Table 2.
CF survival and mortality literature review—studies reporting longitudinal median age at death among people with CF, by country (n = 15 studies)
| Source | Study years | Population description | Ages | Study no. | Sub-group | Age at death Median (IQR) |
Longitudinal trend direction |
|---|---|---|---|---|---|---|---|
| United States | |||||||
| Halliburton 1996 [67] b | 1979–1991 | CF deaths identified via the National Center for Health Statistics | All ages | 6500 | 1979 | 15 | ↑ |
| 1991 | 23 | ||||||
| Hurley 2014 [62] b | 1972–2009 | CF deaths identified via CDC WONDER | All ages | 13,239 | 2009 | 23.4 (16.4–27.6) | ↑ (data in figure only) |
| Stephenson 2017 [45] b | 1990–2013 | U.S. CF Foundation Patient Registry | All ages | 45,448 | 1990–2013 | 24.9 (range 0.0–81.4) | ↑ |
| 32,699 | 2009–2013 | 26.9 (range 0.3–76.9) | |||||
| Singh 2023 [58] a, c | 1999–2020 | Death certificate review of CDC WONDER | All ages | 11,068 | 1999 | 24 (18–33) | ↑ |
| 2020 | 37 (25–55) | ||||||
| Canada | |||||||
| Stephenson 2015 [53] b | 1990–2012 | Canadian CF Registry | All ages | 5787 | 1990 | 21.7 | ↑ |
| 2012 | 32 | ||||||
| Stephenson 2017 [45] b | 1990–2013 | Canadian CF Registry | All ages | 5941 | 1990–2013 | 26.6 (range 0.0–79.3) | ↑ |
| 4662 | 2009–2013 | 31.9 (range 0.3–79.3) | |||||
| Mexico | |||||||
| Bustamante 2021 [60] d | 2000–2020 | People with CF registered and followed at the CF Center at University Hospital, Monterrey | All ages | 205 | 2000–2020 | 12.5 (range 0.1–43.7) | ↑ |
| 2000–2005 | 9.6 (range 0.1–30.6) | ||||||
| 2016–2020 | 15.5 (Range: 0.4–39.3) | ||||||
| Brazil | |||||||
| Santo 2021 [57] a, e | 1999–2017 | CF deaths identified in the Brazilian National Ministry of Health Mortality Database | All ages | 2854 | 1999–2017, underlying cause | 23.5 (7.5–65.5) | ↑ |
| 1999–2017, non-underlying cause | 15.5 (0.3–69.5) | ||||||
| 68 | 1999 | 7.5 (0.5–24.5) | |||||
| 289 | 2017 | 56.5 (18.5–74.5) | |||||
| de Azevedo 2023 [68] a | 2009–2018 | Brazilian CF Register | All ages | 1044 | 2009 | 21.4 (range 6.3–80.0) | ↓ |
| 3359 | 2018 | 18.4 (range 0.3–40.6) | |||||
| England and Wales | |||||||
| Hurley 2014 [62] b | 1972–2009 | CF deaths identified via the Office of National Statistics for England and Wales | All ages | 4622 | 2009 | 22.1 (16.6–32.0) | ↑ (data in figure only) |
| France | |||||||
| Bellis 2007 [52] a | 1994–2003 | Observatoire National de la Mucoviscidose in mainland France and Reunion Island | All ages | 2168 | 1994 | 14.5 | ↑ |
| 4104 | 2003 | 22.0 | |||||
| Germany | |||||||
| Stern 2008 [64] | 1995–2005 | German CF Quality Assessment project | All ages | 6835 | 1995 | 18.5 | ↑ |
| 2005 | 23.7 | ||||||
| Italy | |||||||
| Alicandro 2015 [61] a, e | 1970–2011 | CF deaths identified in the Italian National Institute of Statistics | < 65 years | 338 | 1970–1975 | 0.5 (0.1–4.0) | ↑ |
| 293 | 2006–2011 | 29.0 (21.0–38.0) | |||||
| Spain | |||||||
| Ramalle-Gomara 2008 [63] | 1981–2004 | CF deaths for patients < 30 years identified from the Instituto Nacional de Estadıstica | < 30 years | 528 | 1999–2004, Males | 24 (range 0.0–28.9) | ↑ |
| 1999–2004, Females | 19 (range 0.0–29.3) | ||||||
| 1981, Males | 4.4 | ||||||
| 2004, Males | 20.1 | ||||||
| 1981, Females | 3.8 | ||||||
| 2004, Females | 17.7 | ||||||
| Sweden | |||||||
| Lannefors 2002 [65] b | 1971–1999 | People with CF across Sweden identified via clinical and mortality record review | All ages | 628 | 1991–1998 | 26 (range 0–72) | ↑ (data in figure only) |
| South Africa | |||||||
| Zampoli 2022 [59] a | 1974–2019 | Children with CF diagnosed and managed at the Red Cross War Memorial Children's Hospital | Pediatric | 288 | Before 1990 (1974–1990) | 5.5 (0.5–7.6) | ↑ |
| After 2010 (2010–2019) | 22.5 (19.7–25.2) | ||||||
| Multi-Country (11 countries) | |||||||
| Fogarty 2000 [66] b, f | 1974–1994 | CF deaths identified via national death records | n/s | 18,182 | 1974 | 8 | ↑ |
| 1994 | 21 | ||||||
CDC U.S. Centers for Disease Control and Prevention, CF cystic fibrosis, IQR interquartile range, N/S not stated, U.S. United States; ↑ = increasing over time; ↓ = decreasing over time.
aData also reported for additional years; the first and last years of data available are presented in this table
bData also reported for additional years in figure only
cMedian age at death also reported by gender (males, females), race (white, others), and ethnicity (non-Hispanic, Hispanic)
dMedian age of death also reported by gender (males, females), SES (high, low), pancreatic insufficiency (yes, no), chronic Pseudomonas aeruginosa infection at 6 years (yes, no), and genotype (homozygous F508del, heterozygous F508del, other genotype, missing)
eMedian age at death also reported by gender (males, females)
fCountries include Australia, Belgium, England and Wales, France, Germany, the Netherlands, Ireland, New Zealand, Scotland, Sweden, and the U.S.