University of Glamorgan School of Care Sciences

Bachelor of Nursing (Honours) Degree

04043308

Dissertation Title.

Module Code:

Word Count:

Date:

Acknowledgements:

Abstract:

Table of Contents

 Chapter One

Introduction.

1.1 The reason for the choice of this topic.

Whilst on a community based learning disability placement, I spent a day with a dementia care group.  There were ten patients in the group who were cared for by two health care support workers.  One of the patients had Down syndrome and all of the others were from the general population without a learning disability. 

I asked the health care support workers there if they had had any specific training relating to people with Down syndrome who had dementia.  They all stated that they had not any training at all relating to learning disabilities or Down syndrome. 

Later, I asked the team of local community learning disability nurses (who I was placed with) about the lack of this specific training.  They stated that training was given to the health care support workers in the care setting I was referring to.  However, through conversation it became apparent that the health care support workers who were trained had moved to different posts and were no longer working in the dementia care group. 

The community learning disability team were also conducting a review relating to how they dealt with dementia in people with learning disabilities.  From the meetings I attended it became clear to me that there were problematic issues relating to the assessment of dementia  and people with learning disabilities or Down syndrome. Therefore, the difficulties in diagnosing dementia in Alzheimer’s disease in people with Down syndrome became the subject of my assignment.

1.2  The aim of this assignment is…to be completed at the end.

1.3 Definitions of key terms used.

Learning disability.

The Department of Health (2001) describe learning disability as a condition of impaired intellect.  They also state that social functioning is impaired and that these impairments would have began in childhood and continue to affect intellectual growth throughout adult life. 

In addition to this, learning disability has been classified into subsets such as profound and multiple learning disabilities, severe learning disabilities, moderate learning disabilities and mild learning disabilities.  Each subset is defined by a level of intelligence and anyone with an intelligence quotient of seventy or less is considered to have a learning disability.

However, it is thought problematic to define a person’s level of intelligence through testing (British Institute of Learning Disabilities 2007). This is because levels of intelligence change over time and intelligence itself can manifest itself in different ways (and is therefore immeasurable).  Consequently, it may be said that it is difficult to place anyone with a learning disability in any one category.  With this in mind, it may also be the case that it is problematic to label someone with a learning disability at all where the learning disability may be thought to be mild.

Down syndrome.

Down syndrome is a condition caused by chromosomal abnormalities.  This can either be a shortage of chromosomes or in the majority of cases in Down syndrome too many; as there is an extra element of chromosome twenty one. This gaining of a chromosome is often referred to as ‘Trisomy Twenty One’ (Gates 2003). 

This occurrence of chromosomal abnormalities can mean that physical and mental development can be impaired and therefore people with Down syndrome are often labelled with learning disabilities (National Health Service 2007).

Down syndrome affects approximately one in six hundred and seventy five births, although there is greater incidence of the condition where the mother is aged thirty or over (Gates 2003). However, Harvey (2004) estimates that Down syndrome is present in one in every thousand births which suggests there are conflicting studies as to the prevalence of this condition.  Furthermore, Down syndrome is said to be one of the most common causes of learning disabilities (National Health Service 2007a)

Alzheimer’s disease.

Alzheimer's disease presents as gradual changes and deterioration in the structure of cerebral (brain) tissue.  The onset of this condition is usually subtle but the condition will progressively worsen until death occurs.  The cause of Alzheimer’s disease is unknown (World Health Organisation 2007).

Dementia.

Dementia is a symptom of Alzheimer’s disease as it is with other age related degenerative cerebral conditions; such as Parkinson’s disease or Huntingdon’s disease.

It presents as deterioration in a person’s memory, emotions, behaviour and social functioning (Prasher 2005).  Therefore, the sufferer can expect to undergo changes in personality, and as dementia in Alzheimer’s develops, the sufferer can become incontinent and bedridden.  Those with dementia in Alzheimer’s disease will become increasingly dependant on carers as the condition progresses until full time care becomes necessary (The Royal College of Psychiatrists 2007).

Dementia in Alzheimer’s disease is the most prevalent form of dementia (Alzheimer’s Society 2007).

1.4 Literature search process.

The University of Glamorgan’s internet research databases was utilised to begin the literature search.  The databases initially used in this search were CINAHL and MEDLINE.  Keywords such as Down syndrome (or Down’s syndrome), diagnosis and dementia were used originally; this search produced one hundred and fifty six results via MEDLINE and twenty six on CINAHL.

The search process snowballed from these results; as references and keywords from within the above results were used on the My Athens internet resource.  Research that could not be accessed by any of the above strategies were accessed via the Google or Google Scholar search engines.  This was done by submitting the name of the relevant journal into the search engines.  The journal internet site usually allowed access to their publications via an alternative Athens log in mechanism.  Those research papers which proved impossible to access via the Athens facility were retrieved via the Inter library loans service at The University of Glamorgan’s learning resource centre.

The University of Glamorgan’s learning resource centre was also used to access books and resources relevant to the topic.

The above techniques proved to be an adequate way of finding relevant research material.

Chapter Two

Literature review (one).

The general population in the developed world is living longer and so are people with Down syndrome (Kerr and Wilkinson 2005, Prasher 2005).  With this in mind, it is reasonable to suggest that the prevalence of age related conditions such as dementia in Alzheimer’s disease will also increase. However, people with Down syndrome are not only susceptible to the same age related conditions as their counterparts without a learning disability (Davidson et al 2003); they also are more likely to suffer from dementia in Alzheimer’s disease (Holland 2000, Kerr and Wilkinson 2005). 

To emphasise this statement, Deb et al (2007) and Janicki and Ansello (2000), describe dementia in Alzheimer’s disease as a common condition associated with people with Down syndrome.  However, despite the increased incidence of the condition within the Down syndrome population, the diagnosis remains difficult, particularly at the onset of the condition (Deb et al 2007). This may be because the early symptoms of dementia in Alzheimer’s disease are not fully determined (Margallo Lana et al 2007, Watchman 2007).

