Cystic fibrosis?

[Engineer88]

Has anyone been diagnosed with CF especially adults?


How does it affect BG and control? how do you deal with it?


Apparently I'm a medical mystery and potentially have 3 chronic conditions O-o

 

[Lesleywo]

I thought it was something you were born with?

 

[Engineer88]

it is. Apparently some people only have 'mild' symptoms which might not show up until adulthood and they then progress.

It can also affect selective organs where as full blown Cf affects lungs and digestive system. If its what I have its only on my lungs (currently).

Its basically gene mutations creating all sorts of variances like diabetes (1,1.5,2, MODY, LADA, etc)

 

[xAoifex]

I don't have CF but I am a nurse who works a lot with adults with cf. Sometimes the mutations are so rare that they never show up but you may have one of the more common ones plus a very rare one. CF related diabetes differs from T1 as it's due to pancreatic insufficiency as opposed to beta cell destruction or you could be unlucky and have T1 on top of CFRD but having a double whammy would be very rare. Have you had a sweat test? That is fairly conclusive. There are a few other lung conditions that could be confused with CF for example primary ciliary dyskinesia or pulmonary fibrosis. There are many cases of cf not being diagnosed until adulthood.

 

[Engineer88]

I don't have CF but I am a nurse who works a lot with adults with cf. Sometimes the mutations are so rare that they never show up but you may have one of the more common ones plus a very rare one. CF related diabetes differs from T1 as it's due to pancreatic insufficiency as opposed to beta cell destruction or you could be unlucky and have T1 on top of CFRD but having a double whammy would be very rare. Have you had a sweat test? That is fairly conclusive. There are a few other lung conditions that could be confused with CF for example primary ciliary dyskinesia or pulmonary fibrosis. There are many cases of cf not being diagnosed until adulthood.

Thanks so much xAoifex, I've not been confirmed yet but had blood taken for CFTR test yesterday. Waiting an appointment to get further info/testing as required.

Would it be possible to confuse cf diabetes with t1 in a very young patient without obvious signs of cf?

 

[xAoifex]

Thanks so much xAoifex, I've not been confirmed yet but had blood taken for CFTR test yesterday. Waiting an appointment to get further info/testing as required.

Would it be possible to confuse cf diabetes with t1 in a very young patient without obvious signs of cf?

I don't think so as, if you had cystic fibrosis related diabetes (CFRD), then you would have pancreatic insufficiency and therefore have problems with digestion and need to take enzymes (Creon) to digest fat, without these you would end up severely malnourished with terrible bowel problems (oily floaty diarrhoea, not something you could miss!) It is possible however that you could have CF without pancreatic insufficiency and bog standard t1 diabetes. Have you ever had GAD antibody tests?
At your next appointment ask for a sweat test, it's really quick easy and painless and will give a pretty reliable result, blood tests can take months or years if the mutation is rare which is a distinct possibility

 

[Engineer88]

No I've not had the sweat test but I will ask for it. will it show even with mutations?
Apparently i had a heel prick as a newborn.

Ive never been tested for GAD that I or mum know of, assumption was that HI bg and ketones at 2 and a half was T1

 

[xAoifex]

It should do as it measure the chloride in sweat and cystic fibrosis disrupts the chloride channels in the body plus it won't cause harm and could be used to either diagnose (a positive result or even a borderline positive/negative) or rule out (a definite negative). Definitely ask and see what the doctor thinks. Something like 1 in 25 people carry a CF gene so having one copy is pretty common and normally this will be one of the more common genes (the DeltaF508 is found in around 90% of people with a CF mutation) so a borderline or equivocal sweat test + one of the genes would make CF more like likely, no CF gene and a negative sweat test would make the diagnosis much more unlikely and the doctors should be looking elsewhere. The chances of 2 very rare mutations wold be very small (although not impossible)