life expectation of CF nowadays

Question

My son is born in 2015 with the classical form of CF.
What is the life expectation of a CF patient born in 2015?

Answer

Thank you for your question. We will answer this question in general.

CF still is a life shortening disease. Major differences in severity exist in patients with the classic form of CF, compared to patients with a normal digestion. The type of mutation is the most determining factor, but even then, there are differences in disease severity and life expectancy between patients with exactly the same mutations. It is known that other genetic factors as well as 'environmental’ factors (i.e. age at diagnosis, intensity of treatment, contact with smokers,…) have an important influence on disease severity and life expectancy.

CF affects multiple organs but mainly lung damage determines the disease severity and survival. In very sick patients a lung transplant can be considered. Nevertheless, this doesn’t mean ‘cure’ as the median survival after a lung transplant is only 8 to 10 years.

Although we know a lot about patient groups, it is of course impossible to predict survival for an individual patient. For example: a patient with classic CF and mild symptoms at a younger age has no guarantee that his illness will remain mild during his entire life. No prediction can be made about the survival of a single patient. It can also not be made for an individual healthy person..
At present, the median age at death of patients with CF is in the early 30ties. It is however expected that patients born in 2000 will ‘on average’ reach 50 years even without taking into account the new treatments that emerge. With the advent of therapies targeting the basic CFTR defect, there is hope that survival will even be better.
Yours sincerely,
Prof. K. de Boeck

01.02.2016