Treatment of Acute Pain in Adults With Sickle Cell Disease in an Infusion Center Versus the Emergency Department : A Multicenter Prospective Cohort Study.

Background: Patients with sickle cell disease (SCD) have vaso-occlusive crises (VOCs). Infusion centers (ICs) are alternatives to emergency department (ED) care and may improve patient outcomes.

Objective: To assess whether care in ICs or EDs leads to better outcomes for the treatment of uncomplicated VOCs.

Mortality and Access to Kidney Transplantation in Patients with Sickle Cell Disease-Associated Kidney Failure.

Background And Objectives: Patients with sickle cell disease-associated kidney failure have high mortality, which might be lowered by kidney transplantation. However, because they show higher post-transplant mortality compared with patients with other kidney failure etiologies, kidney transplantation remains controversial in this population, potentially limiting their chance of receiving transplantation. We aimed to quantify the decrease in mortality associated with transplantation in this population and determine the chance of receiving transplantation with sickle cell disease as the cause of kidney failure as compared with other etiologies of kidney failure.

Comparison of US Federal and Foundation Funding of Research for Sickle Cell Disease and Cystic Fibrosis and Factors Associated With Research Productivity.

Importance: Sickle cell disease (SCD) and cystic fibrosis (CF) are severe autosomal recessive disorders associated with intermittent disease exacerbations that require hospitalizations, progressive chronic organ injury, and substantial premature mortality. Research funding is a limited resource and may contribute to health care disparities, especially for rare diseases that disproportionally affect economically disadvantaged groups.

Objective: To compare disease-specific funding between SCD and CF and the association between funding and research productivity.

Metabolic syndrome among adults living with sickle cell disease.

Metabolic syndrome (MetS) is a key risk factor for cardiovascular disease (CVD) incidence and all-cause mortality. MetS prevalence among adults with sickle cell disease (SCD) is not well known. We report initial findings from a cross-sectional study that examined MetS risk factors within a cohort of adults living with SCD.

Clinical Outcomes Associated With Sickle Cell Trait: A Systematic Review.

Background: Although sickle cell trait (SCT) is largely a benign carrier state, it may increase risk for certain clinical outcomes.

Purpose: To evaluate associations between SCT and clinical outcomes in children and adults.

Data Sources: English-language searches of PubMed, CINAHL, the Cochrane Library, Current Contents Connect, Scopus, and Embase (1 January 1970 to 30 June 2018) and bibliographies of review articles.

Correction to: Do Words Matter? Stigmatizing Language and the Transmission of Bias in the Medical Record.

In the HTML version of this paper, Ms. Goddu was incorrectly listed. The correct citation should be AP Goddu.

Increased acute care utilization in a prospective cohort of adults with sickle cell disease.

The ESCAPED (Examining Sickle Cell Acute Pain in the Emergency vs Day Hospital) trial is an ongoing prospective study comparing outcomes of people with sickle cell disease (SCD) seeking care for acute pain management in either an emergency department or specialty infusion clinic. The objective of this paper is to describe the baseline characteristics and health care utilization of patients in the trial. This is a multicenter study across 4 US cities that enrolled all adults with SCD living within 60 miles (96.

Do Words Matter? Stigmatizing Language and the Transmission of Bias in the Medical Record.

Background: Clinician bias contributes to healthcare disparities, and the language used to describe a patient may reflect that bias. Although medical records are an integral method of communicating about patients, no studies have evaluated patient records as a means of transmitting bias from one clinician to another.

Objective: To assess whether stigmatizing language written in a patient medical record is associated with a subsequent physician-in-training's attitudes towards the patient and clinical decision-making.

Examination of the Patient and Hospitalization Characteristics of 30-Day SCD Readmissions.

Objectives: Sickle cell disease (SCD) is associated with a high level of emergency department and hospital utilization, as well as a high rate of hospital readmissions. At Johns Hopkins Hospital, as at other institutions, SCD accounts for a large proportion of readmissions. Our study examined patient and hospitalization factors involved in readmissions at Johns Hopkins Hospital.

