Epilepsy affects approximately 360,000 people in Spain, with an annual incidence of 62.6 cases per 100,000 children.1,2 Among the population with active epilepsy, 25% are children, and although most have brief, self-limited epileptic seizures, there is a subgroup with a predisposition to prolonged convulsive seizures, defined as those that last for more than 5min. In these children seizures are less likely to stop spontaneously unless emergency treatment is administered promptly.3 Delays in administering it are associated with a higher risk of evolving into convulsive status epilepticus. This complication occurs in 10–20% of children with epilepsy at some point in their illness and can require admission to the intensive care unit and lead to the development of neurocognitive sequelae.4–7 It is therefore essential to ensure that children with prolonged convulsive seizures receive rescue medication as quickly as possible, regardless of where their seizure occurs.

Most seizures occur in the community, and therefore administration of rescue medication will depend on the presence of a trained caregiver with access to the drug. Rescue medication is administered in the home by parents, while in special education schools there is commonly a nurse present who assumes this responsibility. On the other hand, in ordinary schools parents have to delegate this responsibility to teaching staff with no specific healthcare training for this purpose.

Benzodiazepines are the first-line emergency treatment and their route of administration will depend on the setting in which the child is treated. The intravenous route is the most effective and is the route of choice in the hospital setting, but in the community the transmucosal route commonly has to be used.6 Rectal diazepam is effective in stopping prolonged convulsive seizures and is the most widely used transmucosal benzodiazepine in Spain. However, the perception exists that appropriate application of emergency treatment in the community is limited in practice by a lack of information on how, when and by whom it has to be administered.

There is little published data on how prolonged convulsive seizures are treated in schools or other areas of the community in Spain and the situation is believed to vary according to the resources and staff available in each school.8 The PERFECT™ initiative (standing for Practices in Emergency and Rescue medication For Epilepsy managed with Community administered Therapy) began in 2011 with the intention of finding out how prolonged convulsive seizures are treated in the community in various European countries and identifying points where there was room for improvement. In the following pages we present some of its results in Spain and establish recommendations for improving care of children with prolonged convulsive seizures in the community, with special emphasis on the school setting.

The PERFECT™ initiative includes 3 phases carried out in 6 European countries (France, Italy, Germany, Spain, Sweden and the United Kingdom). Phase 1 consisted of a review of the clinical and non-clinical guidelines regulating the treatment of prolonged convulsive seizures. Phase 2 comprised a qualitative survey addressed to healthcare professionals on their perception of care of children with prolonged convulsive seizures in the community. Phase 3 will consist of a survey of children and caregivers on their experience with prolonged convulsive seizures in the community. The results of Phase 1 have been published at European level, those of Phase 2 are in press and those of Phase 3 will be published at the beginning of 2014.8 This article presents the results of Phases 1 and 2 in Spain.

For the review of clinical guidelines and non-clinical guidance (Phase 1), searches were carried out in Spanish and English in the medical literature indexed in PubMed, as well as websites of professional associations and patients’ organisations, for relevant clinical guidelines and care protocols. The legal framework governing treatment of prolonged convulsive seizures in educational institutions was also reviewed.

The survey of healthcare professionals (Phase 2) was conducted between March and April 2012 and consisted of a 45-min telephone survey in Spanish. The respondents were 15 neuropaediatricians and 5 paediatricians from Spanish public and private hospitals, the intention being to provide a representative sample of specialists involved in treating children with prolonged convulsive seizures in Spain. The participants were selected using a method of freely searching in various Spanish hospitals. They had to be physicians who devoted more than 10% of their time to the care of children with epilepsy, including at least 5 children per month requiring emergency treatment for prolonged convulsive seizures. A market research company, Insight Research Group, conducted the survey. All the results were obtained anonymously.

The committee of experts of the PERFECT™ initiative, composed of leading European epileptologists, played a part in identifying significant sources of information in participating countries and in consultations with local patients’ organisations and professional associations, as well as in developing the questionnaire for healthcare professionals.8

The review of guidelines and legal framework, as well as the survey of healthcare professionals, in Phases 1 and 2 of the PERFECT™ initiative highlight important limitations in the care of children with prolonged convulsive seizures in community settings in Spain. These results will need to be compared with those to be obtained directly from families in Phase 3 of the study, but they already indicate a need to introduce improvements at various points in the chain of care for these children.

