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Pain Research and Management
Volume 11, Issue 3, Pages 185-192
http://dx.doi.org/10.1155/2006/828964
Original Article

A Survey of the Pain Experienced by Males and Females with Fabry Disease

Andrea L Gibas,1 Regan Klatt,2 Jack Johnson,3 Joe TR Clarke,2 and Joel Katz1,4

1York University, Department of Psychology and School of Kinesiology and Health Science, Canada
2Hospital for Sick Children, Division of Clinical & Metabolic Genetics, Toronto, Ontario, Canada
3Executive Director, Fabry Support & Information Group, Concordia, Missouri, USA
4Toronto General Hospital, Department of Anesthesia & Pain Management, Toronto, Ontario, Canada

Copyright © 2006 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

BACKGROUND: The clinical onset of Fabry disease, a rare, X-linked, multisystemic disorder, is marked by neuropathic pain. Males suffer extensively from this disease. Females, as genetic ‘carriers’, have traditionally been viewed as either asymptomatic or mildly afflicted with this disease.

OBJECTIVES: To describe Fabry-related pain and compare experiences between the sexes. Patients’ perceptions of physician pain assessments were also examined.

METHODS: A disease-specific questionnaire was accessible on-line (www.fabry.org) and mailed to 552 members of a Fabry disease support group.

RESULTS: The response rate was 14.3% for the support group-based mail questionnaire. Females (58.0%) were significantly older (mean ± SD 45.9±13.5 years) than males (mean ± SD 40.0±12.1; t [86]=−2.11, P<0.05). Females were diagnosed with Fabry disease later (31.1±14.0 years) than males (24.2±11.9 years; t [86]=−2.43, P<0.05). Females (mean score for pain disability rating 3.0±1.4) suffered more extensive disability from migraine pain (mean score 2.2±1.3; F [1, 74]=45.0, P<0.005), and, unlike males, did not exhibit a decline in pain intensity with disease duration. Satisfaction with physician pain assessments was moderate.

CONCLUSIONS: Contrary to the traditional view of females as carriers, females with Fabry disease experienced intense disease-related pain; pain produced comparable distress and impairment in both sexes. The diagnostic delay and absence of a decline in pain symptoms over time in females suggest additional disease burden. Females may be triply disadvantaged in the health care system due to disease rarity, devalued carrier status and sex.