INTRODUCTION
Sickle cell disease (SCD) is a genetic, chronic blood disorder that is
characterized by the functioning of red blood cells (RBC). Normal RBCs
are circular in shape, which allows the cell to carry out tasked such as
tissue oxygenation, amongst other vital responsibilities (Conran &
Embury, 2021). Sickled cells, however, take on a C-shape, which leads to
a blockage of the capillaries causing inflammation and severe pain
crises (Knisely et al., 2020). In addition to pain, individuals with SCD
are at risk for chronic organ damage, anemia, and early death (Wallen et
al., 2014).
Pain is often considered the hallmark symptom associated with SCD;
therefore, researchers have attempted to understand the pain experience
among individuals within this population. The Pain in Sickle Cell
Epidemiology Study (PiSCES) highlights this experience and reports that
patients will typically manage their pain at home to avoid going to a
busy ER (Smith et al., 2005). While the pain experience, which has been
shown to increase with age, has been linked to mental health challenges
such as depression and anxiety (Valrie et al., 2020), it is also the
most common reason patients access the hospital (Kanter et al., 2019).
Healthcare utilization among individuals with SCD is increasing along
with the associated costs. One study found the annual cost of managing
individual with SCD ranged from $18,859 for ages 0 – 9 to $43,586 for
ages 20 – 29 annually (Salcedo et al., 2019). In a different study, SCD
accounted for one of the highest rates for readmission among chronic
illnesses and accounted for approximately $2.4 billion dollars annually
(Crego et al., 2020). While the economic burden associated with SCD is
both an individual and national concern, high healthcare utilization
also has psychosocial implications. Kidwell et al. (2021), for example,
found depression and anxiety scores were higher among individuals who
accessed the ED and were admitted to the hospital more frequent. How
patients cope with the many stressors associated with SCD is vital, as
it may determine their overall wellbeing.
Health-related quality of life (HRQOL) is a self-assessment of a
patient’s overall health as it relates to their disease status
(Theofilou, 2013). The literature has been consistent in reporting that
children diagnosed with SCD have poorer HRQOL even when compared with
children without SCD (Menezes et al., 2013; Wrotniak et al., 2014).
However, it is difficult to generalize these findings for two primary
reasons. First, researchers in this field have used various measurement
tools to assess HRQOL within this population. Also, studies have
neglected using a disease-specific measurement tool when assessing
HRQOL. Those interested in this type of research may find it beneficial
to use a disease-specific measurement tool that consist of domains
specifically related to SCD. Secondly, many research studies have
omitted using a theoretical model to guide their study and/or
substantiate their findings. One may find it useful to include a
theoretical model to better understand the connection between youth with
SCD and HRQOL.
One such theoretical model that illustrates the connection between
healthcare utilization and the HRQOL of youth with SCD is the illness
intrusiveness theory. Developed by Devins et al. (1990), the illness
intrusiveness theory posits disease, treatment, psychological, social,
and contextual factors cause illness intrusiveness, which affects the
wellbeing of individuals with chronic illness
(Devins, 2006). Patients who
perceive their illness to be intrusive on their daily functions and
activities they enjoy report lower HRQOL (Devins et al., 2000), even
within children diagnosed with a chronic illness (Fedele et al., 2012).
As children with SCD continue to access the emergency room and/or
require admission to the hospital, they are unable to engage in positive
experiences, which are vital during child and adolescent years (Devins
et al., 1993). Prior research has used illness intrusiveness as a
theoretical framework within the African American population (Bioku et
al., 2020; Hughes et al., 2014), which underscores the value in using
this theory for the current study. Because symptoms such as pain often
leads to emergency room visits and hospital admission, it may be likely
that the way individuals adjust and are able to decrease the need to
access the hospital will aid in improving their overall HRQOL. To
discover if there is a connection between healthcare use and the HRQOL
of youth with SCD, this study will use the Pediatric Quality of Life
Scale (PedsQL) 3.0 SCD module to build on the literature. There are two
identified aims: (1) to investigate whether youth diagnosed with SCD
experience low HRQOL using the PedsQL 3.0 and (2) analyze the
association between healthcare use and the HRQOL of children and
adolescents diagnosed with SCD while also controlling for specific
sociodemographic variables. It is hypothesized that there will be an
impairment in HRQOL and there will be a correlation between healthcare
utilization and the HRQOL of youth with SCD. Findings from this study
may aid healthcare professionals in incorporating mental and emotional
support in their treatment of children with SCD.