Health literacy correlates with abbreviated full-scale IQ in adolescent and young adults with sickle cell disease.

INTRODUCTION: Sickle cell disease (SCD) is a chronic condition with progressive neurocognitive deficits. Health literacy (HL) is essential during adolescence and young adulthood, as the transition to adult care requires healthcare decisions. HL is known to be low in SCD; however, relation between general cognitive ability and HL has not been investigated. METHODS: This cross-sectional study included adolescent and yound adults (AYAs) with SCD from two institutions. Logistic regression measured the association between HL, measured by the Newest Vital Sign tool, and general cognitive ability, measured with abbreviated full-scale intelligence quotient (FSIQ) on the Wechsler Abbreviated Scale of Intelligence. RESULTS: Our cohort contained 93 participants at two sites: 47 (51%) at Memphis, TN and 46 (49%) at St. Louis, MO, ranging from ages 15-45 years (mean = 21 years) and with a majority (70%) possessing a high school education or greater. Only 40/93 participants (43%) had adequate HL. Lower abbreviated FSIQ (p < .0001) and younger age at assessment (p = .0003) were associated with inadequate HL. For every standard score point increase in abbreviated FSIQ, the odds of having adequate HL compared to limited or possibly limited HL increase by 1.142 (95% confidence interval [CI]: 1.019-1.322) and 1.116 (95% CI: 1.045-1.209), respectively, after adjusting for age, institution, income, and educational attainment. CONCLUSIONS: Understanding and addressing HL is imperative in improving self-management and health outcomes. Among AYA with SCD, low HL was prevalent and influenced by abbreviated FSIQ. Routine screening for neurocognitive deficits and HL should be performed to guide development of interventions to adapt to the HL of AYA with SCD.

Transition care continuity promotes long-term retention in adult care among young adults with sickle cell disease.

BACKGROUND/OBJECTIVES: Care continuity prevents increased health care utilization and mortality during transition from pediatric to adult care. Our program employs a co-located care delivery model, in which pediatric provider involvement continues during young adulthood. We tested the hypothesis that individuals who participated in the co-located model have greater retention in adult care compared to those who only received pediatric transition services. METHODS: This study consisted of 311 youth with SCD (51.4% male; 63.0% HbSS/HbSß0 -thalassemia) who transferred to adult care from 2007 to 2017. Retention was defined as continuation with an adult provider for ≥12 or ≥24 months post-pediatric care. Logistic regression estimated the association between co-location status and retention at 12 and 24 months. Logistic regression and t-tests were used to evaluate potential predictors of retention in adult care. RESULTS: Individuals who participated in the co-location model were 1.9 times more likely to remain in adult care 12 (95% CI: 1.01, 3.47) and 24 (95% CI: 1.01, 3.70) months post-pediatric care compared to those who did not participate. Individuals with HbSS/HbSß0 -thalassemia were 1.9 times more likely to be retained at 12 months compared to those with HbSC/HbSß+ -thalassemia/HbS/HPFH (95% CI: 1.12, 3.09). For every clinic encounter in the last 2 years of pediatric care, the odds of being retained at least 24 months after initiating adult care increased 1.1 times (95% CI: 1.02, 1.13). CONCLUSIONS: Continuity of providers from pediatric to adult care may increase long-term retention in adult care. Longitudinal monitoring of adult outcomes is critical to identifying the efficacy of transition services.

Gaps during pediatric to adult care transfer escalate acute resource utilization in sickle cell disease.

