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Maureen Montgomery's Fundraiser:

Secord Lake Skiers

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Maureen's Photo

BENEFITING: ROCK CF FOUNDATION

ORGANIZER: Kimberley

EVENT: Rock CF Rivers Half Marathon

EVENT DATE: Mar 20, 2016

Maureen Montgomery

THE STORY:

Kimberley Wilk wrote -

Anyone who knows me knows I hate running. My general reaction to a friend bragging “I just ran 10 miles” is “wow, the person chasing you sure was persistent.” But many years ago, life became no longer just about me. So my wife, Kim, and I have put together a group to run to raise money for the CF Foundation, which raises money to defeat cystic fibrosis (CF).

 

You may not know that Kim has CF. CF is a genetic disease that, in many cases, leads to decreased lung function. Kim picked the name “Secord Lake Skiers” because her cottage growing up was on Secord Lake in northern lower Michigan, and because her favorite thing to do is waterski. She learned as a small child on Secord Lake, and remains an exceptional water skier today. We are raising money to fight this disease because her goal is to be able to water ski at age 80. We don’t set small goals in this family, as you may know.

 

You may also not know what CF is, what it does, or why money donated towards a cure could be so valuable. The basics:

 

1) CF is a genetic disease. Recall your 9th grade biology class, where you learned about Mendel’s peas. When two recessive genes are passed along by pure chance, the peas were short, even if both parent plants were tall. The same is true here. Two completely unknowing parents can have a child with CF. All they both have to be is carriers: people with one (non-functional) CF gene. As a result, this disease hits parents out of the blue. Because Kim has both recessive genes and I have none (yea! Genetic testing!), both Mark and Elizabeth are carriers, and will have to be careful about their mates.

 

2) The greatest manifestation of this disease is in non- or poorly-functioning lungs. Thus, the running. CF events are often running or athletic events, as a symbol of the patients beating back the main symptom of CF: poor lung function. But not all CF patients are lucky enough to be able to run at all. The median age for CF patients born in the US in 1980 was 12 years. Most succumb to complete lung failure or other complications.

 

3) Because CF has a specific genetic flaw (mostly) and a specific modification caused by that gene, it has been a focus of genetic-based research. Kim’s mutation (delta F508 for those scoring at home) is the most common one genetic defect causing CF. Cells with CF have difficulty transporting sodium across ion channels in the cells. (Trust me, I looked it up!) As a result, CF patients’ lungs have thick mucus, about the consistency of syrup that’s been left on a plate for a few hours. It moves, technically, but it takes a bunch of effort. As a result, CF patients tend to cough.

 

But because the genetic defect has been located, and scientists know how that defect changes proper cellular function, CF has been the focus of a great deal of genetic research. Scientists have looked at pills, mists, and even recoding genes through adenoviruses (the common cold), all to modify the body’s genetic code to reverse CF.

 

4) Because of scientific advances, we are truly on the cusp of a cure. Here’s a look at one of the latest drugs in development:

 

QR-010 is an oligonucleotide designed to repair CFTR encoded mRNA, which could result in a wild-type or normal CFTR protein in people that have one or two copies of the F508del mutation. It is delivered via inhalation. 

 

I have no idea what that means, but I do know that oligonucleotide would be a heck of a scrabble word. In all seriousness, there have been multiple drugs released recently, and there are more on the way. The CF Foundation is one of the leaders in non-profit/private partnerships for drug development; often diseases as rare as CF cannot on their own attract the attention of major drug developers.

 

So what can you do to be a part of history? You can give me another reason to go on a cold, tiring, miserable run. Because if I’ll do this, it must be REALLY important. Please be as generous as you can, knowing that your dollars get us one step closer to a cure, and me one step closer to back on the couch where I belong.  

 

DONATE

To This Fundraiser

$900

MONEY RAISED
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  • Maureen & Jim Montgomery

    $200

  • Nora & Lloyd Even

    $200

  • Denise and Tim Trenary

    $100

  • Jeanette Snyder

    $100

  • Ann and John McNab

    $30

  • John & Ann Miller

    $100

  • Bob and Gail Oust

    $25

  • Mike & Millie Murdock

    $100

  • Carol & Jim Mavity

    $25

  • Fran McClain

    $20

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Fundraise for this Campaign

The Team: $3,482 TOTAL RAISED SO FAR

JOIN THE TEAM
Fundraiser Title

Kimberley Wilk

Amount Raised

$2,332

 

117% Raised of $2,000 Goal

Fundraiser Title

Maureen Montgomery

Amount Raised

$900

Fundraiser Title

John Wilk

Amount Raised

$250

Donor Comments

Fran McClain

Fran McClain

DONATION: $20

1 year ago

Carol & Jim Mavity

Carol & Jim Mavity

DONATION: $25

2 years ago

Mike & Millie Murdock

Mike & Millie Murdock

DONATION: $100

2 years ago

Bob and Gail Oust

Bob and Gail Oust

DONATION: $25

2 years ago

John & Ann Miller

John & Ann Miller

DONATION: $100

2 years ago

Ann and John McNab

Ann and John McNab

DONATION: $30

2 years ago

Jeanette Snyder

Jeanette Snyder

DONATION: $100

Have a great run!!! 2 years ago

Denise and Tim Trenary

Denise and Tim Trenary

DONATION: $100

For my cousin, Kim, and her beautiful family! - You go girl!! 2 years ago

Nora & Lloyd Even

Nora & Lloyd Even

DONATION: $200

2 years ago

Maureen & Jim Montgomery

Maureen & Jim Montgomery

DONATION: $200

For our very special daughter, Kim. 2 years ago