Jefferson University Hospitals

Comprehensive Sickle Cell Program

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Comprehensive Sickle Cell Program

Jefferson's Comprehensive Sickle Cell Program is a collaborative effort between the Division of Hematology and the Division of Internal Medicine. The Program offers longitudinal management for adult patients with sickle cell disease.

Our team includes board-certified internists and hematologists who oversee those in both inpatient and outpatient care. They work alongside our outpatient sickle cell nurse, sickle cell social worker, specialized sickle cell hematologists, and nurse practitioners. 

What is the Sickle Cell?

The function of the healthy sickle cell is as round red blood cells that move through your blood vessels to carry oxygen throughout your body. Sickle cell disease occurs when the red blood cells become hardened and crescent-shaped, thus blocking blood flow and oxygen to the body. Sickle cell is a genetic disorder that occurs at birth.  

Competent & Compassionate Long-Term Care

Our Program focuses on providing you with competent and compassionate long-term care if you are diagnosed with sickle cell disease. Our primary goal is excellence in clinical management and an emphasis on quality of life. We actively participate in research including clinical trials of new therapeutic approaches to sickle cell disease in order to improve the care of present and future patients with the disease.

If you are living with sickle cell disease, you can attend a monthly patient self-support group meeting coordinated by a Jefferson social worker who will teach you techniques to reduce stress and help you learn as much as possible about your disease and its complications.

Our program also includes 24/7 observational care to manage acute uncomplicated vaso occlusive pain crisis. 

Preventative Therapy for Sickle Cell Disease

Preventing the complications of sickle cell anemia are especially important. We offer the following therapies and programs:

  • Hydroxyurea Program – Jefferson participated in the seminal clinical trial, the Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH),  between 1992 and 1996. The Study showed that Hydroxyurea decreased the frequency of painful crises, acute chest syndrome and the need for blood transfusion. Patients with sickle cell anemia who meet the inclusion criteria and agree to the treatment are started on Hydroxyurea and followed regularly to monitor their progress and adjust the dose of Hydroxyurea as needed.
  • Crizanlizumab infusion – Jefferson participated in the phase 2 clinical trial. The study showed decrease in frequency of painful crises and hospital admissions.
  • Voxelotor – Jefferson participated in HOPE trial. The study showed improvement in hemoglobin levels and decrease in hemolysis or destruction of red blood cells.
  • Chelation therapy – Patients with sickle cell can become iron overloaded due to frequent blood transfusions. This iron overload can damage bodily organs including the heart, liver and joints. Programs are available for eligible patients to receive novel therapies to remove the excess iron from the body and thereby prevent organ damage.
  • Blood Transfusion/Blood Exchange Transfusion Program – We have an active Program of red blood cell transfusion/exchange transfusion, which is used to treat and/or prevent many of the complications of sickle cell disease. Patients who have had stroke are routinely managed in this manner to prevent recurrent stroke. Other indications of blood exchange may be acute chest syndrome, complicated pregnancy or major surgery.

Diagnostic Lab

Making the exact diagnosis of sickle cell disease is important for treatment, prognosis and genetic counseling. This often requires sophisticated laboratory testing not routinely available in many hospitals. Our Program has a special diagnostic lab capable of making the exact molecular diagnosis of sickle cell anemia or other inherited disorder of red blood cell hemoglobin.

Community Outreach for Sickle Cell Disease

Our Program maintains close relations with the Philadelphia/Delaware Valley Chapter of the Sickle Cell Disease Association of America (SCDAA). The SCDAA is an advocacy organization for patients with sickle cell anemia. We often involve the SCDAA with some of the psychosocial and personal issues of our patients.

Contact the Sickle Cell Program

Our Program welcomes new patients. We also work with the regional pediatric sickle cell centers to ensure a smooth transition from pediatric care to adult care. Please call 215-955-6180 to schedule an appointment or to receive additional information about the Comprehensive Sickle Cell Program from our program coordinator.

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