Philadelphia hub to aid Allegheny sickle cell care

Thomas Jefferson University in Philadelphia will lead a new federal effort to expand sickle cell care across the mid-Atlantic region. The U.S. Department of Health and Human Services selected Jefferson University and Jefferson Health to manage one of seven regional coordinating hubs nationwide. This designation places the institution at the center of a network designed to improve access to high-quality treatment for patients in Pennsylvania and surrounding states.

The initiative is part of a broader funding program aimed at improving health outcomes for people living with the condition. Dr. Sophie Lanzkron, division director of classical hematology at Thomas Jefferson University, explained that patients who receive care at comprehensive centers typically experience better results than those treated by local hematologists. She noted a specific gap in the region, pointing out that cities like Erie, Pennsylvania, lack a dedicated comprehensive center for the disease.

National data from the Centers for Disease Control and Prevention estimates that 100,000 people across the country live with sickle cell disease. The condition disproportionately affects residents who are Black or of African descent. State data shows that nearly 4,000 people in Pennsylvania are affected by the disease.

Lanzkron described the challenge of providing high-quality care to patients who do not live near major medical centers. She cited Jefferson, Penn Medicine, and the University of Pittsburgh as existing high-quality facilities. The new hub will work to bridge the distance between these centers and patients in more remote parts of the state.

Sickle cell disease is an inherited genetic condition passed down from parents who either have the disease or carry the affected gene. The condition impairs red blood cells, preventing them from carrying oxygen effectively to organs and tissues. This process can lead to anemia and organ damage. The blood cells often become rigid and warp into a C shape, causing them to get stuck in small blood vessels and trigger severe pain.

There are four subtypes of the disease, each with different levels of severity. While medications can help manage pain and other complications, national data indicates that the average lifespan for people with sickle cell disease is about 20 years shorter than the general population.

Lanzkron emphasized that early intervention is key to preventing long-term effects. However, she stated that these efforts fail if patients cannot see specialists with deep knowledge of the disease. She described local community hematologists as caring professionals who do not treat the condition daily. She stressed the need for annual evaluations to ensure patients can live their best lives.

The hub will also connect patients to emerging treatment options, including gene therapies and stem cell transplants. Lanzkron noted that while there are many options before curative therapy, patients require specific evaluation and management at specialized centers. She stated that this process cannot happen outside of these facilities.

Her team has been working with health organizations in the mid-Atlantic region to bridge gaps in care. One strategy involves increasing the use of telemedicine to reach people in rural areas. They are also investigating new ways to use technology to identify more patients and understand where they are seeking care.

Lanzkron said that if the team identifies a specific emergency room where many patients visit, they can reach out to that facility. The goal is to ensure those individuals receive proper referrals for specialized care.