NOTES FROM SMALL DISCUSSION GROUPS
PPA SUPPORT GROUP MEETING
6TH SEPTEMBER 2018
At our PPA Support Group in London on 6th September 2018, we held small group discussions. Each of these groups was facilitated by a member of the Support Group, and somebody in each group also took down notes of questions and answers that we thought would be of interest/ relevance to the wider PPA Support Group membership.
Here, we have attempted to distil some of these questions and answers into a single document that we hope will be helpful. This is by no means meant to be an exhaustive directory of questions and answers related to PPA, and whilst we have tried to ensure that all of the information in here is accurate, we cannot be held responsible for any errors or pieces of advice that you may disagree with.
If you have any comments or questions regarding anything contained in this document, please get in touch with Chris Hardy at firstname.lastname@example.org or leave a message requesting a call back on 07388 220 355.
bvFTD = behavioural variant FTD
FTD = frontotemporal dementia
lvPPA = logopenic variant PPA (aka ‘logopenic aphasia’)
PPA = primary progressive aphasia
nfvPPA = nonfluent variant PPA (aka ‘progressive nonfluent aphasia’)
svPPA = semantic variant PPA (aka ‘semantic dementia’)
Q: How do you educate yourself, your friends and your family about PPA?
A: There is a wealth of information on the internet. Sometimes, this can seem overwhelming, and in one of the small discussion groups people talked about the importance of ‘safe googling’ and using websites that you can trust – e.g. NHS Choices/ Rare Dementia Support. If you want to ask any questions of Chris Hardy then you can always get in touch with him via email (email@example.com) – and he is always 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, it is often the case that many medical professionals will never have met anybody else with the disease. Because of this, people can sometimes be given misleading or inaccurate information about their diagnosis. Some people talked about coming to the support group and questioning the specific diagnosis they had been given based on the other people they met with putatively the same type of PPA as them. On asking for a referral within the NHS to a separate 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.
Q: How does a neurologist come to a decision about a diagnosis?
A: The neurologist will want to conduct several assessments to determine whether your language problems are caused by PPA or something else. The first thing they will do is to ask you for a detailed history of what’s being 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 who can accompany you to that first appointment. They will ask for you to have an MRI brain scan so that they can see a picture of what’s happening inside your head. They will often ask for you to have language testing with a neuropsychologist so as to understand a bit more about how exactly your language is affected – 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 really helpful particularly in identifying whether Alzheimer proteins are circulating in the fluid. The nonfluent and semantic variants of PPA usually have problems with the proteins that we see going wrong in frontotemporal dementia (FTD) – whilst the logopenic variant is typically 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 this is likely to be due to Alzheimer pathology.
Q: It all seems so complicated! What is PPA?
A: It is complicated – and 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 degenerative brain disorders (dementias) in which loss of speech and language abilities is the leading and most prominent problem. ‘Aphasia’ refers to a neurological language problem; ‘progressive’, becoming worse over time; and ‘primary’, due to brain tissue changes rather than an external cause. PPA affects both sexes and usually starts between the ages of 50 and 70. However, it can also affect older people, and rarely, younger people as well. Within the umbrella term of PPA, three main patterns of language loss are recognised: the nonfluent variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). nfvPPA and svPPA 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. lvPPA, 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 kind 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.
Q: Should we be worried about my PPA being genetic?
A: In bvFTD, where problems with behaviour are the prominent and earliest symptoms, we know that around a third of all cases are caused by a faulty gene. This is considerably less likely to be the case in PPA, though nfvPPA can sometimes be genetic in nature. The neurologist who sees you should ask for a detailed family history – if you have one or more first degree relatives who have also had a form of frontotemporal dementia (bvFTD or PPA) then this might be a clue to this possibility. If you’re a concerned family member then you can’t ask for the genetic test to be done on yourself – the person affected with PPA would need to have the genetic test and then, if it were positive, you could be tested for the same gene – although this would only be after genetic counselling.
Q: What are the stages of PPA?
