Active patients
There are 10,655 active patients currently recorded on the Registry. Active patients are people who are currently alive and have had an annual review recorded in the last three years. 10,070 annual reviews were recorded in 2019.
Median age
21 is the median age of people with CF in the UK. 60.6% of the population are aged 16 or over, and 56.7% of the population are aged 18 or over.
Mode of presentation
2,952 (29.3%) people were diagnosed by newborn screening. Aside from newborn screening, the most common three presentations were:
- Abnormal stools/fatty stool (steatorrhea)/malabsorption
- Failure to thrive/malnutrition
- Persistent or acute respiratory infection
Diagnosis
22 days is the median age that people aged under 16 in 2018 were diagnosed with cystic fibrosis. 14.9% of people in the Registry were diagnosed at age 16 or over.
CF population by devolved nation
This map shows how many people with cystic fibrosis there are living in England, Scotland, Wales and Northern Ireland.
Infections
Pseudomonas
39.4% of people aged 16 and over have chronic Pseudomonas. The median age of people with chronic Pseudomonas was 25 years in 2009, compared to 30 in 2019.
88.7% of people with chronic Pseudomonas were on inhaled antibiotic therapy in 2019, compared to 74.3% in 2009.
Non-tuberculous mycobacterium (NTM)
NTM has reduced from 7.1% to 6.7% in the past year. 53.7% of people recorded as having NTM were being treated, which is 4.5% more than in 2018.
Aspergillus
Aspergillus is reported in 15.7% of people.
Allergic bronchopulmonary aspergillosis (ABPA), an immune response to Aspergillus infection, has reduced in prevalence from 15.1% in 2014, to 7.5%.
Median predicted survival age
For people born today, using 2014-2018 data, median predicted survival age is 49.1 years old. The median predicted survival age for females (45.7) is almost 6 years lower than males (51.6).
Of the 114 people with CF who died in 2019, the median age of death was 31 years old.
Pregnancy
45 men with CF became fathers.
58 women with CF became mothers.
Mucus thinners
36.8% of people were on hypertonic saline in 2019, compared to 6.9% in 2009.
67.6% of people were on DNase in 2019, compared to 38.4% in 2009.
IV antibiotics
44.5% of people had at least one course of IV antibiotics in 2019. 38.0% of these were administered in hospital, and 23.1% were administered at home.
Cystic fibrosis-related diabetes (CFRD)
13.1% of children aged 10-15 were on CFRD treatment.
33.9% of adults 16 and over were on CFRD treatment.
Burden of treatment
80.5% of people with CF were on at least one form of inhaled therapy, and 19.5% of people were on no inhaled therapy.
People on CFTR modifiers
646 people were on ivacaftor (Kalydeco)
404 people were on lumacaftor/ivacaftor (Orkambi)
197 people were on tezacaftor/ivacaftor (Symkevi)
13 people were on elexacaftor/tezacaftor/ivacaftor (Kaftrio)
Transplant
2009
144 people were being evaluated for a transplant, 79 people were accepted and 19 had a double lung transplant.
2019
241 people were being evaluated for a transplant, 96 people were accepted and 50 had a double lung transplant.