Numbers of patients with CF

Question

I read in a CF leaflet information on number of patients. 1.300 in the Netherlands, 23.000 in Europe and 25.000 in the US. The numbers are surprising and not related to the population in the different continents. What is the explanation: social standards, healthcare in the specific countries or parts of the world. Or is there an issue of registration?

Answer

This is an interesting question. It is not an easy task to collect correct information about number of patients in a specific country with a specific disease, in this case cystic fibrosis. A specific patient registry is important and useful in this context. In several countries a national register for cystic fibrosis has been created since many years. However, not every country has such a register. Additionally, even in case of a register, it is important to know which patients are classified under the diagnosis of CF and which patients are not. It is well known that milder forms of the disease exist, which may be called CF are not.
The American CF register managed by the Cystic Fibrosis Foundation has a long standing history and includes data on 25.000 patients. However, CF patients in the US not followed in a registered CF centre, are not included in this register. In the UK there is a long standing register, in Belgium about 10 years. There is a German and also recently a French register. Several of these national registers are grouped in the European CF register. In the European report of 2006, only 14.000 patients with CF were registered. Reports are in preparation for the following years, including already over 29.000 patients. Your remark was correct that it is difficult to have a complete and correct overview. Patient associations can also provide data and in Europe they can provide data on 35.000 patients in 28 participating countries. The quality and content of these data are however less specific compared to a register.
On the other hand, there is a variation in the frequency of CF between different countries and areas. In general it can be stated that the disease is less frequent going from the north to south of Europe. Cystic fibrosis is present in every European country. The proportion of the most frequent mutation F508del also decreases from north to south. To evaluate the quality of care in a specific country, the proportion of adult patients is studied. In countries with well organized CF care around 40% are adults, but this percentage is much lower in countries with less well organized CF care. It is easy to understand that in countries with good CF care the life expectancy of a patient is longer and therefore the total number of patients in these countries will be higher.
I hope this information is useful. You can also have a look at the website of the ECFS about the data of the European register. The website is www.ecfs.eu/ecfs_supported_initiatives/european_cf_registry.
With kind regards
K. De Boeck

23.03.2010