In addition to this, the pathology indicating Alzheimer’s disease is thought to be present in all people with Down syndrome over the age of forty years, whether the develop Alzheimer’s disease or not (xxxx).  Consequently, clinical screening procedures that highlight abnormalities in soft tissues, such as magnetic resonance imaging and computerised axial tomography (Alexander et al 2005) have limited use in the diagnosis of Alzheimer’s disease, as these interventions will only confirm what is already known.  However, there is some opportunity with these screening procedures to highlight dementia caused by brain legions (which can be reversible) where dementia in Alzheimer’s disease was previously diagnosed.  Unfortunately, the cost of these clinical procedures may prove prohibitive to the National Health Service and it is unlikely at present for such procedures to take place (Hodges 2003).  Furthermore, there is no standard psychiatric screening measure for dementia in people with learning disabilities (Deb et al 2007). 

Moreover, dementia screening or assessment can only be carried out by suitably qualified health professionals, who may not be in a position to carry out day to day observations, which are an important aspect of assessment (Margallo Lana et al 2007). Therefore, it is understandable that the assessment of the condition may be considered problematic, as stated by Deb et al (2007).

To receive the appropriate care for dementia it is reasonable to suggest that a diagnosis of dementia is confirmed in the first instance.  Unfortunately it can be argued that the afore mentioned obstacles lie in the way of such diagnoses.

The life spans of people with Down syndrome have spectacularly increased since the early twentieth century (Margallo Lana et al 2007, Watchman 2003).  In the 1920’s, a person with Down Syndrome was expected to die before or during their second decade of life, whilst at present it is not uncommon for a person with Down syndrome to live into the seventh decade of life (Holland  2000).  However, as dementia was a recognised health problem for people with Down syndrome in the 1920’s (Holland 2000), it is clear that some people with Down syndrome lived well beyond the average life expectancy of the time, as cases of dementia in Alzheimer’s disease were recorded. 

Consequently, as dementia in Alzheimer’s disease in people with Down syndrome has been a highlighted health issue for at least ninety years (Prasher 2005), it can argued, because of the length time that has elapsed since dementia issues have existed concerning people with Down syndrome, that the existing difficulties in diagnosing the condition is disappointing.  This argument is reinforced by the fact that there has been a great deal of research into the subject (Prasher 2005). 

Aetiology of Alzheimer’s disease.

It is not known what causes Alzheimer’s disease; however, it is known that ‘plaques’ and ‘tangles’ form on the brain and it is these pathological changes which damages brain cells.  It is this damage which causes dementia, and as more brain cells are damaged the dementia progresses. The plaques are a build up of proteins called ‘beta amyloids, which are naturally produced in the human body.  The tangles are a mass of brain fibres which have mutated to become intertwined.  The plaques are usually surrounded by dead or dying nerve cells (as beta amyloids are poisonous to these cells) which fail to function properly. This may manifest itself, for example, as forgetfulness in an Alzheimer’s diseased patient if the plaques have damaged cells which affect memory (Alzheimer’s Society 2007).

This is a simplistic description of the pathology which affects those with Alzheimer’s disease.  However, all people with Down syndrome (where Down syndrome was caused by trisomy twenty one) have developed these pathological changes by the time they have reached their fifth decade of life (Margallo Lana et al 2007, Zigman et al 1997, Janicki et al 1995).

However, not all people with Down syndrome (where Down syndrome was caused by trisomy twenty one) and who have reached forty or more years of age, show clinical signs of dementia (xxxx).  Therefore, it may be said that if such pathology as stated above causes dementia, then there must be other factors which also must be present for dementia to occur.  These other factors of causation are as yet unknown, therefore assessing a person with Down syndrome for Alzheimer’s disease is made difficult; as all of the preliminary contributing factors relating to the disease cannot be identified.

Epidemiology of Alzheimer’s Disease in Down Syndrome.

Epidemiology is the study of the prevalence and distribution of diseases in a population (Martin et al 2004).  This research is considered important as it allows policy makers to determine the appropriate resources to be allocated to the treatment of a particular disease (Prasher 2005). 

Determining the prevalence of dementia in Alzheimer’s disease may be thought of as difficult to ascertain. This is due to the conflicting results of research, and factors which make the epidemiological study of the condition complicated (Janicki and Ansello 2000).

The Down Syndrome Association (2003) suggest that 40% of people with Down syndrome will develop indicators of dementia in Alzheimer’s disease by the time they reach fifty years of age. 

In contrast to this, Janicki and Dalton (1997a) cited in Janicki and Ansello (2000) proposed that only 22% of people with Down syndrome aged between forty and sixty years of age displayed symptoms of dementia in Alzheimer’s disease.

However, Burt et al (1998) claim that studies have shown even more dramatic variances in rates of dementia, per se, in people with Down syndrome, than those studies highlighted above.  These variances ranged form 29% to 83% in those aged of forty years of age.   

By way of contrast, the figure of 40%, as suggested by The Down Syndrome Association, is reflected by those in general population who have reached eighty years of age (The Down Syndrome Association 2003).  Furthermore, a figure within the region of 5 -10% of people over sixty five years of age have been diagnosed with dementia in Alzheimer’s disease within the general population (Burt et al 1998).  These contrasting results may suggest that people with Down syndrome suffer from premature aging, as proposed by Jenkins (2005)

From these studies, it is clear that even though the prevalence of Alzheimer’s disease in people with Down syndrome is difficult to ascertain, it is also far more widespread than it is within the general population.

Additionally, as studies have produced such a varied set of results relating to the prevalence of dementia in Alzheimer’s disease in people with Down syndrome; it may be hypothesised that these studies may have been carried with inconsistent approaches or considerations.  With this in mind it is noticeable that a great deal research studied by the author, fails to distinguish the levels of intellectual ability that were present within the research populations.

The level of a learning disability a person has may be factor in producing the erratic results of the studies above.  This is because of ‘intellectual distortion’.  Intellectual distortion can be explained as the varying degrees of how someone with a learning difficulty can explain phenomena due to their intellectual impairment (Priest and Gibbs 2004).  As ‘self reporting’ of symptoms of dementia is an important part of the overall assessment process or dementia (xxxx), it may be concluded that those who cannot understand, or verbally communicate their feelings may have a diagnosis overlooked or incorrectly identified. 

