Columbus, OH 43229

October 19, 1996

Cystic Fibrosis Update - Bowl-For-Breath Fund-Raiser Ends November 15, 1996

Dear Friends,

Many news items to report this year! Although we won't see results for some time, researchers are looking at ever more devious ways to work around the genetic defect called "cystic fibrosis". There's a lot here, so don't feel bad if you want to just jump to the last paragraph! Here's a brief summary of this year's research reports:

Many research programs are under way investigating different aspects of CF. Two major thrusts involve 1) using chemicals to make the defective "chloride channel" (the defect caused by the CF gene) operate correctly, and, 2) correcting the actual defect in the DNA, itself.

The technical name for the "chloride channel" is CFTR (Cystic Fibrosis Transmembrane Regulator). Chloride channel is a nice simple name for a process which is very complex. We've read about studies of three different chemicals ( phenylbutyrate, CPX - related to caffeine, and genistein) which treat different aspects of the defective "chloride channel". The problem is these drugs affect only three of the many steps involved in making the "chloride channel" work properly.

Research has proved we CAN correct the cell defect causing CF (at least in the lungs). Researchers are now refining the methods to provide a safe and effective delivery vehicle. This research is taking place all over the country. Do you recognize any of these CF research centers? Cornell Medical Center, the University of Pennsylvania, the University of North Carolina at Chapel Hill, the University of Cincinnati, the University of Iowa/University of Washington (joint study), the New England Medical Center/Massachusetts General Hospital/Genzyme (joint study), the University of Alabama at Birmingham, John Hopkins University in conjunction with Targeted Genetics Corp., Stanford University.

Research is future stuff. In the day-to-day struggle of CF patients , the CF center plays a much larger role. CF centers around the country are caring for CF patients as they have cared for us for 18 years. Dedicated and caring doctors, nurses, nutritionists, social worksers - the entire CF team - help patients live productive lives! Without these dedicated professionals in our lives, our journey would be much more difficult!

The next time you hear or read something about research work in cystic fibrosis, you can be proud in the knowledge that your contribution helped make that work possible.

We are continually amazed at the support you have given our family over the last 18 years. Please continue your support by sending us a check payable to the "Cystic Fibrosis Foundation" by November 15, 1996.

With MUCH Love and Thanks,

Jim, Linda, Brent, and Derik Pace