We have gathered advice on a range of topics that may be useful for people affected by rare dementias.
This is not intended to be a complete directory of questions and answers. It is based on the most common questions we receive from members at support group meetings and on some of the strategies that they have found helpful. If you have additional questions that are not mentioned below, please get in touch with us by emailing contact@raredementiasupport.org.
Whilst we have tried to ensure that all of the information on here is accurate, we cannot be held responsible for any errors or pieces of advice that you may disagree with. We strongly advise that you visit the appropriate organisations and resources for the most accurate and up-to-date information.
Causes and symptoms
What is PCA?
Posterior Cortical Atrophy (PCA) is a form of dementia predominantly affecting the processing of visual and spatial information. Common first signs and symptoms include difficulties with seeing what and where things are (such as when driving or reading).
PCA means ‘back of the brain shrinkage’ and it refers to the progressive loss of neural cells, starting in the occipital and parietal lobes. These changes can be caused by a number of different underlying disease processes. The majority of cases are caused by Alzheimer’s disease and PCA is sometimes called the ‘visual variant of Alzheimer’s disease’. PCA can also be caused by Lewy body dementia (LBD), corticobasal degeneration and other causes.
Symptoms usually begin before the age of 65 but it can take people a long time to get a diagnosis.
We feel uncertain about how PCA changes over time and how to plan certain aspects of our lives. What can we expect to happen next?
The experience of PCA differs from one person to the next, which makes it difficult to predict what will happen.
Together with people living with PCA and their carers, we have developed a document to help guide those affected by PCA, based on the established ‘7 stages of Alzheimer’s disease’ framework. This document should act as a guide only as people’s experiences vary, and people do not necessarily go through the stages in this order. View and download ‘The Stages of Posterior Cortical Atrophy’ document.
What do we know about what is happening in the brain in PCA and how can this explain some fluctuations in symptoms and abilities – for example sleeping a lot, and if someone can sometimes hold a conversation but at other times saying random phrases or repeating words or groups of words, and how best to respond and help?
PCA is a syndrome; it is a constellation of different symptoms and signs focused around vision. Causes are normally related to neurodegenerative dementias. The commonest cause is Alzheimer’s (probably about 70%) and then there are a variety of other conditions such as Dementia with Lewy Bodies. All of these illnesses have in common an accumulation of abnormal proteins, the loss of brain cells, and impaired communication between brain cells.
In terms of fluctuation, some causes of dementia which can cause PCA (notably Dementia with Lewy Bodies) are known to have very prominent fluctuations. This issue of fluctuations has been well known for decades, although the exact mechanism behind it is not known. It is likely to be due to fluctuations of the neurotransmitters, which are the brain chemicals that mediate signals within the brain.
However, people in general fluctuate in terms of attention, cognitive performance, irritability, etc. A degree of fluctuation is common to all of us and is affected by many things. For example, whether we are comfortable, hot, cold, well fed, stressed, etc. Like many things, this can become a little more extreme in dementia.
What is happening in the brain – in terms of changes in neurotransmitters and brain pathways – is extremely complicated. We have estimates of between 60 and 100 billion nerve cells in the brain and the tools we have to understand those are fairly blunt. So we don’t know exactly what causes fluctuations. But it is likely to be a combination of how proteins are disrupting the normal brain circuitry and how this is influenced by changes in brain chemistry. However, we should not overlook the impact of external stimuli and how they are modulated.
It can be a mistake to think of things as being exclusively psychological and psychiatric or neurological symptoms. Just because something is going on in the brain, doesn’t mean that changes in people’s everyday physical surroundings and social interactions won’t affect their day-to-day functioning.
In relation to language-related symptoms, even though PCA is known as ‘the visual dementia’, sometimes language symptoms can be the earliest thing people notice. For example, the tip of the tongue phenomenon (difficulty retrieving vocabulary) or mixing up certain sounds e.g. b and p.
Repetitive speaking is also consistent with more intermediate stages of PCA. This may also overlap with people perseverating (repeating a word, phrase or gesture without an ongoing occasion or rationale for that behaviour).
When we live with severe cognitive difficulties our way of interacting and communicating with the world around us changes because our abilities change too.
When we have difficulties choosing the right words to express ourselves, we may end up picking other words that are not an accurate representation of what we want to say but are somehow connected. For instance, if I want to say that I spent the morning having a nice walk in the countryside, I may not be able to find the right words to explain myself and just say that I saw a river and mountain and trees. Even if there were no mountains or trees, they are things related to the countryside and this may be my way to explain my experience.
In addition, communication is not only language. All our behaviour is communication and we can sometimes get relevant clues by observing behaviour too.
Some tips to try and find out what those words or groups of words might mean:
– Pay attention to the stress in a person’s voice: are they anxious, angry, excited?
– Pay attention to body language when the person is speaking: where are they looking? What are they doing with their hands? Maybe they are using gestures that may give you a clue as to what they mean.
– What else is happening at the times when those words are repeated? For example, if you are having lunch it may have something to do with the food.
– As a carer, you probably know the person very well and have many shared memories together– can you link those groups of words with something that you know about them; about their identity, their childhood, their job or the things that they like or are important to them?
– If using all of the above you manage to get a rough idea of the theme the words revolve around, you may start to understand more of what the person wants to say by asking simple questions that they can respond to with yes or no.
Sometimes this may work and sometimes it may not. Do not despair when the latter happens. By doing this, you are training your communication skills and you might gain in confidence and see an increase in your ability to do this over time.
The nurses, and GP, have said seizures are normal in people living with a dementia. Do you have any experience of this from a PCA perspective?
Seizures are not normal but they can be part of the illness. The brain is effectively a highly-tuned and sophisticated electrical circuit. If some of the electrical wires get disrupted as a result of accumulation of abnormal proteins, the breakdown of normal brain networks or changes in brain chemistry, then there is always the enhanced potential of seizures, which are basically little short circuits within the brain. Seizures are certainly not ubiquitous; not everyone with dementia will have them but, as all forms of dementia become more advanced, seizures become more common. Seizures in the early stages are unusual and should be raised with your doctor to make sure there is not something else going on. It is always worth flagging seizures with a health professional even in later stages.
Practical implications relate in particular to driving. However, more pertinently, seizures are very distressing for those who experience and witness them and can relate to impairments in cognition for a little period afterwards. It is important to understand what to do when they happen – for example don’t put things in people’s mouths and, generally speaking, leaving people alone is sensible. Seizures that self-terminate within a minute or two won’t need any further immediate medical intervention but if they go on any longer then you should call an ambulance (more information on the NHS website here and in this St. John’s Ambulance video). Everyone who is having seizures should be assessed by a doctor.
Many medications can be helpful in preventing seizures but, as with many cases, we have to consider the pros and cons of treatments. We need to take a very holistic view about how this impacts the individual, family and day-to-day living.
So, in summary, seizures can be part of the process but certainly not an inevitable part. If seizures do occur, you need to raise it with your doctor and try and make a sort of personalised plan on how to manage them depending on the situation.
Has there been any more info about the causes of PCA?
The term PCA was first coined in a report in 1988. Initially, there was some back and forth about whether PCA was something separate to Alzheimer’s disease, whether it was equivalent to an atypical form of Alzheimer’s disease, or whether it represented its own discrete entity but it could be caused by different things.
The most common cause of PCA is the same pathology as Alzheimer’s disease, but PCA symptoms may arise from other underlying causes, such as Lewy Body pathology, Corticalbasal Degeneration (CBD) and some instances of someone having a rare mutation causing PCA-like symptoms. When we think about causes we are often trying to understand the underlying pathology that is causing PCA. However, you could have the same underlying pathology causing different symptoms.
A key question is why are there certain factors that predispose someone to have Alzheimer’s disease but not have memory symptoms with some regions relatively spared and preserved? What we hope to do is to understand whether we can perhaps influence such factors that can provide a catalyst for any therapeutic strategies.
Has there been any more information about the longevity of PCA?
There are limited longitudinal studies of PCA which have been largely designed to evaluate changes in cognition based on MRI scans over several years. While people with early-onset Alzheimer’s disease (the most common cause of PCA) may have a tendency towards faster disease progression than those with later-onset Alzheimer’s disease, some people with PCA have a protracted course extending over more than a decade.
Can someone address left or right spatial neglect and yet not be paralysed on the side?
If I understand correctly and the question is about whether someone might exhibit particular difficulties perceiving, or locating objects or body parts on one side more than the other, without paralysis, then yes this is correct. There are varying reports of the exact prevalence of neglect in people with PCA, but it has been documented in a number of individuals. There is some rehabilitative literature available regarding techniques to manage neglect based mainly on working with stroke sufferers.
I have had very vivid dreams for a long time, is this normal?
Vivid dreams are not at all uncommon in people with dementia. They can relate to the illness itself disrupting the normal sleep pattern or can be associated with some medications, e.g. cholinesterase inhibitors such as Donepezil. If a medication side-effect is suspected, then sometimes switching taking medications from the evening to the morning can help; this should be discussed with the prescribing doctor before any changes are made.
What is myoclonus and how does myoclonus link to PCA?
Myoclonus refers to brief, involuntary jerks or twitches. Myoclonus is present in some people with PCA and not others. A substantial proportion of people with young-onset Alzheimer’s disease (diagnosed under the age of 65) have myoclonus. If you imagine the brain to be a very complex electrical circuit, and this circuit goes awry, this could lead to brain signals malfunctioning. This in turn can lead to things such as epilepsy and myoclonus.
Some people with advanced Alzheimer’s disease can experience seizures. There are medications which can be used to manage seizures such as Kepra and, as the problem advances, the dosage can be increased. There are side effects, as with any medication, and the balance between risk and benefit should be considered on a case-by-case basis. Keeping medication at a minimum is important. If the jerks do not bother someone, there may not be a need for them to take medication.
Can I have PCA and Lewy body dementia?
PCA has a number of causes. A significant number of people with PCA also have Lewy body dementia (LBD), though it is more common for people with PCA to have an overlap with Alzheimer’s disease.
LBD is a form of dementia closely related to Parkinson’s disease as both Parkinson’s disease and LBD are caused by a build up of proteins called ‘Lewy bodies’ in the brain. The symptoms that people with LBD will experience depend on which part of the brain is affected, and range from issues with movements to visual problems and sometimes hallucinations.
Sometimes the neurologist may ask people with PCA for specialised brain scans, such as a DAT scan, which can be helpful for identifying if Lewy body dementia is the underlying cause of their PCA.
What are Lewy bodies?
Lewy bodies are protein deposits that can be found in the brain of people with LBD and with Parkinson’s disease. Scientists still don’t know exactly how they cause dementia but they may lead to a loss of connections between brain cells. The symptoms that people with LBD will experience depend on where the Lewy bodies are found. When Lewy bodies are mostly found in the deeper parts of the brain, they lead to problems with movements and Parkinson’s disease. When they are found in the outer parts of the brain they tend to lead to LBD.
What are the common symptoms of LBD?
LBD affects different people in different ways. It often causes problems with concentrating and staying alert. Although day to day memory can also be affected, it is less of a problem than in patients with Alzheimer’s disease. Patients and their families often notice that thinking and memory problems vary from day to day or even hour to hour.
LBD can also cause visual hallucinations, such as seeing animals or people that are not really there. In many people, these are not troublesome, and would only need treatment if they become distressing. People with LBD can also sometimes experience delusions which means that they believe ideas which are not true. People with LBD may notice that their movements are slower or stiffer. Some people have problems with balance and may be more prone to falling. Other symptoms can include problems with bladder or bowel function, and sleep troubles. LBD is a progressive condition and symptoms generally worsen over time. The rate at which symptoms progress varies from person to person.
What is the difference between Lewy body disease and LBD?
Lewy body disease is an umbrella term for two closely related clinical diagnoses: dementia with Parkinson’s disease and dementia with Lewy bodies (LBD). The main difference between the two is that in LBD cognitive problems such as problems with concentration develop early on in the disease, whilst in dementia with Parkinson’s disease, cognitive problems can happen more than a year after problems with movement develop.
Does LBD run in families?
LBD is thought to be caused by a complex interaction of genetic factors and environmental factors. Mutations in many genes, such as genes called Apoε4 and GBA, have been linked to Parkinson’s disease and LBD. However no more than 2% of patients with Parkinson’s, and likely even fewer with LBD, carry a gene with a disease-causing mutation. Overall, and particularly where there is no family history, most people with LBD will not have a hereditary illness.
What is the prognosis for LBD?
Prognosis is a term referring to a medical professional’s opinion on the likely course of a medical condition. It is difficult to give an accurate prognosIs for LBD as it can be extremely variable from person to person. Some evidence suggests that the rate of progression in later stages may mirror the rate at which early symptoms develop. Currently there is no reliable method to predict life expectancy or rate of progression in a person with LBD. New research on treatments may also alter prognosis.
What should I expect in the later stages of LBD? How can I manage this?
As with other types of dementia, people with LBD will experience different symptoms at different times, and it is very difficult to predict what will occur and when. If you would like to talk to somebody about how the issues raised here have affected you, please get in touch.
In later stages people with LBD will experience most of the symptoms described previously. Problems with memory and attention can become more noticeable and people may need help with many or most daily tasks. In later stages, hallucinations and delusions can become distressing for the person with LBD and their family, particularly if trying to suggest to a person with LBD that what they are seeing or believing is not true. Treatments for hallucinations and delusions can sometimes be used. Making sure that glasses and hearing aids are checked also helps.
People with LBD can sometimes behave in a challenging manner (agitation or aggression) which can be difficult to manage. This is similar to other forms of dementias and carers and professionals should try to avoid triggers, such as excessive noise or pain. Motor symptoms can also become worse with time and many people with LBD are prone to falling in late stages. Medication can be used in certain cases. Physiotherapy and occupational therapy can also be very helpful in managing problems with movement. Speech and language therapy can be used if problems with swallowing and speech develop. More information can be found on our Support in later stages of LBD page.
Diagnosis
What tools are used to diagnose PCA?
PCA is a condition with a number of symptoms and signs that vary significantly. The specific diagnosis depends on the context – sometimes symptoms are quite consistent, sometimes less so. The neurologist will want to conduct several assessments to determine whether the issues you are presenting, such as visual-spatial issues, are caused by PCA or something else.
The first thing they will do is to ask you for a detailed history of your symptoms – what have you noticed that’s difficult, and when did it start? Ideally, they’ll also ask the same questions of somebody who you know well, and who can accompany you to that first appointment. They may ask you to have an MRI brain scan so that they can see a picture of what’s happening in your brain. They will often ask for you to have cognitive testing with a neuropsychologist so as to understand a bit more about how exactly your visual, spatial or perceptual abilities are affected.
