Trusted Resources: Evidence & Education

Scientific literature and patient education texts

259 resources available:

Sickle Cell Disease: Facts and Stats

Sickle Cell Disease Association of America, Inc. Year: 2021
Sickle cell disease (SCD) is an inherited blood disorder in which red blood cells may become sickle-shaped and hardened. For a baby to be born with sickle cell disease, both parents must carry a sickle cell trait or genes for hemoglobin like HbC, HbE, or beta-thalassemia. Sickle cell disease is not contagious, and there is no universal cure. The SCD Facts:...

Adding Hydroxyurea to Your Treatment Plan

CVS Specialty, Inc. Year: 2022
Living with sickle cell disease (SCD) can be hard. Complications including pain, anemia, acute chest syndrome, and silent organ damage can occur anywhere in your body, at any time, and without warning. One of the most common complications is sudden and intense pain. Pain in SCD is usually caused by the sickle-shaped red blood cells blocking blood flow through the tiny blood vessels throughout your body. The abnormal shape is due to a d...

Sickle Cell Trait Toolkit

Centers for Disease Control and Prevention Year: 2020
CDC, together with the American Society of Hematology (ASH) and the Sickle Cell Disease Association of America (SCDAA), created the Sickle Cell Trait Toolkit. The toolkit is a collection of resources to increase understanding of sickle cell traits.   • Blood in Your Urine? Don’t Delay, See Your Healthcare Provider Today immediately<...
JAN’s Accommodation and Compliance Series is designed to help employers determine effective accommodations and comply with Title I of the Americans with Disabilities Act (ADA). Each publication in the series addresses a specific medical condition and provides information about the condition, ADA information, accommodation ideas, and resources for additional information.  The Accommodation and Compliance Series is a starting point in t...

Sickle Cell School Outreach

Children’s Hospital of Philadelphia Year: N/A
The Sickle Cell Center at Children’s Hospital of Philadelphia has developed comprehensive information on sickle cell disease and how it can affect the school life of children. The information is available to teachers, school nurses, athletic departments, camps, and before- and aftercare providers. Typically, families requ...

Genes for Teens with Sickle Cell Disease

St. Jude Children’s Research Hospital Year: N/A
What are genes? • Genes determine specific traits that are passed down to you, or inherited, from your parents. These traits are things like eye color, hair color, skin tone, physical features, and the type of hemoglobin in your blood. • Genes are pieces of DNA that help make you who y...
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487 resources available:
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 hemolytic anemia and complications often requiring hospitalization and transfusions. In 2019, voxelotor, which inhibits HbS polymerization, was approved for SCD treatment. Sickle cell disease (SCD) i...
Sickle cell disease (SCD) is associated with increased risk of poor health outcomes from respiratory infections, including COVID-19 illness. We used US death data to investigate changes in SCD-related mortality before and during the COVID-19 pandemic. We estimated annual age- and quarter-adjusted SCD-related mortality rates for 2014-2020. We estimated the number of excess deaths in 2020 compared with 2019 using the standardized mortality ratio...
Background: Sickle cell disease is characterized by the painful recurrence of vaso-occlusive events. Gene therapy with the use of LentiGlobin for sickle cell disease (bb1111; lovotibeglogene autotemcel) consists of autologous transplantation of hematopoietic stem and progenitor cells transduced with the BB305 lentiviral vector encoding a modified β-globin gene, which produces an antisickling hemoglobin, HbA...
Young individuals with sickle cell anaemia (SCA) experience sleep disturbances and often experience daytime tiredness, which in turn may impact on their daytime functioning and academic attainment, but there are few longitudinal data. Methods: Data on sleep habits and behaviour were taken on the same day as an in-hospital polysomnography. This study assesses the developmental sleep profiles of children and young adults aged 4–23 years old wi...