The location of those people with Down syndrome studied may be another factor in producing the erratic results of the above research. 

Gathering populations of people with Down syndrome who are already known to learning disability services should be easier than those who are not known.  Therefore, it may be more likely that those studied have probably lived some of their lives within an institution, (as they are known to services).  If this was the case then ‘psychological masking’ may play a part in the inconsistency of results from studies.

Psychological masking takes place when a learning disabilities service user has lead a limited life and therefore lacks the opportunity of personality development (Priest and Gibbs 2004), as theorised by Maslow (1954) cited in Gates (2003).  With such undeveloped characteristics or behaviours, it may be deemed difficult to fully understand personality or behavioural changes in someone experiencing the onset of dementia.  For example, Watchman (2007) states behavioural changes are the first observable sign of dementia in Alzheimer’s disease in people with Down syndrome.  However, how is someone to observe such behavioural changes when behaviours may already be limited?

Conversely, those who have resided in the local community may have had more diverse life experiences. Therefore, they may have developed more distinguishable characteristics or behaviours, and consequently, changes in these characteristics and behaviours may become particularly observable.  

More to the point though, convenient sampling will also serve to overlook those people with Down syndrome and dementia in Alzheimer’s disease who are not known to services (Deb et al 2001).

It seems reasonable to suggest that to diagnose a person with Down syndrome with Alzheimer’s disease in the first instance, there must be diagnostic criteria for Alzheimer’s disease in which to adhere.  However, the diagnostic criteria available itself can cause variations in the epidemiology of Alzheimer’s disease in people with Down syndrome.  Burt et al (1998) state that if strict diagnostic criteria were adhered to then it would be possible to suggest that people with Down syndrome suffer the same prevalence of dementia in Alzheimer’s disease as those in the general population.  Burt et al (1998) further state that as the population of their study was drawn from  pre existing patients suspected of dementia in Alzheimer’s disease (and not from people with Down syndrome per se) then the actual amount of  people with Down syndrome who display characteristics of Alzheimer’s disease were actually less than that of the general population. 

However, as there is an accumulation of studies suggesting Alzheimer’s disease is more prevalent in the Down syndrome population than in the general population (Coppus et al 2006), it is more likely that the diagnostic criteria used is unsuitable for use in the Down syndrome or learning disability populations (Cooper et al 2003). 

Historically the diagnostic criteria used to diagnose Alzheimer’s disease has been either the ‘Diagnostic or Statistical Manual of Mental Disorders (version four)’ or the ‘International Classification of Diseases, (version ten)’.  These diagnostic criterions could possibly be used for people with mild learning disabilities as these people can be argued to have similar intellectual abilities and personality and behavioural development to those in the general population.  Therefore, dementia related criteria could be measured in the same way.  However, Cooper et al (2003) suggest that the ‘Diagnostic Criteria for Psychiatric Disorders for Use with Adults with Learning Disabilities’ should be used for people with moderate to multiple and profound learning disabilities; as it differentiates between existing  abilities and those disabilities caused by Alzheimer’s disease.

With such a myriad of factors to consider when determining the epidemiology of dementia in Alzheimer’s disease in people with Down syndrome, it can be thought understandable why there are such contrary results stated.  However, these results highlight that the ability to diagnose dementia in Alzheimer’s disease in people with Down syndrome is found to be easier for some, than it is for others.  Nevertheless, as resources are required to make care available for those suffering with Alzheimer’s disease, it is important that the amounts of resources are identifiable.  With such confusion it is possible that resources can be over estimated, and therefore, this could possibly make health care budgets smaller for other considerations.  Conversely, if resources are under estimated, the needs of people with Down syndrome who have Alzheimer’s disease may go unmet.

The clinical presentation of the early onset of Alzheimer’s disease, in people with Down syndrome.

Dementia in Alzheimer’s disease in people with Down syndrome manifests via a broad spectrum of cognitive and non cognitive characteristics (Prasher 2005). However, due to other factors such as age, gender, level of learning disability, pre morbid conditions, medication and environment, the presentation of dementia in Alzheimer’s disease in people with Down syndrome may vary from person to person (Prasher 2005).  Therefore the condition can be difficult to diagnose (Deb et al 2007). 

Researches into the symptoms which present at the onset of the disease add validity to these statements by finding a multitude of initial presentations.  Moreover, initial symptoms of dementia in people with Down syndrome can differ from those initial symptoms which present in sufferers from the generic population (Deb et al 2007, Holland 2004, Burt et al 1998).

Watchman (2007) found in a study of thirty eight people with Down syndrome that behavioural changes, deteriorating daily living skills and confusion were the initial symptoms of dementia in Alzheimer’s disease.  However, in a larger study of ninety six individuals with Down syndrome conducted by Lai and Williams in 1989, memory impairment, temporary spatial disorientation and reduced verbalisation were found to be the initial signs of the disease.  These findings were also found by Oliver et al (1998) and by Evenhuis (1990) in a study of seventeen patients, Evenhuis also found apathetic attitudes, diminished social interaction, and changes in daily living skills.  Bigby (2004) states that memory loss, unusual presentations of emotions and impaired cognitive functioning were the first indication of the disease.  Holland in 2000, found that behavioural and personality changes were the first observable indicators of the condition, whilst Prasher (2005) claimed that impaired memory was usually the first sign of dementia in Alzheimer’s disease. 

Deb et al (2007) also found in a study of twenty four people with Down syndrome that memory loss (particularly short term memory loss) was the predominant factor in diagnosing Alzheimer’s disease.  However, this study also found that many symptoms associated with frontal lobe dementia were also observed at the onset of the disease, whereas those in the general population who have Alzheimer’s disease tend to experience these symptoms at a later stage of the condition (Deb et al 2007).

