Cognitive and Emotional Factors and Pain-Related Outcomes in Youth with Sickle Cell Disease

Children with sickle cell disease (SCD) experience acute episodes of pain that may require visits to emergency department (ED) or hospitalizations. SCD is associated with cognitive deficits that are particularly evident in domains of executive functioning (e.g., attention, working memory). Many patients/parents also report emotional concerns related to SCD. It is not known the degree to which these concerns impact disease self-management and healthcare utilization. This study investigated cognitive and emotional factors as contributors to pain-related healthcare utilization (visits to ED and hospitalizations) in children with SCD. The cohort included 112 youth with SCD (all genotypes) between ages 7-16 years who received care at Children's National Hospital in Washington, DC. Measures of socioeconomic status (e.g., parent education, family income, 1- vs. 2-parent households), cognitive abilities, and emotional functioning were extracted from an existing database of participants who had previously enrolled in the IRB approved study of computerized working memory training. Cognitive measures included the Wechsler Intelligence Scale for Children (WISC-V) Full Scale IQ and attention, working memory, and executive functioning tests from Cogstate, a computerized cognitive assessment. Emotional functioning was measured using the Worry 1 (i.e., anxiety about SCD) and Emotions (i.e., frustration and anger about SCD) domains of both the child- and parent-rated Pediatric Quality of Life Inventory Sickle Cell Disease Module. Healthcare utilization was measured through chart review, separated into ED visits for pain and hospitalizations for pain 1 year and 3 years after enrollment. The mean age of participants was 10.61 (SD=2.91) year with majority being females (n=65; 58%). Eighty-three (74%) had sickle cell anemia (HbSS or HbSβ0 thalassemia). The median number of ED visits for pain was 1 visit (IQR=2) across 1 year after enrollment and 3 visits (IQR=6) across 3 years after enrollment. The median number of hospital admissions for pain was 0 admissions (IQR=1) across 1 year after enrollment and 1 admission (IQR=4) across 3 years after enrollment (pain hospitalizations = 1; IQR=4). Linear regression models were applied to explore whether cognitive and emotional variables were predictive of healthcare utilization for pain. Variables included in the final model were selected by using the Akaike information criterion. Results indicated that attentional abilities were significantly associated with ED visits and hospitalizations for pain, both at 1 year and 3 years after enrollment (p's<.009), such that better performance on the Cogstate attention task was associated with fewer ED visits and hospitalizations. Child-rated emotional quality of life significantly predicted ED visits for pain over 1 year (b=-0.004, p=.049) and hospitalizations for pain over 3 years (b=-0.006, p=.013), such that higher emotional quality of life was predictive of fewer ED visits and hospitalizations. Parent-rated child emotional quality of life was also predictive of hospitalizations for pain over 3 years (b=-0.007, p=.020). Neither SCD genotype nor socioeconomic status significantly impacted healthcare utilization. Results demonstrate that children's cognitive and emotional functioning play an important role in pain management and should be an integral part of comprehensive pain management plans for children with SCD. Attentional abilities emerged as a reliable predictor of pain-related healthcare utilization and may signal that poor attention makes it difficult to implement strategies to distract from pain. It could also suggest that children with poor attention have greater difficulty with behavioral aspects of disease self-management (e.g., medication adherence, avoiding pain triggers). These are important considerations, given that youth with SCD are at risk for disease-related neurological sequelae including reduced attentional abilities. Emotional functioning (specifically, frustration and anger about SCD) also predicted healthcare utilization for pain, highlighting the potential impact of stress on pain onset and management. Clinicians and researchers should consider cognitive and emotional factors when evaluating risk for pain in SCD and incorporate these factors when developing strategies to reduce healthcare utilization and costs. Disclosures Darbari: Hilton Publishing: Consultancy, Other: Expert advisory board for developing gaming app for SCD; Global blood therapeutics: Consultancy, Honoraria, Other: participated in advisory board meeting ; Novartis: Consultancy, Honoraria, Other: steering committee for SPARTAN study .

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Healthcare Utilization and the Quality of Life of Children and Adolescents with Sickle Cell Disease

10.22541/au.163795383.34560542/v1 ◽

2021 ◽

Author(s):

Kendall Moody

Keyword(s):

Quality Of Life ◽

Sickle Cell Disease ◽

Healthcare Utilization ◽

Sickle Cell ◽

Cell Disease ◽

Cross Sectional ◽

Patients Âgés ◽

Increased Risk ◽

Lower Hrqol

Introduction: Youth diagnosed with sickle cell disease (SCD) often utilize the healthcare setting to manage disease-related symptoms, yet the connection between healthcare use and their overall wellbeing has been understudied. This study investigates whether healthcare utilization predicts the health-related quality of life (HRQOL) in youth with SCD. It is hypothesized that increased healthcare utilization will predict lower HRQOL in pediatric SCD. Methods: A total of 150 patients, ages 8-17 years old, were enrolled in this cross-sectional quantitative study. Patients completed the Pediatric Quality of Life (PedsQL) 3.0 SCD module, while the researcher conducted a retrospective chart review to gather patient characteristics such as emergency room (ER) and hospitalization occurrences over the past 12 months. Results: A higher frequency of ER visits (p < .05) and hospitalizations (p < .01) predicted lower HRQOL scores. Age (p < .05) also emerged as a significant predictor for both regression models. Discussion: As youth with SCD require ER treatment and/or hospital admission, they are at increased risk for lower HRQOL, specifically as they get older.

