Living With Cystic Fibrosis
by John E. Bonfadini, Ed.D.,
Contributing Columnist
Professor, George Mason University

A Family’s Challenge

Every month thousands of children complete the miracle of life and are born into this world. The families who receive these precious gifts are all hoping for the same outcome, the birth of a son or daughter who’s healthy, intelligent, and beautiful. In most cases their wishes are granted. Maybe their child isn’t the most beautiful, as measured by some beauty pageant standards; or a child may not pass standardized tests with the highest scores; but most parents are grateful if their child doesn’t have any major health problems outside the normal childhood maladies. On rare occasions something goes wrong, and children are brought onto this earth with a problem that can test the faith of all of us. Such was the case with the recent birth of my granddaughter Rachel, who came onto this earth January 4th with cystic fibrosis.

I can remember the commercials with football star Boomer Esiason talking about his son Gunther who has CF, but the advertisement just didn’t hit home until last year when I had my first personal encounter with a person who had cystic fibrosis. Mary Bergere’s (Northern Virginia Electric Cooperative’s government relations liaison) 26- year-old son Jon Capps succumbed to the disease, and I was one of many who wondered why a life should be taken at such a young age. It never occurred to me at the time that the disease would also play an important role in my family’s future. Rachel Will is my daughter Lisa’s fifth child. Four years ago she gave birth to a beautiful little girl named Madi who’s time on this earth was cut short. She was born with an atresia (blockage) of the intestinal track. The problem is somewhat common and operations to repair the blockage are usually successful. Madi remained in the hospital for 6 weeks only to have the surgery rupture, resulting in her death. We now believe that Madi, too, had cystic fibrosis, which contributed to the defective bowel, and was not diagnosed at the time. Rachel’s birth should never have happened, according to doctors. After Madi’s birth they informed my daughter that her reproductive tract was damaged and she would be unable to have any more children. Rachel proved them wrong. She overcame many obstacles to enter this world, and we believe there is a special purpose behind her birth.

Cystic fibrosis is an inherited disorder that affects the way salt and water move into and out of the body’s cells. The most damaging effects are the development of thick mucus, which affects the lungs and digestive tracks. Both parents must have the defective gene for a child to be born with the disease. More than 10 million Americans (1 in 31) are symptom-less carriers of the defective CF gene. In cases where both parents have the defective gene, the probability is one in four that a child will be born with CF. The statistics show that one in every 2,500 live babies will have CF. It’s impossible to predict how long a single patient will live. Science has made great strides in the identification of the defective gene, and new treatments of the symptoms have increased the expected average survival age to 40. Some children are lost at an early age, but the future looks bright since the defective gene has been identified, which may lead to a cure that addresses the defect itself, rather than just treating the secondary causes of CF. The effort to find a cure, as with other diseases, requires the support of the general public. This article is not an effort to request support for one disease over another, but more to remind all of us how fragile and valuable each life is. I’m providing the following story to illustrate this point.

The professor picked up a box of sand and poured it into the jar. Of course, the sand filled up everything else. "Now," said the professor, "I want you to recognize that this is your life. The rocks are the important things —- your marriage, your health, your children, your love of God —- anything that is so important to you that you would be nearly destroyed if it were lost. The pebbles are the other things that matter, like your job, your house, your car. The sand is everything else. The small stuff. If you put the sand into the jar first, there is no room for the pebbles or the rocks." The same goes for your life. If you spend all your time and energy on the small stuff, you will never have room for the things that are really important to you. Pay attention to the things that are critical to your happiness. Play with your children. Take time to get medical checkups. Take your wife out dancing. There will always be time to go to work, wax the car, give a dinner party, and fix the disposal. Take care of the rocks first ... the things that really matter. Set your priorities. The rest is just sand.

When a child like Rachel comes to us, we soon realize the value of the "rocks." Rachel is going to provide us with an opportunity to focus our future efforts on the more important things in life. In my writings I’ve tried to emphasize the rocks in education, and not the sand, as represented by items like SOL testing. My hope is that we will constantly remind ourselves to prioritize our lives, to deal with the items that matter the most.

Mary Bergere is having a Spaghetti Dinner and Silent Auction fundraiser to support the Cystic Fibrosis Foundation on March 30, 2001, at the American Legion Hall, 9918 Cockrell Road, Manassas, VA. Cost for adults is $10; children 2-12, $5; and under 2, free. If you need more information, call Karen Fink toll-free at 1-877-657-8444 x18. If you wish to contribute and cannot attend, send donations to Mary Bergere, Northern Virginia Electric Cooperative, 10323 Lomond Drive, P.O. Box 2710, Manassas, VA 22110-2710. Those of you who would like to learn more about the Cystic Fibrosis Foundation, can get additional information on their Internet site at www.cff.org.