RD306 - Report to the House Appropriations and Senate Finance Committees of the Virginia General Assembly on Community-Based Sickle Cell Programs - September 26, 2016
What is Sickle Cell Disease?
Sickle Cell Disease is an inherited blood disorder where normal round shaped red blood cells change from their normal round shape to a "quarter-moon" or sickle-like 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.
Nationally, approximately 1 in 500 African American children is born with a serious Sickle Cell disorder, 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.”
The Commissioner of Health has the responsibility for sickle cell screening under the Code of Virginia. Screening of all newborns for sickle cell disease began in Virginia during July of 1989. 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.”
Community-based programs provide resources to support the development of coping strategies and support for families impacted by sickle cell disease by addressing unmet social, psychosocial and educational needs. 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 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.
There are four comprehensive sickle cell centers under contract for services 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 of the centers, but generally, do not provide services to non-patients of their hospitals. That means that the centers are only 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.
Those of us involved in community programs define community-based sickle cell programs as ones that work to assist any client needing services who is in the care of a private physician or any client receiving services from sickle cell centers who needs or wants additional service.