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Health‐related quality of life (HRQOL) is a patient‐reported outcome that assesses the impact of a disease or illness on different domains of a patient's life. Different general and disease‐specific measures can be used to evaluate HRQOL. This article aimed to summarize the evidence for HRQOL among patients with transfusion‐dependent (TDT) and non‐transfusion‐dependent thalassemia (NTDT). We included HRQOL data related to standard therapy with blood transfusions, iron chelation, and/or luspatercept in TDT and NTDT, as well as curative therapies for TDT, including hematopoietic stem cell transplant (HSCT) and gene therapy. Patients with thalassemia had worse HRQOL scores compared to the general population, and chronic pain was seen to increase in frequency and severity over time with age. NTDT patients reported worse physical health and functioning, mental health, general health, and vitality than TDT patients. However, TDT patients reported worse pain, change in health, and social support than NTDT. Most therapies improved overall HRQOL among thalassemia patients. Deferasirox, an oral iron chelator, was associated with more HRQOL benefits compared to deferoxamine, an intravenous iron chelator. Luspatercept showed clinically meaningful improvement in physical functioning among TDT and NTDT. Furthermore, HSCT and gene therapy were associated with better physical, emotional, and mental domains scores. [ABSTRACT FROM AUTHOR]

Summary: Sickle cell disease (SCD) is an inherited red blood cell disorder associated with frequent painful events and organ damage. Hydroxyurea (HU) is the recommended evidence‐based treatment of SCD. However, among patients eligible for HU, prescription rates are low. Utilizing a scoping review approach, we summarized and synthesized relevant findings regarding provider barriers and facilitators to the prescription of HU in youth and adults with SCD and provided suggestions for future implementation strategies to improve prescription rates. Relevant databases were searched using specified search terms. Articles reporting provider barriers and/or facilitators to prescribing HU were included. A total of 10 studies met the inclusion criteria. Common barriers to the prescription of HU identified by providers included: doubts around patients' adherence to HU and their engaging in required testing, concerns about side effects, lack of knowledge, cost and patient concerns about side effects. Facilitators to the prescription of HU included beliefs in the effectiveness of HU, provider demographics and knowledge. Findings suggest significant provider biases exist, particularly in the form of negative perceptions towards patients' ability to adhere to taking HU and engaging in the required follow‐up. Improving provider knowledge and attitudes towards HU and SCD may help improve low prescription rates. [ABSTRACT FROM AUTHOR]

BackgroundSuboptimal adherence to 6-mercaptopurine (6-MP) is prevalent in pediatric acute lymphoblastic leukemia (ALL) and associated with increased risk of relapse. Rapid uptake of personal technology makes mobile health (mHealth) an attractive platform to promote adherence. ObjectiveStudy objectives were to examine access to mobile technology and preferences for an mHealth intervention to improve medication adherence in pediatric ALL. MethodsA cross-sectional survey was administered in oncology clinic to parents of children with ALL as well as adolescents and young adults (AYAs) with ALL receiving maintenance chemotherapy. ResultsA total of 49 parents (median age [IQR] 39 [33-42] years; female 76% [37/49]) and 15 patients (median age [IQR] 17 [16-19]; male 80% [12/15]) participated. All parents and AYAs owned electronic tablets, smartphones, or both. Parents’ most endorsed mHealth app features included a list of medications (71%, 35/49), information about 6-MP (71%, 35/49), refill reminders (71%, 35/49), and reminders to take 6-MP (71%, 35/49). AYAs' most endorsed features included refill reminders (73%, 11/15), reminders to take 6-MP (73%, 11/15), and tracking 6-MP (73%, 11/15). ConclusionsParents and AYAs reported ubiquitous access to mobile technology and strong interest in multiple adherence-specific mHealth app features. Parents and AYAs provided valuable insight into preferred features for a multifunctional behavioral intervention (mHealth app) to promote medication adherence in pediatric ALL.

School closures, altered access to health services, and economic stress during the COVID-19 pandemic have likely had an impact on the mental and physical well-being of youth worldwide, particularly among those with chronic health conditions (CHCs). A number of challenges and opportunities have emerged during the COVID-19 pandemic for youth with CHCs. Challenges include heightened anxiety, disrupted routines, academic and social stresses associated with school closure, increased risk of domestic violence and abuse, and reduced access to physical and psychosocial support. On the other hand, opportunities include reduced academic and social stress, increased time with families, reduced access to substances, easier access to health care using technology, and opportunities to build resilience. This viewpoint paper highlights both challenges and opportunities for youth with CHCs during the pandemic and offers recommendations for further research and clinical care.

