A multicenter, matched case-control analysis comparing burden-of-illness in Dravet syndrome to refractory epilepsy and seizure remission in patients and caregivers in Germany

被引：42

作者：

Strzelczyk, Adam ^{[1
,2
,3
,4
,5
]}

Schubert-Bast, Susanne ^{[1
,2
,3
,6
]}

Bast, Thomas ^{[7
,8
,27
]}

Bettendorf, Ulrich ^{[9
]}

Fiedler, Barbara ^{[10
]}

Hamer, Hajo M. ^{[11
]}

Herting, Arne ^{[12
]}

Kalski, Malin ^{[1
,2
]}

Kay, Lara ^{[3
]}

Kieslich, Matthias ^{[1
,2
,6
]}

Klein, Karl Martin ^{[1
,2
,3
,13
,14
,15
,16
]}

Kluger, Gerhard ^{[17
,18
]}

Kurlemann, Gerhard ^{[10
]}

Mayer, Thomas ^{[19
]}

Neubauer, Bernd A. ^{[20
]}

Polster, Tilman ^{[12
]}

von Spiczak, Sarah ^{[21
]}

Stephani, Ulrich ^{[22
]}

Trollmann, Regina ^{[23
]}

Wiemer-Kruel, Adelheid ^{[27
]}

Wolff, Markus ^{[24
]}

Irwin, John ^{[25
]}

Carroll, Joe ^{[26
]}

Pritchard, Clive ^{[26
]}

Rosenow, Felix ^{[1
,2
,3
]}

机构：

[1] Goethe Univ Frankfurt, Epilepsy Ctr Frankfurt Rhine Main, Frankfurt, Germany

[2] Goethe Univ Frankfurt, Dept Neurol, Frankfurt, Germany

[3] Goethe Univ Frankfurt, LOEWE Ctr Personalized Translat Epilepsy Res CePT, Frankfurt, Germany

[4] Philipps Univ Marburg, Epilepsy Ctr Hessen, Marburg, Germany

[5] Philipps Univ Marburg, Dept Neurol, Marburg, Germany

[6] Goethe Univ Frankfurt, Dept Neuropediat, Frankfurt, Germany

[7] Epilepsy Ctr Kork, Kehl, Germany

[8] Univ Freiburg, Fac Med, Freiburg, Germany

[9] Neuropediat Practice, Hirschaid, Germany

[10] Univ Munster, Dept Neuropediat, Munster, Germany

[11] Friedrich Alexander Univ Erlangen, Epilepsy Ctr Erlangen, Erlangen, Germany

[12] Epilepsy Ctr Bethel, Bielefeld, Germany

[13] Univ Calgary, Hotchkiss Brain Inst, Dept Clin Neurosci, Calgary, AB, Canada

[14] Univ Calgary, Hotchkiss Brain Inst, Dept Med Genet, Calgary, AB, Canada

[15] Univ Calgary, Hotchkiss Brain Inst, Dept Community Hlth Sci, Calgary, AB, Canada

[16] Univ Calgary, Cumming Sch Med, Alberta Childrens Hosp, Res Inst, Calgary, AB, Canada

[17] Schon Klin Vogtareuth, Epilepsy Ctr Children & Adolescents, Clin Neuropediat & Neurorehabil, Vogtareuth, Germany

[18] PMU Salzburg, Res Inst Rehabil Transit & Palliat, Salzburg, Austria

[19] Epilepsy Ctr Kleinwachau, Dresden, Germany

[20] Justus Liebig Univ Giessen, Dept Neuropediat, Giessen, Germany

[21] Northern German Epilepsy Ctr Children & Adolescen, Kiel, Germany

[22] Christian Albrechts Univ Kiel, Dept Neuropediat, Kiel, Germany

[23] Friedrich Alexander Univ Erlangen, Dept Neuropediat, Erlangen, Germany

[24] Univ Tubingen, Dept Neuropediat, Tubingen, Germany

[25] Zogenix Int Ltd, Maidenhead, Berks, England

[26] Wickenstones Ltd, Goring Heath, England

[27] Epilepsy Ctr Kork, Kehl, Germany

来源：

EPILEPSIA | 2019年 / 60卷 / 08期

关键词：

costs; depression; encephalopathy; quality of life; Severe myoclonic epilepsy of infants; SEVERE MYOCLONIC EPILEPSY; HEALTH-CARE UTILIZATION; ILAE COMMISSION; POSITION PAPER; LIFE; CLASSIFICATION; COMORBIDITIES; CHILDREN; COSTS;

D O I：

10.1111/epi.16099

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Objective To compare direct and indirect costs and quality of life (QoL) of pediatric and adult patients with Dravet syndrome (DS), with drug-resistant epilepsy (DRE) and in seizure remission (SR), and their caregivers, in Germany. Methods Questionnaire responses from 93 DS patients and their caregivers were matched by age and gender with responses from 93 DRE and 93 SR patients collected in independent studies, and were compared across main components of QoL, direct costs (patient visits, medication use, care level, medical equipment, and ancillary treatments), and indirect costs (quitting job, reduced working hours, missed days). Results Mean total direct costs were highest for DS patients (euro4864 [median euro3564] vs euro3049 [median euro1506] for DRE [excluding outliers], P = 0.01; and euro1007 [median euro311], P SR). Total lost productivity over 3 months was highest among caregivers of pediatric DS (euro4757, median euro2841), compared with those of DRE (euro1541, P < 0.001; median euro0) and SR patients (euro891, P < 0.001; median euro0). The proportions of caregivers in employment were similar across groups (62% DS, 63% DRE, and 63% SR) but DS caregivers were more likely to experience changes to their working situation, such as quitting their job (40% DS vs 16% DRE and 9% SR, P < 0.001 in both comparisons). KINDL scores were significantly lower for DS patients (62 vs 74 and 72, P < 0.001 in both comparisons), and lower than for the average German population (77). Pediatric caregiver EQ-5D scores across all cohorts were comparable with population norms, but more DS caregivers experienced moderate to severe depressive symptoms (24% vs 11% and 5%). Mean Beck Depression Inventory (BDI-II) score was significantly higher in DS caregivers than either of the other groups (P < 0.001). Significance This first comparative study of Dravet syndrome to difficult-to-treat epilepsy and to epilepsy patients in seizure remission emphasizes the excess burden of DS in components of QoL and direct costs. The caregivers of DS patients have a greater impairment of their working lives (indirect costs) and increased depression symptoms.

引用

页码：1697 / 1710

页数：14

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