Understanding chronic pain in Neurofibromatosis Type 1 using the Neurofibromatosis Pain Module (NFPM).

Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder that can cause an individual significant chronic pain (CP). CP affects quality of life and daily functioning, yet there are limited effective treatments for CP within NF1. The current study describes the impact of CP using the Neurofibromatosis Pain Module (NFPM). The NFPM is a self-reported clinical assessment that evaluates the impact of CP across multiple domains (e.g., interference, severity, tolerance, and symptomology) and three prioritized pain regions. A cross-sectional study (N = 242) asked adults with NF1 to describe and rate their pain using the NFPM. The results indicated that they reported moderate pain severity (M = 6.6, SD = 2.0) on a 0-10 scale, that 54% (n = 131) had been in pain at least 24 days in the last 30, for 75% (n = 181) sleep was affected, and 16% reported that nothing was effective in reducing their CP for their primary pain region. The current results extend previously published work on CP within adults with NF1 and indicate that more emphasis on understanding and ameliorating CP is required. The NFPM is a sensitive clinical measure that provides qualitative and quantitative responses to inform medical providers about changes in CP.

The Relationship Between Experiences of Pain Among Youth With Obesity and Health-Related Quality of Life: The Role of Functional Limitation, Sleep, and Depressive Symptoms.

Background: Pediatric obesity is a growing concern in the United States and has been linked to negative psychological health outcomes such as depression, anxiety, and decreased quality of life. Obesity is a complex disease that is influenced by several environmental and social factors that are often out of an individuals' control. The etiology of pain in youth with obesity is not well understood. There are likely many factors that overlap and influence each other, including those related to functional limitation, sleep quality, and psychological health that exacerbate symptoms as a whole. Methods: This study examined the relationship between obesity level (BMI z-score) and youth self reports of: pain, functional limitation, sleep quality, depressive symptoms, and health-related quality of life (HRQoL). Ninety-eight patients completed validated surveys of pain, pain burden, functional disability, sleep, depression, and HRQoL as standard of care during their initial visit in Weight Management Program at Connecticut Children's Medical Center. Indirect effects of pain measures (pain scores and pain burden) on HRQoL through functional limitation, sleep quality, and depressive symptoms, respectively, were tested using bootstrapping according to Hayes.34 Results: Significant indirect effects and full mediation for both models were found. Conclusions: This study uniquely contributes to existing research through the discovery of the serial mediating effects of these variables in the relationship between youth pain and HRQoL. Although these variables have been studied independently as influential in this relationship in past research, this is the first study to examine how they interact through serial mediation models.

A cognitive-behavioral digital health intervention for sickle cell disease pain in adolescents: a randomized, controlled, multicenter trial.

ABSTRACT: Severe acute and chronic pain are the most common complications of sickle cell disease (SCD). Pain results in disability, psychosocial distress, repeated clinic visits/hospitalizations, and significant healthcare costs. Psychosocial pain interventions that teach cognitive and behavioral strategies for managing pain have been effective in other adolescent populations when delivered in person or through digital technologies. Our aim was to conduct a multisite, randomized, controlled trial to improve pain and coping in youth aged 12 to 18 years with SCD using a digital cognitive-behavioral therapy program (iCanCope with Sickle Cell Disease; iCC-SCD) vs Education control. We enrolled 137 participants (ages 12-18 years, 59% female) and analyzed 111 adolescents (107 caregivers), 54 randomized to Education control and 57 randomized to iCC-SCD. Ninety-two percent of youth completed posttreatment assessments and 88% completed 6-month follow-up. There was a significant effect of treatment group (iCC-SCD vs Education) on reduction in average pain intensity from baseline to 6-month follow-up (b = -1.32, P = 0.009, 95% CI [-2.29, -0.34], d = 0.50), and for the number of days with pain, adolescents in the iCC-SCD group demonstrated fewer pain days compared with the Education group at 6-month follow-up (incident rate ratio = 0.63, P = 0.006, 95% CI [0.30, 0.95], d = 0.53). Treatment effects were also found for coping attempts, momentary mood, and fatigue. Several secondary outcomes did not change with intervention, including anxiety, depression, pain interference, and global impression of change. Future studies are needed to identify effective implementation strategies to bring evidence-based cognitive-behavioral therapy for sickle cell pain to SCD clinics and communities.

