Self-administered version of the Fabry-associated pain questionnaire for adult patientsReport as inadecuate

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Orphanet Journal of Rare Diseases

, 10:113

Lysosomal storage diseases


BackgroundFabry-associated pain may be the first symptom of Fabry disease FD and presents with a unique phenotype including mostly acral burning triggerable pain attacks, evoked pain, pain crises, and permanent pain. We recently developed and validated the first Fabry Pain Questionnaire FPQ for adult patients. Here we report on the validation of the self-administered version of the FPQ that no longer requires a face-to-face interview but can be filled in by the patients themselves allowing more flexible data collection.

MethodsAt our Würzburg Fabry Center for Interdisciplinary Treatment, Germany, we have developed the self-administered version of the FPQ by adapting the questionnaire to a self-report version. To do this, consecutive Fabry patients with current or past pain history n = 56 were first interviewed face-to-face. Two weeks later patients’ self-reported questionnaire results were collected by mail n = 55. We validated the self-administered version of the FPQ by assessing the inter-rater reliability agreement of scores obtained by supervised administration and self-administration of the FPQ.

ResultsThe FPQ contains 15 questions on the different pain phenotypes, on pain development during life with and without therapy, and on impairment due to pain. Statistical analysis showed that the majority of questions were answered in high agreement in both sessions with a mean AC1-statistic of 0.857 for 55 nominal-scaled items and a mean ICC of 0.587 for 9 scores.

ConclusionsThis self-administered version of the first pain questionnaire for adult Fabry patients is a useful tool to assess Fabry-associated pain without a time-consuming face-to-face interview but via a self-reporting survey allowing more flexible usage.

KeywordsFabry disease Fabry-associated pain Pain questionnaire Electronic supplementary materialThe online version of this article doi:10.1186-s13023-015-0325-7 contains supplementary material, which is available to authorized users.

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Author: Barbara Magg - Christoph Riegler - Silke Wiedmann - Peter Heuschmann - Claudia Sommer - Nurcan Üçeyler


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