Oral Session 3
Sunday, April 29, 2018 | 10:40am-12:10pm | Room 342B
PREDICTORS OF TIME TO NARCOLEPSY DIAGNOSIS IN PARTICIPANTS WITH PEDIATRIC ONSET OF SYMPTOMS: RESULTS FROM THE NEXUS NARCOLEPSY REGISTRY
Michael J. Thorpy (United States)*
David J. Pasta (United States)
Miriam G. Cisternas (United States)
Ginger Carls (United States)
Jed Black (United States)
Kathleen F. Villa (United States)
Shay Bujanover (United States)
Danielle Hyman (United States)
Maurice Ohayon (United States)
Recent studies have found that symptom onset for about half of narcolepsy patients occurs before age 18 years, yet diagnosis does not occur until years later. Little is known about the reasons for this delay in diagnosis. This analysis aimed to determine the factors associated with delays in evaluation and diagnosis in narcolepsy patients with pediatric symptom onset.
Materials and methods
The Nexus Narcolepsy Registry is an ongoing, online registry of adults with narcolepsy. Participants were grouped based on whether they reported pediatric (<18 years) or adult (≥18 years) symptom onset. Regression analysis in the pediatric onset group was conducted to predict the number of years (1) between symptom onset and first consultation and (2) between first consultation and diagnosis. Predictors examined included: demographics (sex, ethnicity), symptoms leading to first consultation, provider at first consultation, number and type of physicians seen between onset and diagnosis, and misdiagnoses before narcolepsy diagnosis. Statistically significant predictors were retained in the analysis.
Among all registry participants (N=985), mean time from symptom onset to first consultation was 8.2 years, with an additional 3.5 years from first consultation to diagnosis. 58.8% reported pediatric symptom onset, which was associated with significantly longer mean time from symptom onset to first consultation (10.7 years, 95% confidence interval [CI]: 9.8-11.5) versus adult symptom onset (4.6 years, 95% CI: 4.0-5.3). Pediatric symptom onset was also associated with significantly longer mean time from first consultation to diagnosis (4.5 years, 95% CI: 3.9-5.1) versus adult onset (2.2 years, 95% CI: 1.8-2.6). In the pediatric onset group, significant predictors of a longer mean time from symptom onset to consultation included male sex, excessive sleepiness as a symptom leading to first consultation, and seeing a neurologist or sleep specialist at first consultation (P≤0.015 for all). First consultation with a pediatrician was associated with a mean of 2.9 years from symptom onset to first consultation, which was significantly faster than participants who first consulted a neurologist (13.3 years) or sleep specialist (12.4 years; P<0.001 for both). Significant predictors (P<0.01) of a shorter time from first consultation to diagnosis in the pediatric onset group included episodes of knee buckling that led to the first consultation, seeing a neurologist or sleep specialist for first consultation, and fewer total physicians seen before diagnosis. Seeing a pediatrician at first consultation was associated with a mean of 8.8 years (95% CI: 6.8-10.7) to diagnosis, compared with 4.8 years for a general practitioner (95% CI: 4.1-5.5), 2.8 years for a neurologist (1.5-4.1), and 2.3 years for a sleep specialist (95% CI: 1.1-3.5).
More than half of participants experienced onset of narcolepsy symptoms before 18 years of age, yet these participants took significantly longer to seek medical care and reach a narcolepsy diagnosis compared with the adult symptom onset group. Greater awareness of the pediatric nature of narcolepsy appears to be needed, particularly among pediatricians.
Funded by Jazz Pharmaceuticals.