Symptoms of frontal lobe dementia include apathy, behavioural changes and changes in daily living skills (Alzheimer’s Society 2003).  As these symptoms have also been observed by the some of the above studies, there appears to be common characteristics in frontal lobe dementia and dementia in Alzheimer’s disease, when suffered by people with Down syndrome. 

In addition to this, whilst on placement in a supported living setting, I supported a woman with Down syndrome who began carrying out overly habitual activities of daily living.  The woman had established a routine of polishing furniture and general cleaning kitchen surfaces as part of her day time activities.  However, she became obsessive in her cleaning. For example, she would continuously polish a table top long after it had become clean.  This kind of ritualistic behaviour is also a symptom of frontal lobe dementia (Alzheimer’s Society 2003).

To be finished off….

Chapter Three

Literature review (two).

Primary care.

Following the move from institutional care to communities, people with learning disabilities are now expected to access generic services to a far greater extent than ever before (Department of Health 2001a).  Consequently, the majority of the people within Down syndrome population should now be accessing general practitioner’s surgeries (as opposed to using doctors practising in learning disability institutions). However, the number of people with Down syndrome on general practitioner’s caseloads is small. For example Watchman (2007) states that a typical general practitioner’s caseload in Scotland is unlikely to have more five people with Down syndrome.  From these figures it may be assumed that people with Down syndrome form a minority group on a GP’s caseload; and as the number of people with Down syndrome is so small, a question may be asked that ‘are all people with Down syndrome accessing primary health care’?  To emphasise this point, Melville et al (2005) found that general nurses in general practitioner’s surgeries felt they had infrequent contact with people with learning disabilities and Jones and Kemp (2007) found that general practitioners were not consulting with greater numbers of people with Down syndrome than in the past, which is surprising, if the removal of institutional care is considered.

During a community placement, as a student learning disability nurse, I visited people with Down syndrome who were living alone, or living with a partner who also had learning disabilities.  On one of the occasions, it was suggested by the learning disabilities community nurse (who was my mentor), that a lone client, who previously resided in a learning disabilities institution, may be discharged from the service.  This suggestion was made as the learning disabilities community nurse felt there were no nursing interventions to which the lone client would consent. Furthermore, the learning disabilities community nurse was unsure if there were any nursing interventions that were important enough to maintain a nursing presence.

It was clear from talking to the lone client about his general lifestyle, that he did not enjoy living alone, even though he wanted to live alone.  He appeared to live a secluded lifestyle, where he had no friends and he did not go out of his house and into the wider community.  The lone client’s may have been reticent about leaving his home because he had been tormented by local children shortly after his move to his current setting.  The lone client was suspected to be suffering with mild depression, but this could not be confirmed; he was also of an age where he was at risk of developing Alzheimer’s disease.  His move form institutional care to living alone can therefore be criticised, because if he showed the early signs of dementia in Alzheimer’s disease (or if depression became clear) then there would be no one present to observe this.  Subsequently, his health care needs would go unmet, at least during the onset of the condition.  Although it was the lone client’s wish to remain living alone and have limited or no contact with others, it can be argued that this case should be re evaluated.  This re evaluation could then be used to guide other possible placements of people with Down syndrome in the future.

Although the lone client was living in a community setting, he was not enjoying aspects of a community based lifestyle, which were relevant to that setting.  These aspects may include accessing primary care settings such as a general practitioner’s surgery, where the lone client would have gained access to health care as intended by policy makers. 

Prasher and Janicki (2002), claim that primary health care is essential for the provision of effective health care for people with learning disabilities.  However, does a primary health setting provide suitable care for someone with a learning disability or Down syndrome?

Watchman (2007) stated that in a Scottish survey nearly half of all people with Down syndrome, who were suspected of having dementia in Alzheimer’s disease, would seek health care at a general practitioner’s surgery in the first instance.  This however can be seen as problematic for number of reasons.

First, how can a general practitioner diagnose dementia in Alzheimer’s disease if he has not been specifically trained in aspects regarding learning disabilities (Northway et al 2006), or the nuances of Alzheimer’s disease in people with Down syndrome?  Even though the characteristic signs of the condition in people with Down syndrome are similar to those in people in the general population (Deb et al 2007, Prasher 2005), the diagnoses of Alzheimer’s disease in people with Down syndrome, can and does get missed (Wilkinson 2004).  This is another example of how the move from institutional health care may have failed people with Down syndrome or learning disabilities per se.

Second, despite the belief that most general practitioner’s feel that people with learning disabilities are best cared for in primary health care (Jones and Kemp 2007, Melville et al 2005), the attitudes of some doctors in the generic health services towards people with Down syndrome per se may not allow for a thorough investigation of the symptoms that may indicate dementia in Alzheimer’s disease.  Some doctors or health professionals in primary care have unfortunately formed opinions were they might believe health care is unnecessary for people with Down syndrome in certain circumstances (The Down Syndrome Association 1999).  Where these attitudes prevail, it can be found that those in primary care may think symptoms of Alzheimer’s disease are merely presentations of a learning disability; for example, forgetfulness may be attributed to a person’s intellectual ability as opposed to a symptom of dementia in Alzheimer’s disease (Prasher 2005).  These issues can be considered an obstacle in the way of a diagnosis for dementia in Alzheimer’s disease for people with Down syndrome.

Third, Powrie (2003) and Martin et al (1997), state that general nurses where unclear about their roles with people with learning disabilities (especially where consent was concerned), this viewpoint was also endorsed by Melville et al (2005). It is clear from this research that those within generic services have not fully appreciated or understood government reports and documents on learning disabilities.  Further, the DOH (2001a) has found that generic services for people with learning disabilities have attained different levels of quality across various geographical regions of the United Kingdom and therefore greater understanding of health care issues surrounding learning disabilities is required from those in primary care and regional health care trusts need to review their primary health care of people with learning disabilities. 

The current state of provision can be regarded as disappointing, as those in primary care have been the subject of specific guidelines such as those in ‘Once a Day’ (NHSE 1999a), that attempt to inform primary care workers of best practice when dealing with people with learning disabilities.  

