Relationship between bronchiectasis and cystic fibrosis

Maybe someone out there can answer you? If I remember correctly, both @ckscoville & @ritapearl have been diagnosed with both CF and Bronchiectasis.

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.

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.

@sueinmn I was wondering if a different dose of Trikafta has been tested on non-CF Bronchiectasis patients needing to clear a lot of mucus.. but it’s likely that trials were done, if it was possible with that medication. Game changer for CF patients!

@ckscoville Can you help here? This is not a medication I am familiar with.

It is a relatively new medicine specifically for Cystic Fibrosis patients, with genetic deficiencies that are successfully treated with this particular med (a majority of CF patients). It's called a regulator, in that it compensates for a genetic deficiency, allowing mucus to be expelled naturally from the lungs. As I noted, it is extremely expensive. The "retail" cost is something like $340,000 a year! I'm on Medicare, and my monthly copay for this drug is just under $2,000 a month, although the ceiling for out-of-pocket expenses this year is a great help to people in my situation.

I've had Bronchectasis since I was 16, and when I was in my 50's I got a new pulmonologist who sent me to get tested for cystic fibrosis. The test came back that I was right on the margin, but received a negative diagnosis. I certainly would be interested in any cystic fibrosis medication that addresses the excess mucus production.

@vmorton It might be worth getting a new lab to do the genetic testing again for you.

@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.

You asked, "I wonder if one of the BE specialists might have an answer." Maybe this is a question for your pulmonologist?

I think it is early days yet to generalize use of Trikafta to a wider population, but certainly some testing might be in order.