Survey results provide new data on risks, impact and outcome of COVID-19 in people affected by Dravet Syndrome

Data from a survey conducted by Dravet Syndrome UK (DSUK), an independent charity, provides new evidence on the impact of COVID-19 among children and adults with Dravet Syndrome, including the social and psychological impact of shielding.

Dravet Syndrome is a rare form of epilepsy that encompasses a range of comorbidities. Fever is a common trigger for seizures. In addition, some people with Dravet Syndrome may have comorbidities that make them potentially more susceptible to adverse outcomes from COVID-19 infection, such as respiratory problems, swallowing difficulties and spinal abnormalities (1).

Galia Wilson, Trustee and Chair of Dravet Syndrome UK, said: “As COVID-19 infections are once again rising, we hope these survey results provide some timely insights that will be helpful for families living with Dravet Syndrome. The initial outbreak and lockdown was a frightening time for the Dravet community, partly due to uncertainties as to how coronavirus would affect children and adults with this very complex condition. The aim of our survey was to better understand the risks, impact and outcome of COVID-19 in people affected by Dravet Syndrome, to help inform future responses”.

An anonymous cross-sectional online survey was conducted between 17th June and 13th July 13 2020, with responses from 116 parent/carers of children (74%) and adults (26%) with Dravet Syndrome. 

Key findings from the survey include:

• ‘Extremely vulnerable’ status for risk of severe forms of COVID-19 was given to 43% people with Dravet Syndrome by a GP or neurologist
• Even if not deemed ‘extremely vulnerable’ by a medical professional, families adhered to shielding, with 91% of respondents shielding their child/adult at the family home during lockdown
• Symptoms compatible with COVID-19 were reported in 19% of individuals (with no significant difference between children and adults with Dravet Syndrome)
• Among symptomatic individuals, only 4 had a (swab) test, none of which was positive; one person had antibody testing (but not swab testing), which was positive for COVID-19.
• There were no deaths and in most cases, the outcome of infection was ‘benign’ (only 5% required hospitalisation due to severity of symptoms and all recovered) 
• Seizure worsening was reported (increased seizure frequency or duration or both) in 50% of those who developed possible or probable COVID-19 symptoms
• The presence of comorbidities, including susceptibility to respiratory infections, swallowing difficulties, and spinal abnormalities, was not significantly associated with the presentation of COVID-19 symptoms

Professor Sanjay Sisodiya, a member of the DSUK Medical Advisory Board and Professor of Neurology at UCL Queen Square Institute of Neurology, commented. “While this is only a small, retrospective survey and lacks test data on infection and antibody status, it nevertheless provides valuable insights that help us better understand the impact of COVID-19 on the Dravet Syndrome community”. 

Professor Sisodiya continued: “The study adds to the emerging evidence that, overall, people with Dravet Syndrome do not appear to be at increased risk of severe outcomes from COVID-19, though individual circumstances should always be taken into account for each person. In addition, it highlights the potential impact of shielding on mental well-being. Most families in our survey were shielding although less than half were medically advised to do so. Whilst this may have contributed to prevention of infection, there was anecdotal evidence of social and psychological issues related to shielding. For example, several parents/carers reported problems due to increased anxiety and issues in accessing care for these individuals, who have complex needs and may need extensive monitoring”. 

Simona Balestrini, Consultant Neurologist at the Chalfont Centre for Epilepsy and National Hospital for Neurology and Neurosurgery, UCLH, and lead author of a paper summarising the survey findings (2), agreed: “We do not know what adverse effects there may be of prolonged or repeated shielding, and such consequences need consideration. Should there be a further wave of infection, we recommend prompt testing of symptomatic individuals, and regular surveillance for people living in residential care facilities. In addition, given the clinical spectrum of severity in Dravet Syndrome, a holistic risk/benefit assessment of shielding should be conducted on an individual basis, including health and social care needs”.

Summing up, Galia Wilson said: “We want say a heartfelt thank you families for participating in this survey at a time when they’re facing the additional pressures and challenges of coping with COVID-19. Living with a rare condition can feel very isolating, particularly in these unprecedented times. At DSUK, we’ve made it a priority to support families since the start of the pandemic with staff working on without furlough. This survey and the outcomes we’re sharing in today’s report is just one of the ways in which we hope to make a difference to the families we support”.

For more information about Dravet Syndrome and COVID-19, please visit the information hub on our website.

References: 

• In the UK, shielding and additional support are 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, i.e. clinically ‘extremely vulnerable’ status. Epilepsy (including Dravet Syndrome) 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.
• Balestrini S et al. ‘The impact of COVID-19 in Dravet Syndrome: a UK survey’. October 2020. Publication pending. Available to read online here.

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Notes for editors:

About Dravet Syndrome

Dravet Syndrome (DS) is a rare, life-long, life-limiting, catastrophic disorder that places a huge burden on children/adults with the condition, their parents/carers and the entire family.  

DS affects around one in every 15,000 people in the UK. As a spectrum disorder, DS is complex and unpredictable. The key features of DS are treatment-resistant seizures, intellectual disability, autism, behavourial problems, and difficulties with speech, mobility, feeding and sleep. In around 85 % of cases, Dravet Syndrome is caused by a mutation in a gene known as SCN1A.

Seizure-related premature mortality is a major issue in DS. Prolonged, recurrent seizures usually start in the first year of life. As the condition progresses, other seizure types occur. 15% of individuals with DS die of SUDEP (Sudden Unexpected Death in Epilepsy) or status epilepticus before their 20th birthday. Given the high seizure burden, high risk of mortality and associated comorbidities, many children/adults with DS require 24-hour supervision, and additional family support or home care is likely to be required.

This combination of treatment-resistant seizures, debilitating comorbidities and the requirement for 24-hour monitoring causes DS to have a catastrophic impact, not only on health-related quality of life but overall quality of life - for the person with the condition, their parents/carers and the entire family. 

See https://www.dravet.org.uk/about-dravet-syndrome/ for more information.

About Dravet Syndrome UK

Dravet Syndrome UK (DSUK) is an independent charity dedicated to improving the lives of those affected by Dravet Syndrome through support, education and medical research. We do this by:

• Supporting families affected by Dravet Syndrome emotionally, practically and financially. 
• Raising awareness and understanding of Dravet Syndrome among medical professionals
• Funding medical research to increase understanding of Dravet Syndrome, improve its management, work towards better outcomes and to hopefully one day find a cure

See www.dravet.org.uk for more information.