CATEGORIES: CMT Update

A Rare Disease Patient That Does It All!

by | Dec 18, 2014 | 2 comments

Kim-Goodsell-990x670A movie script could not play out like this. There are very few occasions when a rare disease patient becomes the topic of a story that is truly uplifting. Often the stories are tragic or disheartening. That was not the case, however, with CMT patient Kim Goodsell.

Kim recently became the subject of a story by the acclaimed science writer Ed Yong that simultaneously appeared in National Geographic online (http://phenomena.nationalgeographic.com/2014/08/19/how-an-extremeathlete-uncovered-her-own-genetic-flaw/) as well as the Welcome Trust blog, Mosaic (http://mosaicscience.com/ story/diy-diagnosis-how-extreme-athlete-uncoveredher-genetic-flaw). The article describes how Kim was a competitive triathlete until she was diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC), predisposing her to sudden cardiac death. In the years that followed Kim’s motor skills became progressively compromised and thirteen years after her ARVC diagnosis, she was diagnosed with CMT 2B1, co-segregating with an Emery Driefuss Muscular Dystrophy (EDMD)-like myopathy. As unbelievable as it sounds, Kim had multiple rare diseases.

So Kim set out to learn as much as possible to understand whether her diseases were related. She eventually narrowed down the culprit to a gene called LMNA. She then had to convince scientists to dig deeper and went as far as funding the sequencing of the DNA of her LMNA gene. The results showed that Kim indeed had a mutation in LMNA. Kim ultimately wrote up a thesis on her whole experience and is trying to get her experience published. This unique ability to not only survive, but thrive, and drive research to diagnose the culprit for her own diseases has attracted interest from prominent scientists for several reasons. For one it shows how much a patient can do themselves when empowered to do so. And second, as a patient Kim knew more about her disease than anyone else including the specialists that looked into her diseases. CMT (and rare diseases in general) need patients like Kim that can make discoveries that could lead to a greater understanding of the disease. This inspirational story is not over and it will be fascinating to see what Kim does next on her voyage of rare disease discovery.

Thank you, Kim, for sharing your story with HNF…you are an inspiration!

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2 Comments

  1. Jeannie Hoots

    We are all individuals and each of us know what we feel, what hurts, what helps and the more of us that do what Kim has done the quicker we can find ways to help others. Gaining knowledge of the science behind CMT and other diseases mixed with each person’s day to day battles is the best medicine. Only we truly know how and what is going on in our bodies. Good luck and I look forward to seeing or hearing more soon.

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  2. Marianne De Sterano-Hill

    It just goes to show you that knowledge is indeed power, and one person CAN make a difference! Kim, you rock!…. ….and inspire the rest of us to action!

    Reply

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