Browsing by Author "Strouse, John J"
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Item Open Access A Comparison of the effect of patient-specific vs. weight-based protocols to treat vaso-occlusive episodes (VOE) in the emergency department.(Academic emergency medicine : official journal of the Society for Academic Emergency Medicine, 2023-09) Tanabe, Paula; Ibemere, Stephanie; Pierce, Ava E; Freiermuth, Caroline E; Bosworth, Hayden B; Yang, Hongqui; Osunkwo, Ifeyinwa; Paxton, James H; Strouse, John J; Miller, Joseph; Paice, Judith A; Veeramreddy, Padmaja; Kavanagh, Patricia L; Wilkerson, R Gentry; Hughes, Robert; Barnhart, Huiman XBackground
Vaso-occlusive episodes (VOC) cause debilitating pain and are a common cause of emergency department (ED) visits, for people with sickle cell disease (SCD). Strategies for achieving optimal pain control vary widely despite evidence-based guidelines. We tested existing guidelines and hypothesized a patient-specific protocol (PSP) written by their SCD provider, may be more effective than weight-based (WB) dosing of parenteral opiate medication, in relieving pain.Methods
Prospective, randomized controlled trial comparing a PSP versus WB protocol for patients presenting with VOC to six EDs. Patients were randomized to a PSP or WB protocol prior to an ED visit. SCD provider wrote their protocol and placed in the electronic health record for future ED visits with a VOC Exclusion criteria included: pre-existing PSP excluding parenteral opioid analgesia or out-patient use of buprenorphine or methadone, or highly suspected for COVID-19. Pain intensity scores, side effects and safety were obtained every 30 minutes for up to 6 hours post-ED bed placement. The primary outcome was change in pain intensity score from placement in an ED space to disposition or six hours.Results
328 subjects were randomized, 104 participants enrolled (ED visit, target n=230) with complete data for 96 visits. The study was unable to reach the target sample size and stopped early due to the impact of COVID-19. We found no significant differences between groups in the primary outcome; patients randomized to a PSP had a shorter ED length of stay (p=.008); the prevalence of side effects was low in both groups. Subjects in both groups experienced both a clinically meaningful and statistically significant decrease in pain (27 mm on a 0-100 mm scale) CONCLUSIONS: We found a shorter ED length of stay for patients assigned to a PSP. Patients in both groups experienced good pain relief without significant side effects.Item Open Access American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain.(Blood advances, 2020-06) Brandow, Amanda M; Carroll, C Patrick; Creary, Susan; Edwards-Elliott, Ronisha; Glassberg, Jeffrey; Hurley, Robert W; Kutlar, Abdullah; Seisa, Mohamed; Stinson, Jennifer; Strouse, John J; Yusuf, Fouza; Zempsky, William; Lang, EddyBACKGROUND:The management of acute and chronic pain for individuals living with sickle cell disease (SCD) is a clinical challenge. This reflects the paucity of clinical SCD pain research and limited understanding of the complex biological differences between acute and chronic pain. These issues collectively create barriers to effective, targeted interventions. Optimal pain management requires interdisciplinary care. OBJECTIVE:These evidence-based guidelines developed by the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in pain management decisions for children and adults with SCD. METHODS:ASH formed a multidisciplinary panel, including 2 patient representatives, that was thoroughly vetted to minimize bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic reviews. Clinical questions and outcomes were prioritized according to importance for clinicians and patients. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. RESULTS:The panel reached consensus on 18 recommendations specific to acute and chronic pain. The recommendations reflect a broad pain management approach, encompassing pharmacological and nonpharmacological interventions and analgesic delivery. CONCLUSIONS:Because of low-certainty evidence and closely balanced benefits and harms, most recommendations are conditional. Patient preferences should drive clinical decisions. Policymaking, including that by payers, will require substantial debate and input from stakeholders. Randomized controlled trials and comparative-effectiveness studies are needed for chronic opioid therapy, nonopioid therapies, and nonpharmacological interventions.Item Open Access Assessing Feasibility of a Focused Geriatric Assessment in Older Adults with Sickle Cell Disease to Address Functional Risk Factors for Morbidity and Mortality(Blood, 2019-11-13) Oyedeji, Charity I; Pieper, Carl; Hall, Katherine; Morey, Miriam; Whitson, Heather; Strouse, John JBackground During the last five decades the life expectancy for people living with sickle cell disease (SCD) has improved markedly, with median survival of 61 years in recent cohorts enrolled from academic centers. Older adults with SCD (defined herein as age ≥ 50 years) make up 13% of the adult population at four major academic medical health systems in North and South Carolina. As this population continues to grow, more data are needed to guide medical management appropriate to their needs, as a lifetime of vaso-occlusion often leads to functional decline and premature development of complications seen in geriatric populations. There are no validated assessment tools and interventions to improve physical function in older adults with SCD. In this study we evaluated the feasibility of a focused geriatric assessment (FGA) for older adults with SCD. Methods Twenty adults with SCD ≥ 50 years old were enrolled in a prospective cohort study. Measures previously validated in the oncology FGA were included and enriched with additional physical and cognitive functional measures. Activity monitoring was performed for 7 days using the Actigraph wT3X-BT, and biomarkers were collected at each study visit (baseline, 10-20 days after a hospitalization [for those who experienced a hospitalization during the study period] and 12-month after baseline). The primary endpoint was the proportion of subjects who complete the assessment. Secondary endpoints were duration of the FGA, proportion who completed the activity monitoring and lab collection, and acceptability. Results Twenty-one of 25 older adults approached for the study consented. Nearly all (20/21, 95%) completed the FGA. One was removed after missed study visits. The median duration of the assessment was 97 minutes (range 73-175 minutes), and there were no adverse events. The mean age was 57 years (range 50-71), 50% (10) were female, 30% (6) were on disability and 50% (10) were working. 45% (9) were former smokers but only 5% (1) were current smokers (Table). On the acceptability survey, 95% (19) reported the length of the assessment as appropriate. 10% (2) subjects reported a portion of the Montreal Cognitive Assessment (MoCA) as difficult. No subject found the questions upsetting, and 2 subjects reported that they would remove redundant questions. All subjects had a Karnofsky Performance Score of at least 80% and were able to complete activities of daily living. A third (35%) had been admitted in the last 6 months, and most (75%) had had severe pain crises at home that limited their activity. 40% (8) had > 4 severe crises at home in the last 6 months. The mean usual gait speed was 1.14 m/sec (range 0.90-1.50) (Figure). Mean Timed Up and Go (TUG) was 10.1 seconds (range 7.7-14.0). Two (10%) subjects had a TUG consistent with increased fall risk (≥ 12 seconds). Mean grip strength of the dominant hand was 39 kg (range 22-54 kg) for males and 25 kg (range 20-34 kg) for females, which is 38% and 43% lower than expected for age and gender. Mean six-minute walk (6MW) was 465m for males and 499m for females, which is 22% and 4% lower than expected for age/gender/height/weight. In the 17 subjects with heart rate recovery (HRR) recorded after 6MW, the mean HRR at 1 minute (HRR1) was 20 bpm (range 0-46). 