What Is Sickle Cell Disease?
Sickle cell disease is a group of disorders passed down through families. People with sickle cell disease have abnormal hemoglobin in their red blood cells, called hemoglobin S or sickle hemoglobin.
Approximately 100,000 Americans are affected by sickle cell disease (SCD). It is most common among those of African descent, occurring in about 1 out of every 365 African-American births.
Sickle cell anemia arises when a person has two hemoglobin S genes, known as hemoglobin SS. This is the most common and often most severe type of sickle cell disease.
Learn more about causes, signs and symptoms of sickle cell disease
How We Treat Sickle Cell Disease
The Penn Comprehensive Sickle Cell Program provides exceptional, comprehensive medical care for adult patients with sickle cell disease. We offer cutting-edge care to meet each patient’s individual needs.
Treatment methods include:
- Comprehensive pain management
We provide multi-level care focused on goal-oriented pain management. Our treatment incorporates physical, mental and emotional well-being, allowing patients the best possible quality of life.
Everyone experiences pain differently, requiring an individualized approach to treatment. These methods can include: medications, lifestyle changes, mindfulness and other non-pharmacologic options. The Comprehensive Sickle Cell Team works with patients to develop plans that work with their needs to optimize quality of life, safety and function.
- Blood transfusions
The Hospital of the University of Pennsylvania (HUP) Blood Bank provides safe blood products for more than 65,000 transfusions per year. These products include red-blood cell transfusions for sickle cell disease patients who require them.
SCD patients who require red-blood cell exchange on a regular basis receive treatment in a state-of-the-art 19-bed apheresis/infusion unit.
SCD patients have an increased risk of infection by seasonal influenza, pneumococcus, meningococcus and haemophilus bacteria. These bacteria can cause pneumonia, meningitis, sepsis, and other diseases that can affect breathing, bones, joints and the nervous system.
We aim to reduce the frequency and severity of these infections by providing periodic vaccinations that protect against them.
Meet Penn's Sickle Cell Disease Providers
Why Penn’s Sickle Cell Disease Program Is Different
Our program is special because it offers a multidisciplinary approach, providing patients with attentive care tailored to their specific needs. Our providers, programs and services are designed to offer guidance every step of the way.
Coordinated Subspecialty Care
Program physicians coordinate patient care with physician-specialists in areas of particular importance to patients with sickle cell disease. These include:
- Cardiology (heart)
- Pulmonology (lungs)
- Endocrinology (bone health, diabetes, reproduction)
- Gastroenterology (liver and gastrointestinal tract)
- Ophthalmology (eyes)
- Renal (kidney)
- Orthopedics (bones and joints)
Psychosocial issues can often affect patients who have a chronic illness such as SCD. Our dedicated program social workers and community health workers provide a wide range of services to help patients and their families cope.
The social workers in our Comprehensive Sickle Cell Program lead from a strength-based perspective, supporting and assisting patients on their wellness journey. They help facilitate transitions from pediatric to adult care, collaborate with multidisciplinary teams and community partners, and provide individualized psychosocial support for all patients.
Infusion for Sickle Cell Patients at the Oncology Evaluation Center (OEC)
The SCD Infusion Center at the Oncology Evaluation Center (OEC) is a same-day, urgent evaluation and treatment center for Penn Medicine patients with conditions like sickle cell disease. When sickle cell disease patients are experiencing uncomplicated pain or mild dehydration, the evaluation center provides rapid treatment with fluids and/or pain medications to avoid the need for hospital admission.
The OEC is located at the Perelman Center for Advanced Medicine.
Pediatric to Adult Transition Program
The Comprehensive SCD Program coordinates the transition of care for young adults who presently receive treatment at The Children’s Hospital of Philadelphia (CHOP) or St. Christopher’s Hospital for Children.
The transition program aims to provide a seamless move from pediatric to adult care, with close communication and collaboration between the two teams.
SCD Support Group
The SCD Program social workers coordinate a patient support group every two weeks. Patients are able to share their thoughts and feelings in a nonjudgmental setting while sharing fellowship with others that share their experiences. Our team helps participants with stress management and coping with life challenges that are common among patients with SCD.
Sickle Cell Disease Research and Clinical Trials
Providers in the Comprehensive SCD Program are at the forefront of research designed to understand how SCD affects patients. They then use this information to bring novel treatments into clinical practice.
The SCD program actively participates in clinical trials and studies conducted by colleagues in hematology, radiology, emergency medicine and pediatrics. We also have plans to expand research programs to include bone marrow transplantation and gene therapy approaches.