Lost Wisdom from the Pediatric Cystic Fibrosis Clinic

For the first time, we see that about 50% of people with cystic fibrosis (CF) are adults. While CF still may ring as a pediatric disease to those who have been familiar with CF for many years, we must adapt our mindset to accommodate the adult population.

When I was first diagnosed with CF, I was nearly 11 years old. I was diagnosed because my ENT doctor noticed inflammation and polyp tissue in my sinuses, and immediately noted how this was not common. After my blood test, I was diagnosed with CF, but my family was confused. I never had issues with my breathing or digestion, and my family dismissed this diagnosis because to them I seemed “fine.” The doctor ordered sputum culture and PFTs (pulmonary function tests), and all came back normal. At the time, my family and I lived hours from the closest clinic and we weren’t familiar with the devastation of CF. We wrote off the diagnosis and started to treat my chronic sinusitis as if that were all my CF was.

However, it wasn’t until I was in high school that I noticed my breathing changed. I was more congested and felt like I couldn’t get the mucus out. It wasn’t until I went to college that I started to see a CF clinic. It turns out that I now cultured pseudomonas and was feeling the impacts of its colonization. But I was 18 when I went to college. I was an adult, and with that came the classification of an adult with CF.

It wasn’t until recently that I was searching the CF Foundation’s websites for ways to help expel mucus from the lower airways. To my discovery, there were protocols that have been designed to optimize airways clearance treatments, procedures to help clear mucus through various types of coughs and drainage, and various other tidbits that seemed so simple, yet insightful. For me, as a scientist and engineer, I guess that I wrote off looking for the “science” behind airways clearance. I knew what my medications and airways clearance devices did, but I never seemed to look into ways to optimize them.

However, why is it that I missed these perhaps “common sense” actions for CF? After some thinking I came to the conclusion that many of these gaps in knowledge may come from my lack of history in a pediatric clinic. I never learned the common sense of CF. I never learned there were such techniques as autogenic drainage, or active breathing technique. I never learned the difference between coughing and huffing. When I go to my adult CF clinic, we never discuss the techniques associated with devices that many have had their entire lives.

For those of you who didn’t’ start your CF journey until adulthood, please check out the CF Foundation’s website. Since learning of these techniques, I’ve seen an improvement in my PFTs. For those of us who started as adults, we must not think we’re above the techniques taught in the pediatric clinic.

Note: Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Cystic Fibrosis News Today, or its parent company, Bionews Services, and are intended to spark discussion about issues pertaining to cystic fibrosis.