$61,000 Raised to Support Pediatric CMT Trials

by | Apr 9, 2020 | 1 comment

bike tourLast summer HNF teamed up with the Penn Medicine Orphan Disease Center (ODC) for the Million Dollar Bike Ride (MDBR) in Philadelphia. Our fundraising goal of over $30k was achieved and matched by the ODC, granting HNF over $60k to allocate to CMT research. Thanks to our generous sponsors and riders!

After evaluating many different potential avenues, HNF chose to direct the research funds to a very underserved, yet vital area — pediatric clinical trials. “We have very promising developments taking place in pharma and genetic therapy research right now, but without the collection of more pediatric data to measure outcomes and efficacy, these treatments will never make it to our community.” 

“It is imperative to shed more light on pediatrics and the urgency of delivering treatments to these patients and families. There are a million moving parts to produce successful clinical trials. It takes lots of work behind the scenes that few people are aware of unless they are deep in the trenches. We’re confident we’ll be able to significantly alleviate future hurdles and add significant knowledge and resources. 

When it comes to CMT in children, it’s even more important to get treatments out swiftly and halt/reverse progression early on” Allison Moore, HNF Founder and CEO.

HNF is pleased to provide funding to continue the promising research being conducted at the prestigious Connecticut Children’s Gait Lab. The project will be overseen by Director of Research and Education, Sylvia Ounpuu, MSC, and Gyula Acsadi, MD, PhD, Division Head of Neurology. Both investigators are a part of the Inherited Neuropathy Consortium, as is HNF. 

The study will measure abnormalities in gait function using comprehensive gait analysis techniques and wearable activity monitoring and then compare them to CMTPedS scores. The research will also compare the changes in these parameters over time and study the relationship between the patients’ measureable gait function and a promising biomarker, neurofilament light chain (NfL) level. The information gained from these investigations will support not only the understanding of the natural history of gait and community function, but also clinical trial readiness for new treatments. “To conduct a successful therapeutic trial for children and youth with CMT, a detailed understanding of the natural history of walking decline is needed, along with predictable benchmarks to support the development and translation of emerging disease-modifying therapies,” Sylvia Ounpuu, MSc.

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Quality of Life and CMT Research

Did you know that 95% of clinical trials fail? There are multiple causes, most related to efficacy or safety, which obviously can be harmful and risky for patients. The risk-reward of enrolling in trials is a judgment call based on the devastating effects of disease related to quality of life (QoL) or life-threatening disease. With CMT, the risk-reward is more of a challenging question for many, as CMT in most cases is non-fatal.

Help us answer questions that your doctors and the CMT Research Community aren’t too sure about.

Did you know that you can become part of a community in therapy development and further research for all forms of CMT and inherited neuropathies? The mission of the Global Registry for Inherited neuropathies (GRIN) is to collect clinical and genetic information from patients with ALL forms of Charcot-Marie-Tooth (CMT) and other related rare and ultra rare inherited neuropathies.

Hot off the press

It seems like almost weekly there is another new publication on CMT with interesting basic biology. While an earlier “Hot off the press” highlighted the work of Cherry and co-workers at the University of Texas Southwestern Medical Center who showed show that neurons lacking a gene for rab7 result in neuropathy.

New Study on CMT Type 2B

If we are to learn more about CMT and the effectiveness of rehabilitation it is worth asking the patient and their caregiver. A recent Italian study by Padua et al recently described a survey of CMT patients and caregivers and their perspectives and perceptions of rehabilitation efficacy and needs.

Hot off the press

Some recent papers on Charcot-Marie-Tooth (CMT) disease go to show that we are steadily and impressively peeling back the complexity of the biology even though it is a relatively common rare disease with several thousand publications on it.

Allison Moore is going to be published!

HNF’s CSO (Chief Scientific Officer), Sean Ekins wrote a blog about his work with Allison Moore and her two “fighter Mom” friends Lori Sames and Jill Wood. He named his blog: “Rare disease heroes – Extraordinary collaborators we should be listening too.” Sean helped my friends and I write a paper called: “Multifaceted roles of ultra rare and rare disease patients/parents in drug discovery.” YES, Allison Moore is going to be published! It will be in Drug Discovery Today, soon. The link to the reprint is below.

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1 Comment

  1. Claudia Herrera

    My son is 14 years old, he was diagnosed 4 months ago, I would like to know more about what kind of PT it’s better for him, we are going to a place that my insurance cover but it doesn’t seem to me that they know much about CMT.
    Thanks

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