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Thursday, January 30, 2025

Expert discusses new developments in sickle cell disease treatment

Dr. Shawn Jobe, a pediatrician with expertise in hematology and oncology, serves as the director of the MSU Health Care Center for Bleeding and Clotting Disorders and the Sickle Cell Disease Lifespan Clinic. He is also an associate professor at Michigan State University College of Human Medicine and part of the Cell and Molecular Biology program in the College of Natural Science. His research focuses on platelet disorders and unexplained bleeding disorders. In his clinical work, Dr. Jobe is dedicated to supporting individuals with sickle cell disease (SCD), particularly during their transition from pediatric to adult care.

Dr. Jobe explained that a common misperception about SCD involves its impact on blood cells: "With SCD, red blood cells and other blood components end up being 'stickier,' so they accumulate in the small vessels of almost every organ." This accumulation can cause blockages leading to mini-strokes, organ damage, and acute pain crises that often result in emergency department visits.

SCD primarily affects Black individuals in the U.S., along with some people of Middle Eastern descent. While there is no cure for this inherited blood disorder, recent advancements have been made. "In late 2023, the U.S. Food and Drug Administration approved two milestone gene therapies for SCD, Casgevy and Lyfgenia," stated Dr. Jobe. These therapies are designed for patients aged 12 years or older but remain economically challenging due to their high cost.

Until these therapies become more accessible, traditional treatments like Hydroxyurea continue to be used effectively in pediatric patients over 15 years old, alongside newer drugs such as Oxbryta for younger children.

Patients with SCD face significant healthcare challenges, notably having their pain taken seriously by medical professionals who may hesitate to prescribe opioids due to lack of visible symptoms. Dr. Jobe emphasized that "the Centers for Disease Control and Prevention specifically says that the general guidelines for prescribing opioids for pain is NOT applicable to management of pain related to SCD."

Transitioning from pediatric care also presents hurdles as young adults navigate a complex medical system independently after turning 18. To address this issue, MSU Health Care has established the Sickle Cell Disease Lifespan Clinic aimed at bridging care between pediatric and adult stages by providing comprehensive support from various specialists.

Dr. Jobe advises his patients not to let one gene define them: "You are defined by the other 29,999 genes and everything else that impacts you."

Photo credit: istock.com/Rujirat Boonyong

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