Publications by authors named "Susan Creary"

30 Publications

  • Page 1 of 1

Primary Immunization Series Coverage of Children With Sickle Cell Disease.

Am J Prev Med 2021 Mar 11. Epub 2021 Mar 11.

Susan B. Meister Child Health Evaluation and Research (CHEAR) Center, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan; Department of Epidemiology, School of Public Health, University of Michigan, Ann Arbor, Michigan.

Introduction: Children with sickle cell disease are at increased risk of serious infections, many of which can be prevented by receipt of recommended immunizations. Study objectives were to (1) assess the primary immunization series coverage among children with sickle cell disease and (2) compare the coverage with that of those without sickle cell disease.

Methods: The Michigan Care Improvement Registry was used to obtain primary immunization series doses and sickle cell disease status for all eligible children in Michigan born in 2001-2016 (analysis was conducted in 2019). Completion of series and each individual vaccine were assessed for every child at ages 19 and 35 months. Proportions were stratified by sickle cell disease status and compared using chi-square tests. Logistic regression was used to model the odds of completing the series at each age, as predicted by sickle cell disease status and adjusting for the presence of Medicaid identification number.

Results: The proportion of children who completed the immunization series was higher for those with sickle cell disease than for those without sickle cell disease at 19 months (58.5% vs 48.0%) and 35 months (74.7% vs 59.6%) (both p<0.0001). The odds of completing the series were 1.4 times higher at 19 months (95% CI=1.2, 1.6) and 1.7 times higher at 35 months (95% CI=1.5, 2.0) for children with sickle cell disease than for those without, adjusting for the presence of Medicaid identification number.

Conclusions: Although children with sickle cell disease had higher immunization rates than those without sickle cell disease, >40% of children with sickle cell disease did not receive all recommended immunizations by age 19 months. Immunization information systems should be utilized to improve routine immunization coverage of children with sickle cell disease.
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http://dx.doi.org/10.1016/j.amepre.2021.01.015DOI Listing
March 2021

Hydroxyurea Optimization through Precision Study (HOPS): study protocol for a randomized, multicenter trial in children with sickle cell anemia.

Trials 2020 Nov 27;21(1):983. Epub 2020 Nov 27.

University of Cincinnati College of Medicine, Cincinnati, OH, USA.

Background: Sickle cell disease (SCD) is a severe and devastating hematological disorder that affects over 100,000 persons in the USA and millions worldwide. Hydroxyurea is the primary disease-modifying therapy for the SCD, with proven benefits to reduce both short-term and long-term complications. Despite the well-described inter-patient variability in pharmacokinetics (PK), pharmacodynamics, and optimal dose, hydroxyurea is traditionally initiated at a weight-based dose with a subsequent conservative dose escalation strategy to avoid myelosuppression. Because the dose escalation process is time consuming and requires frequent laboratory checks, many providers default to a fixed dose, resulting in inadequate hydroxyurea exposure and suboptimal benefits for many patients. Results from a single-center trial of individualized, PK-guided dosing of hydroxyurea for children with SCD suggest that individualized dosing achieves the optimal dose more rapidly and provides superior clinical and laboratory benefits than traditional dosing strategies. However, it is not clear whether these results were due to individualized dosing, the young age that hydroxyurea treatment was initiated in the study, or both. The Hydroxyurea Optimization through Precision Study (HOPS) aims to validate the feasibility and benefits of this PK-guided dosing approach in a multi-center trial.

Methods: HOPS is a randomized, multicenter trial comparing standard vs. PK-guided dosing for children with SCD as they initiate hydroxyurea therapy. Participants (ages 6 months through 21 years), recruited from 11 pediatric sickle cell centers across the USA, are randomized to receive hydroxyurea either using a starting dose of 20 mg/kg/day (Standard Arm) or a PK-guided dose (Alternative Arm). PK data will be collected using a novel sparse microsampling approach requiring only 10 μL of blood collected at 3 time-points over 3 h. A protocol-guided strategy more aggressive protocols is then used to guide dose escalations and reductions in both arms following initiation of hydroxyurea. The primary endpoint is the mean %HbF after 6 months of hydroxyurea.

Discussion: HOPS will answer important questions about the clinical feasibility, benefits, and safety of PK-guided dosing of hydroxyurea for children with SCD with potential to change the treatment paradigm from a standard weight-based approach to one that safely and effectively optimize the laboratory and clinical response.

Trial Registration: ClinicalTrials.gov NCT03789591 . Registered on 28 December 2018.
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http://dx.doi.org/10.1186/s13063-020-04912-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7691962PMC
November 2020

Author Correction: Baseline and Disease-Induced Transcriptional Profiles in Children with Sickle Cell Disease.

Sci Rep 2020 Aug 6;10(1):13530. Epub 2020 Aug 6.

Center for Microbial Pathogenesis, The Abigail Wexner Research Institute, Columbus, OH, USA.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.
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http://dx.doi.org/10.1038/s41598-020-70341-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7413249PMC
August 2020

American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain.

Blood Adv 2020 06;4(12):2656-2701

Department of Emergency Medicine, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.

Background: 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.
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http://dx.doi.org/10.1182/bloodadvances.2020001851DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322963PMC
June 2020

Baseline and Disease-Induced Transcriptional Profiles in Children with Sickle Cell Disease.

Sci Rep 2020 06 2;10(1):9013. Epub 2020 Jun 2.

Center for Microbial Pathogenesis, The Abigail Wexner Research Institute, Columbus, OH, USA.

