Appearance of cystic fibrosis

Question

My uncle is 83 years old and only now he was diagnosed with cystic fibrosis. I cannot imagine that because I read that this disease is congenital. Is it possible, though, that this diagnosis is correct?

Many thanks
N. H.

Answer

Dear Ms H.,

Receiving the diagnosis of cystic fibrosis at the age of 83 is very extraordinary but not impossible. The disease has a genetic reason and therefore it exists in the affected individuals since birth. However, the troubles can also occur at a later point, in this case the diagnosis is also given at a later date. If the course of the disease is mild or if nobody has the idea that CF could be the reason for existing symptoms, diagnosis can happen very delayed in time (maybe only at the age of 83?!).
For us experts this case is very interesting. We would like to know how and in connection with which troubles your uncle was diagnosed with CF. If the diagnosis is confirmed and save then your uncle would currently be the oldest known CF patient in Germany.

For further questions please call [… German telephone number is given in the original answer].

Best regards,
Dr. Christina Smaczny

20.09.2012

2.10.12
The questioner informed us that the diganosis cystic fibrosis could be ruled out-the patient in question suffers from a pulmonary fibrosis.
D. d'Alquen