Category Archives: younger onset dementia

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“Young onset dementia” – a label too far?

 

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“Will you still need me when I’m 64?”

Many younger people can think they will live forever.

When I was at Chelsea and Westminster Hospital yesterday, a senior clinician in care of the elderly joked that this teenage children define ‘old people’ as ‘five years older than my dad’.

The definition of ‘young onset dementia’, referring to people living with dementia below the age of  65, I feel is arbitrary. I think it defies a general wish not to define people by a diagnosis or a label. I don’t live with young onset dementia so my views are compromised, but I believe the phrase ‘young onset dementia’ is potentially compounding a stigma potentially provided by the word ‘dementia’.

I suppose my biggest problem is the cliff-edge phenomenon which comes with such a definition. This can mean you are living with ‘young onset dementia’, but the following day with ‘dementia’.

This can mean totally different funding mechanisms, and subsequent organisation of your care. I know somebody who said to a man with young onset dementia last week, ‘Strictly speaking, I’m not supposed to look after you next week, as your CPN, as you no longer have ‘young onset dementia’ but ‘dementia’.”

She then, as it happens, then promised to carry on visiting, which pleased that person living with dementia no end. With over hundred different diagnostic causes of dementia, it is unwise to generalise, but many people with dementia can find themselves very disorientated very easily. This matters too from the organisation of good quality of care, in other words the need to mitigate against discontinuities in care.

But why especially produce a class of ‘young onset dementia’?

A key message is “Dementia is not just about old people”.  But does distinguishing a group of “young onset dementia” reinforce or compromise this key message?

Members of the public aren’t on the whole aware of “young onset” other conditions, e.g. cancer, where the syndromes may affect a diverse range of people. One hears of ‘juvenile arthritis’ but this is rather different. Of course it is reasonable to identify a group of patients from a certain age, such as department of medicine of the elderly, if specific ‘needs’ of such patients can be addressed such as co-morbidity, polypharmacy, increased likelihood of falls, increased prevalence of dementia or frailty. But it’s worth thinking that 30-40% of an acute medical take routinely involves people aged 65 or above, and if you go onto any general medical ward the bay can resemble a bay of ‘geriatric patients’.

The age of 65 might be considered a completely arbitrary cut-off from a number of perspectives. For example, most of us can think of spritely 90 year-olds who look no older than 48 year-olds living with several long term conditions (indeed might look younger). And one wonders whether this cut off has taken on board other trends in society, such as trends for increased retirement or pension ages, or general trends in longevity?

‘Young onset dementia’ tends to be younger patients with dementia which normally presents in the older age group such as Alzheimer’s disease or vascular dementia, or older people with conditions normally associated with younger people e.g. Down’s syndrome or Niemann-Pick. An argument for catering for specific ‘needs’ in young onset dementia, such as genetic counselling where there is a strong clear pattern of inheritance for the leukodystrophies, is feasible. But this wanders into tricky ground if you argue that people with ‘young onset dementia’ demand special consideration because of ’employment and family’ – older people of course have concerns too about employment and family.

The ‘catering for needs’ argument is pivotal to lobbying and charities. But there might be inadvertent consequences if, say, you argue that younger people with dementia are entitled to supported living with special housing and assistive technology, whereas older people ‘ought to be in care homes’. There are very good clinical reasons for why younger patients might benefit from residential settings, such as complex medical history or repeated falls. There is a problem if is a societal expectation that older patients in care are seen rather as ‘warehoused’, a particularly real concern if such persons were inappropriated prescribed psychotics to keep them quiet.

I personally would much rather speak with a 76 year-old worldly man with life histories to share and wisdom to impart, than spend time with a 45 year-old racist, sexist bigoted arsehole. Everyone is entitled to the highest quality of care and support in dementia, and, quite frankly, ageism in either direction can be found as indirect discrimination in procedures, policies or processes which act to the detriment of a class of people under the Equality Act. This potentially is amenable to judicial review.

Finally, I do wonder whether the term is actively misleading. That is, people think of ‘young onset dementia’ as clinically comprising a totally different set of conditions – but which are quite homogenous in pattern, time course and outcome. I meet quite a lot of people who don’t realise that many of the conditions which cause young onset dementia also cause late onset dementia, such as Alzheimer’s disease or vascular dementia.

I really feel that there should be constructive scrutiny of the term ‘young onset dementia’. As with all labels, one feel intuitively it should be enabling rather disabling. Some people will approve of the label; others won’t. But it’s a debate I feel we should have in the open?

