CF

Question

Can you also have a mild form of CF? I mean that you are not sick even if you have a low lung capacity.

Answer

Dear Questioner,

Thank you for your question.

CF is a disease with a very variable expression as to age of onset of symptoms as well as to the number and severity of organs involved. Also the severity of lung disease varies between patients.

Since the discovery of the CFTR gene, it has become clear that many different errors in this gene can cause the disease.

Besides the classic clinical symptoms (disease in the lungs and the digestive system from early age on, a clearly abnormal sweat test and male infertility), there are also patients with 2 mutations in the CFTR gene and a much milder disease or disease limited to one organ (e.g. only male sterility). In such a case of 'mild CF' there is even discussion among physicians whether the term CF should be used or not. In such cases terms as CFTR-related disease or atypical CF are also used.

In order to follow up the evolution of CF we use lung function tests: we measure the content of air in the lungs (lung capacity) as well as the maximal volume that can be exhaled in one second.

A mild form of CF implies that is a normal or near to normal lung function, that remains well preserved over time.
So a low lung capacity and a mild form of CF don’t go together.

We hope this response has clarified your question.
Best regards,
Prof. K. De Boeck

06.05.2013