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A Scoping Review of Healthcare Costs for Patients with Cystic Fibrosis

  • Scoping Review
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Abstract

Background

Cystic fibrosis (CF), one of the most common life-shortening genetic diseases, has no cure, but people living with it have seen improvements in their health and survival. The rising life expectancy and increased availability of treatment options has likely increased the lifetime costs of people living with CF. In addition, a recent drug approval for a therapy that targets the cause of the disease is one of the most expensive drugs worldwide. In light of these circumstances, it is important to have an updated understanding of the costs of CF therapy and management. This study aims to determine the extent of available literature that quantifies CF costs.

Methods

We used a scoping review framework to identify the sources and types of evidence available to determine the costs of CF therapy and management compared to the general population or a comparable population of people with other complex chronic conditions. We searched 14 databases for peer-reviewed studies and grey literature published in English since 1998. The search was conducted in August 2013 and updated in October 2014.

Results

We identified 28 studies that estimated overall, general CF costs. Of these, three studies compare CF costs to healthcare costs of a general population and only one of those provides a direct comparison of CF costs to the general population in order to calculate the incremental cost associated with CF. We estimate there are 98 systematic reviews that quantify the costs of comparable conditions and potentially provide a comparison group for people with CF.

Conclusions

There is evidence available that attempts to quantify overall, general healthcare costs of people with CF, although much of it is outdated. However, there is much less evidence available that provides a comparison of these costs with either the general population or people with comparable conditions.

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Acknowledgements

The research and one author were funded by a grant from the Cystic Fibrosis Foundation. There was no funding for manuscript preparation and the funding body played no role in the collection, analysis, or interpretation of data or in the manuscript preparation or decision to submit the manuscript for publication. The funding body did provide expert feedback on the protocol design.

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Authors and Affiliations

  1. Department of Health Policy and Management, Johns Hopkins Bloomberg School of Public Health, 624 N Broadway, Room 691, Baltimore, MD, 21205, USA

    Ilene L. Hollin & Karen A. Robinson

  2. Department of Medicine, Johns Hopkins School of Medicine, 1830 E. Monument Street., Room 8068, Baltimore, MD, 21287, USA

    Karen A. Robinson

Authors
  1. Ilene L. Hollin
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Correspondence to Ilene L. Hollin.

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Conflict of Interest

ILH has a pending grant from the study funder (Cystic Fibrosis Foundation) and has received honoraria from the same foundation for unrelated work. KAR declares no conflicts of interest.

Authors’ contributions

ILH carried out the literature search and drafted the manuscript. KAR conceived of the study, secured funding and participated in its design and coordination and helped to draft the manuscript. All authors read and approved the final manuscript.

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Hollin, I.L., Robinson, K.A. A Scoping Review of Healthcare Costs for Patients with Cystic Fibrosis. Appl Health Econ Health Policy 14, 151–159 (2016). https://doi.org/10.1007/s40258-015-0211-4

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