Google
Trusted Resources: Evidence & Education
Scientific literature and patient education texts
CDC grand rounds: Improving the lives of persons with sickle cell disease
source: Morbidity and Mortality Weekly Report
year: 2017
authors: Mary Hulihan, Kathryn L. Hassell, Jean L. Raphael, Kim Smith-Whitley, Phoebe Thorpe
summary/abstract:Approximately 100,000 Americans have sickle cell disease (SCD), a group of recessively inherited red blood cell disorders characterized by abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in the red blood cells. Persons with hemoglobin SS or hemoglobin Sß0 thalassemia, also known as sickle cell anemia (SCA), have the most severe form of SCD. Hemoglobin SC disease and hemoglobin Sß+ thalassemia are other common forms of SCD. Red blood cells that contain sickle hemoglobin are inflexible and can stick to vessel walls, causing a blockage that slows or stops blood flow. When this happens, oxygen cannot reach nearby tissues, leading to attacks of sudden, severe pain, called pain crises, which are the clinical hallmark of SCD.
The red cell sickling and poor oxygen delivery can also cause damage to the brain, spleen, eyes, lungs, liver, and multiple other organs and organ systems. These chronic complications can lead to increased morbidity, early mortality, or both. Tremendous strides in treating and preventing the complications of SCD have extended life expectancy. Now, nearly 95% of persons born with SCD in the United States reach age 18 years; however, adults with the most severe forms of SCD have a life span that is 20–30 years shorter than that of persons without SCD.
organization: National Center on Birth Defects and Developmental Disabilities, CDC; University of Colorado, Aurora, Colorado; Baylor College of Medicine, Houston, Texas; The Children’s Hospital of Philadelphia, Pennsylvania; Office of the Associate Director for Science, CDCDOI: 10.15585/mmwr.mm6646a2
read more full text
+myBinderRelated Content
-
A Clinically Meaningful Fetal Hemoglobin Threshold for Children With Sickle Cell Anemia During Hydroxyurea TherapyHydroxyurea has proven clinical benefits...
-
Emergency Room 101 (tips for the empowered patient)Millions of people find themselves in a ...
-
With Cincinnati Children’s Hospital – What is Sickle Cell Trait?https://www.youtube.com/watch?v=MDepyJP5...
-
‘Every Time It’s A Battle’: In Excruciating Pain, Sickle Cell Patients Are Shunted AsideAmy Mason had toughed it out for hours o...
-
Improving Quality of Life Through Sickle Cell Research: Marsha J. Treadwell, MDhttps://www.youtube.com/watch?v=DA51AAUH...
-
Sickle Cell and Its Peculiarities (I)Whenever I shared other people’s disti...
-
The challenges of being a caregiver to my cousin with sickle cellWhen I embarked on this journey as a c...
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.
Powered by
Support for this site is provided by
This platform is made possible through a partnership with the Sickle Cell Disease Association of America, Inc. (SCDAA) and its member organizations. SCDAA's mission is to advocate for people affected by sickle cell conditions and empower community-based organizations to maximize quality of life and raise public consciousness while advancing the search for a universal cure.