For Families and Carers : Alzheimer’s disease

Alzheimer’s Disease

Parents and carers often get in touch because they have heard that that everyone with Down’s syndrome will develop Alzheimer’s disease. It is important to be aware of the link between this particular condition and people with Down’s syndrome but it is certainly not inevitable that everyone with Down’s syndrome will develop it. There are a number of physical and mental health problems that singly, or in combination, can result in symptoms that are similar to those seen in Alzheimer’s disease. It is therefore really important to talk through your concerns with the person’s GP and ensure that other causes for changes in behaviour and ability are ruled out before Alzheimer’s is considered as a possibility. Although we have heard of a very small number of cases where people with Down’s syndrome have developed Alzheimer’s very early on in life, if you are noticing changes in a person before the age of 40 years the greater likelihood is that what you seeing is not as a result of Alzheimer’s.  If you have concerns about the person for whom you care, the first step is to talk to their GP.

What is Alzheimer’s disease?


Alzheimer’s disease is a type of dementia that gradually destroys brain cells, affecting a person’s memory and their ability to learn, make judgments, communicate and carry out basic daily activities. Alzheimer’s disease is characterized by a gradual decline that generally progresses through three stages: early, middle and late stage disease.

What is the difference between Dementia and Alzheimer’s disease?


Dementia is is not a disease in its own right. Dementia is an umbrella term covering more than 100 different conditions associated with a cognitive decline. One of these conditions is Alzheimer’s disease.

What is the connection between Down’s syndrome and Alzheimer’s disease?


Down’s syndrome occurs when an individual has a full or partial third copy of chromosome 21. Chromosome 21 plays a key role in the relationship between Down’s syndrome and Alzheimer’s disease as it carries a gene that produces one of the key proteins (APP gene – Amyloid precursor protein) involved with changes in the brain caused by Alzheimer’s. Additionally, scientists have located several genes on chromosome 21 that are involved in the ageing process and that contribute to the increased risk of Alzheimer’s disease. It is this unique property of chromosome 21 that makes the disease a more acute concern for people with Down’s syndrome than those with other forms of learning disability.

Is Alzheimer’s disease inevitable for people with Down’s syndrome?


Alzheimer’s disease is not inevitable in people with Down’s syndrome. While all people with Down’s syndrome are at risk, many adults with Down’s syndrome will not manifest the changes of Alzheimer’s disease in their lifetime. Although risk increases with each decade of life, at no point does it come close to reaching 100%. This is why it is especially important to be careful and thoughtful about assigning this diagnosis before looking at all other possible causes for why changes are taking place with ageing. Estimates show that Alzheimer’s disease affects about 30% of people with Down’s syndrome in their 50s. By their 60s, this number comes closer to 50%.

Can other conditions look like Alzheimer’s disease?


It is always important to first discount other reasons behind an individual’s decline before Alzheimer’s disease is considered as a possibility. This is sometimes referred to as a ‘differential diagnosis’.  If you notice a decline in abilities and/or loss of skills, deterioration in personality or behaviour or poor memory and/or confusion, you should consider the following:

  • Changes in sensory impairments (hearing and vision difficulties) which inhibit social engagement / lead to loss of skills;
  • Thyroid disorder (treatable)
  • Life changes e.g. reaction to a bereavement, changes in an individual’s day service / staff / support, retirement.
  • Depression (treatable, with the right interventions)
  • Menopause
  • Side effects of medications / drug interactions
  • Urinary infection (if sudden onset of confusion)
  • Poor Sleep
  • Vitamin B12 deficiency

 Since these are treatable, it is vitally important to have a full medical assessment at an early stage in order to rule them out.

What are the symptoms that may indicate Alzheimer’s disease?


Symptoms that may indicate the onset of Alzheimer’s disease:

  • Loss of interest in activities
  • Short-term memory loss
  • Withdrawal of spontaneous communication
  • Loss of amenability and sociability
  • Loss of domestic skills
  • Increase in wandering
  • Loss of road sense
  • Problems in unfamiliar places
  • Epileptic fits
  • Loss of comprehension
  • Confusion/Disorientation

Is it difficult to diagnosis Alzheimer’s disease in someone with Down’s syndrome?


Diagnosis of Alzheimer’s disease can be made with a high degree of certainty in situations where there is good and reliable information available about how someone has changed and how/she is now. Alzheimer’s disease affects a person’s cognitive abilities and his/her ability to understand task or communicate.  Whilst a decline in memory or the slow loss of specific abilities may be readily apparent in those without a pre-existing disability, it may be less easily noticed in people with Down’s syndrome. There are two main reasons for this. First, unless someone has known that person for some time and has observed the changes, the inability to do something is all too easily put down to his or her learning disability. The crucial question, in the case of an older person with Down’s syndrome, is has he/she been able to do this in the past and now cannot? If so, why has this change occurred? It can be more difficult to track long-term changes in a person with Down’s syndrome who is living in a residential home due to changes of staff, changes of residential home and the lack of long-term record keeping about the individuals’ skills. Secondly, loss of ability may go unnoticed if the person is leading a life where he/she is, for example, not expected to take part in household activities or do tasks that require good memory. The fact that someone’s memory may have deteriorated would under these circumstances go unnoticed.

