2005 Letter

Everyone deserves a pat on the back every now and then… whether it’s for a good report card, a promotion at work, or simply from a proud parent to a son or daughter. But to give someone a pat on the back 4 million times, now that’s a bit much! Well, over the past fours years of Deirdre’s life, we I have patted her back, front, left-side and right-side as part of her daily physiotherapy (PT). Four million times and counting! We do this to clear her lungs of mucus buildup caused by her cystic fibrosis (CF). In healthy people, this secretion is thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. And instead of acting as a lubricant, the secretions may plug up tubes, ducts and passageways, especially in the pancreas and lungs.

CF is an inherited genetic disease affecting the glands that produce mucus, tears, sweat, saliva and digestive juices. The number one leading cause of death (at a median age of 30) in CF patients is respiratory failure caused by this thick mucus buildup which harbors bacteria which causes infections. With each infection her lung function will diminish and may deteriorate anywhere between 1 – 4% each year. Deirdre, and the other 30,000 Americans with CF, deserves much more than a pat on the back. She deserves to grow up healthy and live a long life.

This past year Deirdre tested positive for one of the most dangerous bacteria: Pseudomonas. There’s good news in everything and what saved Deirdre from getting much worse was a drug by the name of Tobi which was developed just 10 years ago. After a month long treatment, and even more PT, she tested negative. Without the proper funding, drugs like Tobi may never have been discovered. And besides, Tobi is a treatment – not a cure!

People who meet Deirdre are amazed at how healthy she looks and how well adjusted she is in dealing with CF. She follows a strict daily regimen including taking medications such as inhalation therapy, enzymes to aid in her food digestion, and vitamins to improve her general health. All this is in addition to her daily pats on the back. Her sisters bear an inordinate amount of responsibility as they assist us in her care with willingness and pride. Recently, one of our daughter’s Brownie Troop was looking for ideas on how to best allocate proceeds from the cookie sales and our daughter raised her hand to suggest the Cystic Fibrosis Foundation – she got a pat on the back for that (and a few tears from us).

Deirdre recently said that she wished she didn’t have CF. Knowing that Deirdre has a fighting chance to lick this disease because of you leaves us humbled and speechless. After you send in your tax-deductible donation give yourself a well-deserved pat on the back.