Acute chest syndrome (ACS) is a significant cause of morbidity and mortality in sickle cell disease (SCD), but preventive, diagnostic, and therapeutic options are limited. Further, ACS and acute vasoccclusive pain crises (VOC) have overlapping features, which causes diagnostic dilemmas. We explored changes in gene expression profiles among patients with SCD hospitalized for VOC and ACS episodes to better understand ACS disease pathogenesis. Whole blood RNA-Seq was performed for 20 samples from children with SCD at baseline and during a hospitalization for either an ACS (n = 10) or a VOC episode (n = 10). Respiratory viruses were identified from nasopharyngeal swabs. Functional gene analyses were performed using modular repertoires, IPA, Gene Ontology, and NetworkAnalyst 3.0. The VOC group had a numerically higher percentage of female, older, and hemoglobin SS participants compared to the ACS group. Viruses were detected in 50% of ACS cases and 20% of VOC cases. We identified 3004 transcripts that were differentially expressed during ACS episodes, and 1802 transcripts during VOC episodes. Top canonical pathways during ACS episodes were related to interferon signaling, neuro-inflammation, pattern recognition receptors, and macrophages. Top canonical pathways in patients with VOC included IL-10 signaling, iNOS signaling, IL-6 signaling, and B cell signaling. Several genes related to antimicrobial function were down-regulated during ACS compared to VOC. Gene enrichment nodal interactions demonstrated significantly altered pathways during ACS and VOC. A complex network of changes in innate and adaptive immune gene expression were identified during both ACS and VOC episodes. These results provide unique insights into changes during acute events in children with SCD.
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http://dx.doi.org/10.1038/s41598-020-65822-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7265336PMC
June 2020

Measuring hydroxyurea adherence by pharmacy and laboratory data compared with video observation in children with sickle cell disease.

Pediatr Blood Cancer 2020 08 9;67(8):e28250. Epub 2020 May 9.

Division of Pediatric Hematology/Oncology/BMT, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio.

Background: Hydroxyurea nonadherence is common among children with sickle cell disease (SCD), but it is unclear if current adherence measures are valid compared with video directly observed therapy (VDOT), a reference method. The objectives were to evaluate if hydroxyurea adherence by pharmacy records, urine assay, mean corpuscular volume (MCV), and/or fetal hemoglobin (HbF) correlated with and was sensitive and specific compared with VDOT.

Methods: This was a cross-sectional analysis of adherence data from 34 children with SCD on a single-arm, six-month hydroxyurea adherence study. Spearman correlation coefficient compared participants' adherence by pharmacy records, MCV, and HbF to adherence by VDOT. The sensitivity and specificity of ≥80% adherence by pharmacy records, two urine samples with hydroxyurea, MCV ≥100 fl/L, and HbF ≥20% compared with ≥80% VDOT adherence were also calculated.

Results: Median pharmacy and VDOT adherence rates were similar (87.8% vs 88.1%, P = 0.75) and mildly correlated (r  = 0.45; P = 0.008) but the sensitivity of ≥80% adherence by pharmacy records was 72.7% and specificity was 45.5%. MCV (r = -0.02, P = 0.92) and HbF (r = -0.2, P = 0.33) did not significantly correlate with VDOT adherence. Sensitivity and specificity were 83.3% and 33.3% for having two urine samples with hydroxyurea, 35% and 71.4% for MCV ≥100 fl/L, and 75% and 0% for HbF ≥20%, respectively.

Conclusions: Commonly used tools to measure hydroxyurea adherence may not correlate with or be valid compared with video adherence. Future studies to refine these measures are needed to effectively target adherence interventions to children with SCD who have the potential to benefit. (ClinicalTrials.gov NCT02578017).
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http://dx.doi.org/10.1002/pbc.28250DOI Listing
August 2020

Progression of albuminuria in patients with sickle cell anemia: a multicenter, longitudinal study.

Blood Adv 2020 04;4(7):1501-1511

Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

Sickle cell nephropathy results in chronic kidney disease (CKD), which is associated with significant morbidity and mortality in sickle cell anemia (SCA). Albuminuria is an early manifestation of sickle nephropathy; however, little is known about progression of albuminuria or its correlation with glomerular filtration rate (GFR) decline or CKD. We studied nephropathy progression in 303 SCA participants in a prospective, multicenter, longitudinal study. We collected steady-state urine and serum samples yearly and assessed albumin/creatinine ratio (ACR), estimated GFR (eGFR), and SCA and nephropathy biomarkers. Participants with albuminuria (ACR ≥30 mg/g) for ≥2 annual measurements were classified as having persistent albuminuria (PA). At baseline (mean age, 21 years; range, 2-64 years), 32% had albuminuria. In longitudinal multivariate analysis, ACR was associated with sex, anemia, older age, and higher bilirubin and kidney injury molecule-1 levels. Albuminuria increased with age by 3.5 mg/g per year (P < .0001). Of 175 participants with ≥3 annual samples, 81% with baseline albuminuria ≥100 mg/g developed PA. Decreased eGFR and adult CKD were associated with PA (P = .002 and P = .02, respectively), but not with baseline albuminuria. Rate of eGFR decline was steeper among adults (but not children) with albuminuria, compared with those without (P = .02). Participants with PA were more likely to have rapid eGFR decline compared with those without (P = .03). In this longitudinal study, albuminuria progressed with age, and adults with albuminuria had worse eGFR decline than those without. Albuminuria ≥100 mg/g predicted PA, which was associated with rapid eGFR decline and CKD development in adults with SCA. This trial was registered at www.clinicaltrials.gov as #NCT02239016.
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http://dx.doi.org/10.1182/bloodadvances.2019001378DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160281PMC
April 2020

Opioid Prescription Filling Trends Among Children with Sickle Cell Disease After the Release of State-Issued Guidelines on Pain Management.

