Affecting approximately 1 in every 100,000 children, juvenile localized scleroderma (LS) is often associated with severe morbidities, such as inflammation and fibrosis. And while that can be managed over a lifespan, a more severe condition called systemic sclerosis (SSc) can demonstrate symptoms in blood vessels, internal organs, and the digestive tract. Both conditions are relatively rare among pediatric populations, which can therefore lead to delayed diagnoses, increasing the risk for permanent damage and worsened outcomes.
And due to their rarity, these related conditions are relatively under-investigated and not well understood by many providers. In order to begin to better understand LS and SSc, and more quickly diagnose patients suffering from them, Kathryn S. Torok, MD, of the Division of Rheumatology and the Director of the Pediatric Scleroderma Clinic at UPMC Children's Hospital of Pittsburgh, collaborated on a complex study exploring caregiver perspectives on barriers faced while navigating diagnosis and care for their child’s scleroderma. 
Published in Pediatric Rheumatology and titled, “Barriers to Care in Juvenile Localized and Systemic Scleroderma: An Exploratory Survey Study of Caregivers’ Perspectives,” this collaboration brings together over twenty other researchers from nearly as many medical institutions, this cross-sectional study gathered caregivers of juvenile LS or SSc patients to answer surveys regarding child’s condition and their experience in receiving a diagnosis and treatment. Caregivers were recruited through a virtual family scleroderma educational conference and a juvenile scleroderma online interest group.
Ultimately, families identified several factors as significant barriers to care, including lack of knowledge about scleroderma within the medical community, finding reliable information about LS and SSc, long wait times for specialty care, and identifying effective medications, among other things. The barrier most frequently identified by caregivers was the lack of knowledge about scleroderma within the medical community, which led to over a quarter (28%) of LS patients and 36% of SSc patients to wait over a year for accurate diagnosis.
While demonstrating an important gap in knowledge among medical providers, this research also indicates a need for an expanded understanding of caregivers’ experience in receiving a diagnosis and treatment for their LS or SSc family members. Torok and her co-authors conclude the study with a call to broaden the scope of the study to include larger proportions of participants with limited English proficiency, as well as geographic and financial constraints. Once barriers to care become generalizable, then disparities can begin to be addressed.
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