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Anything can happen to anyone at any time

I have some personal form on this. At the beginning of 2007, I was physically able bodied. At the end of 2007, I was learning how to walk and again, getting used to a new physical disability.

I do feel that anything can happen to anyone at any time. It just happens to be my experience that the nastiest of events can happen to the nicest of people (and the opposite is probably true too.)

It is a useful art to know how you personally would react to the unexpected. It’s well known that personal views of immortality and invincibility wane with time, even if they lag behind a temperance of personal ambition.

I attended yesterday an afternoon in a pleasant church in Wimbledon, to celebrate the launch of the Dementia Pathfinders document “Approaching an unthinkable future”. Having now read this original contribution, I do very strongly recommend it.

I took along a copy of my own book to give as a present to someone, so please forgive me for the gratuitous product placement.

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This booklet is in two parts: what a diagnosis of young onset dementia has meant to recipients of that diagnosis in terms of getting on with their lives thereafter, and, secondly, how needs of people with young onset dementia are (or are not) met.

The afternoon was stimulating and refreshing.

The panel discussion consisted entirely of people “living beyond a diagnosis of dementia” (a term coined by Kate Swaffer, leading advocate and co chair of the Dementia Alliance International) and those closest to them. As before, many whom would be perceived as ‘carers’ by others did not see themselves as carers. It was later remarked that co-production lies at the heart of all of Dementia Pathfinders’ work from start to completion.

The contributions were candid, thought provoking and, most of all, useful – they all involved with lives affected by dementia personally.

It was lovely to see Keith and Rosemarie Oliver there. I chatted with Keith for some time. Keith like me is very keen on society hearing about personal narratives (and I should like to commend this new book by Lucy Whitman from my publishers). Also, Keith like me is to keen to tell the world about cognitive treatment approaches in dementia as, for example, advanced by the British Psychological Society faculty for older people.

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It was also lovely to see again Peter Watson, of whom I have fond memories from the Dementia Action Alliance Carers Call to Action.

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And of course Suzy Webster – simply great company, and a true ‘expert by experience’ for care (Suzy’s geographically on the far right of this picture.)

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Dr Jeremy Isaacs gave an excellent overview of young onset dementia. I immediately recognised a slide of his Consultant colleague, Dr Peter Garrard, as he’d been at Cambridge under my own PhD supervisor, Prof John Hodges, and had been on the dementia and cognitive disorders firm at Queen Square under Prof Martin Rossor at the same time I had been there in the early 2000s.

In fact Peter’s work is directly related to one of the questions which was asked about how might the worried well prevent dementia. It’s my view that the best of the previous work has been equivocal (see the EClipSE study paper in Brain 2010); but clearly more research is needed. Peter had been involved in the seminal research studies in language breakdown in some forms of temporal variant frontotemporal dementia. Iris Murdoch, married to Prof John Bayley, Emeritus Professor of English at Oxford, was indeed highly educated – and developed dementia.

On a different note, it was very nice to be in Oxford on the day previous to that to learn of the work of Young Dementia UK. I also had a terrific time visiting the Ashmolean Museum there.

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Here are Barbara Stephens, CEO of Dementia Pathfinders, and Dr Jeremy Isaacs at the beginning of the event.

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I was pleased to witness the overall direction of travel, as explained in Jeremy’s initial summary slide.

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In no way do I wish to undermine the excellent work being done into the genetics of young onset dementia. Understanding the products of these faulty genes is a big step towards developing innovative drugs one day in the future. I dare say, with enough motivation and resources, it might be possible to identify those people who are particularly at risk – say for example those people with a very strong history for the presence and manifestation of particular genes – and use new drugs as a way of slowing down the build up of the products of these faulty genes.
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But I do think EEG (measuring brain waves) can have a particular role to play in say determining whether the process a person presents with in ‘memory clinic’ is indeed a dementia (loss of particular rhythms), and indeed whether some brain wave patterns are that of rapidly progressive dementias such as Creutzfeld Jacob.

I am mindful of the service as a whole, so Jeremy’s point that the national campaign had sustained memes like ‘are you worried with your memory?’ such that many people with other causes of memory problems, for example depression, were coming to be assessed in memory clinic. I know also the converse is true – many people come to the memory clinic as the GP felt dementia had to be excluded but such people show neuropsychiatric symptoms including depression and anxiety. And some of them do later develop a full picture of a dementia.

As Jeremy correctly said, many of the young onset dementias do not indeed present with profound memory loss. I am mindful of the behavioural variant frontotemporal dementia – the subject of my own PhD – heralded by changes in personality and behaviour typically. Another example is posterior cortical atrophy – where the initial problems can be in fact perception of objects, judging depth and so on. Also memory can be quite good, although variable from day to day, for many people who’ve just received a diagnosis of diffuse Lewy body dementia.

