Figure A shows the organs that cystic fibrosis can affect. Figure B shows a cross-section of a normal airway. Figure C shows an airway with cystic fibrosis. The widened airway is blocked by thick, sticky mucus that contains blood and bacteria. National Heart Lung and Blood Institute (NIH) – National Heart Lung and Blood Institute (NIH)

“Dr Welsh: This journey really began for me when I was a junior medical student on my pediatrics rotation. I’m walking down the hall, and before I get to the room where I’m supposed to see a patient, I can hear harsh coughing. I go in the room, and there’s a 7- or 8-year-old little girl. It’s obvious she’s breathing hard. I can see her using her accessory muscles of ventilation. I hear her coughing and then I smell for the first time the odor of Pseudomonas aeruginosa, a common organism that affects the lungs of people with CF. I hear from her and her parents about all the things she can’t do and how much of her day is spent with a variety of different therapies.

The sobering part was when we left the room because then my attending told me that she wouldn’t make it to her teens. If she did make it to her teens, she almost certainly would never make it out of her teens. There are certain patients that are burned into your memory. That little girl is burned into my memory.”

How Cystic Fibrosis Went From Fatal to Treatable via JAMA.