Brinsupri experience after 3+ months

@taracronwall
Brinsupri (brensocatib) works by blocking the enzyme DPP1 (dipeptidyl peptidase 1) to reduce inflammation in non-cystic fibrosis bronchiectasis (NCFB) by stopping neutrophils from activating damaging proteins, leading to fewer lung flare-ups and less lung destruction. It works by preventing the overreaction of these immune cells that cause chronic airway damage.

@cwal
Thank you for describing the physiology behind that drug. I guess it just blows my mind that it could possibly let prevent flare ups. With my body it’s a virus when I get sick in 2 days Ive got pneumonia thick green can’t breathe. So I’m trying to weigh the side effects with what it would mean in the long run. Will it extend my lung life? Will it actually prevent the quick infections that happen as soon as I get sick? Otherwise when I breathe in someone’s vaping or cigarette smoke or perfume cologne gas exhaust febreeze bathroom scented chemicals at providers offices that usually causes me extra coughing and more time on the vibration vest, reactions to environmental things don’t cause me to have infections only catching a virus. So I’m on the fence. 🧐I’m just glad that there is this forum so we can help one another, grateful!

@crepass Glad to hear you are doing so well on Brinsupri. I can imagine it would be disconcerting to not produce sputum after having been productive for several years. I'd be curious what your doctor thinks. Maybe not an issue unless you have an exacerbation? When's your next CT scan? I'd wonder if you could drop down to 10mg if you go for a while without a flare up.

As for me, I've been in Brinsupri 25mg for a little over 4 months. At the 4 month mark, I saw a significant reduction in sputum, resulting in less time with airway clearance. Yay! So far, I have BE only and leave regular sputum samples. The last time I had a flare and was treated with abx, I was not nearly as ill as I usually get, and got better faster. As of now, I still produce sputum easily. I've had BE for over 2 decades. I still have some early morning coughing (during sleep) but coughing is greatly diminished during the day. When I cough it seems something sets it off and I wonder if it's my asthma. Airway clearance 2x day with 7% saline, mucinex 1200mg 1-2x/day, and mullein tea 5 out of 7 days a week, (I have a giant bag of loose tea to use up!) and daily movement/exercise.

@scoop Just found your reply to Crepass.
When do you have your next C Scan?
My mornings are filled with nearly continuous clearing after a nights sleep....and that's with waking up at least two times a night and clearing mucus for a short period of time before I feel I can go back to sleep.
Do I understand, in the two decades you have not had a MAC infection and therefore never have been on the big 3?
Barbara

@scoop Always good to hear that others are doing well as well. I think it’s only been a couple of weeks that I have dried up. Had quit mucinex around the end of september but still do beaucoup liquids. tonight, went to a support group where all 3 be doctors were in attendance. Dr. Ali said it was a good clinic question, but also that since brinsupri has 2 functions at this time. To decrease exacerbations, and to slow down decreasing lung function over time. So maybe there will be trade offs on dosage as they learn more. not really an answer, but I will be very interested in all new information at these upcoming bronchiectasis conferences.

@blm1007blm1007 I'll probably have a CT later this year. To my knowledge I have not had a NTM infection. Over the years I've submitted a lot of sputum samples and none has picked it up.

Have you considered Brinsupri? Sounds like you might benefit from it.

@scoop I am not ready to start Brinsupri.
I do understand "you might benefit from it."
With my feeling fine and BE my only problem at 83 years of age.... and just that the mucus is annoying......... I am sticking with my life long 'rule' ....no matter what I am considefing...... including drugs......" If it hasn't been out for a good period of time I'm not ready to try it."
I have not had to be on any medications prior to the BE diagnosis. So for now it's just the nebulizing of the saline.
I have had a low load of MAI that was diagnosed in 2023 but I am sure I had it for a couple of years before that due to what I brought up once in a while. It was a small green sticky jell substance, 1/2 the size of a dime.....I no longer have that...the green. My mucus is light yellow now.
I will look forward to hear about your journey as you go along and especially results of the CT.
Thanks scoop for your reply and all you have helped with your posts.
Barbara

As an addition to discussion regarding skin changes on brinsupri:
Research found in the autosomal recessive immunodeficiency Papillon–Lefevre syndrome (PLS), the affected children almost invariably present with intractable suppurative gum infections (chronic gingivitis) that lead to loss of teeth by the age of 6–8 years (Pham et al., 2004; Pham, 2006). DPP1 is also expressed at high levels in the skin, and marked thickening of the skin of the palms and soles (hyperkeratosis) can result from failed keratinocyte desquamation at these sites.
a mutation in the DPP1 gene (CTSC), results in diffuse palmoplantar hyperkeratosis, severe prepubertal periodontitis, and premature loss of both deciduous and permanent teeth
Drugs like brinsupri depresses DPP 1 causing the skin and gum problems in people with PL.

@robot Should we be tested for this gene mutation before taking Brinsupri?