Scholars@Duke
Nirmish Ramesh Shah

Nirmish Ramesh Shah

Associate Professor of Medicine
Medicine, Hematology
nirmish.shah@duke.edu
Department of Medicine, Duke S, Durham, NC 27710
Department of Medicine, Duke S, Durham, NC 27710

Scholarly Works - Journal articles

Pre-implementation Barriers and Facilitators to Integrating Complementary and Integrative Health Interventions into Clinic Workflow: The GRACE Trial, NIH Pragmatic Trials Collaboratory.

Journal article Pain Manag Nurs · August 2026 BACKGROUND: Even though there is clear evidence supporting the use of complementary and integrative health (CIH) interventions for chronic pain, their integration into clinical care is challenging. Thus, the purpose of this study was to understand how the ... Full text Link to item Cite

Evaluating a Pediatric to Adult Care Transition Program for Adolescents and Young Adults With Sickle Cell Disease: A Program Assessment at Duke University.

Journal article J Pediatr Hematol Oncol · August 1, 2026 BACKGROUND: Only 33% to 53% of adolescents and young adults (AYAs) with sickle cell disease (SCD) complete the transition process to adult health systems. OBJECTIVE: Examine transition-readiness practices in a pediatric SCD ambulatory clinic to identify st ... Full text Link to item Cite

A new method for augmenting short time series, with application to pain events in sickle cell disease.

Journal article PLoS Comput Biol · June 2026 Researchers across different fields, including but not limited to ecology, biology, and healthcare, often face the challenge of sparse data. Such sparsity can lead to uncertainties, estimation difficulties, and potential biases in modeling. Here we introdu ... Full text Link to item Cite

Safety and effectiveness of voxelotor in individuals with sickle cell disease in the RETRO and PROSPECT US registries.

Journal article Blood Adv · May 12, 2026 Two registry studies, RETRO and PROSPECT, were conducted to evaluate real-world safety and effectiveness outcomes in US participants with sickle cell disease (SCD) who received voxelotor as standard of care. RETRO collected retrospective data of participan ... Full text Link to item Cite

Sustainment of Hydroxyurea Adherence in Patients With Sickle Cell Disease.

Journal article JAMA Netw Open · May 1, 2026 IMPORTANCE: Despite established treatment guidelines and strong therapeutic benefit, hydroxyurea remains underused among patients with sickle cell disease. OBJECTIVE: To assess the change in self-reported hydroxyurea use (ie, taking the medication at all) ... Full text Link to item Cite

Ciliary protein ARL13b detected on RBCs as a potential indicative biomarker of vaso-occlusive crisis and disease severity in SCD: a retrospective pilot study.

Journal article J Sick Cell Dis · 2026 OBJECTIVES: Assessing SCD phenotype is challenging, hindering progress in care and quality of life. Pain crises are the hallmark of SCD, but no reliable prognostic biomarker exists due to its complex pathophysiology. ADP-ribosylation factor-like protein 13 ... Full text Link to item Cite

Digital cognitive behavioral therapy versus Education for chronic pain in adults with sickle cell disease: 12-month randomized trial results

Journal article Annals of Behavioral Medicine · January 1, 2026 Background: Chronic pain affects up to 40% of adults with sickle cell disease (SCD), yet treatment options remain limited. While cognitive behavioral therapy (CBT) is effective in other chronic pain conditions, it is underutilized for SCD pain, and its eff ... Full text Cite

Evidence and gaps in clinical outcomes of novel pharmacologic therapies for sickle cell disease: A systematic literature review highlighting insights from clinical trials and real-world studies.

Journal article Blood Rev · August 2025 This systematic review aims to summarise the clinical outcomes of l-glutamine, crizanlizumab, and voxelotor in the treatment of sickle cell disease (SCD) based on clinical trials and real-world data and to identify any gaps in the observations. The review ... Full text Link to item Cite

User Perceptions of a Multilevel mHealth Intervention to Boost Adherence to Hydroxyurea in Sickle Cell Disease.

Journal article Pediatr Blood Cancer · July 2025 BACKGROUND: Adherence to hydroxyurea has been low historically. The InCharge Health app demonstrates increased adherence with hydroxyurea, but engagement has not been fully investigated. This study investigated the association between InCharge Health app p ... Full text Link to item Cite

Implementing ASH's guidelines for acute medical care for incarcerated children and adults with sickle cell disease.

Journal article Blood Adv · June 10, 2025 Between 2019 and 2021, the American Society of Hematology (ASH) developed clinical guidelines for managing sickle cell disease (SCD), covering acute pain, acute neurological events, and other complications. However, these guidelines lacked implementation s ... Full text Link to item Cite

Patient-reported pregnancy loss and maternal complications: Insights from the sickle cell disease implementation consortium.

Journal article Int J Gynaecol Obstet · March 2025 OBJECTIVE: Sickle cell disease (SCD) is associated with complications during pregnancy and can negatively influence maternal outcomes. Our study aimed to determine the prevalence and predictors of maternal morbidity among participants enrolled in an eight- ... Full text Link to item Cite

Depression, sleep and pain affect instrumental activities of daily living through cognitive functioning in adults with sickle cell disease: A report from the Sickle Cell Disease Implementation Consortium.

Journal article Br J Haematol · March 2025 Depression, disrupted sleep and pain are common comorbidities in sickle cell disease. We tested (1) if these comorbidities are associated with attention/executive functioning, processing speed and instrumental activities of daily living (IADLs), which desc ... Full text Link to item Cite

Duration of anticoagulation for venous thromboembolism in pediatric patients: Evaluation of the Duration of Therapy for Thrombosis in Children (Kids-DOTT) trial outcomes at 2 years.

Journal article J Thromb Haemost · February 2025 BACKGROUND: The Multicenter Evaluation of the Duration of Therapy for Thrombosis in Children multinational, randomized clinical trial revealed noninferiority of a 6-week vs 3-month duration of anticoagulation for the treatment of provoked venous thromboemb ... Full text Link to item Cite

The use of abstract animations and a graphical body image for assessing pain outcomes among adults with sickle cell disease.

Journal article J Pain · January 2025 Painimation, a novel digital pain assessment tool, allows patients to communicate their pain quality, intensity, and location using abstract animations (painimations) and a paintable body image. This study determined the construct validity of painimations ... Full text Link to item Cite

Careful considerations for digital health innovation: developing Nanbar Health-a digital health solution empowering clinical decisions with data-driven insights.

Journal article Mhealth · 2025 BACKGROUND: Digital health tools have grown in their usage and popularity since the coronavirus disease 2019 (COVID-19) pandemic, when healthcare providers were required to find methods of monitoring and engaging with their patients while also practicing s ... Full text Link to item Cite

Digital cognitive behavioral therapy vs education for pain in adults with sickle cell disease.

