Background and Epidemiology

Abstract

Cystic fibrosis (CF) is the most common autosomal-recessive disease in white persons. Significant advances in therapies and outcomes have occurred for people with CF over the past 30 years. Many of these improvements have come about through the concerted efforts of the CF Foundation and international CF societies; networks of CF care centers; and the worldwide community of care providers, researchers, and patients and families. There are still hurdles to overcome to continue to improve the quality of life, reduce CF complications, prolong survival, and ultimately cure CF. This article reviews the epidemiology of CF, including trends in incidence and prevalence, clinical characteristics, common complications, and survival.

Keywords: BMI; Cystic fibrosis; FEV(1); MRSA; Mortality; Newborn screening; Pseudomonas aeruginosa.

Figure 1

Number of people with CF and new diagnoses of CF in the US each year. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 2

Map indicating distribution of individuals with CF in the US. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 3

Proportion of pediatric (green) and adult patients (blue) in the CFFPR every five years from 1988 – 2013 From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 4

Distribution of patients with CF by age in the CFFPR. The number receiving Medicare and/or Medicaid insurances is highlighted From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 5

Mean FEV₁ % predicted by age for birth cohorts of people with CF. The population average for people without CF is 100% predicted at every age. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 6

Median FEV₁ % predicted versus median BMI percentile for children ages 6–19 years of age. The CF Foundation goal is a BMI ≥50^th percentile. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 7

Prevalence of bacteria in respiratory secretions by age in 2014. MRSA = methicillin resistant S. aureus. MDR-PA = multi-drug resistant P. aeruginosa. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 8

Prevalence of bacteria in respiratory secretions from 1989 to 2014. MRSA = methicillin resistant S. aureus. MDR-PA = multi-drug resistant P. aeruginosa. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 9

Proportion of patients with CF who meet CF Foundation-recommended eligibility requirements and who are prescribed chronic CF therapies. Each line starts in the year that FDA approval was received or when high-level evidence was published supporting its use in CF. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 10

Proportion of patients treated for a pulmonary exacerbation with IV antibiotics. From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.

Figure 11

Median Predicted Survival among individuals included in the CFFPR from 1986–2014 From Cystic Fibrosis Foundation. Patient Registry 2014 Annual Report. Bethesda 2015; with permission.