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5 Tips to help prevent infections

Centre for Disease Control and Prevention

Common illnesses, like the flu, can quickly become dangerous for a person with sickle cell disease. The best defense is to take simple steps to help prevent infections. Hand Washing: Washing your hands is one of the best ways to help prevent getting an infection. People with sickle cell disease, their family, and other caretakers should wash their hands with soap and clean water many times each day. Food Safety: Bacteria, called s...

504 plan for education because of sickle cell disease

St. Jude Children's Research Hospital

Your child qualifies for an education plan called a Section 504 plan because of sickle cell disease. What is a Section 504 plan? Section 504 is part of a federal law called the Rehabilitation Act of 1973. It allows students with medical concerns to get “accommodations” to help them learn better. Accommodations are changes made in the classroom to raise the chances that your child will do well in school. Possibl...

7 Foods SC Patients Need

Sickle Cell Warriors

There are many different foods that help to improve circulation and red blood cell production. But for those of us that aren’t so adventurous here are a few foods that you probably use everyday, and have so many positive benefits for sickle cell patients. Try to incorporate at least 2 of these in every meal you eat, and seriously avoid fatty foods…I cannot stress that enough! 1. Green, leafy vegetables like Kale, Spinach, Bok Choy, Turnip Gr...

A Parents’ Handbook for Sickle Cell Disease

State of California Department of Health Services, Genetic Disease Branch

This is a parents handbook for sickle cell disease from The Virginia Sickle Cell Awareness Program. It contains: -Parent Handbook Part I -Parent Handbook Part II Part I is relevant for parents with kids from birth to six years of age, and Part II is relevant for parents with kids of age six to eighteen years. This handbook was developed in response to requests from parents of children with sickle disease for more detailed information...

Alcohol, Tobacco, Street Drugs and Sickle Cell Disease “A Harmful Combination”

St. Jude Children’s Research Hospital

Cocaine: is a potent stimulant. It can cause your heart to race, and it can make you feel nervous and agitated. It can increase heart rate, breathing rate, and blood pressure. Street names include coke, snow, nose candy, flake blow, big C, lady, and snow birds.Cocaine increases the risk of major organ damage. It can cause stroke and even death in people with sickle cell disease. Crack: is a solid (rock) form of cocaine that is smoked, which is...

Ask Dr. Kevin: Debunking the Myths Around Sickle Cell Disease

Pfizer Rare Disease in collaboration with the National Newspaper Publishers Association

This article is the second installment in the “Ask Dr. Kevin” series brought to you by Pfizer Rare Disease in collaboration with the National Newspaper Publishers Association (NNPA) to increase understanding of sickle cell disease. In the United States, sickle cell disease (SCD) is considered rare because it affects only about 100,000 people nationwide. As such, not many people in the U.S. are aware of what SCD is, or if they are aware, th...

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95% of women in England not screened for sickle cell anaemia or thalassaemia by 10 week target

British Medical Journal

Most women fail to receive antenatal screening for sickle cell anaemia or thalassaemia within the recommended time of 10 weeks, a study of general practice in England has found (British Journal of General Practice 2008;58:154-9). This indicates that screening may often occur too late to allow couples a choice regarding termination of affected fetuses. The study assessed antenatal screening for the two inherited disorders in all pregnancies rep...

A family-based randomized controlled trial of pain intervention for adolescents with sickle cell disease

Journal of Pediatric Hematology/Oncology

The study had 2 aims—to determine the efficacy of a family-based cognitive-behavioral pain management intervention for adolescents with sickle cell disease (SCD) in (1) reducing pain and improving health-related variables and (2) improving psychosocial outcomes. Each adolescent and a family support person were randomly assigned to receive a brief pain intervention (PAIN) (n=27) or a disease education attention control intervention (DISEASE ED)...

A hemodynamic study of pulmonary hypertension in sickle cell disease

The New England Journal of Medicine

BACKGROUND: The prevalence and characteristics of pulmonary hypertension in adults with sickle cell disease have not been clearly established. METHODS: In this prospective study, we evaluated 398 outpatients with sickle cell disease (mean age, 34 years) at referral centers in France. All patients underwent Doppler echocardiography, with measurement of tricuspid-valve regurgitant jet velocity. Right heart catheterization was performed in 96 patien...

A multicenter randomized controlled trial of intravenous magnesium for sickle cell pain crisis in children


Magnesium, a vasodilator, anti-inflammatory, and pain reliever, could alter the pathophysiology of sickle cell pain crises. We hypothesized that intravenous magnesium would shorten length of stay, decrease opioid use, and improve health-related quality of life (HRQL) for pediatric patients hospitalized with sickle cell pain crises. The Magnesium for Children in Crisis (MAGiC) study was a randomized, double-blind, placebo-controlled trial of intra...