Sometimes, they might ask you to have a lumbar puncture or “spinal tap”. This can be really helpful, particularly in identifying whether proteins that can cause PCA, such as Alzheimer proteins, are circulating in the fluid. It’s important to know about this difference because there are some medications that can help to manage symptoms in Alzheimer’s disease that may be worth trying in PCA, where Alzheimer proteins are the cause.
Talking about your diagnosis
Who should we tell about the diagnosis?
This is a question that many people from our PCA support groups have struggled with. Some people have said that they preferred to just tell the friends and family they were closest to, and then slowly tell more people outside of their immediate circle, whilst others said that it was better to make it known to as many people as possible to avoid confusion. Some people choose to tell the people they think will understand the most, as PCA can be really difficult to explain.
Some of our members have described how people they once regarded as good friends no longer stay in touch. Identifying why this was the case was difficult. People may find it uncomfortable to deal with a person who has dementia and, while they can still care about a person, they find it easier to withdraw. However, many people have shared positive stories of friends who have been particularly supportive. Members talked about how relationships changed, but that these relationships can still be positive.
In terms of telling strangers about the diagnosis, partners and loved ones of people with PCA have reported taking up the role of being an ‘ambassador’ for the person with a diagnosis. For example if they are having difficulty seeing someone’s hand to shake it, or taking a while to count change, their ambassador can explain that this is because they have a type of dementia that affects their vision.
We have included some additional advice on ‘Talking about your diagnosis’ for people living with all forms of dementia on our General Advice page. We also created a short animation, narrated by people living with PCA that people have found extremely useful in explaining their experience. Watch the film ‘Do I see what you see?’ on YouTube.
How do I educate myself and others about PCA?
Lots of members talk about the confusion around PCA. As it is a rare condition, and it tends to affect people at an earlier age than Alzheimer’s disease, not many people will have heard of it before. People are often in their mid 50s or early 60s at the time of their first symptoms (although it can also affect older people).
There is a wealth of information on the internet. Sometimes, this can seem overwhelming, and members in the past have discussed the importance of ‘safe googling’ and using websites that you can trust such as Rare Dementia Support or Alzheimer’s Research UK (also see their helpful booklet about what PCA is). If you want to ask any questions you can always get in touch with the Rare Dementia support team. We are always happy to speak to people living with PCA and their friends and family. We can also put information packs in the post if this would be helpful.
To raise awareness and increase understanding about PCA, Director Simon Ball created a short animation for us called ‘Do I see what you see?’, which is narrated by people living with PCA. Many people have found this extremely useful in educating their family, friends and professionals about PCA. You can watch the film on YouTube.
My doctor has never heard of PCA. Can I see a doctor who understands PCA?
Unfortunately, because PCA is so rare, it is often the case that medical professionals will never have met anybody else living with this condition.This means that people can sometimes be given misleading or inaccurate information about their diagnosis.
It is important to remember that it is entirely within your rights to ask for a referral to see a neurologist elsewhere. For example, referrals to the Cognitive Disorders Clinic in Queen Square are made via a GP, and the information they would need to make a referral can be found on the UCLH website.
Management strategies
We’ve found it difficult to get assistance when we are out and about. How can I quickly let people know I may need help?
Not many people know about PCA and it is not always immediately obvious when a person living with PCA needs assistance. We can provide you with PCA ID cards to quickly explain your diagnosis and possible needs to others, If you would like us to send you some ID cards, please get in touch with the Rare Dementia Support team.
A symbol cane has been described as useful for making others aware of visual difficulties. You can find out more on the Royal National Institute of Blind People website.
To ensure your condition is easily identified you can also buy a badge online saying ‘please be patient I have dementia’, which has made a difference for some people.
Should people with PCA registered as ‘sight impaired’ be entitled to all the same concessions and benefits as someone who is registered blind?
There is one published recommendation from a neuro-ophthalmologist that states that people with PCA should qualify as being severely sight impaired, which is equivalent to being considered blind. This is with the rationale that people might be getting visual information from the eyes to the back of the brain but the brain has problems making sense of them.
People with PCA may also have difficulty making a mental map of a place. Jon and Seb, amongst others, are putting together training for people on the eye health site, and we are starting to see more people with PCA getting registered as sight impaired. Historically, this was a very contentious issue, and although we are starting to see improvements, there is still a way to go.
The Royal National Institute for the Blind (RNIB) has been contacted for input and they mention that an enhanced rate for PIP is not based on registration status; it is down to mobility and daily living, including wayfinding difficulties. Mentioning things like getting lost and the risk to safety may be helpful in getting people to understand. Remember, you can always seek a second opinion. Also see the RNIB booklet here about benefits, concessions and registration which explains differences in entitlement according to whether the person is registered as sight impaired or severely sight impaired.
You are the experts. Sometimes it is the situation of ‘he who shouts loudest gets heard’. We at RDS are happy to support you with that. Go in with a list of what you can and cannot do; really list out what the issues are as sometimes this can influence people and help them to take you more seriously.
Speaking from the experience of being a carer in this situation – don’t accept no for an answer, really shout. The people that are coming to see you more than likely won’t have a good understanding of PCA, so you really have to stand up for yourself, which is difficult, but the louder you shout the better off you will be.
Many times people get denied access and want to give up, but often on the second time appeal the success rate is much higher. When applying, expect to be turned down the first time, and then go back and appeal.
Comment from RDS member regarding benefits in Scotland: No free TV licence but free bus pass and free rail travel in Scotland.
So many of the resources available for support during this time in particular but at other times too, are visual or rely on visual skills/abilities. As vision is an early victim in PCA we need to address this.
Absolutely, and while there is still a lot of work to be done, please be reassured this isn’t an unrecognised need. This is reflected in conversations Dr Keir Yong and others are having with the College of Optometrists in terms of barriers to service use. Keir has also raised this at the Vision UK Dementia and Sight Loss Committee which has representatives from professional bodies for psychiatrists, nurses and neurologists and eye health professionals, as well as links to memory clinics.
Support resources which are also available in audio format may be particularly helpful at the moment, although we appreciate that people with PCA can have difficulties with getting these started and that not all steps of the process are always completely accessible or able to be completed independently. We have created some short video clips about managing during lockdown here.
The Thomas Pocklington Trust has also put together a list of accessible apps here to support people who are partially sighted or blind to stay connected and informed.
It may be especially difficult at the moment to do the usual activities which you enjoy, and which perhaps don’t rely so heavily on visual skills and abilities. We have collated a list of Arts and Culture resources and experiences which can be enjoyed from home here. Some of those which may be less reliant on visual skills or may still be enjoyable without the visual component include the following.
Make a favourite music playlist using one of the below:
– Playlist for Life
– BBC Music Memories
Or join an online singing group such as The Sofa Singers here.
Classical music:
The Philharmonie Berlin is closed but has opened its digital library of performances containing more than 600 shows. Use the code BERLINPHIL to get 30-day access to the orchestra’s stunning work (look for performances conducted by Sir Simon Rattle, the orchestra’s principal for 16 years).
The Melbourne Symphony Orchestra is live-streaming its performances on YouTube, as it is closed to the public until June 30. While the 7 p.m. AEDT (or 4 a.m. EDT) showtimes may not be an ideal time to watch a symphony performance, you can rewatch the show later on the orchestra’s YouTube channel. So far, they’ve hosted performances of Rimsky-Korsakov’s Scheherazade, with plans for more throughout the closure. More information can be found here.
BBC iPlayer: Audio description is a voiceover which uses the gaps in dialogue to describe the main visual elements happening on the screen, and can help viewers with visual impairments to follow what is going on. You can search for all programmes on BBC iPlayer which have audio description enabled here.
When my husband was registered blind he received initial support from a local Vista group but they could not cope with his limited ability to learn or loss of spatial awareness. What resources are available for this transition?
One consequence of people now being registered as partially sighted or blind is that they are getting more support, but some people mention this support seems predominantly aimed at people experiencing a specific visual problem, without the changes in spatial awareness or memory that often arise in PCA.
The Vista website mentions rehabilitation officers and support workers. We have put out training for Occupational Therapists and Optometrists and presented to Visual Rehab Officers, but training is only available to members of the Royal College of Occupational Therapists/College of Optometrists.
Two RDS members have made free e-learning resources here on understanding and managing PCA that have been updated recently. These signpost to the aforementioned resources, as well as freely available ones such as the MOOC online course ‘The Many Faces of Dementia’ and the UCL/Vision UK conference from 2018.
We are happy to be contacted by Vista, and to be put in touch with other health professionals to provide information and training – please contact us to discuss at contact@raredementiasupport.org.
Several carers have reported showing the short animated film ‘Do I See What You See’ here to different health and care professionals to help explain the visual symptoms of PCA.
Comment from RDS member: I would suggest keeping in touch with the RNIB. They helped us a lot before we knew the cause of the vision problems. They have Rehab Officers for Visually Impaired people (ROVIs). Ours didn’t know about PCA and the RNIB office at St Pancras didn’t have any leaflets about PCA at the time. You may already know this, but it would have hastened the diagnosis if they’d recognised the symptoms. The Ophthalmology Clinic we attended also appeared not to recognise what the visual symptoms might mean.
I am sorry to hear of the delays in diagnosis you experienced. In addition to the training we have delivered via the College of Optometrists, I presented to the London Visual Impairment Forum in 2019 which was mainly made up of ROVIs who were eager to learn more about PCA. Seb and I are involved in the UK Dementia and Sight Loss Committee to raise awareness of how dementia may cause sight loss; this committee has representatives from professional bodies for psychiatrists, nurses, neurologists and eye health professionals.
Additionally, you can find some home safety tips and recommendations for people with dementia experiencing visual dysfunction here, in case this is helpful.
Comment from RDS member: the Ophthalmologist who tested my husband and found him Severely Sight Impaired said he was grateful for the information on PCA. He would otherwise have thought he had had a stroke.
We can provide information specific to Ophthalmologists about assessing and recognising PCA – please contact us if this would be helpful at contact@raredementiasupport.org.
Is there anything you can do to help with eyesight deterioration and spatial awareness for PCA (newly diagnosed)?
It might be of interest to look at materials here from the UCL & Vision UK Dementia and Sight Loss Conference, June 2018, which was linked to one of the PCA Support Group meetings. This features talks on environmental adaptations (Videos 5 and 15) and assistive technology (Videos 13 and 14).
You can ask for a referral to an Occupational Therapist who may be able to advise on adapting activities to compensate for changes in sight and spatial awareness. If you register as partially sighted, you may be eligible for some support and referral to a Visual Rehabilitation Officer. It is worth emphasising to professionals, particularly those who are not familiar with PCA, that you are experiencing both sight difficulties and changes in spatial awareness.
Comment from an RDS member: It can be helpful to carry a cane when you are out so other people know that you cannot see.
Diagnosis
How is LBD diagnosed?
In the early stages it can be difficult to make a diagnosis of Lewy body dementia (LBD) and people can be diagnosed as having Alzheimer’s disease or Parkinson’s disease. The neurologist will want to conduct several assessments to determine whether the symptoms are caused by LBD or something else. The first thing they will do is to ask you for a detailed history of what’s been happening – what have you noticed that’s difficult, and when did it start? Ideally, they’ll ask the same questions of somebody who you know well and who can accompany you to your first appointment.
They may ask you to have an MRI brain scan so that they can see a picture of what’s happening inside the brain. If the neurologist feels that LBD may be the likely cause of the symptoms, they may also ask for a DAT scan, which looks at changes is the amount of dopamine in the brain, as dopamine has been shown to be reduced in people with LBD. In some circumstances, other tests such as a lumbar puncture (spinal tap) may also be needed.
Diagnosis
Do I need a formal diagnosis of FTD, rather than ‘dementia’ or potential misdiagnosis of Alzheimer’s, to access the help we need?
It depends what help you are seeking, but most disability benefits and care services assess how a condition affects you, and not what diagnosis you have. Therefore, it is the symptoms that are most relevant to eligibility, and not the ‘label’ those symptoms are given.
However, it is important to have the correct formal diagnosis to ensure the person’s needs are being met, and to assist with care planning.
Who should we tell about the diagnosis?
This is something that lots of people have talked about struggling with in the past. Some people choose who to tell based on who they think will understand – rare dementias can be a difficult subject to explain. Other people have described how people who were once regarded as good friends would withdraw and not stay in touch. Identifying ‘why’ this is the case was difficult. Sometimes, people find it uncomfortable to deal with a person who has dementia, but this does not mean they no longer care. Many of our members have shared positive stories of friends and family who have continued to engage and communicate with them. They have talked about how relationships have changed, but that these relationships can still be positive.
Many people have found it difficult to choose who to tell about their diagnosis. Some said that they preferred to just tell the friends and family they were closest to, whilst others said that it was better to tell as many people as possible to avoid confusion. Some members talked about telling close family initially, and then slowly telling more people outside of your immediate circle of friends and family.
In terms of telling strangers about a LBD diagnosis, partners and loved ones have reported taking up the role of being an ‘ambassador’ for the person with a diagnosis. For example if they aren’t answering a question or acting in a slightly odd way, their ambassador can explain that they are not being rude, and that they have a dementia that causes this behaviour. We have included some additional advice on ‘Talking about your diagnosis’ for people living with all forms of dementia on our General Advice page.
I have noticed that my partner with LBD has become increasingly anxious after their diagnosis. Is this common? What can I do to help manage this?
Sometimes traits that were already present can be heightened when people have LBD. So, if they have always been anxious, this can increase with LBD. Equally, people with LBD that had not previously experienced anxiety can develop it as a result of LBD. It is also not uncommon for someone’s anxiety to increase as a response to receiving a diagnosis and the changes they may notice in their behaviour.
When a person with LBD is experiencing anxiety, it is important to validate the emotions that they are feeling, and to offer help. The carer can often play a key role in motivation and support. While medication may not be appropriate for everyone, both carers and people living with LBD have spoken about how antidepressants can make a very positive difference if people are really struggling. It is important to note that anxiety and depression are treatable parts of LBD, and it is just about finding the right treatment.
Who do I contact for additional support with my LBD symptoms?
