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Hi Wendy,
Your journey both in the lead up to & after you finally got your CF diagnosis sounds so similar to mine & like you, I wished I had been diagnosed earlier but feel at least now we know, it enables us to get the treatment we need going forward. As they say…. knowledge is power! In response to your question regarding fungal sinus & lung infections, I thought I’d share my experience and hope it is helpful to you. I started having frequent sinus infections from around 18 years of age and just before getting my CF diagnosis 7 years ago, I started having strange fluid & blackish/brown discharge sinus discharge accompanied by severe sinus headache & was feeling very unwell. Saw my wonderful ENT specialist, who via a scope, saw that I had a huge fungal ball in my left sinus & after unsuccessfully trying to remove it in his rooms, he removed it under general anaesthetic & gave my sinuses a wash out at the same time & I felt so much better immediately after the procedure. The pathology showed it was Aspergillus. Because the fungal ball was able to be removed successfully, I wasn’t given any anti-fungal medication after the procedure. Initially after that, I didn’t have too many sinus issues for a while, but then five years ago, I began having frequent & severe sinus infections again that made me feel quite unwell most of the time & proved extremely difficult to treat & I would only get a brief reprieve from sinus symptoms & infections after each course of antibiotics. Unfortunately, I am allergic to Bactrim & Augmentin, which can apparently be quite effective for sinus infections so had to rely on other antibiotics such as Klacid, Ceclor & after my CF diagnosis, I was given Ciprofloxacin for it, which was a bit more effective than previous antibiotics I had tried, but again quite often, only gave me brief reprieve. My ENT recommended I also try using twice daily sinus flo powder washes to help keep the sinuses as clear of troublesome mucus as possible. Since my CF diagnosis, what we started to notice was that every time I got another sinus infection, (which caused severe pain & sometimes secondary swelling requiring Prednisone to reduce it), it would trigger & exacerbate my respiratory symptoms (from the sinus gunk dripping down into my lungs) & I would then develop another chest infection & once had pneumonia as well. When this pattern became more frequent & I required multiple hospital admissions for IV antibiotics etc, my ENT suggested doing minor sinus surgery to “open/widen” the sinus cavities to allow the sinus washes to be more effective in removing the sinus mucous build up. This gave me some relief for a longer period of time which we were pleased about, but unfortunately eventually the sinus infections began again & the same sinus/chest infection pattern started again & my sinuses would bleed as well. I then had more major sinus surgery & this time my ENT surgeon opened/widened the sinus cavity area even more and thankfully that was more successful & I was sinus/fungal infection free for a considerable amount of time (about 18 months) & I also then had less chest infections as well during that time which was great. For the past two years, I’ve had a couple of sinus infections again & for the past few months I’ve had that blackish/brown sinus discharge off & on again & a sputum sample pathology showed I had a fungal called Purpureocilium Lilacinum, which I was told was apparently quite common & usually doesn’t cause problems (but it seems to with me!). It’s likely that it was in my sinuses as well, but my sinuses weren’t swabbed at the same time). My ENT specialist has organised for me to have a sinus swab next time I have that same discharge & hopefully if we can identify any other fungal infections, we can try & successfully treat it with a more specific medication. My current sinus management treatment plan is: Twice daily sinus flo granules with an added nasal steroid once a day (Pulmicort Respules), twice daily hypertonic saline sinus inhalation (6% Hypersal) via a Pari Sinus System machine (which has been a Godsend in treating my sinus issues!) & my CF team also suggested we try Inhaled Colistin (antibiotic) into my sinuses via my Pari Sinus System, one month on, one month off. This treatment plan seems to be relatively effective in at least reducing the frequency & severity of my sinus symptoms & infections. Having a very caring, empathetic, highly skilled & ENT specialist who has experience & knowledge about CF related sinus problems, who is always prepared to see me any time that my sinuses cause problems, makes dealing with this a bit easier & I feel very fortunate to have him as my specialist. At each appointment, he always views my sinuses via a scope & clears out any troublesome/infected sinus mucous that’s there which always brings me relief and helps me to feel better. If at any time I have symptoms & his scope prevents him from seeing the possible area of concern, he gets me to have a Sinus CT which can detect fungal balls, absesses etc. Like you, due to my rare mutation (R117H/1210-34TG (12T (5), I’m ineligible for Trikafta, so unfortunately that isn’t a possible treatment option, but Wendy, I hope me sharing my experience is helpful to you in some way, as I know only too well how ongoing sinus/fungal problems can make you feel quite lousy. I wish you all the best going forward.