The Cystic Fibrosis Registry of Ireland (CFRI) has just published its 2019 Report.

Many thanks to all the work of the Registry, which is chaired by Professor Ed McKone, and it's CEO Godfrey Fetcher.

CFI are on the Board of CFRI but it is an independent entity, in line with good international practice. 

Click below to download a copy of the report.

The annual reports show some good progress in the survival ages of people with CF which is likely to increase in coming years.

Some key findings include the following:

•  Almost 12% of the CF population in Ireland is now over the age of 40 years of age compared with just 4% in 2009. 
• The median predicted age of survival predicts how long we can expect half the of people with CF born today are predicted to live. This is 45.7 years of age.  The increase in Median Predicted Age of Survival  since 2009 is set out on page 36 of the CFRI Report and summarised below:

• There are 1423 people with CF attending Irish Hospitals, an increase of 21% since 2001 and 89% are registered with the CF Registry of Ireland.
• As a result of developments in the management and care of people with CF, survival among individuals ‘has improved dramatically’ (CFRI, 2019). Since 2008 an average of 16 individuals have died annually. 

Screening
A total of 235 babies with CF have been identified as the result of CF being added to the National Newborn Screening Programme since 2011. This has meant that therapies can be commenced from birth. CFI continues to advocate that this Screening Programme is further widened to other diseases (there are just 8 metabolic diseases including CF screened at present in Ireland- CFI).
 

Genotype
In 2019, 55.5% of people with CF had two copies F508del gene mutation compared with UK 44.4% and USA 48.6%. A further 36.5% of the CF population in Ireland have 1 copy of the F508del , compared with 40.9% in USA and 41.1% in the UK. The remaining 8% of individuals have no copies of the F508del mutation.