Implications for clinical practice – SUDEP in adults with an intellectual disability

[Part 3: Challenges; Kerr M]

 

 

Epilepsy is particularly common in people with an intellectual disability, with one in five adults having a seizure disorder as compared to one in a hundred of the general population. The nature of the epilepsy is often more severe, less likely to be controlled and more likely to need multiple drugs in an attempt to gain control. The impact of the epilepsy can be profound in terms of injury, hospitalization, reduction in social interactions and in an increased risk of SUDEP (Young et al., 2015).

 

UK clinical guidance specifically indicates this population as having higher risk and in need of specific assessment of the risk of SUDEP (NICE 2012). Unfortunately despite this clear message that this population is at most risk from their epilepsy the message from carers and professionals alike is that the needs of this group is not being met (Kerr M, et al 2014). In order to reduce the risk of SUDEP in this population two key areas of care delivery need to be addressed: the recognition and management of risk and the access to high quality treatment. As the epilepsy is frequently more severe, people with an intellectual disability have a high rate of SUDEP risk factors the most important being night time convulsive seizures. The way to reduce this risk focuses on reducing seizure frequency and management of the individual seizures.

 

Recognising risk factors places a responsibility on health professionals to communicate risk to the person with an intellectual disability and to their carers. As many people with an intellectual disability will have significant communication problems, this needs a flexible approach using time and often pictorial materials. Even when using such an approach many individuals will not have the capacity to understand risk and the burden of identification will focus on the interaction between the professional and the carer. At every consultation the health professional must ask about the presence of nocturnal seizures. If carers do not report any then further questioning about possible indicators of such seizures is needed. These include: night time injury and carers noticing that on some days the individual is injured, more confused or sedated in the morning.

 

If there is suspicion but no evidence of seizures, night time monitoring by a waking member of staff or carer may be needed to identify the seizure. Once seizures are recognised two parallel courses of action are needed. The first is to maximise treatment, which is most likely to be through review of the epilepsy medication and discussion of new treatment. The second involves night time care. Whilst as yet unproven, the evidence for immediate seizure support reducing SUDEP risk is compelling. For each individual a night time care plan is needed which focuses on identifying the seizure, managing the seizure and post ictal support. Seizure identification is usually through listening, with or without a listening device. If listening is not possible then other movement sensor-related alarms can be used. The care environment must be able to respond when the seizure is heard. When the individual is not in a family home staff need to be available. There is no set standard for how to deliver care to the individual but staff and parents should use rescue medication as indicated, if prescribed, and are recommended to stay with the individual until he or she is recovered, probably from 30 minutes to an hour.

 

The skills that neurological services need, to deliver the best possible reduction in seizure risk for people with an intellectual disability, are generally those needed to support any person with epilepsy: an understanding of seizure type, an understanding of how to identify SUDEP risk, and an ability to apply high quality epilepsy treatment. However, to apply these skills in a busy neurological clinic with patients who have an intellectual disability can be a challenge. Firstly, the communication barriers mean that increased time is needed in the consultation. There is no avoidance of this and time must be given. Secondly, skill is needed in dealing with families and, most importantly, direct care providers. This skill is not necessarily part of neurological training and needs to be acquired; though it is ideally delivered through specialist nurses working in the clinic.  Thirdly, it is important to have a thorough understanding of the need to assess an individual’s capacity to understand risk and treatment choice. Lastly, neurology teams need to be able to deliver sensible risk plans into care environments to ensure risk reduction is actually carried out.

 

Unfortunately repeated reports in the United Kingdom highlight the failures of care delivery to this population in many aspects of health, including epilepsy. A person with an intellectual disability is at particular risk if they are either unable to communicate their own needs or are not empowered to do so. A particular area of risk is when care provision changes, a not infrequent occurrence for those who do not live in family homes. When there is a change in the social care provider, complete education of the team is often needed.  If there is transition to a new epilepsy provider, either when entering adulthood or for other reasons, it is crucial to ensure that the new provider has the specialist skills needed for this complex population.

 

People with an intellectual disability can so easily fall between the gaps in our epilepsy services and suffer an inverse in their care provision, where those with most need are being managed by those with less skill.  It is this that the global conversation needs to address.

 

 

Mike Kerr

Professor of Learning Disability Psychiatry

Institute of Psychological Medicine and Clinical Neurosciences

University of Cardiff, Wales, UK

Reviewed Apr 2018; Original Dec 2014

 

 

How to cite:

Kerr M. Implications for clinical practice – SUDEP in adults with an intellectual disability. In: Panelli R, Hanna J, Jeffs T, Brockett P, editors. Continuing the global conversation [online]. SUDEP Action &  SUDEP Aware; 2018 [retrieved day/month/year]. Available from: www.sudepglobalconversation.com.

 

 

References

 

 

 

 

 

 

 

Kerr M, Thomson R, Linehan C, Glynn M, Mula M, Gil-Nagel A et al. Listening for a change: medical and social needs of people with epilepsy and intellectual disability. A Joint Report of the International League Against Epilepsy Task Force on Intellectual Disability and the International Bureau for Epilepsy. 2014.

NICE CG137 [National Institute for Health and Care Excellence (NICE) National Clinical Guideline (CG) 137]. Epilepsies: diagnosis and management. CG137. London: NICE; 2012.

Young C, Shankar R, Palmer J, Craig J, Hargeaves C, McLean B, et al. Does intellectual disability increase sudden unexpected death in epilepsy (SUDEP) risk? Seizure 2015;25:112-6.

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Karen was born in April 1975 and died in February 2012 ...an interim cause of death was given as bronchopneumonia, yet we felt this couldn’t be right; everything pointed to epilepsy. Although we had heard of SUDEP, we didn’t think it would happen to Karen as she was always so well...

My whole world changed with one phone call in August 2010. My eldest son, Stephen, had died suddenly. The sheer disbelief, shock and bewilderment was powerfully overwhelming. How could that be? He was a fit, healthy 38 year old guy with no cares in the world...

Katherine was thirty one years old when she died of SUDEP on November 13th, 2011. My introduction to SUDEP was short and shocking...

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