Effect of Free Dental Services on Individuals with Sickle Cell Disease.

Objectives: Poor oral health can have a negative impact on overall health. This is especially concerning for individuals with sickle cell disease (SCD), an inherited blood disorder that affects hemoglobin and can lead to an increased risk of infection and hyperalgesia. Because the majority of individuals with SCD have Medicaid insurance and no dental coverage, we provided free basic dental care to individuals with SCD to determine whether it decreased overall healthcare utilization.

Utility of the Montreal Cognitive Assessment as a Screening Test for Neurocognitive Dysfunction in Adults with Sickle Cell Disease.

Objectives: Neurocognitive dysfunction is an important complication of sickle cell disease (SCD), but little is published on the utility of screening tests for cognitive impairment in people with the disease. The purpose of this study was to evaluate the Montreal Cognitive Assessment (MoCA) as a screening tool and identify predictors of MoCA performance in adults with sickle cell disease.

Methods: We conducted a retrospective, cross-sectional study of the first 100 adult patients with SCD who completed the MoCA as part of routine clinical care at the Johns Hopkins Sickle Cell Center for Adults.

Symptomatic Avascular Necrosis: An Understudied Risk Factor for Acute Care Utilization by Patients with SCD.

Objectives: Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid avascular necrosis (AVN) contributes to high healthcare utilization. We sought to clarify whether AVN independently predicts acute care utilization in adults with SCD and to identify characteristics of those with AVN that predict higher utilization.

Chronic Opioid Therapy and Central Sensitization in Sickle Cell Disease.

Chronic opioid therapy (COT) for chronic non-cancer pain is frequently debated, and its effectiveness is unproven in sickle cell disease (SCD). The authors conducted a descriptive study among 83 adult SCD patients and compared the severity of disease and pain symptoms among those who were prescribed COT (n=29) with those who were not using COT. All patients completed baseline laboratory pain assessment and questionnaires between January 2010 and June 2014.

The Association of Clinician Characteristics with their Attitudes Toward Patients with Sickle Cell Disease: Secondary Analyses of a Randomized Controlled Trial.

Background: A high level of evidence exists to suggest that negative attitudes held by clinicians toward persons with sickle cell disease serve as important barriers to the delivery of high quality care to this patient population. Little is known, though, about the characteristics of clinicians that may be predictive of these negative attitudes.

Methods: During spring and summer 2009, we conducted a randomized controlled trial to test an intervention to improve clinician attitudes toward persons with sickle cell disease.

Psychometric Validation of the Insomnia Severity Index in Adults with Sickle Cell Disease.

Background: The Insomnia Severity Index (ISI) is an instrument to evaluate insomnia symptoms. The psychometric properties have not been established in adults (18 years of age or older) with sickle cell disease (SCD).

Objective: Evaluate the reliability and validity of the ISI among adults with SCD.

Racial differences in acute kidney injury of hospitalized adults with diabetes.

Objective: To determine whether there is a racial difference in the risk of acute kidney injury between hospitalized black and white adults with diabetes mellitus in the United States

Research Design And Methods: We analyzed cross-sectional data from the 2000-2010 National Hospital Discharge Survey (NHDS) to compare the odds of AKI among hospitalized black and white adults with diabetes. After excluding records in which race status was missing, race was other than white or black, discharge status was not provided, or end-stage renal disease was a diagnosis, we identified 276,138 eligible records for analysis. Multivariable logistic regression was used to analyze the association between race, AKI, and in-hospital mortality.

An Evaluation of Central Sensitization in Patients With Sickle Cell Disease.

Unlabelled: Central sensitization (CS), nociceptive hyperexcitability known to amplify and maintain clinical pain, has been identified as a leading culprit responsible for maintaining pain in several chronic pain conditions. Recent evidence suggests that it may explain differences in the symptom experience of individuals with sickle cell disease (SCD). Quantitative sensory testing (QST) can be used to examine CS and identify individuals who may have a heightened CS profile.

Multiple Levels of Suffering: Discrimination in Health-Care Settings is Associated With Enhanced Laboratory Pain Sensitivity in Sickle Cell Disease.

