For people with bleeding disorders, the transition from pediatric to adult health care is somewhat like the broader transition from child to grown-up.
The more time and attention that’s devoted to preparing for the second stage while still in the first, the easier the shift is likely to be. But regardless of how well prepared you are, the change — when it comes — will probably be unsettling at times.
You’re dealing with unfamiliar people, you’re asked for information that you may not have even realized you needed, and you discover that the safety net protecting you from the consequences of your own mistakes seems woven quite a bit less tightly than it used to be.
“The majority of youth are unprepared for the change in care,” warns the American Society of Hematology, the world’s largest professional organization for health care providers specializing in diseases that affect blood, bone marrow, and lymphatic systems. “They may not know their medical history, prescriptions, insurance information, or even how to make a doctor’s appointment.”
Further complicating things is a transition process that varies by region and health care system. Some hemophilia treatment centers allow patients to remain in pediatric care until age 21; others move them to adult care at age 18.
And in either case, challenges outside anyone’s control can disrupt the process.
‘I Don’t Know What to Do’
For Kelly Wornall and her son, who was diagnosed with von Willebrand disease at age 12 and moved into adult care at age 18 — which happened to be in 2020, during the COVID-19 pandemic — the shift was particularly daunting.
Their longtime pediatric specialist had retired the year before, and her son had just one appointment with a new pediatric provider in the Kansas City area before pandemic restrictions took effect and he turned 18.
“It was utter chaos,” Wornall recalls. “During the whole diagnosis process, we saw social workers and there were so many people helping us, and then it wasn’t there after the transition into adulthood. Especially since everything had shut down, it would have been nice to transition very slowly.”
Instead, her son found himself responsible for navigating his own treatment needs, partly because of federal health privacy laws that limited Wornall’s ability to act on his behalf after he reached adulthood.
“He was saying, ‘Mom, I don’t know what to do. Help,’” she recalls.
Bleeding disorders treatment providers and advocates are familiar with the complications that such confusion can cause and have been working for at least a decade to make the move into adult care easier, in part by developing detailed written guidelines.
“In pediatric care, we really wrap our arms around patients and families and invest in helping them navigate barriers, from family dynamics to understanding and being able to navigate the health care system,” says Betsy Boegeman, hematology and oncology clinical social worker at the Children’s Minnesota Center for Bleeding and Clotting Disorders in Minneapolis. “While adult care centers provide great care, too, the expectations around independence, autonomy, and responsibility are different.”
Preparing for Independence — from the Beginning
Children’s Minnesota worked hand in hand with the area’s adult hematology center to develop a joint transition policy and procedures to shepherd patients through the transition, she says.
“We have guidelines about how to talk about transitioning to independence basically from birth,” Boegeman explains. “Those conversations can start with teaching kids about their bleeding disorders and how to talk about them with friends, and maybe someday their employers.”
When children with bleeding disorders reach age 14, the team at Children’s Minnesota completes a transition readiness assessment to gauge where patients are and what they still need to learn, she says.
Health care providers and families use the results to fill in any gaps before adolescents move to adult care, which occurs between ages 18 and 21.
“I always recommend that young adults listen to their parents or guardians as they schedule appointments or order medication, then try doing it together, and then try it on their own,” Boegeman adds. “It’s a good approach for all sorts of things, from doing your own infusions to coming to a visit on your own.”
She often refers to Steps for Living, an education program developed by the National Hemophilia Foundation that offers skills development guidelines based on age groups from birth through adulthood.
The section for 16- to 25-year-olds includes a component on transitioning to independence that covers topics such as emergency preparedness and taking charge of personal health care.
“For young adults and adolescents who grow up with chronic medical conditions, not just bleeding disorders, there comes a point when the care has to become more of their responsibility,” says Lucy Ramirez, a clinical social worker for the Rush Hemophilia and Thrombophilia Center in Chicago.
While many parents realize the process should be gradual, they often lose valuable time to the demands of everyday responsibilities such as holding jobs and managing children’s school and extracurricular activities.
Show and Tell vs. Do-It-Yourself
“You can forget to look at the big picture and think, ‘I have to start teaching them how to do some of these things that I’ve been doing for them,’” Ramirez says. “It’s much easier for the parents to just pick up the phone and call the treatment center themselves or keep inventory of a child’s medications than to take the time to show the child how to do it.”
She says awareness of the challenges of transitioning to adult care for chronic medical conditions has been growing for years.
“The issue was that patients were dropping out of treatment once they became adults, and they were often lost to follow-up for years and years until they actually had a problem,” she explains. “By then, they could face serious consequences from not managing their diabetes or cardiac issues or taking the medications that they needed in order to keep themselves in good health.”
Because Rush provides lifetime hemophilia treatment, patients don’t have to switch to a new center at a certain age unless they go away to college or move from the area, Ramirez says. While they still move to adult care, the comparative consistency allows practitioners to focus largely on teaching the practical skills patients need to take care of themselves.
To assess progress, Rush uses a transition checklist that rates the following capabilities:
- Naming your bleeding disorder and identifying the severity or level
- Describing your bleeding disorder in your own words
- Naming the type of product used to treat your bleeding disorder
- Memorizing your factor dose
- Mixing factor and infusing yourself
- Recognizing a bleeding episode and knowing how to respond
- Knowing whom to call and the number to call for questions about bleeding
- Recognizing the symptoms of an active joint bleed and how to treat it
- Ordering your own factor or other medications
- Keeping a bleeding log
- Scheduling your next clinic appointment
- Naming your health insurance provider and knowing the customer service number
“We start the education process at a fairly young age,” Ramirez says, “encouraging them to become comfortable interacting with our staff, asking our staff questions, and then eventually learning how to do what they have to do in order to infuse themselves and make good decisions about their health care.”