SUDEP and children: implications for clinical practice
[Part 3: Challenges; Cross JH]
Sudden Unexpected Death in Epilepsy in childhood has historically been underplayed. There is no question that in population-based prospective studies of newly diagnosed epilepsy it appears to be a rare occurrence (Callenbach et al., 2001; Nickels et al., 2012). But when reviewing populations of children with complex epilepsy resistant to medication, its prevalence is only too apparent (Donner et al., 2001).
The problem we have is in knowing who may be at risk, and how we may prevent it. We know from experience and study data that children with ongoing tonic-clonic (convulsive) seizures are those most at risk (Tomson et al., 2008). However, the only form of prevention is treatment and optimal control of such seizures. It is the type of seizure, not necessarily the epilepsy syndrome, that indicates the risk. Of course, those children with the more complex epilepsy are more at risk, in view of their ongoing seizures despite regular AED therapy. There has been concern that children with some particular epilepsy syndromes may be at more risk than others. Dravet syndrome is thought to carry a risk of SUDEP of up to 15% (Genton, 2011). However, this group is particularly prone to continuing drug resistant convulsive seizures; further, there is the risk of recurrent status epilepticus as well as heat sensitivity, so they may particularly be at risk during high temperatures.
The issue has always been how to introduce the topic to families and consequently minimise risk. There are professionals who argue that as it is a rare occurrence in children, undue stress and worry should not be imparted onto families by talking about something that may never happen. However, the counterargument would be that, surely, it is easier to discuss at a time when seizure freedom is still the goal of treatment; i.e. at the time of diagnosis of epilepsy. Further, many parents witnessing a convulsive seizure for the first time believe that their child has died. Realising the true risk may be less than previously thought can be more of a comfort than a threat.
There has been long debate as to the role of home monitoring, particularly at night. This has been advocated from the association with nocturnal seizures and an apparent higher risk of SUDEP (Lamberts et al., 2012). However, there is no evidence that overnight monitoring prevents SUDEP. The range of monitors available also has to be taken into consideration; the rate of false alarms may be high and disturbed sleep for the families highly problematic. As children get older, their right and desire for privacy must also be taken into consideration. That aside, it should be the patient and family’s choice as to their use.
When a family experiences a loss through SUDEP their questions are ‘Why didn’t we know?’ ‘How could we have prevented this?’ Information remains limited, but full disclosure to the families of the risks and choices available is the first step to prevention.
There is no question that optimising management of the epilepsy and striving for seizure freedom, where possible, should remain at the forefront of care. This includes accurate diagnosis, both of epilepsy itself and the type of epilepsy, as well as appropriate AEDs and consideration of surgery, where a possible option. This means that all children with a history of a convulsive event, as highlighted in the NICE and SIGN guidelines, should be seen by a paediatrician with special expertise in epilepsy (NICE, 2012; SIGN, 2005). Further, discussion about all risks relating to seizures and treatment should be discussed at an early stage, near to the time of diagnosis (Ramachandrannair, 2013). The need for compliance should be stressed at all stages, particularly in teenagers at a time of transition, both in medical care and in the home. Young adults remain the most vulnerable, possibly as they strive for independence. There is also a need for any discussion to be documented, so those who follow subsequently are aware of what has been discussed.
When a death, presumed SUDEP, does occur, there is often a lack of understanding from those investigating. Medical staff can find it difficult to discuss something about which they have little knowledge. In the UK, the charity SUDEP Action will give help in the event of a SUDEP. They have experienced counsellors who are able to help families through and after the investigation of the death, and provide a supportive environment for families to engage with clinicians and researchers.
J Helen Cross
The Prince of Wales’s Chair of Childhood Epilepsy, UCL-Institute of Child Health, Great Ormond Street Hospital for Children NHS Foundation Trust, London, & Young Epilepsy, Lingfield, UK
How to cite:
Cross JH. SUDEP and children: implications for clinical practice. In: Hanna J, Panelli R, Jeffs T, Chapman D, editors. Continuing the global conversation [online]. SUDEP Action, SUDEP Aware & Epilepsy Australia; 2014 [retrieved day/month/year]. Available from: www.sudepglobalconversation.com.