ABSTRACT: Guidelines recommend transfer to adult health care within 6 months of completing pediatric care; however, this has not been studied in sickle cell disease (SCD). We hypothesized that longer transfer gaps are associated with increased resource utilization. Transfer gaps were defined as the time between the last pediatric and first adult visits. We estimated the association between varying transfer gaps and the rates of inpatient, emergency department (ED), and outpatient visits, using negative binomial regression. Health care utilization was evaluated in a mid-south comprehensive program for a follow-up period of up to 8 years (2012-2020) and was restricted to the first 2 years of adult health care. In total, 183 young adults (YAs) with SCD (51% male, 67% HbSS/HbSß0-thalassemia) were transferred to adult health care between 2012 and 2018. YAs with transfer gaps ≥6 months compared with <2 months had 2.01 (95% confidence interval [CI], 1.31-3.11) times the rate of hospitalizations in the 8-year follow-up and 1.89 (95% CI, 1.17-3.04) when restricted to the first 2 years of adult health care. In the first 2 years of adult care, those with transfer gaps ≥6 months compared with <2 months, had 1.75 (95% CI, 1.10-2.80) times the rate of ED encounters. Those with gaps ≥2 to <6 months compared with <2 months had 0.71 (95 % CI, 0.53-0.95) times the rate of outpatient visits. Among YAs with SCD, a longer transfer gap was associated with increased inpatient and decreased outpatient encounters in adult health care and more ED encounters in the first 2 years of adult health care. Strategies to reduce the transfer gaps are needed.

Using mHealth Technology to Evaluate Daily Symptom Burden among Adult Survivors of Childhood Cancer: A Feasibility Study.

BACKGROUND: Cancer therapies predispose survivors to a high symptom burden. This study utilized mobile health (mHealth) technology to assess the feasibility of collecting daily symptoms from adult survivors of childhood cancer to evaluate symptom fluctuation and associations with future health-related quality-of-life (HRQOL). METHODS: This prospective study used an mHealth platform to distribute a 20-item cancer-related symptom survey (5 consecutive days each month) and an HRQOL survey (the day after the symptom survey) over 3 consecutive months to participants from the Childhood Cancer Survivor Study. These surveys comprised a PROMIS-29 Profile and Neuro-QOL assessed HRQOL. Daily symptom burden was calculated by summing the severity (mild, moderate, or severe) of 20 symptoms. Univariate linear mixed-effects models were used to analyze total, person-to-person, day-to-day, and month-to-month variability for the burden of 20 individual symptoms. Multivariable linear regression was used to analyze the association between daily symptom burden in the first month and HRQOL in the third month, adjusted for covariates. RESULTS: Out of the 60 survivors invited, 41 participated in this study (68% enrollment rate); 83% reported their symptoms ≥3 times and 95% reported HRQOL in each study week across 3 months. Variability of daily symptom burden differed from person-to-person (74%), day-to-day (18%), and month-to-month (8%). Higher first-month symptom burden was associated with poorer HRQOL related to anxiety (regression coefficient: 6.56; 95% CI: 4.10-9.02), depression (6.32; 95% CI: 3.18-9.47), fatigue (7.93; 95% CI: 5.11-10.80), sleep (6.07; 95% CI: 3.43-8.70), pain (5.16; 95% CI: 2.11-8.22), and cognitive function (-6.89; 95% CI: -10.00 to -3.79) in the third month. CONCLUSIONS: Daily assessment revealed fluctuations in symptomology, and higher symptom burden was associated with poorer HRQOL in the future. Utilizing mHealth technology for daily symptom assessment improves our understanding of symptom dynamics and sources of variability.

Using an mHealth approach to collect patient-generated health data for predicting adverse health outcomes among adult survivors of childhood cancer.

Introduction: Cancer therapies predispose childhood cancer survivors to various treatment-related late effects, which contribute to a higher symptom burden, chronic health conditions (CHCs), and premature mortality. Regular monitoring of symptoms between clinic visits is useful for timely medical consultation and interventions that can improve quality of life (QOL). The Health Share Study aims to utilize mHealth to collect patient-generated health data (PGHD; daily symptoms, momentary physical health status) and develop survivor-specific risk prediction scores for mitigating adverse health outcomes including poor QOL and emergency room admissions. These personalized risk scores will be integrated into the hospital-based electronic health record (EHR) system to facilitate clinician communications with survivors for timely management of late effects. Methods: This prospective study will recruit 600 adult survivors of childhood cancer from the St. Jude Lifetime Cohort study. Data collection include 20 daily symptoms via a smartphone, objective physical health data (physical activity intensity, sleep performance, and biometric data including resting heart rate, heart rate variability, oxygen saturation, and physical stress) via a wearable activity monitor, patient-reported outcomes (poor QOL, unplanned healthcare utilization) via a smartphone, and clinically ascertained outcomes (physical performance deficits, onset of/worsening CHCs) assessed in the survivorship clinic. Participants will complete health surveys and physical/functional assessments in the clinic at baseline, 2) report daily symptoms, wear an activity monitor, measure blood pressure at home over 4 months, and 3) complete health surveys and physical/functional assessments in the clinic 1 and 2 years from the baseline. Socio-demographic and clinical data abstracted from the EHR will be included in the analysis. We will invite 20 cancer survivors to investigate suitable formats to display predicted risk information on a dashboard and 10 clinicians to suggest evidence-based risk management strategies for adverse health outcomes. Analysis: Machine and statistical learning will be used in prediction modeling. Both approaches can handle a large number of predictors, including longitudinal patterns of daily symptoms/other PGHD, along with cancer treatments and socio-demographics. Conclusion: The individualized risk prediction scores and added communications between providers and survivors have the potential to improve survivorship care and outcomes by identifying early clinical presentations of adverse events.

