Sickle cell anemia (SCA) is an inherited blood disorder characterized by intermittent vaso-occlusion. Recurrent vaso-occlusive pain episodes (VOEs) are the hallmark feature of SCA and the leading source of morbidity in patients. Recurrent and severe VOEs are associated with many detrimental consequences for children, including frequent health care contacts, frequent school absences, academic attainment problems, reduced opportunity for physical recreation and social interactions, and higher levels of depression and anxiety. A distinct obstacle to managing pain in SCA is the great heterogeneity of VOEs experienced by patients, including the frequency, duration, intensity, and extent of disability from VOEs. Hematological factors, such as hematocrit and fetal hemoglobin, have been shown to predict the frequency of VOEs;however, it is likely that many additional factors, including underlying genetic variability in patients, contribute to the manifestation of severe VOEs. Modern theoretical models of pain in SCA also suggest that, in addition to biological predisposition, psychological and social factors may play a prominent role in understanding individual variation in VOEs, yet few studies have examined the manner in which biological and psychosocial variables interact to produce severe pain. This research employs an interdisciplinary, translational approach that examines biopsychosocial factors and their relation to VOEs in children with SCA. Combining the areas of molecular genetics, neuroscience, psychology, and pediatric hematology, this study aims (a) to understand the role of genetic variants in vaso- regulatory factors in predicting VOE severity and (b) to examine the relationships of biological and psychological factors in the prediction of VOE severity. Specifically, two SNPs (rs5333 and rs5351) for genes coding for components of the endothelin system, a vaso-regulatory system, will be examined in relation to VOE severity. Additionally, using a biopsychosocial approach, the relationships between specific biological (hematocrit, fetal hemoglobin) and child psychological factors (coping and mood) will be examined. Social factors, including family functioning, caregiver coping, and caregiver mood, will also be measured and described in terms of their relationship to the development of responses to pain in children. The goals of this research are (a) to identify predictors of VOEs in pediatric SCA and (b) to develop a model that informs integrated medical and psychosocial approaches to pain management in this condition.

Public Health Relevance

Recurrent, debilitating pain is the hallmark symptom of sickle cell anemia, an inherited blood disorder. The ability of clinicians to predict which children will encounter severe pain is currently limited, and it is likely that a combination of biological, psychological, and social factors contribute to the severity of pain in this condition. Using an interdisciplinary approach, this research aims to assist in the identification of children at risk or severe pain and to inform integrated medical and psychosocial approaches to pain management.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Predoctoral Individual National Research Service Award (F31)
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Special Emphasis Panel (ZRG1-F10A-S (20))
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Chang, Henry
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University of South Carolina at Columbia
Schools of Arts and Sciences
United States
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Schlenz, Alyssa M; Schatz, Jeffrey; Roberts, Carla W (2016) Examining Biopsychosocial Factors in Relation to Multiple Pain Features in Pediatric Sickle Cell Disease. J Pediatr Psychol 41:930-40
Schatz, Jeffrey; Schlenz, Alyssa M; McClellan, Catherine B et al. (2015) Changes in coping, pain, and activity after cognitive-behavioral training: a randomized clinical trial for pediatric sickle cell disease using smartphones. Clin J Pain 31:536-47
Smith, Terika P; Schlenz, Alyssa M; Schatz, Jeffrey C et al. (2015) Modulation of pain in pediatric sickle cell disease: understanding the balance between endothelin mediated vasoconstriction and apelin mediated vasodilation. Blood Cells Mol Dis 54:155-9