This is a legitimate question of national policy therefore, where the overall drive appears to be increasing the dementia rates. But one has to presume that there are sufficient resources in diagnosing young onset dementia with an adequately skilled workforce. The figure for the prevalence of young onset dementia varies – it’s around 40,000 – but I suspect due to the lack of timely diagnosis – the figure is in fact much higher. Jeremy lamented that every doctor should be able to do a good bedside cognitive assessment – and I share this sentiment.

I did say to Jeremy before the event that the philosophy of many doctors did seem to be that not much could be offered through the prism of the ‘prescription pad’. I can immediately think of a whole host of issues which merit discussion beyond the diagnosis: such as employment, housing arrangements, creativity clubs, personal relationships, finances, and other social networks and events.

The diagnosis can be profoundly isolating. Professionals without having organisations to turn to (such as Dementia Pathfinders). It’s my view that many feel that they have no one to turn to other than brilliant organisations such Young Dementia UK. There’s no doubt that third sector organisations, including social enterprises, have a huge role to play in increasing the capacity of services for dementia, as indeed I once argued in the Health Services Journal.

I am sure that there’s a conversation yet to be had about ‘age appropriate services’. Views on this vary: for example, one person living with dementia told me that you wouldn’t mix children with adults on wards, and residential settings should be no exception; on the other hand, another person with young onset dementia, said that having young-only services is ageist in itself, he doesn’t mind talking to people much older people, and he said on principle the difference between 55-65 should not be any different to 65-75 or 75-85 and so on.

Equally critical is the question of how to enhance health and wellbeing regardless of care setting – how to get people in and out of hospital safely and in a timely way is now becoming a burning issue. Dominique Kent’s presentation was superb.

I’m still of the view that anything can happen to anyone at any time. Regardless of the precise ‘care setting’, all persons with dementia do deserve access to the very best standards in NHS healthcare too. And as many people pointed out yesterday the community not only has to be ‘friendly’ or indeed supportive but actually need to be educated in what dementia is. Unfortunately, despite the best efforts of many of us, we’ve still got a long way to go (although it’s fair to say we have come a long way).

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Meeting the needs of persons with younger onset dementia and their supporters

There are many different types of dementia. I happen to believe it’s possible to live well with dementia.

That’s why I wished to travel to Robertsbridge from London Charing Cross to see Charmaine and family, and to give her a copy of my book which I had promised her.

Charmaine and my book

“Living well with dementia” is also the name of the current (five year) dementia strategic framework for England, which is about to be renewed.

The ‘behavioural variant’ of frontotemporal dementia is normally a dementia which occurs in younger people (below the age of 65), with quite a subtle progressive change in behaviour and personality noticed by others.

Early on, in this condition, problems in short term memory and new learning are not in fact common, leading many persons with this dementia to score very highly on the MMSE screening test.

Meanwhile, the ‘temporal variant’ of frontotemporal dementia encompasses several different subtypes.

Primary progressive aphasia (PPA), one of these subtypes, is a language disorder that involves changes in the ability to speak, read, write and understand what others are saying. It is usually acknowledged to be properly described by Mesulam.

Problems with memory, reasoning, and judgment are not thought to be usually apparent at first, but can develop over time.

It is associated with a disease process that causes atrophy in the frontal and temporal areas of the brain, and is distinct from aphasia resulting from a stroke.

In 2011, criteria were adopted for the classification of PPA into three clinical subtypes: nonfluent/agrammatic variant PPA, semantic variant PPA and logopenic variant/phonological PPA.

Therefore the frontotemporal dementias are not usually, early on, characterised by problems in memory for events and facts.

PPA speech not memory

PPA can, as it progresses, be accompanied with anxious behaviours manifest as obsessive-compulsions.

The ‘Dementia Research Centre’ (part of University College London, to which the National Hospital for Neurology and Neurosurgery and Institute of Neurology are associated or attached) hosts a number of superb support groups.

The PPA Support Group holds a number of support groups at regular time intervals. Their newsletter from August 2014 is here.

The convener is  Jill Walton, at jill.walton@ftdsg.org.

I attended yesterday’s meeting.

My close #Twitter pal Charmaine, @charbhardy (Charmaine Hardy), invited me down to Robertsbridge, East Sussex, for Wednesday September 17 2014 from 12 noon to 2pm.

Charmaine

Charmaine describes herself as “I’m a carer to a husband with PPA dementia.”