A Multidisciplinary Approach to Impact Acute Care Utilization in Sickle Cell Disease

American Journal of Medical Quality

Sickle cell disease (SCD), an inherited red blood cell disorder, is characterized by anemia, end-organ damage, unpredictable episodes of pain, and early mortality. Emergency department (ED) visits and hospitalizations are frequent, leading to increased burden on patients and increased health care costs. This study assessed the effects of a multidisciplinary care team intervention on acute care utilization among adults with SCD. The multidisciplin...

A Multinational Trial of Prasugrel for Sickle Cell Vaso-Occlusive Events

The New England Journal of Medicine

BACKGROUND: Sickle cell anemia is an inherited blood disorder that is characterized by painful vaso-occlusive crises, for which there are few treatment options. Platelets mediate intercellular adhesion and thrombosis during vaso-occlusion in sickle cell anemia, which suggests a role for antiplatelet agents in modifying disease events. METHODS: Children and adolescents 2 through 17 years of age with sickle cell anemia were randomly assigned to rec...

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ACSM and CHAMP summit on sickle cell trait: mitigating risks for warfighters and athletes

Medicine & Science in Sports & Exercise

INTRODUCTION: An estimated 300 million people worldwide have sickle cell trait (SCT). Although largely benign, SCT has been associated with exertional rhabdomyolysis and exercise-related sudden death in warfighters/athletes (WA). The National Collegiate Athletic Association’s policy to confirm a student athlete’s SCT status during their preparticipation medical examination prompted reaction from some organizations regarding the rationale and eth...

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

Agency for Healthcare Research & Quality

Definitions for the levels of evidence (high, moderate, low, very low) and the strength of the recommendations (strong, weak) are provided at the end of the “Major Recommendations” field. Estimating Mortality Risk in Sickle Cell Disease (SCD) Conclusions from the Committee: Risk stratification guides decision making. Mortality risk can be determined by noninvasive measurement of the tricuspid regurgitant velocity (TRV) via Doppler echocardiogr...

APHON/ASPHO Policy Statement for the Transition of Patients With Sickle Cell Disease From Pediatric to Adult Health Care

Journal of Pediatric Oncology Nursing

With advances in medical care, the majority of children with sickle cell disease are surviving to adulthood. Patients, families, and providers now face the need for this growing population to move from pediatric- to adult-focused care. In order to facilitate a successful transfer to adult health care, and prepare young adults for greater independence, it is recommended that all pediatric patients with sickle cell disease receive transition prepar...

Blood transfusion in the management of sickle cell disease. In: Evidence-based management of sickle cell disease

Agency for Healthcare Research & Quality

Note from the National Guideline Clearinghouse: This guideline references a drug(s) for which important revised regulatory and/or warning information has been released. December 14, 2016 – General anesthetic and sedation drugs External Web Site Policy: The U.S. Food and Drug Administration (FDA) is warning that repeated or lengthy use of general anesthetic and sedation drugs during surgeries or procedures in children younger than 3 years or in...

Guideline on the management of acute chest syndrome in sickle cell disease

British Journal of Haematology

Sickle cell disease (SCD) affects 12 000–15 000 individuals in the UK. Whilst homozygous SCD (sickle cell anaemia — HbSS) is the most common and severe genotype, and is where most of the evidence exists, this guidance should be used for all genotypes of SCD. Acute chest syndrome (ACS) is defined as an acute illness characterized by fever and/or respiratory symptoms, accompanied by a new pulmonary infiltrate on chest X-ray (Charache et al, 19...

Guidelines for the management of acute pain in sickle-cell disease

American Pain Society

This guideline is the first comprehensive evidence-based guideline to address treatment of the pain of sickle-cell disease. Its contents are: Overview of Sickle-Cell Disease and Related Pain Types and Characteristics of Pain Associated with Sickle-Cell Disease Pain Assessment Treatment of Pain in Sickle-Cell Disease Pharmacological Management of Sickle Pain Acetaminophen and NSAIDs...

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A Comparison of an Individualized and Weight-Based Opioid Protocols for the Treatment of Vaso-Occlusive Episodes in the Emergency Department

American Society of Hematology

Introduction: Vaso-occlusive episodes are the most common complication experienced by individuals with sickle cell disease (SCD). Treatment in an emergency department (ED) is often required and significant variability in care exists. In 2014, NHLBI published evidence based recommendations suggesting treatment with either an individualized opioid dosing or standard weight-based protocol; however the supporting evidence grade was Consensus – Pane...

A Developmental Examination of Pain Among Youth with Sickle Cell Disease

American Society of Hematology

Sickle cell disease (SCD) related pain is a difficult and challenging clinical problem that has significant negative effects on youth’s health-related quality of life (Dampier et al., 2010; Walco & Dampier, 1990). Proper assessment and management of pain in the pediatric population is essential to both improving wellbeing of youth with SCD, and minimizing the development of chronic pain as patients transition into adulthood. Much of the...