31% of tested subjects had a HRR1 consistent with impaired HRR (12 bpm or fewer). HRR negatively correlated with age (HRR1 B=-1.1; 95% CI -0.2 - -2; p<0.05). Half of the subjects had mild cognitive impairment (MoCA score < 26). MoCA scores correlated with literacy testing (B= 0.39; 95% CI 0.10-0.68; p=0.02). The first 12 subjects wore the activity monitor 6 days (range 4-8 days) for an average of 15 hours a day. Average activity was sedentary for 7 hours (47%), light 7.5 hours (50%), and moderate 1 hour (7%) per day. There was a median of 7070 steps/day. Conclusions We found FGA to be feasible, acceptable, and safe. The duration of the FGA in our population was 3 times longer than the FGA for oncology. One of our most remarkable findings is that the older adults with SCD in this study have a physical function similar to non-SCD adults over the age of 80. They also demonstrate impaired HRR, an independent predictor of mortality in the elderly. After completion of the 12-month follow-up assessments, we will develop a briefer assessment to be evaluated in a larger study. Future steps will be to determine if FGA can predict outcomes such as risk of hospitalization and mortality and to develop interventions to improve these outcomes. Disclosures Strouse: Global Blood Therapeutics: Consultancy.Item Open Access Birth Prevalence of Sickle Cell Disease and County-Level Social Vulnerability - Sickle Cell Data Collection Program, 11 States, 2016-2020.(MMWR. Morbidity and mortality weekly report, 2024-03) Kayle, Mariam; Blewer, Audrey L; Pan, Wei; Rothman, Jennifer A; Polick, Carri S; Rivenbark, Joshua; Fisher, Elliott; Reyes, Camila; Strouse, John J; Weeks, Shelby; Desai, Jay R; Snyder, Angela B; Zhou, Mei; Sutaria, Ankit; Valle, Jhaqueline; Horiuchi, Sophia S; Sontag, Marci K; Miller, Joshua I; Singh, Ashima; Dasgupta, Mahua; Janson, Isaac A; Galadanci, Najibah; Reeves, Sarah L; Latta, Krista; Hurden, Isabel; Cromartie, Shamaree J; Plaxco, Allison P; Mukhopadhyay, Ayesha; Smeltzer, Matthew P; Hulihan, MarySickle cell disease (SCD) remains a public health priority in the United States because of its association with complex health needs, reduced life expectancy, lifelong disabilities, and high cost of care. A cross-sectional analysis was conducted to calculate the crude and race-specific birth prevalence for SCD using state newborn screening program records during 2016-2020 from 11 Sickle Cell Data Collection program states. The percentage distribution of birth mother residence within Social Vulnerability Index quartiles was derived. Among 3,305 newborns with confirmed SCD (including 57% with homozygous hemoglobin S or sickle β-null thalassemia across 11 states, 90% of whom were Black or African American [Black], and 4% of whom were Hispanic or Latino), the crude SCD birth prevalence was 4.83 per 10,000 (one in every 2,070) live births and 28.54 per 10,000 (one in every 350) non-Hispanic Black newborns. Approximately two thirds (67%) of mothers of newborns with SCD lived in counties with high or very high levels of social vulnerability; most mothers lived in counties with high or very high levels of vulnerability for racial and ethnic minority status (89%) and housing type and transportation (64%) themes. These findings can guide public health, health care systems, and community program planning and implementation that address social determinants of health for infants with SCD. Implementation of tailored interventions, including increasing access to transportation, improving housing, and advancing equity in high vulnerability areas, could facilitate care and improve health outcomes for children with SCD.Item Open Access Chikungunya as a cause of acute febrile illness in southern Sri Lanka.(PLoS One, 2013) Reller, Megan E; Akoroda, Ufuoma; Nagahawatte, Ajith; Devasiri, Vasantha; Kodikaarachchi, Wasantha; Strouse, John J; Chua, Robert; Hou, Yan'an; Chow, Angelia; Sessions, October M; Østbye, Truls; Gubler, Duane J; Woods, Christopher W; Bodinayake, ChampicaBACKGROUND: Chikungunya virus (CHIKV) re-emerged in Sri Lanka in late 2006 after a 40-year hiatus. We sought to identify and characterize acute chikungunya infection (CHIK) in patients presenting with acute undifferentiated febrile illness in unstudied rural and semi-urban southern Sri Lanka in 2007. METHODOLOGY/PRINCIPAL FINDINGS: We enrolled febrile patients ≥ 2 years of age, collected uniform epidemiologic and clinical data, and obtained serum samples for serology, virus isolation, and real-time reverse-transcriptase PCR (RT-PCR). Serology on paired acute and convalescent samples identified acute chikungunya infection in 3.5% (28/797) patients without acute dengue virus (DENV) infection, 