What is a baseline assessment?


Alzheimer’s disease is suspected when there is a change or a series of changes seen in an individual as compared to their previous level of functioning. Thus, in order to observe change effectively, one must be informed about what the individual was capable of doing at his or her very best. This could be considered the individual’s “baseline.” The primary importance of having a good description and understanding of an individual’s baseline is so it can be used as a basis of comparison if changes are observed as the individual grows older. It is extremely helpful to record baseline information throughout adulthood – noting basic self-care skills, personal achievements, academic and employment milestones, talents, skills and hobbies. You can also ask your Community Learning Disability Team to arrange for a formal baseline assessment.

Down’s syndrome Specific Issues


Alzheimer’s disease in people with Down’s syndrome may present atypically with changes in behaviour and/or personality that can precede the full clinical picture of dementia by some years. Often, it is small changes in routine and the person’s usual activities of daily life that indicate the possible onset of Alzheimer’s disease.   Alzheimer’s disease in people with Down’s syndrome may be associated with the onset of seizures for the first time in that person’s life.

The middle and later course of Alzheimer’s disease in people with Down’s syndrome are similar in characteristics to those experienced by people in a similar stage of the disease but without pre-existing learning disabilities.

The time course of Alzheimer’s disease in people with Down’s syndrome has been reported to be more rapid than in the general population. Whether this is the case or not is uncertain as it may be a manifestation of the difficulties and delay in making an early diagnosis. By the time the diagnosis is made the dementia may already be advanced.

I suspect my relative may have Alzheimer’s disease, who do I turn to for help?


The first point of contact should usually be your GP or Community Team for People with Learning Disabilities. Diagnosis may take time.  It is not possible to make a diagnosis of Alzheimer’s disease from a single assessment. Diagnosis can only be made after careful consideration of a number of factors, including a detailed history, performance on assessments over a period of time and exclusion of all other possible reasons for change.

Are there any specific services for people with Down’s syndrome and Alzhemier’s disease?


The main source of local information and support for families will be from the multi-disciplinary Community Team for people with learning disabilities.  There are some local centres of expertise such as the Down’s syndrome and Dementia Mersey Care Learning Disabilities and Dementia Group. There are a relatively small but increasing number of care homes that provide services for people with learning disabilities and early Alzheimer’s disease. These may be found by using the search mechanism on the Care Quality Commission website.

Where should people with Down’s syndrome and Alzheimer’s disease live?


Preferred option: ‘Staying at home’ where the person can stay where they are currently living with appropriate supports adapted and provided. This means that the person stays with what is familiar in their long-term memory.

Compromise option:  ‘Moving to more specialist learning disability provision’ where the person has had to move from their current home, but moves into provision supported by learning disabilities services.

Least preferred option:  ‘Referral out of learning disability services’ where the person will be moved to services for older people, either residential or nursing.

By staying where they are, the person will stay with familiar people (family, peers, and familiar carers) and in an environment that they know. Although their needs will change as the dementia progresses, every effort should be made to maintain their home life. This may necessitate environmental changes and adaptations to support the person, increases in staffing levels and careful thinking about the supports required.

Where to get support after your relative has received a diagnosis of Alzheimer’s disease


As the needs of the person you care for change and the level of care he or she requires increases, it will be important to have any existing Needs Assessment reviewed by Social Services, or if they have never been assessed, to ask Social Services for a Needs Assessment of their needs. Social Services will then work closely with health services and other organisations where necessary to ensure that the appropriate level of care is given.  You can also request a Carer’s Assessment to look at your own needs.

Ageing and Dementia Training


The DSA can provide settings / services bespoke training on supporting people with Down’s syndrome and dementia. For more information about our ageing and dementia training click here

Research


Cambridge Intellectual and Developmental Disabilities Research Group are conducting a study based in partnership with the Down’s Syndrome Association (DSA) and the Wolfson Brain Imaging Centre (WIBC) Cambridge, to investigate the risk of dementia in people with Down’s syndrome (DS).

Read more about the research project here or watch this short film:

Publications


DSA

Down’s Syndrome and Dementia Workbook | DSA | Please call the DSA to order a copy.

Alzheimer’s Disease | DSA Health Series

Ageing and its Consequences | DSA Health Series

Other organisations

Dementia and People with Learning Disabilities: Guidance on the assessment, diagnosis, treatment and support of people with learning disabilities who develop dementia (Royal College of Psychiatrists)

Easy read factsheet: What is Dementia? (Alzheimer’s Society)

Supporting Derek – A practice development guide to support staff working with people who have a learning difficulty and dementia (Pavilion Publishing)

Support for Carers

Alzheimer’s Society

Dementia UK / Admiral Nursing

Carers UK