Moreover, as a learning disabilities student nurse, I have observed that in present day nursing education, there still remains a negative attitude displayed by general nursing students towards learning disability issues.  Although education about learning disabilities is provided, it may not be uncommon to hear general student nurses complaining about it being on the curriculum.  If a negative attitude towards people with learning disabilities is present in both qualified general nursing staff and the current general nursing student population, then it can be argued that current pre registration general nursing training and needs to be reviewed.  Furthermore, Melville et al (2005) also believe that qualified general nurses require further training relating to aspects of health care for people with learning disabilities.   It may be thought that it is important that a positive attitude towards caring for people with learning disabilities is engendered at the beginning of a nursing career.  These positive attitudes can then lead to the health care that those in general population enjoy being available to those with learning disabilities, as is their entitlement (Department of Health 2001a).

Melville et al (2005), consider heath care and health promotion an essential element of practice for general nurses employed at general practitioners.  This viewpoint is also true of the learning disability nurse.  With this mind, it is important to examine the role of the learning disability nurse relating to health promotion and its relationship with dementia in Alzheimer’s disease for people with learning disabilities and Down syndrome; this is discussed in chapter four.

Differential diagnosis

The diagnosis of Alzheimer’s disease in people with Down syndrome is made difficult as symptoms which may indicate Alzheimer’s disease are also common in a number of other conditions.  Identifying a condition from symptoms which may be present in a number of conditions is known as differential diagnosis. 

There are a number of symptoms which may indicate the presence of Alzheimer’s disease in people with Down syndrome.  Prasher (2005) states that symptoms of dementia in Alzheimer’s disease in people with Down syndrome include:  Forgetfulness, disorientation, Apraxia (making unskilled movements), Aphasia (incoherent language), Agnosia (misunderstanding of touch senses), impaired recognition, impaired judgement, changes in mood, disturbed behaviour, delusions, hallucinations, disturbed sleep, seizures, incontinence and wandering.  These symptoms can also be found in a combination of studies by Watchman (2007), Deb et al (2007), Holland (2004) and Deb (2003).

Further, the studies by Deb and Deb et al expand on these findings to explain many specific instances that relate to this broad list of symptoms.  From these studies, it may be concluded that symptoms of Alzheimer’s disease in people with Down syndrome are well known and apparently not exhaustive, as one person’s ‘confusion’ or ‘change in daily livings skills’ may differ from another’s.   For example, during a community nursing placement I witnessed a confused person with Down syndrome constantly mistake one family member with another.  Confusion however, can also translate into other ways, such as putting on two socks onto the same feet (Deb 2003).  Moreover, as the list of symptoms that can indicate Alzheimer’s disease in people with Down syndrome appears endless, it can be argued that it is unsurprising that Alzheimer’s disease is difficult to diagnose in people with Down syndrome.  This is because there are so many symptoms and therefore they are likely to be indicators of more than one condition.  Conversely, as the symptoms are well documented and agreed upon, it may be said that is disappointing that dementia in Alzheimer’s disease is difficult to diagnose.

With this in mind, for an accurate diagnosis of Alzheimer’s disease to take place, other conditions whose symptoms mimic Alzheimer’s disease must be ruled out, or established as a co existing condition McQuillan (2003).  However, as Alzheimer’s disease is non reversible disease (Burt et al 1998, Pary 1992), it may seem preferable for a person with Down syndrome to receive a diagnosis that indicates another condition other than Alzheimer’s disease.  

There have been a number of conditions identified that display symptoms which imitate those of Alzheimer’s disease.  Kerr (1997) states that depression, hypothyroidism, sensory impairment, grieving, locational displacement, lack of sleep and sleep apnoea, toxicity and acute confusional states are conditions which display symptoms similar to those of dementia.  McQuillan (2003) and Pary (1992) agrees with Kerr, however, both of these authors also state other physical health conditions which may produce symptoms which mimic dementia, such as lesions situated in the brain.  Consequently, it may be said again that the difficulty in diagnosing dementia in people with Down syndrome is disappointing as there appears to be a general agreement on what conditions have symptoms that mimic dementia.  Even so, as most of the conditions listed above have a predilection to people with Down syndrome, an argument can be made that diagnosis of dementia is difficult as there are many variables to consider

Depression is defined by the World Health Organisation (2007) as a mental disorder which displays symptoms such as low mood, passing of interest or enjoyment, low self esteem, altered sleep patterns which can lead to lethargy, poor appetite and concentration.  Furthermore, depression has been described as a common mental health disorder in people with learning disabilities (Priest and Gibbs 2004).  Priest and Gibbs (2004) further state that that people with Down syndrome are three times more likely to suffer with depression than other people with learning disabilities who are not diagnosed with Down syndrome.  Given that (as previously discussed) people with Down Syndrome have higher than average incidences of both dementia in Alzheimer’s disease and depression, and that these two conditions share similar symptoms, such as disturbed sleep, lethargy and concentration, it may be deemed understandable that dementia in Alzheimer’s disease (or  for that matter, depression) is difficult to diagnose.   To further complicate issues around diagnosis, Lai (1992) cited in Raghavan and Patel (2005) states that people with Down syndrome who are suffering from a severe form of depression are more likely to suffer from dementia than those whose depression is less serious.  This hypothesis may suggest that depression causes dementia if the depression is severe enough.  If this is correct, then it can be thought that dementia and depression are genetically intertwined; as dementia in people with Down syndrome (as previously discussed) is caused by biological changes in the brain due to genetic abnormalities, and depression is thought to be more likely to occur if there is a family history of depression (National Health Service 2007).  Therefore, it may be hypothesised that dementia and depression co exist in varying degrees and that symptoms shared by the two conditions are to be expected. 

During a community placement I visited a service user who was diagnosed with dementia.  Support staff at the service user’s accommodation had reported a marked improvement in the service user’s daily activities.  The service user had been prescribed fluoxetine, an antidepressant medication.  The service user may have previously been suffering with depression before dementia was suspected, hence the marked improvement in daily activities, however, given Lai’s statement claiming a link between dementia and depression, it may be thought that the fluoxetine was having a positive effect on both dementia and depression( if depression was present).

