Social determinants of health and treatment center affiliation: analysis from the sickle cell disease implementation consortium registry.

BACKGROUND: Adults with sickle cell disease (SCD) suffer early mortality and high morbidity. Many are not affiliated with SCD centers, defined as no ambulatory visit with a SCD specialist in 2 years. Negative social determinants of health (SDOH) can impair access to care. HYPOTHESIS: Negative SDOH are more likely to be experienced by unaffiliated adults than adults who regularly receive expert SCD care. METHODS: Cross-sectional analysis of the SCD Implementation Consortium (SCDIC) Registry, a convenience sample at 8 academic SCD centers in 2017-2019. A Distressed Communities Index (DCI) score was assigned to each registry member's zip code. Insurance status and other barriers to care were self-reported. Most patients were enrolled in the clinic or hospital setting. RESULTS: The SCDIC Registry enrolled 288 Unaffiliated and 2110 Affiliated SCD patients, ages 15-45y. The highest DCI quintile accounted for 39% of both Unaffiliated and Affiliated patients. Lack of health insurance was reported by 19% of Unaffiliated versus 7% of Affiliated patients. The most frequently selected barriers to care for both groups were "previous bad experience with the healthcare system" (40%) and "Worry about Cost" (17%). SCD co-morbidities had no straightforward trend of association with Unaffiliated status. The 8 sites' results varied. CONCLUSION: The DCI economic measure of SDOH was not associated with Unaffiliated status of patients recruited in the health care delivery setting. SCDIC Registrants reside in more distressed communities than other Americans. Other SDOH themes of affordability and negative experiences might contribute to Unaffiliated status. Recruiting Unaffiliated SCD patients to care might benefit from systems adopting value-based patient-centered solutions.

"The project did not come to us with a solution": Perspectives of research teams on implementing a study about electronic health record-embedded individualized pain plans for emergency department treatment of vaso-occlusive episodes in adults with sickle cell disease.

BACKGROUND: This study aimed to capture the implementation process of the ALIGN Study, (An individualized Pain Plan with Patient and Provider Access for Emergency Department care of Sickle Cell Disease). ALIGN aimed to embed Individualized Pain Plans in the electronic health record (E-IPP) and provide access to the plan for both adult patients with sickle cell disease (SCD) and emergency department providers when a person with SCD comes to the emergency department in vaso-occlusive crises. METHODS: Semi-structured interviews were conducted with research teams from the 8 participating sites from the ALIGN study. Seventeen participants (principal investigators and study coordinators) shared their perspectives about the implementation of ALIGN in their sites. Data were analyzed in three phases using open coding steps adapted from grounded theory and qualitative content analysis. RESULTS: A total of seven overarching themes were identified: (1) the E-IPP structure (location and upkeep) and collaboration with the informatics team, (2) the role of ED champion, (3) the role of research coordinators, (4) research team communication, and communication between research team and clinical team, (5) challenges with the study protocol, (6) provider feedback: addressing over-utilizers, patient mistrust, and the positive feedback about the intervention, and (7) COVID-19 and its effects on study implementation. CONCLUSIONS: Findings from this study contribute to learning how to implement E-IPPs for adult patients with SCD in ED. The study findings highlight the importance of early engagement with different team members, a champion from the emergency department, study coordinators with different skills and enhancement of communication and trust among team members. Further recommendations are outlined for hospitals aiming to implement E-IPP for patients with SCD in ED.

Point-of-Care Ultrasonography for the Assessment of Dehydration in Children: A Systematic Review.

