DISPLACE Study Shows Poor Quality of Transcranial Doppler Ultrasound for Stroke Risk Screening in Sickle Cell Anemia.

Children with sickle cell anemia (SCA) are at increased risk of stroke when compared to age-based counterparts. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) previously demonstrated that with the use of transcranial Doppler ultrasound (TCD; Sickle Stroke Screen) and chronic red cell transfusion, the risk of stroke risk is reduced by over 90%. The STOP criteria detailed the type and method of measurement required; the time averaged mean maximum velocity (TAMMV). Unfortunately, it has been difficult to adhere to the appropriate TAMMV measurements. The objectives of this study were to assess the quality of TCD and transcranial Doppler imaging (TCDi) reports to determine report quality and accuracy. This is a sub-analysis of the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study. Over 12,000 TCD/TCDi reports were collected during this study from 28 institutions; 391 TCDs were reviewed for this sub-analysis. There was significant variation in which vessels were assessed, the velocities used to define abnormal results, and who was interpreting the scans. In 52% of reports, it was impossible to identify whether the TAMMV was what was measured. Similarly, it was only clear in 42% of reports that the TAMMV was used to interpret the exam as normal/abnormal. Given this inconsistency, we strongly recommend standardization of TCD/TCDi reporting, specialized training for those performing and interpreting the scans in the use of TCD/TCDi in patients with SCA, internal quality assurance, and institutional quality improvement work to ensure appropriate use of this potentially lifesaving technology.

Practice Patterns for Addressing Developmental-Behavioral Concerns in Sickle Cell Specialty Care.

Objective: Children with sickle cell disease (SCD) are at elevated risk for neurodevelopmental and behavioral disorders. This report describes developmental-behavioral practice patterns among sites who were part of the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) consortium in the context of current guidelines for addressing these concerns. Methods: An internal survey was developed for the Principal Investigators of the DISPLACE study to identify developmental-behavioral clinical practices across the 28-site consortium, including methods for identification, referral practices, access to psychologists, and barriers to services. Descriptive data were pulled from the survey to describe practice patterns. Results: Most sites used informal methods to detect developmental-behavioral concerns, though over 1/3 of sites were using a structured protocol. The most common referral indications for further developmental and neuropsychological evaluation were parent, provider, or school concerns or stroke. Evaluations were predominantly completed by pediatric neuropsychologists and pediatric psychologists. Despite most sites reporting access to a psychologist within the SCD clinic, sites also reported long waitlists and difficulty accessing providers for evaluation and treatment services. Insurance difficulties were also a common barrier. A range of additional barriers were reported at the patient, provider, organizational, and policy/socio-environmental levels. Conclusions: Many sites in the DISPLACE consortium were adhering to existing care guidelines for pediatric SCD; however, there was also wide variation in practices for which guidelines are absent or unclear. Additional work is needed to inform guidelines, to specify the role of psychology within specialty SCD care, and to overcome barriers to care. Implications for Impact Statement: Optimal developmental-behavioral care for children with SCD involves collaboration among specialty care providers and pediatric psychologists; however, these efforts may be hampered by dispersed or unclear guidelines and several barriers to care. Future studies and guidelines have the potential to improve care by providing clear, consistent, and unified care recommendations specific to psychologists and related care professionals who are supporting children with SCD.

Identifying patterns of neurocognitive dysfunction through direct comparison of children with leukemia, central nervous system tumors, and sickle cell disease.

PURPOSE: To quantify and compare the magnitude and type of neurocognitive dysfunction in at-risk children with central nervous system (CNS) tumors, acute lymphoblastic leukemia (ALL), and sickle cell disease (SCD) using a common instrument and metric to directly compare these groups with each other. METHODS: Fifty-three participants between the ages of 7 and 12 years (n = 27 ALL, n = 11 CNS tumor, n = 15 SCD) were enrolled and assessed using the NIH Toolbox Cognition Battery (NIHTCB). Participants with ALL or CNS tumor were 0-18 months posttherapy, while participants with SCD possessed the SS or Sß0 genotype, took hydroxyurea, and had no known history of stroke. RESULTS: Independent sample t-tests showed that participants with ALL and CNS tumor experienced greatest deficits in processing speed (ALL d = -0.96; CNS tumor d = -1.2) and inhibitory control and attention (ALL d = -0.53; CNS tumor d = -0.97) when compared with NIHTCB normative data. Participants with SCD experienced deficits in cognitive flexibility only (d = -0.53). Episodic memory was relatively spared in all groups (d = -0.03 to -0.32). There were no significant differences in function when groups were compared directly with each other by analysis of variance. CONCLUSIONS: Use of a common metric to quantify the magnitude and type of neurocognitive dysfunction across at-risk groups of participants by disease shows that participants perform below age-expected norms in multiple domains and experience dysfunction differently than one another. This approach highlights patterns of dysfunction that can inform disease- and domain-specific interventions.