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Biopsychosocial Predictors of Quality of Life in Paediatric Patients With Sickle Cell Disease

Frontiers in Psychology ◽

10.3389/fpsyg.2021.681137 ◽

2021 ◽

Vol 12 ◽

Author(s):

Anna M. Hood ◽

Melanie Kölbel ◽

Hanne Stotesbury ◽

Jamie Kawadler ◽

April Slee ◽

...

Keyword(s):

Quality Of Life ◽

Emergency Department ◽

Executive Function ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Pain Coping ◽

Cell Disease ◽

Paediatric Patients ◽

Ed Visits

Sickle cell disease (SCD) refers to a group of inherited blood disorders with considerable morbidity that causes severe pain, reduces life expectancy, and requires significant self-management. Acute painful episodes are the hallmark of SCD, but persistent daily pain is also highly prevalent in this population. Characterising the impact and experience of SCD-related morbidity (i.e., sleep disruption, frequent emergency department visits, cognitive dysfunction) on health-related quality of life (HRQOL) requires multiple assessment methods to best capture the underlying mechanisms. To gain a greater understanding of the effect of common symptom categories on HRQOL and to determine potential pain coping targets, the present study investigated whether demographic, socioeconomic, sleepiness, pain burden, frequency of emergency department (ED) visits, and cognition predicted HRQOL in a paediatric sample of patients with SCD. Our study was a secondary analysis of baseline assessment data of children with SCD aged 8–15 years (n = 30) in the Prevention of Morbidity in Sickle Cell Anaemia Phase 2b (POMSb2) randomised controlled clinical trial of auto-adjusting continuous positive airways pressure. Patients completed cognitive testing (IQ, Processing Speed Index, Delis-Kaplan Executive Function Scale (DKEFS) Tower, Conner's Continuous Performance Test), sleepiness (Epworth Sleepiness Scale), and HRQOL (PedsQL Sickle Cell Module) at baseline. Patients reported pain burden (Sickle Cell Pain Burden Inventory-Youth) each month over 8 visits. Caregivers provided demographic information and reported their child's executive function (Behavioural Rating Inventory of Executive Function) at baseline. Data from our analysis demonstrated that demographic factors (i.e., age, gender, level of neighbourhood deprivation) and treatment variables (i.e., hydroxyurea use) did not independently predict HRQOL, and laboratory values (i.e., haemoglobin, haematocrit, mean oxygen saturation) were not significantly correlated with HRQOL (ps > 0.05). However, sleepiness, pain burden, ED visits, and executive dysfunction independently predicted HRQOL (R2 = 0.66) with large effects (η2 = 0.16 to 0.32). These findings identify specific, measurable symptom categories that may serve as targets to improve HRQOL that are responsive to change. This knowledge will be useful for multimodal interventions for paediatric patients with SCD that include sleep management, pain coping strategies, and executive function training.

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Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease

10.1093/oso/9780195169850.001.0001 ◽

2006 ◽

Keyword(s):

Quality Of Life ◽

Sickle Cell Disease ◽

Childhood Cancer ◽

Children And Adolescents ◽

Peer Relationships ◽

Sickle Cell ◽

Cell Disease ◽

Life Issues ◽

Art Research

Over recent decades, tremendous advances in the prevention, medical treatment, and quality of life issues in children and adolescents surviving cancer have spawned a host of research on pediatric psychosocial oncology. This important volume fulfills the clear need for an up-to-date, comprehensive handbook for practitioners that delineates the most recent research in the field--the first of its kind in over a decade. Over 60 renowned authors have been assembled to provide a thorough presentation of the state-of-the art research and literature, with topics including: -Neuropsychological effects of chemotherapy and radiation therapy -Bone marrow transplantation -Important issues about quality of life during and following treatment -Collaborative research among child-focused psychologists -Standards of psychological care for children and adolescents -Stress and coping in the pediatric cancer experience -The role of family and peer relationships The Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease represents both multidisciplinary and international efforts, an alliance between physicians and parents, and a combination of research and service. With a wealth of information of great interest to patients and their families, this volume will also be a welcome resource to the psychologists, psychiatrists, pediatricians, oncologists, nurses, and social workers who confront these issues as they help children and their families through the treatment, recovery, and grieving processes.

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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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Chronic brain damage in sickle cell disease and its relation with quality of life

Medicina Clínica (English Edition) ◽

10.1016/j.medcle.2016.12.041 ◽

2016 ◽

Vol 147 (12) ◽

pp. 531-536

Author(s):

Elena Cela ◽

Ana G. Vélez ◽

Alejandra Aguado ◽

Gabriela Medín ◽

José M. Bellón ◽

...

Keyword(s):

Quality Of Life ◽

Sickle Cell Disease ◽

Brain Damage ◽

Sickle Cell ◽

Cell Disease

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Hip Dysfunction and Quality of Life in Patients With Sickle Cell Disease

Clinical Pediatrics ◽

10.1177/0009922815586051 ◽

2015 ◽

Vol 54 (14) ◽

pp. 1354-1358 ◽

Cited By ~ 7

Author(s):

Cristiane Dias Malheiros ◽

Luanne Lisle ◽

Marilda Castelar ◽

Katia Nunes Sá ◽

Marcos Almeida Matos

Keyword(s):

Quality Of Life ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Disease

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Anxiety, depressive disorders, and quality of life in adults with sickle cell disease

Middle East Current Psychiatry ◽

10.1097/01.xme.0000426319.48898.03 ◽

2013 ◽

Vol 20 (2) ◽

pp. 80-86 ◽

Cited By ~ 1

Author(s):

H. El-Shinnawy ◽

T. Goueli ◽

M. Nasreldin ◽

A. Meshref

Keyword(s):

Quality Of Life ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Depressive Disorders ◽

Cell Disease

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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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