OBJECTIVES: Accurate estimation of the degree of dehydration remains a diagnostic challenge. The primary objective was to systematically review the literature on the role of ultrasound in assessment of the degree of dehydration in children. METHODS: Data sources included Ovid MEDLINE, Web of Science Core Collection, Current Index to Nursing and Allied Health Literature, Cochrane Library, ClinicalTrials.gov , and Trip Pro Database. Two independent reviewers used screening protocol to include articles on assessment of dehydration in children with the use of point-of-care ultrasonography (POCUS). The level of evidence was assessed in accordance with the "The Oxford 2011 Levels of Evidence." The Quality Assessment of Diagnostic Accuracy Studies-2 tool was used to evaluate risk of bias. RESULTS: We identified 108 studies, and 8 studies met our inclusion criteria. All studies were prospective cohort studies (level of evidence, 3-4). The authors of 5 studies used difference between ill weight and weight after rehydration as the reference standard for dehydration, and the authors of 3 studies used clinical dehydration scale. Two studies from the United States showed acceptable areas under the curve for inferior vena cava to aorta (IVC/Ao) diameter ratio at 0.72 and 0.73 for prediction of significant dehydration (>5% weight loss). The IVC/Ao ratio with cut-off at 0.8 had sensitivity of 67% and 86% and specificity of 71% and 56% for prediction of significant dehydration. Studies from the resource-limited settings were more heterogeneous. One study with acceptable risk of biases reported poor sensitivity (67%) and specificity (49%) of Ao/IVC ratio with cut-off of 2.0 for predicting severe dehydration (>9% weight loss) with area under the curve at 0.6. Three studies showed increase in IVC diameter with fluid resuscitation with mean change in IVC diameter by 30% in children with significant dehydration (>5% weight loss) and by 22% without significant dehydration (<5% weight loss). Metaanalysis was not completed due to high heterogeneity. CONCLUSIONS: This study showed that the quantity and quality of research on the application of POCUS for the assessment of dehydration in children is limited. There is no criterion standard for assessing the degree of dehydration and no universal definition of the degree of dehydration. Thus, more methodologically rigorous studies are required. Current systematic review does not support the routine use of US to determine the severity of dehydration in children. Despite these limitations, the use of POCUS in children with dehydration demonstrates potential. Given the clear increase in IVC size with rehydration, repeated IVC US scans may be helpful in guiding fluid resuscitation in children with dehydration. From different proposed US parameters, IVC/Ao ratio has better diagnostic accuracy in detecting significant dehydration than Ao/IVC ratio and IVC collapsibility index. Despite low to moderate diagnostic performance, US still showed better assessment of dehydration than physician gestalt and World Health Organization score.

Addition of Midthigh Circumference Improves Predictive Ability of Broselow Tape Weight Estimation.

OBJECTIVES: This study aimed to improve the accuracy of Broselow tape (BT) weight prediction by adding midthigh circumference (MTC) and to compare and standardize the methods for measuring MTC. METHODS: This prospective, observational study was conducted in a pediatric emergency department of a tertiary care children's hospital. Children up to 12 years of age presenting to emergency department were included. Children were excluded if obtaining the data would interfere with their acute management. The data collected included MTC, height, BT weight, and actual weight. Three models were built. Broselow tape-only model and MTC plus BT model used 2 methods for MTC measurements: visual approximation of thigh midpoint (visual MTC1) and Centers for Disease Control and Prevention (CDC)-defined method (CDC MTC2). RESULTS: A total of 430 children were enrolled during pre-coronavirus disease era. Of these, 234 were boys (54.4%) and 196 (45.6%) were girls. Data were categorized into children younger than 2 years (224 [52.1%]) and older than 2 years (206 [47.9%]). African American constituted 250 (58%); White, 136 (31%); and unspecified, 27 (6%). For the entire cohort, both models that included MTC were significantly better at weight prediction with larger adjusted R2 (visual MTC1, 0.921; CDC MTC2, 0.928) and smaller root mean squared (RMSE) (visual MTC1, 2.70; CDC MTC2, 2.56) compared with BT-only model (adjusted R2 = 0.843; RMSE, 3.80). Midthigh circumference plus BT models performed even better in children older than 2 years compared with BT-only model with adjusted R2 (visual MTC1, 0.859; CDC MTC2, 0.872 vs 0.616) and RMSE (visual MTC1, 3.18; CDC MTC2, 3.03 vs 5.27). CONCLUSIONS: The inclusion of MTC with BT resulted in a more accurate weight prediction in children especially greater than 2 years old. Midthigh circumference model using CDC-defined method was slightly better predictor of actual weight than visual approximation.

