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

Sue Height,Christina Liossi,Jo Howard,Melanie Kölbel,April Slee,Fenella J Kirkham,Jamie Kawadler,Moji Awogbade,Baba Inusa,Anna M Hood,Subarna Chakravorty,Maria Pelidis,Hanne Stotesbury

doi:10.3389/fpsyg.2021.681137

Abstract

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.

Highlights

Sickle cell disease (SCD) is a group of genetic disorders that affect the structure and oxygen-carrying capacity of haemoglobin in red blood cells (Rees et al, 2010)
Disease Phase 2b (POMS2b) trial (Howard et al, 2018), we aimed to determine whether demographic, socioeconomic, sleep, pain burden, emergency department visits, and cognition predicted health-related quality of life (HRQOL) in patients with SCD
Impulsivity was negatively correlated with HRQOL (r = −0.33), it was not a predictor within regression models

Summary

Introduction

Sickle cell disease (SCD) is a group of genetic disorders that affect the structure and oxygen-carrying capacity of haemoglobin in red blood cells (Rees et al, 2010). Patients with SCD experience recurrent acute painful episodes with persistent pain between episodes (Dampier et al, 2002; Smith et al, 2008). The occurrence, location, severity, and duration of pain episodes and persistent pain can vary substantially in an individual patient tending to worsen with age (Ballas, 2020). These types of pain can sometimes have precipitating factors or objective signs specific to the individual patient (e.g., infection, fever, obstructive sleep apnoea, dehydration) and changes in the environment associated with increased admissions for pain in the population (e.g., increased wind speed, rainfall) (Piel et al, 2017). Patients with SCD generally experience poorer quality of life than national norms and other patients with chronic conditions (i.e., cystic fibrosis, asthma, haemodialysis patients) with worsening quality of life as pain increases (McClish et al, 2005)

Objectives

Methods

Results

Conclusion