What is Sickle Cell Disease and what is its impact?

Sickle Cell Disease is an inherited blood disorder where normal soft round shaped red blood cells change to a hard sticky sickle or quarter-moon shape. This disease is produced when the sickle cell gene is transmitted by both parents to a child. Sickled shaped cells cannot squeeze through small blood vessels so they often jam up, blocking the flow of blood and oxygen to body parts and causing extreme pain. A pain crisis can last for days or even weeks and may occur several times a year. Lack of oxygen flow can also damage muscles, bones and internal organs and lead to strokes and other serious medical problems. There is no universal cure.

Nationally, approximately 1 in 500 African American children is born with Sickle Cell Disease, making it the most common long term illness identified in this population. However, in Virginia, approximately 1 in 325 African Americans suffer with Sickle Cell Disease, far higher than the national average. Up to 100,000 people in the USA suffer with Sickle Cell Disease of which around 4,000 are in Virginia. Over 2,000,000 people in the USA have Sickle Cell Trait and approximately 155,000 are in Virginia.

DALLAS, Sept. 27, 2006 /PRNewswire/ -- “Sickle Cell Disease is one of the most prevalent and costly genetic disorders in the U.S. Today, one in every 4,000 Americans is born with a form of SCD and many experience chronic anemia, stroke, spleen and kidney dysfunction, pain crises, and susceptibility to bacterial infections. Moreover, the National Institutes of Health (NIH) estimates that almost one-third of adults with SCD develop pulmonary hypertension, a life-threatening condition resulting in a 10-fold greater risk of death."

“Due to this high disease burden, the Sickle Cell Disease Association of America (SCDAA) reports that Sickle Cell Disease in which abnormal hemoglobin causes red blood cells to become stiff, sickle-shaped and unable to flow easily through blood vessels -- results in an estimated 750,000 hospitalizations a year. The cost of these hospitalizations is estimated at $475 million annually."

Background

Screening of all newborns for Sickle Cell Disease began in Virginia during July of 1989. Since 1989, an average of 75 newborns have been identified yearly with Sickle Cell Disease. At the beginning of the program, centers for comprehensive follow-up care were not available. In 1994, Virginia began providing statewide comprehensive sickle cell services to decrease the morbidity and mortality among children.

The June 30, 2011 report to the House Appropriations and Senate Finance Committees of the Virginia General Assembly on Community-Based Sickle Cell Programs submitted by the Virginia Department of Health made the following statements. “The provision of comprehensive care is a time-intensive endeavor that includes ongoing patient and family education, periodic comprehensive evaluations and other disease-specific health maintenance services, psychosocial care, genetic counseling, and transition services. However, research demonstrates that the medical management model alone cannot address the multiple social, psychological, and educational needs of individuals living with chronic illnesses such as sickle cell disease."

Statewide Sickle Cell Chapters of Virginia, Inc. (SSCCV), also known as Sickle Cell Chapters of Virginia or Statewide, a non-profit 501(c)(3) tax-exempt community-based organization, has a network of nine (9) community-based Sickle Cell Disease organizations (chapters) that provide a variety of needed services across the Commonwealth. The chapters are located in Danville, Fredericksburg, Hampton, Lynchburg, Norfolk, Richmond, Rocky Mount, South Boston and Northern Virginia. Most of the chapters in this network have operated since 1972.

Our Chapters/Community-based programs provide resources to address unmet social, psychosocial and educational needs. They also provide coping strategies and support for families impacted by Sickle Cell Disease. Many of the community-based programs provide a focus on mental and social well-being as well as stress-reducing activities that have a positive effect on physical well-being. Some of the services include support group meetings, calls to check on clients, visitations, sending sickle cell information, "Birthday" and "Thinking of You" cards, providing referral assistance, assistance with obtaining disability benefits, employment and vocational rehabilitation. All chapters provide sickle cell education in the community through participation in health fairs, forums, church activities and civic or health related events. These services vary from chapter to chapter based in part on funds and man-power available.

All chapters receiving grant awards have previously conducted several years of “Surveys of Needs" with as many of their registered clients as possible. The surveys have enabled chapters to determine what services are needed, by whom and how best they can be delivered to clients.

There are four comprehensive sickle cell centers under contract in the state. The centers are Children’s Hospital of the King’s Daughters in Norfolk, Virginia Commonwealth University Health System in Richmond, University of Virginia in Charlottesville and D. C. Children’s Medical Center in Washington, D.C. which replaced INOVA Hospital in Fairfax, Virginia. It should be noted that only Virginia Commonwealth University Health System provides clinical care for adult clients.

It is estimated that the State of Virginia has about 4,000 sickle cell clients/patients. The centers care for around 1,200 of them. The centers provide care and services to patients, but generally, do not provide services to non-patients of their hospitals. That means that the centers are only fully serving around 30% of the sickle cell population. This demonstrates the very need for a network of community-based programs to serve the 70% of clients who still need services. Community-based sickle cell programs work to assist any client/patient needing services.