Outcomes of an Emergency Department Observation Unit-Based Pathway for the Treatment of Uncomplicated Vaso-occlusive Events in Sickle Cell Disease.

STUDY OBJECTIVE: This was a prospective, pre-post, 13-year observational study documenting the multiyear implementation of an observation unit sickle cell pathway for patients with uncomplicated vaso-occlusive events. METHODS: The sickle cell pathway begins with rapid triage to identify patients with uncomplicated vaso-occlusive events for immediate transfer to the observation unit and initiation of patient-controlled analgesia followed by repeated evaluations of pain and identification of other complications. Data were abstracted from the electronic medical record or observation unit database. The sickle cell pathway was initiated in April 2006. Major revisions of it were carried out in June 2009 (physician evaluation occurs in sickle cell pathway and only patient-controlled analgesia administration of medications) and October 2010 (multidisciplinary management and individual dosing). RESULTS: Annual ED visits ranged between 287 and 528. The preimplementation hospital admission rate was 33% (123/368), 3-day return rate 16% (60/368), and 30-day return rate 67% (248/368). Refinements to the sickle cell pathway have resulted in a decrease in admission rate to 20% (258/1276); 3-day return rate, to 3.6% (46/1,276); and 30-day return rate, to 41% (525/1,276) for the past 3 years. CONCLUSION: The use of a sickle cell pathway for the treatment of uncomplicated vaso-occlusive events has been effective in providing rapid treatment and reducing hospital admissions. However, it was not only the intervention and its refinement that made the sickle cell pathway successful. With the Consolidated Framework for Implementation Research, it was discerned that outer setting factors of organizational commitment to the care of patients with SCD, inner setting factors of learning climate and leadership engagement, individuals, and process contributed to the success of the sickle cell pathway.

A Systematic Review of Testicle Survival Time After a Torsion Event.

BACKGROUND: The time window for possible salvage and survival of a torsed testicle is commonly thought to be 6 to 8 hours. However, survival of torsed testicles with or without subsequent atrophy is known to occur outside that critical time window. In this article, we performed a systematic review of the English literature to provide a more accurate understanding of reported time frames of testicle survival after a torsion event. OBJECTIVES: The primary research question was to determine the relationship between time to treatment and the rate of survival for testicles of male patients presenting with testicular torsion (TT). METHODS: A systematic review of the literature was performed and structured according to PRISMA guidelines. An exhaustive library search was performed after search strategies were developed for multiple databases that included PubMed, Cochrane library, Ovid MEDLINE, Web of Science, and ProQuest Theses and Dissertations. Two different searches were developed including "testicular torsion" and TT with the search term "time" added. Articles specifically reporting TT case series, testicle outcomes, and time to surgical or manual treatment were selected for review. In addition to and preceding the systematic review, an exhaustive manual search of the literature was also performed by the authors. As a result of these searches, a total of 30 studies with data considered relevant to the research question were included. The information extracted from the articles was tabulated with regard to time intervals to treatment and survival outcome. RESULTS: The systematic review process and protocol are reported in this article. A total of 30 studies were found that reported case series of TT patients and their outcomes as well as time to treatment reported in useful time frames. From these reports, a total of 2116 TT patients were culled, and their outcomes and time to treatment are reported. Because the time to treatment was reported variously in different case series, the 3 most common formats for reporting time to treatment and outcome were used. When overlap between the tables existed, the data were tallied and reported cumulatively. When reported in 6-hour intervals (1,283 patients), survival at 0 to 6 hours was 97.2%; 7 to 12 hours, 79.3%; 13 to 18 hours, 61.3%; 19 to 24 hours, 42.5%; 25 to 48 hours, 24.4%; and greater than 48 hours, 7.4%. Moreover, we reported cumulative survival data based on reporting for all 3 groups of patients. Testicular salvage in the first 12 hours is 90.4%, from 13 to 24 hours survival is 54.0%, and beyond 24 hours survival is 18.1%. Testicle survival after TT was significant beyond the commonly held 6 to 8 hour time frame and even after more than 24 hours of ischemia. CONCLUSIONS: Survival of the testicle irrespective of subsequent atrophy, decreased spermatogenesis or impaired endocrine function after TT can be much longer than the 6 to 8 hours that is commonly taught. Our systematic review of the literature demonstrates that survival percentages are significant even past 24 hours of torsion. This information should encourage aggressive management of patients presenting with TT pain that has been ongoing for many hours.

