Analysis of Physician Notes to Examine the Clinical and Humanistic Burden of Patients with Dravet Syndrome and Lennox-Gastaut Syndrome in the United States

Abstract number : 1.229
Submission category : 4. Clinical Epilepsy / 4D. Prognosis
Year : 2023
Submission ID : 42
Source : www.aesnet.org
Presentation date : 12/2/2023 12:00:00 AM
Published date :

Authors :
First Author: Mei Lu, MD, MS – Takeda Pharmaceuticals USA, Inc.

Presenting Author: Satish Rao, MD, MS – Takeda Pharmaceuticals

Dave Iwanyckyj, N/A – Amplity; Sally Wade, MPH – Wade Outcomes Research and Consulting; Pablo Racana, N/A – Amplity; Fernando Otalora, N/A – Amplity; Mei Lu, MD, MS – Takeda Pharmaceuticals

Rationale: Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) are rare, childhood-onset, developmental epileptic encephalopathies that are distinguished by frequent seizures, cognitive, behavioral, and developmental issues, and other health problems profoundly impacting patients’ (pts) and caregivers’ lives (1, 2). This study characterized the clinical and humanistic burden of DS and LGS, for which data are lacking.

Methods: Natural language processing (NLP) technology retrospectively analyzed physician-pt interactions recorded in Amplity Insights' medical transcription database. Eligible pts had ≥1 inpatient or outpatient record and a diagnosis of DS or LGS, based on symptoms and/or genetic or electroencephalography findings. Descriptive pt-level characteristics, seizure and nonseizure burden, and quality-of-life (QOL) impacts were summarized.

Results: Results are depicted in Table 1 and Figure 1. Between January 2010 and January 2022, NLP queries identified 166 pts with DS and 1063 pts with LGS treated with ≥1 antiseizure medication (ASM). Most pts with recorded race were White (DS: 95%; LGS: 90%). Pediatric pts aged < 18 years were more prevalent in the DS group (83%) and adult pts aged ≥18 years were more prevalent in the LGS group (58%) at the time of the recorded healthcare provider visit. For pts with data, mean reported age at diagnosis was 1.1 years old for pts with DS and 2.1 years old for pts with LGS. The two most common comorbidities in pts with DS were autism-spectrum disorder (19%) and cardiovascular disease (CVD) (15%); and CVD (21%) and cerebral palsy (19%) in pts with LGS. Among pts mentioning seizures (DS: n=164; LGS: n=1036), cause of onset was: unknown (DS: 48%; LGS: 55%), generalized (DS: 38%; LGS: 38%), and focal (DS: 15%; LGS: 7%). The 3 most frequent seizure types were convulsive (39%), status epilepticus (32%), and febrile (31%) in the DS group; and convulsive (28%), tonic (24%), and myoclonic (17%) in the LGS group. Nonseizure burden included impaired/delayed communication skills for 31% of pts with DS and 38% of pts with LGS, with nonverbal communication most impacted (DS: 22%; LGS: 34%). Half of all pts (DS: 51%; LGS: 50%) experienced developmental delays - primarily delayed behavior and motor skills and growth and nutrition issues. QOL impacts on pts included: sleep issues (DS: 13%; LGS: 10%), feeding-tube use (DS: 8%; LGS: 8%), and inability or difficulty walking or sitting and/or requiring mobility aids (DS: 3%; LGS: 4%). Caregivers’ burden was mentioned in records for 8% of DS pts and 14% of LGS pts.

Conclusions: Though under-reporting is possible with NLP, analysis of this transcription database revealed that, despite treatment with ASMs, pts with DS and LGS experience seizures, nonseizure symptoms, and QOL burdens that extend to caregivers. Application of NLP to future research merits further consideration.

Funding: Takeda Pharmaceuticals USA, Inc. funded this study and writing support.

Clinical Epilepsy