Longitudinal Predictors of Pain in Pediatric Sickle Cell Disease.

OBJECTIVE: Despite the identified pathophysiology of vaso-occlusive pain in sickle cell disease (SCD), predictors of pain in youth with SCD remain elusive. In this study, we measured changes in pain frequency, intensity, and interference over 1 year and examined biopsychosocial risk factors (SCD disease severity, age, female, depression, and sleep quality) as possible longitudinal predictors. METHODS: Medical history was obtained from retrospective chart review for 79 children with SCD (ages 2-18 years; 48.1% female; 100% Black/African American; 83.5% SCD, SS genotype). As part of a clinical screening protocol, caregivers (n = 79) and youth 8-18 years (n = 43) completed psychosocial questionnaires approximately 1 year apart (M = 15.52 months, SD = 5.69). Zero-order correlations, paired t-tests, and hierarchical linear models examined longitudinal predictors of pain. The longitudinal bidirectional relationship between pain and sleep was also examined. RESULTS: The rate of severe SCD disease increased from 41.8% to 55.7% across the year, while most hematologic medical parameters remained stable. Increased depression and pain interference at survey 1 significantly predicted increased pain interference at survey 2. Poor sleep quality and increased pain frequency at survey 1 predicted increased pain frequency at survey 2. Finally, increased pain interference at survey 1 predicted poor sleep quality at survey 2. DISCUSSION: History of pain, depression, and sleep quality were longitudinal predictors of pain over 1 year in youth with SCD. Identifying longitudinal predictors of pain may lead to earlier identification of patients with a high-risk SCD pain phenotype and earlier medical, psychological, and behavioral interventions.

Patient and Family Opioid Decision-Making for Pain Management in Sickle Cell Disease: A Qualitative Study.

Pain is a significant symptom experienced frequently by individuals with sickle cell disease (SCD). Pain management includes strategies such as oral rehydration, non-pharmacological therapies (eg, massage, relaxation), and oral analgesics and opioids. Shared decision-making around pain management is emphasized repeatedly in recent guidelines; however, research is sparse regarding factors to be considered in shared decision-making approaches including the perceived risks and benefits of opioids. This qualitative descriptive study was conducted to explore perspectives of decision-making for opioid medications in SCD. We conducted 20 in-depth interviews with caregivers of children with SCD and individuals with SCD to elucidate the decision-making processes around home use of opioid therapy for pain management at a single center. Themes were identified in the domains of the Decision Problem (Alternatives and Choices; Outcomes and Consequences; Complexity), the Context (Multilevel Stressors and Supports; Information; Patient-Provider Interactions), and the Patient (Decision-Making Approaches; Developmental Status; Personal and Life Values; Psychological State). Key findings indicated opioid management for pain in SCD is important yet complex and requires collaboration among patients, families, and providers. Elements of patient and caregiver decision-making identified in this study may be applied to shared decision-making strategies in the clinical setting and future study. PERSPECTIVE: This study illustrates the factors involved in decision-making around home opioid use for pain management in children and young adults with SCD. These findings can be applied to determining shared decision-making approaches around pain management between providers and patients, in accordance with recent SCD pain management guidelines.

Sleep quality and pain in adolescents and young adults with sickle cell disease.