Pain Frequency and Health Care Utilization Patterns in Women with Sickle Cell Disease Experiencing Menstruation-Associated Pain Crises.

Background: Pain crises in sickle cell disease (SCD) lead to high rates of health care utilization. Historically, women have reported higher pain burdens than men, with recent studies showing a temporal association between pain crisis and menstruation. However, health care utilization patterns of SCD women with menstruation-associated pain crises have not been reported. We studied the frequency, severity, and health care utilization of menstruation-associated pain crises in SCD women. Materials and Methods: A multinational, cross-sectional cohort study of the SCD phenotype was executed using a validated questionnaire and medical chart review from the Consortium for the Advancement of Sickle Cell Research (CASiRe) cohort. Total number of pain crises, emergency room/day hospital visits, and hospitalizations were collected from a subcohort of 178 SCD women within the past 6 months and previous year. Results: Thirty-nine percent of women reported menstruation-associated pain crises in their lifetime. These women were significantly more likely to be hospitalized compared with those who did not (mean 1.70 vs. 0.67, p = 0.0005). Women reporting menstruation-associated pain crises in the past 6 months also experienced increased hospitalizations compared with those who did not (mean 1.71 vs. 0.75, p = 0.0016). Forty percent of women reported at least four menstruation-associated pain crises in the past 6 months. Conclusions: Nearly 40% of SCD women have menstruation-associated pain crises. Menstruation-associated pain crises are associated with high pain burden and increased rates of hospitalization. Strategies are needed to address health care disparities within gynecologic care in SCD.

Maintaining Engagement in Adults with Neurofibromatosis Type 1 to Use the iCanCope Mobile Application (iCanCope-NF).

INTRODUCTION: Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic condition in which chronic pain is a predominant issue. Given the rarity of the disease, there are limited psychosocial treatments for individuals with NF1 suffering with chronic pain. Using mobile applications can facilitate psychosocial treatments; however, there are consistent issues with engagement. Utilizing a mixed methodology, the current study evaluated the customized iCanCope mobile application for NF1 on increasing engagement through the usage of contingency management. METHODS: A mixed methods study from a subset of data coming from a randomized clinical trial that occurred from January 2021 to August 2022 was undertaken. Two groups (iCC and iCC + CM) were exposed to the customized iCanCope mobile application in which engagement data were captured in real-time with daily check-ins for interference, sleep, mood, physical activity, energy levels, goal setting, and accessing article content (coping strategies). Additionally, semi-structured interviews were conducted to gain insight into the participants' experience at the end of the trial. RESULTS: Adults (N = 72) were recruited via NF patient advocacy groups. Significant differences were noted between the groups in total articles read (p = 0.002), goals achieved (p = 0.017), and goals created (p = 008). Additionally, there were significant differences observed between user-generated goals and those that were app recommended (p < 0.001). Both groups qualitatively reported positive feedback on the customized mobile application, indicating that continued usage and engagement of the mobile application were acceptable. CONCLUSIONS: Employing customized mobile applications for adults with NF1 along with contingency management can leverage self-managed pain treatments while providing auxiliary resources to this population.

Common Selfcare Indications of Pain Medications in Children.

Pain has a multifaceted impact on individuals worldwide, affecting their physical functioning, emotional well-being, and quality of life. Children (age < 18 years) have a high prevalence of conditions associated with pain, such as toothache, headache, earache, sore throat, and respiratory tract infections, many of which may be accompanied by fever. Globally, the pharmacologic treatment of pain in pediatric patients is limited largely to nonopioid analgesics, and dosing must account for differences in age, weight, metabolism, and risk of adverse effects. This narrative review summarizes the findings of a literature search on the pediatric indications, dosing approaches, dosing guidelines, and pharmacokinetics of paracetamol and ibuprofen, which are common pain medications available globally for self-care use in children. The review also discusses the risks and benefits associated with these agents. The current roles of paracetamol and ibuprofen in the symptomatic management of coronavirus disease 2019 (COVID-19) infection and in the management of post-COVID-19 immunization symptoms in children are also discussed. Therefore, while a very large amount of data over several decades is available for paracetamol and ibuprofen, an urgent need exists for well-designed studies of these medications for the management of pain and fever in pediatric patients with COVID-19 to ensure optimal relief with minimal toxicity.

