Rima Nabbout1, Maya Dirani2, Théo Teng2, Florence Bianic3, Monique Martin3, Rowena Holland4, Nicole Chemaly5, Nathalie Coque6. 1. Reference centre for rare epilepsies, Department of Pediatric Neurology, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France; Inserm U1163, Imagine institute, Paris, France. Electronic address: rima.nabbout@aphp.fr. 2. Reference centre for rare epilepsies, Department of Pediatric Neurology, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France. 3. Syneos Health Consulting, London, UK. 4. GW Pharma Ltd, Cambridge, UK. 5. Reference centre for rare epilepsies, Department of Pediatric Neurology, Necker Enfants Malades Hospital, Paris Descartes University, Paris, France; Inserm U1163, Imagine institute, Paris, France. 6. Alliance Syndrome de Dravet, France.
Abstract
BACKGROUND: The aim of this study was to understand the impact of Dravet syndrome (DS) on patients with Dravet syndrome and their families, with a focus on the social and economic impact on both mothers and fathers. METHODS: A French language on-line survey was distributed (October 2014-January 2015) for completion by caregivers of patients aged <18 years with DS. The survey was hosted on the French Dravet Syndrome Alliance website, and the survey link was provided to patients and caregivers during clinics at the Necker Hospital (Paris, France). RESULTS: Survey responses were available for 91 patients (median age 7.6 years; 81.6% SCN1A mutation positive). Total seizure frequency was >2 per week for 16.1% of patients, 1-8 per month for 55.2% and < 1 per month for 28.7%; tonic-clonic and myoclonic were the most frequent seizure types. Patients showed various degrees of intellectual disability and DS had a high impact on concentration and school learning in 70.1% and 80.5%. In addition, patients showed appetite disorders in 73.6%, sleep disorders in 72.4% and behavior disorders in 62.1%. Most parents were married (80.5%) with higher rates than the French general population (53.5%). Educational achievement and socio-professional categories for the parents were higher than observed in the French general population, while monthly net income was similar. Preparation of medication was generally done by the mother and father (46.0% of patients) or the mother only (37.9%). Most caregivers reported very low or no difficulty with treatment preparation and low or no risk of error. Parents typically spent <30 min per day on treatment preparation and administration and around 4 h per week for attending therapy appointments. Although most patients and parents were perceived to have good general health, mothers had a worse perception of their own general health than fathers. Compared with fathers, mothers reported a greater impact of caring for a child with DS on their social life, relationships with family and friends, time and energy, and professional life. CONCLUSION: Families caring for a child with DS experience considerable social and economic impact, with an apparent greater burden of care on the mother than the father.
BACKGROUND: The aim of this study was to understand the impact of Dravet syndrome (DS) on patients with Dravet syndrome and their families, with a focus on the social and economic impact on both mothers and fathers. METHODS: A French language on-line survey was distributed (October 2014-January 2015) for completion by caregivers of patients aged <18 years with DS. The survey was hosted on the French Dravet Syndrome Alliance website, and the survey link was provided to patients and caregivers during clinics at the Necker Hospital (Paris, France). RESULTS: Survey responses were available for 91 patients (median age 7.6 years; 81.6% SCN1A mutation positive). Total seizure frequency was >2 per week for 16.1% of patients, 1-8 per month for 55.2% and < 1 per month for 28.7%; tonic-clonic and myoclonic were the most frequent seizure types. Patients showed various degrees of intellectual disability and DS had a high impact on concentration and school learning in 70.1% and 80.5%. In addition, patients showed appetite disorders in 73.6%, sleep disorders in 72.4% and behavior disorders in 62.1%. Most parents were married (80.5%) with higher rates than the French general population (53.5%). Educational achievement and socio-professional categories for the parents were higher than observed in the French general population, while monthly net income was similar. Preparation of medication was generally done by the mother and father (46.0% of patients) or the mother only (37.9%). Most caregivers reported very low or no difficulty with treatment preparation and low or no risk of error. Parents typically spent <30 min per day on treatment preparation and administration and around 4 h per week for attending therapy appointments. Although most patients and parents were perceived to have good general health, mothers had a worse perception of their own general health than fathers. Compared with fathers, mothers reported a greater impact of caring for a child with DS on their social life, relationships with family and friends, time and energy, and professional life. CONCLUSION: Families caring for a child with DS experience considerable social and economic impact, with an apparent greater burden of care on the mother than the father.
Authors: Adam Strzelczyk; Gerhard Kurlemann; Thomas Bast; Ulrich Bettendorf; Gerhard Kluger; Thomas Mayer; Bernd A Neubauer; Tilman Polster; Sarah von Spiczak; Regina Trollmann; Markus Wolff; Toby Toward; Jens Gruenert; Eddie Gibson; Clive Pritchard; Joe Carroll; Felix Rosenow; Susanne Schubert-Bast Journal: Neurol Res Pract Date: 2022-06-06
Authors: Isabella Brambilla; José Ángel Aibar; Anne Sophie Hallet; Irena Bibic; Elena Cardenal-Muñoz; Igor Prpic; Francesca Darra; Nicola Specchio; Rima Nabbout Journal: Epilepsia Open Date: 2021-01-25