Of course, Charmaine, her husband and me were in attendance as part of this impressive group of twelve delegates.

Charmaine also claims “Love my garden I post too many pics.” I disagree though on the latter half – Charmaine never posts too many pics of her garden. Here is a photograph I took of a very beautiful flower of hers.

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This is in fact the pergola Charmaine’s husband built with Paul.

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The meeting was held in one of the venue rooms at The Ostrich Hotel (“The Ostrich”), Station Road, Robertsbridge, East Sussex, T 32 5DJ.

The Ostrich is an outstanding B&B housed in a gorgeous Victorian location, with a spectacular tropical garden.

Exotic garden

I had the pleasure of sampling a delicious supper with Charmaine and her husband.

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Our couple of hours yesterday was an informal meeting for people affected by a diagnosis of PPA, their family and friends. We all thought the meeting was fantastic.

Jill showed a video for teaching purposes of what the language presentations of dementia, including PPA, are broadly like.

We all participated in an activity where we had to express the meaning of a sentence without using any of the words, e.g. “Part of my leg is hot and painful.” It was difficult!

The meeting was very enjoyable, but we were able to discuss many issues.

We discussed how the diagnosis of dementia could mean a contraction of your friends’ network, leading to loneliness.

We also discussed how greater education of what the dementias are would help to overcome stigma and discrimination against people living with dementia.

Overall, the feeling of the group was each person living with dementia must be treated with dignity as an unique individual with a significant past and present.

We also felt that the ‘one glove fits all’ approach of housing and accommodation doesn’t work, and forcing people with dementia into accommodation solutions they’re not happy with is bound to cause distress.

This can be a particular problem with younger patients with dementia, who wish to lead independent lives as long as possible, not wishing to be forced into an old people’s home.

If a younger person who receives a diagnosis of dementia it can be challenging to deal with your employer; the construct of ‘dementia friendly communities’, which is said to promote inclusivity, cannot adequately protect against unlawful discrimination if people are not aware of their legal rights and have inadequate access to justice.

PPA also has particular resonance in English policy as a noteworthy example of a younger onset dementia. A ‘younger onset dementia’ refers to a dementia which occurs before 65.

This cut-off is completely arbitrary, however (as arbitrary as the “retirement age”).

Younger onset dementia is a distinct group of people living with dementia, because the conditions which cause these dementias tend to be late presentations of “young” conditions or early presentations of “old” conditions.

In theory, the ‘younger onset dementia’ label is inaccurate, in that a dementia might start long before the symptomatic presentation of people with dementia. At one extreme, some people with the very rare genetic presentations of dementia are born with their dementias (or indeed have the genetic make-up in utero.)

It is therefore clear that we need a much greater sophistication in responding to the needs, beliefs, concerns and expectations of people with younger onset dementia.

The unique identity of people with younger onset dementia means that they have distinct research and service provision needs.

This is particularly true as some of the younger onset dementia can be accompanied by obvious movement and psychiatric symptoms, such as forms of prion disease (GSS and CJD) or Huntington’s disease.

A longstanding problem with the organisation of services is that mental illness problems have traditionally played ‘second fiddle’ to medical problems.

This is exacerbated by the drastic cuts in social care which have relentlessly continued in the last few years.

It is hoped that some of these problems in ‘parity of esteem’ might be mitigated against through ‘whole person care’, the expected policy from May 8th 2015 to integrate health and social care properly for the first time.

I had a very nice conversation with Fiona Chaâbane from the University Hospital Southampton NHS Foundation Trust.

Fiona’s rôle there is as a clinical nurse specialist in dementia, and as a clinical coordinator for Huntington’s disease and younger onset dementia.

I am hopeful that the ‘care coordinator’ rôle will be properly fleshed out in the next government, which will see a more substantial rôle for specialist and general nurses within networks comprising ‘dementia friendly communities’.

I feel that, in many of these conditions, cognitive or psychiatric features can be prominent early on.

My concern about the misdiagnosis of these dementias which do not have a strong component of a failure of memory is a very substantial one.

A misdiagnosis (e.g. of a dementia -> non-dementia such as anxiety or depression) can not only mean that person not obtaining the proper medical treatment, but can also mean that that person goes down a clinical pathway for which he or she is not appropriate. This can also impact on that person’s life and job in an utterly destructive way, particularly more so if before retirement age.

Thanks to Jill, Charmaine, her husband and the PPA support group for educating me properly about the dementias. It was particularly helpful to me too in confirming my concerns about research and service provision for the younger onset dementias in England, unfortunately. But at least we can now all begin to address them.

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