Journal article Blood Adv · December 24, 2024 Despite the burden of chronic pain in sickle cell disease (SCD), nonpharmacological approaches remain limited. This multisite, randomized trial compared digital cognitive behavioral therapy (CBT) with a digital pain/SCD education program ("Education") for ... Full text Link to item Cite

Barriers and Facilitators to Integrating Acupuncture into the U.S. Health Care System: A Scoping Review.

Journal article J Integr Complement Med · December 2024 Background: Acupuncture is a widely practiced complementary and integrative health modality that has multiple clinical applications. The use of acupuncture in the United States is rapidly increasing. Although studies have shown the efficacy and effectivene ... Full text Link to item Cite

Black Americans With Sickle Cell Disease (SCD) Demonstrate Accelerated Epigenetic Pace of Aging Compared to Black Americans Without SCD.

Journal article J Gerontol A Biol Sci Med Sci · November 1, 2024 BACKGROUND: Sickle cell disease (SCD) is a chronic medical condition characterized by red blood cell sickling, vaso-occlusion, hemolytic anemia, and subsequently, end-organ damage and reduced survival. Because of this significant pathophysiology and early ... Full text Link to item Cite

Epigenetic Aging Associations With Psychoneurological Symptoms and Social Functioning in Adults With Sickle Cell Disease.

Journal article Biol Res Nurs · October 2024 Objective: Sickle cell disease (SCD), the most common inherited blood disorder in the United States, is associated with severe psychoneurological symptoms. While epigenetic age acceleration has been linked to psychoneurological symptom burden in other dise ... Full text Link to item Cite

The clinical spectrum of HbSC sickle cell disease-not a benign condition.

Journal article Br J Haematol · August 2024 Sickle cell disease (SCD) includes a group of heterogenous disorders that result in significant morbidities. HbSS is the most common type of SCD and HbSC is the second most common type of SCD. The prevalence of HbSC disease in the United States and United ... Full text Link to item Cite

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

Journal article Blood Adv · July 23, 2024 Guidelines recommend transfer to adult health care within 6 months of completing pediatric care; however, this has not been studied in sickle cell disease (SCD). We hypothesized that longer transfer gaps are associated with increased resource utilization. ... Full text Link to item Cite

Cost-Effectiveness of Lovotibeglogene Autotemcel (Lovo-Cel) Gene Therapy for Patients with Sickle Cell Disease and Recurrent Vaso-Occlusive Events in the United States.

Journal article Pharmacoeconomics · June 2024 BACKGROUND AND OBJECTIVE: Gene therapies for sickle cell disease (SCD) may offer meaningful benefits for patients and society. This study evaluated the cost-effectiveness of lovotibeglogene autotemcel (lovo-cel), a one-time gene therapy administered via au ... Full text Link to item Cite

Mortality in adults with sickle cell disease: Results from the sickle cell disease implementation consortium (SCDIC) registry.

Journal article Am J Hematol · May 2024 The cause of death in people affected by sickle cell disease (SCD) is often challenging to define as prior studies have used retrospective or administrative data for analysis. We used a prospective longitudinal registry to assess mortality and clinical co- ... Full text Link to item Cite

Opioid Use Among Children and Adults With Sickle Cell Disease in North Carolina Medicaid Enrollees in the Era of Opioid Harm Reduction.

Journal article J Pediatr Hematol Oncol · May 1, 2024 Adults and children with sickle cell disease (SCD) are predominantly African American, with pain-related health disparities. We examined opioid prescription fill patterns in adults and children with SCD and compared factors associated with fills in North C ... Full text Link to item Cite

Recruitment Strategies in the Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization Study (meSH): Multicenter Survey Study.

Journal article JMIR Form Res · April 16, 2024 BACKGROUND: Hydroxyurea is an evidence-based disease-modifying therapy for sickle cell disease (SCD) but is underutilized. The Integration of Mobile Health into Sickle Cell Disease Care to Increase Hydroxyurea Utilization (meSH) multicenter study leveraged ... Full text Link to item Cite

Mental health, pain and likelihood of opioid misuse among adults with sickle cell disease.

Journal article Br J Haematol · March 2024 Depressive symptoms are prevalent in individuals living with sickle cell disease (SCD) and may exacerbate pain. This study examines whether higher depressive symptoms are associated with pain outcomes, pain catastrophizing, interference and potential opioi ... Full text Link to item Cite

Characterizing epigenetic aging in an adult sickle cell disease cohort.

Journal article Blood Adv · January 9, 2024 Sickle cell disease (SCD) affects ∼100 000 predominantly African American individuals in the United States, causing significant cellular damage, increased disease complications, and premature death. However, the contribution of epigenetic factors to SCD pa ... Full text Open Access Link to item Cite

Biopsychosocial Factors Associated With Pain and Pain-Related Outcomes in Adults and Children With Sickle Cell Disease: A Multivariable Analysis of the GRNDaD Multicenter Registry.

Journal article J Pain · January 2024 Pain is the primary symptomatic manifestation of sickle cell disease (SCD), an inherited hemoglobinopathy. The characteristics that influence pain experiences and outcomes in SCD are not fully understood. The primary objective of this study was to use mult ... Full text Link to item Cite

Supporting data driven translational patient-centered care using network analysis to visualize symptom distress in children with serious illness.

Journal article J Spec Pediatr Nurs · January 2024 PURPOSE: There are an increasing number of techniques and tools to improve the capacity for children to relay their perceptions of their symptom experience while undergoing blood and marrow transplant (BMT). Network analysis (NA) is a tool that can illustr ... Full text Link to item Cite

Pharmacokinetics, pharmacodynamics, safety, and efficacy of crizanlizumab in patients with sickle cell disease: final results from the phase II SOLACE-adults study.

Journal article Ther Adv Hematol · 2024 BACKGROUND: Crizanlizumab is a novel inhibitor of P-selectin, a key player in multicellular adhesion and inflammatory signaling, that leads to vaso-occlusion in sickle cell disease (SCD). OBJECTIVES: The SOLACE-adults study evaluated the pharmacokinetics, ... Full text Link to item Cite

A multilevel mHealth intervention boosts adherence to hydroxyurea in individuals with sickle cell disease.

Journal article Blood Adv · December 12, 2023 Hydroxyurea reduces sickle cell disease (SCD) complications, but medication adherence is low. We tested 2 mobile health (mHealth) interventions targeting determinants of low adherence among patients (InCharge Health) and low prescribing among providers (HU ... Full text Open Access Link to item Cite

Preferences for potential benefits and risks for gene therapy in the treatment of sickle cell disease.

