Development and validation of a disease-specific quality of life scale for adult patients with Fabry disease in Japan
Plain English summary Adults with Fabry disease experience severe challenges, which adversely impact their quality of life (QOL). As it is a rare disease, non-patients lack awareness of the severity of its symptoms and the resultant social difficulties of the patients. Most instruments that measure...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
SpringerOpen
2022-11-01
|
| Series: | Journal of Patient-Reported Outcomes |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s41687-022-00525-z |
| _version_ | 1798016815644803072 |
|---|---|
| author | Yuta Koto Wakana Yamashita Yoko Lee Nozomi Hadano Chikara Kokubu Norio Sakai |
| author_facet | Yuta Koto Wakana Yamashita Yoko Lee Nozomi Hadano Chikara Kokubu Norio Sakai |
| author_sort | Yuta Koto |
| collection | DOAJ |
| description | Plain English summary Adults with Fabry disease experience severe challenges, which adversely impact their quality of life (QOL). As it is a rare disease, non-patients lack awareness of the severity of its symptoms and the resultant social difficulties of the patients. Most instruments that measure QOL are not specific enough to address issues related to Fabry disease. Therefore, in this study, a measurement instrument known as the Adult Fabry QOL (AFQOL) scale was designed and validated. The 39-item scale covers five domains that are congruent with the symptoms of adult Fabry disease. It differs from other QOL scales as it also assesses the impact of work and personal relationships on patients’ QOL and symptoms that progress in adulthood. This study has important implications for healthcare providers who treat adult patients with Fabry disease, enabling them to have a fuller picture of the unique needs of this population. |
| first_indexed | 2024-04-11T15:56:42Z |
| format | Article |
| id | doaj.art-4c2736ccaf374de299727c7e95083be5 |
| institution | Directory Open Access Journal |
| issn | 2509-8020 |
| language | English |
| last_indexed | 2024-04-11T15:56:42Z |
| publishDate | 2022-11-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Journal of Patient-Reported Outcomes |
| spelling | doaj.art-4c2736ccaf374de299727c7e95083be52022-12-22T04:15:08ZengSpringerOpenJournal of Patient-Reported Outcomes2509-80202022-11-016111310.1186/s41687-022-00525-zDevelopment and validation of a disease-specific quality of life scale for adult patients with Fabry disease in JapanYuta Koto0Wakana Yamashita1Yoko Lee2Nozomi Hadano3Chikara Kokubu4Norio Sakai5Child Healthcare and Genetic Science Laboratory, Division of Health Science, Graduate School of Medicine, Osaka UniversityChild Healthcare and Genetic Science Laboratory, Division of Health Science, Graduate School of Medicine, Osaka UniversityChild Healthcare and Genetic Science Laboratory, Division of Health Science, Graduate School of Medicine, Osaka UniversityChild Healthcare and Genetic Science Laboratory, Division of Health Science, Graduate School of Medicine, Osaka UniversityChild Healthcare and Genetic Science Laboratory, Division of Health Science, Graduate School of Medicine, Osaka UniversityChild Healthcare and Genetic Science Laboratory, Division of Health Science, Graduate School of Medicine, Osaka UniversityPlain English summary Adults with Fabry disease experience severe challenges, which adversely impact their quality of life (QOL). As it is a rare disease, non-patients lack awareness of the severity of its symptoms and the resultant social difficulties of the patients. Most instruments that measure QOL are not specific enough to address issues related to Fabry disease. Therefore, in this study, a measurement instrument known as the Adult Fabry QOL (AFQOL) scale was designed and validated. The 39-item scale covers five domains that are congruent with the symptoms of adult Fabry disease. It differs from other QOL scales as it also assesses the impact of work and personal relationships on patients’ QOL and symptoms that progress in adulthood. This study has important implications for healthcare providers who treat adult patients with Fabry disease, enabling them to have a fuller picture of the unique needs of this population.https://doi.org/10.1186/s41687-022-00525-zFabry diseaseQuality of lifeSurveys and questionnairesPatient-reported outcome measuresScale development and validation |
| spellingShingle | Yuta Koto Wakana Yamashita Yoko Lee Nozomi Hadano Chikara Kokubu Norio Sakai Development and validation of a disease-specific quality of life scale for adult patients with Fabry disease in Japan Journal of Patient-Reported Outcomes Fabry disease Quality of life Surveys and questionnaires Patient-reported outcome measures Scale development and validation |
| title | Development and validation of a disease-specific quality of life scale for adult patients with Fabry disease in Japan |
| title_full | Development and validation of a disease-specific quality of life scale for adult patients with Fabry disease in Japan |
| title_fullStr | Development and validation of a disease-specific quality of life scale for adult patients with Fabry disease in Japan |
| title_full_unstemmed | Development and validation of a disease-specific quality of life scale for adult patients with Fabry disease in Japan |
| title_short | Development and validation of a disease-specific quality of life scale for adult patients with Fabry disease in Japan |
| title_sort | development and validation of a disease specific quality of life scale for adult patients with fabry disease in japan |
| topic | Fabry disease Quality of life Surveys and questionnaires Patient-reported outcome measures Scale development and validation |
| url | https://doi.org/10.1186/s41687-022-00525-z |
| work_keys_str_mv | AT yutakoto developmentandvalidationofadiseasespecificqualityoflifescaleforadultpatientswithfabrydiseaseinjapan AT wakanayamashita developmentandvalidationofadiseasespecificqualityoflifescaleforadultpatientswithfabrydiseaseinjapan AT yokolee developmentandvalidationofadiseasespecificqualityoflifescaleforadultpatientswithfabrydiseaseinjapan AT nozomihadano developmentandvalidationofadiseasespecificqualityoflifescaleforadultpatientswithfabrydiseaseinjapan AT chikarakokubu developmentandvalidationofadiseasespecificqualityoflifescaleforadultpatientswithfabrydiseaseinjapan AT noriosakai developmentandvalidationofadiseasespecificqualityoflifescaleforadultpatientswithfabrydiseaseinjapan |