Impact of mentoring relationships on nursing professional socialization.

AIM: This study qualitatively explored the impact mentoring relationships had on the professional socialization of novice clinical nurse leader. BACKGROUND: Professional socialization entails acquisition of the skills, knowledge and values associated with nursing. Model C clinical nurse leaders have completed a bachelor's degree before graduate-level nursing programme acceptance. Thereby, the mentoring needs of model C clinical nurse leaders may differ from that of traditionally educated novice nurses. METHOD: Focus groups were conducted with seven novice model C clinical nurse leaders during their first year of employment. Qualitative data were analysed via a grounded theory approach. RESULTS: The participants described an intense focus on patient care and how multiple mentoring relationships motivated them to become competent bedside clinicians. They described how the mentors' actions enabled them to deal with negative feelings, which increased their confidence, comfort and competence with clinical skills. CONCLUSIONS: Clinical skills improved when a novice model C clinical nurse leader worked with multiple mentors. The qualitative data did not show that the model C clinical nurse leaders needed different mentoring relationships than traditionally educated nurses. IMPLICATION FOR NURSING MANAGEMENT: The results suggest multiple mentors should be used to develop the clinical competences of novice model C clinical nurse leaders.

Systematic Review of Occupational Therapy and Adult Cancer Rehabilitation: Part 2. Impact of Multidisciplinary Rehabilitation and Psychosocial, Sexuality, and Return-to-Work Interventions.

This article is the second part of a systematic review of evidence for the effectiveness of cancer rehabilitation interventions within the scope of occupational therapy that address the activity and participation needs of adult cancer survivors. This article focuses on the use of multidisciplinary rehabilitation and interventions that address psychosocial outcomes, sexuality, and return to work. Strong evidence indicates that multidisciplinary rehabilitation benefits cancer survivors and that psychosocial strategies can reduce anxiety and depression. Moderate evidence indicates that interventions can support survivors in returning to the level of sexuality desired and help with return to work. Part 1 of the review also appears in this issue.

Systematic Review of Occupational Therapy and Adult Cancer Rehabilitation: Part 1. Impact of Physical Activity and Symptom Management Interventions.

This article is the first part of a systematic review of evidence for the effectiveness of cancer rehabilitation interventions within the scope of occupational therapy that address the activity and participation needs of adult cancer survivors. This article focuses on the importance of physical activity and symptom management. Strong evidence supports the use of exercise for cancer-related fatigue and indicates that lymphedema is not exacerbated by exercise. Moderate evidence supports the use of yoga to relieve anxiety and depression and indicates that exercise as a whole may contribute to a return to precancer levels of sexual activity. The results of this review support inclusion of occupational therapy in cancer rehabilitation and reveal a significant need for more research to explore ways occupational therapy can positively influence the outcomes of cancer survivors. Part 2 of the review also appears in this issue.

Rapid initiation of nasal saline irrigation to reduce severity in high-risk COVID+ outpatients.

