To The Editor:
My grandson Owen has cystic fibrosis, a genetic defect that affects multiple organs in the body, mainly the lungs and pancreas. CF causes the body to produce a thick, sticky mucus throughout his body. Thankfully, Owen is an active and healthy 4-year-old. Owen has been prescribed numerous medications to help him to breathe and absorb the food he eats. Included in these medications are digestive enzymes, abuterol, pulmazine, pulmacort, hypertonic saline, etc. The research and development of many of these medications can be directly attributed to the Cystic FibrosisFoundation and its donors. For example, hypertonic saline was developed after the foundation noted people with CF living in Australia had much higher lung function than those from other countries. The foundation did additional investigation to find out that many of the kids with CF who were doing well were surfers. Prolonged exposure to salt water helped to loosen the mucus in their lungs. The foundation funded a drug that is essentially a high salt saline that mimics ocean water in the lungs, resulting in improved lung function. Hypertonic saline has saved Owen from the hospital at least three times. Contributions go directly into improving the care available for Owen and others with CF.
The foundation also funded research for Kalydeco, a medication aimed at correcting the underlying defect in CF. Kalydeco was released last year and is the first drug ever approved by the Food and Drug Administration that corrects a genetic defect. Although this is an amazing breakthrough in CFand medical community as a whole, unfortunately the drug does not work for Owens mutation. However, Kalydeco, in conjunction with VX 770 and/or 661 has been shown to be effective in all CF mutations, including Owens. This year combination testing of Kalydeco and VX 770 will move into FDA phase 3 pivotal trials. This is the final step before FDA approval. Thus, if the trials go well, the drug may be available to patients by 2015.
Please support our spaghetti dinner fundraiser for cystic fibrosis March 5at BOCES Northwest Tech, Park Street, Ogdensburg, from 4:30 to 7:30 p.m.Adult dinner tickets are $8.00, tickets for children 5 to 12 are $5, and pre-sale tickets are available for $7by calling 528-0193. Takeout dinners are available. There will be raffles forpizza for a year and a bucket of toys, and a better used book sale. Thank you for your support.