Children with epilepsy have an increased risk of death but this is largely due to the underlying neurological disorder. In contrast, SUDEP is very rare in children (1-2 per 10,000 patient years) (Camfield & Camfield 2005); neurologically normal children are not at increased risk of death compared to their peers. Of course, these figures are little comfort to parents, many of whom are terrified by SUDEP.

There is no good evidence to guide prevention of SUDEP. Close supervision has been suggested, but in practice this should be combined with training in respiratory stimulation or even in respiratory support (Langan, Nashef & Sander 2000). However, children, especially adolescents, strive for independence and so intrusive supervision for otherwise healthy children is impracticable and mostly undesirable.

Despite the lack of preventative measures, many clinicians report that bereaved relatives frequently express anger that SUDEP had not been discussed with them. However, we do not know if relatives of patients who have not died of SUDEP feel the same. Disclosure of SUDEP information to patients has been recently highlighted in medico-legal cases and by epilepsy management guidelines. The UK National Institute of Clinical Excellence guideline (Stokes et al. 2004) suggests: ‘Information on SUDEP should be included in literature on epilepsy to show why preventing seizures is important. Tailored information on the individual’s relative risk of SUDEP should be part of the counselling checklist for people with epilepsy and their families and/or carers.’ However, it is unclear how this should best be implemented, and whether an individual at low risk benefits by being so informed.

In considering possible negligence claims, Beran (2004) considered the paramount issue to be whether advising a patient of SUDEP would overcome a ‘material risk’ inherent in the treatment of their illness. The uncertainty surrounding mechanisms and effectiveness of potential prevention options precludes sound, evidence-based judgements about specified interventions. Thus, an informed patient and his/her family are aware of the potential risk of SUDEP but have no certain ways to prevent it.

‘Paternalism’ is nowadays frowned upon, and patient autonomy applauded; many clinicians opt for full disclosure. The English requirement to copy clinical letters to patients epitomises this new approach. Beran however, argues that this ignores the ‘right not to know’; unsought knowledge may cause patient and/or family distress, and even prompt negligence claims. Many patients, including children and adolescents fear dying and many erroneously believe that their risk of death is very high; here a detailed SUDEP discussion may actually be reassuring.

So, should we tell children, adolescents and their families about SUDEP? We suggest an individual case-by-case approach, where some form of valued judgement is inevitable. Those actively seeking information (children, adolescents or parents) should be informed in a frank and reassuring way, highlighting the low risk. We do not feel that patients and families should be bombarded with unwelcome information at diagnosis. The decision to discuss SUDEP should be regularly reviewed and documented. Written and website information should be available for those who seek it.

Written by: Charlotte Lawthorn & Phil EM Smith
Epilepsy Unit, University Hospital of Wales, Cardiff

Reprinted with the permission of Epilepsy Australia-the national coalition of Australia epilepsy associations and Epilepsy Bereaved UK.