Summary
The Minnesota Muliphasic Personality Inventory (MMPI-2) is widely used in chronic illness and chronic pain populations to assess psychological functioning. We report the results of the first investigation using the MMPI-2 to assess psychological aspects of patients with Fabry disease. Fabry disease, an X-linked lysosomal storage disorder, is a multisystem progressive disease affecting the kidney, heart, and central nervous system, and is particularly associated with chronic symptoms including pain. In this study, 28 patients with Fabry disease completed the MMPI-2 and a background questionnaire. Fabry disease patients scored significantly higher than the MMPI-2 normative sample on seven clinical scales (Hs, D, Hy, Pd, Pa, Pt, Sc) and two validity scales (L, F). Individuals with elevated scores on the Hs, D, and Hy scales tend to have somatic complaints, sadness, and emotional distress. Under stress, they may experience an increase in physical symptoms. Elevated Pd, Pa, Pt, and Sc scales suggest social maladjustment, suspiciousness, and feelings of isolation. An elevated L scale suggests defensiveness; a high score on F suggests emotional turmoil. When compared with cohorts of patients with Gaucher disease (GD), chronic heart disease (CRHD), and chronic pain, the Fabry disease patients had significantly higher scores than GD patients and CRHD patients on numerous clinical (Hs, D, Si), and validity (F) scales underscoring the relative amount of suffering and pain experienced by Fabry disease patients. No significant differences on any MMPI-2 scales were found between the Fabry disease patients and the pain patients, suggesting that Fabry disease patients may be comparable to pain patient populations.
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Abbreviations
- CRHD:
-
chronic heart disease
- ERT:
-
enzyme replacement therapy
- FD:
-
Fabry disease
- GD:
-
Gaucher disease
- MMPI-2:
-
Minnesota muliphasic personality inventory
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Acknowledgements
This project was supported in part by a grant from the Genzyme Corporation, Cambridge, MA. No personnel from Genzyme participated in the design, data analyses, or interpretation of the results of this study. This work was performed under the auspices of the UCSF-Stanford Lysosomal Disease Center. The Center has received support from the Niemann–Pick Disease Foundation, the Mount Zion Health Fund, the Jewish Community Federation, the State of California Genetic Disease Branch, the Genzyme Corporation, the BioMarin Corporation, the Nutricia Corporation, Shire HGT, the Actelion Corporation, and Amicus Therapeutics. The authors thank Douglas Cort, PhD, for providing CRHD data from the UC Davis Medical Center. We also thank Roger Greene, PhD, for his invaluable assistance with this project. We thank Cecilia Fairley, MS, and Mary Needham, MS, for their contributions to phases of this work. Most importantly, we would like to thank all participants for their cooperation.
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Communicating editor: Gerard T. Berry
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Crosbie, T.W., Packman, W. & Packman, S. Psychological aspects of patients with Fabry disease. J Inherit Metab Dis 32, 745–753 (2009). https://doi.org/10.1007/s10545-009-1254-1
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DOI: https://doi.org/10.1007/s10545-009-1254-1