What about other conditions???????

Diagnostic overshadowing

Chapter Four.

The Role of the Learning Disability Nurse.

Historically, people with learning disabilities received their health care in large-scale institutions (Prasher 2005, Phillips et al 2004).  At these institutions health professionals were readily available to carry out not only specialised interventions such as psychiatry or psychology, but were also able to draw upon their training to provide generic health care (Gates 2003).

 However, since reports such as Better Services for the Mentally Handicapped (DHSS 1971) and The Jay Report (DHSS 1979), people with learning disabilities and their health care have slowly been integrated into the community. 

Furthermore, the documents Valuing People (2001a) in England, Fulfilling the Promises (2001) in Wales and The Same as You (2000) in Scotland have also placed emphasis on supporting community integration and normalisation for people with learning disabilities; as suggested by O’Brien (1986) cited in Gates (2003) and have reiterating the importance of generic health care for this client group. 

With such widespread endorsement of community integration for people with learning disabilities, it is reasonable to suggest that the community is right place for people with learning disabilities to live.  Consequently, it may be deduced, as health services for people with learning disabilities have changed then so have the roles of those who provide health services for people with learning disabilities.

With policy forcing people with learning disabilities into the care of those in primary health care services; problems have arisen due to the unclear roles of learning disability nurses (Powrie 2003, Jukes and Bollard 2003). This transition has greatly affected how learning disability nurses practice.  Indeed, learning disability nursing has now become distinct from other branches of nursing; as it no longer follows a sickness model of care (Mitchell 2000).  As a consequence of this, health promotion is now considered an integral part of the role of the learning disability nurse (Northway et al 2006, DOH 1996).

4.1 Health promotion and Dementia.

The World Health Organisation (1986) defines health promotion as, ‘…the process of enabling people to increase control over, and improve, their health’.  This definition is mirrored by Nightingale (1992) who suggests health promotion can be seen as pro-active ill health prevention, such as screening or observation, early detection of conditions, that can be treated appropriately and encouragement of current treatment regimes, to attempt to improve current ill health. 

Most people within the general population have control over their own health.  They are free to make choices, whether to eat healthily, smoke, consume too much alcohol or partake in exercise.  Moreover, these people can understand the implications of their choices.  However, because of impaired intellectual ability, people with learning disabilities can have difficulties in understanding the rationale of making a health choice concerning any aspect of wellbeing or lifestyle. 

From the above definitions, it is clear that some people with learning disabilities would require someone to support them in promoting their own heath as their learning disability may prove a barrier to this.  Moreover, The Department of Health (2001a) states that all people with learning disabilities must have a health facilitator and action plan in place by 2003.  As learning disability nurses are the only health professionals specifically trained to deal with health matters relating to people with learning disabilities (Northway et al 2006), it is reasonable to suggest that the learning disability nurse is the best placed health professional to be the health facilitator.  To enforce this viewpoint, Powrie (2003), also found that general nurses within primary health care, such as those employed at a general practitioners, thought that they were inadequately trained to deal with people with learning disabilities.  Moreover, the need for a health facilitator for someone with Down syndrome could possibly be exacerbated; as this person may experience not only impaired intellectual ability but the debilitating presence of dementia.

Health promotion for people with learning disabilities can be considered more important than health promotion for those within the generic population because of their greater health needs (Northway et al 2006, Cooper et al 2004, Matthews 2000), but as provision is available in the United Kingdom for health care for all, via the National Health Service, then appropriate health care or promotion should be attainable.  However, there are barriers existing that make health promotion difficult for people with learning disabilities in the community (Finlayson et al 2004).

A barrier to health promotion may be the manner in which supported living accommodation is staffed, or as in some cases; the barrier exists where no staff are present at the home of a people with learning disabilities or people with Down syndrome.

It is an established fact that people with learning disabilities suffer from poor mental health than those in the general population (Cooper et al 2006, Janicki and Ansello 2000, Patel et al 1997) and within this scenario it is also an established fact that people with Down syndrome suffer greater incidence of dementia than those within the learning disability population without Down syndrome (McQuillan 2003, Wilkinson and Janicki 2002, Hutchinson 1999).  With this in mind, it may be said that people with learning disabilities and people with Down syndrome require constant specialist support, as stated by Bigby (2004); now that they do not reside within institutions where constant specialist support was once the norm (Northway 2006). 

However, as a student learning disabilities nurse I have encountered numerous settings in which there isn’t a learning disabilities nurse employed.    These settings are often where people with learning disabilities are suggested (by those providing supported living services) to have no particular health needs and therefore do not require learning disability nursing interventions.  Still, The World Health Organisation (1948) defines health as, ‘a state of complete physical, mental and social well being rather than merely the absence of disease or infirmity’.  From this definition it can be assumed that all people with learning disabilities are not healthy as people with learning disabilities by definition have an intelligence quotient of seventy or less (The Department of Health 2001), and therefore it is clear that this group of people cannot attain mental well being and are for that reason, unhealthy.  Thus, it may be suggested that all people with learning disabilities require nursing support.

To emphasise this point, Alzheimer’s disease in people with Down syndrome can manifest itself differently in the early onset of the disease to that which is observed in the general population (as previously discussed).  Therefore, for the dementia to be detected at the earliest possible time, a learning disability nurse must be present.

Moreover, in settings such as supported living where only health care support workers are employed, it may be stated that people with learning disabilities are receiving second class health care.  This statement is not intended to suggest that that health care support workers are not valued, but how are they able to accurately deliver information relating to behavioural changes in a people with Down syndrome,( relating to the early onset of dementia) when they might not have been trained in aspects of dementia (Wilkinson et al 2004).  Whilst on placement in a community learning disability setting I asked the nurses present if they had ever trained health care support workers in aspects of dementia relating to learning disabilities or Down syndrome.  They stated that they had, however, this training proved pointless as those they had trained had moved to other posts where the training was not relevant. 

Therefore, even though the facility of community learning disability nursing is available, it may be some time before dementia is suspected as dementia can present differently in people with Down syndrome than it does in those within the general population (Davidson et al 2003).   