The global spread of the coronavirus disease (COVID-19) outbreak poses a public health threat and has affected people worldwide in various unprecedented ways, both personally and professionally. There is no question that the current global COVID-19 crisis, now more than ever, is underscoring the importance of leveraging digital approaches to optimize pediatric health care delivery in the era of this pandemic. In this perspective piece, we highlight some of the available digital approaches that have been and can continue to be used to streamline remote pediatric patient care in the era of the COVID-19 pandemic, including but not limited to telemedicine. JMIR Pediatrics and Parenting is currently publishing a COVID-19 special theme issue in which investigators can share their interim and final research data related to digital approaches to remote pediatric health care delivery in different settings. The COVID-19 pandemic has rapidly transformed health care systems worldwide, with significant variations and innovations in adaptation. There has been rapid expansion of the leveraging and optimization of digital approaches to health care delivery, particularly integrated telemedicine and virtual health. Digital approaches have played and will play major roles as invaluable and reliable resources to overcome restrictions and challenges imposed during the COVID-19 pandemic and to increase access to effective, accessible, and consumer-friendly care for more patients and families. However, a number of challenges remain to be addressed, and further research is needed. Optimizing digital approaches to health care delivery and integrating them into the public health response will be an ongoing process during the current COVID-19 outbreak and during other possible future pandemics. Regulatory changes are essential to support the safe and wide adoption of these approaches. Involving all relevant stakeholders in addressing current and future challenges as well as logistical, technological, and financial barriers will be key for success. Future studies should consider evaluating the following research areas related to telemedicine and other digital approaches: cost-effectiveness and return on investment; impact on quality of care; balance in use and number of visits needed for the management of both acute illness and chronic health conditions; system readiness for further adoption in other settings, such as inpatient services, subspecialist consultations, and rural areas; ongoing user-centered evaluations, with feedback from patients, families, and health care providers; strategies to optimize health equity and address disparities in access to care related to race and ethnicity, socioeconomic status, immigration status, and rural communities; privacy and security concerns for protected health information with Health Insurance Portability and Accountability Act (HIPAA)–secured programs; confidentiality issues for some specific populations, especially adolescents and those in need of mental health services; early detection of exposure to violence and child neglect; and integration of training into undergraduate and graduate medical education and subspecialty fellowships. Addressing these research areas is essential to understanding the benefits, sustainability, safety, and optimization strategies of telemedicine and other digital approaches as key parts of modern health care delivery. These efforts will inform long-term adoption of these approaches with expanded dissemination and implementation efforts.

BackgroundSickle cell disease (SCD) is an inherited blood disorder causing acute complications and chronic progressive end organ damage. SCD is associated with significant morbidity, early mortality, impaired health-related quality of life, and increased acute health care utilization. Hydroxyurea is a US Food and Drug Administration–approved medication that reduces disease complications, acute health care utilization, and costs. However, adherence to hydroxyurea is suboptimal. Mobile health (mHealth) interventions have the potential to improve hydroxyurea adherence, but few examples exist that are specific to the SCD population. ObjectiveThis study aimed to design a mHealth intervention for individuals with SCD to improve adherence to hydroxyurea, using a user-centered design that was informed by specific barriers to hydroxyurea adherence and utilization in this population. MethodsThis study consisted of 4 phases. In phase 1, individuals with SCD and health care providers participated in an optimization digital workshop. In phase 2, patients completed surveys pertaining to their interest in mHealth use, barriers and facilitators to hydroxyurea use, and health literacy. Phases 3 and 4 involved semistructured interviews and focus groups, respectively, and used the Health Belief Model (HBM) as the framework to investigate drivers of poor hydroxyurea adherence and to inform the development of an app prototype. In addition, in phase 4, we have incorporated the patients’ feedback on the preliminary app prototype and its features. ResultsBarriers to hydroxyurea adherence were consistent with the literature and included forgetfulness and several specific thoughts and emotions associated with hydroxyurea use (eg, fear of side effects, depression, stigma, and hopelessness). In addition, more than half of the participants reported potentially low health literacy. Preferred patient app features included 7 key components, namely (1) medication reminders and tracker, (2) disease education, (3) communication, (4) personalization, (5) motivation, (6) support during pain episodes, and (7) social support. Utilizing a user-centered design approach, data obtained from patients and providers were translated into features within the app, mapping to components of the HBM and the specific drivers of hydroxyurea adherence and matching the literacy level of the population, resulting in the development of a novel mobile app called InCharge Health. ConclusionsThe InCharge Health app is an mHealth intervention developed with substantial input from users and by mapping the HBM as the framework that guided the choice for its components. InCharge Health is a customized product for the SCD population aimed at optimizing medication adherence, with the end goal of improving quality of life and health outcomes among patients with SCD. The efficacy and implementation of the InCharge Health app as an mHealth intervention to promote hydroxyurea adherence will be tested in a future stepped-wedge multicenter trial for adolescents and adults with SCD.

