in life, and to take time to enjoy the small gifts that come our way each day. Well it's been nine years since I was diagnosed with myasthenia gravis, and I still struggle with losing myself in the whirlwind of activity.

In Oliver Burkeman's book "Four Thousand Weeks: Time Management for Mortals," the author brings to light the fact that if we live to be 80, we have just over 4,000 weeks on Earth. When you look at it like that, you know the truth is really staggering. Our grandson has lived just 100 of those, while Mawmaw and Pawpaw have logged 70% of this total. By the time he's our age, we'll most likely be long gone, and all he will have will be "photographs and memories," to quote Jim Croce. If we ever have a question about how we should invest our resources in our grandson's life, that should answer our question.

I don't know anyone who would say they've lived a life of no regrets. But as I strive to live a truly rare life in the years I have left, my focus needs to be on filling those days with the things that will matter most in the end, both to me and to the people whom I'm blessed to have in my life. Sometimes people are confused by what I mean when I talk about living a rare life. Is that just about learning to live with a rare disease? That's certainly part of it. But the bigger picture is crafting an existence wherein you step outside of the normal and the routine, and you create a world that enriches you and those around you. What does a rare life look like for you? Well, for me, it's pouring myself into my grandson's life to help him become the person God would have him to be.

It's investing in my wife and always being mindful that she's not a supporting cast member in the story of my life, but the leading lady in every scene. It's being a friend and a leader to my children, setting a good example of how a husband and a father conducts himself. It's about taking time for extended family and fri

Hello, I'm Stephen Smith and today we're going to talk about how family is really the best medicine when you're in ICU. I can still see the images today. The noise was unnerving. The banging and the clanging reverberated throughout the dark halls of Trinity Medical Center in Birmingham, Alabama. This is where I'd been an ICU patient for weeks. It was early 2015, and from my bed, I caught glimpses of maintenance crews breaking down the nursing stations, loading panels of equipment on the long, flat carts, and wheeling everything into freight elevators. My mind raced with questions. "Where are they taking everything?" "Will they bring it all back before the morning shift?" And most alarming of all, "Will they remember that I'm here? Or will I be left behind with no one to check on me throughout the night?"

Well, of course, all this commotion, as if they were striking a stage production or something, it was, it was only happening in my mind. And although it'd be several months before I had words and context to understand it, I was suffering from ICU Delirium, fostered by a combination of factors such as sedation, immobility, and isolation. In Tennessee, the Vanderbilt University Medical Center, they have a Critical Illness, Brain Dysfunction, and Survivorship Center. And it focuses on advancing knowledge, education, and models for care for people affected by critical illness. Among the work the center does is the ABCDEF bundle, or the A to F bundle. This bundle is a framework for aligning and coordinating care for critically ill patients. Each letter represents an area of focus, with the F standing for family engagement and empowerment.

If you go to the CIBS center website, it explains it this way. "Good communication with the family is critical at every step of a patient's clinical course. And empowering the family to be part of the team, to ensure best care is adhered to diligently,will improve many aspects of the patient's experience. The F was recently added to keep patients and family as the center and focus of this care." Looking back, my ICU experiences in 2015 served as a study of the various approaches to family engagement. After a few weeks at Trinity Medical Center, I was transferred to the University of Alabama at Birmingham, UAB, Medical Center, where the visitation rules were considerably different. At Trinity, family visitation was restricted to four time slots per day, each one only lasting 30 minutes. This schedule was strictly enforced, except for the occasional nurse who would smile at my wife, Michele, and tell her she could stay a few extra minutes. This schedule created a sometimes dramatic emotional cycle for me with anticipation and excitement then relief, then anxiety, and then depression rotating throughout the day. Those 30-minute periods were blessings and curses, bringing joy at seeing my wife and sometimes other family members, then ending with a crash of loneliness and longing for the next visitation.

I'm sure at some point in the progression of medical protocols that there were many good reasons laid out for limiting family visitation. I mean, among them was likely a concern that visitors would somehow interfere with the health care provider's work of taking care of the patient. After all, ICU patients are critically ill, and the medical team needs to focus on caring for them uninterrupted, right? Well, in my experience, an assortment of monitors did a good bit of the work, and I was not surrounded by nurses and doctors around the clock. With all the time I spent alone in Trinity's ICU, there was no reason for such a restrictive visitation schedule. We were quite surprised when I was transferred to UAB. We were told that Michele could stay in the room with me around the clock. The only exception was a 30-minute window during shift change, when they asked that she go to the waiting room. And in subsequent ICU visits, we've learned that even that restriction has been lifted.

Did this change ma

Hello, I'm Stephen Smith and today we're going to talk about grief. I've been thinking a lot about grief lately. The word often conjures up a widow mourning for her partner. But, you know, grief has a much broader definition. Grief is part of processing the loss of something important. You know, that could be your career, your health, a hometown you left behind, or really any number of things.