Pain Med 2020 10;21(10):2583-2592

Center for Surgical Outcomes Research and Center for Innovation in Pediatric Practice, Abigail Wexner Research Institute at Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio, USA.

Objective: To assess the impact of Ohio's 2012, 2013, and 2016 opioid prescribing guidelines on opioid and nonsteroidal anti-inflammatory drug (NSAID) prescription filling and health care utilization for pain among children with sickle cell disease (SCD).

Design: Quasi-experimental retrospective cohort study.

Setting: Ohio Medicaid claims data from August 2011 to August 2016.

Subjects: Medicaid beneficiaries under age 19 years with SCD.

Methods: Interrupted time series analyses comparing population-level rates of opioids and NSAID prescriptions filled, standardized amounts of opioids dispensed, and acute health care utilization for pain before and after release of each guideline.

Results: In our cohort of 1,505 children with SCD, there was a temporary but significant decrease in the opioid filling rate (-2.96 prescriptions per 100 children, P = 0.01) and in the amount of opioids dispensed (-31.39 milligram morphine equivalents per filled prescription, P < 0.001) after the 2013 guideline but a temporary but significant increase in the opioid filling rate (7.44 prescriptions per 100 children, P < 0.001) and in the amount of opioids dispensed (72.73 mg morphine equivalents per filled prescription, P < 0.001) after the 2016 guideline. The NSAID filling rate did not significantly change after any of the guidelines. Acute health care utilization rates for pain after the 2016 guideline were similar to those before the 2013 guideline (rate ratio = 1.04, P = 0.63).

Conclusions: Our results suggest that Ohio's 2013 and 2016 guidelines were associated with significant but nonsustained changes in opioid prescription filling among children with SCD. Additional studies are needed to confirm that opioid guidelines have a sustained impact on excessive opioid prescribing, filling, and misuse.
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http://dx.doi.org/10.1093/pm/pnaa002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7593803PMC
October 2020

Allocation of Treatment Responsibility and Adherence to Hydroxyurea Among Adolescents With Sickle Cell Disease.

J Pediatr Psychol 2019 11;44(10):1196-1204

Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center.

Objective: Adolescents with sickle cell disease (SCD) are at increased risk for complications. Hydroxyurea is a medication that can ameliorate risk but to benefit, adolescents must adhere to treatment. Study aims were to describe how adolescents and their caregivers decided who was responsible for treatment tasks, to describe adolescents' and caregivers' responsibility for these tasks, and to examine if hydroxyurea adherence was associated with younger adolescent age, less discrepancy between adolescents' and caregivers' reports of adolescent responsibility, and higher caregiver involvement.

Methods: Twenty-nine dyads completed treatment responsibility measures. A combination of laboratory and electronic prescription data were used to determine hydroxyurea adherence and electronic medical records were used to determine appointment adherence.

Results: Few dyads agreed or planned how to complete treatment tasks. Adolescents shared responsibility with caregivers for medication-taking tasks. Adolescents perceived caregivers and caregivers perceived adolescents were overall responsible for treatment, especially for appointment tasks. Half of adolescents were adherent to hydroxyurea and half were adherent to appointments but medication adherence was not associated with age, discrepancy between adolescents' and caregivers' responses, or caregiver involvement.

Conclusions: Despite frequent hydroxyurea and appointment nonadherence, few adolescents and caregivers plan how to manage adolescents' SCD treatment or perceive they are overall responsible. Future studies are needed to determine the factors that influence these perceptions and if increasing adolescent and caregiver treatment planning improves adherence and clinical outcomes.
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http://dx.doi.org/10.1093/jpepsy/jsz061DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6823103PMC
November 2019

A Multidimensional Electronic Hydroxyurea Adherence Intervention for Children With Sickle Cell Disease: Single-Arm Before-After Study.

JMIR Mhealth Uhealth 2019 08 8;7(8):e13452. Epub 2019 Aug 8.

Nationwide Children's Hospital, The Ohio State University, Columbus, OH, United States.

Background: Hydroxyurea is a disease-modifying medication for patients with sickle cell disease (SCD). Despite demonstrated efficacy, hydroxyurea nonadherence in clinical practice is common and results in worse health outcomes for nonadherent patients. Mobile Directly Observed Therapy (Mobile DOT) is a pilot-tested, electronic, multidimensional hydroxyurea adherence intervention for children with SCD. Mobile DOT includes sending daily text message reminders to patients to take hydroxyurea, patients recording and sending daily videos that capture their hydroxyurea administrations for the research team to review and track adherence, providing personalized feedback to patients about their adherence, and providing small monetary incentives to patients if they achieve high hydroxyurea adherence.

Objective: This study aimed to determine if Mobile DOT increases hydroxyurea adherence in children with SCD and to explore its impact on hematologic and clinical outcomes.

Methods: This was a single-arm, 6-month intervention study of patients with SCD on hydroxyurea who were aged ≤19 years and reported having access to an electronic device. Participants' hydroxyurea adherence when they received Mobile DOT was compared with their adherence 6 months before and after receiving Mobile DOT. Participants' medication possession ratio (MPR) was calculated from their pharmacy dispensing records and was used to measure adherence. Laboratory and clinical outcomes were abstracted from participants' electronic medical records. Infrequently hospitalized patients who received at least 160 days of the intervention were considered to be engaged participants.