BackgroundUnintentional medication nonadherence is common and has been associated with poor health outcomes and increased health care costs. Earlier research demonstrated a relationship between habit strength and medication adherence. Previous research also examined a habit’s direct effect on adherence and how habit interacts with more conscious factors to influence or overrule them. However, the relationship between habit and adherence and the role of habit-based mobile health (mHealth) interventions remain unclear. ObjectiveThis review aimed to systematically evaluate the most recent evidence for habit strength, medication adherence, and habit-based mHealth interventions across chronic medical conditions. MethodsA keyword search with combinations of the terms habit, habit strength, habit index, medication adherence, and medication compliance was conducted on the PubMed database. After duplicates were removed, two authors conducted independent abstract and full-text screening. The guidelines for the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) were followed when reporting evidence across the included and reviewed studies. ResultsOf the 687 records examined, 11 met the predefined inclusion criteria and were finalized for data extraction, grading, and synthesis. Most included studies (6/11, 55%) were cross-sectional and used a theoretical model (8/11, 73%). The majority of studies measured habit strength using the self-report habit index and self-report behavioral automaticity index (9/11, 82%). Habit strength was positively correlated with medication adherence in most studies (10/11, 91%). Habit mediated the effects of self-efficacy on medication adherence (1/11, 9%), and social norms moderated the effects of habit strength on medication adherence (1/11, 9%). Habit strength also moderated the effects of poor mental health symptoms and medication adherence (1/11, 9%). None of the included studies reported on using or proposing a habit-based mHealth behavioral intervention to promote medication adherence. ConclusionsHabit strength was strongly correlated with medication adherence, and stronger habit was associated with higher medication adherence rates, regardless of the theoretical model and/or guiding framework. Habit-based interventions should be used to increase medication adherence, and these interventions could leverage widely available mobile technology tools such as mobile apps or text messaging, and existing routines.

The prediction of asphalt mixture dynamic modulus (E*) was investigated based on 1128 E* measurements, using three regression and thirteen machine learning models. Asphalt binder properties and mixture volumetrics were characterized using the same feeding features in the NCHRP 1-37A Witczak model. However, three aggregate gradation characterization approaches were involved in both modelling techniques: the NCHRP 1-37A gradation parameters, Weibull distribution factors, and Bailey method parameters. This study evaluated the performance of these models based on various performance indicators, using both statistical and machine learning regression modeling techniques. K-fold cross-validation and learning curve analysis were conducted to assess the models' generalization capabilities. The conclusions of this study demonstrate the superiority of the ML models, particularly the Catboost ensemble learning regression (CbR). Hyperparameter optimization and residual analysis were performed to fine-tune and confirm the heteroscedasticity of the CbR model. The Bailey-based CbR model showed the highest coefficient of determination (R2) of 0.998 and the lowest root mean square error (RMSE) of 220 MPa. Moreover, SHAP values interpreted the CbR model and showed the relative importance of its feeding features. Based on the findings of this study, the CbR model is suggested to accurately predict E* for a variety of asphalt mixtures. This information can be used to improve pavement design and construction, leading to more durable and long-lasting pavements. [ABSTRACT FROM AUTHOR]

