Objective:

To characterize patients with narcolepsy type 1 (NT1), narcolepsy type 2 (NT2), and idiopathic hypersomnia (IH), their diagnosis, and treatment journey using administrative health data.

Background:

People with NT1, NT2, and IH endure long diagnostic delays (up to 15 years), have many comorbidities, and suffer considerable, poorly recognized disease burden.

Design/Methods:

This retrospective cohort study included patients aged ≥2 years with (non-diagnostic) medical claims for narcolepsy or IH between January 01, 2014 and December 31, 2021 in Optum’s Market Clarity integrated claims and EHR database. Patients with ≥24 months continuous enrollment prior to last narcolepsy/IH diagnosis (index date) were classified hierarchically into NT1; NT2; IH; NT2/IH cohorts. Patient journeys, diagnostic evaluations and treatments were described using all enrolled time, while comorbidities were described during a 12-month baseline period.

Results:

Overall, 51,548 patients (median 45 years, 64% female) were included: 7,742 (15%) NT1; 31,132 (60%) NT2; 12,287 (24%) IH; and 387 (1%) NT2/IH. Cardiovascular disease (52%), depression (39%), anxiety disorders (38%), obesity (32%) and sleep apnea (40%) were similarly common across cohorts during the 12 months pre-index. Substance-abuse disorders were reported among 18–19% (narcolepsy) and 13–14% (IH) of patients.

9,608 (18.6%) patients had diagnosis switches, most commonly NT2→NT1 (n=3,355), NT1→NT2 (n=2,656), NT2→IH (n=1,187), IH→NT2 (n=859), or NT1→IH (n=205). Among patients with these switches (n=8,262), no sleep tests were recorded for 68% between switches and 38% at any time.  

Common treatments included SSRI/SNRI (57%), stimulants (41%), and wake-promoting agents (39%); 15% of NT1 patients received oxybate and 2.6% pitolisant. Among patients without a diagnosis switch (n=41,940), 26% did not receive any NT/IH medication after diagnosis.

Conclusions:

In a large real-world US population, sleep diagnostic evaluations were underutilized and many patients were untreated. A small proportion received narcolepsy-specific medications. These results indicate an unmet need for effective diagnostic and treatment strategies for patients with narcolepsy/IH.