Patients with pediatric dermatomyositis need guidance on disease ‘rollercoaster’

Guiding patients with juvenile dermatomyositis from pediatric to adult care is essential to minimize comorbidities and complications, according to data presented at EULAR 2022 Congress.

“A 4-year-old is very different than a 16-year-old,” Liza McCann, MD, of the Alder Hey Children’s National Health Service Foundation Trust, in the United Kingdom, said in her presentation.

All too often, patients with pediatric and adolescent dermatomyositis in these disparate age groups are treated similarly, according to McCann. Moreover, they are then cast to the colder, less supportive world of adult care with little guidance or understanding of their disease and how it should be managed, she added.

“The impact of developing a chronic disease during the formative years cannot be understated,” she said.

McCann showed a graphic indicating the broad cross-section of challenges, disease flares and setbacks that can occur for young patients.

“This rollercoaster shows the ups and downs of living with a disease like JDM,” she said.

A big question for these patients is how and when they should be given the full truth about their diagnosis and all of the comorbidities that could potentially come with it.

“Kids say that when they first get diagnosed, they are overwhelmed,” McCann said.

However, as they grow and mature, they became open not only to understanding how the disease can impact them, but also how to manage it.

Still, the key concern, according to McCann, is that the quality and nature of the care often does not match the growth and development of these patients. Because of the multi-systemic nature of dermatomyositis, care is often handled by multiple physicians in multiple centers, which can lead to a common issue for patients in this age group: “Handover is often imperfect and incomplete,” McCann said.

The good news is that organizations like the American College of Rheumatology and EULAR have standards for transitional care, according to McCann.

“It should be holistic,” she said. “It should start at age 11 but is essential by 14.”

A multidisciplinary team is recommended, along with provisions for scheduling doctor visits and access to medications. The transition policy for any given health system should be updated every 5 years, she added.

Providers should also be cognizant that mental health comorbidities like depression and anxiety are common. These outcomes are reported in as many as three-quarters of patients. However, McCann noted that less than half of patients with JDM may have a dedicated mental health professional assigned to their care.

Despite these hurdles, she noted that patients with JDM frequently grow resilient. The comprehensive challenge of dealing with a chronic disease increases their mental strength and coping mechanisms. However, they cannot do all of this alone.

“This resiliency is helped by access to counseling and peer support,” she said.

Such support can do much more than help patients stay on course with their treatments. They can encourage healthy lifestyle behaviors, as well.

“Exercise is a safe and important adjunct to treatment,” McCann said.

She added that patients in this group should be encouraged to adopt an attitude of, “I take my meds and I do my exercise.”

However, despite recommendation documents and advice from experts like McCann, many patients with JDM fail to successfully transition to adult service.

“They need increased support around this time, but often they are getting less,” she said. “They are also dealing with a number of factors in their lives at this age.”

Ultimately, McCann called on every rheumatologist who manages a patient with JDM to provide a clear plan for the transition into adult care.

“May doctors see transition as a single point in time, but it is really a process,” she said.