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Quality of Life Research

, Volume 27, Issue 8, pp 2057–2065 | Cite as

Executive function and quality of life in individuals with Marfan syndrome

  • Ileana Ratiu
  • Thomas B. Virden
  • Hope Baylow
  • Melissa Flint
  • Mitra Esfandiarei
Article

Abstract

Purpose

Marfan syndrome (MFS) is a connective tissue disorder that affects skeletal, ocular, pulmonary, cardiovascular, and central nervous systems. Psychological and physiologic symptoms may lead to diminished quality of life (QoL) in individuals with MFS compared with healthy individuals. Currently, there is little evidence regarding the impact of MFS on executive function and QoL. This study examined perceptions of executive function and QoL among persons with MFS.

Method

A total of 318 participants with MFS completed surveys assessing perceptions of executive function abilities and QoL. Responses to executive function questions were grouped using principle component analysis. Responses to QoL questions were separated into overall QoL, questions dealing with satisfaction of QoL, and importance placed on aspect of QoL.

Results

Principle component analysis revealed that executive function difficulties, particularly mental fatigue, associated with MFS symptoms affect QoL satisfaction and total QoL. Interestingly, medication status did not significantly impact QoL, over and above executive function difficulties.

Conclusions

The findings of the current study suggest that individuals with MFS may experience specific executive function difficulties which impact QoL. These findings also have implications for clinicians who work with individuals with MFS.

Keywords

Quality of life Marfan syndrome Executive function 

Notes

Acknowledgements

The authors would like to thank The Marfan Foundation and Genetic Aortic Disease Association (GADA) for their assistance with patient recruitment for this study.

Funding

This research study was not funded by any agency or grant. The authors are employed by the affiliated universities. The lead author presented a portion of this research at the Arizona Speech-Language Hearing Association Convention, for which she received financial support from Midwestern University. No author received honoraria for speaking at a symposium. The Marfan Foundation and Genetic Aortic Disease Association (GADA) assisted with patient recruitment for this study. Two authors, Ileana Ratiu and Melissa Flint, were asked to serve on an expert panel for The Marfan Foundation following this study. These authors do not receive payment for this service. Mitra Esfandiarei has received two grants from The Marfan Foundation to support other translational research projects in the mouse model of MFS. The authors have no other financial or non-financial relationships to disclose.

Compliance with ethical standards

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study. The experimental procedures were approved by the Midwestern University Human Subjects Institutional Review Board. No personally identifying information was link to participant responses on the survey.

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Midwestern UniversityGlendaleUSA
  2. 2.Richmond University Medical CenterStaten IslandUSA
  3. 3.Hofstra UniversityHempsteadUSA

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