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.

#1 Best Seller on Sickle Cell Disease (SCD). Sickle cell disease is a group of blood disorders passed down from parents to children. Sickle cell anemia shortens life expectancy by 30 years via bacterial infections, painful swellings, fever, arthritis, leg ulcers, eye, lung & heart damage. Over 100,000 people, mostly African-Americans, in the United States have sickle cell disease. Over 2 million people have sickle cell trait in America. It is estimated that more than 300,000 children are born each year with SCD around the world. This edition of The Management of Sickle Cell Disease (SCD) is organized into four parts: 1. Diagnosis and Counseling 2. Health Maintenance 3. Treatment of Acute and Chronic Complications 4. Special Topics. The original intent was to incorporate evidence-based medicine into each chapter, but there was variation among evidence-level scales, and some authors felt recommendations could be made, based on accepted practice, without formal trials in this rare disorder. The best evidence still is represented by randomized, controlled trials (RCTs), but variations exist in their design, conduct, endpoints, and analyses. It should be emphasized that selected people enter a trial, and results should apply in practice specifically to populations with the same characteristics as those in the trial. Randomization is used to reduce imbalances between groups, but unexpected factors sometimes may confound analysis or interpretation. In addition, a trial may last only a short period of time, but long-term clinical implications may exist. Another issue is treatment variation, for example, a new pneumococcal vaccine developed after the trial, which has not been tested formally in a sickle cell population. Earlier trial results may be accepted, based on the assumption that the change is small. In some cases, RCTs cannot be done satisfactorily (e.g., for ethical reasons, an insufficient number of patients, or a lack of objective measures for sickle cell "crises"). Thus the bulk of clinical experience in SCD still remains in the moderately strong and weaker categories of evidence. Not everyone has an efficacious outcome in a clinical trial, and the frequency of adverse events, such as with long-term transfusion programs or hematopoietic transplants, might not be considered. Thus, an assessment of benefit-to-risk ratio should enter into translation of evidence levels into practice recommendations. A final issue is that there may be two alternative approaches that are competitive (e.g., transfusions and hydroxyurea). In this case the pros and cons of each course of treatment should be discussed with the patient. This book is B&W copy of the government agency publication.

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.

Sickle cell disease can be severe and disabling. When properly treated, patients live longer and with better quality life. This is a US government publication intended to provide evidence-based guidelines for the care of these patients for the use of all concerned providers as well as patients and family members. This book is available in print here for convenience.

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.

This book provides a comprehensive, state-of-the art review of hematopoietic stem cell transplantation (HSCT) for sickle cell disease (SCD). The book reviews new data about risk prediction for severe SCD, outlines the unique challenges of HSCT for patients with SCD, profiles the supportive care guidelines for patients who are undergoing HSCT, highlights our current understanding of the best transfusion support for SCD patients prior to, during and after HSCT, and provides new perspectives about the ethics of HSCT for pediatric patients with SCD. Published in the last few years, several landmark phase III trials that utilize matched unrelated and haploidentical donors for HSCT in SCD patients are also placed in context with respect to current management. Written by experts in the field, Sickle Cell Disease and Hematopoietic Stem Cell Transplantation is a valuable resource for physicians and researchers dealing with and interested in this challenging, yet exciting, curative therapy for sickle cell disease, that will help guide patient management and stimulate investigative efforts.