Results: Of 91 patients who were approached, 55 enrolled and 34 engaged with Mobile DOT. The median age of the engaged participants was 10 years (range 2-18.8 years), and 21 (62%, 21/34) participants were male, 28 (82%, 21/34) had hemoglobin SS SCD, and 19 (56%, 19/34) were prescribed hydroxyurea for at least a year before enrollment. With Mobile DOT, engaged participants' median MPR increased from 61.7% to 84.4% (P<.001) and significantly more (67% vs 30%; P=.002) achieved ≥80% hydroxyurea adherence compared with baseline values. Engaged participants' mean fetal hemoglobin (HgbF) levels and mean corpuscular volumes (MCV) improved significantly after 6 months of Mobile DOT (P=.04 and P=.001, respectively), but their adherence, HgbF levels, and MCV returned to baseline values during the 6 months after the intervention. Hospitalizations and the clinical outcomes that were measured occurred infrequently during the study. Nonengagement was associated with being female and having a recent SCD complication. In addition, having insufficient electronic data, being unable to quickly complete Mobile DOT each day, and not perceiving that Mobile DOT was beneficial may have further decreased engagement.

Conclusions: Mobile DOT shows promise as an effective intervention for some children with SCD. Modifications that may improve recruitment, reduce attrition, and increase engagement were identified and could increase the impact that Mobile DOT has on children with SCD.

Trial Registration: ClinicalTrials.gov NCT02578017; https://clinicaltrials.gov/ct2/show/NCT02578017.
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http://dx.doi.org/10.2196/13452DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6705009PMC
August 2019

Impact of erythrocytapheresis on natural anticoagulant levels in children with sickle cell disease: A pilot study.

Pediatr Blood Cancer 2019 04 13;66(4):e27588. Epub 2018 Dec 13.

Department of Pediatrics, The Ohio State University, Division of Hematology/Oncology, Nationwide Children's Hospital, Columbus, Ohio.

Venous thromboembolism (VTE) is being increasingly recognized in children with sickle cell disease (SCD). In a retrospective cohort study, we identified bilateral central venous catheter (CVC) placement as an independent risk factor for VTE. At our institution, the only indication for bilateral CVC placement in children with SCD is erythrocytapheresis. To investigate the impact of erythrocytapheresis on coagulation, we measured levels of natural anticoagulants in 11 patients with SCD on chronic erythrocytapheresis, immediately before and after apheresis. We demonstrated a statistically significant reduction in most parameters. Additional studies are needed to further investigate the exact etiology and clinical impact of this acute decrease.
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http://dx.doi.org/10.1002/pbc.27588DOI Listing
April 2019

A pilot study of hormonal contraceptive use and bone mineral density in young women with sickle cell disease.

Pediatr Blood Cancer 2018 12 11;65(12):e27398. Epub 2018 Sep 11.

Division of Pediatric Hematology/Oncology/BMT, Nationwide Children's Hospital, Columbus, Ohio.

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http://dx.doi.org/10.1002/pbc.27398DOI Listing
December 2018

Diverse manifestations of acute sickle cell hepatopathy in pediatric patients with sickle cell disease: A case series.

Pediatr Blood Cancer 2018 08 18;65(8):e27060. Epub 2018 Apr 18.

Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia.

The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis. These complications require a systematic approach to extensive evaluation and coordinated multidisciplinary care.
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http://dx.doi.org/10.1002/pbc.27060DOI Listing
August 2018

Venous Thromboembolism in Children with Sickle Cell Disease: A Retrospective Cohort Study.

J Pediatr 2018 06 28;197:186-190.e1. Epub 2018 Mar 28.

Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH; Department of Pediatrics, The Ohio State University, Columbus, OH. Electronic address:

Objectives: To describe the cumulative incidence of venous thromboembolism (VTE) in children with sickle cell disease (SCD) followed at a single institution and report on the risk factors associated with VTE development.

Study Design: Charts for all patients with SCD, aged 0-21 years, followed at Nationwide Children's Hospital over a 6-year period (January 1, 2009, to January 31, 2015) were reviewed. Data on VTE diagnosis, sex, body mass index/weight-for-length, SCD genotype, SCD clinical complications, central venous catheter (CVC) placement, and thrombophilia testing were collected.

Results: Cumulative incidence of VTE in children with SCD followed at a single tertiary care institution was found to be 2.9% (12/414). Nine of the 12 VTE were CVC-associated. On univariate analysis, hemoglobin SS genotype (OR 10.7, 95% CI 1.4-83.5), CVC presence (OR 34.4, 95% CI 8.9-134.6), central nervous system vasculopathy (OR 19.4, 95% CI 5.6-63.4), chronic transfusion therapy (OR 30.6, 95% CI 8.9-122.2), and older age (P = .03) were associated with VTE. However, presence of CVC was the only independent risk factor identified on multivariable logistic regression analysis (OR 33.8, 95% CI 8.7-130.9).

Conclusion: In our institution, nearly 3% of children with SCD had a history of VTE. CVC is an independent predictor of VTE in children with SCD.
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http://dx.doi.org/10.1016/j.jpeds.2018.01.073DOI Listing
June 2018

Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: an administrative database study.

Blood Adv 2018 02;2(3):285-291

Division of Hematology/Oncology, Department of Pediatrics, The Ohio State University, Nationwide Children's Hospital, Columbus, OH; and.

