Prevalence of Substance Use Disorders in Sickle Cell Disease Compared to Other Chronic Conditions: a Population-Based Study of Black American Adults.

Background: Sickle cell disease (SCD) is a heritable chronic health condition characterized by pain symptoms throughout the life course that are routinely treated with opioids.

Objective: This study examined differences in substance use disorders in Black American adults with SCD compared to those with other chronic conditions or with no chronic conditions.

Design: Data from a population-representative sample of Black Americans with SCD, other chronic conditions, and no chronic conditions were obtained from the National Survey of American Life (NSAL) database.

American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease.

Background: Prevention and management of end-organ disease represent major challenges facing providers of children and adults with sickle cell disease (SCD). Uncertainty and variability in the screening, diagnosis, and management of cardiopulmonary and renal complications in SCD lead to varying outcomes for affected individuals.

Objective: These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about screening, diagnosis, and management of cardiopulmonary and renal complications of SCD.

Phase 1 study of the E-selectin inhibitor GMI 1070 in patients with sickle cell anemia.

Background: Sickle cell anemia is an inherited disorder of hemoglobin that leads to a variety of acute and chronic complications. Abnormal cellular adhesion, mediated in part by selectins, has been implicated in the pathophysiology of the vaso-occlusion seen in sickle cell anemia, and selectin inhibition was able to restore blood flow in a mouse model of sickle cell disease.

Methods: We performed a Phase 1 study of the selectin inhibitor GMI 1070 in patients with sickle cell anemia.

Emotional reactions to pain predict psychological distress in adult patients with Sickle Cell Disease (SCD).

Differentiating somatic from emotional influences on the experience of chronic pain has been of interest to clinicians and researchers for many years. Although prior research has not well specified these pathways at the anatomical level, some evidence, both theoretical and empirical, suggest that emotional reactions influence the experience of disease and non-disease-related pains. Other studies suggest that treatments directed at negative emotional responses reduce suffering associated with pain.

An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.

Background: In adults with sickle cell disease (SCD), an increased tricuspid regurgitant velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro-brain natriuretic peptide (NT-pro-BNP) level, and pulmonary hypertension (PH) diagnosed by right heart catheterization (RHC) are independent risk factors for mortality.

Methods: A multidisciplinary committee was formed by clinician-investigators experienced in the management of patients with PH and/or SCD. Clinically important questions were posed, related evidence was appraised, and questions were answered with evidence-based recommendations.

Evaluation of a train-the-trainer workshop on sickle cell disease for ED providers.

Objective: (1) Determine the difference in pre-test and post-test knowledge scores for attendees of a train-the-trainer workshop and (2) determine the number of attendees who disseminated the content within 6 months of attending the workshop.

Methods: A 1-day, train-the-trainer workshop focusing on sickle cell disease (SCD) was developed. ED nurses and physicians from the emergency departments with the highest number of patients with SCD were invited to participate at no cost.

The association of optimism and perceived discrimination with health care utilization in adults with sickle cell disease.

Objective: We evaluated the effect of perceived discrimination, optimism, and their interaction on health care utilization among African American adults with sickle cell disease (SCD).

Methods: Measures of optimism and perceived discrimination were obtained in 49 African American SCD patients. Multiple regression analyses controlling for sex and age tested effects of optimism and perceived discrimination on the number of emergency department visits (ED) and number and duration of hospitalizations over the past year.

The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up.

A randomized, controlled clinical trial established the efficacy and safety of short-term use of hydroxyurea in adult sickle cell anemia. To examine the risks and benefits of long-term hydroxyurea usage, patients in this trial were followed for 17.5 years during which they could start or stop hydroxyurea.

Exposure to hydroxyurea and pregnancy outcomes in patients with sickle cell anemia.

The Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH) was a randomized double-blind placebo-controlled trial to test whether hydroxyurea could reduce the rate of painful crises in adults who had at least 3 painful crises per year. Because hydroxyurea is known to be carcinogenic, mutagenic, and teratogenic in animals, a major inclusion criterion in MSH was the use of contraceptives both by females and males in order to avoid exposure of the fetus to hydroxyurea. Despite this precautionary measure, some women became pregnant while taking hydroxyurea or their male partners were on hydroxyurea.

Neuropsychological assessment, neuroimaging, and neuropsychiatric evaluation in pediatric and adult patients with sickle cell disease (SCD).

Traditionally, neuropsychological deficits due to Sickle Cell Disease (SCD) have been understudied in adults. We have begun to suspect, however, that symptomatic and asymptomatic Cerebrovascular Events (CVE) may account for an alarming number of deficits in this population. In the current brief review, we critically evaluated the pediatric and adult literatures on the neurocognitive effects of SCD.

Parental substance abuse, reports of chronic pain and coping in adult patients with sickle cell disease.

There is increasing interest from a social learning perspective in understanding the role of parental factors on adult health behaviors and health outcomes. Our review revealed no studies, to date, that have evaluated the effects of parental substance abuse on reports of chronic pain and coping in adult patients with sickle cell disease (SCD). We explored the effects of parental substance (alcohol or drug) abuse on reports of the sensory, affective and summary indices of pain in 67 adult patients, mean age 38.

Moderate chronic pain, weight and dietary intake in African-American adult patients with sickle cell disease.

In this exploratory study, we evaluated weight status and dietary intake patterns during painful episodes in adult patients with SCD. Specifically, we explored the relation between pain severity and body mass index (BMI), and we tested the hypothesis that dietary intake would be reduced and dietary content altered during periods of increased pain. We conducted an analysis of survey data from 62 patients involved in a longitudinal evaluation of the relationship of medical and psychosocial factors to pain.

Religiosity/spirituality and pain in patients with sickle cell disease.

Religion/spirituality has been identified by individuals with sickle cell disease (SCD) as an important factor in coping with stress and in determining quality of life. Research has demonstrated positive associations between religiosity/spirituality and better physical and mental health outcomes. However, few studies have examined the influence religiosity/spirituality has on the experience of pain in chronically ill patients.

Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.

Context: Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality.

Objective: To determine whether hydroxyurea attenuates mortality in patients with SCA.