Relationship between bronchiectasis and cystic fibrosis

@sueinmn
Thanks Sue. I actually meant Bronchiectasis specialists that might be more involved with or knowledgeable about current research into drugs like Trikafta.

I agree though, more testing would be helpful. For those who have a negative diagnosis for CF but are right on the margins, there might be benefit?

Maybe value for others too when someone can’t physically do clearance for a period of time.

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?

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.

The link takes me to a “ not found oage”?

How high were your numbers.l? I understand that as you age the number that indicates a definite CF diagnosis goes up.

@sueinmn I’ll see what I can do here. I’d definitely share with everyone if I can get any information.

CF is caused by mutations in the CFTR gene, leading to a defective protein that regulates the flow of water and chloride in and out of cells, causing a buildup of thick, sticky mucus in the lungs and other organs. These drugs work by directly targeting the defective CFTR protein, helping it function more effectively. It seems unless you have that defective gene the drug is not relevant??

My numbers were 67 and 75.

I was in the above clinical trial Sue mentions above NCT05743946 last August at Emory University. I was diagnosed in 2019 with bronchiectasis and have a great niece with CF who has been on trikafta for 6 years (is s game changer for her). In late 2023 my niece encouraged me to be tested for CF since my symptoms were so similar to my nieces'. I tested medium range for sweat chloride and have the 508del mutation ( which is the disease causing mutation) . The CF pulmonologist decided to prescribe trikafta but insurance denied because of no formal CF diagnosis. I have excessive mucus and exacerbations, and do not have the time or energy to do the necessary airway clearance due to a very demanding job. To make a long story short, the trikafta was a huge game changer for me the month I was on it . Within 24 hours the mucus and coughing stopped. I felt like a new person. Within 24 hrs of going off it, I had a wash out period and it all started again. The trikafta definitely works. Now I am really stuggling again with my regular non cf pulmonologist. From what I have heard regarding the preliminary findings of July 24, the three people who were worse off than me and were in the clinical trial all had hugely promising results just like me. I pray that that another pharma will make the generic version of trikafta once Vertex' patent is expired. But for now I continue to struggle with nebulizing saline acetylysteine, and airway clearance, which is so time consuming and so exhausting.

One more thing, my sweat chloride levels went way down while on the trikafta and way up when off the trikafta. My insurance denied trikafta 3 times because my second variant does not qualify me for CF. I don't have the digestive/ enzyme issues that my great niece has.