I am almost as old as cystic fibrosis (CF). That is, Dorothy Anderson initially described CF as mucoviscidosis in 1939, and I came along in 1944. In this way, we are both over 80. 

Since early childhood, I have had a productive cough, which my mother‘s pediatrician told her was chronic bronchitis and that she should “just get used to it,” which we did.  “Paul just has a funny cough” was her response when asked about it. She was very talented at applying Vick’s salve, front and back, when I caught a cold. She also had me sleep under a sheet tent with a vaporizer when the product was applied. Perhaps I owe my longevity to Vicks. 

In the course of growing up, I coughed–and spit—continually, of course.  I still have pretty good aim with spitting.  I began to develop chest pains that came and went, but worse, at least from the viewpoint of being startling, I began to have episodes of hemoptysis.  At first, it was blood-streaked sputum, which evolved into frank bleeding hemoptysis, and the treatment for it was a right upper lobectomy when I was 16, which was followed by a left upper lobectomy when I was 51.   

After I was diagnosed with CF in early adulthood, I went on to receive my PhD and began investigating the disease—both for myself and others. After years of research, in which we isolated individual sweat glands and placed a micro pipette in the lumen of the sweat chloride tubules from people with CF (including my own cells) and those with no CF, we discovered that the CF mutation caused the CFTR channel to fail to allow either chloride or bicarbonate to pass through the cell membrane.  It is this defect that causes sweat to be characteristically three to four times higher in people with CF than in unaffected persons with normal CFTR. It is this that also causes other CF-related symptoms, such as degeneration of the pancreas, respiratory bronchioles, or absence of the vas deferens, etc., due to the formation of abnormally thick, viscous mucus. Isolated sweat ducts were investigated rather than other affected organs because they consistently expressed the salt sweat defect but are anatomically normal. That is, the function is defective, but not the structure.  

Over the course of my adult life, CF research has been and continues to be a great passion and joy of mine. I have been involved with CFRI since its inception, and I was honored to be the first recipient of CFRI research funding in 1977.  

Despite the fact that cystic fibrosis can be a fatal disease, I do not believe that for me it is all that negative. Living with CF as a constant threat has given me many friends that are probably closer than they might have been. I would like to believe that it has sharpened my senses and feelings for being alive and increased my appreciation for the health that I have had in the years I have been living. There is no question that having devoted my career and research to understanding CF has given a real meaning to life. It is a “cause with purpose.”  

By: Paul Quinton, PhD