Sickle cell disease (SCD) is the most commonly inherited blood disorder in North America, and impacts approximately 100 000 people in the United States (US). Affected individuals generally live on average 30 years less than the general population. These individuals suffer acute, intermittent painful episodes (vaso‐occlusive crisis [VOC]), as well as fatigue, chronic organ damage and chronic pain. Currently, the National Heart Lung and Blood Institute and Centers for Disease Control and Prevention recommend aggressive treatment with opioids during acute VOC and as a therapeutic option for chronic pain. Despite these guideline recommendations for aggressive opioid treatment for acute pain in SCD, the high opioid doses often used for SCD‐related pain may result in a negative perception of affected persons by some healthcare professionals. Additionally, the current national opioid crisis is impacting overall opioid prescribing including for the SCD population. Despite the critical need for and concerns about opioid‐based pain management, there is limited evidence on current opioid treatment patterns among individuals with SCD and any potential link to the U.S. opioid epidemic. This study aims to describe opioid utilization in individuals with SCD within the context of healthcare utilization, and the U.S. opioid epidemic among a published broader U.S. population. Since individuals with SCD are likely to have public health insurance coverage, and literature suggests that utilization (including opioid use) may differ between privately and publicly insured indidviduals, our study examined utilization in both commercially‐insured individuals and those with Medicaid coverage. Abstract source Full text

Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray. The spectrum of clinical manifestations can range from mild respiratory illness to acute respiratory distress syndrome. The presence of severe hypoxemia is a useful predictor of severity and outcome. The etiology of ACS is often multifactorial. One of the proposed mechanisms involves increased adhesion of sickle red cells to pulmonary microvasculature in the presence of hypoxia. Other commonly associated etiologies include infection, pulmonary fat embolism, and infarction. Infection is a common cause in children, whereas adults usually present with pain crises. Several risk factors have been identified in children to be associated with increased incidence of ACS. These include younger age, severe SCD genotypes (SS or Sβ0 thalassemia), lower fetal hemoglobin concentrations, higher steady-state hemoglobin levels, higher steady-state white blood cell counts, history of asthma, and tobacco smoke exposure. Opiate overdose and resulting hypoventilation can also trigger ACS. Prompt diagnosis and management with intravenous fluids, analgesics, aggressive incentive spirometry, supplemental oxygen or respiratory support, antibiotics, and transfusion therapy, are key to the prevention of clinical deterioration. Bronchodilators should be considered if there is history of asthma or in the presence of acute bronchospasm. Treatment with hydroxyurea should be considered for prevention of recurrent episodes. This review evaluates the etiology, pathophysiology, risk factors, clinical presentation of ACS, and preventive and treatment strategies for effective management of ACS. Abstract source Full text

The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV1% predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: (1) FEV1 % predicted declines over time; and (2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ0 thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study. Study-certified pulmonary function technicians performed spirometry and lung volumes. Each assessment was reviewed centrally. Predicted values were determined for TLC, FEV1 , FVC, and FEV1 /FVC ratio. A total of 197 participants, mean age 11.0 years at first testing (range 4-19.3 years), had a minimum of three spirometry measurements, over an average of 4.4 years (range 1.1-6.5 years) from baseline to endpoint. In a multivariable model, FEV1 % predicted declines by 0.3% for every additional year of age (95% CI -0.56 to -0.05, P = .020). Sex, asthma history, hemoglobin, reticulocyte count, white blood cell count, incidence rate of severe acute pain and acute chest syndrome episodes, and hydroxyurea therapy were not associated with a decline in FEV1% predicted. In a large, rigorously evaluated, prospective cohort of an unselected group of children with SCA, FEV1% predicted declines minimally over an average of 4 years, and none of the examined disease features predict the decline. Abstract source Full text

Drug overdose, driven largely by overdose related to the use of opioids, is now the leading cause of unintentional injury death in the United States. The ongoing opioid crisis lies at the intersection of two public health challenges: reducing the burden of suffering from pain and containing the rising toll of the harms that can arise from the use of opioid medications. Chronic pain and opioid use disorder both represent complex human conditions affecting millions of Americans and causing untold disability and loss of function. In the context of the growing opioid problem, the U.S. Food and Drug Administration (FDA) launched an Opioids Action Plan in early 2016. As part of this plan, the FDA asked the National Academies of Sciences, Engineering, and Medicine to convene a committee to update the state of the science on pain research, care, and education and to identify actions the FDA and others can take to respond to the opioid epidemic, with a particular focus on informing FDA's development of a formal method for incorporating individual and societal considerations into its risk-benefit framework for opioid approval and monitoring. Abstract source

Sickle cell trait is a blood disorder that affects the red blood cells. It is not the same as sickle cell disease - in fact, it is not a disease at all. It is a "trait," which means it is inherited. In the case of sickle cell trait (SCT), a person has inherited one abnormal hemoglobin gene from one parent and one normal gene from the other. Hemoglobin is a protein in red blood cells that carries oxygen around the body. In this article, learn how SCT is diagnosed and what it means for the people who have it. What Is Sickle Cell Trait? Sickle cell trait (SCT) is an inherited blood disorder that affects the red blood cells. It affects between 1 and 3 million Americans and 8 to 10 percent of African Americans. People with SCT have inherited one gene for normal hemoglobin and one for sickle hemoglobin.   Abstract source

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During Your Adolescent Years: • When do I start to meet with you on my own for part of the visit to become more independent when it comes to my own health and health care? • What do I need to learn to get ready for adult health care? Do you have a checklist of self-care skills that I need to learn? • Can I work with you to prepare a Medical Summary and Emergency Care Plan? • When I turn 18, what information about privacy and consent do I need to know about? If I need help with making health decisions, where can I get information about this? • At what age do I need to change to a new doctor for adult health care? • Do you have any suggestions of adult doctors to transfer to?   Abstract source