A National Survey Describing Management Patterns for Pediatric Paroxysmal Sympathetic Hyperactivity (PSH)
Hitoshi Koshiya1, Laura Kirkpatrick1, Alyssa Zuziak1, Julie Hauer2, Justin Yu1
1UPMC Children's Hospital of Pittsburgh, 2Boston Children's Hospital / Harvard Medical School
Objective:
To describe current trends in pharmacologic management of paroxysmal sympathetic hyperactivity (PSH) among pediatric physicians practicing in North America.
Background:
Paroxysmal sympathetic hyperactivity (PSH), frequently referred to as dysautonomia and “storming,” is a common source of discomfort among patients with severe neurological impairment. Despite more than 20 classes of medications being described in the literature to treat PSH, there are currently no studies that broadly describe utilization patterns among pediatric clinicians and recommendations rely on expert opinion.
Design/Methods:
From March through August 2023, attending physicians from multiple pediatric disciplines were invited to complete a one-time, web-based survey via Qualtrics. Participants reported their comfort levels in managing PSH, treatment preferences for abortive and maintenance PSH management, and the subspecialties responsible for managing PSH at their institution. Descriptive statistics were calculated; subgroup analyses were conducted using Chi-squared and Fisher’s exact tests.
Results:
Of 295 respondents, 33% were palliative care physicians, 23% were neurologists, 18% were complex care specialists, and 17% were physiatrists. The majority (76%) of respondents felt very/somewhat comfortable managing pediatric PSH. The pediatric subspecialty primarily responsible for inpatient PSH management varied significantly by institution: physiatry (32%), neurology (26%), critical care medicine (15%), palliative care (4%), and general pediatrics (3%). The most frequently used abortive medications were GABA-A agonists (e.g., lorazepam) (85.7%) and alpha-2 agonists (e.g., clonidine) (78.6%). The most used maintenance medications were alpha-2 agonists (89.6%) and gabapentinoids (88.6%). Notably, use of opioid, alpha-2 agonist, and dopamine agonist medications varied significantly for both abortive (p<0.01) and maintenance (p<0.05) management of PSH across pediatric subspecialties.
Conclusions:
Significant heterogeneity in pharmacologic management of pediatric PSH exists, especially across subspecialists. Future work comparing efficacy of pediatric PSH management strategies is needed to improve best-practice recommendations.