When I grow up

Helping chronically ill kids make the jump to adult care

Yana Vaks, MD, was about to begin a year of postdoctoral research at Stanford, studying the health of children with chronic medical conditions after they become too old for pediatric care, when she saw the worst of what can happen.

“There was an 18-year-old who came to the hospital in crisis,” she says. “He had a liver transplant when he was 8, but when he turned 18 he wanted to start a new life and decided he was done with all that extra health consciousness his transplant meant.” The patient had stopped taking the drugs necessary to keep his body from rejecting the transplant and neglected to see his doctor regularly. By the time Vaks saw him, his transplanted liver had begun to fail, starting a catastrophic process that affects all body systems. “It was a shocking case,” she says.

The teenager died the next day.

“His mother said the biggest change had been that because he was 18, she was no longer in control of what medications he took and what he didn’t take,” Vaks says. “Unless there’s a plan for letting go, it can be disastrous.”

Right now, there is no standard road map for doctors and hospitals to help children with chronic illness transition from pediatric to adult care. And the need for a plan is increasing. More and more children are living beyond the bounds of pediatric care, simply because medicine has improved. Many of the specialists who have treated them recognize that their transition from childhood to adulthood is risky: Young adults may be grown in body, but they aren’t always ready psychologically or socially to take full responsibility for consistently following complicated medical routines and practicing lifestyle restrictions. “You can’t just expect patients leaving pediatric care to be an adult patient when they haven’t developed any skills,” says Lynn Kjelson, MPH, a kidney transplant coordinator at Stanford Children’s Health. This recognition is translating into a call to action from medicine’s leaders for specialized training, certification and research into best practices — including a recent project at Stanford’s Clinical Excellence Research Center.

“A problem like this has suffered from a lack of ownership,” says Dennis Lund, MD, chief medical officer at Stanford Children’s Health. “Is it the pediatrician’s problem if there’s a 25-year-old having difficulty finding the right care or that of the adult patient’s doctor with no expertise in pediatric diseases? When you reach adulthood, you don’t want to go to a children’s hospital and sit in an exam room with Pocahontas on the wall. It’s become a national issue.”

A handful of care providers, including several teams at Stanford Children’s Health, have formal transition programs in place. But most do not. A 2011 joint report in Pediatrics from the American Academy of Pediatrics, the American Academy of Family Physicians and the American College of Physicians called for a systematic approach to the problem. The recent project at Stanford’s Clinical Excellence Research Center makes specific recommendations for pediatricians, pediatric specialists, patients and parents to prepare for the jump to adult care.

Some medical conditions, including cystic fibrosis, diabetes, rheumatoid arthritis, movement disorders and transplantation, require lifelong, daily maintenance and ongoing specialized medical treatment. Others, like congenital heart disease, require lifelong but not daily care, and physicians with knowledge particular to the condition — including in adulthood. Doctors have recently begun to call for special attention to transition care for survivors of childhood cancer, who must learn to live with the long-term effects of the treatment that saved their lives, including higher risk of infertility; early-onset bone weakness; chronic problems with heart, liver, kidney and hormone function; impairments in sexual function; and development of other cancers. Doctors are also beginning to think about how to help patients with mental illnesses that emerge in childhood and change with age.

The Clinical Excellence Research Center, established in 2010 to study, design and demonstrate ways to improve health care while reducing costs, identified transition care as a good candidate for the changes it hopes to effect with its work. “We’re trying to figure out how to do the right thing and have it be affordable,” says Dana Steidtmann, PhD, a co-leader of CERC’s two-year project on transition care. Young adults with chronic illness may soon constitute 10 percent of the U.S. population ages 15 to 25, she says, compared with 1 percent before advances in treatment.

To understand the problem, the CERC team did a thorough review of scientific articles; interviewed adolescent patients, their parents and clinicians throughout the United States; reviewed advances in information and communications technology; and visited several hospitals around the country to observe successful transition programs for all major chronic diseases before constructing its recommendations.

