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Quality of life and patient-reported outcomes in chronic severe neutropenia conditions

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Abstract

Quality of life (QOL) and patient-reported outcomes (PROs) assessments in immunodeficiency patients, including those with chronic severe neutropenia conditions, are imperative to determining modifiable health-related features to optimize care. We present the largest study to date of QOL in those with chronic severe neutropenia conditions with further evaluation of patient provider satisfaction and patient-reported outcome measures. Subjects completed electronic surveys assessing QOL, PROs, and patient provider satisfaction. There is a significantly negative impact of a chronic severe neutropenia disorder on QOL, fatigue, physical function, cognitive function and pain in adult patients when compared to controls. Children with a chronic neutropenia condition had comparable QOL to controls, but reported fewer depressive symptoms, improved mobility, and stronger self-reported peer relationships. Adults had worse scores for QOL, depression and fatigue when compared to children. Adult and pediatric chronic severe neutropenia patients or their caregivers felt that their medical provider was compassionate, trustworthy, and accessible. However, less than 50% of adult patients agreed their clinician had excellent expertise in white blood cell disorders. Chronic neutropenia complexly affect QOL and PROs. An analysis of these parameters allows for targeted interventions to improve patient psychosocial, physical and neurocognitive health.

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Abbreviations

G-CSF:

Granulocyte colony-stimulating factor

GM-CSF:

Granulocyte–macrophage colony-stimulating factor

HRQoL:

Health-related quality of life

IQR:

Interquartile range

MID:

Minimally important difference

PROMIS:

Patient-reported outcomes measurement information system

PROs:

Patient-reported outcomes

QOL:

Quality of life

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Acknowledgements

The authors acknowledge the assistance of the National Neutropenia Network, specifically Kate Bottiger, in soliciting patients for study enrollment. The mission of the National Neutropenia Network is to promote awareness, education, and research, and to provide a support system for patients with severe chronic neutropenia and their families through a national resource network.

Patient reported outcome survey questions provided by © 2010-2016 PROMIS Health Organization and PROMIS Cooperative Group. Quality of life assessed via PedsQL™—Pediatric Quality of Life Inventory™, copyright © 1998 JW Varni, Ph.D. and all rights reserved. Study tools administered electronically via the QuestionPro© survey software.

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Authors and Affiliations

  1. Department of Pediatrics, University of Michigan, 1500 E. Medical Center Drive, D4202 Medical Professional Building, Ann Arbor, MI, 48109-5718, USA

    Thomas F. Michniacki & Kelly Walkovich

  2. Department of Internal Medicine, Brigham and Women’s Hospital, Boston, MA, USA

    Lauren E. Merz

  3. Center for Human Growth and Development, University of Michigan, Ann Arbor, MI, USA

    Harlan McCaffery

  4. Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN, USA

    James A. Connelly

Authors
  1. Thomas F. Michniacki
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  2. Lauren E. Merz
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  3. Harlan McCaffery
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  4. James A. Connelly
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  5. Kelly Walkovich
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Contributions

Drs. TFM and KW conceptualized and designed the study. Dr. TFM constructed the manuscript as well as analyzed/interpreted study data. Dr. KW, Dr. JAC, Dr. LEM and Mr. HM assisted in editing the manuscript. Dr. LEM assisted in constructing the online surveys. Mr. HM additionally provided statistical support.

Corresponding author

Correspondence to Thomas F. Michniacki.

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Conflict of interest

The authors note no conflicts of interest.

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 Declaration of Helsinki and its later amendments or comparable ethical standards.

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Michniacki, T.F., Merz, L.E., McCaffery, H. et al. Quality of life and patient-reported outcomes in chronic severe neutropenia conditions. Int J Hematol 113, 735–743 (2021). https://doi.org/10.1007/s12185-021-03089-8

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  • DOI: https://doi.org/10.1007/s12185-021-03089-8

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