A: We know that this is really important but unfortunately we just don’t know the answer to this yet. We have some really basic descriptions of ‘Recognising PPA’, ‘Living with PPA’, and ‘Anticipating problems later on’ on our new webpages (see http://www.raredementiasupport.org/ppa/ppa-early-stages/) but the research needs to be done properly. We would actually like your help with this – you may have heard Prof Warren mention a research study at the start of the meeting that we’ll be starting shortly – and you’ll be receiving an invitation to participate over the next couple of weeks.
Q: 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?
A: Yes – we talked to the people who had made this booklet for the Cognitive Disorders Clinic. They said that they had stopped giving it out because it hadn’t been updated recently, but that we would be welcome to update it ourselves and give it out. So that’s what we’ve done – you can see the updated booklet here: http://www.raredementiasupport.org/wp-content/uploads/2018/09/Essential-information-booklet_webVersion.pdf.
Q: What is a lasting power of attorney and when should we get one?
A: A lasting power of attorney is a legal document that will let the person with PPA appoint one or more people (the ‘attorneys’) to make decisions on their behalf. There are two types: i) Health and welfare; and ii) Property and financial affairs. Members discussing this said that it was important to get both types, 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. You can also add comments at the point of registering, so that e.g. if you are giving one of your children power of attorney, they can read your instructions at the point of exercising it. You can find more information here: https://www.gov.uk/power-of-attorney.
Q: Might we be eligible for a ‘blue badge’ so that we can park closer to supermarkets etc?
A: This scheme is predominantly for people with severe mobility problems, although people with dementia of all kinds are now eligible. You can find more information here: https://www.citizensadvice.org.uk/benefits/sick-or-disabled-people-and-carers/help-for-disabled-travellers1/blue-badge-scheme/applying-for-a-blue-badge/.
Q: These 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?
A: 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 officially endorsed by RDS and we cannot vouch for any of the information or interactions on external sources. The first of these is Facebook – there is a ‘group’ on there called ‘Primary Progressive Aphasia Support Group’ which has more than two thousand members from all over the world. You can find the group here: https://www.facebook.com/groups/441592592551251/. The Alzheimer’s Society website does have an online forum that is moderated, and there are other people who ask questions about PPA and FTD on here: https://forum.alzheimers.org.uk/
Q: Can videos of the talks at support group meetings be put online?
A: Yes. We’re really sorry that we haven’t been doing this routinely for meetings held over the last few years. We have uploaded Dr Sobel’s talk onto Youtube now and you can also access it via our at www.raredementiasupport.org/ppa. The link to watch directly on Youtube is here:
Q: How do I know when is the right time to exercise my power of attorney?
A: Members here talked about the fact that ‘capacity’ for decision making is on a continuum – there isn’t a dichotomous 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 autonomy, but within certain limits.
Q: As the daughter of somebody who has just received a diagnosis of PPA, could I receive any counselling on the NHS?
A:It is always worth going to a GP if on receipt of a diagnosis of a loved one, you as a relative and/or carer are experiencing significant stress or distress. There may be counselling available on the NHS, or the GP may be able to suggest other voluntary sector or private services locally. Having counselling can be invaluable in supporting you through the tough journey of being a caregiver; ideally someone who can offer flexibility of timing and an openness to learning about these rare conditions that they may not previously have come across. Caring for someone who has lost language can be a lonely experience, having supportive relationships with friends, family and/or a professional counsellor can all help. See here for information from BACP about how to access therapy. 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. Trying different things shows that you are making an effort to improve the support and care you provide – and not everything you try will work – but don’t criticise yourself when it doesn’t.
Q: Since we’ve found out about the diagnosis and started to tell friends and family, we’ve noticed that several people drift away and don’t want to stay in touch as much anymore. Why is this and what can we do?
A: This is something that several of the groups mentioned – it was a common theme that people who were once regarded as good friends would melt away 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. However, other people shared positive stories of friends who had been particularly effective at continuing to engage and communicate with the person with PPA. Members talked about how relationships changed, but that these relationships can still be positive. Another group discussed the fact that withdrawal doesn’t necessarily mean that the other person doesn’t care. 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, whilst 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.
