Sickle cell disease patients receive routine care in the Hematology Outpatient Clinic on the 4th floor of the Wood building at the Main Hospital and in specialty care centers in King of Prussia, PA and Voorhees, NJ.
The clinics are staffed by a multidisciplinary team of board-certified pediatric hematologists, hematology nurse-specialists, and a social worker. Each patient is assigned a physician who manages his or her outpatient care. Patient and family education is provided at the time of diagnosis and is incorporated into all clinic visits as part of comprehensive care. To make an appointment, call 215-590-3423.
Because sickle cell disease is a complicated condition with various manifestations, a broad range of specialty services are required to meet the needs of children with the disease. Our patients have access to pediatric general surgeons, ophthalmologists and pulmonologists with expertise in the care of patients with sickle cell disease. All work closely with the sickle cell team.
Asthma is common in children with sickle cell disease and the control of asthma may decrease the risk of pulmonary complications. Upper airway symptoms, such as snoring, are also common and specialists in sleep disorders address such issues. Attention to these symptoms may decrease the incidence of acute chest syndrome.
Members of the stroke team work closely with the sickle cell team to provide comprehensive care to our sickle cell patients who are at risk or have experienced cerebrovascular complications, including stroke.
Access to state-of-the-art imaging techniques plays a major role in the care of sickle cell patients. Services include transcranial Doppler studies for stroke prevention, abdominal ultrasound for gallstones, imaging of the bones to evaluate avascular necrosis, and imaging of the brain for detection of silent infarcts.
Imaging techniques are also used to identify chronic organ damage from red cell sickling. Neuropsychological screening is available to help identify problems, which if not adequately addressed, can affect a child's ability to learn. Consultations with a psychiatrist are arranged as needed.
Sickle cell disease imposes stress on both patient and family. Social workers at the Sickle Cell Center provide a wide range of services related to psychosocial needs in coping with chronic illness as well as navigation through hospital and insurance systems. Assistance is also provided for school, employment and financial issues and in referrals to community-based and support agencies. When needed, social workers coordinate psychiatric and psychological consultations with specialists within the Hospital.
All parents and patients can reach a hematologist on a 24-hour basis. From 8:30 am - 5:00 pm Monday - Friday, call 215-590-3437. After 5 pm and on weekends or holidays, please call 215-590-1000. Parents, caretakers, and older patients should call whenever a patient is ill so that they can be directed to an appropriate site for treatment. For acute illness, patients may go to the Hematology Clinic (for minor problems and by appointment), the Hematology Acute Care Unit, or the Emergency Department.
The Sickle Cell Center's Hematology Acute Care Unit (HACU), which can be reached at 215-590-7311, provides rapid evaluation and treatment for children with blood diseases. Between 7 a.m. and 11 p.m., parents may bypass the Emergency Department and come straight to the HACU at 5 East in the Main Hospital for evaluation and treatment of non-emergent, acute illness. Hematologists and hematology nurse practitioners promptly see the patients to evaluate and initiate management of acute illness. If, after 23 hours in the HACU, the patient is not ready to be managed at home, the child stays on as an inpatient.
Transfusion services have been developed specifically for sickle cell patients. Chronic blood transfusions, usually every three to four weeks, are provided to prevent recurrence of stroke in a child who has already experienced stroke or to avoid stroke in a child who has been determined to be at risk for stroke. Some people with sickle cell disease receive chronic transfusions to reduce the number of painful episodes or the risk of recurrent acute chest syndrome and other complications.
About 15 percent of sickle cell patients at Children's Hospital are on chronic transfusions. Simple transfusions are provided through the Hematology-Oncology Day Hospital adjacent to the Oncology Clinic on the 4th floor of the Wood Building at the Main Hospital.
Automated exchange transfusion, given on the Apheresis Unit, is the preferred method of transfusion for those on chronic transfusions to avoid iron accumulation. When possible, blood is used from a special blood donor program developed at Children's Hospital in cooperation with the American Red Cross Penn-Jersey Region.
The program also serves as a center for newborn testing for sickle cell disease, genetic counseling and treatment following newborn screening.