64.3% (18/28) of which were confirmed by viral isolation and/or real-time RT-PCR. No CHIKV/DENV co-infections were detected among 54 patients with confirmed acute DENV. Sequencing of the E1 coding region of six temporally distinct CHIKV isolates (April through October 2007) showed that all isolates posessed the E1-226A residue and were most closely related to Sri Lankan and Indian isolates from the same time period. Except for more frequent and persistent musculoskeletal symptoms, acute chikungunya infections mimicked DENV and other acute febrile illnesses. Only 12/797 (1.5%) patients had serological evidence of past chikungunya infection. CONCLUSIONS/SIGNIFICANCE: Our findings suggest CHIKV is a prominent cause of non-specific acute febrile illness in southern Sri Lanka.Item Open Access Common data model for sickle cell disease surveillance: considerations and implications.(JAMIA open, 2023-07) Smeltzer, Matthew P; Reeves, Sarah L; Cooper, William O; Attell, Brandon K; Strouse, John J; Takemoto, Clifford M; Kanter, Julie; Latta, Krista; Plaxco, Allison P; Davis, Robert L; Hatch, Daniel; Reyes, Camila; Dombkowski, Kevin; Snyder, Angela; Paulukonis, Susan; Singh, Ashima; Kayle, MariamObjective
Population-level data on sickle cell disease (SCD) are sparse in the United States. The Centers for Disease Control and Prevention (CDC) is addressing the need for SCD surveillance through state-level Sickle Cell Data Collection Programs (SCDC). The SCDC developed a pilot common informatics infrastructure to standardize processes across states.Materials and methods
We describe the process for establishing and maintaining the proposed common informatics infrastructure for a rare disease, starting with a common data model and identify key data elements for public health SCD reporting.Results
The proposed model is constructed to allow pooling of table shells across states for comparison. Core Surveillance Data reports are compiled based on aggregate data provided by states to CDC annually.Discussion and conclusion
We successfully implemented a pilot SCDC common informatics infrastructure to strengthen our distributed data network and provide a blueprint for similar initiatives in other rare diseases.Item Open Access Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity.(JAMA network open, 2020-03-02) Farooq, Faheem; Mogayzel, Peter J; Lanzkron, Sophie; Haywood, Carlton; Strouse, John JImportance:Sickle cell disease (SCD) and cystic fibrosis (CF) are severe autosomal recessive disorders associated with intermittent disease exacerbations that require hospitalizations, progressive chronic organ injury, and substantial premature mortality. Research funding is a limited resource and may contribute to health care disparities, especially for rare diseases that disproportionally affect economically disadvantaged groups. Objective:To compare disease-specific funding between SCD and CF and the association between funding and research productivity. Design, Setting, and Participants:This cross-sectional study examined federal and foundation funding, publications indexed in PubMed, clinical trials registered in ClinicalTrials.gov, and new drug approvals from January 1, 2008, to December 31, 2018, in an estimated US population of approximately 90 000 individuals with SCD and approximately 30 000 individuals with CF. Main Outcomes and Measures:Federal and foundation funding, publications indexed in PubMed, clinical trial registrations, and new drug approvals. Results:From 2008 through 2018, federal funding was greater per person with CF compared with SCD (mean [SD], $2807 [$175] vs $812 [$147]; P < .001). Foundation expenditures were greater for CF than for SCD (mean [SD], $7690 [$3974] vs $102 [$13.7]; P < .001). Significantly more research articles (mean [SD], 1594 [225] vs 926 [157]; P < .001) and US Food and Drug Administration drug approvals (4 vs 1) were found for CF compared with SCD, but the total number of clinical trials was similar (mean [SD], 27.3 [6.9] vs 23.8 [6.3]; P = .22). Conclusions and Relevance:The findings show that disparities in funding between SCD and CF may be associated with decreased research productivity and novel drug development for SCD. Increased federal and foundation funding is needed for SCD and other diseases that disproportionately affect economically disadvantaged groups to address health care disparities.Item Open Access Emergence of Epidemic Dengue-1 Virus in the Southern Province of Sri Lanka.