Adolescents’ experiences of living with sickle cell disease: An integrative narrative review of the literature

Abstract

Sickle cell disease impacts on multiple facets of an adolescent's life. While there are similarities in the experience of living with sickle cell disease and living with other chronic illnesses, there are essential differences in relationship dynamics and healthcare experience. The adolescents expressed less confidence in generic healthcare providers. The review highlights areas relating to symptom management and health service provision that has been under-researched and need further exploration to understand adolescents' experiences and their support needs fully. Nursing care and research should focus more on adolescents' developmental wellbeing, promote peer support network among adolescents with the condition and with adolescents with other chronic illnesses and collaborate with adolescents to ensure service development are developmentally and culturally appropriate.

“Body as a Machine”: How Adolescents With Sickle Cell Disease Construct Their Fatigue Experiences

“…Thus, in young people SCD, social isolation and withdrawal may not necessarily indicate emotional or mental health problems. This might explain the discrepancy between the reported higher prevalence of depressive and anxiety symptoms when using standardized measurement tools, and the clinically insignificant prevalence rate from detailed psychiatric assessment (Poku et al, 2018).…”

“…This is contrary to SCD-related pain, which being time-bound and temporal may not permanently affect self and identity. Therefore, fatigue might be the feature of SCD that has the most profound salience to adolescents' social and self-identity development, possibly playing a significant role in the reported poor selfconcept reported in adolescents with SCD (Poku et al, 2018).…”

“…According to them, adults with major depressive disorder withdraw socially when they are not able to "hold up the mask" of concealing their fatigue from others. Indeed, while social isolation and withdrawal has been commonly identified in the literature about the experiences of adolescents with SCD and adults with depression as a direct negative consequence of the conditions (Poku et al, 2018;Porr et al, 2010), it is a strategy purposively used by adolescents with SCD and adults with major depression disorder to manage fatigue and it seems to be successful in helping them to renegotiate social repsonsibilities in order to preserve/restore energy and maintain positive social identities. Thus, in young people SCD, social isolation and withdrawal may not necessarily indicate emotional or mental health problems.…”

“…SCD is characterized by life-limiting and life-threatening complications such as chronic anemia, acute and severe pains, delayed growth/sexual development, increased vulnerability to severe infections and stroke, leading to significant morbidity, and psychosocial and economic impacts (Mulumba & Wilson, 2015). A recent integrative review of adolescents' experiences of living with SCD highlighted how SCD impacts negatively on adolescents' physical functioning, social relationships, emotional well-being, self-concept, social identity, and schooling (Poku et al, 2018). Among the many SCDrelated symptoms, pain was the most explored.…”

“…Although the distinctive chronic hemolytic anemia, inflammation, and pain that characterize SCD suggest that individuals with the condition are at risk of experiencing substantial fatigue, fatigue is under-explored and not prioritized in research and clinical care compared with SCDrelated pain (Poku et al, 2018). This lack of attention may be due to the nihilistic perceptions that fatigue is an inevitable consequence of the condition, about which nothing can be done and therefore must be endured by those living with SCD (While & Mullen, 2004).…”