Published ahead of print on August 15, 2002, doi:10.1164/rccm.200202-087OC
Am. J. Respir. Crit. Care Med., Volume 166, Number 12, December 2002, 1550-1555
A more recent version of this article appeared on December 15, 2002
Submitted on February 8, 2002
Accepted on August 9, 2002
Developing Cystic Fibrosis Lung Transplant Referral Criteria Using Predictors of Two Year Mortality
Nicole Mayer-Hamblett1*, Margaret Rosenfeld2, Julia Emerson2, Christopher H Goss3, and Moira L Aitken3
1 Pediatrics, University of Washington, Seattle, WA, USA; Statistical Analysis Unit, CF Therapeutics Development Network Coordinating Center, Children's Hospital and Regional Medical Center, Seattle, WA, USA,
2 Pediatrics, University of Washington, Seattle, WA, USA,
3 Medicine, University of Washington, Seattle, WA, USA
* To whom correspondence should be addressed. E-mail: nhambl{at}chmc.org.
The first study objective was to develop a model identifying the best clinical predictors of 2-year mortality among cystic fibrosis patients, in order to assist in selection of appropriate candidates for lung transplantation. Using multivariate logistic regression, age, height, FEV1, respiratory microbiology, number of hospitalizations for pulmonary exacerbations, and number of home intravenous antibiotic courses were all found to be significant predictors of 2-year mortality among 14,572 patients in the Cystic Fibrosis Foundation National Patient Registry 6 years of age in 1996. The second objective was to compare the diagnostic accuracy of our model when used to guide referral for lung transplant to that of the widely used criterion of an FEV1 <30% predicted. Surprisingly, this well-fitting model derived from the largest collection of cystic fibrosis patient data available provided no better diagnostic accuracy than the simpler FEV1 criterion. Both had high negative predictive values (98% and 97%, respectively), but only modest positive predictive values (33% and 28%, respectively). Transplant referral decisions based either on a multivariate logistic model or the criterion of an FEV1 <30% predicted are likely to result in high rates of premature referral. Better clinical predictors of short-term mortality among cystic fibrosis patients are needed.
Key words: Cystic Fibrosis, mortality, lung transplantation, logistic models.
This article has been cited by other articles:

