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 friends. It's about filling our lives with the arts, being creative, enjoying the outdoors and all that God set into motion when He began his work and declared that it was good.

I fail at many of those on pretty much a daily basis. But weeks like the one that we had with our grandson, it's a reminder that being intentional will reap huge returns. Our grandson may not remember a lot of details, if any, from his birthday week, but I believe the time that we spent with him will add to the foundation that we're building in his life. He may not recall with much clarity the big fish at the aquarium, or the prize roosters at the fair, or the trail through the woods. But he'll know that Mawmaw and Pawpaw love him, and that spending time with him is one of our greatest joys.

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 make a difference in my condition and recovery? Absolutely it did. Michele's presence grounded me, providing a point of reference that I could hold on to when my mind tried to create realities of its own. Now, there were likely other factors, including the approach to sedation and mobility, but I'm convinced that the greatest impact came from having my wife by my side. Not only did this ground me in reality, but it also took away the helplessness I felt knowing that she was having to navigate the details of this experience without my help. Did she have somewhere safe and comfortable to rest and stay? Did she have convenient access to food? How was she managing to keep clean clothes and necessary supplies across this extended stay? These sources of anxiety disappeared when she was sharing the ICU room with me.

And just imagine what this did for her. She was there when various doctors made their rounds, stayed better informed about my condition and plan of care, and felt comfortable knowing that she was nearby to help with any small needs that might go unmet in that more restrictive environment. During the spring and summer of this year, I've experienced three separate ICU visits at UAB, totaling 32 days. I was intubated during one of those stays, spending four days on a ventilator to help overcome a myasthenic crisis. Not once during those days did I lose touch with reality. And while I know several factors contributed to avoiding delirium, I credit much of it to having a familiar voice, a friendly face, and the presence of my life partner and best friend there by my side throughout the ordeal.

F may be the last and newest letter added to the A to F bundle, but it may well be the most powerful. I really hope more medical centers adopt this approach to family engagement and empowerment. It not only makes a difference while the patients are in ICU, but it can have a lasting impact on the health of the patient for months and years to come. If you're enjoying the Live Life Rare podcast, please subscribe to its companion newsletter at liveliferare.com. Thanks for joining us on this journey to discover and pursue what a rare life means to us all.

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 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 [email protected] 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.

Hello, I'm Stephen Smith and today we're going to talk about when you're too tired to swing, when there's just not enough fight to get off the mat. Living with a rare disease is often challenging. Sometimes it can be downright exhausting. You know the feeling. You've hit the mat so many times you don't have the strength to lift your shoulder, to pull yourself back up by the ropes, or to take another swing at your adversary. All you want to do is lie there until the room stops spinning and the pounding in your head subsides.

But you can't. There's too much at stake. Too many people are depending on you, and deep inside you wonder whether you'll be able to get up at all if you stay down too long.

How do you cope? For starters, remind yourself that this is a long match. The round you're in at the moment will eventually end. You'll hear the bell and retreat to the corner.

And once you're there, allow your support system to help you prepare for the next round. Let them give you encouragement. Medical care. Strategy adjustments. Be it friends or family or your online community, make sure you have the right people in your corner.

When a new round begins and one always does, you have to put the previous rounds behind you. Focusing on what you didn't do right before only distracts you from the fight ahead.

Some of you listening to this podcast today have been in the ring for decades. My match with a rare disease, myasthenia gravis, started nine years ago. There have been times I thought I was down for the count, and other times when I've prematurely raised my gloves in the victory that I only thought was mine. There's always a surprise. An uppercut you didn't see coming. A quick jab before you smack into the mat. Down again. But not for the count. Close, perhaps, but not for the full count.

Today you may feel like you're too tired to swing. You may be counting to ten in your mind as you tell yourself, "I just can't go another round."

But you can. Take some deep breaths. Listen for that bell that sends you back to your corner. Regroup. Focus on the challenges ahead and how you plan to attack them. And never forget that there's a crowd of fans surrounding the ring. We're cheering you on. Listen to our voices. Hear our words of encouragement. Working together, we can all stay in the fight. Visit liveliferare.com to subscribe to the companion newsletter to this podcast. And leave comments and start a conversation there.

And thank you for visiting and listening, and keep walking with us on this journey as we all discover and pursue what a rare life means to each of us.

Hello, I'm Stephen Smith, and welcome to the premiere episode of Live Life Rare. This podcast is a companion to the newsletter that you will find at liveliferare.com. So what is this work all about? I guess we could sum it up by saying that Live Life Rare is my attempt to help you discover and to pursue what a rare life means to you. Really has its roots in the fact that I wrestle with the rare disease myasthenia gravis, and through the years, living with this disease has taught me a lot about living life in general. About discovering what it means to live outside our comfort zones, to live with the challenges that life brings us. To live in a way that steps outside of the normal, that steps outside of the unintentional, that steps outside the paths that we expect to walk every day, the things that are considered normal and to live a life with intent.

I've been working on this project for a while, and this year in particular, roadblock after roadblock has come my way, including several unplanned trips to the emergency department that landed me in our university hospital. For several days across the spring and summer of this year, in total, I spent 32 days in the hospital, and a few of those days even on a ventilator. And I must admit, there are times that I questioned, "Should I even continue this process?" Every time I go to write a new article for the newsletter or plan to start this podcast, some new challenge would come my way and I would have another setback with MG.

But then I ran across a quote recently from the professor and author Brené Brown, and it goes like this. "One day you will tell your story of how you overcame what you went through, and it will be someone else's survival guide." I thought a lot about those words, and they really did bring me some clarity. And I thought about how when I send out a newsletter, post an article on Facebook, engage with people online who are also dealing with challenges, including rare diseases and and other issues, that it seems to be an encouragement to them to see someone else struggling with the same thing and trying their best to live in a way that is still a very full life.

As I share these issues, writing about it and now podcasting about the rare disease experience, it helps me process these events. But, you know, it's also creating an archive of lived experiences that may well help others along their journeys. There have been times since my diagnosis in the fall of 2014 that I've had all my symptoms under control, to a large degree. Other times, I've floated somewhere between being mildly inconvenienced to fighting for survival. And throughout these stages, yeah, it would have been helpful to read and to listen about others who've walked this path specifically, especially in the early years of my journey. Rare disease is a very personal experience. Myasthenia gravis in particular can present in wildly different fashion among otherwise similar patients. And yet, reading the experiences of others can help patients and caregivers understand what is possible and perhaps be more prepared when circumstances change.

Just as important to the mission of this project with the newsletter and the podcast is the encouragement that I hope readers will take from these stories. MG has put my family and me through some tough times the past few years. And yet we have survived and drawn closer through the challenges, actually. Some of the things I've learned include:. 

It's hard.

You are not alone.

And you can get through this.

You know, I really love Brene Brown's concept of a survival guide. I don't know if that's an accurate label for what this project is trying to be, but I do hope that those who struggle with MG, with rare disease, and with the adversities of life in general, will find something here to help them move forward for one more day. That's what I'm doing right now as I try to regain control of myasthenia gravis. And this podcast and the companion newsletter are a big part of that.

Thanks for joining me on this journey and if you enjoy this show, please keep listening to new episodes. Share them with those that you know who are struggling, caregivers and those who suffer from rare disease. But also, we're going to be touching on topics that are a little more broad in general, in terms of helping people see that the pathway they choose through life can be something unique, something intentional, something rich and beautiful. That they can indeed live in a rare way. Thank you for joining us on this journey. I'm Stephen Smith and you're listening to Live Life Rare.