Journal article Blood Adv · December 12, 2023 Objective of this study is to quantify benefit-risk tradeoffs pertaining to potential gene therapies among adults and parents/caregivers of children with sickle cell disease (SCD). A discrete-choice experiment survey was developed in which respondents sele ... Full text Link to item Cite

Monitoring and responding to signals of suicidal ideation in pragmatic clinical trials: Lessons from the GRACE trial for Chronic Sickle Cell Disease Pain.

Journal article Contemp Clin Trials Commun · December 2023 Sickle cell disease (SCD) is a hemoglobin disorder and the most common genetic disorder that affects 100,000 Americans and millions worldwide. Adults living with SCD have pain so severe that it often requires opioids to keep it in control. Depression is a ... Full text Link to item Cite

Developing an Implementation Blueprint for the NIH HEAL Initiative GRACE Trial: Perspectives on Acupuncture and Guided Relaxation for Chronic Sickle Cell Disease Pain.

Journal article J Integr Complement Med · October 2023 Objective: This study aimed to explore perspectives of people living with sickle cell disease (SCD) and SCD clinic providers and staff about the use of acupuncture and guided relaxation for treating chronic SCD pain. Data obtained were to inform an impleme ... Full text Link to item Cite

Feasibility of Implementation of a Mobile Digital Personal Health Record to Coordinate Care for Children and Youth With Special Health Care Needs in Primary Care: Protocol for a Mixed Methods Study.

Journal article JMIR Res Protoc · September 20, 2023 BACKGROUND: Electronic health record (EHR)-integrated digital personal health records (PHRs) via Fast Healthcare Interoperability Resources (FHIR) are promising digital health tools to support care coordination (CC) for children and youth with special heal ... Full text Link to item Cite

Outcomes of End-User Testing of a Care Coordination Mobile App With Families of Children With Special Health Care Needs: Simulation Study.

Journal article JMIR Form Res · August 28, 2023 BACKGROUND: Care for children with special health care needs relies on a network of providers who work to address the medical, behavioral, developmental, educational, social, and economic needs of the child and their family. Family-directed, manually creat ... Full text Link to item Cite

Infertility and treatment-seeking practices among females and males with sickle cell disease in the Sickle Cell Disease Implementation Consortium registry.

Journal article Pediatr Blood Cancer · July 2023 OBJECTIVE: To describe the prevalence of infertility and infertility treatment seeking among people enrolled in the Sickle Cell Disease Implementation Consortium (SCDIC) registry and identify sociodemographic and clinical correlates of infertility. DESIGN: ... Full text Link to item Cite

Hybrid effectiveness-implementation trial of guided relaxation and acupuncture for chronic sickle cell disease pain (GRACE): A protocol.

Journal article Contemp Clin Trials Commun · April 2023 BACKGROUND: People with sickle cell disease frequently use complementary and integrative therapies to cope with their pain, yet few studies have evaluated their effectiveness. The 3-arm, 3-site pragmatic Hybrid Effectiveness-implementation Trial of Guided ... Full text Link to item Cite

Pharmacokinetics, pharmacodynamics, safety, and efficacy of crizanlizumab in patients with sickle cell disease.

Journal article Blood Adv · March 28, 2023 Crizanlizumab is an anti-P-selectin monoclonal antibody indicated to reduce the frequency/prevent recurrence of vaso-occlusive crises (VOCs) in patients with sickle cell disease (SCD) aged ≥16 years. This analysis of an ongoing phase 2, nonrandomized, open ... Full text Link to item Cite

Predicting Pain in People With Sickle Cell Disease in the Day Hospital Using the Commercial Wearable Apple Watch: Feasibility Study.

Journal article JMIR Form Res · March 14, 2023 BACKGROUND: Sickle cell disease (SCD) is a genetic red blood cell disorder associated with severe complications including chronic anemia, stroke, and vaso-occlusive crises (VOCs). VOCs are unpredictable, difficult to treat, and the leading cause of hospita ... Full text Link to item Cite

Characterising the prevalence of overweight and obese status among adults with sickle cell disease.

Journal article Br J Haematol · March 2023 Individuals with sickle cell disease (SCD) have historically been considered underweight. Despite increasing body mass index (BMI) in the general population, the prevalence of overweight and obese status remains unclear in the adult SCD population. Our pri ... Full text Link to item Cite

Evaluating Associations between Average Pain Intensity and Genetic Variation in People with Sickle Cell Disease: An Exploratory Study.

Journal article Pain Manag Nurs · February 2023 BACKGROUND: Pain is one of the most common and deleterious symptoms experienced by individuals with sickle cell disease (SCD). There is a paucity of studies identifying potential genetic mechanisms of pain in this population. AIM: Examine associations betw ... Full text Link to item Cite

Intrahepatic cholestasis in sickle cell disease: A review of diagnostic criteria, treatments, and case reports.

Journal article J Natl Med Assoc · February 2023 Objective To delineate the etiology, symptomatology, and treatment of sickle cell intrahepatic cholestasis (SCIC). Sickle cell disease (SCD) is the most frequently inherited hematologic disease, and SCIC is one rare and often fatal complication and comorbi ... Full text Link to item Cite

Consensus definition of essential, optimal, and suggested components of a pediatric sickle cell disease center.

Journal article Pediatr Blood Cancer · January 2023 Sickle cell disease (SCD) requires coordinated, specialized medical care for optimal outcomes. There are no United States (US) guidelines that define a pediatric comprehensive SCD program. We report a modified Delphi consensus-seeking process to determine ... Full text Link to item Cite

Use of consumer wearables to monitor and predict pain in patients with sickle cell disease.

Journal article Front Digit Health · 2023 BACKGROUND: In sickle cell disease (SCD), unpredictable episodes of acute severe pain, known as vaso-occlusive crises (VOC), disrupt school, work activities and family life and ultimately lead to multiple hospitalizations. The ability to predict VOCs would ... Full text Link to item Cite

Early Initiation of Sub-Anesthetic Ketamine Infusion in Adults with Vaso-Occlusive Crises Is Associated with Greater Reduction in Sickle Cell Pain Intensity: A Single Center's Experience.

Journal article Pain Med · December 1, 2022 OBJECTIVES: Recurrent, severely painful episodes, known as vaso-occlusive crises (VOCs) are the hallmark of sickle cell disease (SCD) and the primary reason for hospitalization. Opioids have been the gold standard for VOC treatment without significant impr ... Full text Link to item Cite

A clinician's view of voxelotor.

Journal article Br J Haematol · November 2022 Full text Link to item Cite

Impact of the COVID-19 Pandemic on the Implementation of Mobile Health to Improve the Uptake of Hydroxyurea in Patients With Sickle Cell Disease: Mixed Methods Study.