(PLoS Negl Trop Dis, 2016-10) Bodinayake, Champica K; Tillekeratne, L Gayani; Nagahawatte, Ajith; Devasiri, Vasantha; Kodikara Arachichi, Wasantha; Strouse, John J; Sessions, October M; Kurukulasooriya, Ruvini; Uehara, Anna; Howe, Shiqin; Ong, Xin Mei; Tan, Sharon; Chow, Angelia; Tummalapalli, Praveen; De Silva, Aruna D; Østbye, Truls; Woods, Christopher W; Gubler, Duane J; Reller, Megan EBACKGROUND: Dengue is a frequent cause of acute febrile illness with an expanding global distribution. Since the 1960s, dengue in Sri Lanka has been documented primarily along the heavily urbanized western coast with periodic shifting of serotypes. Outbreaks from 2005-2008 were attributed to a new clade of DENV-3 and more recently to a newly introduced genotype of DENV-1. In 2007, we conducted etiologic surveillance of acute febrile illness in the Southern Province and confirmed dengue in only 6.3% of febrile patients, with no cases of DENV-1 identified. To re-evaluate the importance of dengue as an etiology of acute febrile illness in this region, we renewed fever surveillance in the Southern Province to newly identify and characterize dengue. METHODOLOGY/PRINCIPAL FINDINGS: A cross-sectional surveillance study was conducted at the largest tertiary care hospital in the Southern Province from 2012-2013. A total of 976 patients hospitalized with acute undifferentiated fever were enrolled, with 64.3% male and 31.4% children. Convalescent blood samples were collected from 877 (89.6%). Dengue virus isolation, dengue RT-PCR, and paired IgG ELISA were performed. Acute dengue was confirmed as the etiology for 388 (39.8%) of 976 hospitalizations, with most cases (291, 75.0%) confirmed virologically and by multiple methods. Among 351 cases of virologically confirmed dengue, 320 (91.2%) were due to DENV-1. Acute dengue was associated with self-reported rural residence, travel, and months having greatest rainfall. Sequencing of selected dengue viruses revealed that sequences were most closely related to those described from China and Southeast Asia, not nearby India. CONCLUSIONS/SIGNIFICANCE: We describe the first epidemic of DENV-1 in the Southern Province of Sri Lanka in a population known to be susceptible to this serotype because of prior study. Dengue accounted for 40% of acute febrile illnesses in the current study. The emergence of DENV-1 as the foremost serotype in this densely populated but agrarian population highlights the changing epidemiology of dengue and the need for continued surveillance and prevention.Item Open Access Improving the Quality of Care for Adolescents and Adults With Sickle Cell Disease-It's a Long Road.(JAMA network open, 2020-05) Oyedeji, Charity; Strouse, John JItem Open Access Is low dose hydroxyurea the solution to the global epidemic of sickle cell disease?(Pediatr Blood Cancer, 2015-06) Strouse, John JItem Open Access Secondhand smoke is an important modifiable risk factor in sickle cell disease: A review of the current literature and areas for future research(International Journal of Environmental Research and Public Health, 2016-11-12) Sadreameli, S Christy; Kopp, Benjamin T; Creary, Susan E; Eakin, Michelle N; McGrath-Morrow, Sharon; Strouse, John J© 2016 by the authors; licensee MDPI, Basel, Switzerland.Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that causes significant morbidity and mortality related to chronic hemolytic anemia, vaso-occlusion, and resultant end-organ damage. Tobacco smoke exposure (TSE) through secondhand smoke exposure in people with SCD of all ages and through primary smoking in adolescents and adults is associated with significantly increased morbidity, with increased rates of emergency department visits and hospitalizations for painful vaso-occlusive crises and acute chest syndrome (ACS). Secondhand smoke is also associated with pulmonary function abnormalities in children with SCD who are already at risk for pulmonary function abnormalities on the basis of SCD. TSE is emerging as one of the few modifiable risk factors of SCD. This review discusses the current state of the evidence with respect to TSE and SCD morbidity, discusses potential mechanisms, and highlights current gaps in the evidence and future research directions.Item Open Access The Sickle Cell Disease Functional Assessment (SCD-FA) tool: a feasibility pilot study.(Pilot and feasibility studies, 2022-03) Oyedeji, Charity I; Hall, Katherine; Luciano, Alison; Morey, Miriam C; Strouse, John JBackground