STUDY OBJECTIVES: This study evaluated sleep quality in relation to pain and pain-related impairment in adolescents and young adults with sickle cell disease. The purpose was to examine whether increased age was associated with poorer sleep quality and pain and to examine the sleep quality-pain association in this age group. METHODS: Eighty-nine adolescents and young adults with sickle cell disease between the ages of 13 and 25 completed ratings of sleep quality, overall pain, and 2 measures of pain-related impairment (pain impact and pain burden) as part of their clinical care. Retrospective chart reviews were completed to match ratings to demographic and medical characteristics. Correlations and multiple regression were used to examine associations between age, sleep quality, and pain variables, including an exploratory analysis of the sleep-pain association by age. RESULTS: Increased age was associated with poorer sleep quality, worse overall pain, and higher pain burden. Poorer sleep quality was also associated with worse overall pain and pain burden. Using multiple regression, a small, but not statistically significant trend was observed for the interaction of increased age and strengthening of the sleep quality-pain burden association. CONCLUSIONS: Sleep quality and pain are important challenges for adolescents and young adults with sickle cell disease that may persist or worsen with increased age. Early identification of these difficulties in pediatric populations as well as continued screening and intervention as adolescents transition into adult care is important. Additional longitudinal research is needed to better understand the progression of the sleep-pain relationship over time. CITATION: Schlenz AM, Thomas SJ, Gloston G, Lebensburger J, Maxwell SL, Kanter J. Sleep quality and pain in adolescents and young adults with sickle cell disease. J Clin Sleep Med. 2022;18(12):2845-2853.

Barriers and Facilitators to Chronic Red Cell Transfusion Therapy in Pediatric Sickle Cell Anemia.

Background: Chronic red cell transfusion (CRCT) therapy is one of a few effective disease-modifying therapies for children with sickle cell anemia (SCA). CRCT is recommended for primary and secondary stroke prevention for at-risk children with SCA and is sometimes used for other disease-related complications. However, CRCT can be resource- and time-intensive for patients/families, providers, and organizations. This study was conducted to provide a comprehensive, multilevel examination of barriers and facilitators to transfusion therapy in children with SCA from health care provider and caregiver perspectives. Methods: A qualitative descriptive approach was used to conduct key informant interviews in a sample of 26 caregivers and 25 providers across the United States. Interviews were analyzed using directed content analysis with the Multilevel Ecological Model of Health as an initial coding framework and the constant comparison method. Results: Ten barrier themes and 10 facilitator themes emerged across all ecological levels. Themes most commonly occurred on the patient and organizational levels. Key barriers themes included Logistical Challenges, Obtaining and Maintaining Venous Access, Alloantibodies/Alloimmunization and Reactions, and Iron Overload and Adherence to Chelation Therapy. Key facilitator themes included Nursing and Non-nursing Staff Support, Positive Child/Family Experiences, Logistical Help and Social Resources, Blood Bank and Access to Blood, and Transfusion-Specific Resources. Discussion: The comprehensive understanding of multilevel barriers and facilitators to transfusion therapy, including the role of nursing, in children with SCA can inform strategies to improve CRCT for patients/families and providers and can also be applied by organizations seeking to implement transfusion services for SCA.

Identified barriers and facilitators to stroke risk screening in children with sickle cell anemia: results from the DISPLACE consortium.

BACKGROUND: Children with sickle cell anemia are at risk for stroke. Ischemic stroke risk can be identified among children ages 2-16 years with sickle cell anemia using transcranial Doppler ultrasound. Despite strong recommendations for transcranial Doppler screening in guidelines released by the National Heart, Lung, and Blood Institute, implementation of transcranial Doppler screening in sickle cell anemia remains suboptimal. The purpose of this study was to identify barriers and facilitators to transcranial Doppler screening in a large national consortium to inform subsequent implementation interventions. METHODS: A qualitative descriptive approach was used to conduct 52 semi-structured interviews with a sample of patients with sickle cell anemia, their parents or primary caregivers, and healthcare providers dispersed across the United States. Interviews took place from September 2018 through March 2019. Directed content analysis was conducted with an adapted version of the Multilevel Ecological Model of Health as an initial coding framework, completed July 2019. Frequency analysis was conducted to determine predominant barrier and facilitator themes. RESULTS: Fourteen barrier themes and 12 facilitator themes emerged representing all levels of the ecological framework. Two barrier themes (Logistical Difficulties and Competing Life Demands and Gaps in Scheduling and Coordination), and 5 facilitator themes (Coordination, Scheduling and Reminders; Education and Information; Provider and Staff Investment and Assistance; Positive Patient Experience; and Convenient Location) were predominant. CONCLUSIONS: Barriers and facilitators to transcranial Doppler screening in children with sickle cell anemia are complex and occur across multiple ecological levels. One barrier theme and 3 facilitator themes were found to be optimal to address in subsequent implementation interventions.

