Emerging Biobehavioral Factors of Fatigue in Sickle Cell Disease

Abstract

Purpose The symptom most frequently associated with sickle cell disease (SCD) is pain, but recent research is beginning to indicate that fatigue as an increasingly important symptom of this disease upon which to focus research efforts. This article explores biological and behavioral factors that can potentially contribute to fatigue in SCD. Organizing Framework A biobehavioral framework guides this discussion of factors that may contribute to SCD fatigue. Findings The pathophysiology of the disease process, such as the profound hemolytic anemia and unpredictable vasoocclusive crises, suggests that individuals with SCD are at risk for both acute and chronic fatigue. For example, hypoxemia can cause muscle weakness and produce oxidative stress, which, in turn, increases fatigue. Sickled erythrocytes disrupt the vascular endothelium and stimulate proinflammatory cytokines, which are linked to sleep disruptions. Pain, the most notorious symptom of SCD, has a complex and mechanistically poorly understood relationship with fatigue. Conclusions Little is known about the symptom of fatigue in SCD. Considering the biological and behavioral factors of SCD that could potentially contribute to fatigue, there is a great need for research on the nature and potential mechanisms of fatigue in SCD. Clinical Relevance Fatigue in SCD may negatively affect quality of life. Understanding factors that may contribute to fatigue aids the clinician in identifying causes and determining treatment.

Understanding the meaning of fatigue at the end of life: An ethnoscience approach

“…Our findings resonate to a certain extent with findings of others (Piper, Lindsey, Dodd, 1987;Richardson, 1995;Krupp and Christidoulou, 2001;Kralik, Telford, Price et al 2005;Ameringer and Smith, 2011), however none of these studies offered relational dimensions between the themes or segregates nor mention tiredness or exhaustion as part of the conceptualisation. The ethnoscience approach was instrumental to advancing the conceptualisation of the progressive continuum of fatigue through utilising taxonomical displays where relationships between different levels (domains, segregates, sub-segregates) were featured.…”

“…Kralik and colleagues (2005) have described fatigue as a 'multi-dimensional and complex phenomenon that affects physical, cognitive, emotional and social functioning' (p378), which concurs with the some literature (Piper, Lindsey, Dodd, 1987;Richardson, 1995;Krupp and Christidoulou, 2001;Ameringer and Smith, 2011). A more recent approach to the management of fatigue in cancer care has been to view and attempt to treat multiple symptoms simultaneously as a cluster (Dodd, Miaskowski and Paul 2001;Barsevick 2007).…”

The Effectiveness of Self Management Program on Pain, Fatigue, Depression, Anxiety, and Stress in Sickle Cell Patients: A Quasi-Experimental Study

“…Patients with SCD experience multiple disease-related complications; the physical, psychological, and social effects of these expose these patients to an evaluated risk of acute and chronic fatigue (12), as well as depression, anxiety, stress, and other mental health problems (3,6). This study's findings indicate that fatigue is significantly related to depression, anxiety, and stress; that pain frequency during the study period was significantly related to depression, anxiety, and stress; and that duration of pain was significantly related to depression, anxiety, and fatigue.…”

“…Extant research indicates a relationship between pain, fatigue, depression, anxiety, and stress in SCD patients (12). Severe pain in SC patients has been associated with reduced vitality, increased fatigue (11,13) and increased prevalence of depression, anxiety, and stress (3,21,22).…”

“…In managing serious and unpredictable complications, patients with SCD suffer numerous psychosocial problems such as reduced self-esteem, feelings of frustration (7), depression (7)(8)(9), anxiety and stress (8), and disruption of interpersonal relationships (10); these often lead to fatigue (11)(12)(13)(14) and poor quality of life (13,(15)(16)(17) in these patients. Bakri et al (10) showed that depression, anxiety and stress are prevalent in SC patients compared with healthy subjects.…”