This research study aims to investigate the feasibility of incorporating high-density polyethylene waste (HDPEW) into bitumen applications. Two conventional conditions of bitumen, namely, aged bitumen (AB) and virgin bitumen (VB), are rejuvenated and modified, respectively, using post-consumer HDPEW sourced out of bottle crates. The outcome (Pyro oil, PO-HDPEW) of the pyrolysis thermochemical process is used by 10, 20, and 30% to rejuvenate AB, while the fine-ground granules (FG) (FG-HDPEW) are used by 2, 3, 4, and 5% to modify the VB with different percentages. Physical and rheological characterization testing, including penetration, softening point temperature, and rotational viscosity (RV), is conducted to evaluate the performance of the HDPEW-rejuvenated and -modified binders and optimize both rejuvenator and modifier percentages. In addition, physical and chemical tests, including scanning electron microscopy (SEM), energy dispersive X-ray spectroscopy (EDX), and Fourier transform infrared spectroscopy (FTIR) are conducted to analyze the composition, distribution of surface contaminants, and the molecular structure of the bitumen, based on their respective wavelengths. Moreover, advanced mechanical and rheological tests, including dynamic shear rheometer (DSR), multiple stress creep and recovery (MSCR), and linear amplitude sweep (LAS) tests, are conducted to investigate the susceptibility of the rejuvenated and modified bitumen with HDPEW to rutting and fatigue cracking. The testing results demonstrate that the addition of PO-HDPEW to AB and FG-HDPEW modification of VB can enhance the physical, chemical, mechanical, and rheological properties of bitumen; however, this study recommends further research on the aging performance of the PO-HDPEW-rejuvenated bitumen. This research provides insights into using HDPEW as a cost-effective and eco-friendly rejuvenator and modifier on bitumen properties, which can aid in the longevity and performance of pavements. [ABSTRACT FROM AUTHOR]

BackgroundIn recent years, wearable devices have become increasingly attractive and the health care industry has been especially drawn to Google Glass because of its ability to serve as a head-mounted wearable device. The use of Google Glass in surgical settings is of particular interest due to the hands-free device potential to streamline workflow and maintain sterile conditions in an operating room environment. ObjectiveThe aim is to conduct a systematic evaluation of the literature on the feasibility and acceptability of using Google Glass in surgical settings and to assess the potential benefits and limitations of its application. MethodsThe literature was searched for articles published between January 2013 and May 2017. The search included the following databases: PubMed MEDLINE, Embase, Cumulative Index to Nursing and Allied Health Literature, PsycINFO (EBSCO), and IEEE Xplore. Two reviewers independently screened titles and abstracts and assessed full-text articles. Original research articles that evaluated the feasibility, usability, or acceptability of using Google Glass in surgical settings were included. This review was completed following the Preferred Reporting Results of Systematic Reviews and Meta-Analyses guidelines. ResultsOf the 520 records obtained, 31 met all predefined criteria and were included in this review. Google Glass was used in various surgical specialties. Most studies were in the United States (23/31, 74%) and all were conducted in hospital settings: 29 in adult hospitals (29/31, 94%) and two in children’s hospitals (2/31, 7%). Sample sizes of participants who wore Google Glass ranged from 1 to 40. Of the 31 studies, 25 (81%) were conducted under real-time conditions or actual clinical care settings, whereas the other six (19%) were conducted under simulated environment. Twenty-six studies were pilot or feasibility studies (84%), three were case studies (10%), and two were randomized controlled trials (6%). The majority of studies examined the potential use of Google Glass as an intraoperative intervention (27/31, 87%), whereas others observed its potential use in preoperative (4/31, 13%) and postoperative settings (5/31, 16%). Google Glass was utilized as a videography and photography device (21/31, 68%), a vital sign monitor (6/31, 19%), a surgical navigation display (5/31, 16%), and as a videoconferencing tool to communicate with remote surgeons intraoperatively (5/31, 16%). Most studies reported moderate or high acceptability of using Google Glass in surgical settings. The main reported limitations of using Google Glass utilization were short battery life (8/31, 26%) and difficulty with hands-free features (5/31, 16%). ConclusionsThere are promising feasibility and usability data of using Google Glass in surgical settings with particular benefits for surgical education and training. Despite existing technical limitations, Google Glass was generally well received and several studies in surgical settings acknowledged its potential for training, consultation, patient monitoring, and audiovisual recording.