Objective: People living with sickle cell disease (SCD) experience severe episodic and chronic pain and frequently report poor interpersonal treatment within health-care settings. In this particularly relevant context, we examined the relationship between perceived discrimination and both clinical and laboratory pain.

Methods: Seventy-one individuals with SCD provided self-reports of experiences with discrimination in health-care settings and clinical pain severity, and completed a psychophysical pain testing battery in the laboratory.

Quantitative sensory testing and pain-evoked cytokine reactivity: comparison of patients with sickle cell disease to healthy matched controls.

Sickle cell disease (SCD) is an inherited blood disorder associated with significant morbidity, which includes severe episodic pain, and, often, chronic pain. Compared to healthy individuals, patients with SCD report enhanced sensitivity to thermal detection and pain thresholds and have altered inflammatory profiles, yet no studies to date have examined biomarker reactivity after laboratory-induced pain. We sought to examine this relationship in patients with SCD compared to healthy control participants.

Acute Pain and Depressive Symptoms: Independent Predictors of Insomnia Symptoms among Adults with Sickle Cell Disease.

No studies to date have systematically investigated insomnia symptoms among adults with sickle cell disease (SCD). The purpose of this study was to (1) describe the prevalence of insomnia symptoms and (2) identify biopsychosocial predictors in community-dwelling adults with SCD. Cross-sectional analysis of baseline data from 263 African American adults with SCD (aged 18 years or older).

The five key things you need to know to manage adult patients with sickle cell disease.

The lack of a strong evidence base to guide the management of adults with sickle cell disease (SCD) makes it difficult for patients to receive high quality care outside of specialty centers. As there is a dearth of providers with sickle cell expertise, the purpose of this article is to identify some of the key things every provider who manages the care of adults with SCD should know. Managing adults with SCD requires excellent clinical skills, as it can affect every organ and cause life-threatening complications but it also requires a willingness to manage patients who often have psychosocial issues that are complex and impact care and care delivery in very significant ways.

Sickle cell trait diagnosis: clinical and social implications.

The sickle hemoglobin (HbS) point mutation has independently undergone evolutionary selection at least five times in the world because of its overwhelming malarial protective effects in the heterozygous state. In 1949, homozygous Hb S or sickle cell disease (SCD) became the first inherited condition identified at the molecular level; however, since then, both SCD and heterozygous Hb S, sickle cell trait (SCT), have endured a long and complicated history. Hasty adoption of early mass screening programs for SCD, recent implementation of targeted screening mandates for SCT in athletics, and concerns about stigmatization have evoked considerable controversy regarding research and policy decisions for SCT.

Improving Emergency Providers' Attitudes Toward Sickle Cell Patients in Pain.

Background: Provider biases and negative attitudes are recognized barriers to optimal pain management in sickle cell disease, particularly in the emergency department (ED).

Measures: This prospective cohort measures preintervention and postintervention providers' attitudes toward patients with sickle pain crises using a validated survey instrument.

Intervention: ED providers viewed an eight-minute online video that illustrated challenges in sickle cell pain management, perspectives of patients and providers, as well as misconceptions and stereotypes of which to be wary.

Primary Care Providers' Comfort Levels in Caring for Patients with Sickle Cell Disease.

Objective: The purpose of this study was to determine the comfort levels of primary care providers in caring for individuals with sickle cell disease (SCD) and determine factors that improved or lessened provider comfort.

Methods: We surveyed providers at the annual Johns Hopkins Community Physicians retreat. The survey consisted of 19 questions and measured comfort levels in four domains: providing ambulatory care to individuals with SCD, managing SCD comorbidities, managing SCD-specific issues, and managing chronic pain.

Improving Clinician Attitudes of Respect and Trust for Persons With Sickle Cell Disease.

Objective: To test the effect of 1 high-intensity, and 1 reduced-intensity, educational intervention designed to improve health care provider attitudes toward youth with sickle cell disease (SCD).

Methods: We exposed a regional sample of pediatric health care providers to a 2.5-day high-intensity educational and experiential intervention using videos about the SCD patient experience.