Journal article JMIR Form Res · October 14, 2022 BACKGROUND: Hydroxyurea therapy is effective for reducing complications related to sickle cell disease (SCD) and is recommended by National Health Lung and Blood Institute care guidelines. However, hydroxyurea is underutilized, and adherence is suboptimal. ... Full text Link to item Cite

Transitioning Adolescents With Sickle Cell Disease From Pediatric to Adult Care: Results From a New Survey of Health Care Professionals.

Journal article J Pediatr Hematol Oncol · October 1, 2022 Transition from pediatric to adult care for adolescents and young adults (AYA) with sickle cell disease (SCD) comes at a time when a range of biopsychosocial issues occur simultaneously. A new survey sought information from physicians who treat AYA with SC ... Full text Link to item Cite

An evaluation of patient-reported outcomes in sickle cell disease within a conceptual model.

Journal article Qual Life Res · September 2022 PURPOSE: To examine the relations between patient-reported outcomes (PROs) within a conceptual model for adults with sickle cell disease (SCD) ages 18 - 45 years enrolled in the multi-site Sickle Cell Disease Implementation Consortium (SCDIC) registry. We ... Full text Link to item Cite

Improving Pain Assessment Using Vital Signs and Pain Medication for Patients With Sickle Cell Disease: Retrospective Study.

Journal article JMIR Form Res · June 23, 2022 BACKGROUND: Sickle cell disease (SCD) is the most common inherited blood disorder affecting millions of people worldwide. Most patients with SCD experience repeated, unpredictable episodes of severe pain. These pain episodes are the leading cause of emerge ... Full text Link to item Cite

Pregnancy outcomes with hydroxyurea use in women with sickle cell disease.

Journal article Am J Hematol · May 2022 Hydroxyurea reduces pain crises, acute chest syndrome, and blood transfusions in sickle cell disease (SCD), but potential detrimental effects on fertility and birth outcomes impede its use. Data on the effects of hydroxyurea taken for SCD during conception ... Full text Link to item Cite

Real-world effectiveness of voxelotor for treating sickle cell disease in the US: a large claims data analysis.

Journal article Expert Rev Hematol · February 2022 BACKGROUND: Sickle cell disease (SCD) is a genetic disease that impacts patients' quality of life, healthcare costs, and life expectancy. Elevated sickle hemoglobin (HbS), which readily polymerizes, causes red blood cell sickling, leading to chronic hemoly ... Full text Link to item Cite

Severe Persistent Pain and Inflammatory Biomarkers in Sickle Cell Disease: An Exploratory Study.

Journal article Biol Res Nurs · January 2022 BACKGROUND: Severe pain is among the most common and deleterious symptoms experienced by individuals with sickle cell disease (SCD), of whom more than 50% report chronic pain. Despite this, the understanding of the biological contributors to persistent sev ... Full text Link to item Cite

Perspectives of individuals with sickle cell disease on barriers to care.

Journal article PLoS One · 2022 INTRODUCTION: Sickle cell disease (SCD) is an inherited hemoglobinopathy that predominantly affects African Americans in the United States. The disease is associated with complications leading to high healthcare utilization rates, including emergency depar ... Full text Link to item Cite

Usability and Acceptability of the QuestLeukemia Mobile Application: A Pilot Study for An Educational and Psychological Intervention for Children with Chronic Illnesses.

Journal article J Pediatr Hematol Oncol Nurs · 2022 It is widely accepted that educational interventions benefit children with chronic diseases (disease awareness and autonomy) or those undergoing medical procedures (decreased anxiety and improved satisfaction). Hematopoietic cell transplantation (HCT) is a ... Full text Link to item Cite

Careful considerations for mHealth app development: lessons learned from QuestExplore.

Journal article Mhealth · 2022 BACKGROUND: Mobile health (mHealth) applications (app) have proven to be useful in gathering symptom data for a variety of populations living with chronic and serious illnesses. These mHealth tools have been built for a variety of populations but can quick ... Full text Link to item Cite

What does it mean to be affiliated with care?: Delphi consensus on the definition of "unaffiliation" and "specialist" in sickle cell disease.

Journal article PLoS One · 2022 Accruing evidence reveals best practices for how to help individuals living with Sickle Cell Disease (SCD); yet, the implementation of these evidence-based practices in healthcare settings is lacking. The Sickle Cell Disease Implementation Consortium (SCDI ... Full text Open Access Link to item Cite

Evaluating the implementation of a multi-level mHealth study to improve hydroxyurea utilization in sickle cell disease.

Journal article Front Health Serv · 2022 BACKGROUND: Sickle Cell Disease (SCD) is a progressive genetic disease that causes organ damage and reduces longevity. Hydroxyurea is an underutilized evidence-based medication that reduces complications and improves survival in SCD. In a multi-site clinic ... Full text Open Access Link to item Cite

A reanalysis of pain crises data from the pivotal l-glutamine in sickle cell disease trial.

Journal article Contemp Clin Trials · November 2021 The pivotal Endari trial in sickle cell disease showed a reduction in pain crises events. This reanalysis of the l-glutamine phase 3 trial using annual rates of pain crises, consistent with other SCD studies, supported the statistically significant outcome ... Full text Link to item Cite

Severe Pain Profiles and Associated Sociodemographic and Clinical Characteristics in Individuals With Sickle Cell Disease.

Journal article Clin J Pain · September 1, 2021 OBJECTIVE: The aim of this study was to identify classes of individuals with sickle cell disease (SCD) who share distinct severe pain profiles and evaluate differences in demographic, clinical, and psychosocial characteristics between classes. METHODS: Thi ... Full text Link to item Cite

Effective Recruitment Strategies for a Sickle Cell Patient Registry Across Sites from the Sickle Cell Disease Implementation Consortium (SCDIC).

Journal article J Immigr Minor Health · August 2021 Sickle cell disease (SCD) is a genetic disorder predominantly affecting people of African descent and is associated with significant morbidity and mortality. To improve SCD outcomes, the National Heart Lung and Blood Institute funded eight centers to parti ... Full text Link to item Cite

Seriously ill pediatric patient, parent, and clinician perspectives on visualizing symptom data.

Journal article J Am Med Inform Assoc · July 14, 2021 OBJECTIVE: This study examined the perspectives on the use of data visualizations and identified key features seriously ill children, their parents, and clinicians prefer to see when visualizing symptom data obtained from mobile health technologies (an App ... Full text Link to item Cite

Symptom Monitoring in Children With Life-Threatening Illness: A Feasibility Study Using mHealth.

