my life expectation

Question

Hello ECORN-CF experts,

I am 13 years old and I suffer from mild CF. Only my lungs are infected. My liver and other organs are all right.
Everywhere I see things on the net about life expectations of 40. I am a normal child and want also a future. What is my life expectation?
Gr.
Imar

Answer

Thank you for your question. We will answer this question generally. Specific questions about your own case should be discussed with your doctor.

There are several ways in which the term mild CF (cystic fibrosis) is used. First of all, we know that, besides the classical 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 a much milder disease or disease limited to one organ (e.g. only male infertility). In such a case of 'mild CF' there is even discussion among physicians whether the term CF should be used or not. For these people there is no evidence that their life will be shortened. In such cases terms as CFTR-related disease or atypical CF are also used.
But also among patients with the classic clinical symptoms, there are major differences in severity, also between patients with a normal digestion. One reason for these differences is the type of mutation of those patients. But even then, there are also differences in disease severity and life expectations between patients with the exact same mutations. We know that also others than genetic factors determine the severity of the disease such as 'environmental’ factors, age at diagnosis, intensity of treatment, contact with smokers,…) .
Another way to define mild CF may be: a patient who is less ill than the average patient with CF. This can e.g. be expressed as: a better lung function than 3/4 of the other CF patients of the same age. Or a better life expectancy than the average patient with CF, e.g. a patient older than 40 years with two F508del mutations.

Though we can tell a lot about groups of patients, it is very difficult to make a prediction for each individual patient. For example: a patient with classic CF and mild symptoms at younger age has no guarantee that his illness will remain mild during his entire life.

CF is indeed still a life-shortening illness . The median survival age of patients with cystic fibrosis is 37 years. For patients who are born in 2000 we expect that they will reach the age of 50 years.
CF affects multiple organs in the body but it is mainly the lung damage that determines the disease severity and life expectation. In very sick patients a lung transplant can be considered. This gives a second chance but still not a normal life. Also, the median survival after a lung transplantation is only 8 to 10 years.
There is hope that treatments that deal with the basic defect of CF will improve the life expectations. The first such promising treatment with ivacaftor is already on the market for patients with a very specific mutation.
Prof. K. de Boeck

30.09.2013