OBJECTIVE: To determine whether initiating saline nasal irrigation after COVID-19 diagnosis reduces hospitalization and death in high-risk outpatients compared with observational controls, and if irrigant composition impacts severity. METHODS: Participants 55 and older were enrolled within 24 hours of a + PCR COVID-19 test between September 24 and December 21, 2020. Among 826 screened, 79 participants were enrolled and randomly assigned to add 2.5 mL povidone-iodine 10% or 2.5 mL sodium bicarbonate to 240 mL of isotonic nasal irrigation twice daily for 14 days. The primary outcome was hospitalization or death from COVID-19 within 28 days of enrollment by daily self-report confirmed with phone calls and hospital records, compared to the CDC Surveillance Dataset covering the same time. Secondary outcomes compared symptom resolution by irrigant additive. RESULTS: Seventy-nine high-risk participants were enrolled (mean [SD] age, 64 [8] years; 36 [46%] women; 71% Non-Hispanic White), with mean BMI 30.3. Analyzed by intention-to-treat, by day 28, COVID-19 symptoms resulted in one ED visit and no hospitalizations in 42 irrigating with alkalinization, one hospitalization of 37 in the povidone-iodine group, (1.27%) and no deaths. Of nearly three million CDC cases, 9.47% were known to be hospitalized, with an additional 1.5% mortality in those without hospitalization data. Age, sex, and percentage with pre-existing conditions did not significantly differ by exact binomial test from the CDC dataset, while reported race and hospitalization rate did. The total risk of hospitalization or death (11%) was 8.57 times that of enrolled nasal irrigation participants (SE = 2.74; P = .006). Sixty-two participants completed daily surveys (78%), averaging 1.8 irrigations/day. Eleven reported irrigation-related complaints and four discontinued use. Symptom resolution was more likely for those reporting twice daily irrigation (X2 = 8.728, P = .0031) regardless of additive. CONCLUSION: SARS-CoV-2+ participants initiating nasal irrigation were over 8 times less likely to be hospitalized than the national rate.

Transition from pediatric to adult care in sickle cell disease: establishing evidence-based practice and directions for research.

Transition of young adults with sickle cell disease (SCD) from pediatric to adult medical care is an important priority, given medical advances that have transformed SCD into a lifelong chronic condition, rather than a disease of childhood. Successful transfer from pediatric to adult care has its foundation in collaboration among the young adult, the family, and the health care system to support building skills in positive disease management and independent living. Systemic issues in transition from pediatric to adult care for individuals with SCD include limited access to adult providers with the skills and/or interest in caring for people with SCD; poor communication and follow-up between pediatric and adult providers; and insurance coverage and reimbursement for care coordination. Family and patient issues in transition include lack of skill development for successful transition into adulthood; absence of financial independence; fear of the unknown; and increasing morbidity with age. The design and evaluation of successful transition programming in SCD requires clarity in conceptual frameworks and consistent measurement, both before and after transfer to adult care. Strategies used by three SCD transition programs and future directions for research and program development are presented.

Occupational therapy interventions for recovery in the areas of community integration and normative life roles for adults with serious mental illness: a systematic review.

This systematic review investigated research literature evaluating the effectiveness of occupational therapy interventions focusing on recovery in the areas of community integration and normative life roles for people with serious mental illness. The review included occupation- and activity-based interventions and interventions addressing performance skills and performance patterns, aspects of context and environment, activity demands, and client factors. The results indicated that the evidence of the effectiveness of social skills training is moderate to strong. The evidence for the effectiveness of life skills and instrumental activities of daily living (IADLs) training to improve performance is moderate, as is the evidence for neurocognitive training paired with skills training in the areas of work, social participation, and IADLs. The evidence for client-centered intervention and increased intensity and duration of treatment is limited but positive, and the evidence that providing intervention in the natural context is more beneficial than in the clinic setting is inconclusive.

Hydroxyurea Adherence Strategies for Persons with Sickle Cell Disease: A Systematic Review.

INTRODUCTION: Adherence to hydroxyurea is essential to modify the pathology of sickle cell disease. OBJECTIVE: To identify best strategies to support adherence to hydroxyurea in persons with sickle cell disease. METHODS: A systematic review was conducted. PubMed, Cumulative Index of Nursing and Allied Health Literature, PsycArticles, PsycInfo, Embase databases and Cochrane Library were searched for studies between 1998 to 2018. RESULTS: Six studies met inclusion criteria: two randomized controlled trials, three prospective studies, and one retrospective chart review. DISCUSSION: Few studies addressed strategies that tested adherence interventions primarily composed of behavioral change resulting in medication adherence. More studies on hydroxyurea adherence are needed.