Journal article ANS Adv Nurs Sci · July 2021 Children with life-threatening illness (C-LTI) experience considerable symptom distress. Mobile technology may offer opportunities to better obtain symptom data that will lead to better symptom management. A mixed-methods study was conducted to explore the ... Full text Link to item Cite

Comparing the Effectiveness of Education Versus Digital Cognitive Behavioral Therapy for Adults With Sickle Cell Disease: Protocol for the Cognitive Behavioral Therapy and Real-time Pain Management Intervention for Sickle Cell via Mobile Applications (CaRISMA) Study.

Journal article JMIR Res Protoc · May 14, 2021 BACKGROUND: Patients with sickle cell disease (SCD) experience significant medical and psychological stressors that affect their mental health, well-being, and disease outcomes. Digital cognitive behavioral therapy (CBT) has been used in other patient popu ... Full text Link to item Cite

The impact of vaso-occlusive crises and disease severity on quality of life and productivity among patients with sickle cell disease in the US.

Journal article Curr Med Res Opin · May 2021 AIM: Sickle cell disease (SCD) is a lifelong blood disorder affecting approximately 100,000 individuals in the United States (US). A number of new treatments have recently become available to improve SCD clinical outcomes, but it is unclear how treatment i ... Full text Link to item Cite

Effect of Poloxamer 188 vs Placebo on Painful Vaso-Occlusive Episodes in Children and Adults With Sickle Cell Disease: A Randomized Clinical Trial.

Journal article JAMA · April 20, 2021 IMPORTANCE: Although effective agents are available to prevent painful vaso-occlusive episodes of sickle cell disease (SCD), there are no disease-modifying therapies for ongoing painful vaso-occlusive episodes; treatment remains supportive. A previous phas ... Full text Link to item Cite

Adherence to Iron Chelation Therapy with Deferasirox Formulations among Patients with Sickle Cell Disease and β-thalassemia.

Journal article J Natl Med Assoc · April 2021 BACKGROUND: Individuals with hemoglobinopathies experience complications that often require management with multiple transfusions. These chronic transfusions can lead to iron overload, which places them at increased risk of organ damage, malignancy, and ev ... Full text Link to item Cite

Patient Perspectives of Sickle Cell Management in the Emergency Department.

Journal article Crit Care Nurs Q · April 2021 Sickle cell disease (SCD) is a common genetic blood disorder predominantly affecting African Americans in the United States. The objective of this study was to use a multimethods approach to describe how patients with SCD in North Carolina perceive the car ... Full text Link to item Cite

Dissemination of Evidence-Based Recommendations for Sickle Cell Disease to Primary Care and Emergency Department Providers in North Carolina: A Cost Benefit Analysis.

Journal article J Health Econ Outcomes Res · April 1, 2021 Background: Sickle cell disease (SCD) is a genetic condition affecting primarily individuals of African descent, who happen to be disproportionately impacted by poverty and who lack access to health care. Individuals with SCD are at high likelihood of high ... Full text Open Access Link to item Cite

"Pain is Subjective": A Mixed-Methods Study of Provider Attitudes and Practices Regarding Pain Management in Sickle Cell Disease Across Three Countries.

Journal article J Pain Symptom Manage · March 2021 CONTEXT: Sickle cell disease (SCD), an autosomal recessive blood disorder, affects millions of people worldwide. Approximately 80% of all cases are located in Africa. OBJECTIVES: This cross-national, interdisciplinary, collaborative study investigated prov ... Full text Link to item Cite

Can subjective pain be inferred from objective physiological data? Evidence from patients with sickle cell disease.

Journal article PLoS Comput Biol · March 2021 Patients with sickle cell disease (SCD) experience lifelong struggles with both chronic and acute pain, often requiring medical interventMaion. Pain can be managed with medications, but dosages must balance the goal of pain mitigation against the risks of ... Full text Link to item Cite

Sickle cell disease is a global prototype for integrative research and healthcare.

Journal article Adv Genet (Hoboken) · March 2021 Differences in health outcomes and treatment responses within and between global populations have been well documented. There is growing recognition of the need to move beyond simple inventories and descriptions of these differences and our linear explanat ... Full text Open Access Link to item Cite

Assessing the Feasibility of a Novel mHealth App in Hematopoietic Stem Cell Transplant Patients.

Journal article Transplant Cell Ther · February 2021 Hematopoietic stem cell transplantation (HCT) is a curative treatment option for patients with hematologic conditions but presents many complications that must be managed as a complex, chronic condition. Mobile health applications (mHealth apps) may permit ... Full text Link to item Cite

An Interrupted Time Series Analysis of the Dissemination of a Sickle Cell Vaso-Occlusive Episode Treatment Algorithm and a Case Management Referral Form for Individuals With Sickle Cell Disease in the Emergency Department.

Journal article J Emerg Nurs · January 2021 BACKGROUND: Sickle cell disease is associated with frequent vaso-occlusive episode and emergency department visits. Our group developed (1) a vaso-occlusive episode treatment algorithm based on the National Heart, Lung, and Blood Institute recommendations, ... Full text Link to item Cite

Voxelotor: alteration of sickle cell disease pathophysiology by a first-in-class polymerization inhibitor.

Journal article Ther Adv Hematol · 2021 Sickle cell disease, despite its recognition as a severely debilitating genetic condition affecting hundreds of thousands of neonates throughout the world each year, was not a target of pharmaceutical research focus for most of its 100-year existence in th ... Full text Link to item Cite

A Needs Assessment of Persons With Sickle Cell Disease in a Major Medical Center in North Carolina.

Journal article N C Med J · 2021 BACKGROUND Sickle cell disease (SCD) is a complex disease associated with many complications and a shortened lifespan. In 2016, the National Heart, Lung and Blood Institute funded 8 centers in the United States to form the Sickle Cell Disease Implementatio ... Full text Link to item Cite

Awareness and Use of the Sickle Cell Disease Toolbox by Primary Care Providers in North Carolina.

Journal article J Prim Care Community Health · 2021 BACKGROUND: Sickle cell disease (SCD) is a complex chronic blood disorder characterized by severe disease complications ideally managed by both hematologists and primary care providers (PCP's). PCP's report knowledge gaps and discomfort with SCD management ... Full text Link to item Cite

Sex-based differences in the manifestations and complications of sickle cell disease: Report from the Sickle Cell Disease Implementation Consortium.

Journal article PLoS One · 2021 INTRODUCTION: Sex-based clinical outcome differences in sickle cell disease (SCD) remain largely unknown despite evidence that female sex is associated with an increased lifespan. To better characterize sex-based differences in SCD, we assessed pain, treat ... Full text Link to item Cite

Vaso-occlusive crises and costs of sickle cell disease in patients with commercial, Medicaid, and Medicare insurance - the perspective of private and public payers.

