Sleep and fatigue among youth with sickle cell disease: A daily diary study.

Youth with sickle cell disease (SCD) experience disease effects including vaso-occlusive pain crises, poor sleep quality, and fatigue. The present study examines how sleep quality and pain medications impact fatigue in youth with SCD. Daily diaries assessing pain, fatigue, sleep quality, mood, and use of pain medications from 25 youth with SCD ages 11 to 18 years were collected for eight consecutive weeks.

A Randomized Controlled Trial of Working Memory Training in Pediatric Sickle Cell Disease.

Objective: Youth with sickle cell disease (SCD) are at risk for neurocognitive deficits including problems with working memory (WM), but few interventions to improve functioning exist. This study sought to determine the feasibility and efficacy of home-based, digital WM training on short-term memory and WM, behavioral outcomes, and academic fluency using a parallel group randomized controlled trial design.

Methods: 47 children (7-16 years) with SCD and short-term memory or WM difficulties were randomized to Cogmed Working Memory Training at home on a tablet device (N = 24) or to a standard care Waitlist group (N = 23) that used Cogmed after the waiting period.

Feasibility of Home-Based Computerized Working Memory Training With Children and Adolescents With Sickle Cell Disease.

Background: Children with sickle cell disease (SCD) are at increased risk for neurocognitive deficits, yet the literature describing interventions to ameliorate these problems and promote academic achievement is limited. We evaluated the feasibility and preliminary efficacy of a home-based computerized working memory (WM) training intervention (Cogmed) in children with SCD.

Procedure: Youth with SCD between the age of 7 and 16 years completed an initial neuropsychological assessment; those with WM deficits were loaned an iPad on which they accessed Cogmed at home.

Association between somatic growth trajectory and cognitive functioning in young children with sickle cell disease.

Children with sickle cell disease are at risk of cognitive deficits and somatic growth delays beginning in early childhood. We examined growth velocity from age 2 years (height and body mass index progression over time) and cognitive functioning in 46 children with sickle cell disease 4 to 8 years of age. Height-for-age velocity was not associated with cognitive outcomes.

Modulation of pain in pediatric sickle cell disease: understanding the balance between endothelin mediated vasoconstriction and apelin mediated vasodilation.

Children with sickle cell disease (SCD) have painful vaso-occlusive episodes (VOEs), which often reoccur across the individual's lifespan. Vaso-constrictive and vaso-dilatory molecules have been hypothesized to play a role in VOEs. Endothelin-1 (ET-1) is a potent vasoconstrictor that is released during VOEs and is correlated with pain history.

Sensitization to acute procedural pain in pediatric sickle cell disease: modulation by painful vaso-occlusive episodes, age, and endothelin-1.

Unlabelled: The impact of pain early in life is a salient issue for sickle cell disease (SCD), a genetic condition characterized by painful vaso-occlusive episodes (VOEs) that can begin in the first year of life and persist into adulthood. This study examined the effects of age and pain history (age of onset and frequency of recent VOEs) on acute procedural pain in children with SCD. Endothelin-1, a vaso-active peptide released during VOEs and acute tissue injury, and its precursor, Big Endothelin, were explored as markers of pain sensitization and vaso-occlusion.

Relationships between somatic growth and cognitive functioning in young children with sickle cell disease.

Objective: Children with sickle cell disease (SCD) exhibit poor somatic growth due to nutritional and metabolic effects, but potential relationships between growth and other areas of development are unclear. We examined whether growth is related to cognition and whether growth might be one marker of neurocognitive risk.

Methods: Sixty-four children with SCD and eighty-one demographically similar controls, ages 4 to 8 years, completed cognitive and anthropometric measures.

Criterion and convergent validity for 4 measures of pain in a pediatric sickle cell disease population.

Objective: To evaluate the psychometric properties of 4 measures of acute pain in youth with sickle cell disease (SCD) during a medical procedure.

Methods: Heart rate, child self-report, parent proxy-report, and observable pain behaviors were examined in 48 youth with SCD ages 2 to 17 years. Criterion validity for acute pain was assessed by responsiveness to a standardized painful stimulus (venipuncture) in a prospective pre-post design.

Use of handheld wireless technology for a home-based sickle cell pain management protocol.

Purpose: To evaluate use of a handheld electronic wireless device to implement a pain management protocol for participants with sickle cell disease (SCD).

Methods: Participants were 19 patients with SCD aged 9-20 who experienced vaso-occlusive pain. A single-session training on the use of cognitive-behavioral coping skills was followed by instruction on how to practice these skills and monitor daily pain experience using the device.