Impact of childhood Dravet syndrome on care givers of patients with DS, a major impact on mothers

Nabbout, Rima; Dirani, Maya; Teng, Théo; Bianic, F.; Martin, Monique; Holland, Rowena; Chémaly, Nicole; Coqué, Nathalie

doi:10.1016/j.yebeh.2020.107094

Cited by 34 publications

(38 citation statements)

References 25 publications

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“…Quantitative studies support the qualitative findings that caregivers' social lives can be significantly affected, with one survey finding that more than 50% of parents reported that having a child with DS affected their social life and relationships with family and friends [35]. Mothers reported a greater impact on social life compared with fathers [35].…”

Section: Physical Health and Daily Activitiesmentioning

confidence: 77%

“…All abstracts were reviewed, and any that fulfilled the selection criteria were included in the full text review. Inclusion criteria were: study reporting the impact of caring for an individual with a refractory DEE, including the impact on parents, siblings, or other family members; qualitative or quantitative methods used to assess the caregiver/family impact, including HRQL, psychosocial Modified OCBS; EQ-5D-5L; WPAI; modified NHIS Nabbout et al 2020 [35] To understand the impact of DS on patients with DS and their families, with a focus on the social and economic impact on both mothers and fathers burden, work, or productivity impact; and English language. Exclusion criteria were conference abstracts that did not report data relating to the caregiver in the abstract, and studies reporting results for formal or paid care (non-family members).…”

Section: Abstract Reviewmentioning

confidence: 99%

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Impact of developmental and epileptic encephalopathies on caregivers: A literature review

Gallop

Lloyd

Olt

et al. 2021

Epilepsy & Behavior

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Objectives: Developmental and epileptic encephalopathies (DEEs) are rare neurodevelopmental disorders characterized by early-onset seizures and numerous comorbidities. Due to the complex requirements for the care of a child with a DEE, these disorders would be expected to impact health-related quality of life (HRQL) for caregivers as well as for patients. The objective of this literature review was to describe the impact of DEEs on the HRQL, emotional wellbeing, and usual activities (social, work, relationships, etc.) of caregivers, including the wider impact on other family members such as siblings. Methods: A literature search was conducted in May 2020 using MEDLINE Ò and Embase Ò databases. Quantitative and qualitative studies were identified using search terms related to family, disease type (including >20 specific DEEs), and quality of life/methodology. Each study was assessed for relevance and was graded using customized critical appraisal criteria. Findings from studies that were given the highest quality ratings were summarized and used to develop a conceptual model to illustrate the complex impact of DEEs on caregiver HRQL. Results: Sixty-seven relevant studies were identified, of which 39 (27 quantitative, 12 qualitative) met the highest appraisal criteria. The studies recruited caregivers of patients with one of eight individual DEEs, or pediatric intractable or refractory epilepsy. Most studies reported negative impacts on HRQL and emotional wellbeing in caregivers. The wide-ranging impact of a DEE was highlighted by reports of negative effects on caregivers' physical health, daily activities, relationships, social activities, leisure time, work, and productivity. Factors that influenced the perceived impact included demographic characteristics (e.g., child's age, living arrangements, family income) and clinical factors (e.g., feeding or sleep difficulties, disease severity). Few studies evaluated the impact on siblings. Conclusions: There is evidence that DEEs can impact HRQL and emotional wellbeing and can limit usual activities for the primary caregiver and their wider family. However, no research was identified regarding many individual DEEs, and only limited research assessed the impact on different family members with most studies focusing on mothers. Further research is required to understand the influence of certain factors such as the age of the patient, disease severity, and seizures on caregiver burden. Furthermore, the review highlighted the lack of appropriate measurement tools to assess caregiver HRQL in this population.

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Section: Physical Health and Daily Activitiesmentioning

confidence: 77%

Section: Abstract Reviewmentioning

confidence: 99%

Impact of developmental and epileptic encephalopathies on caregivers: A literature review

Gallop

Lloyd

Olt

et al. 2021

Epilepsy & Behavior

View full text Add to dashboard Cite

show abstract

“…This might explain the child care arrangement for children with DS with in many reports an impact on the parents carrier as they choose to avoid community child care modes at least in the first years of age were the febrile events and possible consequent status and long lasting seizures are frequent. 13 Behavior disorders worsened in over one-third of patients, in almost all children with worsened seizures' frequency, but also with stable or improved seizures' frequency. Despite a trend in correlation to the adolescents, there was no significant relation with the different age groups and this worsening was reported in all 4 groups.…”