There are a number of different health and social care professionals that should be involved in supporting a person living with LBD. These include (but are not limited to):
Speech and Language Therapists (SALT):
– Can assist with managing speech and swallowing difficulties
– Getting a referral to a SaLT through the NHS or local authority can sometimes depend on the area that you live in, and they may only be funded to provide support for swallowing difficulties and not for language problems. You can also pay privately for speech and language therapy
Dietitians
– Qualified health professionals who work with people with dietary needs due to health conditions – you can ask your GP for a referral
– In LBD, food preferences often change, such as starting to prefer sugary and sweet foods. It’s important to make sure people are getting enough fluids, as dehydration is a common issue, and can increase or cause symptoms of delirium, as well as increasing the risk of urinary tract infections.
Find out more about available dietician and nutritionist services on the NHS website.
Physiotherapists
– Provide education and advice on movement, exercise and manual therapy
– Can be accessed with a referral from the GP, or can be engaged privately
Find out more about Physiotherapy on the NHS website.
Occupational Therapists
– Provide assistance with everyday tasks and are important in terms of identifying areas where help is needed
– Referred by health professional, or social services/local authority. They can also be engaged through private healthcare
Find out more about Occupational Therapy on the NHS website.
Admiral Nurses
Provide specialist dementia support to people living with dementia and their families, including:
– One-to-one support
– Practical solutions to overcome challenges
– Information provision
– Help to get additional care and support
– Expert guidance, for example the best ways to prevent and manage distress
Find out more about Admiral Nurses on the Dementia UK website. People can self-refer and the service is free. The service is area dependent. Find out if there is one in your local area on the Dementia UK Find a local Admiral Nurse page.
Continence Clinics
– People with LBD can start to have issues with bladder and bowel function.
– You can get support with incontinence issues through an NHS continence service, or your GP may refer you to a district nurse. You do not always need a referral from your GP to be seen at a continence clinic
You can find out more about incontinence symptoms and available support and products to help manage this on the NHS website.
Falls Clinic
– Clinic and referral process is area dependent
– It may be helpful to ask your GP about your local service. For example: Bromley Falls and Fracture prevention service or Richmond Falls and Bone Health Service.
What should I do if I notice a sudden change in my partner with LBD?
There are many reasons why there can be sudden changes in behaviour in a person with LBD. It could be because they have a secondary problem, for example a urinary tract infection (UTI). Alternatively it could be related to the LBD. It is essential for them to see their GP to help uncover what could be behind the change.
Strategies and support
How can I make it easier to get around at home?
There are many ways to help guide someone’s way within the home when they have PCA. It may involve some trial and error to see what works specifically for you. Some strategies our members have found helpful include using bamboo screens, or arranging furniture in a way that helps to make pathways clear and minimises confusion by not having too many options. Other adaptations mentioned included high ridged plates, grab rails and raised seating areas. People often found stair lifts tricky, including the sensation of going up and down as well as the transfer into the seat. An occupational therapy (OT) assessment can be helpful, to see what equipment could be useful in the home. Find out more about occupational therapy on the NHS website.
An even spread of lighting and minimal shadows as far as possible can be helpful. LEDs and down-lighters were recommended by some of our members. Sensor lights used to guide the way around the home, such as down the hallway or to the bathroom, have also been recommended. You might be interested in a factsheet about home safety tips and recommendations for people with dementia-related visual problems. View and download the ‘Home Safety Tips & Recommendations’ factsheet.
I have a diagnosis of LBD and like to drink alcohol occasionally. Will this make my symptoms worse?
Drinking alcohol can affect sleep, which is often already affected in people with LBD, so this may be something to consider if sleep disturbance is a significant issue. You may also need to re-assess alcohol consumption if it is causing physical issues, for example, balance and coordination. However, there is no reason to cut alcohol out completely if someone is drinking a small amount, especially if it brings enjoyment.
I find it hard to find my way when I get up in the night. What can I use to guide me?
This is something lots of people have talked about having difficulties with. People have found it useful to plug in sensor lights or night lights for when they go to the bathroom and a motion-activated light for the toilet bowl. Glow in the dark stickers to help with finding light switches at night have also been helpful.
I worry about falling over and hurting myself. What can I do to stay safe?
If you are concerned about falls you can get an assessment and advice from a falls risk screening. This will help to identify ways for you to stay safe and lower the risk of falling, allowing you to continue with the activities you find important. The clinic and referral process is area dependent, so it may be helpful to ask your GP about your local service. Examples of these services include the Bromley Falls and Fracture prevention service and the Richmond Falls and Bone Health Service.
I find it hard to put down and pick up a glass or pick up food with cutlery. Do other people find this hard and how can I make it easier?
Mealtimes can be tricky for lots of people living with PCA. Strategies that have helped people with PCA include:
– Using a spoon rather than a fork.
– Picking easier or softer foods such as rice
– Using a steak knife to cut all food
– Using a pasta dish with a curved edge rather than plate so the food doesn’t spill off
– Clipping on a lipped edge for a plate
– Using a bowl which curves inwards at the top to help with spillages when eating soup, for example
We are going to move house and I feel anxious about my partner adapting to the new surroundings. What can I do to make the move straight-forward and minimise distress as much as possible?
People highlighted that familiarity was key and to try and make their new house’s interior as similar as possible to the previous one. For example, having the same paint colours or furniture. It is important to remember that people living with PCA experience symptoms differently and, while some will adapt to new surroundings, this is often easier in the earlier stages.
Is there anything we can do to make cooking safer?
Lots of people with PCA can find cooking increasingly difficult. SGN offer a free safety device to help vulnerable people keep gas safe in their own homes called the locking cooker valve. This device prevents someone from unintentionally turning on or leaving on a gas cooker, and allows someone with PCA to retain their independence whilst remaining safe at home. SGN also have a priority services register for people in need of priority support in a gas emergency situation. Find out more about what is available to make cooking safer on the SGN website.
Can I use a white cane?
People have found using a white cane very helpful and often use a ‘sweeping technique’ to let people know to get out of the way! Using one with the ball on the end (‘ball cane’), which makes a noise when it rolls along, tells other people that you can’t see and can make people feel more confident. It can be useful on stairs as well to help with judging distances. Members have noted that it can take a while to get used to and have had mixed success with the shorter cane. Several members have found that using a white symbol cane to let others know of their visual problems when out and about is useful, particularly foldable ones which can be used at a number of lengths. You can search for a cane suited to your needs on the Royal National Institute of Blind People website.
Are alarms and trackers useful for people with PCA?
Trackers and alarms have proved useful for people living with PCA and their families and carers. However, they will not be useful for everyone. There are lots of options available. Most mobile phones (iPhone and Androids) have tracking ability through GPS which some have found very useful. For alarms, one member had an alarm fitted to the bottom of their door, which goes off if the person with PCA is walking around at night. You should contact your local council or social services about setting up an alarm and details of who should be contacted if an alarm goes off.
What other technologies are available for people living with PCA and their carers?
Smart-home voice assistants like Alexa and Siri have been recommended for people with PCA. The Amazon Echo has been recommended for its handy features that allow people to, among other things: ask about the weather; ask for the time and date; add items to a shopping list; access entertainment such as quizzes; and turn lights off and on. Doro has been recommended for easy-to-use phones and they now have a range of smart phones available with useful additional features. For example, they can sync up with the Amazon Echo app. Prices for these smart phones are around £200 and you can find out more on the Doro website.
Members have also found audiobooks and wireless earphones helpful as well as a number of apps. You can access the following apps at:
– Jointly app: An app designed by carers for carers. This is particularly useful for storing information (such as medication, photos, appointment times) in a way that’s accessible to a whole group such as a family or care team.
– The armchair gallery app: Designed for people living with dementia who struggle to get around and access arts and culture services such as museums and galleries. The app brings arts and culture to the person when they are at home, including artwork and artefacts from a range of collections across the UK.
People mentioned designating ‘In case of emergency’ contacts in their mobile phone contact list (saving them as ‘ICE [name]’) and also the importance of completing ‘Medical ID’ information on their mobile phone so that emergency services can access the information if they need to. Medical ID wristbands can also be helpful and reassuring.
Treatment
What treatments are currently being investigated in trials or planned for the future?
There are multiple different avenues being investigated, including gene therapies and drugs to try to decrease the amount of abnormal protein in the brain. Many of these will be coming to trials in the next couple of years. See the FTD talk link for more information: https://www.ftdtalk.org/.
What treatment is available for LBD?
There is currently no cure for LBD but there are treatments that can help manage symptoms and improve day-to-day life. Managing symptoms as best as possible, with or without medications, is very important in helping people with LBD to live well. There is some evidence that a group of medications called acetylcholinesterase inhibitors, that are used to treat Alzheimer’s disease, can help with memory problems and hallucinations in LBD. Depression, anxiety and changes in behaviour that can occur in LBD can often be managed effectively with a combination of medicines and non-medical strategies.
Additionally, medications used for Parkinson’s disease, such as levodopa, can be used in LBD to help with motor symptoms. However, levodopa could make hallucinations worse in some cases and these treatments should be monitored by a specialist. Treatment for LBD can be problematic because certain medications, particularly antipsychotic medications, can significantly worsen LBD symptoms. Due to the variety of symptoms that can occur in LBD, people living with a diagnosis often need tailored support from several health professionals and specialists.
Treatment
Causes and symptoms
What is FAD?
Familial Alzheimer’s disease (FAD) is a rare disease, probably accounting for less than 1% of cases of Alzheimer’s disease overall. It generally affects people at a younger age than the more common, non-inherited forms of Alzheimer’s disease. It is caused by a fault (mutation) within a gene. Three genes have been found to contain mutations that lead to the disease. These are called the Presenilin 1 (PSEN1) and Presenilin 2 (PSEN2) and Amyloid precursor protein (APP) genes. These mutations run in families and the risk of a parent passing a mutation on to their children is 50% (1 in 2 chance) for each child. An affected parent could have all children affected, all children unaffected, or a mixture.
What does ‘autosomal dominant inheritance’ mean?
Autosomal dominant is one of several ways that a trait or disorder can be passed down (inherited) through generations. In an autosomal dominant disease, if you inherit one copy of the mutation or faulty gene you, depending on the gene penetrance, get the disease. Unfortunately, mutations in FAD are almost 100% penetrance, which means if you inherit the mutation, you will develop the condition within your lifetime.
How many different genetic mutations are there? Which are the most common?
FAD is due to mutations in 3 main genes – the presenilin 1 (PSEN1), presenilin 2 (PSEN2) or amyloid precursor protein (APP) genes, or APP duplications. To date, over 200 different mutations have been found in the PSEN1 gene, over 50 in APP and less than 20 in PSEN2. There are mutations that affect many different families and some that may only affect one family (to our knowledge). However, more mutations continue to be discovered.
What are the first symptoms of FAD?
Initial symptoms vary from person to person. Most people with familial Alzheimer’s disease have symptoms of memory loss from an early stage. In some individuals, memory impairment remains the only problem for many years. Changes in behaviour and personality may occur later in the disease, though sometimes they may be the earliest symptom. The same is true of speech. In most individuals speech is not affected until later in the disease, but in some people difficulty with speech and conversation may be an early symptom. The symptoms which occur first often depends on the gene involved.
Many people with familial Alzheimer’s disease have no physical signs of the disease. Rarely, however, it can affect an individual’s walking by causing stiffness of the legs, unsteadiness, or slowing down of movements. Involuntary jerking movements (called myoclonus) may also develop. These may begin very subtly in the fingers and become more prominent in the limbs later in the disease course. Seizures can also occur, particularly in the later stages of the disease.
Is there a way of telling how long someone with FAD will survive?
The length of time an individual survives after they first develop symptoms of FAD varies widely. At present, there is no accurate way to predict how long an individual with FAD will survive. Longitudinal research studies are working to understand this by following individuals as their condition progresses.
Being at risk
How is FAD diagnosed?
Genetic mutations that cause FAD can be tested for with a blood test which screens for genetic mutations. If a person already presents with symptoms and it is known that there is a gene mutation in their family, that gene can be tested for.
My parent developed Alzheimer’s in their 50s. Am I at risk of FAD?
Young onset Alzheimer’s disease is defined by a person developing symptoms before the age of 65. Most commonly, cases of young onset Alzheimer’s disease are sporadic meaning they are not inherited. Some cases of young onset Alzheimer’s disease are familial, meaning they are caused by a genetic, inherited mutation. In these cases, there is almost always a family history of young Alzheimer’s disease, with memory problems usually starting at around the same age across generations.
Will I develop the exact same symptoms as my parent if I have the same genetic mutation?
Not necessarily. The symptoms and disease progression rate vary significantly between mutations and within the same mutation, and even within the same family.
At what age can FAD develop? If my relative started showing symptoms at a certain age and I end up having the mutation, am I likely to start showing symptoms at the same age?
The age at which people develop symptoms varies considerably between different families, but in some families, individuals may be as young as their early 30’s when they become affected. Within a single family, people tend to develop the disease at broadly similar ages.
The age of symptom onset – when a person starts showing symptoms of FAD – often depends on the genetic mutation. Studies suggest that people with the PSEN1 mutation have symptom onset at an younger age, compared to APP mutation carriers. However the age of symptom onset varies a lot between different PSEN1 mutations. The oldest ages of symptom onset is mostly seen in people with PSEN2 mutations, which are rarer than APP mutations or PSEN1 mutations. PSEN1 mutations are the commonest cause of FAD.
I look the most like my parent, who has the genetic mutation. Does that mean I am more likely to have inherited the mutation?
The simple answer is no. The genetic mutations that cause familial Alzheimer’s disease are not responsible for all of your other characteristics, such as how you look, your height or your personality. Therefore, there is no evidence to suggest that those who look most like the person with the mutation are more likely to inherit the mutation.
Is the mutation more likely to be inherited by the women or men in my family?
There is no evidence of a link between biological sex and the likelihood of inheriting a mutation. Therefore, neither men nor women are more likely to inherit the mutation than the other.
Can FAD skip a generation? If I do not have the genetic mutation are my children still at risk of the disease?
Autosomal dominant genetic mutations do not skip a generation. Therefore, if you did not inherit the genetic mutation that causes FAD from your parents, then your children are not at risk of the disease. The mutation may appear to skip a generation if a person with the faulty gene dies of another cause before the illness develops. However individuals who inherit the faulty gene will almost certainly go on to develop the disease within their lifetime.
Genetic testing and counselling
Who should have genetic testing?
Diagnostic genetic testing is only appropriate for a very small proportion of people with Alzheimer’s disease. The vast majority of cases of Alzheimer’s disease are sporadic, meaning that a single genetic mutation is not responsible for the condition. Therefore, diagnostic testing is typically offered when someone has developed symptoms of Alzheimer’s disease at a young age and has a strong family history of other relatives developing a similar illness at a similar age. It may also be offered to individuals with early onset Alzheimer’s disease who have a censored family history, such as a family member dying at a age younger than the expected onset of symptoms of FAD.