Short-term insomnia disorder in health care workers in an academic medical center before and during COVID-19: rates and predictive factors.

STUDY OBJECTIVES: This study investigated risk factors and estimated rates of acute insomnia disorder in health care workers at the onset of the coronavirus disease 2019 (COVID-19) pandemic. METHODS: A Qualtrics survey of more than 2,300 health care providers was conducted in a single academic health system on May 15, 2020, including practicing attending physicians, residents and fellows in training, advanced practice providers, and nurses. Six hundred and sixty-eight responded (29% response rate). The survey employed the Research Diagnostic Criteria for insomnia disorder to diagnose acute insomnia disorder. RESULTS: Five hundred seventy-three respondents had no missing data pertaining to sleep, with a mean age of 43.4 ± 12.5 years and 72% women. The rate of insomnia disorder before COVID-19 was 44.5%, while after COVID-19 it was 64.0%. Of persons with insomnia disorder before COVID-19 10.2% stated it had resolved during COVID-19, while 43.4% of persons who did not have insomnia disorder before COVID-19 developed acute insomnia disorder during COVID-19 (χ² = 145.2; df = 1; P < .0001). New cases of acute insomnia disorder were related positively to female sex, advancing age, and less time spent in direct patient care. CONCLUSIONS: Acute insomnia disorder was exceptionally common in this sample of tertiary care health care workers. The effects of sex and age were similar to what has been generally described as risk factors for insomnia. The surprising finding that less time spent in direct patient care was associated with more cases of acute insomnia disorder might be explained by the poorly understood stresses of working from home during COVID-19.

Electronic Health Record-Embedded Individualized Pain Plans for Emergency Department Treatment of Vaso-occlusive Episodes in Adults With Sickle Cell Disease: Protocol for a Preimplementation and Postimplementation Study.

BACKGROUND: Individuals living with sickle cell disease often require aggressive treatment of pain associated with vaso-occlusive episodes in the emergency department. Frequently, pain relief is poor. The 2014 National Heart, Lung, and Blood Institute evidence-based guidelines recommended an individualized treatment and monitoring protocol to improve pain management of vaso-occlusive episodes. OBJECTIVE: This study will implement an electronic health record-embedded individualized pain plan with provider and patient access in the emergency departments of 8 US academic centers to improve pain treatment for adult patients with sickle cell disease. This study will assess the overall effects of electronic health record-embedded individualized pain plans on improving patient and provider outcomes associated with pain treatment in the emergency department setting and explore barriers and facilitators to the implementation process. METHODS: A preimplementation and postimplementation study is being conducted by all 8 sites that are members of the National Heart, Lung, and Blood Institute-funded Sickle Cell Disease Implementation Consortium. Adults with sickle cell disease aged 18 to 45 years who had a visit to a participating emergency department for vaso-occlusive episodes within 90 days prior to enrollment will be eligible for inclusion. Patients will be enrolled in the clinic or remotely. The target analytical sample size of this study is 160 patient participants (20 per site) who have had an emergency department visit for vaso-occlusive episode treatment at participating emergency departments during the study period. Each site is expected to enroll approximately 40 participants to reach the analytical sample size. The electronic health record-embedded individualized pain plans will be written by the patient's sickle cell disease provider, and sites will work with the local informatics team to identify the best method to build the electronic health record-embedded individualized pain plan with patient and provider access. Each site will adopt required patient and provider implementation strategies and can choose to adopt optional strategies to improve the uptake and sustainability of the intervention. The study is informed by the Technology Acceptance Model 2 and the Reach, Effectiveness, Adoption, Implementation, and Maintenance framework. Provider and patient baseline survey, follow-up survey within 96 hours of an emergency department vaso-occlusive episode visit, and selected qualitative interviews within 2 weeks of an emergency department visit will be performed to assess the primary outcome, patient-perceived quality of emergency department pain treatment, and additional implementation and intervention outcomes. Electronic health record data will be used to analyze individualized pain plan adherence and additional secondary outcomes, such as hospital admission and readmission rates. RESULTS: The study is currently enrolling study participants. The active implementation period is 18 months. CONCLUSIONS: This study proposes a structured, framework-informed approach to implement electronic health record-embedded individualized pain plans with both patient and provider access in routine emergency department practice. The results of the study will inform the implementation of electronic health record-embedded individualized pain plans at a larger scale outside of Sickle Cell Disease Implementation Consortium centers. TRIAL REGISTRATION: ClinicalTrials.gov NCT04584528; https://clinicaltrials.gov/ct2/show/NCT04584528. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/24818.