The number of children and adolescents with chronic health conditions (CHCs) has doubled over the past two decades. Medication adherence is a key component of disease management within these groups. Low adherence to prescribed medications is a known problem in adolescents with CHCs and is related to health outcomes, including quality of life, disease complications, and mortality. Adolescence is a critical time to create routines and health behaviors that optimize disease self-management and transition to adult care. The mounting interest in the development and use of mobile health tools provides novel opportunities to connect patients, particularly adolescents, with their providers outside of the clinic and to improve health outcomes. There is growing evidence to support the efficacy of technology-based approaches, in particular text-messaging and mobile apps, to improve adherence behavior in adolescents, although cost-effectiveness and long-term health benefits remain unclear. In this short viewpoint article, we review some important considerations for promoting medication adherence in adolescents with CHCs using technology-based approaches.

BackgroundWearable technologies provide users hands-free access to computer functions and are becoming increasingly popular on both the consumer market and in various industries. The medical industry has pioneered research and implementation of head-mounted wearable devices, such as Google Glass. Most of this research has focused on surgical interventions; however, other medical fields have begun to explore the potential of this technology to support both patients and clinicians. ObjectiveOur aim was to systematically evaluate the feasibility, usability, and acceptability of using Google Glass in nonsurgical medical settings and to determine the benefits, limitations, and future directions of its application. MethodsThis review covers literature published between January 2013 and May 2017. Searches included PubMed MEDLINE, Embase, INSPEC (Ebsco), Cochrane Central Register of Controlled Trials (CENTRAL), IEEE Explore, Web of Science, Scopus, and Compendex. The search strategy sought all articles on Google Glass. Two reviewers independently screened titles and abstracts, assessed full-text articles, and extracted data from articles that met all predefined criteria. Any disagreements were resolved by discussion or consultation by the senior author. Included studies were original research articles that evaluated the feasibility, usability, or acceptability of Google Glass in nonsurgical medical settings. The preferred reporting results of systematic reviews and meta-analyses (PRISMA) guidelines were followed for reporting of results. ResultsOf the 852 records examined, 51 met all predefined criteria, including patient-centered (n=21) and clinician-centered studies (n=30). Patient-centered studies explored the utility of Google Glass in supporting patients with motor impairments (n=8), visual impairments (n=5), developmental and psychiatric disorders (n=2), weight management concerns (n=3), allergies (n=1), or other health concerns (n=2). Clinician-centered studies explored the utility of Google Glass in student training (n=9), disaster relief (n=4), diagnostics (n=2), nursing (n=1), autopsy and postmortem examination (n=1), wound care (n=1), behavioral sciences (n=1), and various medical subspecialties, including, cardiology (n=3), radiology (n=3), neurology (n=1), anesthesiology (n=1), pulmonology (n=1), toxicology (n=1), and dermatology (n=1). Most of the studies were conducted in the United States (40/51, 78%), did not report specific age information for participants (38/51, 75%), had sample size

The medical needs of individuals with sickle cell disease (SCD) are complex. Patients with SCD experience complications such as recurrent pain episodes and increased hospitalizations. Over 70% of AYA and their parents seek medical information from the Internet; 83% inquire on sites that have interactive/social features, such as Facebook or Twitter, yet accuracy remains unclear. Our objective was to assess the accuracy of the SCD-information posted on social media. We hypothesized that most of the posted information is inaccurate. We coded one month of threads from two common SCD Facebook groups (Sickle Cell Warriors Unity and Sickle Cell Anemia) to identify the purpose of each post and the accuracy of medical information posted. Amongst both social media sites, there were 487 posts. Most of the posts were directed toward socializations (n = 311, 63.8%), while other posts mainly focused mainly on SCD and its management (n = 173, 35.5%). When looking at the medical posts, 44.9% were accurate, whereas 55.1% of the posts included inaccurate information. We found that less than half of the medical information posted on interactive social media is inaccurate. Our findings raise potentially serious implications for individuals with SCD and/or their caregivers who may rely on social media to gather more information about their or their child's disease. Our data highlight the importance of health care providers encouraging patients and parents to ask any questions they may have about SCD, given they may consult social media and Internet site that provide inaccurate information. [ABSTRACT FROM AUTHOR]