To be finished off……………

Chapter Five

Recommendations for Practice and Summary.

Summary here…..

The inclusion of more learning disability training given to student general nurses, health care support workers and qualified health professionals, who can expect to offer health care to people with learning disabilities.

This is needed to engender a greater understanding of the health care issues relating to those with a learning disability.  Furthermore, this is needed to promote a more caring attitude towards people with learning disabilities that can already be present within non learning disability health care specialities.  In addition to this, there should be an evaluation of all people with Down syndrome who live alone.  This evaluation should ascertain whether the lone person is living in suitable accommodation, and that the lone person is part of an adequate social network which has the best interest of the lone person in mind.

The placement of qualified learning disability nurses in all supported living settings where people with Down syndrome reside.

This is needed to ensure that the early symptoms of Alzheimer’s disease are recognised, which may be missed if unqualified or untrained staff are the only support staff who care for people with Down syndrome on a day to day basis.  Furthermore, as only qualified health professionals are allowed to carry out assessments relating to dementia in people with Down syndrome, the opportunity is afforded to carry out these assessments, at regular intervals.

A greater emphasis to be put on staff retention, by organisations that provide support for people with Down syndrome.

This needed to reduce staff turnover that may occur in these settings.  The need for client observation on a day to day, or year to year basis is required to form an informed opinion on the possible deterioration in a person’s memory or emotional, behavioural and social functioning.  Moreover, the familiarity of long serving staff will not facilitate or exacerbate confusion in the sufferer of Alzheimer’s disease, as new staff to the health care setting may do.

A greater emphasis to be put on referring possible cases of suspected Alzheimer’s disease to learning disability services as opposed to a general practitioner’s practice.

This emphasis is needed to ensure that people with Down syndrome who may have Alzheimer’s disease are met by health professionals with the appropriate understanding of the relevant issues.  However, this recommendation is not intended to take away the importance of people with Down syndrome accessing primary care services for complaints of a general nature.

·        Standardisation

·        Research; no distinction between severity of ld

References.

Alexander et al (2005) Nursing Practice: Hospital and Home, London: Churchill Livingstone.

Alzheimer’s Society. (2003) http://www.alzheimers.org.uk/Facts_about_dementia /What_is_dementia/info_fronto.htm  [Accessed 1 November 2007].

Alzheimer’s Society. (2007) http://www.alzheimers.org.uk /Facts_about_dementia /What_is_dementia/info_dementia.htm [Accessed 19 October 2007].

Alzheimer’s Society. (2007) http://www.alzheimers.org.uk/research/Cause/ plaques. htm [Accessed 30 October 2007].

Bigby, C. (2004) Ageing with a Lifelong Disability. Gateshead: Athenaeum.

British Institute of Learning Disabilities. (2007) http:/ /www.bild.org.uk /pdfs /05faqs /ld.pdf. [Accessed 19 October 2007].

Burt et al. (1998) Dementia in Adults with Down’s Syndrome: Diagnostic Challenges. American Journal on Mental Retardation, 103 (2): pp.130-145.

Cooper et al. (2004) People with Intellectual Disabilities: Their Health Needs Differ and Need to be Recognised and Met. British Medical Journal, 239, pp. 414-415.

Cooper et al. (2006) Improving the Health of People with Intellectual Disabilities: Outcomes of a Health Screening Programme after One Year. Journal of Intellectual Disability Research, 50 (9): pp. 667 – 677.

Coppus et al. (2006) Dementia and Mortality in Persons with Down Syndrome. Journal of Intellectual Disability Research, 50 (10): pp. 768 – 777.

Davidson et al. (2003) Mental Health, Intellectual Disabilities and the Aging Process. Oxford: Blackwell.

Deb et al. (2001) Practice Guidelines for the Assessment and Diagnosis of Mental Health Problems in Adults with an Intellectual Disability. Brighton: Pavilion.

Deb, S. (2003) Dementia in People with Intellectual Disability. Clinical Gerontology, 13: pp. 137-144.

Deb et al. (2007) Symptoms of Dementia among Adults with Down’s Syndrome: a Qualitative Study. Journal of Intellectual Disability Research, 51 (9): pp.726-39.

Department of Health. (1996) Continuing the Commitment: The Report of the Learning Disability Nursing Project, London: HMSO.

Department of Health. (2001) Seeking Consent: Working with People with Learning Disabilities, London: HMSO.

Department of Health. (2001a) Valuing People: A New Strategy for Learning Disability for the 21^st Century, London: HMSO.

Department of Health and Social Security. (1971) Better Services for the Mentally Handicapped, London: HMSO.

Department of Health and Social Security. (1979) Report of the Committee

of Enquiry into Mental Handicap Nursing and Care. Cmnd 7468,

London: HMSO.

Evenhuis, H. (1990) The Natural History of Dementia in Down Syndrome. Archives of Neurology, 47: pp. 263 – 267.

Finlayson et al. (2004) Learning disabilities: Enhancing Care for Adults. Practice Nurse, 28 (10): pp. 21-7.

Gates, R. (2003) Learning Disabilities: Toward Inclusion, London: Churchill Livingstone.

Harvey, B. (2004) Down’s Syndrome: a Biopsychosocial Perspective. Nursing Standard. 18 (30): pp. 43 – 5.

Hodges, J. (2003) http://news.bbc.co.uk/1/hi/health/3234714.stm [Accessed 4 November 2007].

Holland, A. (2000) Ageing and Learning Disability. British Journal of Psychiatry,  176: pp.26 - 31.

Holland, A. (2004) Down’s Syndrome and Alzheimer’s Disease: A Guide for Parents and Carers. Teddington: Down’s Syndrome Association.

Hutchinson, N. (1999) Association between Down’s syndrome and Alzheimer’s Disease: A Review of the Literature. Journal of Learning Disabilities for Nursing, Health and Social Care, 3 (4): pp. 194 – 203.

Janicki et al. (1995) Practice Guidelines for the Clinical Assessment and Care Management of Alzheimer and other Dementias among Adults with Mental Retardation. Washington: American Association.