Journal article J Med Econ · November 2020 AIM: To characterize vaso-occlusive crises (VOCs) and describe healthcare costs among commercially-insured, Medicaid-insured, and Medicare-insured patients with sickle cell disease (SCD). MATERIALS AND METHODS: The IBM Truven Health MarketScan Commercial ( ... Full text Link to item Cite

Crizanlizumab and comparators for adults with sickle cell disease: a systematic review and network meta-analysis.

Journal article BMJ Open · September 17, 2020 OBJECTIVES: Treatment options for preventing vaso-occlusive crises (VOC) among patients with sickle cell disease (SCD) are limited, especially if hydroxyurea treatment has failed or is contraindicated. A systematic literature review (SLR) and network meta- ... Full text Link to item Cite

SickleFit: Adherence, Feasibility and Validation of Fitbit Charge 3 Metrics in Steady-State Sickle Cell Patients (Preprint)

Journal article · September 17, 2020 BACKGROUNDWhile the relationship of pain and sleep in sickle cell disease (SCD) has been described, the data are evolving around this complex relationship. In a prior study, using actig ... Full text Cite

Patient-reported outcomes in sickle cell disease and association with clinical and psychosocial factors: Report from the sickle cell disease implementation consortium.

Journal article Am J Hematol · September 2020 Understanding patient experiences, quality of life, and treatment needs in individuals with sickle cell disease (SCD) is essential in promoting health and well-being. We used measures from the Adult Sickle Cell Quality of Life Measurement Information Syste ... Full text Link to item Cite

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

Journal article JMIR Res Protoc · July 14, 2020 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 w ... Full text Open Access Link to item Cite

Publication of data collection forms from NHLBI funded sickle cell disease implementation consortium (SCDIC) registry.

Journal article Orphanet J Rare Dis · July 7, 2020 BACKGROUND: Sickle cell disease (SCD) is an autosomal recessive blood disorder affecting approximately 100,000 Americans and 3.1 million people globally. The scarcity of relevant knowledge and experience with rare diseases creates a unique need for coopera ... Full text Link to item Cite

Effects of repleting organic phosphates in banked erythrocytes on plasma metabolites and vasoactive mediators after red cell exchange transfusion in sickle cell disease.

Journal article Blood Transfus · May 2020 BACKGROUND: Red blood cell (RBC) exchange (RCE) transfusion therapy is indicated for certain patients with sickle cell disease (SCD). Although beneficial, this therapy is costly and inconvenient to patients, who may require it monthly or more often. Identi ... Full text Link to item Cite

Feasibility of implementing mobile technology-delivered mental health treatment in routine adult sickle cell disease care.

Journal article Translational behavioral medicine · February 2020 Sickle cell disease (SCD) is a severe hemoglobinopathy characterized by acute and chronic pain. Sufferers of the disease, most of whom are underrepresented minorities, are at increased risk for mental health disorders. The purpose of this study is to test ... Full text Cite

Sickle-Cell Disease Co-Management, Health Care Utilization, and Hydroxyurea Use.

Journal article J Am Board Fam Med · 2020 BACKGROUND: Sickle-cell disease (SCD) causes significant morbidity, premature mortality, and high disease burden, resulting in frequent health care use. Comanagement may improve utilization and patient adherence with treatments such as Hydroxyurea. The pur ... Full text Link to item Cite

Mobile Health Technology for Pediatric Symptom Monitoring: A Feasibility Study.

Journal article Nurs Res · 2020 BACKGROUND: Pediatric blood and marrow transplant (PBMT) patients experience significant symptom distress. Mobile health (mHealth) technologies can be leveraged to improve understanding of the patient's symptom experience by providing continuous, real-time ... Full text Link to item Cite

User-Centered App Design for Acutely Ill Children and Adolescents.

Journal article J Pediatr Oncol Nurs · 2020 Background and Objectives: The high level of acceptance and consistent use of smartphones by children and adolescents present new opportunities to monitor and collect health data. For acutely ill children and adolescents, collecting symptom data via smartp ... Full text Link to item Cite

Development of a Severity Classification System for Sickle Cell Disease.

Journal article Clinicoecon Outcomes Res · 2020 PURPOSE: There is no well-accepted classification system of overall sickle cell disease (SCD) severity. We sought to develop a system that could be tested as a clinical outcome predictor. PATIENTS AND METHODS: Using validated methodology (RAND/UCLA modifie ... Full text Link to item Cite

Medical resource use and costs of treating sickle cell-related vaso-occlusive crisis episodes: A retrospective claims study

Journal article Journal of Health Economics and Outcomes Research · January 1, 2020 Background: The study investigated the economic burden of vaso-occlusive crisis (VOC) among sickle cell disease (SCD) patients, through assessment of overall utilization and costs and costs per VOC episode (regarding the number of VOC episodes and health c ... Full text Cite

Use of Mobile Health Apps and Wearable Technology to Assess Changes and Predict Pain During Treatment of Acute Pain in Sickle Cell Disease: Feasibility Study.

Journal article JMIR Mhealth Uhealth · December 2, 2019 BACKGROUND: Sickle cell disease (SCD) is an inherited red blood cell disorder affecting millions worldwide, and it results in many potential medical complications throughout the life course. The hallmark of SCD is pain. Many patients experience daily chron ... Full text Link to item Cite

Treatment patterns and economic burden of sickle-cell disease patients prescribed hydroxyurea: a retrospective claims-based study.

Journal article Health Qual Life Outcomes · October 16, 2019 BACKGROUND: This study aimed to evaluate sickle-cell disease (SCD) treatment patterns and economic burden among patients prescribed hydroxyurea (HU) in the US, through claims data. METHODS: SCD patients with pharmacy claims for HU were selected from the Me ... Full text Link to item Cite

Where Did They Go? Tracking Young Adult Follow-up During the Transition From Pediatric to Adult-Oriented Care.

Journal article Clin Pediatr (Phila) · October 2019 Continuity of care during health care transition is critical. We sought to evaluate electronic medical record clinic attendance data to examine this outcome. We identified 1623 patients (ages 18-27 years) with 1 of 6 childhood-onset chronic conditions and ... Full text Link to item Cite

Metabolic impact of red blood cell exchange with rejuvenated red blood cells in sickle cell patients.

Journal article Transfusion · October 2019 BACKGROUND: Red blood cell exchange (RCE) transfusions are a mainstay in the treatment of sickle cell anemia (SCA), and allow a temporary restoration of physiological parameters with respect to erythrocyte oxygen carrying capacity and systems metabolism. R ... Full text Link to item Cite

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

Journal article · September 24, 2019 BACKGROUNDHydroxyurea prevents disease complications among patients with sickle cell disease (SCD). Although its efficacy has been endorsed by the National Health Lung and Blood Institute ... Full text Cite

Interval decline in hemoglobin A is associated with annual clinical event rate in sickle cell anemia patients receiving maintenance apheresis RBC exchange.

