Dear Family & Friends,
I would like to take a minute to invite each and every one of you to the Olympia Cystic Fibrosis (CF) walk on May 22nd` . As most of you know, CF is an illness very close to our hearts as our one and only daughter, Lucia Grace, deals with it everyday. Here is a brief explanation of CF from the foundation’s website:
Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
clogs the lungs and leads to life-threatening lung infections; and
obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond. –
www.cff.orgCall me crazy, but I live every single day as an optimist. Even though this is an incredibly scary, life shortening and at times pain inducing disease, the progress that has been made (great strides if you will) is absolutely remarkable. I feel oddly blessed to have an undeniable passion to learn more about something so profound – and here’s an example of why. There are over 400 genetic mutations that cause CF. 60 of these are tested for on the standard CF DNA screen (which Lucy had in her first few days of life, results came to us at 6 days old), and the remaining 300+ can be identified on a highly advanced Ambry full genetic panel. Lucy has a CF gene (mutation) from Randy (G551D) & one from me (N1303K), because we are both carriers. These are both relatively rare mutations, the G551D mutation is the one we are mainly focused on because there is an important med on the horizon aimed at treating this mutation. Only 3-4% of all CF patients have this G551D mutation, the vast majority have delta F508. The medication we are closely following go through the CF drug development pipeline was created by Vertex Pharmaceuticals with funding from the Cystic Fibrosis Foundation; it is called VX-770. On a cellular level specific to G551D, this medication is able to essentially correct the salt imbalance that is the root problem of CF. The effect is no more “dehydrated”/thick mucous clogging up parts of the body, most importantly the lungs and digestive system – it is basically a cure as it is able to eliminate symptoms. We have known about this med since Lucy was diagnosed and have been following it closely as it goes through the different phases towards final FDA approval. It looks very promising and about 2 years away from being within our reach. The ultimate goal is for researcher to also apply this knowledge to other mutations, it is very exciting!
Over the past year, I have been lucky enough to meet two amazing adults with CF. The first is a 50 year old female physician with CF who spoke at a parent education day at UW, her topic was the importance of physical exercise in the life of a CF patient – she is incredibly vibrant and did a fantastic job. The other amazing individual is a 54 year old male, former computer wiz during the dot com craze, now retired & living happily in the Seattle area, working hard everyday to battle his CF. Seeing these very positive, high achieving, life loving & OLD (I say that with every positive meaning of the word!) adults with CF, I can’t help but realize the potential Lucy has for a long healthy life simply because of the era which she was born into.
So, how does the medical/genetics talk relate to the Olympia Great Strides CF Walk? Great Strides is the CF Foundation’s largest national fundraiser and it provides the money necessary to fund incredibly important research including the development of the med mentioned above and others like the inhaled antibiotics Lucy has taken this year.
Although we would love to see zillions of dollars raised, Randy and I would most like to see awareness increased and invite people to learn more about our reasons for being so optimistic about Lucy’s future with CF. The Olympia walk is a lot of fun, last year it fell on an absolutely beautiful sunny spring day and we had 75 walkers in bright yellow TEAM LUCY tshirts. I was amazed by the incredible support we received from friends and family!
To register for the walk, go to
http://www.cff.org/, then register for Great Strides, select WA state, then Olympia & then Team Lucy (the shortcut is
http://www.cff.org/great_strides/christywebster ). Technically the walk is free, but donations are encouraged. There is a live band, BBQ & fun projects for the kids to do as well. The full walk is 4.5 miles although there is a beautiful 1 mile option around Capitol Lake itself.
Thank you to all of our much loved friends and family, your support means the world to us!
Love,
Christy, Randy & Lucy Webster
websterhome@comcast.net
Posts