Feasibility of Fitbit Use in Adolescent Survivors of Pediatric Cancer: Who Consistently Uses It and for How Long?

Purpose: Wearable activity trackers with real-time feedback and goal-setting features are being incorporated into programs to increase physical activity among childhood cancer survivors. This analysis describes the adoption and use of a Fitbit® Flex™ among adolescent-aged survivors of childhood cancer without incentives, reminders, or interventions to encourage use. Procedures: Cancer survivors aged 13-18 and ≥2 years from therapy received a Fitbit Flex with instructions to wear it daily. Researchers downloaded participants' daily total steps and active minutes for 1 year. Participants were classified as consistent (≥5 days/week for >4 weeks during the first 12 weeks) or inconsistent users, and comparisons between user types were made. Longitudinal use of the Fitbit and participants' 1-year acceptability evaluations are described. Results: Overall, 67.1% (47/70) of survivors enrolled, and Fitbit data were available for 36 participants. Initially, 30.6% (11/36) were consistent users. Consistent users had lower body mass index z-scores at enrollment (0.4 ± 0.7 vs. 1.2 ± 0.9; p = 0.01), but were otherwise comparable with inconsistent users. Over time survivors' use declined; at 12 months, only one participant was using his or her Fitbit. Survivors who completed a survey (n = 22) reported their Fitbit helped them self-monitor their exercise (72.7%, 16/22) and lead a more active lifestyle (63.6%, 14/22), but found it challenging to charge and not lose, forget about, or break the device. Conclusions: In the real-world setting, only a small subset of adolescent-aged survivors will initially consistently use a Fitbit and their interest diminishes over time. To maximize engagement, interventions incorporating wearable activity trackers likely need to include additional behavior change strategies.

Assessment of transition readiness to predict health care utilization during transition to adult care in sickle cell disease.

BACKGROUND: Transition-age patients with sickle cell disease (SCD) are at risk for poor outcomes associated with incomplete transition readiness and neurocognitive deficits. Study objectives were to: 1) test if a SCD-specific measure of self-management skills was associated with transition outcomes and 2) evaluate if caregiver-reported executive functioning was associated with self-management skills and transition outcomes among youth with SCD. RESEARCH DESIGN AND METHODS: Youth/caregivers were selected from a longitudinal cohort study. Caregivers completed the Behavior Rating Inventory of Executive Function (BRIEF); caregivers and youth completed the Self-Management Skills Checklist (SMSC) at a median age of 16.8 ± 0.6 years. Non-parametric tests compared SMSC and transition outcomes. Regression assessed the incremental validity of SMSC in predicting transition outcomes. RESULTS: In total, 95 participants (54% male, 55% severe genotype) completed the SMSC assessment. Most participants (87%) transferred to adult care within six months and 87% were retained for at least 12 months. BRIEF and caregiver-reported SMSC assessments were weakly, negatively correlated (ρ = -0.25, p = 0.0392) but were not significant in predicting transition outcomes (p > 0.05). CONCLUSIONS: The SMSC and executive function did not predict adult care engagement. Development of readiness assessments that predict care engagement and reflect self-efficacy is important for monitoring transition-aged patients with SCD.