A hypercoagulable state resulting in increased venous thromboembolism (VTE) has been described in adults with sickle cell disease (SCD), but similar data for children are lacking. The objective of this retrospective cohort study was to describe the rate of VTE and risk factors associated with VTE in children with SCD across tertiary-care children's hospitals in the United States between the years 2009 and 2015. We used the Pediatric Health Information System database to investigate all pediatric patients with SCD admitted to 1 of 48 participating institutions between 1 January 2009 and 30 September 2015. International Classification of Disease, Ninth Edition, Clinical Modification codes were used to identify index thromboembolic events and chronic medical conditions known to be associated with VTE. Billing codes were used to identify central venous line (CVL) placement and pharmaceutical billing codes to identify estrogen containing oral-contraceptive use. Logistic regression analysis was used to study the association among unique patient characteristics, VTE, and death. 10 454 eligible subjects with SCD were identified. Median age (±interquartile range) of study cohort was 10 (±11) years. 181 subjects (1.7%) developed an index venous thromboembolic event during the study period. Median age at VTE diagnosis was 15.9 (±7.4) years. On multivariable logistic regression analysis, CVL placement, chronic renal disease, history of stroke, female sex, length of hospitalization, intensive care unit utilization, and older age were associated with VTE. After adjusting for other variables, VTE was independently associated with death. In summary, VTE can occur in pediatric patients with SCD. CVL placement is a modifiable risk factor for VTE development.
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http://dx.doi.org/10.1182/bloodadvances.2017012336DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5812330PMC
February 2018

A Retrospective Review to Determine If Children with Sickle Cell Disease Receive Hydroxyurea Monitoring.

Pediatr Qual Saf 2017 May-Jun;2(3). Epub 2017 May 25.

Nationwide Children's Hospital, The Ohio State University, Columbus, OH.

Introduction: Sickle Cell Disease (SCD) guidelines recommend that patients on hydroxyurea receive monitoring at least every 2-3 months, but it is unknown if this occurs in clinical practice. This study aimed to determine if patients with SCD at Nationwide Children's Hospital (NCH) had at least four, in-person monitoring visits during a 12-month period and if frequent monitoring was associated with hydroxyurea adherence and clinical outcomes.

Methods: We performed a retrospective analysis of children on hydroxyurea for at least 12 months during 2010-2015. Patients' demographics, laboratory studies, prescriptions, and number of hydroxyurea and acute visits were recorded from their 12-month period that met eligibility criteria. Patients were considered frequently monitored if they had ≥4 hydroxyurea visits and adherent if they had prescriptions for hydroxyurea for ≥80% of the days in their 12-month period.

Results: Seventy-four children met the eligibility criteria and 57 (77%) had frequent monitoring. The most common reason for not obtaining frequent monitoring was missing a scheduled appointment. A greater proportion of frequently monitored patients were adherent to hydroxyurea (66.7% vs. 17.7%, p<0.001) and they had significantly fewer acute visits (median 1 vs. 2 visits, p=0.032) compared to infrequently monitored patients.

Conclusions: Our study shows that most children on hydroxyurea at NCH received frequent monitoring and that it was associated with improved adherence and outcomes. Our results suggest that frequent in-person monitoring could be an opportunity to identify poorly adherent patients. These data inform our next quality improvement initiative that will maximize adherence to these monitoring guidelines.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5793862PMC
http://dx.doi.org/10.1097/pq9.0000000000000024DOI Listing
May 2017

Sickle cell trait knowledge and health literacy in caregivers who receive in-person sickle cell trait education.

Mol Genet Genomic Med 2017 11 23;5(6):692-699. Epub 2017 Aug 23.

Nationwide Children's Hospital, Columbus, Ohio.

Background: Despite universal screening that detects sickle cell trait (SCT) in infancy, only 16% of Americans with SCT know their status. To increase SCT status awareness, effective education for patients and their families is needed. The objective of this study was to assess caregivers' SCT knowledge before and after an in-person SCT education session.

Methods: A trained educator provides in-person SCT education to caregivers of referred infants with SCT at Nationwide Children's Hospital. From August 2015 to July 2016, primarily English-speaking caregivers of infants with hemoglobin S-trait were recruited and completed a health literacy assessment and a SCT knowledge assessment (SCTKA) before and after receiving education. Caregivers repeated the SCTKA again after ≥6 months, if they could be contacted.

Results: Thirty-eight (38.1%) percent of 113 caregivers had high SCTKA scores (≥75%) before education but 90.3% achieved high scores after education. Caregivers with low SCTKA scores after education had significantly lower health literacy (P = 0.029) and baseline SCTKA scores (P = 0.003) compared to those with higher scores after education. At ≥6 months, caregivers' scores were significantly higher (P = 0.014) than baseline, but only 73.3% scored ≥75%.

Conclusion: Our results suggest that caregivers' baseline SCT knowledge is low, improves with in-person education but may decline with time. Caregivers who do not achieve high SCT knowledge after education had lower health literacy and baseline knowledge. Future studies should determine if adapting in-person education to caregivers' health literacy and knowledge levels results in high and sustained SCT knowledge among all caregivers and more individuals who know their SCT status.
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http://dx.doi.org/10.1002/mgg3.327DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5702560PMC
November 2017

Desire for parenthood and reproductive health knowledge in adolescents and young adults with sickle cell disease and their caregivers.

Pediatr Blood Cancer 2018 Feb 25;65(2). Epub 2017 Sep 25.

Center for Innovation in Pediatric Practice, The Research Institute at Nationwide Children's Hospital, Columbus, Ohio.

Background/objective: Sickle cell disease (SCD) and hydroxyurea have implications for fertility and reproductive health. The goal of this study was to examine desire for parenthood and reproductive health knowledge among a cohort of adolescent and young adult (AYA) with SCD receiving hydroxyurea and their caregivers at a large pediatric academic center.

Methods: Patients with SCD were approached from September 2016 to July 2017 if they were: (1) 12-20 years old, (2) prescribed hydroxyurea for at least 6 months, (3) proficient in English, and (4) accompanied by a caregiver who was proficient in English and willing to participate.  Participants self-reported sociodemographic characteristics and completed surveys to assess their/their child's desire for parenthood and other life goals, and reproductive health knowledge.