The life expectancy for individuals with sickle cell disease (SCD) has greatly increased over the last 50 years. Adults with SCD experience multiple complications such as cardiopulmonary disease, strokes, and avascular necrosis that lead to limitations that geriatric populations often experience. There are no dedicated instruments to measure functional decline and functional age to determine risk of future adverse outcomes in older adults with SCD. The objective of this study was to assess the feasibility of performing the Sickle Cell Disease Functional Assessment (SCD-FA).Methods
We enrolled 40 adults with SCD (20 younger adults aged 18-49 years as a comparison group and 20 older adults aged 50 years and older) in a single-center prospective cohort study. Participants were recruited from a comprehensive sickle cell clinic in an academic center in the southeastern United States. We included measures validated in an oncology geriatric assessment enriched with additional physical performance measures: usual gait speed, seated grip strength, Timed Up and Go, six-minute walk test, and 30-second chair stand. We also included an additional cognitive measure, which was the Montreal Cognitive Assessment, and additional patient-reported measures at the intersection of sickle cell disease and geriatrics. The primary outcome was the proportion completing the assessment. Secondary outcomes were the proportion consenting, duration of the assessment, acceptability, and adverse events.Results
Eighty percent (44/55) of individuals approached consented, 91% (40/44) completed the SCD-FA in its entirety, and the median duration was 89 min (IQR 80-98). There were no identified adverse events. On the acceptability survey, 95% (38/40) reported the length as appropriate, 2.5% (1/40) reported a question as upsetting, and 5% (2/40) reported portions as difficult. Exploratory analyses of physical function showed 63% (25/40) had a slow usual gait speed (< 1.2 m/s).Conclusion
The SCD-FA is feasible, acceptable, and safe and physical performance tests identified functional impairments in adults with SCD. These findings will inform the next phase of the study where we will assess the validity of the SCD-FA to predict patient-important outcomes in a larger sample of adults with SCD.Item Open Access Treatment of an acquired Factor XIII inhibitor in an adolescent with systemic lupus erythematosus and renal failure(Transfusion, 2017-09-01) Rabik, Cara A; Atkinson, Meredith A; Sule, Sangeeta; Strouse, John JFactor (F)XIII deficiency is a rare inherited bleeding disorder, but can also be acquired due to the development of inhibitors. CASE REPORT: A 17-year-old female with systemic lupus erythematosus and end-stage kidney disease secondary to Class IV lupus nephritis developed spontaneous subcutaneous and muscular hematomas and delayed major bleeding after invasive procedures. She had abnormal kaolin thromboelastography (kTEG; decreased maximal amplitude, representative of clot strength) initially attributed to thrombocytopenia and uremic platelet dysfunction, but her FXIII activity was undetectable, and a high-titer antibody against FXIII was identified. She had improvement in clinical bleeding and in kaolin thromboelastogram result and transient improvement in FXIII activity after each dose of plasma-derived FXIII concentrate (Corifact) or cryoprecipitate. Her inhibitor titers gradually improved with multiple immunosuppressive therapies and plasma exchange. While her FXIII activity level remained mildly decreased, she has not had additional significant bleeding. CONCLUSION: Treatment with either plasma-derived FXIII or cryoprecipitate is an effective treatment to normalize the kTEG variables and clinical bleeding diatheses associated with acquired FXIII inhibitors. Higher doses may be needed in patients with high-titer inhibitor.