October, 2011

Dear Friends and Family,

Fall is here and the changing leaves and cool breezes tell us that the Annual Bowl for Breath is coming up. Our family has committed to fundraising for the Cystic Fibrosis Foundation since Derik's diagnosis 33 years ago. This year we will be bowling on Sunday, November 13. With the support of our friends and family, we look forward to raising money for research for this incurable disease.

The Family Update: Jim and Linda are enjoying (most of the time) more time together since Linda retired from teaching in June. Linda is volunteering with kids, taking cooking classes, and even trying out yoga. Jim is taking an astronomy class at OSU and enjoying college more than the first time.

Brent continues to love living in Charleston, South Carolina. Hard to believe he has been there eight years. Jim and I enjoyed a fall trip to see him and he was a speaker at a CF family conference. It was great to meet the doctors and nurses that support him. Brent continues as an environmental engineer with BP Chemical, playing in bands, and playing soccer. How many South Carolinians do you know that have a season pass (snow boarding) to Vail? Maybe some of you saw Brent on his second guest appearance on Army Wives (filmed in Charleston). Brent also puts on his own fundraiser every year around his birthday in April. Last year we attended The Champagne Campaign along with Misty and Derik. And Brent continues to be an active member of the South Carolina CF Board. He was in charge of the fall fundraiser - a fishing tournament. Brent has had a very healthy year.

Derik is now working in the cafe at the Limited Stores Victoria's Secret Distribution Center. The Limited headquarters are in Columbus and Derik says his days are busy. He now works the breakfast/lunch shift and has evenings and weekends off... Chef jobs don't all come with this perk! He already knows who his "regulars" are and starts their order as soon as he sees them in line. He has put on some weight this year (a good thing) and is feeling better with daily exercise. Derik is involved with the Adult Advisory Committee for CF at Children's Hospital. Misty, Derik's #1 supporter, graduated from Ohio Dominican University in December and is now teaching at a Montessori School. She is also attending classes for Montessori certification along with her teaching responsibilities. Derik and Misty have a bake sale (Pacetrees Home Baking) at the Bowl-for-Breath. 100% of the proceeds goes to CF. If you are in the Columbus, come to the Columbus Bowling Palace on Nov 13 - 1:30-3:30 to buy some goodies!!

We are very proud of our sons and their aggressiveness to handle their own illness and work to improve the health of others with CF!

And a New CF Connection! Linda's sister, Carol, has been diagnosed with "borderline CF". Carol has battled lung infections and other respiratory problems for many years. In the past year, Carol's condition was finally diagnosed and she now has added some of the same CF care regimen to her life as Brent and Derik have done for over 30 years.

Research Update:

"A rose by any other name" - In the past several years I've talked about a drug called VX-770 which helps the defective CFTR protein work better. VX-770 has been in clinical trials, but has now been submitted to the Food and Drug Administration under the name, KALYDECO, for approval, hopefully in 2012. If approved, it will be the first drug on the market that targets the underlying cause of cystic fibrosis. Therapies currently available to people with cystic fibrosis treat only the symptoms of the disease.

CF Mutations: CF is caused by a defect on the CF gene also known as the CFTR (cystic fibrosis transmembrane conductance regulator). This gene is on chromosome 7 in the human DNA. The CFTR gene is composed of over 230,000 base pairs. These base pairs are the "instructions" to make a protein that is 1480 amino acids long.

Brent and Derik have a defect called delF508. This defect is a deletion (del) of phenylalanine (F) at the 508th (508) position on the protein.. This one defect accounts for two-thirds (66-70%) of CF cases worldwide and over 90% of cases in the USA. There are over 1500 other mutations that can produce CF.

Please join us in our money raising efforts. We encourage you to use your credit card to donate online. Click on the orange "Donate Now" button near the bottom of our web page at: http://jimpace.eml.cc The "Donate Now" button will take you directly to a secure page at the CF Foundation dedicated to recording our collections for CF.

If you prefer, you can send your donations to us by November 21st. Checks should be made to The Cystic Fibrosis Foundation. Mail your check to:

James M. Pace
5358 Torchwood Loop W
Columbus, OH 43229

Together we can make a difference in many lives and conquer this disease.

As always, thanks for your support,

Jim and Linda Pace (and the rest of the Pace family: Derik, Misty, and Brent)

PS: More information about cystic fibrosis research is available at the CF Foundation web site at: http://www.cff.org

PPS: Would you like to read (or reread) some of our previous year's letters just to see how things have changed (or how our letters keep getting longer? All but one of our letters back to 1996 are available at: http://jimpace.eml.cc