A Multi-Center Randomized Controlled Trial of Intravenous Magnesium for Sickle Cell Pain Crisis in Children


Introduction: There are approximately 18,000 hospitalizations and 75,000 hospitalization days annually in the United States for children experiencing sickle cell vasoocclusive crises. Despite advances in the management of other comorbidities of sickle cell disease, little has changed in the management of sickle cell pain crises. Magnesium, a known vasodilator, anti-inflammatory and pain reliever, has the potential to alter the pathophysiology of...

A Multicenter Phase II Trial of Unrelated Donor Reduced Intensity Bone Marrow Transplantation for Children with Severe Sickle Cell Disease (SCURT): Results of the Blood and Marrow Transplant Clinical Trials Network (BMT CTN 0601) Study

American Society of Hematology

Background: Children with severe sickle cell disease (SCD) experience organ damage, poor quality of life, and a risk of premature mortality. Allogeneic bone marrow transplantation (BMT) halts disease progression when stable donor erythropoiesis is established. Myeloablative conditioning (MAC) followed by HLA matched sibling donor BMT is effective, but most patients with SCD lack a matched sibling donor and long term side effects...

A Phase 3 Study of L-Glutamine Therapy for Sickle Cell Anemia and Sickle ß0-Thalassemia


Background:There remains an unmet medical need for new treatments for patients with sickle cell disease (SCD). Increased oxidant stress plays a major part in the pathophysiology of sickle cell disease. It can lead to disturbance of cell membranes, exposure of adhesion molecules and damage to the contents of the sickle red blood cells (sRBC). Previous work from our laboratory has demonstrated enhancement of nicotinamide adenine dinucleotide (NAD)...

A single center experience of GBT440 treatment of severe anemia in sickle cell disease

American Society of Hematology

Background: GBT440 is an oral, once-daily therapy that modulates hemoglobin’s affinity for oxygen, thereby inhibiting HbS polymerization. Clinical trials assessing efficacy and safety in SCD are underway including a randomized Phase 3 study. Patients with SCD and severe anemia, i.e. Hb < 6.5 g/dL, are excluded from the trials of GBT440 but represent a major unmet need. To further describe the emerging clinical profile of...

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Acute Vaso-Occlusive Episode in Sickle Cell Anemia

Family Practice Notebook

Acute Vaso-Occlusive Episode in Sickle Cell Anemia: II. Epidemiology Affects almost all Sickle Cell Anemia patients May occur as early as age 6 months III. Pathophysiology Deoxygenated Hemoglobin polymerizes in triple helix chains Results in Red Blood Cells assuming sickled shape Results in Occlusion of small vessels and capillaries IV. Precipitating Factors A. Preceded by infection 25% of the time 1. Human Parvovirus B19 is a common viral caus...

Athlete and Coach Knowledge, Attitudes, and Perceptions of Sickle Cell Trait and National Collegiate Athletic Association Mandated Testing: Recommendations for Intervention

Georgia Southern University

Author’s abstract: Individuals with Sickle Cell Trait have died suddenly after extreme exertion during military training, athletic practice or games (Kerle & Nishimura, 1996; Harrelson, Fincher & Robinson, 1995; Howe & Bowden, 2007). One of those deaths, Dale Lloyd, a football player at Rice University, prompted a change in the National Collegiate Athletic Association’s (NCAA) Sports Medicine Handbook Guideline 3c: The Student Athlete...

Biochemical and Clinical Aspects of Hemoglobin Abnormalities


Biochemical and Clinical Aspects of Hemoglobin Abnormalities contains the proceedings of a symposium held on the Pingree Park campus of Colorado State University on October 2-7, 1977. Contributors discuss the biochemical and clinical aspects of hemoglobin abnormalities and cover topics ranging from amino acid substitutions to sickle cell disease, glycosylated hemoglobins, cystamine inhibition of sickling, and gelation of sickle cell hemoglobin. T...

Blood Transfusion for Preventing Primary and Secondary Stroke in People with Sickle Cell Disease

Cochrane Library

Background: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting th...

Cerebrovascular Accident in Sickle Cell Anemia

Family Practice Notebook

Cerebrovascular Accident in Sickle Cell Anemia: II. Epidemiology: Prevalence Children: 5% Highest risk at ages 2 to 5 years old with Sickle Cell Anemia A. Silent cerebral infarct occurs in up to 25% of children by age 6 years old B. Young adult (age <20): 11% C. Age 45 years old: 24% D. Lifetime risk: 25% III. Pathophysiology A. Silent Cerebral Infarction 1. Silent CVA may occur in up to 25% of children with Sickle Cell Anemia by age 6 years...

Comprehensive Handbook of Childhood Cancer and Sickle Cell Disease: A Biopsychosocial Approach

Oxford University Press, USA

Over recent decades, tremendous advances in the prevention, medical treatment, and quality of life issues in children and adolescents surviving cancer have spawned a host of research on pediatric psychosocial oncology. This important volume fulfills the clear need for an up-to-date, comprehensive handbook for practitioners that delineates the most recent research in the field–the first of its kind in over a decade. Over 60 renowned authors have...

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