BackgroundThe number of adolescents with chronic health conditions (CHCs) continues to increase. Medication nonadherence is a global challenge among adolescents across chronic conditions and is associated with poor health outcomes. While there has been growing interest in the use of mHealth technology to improve medication adherence among adolescents with CHCs, particularly text messaging and mobile phone apps, there has been no prior systematic review of their efficacy. ObjectiveThe purpose of this review was to systematically evaluate the most recent evidence for the efficacy of text messaging and mobile phone apps as interventions to promote medication adherence among adolescents with CHCs. MethodsPubMed, Embase, CENTRAL, PsycINFO, Web of Science, Google Scholar, and additional databases were searched from 1995 until November 2015. An additional hand search of related themes in the Journal of Medical Internet Research was also conducted. The Preferred Reporting Results of Systematic Reviews and Meta-Analyses guidelines were followed. Two reviewers independently screened titles/abstracts, assessed full-text articles, extracted data from included articles, and assessed their quality using Grades of Recommendation, Assessment, Development, and Evaluation criteria. Included studies were described in original research articles that targeted adherence in adolescents with CHCs (12-24 years-old). ResultsOf the 1423 records examined, 15 met predefined criteria: text messaging (n=12) and mobile phone apps (n=3). Most studies were performed in the United States (11/15, 73%), were randomized-controlled trials (8/15, 53%), had a sample size

BackgroundMany preventable behaviors contribute to adolescent mortality and morbidity. Non-adherence to preventive measures represents a challenge and has been associated with worse health outcomes in this population. The widespread use of electronic communication technologies by adolescents, particularly the use of text messaging (short message service, SMS) and mobile phones, presents new opportunities to intervene on risk and preventive risk behavior, but little is known about their efficacy. ObjectiveThis study aimed to systematically evaluate evidence for the efficacy of text messaging and mobile phone app interventions to improve adherence to preventive behavior among adolescents and describe intervention approaches to inform intervention development. MethodsThis review covers literature published between 1995 and 2015. Searches included PubMed, Embase, CENTRAL, PsycINFO, CINAHL, INSPEC, Web of Science, Google Scholar, and additional databases. The search strategy sought articles on text messaging and mobile phone apps combined with adherence or compliance, and adolescents and youth. An additional hand search of related themes in the Journal of Medical Internet Research was also conducted. Two reviewers independently screened titles and abstracts, assessed full-text articles, and extracted data from articles that met inclusion criteria. Included studies reflect original research—experimental or preexperimental designs with text messaging or mobile phone app interventions—targeting adherence to preventive behavior among adolescents (12-24 years old). The preferred reporting items of systematic reviews and meta-analyses (PRISMA) guidelines were followed for reporting results, and findings were critically appraised against the Oxford Centre for Evidence-based Medicine criteria. ResultsOf 1454 records, 19 met inclusion criteria, including text messaging (n=15) and mobile phone apps (n=4). Studies targeted clinic attendance, contraceptive use, oral health, physical activity and weight management, sun protection, human papillomavirus (HPV) vaccination, smoking cessation, and sexual health. Most studies were performed in the United States (47%, 9/19), included younger adolescents (63%, 12/19), and had sample size

BackgroundThe rate of chronic health conditions (CHCs) in children and adolescents has doubled in the past 20 years, with increased health care costs. Technology-based interventions have demonstrated efficacy to improving medication adherence. However, data to support the cost effectiveness of these interventions are lacking. ObjectiveThe objective of this study is to conduct an economic evaluation of text-messaging and smartphone-based interventions that focus on improving medication adherence in adolescents with CHCs. MethodsSearches included PubMed MEDLINE, Embase, Cochrane Central Register of Controlled Trials, Cumulative Index to Nursing and Allied Health Literature, PsycINFO, Web of Science, and Inspec. Eligibility criteria included age (12-24 years old), original articles, outcomes for medication adherence, and economic outcomes. ResultsOur search identified 1118 unique articles that were independently screened. A total of 156 articles met inclusion criteria and were then examined independently with full-text review. A total of 15 articles met most criteria but lacked economic outcomes such as cost effectiveness or cost-utility data. No articles met all predefined criteria to be included for final review. Only 4 articles (text messaging [n=3], electronic directly observed therapy [n=1]) described interventions with possible future cost-saving but no formal economic evaluation. ConclusionsThe evidence to support the cost effectiveness of text-messaging and smartphone-based interventions in improving medication adherence in adolescents with CHCs is insufficient. This lack of research highlights the need for comprehensive economic evaluation of such interventions to better understand their role in cost-savings while improving medication adherence and health outcomes. Economic evaluation of technology-based interventions can contribute to more evidence-based assessment of the scalability, sustainability, and benefits of broader investment of such technology tools in adolescents with CHCs.

