ALL HEART

Recently I was invited to speak at a cardiac conference on the campus of Stanford University in Palo Alto California. Today I would like to share with you why this was a significant event for individuals living with different types of muscular dystrophy.

First of all, there is only one way I am ever getting to see a place like Stanford: as an invited guest. The 8000 acre campus is a small village, and very beautiful. I read that the current acceptance rate is just over 4% and undergraduate tuition, including room, board, and food, is a shade north of $92,000 a year. Ouch. I’m also going to assume that no one pays that much since the place is full of wicked smahht kids. (as we say in New England). My wife and I were struck by the spacious parks, bike trails, and gothic design of many campus buildings. Because the place is huge and I had a job to do, we only saw the medical college area and did a quick drive by of some other parts. We actually got lost….that’s how big it is. This could also possibly have to do with the fact that neither one of us is bright enough to be on the grounds in the first place.

On with the show. I was asked to attend in order to speak to a group of 40 cardiologists from around the country who specify in heart function in kids and adults living with different forms of muscular dystrophy. As the condition progresses, the heart, since it is a muscle, can get to the point where it has trouble keeping up with the needs of circulating blood through the body. The heart pumping force diminishes, leading to the use of medication to regulate the rhythm and help the heart squeeze. As one grows older, it becomes harder to exercise, further weakening the heart, and causing it to retain fluid which can lead to swelling of the ankles, and a host of other issues.

When medication no longer holds the answer, many cardiologists begin to talk about “advanced therapies”, which is a nice way of saying have surgery to (A) implant a pacemaker/defibrillator, (B) have surgery to attach an LVAD, or (C) perform a heart transplant if the individual’s lungs are functional enough to handle the surgery.

My role was to speak about my illness last winter, which resulted in scenario A above, and to present a video interview that I did with a young man living with Duchenne muscular dystrophy (a different type than I have) who had a heart transplant when he was 13. He is now 26 and living a full life, while feeling good. Let me explain to you why this meeting has far reaching implications.

As of now, there are very few medical centers/hospitals in the United States that will consider heart transplant in any form of muscular dystrophy. Twenty years ago, there were none. The simple fact is that, as science and researchers better understand the condition, patients are living longer, stronger lives. Science is now lagging behind. We have created a generation of people living with this muscle disease who are here well into adulthood. When I was diagnosed in 1985, the average lifespan for my type of MD was foggy at best, and people living with Duchenne (the most severe form) were lucky to live a quarter century. Thankfully, in most cases, with treatment and quality care, this history has been rewritten. I have friends living with Duchenne that are in their 40’s and feeling good. It’s awesome. So now what? These people begin to look at how long their heart and lungs will go before the disease threatens the function of both.

Enter the cardiologists that assembled at Stanford. All of the men and women in the room were highly intelligent, motivated, forward thinking human beings who were tasked with breaking into working groups to begin to come up with a way to communicate to the medical (and general) public that heart transplant in a human living with different forms of muscular dystrophy should, at the least, be considered in centers that have had success in limited cases already. To move the needle, the group is coming up with a set of suggestions, which will then be shared with more centers around the country that have been certified as centers of excellence in the care of people living with muscular dystrophy. If a center is unable, or reluctant to take on a transplant patient, the hope is that there will be a network to refer them to so they can make the best decision for themselves and their family while weighing all of the risks, and determining if their quality of life before and after major surgery would live up to their expectations.

I was honored to be asked to attend, along with Vanessa, to speak about my feelings, observations, and take questions from the group. The simple fact that this is being discussed increases my hope for the future for all of us living with muscular dystrophy. Progress is being made, and it’s exciting to be in on the ground level with a small bunch of people who are changing the world.

No, I am not considering a heart transplant. I’m doing fine.

Stay safe, stay awesome, and stay tuned. Did I mention that the Golden Gate Bridge is lovely at this time of year?