Does Diagnostic Certainty Matter?: Pain-Related Stigma in Adolescents with Juvenile Idiopathic Arthritis.

OBJECTIVES: Childhood chronic pain conditions are common and vulnerable to stigma. Adolescents with chronic primary pain experience diagnostic uncertainty and describe pain-related stigma experiences across multiple social contexts. Juvenile idiopathic arthritis (JIA) is a childhood autoimmune, inflammatory condition with associated chronic pain, but with well-defined diagnostic criteria. The current study examined pain-related stigma experiences in adolescents with JIA. METHODS: Four focus groups of 3-7 adolescents with JIA (N = 16), ages 12-17 (Mage = 15.42, SD = 1.82), and parents (N = 13) were conducted to examine experiences of, and reaction to, pain-related stigma. Patients were recruited from an outpatient pediatric rheumatology clinic. Focus group length ranged from 28 to 99 minutes long. Two coders used directed content analysis resulting in 82.17% inter-rater level of agreement. RESULTS: Adolescents with JIA described pain-related stigma experiences predominantly from school teachers and peers, and less from medical providers (e.g., school nurses), and family members after a diagnosis. The primary categories that emerged were (1) Felt Stigma, (2) Internalized Stigma, (3) Anticipatory Stigma/Concealment, and (4) Contributions to Pain-Related Stigma. A common experience of pain-related stigma was the perception by others that the adolescent was too young to have arthritis. CONCLUSIONS: In common with adolescents with unexplained chronic pain, our findings indicate that adolescents with JIA experience pain-related stigma in certain social contexts. Diagnostic certainty may contribute to greater support among medical providers and within families. Future research should investigate the impact of pain-related stigma across childhood pain conditions.

Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities, and Networks-American Pain Society-American Academy of Pain Medicine Pain Taxonomy Diagnostic Criteria for Acute Needle Pain.

Needle procedures are among the most common causes of pain and distress for individuals seeking health care. While needle pain is especially problematic for children needle pain and associated fear also has significant impact on adults and can lead to avoidance of appropriate medical care. Currently there is not a standard definition of needle pain. A taxonomy, or classification system, for acute needle pain would aid research efforts and enhance clinical care. To meet this need, the Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities, and Networks public-private partnership with the U.S. Food and Drug Administration, the American Pain Society, and the American Academy of Pain Medicine formed the Analgesic, Anesthetic, and Addiction Clinical Trial Translations, Innovations, Opportunities, and Networks-American Pain Society-American Academy of Pain Medicine Pain Taxonomy initiative. One of the goals of this initiative was to develop taxonomies for acute pain disorders, including needle pain. To accomplish this, a working group of experts in needle pain was convened. Based on available literature and expert opinion, the working group used a 5-dimenional structure (diagnostic criteria, common features, modulating factors, impact and/or functional consequences, and putative mechanisms) to develop an acute pain taxonomy that is specific needle pain. As part of this, a set of 4 diagnostic criteria, with 2 modifiers to account for the influence of needle associated fear, are proposed to define the types of acute needle pain. PERSPECTIVE: This article presents a taxonomy for acute needle pain. This taxonomy could help to standardize definitions of acute pain in clinical studies of patients undergoing needle procedures.

A Systematic Approach to Developing Virtual Patient Vignettes for Pediatric Health Equity Research.