Sue Fernandes, LPD, a clinical associate professor of pediatric cardiology and the program director of the Adult Congenital Heart Program at Stanford Children’s Health and Stanford Health Care, became one of the team’s advisers for the project. Previously, Fernandes had conducted research that identified gaps in the transition process. Among her findings: More than half of parents reported their child’s pediatric cardiology team had never mentioned the need for lifelong care.

Then there are the adolescents themselves. Being responsible for making medical appointments, renewing medications, negotiating insurance coverage and learning how to take an active part in health-care decisions — these tasks may become second nature with age, but many young adults don’t want or know how to do them, especially at the intense and ongoing level certain chronic illnesses demand. “Young adults do not want to have to think about their health every day,” says Steidtmann. “They have a strong desire to feel normal.”

Their parents, meanwhile, aren’t often prepared to change doctors or cede control. “As the parent of a child with a serious chronic illness, you are a warrior who has gone through years of learning the ins and outs of your child’s condition,” says Elizabeth Malcolm, MD, director of implementation and evaluation at CERC. “By the time your child graduates from high school, you think you have it all wired and it’s incredibly scary to think about your child moving to a new doctor or to a new place.”

The CERC team’s recommendations emphasize that pediatricians and pediatric specialty teams must be guides in this process: equipping patients and parents with information so they can anticipate the transition, coaching patients to develop the confidence and skills needed to manage their health, and locating and being available to specialists and primary care physicians who will need certain medical knowledge to care for their patients as adults.

The recommendations provide a structure to organize transition care for any condition. They suggest using a checklist to tailor plans; identifying a transition team leader to oversee the process and a transition guide to ensure each step is carried out; and using health coaches, telemedicine and other technologies like cellphones with health-tracking apps to make the transition steps as feasible as possible for patients, families and doctors. The team calculated that if these transition programs were widely implanted, they could avoid preventable disability, hospital visits and other medical expenses totaling $200 million a year.

Pioneering programs

Stanford Children’s Health has pioneered transition programs for patients, parents and pediatric physicians. As soon as children go on the kidney-transplant list, for example, post-transplant coordinators begin working with them and their parents. “We start our teaching early so it will be part of their life,” says Kjelson, the physician assistant who coordinates the team. “We do that to establish rapport and to build familiarity with the transition process.” After a post-transplant patient turns 12, the clinical team begins split appointments, seeing the patient and parents separately. “We do that so patients can begin to recognize their voice as a patient,” she says.

The team pays attention to the patient’s psychological and social needs. In 2013, the program asked its adolescent patients who had experienced a transplant rejection or loss what they had most struggled with. Those struggles typically were not medical, Kjelson says. They were more often linked to poor preparedness for the challenges of adult life, including powerful emotions.

“The biggest fears are usually about working with a new medical team and navigating a new medical system — and feeling overwhelmed by that,” says Sarah Kinnier, a pediatric social worker who leads separate groups for teen transplant patients and their parents.

To determine how well patients are learning the techniques they’ll need, the kidney transplant team uses readiness-assessment questionnaires. One form focuses on skills. “Do you know what to do if you are having a bad reaction to your medicine?” “Do you make a list of questions before the doctor’s visit?” The other tracks attitudes. Does a patient want others to notice him or her taking medicine? Does a patient have a hard time sticking to a fixed medication schedule?

The transition plan also includes a 10-step checklist that covers matters such as transferring medical history and ensuring patients and parents understand how insurance coverage can change in adulthood. The final step: a goodbye visit at Stanford Children’s before the patient is accompanied to his or her first adult appointment by someone from the pediatric team.

‘Young adults do not want to have to think about their health every day. They have a strong desire to feel normal.’

Linden Moore was born with a congenital heart defect and has had six surgeries. Her first was 11 days after her birth. Her last was quite recent — after her 18th birthday — so she had to sign her own consent form for the first time. It was a symbolic moment. “My parents and I were going, ‘Oh, my God!’ she says. “It was kind of surreal — that I’ve grown up with this and now it’s in my hands.”

Linden’s latest care transition is more complicated than moving from Stanford Children’s Health to Stanford Health Care: She began college in Oregon this fall. “You have a twinge of worry,” says her mother, Mary Moore. “No mom ages out of that. And you have to make sure that your child understands, as they take the reins, that they have certain rights. You have to school them on how to find information, to tap into resources and to ask questions.”