Journal article Transfusion · August 2019 BACKGROUND: Apheresis red blood cell (RBC) exchange (RCE) is a standard intervention for patients with sickle cell anemia (SCA) who have had previous thromboembolic stroke or intractable chronic pain. Replacing sickling cells with those containing hemoglob ... Full text Link to item Cite

Double-Blind, Randomized, Placebo-Controlled Trial Comparing the Effects of Antithrombin Versus Placebo on the Coagulation System in Infants with Low Antithrombin Undergoing Congenital Cardiac Surgery.

Journal article J Cardiothorac Vasc Anesth · February 2019 OBJECTIVES: To determine whether precardiopulmonary bypass (CPB) normalization of antithrombin levels in infants to 100% improves heparin sensitivity and anticoagulation during CPB and has beneficial effects into the postoperative period. DESIGN: Randomize ... Full text Link to item Cite

Transition to adult care in sickle cell disease: A longitudinal study of clinical characteristics and disease severity.

Journal article Pediatr Blood Cancer · January 2019 BACKGROUND: Sickle cell disease (SCD) is a chronic blood disorder in which mortality has increased for adolescents and young adults (AYA). PROCEDURE: A longitudinal analysis of medical records was conducted to describe the clinical course among AYAs (ages ... Full text Link to item Cite

A Protocol to Assess Feasibility, Acceptability, and Usability of Mobile Technology for Symptom Management in Pediatric Transplant Patients.

Journal article Nurs Res · 2019 BACKGROUND: Pediatric blood and marrow transplant (PBMT) patients experience significant symptom distress, and the use of mobile health (mHealth) technologies may enhance symptom management by providing patient-generated health data to foster personalized ... Full text Link to item Cite

Barriers and facilitators to care for individuals with sickle cell disease in central North Carolina: The emergency department providers' perspective.

Journal article PLoS One · 2019 BACKGROUND: Sickle cell disease (SCD) is an inherited blood disorder associated with acute pain crisis and other complications that lead to frequent emergency department (ED) visits. To improve outcomes, the National Heart, Lung and Blood Institute (NHLBI) ... Full text Link to item Cite

Sickle cell disease complications: Prevalence and resource utilization.

Journal article PLoS One · 2019 OBJECTIVES: This study evaluated the prevalence rate of vaso-occlusive crisis (VOC) episodes, rates of uncomplicated and complicated VOC episodes, and the primary reasons for emergency room (ER) visits and inpatient admissions for sickle cell disease (SCD) ... Full text Link to item Cite

Evaluation of Vaso-occlusive Crises in United States Sickle Cell Disease Patients: A Retrospective Claims-based Study

Journal article Journal of Health Economics and Outcomes Research · January 1, 2019 Sickle cell disease (SCD) is a life-threatening vascular disease that burdens affected persons physically. SCD-related vaso-occlusive crises (VOCs) are one of the primary causes of morbidity and mortality. Our objective was to examine the epidemiology of p ... Full text Cite

Effects of red blood cell (RBC) transfusion on sickle cell disease recipient plasma and RBC metabolism.

Journal article Transfusion · December 2018 BACKGROUND: Exchange transfusion is a mainstay in the treatment of sickle cell anemia. Transfusion recipients with sickle cell disease (SCD) can be transfused over 10 units per therapy, an intervention that replaces circulating sickle red blood cells (RBCs ... Full text Link to item Cite

Abstract Animations for the Communication and Assessment of Pain in Adults: Cross-Sectional Feasibility Study.

Journal article J Med Internet Res · August 3, 2018 BACKGROUND: Pain is the most common physical symptom requiring medical care, yet the current methods for assessing pain are sorely inadequate. Pain assessment tools can be either too simplistic or take too long to complete to be useful for point-of-care di ... Full text Link to item Cite

Mobile health intervention for youth with sickle cell disease: Impact on adherence, disease knowledge, and quality of life.

Journal article Pediatr Blood Cancer · August 2018 BACKGROUND: Adherence to illness self-management among youth with sickle cell disease (SCD) positively impacts health outcomes and decreases overall healthcare costs. Despite this, children with SCD face several barriers to adherence, with adherence rates ... Full text Link to item Cite

Patient-Centered eHealth Interventions for Children, Adolescents, and Adults With Sickle Cell Disease: Systematic Review.

Journal article J Med Internet Res · July 19, 2018 BACKGROUND: Sickle cell disease is an inherited blood disorder that affects over 100,000 Americans. Sickle cell disease-related complications lead to significant morbidity and early death. Evidence supporting the feasibility, acceptability, and efficacy of ... Full text Link to item Cite

Improving Pain Management in Patients with Sickle Cell Disease from Physiological Measures Using Machine Learning Techniques.

Journal article Smart Health (Amst) · June 2018 Pain management is a crucial part in Sickle Cell Disease treatment. Accurate pain assessment is the first stage in pain management. However, pain is a subjective response and hard to assess via objective approaches. In this paper, we proposed a system to m ... Full text Link to item Cite

Patient-Centered eHealth Interventions for Children, Adolescents, and Adults With Sickle Cell Disease: Systematic Review (Preprint)

Journal article · May 2, 2018 BACKGROUNDSickle cell disease is an inherited blood disorder that affects over 100,000 Americans. Sickle cell disease–related complications lead to significant morbidity and early death. ... Full text Cite

Immunogenicity and safety of the quadrivalent meningococcal ACWY-tetanus toxoid conjugate vaccine (MenACWY-TT) in splenectomized or hyposplenic children and adolescents: Results of a phase III, open, non-randomized study.

Journal article Vaccine · April 19, 2018 BACKGROUND: Individuals with functional or anatomic asplenia are at high risk for meningococcal disease. We evaluated the immunogenicity and safety of 1 and 2 doses of the quadrivalent meningococcal serogroups A, C, W, Y tetanus toxoid-conjugate vaccine (M ... Full text Link to item Cite

A Cross-sectional Feasibility Study Testing the Use of Abstract Animations for the Communication and Assessment of Pain (Preprint)

Journal article · February 7, 2018 BACKGROUNDPain is the most common medical symptom requiring care, yet the current methods for assessing pain are sorely inadequate. Pain assessment tools either take too long to complete ... Full text Cite

Customization of the TRU-PBMT App (Technology Recordings to better Understand Pediatric Blood and Marrow Transplant).

