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 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 the world’s first 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. Patient education is performed by all staff during clinic visits. There are special educational meetings for patients and family members on special topics. Health care providers are educated by annual two day conferences. 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.

Dr. Eckman and JoAnn Beasley RN, have championed national newborn screening for sickle cell disease. 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. Data from the Georgia program demonstrates a lower infant mortality for those babies detected with sickle cell disease that the general population.

JoAnn Beasley,RN is one of two Newborn Screening Coordinators for the State of Georgia. Parents of newborns detected through mandatory screening are notified and referred to a health care provider for counseling, education, and preventive care. Parents in the Atlanta metro area are counseled by Ms. Beasley. Parents are taught what to expect and how to detect and prevent complications. The simple act of taking a temperature and reading a thermometer can save a child from fatal sepsis.

The Parent-Patient support group has existed since the beginning of the center. One parent of a son with sickle cell disease, Berrutha Harper, helped lobby the Georgia Legislature to fund the 24-hour sickle cell center at Grady. The support group functions by providing financial support, food, clothing and special needs for patients who have no other resources. A Christmas party for all of the center’s children is co-sponsored by the center staff and the Parent Patients group. The Pediatric social worker and other interested staff members have formed a teen peer support group that involves monthly meetings, fun outings and teen topics. Adult support groups have formed as patient's needs change and it is led by the adult social worker and the clinical nurse specialist in psychiatry.

A new support model pioneered in Denver by a Pediatric Hematology fellow, pairing medical students with sickle cell children is underway. Students from Emory and Morehouse medical schools are buddy paired with sickle cell children for several social outings. The benefits are mutual, educating future physicians about the trials of a lifelong chronic disease, and the patients have a role model to help inspire educational pursuits.

A major problem for adult patients is the ability to obtain employment that will not interfere with their disease, and will allow time off for prolonged crisis. A one-time grant was awarded to establish a multimedia-training center for sickle cell patients. A multimedia teacher was placed on the team to provide computer based training and establish a small patient run business. The multimedia center has been designated a State of Georgia Vocational Rehabilitation training site and allows patients to come to a familiar location for training. Patients have been trained in typing skills, photograph development, job interview skills, resume writing, and catalog assembly. Patient education is available on CD-ROM on sickle cell disease and a variety of medical topics.

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, Atlanta is 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 a patient followed at the Center in 1998. The child is disease free and doing well one year post transplant.

An extensive computerized patient database was developed to help track clinical care, costs and utilization of services. 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, patients treated with concentrated fish oil had a 50% reduction in pain episodes when compared to the control group. The results have prompted a national multicenter trial now underway.

New psychosocial interventions involving family teaching and testing were established. It was documented with pre and posttest evaluations that the level of education about sickle cell disease improved with the intervention. New patient and professional educational materials on computer based CD-ROM and Internet technology including a partnership with the Starbright Foundation to provide web based education for hospitalized children worldwide.

Through special kidney studies, it was determined that small amounts of urine protein is an early detection test that can alert clinicians to the problem of kidney damage, which may be as high as 60% in adults with sickle cell disease. Preventive use of ACE inhibitors, medications used to control hypertension; to prevent progressive renal disease is currently being studied.

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.

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. This study clearly documented the benefit of early detection and treatment with 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 24-hour clinic staff allows 80% of the patients with acute pain to return home without the need for inpatient admission.

. Pain is the most common presentation for those with sickle cell disease as well as all patients presenting to hospitals. Patients have stated that their level of pain is rarely assessed and not believed by health care professionals. Patients are usually inadequately treated and require prolonged hospitalizations for pain management. James Eckman and Allan Platt have worked seven years developing a new multidimensional pain assessment system utilizing palm touch screen computers. Using the palm computers, caregivers can assess patient’s pain intensity, mood, relief and side effects in less than two minutes, generating a computer history of pain episodes. Patients are also asked to numerically rank the importance of each pain dimension. This led to the development of a new, computer generated, pain score the MPS, or Multidimensional Pain Score, a number from 0 to 10 that correlates with the sensory portion of McGill Pain Questionnaire. This development has set a new pain standard and pain assessment method that exceeds the new JCAHO pain assessment requirements and provides documentation and outcome analysis for patients in pain. It allows vulnerable patient populations to have their pain quantified and easily documented over time.

The pain system has been incorporated into a palm based software program that allows care givers to document all of the patient’s vital signs, weight, intake/output, therapy given and patient satisfaction. Putting the pain assessment with the vital signs in an easy to use bedside tool and allows pain levels to become the fifth vital sign. The ease of documentation encourages frequent pain assessment and real time pain intervention.