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A standardized approach to estimating survival statistics for population-based cystic fibrosis registry cohorts.

ABSTRACT: Our objective was to quantify the effect of different statistical techniques, inclusion/exclusion criteria, and missing data on the predicted median survival age.Using the Canadian cystic fibrosis registry (CCFR), the median age of survival was calculated using both the Cox proportional hazards (PH) and the life-table methods. Through simulations, we examined how the median age of survival would change when: (1) patients were excluded, (2) death dates were inaccurate, (3) patients were lost to follow-up, (4) entire years with no clinic visits were excluded even if the? patient had a visit in subsequent years, and (5) censoring patients at their date of transplant. Simulations were run assuming 5-35% of data were affected by each scenario.Over the period 2009-2013, there were 4,666 individuals in the CCFR with 240 deaths. The observed median age of survival calculated by the Cox PH method was 50.9 [95% confidence interval (CI): 47.4, 54.3] and 50.5 from the life-table method (95% CI: 47.5, 53.5). Censoring patients at their transplant date overestimated the median age of survival by 7.2 years (58.1; 95% CI: 53.3, 64.7). Simulations determined that by missing just 15% of deaths, the median age of survival can be overestimated by 3.5 years (54.4; 95% CI: 54.2, 56.1), and having 25% of patients lost to follow-up can underestimate the median age of survival by 3.3 years (47.6; 95% CI: 46.8, 47.7).We present several recommendations to assist national cystic fibrosis registries in calculating and reporting the median age of survival in a standardized fashion. It is imperative to state the statistical method used as well as the proportion lost to follow-up and the treatment of missing data and transplanted patients. Registries must be diligent in their data collection as incomplete data can lead to overestimation and underestimation of survival.

SUBMITTER: Sykes J

PROVIDER: S-EPMC4889445 | biostudies-literature | 2016 Feb

REPOSITORIES: biostudies-literature

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A standardized approach to estimating survival statistics for population-based cystic fibrosis registry cohorts.

Sykes Jenna J Stanojevic Sanja S Goss Christopher H CH Quon Bradley S BS Marshall Bruce C BC Petren Kristofer K Ostrenga Josh J Fink Aliza A Elbert Alexander A Stephenson Anne L AL

Journal of clinical epidemiology 20151003

<h4>Objectives</h4>Our objective was to quantify the effect of different statistical techniques, inclusion/exclusion criteria, and missing data on the predicted median survival age.<h4>Study design and setting</h4>Using the Canadian cystic fibrosis registry (CCFR), the median age of survival was calculated using both the Cox proportional hazards (PH) and the life-table methods. Through simulations, we examined how the median age of survival would change when: (1) patients were excluded, (2) deat ...[more]

PMID: 26434789

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Project description:BACKGROUND: over the last 25 years several national registries of CF have been set up. Such systems can be very useful in providing an integrated resource for improving patient care and conducting research on the disease. Minimum Data Set is a common set of data items that should be used to collect and report data in the registry. The principal aim of this research was to determine minimum data set for the CF registry in north-west of Iran. METHODS: data items collected by several selected registries of cystic fibrosis were studied and an initial set of data was selected by the researchers. A group of experts including epidemiologists, pediatricians, and CF specialists were asked to review the proposed data elements and score them based on their importance by using a nine-point Likert scale. The items scored as important or highly important by more than 50 % of the experts, were included in final list of minimum data set. Availability of data was evaluated through reviewing medical records of 144 patients hospitalized in Children Hospital located in Tabriz. RESULTS: overall six classes of data (46 items) were identified in the selected registry systems for cystic fibrosis: patient demographics, administrative data, survival status, diagnostic procedures, genetic and clinical manifestations, and therapeutics. Thirty two data elements from all six categories of data were approved by the experts as the minimum data set for cystic fibrosis registry system. Availability of data in administrative category and survival class was 100 percent. Collecting data on medications was feasible in 100% of the cases as well. In class of demographic data, accessibility of patient name, age, gender, place of birth, and date of birth was 100 percent. In group of diagnostic procedures, partial availability of data was found for sweat test and genetic test. No data was found on the antenatal screening, exercise tolerance test, and glucose tolerance test. CONCLUSION: this work can be considered as a first step toward establishing CF registry system in Iran. Minimum data set can be also useful in designing electronic registry or electronic patient records for those suffering from CF toward integration of their fragmented records across continuum of the health care system in order to improve quality of shared patient care.

Project description:The effects of lung transplantation on the survival and quality of life in children with cystic fibrosis are uncertain.We used data from the U.S. Cystic Fibrosis Foundation Patient Registry and from the Organ Procurement and Transplantation Network to identify children with cystic fibrosis who were on the waiting list for lung transplantation during the period from 1992 through 2002. We performed proportional-hazards survival modeling, using multiple clinically relevant covariates that were available before the children were on the waiting list and the interactions of these covariates with lung transplantation as a time-dependent covariate. The data were insufficient in quality and quantity for a retrospective quality-of-life analysis.A total of 248 of the 514 children on the waiting list underwent lung transplantation in the United States during the period from 1992 through 2002. Proportional-hazards modeling identified four variables besides transplantation that were associated with changes in survival. Burkholderia cepacia infection was associated with a trend toward decreased survival, regardless of whether the patient underwent transplantation. A diagnosis of diabetes before the patient was placed on the waiting list decreased survival while the patient was on the waiting list but did not decrease survival after transplantation, whereas older age did not affect waiting-list survival but decreased post-transplantation survival. Staphylococcus aureus infection increased waiting-list survival but decreased post-transplantation survival. Using age, diabetes status, and S. aureus infection status as covariates, we estimated the effect of transplantation on survival for each patient group, expressed as a hazard factor of less than 1 for a benefit and more than 1 for a risk of harm. Five patients had a significant estimated benefit, 283 patients had a significant risk of harm, 102 patients had an insignificant benefit, and 124 patients had an insignificant risk of harm associated with lung transplantation.Our analyses estimated clearly improved survival for only 5 of 514 patients on the waiting list for lung transplantation. Prolongation of life by means of lung transplantation should not be expected in children with cystic fibrosis. A prospective, randomized trial is needed to clarify whether and when patients derive a survival and quality-of-life benefit from lung transplantation.

Dataset Information

A standardized approach to estimating survival statistics for population-based cystic fibrosis registry cohorts.

Publications

A standardized approach to estimating survival statistics for population-based cystic fibrosis registry cohorts.

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