Journal article J Pediatr Nurs · 2018 PURPOSE: Our investigative team is integrating mobile health technologies into pediatric blood and marrow transplant (PBMT) care. We aim to evaluate whether patient-generated health data can be used to monitor health status and enhance symptom management. ... Full text Link to item Cite

Development of quality indicators for transition from pediatric to adult care in sickle cell disease: A modified Delphi survey of adult providers.

Journal article Pediatr Blood Cancer · June 2017 BACKGROUND: Transition from pediatric to adult care is a vulnerable time for young adults with sickle cell disease (SCD); however, improvements in transition are limited by a lack of quality indicators. The purpose of this study was to establish quality in ... Full text Link to item Cite

Utilizing a Novel Mobile Health "Selfie" Application to Improve Compliance to Iron Chelation in Pediatric Patients Receiving Chronic Transfusions.

Journal article J Pediatr Hematol Oncol · April 2017 Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and di ... Full text Link to item Cite

Attitudes of Primary Care Physicians Toward Sickle Cell Disease Care, Guidelines, and Comanaging Hydroxyurea With a Specialist.

Journal article J Prim Care Community Health · January 2017 BACKGROUND: Sickle cell disease (SCD) is a complex chronic disease requiring multidisciplinary care that involves primary care physicians (PCPs) working with a hematologist or SCD specialists. However, PCPs often lack access to SCD specialists and are unaw ... Full text Link to item Cite

Advances in iron chelation therapy: transitioning to a new oral formulation.

Journal article Drugs Context · 2017 Iron overload is a concern for patients who require repeated red-blood-cell transfusions due to conditions such as sickle cell disease, thalassemia, or myelodysplastic syndromes. The recommended treatment for removing excess iron in these patients is iron ... Full text Link to item Cite

Trends in Sickle Cell Disease-related Priapism in U.S. Children's Hospitals.

Journal article Urology · March 2016 OBJECTIVE: To define rates of priapism diagnosis and inpatient admission among males with sickle cell disease (SCD). PATIENTS AND METHODS: We retrospectively reviewed the Pediatric Health Information System database for males aged <21 years treated 2004-20 ... Full text Link to item Cite

Challenges in Shifting Management Responsibility From Parents to Adolescents With Sickle Cell Disease.

Journal article J Pediatr Nurs · 2016 UNLABELLED: This study explored the challenges faced by adolescents with sickle cell disease (SCD) and their parents and the work they engage in to progressively shift from parent management to independent adolescent self-management. DESIGN AND METHODS: A ... Full text Link to item Cite

Multicenter randomized controlled trial on Duration of Therapy for Thrombosis in Children and Young Adults (the Kids-DOTT trial): pilot/feasibility phase findings.

Journal article J Thromb Haemost · September 2015 BACKGROUND: Randomized controlled trials (RCTs) on pediatric venous thromboembolism (VTE) treatment have been challenged by unsubstantiated design assumptions and/or poor accrual. Pilot/feasibility (P/F) studies are critical to future RCT success. METHODS: ... Full text Link to item Cite

Sickle cell disease is associated with iron mediated hypercoagulability.

Journal article J Thromb Thrombolysis · August 2015 Sickle cell disease (SCD) is associated with a significant hypercoagulable state and several hemostatic anomalies have been identified in this disease state. Of interest, SCD patients can become iron overloaded after transfusion, and iron can enhance fibri ... Full text Link to item Cite

Usability and Feasibility of an mHealth Intervention for Monitoring and Managing Pain Symptoms in Sickle Cell Disease: The Sickle Cell Disease Mobile Application to Record Symptoms via Technology (SMART).

Journal article Hemoglobin · 2015 Patients with sickle cell disease frequently experience severe pain events that lead to unplanned healthcare utilization. Mobile health tools (mHealth) may help prevent these events by providing remote monitoring and self-management support. This article d ... Full text Link to item Cite

Differences in pain management between hematologists and hospitalists caring for patients with sickle cell disease hospitalized for vasoocclusive crisis.

Journal article Clin J Pain · March 2014 OBJECTIVES: Sickle cell disease (SCD) is a chronic disease characterized by multiple vaso-occlusive complications and is increasingly cared for by hospitalists. The purpose of this study is to examine differences in pain management between hematologists an ... Full text Link to item Cite

Patients welcome the Sickle Cell Disease Mobile Application to Record Symptoms via Technology (SMART).

Journal article Hemoglobin · 2014 The widespread use of mobile phones among patients provides a unique opportunity for the development of mobile health intervention designed specifically for sickle cell disease, which will improve self-management as well as health care delivery. Our object ... Full text Link to item Cite

Characteristics of abdominal vein thrombosis in children and adults.

Journal article Thromb Haemost · April 2013 The demographic and clinical characteristics of adults and children with lower extremity deep-vein thrombosis and/or pulmonary embolism (LE DVT/PE) may differ from those with abdominal vein thrombosis (abdominal VT). Abdominal VT can be a presenting sign o ... Full text Open Access Link to item Cite

Severe fetal and neonatal hemolytic anemia due to a 198 kb deletion removing the complete β-globin gene cluster.

Journal article Pediatr Blood Cancer · November 2012 Fetal and neonatal hemolytic anemia can be caused by (γδβ)(0)-thalassemia deletions of the β-globin gene cluster. Many of these deletions have not been well characterized, and diagnostic tests are not readily available, thus hampering carrier detection, fa ... Full text Link to item Cite

Characterization of the hypercoagulable state in patients with sickle cell disease.

Journal article Thromb Res · November 2012 BACKGROUND: The pathophysiology of sickle cell disease (SCD) is complex, with increasing evidence of a pronounced prothrombotic state. OBJECTIVE: We investigated thrombin generation in SCD utilizing calibrated automated thrombography (CAT) and D-dimer, wit ... Full text Link to item Cite

Antithrombin Levels Are Significantly Lower During Pregnancy

Journal article AMERICAN JOURNAL OF HEMATOLOGY · May 1, 2012 Link to item Cite

Complications of implantable venous access devices in patients with sickle cell disease.

Journal article Am J Hematol · February 2012 Implantable venous access devices (VADs) are used in sickle cell disease (SCD) for patients with poor venous access to facilitate chronic blood transfusions and manage acute complications. We attempted to define the frequency of bloodstream infections (BSI ... Full text Open Access Link to item Cite

Presentation and outcomes for children with bone marrow necrosis and acute lymphoblastic leukemia: a literature review.

Journal article J Pediatr Hematol Oncol · October 2011 Bone marrow necrosis is a rare histopathology finding with the majority of cases occurring in the setting of a hematologic malignancy. This article reports a case of diffuse marrow necrosis in a child secondary to acute lymphoblastic leukemia and summarize ... Full text Open Access Link to item Cite