Children with primary immunodeficiency (PI) often require a high level of medical care, and parents dedicate themselves to managing their children’s health needs. Eventually, though, children grow into adults and must coordinate their own care. How can parents help prepare them for the challenges of staying healthy while navigating a sometimes-complicated diagnosis?
In Growing Pains: Transitioning from Pediatric to Adult Care, a SCID Compass Lunch & Learn presentation, Dr. Lauren Sanchez, an immunologist at the University of California San Francisco (UCSF) Benioff Children’s Hospital, discussed how to provide families and children with the knowledge and skills they need to leave pediatric care and enter adult care.
Transition of care is much more than handing a folder to the next doctor, said Sanchez. It’s a planned, multi-step process involving the transfer of care of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented healthcare systems.
“When a child turns 18, we don’t just drop them off at college and expect them to be instantly independent. Over 18 years, you learn the skills necessary to become independent learners and take care of yourself in college, and the process doesn’t just stop in college. You continue to learn and develop,” she explained.
“Similarly, transition of care is a process for families. Parents and the clinical team work together over time, over many years, to empower the patient to be able to take care of themselves.”
In transition of care, the responsibility of healthcare gradually shifts from parents only, to shared responsibility, to the young adult patient. This process can start as young as ages 11 to 14 and lasts for several years as children learn health literacy and self-management skills.
An important resource for those families whose children have PI and are transitioning to adult care is the Immune Deficiency Foundation Guide for Young Adults.
“It’s a great guide to spark discussion with your young adult or your teen about the things that they need to do to start taking care of themselves in the medical aspect,” said Sanchez. “It’s also a great guide to spark discussion with your clinician about preparing your child for adult care.”
Families may also utilize GotTransition.org, a federally funded initiative to help with the change from pediatric to adult care.
“Transition of care is poised to occur at a really important developmental period where adolescents learn to become adults. They learn to become independent and navigate adult experiences like self-care, independent study, independent learning, driving a car, and going to the doctor,” said Sanchez.
Transition to a dedicated adult health team is critical to maintaining a high quality of care for long-term follow-up of chronic illness, said Sanchez. Unfortunately, many young adults experience delays in that transition. In the U.S., less than 20% of youth with special healthcare needs receive transition preparation, and 50% establish care after the recommended ages of 18 and 22, with some taking several years before finding a primary care provider.
A lack of a structured transition of care can be associated with loss of follow-up, poor compliance with medication, and increased emergency room visits and hospitalizations.
“For kids with chronic illness, these long gaps, a year to three years, could put patients at risk for health complications,” said Sanchez.
Those with PI can be even more at risk. Depending on the immune deficiency, patients might be affected by multiple and complex health problems that can progress with age. These patients need access to a specialized adult center and multiple subspecialists.
For severe combined immunodeficiency (SCID) specifically, follow-up may vary depending on the type of SCID the person has and the treatment they’ve received. Immunity may wane post-transplant, so monitoring of the patient is critical. Patients with SCID need to be transitioned to a team with specialized immunologists and transplanters.
Although no clinical guidelines exist to transition care of children with PI, providers and parents can set age-appropriate goals for adolescents that promote shared decision-making, participation in visits, and communication with the healthcare practice.
“We just want them to start thinking about the differences between pediatric and adult care, but it doesn't have to be a big deal. It doesn't have to be scary. It doesn't have to be abrupt,” said Sanchez.
Start with small steps like encouraging the child to learn the name of their medical condition and the names of medications, their dosages, and purposes. Have the child write a question on an index card for the doctor. As they get older, have the teen use their phone to record the doctor’s contact information, their diagnosis, the name and dosage of medication, and whom to call for after-hours sickness.
UCSF established an immunodeficiency transition clinic for 18 to 22-year-olds because they noticed young adults in that age group were experiencing their first adult care by abruptly transitioning during acute hospitalizations, said Sanchez. As part of the transition clinic, participants take a readiness questionnaire with questions such as:
- Do they take their own medications?
- Do they fill their own prescriptions?
- Do they make their own appointments?
- Do they track their own health issues and communicate with providers?
UCSF transition clinic providers also prepare medical transfer summaries detailing the patient’s entire health history, as well as health team contacts, health insurance information, specialty pharmacy information, and emergency contacts. Medical summaries include:
- Diagnoses and the date of diagnoses.
- Names of the current specialists treating the patient.
- Therapies used both currently and in the past.
- Any prescribed antibiotic prophylaxis.
- Type of immune globulin (Ig) replacement therapy (if applicable).
- Any prescribed immunomodulatory therapy taken in the past, like steroids, graft versus host medications, or biologics.
- Any major hospitalizations or surgeries.
- Dates and the location of the transplant or gene therapy.
- Source of the transplant. Was it a stem cell? Was it gene therapy? Was it a matched unrelated donor? Was it a sibling donor?
- Type of conditioning and the type of chemotherapies and/or radiation used.
- Complications of the transplant or gene therapy.
When the time comes for changing providers, Sanchez suggests that families coordinate a “warm handoff.”
“A ‘warm handoff’ means you have a visit with both your pediatric specialist and your adult specialist in the same room with you, so it's more of a meeting. This reduces gaps in the transition of care and gaps in knowledge regarding the patient,” she said.