Quality of life and comorbidities associated with Dravet syndrome severity: a multinational cohort survey

Lagae, Lieven; Brambilla, Isabella; Mingorance, Ana; Gibson, Eddie; Battersby, Alysia

doi:10.1111/dmcn.13591

Cited by 135 publications

(253 citation statements)

References 27 publications

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“…Seizure frequency and severity are common measures of seizure burden. [10][11][12][13][14] From a treatment perspective, these are relatively clear and measurable outcomes. Our qualitative data from a sample of parents of children with severe epilepsies indicate that unpredictability is an equally important aspect of seizure burden.…”

Section: Discussionmentioning

confidence: 99%

Seizure burden in severe early‐life epilepsy: Perspectives from parents

et al. 2019

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Objectives Seizure burden is typically measured by seizure frequency yet it entails more than seizure counts, especially for people with severe epilepsies and their caregivers. We aimed to characterize the multi‐faceted nature of seizure burden in young people and their parents who are living with severe early‐life epilepsies. Methods A one‐day workshop and a series of teleconferences were held with parents of children with severe, refractory epilepsy of early‐life origin and providers for children with epilepsy. The workshop sessions were structured as focus groups and aimed to identify components of seizure burden and their impact from the perspective of parents and providers. Data were gathered, organized, and refined during the workshop using an iterative 4‐step process that drew upon grounded theory. Results Three primary components of seizure burden were identified: frequency, severity, and unpredictability, which was as important if not more important at times than frequency and severity. Caregivers noted that the impacts of seizures were experienced as acute‐immediate consequences, longer‐term consequences, and as chronic effects that develop and evolve over time. The severity of the child's neurological and medical status as well as where in the disease journey a family was represented additional contextual factors that influenced the experience of seizure burden. Significance Patient‐reported and patient‐centered outcomes are increasingly incorporated into the evaluation of treatment effectiveness. Without understanding how the disease creates burden for the patient (or family), it is difficult to know how to assess the impact of treatment. Our preliminary findings indicate seizure burden is a complex construct and unpredictability can be as important as frequency and severity.

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Section: Discussionmentioning

confidence: 99%

Seizure burden in severe early‐life epilepsy: Perspectives from parents

et al. 2019

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“…Average care grade allowances (insurance payments determined by patient care grade level I, II or III on the Pflegebedü rftigkeit scale [a categorisation of need for care, on the basis of which care allowances are paid]), 31 were calculated under the assumption that nursing services are provided by family members. 32 Care grade costs are interpreted here as a proxy for informal care costs.…”

Section: Care Grade (Informal Care) Costsmentioning

confidence: 99%

Burden-of-illness and cost-driving factors in Dravet syndrome patients and carers: A prospective, multicenter study from Germany

Strzelczyk

Kalski

Bast

et al. 2019

European Journal of Paediatric Neurology

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Seizure CostsQuality of life Depression a b s t r a c t Introduction: Dravet syndrome (DS) is a rare developmental and epileptic encephalopathy.This study estimated cost, cost-driving factors and quality of life (QoL) in patients with Dravet syndrome and their caregivers in a prospective, multicenter study in Germany. Methods:A validated 3e12-month retrospective questionnaire and a prospective 3-month diary assessing clinical characteristics, QoL, and direct, indirect and out-of-pocket (OOP) costs were administered to caregivers of patients with DS throughout Germany.Results: Caregivers of 93 patients (mean age 10.1 years, ±7.1, range 15 monthse33.7 years) submitted questionnaires and 77 prospective diaries. The majority of patients (95%) experienced at least one seizure during the previous 12 months and 77% a status epilepticus (SE) at least once in their lives. Over 70% of patients had behavioural problems and delayed speech development and over 80% attention deficit symptoms and disturbance of motor skills and movement coordination. Patient QoL was lower than in the general population and 45% of caregivers had some form of depressive symptoms. Direct health care costs per three months were a mean of V6,043 ± V5,825 (median V4054, CI V4935-V7350) per patient. Inpatient costs formed the single most important cost category (28%, V1,702 ± V4,315), followed by care grade benefits (19%, V1,130 ± V805), anti-epileptic drug (AED) costs (15%, V892 ± V1,017) and ancillary treatments (9%, V559 ± V503). Total indirect costs were V4,399 ±V 4,989 (median V0, CI V3466-V5551) in mothers and V391 ± V1,352 (median V0, CI V195-V841) in fathers. In univariate analysis seizure frequency, experience of SE, nursing care level and severe additional symptoms were found to be associated with total direct healthcare costs. Severe additional symptoms was the single independently significant explanatory factor in a multivariate analysis. Conclusions:This study over a period up to 15 months revealed substantial direct and indirect healthcare costs of DS in Germany and highlights the relatively low patient and caregiver QoL compared with the general population.

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“…Approximately 30% of patients with epilepsy in the general population have drug-resistant seizures that are persisting despite treatment with at least two adequately dosed and tolerated antiepileptic drugs (AEDs) [4]. Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), and tuberous sclerosis complex (TSC) represent three conditions associated with substantially higher rates of treatment-resistant seizures, ranging from 65% in TSC [5] to N80% treatment resistance in LGS [6] and DS [7].…”

Section: Introductionmentioning

confidence: 99%

The direct cost of seizure events in severe childhood-onset epilepsies: A retrospective claims-based analysis

Reaven¹,

Funk²,

Lyons

et al. 2019

Epilepsy & Behavior

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Objective: The objective of the study was to assess the direct cost of medically treated seizure events in severe childhood-onset epilepsies. Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), and tuberous sclerosis complex (TSC) are representative conditions associated with frequent intractable seizures. Methods: Commercial and Medicaid insurance claims from 2010 to 2015 were queried to identify patients with possible LGS, possible DS, or TSC, having ≥2 years of continuous insurance from the date of first epilepsy/seizure diagnosis or antiepileptic drug (AED) fill (index date). Utilization and cost data in patients with and without seizure events requiring acute treatment were evaluated for two years postindex. Medically treated seizure events resulting in minor, moderate, severe, and no injury were included. Average costs were normalized to 2017 dollars at 3% per annum and reported for each cohort, by insurance type and degree of injury. Results: Among 9754 patients, 55.4-58.8% of LGS, 47.7-55.8% of DS, and 13.7-28.0% of TSC cohorts had ≥ 1 medically treated seizure event, depending on insurance type. Events during two-year postindex averaged 2.Conclusions: Patients with intractable seizures having at least one medically treated seizure event incur substantial all-cause costs. Our results can be used to inform cost effectiveness and budget impact models to estimate the value of existing and future treatments for these and similar conditions.

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Quality of life and comorbidities associated with Dravet syndrome severity: a multinational cohort survey

Cited by 135 publications

References 27 publications

Seizure burden in severe early‐life epilepsy: Perspectives from parents

Seizure burden in severe early‐life epilepsy: Perspectives from parents

Burden-of-illness and cost-driving factors in Dravet syndrome patients and carers: A prospective, multicenter study from Germany

The direct cost of seizure events in severe childhood-onset epilepsies: A retrospective claims-based analysis

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