Predictive genetic testing is offered to members of FAD families who do not have any symptoms of FAD, in other words are presymptomatic. For an individual to undergo predictive genetic testing they must have a first-degree relative (a sibling or parent) with a proven FAD-causing genetic mutation. We need to know the specific mutation in the family in order to check for its presence or absence. Predictive genetic testing involves a period of genetic counselling.
What is genetic counselling and how do I access it?
Genetic counselling is not counselling in the psychological sense of the word. It is a process by which a person is given accurate information, explaining all the facts and options to an individual as clearly as possible, so that they may make their own informed decision about whether to proceed with genetic testing.
The sessions are spread over a minimum of 3-6 months and are followed by a ‘cooling off’ period before the test is actually done, during which people may decide to decline or postpone taking the test. If a relative is thinking about finding out whether they have inherited a faulty gene that causes FAD, they will need to be referred to a genetics or neurogenetics clinic by a GP or hospital consultant.
When should I consider genetic testing?
Choosing to undergo genetic testing is a very personal decision and requires careful consideration. It is not the right decision for everyone. It is also perfectly acceptable for an individual to decline testing after undergoing a period of genetic counselling. Reasons why people may want to have a genetic test include wanting to end uncertainty, to help them in making plans for the future, to make family planning choices and to inform other family members of their risk such as children. If someone is considering genetic testing, it is a good idea to think about how the testing procedure and test results may affect their relationship with their partner and other family members. In many cases, the genetic testing process brings families closer together, but in some cases, the process can cause tension and complications within a family. An individual’s test result can give other family members unwanted information about their own risk. It is important to remember that different members of a family may have different feelings about testing and that these feelings should be respected. Equally, it should be remembered that taking a genetic test is a very personal choice and an individual should never feel pressured into taking it by family, friends or healthcare professionals.
Does having a positive genetic test (knowing I have the mutation) affect my chances of getting life insurance, a mortgage, or a job, for example?
According to Genetic Alliance UK, if you have taken a predictive genetic test and the test result is “positive”, but you do not have any symptoms of Alzheimer’s disease, you do not have to disclose the results to the insurance company as you currently do not have the disease.
If the insurance application asks about your other family members’ diagnoses, you must disclose this in your application to prevent your policy becoming void. You can find more information about issues around genetic testing from the Genetic Alliance UK website.
Support for families (including children)
When should I tell my children they could be at risk?
Discussing genetic risk can be a difficult conversation, and the “right time” to broach this with children differs between families. Some people choose to discuss this issue with children from a young age, while other people prefer to wait until they are older.
If one of their family members has symptoms of FAD, children may have many questions about the changes they notice in their relative. It is important to give them opportunities to talk and ask questions and there are some useful resources online to help explain dementia to children of different ages. Look at the FAQ below for a list of resources.
What support is available for my children?
The children of a person living with FAD may face difficulties around managing the changes in their family situation that comes with a diagnosis. Furthermore, the inheritance risk associated with this disease will naturally add to their anxieties and carry with it psychological and emotional factors, that will play a part in how young people deal with its effect on a parent. We have listed a few online resources to help children manage some of these challenges:
Understanding dementia, a guide for young people
Dementia for children and young people
Carers Trust: Young Carers
Information for young carers from the NHS
Alzheimer’s association, kids and teens
Explaining dementia to children and young people
How dementia in someone close can affect children or young people
How to help children affected by dementia
If I have a mutation, how can I stop my children from inheriting the mutation?
It is possible to prevent your children from inheriting the mutation by undergoing pre-implantation genetic diagnosis (PGD). The Human Fertility and Embryology Authority (HFEA) have granted a license allowing testing for PSEN1, PSEN2 or APP mutations for couples who wish to pursue PGD. PGD is available to couples if there is a specific genetic mutation known to run in the family, although the individual undergoing PGD does not necessarily need to find out whether they carry the mutation themselves. It is a lengthy and complex process involving assisted reproductive technology, more commonly known as IVF (in vitro fertilization). The chances of success depend on various factors and there are a number of criteria that must be fulfilled if a couple is to be considered for PGD. Unfortunately, at present the NHS only covers for the costs of one child per couple.
How can I access pre-implantation genetic diagnosis (PGD) in the UK?
You can be referred to a PGD clinic through your geneticist or genetic counsellor. More information about PGD can be found on the Genetic Alliance UK website.
Do I need to have had genetic testing myself to have PGD?
No. As long as there is a confirmed genetic mutation that runs within your family, you can have PGD without having a genetic test yourself.
How do you live knowing you might have a gene for Alzheimer’s disease?
Genetic counselling and the appropriate support from carers, professionals and individuals in a similar situation is essential. You can find an article which tells the experience of one individual in a similar situation on the New York Times website.
What support is available for a person with a diagnosis of FAD?
The needs of the person with the diagnosis will certainly change as the disease progresses. Genetic counselling and psychological support are likely to be very important especially in the early stages.
Admiral Nurses are provided by the charity Dementia UK to provide specialist dementia support for families living with a diagnosis. For more useful information about Admiral Nurses and their availability across the UK, visit the Dementia UK website.
What support is available for the carer?
Receiving a diagnosis of a genetic condition can also have a large impact on the person’s caregiver. They may have to manage the practical difficulties of the diagnosis, along with the emotional implications this has for themselves and for the rest of the family. The emotional impact of dementia is hard – many people go through a wealth of feelings from guilt, anger, and annoyance, to sadness and despair.
Coming along to a peer support group is one way of realising that it is normal to feel like this, and that many other carers have those feelings from time to time. The RDS FAD Support Group aims to provide a forum where it is possible for people living with, or at risk of FAD, and their loved ones to share their experiences and coping strategies with each other. The Carers’ Support Group also provides a forum for carers of people with different types of dementia to meet and discuss issues around supporting a person living with dementia. You can find out more information on the Meetings page.
Some carers find online forums a useful place to meet others who empathise with and share their experiences. To see what one popular UK-based forum is like, visit the Alzheimer’s Society Talking Point website.
National Institute for Health and Clinical Excellence (NICE) guidelines recommend that people caring for a person living with dementia should have an assessment of their needs (which includes assessing for and treating psychological distress if necessary), and should be given education about dementia, access to peer-support groups and information about benefits and legal matters. You may wish to access local psychological support via your GP.
You may find other relevant regional support groups on the Young Dementia UK website or on the Alzheimer’s society website. Both of these sites allow you to search by region.
Treatment
Is there any treatment for FAD?
There are a number of treatments available to help manage symptoms. These medications (cholinesterase inhibitors and memantine) can improve symptoms of the disease but do not slow the disease progression.
Symptoms of depression and anxiety are relatively common in FAD and may be helped by medication. It is important to treat these symptoms as, if present, they may exacerbate an individual’s memory impairment. Other medications may also be helpful for treating specific additional symptoms in FAD if they are present, including myoclonic jerks, seizures or leg stiffness.
A progressive deterioration does eventually occur due to the ongoing loss of brain cells. However, the rate of decline can vary considerably between individuals. In the later stages of the disease, full-time care may be required.
Is there anything I can do to prevent or delay the start of symptoms if I have a mutation?
FAD is an autosomal dominant inherited condition, which means if an individual has inherited the mutation, they will develop the condition within their lifetime. At present there are no disease modifying treatments that slow or stop the progression of the disease. However, there is considerable ongoing research into finding new treatments, including clinical trials that individuals who are at risk of FAD may participate in.
Causes and symptoms
It all seems so complicated! What is PPA?
It is complicated! We often reflect on the irony of the fact that PPA – a disorder that affects language – is associated with so many different complicated terminologies. The term primary progressive aphasia (PPA) refers to a group of dementias in which loss of speech and language abilities is the leading and most prominent problem.
‘Aphasia’ refers to a neurological language problem; ‘progressive’ means becoming worse over time; and ‘primary’ refers to the fact that it is caused by brain tissue changes rather than an external cause. PPA affects both men and women and usually starts between the ages of 50 and 70. However, it can also affect older people and, though less likely, younger people as well.
Within the umbrella term of PPA, three main forms are recognised. These are progressive nonfluent aphasia (PNFA), semantic dementia (SD) and logopenic aphasia (LPA). PNFA and SD fall within a larger group of brain disorders collectively called frontotemporal dementia (FTD), indicating the parts of the brain mainly affected: the frontal and temporal lobes. LPA, by contrast, is usually caused by the same proteins that cause Alzheimer’s disease (it is an unusual form of Alzheimer’s disease). Some people might have a form of PPA that doesn’t neatly fit into one of these three main categories. In most cases, we do not know what causes PPA in a particular person.
What are the stages of PPA?
We know that this is really important, and conversations with many of you at Support Group meetings over the years inspired us to try to identify the different stages that people with different forms of PPA go through. We have now nearly completed the first phase of this work, and Dr Chris Hardy gave an update on the PPA stages at our Support Group meeting in May 2023. You can see his slides from the talk he gave here. Since that meeting, the stages of nonfluent/agrammatic variant PPA (progressive nonfluent aphasia) and semantic variant PPA (semantic dementia) have been published in a scientific journal – you can see the paper here. We are currently producing a more accessible version of these stages, as well as those for logopenic variant PPA (logopenic aphasia) which will be made available on our website as soon as possible.
Should I be worried about my PPA being genetic?
In most cases of PPA, it is unlikely to have a genetic cause. The neurologist who sees you should ask for a detailed family history. If you have one or more first degree relatives (parent or sibling) who have also had a form of PPA, then this might suggest a genetic factor. If you’re a concerned family member then you can’t ask for the genetic test to be done on yourself. Instead, the person affected with PPA would need to have the genetic test and then, if it was positive, you could be tested for the same gene. Genetic testing can only be done after genetic counselling.
What is the prognosis for someone with PPA?
Prognosis is a term referring to a medical professional’s opinion on the likely course of a medical condition. It is really difficult to give an accurate prognosis for PPA. One person might progress quite quickly, whilst for another person their rate of progression could be much slower. Neurologists can give a very general overview of what symptoms may be experienced in PPA, but much more needs to be discovered in order to give more accurate prognostic information. This is one of the biggest gaps in clinical care at the moment. We are currently working on a project that will define the stages of each form of PPA. Members of the support group will be the first to hear when this is available.
Sensation of an ‘itchy throat’ in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Symptom progression in logopenic aphasia
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
What is the role of anger and frustration in PPA?
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Symptom progression in semantic dementia
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Sensitivity and pain in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Choking, pneumonia and COVID-19 in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Diagnosis
How does a neurologist come to a decision about a diagnosis?
A neurologist will want to conduct several assessments to determine whether your language problems are caused by primary progressive aphasia (PPA) or something else. The first thing they will do is to ask you for a detailed history of what’s been happening – what have you noticed that’s difficult, and when did it start? Ideally, they’ll ask the same questions of somebody who you know well and who can accompany you to that first appointment. They may ask you to have an MRI brain scan so that they can see a picture of what’s happening within the brain. They will often ask for you to have language testing with a neuropsychologist so as to understand more about how exactly your language is affected. For example, is it your ability to produce speech, problems finding the right word to say, your understanding of words, or a combination of all three?
Sometimes they might ask for you to have a lumbar puncture or ‘spinal tap’. This can be particularly helpful in identifying whether Alzheimer proteins are circulating in the fluid. The nonfluent (PNFA) and semantic variants (SD) of PPA usually have problems with the same proteins that we see going wrong in frontotemporal dementia (FTD). In contrast, the logopenic variant (LPA) is usually caused by the same proteins that we see in typical Alzheimer’s disease. It’s important to know about this difference because we know that there are some medications that can help to manage symptoms in typical Alzheimer’s disease that may be worth trying in PPA, where it is caused by Alzheimer proteins.
Strategies and support
The PPA meetings are great, but they only happen 3 times a year and I have so many questions that I would love to ask of other people going through the same things as me. Is there any sort of forum where people can exchange experiences with each other?
We would really like to set this up, and do hope to do exactly that in the future. For now, there are a few different options that you might want to consider, but please note that none of these are organised by Rare Dementia Support and we cannot endorse any of the information or interactions on external sources.
Some members have found the PPA Facebook group useful. It has more than a thousand members from all over the world. Access the Primary Progressive Aphasia Support Group Facebook group. The Alzheimer’s Society website does have an online forum that is moderated, and there are people who ask questions about PPA and FTD. You can access the forum on the Alzheimer’s Society Dementia Talking Point page.
How do I educate myself, my friends and my family about PPA?
Lots of our members talk about the confusion around PPA as not many people will have ever heard of it before. Although there is a wealth of information on the internet, sometimes this can seem overwhelming, and members in the past have discussed the importance of ‘safe googling’ and using websites that you can trust. For example, NHS Choices and Rare Dementia Support. If you want to ask any questions, then you can always get in touch with the Rare Dementia Support team. We are more than happy to speak to friends and family about PPA. We can also put information packs in the post if this would be helpful.
Unfortunately, because PPA is so rare, many medical professionals will never have met anybody else with the disease. Therefore, people can sometimes be given misleading or inaccurate information about their diagnosis. After hearing the experiences of other members with the same form of PPA as them at our PPA Support Group meetings, some people have then gone back to their healthcare professional and questioned their diagnosis. Following this, upon asking for a NHS referral to a neurologist for a second opinion, their diagnosis was changed. It is important to remember that it is entirely your prerogative if you should want a referral to see a neurologist elsewhere.
Referrals to the Cognitive Disorders Clinic in Queen Square are made via the GP, and the information they would need to make a referral is available on the UCLH website. We do understand that it is not always possible to come to the clinic in London, so please do get in touch if you would like information on how to access a specialist clinic closer to where you live. We are currently working on building a list of specialist clinics outside of London.
Who should I tell about a PPA diagnosis?
This is something that lots of people have talked about struggling with. Some people choose who to tell based on who they think will understand – PPA can be really difficult to explain. Other members have described how people who were once regarded as good friends would withdraw and not stay in touch. Identifying ‘why’ this is the case was difficult. Sometimes, people find it uncomfortable to deal with a person who has dementia, but this does not mean they do not care.
Many of our members have shared positive stories of friends who had been particularly effective at continuing to engage and communicate with the person with PPA. Members talk about how relationships have changed, but that these relationships can still be positive. Choosing who to tell was regarded as difficult as well – some people said that they preferred to just tell the friends and family they were closest to, while others said that it was better to make it known to as many people as possible to avoid confusion. Some members talked about telling close family initially, and then slowly telling more people outside of your immediate circle of friends and family.