Regaining Control: Anxiety in Sickle Cell Disease

Journal of Child and Adolescent Psychopharmacology Year: 2020
B. was a 15-year-old adolescent girl living with her parents and attending 10th grade referred for outpatient psychiatric services after superficially cutting herself with a razor blade. Medical history was notable for sickle cell disease (SCD) and multiple related complications, but no previous psychiatric evaluation or treatment. Her chief complaint on presentations was “I want to be in control of my emotions.” History of P...
Introduction: The main therapeutic intervention for sickle cell disease (SCD) is hydroxyurea (HU). The effect of HU is largely through dose-dependent induction of fetal hemoglobin (HbF). Poor HU adherence is common among adolescents. Methods: Our 6-month, two-site pilot intervention trial, “HABIT,” was led by culturally aligned community health workers (CHWs). CHWs performed support pri...
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11 resources available:

A Travel Guide for Adults With Sickle Cell Disease

South Thales Sickle Cell & Thalassaemia Network Year: N/A
People with sickle cell disease generally adapt well to air travel, but it can create difficulties for some. For this reason, it is important to plan ahead and know about potential problems so you can take preventative measures or deal with them should they occur. Choose a travel agent that is registered with ABTA or ATOL. You will need to inform the airline, travel agent and insurance company that you have sickle cell disease. If you...
The American Society of Hematology (ASH) believes it is essential to provide updated treatment guidelines that reflect the newest evidence about the disease, ensuring the medical community can better treat SCD and people living with SCD can make the best decisions for their care. In partnership with the Evidence-Based Practice Research Program at Mayo Clinic, the 2019-2021 ASH Clinical Practice Guidelines on Sickle Cell Disease (SCD) w...
Sickle cell disease (SCD) is an inherited blood disorder that affects the red blood cells and occurs when a person has inherited a sickle cell gene from each parent. The sickle-shaped red blood cells break apart easily, clump together, and stick to the walls of blood vessels, blocking the flow of blood and causing a range of complications, including severe pain, acute chest syndrome (a condition that affects the lungs), stroke, organ damage, a...
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 int...

Sickle cell pain in the emergency department

National Institute for Children’s Health Quality Year: 2015
Sickle cell disease is characterized by unpredictable episodes of extreme pain, known as acute vaso-occlusive episodes (VOE) or pain episodes, caused when sickled red blood cells are unable to move freely through blood vessels. In addition to pain, these changes at the cellular level can also lead to organ damage, stroke and even death. Pain episodes are the most common reason for emergency department (ED) visits and hospitalizations for patients...
Sickle cell disease (SCD) is a generic term for an inherited group of disorders that includes homozygous sickle cell anaemia (SS), sickle cell/haemoglobin C (SC) sickle cell/β-thalassemia (S/β thal) and other compound heterozygous conditions. SCD is characterised by the presence of the mutated β‐globin gene, HBBs (also termed βs‐globin). On de‐oxygenation, this forms a polymeric structure r...
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158 resources available:
This paper reports on the findings of a study designed to establish website‐based self‐management recommendations for sickle cell disease. Google and Yahoo search engines were used to search the World‐Wide‐Web. Purposive sampling was utilized to select 28 websites that met the inclusion criteria. Data were manually collected from health education materials and subjected to qualitative content analysis. Self‐management was con...

Gabapentin for Pain in Sickle Cell Disease: Results of a Randomized Phase ll Clinical Trial

