Leaving the Pediatric Cocoon

How Teams in NICHQ’s WISCH Project Are Helping Prepare Sickle Cell Patients for Adult Care

July 12, 2013
By Cindy Hutter

Transition Curriculum for Sickle Cell Disease
WISCH teams created the Transition Curriculum for Sickle Cell Disease to fill the void where national guidelines for healthcare providers are lacking.
Transitioning from pediatric to adult care for patients with sickle cell disease is a matter of life and death—literally. With data showing there is an increase in mortality during the time individuals with sickle cell disease transition into the adult world1,2, teams on NICHQ’s Working to Improve Sickle Cell Healthcare (WISCH) program are working together to address various aspects of sickle cell healthcare, in particular care transition and patient advocacy.

Improvements in treatment for sickle cell disease have led to longer life expectancies, but doctors trained in treating adults with the disorder lag behind. The genetic blood disorder causes blood cells to change shape, which can cause intense pain and eventually tissue damage or organ failure. Patients need coordinated medical care to manage the disease, but many patients lose this once they become adults for a variety of reasons.

“Young people are needlessly dying,” says Marsha Treadwell, PhD, clinical scientist and project director of the Northern California Network of Care for Sickle Cell Disease at Children’s Hospital & Research Center Oakland. “When individuals with sickle cell disease are in their early 20s, there is a peak of emergency department visits and mortality. Whether it is because of the disease, the failure of the system in transitioning people successfully or a combination doesn’t matter. There is a real urgency to improve that process.”

A handful of WISCH teams are piloting tools, including the recently developed Transition Curriculum for Sickle Cell Disease (see right), to fill the void where national guidelines for healthcare providers are lacking. The transition curriculum is the first set of sickle cell disease-specific guidelines to be created and implemented, and assists pediatric providers in preparing their patients with sickle cell disease, ages 12-25, to move to adult providers. The transition curriculum, along with an adapted version of the more general Got Transition Youth Readiness Checklist, aims to empower young adults to take an active role in managing their disease by developing skills in the medical, social and academic domains.

Michael Regier, clinical care coordinator of the Colorado Sickle Cell Treatment and Research Center, says it’s not uncommon for children to be raised in the “warm, caring cocoon” of the pediatric setting where so much is done for the patient and the family. It then comes as a shock when patients go to an adult system and realize all the rules have changed. Patients become responsible for scheduling appointments; no one necessarily follows up if appointments are skipped, unlike with pediatric patients.

The recently developed Transition Curriculum for Sickle Cell Disease aims to help patients at the younger ages become active participants in their care by encouraging them to learn how to explain the type of sickle cell they have, know what medications they are on, and recognize which symptoms need quick medical attention. Providers are also recommended to encourage patients to carry a health insurance card, know their medical coverage, and learn to book their own doctor’s appointments and track prescription refill expiration dates. As patients get closer to adulthood, topics of sexuality, adult care options and disease complications—such as chronic organ dysfunction—are introduced.

Andrea Williams, founder and executive director of Children’s Sickle Cell Foundation in Pittsburgh, incorporates other life lessons into the foundation’s transition program. Not only are participants introduced to a primary care physician and given tours of the local federally qualified health center, patients also attend sessions on meal planning, shopping on a budget and receive CPR certification.

“Medical transition from pediatric to adult care is only one aspect of life, but an important part,” says Williams. “We believe you are a person first and then a person with sickle cell disease. Everything you need to know as a transitioning young adult should be part of the program so that you become a productive citizen. We incorporate everything from understanding health literacy and insurance issues to enrolling in college or finding employment.”

Some of the challenges teams are facing are no different than ones that are experienced with a typical teenager making a care transition. At this stage in life teenagers tend to take risks and push boundaries. Getting across that some risks taken with sickle cell disease could be very disastrous is not often easy.

“Part of successful transition care is getting patients to understand that coming to clinic every three months or six months wasn’t because we didn’t think you had nothing to do, it was to monitor health issues that might not be apparent on the outside,” says Regier. “It has to be an internalized thing, not just ‘my social worker told me to do it.’ We can spend a lot of time teaching skills and giving facts, but the patient still has to make the choice to do it, to understand why it is important. That’s the tricky part.”

To address the challenge, the focus has moved from a single point of transition to a view of long-term preparation, which is similar to what some of NICHQ’s projects working on medical homes are doing. The WISCH teams are finding the adapted Got Transition Youth Readiness Checklist tool to be useful in addressing transition readiness.

“It’s important for systems to not arbitrarily transition based on age,” says Treadwell. “Staff need to start earlier and assess how ready someone is in various areas, such as general life skills and self-care. To be successful, the patient really has to be ready for the process. Families are also important. You have to make sure that parents are on board and are prepared for their young person to transition.”

The efforts are working, according to Jane Hankins, MD, associate faculty at St. Jude Children’s Research Hospital and co-leader of the WISCH group focused on improving transitions. Hankins’ most recent success was with a 19-year-old patient that she describes as having very bad sickle cell disease with lots of complications. Adding to that, Hankins says she has a very disinterested family and she has always been very angry at her disease, and has transferred a lot of the anger toward her medical team. At 18, the medical team—using the assessment tool—decided the patient wasn’t ready for transition and the team devised an individualized transition plan for her with time-bound goals. The team worked closely with her for a year. At the end of the year, she said she was ready to go.

“I had a fear that she was saying that to please us,” says Hankins. “She came back for a celebration graduation this past Thursday [six months since we last saw her] and she looked well. She has been making her appointments herself and attending them. She has been taking her medicine and is even off one altogether. This was a successful example of all our work pays off. She was such a happy person that day. We all were.”

Other teams in the project have shared success stories too, now into the dozens of patients. With a set curriculum in place, the hope is it will continue to spread beyond WISCH project teams to sickle cell care providers around the world.

  1. Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. 2004;103(11):4023–4027
  2. Brousseau DC, Owens PL, Mosso AL, Panepinto JA, Steiner CA. Acute care utilization and rehospitalizations for sickle cell disease. JAMA. 2010;303(13):1288–1294