Table 1.

CF survival and mortality literature review—studies reporting longitudinal median survival age among people with CF, by country (n = 10 studies)

Source	Study years	Population description	Ages	Study no.	Sub-group	Age of survival median (95% CI)	Longitudinal trend direction
United States
FitzSimmons 1994 and FitzSimmons 1993 [47, 48]a, b	1969–1990	U.S. CF Foundation Patient Registry	All ages	7795	1969	14	↑
				17,857	1990	27.6
Kulich 2003 [50]	1985–1999	U.S. CF Foundation Patient Registry	All ages	31,012	1985, Males	34.5	↑
					1999, Males	37.1
					1985, Females	31.5
					1999, Females	34.9
Jackson 2011 [46] a	1986–2008	U.S. CF Foundation Patient Registry patients with death records	All ages	8849	1986	26.7 (25.3–28.3)	↑
					2008	37.4 (35.0–40.1)
Stephenson 2017 [45] c	1990–2013	U.S. CF Foundation Patient Registry	All ages	32,699	2009–2013, Includes post-transplant follow-up	40.6 (39.1–41.8)	↑ (data in figure only)
					2009–2013, Censoring at transplant	44.0
Ostrenga 2023 [49] a, d	1986–2017	U.S. CF Foundation Patient Registry linked to the National Death Index	All ages	30,788	1986–1990	29.7	↑
					2013–2017	45.8
Canada
Corey 1996 [54]	1970–1989	Canadian Patient Data Registry	All ages	3795	1970–1989, Males	29.5	↑
					1970–1974, Males	26.6
					1985–1989, Males	36.7
					1970–1989, Females	21.3
					1970–1974, Females	19.7
					1985–1989, Females	27.8
Stephenson 2015 [53] c	1990–2012	Canadian CF Registry	All ages	5787	1990	31.9 (28.3–35.2)	↑
					2008–2012	49.7 (46.1–52.2)
Stephenson 2017 [45] c	1990–2013	Canadian CF Registry	All ages	4662	2009–2013, Includes post-transplant follow-up	50.9 (50.5–52.2)	↑ (data in figure only)
					2009–2013, Censoring at transplant	57.1
France
Bellis 2007 [52] a	1994–2003	Observatoire National de la Mucoviscidose in mainland France and Reunion Island	All ages	n/s	1994–1996	28.1	↑
					2001–2003	36.4
Ireland
Jackson 2011 [46] a	1986–2008	CF Registry of Ireland patients with death records	All ages	421	1986	20.1 (13.0–34.4)	↑
					2008	35.2 (24.5-N/A)
Australia
Ruseckaite 2022 [51] a	2005–2020	Australian CF Data Registry	All ages	4601	2005–2009	48.9 (44.7–53.5)	↑
					2016–2020	56.3 (53.0–60.4)
Japan
Kozawa 2023 [55] b	1994–2022	National epidemiological survey and Japan CF Registry	All ages	132	2012	18.8 (16.6–21.1)	↑
					2022	25.2 (18.7–31.7)

CF cystic fibrosis, CI confidence interval, N/A not available, 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

^bMedian survival age also reported by gender (males, females)

^cData also reported for additional years in figure only

^dMedian survival age also reported for CFFPR standard reporting with no linkage to NDI