A sickle cell disease is a group of blood disorders that a person inherits from parents. They usually occur when the person inherits two abnormal copies of the hemoglobin gene. Sickle cell anemia is the most common type of sickle cell disease. It causes an abnormality in hemoglobin, the oxygen-carrying protein found in red blood cells. The problems due to sickle cell anemia begin to appear around 5 to 6 months of age. It leads to problems such as sickle cell crisis, swelling in hands and feet, stroke and bacterial infections. The care of people suffering from sickle cell anemia includes infection prevention with vaccination and antibiotics, folic acid supplementation and pain medication. A bone marrow transplant is also used in certain cases. This book consists of contributions made by international experts. It contains some path-breaking studies in sickle cell anemia. It will serve as a valuable source of reference for graduate and post graduate students.

Although sickle cell anemia was the first molecular disease to be identified, its complex and fascinating pathophysiology is still not fully understood. A single mutation in the beta-globin gene incurs numerous molecular and cellular mechanisms that contribute to the plethora of symptoms associated with the disease. Our knowledge regarding sickle cell disease mechanisms, while still not complete, has broadened considerably over the last decades. Sickle Cell Anemia: From Basic Science to Clinical Practice aims to provide an update on our current understanding of the disease’s pathophysiology and use this information as a basis to discuss its manifestations in childhood and adulthood. Current therapies and prospects for the development of new approaches for the management of the disease are also covered.

Written by 80 of the world's foremost basic scientists and clinicians, this volume is the first comprehensive reference on sickle cell disease. Because this disease has diverse manifestations and involves many medical specialties, the contributors were chosen for their expertise in specific areas. Their discussions cover virtually every aspect of the disease - its molecular and cellular biology, pathophysiology, diagnosis, organ-specific complications, and clinical management.

Sickle Cell Disease is the most common genetic disease world wide and in the UK. It has marked geographical variation in its distribution in the UK, with a concentration in London and other major conurbations (Birmingham and Manchester). In these areas, specialist centres have become established offering expert, up to date care for both inpatients and out patients with Sickle Cell Disease. Although patient numbers are increasing outside these areas, the expertise of health professionals can be patchy. This book aims to provide a user friendly, accessible resource for areas with smaller numbers of patients, to allow them to provide equitable care with the larger well established centres. Sickle Cell Disease can be associated with acute life threatening complications, when clear, easily available advice is needed, and with chronic long term complications which may need liaison with other health professionals. Clear treatment protocols for all the common complications of sickle cell disease, are outlined here, with summaries of key evidence and references.

The most comprehensive, current sickle cell disease resource—for both clinicians and researchers A Doody's Core Title for 2023! The first and only resource of its kind, Sickle Cell Disease examines this blood disorder through both clinical and research lenses. More than 80 dedicated experts in the field present their combined clinical knowledge of basic mechanisms, screening, diagnosis, management, and treatment of myriad complex complications of a single base point mutation in the human genome. Case studies with “How I Treat” authoritative insights provide overviews of common and rare complications, and Key Facts offer at-a-glance high-yield information. Filled with clinical photos, illustrations, numerous original diagrams, and with free updates available online, this unmatched resource covers: Mechanisms of sickle cell disease Historic and current research approaches The latest work in gene therapy and editing Guidelines for patient care, diagnosis, unique cases, and therapies Rare and common complications, including domestic and internationally relevant topics Psychosocial and supportive care The newest standards of therapy and future treatment options in children and adults Cardiopulmonary complications

Sickle Cell Pain is a panoramic, in-depth exploration of every scientific, human, and social dimension of this cruel disease. This comprehensive, definitive work is unique in that it is the only book devoted to sickle cell pain, as opposed to general aspects of the disease. The 752-page book links sickle cell pain to basic, clinical, and translational research, addressing various aspects of sickle pain from molecular biology to the psychosocial aspects of the disease. Supplemented with patient narratives, case studies, and visual art, Sickle Cell Pain’s scientific rigor extends through its discussion of analgesic pharmacology, including abuse-deterrent formulations. The book also addresses in great detail inequities in access to care, stereotyping and stigmatization of patients, the implications of rapidly evolving models of care, and recent legislation and litigation and their consequences.

Since the first case of sickle cell disease was described in 1910, several efforts have been made to improve its management. However, it remains the leading scourge of our times, with a high level of morbidity and mortality in Sub-Saharan Africa, the Middle East and India. There have been few efforts by academia in developing countries towards contributing to in-depth knowledge of sickle cell disease. This volume rectifies this by providing a comprehensive review of sickle cell disease from a multidisciplinary point of view. Bringing together a number of experts in the field, the text highlights details of what is known and areas in which future work and advances are needed. The contributions contain comprehensive information on all aspects of the disease, and provides a solid foundation for future studies.

In this issue of Hematology/Oncology Clinics, guest editors Drs. Sophie Lanzkron and Jane Little bring their considerable expertise to the topic of Sickle Cell Syndromes. Top experts in the field cover key topics such as structural racism and impact on sickle cell disease (SCD); pathophysiology and biomarkers of SCD; genetic modifiers of SCD; allogeneic transplant and gene therapy: reproductive health; chronic pain; and more. Contains 16 relevant, practice-oriented topics including innovative therapies, addressing challenging complications, novel science on mechanisms of disease; preventing cognitive decline in people with SCD; quality of life in SCD; and more. Provides in-depth clinical reviews on sickle cell syndromes, offering actionable insights for clinical practice. Presents the latest information on this timely, focused topic under the leadership of experienced editors in the field. Authors synthesize and distill the latest research and practice guidelines to create clinically significant, topic-based reviews.

Sickle cell anemia is an inherited disorder of the globin chains that causes hemolysis and chronic organ damage. Sickle cell anemia is the most common form of sickle cell disease (SCD), with a lifelong affliction of hemolytic anemia requiring blood transfusions, pain crises, and organ damage. Since the first description of the irregular sickle-shaped red blood cells (RBCs) more than 100 years ago, our understanding of the disease has evolved tremendously. Recent advances in the field, more so within the last three decades, have alleviated symptoms for countless patients, especially in high-income countries. Although there is evidence of several important therapies in the pipeline, greater investment in research is needed into both of these therapies and the dissemination of effective care to the affected population, especially because of historical mistrust. In this book, we present an overview of sickle cell disease, pathogenesis, clinical presentation, complications, and recent treatment modalities and prospective research that will enable the reader to get a better understanding of this hot topic.

Sickle cell disease is a complex disease with a genetic mutation producing a wide variety of manifestations. It is of great public health importance nationally and internationally. While the conclusions of this study support the generally accepted views on the disease, the book provides the most recent information and state-of-the-art knowledge on the subject. The book is unique in that it presents the genetic, molecular, cellular, metabolic, rheologic, clinical, diagnostic, therapeutic, and psychosocial dimensions of sickle cell disease. Its synthesis of micro to macro, molecular to cellular, individual to social relationships provides a comprehensive study of sickle cell disease. The book offers a synthesis of basic science describing genetic, molecular, metabolic, and rheologic aspects of sickle cell disease. Clinical aspects, including diagnosis and treatment, and psychosocial aspects, covering the interaction of the patient with sickle cell disease with society, are also treated. Physicians, scientists, allied health workers, sociologists, and social workers, epidemiologists and anthropologists as well as administrators of sickle cell centers will find this volume useful. The ultimate goal of the book is to improve the understanding of sickle cell disease and to contribute to a better quality of life for those who have the disease or who are at risk of getting it.