Shortening the gap between pediatric and adult care improves sickle cell outcomes

Caring for a chronic disease is a marathon, not a sprint. Typically, these conditions are managed but never fully resolved. For sickle cell disease, which is diagnosed at birth, care is a marathon. Growing up has a major impact on patients as treatment must continue throughout the peaks and valleys of life. One critical crossroad faced by individuals with sickle cell is the transition from pediatric to adult care. The consequences of not transitioning quickly enough to adult care were recently documented by St. Jude scientists in Blood Advances.

“I observed patients in the clinic on the adult side, who arrived with chronic pain and were developing organ dysfunction,” said senior author Jane Hankins, MD, St. Jude Department of Global Pediatric Medicine member and Global Hematology transversal program director. “Those patients with an interruption between pediatric and adult care did not fare well. But no one had truly documented it.”

Once the researchers began looking, the clinical observation was reinforced. In a group of 183 patients with sickle cell disease followed across six years, those who had more than six months between their last pediatric and first adult appointments experienced worse outcomes. This group had more emergency department and inpatient visits and fewer outpatient visits than those who transitioned to adult care within six months.

“Now institutions can fight for resources because they can show that it’s bad to go too long without seeing your doctor,” Hankins said. “Sometimes you must document the obvious to get resources and change policies. We’ve given the data to the field; now they can use it as an argument to fight for the right course of action.”

Current guidelines are necessary but not sufficient

That six-month period is derived from current guidelines, which recommend that patients transition to adult care within six months. The research confirms the importance of transitioning within six months, but it also went further. 

“I questioned if maybe six months was too long,” Hankins said. “Maybe the interval should be one month, or two or three. So, we looked at people who took two, three, six and more than six months to transition to adult care. And then we showed a very direct relationship, with the longer you go without seeing a doctor, as you leave pediatric care and begin adult care, the more emergency department visits and admissions to the hospital you have.”

The group with the best outcomes were patients who transitioned within the first two months after pediatric care ended. Outcomes worsened the longer the gap between pediatric and adult care persisted, with a three-month gap leading to more hospital visits than the two-month gap and worsening in the six- and greater-than-six-month groups. Therefore, the evidence also suggests that a shorter transition period than currently recommended in care guidelines may improve outcomes.

“We documented that, yes, a gap of more than six months is bad, but we also found that a gap of more than two months is bad too,” Hankins said. “So, our conclusion was, definitely don’t wait more than six months to transition to adult care, but also try to go within the first two months.”

Patients with longer gaps in care appear to do worse for several reasons. For example, whatever prescriptions may have lapsed for greater periods. In addition, minor issues that could be addressed early are instead allowed to worsen. Patients are then forced to seek acute or emergency care as crises arise, contributing to extended hospital stays. Successfully transitioning to adult care can significantly reduce these issues.

Setting up structures for sickle cell disease care success

While the study provides strong evidence to support helping patients transition from pediatric to adult care quickly, it cannot address the major barriers to that transition. Some are unique challenges, such as the patients’ age and maturity at this point in their lives (18 to 20 years old), which present their own unique challenges when it comes to navigating health care. Adding to that load are structural differences in pediatric and adult care, such as the shift from careful guidance in pediatrics to patient-led care as adults. The key to a smooth transition is to provide patients with knowledge, resources and direct help.

“The problem seems to be a lack of structure,” Hankins said. “That’s what good transition programs do. They put structure in place.” 
 
Due to observant physicians, such as Hankins, St. Jude and the local Regional One Health and Methodist University Hospital have a transition program for patients with sickle cell disease. A transition navigator, a position created for such purpose, helps patients and families transition from pediatric to adult care, minimizing the patient’s burden and facilitating a timely process. The program currently boasts an average transition time of less than 30 days, well within the optimal two-month window identified by the study. That example shows the potential promise of taking a structured approach to helping newly minted adults with sickle cell disease transition to adult care quickly.

“We do all these beautiful things in pediatrics, and we want to see our kids succeed as adults,” Hankins said. “We want our kids to have a long and good life. We want to see kids grow up and graduate high school, go to college and pursue the careers and families that they want. Helping them transition to care early is one way to do that.”