Rare Leader: Kyle Dempsey, President, Superficial Siderosis Research Alliance
May 27, 2021
Name: Kyle Dempsey
Organization: Superficial Siderosis Research Alliance
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Disease focus: Superficial siderosis is a slowly progressive chronic neurodegenerative condition due to iron toxicity from persistent subarachnoid bleeding. The human body has a limited capacity to rid itself of blood products in the subarachnoid space, but in ultra-rare cases, chronic or intermittent bleeding over the years to decades eventually leads to overwhelming amounts of toxic free-iron molecules. These toxic free-iron molecules are curbed by a protein called ferritin which together forms a layer of hemosiderin around the brain, brain stem, spinal cord, and optic nerves. Long-term exposure is toxic to the tissue underneath resulting in hearing loss, imbalance, dizziness, weakness, numbness and bowel/bladder dysfunction. Although easily identified by magnetic resonance imaging, superficial siderosis is often confused for other progressive neurological conditions such as multiple sclerosis, Parkinson’s, or multiple system atrophy.
Headquarters: Pewaukee, Wisconsin
How did you become involved in rare disease: Unfortunately, our daughter Mackenzie was diagnosed with superficial siderosis approximately eight years ago. We had the same reaction any parent would have trying to find out more information. When you’re in the rare disease world, there isn’t much information. You play that for a while, and you try to see as many doctors as possible who have experience. And when you find out there’s only two in the United States and just a few more in the world that have even heard of it, you’ve got to do a lot on your own. That’s how we started getting involved. The major first step was four years ago. We went to Washington D.C. for Rare Disease Week and the event put on by EveryLife Foundation.
Previous career: Currently work as a manager of business development in the meals and services industry.
Education: B.B.A. in production operations management from the University of Wisconsin-Whitewater and an MSEM in project and program management from the Milwaukee School of Engineering
Organization’s mandate: Our mandate is to find hope and treatment options for people with superficial siderosis. Except for one very outstanding doctor, Dr. Levy at Massachusetts General in Boston who found a drug called Ferriprox, there hasn’t been much research or anything new to come out except that you can have surgery to repair a dura leak to your cerebral spinal fluid. That’s a surgical procedure that just about any surgeon could probably do that has experience with dura and spinal cord surgery, there are surgeons at the Mayo clinic that are very experienced and experience is a key factor. There’s no research being done on superficial siderosis.
Organization’s strategy: Our strategy is changing, but we’re focused on medical research. That’s the piece that we feel we can have long-term impact on the most people and provide the most hope to people.
Funding strategy: We’re doing all of the grassroots fundraising that is done in the rare disease community. We’re also looking at NIH grants and grants from charitable foundations that focus on medical research. The grant part is still developing, but the grassroots fundraising has been very good considering the pandemic and everything.
What’s changing at your organization in the next year: We’re getting more organized. Because we’re so spread with basic email and Facebook and the ways people communicate, we needed to take it to the next level. We’ve implemented more technology—Microsoft teams, Salesforce—and we’re just trying to have more regular meetings. When you look at a condition like superficial siderosis, you’re talking about maybe two or three people in each state that have this condition. We’re spread out. And then when you look at the world, it makes it that much more difficult.
Management philosophy: My management philosophy for this endeavor is involvement. I’m looking for everybody in the SSRA organization to put their own spin on everything. I’m somebody who puts it together at the top, even though I don’t look at it as a pyramid. I try to help and consult where I can. I have some good management background, so that’s my personal strength. I try to get people where they feel most comfortable and where they can succeed. Rori Daniel has been outstanding in implementing technology and our website.
Guiding principles for running an effective organization: It’s the golden rule. I’ve looked at the way some of the Fortune 500 companies I’ve worked for in the past have done guiding principles. Some have been good at it and some not so good, but I believe that we’ve got to treat people how we want to be treated. We just need to be transparent. We need to be honest with what we’re doing. There’s no doubt that medical research is expensive, and the results are many, many years down the line, but if we do nothing, that’s exactly what’s going to happen.
Best way to keep your organization relevant: We’re relevant because we’re dealing with people’s lives. We’re trying to give people hope. Superficial siderosis is not a fatal condition. We’re lucky in that sense, but the folks who actually have superficial siderosis struggle and have some serious issues that are similar to MS or Parkinson’s as they progress. It’s a long progression. It’s not quick, but over time they do have some serious medical conditions.
Why people like working with you: I’m down to earth. I’m able to speak to people at all levels. I try to be inclusionary, bring people in, and listen. I’m always looking for ideas. I try to make it fun. I try to make everything I do fun in some regard. What we’re doing is very serious and it can get depressing if you think about it too much. We try to have some fun while in whatever we’re doing.
