Quality of Life of Patients with Wiskott Aldrich Syndrome and X-Linked Thrombocytopenia: a Study of the Primary Immune Deficiency Consortium (PIDTC), Immune Deficiency Foundation, and the Wiskott-Aldrich Foundation
In: Journal of Clinical Immunology, Jg. 39 (2019-11-01), Heft 8
academicJournal
- 786 - 794
Zugriff:
BackgroundWe undertook a study to determine the impact of Wiskott Aldrich Syndrome (WAS) and X-linked thrombocytopenia (XLT) and their therapies upon the health-related quality of life (HRQOL) of patients and their families.Materials and methodsWe undertook a survey of patients and their families, who self-identified as having either WAS or XLT. We assessed the PedsQL™ 4.0, the parent proxy form, and the family impact module. These results were compared with normative data from previously published reports.ResultsSixty-eight patients (29 patients completed both the PedsQL™ 4.0 and the parent proxy form; 21 completed only the PedsQL™ 4.0; and 18 completed only the parent proxy form) were included. In contrast to patient-reported outcomes, parents of patients who had a bone marrow transplant (BMT) reported that their children had better QOL scores compared with those who did not (82.6 vs. 73.3, p = 0.023). The QOL of patients vs. previously published normative data showed decreases in patient scores for psychosocial health (72.62 vs. 86.58, p =
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Quality of Life of Patients with Wiskott Aldrich Syndrome and X-Linked Thrombocytopenia: a Study of the Primary Immune Deficiency Consortium (PIDTC), Immune Deficiency Foundation, and the Wiskott-Aldrich Foundation
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Autor/in / Beteiligte Person: | Shah, Ami J ; Sokolic, Robert ; Logan, Brent ; Yin, Ziyan ; Iyengar, Sumathi ; Scalchunes, Chris ; Mangurian, Christina ; Albert, Michael ; Cowan, Morton J |
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Zeitschrift: | Journal of Clinical Immunology, Jg. 39 (2019-11-01), Heft 8 |
Veröffentlichung: | eScholarship, University of California, 2019 |
Medientyp: | academicJournal |
Umfang: | 786 - 794 |
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