Identifying barriers to evidence-based care for sickle cell disease: results from the Sickle Cell Disease Implementation Consortium cross-sectional survey of healthcare providers in the USA.

OBJECTIVES: Sickle cell disease (SCD) leads to chronic and acute complications that require specialised care to manage symptoms and optimise clinical results. The National Heart Lung and Blood Institute (NHLBI) evidence-based guidelines assist providers in caring for individuals with SCD, but adoption of these guidelines by providers has not been optimal. The objective of this study was to identify barriers to treating individuals with SCD. METHODS: The SCD Implementation Consortium aimed to investigate the perception and level of comfort of providers regarding evidence-based care by surveying providers in the regions of six clinical centres across the USA, focusing on non-emergency care from the providers' perspective. RESULTS: Respondents included 105 providers delivering clinical care for individuals with SCD. Areas of practice were most frequently paediatrics (24%) or haematology/SCD specialist (24%). The majority (77%) reported that they were comfortable managing acute pain episodes while 63% expressed comfort with managing chronic pain. Haematologists and SCD specialists showed higher comfort levels prescribing opioids (100% vs 67%, p=0.004) and managing care with hydroxyurea (90% vs 51%, p=0.005) compared with non-haematology providers. Approximately 33% of providers were unaware of the 2014 NHLBI guidelines. Nearly 63% of providers felt patients' medical needs were addressed while only 22% felt their mental health needs were met. CONCLUSIONS: A substantial number of providers did not know about NHLBI's SCD care guidelines. Barriers to providing care for patients with SCD were influenced by providers' specialty, training and practice setting. Increasing provider knowledge could improve hydroxyurea utilisation, pain management and mental health support.

A meta-analysis of toxicities related to hydroxycarbamide dosing strategies.

Due to fear of short-term toxicities, there is nonconsensus of hydroxycarbamide dosing strategy (escalated vs fixed-dosing methods), which contributes to its suboptimal use. We performed a meta-analysis to summarize the incidence rates of toxicities associated with both dosing methods. Summarized incidence rates could not be statistically compared between dosing methods due to sparse data. Summarized neutropenia and thrombocytopenia incidence rates were slightly higher when using escalated dosing than with fixed. Summarized reticulocytopenia was comparable. Summarized hepatic and renal toxicities' incidence rates were slightly higher when using fixed doses than with escalated. We recommend diligent and transparent reporting of toxicities.

The International Association for the Study of Lung Cancer Global Survey on Molecular Testing in Lung Cancer.

INTRODUCTION: Access to targeted therapies for lung cancer depends on the accurate identification of patients' biomarkers through molecular testing. The International Association for the Study of Lung Cancer (IASLC) conducted an international survey to evaluate perceptions on current practice and barriers to implementation of molecular testing. METHODS: We distributed the survey to IASLC members and other health care professionals around the world. The survey included a seven-question introduction for all respondents, who then answered according to one of three tracks: (1) requesting tests and treating patients, (2) performing and interpreting assays, or (3) tissue acquisition. Barriers to implementing molecular testing were provided in free-response fields. The chi-square test was used for regional comparisons. RESULTS: A total of 2537 respondents from 102 countries participated. Most respondents who test and treat patients believe that less than 50% of patients with lung cancer in their country receive molecular testing, but reported higher rates within their own practice. Although many results varied by region, the five most frequent barriers cited in all regions were cost, quality and standards, access, awareness, and turnaround time. Many respondents expressed dissatisfaction with the current state of molecular testing for lung cancer, including 41% of those performing and interpreting assays. Issues identified included trouble understanding results (37%) and the quality of the samples (23% reported >10% rejection rate). Despite concerns regarding the quality of testing, 47% in the performing and interpreting track stated there is no policy or strategy to improve quality in their country. In addition, 33% of respondents who request tests and treat patients were unaware of the most recent College of American Pathologists, IASLC, and Association for Molecular Pathology guidelines for molecular testing. CONCLUSIONS: Adoption of molecular testing for lung cancer is relatively low across the world. Barriers include cost, access, quality, turnaround time, and lack of awareness.