Content validation of a self-report daily diary in patients with sickle cell disease

Abstract Background Sickle Cell Disease (SCD) is a genetic progressive vascular disease that impacts patients overall health and quality of life. Sickle-cell pain crises (SCPCs) are a hallmark clinical presentation of SCD and have been associated with increased morbidity and mortality. The Sickle Cell Pain Diary- Self Report (SCPD-S) was developed as a daily patient-reported outcome (PRO) measure primarily intended to capture the frequency and severity of SCD-related pain during and outside of a SCPC. The SCPD-S also examines the impact of the pain associated with an SCPC on other health-related quality of life concepts. The objective of this study was to investigate the content validity of the SCPD-S. Methods The content validation testing included 18 in-depth hybrid concept elicitation and cognitive debriefing interviews conducted with SCD patients in the US aged 12 years and older. Interviewers used a semi-structured interview guide and a think-aloud approach for the cognitive debriefing portion. All interviews were recorded, transcribed, coded and analyzed. Results Eighteen interviews across two rounds were conducted. Round 1 hybrid interviews (n = 12) resulted in the expansion of the SCPD-S from 13 to 19 items. Items on the impact of an SCPC on social and recreational activities, sleep, and emotional well-being were added. Five items were significantly revised, as were three response choice sets. Round 2 hybrid interviews (n = 6) confirmed the comprehensiveness of the revised diary, understandability of the wording, and appropriateness of the recall period and response sets. Saturation analyses specific to concept elicitation revealed that no additional interviews were needed. Conclusions This study provided evidence to support the content validity of the SCPD-S, a self-report daily diary. Data gathered during patient interviews indicated that the SCPD-S is a fit for purpose measure of SCD and SCPC-related pain frequency and severity and the impact of this pain on other health-related quality of life concepts including fatigue and emotional health. The numerous changes to the SCPD-S as a result of the study findings highlight the importance of the content validation process when developing a PRO measure.

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Health-related quality of life and neuropathic pain in sickle cell disease in Jamaica

Disability and Health Journal ◽

10.1016/j.dhjo.2021.101107 ◽

2021 ◽

pp. 101107

Author(s):

Rachel Bartlett ◽

Zachary Ramsay ◽

Amza Ali ◽

Justin Grant ◽

Angela Rankine-Mullings ◽

...

Keyword(s):

Quality Of Life ◽

Neuropathic Pain ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Health Related Quality ◽

Cell Disease ◽

Related Quality ◽

Health Related

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Parent-reported sleep problems in children and adolescents with sickle cell disease: relationship to health-related quality of life

Archives of Medical Science ◽

10.5114/aoms/124154 ◽

2021 ◽

Author(s):

Serkan Gunes ◽

Rahime Aldemir ◽

Adem Gunes ◽

Ozalp Ekinci

Keyword(s):

Quality Of Life ◽

Children And Adolescents ◽

Sickle Cell ◽

Sleep Problems ◽

Well Being ◽

Health Related Quality ◽

Cell Disease ◽

Related Quality ◽

Health Related

IntroductionChildren with sickle cell disease (SCD) can present a variety of clinical symptoms that may affect their sleep and health-related quality of life (HRQOL). This study aims to investigate the relation between sleep problems and HRQOL in children and adolescents with SCD.Material and methodsThe sample included 86 children and adolescents in the SCD patient group and 82 healthy controls, with an age range of 8-16 years. Subjects for the study were recruited from the Sickle Cell and Thalassemia Center of Hatay State Hospital, Hatay, Turkey. The Children’s Sleep Habits Questionnaire (CSHQ) was used to evaluate sleep problems and Kinder Lebensqualitätsfragebogen: Children’s Quality of Life Questionnaire – revised (KINDL-R) was used to examine HRQOL.ResultsTotal score, bedtime resistance, and night waking subscores of CSHQ were significantly higher in children with SCD when compared to healthy children. Total score, physical well-being, emotional well-being, social, and school subscores of KINDL-R were significantly lower in the patient group. Among SCD children, total score, bedtime resistance, sleep onset delay, daytime sleepiness, and parasomnias subscores of CSHQ were negatively correlated with KINDL-R total score. In the regression model, disease severity and CSHQ total score had significant negative associations with KINDL-R total score.ConclusionsSleep problems in SCD children appear to be negatively linked with HRQOL. Disease severity and sleep problems may be predictors of overall HRQOL in children and adolescents with SCD.

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Health Related Quality of Life of Children with Sickle Cell Anemia and Their Parents; Quantitative Study in Albaha; Saudi Arabia

Journal of Pharmaceutical Research International ◽

10.9734/jpri/2021/v33i27a31487 ◽

2021 ◽

pp. 9-17

Author(s):

Turki Alzahrani ◽

Raed Alzahrani ◽

Amer Alzahrani ◽

Abdullah Alzahrani ◽

Abdu Adawi ◽

...

Keyword(s):

Quality Of Life ◽

Saudi Arabia ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Healthy Children ◽

Health Related Quality ◽

Cell Disease ◽

Related Quality ◽

Health Related

Aims: To gain a better understanding of the quality of life (QoL) of children and impact of this disease on parents QoL. Study Design: Cross-sectional study. Place and Duration of Study: The study was conducted in King Fahd Hospital, Albaha city, Albaha, Saudi Arabia, between March2020 and February 2021. Methodology: We included 95 responses. Two different tools were used for the purpose of this study. PedsQL™ Sickle Cell Disease Module was used to measure health-related quality of life (HRQoL) in healthy children and adolescents and those with acute and chronic health conditions. Moreover, PedsQL™ Family Information Form was completed by caregivers. Median and interquartile range were used for numerical variables since they were skewed. Bivariate analyses were carried out using non-parametrical tests and Pearson correlation. The prediction of QoL was accomplished through multivariate analysis. Results: A total of (95) responses were analyzed. Female respondents were 52.6%. The age median was 12 (IQR=10-14). Mothers represented the most frequent informant 46.3% in this current study. Significant association was found between QoL and certain independent factors, some of which is parental level of education (P< .001) and marital support (P< .001). Conclusion: Sickle cell disease (SCD) is a major condition accounts for a huge burden on variable levels. This study reported that low QoL among children affected by SCD. Higher education and current marital status of the parents were significantly associated with high QoL in SCD patients. Number of workdays affected due to child health was significantly correlated with low QoL.

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