Sickle cell disease (SCD) state level surveillance data are limited. We performed a retrospective review of emergency department (ED) visits and hospitalizations from individuals with SCD in Illinois (2016–2020) using the Illinois Health and Hospital Association's Comparative Health Care and Hospital Data Reporting Services. There were 48,094 outpatient ED visits and 31,686 hospitalizations. Most visits (67%) occurred in Cook County, were covered by public insurance (77%) and were from individuals with medium high (40.3%) or high (36.1%) poverty levels. SCD healthcare utilization remains high and surveillance data may inform SCD program development and resource allocation at the state level. Abbreviations CDC Centers for Disease Control and Prevention ED Emergency Department FDA Food & Drug Administration ICD International Classification of Diseases IL Illinois SCD Sickle cell disease [ABSTRACT FROM AUTHOR]

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children and young adults. Treatment is long and involves 2–3 years of a prolonged maintenance phase composed of oral chemotherapies. Adherence to these medications is critical to achieving good outcomes. However, adherence is difficult to determine, as there is currently no consensus on measures of adherence or criteria to determine nonadherence. Furthermore, there have been few studies in pediatric B-ALL describing factors associated with nonadherence. Thus, we performed a systematic review of literature on oral chemotherapy adherence during maintenance therapy in ALL following PRISMA guidelines. Published studies demonstrated various objective and subjective methods of assessing adherence without generalizable definitions of nonadherence. However, the results of these studies suggested that nonadherence to oral maintenance chemotherapy was associated with increased risk of relapse. Future studies of B-ALL therapy should utilize a uniform assessment of adherence and definitions of nonadherence to better determine the impact of nonadherence on B-ALL outcomes and identify predictors of nonadherence that could yield targets for adherence improving interventions. [ABSTRACT FROM AUTHOR]

Sickle cell disease (SCD) results in severe complications, such as anaemia and pain episodes. Hydroxyurea (HU) is efficacious in SCD, yet adherence remains low. To assess the relationship of HU adherence to health care utilization and patients' characteristics. This is a 5-year retrospective chart review. Patients' demographics and medical history were collected from the electronic medical record (EMR). HU adherence was evaluated using foetal haemoglobin "HbF%", mean corpuscular volume "MCV", and absolute neutrophil count "ANC". Age groups included children (<12 years), adolescents (12–17 years), and young adults (≥18 years). A total of 113 SCD patients on HU were included (median age 14 years, IQR 10–20; 50% female; 88% HbSS). Young adults had significantly higher HU adherence compared to adolescents and children, including higher median HbF% (24.2 vs. 12.4 vs. 8.6, p =.003), MCV (fl) (106.4 vs. 96.2 vs. 95.4, p =.01) and lower ANC (103/ml) (3.25 vs. 4.9 vs. 4.2, p =.01), respectively. Patients with chronic pain had lower HU adherence (HbF% 15.3 vs. 10.7, p =.04; ANC 3.6 vs. 6.3, p =.002; MCV 102.3 vs. 93.1, p =.1). Patients with higher HbF or MCV and lower ANC had significantly less frequent emergency room visits (rs=–0.26, p =.01; rs=–0.23, p =.01; rs=0.24, p =.01) and hospitalizations (rs=–0.27, p =.01; rs=–0.31, p =.01; rs=0.21, p =.02) as well as shorter length of stays (rs=–0.27, p =.0045; rs=–.34, p = 0.004; rs=0.23, p =.02), respectively. Similar trends in HU adherence and health care utilization were seen in subgroup analysis of only HbSS patients. There was no significant association of HU adherence to patients' sex, socio-economic status, distance from hospital, and HU duration. Young adults with SCD had significantly higher HU adherence compared to children and adolescents. Patients with lower HU adherence and/or chronic pain had increased health care utilization. Future studies examining barriers to adherence and evaluating interventions to optimize HU adherence in SCD are warranted. Young adults with SCD had significantly higher HU adherence, as reflected in their laboratory markers, compared to children and adolescents. Patients with higher HU adherence and/or those without chronic pain had lower or less frequent health care utilization. No significant association of HU adherence to patients' sex, socio-economic status and distance from hospital. [ABSTRACT FROM AUTHOR]