In terms of telling strangers about the diagnosis, partners and loved ones have reported taking up the role of being an ‘ambassador’ for the person with a diagnosis. For example if they aren’t answering a question or are acting in a slightly odd way, their ambassador can explain that they are not being rude, but that they have a dementia that causes this behaviour. Some people with PPA at our meetings have noted that they worry about not being able to communicate with strangers and this has made them much more reliant on their partner, carer or family member. We have also included some additional advice on ‘Talking about your diagnosis’ for people living with all forms of dementia on our General Advice page.
We’ve seen some people here with cards that say “I have primary progressive aphasia” written on them. These look really useful – where can I get some?
We make these ourselves at Rare Dementia Support and if you would like to be sent a couple in the post just let us know by emailing us at contact@raredementiasupport.org. These are also always available at our PPA Support Group meetings.
I’m worried about my partner falling ill when they’re out and about and not being able to explain to other people what’s wrong because of their language difficulties. Is there anything I can do about this?
One option would be to use Medic Alert, a charity that provides custom-made medical ID jewellery. These have an internationally-recognised symbol on them and a telephone number. Any paramedic who sees this will phone the number, ring Medic Alert and be able to access the medical information stored on your secure electronic record, 24 hours a day, 365 days a year. You can find more information on the Medic Alert website. There are other options too, for example on the ID Band Company website
Is there anything we can do to make cooking safer?
Lots of our members with PPA have said they have found cooking increasingly difficult as their PPA develops. Although it’s something that we often take for granted, cooking is a really complex process – we have to hold information in memory, sequence lots of things to happen at different times and follow instructions. So it isn’t surprising that it can be affected in PPA. Some members have talked about cooking as a pair so that the person with PPA can focus on one task at a time while the person without PPA is in charge of coordinating the cooking process.
In terms of safety, SGN offer a free safety device to help vulnerable people keep gas safe in their own homes. The device is called a locking cooker valve and it prevents someone from unintentionally turning on or leaving on a gas cooker. This allows someone with PPA to retain their independence whilst remaining safe at home. For more information visit the SGN website.
What activities can people continue to enjoy after a diagnosis of PPA?
This can vary quite a lot, depending on what the person enjoyed before their diagnosis, and the specific form of PPA they have. Some people in the past have reported enjoying playing games such as table-tennis. People living with progressive nonfluent aphasia (PNFA) can sometimes sing better than they can speak, meaning that choirs and singing groups can be helpful and enjoyable for them. Walking and other physical activities like yoga, pilates, and tai-chi can be performed without any need for language and so can be enjoyable for some people living with PPA. Though it is important to note that they may not be appropriate for people who have difficulties with movements. Many cinemas now have ‘dementia-friendly’ screenings, which some of our PPA members have found to be an enjoyable activity.
Members have found it really powerful and helpful to use their diagnosis to promote good – advocating for others and becoming a ‘PPA ambassador’, as well as being involved with scientific research. Dementia cafés tend to have a mixed reception. Some people think these are nice as the person with PPA doesn’t have to put on an act, while for others it can be an unnerving experience, as most of the attendees will have typical memory-led Alzheimer’s disease and so do not experience the same problems as them. Some members who care for partners with PPA talk about the importance of establishing a routine, and ensuring that every day of the week has a focus.
Has anybody had experience of getting a dog or a cat after receiving a PPA diagnosis?
Some members have found that having a dog and a cat can be really helpful for somebody with PPA. We don’t always need language to enjoy the company of animals, and they can provide a daily structure of walks and other activities which can have a positive impact.
What have other people decided about driving after they have received a diagnosis of PPA?
This is really tricky. If you have been diagnosed with any kind of dementia, you have a legal obligation to tell the DVLA. They will then write to your consultant neurologist or other nominated doctor and to your insurance company. That doesn’t mean that you will have to stop driving necessarily. This really depends on whether you are safe to drive. Some members discussing this in the past have told us that even though they hadn’t been told to stop by the DVLA, they had decided to stop anyway. One way that helped people make this decision was to think about whether they would be confident to drive children or grandchildren around (or, if you are a caregiver, would you be happy to let the person with PPA do this). Other people have agreements in place so that the person with a diagnosis will only drive when accompanied by their partner or caregiver, though again it is important to be confident they are safe, irrespective of whether they are accompanied.
If there is any doubt, and the person wants to drive, a practical driving test can give peace of mind. Some people here advocated keeping a driving licence even after stopping driving as a useful form of photographic ID. It is important to recognise that different forms of PPA have different implications for driving – for example, manual coordination and spatial awareness can be more of an issue in LPA or PNFA, while difficulty understanding road signs and signals can be key in semantic dementia (SD).
My wife has become quite obsessed with knowing the time. Is this part of her PPA?
Yes, many people have reported that PPA has led to changes in attitude towards time. Some people do indeed seem to become more obsessed with time. They may feel they need to know the exact time of day, or be fixated with things being done at the same time every day. In others, the concept of time seems to go completely, and some people may have difficulties with recalling past events or imagining future ones. There is more research needed in this area – one of Professor Warren’s current PhD students at the Dementia Research Centre is studying time perception in PPA for her PhD. If you have any thoughts or observations about this, you can email Mai-Carmen directly at mai-carmen.requena-komuro.17@ucl.ac.uk.
I am having a lot of difficulties with reading and writing. What can I do to help manage this?
Difficulties with reading and writing can occur in all types of PPA, and it is something that is understandably very frustrating. With writing, sometimes people can still use keyboards and iPads, however some people may still have difficulties with the complex gestures needed to use these devices. In terms of reading, if the issue is tracking words on the page, audiobooks can be a good alternative.
Additionally, some of our members have come across an app called iReadMore, which is a new single-word reading therapy for improving reading accuracy and speed. It is being developed at UCL for individuals with acquired reading problems. Research into iReadMore has shown that it is effective in improving reading functioning when used regularly. The app has been tested with patients with reading difficulties following a stroke and the researchers believe that it may be beneficial for people with primary progressive aphasia (PPA) also. iReadMore will be available to download on android tablets and mobile devices for £5 a month (though the first week is free), and it will be released as soon as it is ready. More information is available on the iReadMore website. If you would like to be notified when iReadMore is available on the Google Play store, please e-mail them on ireadmore@ucl.ac.uk.
If reading is becoming very difficult and frustrating, it might be more helpful to focus more on other stimulating activities, such as listening to music. It is important to access support such as speech and language therapy for suggestions of suitable adjustments and alternatives, so that you can maintain your independence and keep doing the activities that you enjoy.
Is hearing affected in PPA?
Yes. The same parts of the brain that we use to understand and produce speech are involved in understanding and hearing more basic sounds, so hearing can often be affected in PPA. We know that people with PPA, who have damage to these language regions, can also find it difficult to concentrate in an environment where there is lots of background noise. Many of our members report struggling to hear what is said at large tables or at a party. Some people have reported using strategies where only one person at a family dinner is allowed to talk at a time. So, for example, using a ball to regulate who is allowed to talk and when – it’s your turn to speak if you’re holding the ball.
If you think your hearing, or somebody else’s hearing is affected, you should have this tested, as it is possible that the hearing problems are due to something treatable, like a build-up of earwax. PPA affects the hearing regions of the brain, but as we get older, we can also have problems with our cochlea – an important part of our ear. In some people, hearing aids may be useful, but this often isn’t tested for if the person has problems with language.
Other people have found noise-cancelling headphones really helpful if a person with PPA wants to concentrate on something without the distractions of other noises. Unlike conventional headphones, which are essentially just ‘soundproofing’ (reducing both external noise and also interesting sounds), these headphones actively and selectively reduce background noise entering the ears. They do this by generating a signal that is the acoustic ‘negative’ of the noise and therefore smooth it out – a bit like filling in a crack with plaster. They are, predictably, more expensive than standard headphones.
I’ve noticed that my partner, who has a diagnosis of PPA, seems to have a problem with their eyesight. What should I do about this?
You should take them for an eye test. You can receive a free test every year when you’re over the age of 60. Evidence shows that both hearing and vision can affect cognition (thinking abilities) dramatically so it may be the case that the person feels better after they’re vision has been corrected. Sometimes, opticians can run home visits for people who can’t leave the house. If possible, you should explain to them about the need to speak more slowly and to make adjustments for the person with PPA. You can find more information on the Specsavers website.
My partner with moderate PPA has become apathetic and he doesn’t seem to enjoy most of the things that he used to. What should I do?
Unfortunately, this is something that several of our PPA members have reported. Dr Sobel spoke about apathy a little bit in her talk at our Support Group meeting on 6th September 2018 – you can view this talk on our YouTube channel. This can be one of the most challenging behavioural symptoms to deal with. Apathy is a particular problem where it interferes with caring for the person, for example managing their personal hygiene. Milder forms of apathy, though frustrating to live with, may be relatively harmless.
Established routines and environmental ‘cues’ may help trigger activity in some people. It is very important to consider the possibility that the ‘apathetic’ person may be depressed, as this can look like apathy but is treatable with antidepressant medication and psychological therapy. Apathy and depression can coexist so it may be worth considering this possibility. Your GP or local psychiatry team may be helpful.
We’ve read on the internet about cannabis therapy helping with Alzheimer’s disease. Could this help with PPA too?
It is true that there have been some studies suggesting that cannabis might help manage some of the behavioural symptoms associated with dementia. However, there is no evidence to suggest that cannabis can help to prevent the disease. If you’re interested in reading more about this, there is a helpful article – available on the Alzheimer’s Society website.
Our neurologist told us that there was no point in having speech and language therapy as PPA is progressive. Is that right?
No, speech and language therapy (SALT) can be really helpful for people with PPA and their families. However, some members who have had speech and language therapy themselves have noted that they were surprised that the emphasis had not really been on speech exercises – but rather on training to use communication aids. Assessment of swallowing safety is another very important role for the speech therapist in some people with PPA. The charity Dyscover, based in Surrey, offers SALT-led workshops and courses for people living with PPA. You can find out more on the Dyscover website.
It is true that the goals of speech therapy are different in PPA than in, for example, stroke aphasia. After a stroke, there is much more intensive ‘rehabilitation’ to help boost brain recovery. This type of rehabilitation may not be appropriate for somebody with PPA, and in fact may contribute to stress and anxiety. This is one reason why speech and language therapists who have a special interest in, and knowledge of, PPA are invaluable. Speech and language therapy isn’t just for the person with PPA. Training one’s partner or family members in communicating more effectively with the person with PPA is also very important.
What sorts of aids are there that might help people with PPA to communicate?
There are different sorts of communication aids and a speech and language therapist can be instrumental in helping people with PPA to develop strategies to communicate, even if producing speech is really difficult. Some members talk about having success with communication books, while others cited success with an app called proloquo2go, which they had been given access to via the NHS. You can find our more about the proloquo2go app on the Assistive Ware website.
Another option used by members is an app called React2 – available on the React2 website. However, there are many others and this should ideally be discussed with a speech and language therapist. Some members have noted that some common technologies can be difficult for people with PPA. Phones and tablets often use screenlocks and have a language-based navigation system. Picture books can be helpful if the person can’t find the word they’re looking for but know what the object is, and these can be personalised to the individual with PPA. Others have noted the importance of learning ‘new’ communication strategies early, so getting to grips with an iPad or phone in the early stages of the disease can help later on.
How do we find a speech and language therapist ourselves?
One option for finding a speech and language therapist is to search the directory of therapists working in private practice. This way, you can specify that you want somebody with expertise of working with progressive neurological conditions like PPA in your area. Access the speech and language therapist directory. If you are going through the NHS, it is absolutely within your right to request a referral to be made to a speech and language therapist. This referral can come either via the GP or the neurologist, and the neurologist might be slightly better-placed to make a referral to somebody with expertise in neurological disorders.
However, one thing to bear in mind is that, because PPA is so rare, you may see a speech and language therapist who has never worked with somebody with PPA. They are often very motivated to learn about how to best help you, and you could recommend that they get in touch with us at Rare Dementia Support. RDS researcher Chris Hardy would always be very happy to explain to them about what PPA is and how speech and language therapy can help. Contact Chris at c.hardy.12@ucl.ac.uk.
There is also a book that could be helpful, written by a highly specialist speech and language therapist and a regular contributor to our support group meetings, Anna Volkmer. Find the ‘Assessment and Therapy for Language and Cognitive Communication Difficulties in Dementia’ book on Amazon.
I sometimes get ‘yes’ and ‘no’ mixed up – why is this and what can I do?
This can be really tricky. Scientists aren’t sure at the moment as to why this happens. What we do know is that this mixing up of “yes” and “no”, and also other word pairs such as “hot” and “cold”, tends to be most common in progressive nonfluent aphasia (PNFA). It can be incredibly frustrating. Some members have reported finding it helpful to try using ‘non-verbal’ responses, such as using thumbs up or down, or pointing up or down to indicate “yes” or “no”, but these strategies don’t work for everybody. Some have also suggested avoiding using direct questions. A speech and language therapist may be able to help with alternative communication strategies.
Questions to ask care agencies when seeking support from paid carers
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
What is continuing healthcare funding and how can people access it?
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Issues around hygiene and anger in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Arranging care for somebody with PPA who lives alone
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Support for balance issues in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Dental hygiene in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Vomiting in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Treatment
What medications work for PPA?
Unfortunately, there are no medications currently available that will prevent or cure PPA. Research is ongoing and we hope that we will soon be able to start clinical trials of new drugs. There are different drugs available that might help manage symptoms. For instance, if a person’s PPA is caused by the same proteins that cause typical Alzheimer’s disease (as is likely in logopenic aphasia or LPA), they may benefit from a class of drugs called cholinesterase inhibitors. Other medications may be helpful in managing some of the behavioural symptoms associated with PPA. Dr Sobel, who spoke at our September 2018 meeting, gave an overview of some of these and you can see her talk on our YouTube channel here.
Findings from drugs trials in PPA
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Will tau protein trials take place in 2021?
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Diagnosis
How does a neurologist come to a decision about a diagnosis?
The neurologist will want to conduct several assessments to determine whether the symptoms are caused by a rare dementia or something else. The first thing they will do is to ask you for a detailed history of what’s been happening – what have you noticed that’s difficult, and when did it start? Ideally, they’ll ask the same questions of somebody who you know well and who can accompany you to that first appointment. They may ask you to have an MRI brain scan so that they can see a picture of what’s happening within the brain. They will often ask you to have cognitive testing with a neuropsychologist so they can understand more about how exactly your skills are affected.
