Children with sickle cell disease (SCD) now live longer and experience a much better quality of life than ever before.
“Children with SCD living in high-income countries no longer have a life-threatening disease, but rather a chronic disease with disease-associated, life-threatening events,” wrote Michael DeBaun, MD, MPH, of Vanderbilt University Medical Center in Nashville, in a column in The Hematologist.
Consequently, much attention has been placed on how to transition these children to adult care, but that process has not been without its challenges.
For example, in a 2013 study, researchers found that patients who were transitioning to adult care (age 18) had fewer transfusions, took less hydroxyurea, less iron chelation therapy when eligible for it, had higher healthcare costs, and suffered from more frequent SCD-related complications than pediatric patients.
DeBaun and Joseph Telfair, DrPH, MPH, MSW, of Georgia Southern University in Statesboro, pointed out in a Pediatrics article on transition and SCD, that a number of “unique features” characterize the experience of adolescents who are ready to transition from pediatric management of their disease. These include the fact that:
- A high proportion of SCD adolescents receive public health insurance when compared with any other pediatric genetic or chronic disease.
- A high proportion of these patients experience overt strokes or silent cerebral infarcts affecting cognition.
- They have a high rate of comorbid disease, such as asthma.
“Young adults are living longer,” wrote DeBaun and Telfair. “Consequently, the importance of transitioning from a pediatric primary care provider to adult primary care physician has become a critical step in the health care management plan.”
And the consequences of failure when it comes to transition can be dire.
“When you look at patients who fail to transition well, we think they are substantially at greater risk of complications of their disease when they don’t have a coordinated process that takes them from pediatric to adult system of care,” said John J. Strouse, MD, PhD, of Duke University School of Medicine in Durham, North Carolina. “And we all worry about patients who are getting intense services as pediatric patients, and then fail to connect with adult providers, and those services stop — patients with chronic transfusions who just don’t show up for their first adult visit or first adult transfusion, and end up in the hospital. “
In another study, Joseph F. McLaughlin, MD, and Samir K. Ballas, MD, both of Thomas Jefferson University in Philadelphia, reported on the outcome of 22 patients who transitioned from pediatric care to an adult clinic over a 16-year period. They found that while patients who were compliant with blood transfusion were rarely hospitalized, eight patients who refused to be maintained on chronic blood transfusion died within 1 to 5 years after transition.
Transition represents a high bar for patients and their families to hurdle, Strouse said. “We want them to be prepared to navigate the adult healthcare system, which is very different from the pediatric healthcare system, while they are still in adolescence.”
Sophie Lanzkron, MD, of the Sickle Cell Center for Adults at Johns Hopkins Hospital in Baltimore, pointed out that one issue complicating transition is that there is often no place to transition children to. “Certainly, in parts of this country, that is absolutely true,” she said.
In 2011. researchers conducted a survey of pediatric sickle cell clinics and found that only 60% of pediatric sickle cell clinics routinely transferred their patients to a hematologist specializing in SCD.
And, many primary care providers (PCP) are simply not that familiar with the disease or comfortable managing it.
In 2015, when researchers surveyed community physicians to determine their level of comfort in providing care to patients with SCD, they found that the majority of respondents lacked confidence in basically all aspects of SCD, including providing ambulatory care to patients with SCD, managing their comorbidities, managing SCD-specific issues, and managing chronic pain.
One of their conclusions was that it is essential that continuing medical education on SCD be available so that providers stay up-to-date on the disease.
“It’s a rare disease,” she said. “If you are a PCP and you have 100 people with diabetes, and one person with SCD, and you want to improve your knowledge base, you are going to improve your knowledge base for the 100 and not the one. And that’s not necessarily inappropriate.”
“So, we need to start thinking of different ways of approaching this problem rather than teach all PCPs how to do this, because I think that’s impractical and just not possible,” she added.
How should transition work?
DeBaun and Telfair recommended that transition “should be a gradual process with a flexible timeline of goals in partnership with the patient, family, and future primary care provider.”
Jeffrey Lebensburger, MD, of the University of Alabama in Birmingham, and colleagues suggested in the International Journal of Hematology and Therapy that transition planning “should occur years prior to transferring a patient to an adult center” and employ strategies that not only teach patients and their families about the disease, but should focus on issues related to employment, insurance, personal relationships, and adult physicians.
ASH has also designated this issue as a priority and has developed a toolkit to help hematologists transition their patients from pediatric to adult care.