The purpose of this website is to track the adventures of Doug (that’s me) and Gracie (my 3-year-old Bernese Mountain Dog mix) as we set out to travel from New York City to Washington DC on a mobility scooter to raise awareness of, and research funding for, a little-known type of muscular dystrophy—facioscapulohumeral muscular dystrophy, or FSHD. This will be the main place where I’ll post a journal of our travels with photographs, video, and stories of the place we see and the people we meet along the way.
I have also included posts about a trial run we did in June of this year from NYC to Philadelphia. The purpose of that 5-day journey was to assess our readiness for this type of trip and to convince ourselves and others that we could actually complete a longer journey. There were a number to things we needed to know: what was the actual range of my scooter after adapting it for an external lithium battery, could I travel safely on the route as planned using Google Maps selected for bicycles, what type of endurance would Gracie have for distance running after some weeks of training. In addition, I wanted to assess my own stamina for repeated days of 6+ hours on the road and to also get a sense of the role of the support van in our travels.
In addition to this site, much of the information will also appear on the Doug & Gracie Coast-to-Coast facebook page, as well as my personal facebook page. In fact, the facebook page will probably have more up to the minute information during the trip, while my intention is to have more extensive write-ups and more images/videos on this site.
Recent research has shown FSHD to be the most prevalent form of muscular dystrophy. FSHD results from a genetic alteration that leads to the progressive loss of skeletal muscle cells, and a critical and disabling loss of strength over time. We hope to convey the cumulative impact this disease has on individuals over their lifetime.
We also want to talk about the complex, fascinating science that underlies this disease in terms that scientists and non-scientists can understand and appreciate. Recent breakthroughs have identified a key event that ultimately leads to the death of muscle cells. This has allowed scientists, for the first time, to begin thinking specifically about developing the first treatments for FSHD, and patient participation will be critical as they refine theories, establish the significance of cellular targets, and define clinical outcome measures that can be used to assess the effectiveness of treatments undergoing clinical evaluation.
Our financial goal is to raise $100,000 to support FSHD research. Researchers have at long last converged on a consensus view of the cellular events that initiate the dystrophic process—not a gene defect, but the aberrant activation of a gene called DUX4. This consensus has brought about a shift in focus from probing how the disease occurs to examining ways to stop its progression, or even to reverse the damage. Funding to support this next phase of research is urgently needed. Money raised in response to our cross-country journey will go directly to the FSH Society, recognized with a four-star rating by Charity Navigator as an outstanding organization with a 24-year history of funding research and promoting awareness of FSHD.