Sometimes, they might ask you to have a lumbar puncture or “spinal tap”. This can be really helpful particularly in identifying whether Alzheimer proteins are circulating in the fluid. There are types of dementia, like frontotemporal dementia (FTD), that are not caused by these proteins. It’s important to know about this difference because we know that there are some medications that can help to manage symptoms in Alzheimer’s disease that may be worth trying if the type of dementia is caused by Alzheimer proteins.
Can I ask for a second opinion about the diagnosis?
As these dementias are so rare, it may be that some medical professionals have never met someone with your type of dementia before. As a result, people can sometimes be given misleading or inaccurate information about their diagnosis. It is important to remember that it is entirely your prerogative if you should want a referral to see a neurologist elsewhere. Referrals to the Cognitive Disorders Clinic in Queen Square are made via the GP, and the information they would need to make a referral can be found on the UCLH website.
We understand that it is not always possible to come to the clinic in London, so please do get in touch if you would like information on how to access a specialist clinic closer to where you live, as we are currently working on building a list of specialist clinics outside of London.
Talking about your diagnosis
Who should we tell about the diagnosis?
This is something that lots of people have talked about struggling with in the past. Some people choose who to tell based on who they think will understand – rare dementias can be a difficult subject to explain. Other members have described how people who were once regarded as good friends would withdraw and not stay in touch. Identifying ‘why’ this is the case was difficult – sometimes people find it uncomfortable to deal with a person who has dementia, but this does not mean they no longer care. Many of our members have shared positive stories of friends and family who have continued to engage and communicate with them. They have talked about how relationships have changed, but that these relationships can still be positive.
Many people have found it difficult to choose who to tell about their diagnosis. Some said that they preferred to just tell the friends and family they were closest to, whilst others said that it was better to tell as many people as possible to avoid confusion. Some members talked about telling close family initially, and then slowly telling more people outside of your immediate circle of friends and family.
In terms of telling strangers about a diagnosis, partners and loved ones have reported taking up the role of being an ‘ambassador’ for the person with a diagnosis. For example if they aren’t answering a question or are acting in a slightly odd way, their ambassador can explain that they are not being rude, and that they have a dementia that causes this behaviour. We have included some additional advice on ‘Talking about your diagnosis’ for people living with all forms of dementia within our General Advice page.
I don’t live with my parent, who has a diagnosis of dementia, and I’m worried that they won’t be able to keep me in the loop about medical results, for example. How can we manage this?
Your parent can give consent for their GP and other medical professionals to discuss their health with you. You could ask to be copied in on any letters that they send out too. To find out more, please see the NHS website.
Research
How do I get involved with research?
Several members have taken part in research studies into PPA with the same team who run the PPA Support Group. Much more research into PPA is needed and will be crucial if we are to arrive at effective treatments. There is currently a team of neurologists, neuropsychologists, brain imaging experts and geneticists researching PPA at the Dementia Research Centre and Institute of Neurology in London.
The current studies focus on understanding how symptoms arise in PPA, how abnormalities of brain physiology develop and how these can be detected, how the diagnosis can be made earlier and more reliably, and how PPA can be tracked over time. Our research projects generally involve clinical and psychology assessments and MRI brain scanning, and we may ask people to have a lumbar puncture (also know as a ‘spinal tap’) and take part in other specialised tests. We ask people volunteering for research to come to a research visit which is spread over 2 or 3 days, and if possible, repeat the visit the following year so we can measure changes directly. If you would be interested in learning more about the current research, you can email Chris Hardy at chris.hardy@ucl.ac.uk.
How can people get involved with research into PPA at UCL?
Answers from the Q&A session of our PPA Support Group webinar in June 2020.
Causes and symptoms
I’ve noticed that my partner, who has a diagnosis of dementia, seems to have a problem with their eyesight. What should I do about this?
You should take them for an eye test. You can receive a free test every year when you’re over the age of 60. Evidence shows that both hearing and vision can affect thinking abilities dramatically, so testing both regularly is important. Sometimes, opticians can run home visits for people who can’t leave the house. If possible, you should explain to them about the need to speak more slowly and to make adjustments for the person with dementia.
If someone already has problems with their hearing, are they more likely to develop dementia?
There has been some research, particularly focused on Alzheimer’s disease, indicating that hearing loss in mid-life can increase the risk of developing dementia later on. Problems with hearing might make symptoms of dementia worse, and vice versa. The relationship between hearing loss and dementia is very complicated and is an area that is currently being explored in research – but it definitely isn’t the case that everybody with hearing difficulties will go on to develop dementia. Again, it is very important to have your hearing checked if you are experiencing difficulties.
Several carers have mentioned considerable weight loss in their partners and they are worried about what to do. Some of those are in residential care; some at home but still have good appetites and are eating well-balanced meals. Is it OK to give foods with higher fat content or more sweet options? Do they need to be taking supplements?
Loss of weight is a common feature in many dementias, and indeed may precede the development of symptoms. The reasons behind this are not entirely clear. Additional reasons for weight loss can include individuals forgetting to eat, having changes in what they like to eat, or not being given sufficient time and help to finish their meals.
While it is important of course to have a balanced diet, finding foods that people enjoy is very important, as eating is both about nutrition and enjoyment. If individuals are eating a balanced diet, then additional supplements are usually not needed, but if there is lots of weight loss then sometimes drink supplements with lots of calories may be needed. This should be discussed with the patient’s doctor. If the patient is in a care home, it is important to ensure that staff give sufficient help, time and encouragement as needed.
Specialist support
What support is available for someone with a rare dementia?
There are lots of types of support out there for people living with a rare form of dementia. Here are a few sources of support people have found helpful:
Occupational Therapy
– Provides assistance with everyday tasks and is important in terms of identifying areas where help is needed
– Referred by a health professional, or social services/local authority. It can also be paid for privately
For more information, please see the NHS website.
Physiotherapy
– Provides assistance with movement, and helps to reduce the risk of injury in the future
– Provide education, tailored exercises and manual therapy
You can access physiotherapy through the NHS, usually through a referral from your GP, although sometimes you can also refer yourself directly. You can also pay for it privately.
For more information, please see the NHS website.
Local Authority
Your local authority can assist with:
– Needs Assessment: This looks at what support is needed to help people live as safely and independently as possible. It may advise on areas such as: help with cooking, cleaning or personal care, equipment and adaptations to the home (usually through an occupational therapist assessment), day centres and care homes. For more information, please visit the gov.uk website.
– Carers Assessment: These are available alongside the needs assessment for the person living with dementia. These can advise on areas such as: benefits, respite services and local support organisations. For more information, please see the NHS website.
– Emergency Carers Schemes: Some carers organisations work in partnership with the local authority to provide emergency carers cards. You carry this card with you so that if there is an accident or health emergency, people will know that you are a carer and someone relies on you for support. If they ring the number on the card, they will be able to find out your emergency plan and who you wish to be contacted. Please see the Carers Trust website for more information.
– Financial Assessments: Local authorities are required to do a means test to determine if they should be contributing towards care costs. They may provide some financial assistance if the person with dementia has less than a certain amount in savings (the amount differs across different parts of the UK). If the person with dementia needs care in the home, the value of the house will not be included in the means test. For more information, please see the NHS website.
You can find details on how to contact your local council on the gov.uk website.
Admiral Nurses
Admiral Nurses provide specialist dementia support to people living with dementia and their families, including:
– One-to-one support
– Practical solutions to overcome challenges
– Information provision
– Help to get additional care and support
– Expert guidance, for example, tips on preventing and managing distress
People can self-refer and the service is free. The service is area dependent, so check here to see if there is one in your local area by visiting the Dementia UK website or calling 0800 888 6678.
How can we request help in accessing health and social care services? We are struggling to get any support at all.
Everyone is entitled to receive health and social care support. Regarding support for heath needs, the GP is the gatekeeper to enabling this. The GP initiates the referral to the Psychiatry or Neurology Teams in the beginning, but also has a duty to offer invaluable services to assist when they are required. Open communication with the GP is needed to express your difficulties and ensure your needs are met.
This can be any of the following:
– Community/District or Mental Health Nurses to assist with extremely difficult situations or to administer medications
– Counsellors and psychologists for talking therapies
– Audiologists for hearing problems/ Ophthalmologists for vision/perception (especially helpful in PCA)
– Occupational Therapists and Physiotherapists for movement, balance and ability; ensuring you stay safe
– Speech and Language Therapists for speech problems and swallowing issues
– Dieticians for advice on nutrition and staying healthy.
Some of these services may be restricted or have criteria for accessibility, but this can be discussed with the GP.
Social Services have a duty of care to assess your care needs as a person living with dementia and a carer. Everyone in this category is entitled to an assessment and we would advise to have a carers or needs assessment as soon as possible so you are known to their service and they can benchmark the current situation to any future situations. You can arrange a carers or needs assessment by contacting your local Social Services directly or by asking your GP or Care Coordinator to do so on your behalf.
A social worker will assess your abilities and determine what services would be available to meet the needs to help in your everyday life. This could include help around the home, such as shopping, cooking and cleaning, or personal care such as washing and dressing. They can also arrange for adaptations to the house, such as raised toilet seats or handrails. They can provide carers coming in up to 4 times a day if needed, but this may incur a cost.
Social services can also arrange a placement at a Day Centre or suggest support groups and arrange respite care when needed. They will also work with the health service to provide aftercare when people are discharged from hospital.
Social services can also help with finances if someone is struggling to pay bills and claim benefits.
Each local authority will have a slight variation in options of services and some may have higher criteria than others due to local financial restrictions.
A close family member has just received a rare dementia diagnosis. Can I receive counselling on the NHS?
When a loved one receives a diagnosis of a rare dementia, you as a relative and/or carer may experience significant stress or distress. It is always worth contacting your GP if you feel like this. There may be counselling available on the NHS, or the GP may be able to suggest other services locally. Having counselling can be invaluable in supporting you through the tough journey of being a caregiver. Caring for someone with a rare dementia can be a lonely experience, and having supportive relationships with friends, family and/or a professional counsellor can all help. For more information on how to access therapy, visit the British Association for Counselling and Psychotherapy website.
One really important thing to remember is that you need to be kind to yourself. You will make mistakes – you are learning to cope with something that you never expected you would have to cope with. Trying different things shows that you are making an effort to improve the support and care you provide. Not everything you try will work – but try not to criticise yourself when it doesn’t.
What should I do if I notice a sudden change in my partner, who already has a diagnosis of dementia?
There are many reasons why there can be sudden changes in a person living with dementia. It could be that they have a secondary problem, for example a urinary tract infection (UTI) which is causing a change in behaviour. Or it could be related to their condition. It is essential for them to see their GP to unravel what could be behind the change.
Where can I find support for home care?
Often people seek information on home care from a range of sources, including: asking for recommendations in online forums, searching online for home care agencies, or from schemes that involve having a live-in helper in exchange for low rent costs and word of mouth recommendations in the local community (although reliability has been an issue for some people).
Specific examples shared were:
– Asking via dementia-specific Facebook groups
– Using sites such as Home Instead for private care support, or Share and Care who match up households who have a spare room with someone happy to provide some hours of companionship and support – this is in return for an affordable place to rent
– Some local charities also provide befriending services, you can check what if it is operating in your area on the Alzheimer’s Society website. Some people find it helpful to have a personal assistant to provide them with support. You can find out more about personal assistants here and here
I have dementia and live by myself. I sometimes have problems understanding what people say to me – especially with complicated medical words and I’m worried that I won’t understand what the doctors say to me. Who could support me?
You are entitled to have an ‘advocate’ appointed for you by your council. They can help you to understand your care and support process. They can talk to you about how you feel about your care, help you to make or challenge decisions about your care, and stand up for your rights. You can access more information on the NHS website.
There used to be a booklet called the ‘Essential Information Booklet’ that was handed out by the nurses working in the Cognitive Disorders Clinic at Queen Square. What has happened to this – can this be used again?
Yes – we talked to the people who had made this booklet for the Cognitive Disorders Clinic. They have stopped giving it out, but other members have found the Alzheimer’s Society Dementia Guide a great resource. You can find this on the Alzheimer’s Society website.
Financial and legal support
I have heard that sometimes the NHS will pay for care. How does this work?
This is what is called ‘NHS Continuing Healthcare’ (CHC), where the NHS will pay for care services provided at home or in a care home. People that have been through the process say that the funding is very difficult to get, and that it is very common to be rejected when first applying. However it is possible to appeal if rejected, and some people do go on to receive Continuing Healthcare (CHC) as a result of this appeal process. More information can be found on the Beacon CHC website.
It’s a good idea to make sure you’re known to social services, district nurses and palliative care teams ahead of applying. Some people have also noted that the assessment can sometimes take place when only the person living with dementia is present, so it is important to try and find out when this will take place so that a representative (friend or family member) can also be present.
If you have questions about Continuing Healthcare (CHC) you might want to take a free 90 minute phone consultation with an organisation which specialises works on helping people with their CHC claims. For more information, visit the Beacon website.
Will I be eligible for Continuing Healthcare (CHC) funding?
*Warning: there is the likelihood of being rejected when first applying for CHC funding, although you can attempt to appeal if rejected. Only a small number of people do actually get CHC funding and you do need to have quite a severe health need to get it, but people should definitely still be assessed if it seems at all likely that they will be eligible.
An Initial Screening Test is carried out and if a possible likelihood is found then a more thorough assessment is explored by two or more health professionals. There are 12 categories in the assessment:
– Breathing
– Nutrition
– Continence
– Skin integrity (including wounds, ulcers, tissue viability)
– Mobility
– Communication
– Psychological and emotional needs
– Cognition
– Behaviour
– Drug therapies and medication; symptom control
– Altered states of consciousness
– Other significant care needs
Each are rated from low/moderate/high/ severe/priority.
You have to score in the high/severe/priority range, with at least five categories at ‘high’ or one at ‘priority’ to qualify.
With regard to CHC entitlement, guidance can be found online here.
Regarding other funding, you can check eligibility for PIP (Personal Independence Payment) here and for Attendance Allowance (over state pension age) here.
There are also online benefits eligibility calculators here and here that might assist.
We would be happy to arrange a one-off advice appointment with you as part of the RDS Advice Service to advise further on the eligibility criteria for CHC and PIP, how to claim, and other advice on your benefits entitlement tailored to your circumstances. Please request an enquiry form from contact@raredementiasupport.org.
Comment from RDS member: If filling out PIP or ESA (Employment and Support Allowance) forms you need to put down your worst days.