Objective: The aim of this study was to describe a systematic approach to developing virtual patient (VP) vignettes for health equity research in pediatric pain care. Methods: VPs were initially developed to depict the body posture and movements of actual children experiencing pain. Researchers and clinicians with expertise in pediatric pain worked closely with a professional animator to portray empirically supported pain expression in four, full-motion, virtual male characters of two races (i.e., White and Black). Through an iterative process, VPs were refined to (1) appear realistic in a clinical setting and (2) display archetypal pain behavior and expression during a 1-min video clip without sound. Text vignettes were developed with consultation from experts in pain care and presented alongside VPs to assess clinical decision-making. VP vignettes were piloted in a sample of pediatric providers (N=13). Results: Informed by the literature and expertise of stakeholders, several revisions were made to improve VPs' facial grimacing and realism before piloting. VPs appeared to accurately capture important aspects of pain expression and behavior common among pediatric patients with pain disorders. Additional refinements to the text vignettes were made based on provider feedback to improve clarity and clinical relevance. Conclusions: This article presents a working framework to facilitate a systematic approach to developing VP vignettes. This framework is a first step toward advancing health equity research by isolating psychosocial and interpersonal factors affecting provider behavior and decision-making. Future research is needed to validate the use of VP vignettes for assessing provider behavior contributing to health inequities for youth with pain disorders.

Characterizing User Engagement With a Digital Intervention for Pain Self-management Among Youth With Sickle Cell Disease and Their Caregivers: Subanalysis of a Randomized Controlled Trial.

BACKGROUND: Sickle cell disease (SCD) is characterized by severe acute pain episodes as well as risk for chronic pain. Digital delivery of SCD pain self-management support may enhance pain self-management skills and accessibility for youth. However, little is known about how youth with SCD and their caregivers engage with digital health programs. iCanCope with pain is a digital pain self-management platform adapted for youth with SCD and caregivers through a user-centered design approach. The program was delivered via a website (separate versions for youth and caregiver) and mobile app (youth only). OBJECTIVE: We aimed to characterize patterns of user engagement with the iCanCope with SCD program among youth with SCD and their caregivers. METHODS: A randomized controlled trial was completed across multiple North American SCD clinics. Eligible youth were aged 12-18 years, diagnosed with SCD, English-speaking, and experiencing moderate-to-severe pain interference. Eligible caregivers were English-speaking with a child enrolled in the study. Dyads were randomized to receive the iCanCope intervention or attention-control education for 8-12 weeks. This report focused on engagement among dyads who received the intervention. User-level analytics were captured. Individual interviews were conducted with 20% of dyads. Descriptive statistics characterized quantitative engagement. Content analysis summarized qualitative interview data. Exploratory analysis tested the hypothesis that caregiver engagement would be positively associated with child engagement. RESULTS: The cohort included primarily female (60% [34/57] of youth; 91% [49/56] of caregivers) and Black (>90% of youth [53/57] and caregivers [50/56]) participants. Among 56 dyads given program access, differential usage patterns were observed: both the youth and caregiver engaged (16/56, 29%), only the youth engaged (24/56, 43%), only the caregiver engaged (1/56, 2%), and neither individual engaged (16/56, 29%). While most youth engaged with the program (40/57, 70%), most caregivers did not (39/56, 70%). Youth were more likely to engage with the app than the website (85% [34/57] versus 68% [23/57]), and the most popular content categories were goal setting, program introduction, and symptom history. Among caregivers, program introduction, behavioral plans, and goal setting were the most popular content areas. As hypothesized, there was a moderate positive association between caregiver and child engagement (χ21=6.6; P=.01; ϕ=0.34). Interviews revealed that most dyads would continue to use the program (11/12, 92%) and recommend it to others (10/12, 83%). The reasons for app versus website preference among youth were ease of use, acceptable time commitment, and interactivity. Barriers to caregiver engagement included high time burden and limited perceived relevance of content. CONCLUSIONS: This is one of the first studies to apply digital health analytics to characterize patterns of engagement with SCD self-management among youth and caregivers. The findings will be used to optimize the iCanCope with SCD program prior to release. TRIAL REGISTRATION: ClinicalTrials.gov NCT03201874; https://clinicaltrials.gov/ct2/show/NCT03201874.

Perspectives on adapting a mobile application for pain self-management in neurofibromatosis type 1: results of online focus group discussions with individuals living with neurofibromatosis type 1 and pain management experts.

