Relationship between Adherence Behaviors and Health-related Quality of Life in Youth with Sickle Cell Disease
Authors:   , Ding Ke , Daly Brian P. 
Published: July 2021

Background: Sickle cell disease (SCD) is the most common inherited blood disorder in the United States (Center of Disease Control and Prevention, 2016). There has been an increase in the life expectancy of individuals with SCD. However, individuals still experience a variety of serious medical complications and are at risk for developmental delays, cognitive difficulties, and/or academic deficits (e.g., Hogan et al., 2006; Thompson et al, 2002). SCD management plans vary in intensity and invasiveness (Barakat et al., 2006). Despite the serious consequences, adherence to SCD management is highly variable. Given that SCD is a chronic illness with a complex treatment regimen and serious complications, it is important to assess the impact of the illness on individuals' health related quality of life (HRQoL). Research has found that children with SCD experience poorer HRQoL compared to healthy peers (Dale et al., 2011). However, there is a lack of conclusive findings on the relationship between adherence and HRQoL among children with SCD. Additionally, limited studies have explored the potential moderation effects of health-related variables on the relationship between adherence and patient's HRQoL in an SCD population (e.g., Fisak et al., 2010). Aims: The first aim of the current study was to examine the relationship between caregiver-report on adherence and child HRQoL in a school-age population with SCD. It was hypothesized that endorsement of more adherence would be positively related to child HRQoL. The second aim explored the potential moderation effect of cognitive functioning, academic functioning, and academic accommodations on the previous relationship, respectively. It was hypothesized there would be a stronger relationship between adherence and HRQoL for individuals who had a higher level of cognitive functioning as compared to individuals who had a lower level. Similarly, it was predicted that there would be a stronger relationship between adherence and HRQoL for individuals who had higher levels of academic functioning as compared to individuals who had lower levels. Lastly, it was predicted that there would be a stronger relationship between adherence and HRQoL for individuals who received fewer academic accommodations comparing to individuals who received more accommodations. Methods: The current study utilized baseline data from a completed randomized controlled trial that examined the efficacy of a problem-solving intervention for families with a school-age child with SCD. Children and caregivers completed questionnaires assessing child HRQoL. Children were administered a performance-based measure of cognitive and academic functioning. Caregivers completed measures assessing child adherence. Results: The current study found mixed results on the relationship between adherence and HRQoL. From caregivers' perspective, better adherence was associated with better HRQoL (r= .34, p= .002). No relationship was revealed based on child self-report (r= -.01, p= .929). No significant moderation effects were found. However, academic functioning was positively associated with caregiver proxy-report on child HRQoL. Academic needs were negatively associated with caregiver proxy-report on child HRQoL, suggesting that increasing number of needed accommodations was associated with lower HRQoL. Conclusions: Child and caregiver informants reported different perspectives on child HRQoL and might assess child HRQoL differently as the child ages. Without strong communication between children and caregivers, caregivers may have a different understanding of their children's overall HRQoL. This highlights the importance for psychologists and health care workers of integrating different perspectives when discussing HRQoL. Adherence was only found to be significantly correlated with caregiver proxy-report on child HRQoL. Future research should further explore this relationship using different informants and continue to explore the potential influence of cognitive and academic functioning on adherence and HRQoL within a SCD population. Additional data may help us better understand how to improve children's HRQoL and develop strategies for early detection and intervention.