Mentor: I have a lot of mentors. The one I want to emphasize here is my dad. He just turned 83 and he goes in a straight line and does not to let things bother him too much. Nothing ever seems to get him down. He has a lot of reasons to have been depressed. I had an older brother who died very young. My son died in a car accident that caused the superficial siderosis for my daughter. My mom died fairly young of a heart condition. If you look through his life, he has a lot of reason to have things affect him, but if you ever met him, you’d never, never think of that. He’s very positive.
On the Job
What inspires you: I like people’s reactions and to see a smile come across someone’s face. We had a Microsoft Team conference with someone from the U.K. this morning. That’s the first time I ever met him, aside from email. He has superficial siderosis and I could see his face. He’s so happy that he’s getting to participate in our organization. That makes my day to see somebody who wants to help out. You can see that they’re excited about it and they’re going to do something.
What makes you hopeful: At the end of the day, science will prevail. I completely believe that. We’re living in probably the best time ever. You can say that every time in the future unless something drastically changes because there’s always breakthroughs being made. There’s always some type of research that’s being done. That’s encouraging. That’s why I’m hopeful.
Best organization decision: To get started. We’re rare within rare. There’s been a few people in the last 20 years who have tried to do things and it never got off the ground because it’s so difficult. My partners in this are my wife Sue, and Rori and Gary Daniel—Gary has superficial siderosis. I met Rori and Gary three years ago at a rare conference. She got us off the ground because she’s very technical and very good at website development. She was able to get us to expand our communication links.
Hardest lesson learned: I learn one of those lessons every day. Unfortunately, there’s a lot of difficult things and the time commitment. I have a full-time job. I do this on the off hours. The time commitment has been very difficult. Hearing “no” is difficult, although we hear “yes” a lot. There’s always a reason somebody has for saying “no.” That’s even more difficult to hear because you think everything you’re doing is the most important thing in the world, but then you hear somebody has a baseball game with their 10- or 12-year-old. At that time in their life, that’s more important to them, which is totally understandable. I get that. Probably everybody in the rare disease community feels that whatever they’re doing is the most important thing in the world at that time.
Toughest organization decision: We can’t do everything that people want us to do. We’ve been laser focused on medical research. Until we get that, we can’t be as good at advocacy, public policy, education, even though we tackle all of those things. You can’t spread yourself too thin. The difficult decision is that we can’t do as much as we want to do.
Biggest missed opportunity: One of the clinical trials that we need to do is on Ferriprox, which is an iron chelation drug. It turns out that our story would have been very compelling to the founder of the company that made Ferriprox if he ever heard it, but he passed away about three years ago. I did not know that at the time, but I heard about his story through people in his organization when I tried to approach them about doing a double-blind study on the drug. It sounded like he would have taken a chance on us, but the company was selling the drug at the time. We had to start all over with the new company.
Like best about the job: I would say definitely it’s working with the people that I’ve met throughout the world who are happy that something’s being done. People throughout the world express their thankfulness that something’s being done and that they want to participate. There are some estimates that there are only 120 people in the world that have this condition. I feel that if there was more MRI, diagnosis and scanning done that that number would be a lot bigger, but there’s only 120 people in the world. I’ve talked to probably 20 or 30 of them. That’s a pretty big chunk of the patient population to have gone through. Everybody has such a positive outlook that at least we’re getting one voice and trying to all do this together.
Like least about the job: The fact that people suffer is difficult. People lose their hearing, any function that the cerebellum and the brain has—they lose that type of function. It’s a very debilitating disease for people that progresses. It’s usually slow, but everybody’s a little different, depending on how severe their case is. It’s very difficult to see that and not have more treatment options available for these people that suffer with superficial siderosis.
Pet peeve: Negativity. I can’t tell you how many times I’ve told my story and someone well-meaning wants to tell me why it can’t be done, how things are more difficult and even impossible at times. Maybe at the same time they’re giving me more motivation.
First choice for a new career: I’d like to do this full time if that’s ever possible. We need to find more funding and grants, everyone is in that boat. If I don’t do this full time, I would like to fund someone that can, we are a ways off before that happens but hopefully sooner than later.
Most influential book: Think and Grow Rich by Napoleon Hill, he has some classic inspirational quotes.
Favorite movie: I enjoy action-adventure movies.
Favorite music: I’m a product of the ‘80s. One of my interesting stories is I got to see the Grateful Dead toward the end of their career with Jerry Garcia while working security at a concert venue.
Favorite food: I try to eat the Mediterranean diet now and don’t always succeed, but roast beef and mashed potatoes would be high on the list if I was 20 years younger.
Guilty pleasure: Being from Wisconsin, we’re famous for our frozen custard. I stop in the afternoon for vanilla custard occasionally.
Favorite way to spend free time: Family time on the boat as much as possible on the weekends.
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