OBJECTIVE: Neurofibromatosis type 1 (NF1) is a genetic disorder in which chronic pain commonly occurs. The study sought to understand the needs of individuals with NF1 and pain management experts when adapting a pain self-management mobile health application (app) for individuals with NF1. DESIGN: We conducted a series of online, audio-recorded focus groups that were then thematically analysed. SETTING: Online focus groups with adults currently residing in the USA. PARTICIPANTS: Two types of participants were included: individuals with NF1 (n=32 across six focus groups) and pain management experts (n=10 across three focus groups). RESULTS: Six themes across two levels were identified. The individual level included lifestyle, reasons for using the mobile app and concerns regarding its use. The app level included desired content, desired features and format considerations. Findings included recommendations to grant free access to the app and include a community support feature for individuals to relate and validate one another's experience with pain from NF1. In addition, participants noted the importance of providing clear instructions on navigating the app, the use of an upbeat, hopeful tone and appropriate visuals. CONCLUSIONS: Both participant groups endorsed the use of iCanCope (iCC) as an NF1 pain self-management mobile app. Differences between groups were noted, however. The NF1 group appeared interested in detailed and nuanced pain tracking capabilities; the expert group prioritised tracking information such as mood, nutrition and activity to identify potential associations with pain. In tailoring the existing iCC app for individuals with NF1, attention should be paid to creating a community support group feature and to tailoring content, features and format to potential users' specific needs.

Pain Burden in the CASiRe International Cohort of Sickle Cell Patients: United States and Ghana.

OBJECTIVES: Sickle Cell Disease (SCD) is a genetic blood disorder affecting over 1 million people globally. The aim of this analysis is to explore the pain burden of patients with SCD in two countries: the United States and Ghana. METHODS: The Consortium for the Advancement of Sickle Cell Research (CASiRe) was created to better understand the clinical severity of patients with SCD worldwide. Data regarding gender, SCD genotype, prior medical diagnoses, and validated pain burden measures were analyzed from the CASiRe database. The Sickle Cell Pain Burden Interview (SCPBI) was used to assess pain burden, the impact of pain on physical, emotional, and social function. RESULTS: Most subjects identified as Black/African American (n = 298, 97.0%). Patient ages ranged from 6 to 73 years. 35.9% resided in the United States, 64.1% resided in Ghana, 40.9% were men, and 58.7% were women. The mean SCPBI score for US SCD patients was 6.53(±5.89) vs 4.04(±5.10) for Ghanaian patients, P <0.001. Pain burden was higher in US men vs Ghanaian men (6.74(±5.68) vs 3.54(±4.46), P = .003) and in US women vs Ghanaian women (6.37 ± 6.06 vs 4.44(±5.54), P = .032). Pain burden was higher in US patients than Ghanaian patients for both the Hb SC/SBeta+ genotype (5.40(±5.29) vs 2.82(±4.86), P = .054) and Hb SS/SBeta0 genotype (6.79(±6.01) vs 4.49(±5.13), P = .003). Pain burden was significantly higher in SCD patients with comorbid conditions independent of geographic origin including stroke, cholecystectomy, gallstones, depression, and headache. DISCUSSION: US patients with SCD have a higher pain burden than Ghanaian patients. Further studies should investigate underlying contributors to pain burden in these populations and further explore the etiology of geographic differences in pain.

Innovation in the treatment of persistent pain in adults with Neurofibromatosis Type 1 (NF1): Implementation of the iCanCope mobile application.

Neurofibromatosis Type 1 (NF1) is a genetic disorder presenting with chronic pain symptoms that has limited treatment options for addressing the pain. The utilization of a mobile application allows for greater reach and scalability when using empirically valid psychosocial self-management treatments for pain. The iCanCope mobile application has been utilized in several different populations dealing with pain symptoms and has demonstrated initial effectiveness. To address the need for this population, we have customized the iCanCope mobile application for the NF1 population and included additional tailored features. We describe the rationale and design of a pilot randomized control study with a sample of 108 adults with NF1, in which two groups will receive access to the mobile application, of which one group will be incentivized to engage in the mobile application and the third group will treatment as usual over the course of 8-week period with a six-week follow-up. Outcomes will focus on the acceptability of the iCanCope-NF mobile application within the NF1 population and the impact of pain related activity on psychometric evaluations to determine if the contingency management will impact the engagement of mobile application, as well as to identify the participants' experiences in relationship to their treatment satisfaction and perceived support.