The clinical guidelines do not offer practical guidance on the need for training or the conditions for authorising caregivers to administer rescue medication. Recommendations to schools as to whether teachers can administer rescue medication to children are ambiguous and contradictory. On the one hand, the law protects the right to health of children with epilepsy during school hours; on the other, there is no legal obligation for teaching staff to administer medication to children under their care. The result of this contradiction is often that rescue medication is not administered and an ambulance is called when a child has a prolonged convulsive seizure in school. This leads to delay in administering treatment and a consequent risk of morbidity, and it contributes to stigmatising the children affected and limiting their social integration.6,25

Many of these problems are common to other countries, and Table 5 lists some of the recommendations of the committee of experts of the PERFECT initiative for improving the care of children with prolonged convulsive seizures.8 More exhaustive guidelines are needed covering both healthcare and educational areas. As well as specifying the indicated treatment, these guidelines ought to make explicit recommendations on the role of teachers and caregivers in schools. A special role should also be introduced to facilitate interaction between the two areas, as happens in the United Kingdom and Sweden, where epilepsy specialist nurses play this role; unfortunately, that is not the case in Spain, and the person who assumes this function varies from child to child.

The need to administer rescue medication rapidly in order to avoid progression to status epilepticus and its complications must be brought to the attention of everyone involved in the chain of care for children with epilepsy, from the specialists who prescribe the rescue medication to all the settings in which children at risk may experience a prolonged convulsive seizure. Having clear recommendations may make it easier for parents to convey their needs to schools and schools to be provided with the appropriate resources. This information must be an integral part of an overall effort to promote understanding of epilepsy in general. For example, the campaign entitled Conocer la epilepsia nos hace iguales (Knowing about epilepsy makes us equal), which was launched in 2011 and has reached over 25,000 children in 2000 schools all over Spain, has contributed to fostering awareness of epilepsy in schools and has provided guidance on what to do in the event of a convulsive seizure occurring in school. However, the campaign does not make specific reference to prolonged convulsive seizures or to their treatment.26

Paediatricians have an obligation to identify children at risk of suffering a prolonged seizure and to instruct their parents in the administration of emergency medication. Providing parents with rescue medication reduces their fears for the safety of their children, and administering treatment at home, as well as limiting the morbidity of prolonged seizures, may reduce ambulance calls, emergency department visits, and hospital admissions, with the resulting decrease in costs.27,28 Even in patients for whom the treatment does not stop the seizure, its administration seems to make seizures easier to control subsequently in the hospital setting.6 However, some paediatricians are reluctant to prescribe rescue medication for children at risk of suffering prolonged convulsive seizures for fear of respiratory depression which is a side effect of treatment. Respiratory depression associated with the administration of transmucosal benzodiazepines occurs in 5% of cases, whatever the route of administration, but increases significantly after administration of more than 2 doses of benzodiazepines.6,7,29 This must be weighed against the consequences of progression to status epilepticus and the risk of respiratory depression directly attributable to the seizure. In the benchmark study by Alldredge et al.,30 the incidence of respiratory depression in adults suffering a prolonged convulsive seizure was greater in those who received a placebo than in those treated with lorazepam or diazepam intravenously. However, the prescription of emergency treatment cannot be indiscriminate and must be limited to children with an objective risk of prolonged convulsive seizures. A history of prolonged seizures is the main risk factor, and the United Kingdom NICE guidelines limit the prescription of rescue medication to children who have had a previous episode.31

An important consideration is that the only transmucosal benzodiazepine marketed in Spain so far has been rectal diazepam, which presents obvious problems of social acceptance when it is to be administered in the community by non-healthcare staff. Rectal administration and the need to undress the child make many teachers uncomfortable and represent an additional limitation to achieving appropriate emergency treatment in schools. Perhaps the reluctance of schools to administer rescue medication may be partly solved by the availability of benzodiazepines that are easy to administer and socially acceptable, such as buccal or intranasal midazolam, which are preferred by patients and their caregivers.32,33 Buccal midazolam, which has recently been approved for use in Spain for the treatment of prolonged convulsive seizures in children between the ages of 3 months and 18 years, has been shown in some studies to be more efficacious and cost-effective than rectal diazepam.29,34,35 Indeed, the Cochrane guidelines on the treatment of convulsive status epilepticus in children conclude that buccal midazolam is the treatment of choice for children in whom venous access is not possible, and in the United Kingdom, for example, buccal midazolam is now more frequently prescribed than rectal diazepam. 32,36

In conclusion, we need guidelines containing explicit information for parents and schools about the indicated emergency treatment; the ambiguities over administration of treatment in schools must be resolved and caregivers must be trained so that children with prolonged convulsive seizures receive appropriate emergency treatment. Achieving this requires collaboration between the healthcare and education systems, and the existing care protocols are a first step towards that objective. Extending the principles of the non-binding parliamentary resolution on integration of children with diabetes into schools to children with epilepsy requiring rescue medication would represent an important step forward for this vulnerable group of children.

M. Raspall-Chaure, A. Martínez-Bermejo, R. Sánchez-Carpintero and S. Wait have received fees from ViroPharma as consultants in the past.