October, 2012

Dear Friends and Family,

We are enjoying beautiful fall trees and OSU football in Ohio! This is our 34th year to be involved in the Cystic Fibrosis Foundation's Bowl for Breath. We will be bowling on Sunday, November 11. With the support of our friends and family (that means YOU), we look forward to raising money for research for this incurable disease.

The Family Update: Linda won't be bowling this year because in September she broke a bone in her foot. After a month in a hard cast, she now gets around in a boot. She is still busy with flute lessons, tutoring, volunteering at Wesley Glen (where her mom is in Special Care), and involvement with Stratford Ecological Center. Jim continues to take classes at OSU and this semester he is taking philosophy. Misty (daughter-in-law) asked him to build some tables for her classroom so that meant some new power tools. Jim and Linda spend most of their days taking care of Misty and Derik's foster children. Two and three year old siblings keep the Paces busy and realizing why you have kids when you are young! Allison, Linda's niece and recent Otterbein College graduate, is living with us now. This will be her 5th year of bowling with us!

Brent (37) had a very healthy year. From his involvement in a human trial for a new inhaled antibiotic, he was asked in September to speak before the advisory subcommittee for approval of the new drug. The meeting was the final step in gathering information for FDA approval of the drug. The CF Foundation flew him to Washington DC, where he spoke what he does in his daily routine to try to stay healthy and the time saving benefits of the new antibiotic. Brent has been working with the drug company (more specifics to come later, and they are good specifics. Brent can't talk about them until drug approval) and hopefully the drug will be available soon. Girlfriend, Christine, has also become very involved in the SC CF Chapter. She has chaired several events and spreads the word about CF research everywhere. Brent again chaired the fishing tournament fundraiser for the foundation along with his own fundraiser, the Champagne Campaign. Brent and Christine have just moved to another condo in Charleston. This one has a water view from the outside space. Brent has now been with BP Chemical for 17 years and is still enjoying new challenges. And of course, the "getting up there near 40 guy" is still rocking with his bass guitar!

Derik (34) had a more routine year with his CF. His weight remained "steady" with fewer infections... all good things! Some tests he and other CF adults go through include: bone density tests for osteoporosis, sugar screenings for diabetics, and a colonoscopy every 5 years to check for colon cancer. Liver scans are routine. CF adults are more likely to development some of these diseases. The biggest change in Derik and Misty's 12 year marriage is the addition of foster children. After taking classes and completing interviews, their first foster baby arrived in January. He was with them for 7 months. Now their house is busy with two preschoolers. They attend preschool at Columbus Montessori, where Misty teaches. Our whole family is learning a lot about foster care and giving love to kids. Their return love makes for a win-win! Derik is in his second year at The Limited Corporate Cafeteria and enjoys busy days. Derik and Misty will have their annual bake sale (Pacetrees Home Baking) at the Bowl-for-Breath. 100% of the proceeds goes to CF. If you are in Columbus, come to the Columbus Bowling Palace on Nov. 11 - 1:30-3:30 to buy some goodies!!

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

The Cystic Fibrosis Foundation provides funding for and accredits more than 110 cystic fibrosis care centers and 55 affiliate programs nationwide, including 96 programs for treating adults with CF. Ohio has 6 centers, each providing pediatric AND adult care programs. Outside of the US, you can find another 358 or more centers. Many CF centers have not only patient care, but research projects connected to the centers. Your generous donations are what funds these research projects!

Research Update:

I HAVE A STORY TO TELL - and you've heard part of it before, so bear with me... Also, I'm not a geneticist, so I use generalities. Here we go:

DNA: Human DNA is coded, primarily, in 23 pairs of chromosomes in each cell. Chromosomes are visible in a microscope, but only during cell division. Each chromosome contains between 379 and 4,220 genes. Genes are used to build the proteins that determine our body's characteristics.

Human DNA contains over 32,000 genes. Each gene contains, on average, 10,000-15,000 instructions to create a specific protein. The instructions are formed from 4 nucleic acids (represented by the letters A, T, C, G). The 4 "letters" are combined to form 3 letter "words" (like ATC, TTT, GTG, etc.). Each 3-letter word is one of the 10,000+ instructions required to make a single protein. Is your mind spinning yet?

Folding is important! So, how does all this information get stuffed into that little tiny cell? Folding! Take each one of those genes (with their average of 10,000+ "instructions") and start folding them. Pretty soon they take up a lot less space!

CF is caused by a defect (mutation) on the CF gene (also known as the CFTR - cystic fibrosis transmembrane conductance regulator). This gene is on chromosome 7 in human DNA. The CFTR gene is composed of over 230,000 base pairs (the letters). So, divide by 3 and you get almost 77,000 "instructions" to make a protein that is 1480 amino acids long. A lot more than the average set (10,000-15,000) of instructions to build a protein

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 (the one that is 1480 amino acids long). This one defect accounts for over 90% of cases of CF in the USA. There are over 1500 other mutations that can produce CF.

That was all background. Next is why it's important to us!

In 2007, I started telling you about a drug called VX-770 (now named KALYDECO) which has proven to significantly improve the health of CF patients with a different CF mutation (G551D, if you're interested). Kalydeco helps the defective CFTR protein work better. But it won't work if it's not at the right place on the cell surface. Proteins have shapes. The defective CFTR protein doesn't have a certain fold in the right place (remember, "Folding is important!").

Enter VX-809. VX-809, another drug, makes the protein fold correctly. The combination of VX-809 and VX-770 get the defective CFTR protein to the right location on the cell surface and then make it work better! VX-809 used in combination with Kalydeco has significant benefit for CF patients with defect delF508! That means it could help Brent and Derik and most other CF patients in the US! HURRAY!! Can't wait for that drug to become available!!!

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