Efficacy and safety of pharmacological, physical, and psychological interventions for the management of chronic pain in children: a WHO systematic review and meta-analysis.

ABSTRACT: Chronic pain in childhood is an international public health problem. We conducted a systematic review and meta-analysis to provide a summary of the published evidence of pharmacological, physical, and psychological therapies for children with chronic pain conditions. We searched CENTRAL, MEDLINE, EMBASE, and PsycINFO from inception to April 2020; clinical trial registries; and other sources for randomised controlled trials or comparative observational trials. We extracted critical outcomes of pain intensity, quality of life, physical functioning, role functioning, emotional functioning, sleep, and adverse events. We assessed studies for risk of bias and certainty of the evidence using GRADE. We included 34 pharmacological (4091 participants), 25 physical therapy (1470 participants), and 63 psychological trials (5025 participants). Participants reported a range of chronic pain conditions. Most studies were assessed to have unclear or high risk of bias across multiple domains. Pharmacological, physical, and psychological therapies showed some benefit for reducing pain, posttreatment, but only physical and psychological therapies improved physical functioning. We found no benefit of any treatment modality for health-related quality of life, role functioning, emotional functioning, or sleep. Adverse events were poorly reported, particularly for psychological and physical interventions. The largest evidence base for the management of chronic pain in children supports the use of psychological therapies, followed by pharmacological and physical therapies. However, we rated most outcomes as low or very low certainty, meaning further evidence is likely to change our confidence in the estimates of effects. This protocol was registered on PROSPERO (CRD42020172451).

"There's Nothing Wrong With You": Pain-Related Stigma in Adolescents With Chronic Pain.

OBJECTIVE: Adolescents with chronic pain often experience symptom disbelief and social rejection by others secondary to "medically unexplained" symptoms. Although chronic pain is common in adolescents, limited research has conceptualized these social experiences as pain-related stigma in this population. The purpose of this study was to identify and describe pain-related stigma among adolescents with chronic pain and their parents using focus group methodology. METHODS: Five adolescent focus groups (N = 18; Age M = 15.33 years, SD = 1.28) and three parent focus groups (N = 9) were conducted. Directed content analysis was used to analyze focus group transcripts. Stigma categories were developed a priori (Felt Stigma, Anticipated Stigma, Internalized Stigma, Concealment, and Controllability) and new categories emerged during analysis. Two coders reached 87.16% agreement for all groups (adolescent group: 90.34%; Parent group: 79.55%) and consensus was achieved for discordant codes. RESULTS: Adolescents and their parents endorsed pain-related stigma across all social domains. Analyses revealed four main categories for both groups (a) Felt Stigma (subcategories: pain dismissal, faking or exaggerating, and mental health stigma), (b) Anticipated Stigma and Concealment, (c) Internalized Stigma, and (d) Sources of Pain-Related Stigma (subcategories: pain invisibility, lack of chronic pain knowledge, lack of understanding, and controllability). CONCLUSIONS: Adolescents with chronic pain experience pain-related stigma from medical providers, school personnel, family members, and peers, which may have negative social and health implications. More research is needed to evaluate the link between pain-related stigma and health outcomes for adolescents with chronic pain. Clinical approaches targeting pain-related stigma are discussed.

Navigating Ethical Challenges for Pediatric Sickle Cell Pain Management in the Context of the Opioid Epidemic.

