Incidence, population, and survival of cystic fibrosis in the UK, 1968-95

Abstract

The UK Cystic Fibrosis Survey holds data on all people resident in the UK who were diagnosed as having cystic fibrosis and born either since 1968 or before 1968 and alive in 1977. Thus, incidence may be reported from 1968 and prevalence from 1977.The previous estimates are updated to the end of 1995 from data held in the database on 23 August 1996.The incidence is now calculated as one in 2415 live births. The 1992 mid-year population was 6500 people with 65% aged under 16 years. Births outnumber deaths by 160 per year, which suggests a population of 7750 by the year 2000, with all the increase being in the adult age range.The survival of successive cohorts continues to be better than earlier cohorts, the linear descent of the curves is still evident. The infant mortality rate for cystic fibrosis is now under 20 per thousand per year and early childhood mortality is under five per thousand per year.The crude mortality rate for 1995 was 21 per thousand per year, but the standardised mortality ratio was about 3300.

Trials and tribulations: Understanding motivations for clinical research participation amongst adults with cystic fibrosis

“…Cystic fibrosis (CF), the UK's most common autosomal recessive genetic disease with an incidence of 1:2415 births (Dodge et al, 1997), represents an excellent opportunity to examine research participation within a defined patient group. The major symptoms of CF arise from abnormal sticky secretions in the respiratory and digestive tracts, the most common symptom being lung damage from recurring chest infections.…”

“…The mainstays of a wide array 6 of daily therapies are chest physiotherapy, regular courses of antibiotics and pancreatic enzymes, with patients attending specialist centres for paediatric and adult care reported to achieve a better clinical outcome (Mahadeva et al, 1998). Survival age has steadily increased from under one year in the late 1930s (Anderson, 1938) to 31.5 years currently (Dodge et al, 1997), as the disease is better understood and treatment and care are developed. Adults are generally knowledgeable about their disease and treatment and most have well-established relationships with healthcare staff.…”

‘Double or quits’: perceptions and management of organ transplantation by adults with cystic fibrosis

“…The incidence of the disease in the UK has been estimated at 1:2415 births (Dodge et al, 1997). CF is most commonly found in Caucasian populations; a lower occurrence of 1:3500 live births has been described amongst people from Mediterranean countries (Bossi, Battistini, & Braggion, 1999).…”

Demographics of the UK cystic fibrosis population: implications for neonatal screening

“…This article presents the demographics of the currently registered UK CF population focusing on the gender, ethnicity, mode of presentation and genotypes of the 5274 patients from an estimated UK population of approximately 7500 patients. 3 The considerable impact of ethnicity on CFTR mutations in the UK is shown.…”