American Society of Pediatric Hematology/Oncology Year: N/A
Background: Pain in sickle cell disease (SCD) can have a significant neuropathic component. Therapies targeting neuropathic pain have not been extensively studied in this setting. We report results of a randomized controlled trial evaluating utility of gabapentin for acute vaso-occlusive crisis (VOC) pain. Objectives: The primary objective was to evaluate efficacy of gabapentin when added...
Background: Vasoocclusive pain leading to frequent emergency department visits and hospital admissions are the hallmark of sickle cell disease (SCD). Adolescents with SCD are known to have higher rates of emergency department (ED) visits and transition to adult care are associated with significant challenges. Adults with SCD have reported higher rates of stigmatization and perception of neglect when receiving care....
Background: Sickle cell disease (SCD) is an inherited disorder caused by a single amino acid substitution producing sickle hemoglobin (HbS). Deoxygenation of HbS causes polymer formation and red blood cell sickling, which lead to anemia, hemolysis, and vaso-occlusion. These clinical features contribute to the chronic and acute manifestations of SCD. Voxelotor is a once-daily oral therapy designed to modify HbS, interfer...
Background: Vaso-occlusive pain crises account for the majority of hospitalizations in patients with sickle cell disease (SCD), and opioids are the mainstay of chronic pain control in SCD. The study sought to: i) examine temporal trends in hospitalization rates and in-hospital mortality rates among adults with SCD ii) compare in-hospital mortality rates for SCD with the rates of opioid prescription...
Sickle cell disease (SCD) is a genetic disorder in which deoxygenation results in polymerization of mutated hemoglobin S (HbS) and triggers the downstream effects of red blood cell (RBC) deformation (sickling), hemolytic anemia, vaso-occlusion, inflammation, predisposition to infection, and chronic organ damage. Two distinct pathophysiologic mechanisms of SCD—severe anemia and vasculopathy—overlap to cause severe morbidity. Chronic anemia...
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47 resources available:

Sickle Cell Disease Implementation Guide

Society of Hospital Medicine Year: N/A
The chronic and potentially debilitating nature of SCD can pose significant challenges for the inpatient management of this patient population. A multidisciplinary hospital team comprised of hospitalists, hematologists, case management, social workers, nursing, and pharmacy must work together collaboratively to improve outcomes for these patients. Patients with SCD face physical, psychosocial, and socioeconomic challenges. Chronic pain with ac...
Several different types of resources are included.  These resources are designed to be beneficial for healthcare professionals (e.g. physicians, nurses, nurse practitioners, physician assistants) who are not sickle cell experts and practice in a variety of settings (e.g. hospitals, primary care clinics, and emergency departments).  • “SCD Conferences” include select presentations from a two day, yearly SCD conference held at Du...
Rationale: The rationale for this article is that one of the most important ways to measure the quality of healthcare is to ask the individuals who actually receive the care. It is beneficial to have a survey that can be easily understood, tells about what people experience and can be used in different places to allow comparisons. In some cases, standard surveys like the Consumer Assessments of Health Care Providers and Systems (CAHPS) and the...

Medicaid and CHIP Sickle Cell Disease Report, T-MSIS Analytic Files (TAF) 2017

Centers for Medicare and Medicaid Services Year: 2021
Sickle cell disease (SCD), the most prevalent lifelong inherited blood disorder in the United States, causes the body to produce red blood cells shaped like crescents or sickles rather than discs. These sickle-shaped blood cells tend to stick to vessel walls leading to blockage and impeding blood flow. When this occurs, oxygen is not delivered to body tissues, which ultimately leads to severe acute and chronic pain episodes known as crises....

Management of the Sickle Cell Spectrum in Basketball Players

Basketball Sports Medicine and Science Year: 2020
Sickle cell spectrum includes sickle cell trait (SCT) and sickle cell disease (SCD) and manifestations range from relatively benign to life threatening. SCT is common affecting almost 3,000,000 Americans and SCD affects approximately 100,000. There are important considerations for sports, exercise, and conditioning in individuals on the sickle cell spectrum. This chapter explores the epidemiology and pathogenesis of SCT and SCD and reviews the...

Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action

National Academies of Sciences, Engineering, and Medicine Year: 2020
This consensus study report was commissioned by the Office of Minority Health at the Office of the Assistant Secretary for Health at the U.S. Department of Health and Human Services to provide a comprehensive approach to the management and potential interventions for sickle cell disease (SCD), a genetic condition affecting approximately 100,000 people in the United States and millions worldwide. The report sets forth a substantial agenda begin...
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