|
 |

|
 |
 
M Loeve, P T. W van Hal, P Robinson, P A de Jong, M H Lequin, W C Hop, T J Williams, G D Nossent, and H A Tiddens
The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease
Thorax,
October 1, 2009;
64(10):
876 - 882.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. Bourke, S. Doe, A. Gascoigne, K Heslop, M Fields, D Reynolds, and K Mannix
An integrated model of provision of palliative care to patients with cystic fibrosis
Palliative Medicine,
September 1, 2009;
23(6):
512 - 517.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
M. Kreider and R. M. Kotloff
Selection of Candidates for Lung Transplantation
Proceedings of the ATS,
January 15, 2009;
6(1):
20 - 27.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. M. Levine
Lung Transplantation
ACCP Pulmonary Med Brd Rev,
January 1, 2009;
25(0):
563 - 584.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. T. Saavedra, G. J. Hughes, L. A. Sanders, M. Carr, D. M. Rodman, C. D. Coldren, M. W. Geraci, S. D. Sagel, F. J. Accurso, J. West, et al.
Circulating RNA Transcripts Identify Therapeutic Response in Cystic Fibrosis Lung Disease
Am. J. Respir. Crit. Care Med.,
November 1, 2008;
178(9):
929 - 938.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. von Drygalski and J. Biller
Anemia in Cystic Fibrosis: Incidence, Mechanisms, and Association With Pulmonary Function and Vitamin Deficiency
Nutr Clin Pract,
October 1, 2008;
23(5):
557 - 563.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J W Wilson, R M du Bois, and T E King Jr
Challenges in pulmonary fibrosis: 8 {middle dot} The need for an international registry for idiopathic pulmonary fibrosis
Thorax,
March 1, 2008;
63(3):
285 - 287.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. Stern, B. Wiedemann, P. Wenzlaff, and on behalf of the German Cystic Fibrosis Quality As
From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995 2006
Eur. Respir. J.,
January 1, 2008;
31(1):
29 - 35.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
N. Mayer-Hamblett, B. W. Ramsey, and R. A. Kronmal
Advancing Outcome Measures for the New Era of Drug Development in Cystic Fibrosis
Proceedings of the ATS,
August 1, 2007;
4(4):
370 - 377.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. H. Goss and A. L. Quittner
Patient-reported Outcomes in Cystic Fibrosis
Proceedings of the ATS,
August 1, 2007;
4(4):
378 - 386.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
N. Mayer-Hamblett, M. L. Aitken, F. J. Accurso, R. A. Kronmal, M. W. Konstan, J. L. Burns, S. D. Sagel, and B. W. Ramsey
Association between Pulmonary Function and Sputum Biomarkers in Cystic Fibrosis
Am. J. Respir. Crit. Care Med.,
April 15, 2007;
175(8):
822 - 828.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. Hadjiliadis
Special Considerations for Patients With Cystic Fibrosis Undergoing Lung Transplantation
Chest,
April 1, 2007;
131(4):
1224 - 1231.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. H Goss and J. L Burns
Exacerbations in cystic fibrosis {middle dot} 1: Epidemiology and pathogenesis
Thorax,
April 1, 2007;
62(4):
360 - 367.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
K. Buranawuti, M. P Boyle, S. Cheng, L. L Steiner, K. McDougal, M D. Fallin, C. Merlo, P. L Zeitlin, B. J Rosenstein, P. J Mogayzel Jr, et al.
Variants in mannose-binding lectin and tumour necrosis factor {alpha} affect survival in cystic fibrosis
J. Med. Genet.,
March 1, 2007;
44(3):
209 - 214.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
E. F. McKone, C. H. Goss, and M. L. Aitken
CFTR Genotype as a Predictor of Prognosis in Cystic Fibrosis.
Chest,
November 1, 2006;
130(5):
1441 - 1447.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J K Block, K L Vandemheen, E Tullis, D Fergusson, S Doucette, D Haase, Y Berthiaume, N Brown, P Wilcox, P Bye, et al.
Predictors of pulmonary exacerbations in patients with cystic fibrosis infected with multi-resistant bacteria
Thorax,
November 1, 2006;
61(11):
969 - 974.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. A. Belkin, N. R. Henig, L. G. Singer, C. Chaparro, R. C. Rubenstein, S. X. Xie, J. Y. Yee, R. M. Kotloff, D. A. Lipson, and G. R. Bunin
Risk Factors for Death of Patients with Cystic Fibrosis Awaiting Lung Transplantation
Am. J. Respir. Crit. Care Med.,
March 15, 2006;
173(6):
659 - 666.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. H. Goss, G. D. Rubenfeld, B. W. Ramsey, and M. L. Aitken
Clinical Trial Participants Compared with Nonparticipants in Cystic Fibrosis
Am. J. Respir. Crit. Care Med.,
January 1, 2006;
173(1):
98 - 104.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
P. A. Flume, C. Strange, X. Ye, M. Ebeling, T. Hulsey, and L. L. Clark
Pneumothorax in Cystic Fibrosis
Chest,
August 1, 2005;
128(2):
720 - 728.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
T. M. Egan and R. M. Kotloff
Pro/Con Debate: Lung Allocation Should Be Based on Medical Urgency and Transplant Survival and Not on Waiting Time*
Chest,
July 1, 2005;
128(1):
407 - 415.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. D. Nathan
Lung Transplantation: Disease-Specific Considerations for Referral
Chest,
March 1, 2005;
127(3):
1006 - 1016.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. Kraemer, A. Blum, A. Schibler, R. A. Ammann, and S. Gallati
Ventilation Inhomogeneities in Relation to Standard Lung Function in Patients with Cystic Fibrosis
Am. J. Respir. Crit. Care Med.,
February 15, 2005;
171(4):
371 - 378.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. B. Rosenbluth, K. Wilson, T. Ferkol, and D. P. Schuster
Lung Function Decline in Cystic Fibrosis Patients and Timing for Lung Transplantation Referral
Chest,
August 1, 2004;
126(2):
412 - 419.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A.R. Glanville and M. Estenne
Indications, patient selection and timing of referral for lung transplantation
Eur. Respir. J.,
November 1, 2003;
22(5):
845 - 852.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. L. Gibson, J. L. Burns, and B. W. Ramsey
Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis
Am. J. Respir. Crit. Care Med.,
October 15, 2003;
168(8):
918 - 951.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. J. Tobin
Pediatrics, Surfactant, and Cystic Fibrosis in AJRCCM 2002
Am. J. Respir. Crit. Care Med.,
February 1, 2003;
167(3):
333 - 344.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. J. Tobin
Chronic Obstructive Pulmonary Disease, Pollution, Pulmonary Vascular Disease, Transplantation, Pleural Disease, and Lung Cancer in AJRCCM 2002
Am. J. Respir. Crit. Care Med.,
February 1, 2003;
167(3):
356 - 370.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
P. G. Noone and T. M. Egan
Cystic Fibrosis: When to Refer for Lung Transplantation-Is the Answer Clear?
Am. J. Respir. Crit. Care Med.,
December 15, 2002;
166(12):
1531 - 1532.
[Full Text]
[PDF]
|
 |
|
Copyright © 2002 American Thoracic Society
|
|
|