Mental health evidence in the American Journal of Occupational Therapy.

We reviewed the mental health articles published in the American Journal of Occupational Therapy (AJOT) from 2008 through 2009 in light of meeting the Centennial Vision charge of supporting practice through evidence. Seven articles that addressed mental health practice were published in AJOT over these years. Review of the articles found that only two addressed effectiveness of occupational therapy intervention; one was rated as Level II evidence, and the other was rated as Level V evidence. Two articles addressed instrument development and testing. Three articles were basic research studies that expanded consideration about the needs of people with mental health conditions. Scholars and clinicians have begun to embrace the charge of the Centennial Vision to support practice with evidence and continue to embrace mental health practice but have a distance to travel. We hope that in the coming years, the profession will see more evidence published in AJOT supporting mental health as practiced by occupational therapists.

Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization: Protocol for an Efficacy and Implementation Study.

BACKGROUND: Hydroxyurea prevents disease complications among patients with sickle cell disease (SCD). Although its efficacy has been endorsed by the National Health Lung and Blood Institute evidence-based guidelines, its adoption is low, both by patients with SCD and providers. Mobile health (mHealth) apps provide benefits in improving medication adherence and self-efficacy among patients with chronic diseases and have facilitated prescription among medical providers. However, mHealth has not been systematically tested as a tool to increase hydroxyurea adherence nor has the combination of mHealth been assessed at both patient and provider levels to increase hydroxyurea utilization. OBJECTIVE: This study aims to increase hydroxyurea utilization through a combined two-level mHealth intervention for both patients with SCD and their providers with the goals of increasing adherence to hydroxyurea among patients and improve hydroxyurea prescribing behavior among providers. METHODS: We will test the efficacy of 2 mHealth interventions to increase both patient and provider utilization and knowledge of hydroxyurea in 8 clinical sites of the NHLBI-funded Sickle Cell Disease Implementation Consortium (SCDIC). The patient mHealth intervention, InCharge Health, includes multiple components that address memory, motivation, and knowledge barriers to hydroxyurea use. The provider mHealth intervention, Hydroxyurea Toolbox (HU Toolbox), addresses the clinical knowledge barriers in prescribing and monitoring hydroxyurea. The primary hypothesis is that among adolescents and adults with SCD, adherence to hydroxyurea, as measured by the proportion of days covered (the ratio of the number of days the patient is covered by the medication to the number of days in the treatment period), will increase by at least 20% after 24 weeks of receiving the InCharge Health app, compared with their adherence at baseline. As secondary objectives, we will (1) examine the change in health-related quality of life, acute disease complications, perceived health literacy, and perceived self-efficacy in taking hydroxyurea among patients who use InCharge Health and (2) examine potential increases in the awareness of hydroxyurea benefits and risks, appropriate prescribing, and perceived self-efficacy to correctly administer hydroxyurea therapy among SCD providers between baseline and 9 months of using the HU Toolbox app. We will measure the reach, adoption, implementation, and maintenance of both the InCharge Health and the HU Toolbox apps using the reach, effectiveness, adoption, implementation, and maintenance framework and qualitatively evaluate the implementation of both mHealth interventions. RESULTS: The study is currently enrolling study participants. Recruitment is anticipated to be completed by mid-2021. CONCLUSIONS: If this two-level intervention, that is, the combined use of InCharge Health and HU Toolbox apps, demonstrates efficacy in increasing adherence to hydroxyurea and prescribing behavior in patients with SCD and their providers, respectively, both apps will be offered to other institutions outside the SCDIC through a future large-scale implementation-effectiveness study. TRIAL REGISTRATION: ClinicalTrials.gov NCT04080167; https://clinicaltrials.gov/ct2/show/NCT04080167. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/16319.