“In health matters, most parents do everything and children do what they’re told,” says Christy Sillman, the nurse coordinator for Stanford’s Adult Congenital Heart Program. “The child isn’t expected to advocate.” To help young adults develop those self-care and advocacy skills, Stanford Children’s Health has organized an Adolescent and Young Adult Heart Clinic. There, the care team understands patients’ concerns — even those that might seem small to others. “We know that life expectancy for most adult congenital heart survivors is fairly normal, but the life experience is not normal,” Sillman says. “While all their friends are going out for pizza, they’re restricted to a low-sodium diet.”

At Stanford’s Bass Center for Childhood Cancer and Blood Diseases, co-director Sheri Spunt, MD, oversees a Health After Therapy Program designed to ease patients’ transition from cancer treatment to survivorship. Her team equips its patients with a notebook containing their entire health history and a treatment summary, as well as personally tailored care guidelines for adult survivors of childhood cancer. The guidelines come from the Children’s Oncology Group, an international organization of 9,000 experts in childhood and adolescent cancer and a National Cancer Institute clinical trials research group. “Many health-care practitioners are unaware that these guidelines exist,” says Spunt, the Endowed Professor of Pediatric Cancer. “With about one in 650 young adults who’ve become cancer survivors, doctors need to be educated about this. People think the cure is the victory, but cure is not sufficient; you need cure with long-term care that supports quality of life.”

Piloting the plan

After analyzing the state of transition care, the CERC team has launched pilot programs to test its recommendations. A pilot at Stanford Children’s Health focuses on the effectiveness of a health coach in strengthening the health management skills of chronically ill young adults, specially crafted to the feelings that can drive neglect of medical needs. “We coach from a different perspective than a medical team would,” says health coach Jacqueline Nguyen, a former CERC research assistant. “We say, ‘We know it’s hard for you to be responsible for your care, but let’s think about all the reasons why it might be important for you. Instead of saying, ‘If you don’t take your meds. … ,’ we say, ‘If you take your meds, you’re going to feel better and do better in school and go away to college.’”

The CERC team’s recommendations will be piloted on a larger scale in the diabetes clinics of Intermountain Healthcare, a Salt Lake City-based system of 22 hospitals. The region has a high and increasing rate of diabetes, says Carolyn Reynolds, the registered nurse who directs Intermountain’s Pediatric Specialties Clinical Program. The 250 patients in the pediatric diabetes program will be given an experienced care-management clinician, a transition guide and a health coach to support them as they take over their own care.

​Intermountain’s multi-hospital system could eventually ​serve as a testing ground for the ability of the Stanford plan to be adapted to various medical specialties. “Everyone we’ve talked with has thought this program is needed,” Reynolds says. “When it was presented to our Parent Advisory Council, they said, ‘Let’s do it today.’”

When transition works

Jon Michaiel, 23, was born with kidney failure and received his first kidney transplant when he was 11. As he approached his 18th birthday, he needed a second one — which meant he stayed with his Stanford Children’s Health doctors for another three years. As comfortable as he felt with them, he wanted to be more independent. “I didn’t have the autonomy to make decisions and my parents were with me in appointments, which wasn’t very cool for someone my age,” he says. “I was ready to go to the adult side, but I was apprehensive about meeting a whole new care team.”

Once he made the transfer, he felt the difference immediately. “The pediatric team is very hawk-eyed with its patients,” he says. “On the adult side, there’s no social worker or liaison to remind you if you’re late on your meds. There is a lot more you have to think about.”

A transplant “is a solution, not a fix,” Michaiel says. “It’s something you need to deal with and manage, just like people manage jobs responsibly. You do have to grow up a lot faster than most people do.” When the rigors of maintaining his health become challenging, he reminds himself of the dedication of his family and his pediatric team. “It took a lot to get me here, so I don’t want to mess up,” he says. “I am one of the lucky ones.”

Sara Wykes is a writer for the Stanford Hospital & Clinics communications office. Email her at swykes@stanfordhealthcare.org.

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