Janicki, M. and Ansello, E. (2000) Community Supports for Aging Adults with Lifelong Disabilities. Baltimore: Brookes.

Jenkins, R. (2005) Older People with Learning Disabilities. Nursing Older People, 16 (10): pp. 30 – 34.

Jones, J and Kemp, K. (2007) Down Syndrome: Exploring the Knowledge, Attitudes and Practice of GP’s. Learning Disability Practice, 10 (8): pp. 18 – 21.

Jukes M. and Bollard M. (2003) Contemporary Learning Disability Practice. Salisbury: Quay.

Kerr, D. (1997) Down’s Syndrome and Dementia. Birmingham: Venture.

Kerr, D. and Wilkinson, H. (2005) In the Know: Implementing Good Practice. Brighton: Pavilion.

Margallo Lana et al. (2007) Fifteen Year Follow-up of 92 Hospitalised Adults with Down’s Syndrome; Incidence of Cognitive Decline, its Relationship to Age and Neuropathology, Journal of Intellectual Disability Research, 51 (6): pp. 463 – 477.

Martin et al. (1997) Health Gain Through Health Checks: Improving Access to Primary Health Care for People with Intellectual Disability. Journal of Intellectual Disability Research, 41 (5): pp. 401 – 408.

Martin et al. (2004) Oxford Dictionary of Nursing, Oxford: Oxford University Press.

Matthews, D. (2002) Learning Disabilities: the Need for Better Health Care. Nursing Standard, 16(39):pp. 40 – 1.

McFerran et al. (2004) Oxford Dictionary of Nursing. Oxford: Oxford University Press.

McQuillan, S. (2003) Adults with down’s Syndrome and Alzheimer’s Disease. Tizard Learning Disability Review. 8:  pp. 4-13.

Melville et al. (2005) Enhancing Primary Health Care Services for Adults with Intellectual Disabilities. Journal of Intellectual Disability Research, 49 (3): pp. 190-198.

Mitchell, D. (2000) Parallel stigma? Nurses and People with Learning

Disabilities. British Journal of Learning Disabilities, 28: pp. 78–81.

National Health Service. (2007a) http:/ /www.nhsdirect.nhs.uk/articles /article.aspx? articleId=136. [Accessed 19 October 2007].

National Health Service. (2007) http: //www.nhsdirect.nhs.uk /articles /article.aspx?articleId =127&sectionId=5  [Accessed 27 September 2007]

NHSE. (1999) Once a Day. London: HMSO.

Nightingale, C. (1992) Pointing the Way Ahead in Health Education for People with Learning Disabilities. Professional Nurse, 7(9): pp. 612 – 5.

Northway et al. (2006) Shaping the Future: A Vision for Learning Disability Nursing. United Kingdom: UK Learning Disability Consultant Nurse Network.

Oliver et al (1998) A Four Year Prospective Study of Age Related Cognitive Change in Adults with Down Syndrome. Psychological Medicine, 28: pp. 1365 – 1377.

Pary, R. (1992) Differential Diagnosis of Functional Decline in Down’s Syndrome. The Habilatative Mental Healthcare Newsletter. 11 (6): pp. 37- 41.

Patel et al. (1997) Psychiatric Morbidity in Older People with Moderate and Severe Learning Disability: The Prevalence Study. British Journal of Psychiatry, 163: pp. 481 – 491.

Phillips et al. (2004). General Practitioners’ Educational Needs in Intellectual Disability Health. Journal of Intellectual Disability Research, 48 (2): pp. 142-149.

Powrie, E. (2003) Primary Health Care Provision for Adults with a Learning Disability. Journal of Advanced Nursing, 42(4): pp. 413 - 423.

Prasher, V. (2005) Alzheimer’s Disease and Dementia in Down Syndrome and Intellectual Disabilities. Abingdon: Radcliffe.

Prasher, V. and Janicki, M. (2002) The Physical Health of People with Intellectual Difficulties. London: Blackwell.

Priest, H. and Gibbs, M. (2004) Mental Health Care for People with Learning Disabilities, London: Churchill Livingstone.

Raghavan, R. and Patel, P. (2005) Learning Disabilities and Mental Health: A Nursing Perspective, Oxford: Blackwell.

Scottish Executive. (2000) The Same as You: A Review of Services for People with Learning Disabilities, Edinburgh: Stationary Office.

The Down Syndrome Association. (2003) http://www.dsa-uk.com/txtOnly .aspx ?process=::_ROOTPATH_::DSA_detPressRelease.aspx::pr=19 [Accessed 26 October 2007]

Thomas, K. and Waights, S. (1999) He’ll Never join the Army. Teddington: The Down Syndrome Association.

Watchman (2003) Why Wait for Dementia. Journal of Learning Disabilities, 7 (3): pp. 221 – 230.

Watchman, K. (2007) Dementia and Down Syndrome: the Diagnosis and Support Needed. Learning Disability Practice, 10 (2): pp.10 - 14.

Welsh Assembly. (2001) Fulfilling the Promises, Cardiff: The Welsh Assembly.

Wilkinson, H and Janicki, M. (2002) The Edinburgh Principles and Accompanying Guidelines and Recommendations. Journal of Intellectual Disability Research, 46 (3): pp. 279 – 284.

Wilkinson, H. Kerr, D. Rae, C. Cunningham, C. (2004) Preparing to Support People with Learning Difficulties in Residential Settings when they Develop Dementia. Brighton: Pavilion.

World Health Organisation. (1948) http://www.who.int/suggestions/faq/en/ [Accessed 04 December 2006]

World_Health_Organisation. (1986)http://www.who.int/hpr/NPH/docs /ottawa_ charter_hp.pdf [Accessed 06 December 2006]

World Health Organisation (2007) http://www.who.int/mental_ health/ management /depression/definition/en/  [Accessed 27 September 2007]

Zigman et al. (1997) The Epidemiology of Alzheimer Disease in Intellectual Disability: Results and Recommendations from an International Conference. Journal of Intellectual Disability Research, 41 (1): pp. 76 – 80.