key: cord-0813946-qzkmg7jv authors: Balestrini, Simona; Wilson, Galia; Eldred, Claire; Evans, Helen; Sisodiya, Sanjay M. title: The impact of COVID‐19 in Dravet syndrome: A UK survey date: 2021-02-11 journal: Acta Neurol Scand DOI: 10.1111/ane.13405 sha: 90e7ce93ec394e54a1a0ed5b6e23fcf13ff69c68 doc_id: 813946 cord_uid: qzkmg7jv OBJECTIVES: To understand the risks, impact and outcome of COVID‐19 in people affected by Dravet Syndrome (DS). MATERIALS AND METHODS: An anonymous cross‐sectional online survey was conducted between June 17 and July 13, 2020, addressed to families of people with DS. RESULTS: A total of 116 responses were collected, from families of children (n = 86; 74%) and adults (30; 26%) with DS. The majority (106; 91%) were shielded at the family home during lockdown. Symptoms compatible with COVID‐19 were reported in 22 (19%) individuals. Only four individuals with symptoms had a PCR swab test, none of which was positive. Only one symptomatic person had antibody testing (but not swab testing), which was positive. One person had repeatedly positive swab tests whilst in hospital for renal failure, but had no typical symptoms of COVID‐19. In 50% of people with DS who developed possible or probable COVID‐19 symptoms, seizure worsening was reported, in terms of increased seizure frequency or duration or both. Medical attention was required in 9/22 (41%), all of whom were children. CONCLUSIONS: In this cohort of people with DS, we observed an infection rate, determined by compatible symptoms, of 19%, with no deaths and benign outcome in most cases despite the underlying complex epilepsy although children often required medical attention. Early adoption of preventative measures, including testing of symptomatic individuals, regular surveillance for people living in residential care facilities, and shielding of individuals with comorbidities increasing the risk of severe outcome, may limit the impact of COVID‐19. Dravet Syndrome (DS), is one of the most common developmental and epileptic encephalopathies (DEEs), with early onset of seizures and developmental issues. Fever is one of most common precipitants for seizures. 1 Severe peri-ictal hypoxaemia can complicate seizures in DS. 2 People with DS have a high burden of morbidity and risk of premature mortality, mainly epilepsy-related. 3 In an unprecedent catastrophic event such as the SARS-CoV-2 pandemic, people with epilepsy have faced multiple challenges, including the risk of infection itself but also indirect effects related to their comorbidities, reduced access to treatment and health-care services and stress. 4 In the UK, shielding and additional support have been recommended for people with specific medical conditions that have been established or considered to be associated with greatest risk of severe illness from COVID-19, that is clinically 'extremely vulnerable' status. In the UK, epilepsy broadly is not included in this category, but people can also be classed as clinically extremely vulnerable, based on clinical judgement and an assessment of their needs. 5 Additional guidance in people with severe epilepsies is needed. Here we present a survey of people with DS and their carers, to understand the risks, impact and outcome on one of the most severe epilepsy syndromes. A total of 116 responses were collected, from families of children (n = 86; 74%) and adults (30; 26%) with DS, from various parts of the UK and one from Ireland. Age ranges were as follows: under 5 years (n = 23), 5-11 (n = 32), 12-17 (n = 31), 18-24 (n = 16), 25-34 (n = 11), 35-44 (n = 3). The majority (106; 91%) were shielded at the family home during lockdown, whilst a few people with DS remained at their residential home (9; 8%) and one DS person stayed at the family business (1%). 'Extremely vulnerable' status for risk of severe forms of COVID-19 was given to 50 (43%) people with DS, whilst 47 (40%) were not and no request was made, 10 (9%) were not despite a request having been made by the GP or neurologist, and 9 (8%) families were uncertain about the declared vulnerability status of their child. At least three DS people without 'extremely vulnerable' status have been shielding anyway. complications were reported in some people with DS; these included a tendency to respiratory problems, for example history of recurrent chest infections, in 28 (24%); swallowing difficulties, for example percutaneous endoscopic gastrostomy (PEG) in 25 (22%); spinal abnormalities, for example scoliosis, curved or twisted spine, in 33 (29%), ranging from mild (n = 24) to severe (n = 2). Statistical analysis did not reveal significant association between any of these comorbidities and presentation of COVID-19 symptoms. Contact with people who displayed COVID-19 symptoms, necessitating self-isolation, was reported for 10 DS people (9%), and six of these 10 subsequently developed symptoms themselves. asymptomatic people had negative swab tests. One asymptomatic person was admitted to hospital due to a seizure and had an antibody test which was negative. Of the people with DS with COVID-19 symptoms, during the illness five had more frequent and longer seizures (of these, in one also a longer postictal recovery was