Survival comparison of patients with cystic fibrosis in Canada and the United States: A population-based cohort study

Annals of Internal Medicine

Volumn 166, Issue 8, 2017, Pages 537-546

  Stephenson, Anne L ^a   Sykes, Jenna ^a   Stanojevic, Sanja ^b   Quon, Bradley S ^c   Marshall, Bruce C ^d   Petren, Kristofer ^d   Ostrenga, Josh ^d   Fink, Aliza K ^d   Elbert, Alexander ^d   Goss, Christopher H ^e  

^a ST MICHAEL'S HOSPITAL   (Canada)

^b UNIVERSITY OF TORONTO   (Canada)

^c UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^d CYSTIC FIBROSIS FOUNDATION   (United States)

^e UNIVERSITY OF WASHINGTON MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AZITHROMYCIN; AZTREONAM LYSINE; COLISTIN; DORNASE ALFA; SODIUM CHLORIDE; TOBRAMYCIN;

ARTICLE; CANADA; CHILD; COHORT ANALYSIS; CYSTIC FIBROSIS; FEMALE; FOLLOW UP; HUMAN; LONGITUDINAL STUDY; LUNG FIBROSIS; LUNG TRANSPLANTATION; MALE; MEDICAID; MEDICARE; MULTICENTER STUDY; NEWBORN SCREENING; POPULATION RESEARCH; PRESCHOOL CHILD; PRIORITY JOURNAL; SURVIVAL; SURVIVAL TIME; TREND STUDY; UNITED STATES; COMPARATIVE STUDY; EPIDEMIOLOGY; HEALTH INSURANCE; INSURANCE; MORTALITY; PROPORTIONAL HAZARDS MODEL; RISK FACTOR; SURVIVAL RATE;

CANADA; CYSTIC FIBROSIS; HUMANS; INSURANCE COVERAGE; INSURANCE, HEALTH; LUNG TRANSPLANTATION; PROPORTIONAL HAZARDS MODELS; RISK FACTORS; SINGLE-PAYER SYSTEM; SURVIVAL RATE; UNITED STATES;

EID: 85015717144     PISSN: 00034819     EISSN: 15393704     Source Type: Journal    
DOI: 10.7326/M16-0858     Document Type: Article

References (37)