Transcranial Doppler Screening in a Current Cohort of Children With Sickle Cell Anemia: Results From the DISPLACE Study.

Stroke prevention guidelines for sickle cell anemia (SCA) recommend transcranial Doppler (TCD) screening to identify children at stroke risk; however, TCD screening implementation remains poor. This report describes results from Part 1 of the 28-site DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study, a baseline assessment of TCD implementation rates. This report describes TCD implementation by consortium site characteristics; characteristics of TCDs completed; and TCD results based on age. The cohort included 5247 children with SCA, of whom 5116 were eligible for TCD implementation assessment for at least 1 study year. The majority of children were African American or Black, non-Hispanic and received Medicaid. Mean age at first recorded TCD was 5.9 and 10.5 years at study end. Observed TCD screening rates were unsatisfactory across geographic regions (mean 49.9%; range: 30.9% to 74.7%) independent of size, institution type, or previous stroke prevention trial participation. The abnormal TCD rate was 2.9%, with a median age of 6.3 years for first abnormal TCD result. Findings highlight real-world TCD screening practices and results from the largest SCA cohort to date. Data informed the part 3 implementation study for improving stroke screening and findings may inform clinical practice improvements.

Changes in Care Delivery for Children With Sickle Cell Anemia During the COVID-19 Pandemic.

BACKGROUND: Specialty care for children with sickle cell disease (SCD) may be disrupted during the coronavirus (COVID-19) pandemic. Our DISPLACE consortium includes 28 pediatric SCD centers. METHODS: In May 2020, we surveyed the consortium on the impact of COVID-19 on their practice focusing on transcranial Doppler ultrasound, chronic red cell transfusions, telehealth, and COVID-19 testing. OBSERVATION: Twenty-four DISPLACE providers completed the survey. Transcranial Doppler ultrasound screening decreased to 67% but chronic red cell transfusions remained at 96%. Most investigators (92%) used telehealth and 40% of providers had patients test positive for COVID-19. CONCLUSION: The COVID-19 pandemic has affected routine care and necessitated changes in practice in SCD.

Practice patterns for neuroimaging and transfusion therapy for management of neurologic complications in sickle cell anemia: DISPLACE consortium.

BACKGROUND: Children with sickle cell anemia (SCA) are at risk for neurologic complications (stroke and silent cerebral infarct). The 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines for sickle cell disease include recommendations for initiation and maintenance of chronic red cell transfusion (CRCT) therapy for children with SCA at risk for or with ischemic stroke. The guidelines do not include well-delineated recommendations for cerebral imaging for stroke screening. The purpose of this study was to evaluate current stroke risk screening, prevention, and intervention practices amongst the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) study sites. PROCEDURE: A survey was administered to DISPLACE site principal investigators to identify current stroke prevention practices relative to the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study protocols and the 2014 NHLBI guidelines. Data were analyzed using descriptive statistics and line-by-line analysis of comments. RESULTS: Sites consistently applied NHLBI recommendations to initiate CRCT for ischemic stroke and abnormal transcranial Doppler ultrasound (TCD) results. Similarly, nearly all sites reported obtaining an magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) following an abnormal TCD result. There was wide variation for other indications for MRI/MRA, frequency of MRI/MRA, and other neurological indications for initiating CRCT. CONCLUSIONS: Guideline-based practices for preventing ischemic stroke through TCD and CRCT initiation were evident in nearly all sites. Wide variation in practices pertaining to MRI/MRA exists, potentially influenced by more recent stroke prevention trials. New guidelines from the American Society of Hematology were published in April 2020, which may reduce practice variation.

Practice patterns for stroke prevention using transcranial Doppler in sickle cell anemia: DISPLACE Consortium.