Iron chelation therapy (ICT) is essential to prevent complications of iron overload in patients with transfusion-dependent thalassaemia. However, there is currently no standard for how to best measure adherence to ICT, nor what level of adherence necessitates concern for poor outcomes, especially in paediatric patients. The objectives of this review are to identify rates of adherence to ICT, predictors of adherence, methods of measurement, and adherence-related health outcomes in children and adolescents. This review covers the literature published between 1980 and 2020 on ICT in thalassaemia that assessed adherence or compliance. Included studies reflect original research. The preferred reporting items of systematic reviews and meta-analyses (PRISMA) guidelines were followed for reporting results, and the findings were critically appraised with the Oxford Centre for Evidence-based Medicine criteria. Of the 543 articles, 37 met the inclusion criteria. The most common methods of assessing adherence included patient self-report (n = 15/36, 41.7%), and pill count (n = 15/36, 41.7%), followed by subcutaneous medication monitoring (5/36, 13.8%) and prescription refills (n = 4/36, 11.1%). Study sizes ranged from 7 to 1115 participants. Studies reported adherence either in "categories" with different levels of adherence (n = 29) or "quantitatively" as a percentage of medication taken out of those prescribed (n = 7). Quantitatively, the percentage of adherence varied from 57% to 98.4% with a median of 89.5%. Five studies focussed on interventions, four of which were designed to improve adherence. Studies varied in sample size and methods of assessment, which prohibited performing a meta-analysis. Due to a lack of clinical consensus on how adherence is defined, it is difficult to compare adherence to ICT in different studies. Future studies should be aimed at creating guidelines for assessing adherence and identifying suboptimal adherence. These future efforts will be crucial in informing evidence-based interventions to improve adherence and health outcomes in thalassaemia patients. Predictive factors associated with ICT adherence in the paediatric population include age, social perception of ICT, social support, and side effects/discomfort. Increased adherence in the paediatric population is associated with decreased serum ferritin and improved cardiac, hepatic, and endocrine outcomes. Inadequate adherence to ICT is associated with increased lifetime health costs. There are few studies that focussed on interventions to increase adherence in the paediatric population, and the studies that do exist all focussed on different types of interventions; successful interventions focussed on consistent, long-term engagement with patients. [ABSTRACT FROM AUTHOR]

Sickle-cell disease (SCD) is an inherited hemoglobinopathy, causing lifelong complications such as painful vaso-occlusive episodes, acute chest syndrome, stroke, chronic anemia, and end-organ damage, with negative effects on quality of life and life expectancy. Within the last five years, three new treatments have been approved: L-glutamine in 2017 and crizanlizumab and voxelotor in 2019. We conducted a literature search of these three medications, and of the 31 articles meeting inclusion criteria, 6 studied L-glutamine, 9 crizanlizumab, and 16 voxelotor. Treatment with L-glutamine was associated with decrease in pain crises, hospitalizations, and time to first and second crises, with a decrease in RBC transfusion rate. Barriers to filling and taking L-glutamine included insurance denial, high deductible, and intolerability, especially abdominal pain. Crizanlizumab was associated with a reduction in pain crises and time to first crisis, with reduction in need for opioid use. Adverse effects of crizanlizumab include headache, nausea, insurance difficulty, and infusion reactions. Voxelotor was associated with increased hemoglobin and decreased markers of hemolysis. Barriers for voxelotor use included insurance denial and side effects such as headache, rash, and diarrhea. These three medications represent exciting new therapies and are generally well-tolerated though price and insurance approval remain potential barriers to access. Other studies are ongoing, particularly in the pediatric population, and more real-world studies are needed. The objective of this article is to evaluate post-approval studies of crizanlizumab, voxelotor, and L-glutamine in SCD, with a focus on real-world efficacy, side effects, and prescribing data. [ABSTRACT FROM AUTHOR]