OBJECTIVES: The aim was to describe the impact of the opioid epidemic on pain management practices in pediatric sickle cell disease (SCD) and propose a conceptual framework for navigating ethical decision-making in pediatric sickle cell pain management. METHODS: A review of the literature on ethical challenges in the management of sickle cell pain was conducted and considered in the context of the opioid epidemic and psychosocial factors affecting youth with SCD. The Integrated Ethical Framework for Pain Management (IEFPM) was applied to pediatric sickle cell pain management using a clinical case example. RESULTS: Implicit bias, health-related stigma, and potential neurocognitive impairment all present unique challenges in ethical decision-making for youth with SCD. National guidelines for prescribing opioid medication may complicate providers' clinical decision-making and affect their sickle cell pain management practices. The IEFPM was found to be applicable to ethical decision-making for pediatric sickle cell pain and captures both patient-related and provider-related aspects of clinical pain management. DISCUSSION: The opioid epidemic has exacerbated existing ethical challenges for pain management among youth with SCD. The IEFPM provides a conceptual model that can be integrated into health care settings to facilitate ethical decision-making and promote greater health equity in the clinical management of pediatric sickle cell pain.

Global geographic differences in healthcare utilization for sickle cell disease pain crises in the CASiRe cohort.

BACKGROUND: Sickle cell disease (SCD) is characterized by frequent, unpredictable pain episodes and other vaso-occlusive crises (VOCs) leading to significant healthcare utilization. VOC frequency is often an endpoint in clinical trials investigating novel therapies for this devastating disease. PROCEDURE: The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaboration investigating clinical severity in SCD using a validated questionnaire and medical chart review standardized across four countries (United States, United Kingdom, Italy and Ghana). RESULTS: This study, focused on pain crisis incidence and healthcare utilization, included 868 patients, equally represented according to age and gender. HgbSS was the most common genotype. Patients from Ghana used the Emergency Room/Day Hospital for pain more frequently (annualized mean 2.01) than patients from other regions (annualized mean 1.56 U.S.; 1.09 U.K.; 0.02 Italy), while U.K. patients were hospitalized for pain more often (annualized mean: U.K. 2.98) than patients in other regions (annualized mean 1.98 U.S.; 1.18 Ghana; Italy 0.54). Italy's hospitalization rate for pain (annualized mean: 0.57) was nearly 20 times greater than its emergency room/day hospital only visits for pain (annualized mean: 0.03). When categorized by genotype and age, similar results were seen. CONCLUSIONS: Geographic differences in pain crisis frequency and healthcare utilization may correlate with variable organization of healthcare systems among countries and should be considered regarding trial design, endpoints, and analysis of results when investigating novel agents for clinical benefit.

Gender Dysphoria and Chronic Pain in Youth.

Chronic pain in youth with gender dysphoria (GD) is poorly understood. The aim of our study was to review the clinical presentation of 8 youth with GD in a multidisciplinary chronic pain clinic. A single center retrospective chart review was conducted to obtain information on demographics, clinical care, previous diagnoses, and validated clinical measures. We present the trajectory of pain in this population with treatment of GD. Recognition and treatment of GD in youth with pain may improve pain outcomes.

Preliminary Validation of the Pain Burden Inventory in a Pediatric Chronic Pain Population.

OBJECTIVE: To determine the preliminary reliability and validity of the Pain Burden Inventory-Youth (PBI-Y), a 7-item measure of the impact of chronic pain in adolescents with chronic pain that was, initially validated in youth with sickle cell disease. MATERIALS AND METHODS: A retrospective chart review of 130 patients presenting to an interdisciplinary pain clinic were examined with 98 (mean age=14.49; 66% female) comprising the final sample. Demographic information as well as adolescent and parent self-reported measures were examined. RESULTS: Youth reported a variety of pains including abdominal pain, headaches, back pain, and amplified musculoskeletal pain. The PBI-Y demonstrated strong internal reliability (α=0.792) and strong cross-informant concordance (r=0.822; P=0.000). Good construct validity was seen where higher scores on the PBI-Y were correlated with higher scores of (1) functional disability (r=0.689; P=0.000), (2) pain catastrophizing (r=0.494; P=0.000), (3) pain frequency days (r=0.526; P=0.000), (4) usual pain intensity (r=0.467; P=0.000), and (5) the worst pain intensity (r=0.485; P=0.000). Similar results were seen with caregiver assessments. Neither caregiver nor youth reports differed based on the sex or age of the child. DISCUSSION: The results support the psychometric properties of a brief self-report measure of pain impact in a pediatric chronic pain population. This measure may have great utility for clinicians caring for youth with chronic pain.