Treatment of Depression in Adults with Fabry Disease
Fabry disease (FD) is a genetic X-linked, multisystemic, progressive lysosomal storage disorder (LSD). Depression has emerged as a disease complication, with prevalence estimates ranging from 15 to 62%. This is a pilot study examining the effects of psychological counseling for depression in FD on depression, adaptive functioning (AF), quality of life (QOL), and subjective pain experience. Telecounseling was also piloted, as it has beneficial effects in other chronic diseases which make in-person counseling problematic. Subjects completed 6 months of in-person or telecounseling with the same health psychologist, followed by 6 months without counseling. Self-report measures of depression, AF, QOL, and subjective pain were completed every 3 months. All subjects experienced improvements in depression, which were sustained during the follow-up period. Improvements in depression were correlated with improvements in mental health QOL and subjective pain severity, while improvements in mental health QOL were correlated with improvements in AF. While statistical comparison between counseling modes was not possible with the given sample size, relevant observations were noted. Recommendations for future research include replication of results with a larger sample size and a longer counseling period. The use of video counseling may be beneficial. In conclusion, the present pilot study supports the efficacy of psychological treatment for depression in people with FD, highlighting the importance of having health psychologists housed in LSD treatment centers, rather than specialty psychology/psychiatry settings, to increase participation and decrease potential obstacles to access due to perceived stigma.
KeywordsAdaptive functioning Depression Fabry Quality of life Telecounseling
The authors thank all the individuals with Fabry disease who participated in this study.
This study was supported by Shire Pharmaceuticals and by the National Center for Advancing Translational Sciences of the National Institutes of Health under Award Number UL1TR000454. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
- Choi NG, Wilson NL, Sirrianni L, Marinucci ML, Hegel MT (2014b) Acceptance of home-based telehealth problem-solving therapy for depressed, low-income homebound older adults: qualitative interviews with the participants and aging-service case managers. Gerontologist 54(4):704–713CrossRefPubMedGoogle Scholar
- Cleeland CS (2009) The brief pain inventory user guide. MD Anderson Cancer Center, HoustonGoogle Scholar
- Hughes DA, Evans S, Milligan A et al (2006) A multidisciplinary approach to the care of patients with Fabry disease. In: Mehta A, Beck M, Sunder-Plassmann G (eds) Fabry disease: perspectives from 5 years of FOS. Oxford PharmaGenesis, OxfordGoogle Scholar
- Maruish ME, DeRosa MA (2009) A guide to the integration of certified Short Form survey scoring and data quality evaluation capabilities. QualityMetric Incorporated, LincolnGoogle Scholar
- Maruish ME, Kosinski M (2009) A guide to the development of certified short form interpretation and reporting capabilities. QualityMetric Incorporated, LincolnGoogle Scholar