100 YRS

100 YRS

World Sickle Cell Day: 100 Years of Research

Sickle Cell Disease is a serious condition that affects people around the world. Although considered a childhood disease, people with severe Sickle Cell Disease are now living into adulthood but can experience a life expectancy that is 30 years shorter than those without disease.

A Historical Discovery

In November 1910, the Archives of Internal Medicine published a report by Dr. James B. Herrick titled 'Peculiar Elongated and Sickle-shaped Red Blood Corpuscles in a Case of Severe Anemia'. In this publication, Dr. Herrick described the experience of a young dental student from Grenada attending the Chicago College of Dental Surgery. Soon after arriving to dental school, the student sought medical attention and the abnormal shaped red blood cells in the student were first noted. These cells, along with anemia and symptoms typical of the disease soon became known as 'sickle cell anemia'. This year marks the 100th anniversary of this important case report.

Sickle Cell Disease (SCD) is a group of inherited red blood cell disorders. In SCD, red blood cells become hard and sticky and look like a C shaped farm tool called a "sickle." The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause chronic pain and other serious problems such as infections, stroke, and acute chest syndrome.

World Sickle Cell Day 2010

Although the severity of disease varies from person to person, complications from SCD are often life-threatening. Unfortunately, the distribution of the disease is such that it occurs among populations that have limited access to comprehensive care due to social, economic, cultural, and geographical barriers. People with severe SCD are now living into adulthood but can experience a life expectancy that is 30 years shorter than those without disease.

On December 22, 2008, the General Assembly of the United Nations adopted a resolution recognizing sickle cell disease as a public health problem. As a result of this resolution "World Sickle Cell Day" is celebrated on June 19th each year to increase awareness about sickle cell disease at the national and international level.

International Public Health Approach

The World Health Organization (WHO) has begun work to promote a global agenda to address hemoglobin disorders, also called hemoglobinopathies, including SCD. In May 2006, a resolution on Sickle Cell Disease was adopted that called upon affected countries and the secretariat of WHO to strengthen their response to these conditions. Specifically, WHO has made a commitment to:

•Increase awareness of the international community of the global burden of SCD;

•Promote equitable access to health services for people with SCD;

•Provide technical support to countries for the prevention and management of SCD, and ;

•Promote and support research to improve quality of life for those affected

Public Health Approach to SCD in the United States

Research to determine the number of people with SCD and to identify risk factors for complications can ultimately lead to a better understanding of the burden of SCD. The Centers for Disease Control and Prevention's (CDC) current activities in SCD include the following:

•Developing a national surveillance system to collect information on SCD and its complications to help determine occurrence rates and healthcare needs of people affected.

•Developing registries at clinical sites to identify people with SCD and to gather information about the health impact of living with SCD.

•Providing quality assurance and proficiency testing for newborn screening to ensure accurate early diagnosis of SCD.

Informatics and Infrastructure

Currently, there are no data systems that can provide an accurate count of the number of people with SCD in the United States or to fully describe the health impact of this condition on individuals. CDC has recently entered into partnership with the National Institutes of Health to plan and implement a pilot registry and surveillance system for hemoglobinopathies in six U.S. states called RuSH (Registry and Surveillance System for Hemoglobinopathies).

Health Education and Health Literacy

Because there is no cure for SCD, the goal for treatment of SCD

is the prevention of complications. Educating health care professionals

about treatment, prevention, research, and resources is essential to

improve the quality of care available to patients. Partnerships with

community-based organizations and with experts in SCD will help CDC

create messages tailored for different patient and family groups affected

by the condition.

Laboratory Capacity and Support

An important component of RuSH includes strengthening and

enhancing the information collected in the public health infrastructure in a way that will be linked and have the capacity to provide us with more information on people living with SCD.

Federal Interagency Partnership

A federal interagency collaborative has been established to address issues related to hemoglobinopathies including SCD. The hemoglobinopathy federal interagency partnership involves:

•National Institutes of Health (NIH),

•Food and Drug Administration (FDA),

•Health Resources and Services Administration (HRSA),

•Centers for Disease Control and Prevention (CDC),

•Centers for Medicare and Medicaid Services (CMS),

•Agency for Healthcare Research and Quality (AHRQ)

CDC considers SCD a major public health concern and is committed to conducting surveillance, raising awareness, and promoting health education. For more information visit our website at www.cdc.gov/blooddisorders or send an email to info@cdc.gov.

Content source: National Center on Birth Defects and Developmental Disabilities, Division of Blood Disorders