Pancreatic sufficient with pa?

[CFSanDiego]

Ok, I know pancreatic sufficiency isn't supposed to predict lung involvement, but thought I'd ask anyway, since in theory secretions are at least a little easier to clear due to some cftr function.

My daughter (6) has had pa since December and we can't get rid of it, although it has been I was in a good state of semi-denial about the severity of cf with her until now.

Anyway, how rapid is this proposed lung function decline supposed to happen? So far her fev1 is still holding steady (checked this week), but then again it has only been a few months...

This pa thing had been a new cloud hanging over my head and I just want the sunny skies back, you know? Hope is everything!!

Thanks,
Edan

 

[Aboveallislove]

Can't answer re the lung function other than to say I see lots on her with pseudomonas who do great. But I did want to suggest pushing to get Kalydeco off label if she is pancreatic sufficient since she must have some residual CFTR function. And I know several on Kalydeco who have cleared bacteria they were culturing regularly (and for years), after starting Kalydeco.

 

[nocode]

Hi. I am a 29 yr old female and I have had Pseudomonas since I can remember. I was diagnosed at 15 and I think I already had it then, and have never been able to get rid of it. I am pancreatic sufficient. My lung function is not good. I'm around 32% FEV1. Each person is different but I have been managing to live a good, active life until now. (Relatively active).
I wish I had been diagnosed earlier.

 

[CFSanDiego]

Aboveallislove, thanks for the suggestion. I have been considering Kalydeco. I need to get her Dr. on board. I really respect him (Dr. Milla, Stanford).

Nocode, thanks for responding. I am sorry things have progressed so far for you but glad you are managing ok. Can I ask, how progressed was your CF when you were diagnosed at 15? Also, have you been culturing anything else, or is PA it and is it mucoid or non-mucoid?

Thanks,
Edan

 

[nocode]

Hi Eden,
Believe it or not, they didn't use to talk to us about FEV1 or other lung function values at my hospital, so I was never really aware of my condition in terms of 'numbers' (this was also 15 years ago and things have come a long way). The earliest I can remember knowing what my FEV1 was was when I was around 20, and I was at 40%! As you can see, it was never very good, and I honestly cannot remember going through a sudden decline since. It has been very gradual in the last 9 years.
But take this into consideration - I started showing symptoms at 9 years old, and I was misdiagnosed for 6 years while in very poor health! So I believe the biggest decline happened during these years when I was not getting treated for CF, or anything for that matter.

PA is the only bacteria that has been cultured since diagnosis, and very seldom staph, which goes away after an antibiotic cycle. I am not sure if my PA is mucoid or non-mucoid, and I am not familiar with these terms..hmm... care to explain further?

I also have 2 mutations that are not that common: N1303K and R334W.

 

[CFSanDiego]

Hi Nocode. Thanks for your reply. The difference between mucoid and non-mucoid is that mucoid has the biofilm that is harder to penetrate with antibiotics (from what I understand). My daughter's is currently non-mucoid which means anitibiotics can beat it back a little better, but over time non-mucoid usually turns into mucoid.

Edan