1. George PM, Banya W, Pareek N., Bilton D, Cullinan P, Hodson M.E., et al. Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007. BMJ. 2011; 342:d1008. [PMID: 21357627] doi:10.1136/bmj.d1008
2. Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013; 162:530-535. [PMID: 23062247] doi:10.1016/j.jpeds.2012.08.040
3. MacKenzie T, Gifford AH, Sabadosa K.A., Quinton HB, Knapp EA, Goss CH, et al Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the Cystic Fibrosis Foundation patient registry. Ann Intern Med. 2014; 161:233-41. [PMID: 251 33359] doi:10.7326/M13-0636
4. Liou TG, Adler FR, Fitzsimmons S.C., Cahill BC, Hibbs JR, Marshall BC. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol. 2001; 153:345-52. [PMID: 11207152]
5. Corey M, Farewell V. Determinants of mortality from cystic fibrosis in Canada, 1970-1989. Am J Epidemiol. 1996; 143:1007-17. [PMID: 8629607]
6. Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988; 41:583-91. [PMID: 3260274]
7. Goss CH, MacNeill SJ, Quinton H.B., Marshall BC, Elbert A, Knapp EA, et al Children and young adults with CF in the USA have better lung function compared with the UK. Thorax. 2015; 70:229-36. [PMID: 25256255] doi:10.1136/thoraxjnl-2014-205718
8. Fogarty A, Hubbard R, Britton J. International comparison of median age at death from cystic fibrosis. Chest. 2000; 117:1656-60. [PMID: 10858398]
9. Kollberg H. Incidence and survival curves of cystic fibrosis in Sweden. Acta Paediatr Scand. 1982; 71:197-202. [PMID: 7136627]
10. Jackson AD, Daly L, Kelleher C., Marshall BC, Quinton HB, Foley L, et al The application of current lifetable methods to compare cystic fibrosis median survival internationally is limited. J Cyst Fibros. 2011; 10:62-5. [PMID: 20888308] doi:10.1016/j.jcf.2010.08.021
11. McCormick J, Mehta G, Olesen H.V., Viviani L., Macek M Jr, Mehta A; European Registry Working Group. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet. 2010; 375:1007-13. [PMID: 20304245] doi:10.1016/S0140-6736(09)62161-9
12. Martin B, Schechter MS, Jaffe A, Cooper P., Bell SC, Ranganathan S. Comparison of the US and Australian cystic fibrosis registries: the impact of newborn screening. Pediatrics. 2012; 129:e348-55. [PMID: 22250024] doi:10.1542/peds.2011-0567
13. Cystic Fibrosis Canada. Annual Registry Report. Toronto, ON, Canada: Cystic Fibrosis Canada; 2011.
14. Cystic Fibrosis Foundation. Cystic Fibrosis Foundation National Patient Registry Annual Data Report. Bethesda: Cystic Fibrosis Foundation; 2011.
15. Sykes J, Stanojevic S, Goss C.H., Quon BS, Marshall BC, Petren K, et al A standardized approach to estimating survival statistics for population-based cystic fibrosis registry cohorts. J Clin Epidemiol. 2016; 70:206-13. [PMID: 26434789] doi:10.1016/j.jclinepi.2015.08.026
16. McCormick J, Sims EJ, Green M.W., Mehta G., Culross F, Mehta A. Comparative analysis of Cystic Fibrosis Registry data from the UK with USA, France and Australasia. J Cyst Fibros. 2005; 4:115-22. [PMID: 15978536]
17. Farrell PM, Rosenstein BJ, White T.B., Accurso FJ, Castellani C, Cutting GR, et al; Cystic Fibrosis Foundation. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008; 153:S4-S14. [PMID: 18639722] doi:10.1016/j.jpeds.2008.05.005
18. Aaron SD, Stephenson AL, Cameron D.W., Whitmore GA. A statistical model to predict one-year risk of death in patients with cystic fibrosis. J Clin Epidemiol. 2015; 68:1336-45. [PMID: 25655532] doi: 10.1016/j.jclinepi.2014.12.010
19. Stanojevic S, Wade A, Stocks J., Hankinson J, Coates AL, Pan H, et al Reference ranges for spirometry across all ages: a new approach. Am J Respir Crit Care Med. 2008; 177:253-60. [PMID: 180 06882]
20. Kuczmarski RJ, Ogden CL, Grummer-Strawn LM, Flegal K.M., Guo SS, Wei R, et al. CDC growth charts: United States. Adv Data. 2000: 1-27. [PMID: 11183293]
21. Cole TJ, Bellizzi MC, Flegal K.M., Dietz WH. Establishing a standard definition for child overweight and obesity worldwide: international survey. BMJ. 2000; 320:1240-3. [PMID: 10797032]
22. World Health Organization. BMI classification. 2006. Accessed at http://apps.who.int/bmi/index.jsp?introPage=intro-3.html on 26 September 2016.
23. Moran A, Hardin D, Rodman D., Allen HF, Beall RJ, Borowitz D, et al Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report. Diabetes Res Clin Pract. 1999; 45:61-73. [PMID: 10499886]
24. Kerem E, Kerem B. Genotype-phenotype correlations in cystic fibrosis. Pediatr Pulmonol. 1996; 22:387-95. [PMID: 9016472]
25. Normand ST, Landrum MB, Guadagnoli E, Ayanian J.Z., Ryan TJ, Cleary PD, et al. Validating recommendations for coronary angiography following acute myocardial infarction in the elderly: a matched analysis using propensity scores. J Clin Epidemiol. 2001; 54:387-98. [PMID: 11297888]
26. Muggeo VM. Estimating regression models with unknown breakpoints. Stat Med. 2003; 22:3055-71. [PMID: 12973787]
27. van Buuren S., Groothuis-Oudshoorn K. MICE: multivariate imputation by chained equations in R. J Stat Softw. 2011; 45:1-67.
28. Lai HJ, Cheng Y, Cho H., Kosorok MR, Farrell PM. Association between initial disease presentation, lung disease outcomes, and survival in patients with cystic fibrosis. Am J Epidemiol. 2004; 159: 537-46. [PMID: 15003957]
29. Quon BS, Psoter K, Mayer-Hamblett N, Aitken M.L., Li CI, Goss CH. Disparities in access to lung transplantation for patients with cystic fibrosis by socioeconomic status. Am J Respir Crit Care Med. 2012; 186:1008-13. [PMID: 22983958] doi:10.1164/rccm.201205-0949OC
30. Schechter MS, Shelton BJ, Margolis P.A., Fitzsimmons SC. The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States. Am J Respir Crit Care Med. 2001; 163: 1331-7. [PMID: 11371397]
31. Egan TM, Murray S, Bustami R.T., Shearon TH, McCullough KP, Edwards LB, et al Development of the new lung allocation system in the United States. Am J Transplant. 2006; 6:1212-27. [PMID: 1661 3597]
32. Stephenson AL, Sykes J, Berthiaume Y., Singer LG, Aaron SD, Whitmore GA, et al Clinical and demographic factors associated with post-lung transplantation survival in individuals with cystic fibrosis. J Heart Lung Transplant. 2015; 34:1139-45. [PMID: 26087666] doi:10.1016/j.healun.2015.05.003
33. Merlo CA, Clark SC, Arnaoutakis G.J., Yonan N., Thomas D, Simon A, et al National healthcare delivery systems influence lung transplant outcomes for cystic fibrosis. Am J Transplant. 2015; 15:1948-57. [PMID: 25809545] doi:10.1111/ajt.13226
34. Ramos KJ, Quon BS, Psoter K.J., Lease ED, Mayer-Hamblett N, Aitken ML, et al Predictors of non-referral of patients with cystic fibrosis for lung transplant evaluation in the United States. J Cyst Fibros. 2016; 15:196-203. [PMID: 26704622] doi:10.1016/j.jcf.2015.11.005
35. Merlo CA, Weiss ES, Orens J.B., Borja MC, Diener-West M, Conte JV, et al Impact of U.S. Lung Allocation Score on survival after lung transplantation. J Heart Lung Transplant. 2009; 28:769-75. [PMID: 19632571] doi:10.1016/j.healun.2009.04.024
36. Gries CJ, Mulligan MS, Edelman J.D., Raghu G., Curtis JR, Goss CH. Lung allocation score for lung transplantation: impact on disease severity and survival. Chest. 2007; 132:1954-61. [PMID: 18079228]
37. Nick JA, Chacon CS, Brayshaw S.J., Jones MC, Barboa CM, St Clair CG, et al. Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis. Am J Respir Crit Care Med. 2010; 182:614-26. [PMID: 20448091] doi:10.1164/rccm.201001-0092OC