INTRODUCTION: Quality-of-life (QOL) assessments in frequently bleeding patients with congenital hemophilia with inhibitors and their families are confounded by preexisting arthropathy and family circumstances. Periodic QOL assessments typically made on nonbleed days may not provide complete reflections of the burden on patients/families. AIM: To evaluate the impact of bleeding episodes on patients/caregivers/families and the association between monthly QOL scores and patients' average diary experiences. METHODS: Frequently bleeding inhibitor patients (≥four bleeds in 3 months), or their caregivers, provided daily assessment of EuroQol five-dimensional questionnaire and EuroQol five-dimensional questionnaire visual analogue scale, pain (11-point Likert scale), and family anxiety/stress/activity change over 3 to 6 months. QOL scores were stratified by bleed/nonbleed days. RESULTS: Patient QOL assessments were recorded by 37 of the 39 enrolled patients/caregivers (3771 of 3777 eligible dairy days, 472 bleed/3299 nonbleed days). Median (range) diary duration was 91 (66-180) days, with 8.2% (0%-72.2%) bleed days. Mean health scores were significantly worse on bleed days than on nonbleed days (P < 0.0001 for all): EuroQol five-dimensional questionnaire index, 0.66 versus 0.82; visual analogue scale health, 69.7 versus 77.4; and pain score, 4.1 versus 1.8. Bleed days also had higher (P < 0.001) proportions of days with abnormalities in family anxiety/stress (42% vs. 30%) and family activity changes (34% vs. 21%). CONCLUSIONS: Assessing the impact of hemophilia with inhibitors on patient/family QOL typically includes periodic (likely nonbleed day) evaluations reflecting baseline abnormalities. Daily assessment, however, indicated that frequent acute bleeds impair QOL beyond patient's nonbleed day baseline. New approaches are required to assess the cumulative impact of frequent acute bleeds on patients and their families.
INTRODUCTION: Quality-of-life (QOL) assessments in frequently bleedingpatients with congenital hemophilia with inhibitors and their families are confounded by preexisting arthropathy and family circumstances. Periodic QOL assessments typically made on nonbleed days may not provide complete reflections of the burden on patients/families. AIM: To evaluate the impact of bleeding episodes on patients/caregivers/families and the association between monthly QOL scores and patients' average diary experiences. METHODS: Frequently bleeding inhibitor patients (≥four bleeds in 3 months), or their caregivers, provided daily assessment of EuroQol five-dimensional questionnaire and EuroQol five-dimensional questionnaire visual analogue scale, pain (11-point Likert scale), and family anxiety/stress/activity change over 3 to 6 months. QOL scores were stratified by bleed/nonbleed days. RESULTS:Patient QOL assessments were recorded by 37 of the 39 enrolled patients/caregivers (3771 of 3777 eligible dairy days, 472 bleed/3299 nonbleed days). Median (range) diary duration was 91 (66-180) days, with 8.2% (0%-72.2%) bleed days. Mean health scores were significantly worse on bleed days than on nonbleed days (P < 0.0001 for all): EuroQol five-dimensional questionnaire index, 0.66 versus 0.82; visual analogue scale health, 69.7 versus 77.4; and pain score, 4.1 versus 1.8. Bleed days also had higher (P < 0.001) proportions of days with abnormalities in family anxiety/stress (42% vs. 30%) and family activity changes (34% vs. 21%). CONCLUSIONS: Assessing the impact of hemophilia with inhibitors on patient/family QOL typically includes periodic (likely nonbleed day) evaluations reflecting baseline abnormalities. Daily assessment, however, indicated that frequent acute bleeds impair QOL beyond patient's nonbleed day baseline. New approaches are required to assess the cumulative impact of frequent acute bleeds on patients and their families.
Authors: J M Soucie; S D Grosse; A-E-A Siddiqi; V Byams; J Thierry; M M Zack; A Shapiro; N Duncan Journal: Haemophilia Date: 2017-06-02 Impact factor: 4.287
Authors: Miguel Escobar; James Luck; Yevhenii Averianov; Jonathan Ducore; Maria Fernanda López Fernández; Adam Giermasz; Daniel P Hart; Janna Journeycake; Craig Kessler; Cindy Leissinger; Johnny Mahlangu; Laura Villarreal Martinez; Wolfgang Miesbach; Ismail Haroon Mitha; Doris Quon; Mark T Reding; Jean-François Schved; Oleksandra Stasyshyn; Kateryna V Vilchevska; Michael Wang; Jerzy Windyga; W Allan Alexander; Ahmad Al-Sabbagh; Daniel Bonzo; Ian S Mitchell; Thomas A Wilkinson; Cédric Hermans Journal: Haemophilia Date: 2021-10-06 Impact factor: 4.263
Authors: Christine L Kempton; Michael Wang; Michael Recht; Anne Neff; Amy D Shapiro; Amit Soni; Roshni Kulkarni; Tyler W Buckner; Katharine Batt; Neeraj N Iyer; David L Cooper Journal: Patient Prefer Adherence Date: 2017-09-19 Impact factor: 2.711
Authors: Michael Wang; Katharine Batt; Craig Kessler; Anne Neff; Neeraj N Iyer; David L Cooper; Christine L Kempton Journal: Patient Prefer Adherence Date: 2017-10-25 Impact factor: 2.711
Authors: Tyler W Buckner; Michael Wang; David L Cooper; Neeraj N Iyer; Christine L Kempton Journal: Patient Prefer Adherence Date: 2017-10-11 Impact factor: 2.711
Authors: María Mareque; María Eva Mingot-Castellano; María Fernanda López-Fernández; María Teresa Álvarez-Román; Itziar Oyagüez Journal: Eur J Haematol Date: 2020-04-16 Impact factor: 2.997