Q: Should we tell the police about the diagnosis?
A: You don’t need to tell them directly, but you might want to consider the ‘Herbert Protocol’. This is a national scheme being introduced by the Met in partnership with other agencies which encourages carers to compile useful information 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 here: https://www.met.police.uk/herbertprotocol
Q: How can I find my husband if he goes for a walk and gets lost?X has started to have difficulties navigating recently and I’m worried that they might get lost. Is there an easy way that I could ‘track’ them if this happens?
A: There are several different devices and trackers that might be helpful in situations like this. One thing suggested in the discussion groups 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. You can find more information here: https://www.doro.co.uk/doro-secure-580.html
Q: 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?
A: We make these ourselves at Rare Dementia Support and if you would like to be sent a couple in the post just let Chris Hardy know – firstname.lastname@example.org. We’ve had a lot of requests for these, so will make sure to bring some to our next meeting as well.
Q: I’m worried about X 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 we could do about this?
A: 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 here: https://www.medicalert.org.uk/new-to-medic-alert/new-to-medic-alert, and there are other options too – e.g. http://www.theidbandco.com.
Q: What is a ‘CEA Card’?
A: 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 https://www.ceacard.co.uk/
Q: What sort of activities have other people found helpful for someone who has had PPA for a number of years?
A: Members here talked about many different things that people with PPA can still enjoy. Walking was mentioned by a number of people, as was going to the cinema – many cinemas now have ‘dementia-friendly’ screenings. Physical activities like yoga, pilates, tai-chi etc can be perfomed without any need for language and so these can be really nice for people with PPA. Other people said that they had 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 had a mixed reception – some people thought these were nice as the person with PPA didn’t have to put on an act – but for other people, they found it really unnerving that most of the attendees had typical memory-led Alzheimer’s disease and so were not experiencing the same problems as them. Some members who cared for partners with PPA talked about the importance of setting a routine, and ensuring that every day of the week had a focus, which worked really well for them.
Q: Somebody mentioned that noise-cancelling headphones had been helpful – but I don’t understand why?
A: Yes this is something that many people with PPA tell us. The same parts of the brain that we use to understand and produce speech are involved in understanding and hearing more basic sounds. We know that people with PPA – who have damage to these language regions – can also find it really difficult to concentrate in an environment where there is lots of background noise. So sometimes, if the person with PPA wants to concentrate on something without the distractions of other noises, noise-cancelling headphones can be really helpful. They are based on a very clever idea – unlike conventional passive headphones which are essentially just ‘soundproofing’ (which reduces both external noise and also interesting sounds), these headphones actively and selectively reduce background noise entering the ears, by generating a signal that is the acoustic ‘negative’ of the noise, smoothing it out (a bit like filling in a crack with plaster – more information here: https://en.wikipedia.org/wiki/Noise-cancelling_headphones). They are also, predictably, more expensive than standard headphones.
Q: Has anybody had experience of getting a dog or a cat after receiving a diagnosis?
A: Some members shared that dogs and other animals could be really helpful for somebody with PPA. We don’t need names or words of animals to be able to enjoy their company, and they can provide a daily structure of walks and other activities which can be an incredible therapy.
Q: What have other people decided about driving after you’ve received a diagnosis of dementia?
A: 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. But 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 said that even though they hadn’t been told to stop by the DVLA, they had decided to stop anyway. A helpful way to think about whether or not it’s safe for some people was to think about whether you would be confident to drive children/ grandchildren around (or, if you are a caregiver, would you be happy to let the affected person do this). Other people had agreements in place so that the person with a diagnosis would only drive when accompanied by their spouse – though again, it is important to be confident they are safe, irrespective of whether they are accompanied. If there is 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 lvPPA or nfvPPA while difficulty understanding road signs or signals or behavioural issues can be key in svPPA. For more information visit the DVLA website, or the Alzheimer's Society advice pages.
Q: 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?
