The Irish Times reported in 2004 that it can take up to two and a half years to get an appointment with some neurology and neurophysiology services in Ireland (Houston 2004). People with intellectual disabilities and epilepsy have particularly complex needs. Families and carers need all the possible advice and treatment on epilepsy, to enhance the quality of life for people with severe intellectual disabilities. The following article gives a synopsis of some recent concepts discussed in the literature.
The problems noted in The Irish Times are not unique to Ireland. Eucare (2001) produced the European White Paper on Epilepsy which assessed the situation throughout Europe; it indicated that even with their problems our services are much better than in some of our developing countries. The World Health Organisation (WHO), the International League Against Epilepsy (ILEA) and the International Bureau of Epilepsy (IBE) in 2003 published Global campaign against epilepsy: Out of the shadows. Even though a global campaign against epilepsy was launched in 1998, in many countries fewer than 50% of the people with epilepsy are being treated properly.
Definition of epilepsy
McMenamin and Bird (1993) define epilepsy as ‘recurrent episodic, uncontrolled electrical discharges from the brain’. This means that recurrent, unprovoked seizures or convulsions occur in one or more parts of the brain, during the transmission of messages within the brain. Effective medical and surgical treatment may alleviate some or all of the symptoms. Progress with new choices in medications is helping to control the symptoms of a greater population of people. However, the research literature demonstrates that a small population of people with severe and profound intellectual disabilities are not benefiting from these new medications. Everitt and Sander (1998) have discovered that the life expectancy of people with both intellectual disabilities and epilepsy is increasing in line with that of the general population. As a result, the incidence of epilepsy is now higher in elderly people than in children.
Brainwave Ireland (2004) states that at least 50% of the general population in Ireland with epilepsy have no explanation for the cause of their problem. For people with intellectual disabilities, epilepsy is one of the most frequently occurring additional major handicaps (Craft et al.1981). The frequency with which these occur together depends partly on the severity of the intellectual disability. The overall prevalence of epilepsy is approximately 15% of those with mild intellectual disabilities, and up to 30% for people with severe intellectual disabilities (Eucare 2001). The care and treatment available should be dictated by the individuals needs, assessments of their overall lifestyle should dictate future plans. However, in spite of the common association between epilepsy and intellectual disabilities, research into the subject and guidelines for effective clinical practice are badly neglected (Espie et al. 1998).
Some people with intellectual disability and epilepsy are said to have ‘epilepsy plus’—that is, they require access to specialist services because: (1) their seizures continue at an unacceptably high rate despite treatment, (2) their diagnosis remains uncertain, or (3) they have additional neurological or mental health problems (Espie et al. 1998). These service users may require anti-epileptic and psychotropic medications.
Whilst the majority of people with intellectual disabilities have controlled epilepsy, for a small number there can be difficulties in treating or attaining management of seizures. More than one anti-epileptic drug may be needed to regain a safe management regime. Generally the greater number of anti-epileptic medications used, the higher risks of side effects occurring (Eucare 2001). Seizures themselves, and the required long-term anti-epileptic treatment, may further impair learning in people with intellectual disabilities. To improve the overall care of the person with epilepsy, careful monitoring of side effects is needed, along with the close observation of seizure patterns.
Ross (2002) highlights a further complication in the diagnosis of epilepsy. She states that as many as 36% of patients diagnosed with epilepsy may have ‘pseudo-epilepsy’ episodes instead of, or as well as, true epileptic attacks. A more favoured term is ‘non-epileptic attack disorder’ (NEAD), which is considered less pejorative than other terms. Betts and Boden (1992) define non-epileptic attacks as a ‘sudden disruptive change in a person’s behaviour, which is usually time-limited, and which resembles, or is mistaken for, epilepsy—but which does not have the characteristic electrophysiological changes in the brain detectable by electroencephalography (EEG), which accompany a true epileptic seizure’. NEADs can be related either to a physiological problem such as diabetes or to mental health issues—e.g. panic attacks.
Ross (2002) recognises that people with intellectual disabilities have poor access to specialist services and investigations. The precise nature of the problem can be unclear, as the person may have multiple disabilities. This can delay diagnosis. Specialist services are needed as difficulties range from distinguishing epileptic phenomena and mannerisms to behaviour disorders such as attention seeking.
Health and safety are central issues for every person, but such interventions for people with special needs require even greater planning and reflection. This situation applies even more acutely in people who have intellectual disability and epilepsy. An English study by Jancar and Jancar (2001) explains that a higher incidence of age-related fractures was noted in people with intellectual disabilities and epilepsy (26%), compared to people with intellectual disabilities and no accompanying epileptic problems. (15%). The authors recommend that prevention of osteoporosis and fractures can be achieved in this population by ensuring regular health checkups, dietary improvements, hormone replacement therapy, the provision of ground-floor accommodation, and any other relevant interventions that may address the individual’s needs. Hormone replacement therapy may be a contentious issue; some major studies have identified adverse reactions with some patients. Sherrard et al. (2002) found similar results when they studied injury risks in young people with intellectual disabilities. The findings revealed that few social and family factors influenced injury risk. They suggested that neurological problems have the most powerful influence on behaviours and emotions of persons with an intellectual disability and, subsequently, on their injury experience. While the person with epilepsy is concerned about their own health and safety, controlled and uncontrolled epilepsy places additional stress on families and carers of people with intellectual disabilities. The level of supervision and other safety factors are important to their quality of life, as the literature highlights this is a concern when carers are planning safety policies specifically in the prevention of accidents and injuries.
The prevalence of epilepsy is higher (35-69%) among those living within institutions for people with severe intellectual disabilities (Eucare 2001). Irish services have moved away from this model of care; community-based residences with small numbers of inhabitants is now the way forward. Therefore, one rarely sees large groups of people with both epilepsy and intellectual disability living together, segregated from the rest of the community.
Sometimes, because of lack of resources, people with intellectual disabilities may not have access to mainstream epilepsy services. Liaison with epilepsy services should be available to all people with epilepsy no matter where they live or who they are. Eucare (2001) explain that there is a selection of assessment tools available to measure people’s quality of life. These tools emphasise the need to progress onto reviewing how epilepsy should not take over every aspect of people’s lives. Eucare (2001) also stress that there has been a growing recognition that assessing the impact of epilepsy must extend beyond counting seizures. Resources are needed for such a worthwhile activity to occur in order to ensure that planning services from needs based assessments enhance peoples’ quality of life. Perhaps the only way forward is to document and produce evidence by assessment of the essential needs of people with both epilepsy and intellectual disabilities.
Great progress has been made in the care and pharmacological treatment of people with epilepsy. However, some still have not gained control of their epilepsy. In particular, people with epilepsy and intellectual disabilities need specialised services to address the specific complications of having both these problems. Families and carers can access information from a variety of sources—via websites and certain organisations such as Brainwave Ireland and The World Health Organisation. However, access to literature is not enough, resources for specialist centres are necessary. Since care is our ultimate concern throughout the lifespan of people with intellectual disabilities, the development of such specialised services could have far-reaching benefits.