Section: Discussionmentioning

confidence: 99%

“…This was an expected result as the frequency of status epilepticus induced by fever and febrile illness remains the major burden in the patients’ group with frequent febrile episodes. This might explain the child care arrangement for children with DS with in many reports an impact on the parents carrier as they choose to avoid community child care modes at least in the first years of age were the febrile events and possible consequent status and long lasting seizures are frequent 13 …”

Section: Discussionmentioning

confidence: 99%

Impact of the COVID‐19 lockdown on patients and families with Dravet syndrome

Brambilla

Aibar²,

Hallet³

et al. 2021

Epilepsia Open

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We explored the impact of coronavirus virus 2019 (COVID‐19) pandemic on patients with Dravet syndrome (DS) and their family. With European patient advocacy groups (PAGs), we developed an online survey in 10 languages to question health status, behavior, personal protection, and health services before and after lockdown. Approximately 538 European PAG members received electronic invitations. Survey ran from April 14, to May 17, 2020, with 219 answers; median age 9 year 10 months. Protection against infection was highly used prior to COVID‐19, but 88% added facemask‐use according to pandemic recommendations. Only one patient was tested positive for COVID‐19. Most had stable epilepsy during lockdown, and few families (4%) needed emergency care during lockdown. However, behavior disorder worsened in over one‐third of patients, regardless of epilepsy changes. Half of appointments scheduled prior to lockdown were postponed; 12 patients (11%) had appointments fulfilled; and 39 (36%) had remote consultations. Responders welcomed remote consultations. Half of responders were unsatisfied with psychological remote support as only few (21 families) received this support. None of the five of patient in clinical trials stopped investigational treatment. Prior adoption of protective measures against general infection might have contributed to avoiding COVID‐19 infections. Protocols for the favored remote contact ought to now be prepared.

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“…This study resonates with the search by Nabbout et al [ 9 ] for a limited set of clinically meaningful endpoints which can potentially be measured and detect change over time with treatment in DS. In addition to seizures, they propose patient cognitive functioning (expressive and receptive communication) alongside caregiver daily activities and caregiver social functioning as measures which could usefully be incorporated into clinical trials [ 11 ] [ 10 ]. In the future, it is envisaged that the chosen endpoints could be collapsed into a single endpoint for use in clinical trials [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Exploring the relationships between composite scores of disease severity, seizure-freedom and quality of life in Dravet syndrome

Strzelczyk

Kurlemann

Bast

et al. 2022

Neurol. Res. Pract.

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Background In Dravet syndrome (DS), a rare epileptic and developmental encephalopathy, the effectiveness of a new treatment is predominantly measured in terms of seizure frequency. However, this may not fully capture the impact of a treatment on the broader aspects of the syndrome and patients’ health-related quality of life (HRQoL). Using a previously published survey which collected data from DS patients and their carers on the broader manifestations of their syndrome, their HRQoL, and their experience of seizures, this study created composite measures of symptom severity to offer new perspectives on the multifaceted aspects of this rare condition. Methods Survey responses on the severity of physical and psychosocial symptoms were combined with independent assessments of disability and care need, to generate three composite symptom scores assessing the manifestations of DS (physical, psychosocial and care requirements). Variation in HRQoL was investigated in multiple regression analyses to assess the strength of association between each of these composite measures and three forms of seizure measures (seizure frequency, days with no seizures and longest interval without seizures), as experienced over a 4- and 12-week period. Results Composite scores were calculated for a cohort of 75 primarily paediatric patients who were enrolled in the study. Strong associations were found between each of the three composite symptom scores and each of the three seizure measures, with the regression coefficient on symptom score highly significant (p ≤ 0.001) in all nine comparisons. Separate regressions using predictors of HRQoL (Kiddy KINDL and Kid KINDL) as the dependent variable were inconclusive, identifying only behavioural/attention problems and status epilepticus as significant predictors of HRQoL. Conclusions These results allow the development of a composite score that may be useful in developing a clinical understanding of the severity of DS for an individual patient and establishing their treatment goals. Where measurement of long-term sequalae of disease is not feasible, such as clinical trials, correlation of the composite score with experience of seizures and seizure-free periods may allow a better contextualisation of the results of short-term assessments. Trial registration German Clinical Trials Register (DRKS), DRKS00011894. Registered 16 March 2017, http://www.drks.de/ DRKS00011894.

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Impact of childhood Dravet syndrome on care givers of patients with DS, a major impact on mothers

Cited by 34 publications

References 25 publications

Impact of developmental and epileptic encephalopathies on caregivers: A literature review

Impact of developmental and epileptic encephalopathies on caregivers: A literature review

Impact of the COVID‐19 lockdown on patients and families with Dravet syndrome

Exploring the relationships between composite scores of disease severity, seizure-freedom and quality of life in Dravet syndrome

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