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Am Fam Physician. 2010;81(4):421

I didn't consider myself a sick child; however, I did have a lot of colds, and I was hospitalized several times with pneumonia. It was the crippling abdominal pain that I remember most. I had surgery for intussusception when I was six years old, and throughout my childhood I always had a lot of large, unpleasant bowel movements. When I first got to college and had a few beers with friends, they all got drunk and I got stomach pain that was so severe I ended up in the hospital.

I was treated for Crohn disease for several years, but when I was 27 years old, a nurse saw my chart and thought that there was something else going on. I had more tests and then got my diagnosis from the gastroenterologist. He said, “The good news is that it's not Crohn disease. You have cystic fibrosis (CF).”

I didn't really know about CF at that time, and I went home to my wife and said, “Great news—I have CF!” She was devastated. She remembered a friend's sister who died from CF when she was six years old.

Throughout this process, my wife has been very supportive and is my best coach. She encourages me to play racquetball, and we go for long bike rides and hikes. My CF doctor and his nurse practitioner have done so much to help. A few years ago, my primary care doctor told me that my blood pressure was elevated. She wanted to start me on a new medicine, but she called my CF doctor first. I was really impressed that she did that.

CF has had a major impact on my life as an adult. It has affected my jobs, because I was always sick and had to find a job that would understand. I now have a job where the air quality is good. This better work environment, as well as regular exercise and a healthy diet, has decreased my exacerbations. When I was in my 30s, I thought that I wouldn't live very long, but my wife and I have strong faith. Now, I am not worried about dying because I am in good hands.—j.w., 46


Although most cases of CF are diagnosed in childhood, some are not recognized until adulthood. J.W. is the only one of his family members who has CF, although several of his siblings are carriers of the mutation. During our interview, he noted that CF is only a small part of who he is. J.W. has been deaf since birth, and feels that his identity has been shaped more by his experiences as a deaf man and participation in the deaf community than by his diagnosis of CF. Whether this would be true if he'd received that diagnosis earlier is uncertain, even to J.W.

The average life expectancy for persons with CF is now 37.4 years, up from 32 years in 2000.1 Living longer puts patients at risk of many chronic diseases of aging, including hypertension. In treating J.W.'s hypertension, it seemed appropriate to partner with the CF team, who admitted that this was new territory for them as well.

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