Impact of Red Blood Cell Antigen Matching on Alloimmunization and Transfusion Complications in Patients with Sickle Cell Disease: A Systematic Review.

Red blood cells (RBC) transfusion is critical in managing acute and chronic complications in sickle cell disease (SCD); however, it is complicated by RBC alloimmunization, iron overload, transfusion reactions and infection. Several reports documented an increased incidence of alloantibodies in transfused individuals with SCD, especially for Rh and Kell antigens. As a result, the National Institutes of Health Expert Panel and British Society for Haematology guidelines recommend primary matching for C/c, E/e and K antigens in addition to ABO/RhD for RBC transfusions. However, the evidence supporting these recommendations was cited as limited and understanding of alloimmunization in SCD is evolving. To examine the limitations of the evidence, we undertook a systematic review of evidence behind recommendations for limited and extended serologic and genotypic RBC antigen matching to reduce alloimmunization, autoimmunization and transfusion reactions. Searches of PubMed, Embase, Cochrane, and Web of Science databases using MeSH index and free text terms between 1976 through October 2015 and papers and captured through July 2016 through review references in papers, word of mouth, and ongoing Google Scholar and Medline Alerts identified 303 unique articles. Nineteen articles met inclusion criteria and were classified by the Oxford Centre Evidence Based levels of evidence. Strengthening the Reporting of Observational Studies in Epidemiology checklists were completed for 18 of the 19 studies. There were no prospective randomized controlled trials. Sixteen of the articles were cohort studies, two were cross-sectional studies, and one decision tree model examining costs. Low-quality evidence from observational cohort studies supports that alloimmunization prevalence can be decreased by extending serological RBC antigen matching. Transfusion reactions are generally poorly and inconsistently reported. There was no evidence reporting the effect prophylactic genotypic matching has on alloimmunization, autoimmunization or transfusion reactions. There were no studies comparing prophylactic genotypic matching to serologic matching. High-quality evidence was lacking to support clinical decision making regarding best transfusion practices. Multicenter prospective randomized clinical trials are needed to determine best strategies for reducing the rate of alloimmunization using serologic and genotypic matching.

Increased Intracranial Pressure Damages Optic Nerve Structural Support.

Optic nerve sheath diameter (ONSD) is used clinically as a noninvasive measure for elevated intracranial pressure (ICP). This study had two purposes: to investigate the immediate effects optic nerve sheath (ONS) dilation post-ICP increase on trabecular fibers connecting the optic nerve to the ONS and to document any changes in these fibers 30 days post-increased ICP. In a swine model, ICP was increased by inflating a Foley catheter balloon in the epidural space. Three control pigs received the catheter insertion without inflation (no increase in ICP) and four experimental pigs received the catheter with inflation (increased ICP). The control and two randomly selected pigs with increased ICP were euthanized immediately after the procedure. The two other pigs were euthanized 30 days post-catheter inflation. For all pigs, the ONS was removed and imaged using a scanning electron microscope, calculating percent porosity values. Porosity values for the experimental groups (Immediately measured [IM] µ = 0.5749; Delayed measured [DM] µ = 0.5714) were larger than the control group (µ = 0.4336) and statistically significant (IM vs. Control, p = 0.0018; DM vs. Control, p = 0.0092). There was no significant difference (p = 0.9485) in porosity of the DM group when compared with the IM group. This study demonstrated that the trabecular fibers immediately post-increased ICP (ONS dilation) were more porous than the control and remained statistically different (more porous) after 30 days. These results suggest a structural change that occurs in the ONS with elevations in ICP.