Sickle Cell Disease: Transitioning Patients From Pediatric to Adult Care

How to combat the rise in mortality when patients become adults

Thanks to newborn screening, preventive antibiotics and updated immunization practices, pediatric deaths from sickle cell disease have dropped significantly over the past 35 years. Almost all children with the disease now survive to adulthood. But transitioning from pediatric to adult care remains a challenge for both patients and providers, contributing to a rise in mortality once patients enter their 20s and 30s.

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Teenage sickle cell patients struggle with the same challenges as other adolescents, such as increasing risk-taking, learning life skills and developing executive function, says Crawford Strunk, MD, a pediatric hematologist-oncologist at Cleveland Clinic. Taking on responsibility for managing their own care and medical decision-making at a time when their health remains fragile can be perilous.

“Any adolescent with a chronic disorder has issues with transitioning to adult care,” says Dr. Strunk. “They may think ‘I want to be independent; I want to be normal; I don’t want to worry about this anymore.’”

Compounding those concerns, many young adults with sickle cell disease also have neurocognitive impairment due to complications of the disease.

“That makes it even more challenging for these patients to get the care they need,” he says.

Start transitions early

Best practices for providers include starting the transition to adult care as early as age 12 to 14 so the patient has years to learn skills like making appointments, navigating visits with their providers and following their plan of care.

“We don’t want them, all of a sudden at age 18, to be pushed to start going to appointments on their own,” says Dr. Strunk. “It should be a gradual process.”

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Providers should follow transition guidelines like the toolkit created by the American Society of Hematology, which outlines a series of steps for making the shift, he adds. Steps include creating a transition policy, monitoring and tracking progress, assessing the patient’s readiness, and creating a plan for transition to adult care.

“Even after the patient’s first meeting with their adult provider, there should be a crossover period where the new and old providers maintain open communication and ensure the patient’s needs are being met,” he says.

Making transitions seamless

Cleveland Clinic has taken steps to make the transition of care seamless with an approach involving both pediatric and adult physicians, including pulmonologists, nephrologists, ophthalmologists and cardiologists with expertise in sickle cell disease. The team meets regularly to coordinate each patient’s care.

“We are a life-span clinic, which means we take care of patients from birth through adulthood, so patients don’t have to worry about transitioning from pediatric to adult practice,” says Dr. Strunk. “However, they still have to learn all the basics about how to be an adult, how to take care of themselves, and how to engage in medical decision-making.”

These steps are facilitated by two dedicated care coordinators and two social workers, who support patients with the transition and help them learn key skills for managing their care.

Disease progression causes organ damage

While transition challenges are one reason mortality increases in early adulthood, disease progression also contributes to deaths. Dr. Strunk notes that, while the most visible symptom of sickle cell in children is pain, these patients also experience ongoing damage to their kidneys, lungs, heart, brain and other organs.

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“We know that mortality peaks in the third decade of life,” he says. “Some of that is related to sudden death, but some is related to organ damage that has been developing all along, becoming more apparent in adulthood.”

Dr. Strunk notes that after nearly two decades without new treatment options for sickle cell disease, new medications are now available, with more therapies on the horizon. The most recent treatments, two gene therapies approved for use in patients in late 2023, are available for patients age 12 and older.

“They promise to be transformative for many patients,” he says. “They’re some of the most exciting new therapies to come along.”

Additional research is underway on treatments to improve the lives of people with sickle cell disease, with Cleveland Clinic physicians participating in studies. In addition to research, Cleveland Clinic providers are working to address a shortage of hematologists who care for adults with sickle cell disease.

“We’re working to build on the hub-and-spoke model of caring for patients by participating in larger networks to help standardize practice and education,” he says. “We want care across the country to be the same no matter where you are.”