Down syndrome and dementia: ‘Don’t break the link between the carers and the client’

People with Down syndrome and other intellectual disabilities (ID) are living longer than before, and this is good news. However, some are developing serious medical conditions such as dementia much earlier in life than the general population, which brings about a unique set of challenges in the care of this particularly vulnerable group.

Studies have shown that 15 to 40 per cent of people with Down syndrome over the age of 40 have dementia, compared with 4 to 8 per cent of the general population over the age of 65.

The 2014 Intellectual Disability Supplement to the Irish Longitudinal Study on Ageing (IDS-TILDA) revealed that the prevalence of dementia among people with Down syndrome almost doubled from 15.8 to 29.9 per cent compared with the previous study in 2010.

It also found that the average age of the onset of dementia for people with Down syndrome was 55, with some cases presenting in their early 40s. By comparison, the majority of people with dementia in the general population are over 65.

People in the general population between the ages of 30 and 64 can also develop “young” or early-onset Alzheimer’s disease or dementia, but it is increasingly common in the ID population.

Not everyone with Down syndrome will develop dementia, but the risk increases significantly with age. One Irish study published last year revealed that the risk of someone with Down syndrome developing dementia increased from 20 per cent at the age of 50 to 80 per cent by the age of 65.

Increased prevalence

The reasons for the increased prevalence of dementia in people with ID are multifactorial, but the most common is that people with Down syndrome have a genetic predisposition to the condition.

The National Dementia Strategy, which was published last year, highlighted the need for “systems, structures and age-appropriate services specifically to promote timely diagnosis of people experiencing early onset dementia, including people with Down syndrome”.

The College of Psychiatrists of Ireland recommends that every service for people with ID should have a plan for the recognition and management of dementia in people with ID, and that this population should undergo baseline screening and regular repeat assessments.

Dr John Hillery is a consultant psychiatrist with a special interest in intellectual disability who works with Stewart’s Care Ltd in Dublin and St John of God Kildare Services.

Together with a multidisciplinary team, Hillery runs a dedicated memory clinic for people with ID in the St John of God Kildare Services.

In the past two years the clinic has carried out baseline assessments of all clients in the service who are over the age of 40, as well as others who are presenting with possible signs of dementia.

While a number of other ID services run similar memory clinics, they are not available in all services throughout the country and, according to Hillery, the availability and access to other specialist services for people with ID and dementia, such as multifactorial assessments and supports, are also not widely available.

He says there is a lack of residential and specialist beds for people with ID and dementia, with access very much depending on geography.

Unlike the general population, dementia in someone with an ID or Down syndrome can be more difficult to detect and the subtle changes in someone’s cognitive ability may go unnoticed until all of a sudden there is a dramatic decline in their condition, he says.

“Then you have a family who are suddenly being called on to care for someone with huge support needs.”

According to Hillery, one of the most difficult things for families and carers is that having cared for their loved one with ID at home for many years and supporting them to live as independently as possible, when a diagnosis of dementia forces them to seek residential or respite care, the supports they need are not always available.

“It is very positive when a family member with an ID stays at home with their family but the sad thing for me is when something does go wrong; we don’t have the supports to offer,” he says.

Bridget’s story

Vincent Quinn’s cousin Bridget Connolly had Down syndrome and lived relatively independently for many years in the care of St Christopher’s residential services in Longford.

Predeceased by her doting parents, Bridget was a sociable and popular young woman who developed dementia at the age of 46.

“Her carers were saying that she was getting up and not knowing what to do, not being able to put on her own clothes properly . . . just little things. They began to notice sometimes she wouldn’t recognise things, or people, or what to do next,” says Quinn.

First, Connolly developed epilepsy, a common feature of dementia in Down syndrome people that is not seen in the general population.

“She went down very quickly because she ended up . . . in a wheelchair . . . she didn’t recognise us at all,” says Quinn.

Connolly died in hospital in March at the age of 50. Her family is anxious to raise awareness of dementia in the Down syndrome population and the need for dedicated specialist support services to be available to everyone who needs them.

“The thing I did learn most was how dedicated the staff within the service are to looking after their clients. They are very dedicated and . . . the link between them and the client is very, very strong,” says Quinn.

He believes it is imperative that that link between carer and client is not broken if, for example, someone with Down syndrome is moved to an acute hospital. He also believes ID services should be equipped to provide palliative care.

“Don’t break the link between the carers and the client; that gives them more comfort than anything. You can’t replace that with hospital staff,” he says.

Central importance

Prof Mary McCarron, dean of the faculty of health sciences at

Trinity College Dublin

and principal investigator of IDS-TILDA, echoes Quinn’s view of the central importance of relationship-based care for people with Down syndrome and dementia.

According to McCarron, “The other peers with ID also need support and training because they often have great difficulty in understanding what is happening to their friend and this can be very stressful for them.

“People with dementia who have Down syndrome should be supported to remain in the home of their choice with the family and friends that they know. Relationship-based care is absolutely key.

“We need to make greater efforts in ensuring those long-standing relationships are maintained,” she said.

Like many people born with Down syndrome in the 1960s, Connolly was not expected to outlive her parents. However, thanks to advances in medical and social care, she did.

Her cousin says parents and carers of people with Down syndrome need to plan for the future, to ensure that their loved ones are supported to live as independently as possible, despite the devastation of dementia.

Scientists study effects of brain-training Researchers at Trinity College Dublin have just begun a new study to examine whether cognitive training for adults with Down syndrome can have a protective effect for healthy ageing.

The study is being conducted in the context of a growing concern by the researchers with regard to the levels of dementia in an ageing Down syndrome population in Ireland and varying standards of care, support and diagnostic pathways around the country.

The Brain Exercises for Adults with Down syndrome (Beads) study will investigate the feasibility of using existing brain-training games with a cohort of older adults with Down syndrome without dementia, and measure the effectiveness of the training in positively influencing levels of executive functioning such as planning, attention and memory.

A lot of research into the protective value of cognitive stimulation, or brain training, and its importance in healthy ageing, has been conducted in the general population. This is even more vital in a population of adults with Down syndrome as, typically, fewer opportunities for cognitive training and development were presented throughout their lives. To date there has been little work, in Ireland or internationally, on cognitive training and its influence on executive functioning for older adults with intellectual disabilities.

Prof Mary McCarron, the principal investigator, says: "Dementia has become such a significant challenge to the successful ageing of people with Down syndrome and we must do more than simply provide care.

“Other successes in the lives of people with Down syndrome occurred because we found new ways to increase opportunities; we can do no less as we confront the challenge of dementia.”

The Beads study is being led by Prof Mary McCarron and Eimear McGlinchey, a PhD student within IDS-TILDA. The project is supported by Down syndrome Ireland and the work is a collaboration with the University at Albany, New York and the University of Cambridge.