BACKGROUND: Children with sickle cell anemia (SCA) are at increased risk for stroke. In 2014, the National Heart, Lung, and Blood Institute (NHLBI) developed guidelines for stroke prevention in SCA informed by the Stroke Prevention Trial in Sickle Cell Anemia (STOP) and Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP II) trials. The guidelines specify the use of transcranial Doppler (TCD) screening and intervention with chronic red cell transfusions (CRCT) in children with SCA who have TCD indication of high stroke risk. The purpose of this study was to describe real-world practice patterns of stroke risk screening and intervention in sites that participated in the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) Consortium. PROCEDURE: Site investigators completed a survey during the formative stages of the study to evaluate their TCD practices relative to the STOP studies. Descriptive statistics and analysis of free-text comments for more complex practices were evaluated. RESULTS: Results suggested universal acceptance of annual TCD screening and initiation of CRCT following an abnormal result among the DISPLACE Consortium, consistent with NHLBI recommendations. However, there was wide variation in methods for conducting TCD screenings (eg, dedicated Doppler vs TCD imaging), classifying TCD results, and actions taken for conditional and inadequate results. CONCLUSIONS: Annual TCD screening and initiation of CRCT are critical stroke prevention practices that were universally embraced in the consortium. Additional research would be beneficial for informing clinical practices for areas in which guidelines are absent or unclear.

Health-related Quality of Life in Children With Sickle Cell Disease Undergoing Chronic Red Cell Transfusion Therapy.

Chronic red cell transfusion (CRCT) therapy is one of few disease-modifying treatments for sickle cell disease (SCD). This study evaluated health-related quality of life (HRQL) in children receiving CRCT relative to 2 comparison groups: children with similar, severe SCD and children with milder disease risk defined by SCD genotype. For this study, 67 children with SCD between the ages of 8 and 18 completed the self-report Pediatric Quality of Life Sickle Cell Disease module (PedsQL SCD) as part of a pilot clinical program during routine hematologic visits. A medical chart review was also performed. Linear regression suggested that children in the CRCT group had significantly higher self-reported HRQL ratings for domains related to pain, F2,64=4.07 (P=0.022) and pain-related functioning, F2,64=4.32 (P=0.017), compared with children with similar and milder disease risk. Exploratory analyses implied that children in the CRCT group also had fewer worries about SCD-related complications, F3,63=9.68 (P<0.001). These patient-perceived benefits of CRCT may have important implications for treatment decisions and for providing ancillary support for children with SCD and their families.

Predictive validity of developmental screening in young children with sickle cell disease: a longitudinal follow-up study.

AIM: To assess the predictive validity of developmental screenings in children with sickle cell disease (SCD) for academic outcomes and stroke risk. METHOD: Parent questionnaires and medical record data were collected for a cohort receiving developmental screenings between September 2004 and May 2008 as toddlers or early school age. Screening outcomes were dichotomized (positive, negative) by a priori criteria. Questionnaires assessed school and social functioning, services received, and quality of life. Medical record data assessed general SCD morbidity and stroke risk. RESULTS: Forty-one toddlers (mean age 2y 5mo; 25 males, 16 females) and 49 early school-age children (mean age 6y 5mo; 26 males, 23 females) completed follow-up. The mean follow-up period was 8 years 6 months (range 6.1-10.8y). For toddlers, positive screenings for language delays predicted lower academic performance (p=0.023). For older children, positive screenings for cognitive delays predicted more frequent academic/attentional problems at school (p<0.001), grade retention (p=0.007), and lower academic performance (p=0.001). Positive screenings were associated with an earlier onset of school problems and lower quality of life. Positive screenings for language/cognitive delays predicted increased stroke risk (both p<0.05). INTERPRETATION: Screening for language or cognitive development in young children with SCD predicts academic outcomes and stroke risk. WHAT THIS PAPER ADDS: Developmental screening predicts academic outcomes in sickle cell disease. Children with concerning language/cognitive screenings have early-onset school difficulties. Developmental screenings may help predict cerebrovascular complications.

Examining Biopsychosocial Factors in Relation to Multiple Pain Features in Pediatric Sickle Cell Disease.

OBJECTIVE: To examine biopsychosocial variables in relation to multiple pain features in pediatric sickle cell disease (SCD). METHODS: 76 children with SCD (M = 14.05, SD = 3.26), ages 8-19 years, and 70 caregivers completed measures of coping, mood, and family functioning and reported on multiple pain features via retrospective interviews during routine hematological visits. Sickle cell genotype and health care utilization were collected via medical record review. Using hierarchical regression, biological (genotype), child psychological (coping and mood), and social factors (caregiver coping and family functioning) were evaluated in relation to multiple pain features. RESULTS: Genotype was associated with pain intensity, and child psychological factors were associated with pain frequency. Multiple biopsychosocial factors were related to health care utilization. CONCLUSIONS: Biopsychosocial factors may have distinct relationships with pain features in pediatric SCD. Understanding these relationships may refine the biopsychosocial model and inform integrated medical and psychosocial approaches in SCD.