A: You should take them for an eye test – you can receive a free test every year when you’re over the threshold age of 60. Evidence shows that both hearing and vision can affect cognition dramatically so it may be the case that the person seems a bit better if you can correct something that’s stopping them seeing correctly. One member reported that cataract surgery had made her feel much better in herself. 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 here: https://www.specsavers.co.uk/home-eye-tests.
Q: My partner with moderate PPA has become apathetic and he doesn’t seem to enjoy most of the things that he used to. Should I just let him sit in his chair watching television for 12 hours at a time and let him be – or should I keep encouraging him to stay active?
A: Quite a few different people said that this was something they were having to confront and wanted to know what other people thought. Dr Sobel spoke about apathy a little bit in her talk at the start of the meeting on 6th September – and you can view that here now: https://www.youtube.com/watch?v=IqV1nlgAkV8&t=4s. 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 (e.g., their personal hygiene) – milder forms, 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 eminently treatable with antidepressant medication. Apathy and depression can coexist so it may be worth covering this possibility, just in case – your GP or local psychiatry team may be helpful.
Q: We’ve read on the internet about cannabis therapy helping with Alzheimer’s disease. Could this help with PPA too?
A: 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, Alzheimer’s Society have written a nice article here: https://www.alzheimers.org.uk/about-dementia/treatments/alternative-therapies/cannabis-and-dementia
Q: What drugs do work in PPA?
A: Unfortunately, there are no drugs available yet 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 lvPPA), 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 gave a nice overview of some of these in her talk here: https://www.youtube.com/watch?v=IqV1nlgAkV8&t=4s
Q: I have PPA and I 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?
A: You are entitled to have an ‘advocate’ appointed for by your council. They can help you to understand your care and support process, talk to you about how you feel about your care, help you to make decisions, challenge decisions about your care and support if you don’t agree with them, and stand up for your rights. There is more information here: https://www.nhs.uk/conditions/social-care-and-support-guide/help-from-social-services-and-charities/someone-to-speak-up-for-you-advocate/
Q: I don’t live with my mum, who has a diagnosis of PPA, and I’m worried that she won’t be able to keep me in the loop about medical results etc. How can we manage this?
A: Your mum 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. You can find more information here: https://www.nhs.uk/common-health-questions/caring-carers-and-long-term-conditions/can-i-speak-to-a-gp-about-someone-elses-health/
Q: Our neurologist told us that there was no point in having speech and language therapy as PPA is progressive. Is that right?
A: No, speech and language therapy can be really helpful for people with PPA and their families. However, some members who had had speech and language therapy themselves noted that they had been really 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. One group talked about how a speech and language therapist should help the person with PPA to consider research into word drills, conversation therapy, and training the partner of the person with PPA to communicate effectively as well. The charity Dyscover, based in Surrey, offers SLT led workshops and courses for people living with PPA: https://dyscover.org.uk/?page_id=39. It is true that the goals of speech therapy are different in PPA than in, for example, stroke aphasia – after a stroke, much more intensive ‘rehabilitation’ to help boost brain recovery may be the objective. 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 PPA and knowledge about it are invaluable.
Q: What sorts of communication aids are there that might help people with PPA to communicate?
A: There are different sorts of communication aids and a speech and language therapist can be instrumental in helping people with PPA to communicate – even if producing speech is really difficult. Some members talked 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 more information about that here: https://www.assistiveware.com/products/proloquo2go, but there are many others and this should ideally be discussed with a speech and language therapist.
Q: How do find a speech and language therapist ourselves?
A: In terms of finding a speech and language therapist, one option could be 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 in your area. That directory is here: https://www.helpwithtalking.com/. Via the NHS, it is absolutely your prerogative to request a referral to be made for you to have access to speech and language therapy. 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 – Chris Hardy (email@example.com) would always be very happy to explain to them about what PPA is and how speech and language therapy has helped other people with PPA. If they are very motivated, they could even read a book written by a highly specialist speech and language therapist and a regular contributor to our support group meetings, Anna Volkmer: https://www.amazon.co.uk/Assessment-Cognitive-Communication-Difficulties-Progressive/dp/1907826181. Speech and language therapy can also be aimed toward a person’s carer so that communication can continue effectively.
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