Normal Spirometry (PFTs) Frequent Infections?

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UNCLoveCF

New member
My spirometry is normal yet I am on IV antibiotics every 4-6 weeks. My FEV1 is in the 90's yet I am frequently coughing up blood and sick. I used to believe PFTs were indicative of one's overall pulmonary status with CF, but now I realize this is not always true. My CF doctor told me that I have "atypical CF lung disease" due to my lung function(spirometry) and yet I am frequently symptomatic for infections and experience hemoptysis. I culture: Ralstonia Mannitolilytica, Pseudomonas (on occasion), Aspergillus, and Oropharyngeal Flora. I use my Vest and Hypertonic Saline 3% three times daily and Pulmozyme once daily. I am compliant with my medications and therapy. One year ago I experienced massive hemoptysis and was embolized. I continued to experience hemoptysis, but now my bleeds are getting closer together and worse with each recent episode. To say that I am concerned is an understatement. I cannot tolerate -Azole antifungals due to adverse side effects and interaction with Motilium (Domperidone) which I take for Gastroparesis. I was on IV Amphotericin B for two weeks last Fall, but had to stop taking it due to renal (kidney) failure. Anyone else have good spirometry numbers and yet experience frequent exacerbations and/or hemoptysis reguarly?

Update: Frequent infections were actually caused by Aspergillus. My IGE levels are normal, yet extensive testing proved that Aspergillus indeed was causing all of my pulmonary issues. I also believe Advair was contributing to my hemoptysis as I tend to experience airway irritation from different inhaled meds.
 
M

moxie1

Guest
My pfts aren't as good as yours, but my FEV1 is around 70%. I had alot of years when I had hemoptysis with no symptoms of an exacerbation. Everything was fine, pfts were fine, just bleeding. I had 3 emoblizations and was still bleeding after the 3rd one. Mine were getting worse too.
For me, the bleeding shut off like a faucet when I stopped the Pulmozyme. I lost lung function without it, so experimented for awhile and found that I can tolerate Pulmozyme once every 3 days and maintain lung function without having bleeds. Pulmozyme is an enzyme. Itbreaksdown things. My theory is that when your FEV1 is higher, the airways are clearer, and the pulmozyme is breaking down at the lung tissue as well as mucus.
Note:If you decide to decrease the Pulmozyme, do it slowly. My lungs did not like it when I stopped cold turkey!
 
M

moxie1

Guest
My pfts aren't as good as yours, but my FEV1 is around 70%. I had alot of years when I had hemoptysis with no symptoms of an exacerbation. Everything was fine, pfts were fine, just bleeding. I had 3 emoblizations and was still bleeding after the 3rd one. Mine were getting worse too.
For me, the bleeding shut off like a faucet when I stopped the Pulmozyme. I lost lung function without it, so experimented for awhile and found that I can tolerate Pulmozyme once every 3 days and maintain lung function without having bleeds. Pulmozyme is an enzyme. Itbreaksdown things. My theory is that when your FEV1 is higher, the airways are clearer, and the pulmozyme is breaking down at the lung tissue as well as mucus.
Note:If you decide to decrease the Pulmozyme, do it slowly. My lungs did not like it when I stopped cold turkey!
 
C

cfsucks

Guest
it may be the meds your on causing your hemotopysis- for me tobi is a huge culprit. the inhalation of the drugs may be causing irritation to your airways.
 
C

cfsucks

Guest
it may be the meds your on causing your hemotopysis- for me tobi is a huge culprit. the inhalation of the drugs may be causing irritation to your airways.
 
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