Needs Assessment for Patients with Sickle Cell Disease in South Carolina, 2012.

OBJECTIVE: We conducted a needs assessment for patients with sickle cell disease (SCD) in South Carolina using statewide administrative data to examine acute care utilization during a defined 12-month period. The data were collected to provide information for state and regional service providers, managed care companies, and policy makers to identify demographic gaps in care and inform policy and educational efforts to improve care. METHODS: We obtained records on emergency department visits and hospitalizations through patient-based uniform billing data. We stratified analyses of acute care utilization and 30-day readmission rates by patient age, region, and expected payer. RESULTS: Young adults, those with public insurance, and those who resided in a region with the largest number of patients had the highest rates of acute care utilization and 30-day readmissions. Patients who resided in a largely rural area without access to comprehensive care also had high rates of acute care utilization and readmissions. The pattern of readmissions data suggested that data on 7- or 14-day readmission rates, in addition to data on 30-day readmission rates, could be used as benchmarks of quality of care for adult patients with SCD. CONCLUSION: Administrative datasets can provide important information on demographic gaps in care for patients with SCD. The results highlight both national and regional issues in the provision of health-care services for patients with SCD.

Age Differences in Emergency Department Visits and Inpatient Hospitalizations in Preadolescent and Adolescent Youth with Autism Spectrum Disorders.

This paper evaluated age differences in emergency department care and inpatient hospitalizations in 252 preadolescent and adolescent youth with autism spectrum disorders (ASDs; ages 9-18). Records from youth with ASDs were linked to acute care utilization records and were compared to a demographically similar comparison group of youth without ASDs (N = 1260). A particular focus was placed on utilization for psychiatric concerns and injuries or accidents. Results suggested that psychiatric care was more likely for youth with ASDs in both the preadolescent and adolescent cohorts versus comparison youth, with no significant differences between age cohorts. In contrast, results for the accident and injury categories suggested age-specific findings. Results suggest opportunities for prevention efforts for youth with ASDs.

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. Apelin is a vaso-dilatory peptide that also has a modulatory role in pain processing. We hypothesize that the ratio between vaso-dilatory and vaso-constrictive tone in children with SCD may be a marker of pain sensitization and vaso-occlusion. Plasma endothelin and apelin levels were measured in 47 children with SCD. Procedural pain and baseline pain were assessed via child- and caregiver-reports and observational distress. Pain history was assessed using retrospective chart review. Plasma apelin was related to age, with decreased levels in older children. The ratio between apelin and ET-1 was negatively correlated to observational baseline pain. The ratio between apelin and Big ET was negatively correlated to caregiver ratings of baseline pain and positively correlated to history of VOEs, which is possibly due to hydroxyurea treatment. These results suggest that an imbalance in the apelin and endothelin systems may contribute to an increasing number of VOEs and baseline pain in children with SCD.

Changes in coping, pain, and activity after cognitive-behavioral training: a randomized clinical trial for pediatric sickle cell disease using smartphones.

OBJECTIVES: We examined the outcomes of a cognitive-behavioral therapy (CBT) intervention for pain in pediatric sickle cell disease (SCD) using smartphones as a novel delivery method. MATERIALS AND METHODS: Forty-six children with SCD received CBT coping skills training using a randomized, waitlist control design. The intervention involved a single session of CBT training and home-based practice using smartphones for 8 weeks. Pre-post questionnaires between the randomized groups were used to evaluate changes in active psychological coping and negative thinking using the Coping Strategies Questionnaire. Daily diaries completed by the full sample during the treatment period were used to assess whether CBT skill use was related to reductions in next-day pain intensity and increases in same-day functional activity. RESULTS: The pre-post group comparison suggested that the youth increased active psychological coping attempts with the intervention. Daily diary data indicated that when children used CBT skills on days with higher pain, there were reductions in next-day pain intensity. There was no such association between skill use and functional activity. DISCUSSION: CBT coping skills training supported using smartphones can increase coping and reduce pain intensity for children with SCD; however, additions to the study protocols are recommended in future studies. Advantages and caveats of using smartphones are also discussed.