Results: Eighteen patient-caregiver dyads completed the study (78.3% of those eligible); 61.1% indicated that they wanted to have future biological children.  Few participants reported receiving information about fertility (16.7% of AYA and 27.8% of caregivers) or birth control (11.1% of AYA and 22.2% of caregivers) from their/their child's health care provider, and the majority had received no information on these topics. Less than half of participants reported that SCD (22.2% of AYA and 50.0% of caregivers) or hydroxyurea (11.1% of AYA and 27.8% of parents) could potentially impair fertility.

Conclusions: Biological parenthood was important to this cohort yet fertility and reproductive health knowledge was low, suggesting that clinicians should prioritize conversations about infertility risk and birth control options with AYA with SCD on hydroxyurea and their caregivers.  More research is needed to identify optimal approaches to these discussions.
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http://dx.doi.org/10.1002/pbc.26829DOI Listing
February 2018

Losartan for the nephropathy of sickle cell anemia: A phase-2, multicenter trial.

Am J Hematol 2017 Sep 19;92(9):E520-E528. Epub 2017 Jul 19.

Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Nephropathy is a common and progressive complication of sickle cell anemia (SCA). In SCA mice, we found that hyperangiotensinemia in the absence of hypertension underlies nephropathy, and its downregulation by losartan, an angiotensin-II-receptor-1 blocker, reduced albuminuria and progression of nephropathy. Therefore, we performed a phase-2 trial of oral losartan, given for 6 months, to explore whether it reduced albuminuria in children and adults with SCA. Participants were allocated to groups defined by class of baseline urinary albumin-to-creatinine ratio (UACR): no albuminuria (NoA), microalbuminuria (MicroA), and macroalbuminuria (MacroA). The primary endpoint was a ≥25% reduction UACR from baseline. There were 32 evaluable participants (mean age 24 years; NoA = 14, MicroA = 12, MacroA = 6). The primary endpoint was met in 83% of the MacroA group (P < 0.0001) and 58% of the MicroA group (P < 0.0001). Median fold-change in UACR was -0.74 for MacroA and -0.46 for MicroA. In MacroA and MicroA, UACR classification improved in 50% but worsened in 11%. Urine osmolality and estimated glomerular filtration rate (eGFR) did not change significantly. Losartan was discontinued in three participants [leg cramps, N = 1; decline in eGFR >25% (142➝104 mL/minute/1.73 m ), N = 1; rise in serum creatinine >50% (0.2➝0.3 mg/dL), N = 1]. Albuminuria was associated with diastolic dysfunction and impaired functional capacity, although cardiopulmonary status was unchanged after 6 months of losartan therapy. In summary, losartan decreased urinary albumin excretion in most participants with albuminuria. Those with macroalbuminuria had the greatest benefit. This study forms the basis for a phase-3, randomized, placebo-controlled trial of losartan for the nephropathy of SCA.
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http://dx.doi.org/10.1002/ajh.24810DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5546943PMC
September 2017

Secondhand Smoke Is an Important Modifiable Risk Factor in Sickle Cell Disease: A Review of the Current Literature and Areas for Future Research.

Int J Environ Res Public Health 2016 11 12;13(11). Epub 2016 Nov 12.

Divisions of Hematology and Pediatric Hematology/Oncology, Duke University School of Medicine, Durham, NC 27710, USA.

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.
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http://dx.doi.org/10.3390/ijerph13111131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5129341PMC
November 2016

ENHANCE-(Electronic Hydroxyurea Adherence): A Protocol to Increase Hydroxyurea Adherence in Patients with Sickle Cell Disease.

JMIR Res Protoc 2016 Oct 3;5(4):e193. Epub 2016 Oct 3.

Nationwide Children's Hospital, The Ohio State University School of Medicine, Columbus, OH, United States.

Background: Hydroxyurea (HU) is the only disease-modifying medication for patients with sickle cell disease (SCD). HU can reduce SCD-related complications but only 35% to 50% of pediatric patients adhere to HU at the rates achieved in clinical trials and this limits its clinical effectiveness. Mobile Directly Observed Therapy (Mobile DOT) is a pilot-tested, electronic, multidimensional, HU adherence intervention that targets many components of the Health Behavior Model.

Objective: The aim of this study is to evaluate the impact of Mobile DOT on HU adherence in children with SCD. The objective of our study is to inform the development of future adherence interventions and pediatric SCD protocols.

Methods: This is a single-arm crossover study of pediatric patients with SCD. Participants self-record videos of their daily HU administrations and receive text message alerts to take HU, feedback on their HU adherence, and incentives when they achieve adherence goals during the 6-month Mobile DOT phase. Participants' HU adherence during the Mobile DOT phase is compared with their baseline HU adherence (6 months prior to study entry) and to their HU adherence 6 months after completing the Mobile DOT phase. The primary outcome of this study is HU adherence measured by medication possession ratio.

Results: The trial is ongoing. Preliminary review of participant satisfaction results suggest that most participants can complete Mobile DOT in less than 5 minutes per day and are satisfied with the intervention.

Conclusions: If effective, the Mobile DOT strategy will increase HU adherence and this could improve patients' clinical outcomes and reduce costs of care.
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http://dx.doi.org/10.2196/resprot.6403DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5067359PMC
October 2016

Thrombocytopenia Pitfalls: Misdiagnosing Type 2B von Willebrand Disease as Ethylenediaminetetraacetic Acid-Dependent Pseudothrombocytopenia.

J Pediatr 2016 Aug 20;175:238-238.e1. Epub 2016 May 20.

Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio.

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http://dx.doi.org/10.1016/j.jpeds.2016.04.077DOI Listing
August 2016

Method-dependent Discrepancies in Fetal Hemoglobin Quantification in Patients With Hemoglobin S.

