Relationship between bronchiectasis and cystic fibrosis

Wonder what kind of CF test involved numbers? Was that a skin test? Usually the genetic test is the only way to know for sure. If you are positive then the new drug that people with CF take has made a HUGE difference for them. If only Non Cystic Fibrosis Bronchiectasis had something like it! I have also read that new genetic variants are being discovered all the time for CF.

@ckscoville @sueinmn
Thanks for the response @ckscoville
It is a very expensive medication. I wonder though if the same mechanism in the drug that allows it to be called a regulator, allowing mucus to be expelled naturally, could be helpful for those where mucus clearance is challenging.

I use postural drainage a lot. If I’m ever in a position where I can’t do this in the future, I think I’d be more prone to infection and a decline in health. Many of us would be.

I suppose there aren’t enough of us to warrant the cost of a new drug that could help with mucus clearance rather than having to position oneself and huff cough with such force. How do people do that with broken ribs? That said, the numbers of BE patients being diagnosed around the world is increasing.

Would there not be other people in circumstances or with illnesses who would benefit though from a targeted medication in addition to Bronchiectasis patients?

I’m still wondering if this particular medication might work even on a short term basis to prevent mucus build up, infection and progression of disease for BE patients, especially if circumstances prevent regular airway clearance. Thanks.

I wonder if one of the BE specialists might have an answer.

In my case the diagnosis of CF (after genetic testing) definitely changed treatment options. It was determined that I am eligible for treatment with a very expensive CF drug called Trikafta, and that drug has been a real life-saver for me. My constant coughing and struggles with mucus have almost disappeared. In an odd way, my CF diagnosis has improved my life and my outlook considerably -- at least my daily struggles with lung disease.

Yes, I have Bronchiectasis and had a genetic test at Mayo that was positive for one CF gene. I had sweat chloride test at NJH that was positive for CF. I am a 72 year old woman - surprise! I also am immune deficient and have CLL. Weekly immunoglobulin infusions (at home) and airway clearance have helped tremendously.

I haven't experience any significant side-effects from Trikafta in the four years I've been taking it. You've probably been warned already about the "purging" that you will experience during the first few days on the drug ...expelling a LOT of mucus from your lungs! My bloodwork also indicates macrocytic anemia, which has worsened since I started Trikafta, but I don't know if that's simply coincidental or a side-effect of the drug; nobody has been very concerned about it. I hope the Trikafta gives you some relief; it certainly did for me. Before starting it, I couldn't lie down to go to sleep at night without first going through a 30-minute coughing fit. Now I rarely have any coughing, day or night!