Many places can offer help with completing forms too, such as Carers Centres and Citizens Advice Bureaus – your local Alzheimer’s Society Dementia Support Worker will be able to identify who is commissioned in your area to help.
What is the advantage of CHC (continuing healthcare) funding apart from the financial benefits?
Please note: the below has been drafted by the RDS Legal Advice Service as general information and should not be treated as legal advice for specific individuals. Please contact the RDS Legal Advice Service if you would like any specific advice relating to your personal circumstances: accesstojustice@ucl.ac.uk.
The principal advantage of NHS CHC is financial: it is free at the point of use. Care provided by a Local Authority under the Care Act 2014 is subject to a means test with a very low threshold for private contribution, and the private provision of care can be extremely expensive.
Accessing NHS CHC is much more difficult, however, as the eligibility criteria are narrower than those used for assessing eligibility for local authority care:
1. The individual must have ongoing significant physical and/or mental health needs, and
2. Having taken into account all of the individual’s needs, it must be said that the main aspects or majority part of the care they need is focused on addressing and/or preventing health needs.
The non-financial benefits of NHS CHC are mostly apparent when compared to local authority care, rather than private care. NHS CHC allows for individuals with complex health needs to receive treatment and care from people with particular expertise. It also allows for greater communication between the skilled staff who are providing care to an individual, which may improve the overall service and quality of care that the individual receives.
A further benefit of seeking Continuing Healthcare is that, even where an individual is assessed as not being eligible, the NHS may still contribute to the care under a ‘joint package’ with the local authority. This allows for direct input from the NHS in the care provided to the individual, and again may be helpful where an individual has complex health and care needs.
How does the council decide whether someone needs to pay for their own care or not?
The council will need to do a means test (financial assessment) to determine how much you need to contribute to your care costs. The amount differs across areas, so please search for information about your local area to see what you are entitled to. In England, the council will help to pay for your care if you have less than £23,250 in savings, although you may still have to contribute towards the care costs. It is important to know that if you have a joint account with a spouse/partner, the council will count your savings as being half of this, so £50,000 in a joint account will count as £25,000 in savings. For more about the local authority financial assessment, please see the NHS website.
For more information on paying for care, please see the AgeUK website.
Will I have to sell my house to pay for care?
If you are paying for care in your own home, the value of your home won’t be taken into account to pay for care fees. If you are living in a care home, the value of your home may be taken into account in a financial assessment. It will not be taken into account if:
– You need temporary or short-term care
– Someone is still living in the home, such as: your partner, a relative who is 60 or older or a child of yours under the age 18
The council cannot include the value of your property in their financial assessment for the first 12 weeks that you are in a care home, to give you some extra time to decide what to do with your property.
For more information on how your property may be taken into account when paying care home fees, please see the AgeUK factsheet.
When should I start thinking about financial and legal affairs?
Many support group members find it beneficial to think about financial and legal affairs as soon as possible. Some people encounter difficulties in trying to make arrangements further down the line when it is less clear whether they have the mental capacity to do so (this refers to your ability to make your own decisions based on your understanding, communication and ability to remember the decision). Therefore, we would always recommend making arrangements before your mental capacity can be called into question. Organising your financial and legal affairs also allows you to have peace of mind that all issues can be dealt with in the way you have chosen. You may wish to consider the following issues:
– Arrange bills to be paid by direct debit and benefits to be paid directly into the bank accounts
– Consider joint bank accounts
– Seek advice from a solicitor or financial adviser if needed (especially with complicated matters such as setting up a trust, for example)
– Make sure you are receiving all the benefits to which you are entitled
– Make a will
– Consider arranging Lasting Power of Attorney (LPA). This is a legal document which gives the person/persons you have chosen the right to make decisions for you on financial and/or health care matters in case you lose the capacity to make decisions yourself. You may choose to have only one LPA (financial or health) or both. You can find out more about this at the gov.uk website.
What is a lasting power of attorney and when should we get one?
A: A Lasting Power of Attorney (LPA) is a legal document that lets you appoint one or more people (the ‘attorneys’) to make decisions on your behalf.
Lasting Power of attorney is not the same as being next of kin. Being next of kin does not legally entitle you to make health or financial decisions on behalf of your relative. In many instances, in order to represent your loved one, you will need a Lasting Power of Attorney in place. We advise members to discuss LPA with their family and loved ones as early as possible in order to make an informed decision. An LPA is only valid if the individual has the mental capacity to set it up and hasn’t been put under any pressure to create it. Once in place this will ensure that financial and health decisions are made by an individual your relative trusts rather than an external team of professionals having to make decisions on what they believe will be in the patient’s “best interests”. It is important to remember that you can only set up a Lasting Power of Attorney when you have mental capacity.
There are two types of LPA:
– Health and Welfare: who can deal with daily care, for example
– Property and Financial Affairs: who can deal with bills and bank accounts, for example.
A Health and Welfare LPA is the most useful when pursuing a claim for NHS Continuing Healthcare Funding, as most NHS-related establishments will attempt to insist on it before liaising with someone on behalf of a patient. They can refuse to disclose medical records relating to an individual if the family are not in receipt of a Health and Welfare LPA. making decisions about future medical care will become very difficult. In such cases, family or friends must obtain a Deputyship through the Court of Protection if they wish to be involved with decisions surrounding their relative’s ongoing care.
A Deputyship works in the same way as a Power of Attorney, authorising a person to make decisions for Health and Welfare and/or Property and Finance on behalf of their incapacitated relative. The Court of Protection will decide if it is necessary for ongoing decisions to be made on an individual’s behalf, and whether the nominated person is suitable to be appointed to that role. They will also ascertain that there are no objections to the Deputy’s appointment. the Court of Protection route is always more expensive, time-consuming and inconvenient. In particular it requires a detailed examination and background check of the person nominated to act as a Deputy and is far more specific in the permissions it provides.
A Deputy must fulfil a number of ongoing legal obligations including the payment of an annual fee and the submission of an annual report to the Court of Protection which is not required under the LPA option.
Further, the Court of Protection is exceptionally reluctant to provide a Health and Welfare Deputyship which can cause issues when attempting to secure the necessary medical records needed when making an NHS Continuing Healthcare Funding application.
Members discussing this said that it was important to get both types of LPA, and that it was really important to think about doing this as soon as you can. Another piece of advice that was shared was that it’s a good idea to have multiple people named with power of attorney (see below). You can also add comments at the point of registering, so that your attorney(s) can read your instructions at the point of exercising it.
It is important to note that this applies to everybody – not just people living with dementia. We should all have a lasting power of attorney set up for ourselves and should have this as early as possible.
You can find more information about this at the gov.uk website.
How many people should you have acting for you in LPAs?
Please note: the below has been drafted by the RDS Legal Advice Service as general information and should not be treated as legal advice for specific individuals. Please contact the RDS Legal Advice Service if you would like any specific advice relating to your personal circumstances: accesstojustice@ucl.ac.uk.
There is no single answer to this question and it depends on each person’s individual circumstances. There are, however, some important things to bear in mind when deciding how many attorneys to appoint.
First, it is very important for a donor to have complete trust in their attorney or attorneys. If the donor has any doubts whatsoever, they should consider whether there is someone better placed to take on the role. This might be a professional person such as a solicitor or accountant, although these people will charge for their services.
There is a balance to be struck between giving attorneys freedom and flexibility to effectively act on a donor’s behalf, and ensuring that the donor’s interests are well protected. If more than one attorney is appointed, the donor can choose whether they will act jointly or severally, or jointly for some matters and severally for others.
‘Jointly’ means that all attorneys must agree every decision together. This may provide the donor with a greater sense of certainty, but it may also slow down the decision-making process.
‘Severally’ allows each attorney to act for the donor independently from other(s). This gives each attorney greater freedom to act for the donor, but provides less oversight of the attorney’s decisions.
Finally, it is worth considering what might happen if an attorney can no longer act for the donor. Where an attorney is appointed alone, or jointly with others, if he or she is no longer able to act for the donor the power will come to an end. This is not the case where attorneys are appointed severally – in this case the power will continue even where one attorney can no longer act.
Lasting Powers of Attorney also allow the donor to name substitute attorneys on their application form in case of this eventuality. This means that the power will continue to be exercisable by the substitute attorney if an attorney can no longer act. As with an attorney, a substitute must consent to being included on the form and should be chosen with the same amount of care.
How do I know when is the right time to exercise my power of attorney?
Members here have talked about the fact that ‘capacity’ for decision making is on a continuum – there isn’t always a distinct split between somebody having capacity one day and then not having it the next. For this reason, it can be really hard to think about taking control of things that your partner / family member would have taken for granted previously. One person gave the example of taking money out of an ATM: they set a daily limit for their partner, meaning that they still had independence, but within certain limits.
What type of benefits are available to me?
There are a number of different benefits someone with dementia may be eligible to apply for.
– If you are under State Pension age, you may be eligible for the Personal Independence Payment (PIP), which is not means tested, and is assessed based on the effect that your condition has on your life. There are two components to PIP: Daily Living, and Mobility. Find out more about PIP on the Citizen’s Advice website.
– If you are over State Pension age, you may be eligible to apply for Attendance Allowance (AA), which is not means tested, and is based on care needs. There are two rates of AA: the lower rate, for people that have care needs in the day OR night, and the higher rate, for people that have care needs in the day AND night. Find out more about Attendance Allowance on the gov.uk website.
It is important to note that as there is no mobility element of AA, if you are under state pension age then you should apply for PIP if you have mobility needs, rather than waiting to apply for AA. PIP will continue past state pension age if you are already receiving it.
The forms for PIP and AA can be difficult to complete, so if you would like help completing these forms you can contact the Citizens Advice Bureau website for local support. Members have also recommended keeping a 2 week diary of details of the ‘help’ required/provided, for example, every time you get up in the night, how long for and so on. This will help to ensure you paint an accurate picture of how much assistance you’re providing or requiring. It is also important that you think about the ‘worst day’ when describing symptoms, so that the assessors can get a clearer picture of the difficulties the person with dementia may experience.
PIP and AA are often seen as a gateway to other benefits/support, such as (but not limited to):
– Carer’s Allowance: if you care for someone for 35 hours or more and they receive particular benefits. Carer’s allowance is dependent on income. Visit the gov.uk website for more information.
– Council tax exemption: if the person with dementia lives on their own, they may qualify for an exemption in council tax. If they live with someone else, they may qualify for a 25% reduction in council tax. Visit the gov.uk website for more information.
– VAT Relief: people over 60 or that have disabilities may be eligible for VAT reliefs on certain items that are for personal or domestic use. Visit the Low Incomes Tax Reform Group website for more information.
– Vehicle tax exemption: if you get certain benefits you can apply for an exemption or reduction in vehicle tax. Visit the gov.uk website for more information.
What should I do if the person that I support is starting to struggle with finances?
Some people have found it helpful to set up automatic payments for bills that regularly need to be paid. Members have also suggested pre-paid cards or putting a limit on the amount that can be withdrawn each day (For example banks such as Monzo offer card arrangements which be topped up and frozen remotely).
What is a ‘CEA Card’?
This is a national cinema card scheme that enables a person with dementia to receive a complimentary ticket for someone to go with them when they see a film in a participating cinema. CEA stands for ‘Cinema Exhibitors Association’. It costs £6 and you can find more information on the CEA Card website.
Is there a way that I can record what I want to happen in the future if I have difficulty making decisions for myself?
There is a document called an ‘Advance Statement’ where you can note down things that are important to you, like where you would like to live, who you would like to visit you, and if you have any religious or spiritual beliefs. This can be as specific or as general as you would like. It is not a legally binding document, but whoever is making decisions on your behalf has to take your wishes into account. You can find a template Advance Statement document available on the Alzheimer’s Society website. For decisions about medical treatment, such as refusing resuscitation, you can complete an ‘Advance Decision’ form. More information about Advance Decisions can be found on the NHS website.
Financial and legal support
Do you think that judges know enough about FTD – is that important to avoid people being locked up who are ill?
I am fairly sure that the judiciary receive some training on the issue, but there is always scope to feed more information in to various forms of judicial training so that the judiciary can appropriately take into account issues such as FTD. In considering how to deal with an individual, they would probably obtain medical opinion.
If someone with FTD resigned from work – when actually they were ill with FTD – is there anything that can be done even a year later?
What can be done depends on the circumstances of the resignation. There may be grounds for discrimination/constructive dismissal, however, these avenues are subject to three month time limits for claims, which can only be extended in specific circumstances.
Safety and accessibility
Might we be eligible for a ‘blue badge’ so that we can park closer to supermarkets, for example?
This scheme is predominantly for people with severe mobility and sight problems. Although people with dementia of all kinds are now eligible due to changes to the guidelines for people with hidden disabilities, this is somewhat dependent on the local authority, and people living with dementia may sometimes still be turned down for a blue badge. You can find more guidance on applying for a blue badge for someone with dementia on the Alzheimer’s Society website.
Can I make changes at home to make it easier to get around?
There are many adaptations that can be made to the home to make it easier to get around. It might be helpful to speak to an occupational therapist about what changes can be made to help maintain independence around the home, such as installing rails in the bath or shower to help the person get in and out.
For more information, please see the NHS website.
Is there anything we can do to make cooking safer?
Lots of people have said that they find cooking increasingly difficult as their dementia develops. Although it’s something that we often take for granted, cooking is a really complex process – we have to hold information in memory, sequence lots of things to happen at different times, follow instructions – and so it isn’t surprising that it can be affected in a number of different types of dementia. Some members have talked about cooking as a pair so that the person with dementia can focus on one task at a time while the person without dementia is in charge of coordinating the cooking process. In terms of safety, SGN offer a free safety device to help vulnerable people keep gas safe in their own homes. The locking cooker valve prevents someone from unintentionally turning on or leaving on a gas cooker. This allows someone with dementia to retain their independence whilst remaining safe at home. They also have a priority services register for people in need of priority support in a gas emergency situation. For more information please visit the SGN website.
I’m worried about my sister falling ill when she’s out and about and not being able to explain to other people what’s wrong because of her language difficulties. Is there anything we could do about this?
One option would be to use Medic Alert – a charity that provide custom-made medical ID jewellery. These have an internationally-recognised symbol on them and a telephone number. Any paramedic who sees this will phone the number, ring Medic Alert and be able to access the medical information stored on your secure electronic record, 24/7, 365 days a year. You can find more information on the Medic Alert website. You can also explore other options such as by visiting The ID Band Company website.You can also request an RDS Helpcard at contact@raredementiasupport.org.
I worry about falling over and hurting myself. What can I do to stay safe?
