I don't have CF diagnosis yet, although my CF doctor believes I have CF related syndrome. I didn't push to get the CF diagnosis yet as I thought it might influence future insurance options.
I have been on trikafta for 4 months , off label drug use, and it is helping me alot. Insurance had denied for 3 years and then overturned their decision when my cf pharmacist went to bat for me and wrote a 3.5 page appeal letter. I am also trying pulmozyme instead of or in addition to sodium chloride nebs. My regular BE pulmonologist is silently going along with this since I am doing so well and much better than on the regimen they had me on, which was just airway clearance.
Before trikafta I was on the brink of giving my notice to my job because I could not control the amount and frequency of green phlegm and airway clearance through nebulizing. Insurance may or may not re-approve trikafta starting in May for the remainder of the year, but I am hopeful it will, since I am doing so much better now. Fatigue is still very much a problem, but my job is terribly demanding on my fragile body, and my goal was always to work til 65. I don't have much energy for other things besides work, but I am ok with that for now, knowing that retirement is coming in Jan 0f 2027. You have to save alot of money to plan for expensive copays and out of pocket maxes when on expensive meds. Reason I qualified, finally for the trikafta is because I have the 508del mutation and borderline sweat chlorides and 90 % of CF symptoms including bronchiectasis and because the clinical trial I went on for trikafta proved to help me.