Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Sickle Cell Disease: Current Treatment and Emerging Therapies
source: The American Journal of Managed Care (AJMC)
year: 2019
authors: Lynne D. Neumayr, Carolyn C. Hoppe, Clark Brown
summary/abstract:Sickle cell disease (SCD) is among the most common genetic diseases in the United States, affecting approximately 100,000 people. In the United States, SCD is characterized by a shortened life expectancy of only about 50 years in severe subtypes, significant quality-of-life impairments, and increased healthcare utilization and spending. SCD is characterized by chronic hemolytic anemia, vaso-occlusion, and progressive vascular injury affecting multiple organ systems. The pathophysiology is directly related to polymerization of deoxygenated hemoglobin, leading to a cascade of pathologic events including erythrocyte sickling, vaso-occlusion, tissue ischemia, and reperfusion injury as well as hemolysis, abnormal activation of inflammatory and oxidative pathways, endothelial dysfunction, increased oxidative stress, and activation of coagulation pathways.
These multifactorial abnormalities have both acute and chronic clinical consequences across multiple organ systems, including acute pain episodes, chronic pain syndromes, acute chest syndrome, anemia, stroke and silent cerebral infarcts, cognitive dysfunction, pulmonary hypertension, and a wide range of other clinical consequences.
organization: UCSF Benioff Children’s Hospital Oakland, USA; Global Blood Therapeutics, USA; Emory University, USA; Children’s Healthcare of Atlanta, Scottish Rite Hospital, USAread more
Related Content
-
Big Jump in Success for Sickle Cell TransplantsDoubling the dose of total-body irradiat...
-
A new Sickle Cell Disease Drug Holds Much Promise but Most Sufferers Won’t be Able to Afford itThe regulatory approval of a groundbreak...
-
Medical and Surgical Complications of Sickle Cell AnemiaThis book is a wide-ranging guide to the...
-
Blood exchange transfusion safety for priapism in sickle cell disease: a single institution reviewBackground: Males with sickle cell disea...
-
Johns Hopkins researchers offer new protocol to potentially cure sickle cell diseaseThere's new hope for patients who have b...
-
Older Blood Used in Transfusions May Be Harmful to Adult Sickle Cell Patients, Review FindsIn an analysis of adults with sickle cel...
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.