described), six had more frequent seizures without change in seizure length, and nine had no changes in seizure frequency or length; there was no information provided for two (Table 1) . Interestingly, the person asymp- (Table 1) . There was no significant difference in the prevalence of symp- In one case, it was reported that the use of clobazam for increased seizure frequency was an effective treatment measure. Whilst there was no difference in the age of individuals who had symptoms compatible with COVID-19, we note as an observation that all those who required medical attention were children (the majority below the age of 12 years). In a cross-sectional study of 48 critically ill children affected by COVID-19 and admitted to North American paediatric intensive care units, an uncharacterized 'neurological presentation' was reported in two children, and three had 'seizures' as pre-existing comorbidity; this study showed that rates of COVID-related severe illness and mortality in children are lower than in adults. 8 In the paediatric population, there are a small number of children who present with a Kawasakilike disease, also known as toxic shock syndrome, which includes persistent fever, single or multi-organ dysfunction, headache, and meningeal signs; no seizures have been reported to date associated with this Kawasaki-like disease. 9, 10 No such presentation was reported in our cohort. This observation should be interpreted with caution due to the small sample size and the possibly different threshold for parents to seek medical attention for younger children. Limitations in our study include the small sample size, the retrospective design of the survey, and the lack of data on infection status and immunity due to limited testing having been performed. There is no information available on the occurrence of similar symptoms in previous years; therefore, for most subjects who developed symptoms the association with COVID-19 infection remains purely TA B L E 2 Most relevant comments related to shielding, reported from the open comment section (reported as original comments, anonymized). As a parent I am experiencing anxiety as shielding due to end in August. We have a care agency supporting the 24/7 care needs our daughter has, and I'm also very worried about the risks that having carers come in presents to our daughter and us as a family. My other children are expected to resume school in Sept, as am I as I work in school and I'm terrified about the risks this presents. Been in lockdown since 4th of March no carers or respite. But carers slowly coming back in wearing full ppe. We have kept daughter as isolated as possible during lockdown, and continue to do so, to protect her health; whilst balancing out her and our wellbeing need and taking occasional respite support and some brief visits to school during July. We have had one Carer helping who has remained in our bubble as best we can. We feel this careful but pragmatic approach has worked for us. We are opening up gradually but remain cautious. Feel totally let down from people that are supposed to support us, no care workers, no disability social worker contactable, no help, absolutely exhausted. Just far more behavioural issues due to lockdown and change routine. Seizure control good. Do not see GP or specialist. Was refusing to leave house for months before lockdown so has not been upset by it in fact has been happy with her parents as sole Carers!!! Mental health nurse, psychologist and psychiatrist in frequent telephone and video contact. We stopped PAs [personal assistants] coming before lockdown. speculative. This was not a controlled study, which would be difficult to justify. We should also note that our cohort mostly included children and young adults and this age distribution may have contributed to the benign outcome observed in all cases. Most families in our survey were or have been shielding even when not medically advised to do so, and although this may have contributed to prevention of infection, there was anecdotal evidence of social and psychological issues related to shielding ( whilst the risk of subsequent vaccination-associated seizures seems to be vaccine-specific. 27 In conclusion, our study provides additional evidence that risk of severe outcomes of COVID-19 in people with DS may not be not significantly increased, although medical attention may still be required. However, prospective studies are lacking. We abided by national regulations in the UK where the survey was undertaken. Data handling information was provided at the start of the survey and detailed in the following link: https://www.dravet. org.uk/priva cy-polic y/. This meets the information and consent requirements for survey data sharing, as stated in the UK Data Service regulation (https://www.ukdat aserv ice.ac.uk/manag e-data/legal -ethic al/conse nt-data-shari ng/surve ys.aspx). No personal information is included in this report of the results of the survey. We thank all the families who participated to the survey. S Balestrini was supported by the Muir Maxwell Trust and Epilepsy Society. None of the authors have competing interests to disclose. 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