1. < MAC & Bronchiectasis

Relationship between bronchiectasis and cystic fibrosis

Posted by lenorj @lenorj, Mar 25 8:26am

I recently was tested for cystic fibrosis as part of the on going treatment of . bronchiectasis. The test came back with out a definitive answer but the numbers did show that I was not in negative territory either! I am wondering if anyone on the forum has any experience with this and does the results change treatment options?

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mtyler | @mtyler | 3 days ago
In reply to @peggyri "I was diagnosed with non-CF bronchiectasis about 10 years ago. Last year after looking at my..." + (show)
@peggyri

I was diagnosed with non-CF bronchiectasis about 10 years ago. Last year after looking at my CT scan, my new pulmonologist recommended that I get tested for CF. First I was given the sweat test, and the numbers were high enough to make the diagnosis likely and warrant genetic testing, which confirmed CF. Unfortunately I do not qualify for Trikafta because of my particular genetic mutations, but I'm still relieved in a strange way to have a definitive diagnosis of a disease I've had my entire life.

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I started searching yesterday and found that Alyftrek (Elexacaftor/tezacaftor/ ivacaftor), which is basically the same, if I am understanding correctly, as Trikafta, is now approved for 31 more CF mutations. If you look this up, you can tell which mutations qualify you. It might be worth looking at this especially if you already have the diagnosis of CF.

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2 replies
lenorj | @lenorj | 3 days ago
In reply to @mtyler "I started searching yesterday and found that Alyftrek (Elexacaftor/tezacaftor/ ivacaftor), which is basically the same, if..." + (show)
@mtyler

I started searching yesterday and found that Alyftrek (Elexacaftor/tezacaftor/ ivacaftor), which is basically the same, if I am understanding correctly, as Trikafta, is now approved for 31 more CF mutations. If you look this up, you can tell which mutations qualify you. It might be worth looking at this especially if you already have the diagnosis of CF.

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Great information, thanks for you time and effort.

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PeggyRI | @peggyri | 3 days ago
In reply to @mtyler "I started searching yesterday and found that Alyftrek (Elexacaftor/tezacaftor/ ivacaftor), which is basically the same, if..." + (show)
@mtyler

I started searching yesterday and found that Alyftrek (Elexacaftor/tezacaftor/ ivacaftor), which is basically the same, if I am understanding correctly, as Trikafta, is now approved for 31 more CF mutations. If you look this up, you can tell which mutations qualify you. It might be worth looking at this especially if you already have the diagnosis of CF.

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Thanks. I have been keeping up with the new approvals but my mutations aren't yet on the list. I'm still waiting for results of theratyping being done at Cincinnati Children's Hospital.

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paxmundi | @paxmundi | 3 days ago
In reply to @sueinmn "I did a little digging and found one nearly completed research study. You may want to..." + (show)
@sueinmn

I did a little digging and found one nearly completed research study. You may want to contact the principle researcher and ask to be notified when the results come out:
https://www.centerwatch.com/clinical-trials/listings/NCT05743946/trikafta-for-patients-with-non-cystic-fibrosis-bronchiectasis/trikafta-for-patients-with-non-cystic-fibrosis-bronchiectasis
If you learn anything exciting will you share it with the group?

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Link went to a “not found” link for me also.

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jnmy | @jnmy | 2 days ago

I could not open the link that you included in your post @sueinmn however I found this lengthy article. This is a link to read about the study protocol. It’s a detailed, scientific study published in 2025.

Evaluating elexacaftor/tezacaftor/ivacaftor (ETI; Trikafta™) for treatment of patients with non-cystic fibrosis bronchiectasis (NCFBE): A clinical study protocol
https://search.app/RDi5Rkte4anvq68SA

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2 replies
jnmy | @jnmy | 2 days ago
In reply to @jnmy "I could not open the link that you included in your post @sueinmn however I found..." + (show)
@jnmy

I could not open the link that you included in your post @sueinmn however I found this lengthy article. This is a link to read about the study protocol. It’s a detailed, scientific study published in 2025.

Evaluating elexacaftor/tezacaftor/ivacaftor (ETI; Trikafta™) for treatment of patients with non-cystic fibrosis bronchiectasis (NCFBE): A clinical study protocol
https://search.app/RDi5Rkte4anvq68SA

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@lenorj I just read your comments. The article I posted about Trikafta could be of interest to you. You may want to pursue it with your insurance company provider. Good luck.

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1 reply
jnmy | @jnmy | 2 days ago

This was the Objective of the Study at Emory. @paxmundi

“Objective
Based on a considerable body of evidence, we believe many patients with NCFBE have disease likely to benefit from drugs such as elexacaftor/tezacaftor/ivacaftor (ETI) that activate CFTR-dependent ion transport. ETI is currently prescribed solely for treatment of CF and has not been adequately tested or proposed for patients with NCFBE, many of whom exhibit decreased CFTR function. Accordingly, we are conducting a clinical trial of ETI in subjects carrying a diagnosis of NCFBE.“

Evaluating elexacaftor/tezacaftor/ivacaftor (ETI; Trikafta™) for treatment of patients with non-cystic fibrosis bronchiectasis (NCFBE): A clinical study protocol
https://search.app/RDi5Rkte4anvq68SA
This could help people with other CF gene mutations in the future. It sounds like more research is underway. That’s good news.

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lenorj | @lenorj | 2 days ago
In reply to @jnmy "@lenorj I just read your comments. The article I posted about Trikafta could be of interest..." + (show)
@jnmy

@lenorj I just read your comments. The article I posted about Trikafta could be of interest to you. You may want to pursue it with your insurance company provider. Good luck.

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I spoke to my pulmonologist about it and she told me that because of my marginal test numbers I do not qualify for the medication.

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1 reply
Mentor
Sue, Volunteer Mentor | @sueinmn | 2 days ago
In reply to @jnmy "I could not open the link that you included in your post @sueinmn however I found..." + (show)
@jnmy

I could not open the link that you included in your post @sueinmn however I found this lengthy article. This is a link to read about the study protocol. It’s a detailed, scientific study published in 2025.

Evaluating elexacaftor/tezacaftor/ivacaftor (ETI; Trikafta™) for treatment of patients with non-cystic fibrosis bronchiectasis (NCFBE): A clinical study protocol
https://search.app/RDi5Rkte4anvq68SA

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Thank you

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jnmy | @jnmy | 2 days ago
In reply to @lenorj "I spoke to my pulmonologist about it and she told me that because of my marginal..." + (show)
@lenorj

I spoke to my pulmonologist about it and she told me that because of my marginal test numbers I do not qualify for the medication.

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@lenorj Perhaps that will change in the future for you and others. Hopefully with additional research, more gene mutations will be covered.

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