J Pediatr Hematol Oncol 2016 07;38(5):402-5

*Division of Pediatric Hematology/Oncology/BMT †Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital ‡Department of Pathology, Ohio State University Wexner Medical Center, Columbus, OH §The Permanente Medical Group Regional Laboratories, Kaiser Permanente Northern California, Berkeley, CA ∥Department of Pathology and Laboratory Medicine, St Paul's Hospital ¶Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada.

Hemoglobin F (HbF) concentration is used in the diagnosis of certain hemoglobinopathies and accurate quantification is central to treatment of patients with sickle cell disease. The 2 most commonly used methods to quantify HbF are high performance liquid chromatography and capillary zone electrophoresis. This study reports discrepancies in HbF quantification between these methods when hemoglobin S is present in the sample. Clinicians and investigators should be mindful of the method used for HbF quantification when evaluating and treating patients who produce hemoglobin S.
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http://dx.doi.org/10.1097/MPH.0000000000000575DOI Listing
July 2016

Hydroxyurea use in Children with Sickle Cell Disease: Do Severely Affected Patients Use It and Does It Impact Hospitalization Outcomes?

Pediatr Blood Cancer 2016 May 21;63(5):844-7. Epub 2016 Jan 21.

Division of Pediatric Hematology and Oncology, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio.

Background: Expert guidelines recommend that hydroxyurea (HU) be offered to all children with hemoglobin SS and Sβ(0) sickle cell disease (SCD) and be considered for children with clinically severe hemoglobin SC or Sβ(+) . This study aims to determine the rate of HU use in hospitalized children, if HU is differentially used in children with clinically severe SCD, and if HU users have shorter length of stay (LOS), fewer intensive care unit (ICU) admissions, and fewer inpatient transfusions compared to nonusers.

Procedure: Using the Pediatric Health Information System, we performed a retrospective analysis of children ages 2-18 years with SCD discharged between January 1, 2011 and September 30, 2014. We defined patients as having clinically severe SCD if they had a recent ICU admission or ≥3 admissions in the preceding year.

Results: Of the 2,665 unique children identified, approximately 80% had an inpatient code indicating HU use. Significantly more (p < 0.001) nonusers (30.1%) had a recent ICU admission compared to HU users (18.7%). More nonusers (33.9%) had a history of ≥3 admissions compared to HU users (21.5%) (p < 0.001). After applying propensity score weighting, the groups did not differ in their LOS, prevalence of ICU admissions, or prevalence of transfusions.

Conclusions: HU use is high among hospitalized children with SCD. However, HU is not utilized by many children with clinically severe SCD. These results support that HU be considered in children with SCD to prevent hospitalization rather than as a treatment to improve hospitalization outcomes.
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http://dx.doi.org/10.1002/pbc.25894DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4801693PMC
May 2016

Hydroxyurea therapy for children with sickle cell disease: describing how caregivers make this decision.

BMC Res Notes 2015 Aug 25;8:372. Epub 2015 Aug 25.

Division of General Academic Pediatrics, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, USA.

Background: Hydroxyurea (HU) is underutilized in children with sickle cell disease (SCD) because caregivers frequently decline HU when it is offered. This study explores what impacts this decision.

Results: Caregivers of children with clinically severe SCD whose children were offered HU previously were interviewed. We used a qualitative analytical approach to analyze their telephone interview transcripts. Caregivers who chose HU (n = 9) reported their children had severe SCD, sought detailed information about HU, and accepted HU as a preventative therapy. In contrast, caregivers who did not choose HU (n = 10) did not perceive their children as having severe SCD and did not question their child's provider about HU.

Conclusions: This study identifies specific areas that providers should address to when they discuss HU with families so that they can make informed decisions. Our study also uncovered factors that are important to consider when designing future interventions to improve hydroxyurea acceptance and when developing decision-aid tools to assist caregivers of children with SCD who are considering disease modifying therapies.
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http://dx.doi.org/10.1186/s13104-015-1344-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4548690PMC
August 2015

Identification of Unique, Heterozygous Germline Mutation, STK11 (p.F354L), in a Child with an Encapsulated Follicular Variant of Papillary Thyroid Carcinoma within Six Months of Completing Treatment for Neuroblastoma.

Pediatr Dev Pathol 2015 Jul-Aug;18(4):318-23. Epub 2015 Mar 9.

1 Division of Endocrinology, Department of Pediatrics, Children's Hospital of Pittsburgh, UPMC, 4401 Penn Ave, Pittsburgh, PA, USA.

Papillary thyroid carcinoma (PTC) is rare in children, although it is a known secondary malignancy after treatment for neuroblastoma (NB). The interval between NB treatment completion and PTC is usually more than 5 years. A 4-year-old, female patient with a high risk adrenal NB was found to have a 2.9-cm, right thyroid nodule on surveillance chest computed tomography (CT) 6 months after completion of her NB treatment (induction chemotherapy, tumor resection, autologous stem cell transplantation, external beam radiation to the abdominal tumor site, immunotherapy, and retinoic acid). Posttreatment surveillance included iodine-123-metaiodobenzylguanidine scans and CT scans. Fine-needle aspiration of the thyroid nodule diagnosed a follicular neoplasm, which was negative for BRAF, NRAS, KRAS, HRAS, PAX8/PPARg, and RET/PTC mutations, without evidence of metastatic NB. Nodule histology demonstrated an encapsulated follicular variant of PTC (FVPTC). Next-generation sequence analysis for a 46 cancer-gene profile was performed on both tumors with subsequent peripheral blood DNA testing. A heterozygous missense mutation in STK11 (F354L) was identified in both the NB and FVPTC. This mutation was also detected in peripheral blood mononuclear cells. Two additional heterozygous somatic missense mutations of uncertain significance were identified: KDR/VEGF receptor 2 (Q472H) on chromosome 4 and MET (N375S) on chromosome 7. To our knowledge, this is the shortest reported duration from completion of NB treatment to detection of thyroid cancer. The association of the STK11 gene with Peutz-Jeghers syndrome, lung adenocarcinomas, and medullary thyroid cancer leads to a possible association between this genetic variant and our patient's tumors.
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http://dx.doi.org/10.2350/15-01-1597-CR.1DOI Listing
September 2015

Prodromal illness before acute chest syndrome in pediatric patients with sickle cell disease.

