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Sickle Cell Research and Publications from the Staff of The Georgia Comprehensive Sickle Cell Center at Grady Health System in Atlanta, Georgia

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History of the Research Effort

Sickle cell disease is one of the most common genetic diseases in the United States, affecting one out of four hundred African Americans. This population is usually undeserved with a lack of specialty care available in the communities where the disease is prevalent. The disease causes recurrent pain episodes, increased infections and other complications requiring emergency medical intervention. Prior to 1984, patients presenting with uncomplicated sickle cell pain crisis were seen in the Grady Memorial Hospital Emergency Care Center and other Atlanta emergency rooms. With an excess of a quarter of a million patients in the Grady ER annually, sickle cell patients were often placed low on triage lists. Evaluation procedures were erratic and continuity of care was usually absent.

For this reason, an intense lobbying effort was led by the Sickle Cell Patient/Parent Group, Grady hospital administration and medical staff convinced members of the Georgia General Assembly of the need for a specialized clinic in the State of Georgia. In 1984, the Georgia General Assembly provided the original state grant of $550,000 to Grady Memorial Hospital to fund a 24-hour comprehensive acute care, Sickle Cell Center. The goals of the Center were to provide 24-hour acute care in a designated area with a dedicated staff, health care consultation, research new treatments, education and support services to residents of the state of Georgia with sickle cell syndromes. Pediatric and Adult Health Maintenance Appointment clinics deliver primary care to this population with the same staff and facilities. Center staff also visit inpatients with sickle syndromes on a daily basis to assist in management of specific problems and to maintain continuity of care. A number of group and individual counseling services are provided. Job training is provided for patients on site in a unique multimedia training room. The combination of these acute, primary care, and health maintenance services provide for an uncommon mix that has proven to serve the patient population in a very cost-effective manner. The program provides a wellness model for treatment of patients with a severe chronic disease, providing a practical solution to many problems faced in modern health care delivery.

Having established the clinic on this foundation, during the past 14 years the Center has grown in patient numbers, expanded its scope of services, and become an international leader in the care of sickle cell patients. This has occurred through the development of clinical guidelines that were first published and distributed world wide in 1991 and are now available on the World Wide Web for all providers to access (http://www.emory.edu/PEDS/SICKLE). An extensive computerized patient database was developed to help track clinical care, costs and utilization of services. Computer based multimedia teaching materials about sickle cell disease were developed and offered to patients in a dedicated multimedia teaching center. Educational conferences are provided annually to update health care providers about the latest in sickle cell treatment. This educational material is captured in video and in CD-ROM format for international distribution.

The clinical success of the center, led by Dr. James Eckman, with a dedicated staff of hematologists, physician assistants, nurse practitioners, nurses, clinic assistants, social workers and clinical nurse specialists in psychiatry, allowed the staff to apply for Federal research funding. In 1993, The National Institutes of Health awarded Dr. Eckman, Emory University, and the Sickle Cell Center $7 million over five years in research funding for projects to discover new treatments and prevention of complications. From this research grant, we are now the leading center with five successful transplants of the fifty now transplanted in the twenty-five centers providing bone marrow transplants to cure sickle cell disease in the United States and Europe. The first unrelated cord blood stem cell transplant in the world for sickle cell disease was performed on one of our patients, who is still in the monitoring phase. New treatments were studied such as concentrated fish oil, or N3 fatty acids, that prevent pain events with little or no side effects. Over a one year period ten adult patients were randomized to receive the fish oil or the control substance, olive oil. Those receiving the fish oil had a 50% reduction in pain episodes when compared to the control group. This study also demonstrated the platelet activation and procoagulant activity in sickle cell patients redefining the role of thrombosis in sickle complications.

New psychosocial interventions involving family teaching and testing were established. Twenty five families completed a ten week educational intervention with nineteen families as controls. It was documented with pre and post test evaluations that the level of education about sickle cell disease improved with the intervention. One hunded and fifteen children were evalusted with extensive neurocognitive and psycological tests demonstrating a bidirectional interaction with the mothers’ coping correlating both to the childrens’ adaptation and the severity of disease. New patient and professional educational materials on computer based CD-ROM and Internet technology was developed.

Fifty-three sickle cell patients and seventeen controls were evaluated for kidney damage. The cause of damage in sickle cell disease was characterized as a loss of permselectivity and ultrafiltration capacity. Mirco-albinuria is an early detection test that can alert clinicians to this problem. This study determined that the prevalence of glomerular damage may be as high as 60% in adults with sickle cell disease. Preventive use of ACE inhibitors to prevent progressive renal disease is currently being studied.

Engineers at Georgia Tech, describing the causes and possible interventions, studied sickle red blood cell adhesion to blood vessel walls. Four major pathways of adherence have been characterized under flow conditions. The engineers have delveloped a cone and plate adhesion apparatus that simulates flow conditions inside blood vessels. They monitor sickle red cell adhesion to endothelial cells and have monitored the effect of nonionic poloxamer surfactant in blocking adhesion. This will help guide future therapy targeted at reducing sickle red cell adherance which may provide clinically useful treatment for acute complications.

The Center participated in the national multicenter study of the preventive drug hydroxiurea, now the only FDA approved therapy for preventing sickle cell pain events. Hydroxyurea was found to reduce pain episodes by one-half, the need for inpatient admission by one-half, and the need for transfused blood by one-half.

Dr. Eckman has championed newborn screening for sickle cell. This has saved the lives of many sickle cell children who would have died from pneumococcal sepsis if timely preventive penicillin was not started. It was through his efforts that Georgia instituted universal mandatory sickle cell screening for newborns in October of 1998.

The Center was one of the leading research facilities for the landmark transcranial doppler (TCD) ultrasound study to detect sickle cell children at high risk for strokes. One hundred and seventy children were screened, twenty-two were found to be at high risk, sixteen parents agreed to enter the protocol of monthly transfusion or standard therapy. This study clearly documented the benefit of chronic transfusion to prevent first stroke in these high risk children.

The Sickle Cell Center is an experiment in itself as a new model for chronic disease management. The computerized patient database has tracked the clinic population over several years. Over the last ten years, the annual cost of treating adult patients has been reduced by over two-thirds by reducing the need for hospitalization and increasing the compliance with health maintenance. The multidisciplinary team, the case management, the role of specialists acting as the primary care givers has created these beneficial trends. The aggressive treatment of pain events by the same clinic staff allows 80% of the patients with pain to return home without inpatient admission.

Improved Health Care Reduces Costs for Treatment

Year

Outpatient Visits

Admissions/100 Patient Years

Cost/Active Adult

1985

1,134

215

$15,932

1996

2,032

83

$4,981

Currently, the Center is investigating the use of inhaled nitric oxide for treating pain episodes, a new pain meter for assessing pain in four dimensions over time, a therapeutic intervention to prevent kidney damage, and a teen intervention group. A unique vocational rehabilitation program is underway to train sickle cell patients in the Center’s multimedia center. Three classes of four patients each have graduated from a six week training course in photo processing. This program is one of the first hospital based vocational rehabilitation training program funded by the State of Georgia. The Sickle Cell Center has partnered with several Atlanta institutions to form The Atlanta Sickle Cell Consortium. This partnership will provide the best possible care and the latest advances for sickle cell care. The attached list represents the publications and presentations generated from the research.

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Research Publications

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Monographs, Position Papers, and Guidelines:

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Books and Book Chapters:

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Copyright © 1997 Sickle Cell Information Center
Last modified: July 20, 2003