Brinsupri experience after 3+ months

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.

@cayenne May I message you regarding hemoptysis? I too frequently bleed 🙁

Regarding brinsupri: by my pulmonologist at NJH 10 mg and 25 mg are equally successful in diminishing the frequency of exacerbations but only 25mg dose was associated with the maintenance of lung function. So when my blood pressure went up with the 25 mg dose I was advised to watch it regularly and if not stabilized get the blood pressure med. So far it is around 130 and I still take 25 mg of brinsupri. The skin bump, itchy, is gone so may be it was something else.

I’m approaching 5 months on brinsupri 25mg. The last 2 months , have been really great. No URIs, no exacerbations. My question at this time. Is anyone having a hard time getting a sputum sample? This has never happened to me in the past. I was diagnosed with BE and mac. 6 years ago (tho .I was having lots of mucus come up when doing shoulder stands in yoga for 10 years before that.) Don’t do those anymore. But I do bridges to help get up mucus after saline nebs. no sputum in sight. except a random cough every couple days.

not macular degeneration! MAC!

@crepass I have always had problems producing sputum sample but I could cough up a little. Now in my fourth month of brinsupri, I am dryyy, cannot spit out any that could be sent for testing. I drink a lot, I take mucinex and NAC and mullein tea- still desert

@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 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.

Just went to my pulmonologist and he suggested dupixent🤦🏻‍♀️ and Brinsupri. Being a medical assistant I have seen negative side effects of that biologic. Does anyone know exactly what Brinsupri does? Is that a good thing to dry up secretions? Is it killing bacteria? I have pneumonia currently and just die my first sputum culture. I’m already on two antibiotics and two inhalers, steroids and cough medicines. What can the culture tell at this point? From what I’m coughing up now it’s less and lighter not dark green. Other than NAC and bronchial clear tabs mystery naturally, I have been looking into getting a hydrogen machine and a portable oxygen machine as well for when I get sick and or get sick or exacerbated by something in the environment. Thank you for all the posts and sharing as it helps those navigate in various stages🙏💫💕 Tara

@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.