A psychologist who worked with me as a business coach, introduced this concept to me a couple of years ago. My wife and I had sold the marketing agency that we started, and we ran for 24 years, and I was working through some career shifts. During one of our coaching sessions, she said to me, "You haven't given yourself space to grieve." "Grieve? I said. I was really surprised at her statement. I said, "Well what do I have to grieve?" I mean, after all, this was exactly what I wanted to grow a business and find a strategic acquirer who could take our business to the next level. Someone who would care for employees and our clients. A company and a partner that would preserve the legacy of what we had built.

Well, my coach went on to explain that something very close to my heart was now gone. And it was no longer part of my life, and that I needed to acknowledge the impact of that. This business that had been the core of our family for a quarter century, you know, it was it was part of our lives and all of the wins and the losses and the highs and lows, those were very personal. Well, she was right. And I realized that despite my satisfaction with the deal, that part of me ached at a hole that was left inside of me. Something that we had built from scratch was no longer ours and I had to process that loss.

I thought of that recently when I reached a turning point in my journey with the rare disease myasthenia gravis. There have been times I believe the disease was totally under control and that my life could go back to what it was. Multiple work projects and home projects, traveling, networking, growing my business, enjoying recreational activities and such. I've been in a particularly challenging season with the disease, especially this year, and, you know, I've had to force myself to admit the truth. That my life's never going to be the same again. I've got to pace myself. I've got to adjust to a schedule that's less packed. I've got to take on fewer projects and generally just slow down. That realization, I guess, has been years in the making. But, you know, immediately after this became clear to me and I acknowledged it out loud... yeah, that's when the grief set in.

Some of the things now lost to me were important parts of my identity. This podcast and its companion newsletter are part of that redefining process. Along the way, I'll be talking with others who are on similar journeys and sharing more of my personal experiences. Thanks for coming along. I invite you to visit liveliferare.com and subscribe to the newsletter. And please share these resources with others you know who are looking to discover and pursue their own version of a rare life.

Hello, I'm Stephen Smith and today we're going to talk about living life inside the margins. As a child, I struck an unusual balance between grasping a lot of concepts for my age and completely overlooking the obvious. In school. When we started using ruled three-hole punched sheets of paper, I would turn in work that would get high marks along with comments that I needed to stay within the margins. I had no idea what the teachers were talking about. How I made it that far without understanding the role of those vertical lines down each side of the page. I'll never know.

It actually took several such notes from teachers before I understood. I was too quiet and reserved to ask, so I just kept writing and turning in pages with text from edge to edge. When a teacher finally showed me personally what I was doing wrong, I felt the liberty that only proper guidelines can bring.

For the past 30-plus years, I've written for newspapers, magazines, and clients, and I've really grown to appreciate those margins more and more. Staying within the margins as I take notes gives me more space on the sides to fill up with more notes and thoughts and follow-up questions and drawings and,  well ... that's the pattern of my life, see. Space is there for me to fill up.

Now that's a great way to live until life hands you something that's too large to fit in the margins. When you've filled up the page and you have notes and drawings all up and down the sides, top the bottom, what do you do when, for instance, you're diagnosed with a rare and incurable disease? That's a question I could not find a satisfactory answer to when it came my way in 2014. In fact, the only answer was to grab a clean sheet of paper and start over.

I pushed the old sheet to the side and with a new sheet in front of me, began to sketch out what life would look like moving forward. My first inclination was, "Everything." I can just write smaller and move everything over. When my condition grew worse and the complications became more complicated, I had to face the reality that this approach would simply not work. I'm far from finished with the task of moving things over, but the first thing I had to do was learn a new respect for those margins. If you fill them up right off the bat, there's no space for surprises. And surprises will come. So for me, I've started by moving those margins further in, creating more space around the edges.

Since being diagnosed with myasthenia gravis, the disease has taught me much about margins. MG will demand more of me at times and I will adjust or I will pay the price. Those are difficult words for me to say, because I want to walk to the edge and have a look for myself. I want to take opportunities to learn more, grow more, to do more, to see more, to be more. I want to see how many plates I can keep spinning while I go find sticks to add more.

But that's no longer a reasonable plan for my life. If I fill it up, margins and all, I'll end up missing out later on. I must learn to sacrifice the good in order to experience the great. Read John 15:1-2 for a great example. Life can be rich without being stuffed. I'm still learning how to pull back my margins. How are you doing with margins? Visit liveliferare.com to subscribe to the companion newsletter to this podcast and leave comments there to start a conversation. Thank you for joining us on this journey as we go along together trying to discover and pursue what a rare life means to us all.

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 tha