Young woman with sickle cell disease starts over

In the 1970s, the average life span for a person with sickle cell disease was 14 years. Now, many people, including Jalen Matthews, are living long and full lives with the congenital blood disorder.

Jalen Matthews is embarking on a new journey in life. A University of Kentucky graduate, she is living on her own for the first time in a new city. She is pursuing a master’s degree in exercise science at the University of Indianapolis, with a focus on a career in physical therapy. She’s also now managing her sickle cell disease as an adult patient thanks to the care she received through Norton Children’s Cancer Institute, affiliated with the UofL School of Medicine, and Norton Cancer Institute.

In the 1970s, the average life span for a person with sickle cell disease was 14 years. Now, many people are living a long and full life with the congenital blood disorder, but finding ongoing follow-up care can be a concern for some of these adults.

Sickle cell disease has had an impact on Jalen’s life. In 2009, she had a blood clot on her spine that led to a stroke that caused paralysis on her left side. She was hospitalized for 35 days.

“The stroke is what made me want to be a physical therapist,” Jalen said. “I saw how exercise helped me and other people I was in therapy with. I worked with exercise physiologists, and I really thought it was cool how exercise helped people who didn’t experience paralysis. It made a big impact, and it made me want to help others recover.”

People with sickle cell disease have red blood cells that are curved, or sickle-shaped, and cannot bend. They are more likely to stick to vessel walls, which can slow or stop vital blood flow.

AThe lack of oxygen can cause a sudden, severe pain attack called a “crisis.” Most children with sickle cell disease are pain-free between crises, but adolescents and adults may experience chronic pain, according to the National Heart, Lung and Blood Institute.

Transitioning from pediatric to adult sickle cell disease care

Since her diagnosis as a baby, Jalen has received red blood cell exchange treatments at Norton Children’s Infusion Center. She’s been cared for by Ashok B. Raj, M.D., pediatric hematology/oncology specialist with Norton Children’s Cancer Institute. Apheresis is a treatment which removes the sickle cells in the blood and replaces them with normally shaped red blood cells. In 2017, as a young adult, it was time for Jalen to graduate to a new phase of care. Through the Norton Children’s Sickle Cell Disease Program, she graduated to adult care under Shawn D. Glisson, M.D., hematologist and sickle cell disease specialist with Norton Cancer Institute.

This comprehensive transition program gives children with the condition a structured, education-based curriculum starting at 12 years of age. It’s designed to prepare patients for management of their own health care needs into young adulthood and an eventual change to care with adult providers. Our approach for transition includes the patient; pediatric and adult providers; and members of the Sickle Cell Association of Kentuckiana, a community-based organization; to help encourage self-management and independence and other positive ways to prepare adolescents for transition.

These transition efforts are designed to help patients have lifelong well-being. The approach can help tackle health care disparities by providing support; promote independent knowledge and skills in young adults; and help set expectations for an adult model of care. The program provides interventions that help ensure sickle cell patients have uninterrupted care when they’re ready for adult providers.

“Our hope is that our transition program gives young adults with sickle cell disease the resources, knowledge and support so they can overcome challenges and stay healthy during transition and beyond,” Dr. Raj said.

With Dr. Glisson and Norton Cancer Institute, Jalen has had great outcomes. She has been able to extend the time she goes between apheresis or red blood cell exchange treatment. Jalen goes for treatment every seven weeks. These five- to six-hour transfusions rely on blood donations, which have been affected by the COVID-19 pandemic.

“Due to the national blood shortage, it looked like I was going to have to miss a treatment,” Jalen said. “However, luckily last-minute it came through and I was able to get the exchange. But it’s scary to know that with people donating blood less, it could mean I might not be able to get my exchange due to COVID-19. Blood donation is critical not only for people who have emergencies, but for people with blood disorders as well.”

Focus on the future

Given that Jalen experienced a childhood filled with countless doctor visits, transfusions and regular assistance from her parents, starting over in a new city is both scary and thrilling.

Norton Children’s Sickle Cell Disease Program

(502) 588-3600

As she adapts to her new truly independent life, she will begin transitioning her care again — this time with providers in her new city. While being treated by Norton Cancer Institute, she still got to see many of the nurses who have provided her care over the past two decades through the Norton Children’s Infusion Center. During her treatments, nurses and staff would stop by her room to check on her and say hello.

“It will be different,” Jalen said. “I’ve known Dr. Raj since I was a baby. I called Dr. Glisson just this week to ask a question. It’s scary to be on your own, but it’s exciting, too. I know what I need to do to keep myself healthy, and I learned it from them.”