J Pediatr Hematol Oncol 2014 Aug;36(6):480-3

*Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital, Columbus, OH †Division of Pediatric Hematology/Oncology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.

Background: Acute chest syndrome (ACS) is associated with morbidity and mortality in children with sickle cell disease. We hypothesize that children with sickle cell disease have a distinct prodromal illness before their ACS episode.

Materials And Methods: We performed a chart review of ICD-9-CM identified ACS episodes at a pediatric hospital from 2005 to 2010. Prodromal visits were defined as acute visits that resulted in a discharge from care and occurred within 2 weeks of a hospitalization that included ACS. We reviewed the documented history, examination, laboratory studies, and radiographs for each prodromal visit.

Results: We identified 196 ACS episodes. Children received prodromal care in 29% of the ACS episodes. Painful vaso-occlusive crisis was a common reason for seeking this care (61%) and was commonly located in the chest or back (81%). We also observed that patients were hypoxic (53%), tachypneic (29%), had a history of asthma (39%) or ACS (80%), and presented during the winter months (38%).

Conclusions: These data suggest that nearly one third of patients who develop ACS seek care for a prodromal illness. Further research is needed to confirm and better define an ACS prodromal illness that may help to identify patients at high risk for developing ACS.
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http://dx.doi.org/10.1097/MPH.0000000000000146DOI Listing
August 2014

A pilot study of electronic directly observed therapy to improve hydroxyurea adherence in pediatric patients with sickle-cell disease.

Pediatr Blood Cancer 2014 Jun 16;61(6):1068-73. Epub 2014 Jan 16.

Division of Pediatric Hematology-Oncology, Nationwide Children's Hospital, Columbus, Ohio.

Background: Poor hydroxyurea (HU) adherence limits effective HU use in patients with sickle cell disease (SCD). Electronic directly observed therapy (DOT) may limit costs and achieve high HU adherence in children with SCD. This study aimed to determine if electronic DOT was feasible, acceptable, and could achieve ≥ 90% HU adherence.

Procedure: Children with SCD were recruited for this single institution, 6-month pilot study if they had been prescribed HU for ≥ 6 months and had daily access to a smartphone or computer. Participants submitted HU administration videos daily and received electronic reminder alerts, personalized feedback, and incentives to encourage adherence as part of electronic DOT. Primary outcomes were feasibility, participant satisfaction with electronic DOT, and HU adherence. Secondary outcomes included mean corpuscular volume (MCV), hemoglobin F percentage (HbF), and overall participant satisfaction with HU therapy.

Results: Of 15 enrolled participants, 14 completed the study. Satisfaction surveys showed electronic DOT reminded participants to take HU and could be completed in fewer than 5 minutes daily. Participants' median medication possession ratio at study entry improved from 0.75 (0.59-0.82) to 0.91 (0.85-1.00) (P = 0.02) at the end of the study. Overall median observed HU adherence with electronic DOT was 93.3%. Median MCV and HbF increased from 96.0 to 107.2 (P = 0.009) and 10.5 to 11.4 (P = 0.03), respectively.

Conclusions: This study demonstrates electronic DOT is feasible, acceptable, and can achieve high HU adherence. Further study is needed to confirm that electronic DOT can improve HU adherence and impact clinical outcomes in children with SCD.
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http://dx.doi.org/10.1002/pbc.24931DOI Listing
June 2014

Clinical course of postthrombotic syndrome in children with history of venous thromboembolism.

Blood Coagul Fibrinolysis 2012 Jan;23(1):39-44

Department of Pediatrics, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA.

Postthrombotic syndrome (PTS) is a chronic morbidity of venous thromboembolism (VTE) in children. Information about the evolution of PTS is lacking in children. Present study was aimed to evaluate the time-course of extremity PTS in children who were serially followed in a hematology clinic. This retrospective cohort study included 69 consecutive children with documented VTEs that presented with symptoms of extremity VTE: 67 extremity VTEs with or without extension to vena cava, 2 inferior vena cava VTEs. Severity of PTS was assessed using modified Villalta scale. Median age of the cohort was 12.6 years (interquartile range 1.6-15 years) while median follow-up was 28.7 months (interquartile range 13.3-33.4 months. PTS prevalence was 46.8% [95% confidence interval (CI) 37.9-57.7%]. Lower extremity VTE was associated with development of PTS compared to upper extremity VTE regardless of catheter use (P = 0.002). The time-course of PTS fluctuated in 11 of 33 children (33%; 95% CI 20-47%) at a median interval of 12 months from diagnosis of VTE (range 4-14 months): three progressed from mild/moderate to severe, one improved from moderate to mild, seven fluctuated between mild and moderate. Recurrence and incomplete resolution of VTE were associated with variability in PTS severity (P < 0.05). In summary, this study suggested that almost 50% of study cohort developed PTS, and the time-course of PTS was not static in one third of children. Future research should focus on identifying the predictors contributing to the worsening of PTS and developing risk-stratified treatment interventions so as to improve the outcome of children with VTE.
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http://dx.doi.org/10.1097/MBC.0b013e32834bdb1cDOI Listing
January 2012