There are a number of things that can increase the likelihood of falls, such as; low blood pressure, mobility and balance issues, visual impairments and taking medications that increase drowsiness. It is important to try and minimise these risks where possible, such as; using mobility aids, ensuring good lighting around the home and ensuring medication is monitored closely. For more information on fall prevention, please see the NHS website.
If you are concerned about falls you should ask your GP for a falls risk screening, which will help to identify ways for you to stay safe and lower the risk of falling, allowing you to continue with the activities you find important. Clinic and referral process is area dependent, so may be helpful to ask your GP about your local specialist falls service. You can also complete a self-assessment on the NHS website.
Should we tell the police about the diagnosis?
You don’t need to tell the police directly about the diagnosis, but you might want to consider the ‘Herbert Protocol’. This is a national scheme introduced by the Met in partnership with other agencies which encourages carers to compile useful information, such as medication, contact numbers and a photo, which could be used in the event of a vulnerable person going missing. The idea is to supply information at any given time, ahead of an incident happening, so that the police already have the information that they need. You can find more information and download the form at the Met Police website.
I am worried about the person that I support getting lost. What could help me with this?
There are a number of GPS locating devices that can be used to find people when they are out walking if there is a concern that they will become lost. For example you can use:
– Find my Friends
– Life 360
– GPS smart sole
– Watches with GPS trackers (various companies provide these)
The carer can access the tracker remotely on these products rather than the person living with dementia having to press a button.
One suggestion was a specific device called the ‘Doro Secure 580’. This particular device is a simple mobile phone that has four numbers written on it. Five nominated individuals can use the tracking facility to find out where the person is. Find out more on the Doro 580 website.
Support group members have mentioned the importance of designating ‘In Case of Emergency’ contacts in their mobile phone contact list (saving them as ‘ICE [name]’). It is also important to complete your ‘Medical ID’ information on your mobile phone so that emergency services can access the information in case of an emergency. Medical ID wristbands can also be helpful and reassuring for people.
Importantly, walking can be a huge source of independence, as well as a good form of exercise, and members have said that they would like to maintain their independent walking as long as it is safe to do so.
Travel
If I have a diagnosis of dementia, does that mean that I have to stop driving?
Driving is a huge source of independence, and can be a very difficult thing to decide to give up. Having a diagnosis of dementia does not necessarily mean that you have to give up driving immediately, however you do need to inform the DVLA (Driver and Vehicle Licensing Agency) and your car insurance company of your diagnosis. The DVLA then makes a decision about whether you should still be driving, based on information they receive from your GP or consultant. They may decide to renew your license for a short period, ask you to take a test or cancel your license.
There are a lot of processes involved in driving, including; memory, decision making, vision, motor skills and concentration. Any of these processes can be affected in someone with a diagnosis of dementia, so it is important that your safety, and the safety of people around you, is assessed. If the decision is made that you should stop driving, you will likely need some support to help you adjust to this, and may need to find out more information about alternatives, such as taxis and community transport, to help you get around. For more information on driving and dementia, please see the Alzheimer’s Society factsheet.
My partner and I have lost motivation to travel because it can be difficult and stressful. Are there any tips to help us?
Many of our members talk about needing extra time to plan and prepare for travel, and people’s tips include:
– Getting taxis to avoid public transport (escalators, trains and tubes can be really stressful)
– If using escalators, someone standing in front of the person with dementia can decrease anxiety
– Allowing plenty of time
– Pre-planning/thinking ahead
– Finding strategies to overcome frequent areas of difficulty can reduce a carer’s stress, but also planning workarounds in advance can often be helpful
– Choosing routes that involve less transfers and changes
– Using the Mobility Exemption Pass for the M6 toll if that affects you, and looking for similar arrangements for other routes. You can find details on a number of major toll roads by visiting the Blue Badge Company website has details on a number of major toll roads.
– The locks on public toilets can also be challenging and a number of members have purchased RADAR keys, which allow independent access to accessible toilets all over the UK. You can purchase RADAR keys on the Disability Rights website.
What about tips for travelling abroad?
It is best to contact an airline/airport in advance (at least 48 hours) to let them know of any difficulties and to see what support can be offered. It is also worth mentioning to cabin staff when boarding so they are aware and can provide assistance where required. Some airports offer a special service such as using a sunflower lanyard to make staff aware you need extra help, which several people have found beneficial. To order a sunflower lanyard and search for which organisations are participating, visit the Hidden Disabilities Sunflower Lanyard Scheme website.
To find out more information about what airports offer, you can visit the individual websites of the airports you are travelling with. For example, visit the Gatwick, Manchester and Heathrow websites.
We have heard mixed experiences from people who have requested passenger assistance when travelling. This needs to be booked in advance (at least 48 hours) and there is some more information about how to organise this from the UK Civil Aviation Authority website. Customs was mentioned as an especially difficult part of airport journeys and one couple were directed to a quiet route once they asked for assistance. Another group member mentioned that he tells airport staff at customs about his wife’s visual impairment at the electronic passport gates so they can be taken to a manual gate.
For travel insurance, support group members have recommended Staysure, Holidaysafe and Able2Travel. Others arranged their travel insurance via their bank.
I would like to go on holiday with my wife who has dementia. Are there organisations that will help me with this?
There are a number of organisations that offer supported holidays for people with dementia. Members have recommended:
– Dementia Adventure: Offer small group adventures and bespoke holidays for people living with dementia and their friends/family/carers. They also run free events for family carers – visit the Dementia Adventure website for more information.
– Revitalise: Provide respite care in a holiday setting for people with disabilities and their carers, as well as people with different forms of dementia. Visit the Revitalise website for more information.
Management strategies
What tips do you have around managing anxiety?
We know that many people living with dementia and their carers experience anxiety.
The Alzheimer’s Society has some helpful information about managing anxiety for people living with dementia here, and they acknowledge that anxiety can be especially common for people with dementia who have good insight into and awareness of their condition.
There is also some information for carers here.
If you are feeling overwhelmed, don’t hesitate to contact your GP, NHS 111 or a helpline for support:
Samaritans: Call 116 123 or email jo@samaritans.org – Open 24 hours, 365 days a year
Age UK: Call 0800 169 2081 – Open 8am- 7pm, 365 days a year
Alzheimer’s Society Dementia Connect support line: Call 0333 150 3456
Dementia UK: Call 0800 888 66 78 or email direct@dementiauk.org
Mind: Call 0300 123 3393, email info@mind.org.uk or text 86463.
Has anybody had experience of getting a dog or a cat after receiving a diagnosis?
Some members have found that having a dog and a cat can be really helpful for somebody who has received a diagnosis of a rare dementia. Many of us enjoy the company of animals, and they can provide a daily structure of walks and other activities which can have a positive impact.
Is there any technology to help people with dementia that have problems with their vision?
There are a number of different options of assistive technology that can help people with visual impairments. Here are some technologies that members have previously recommended:
– Audio Labelling: particularly useful in the early stages
– Amazon Echo: good for voice calling, calendar reminders
– Voice activation on smartphones (Siri on iPhones, Cortana on Android) for making calls, writing and reading texts and emails
– Microsoft Accessibility: vision, hearing and mobility related tools
– TV: audio description and an easy to use remote
– Reading: the Read-Clear application has adjustable display settings to assist with gaze stability, gaze direction and trouble seeing cluttered letters. Members also recommend audiobooks and radio plays, for example from Amazon or BBC. Large print books may help for some people, but can make reading harder for others.
Where can I find useful aids/adaptations?
The following online shops were recommended as sources of some useful aids and adaptations for managing a range of dementia challenges:
– Daily Living Aids (Age UK) or call them on 01214 370 033
– Complete Care Shop or call them on 03330 160 000
– Adaptive Clothing at The Able Label and Tommy Hilfiger
Management strategies
Can you offer any advice with dealing with constant singing (not always tuneful)?
Sometimes the biggest challenges for carers don’t come in the form of the big things, but rather in the form of little things that can push us ever closer to the edge. Repetitive singing (and also humming) definitely falls into this category – and all the more so if the singing isn’t tuneful!
It might help to know that there is in fact a neurological ‘reward system’ that is triggered within the brain by humming and singing. Repetitive humming and singing is in part to do with this system being not only triggered, but caught in a sort of ‘loop system’ from which the singer (or hummer) can’t extract themselves.
We all ‘feel good’ when we sing – and remembering this whilst you are trying to live with this behaviour might help to a degree! At least someone is feeling good – even if it’s not you!
In addition to the above, it is worth considering what other causes there may be for the singing:
With the impact that FTD has upon words and language, singing may in fact be providing a simple form of communication, making up for limited vocabulary and a decreasing ability to express oneself.
Compulsive and repetitive behaviours are part of the FTD template…so it isn’t surprising that we find repetitive singing in people living with FTD.
Neither asking the person to stop, reasoning with them, nor criticising them will be helpful. A more therapeutic approach will be for you to find ways you can discreetly and productively ‘manipulate’ the situation whilst ultimately accepting that which can’t be changed – and holding on to the knowledge that this is likely to be a phase and it won’t last for ever.
What might help?
• You could you try asking the person some questions about the music they are singing, or talking about music more generally. Sometimes getting a person to talk may break the repetitive cycle.
• Try something like ‘Who sings that song?’, ‘I remember liking xxxx (an artist you know they like) and the songs he sang…what was that one we both liked…xxxxx? Let’s listen to that now?’, ‘My favourite singer is xxxxx. Let’s listen to a song by him now’, etc.
• You could try playing other songs which you know the person likes, and actively engaging them in trying to sing along with the lyrics. Perhaps you could make it a joint activity and sing along too!
• Try to increase the sensory stimulation available in other domains and direct the person’s attention towards it, or to other things that are happening around them. External auditory cues or alternative sensory inputs/distractions might help distract from the singing. For example, ‘Look at that red car driving slowly down the road’, ‘Listen to the birds outside’, ‘Can you smell the cake I am baking…remind me what your favourite cake is’, etc.
• Other things you might want to try could include talking books, providing access to a musical instrument (ideally one you can tolerate – chimes, xylophone, etc.) Think about ways in which you can actively engage all 5 of the senses and try to find inputs that will engage these.
• Looking through photographs or magazines and talking about the times/ images they reflect might provide a distraction from the singing.
• Under the present circumstances it is difficult to get out and about, but if you are able to get outside at all, this might help.
• Can you engage the person in meaningful or helpful activities/chores? Sweeping the floor, polishing, sorting are all activities which might distract.
• Try having music, audio books, podcasts, etc. playing in the home as a distraction and alternative input.
• A final suggestion might be to try using personal headphones – either for the singer to be possibly distracted by or for you to listen to something other than the singing!
My wife who has FTD is confused about day and night times. She can close curtains and start to undress to go to bed at 2 in the afternoon.
Being disorientated about day and night time and having disturbed sleep patterns is a relatively common symptom in people living with a diagnosis of dementia, including FTD. More specifically, it is not uncommon for people with FTD to want to sleep for longer than they previously did. Daytime sleepiness is also common.
It is however, always worth taking a step back and looking at the bigger picture.
Could it be that your wife is actually tired at this point in the day? Is she sleeping well enough and for long enough at night? Are there medications that she is taking which could be causing her to feel sleepy at this time of day?
If any of the above are possibilities, it is worth addressing changes in these areas wherever possible.
Generic advice would include making sure that the room where your wife spends the day is well lit and with as much natural light as possible. Some studies suggest that light therapy may be helpful, and whilst this isn’t conclusive, it might be worth trying. Access to a clear and readable clock is also advisable.
In terms of practically managing the 2pm slot at which your wife performs the ‘return to bed’ ritual you describe, it may be helpful to create a routine to the day which means that she is actively engaged in other meaningful activities at 2pm.
Some suggestions are:
• Consider adjusting your mealtimes so that lunch is happening over this time period, or making 2pm the time where you make a drink and get a snack together.
• Plan to be outside of the house at 2pm.
• Try to get your wife engaged in activities which engage as many other sensory domains as possible at this time. Providing external auditory cues or alternative sensory inputs/distractions might help to shift away from the 2pm ritual you describe. Perhaps consider: Looking through photographs or magazines and talking about the times/ images they reflect
• Listen to music together – perhaps having a singalong at the same time! You may find that headphones are an alternative and can be useful too
• Set up a ‘painting by numbers’ activity or other manageable craft activity to engage her attention at this time. Craft shops have all sorts of activity kits designed for people across a wide range of abilities – even Play-Doh can be a therapeutic medium for adults as well as children!
• Engage your wife in meaningful and helpful chores – for example, helping you prepare ahead for the evening meal, sweeping, polishing and sorting are all activities which might distract from the 2pm ritual.
Getting enough sleep is critical for the well-being of both your wife and you.
Try to keep a good sleep routine, keep the bedroom just for sleeping if possible. Shut off screens at least an hour before bedtime and have a warm drink or bath to aid restful sleep.
If you are fitful and waking during the night, try getting up and leaving the bedroom (if it is safe to do so) and make yourself a drink, perhaps read a book, or listen to some quiet music or a podcast, and then go back to bed.
How do I manage if the person I care for needs to go to hospital but cannot speak in any meaningful way to hospital staff?
The RDS Direct Support Team has put together a Hospital Resources Pack for each rare dementia subtype – please contact us directly if you would like to access these at contact@raredementiasupport.org.
You can also create a brief booklet with essential and relevant information about your husband and tips to support the healthcare professionals to communicate with him. You can also ask them to video call you when they need to interact with him, so you can provide support. It is important that the staff looking after him are aware of his communication difficulties and provided with some guidance about how to handle them.
If possible, write in advance to the medical teams your husband is due to see and, if he is an in-patient, provide a notice to be tied/placed securely at the head of the bed that explains your husband’s diagnosis and the support he needs (notices are available in the Hospital Resources Packs we have created – please contact us at contact@raredementiasupport.org for access.)
Do not assume that people on the ward will know what PCA is; the patient may know more about the condition than the healthcare professional who may be anxious about new conditions, so use your expertise. You can also direct them to the RDS website here.
Should we be pressing for a better solution for care homes? How?
You can find advice on how to contact your local MP here.
Research
How can I get involved in research?
Several members of our support group have also taken part in research studies into PCA. Much more research into PCA is needed and it will be crucial if we are to establish effective support strategies and treatments. There is currently a team of neurologists, neuropsychologists, brain imaging experts and geneticists researching PCA at the Dementia Research Centre and Institute of Neurology in London. You can find out more about current studies on the Dementia Research Centre website.
If you would be interested in getting involved with current research, please get in touch.
