NOTES FROM SMALL DISCUSSION GROUPS
We regularly devote time during our London meetings for ‘small discussion groups’, during which we encourage members to talk in small groups about whatever they would find most helpful. We know that the advice and support shared during these groups can be incredibly helpful, and so we will often ask one of our team to quietly sit in on each group and take notes about the themes, questions, and advice being shared.
Here, we have attempted to distil some of these questions and answers into a format that we hope will be helpful to all of our members, whether or not you are able to attend our London meetings.
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.
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: Lots of members talk about the confusion around PPA – not many people will have ever heard of it before. 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 – e.g. NHS Choices/ Rare Dementia Support. If you want to ask any questions of Nikki Zimmermann then you can always get in touch with her via email (firstname.lastname@example.org) – she 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 at previous meetings have talked about coming to the support group and questioning the specific diagnosis they had been given based on the other people they met with apparently 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. 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 here: https://www.uclh.nhs.uk/OurServices/ServiceA-Z/Neuro/CDC/Pages/refer.aspx. 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, as we are currently working on building a list of specialist clinics outside of London.
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 may 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 proteins.
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. 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 are currently undertaking some research into this – many of you will have helped us by answering an online survey that we sent round at the beginning of 2019 – and we’re hoping to move forward with the next phase of this research very soon.
Q: What is the prognosis for someone with PPA?
A: It is really difficult to answer this question, mostly because we just don't know what the prognosis is for each person with PPA: one person might progress quite quickly, whilst for other people 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 done in order to give more accurate prognostic information, as 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.
Q: How do I get involved with research?
A: Several members have taken part in research studies into PPA with the same team who run the Support Group. Much more research in 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 (see below) and we may ask people to have a lumbar puncture and take part in other specialised tests. We ask people volunteering for research to come to a research visit spread over two or three days, and if possible, repeated 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 email@example.com
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: 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: Videos from all of our support group meetings are now routinely being put on our Youtube channel here:
We are hoping to make some big changes to our website and online provision over the next few months so please do let us know if there is anything you would like to see covered that we don’t offer at the moment.
Q: As the family member 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, and having supportive relationships with friends, family and/or a professional counsellor can all help. See https://www.bacp.co.uk/about-therapy/how-to-get-therapy/ 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: Who should we tell about the diagnosis?
A: 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 – PPA can be really difficult to explain. Other members have described how 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.
In terms of telling strangers about the diagnosis, partners of some people with PPA have reported often taking up the role of being an ‘ambassador’ for the person with a diagnosis, perhaps explaining that they are not being rude – but that they aren’t answering a question, or behaving in a slightly odd way, because of the PPA. Some people with PPA at our meetings have noted that worry about not being able to communicate with strangers has made them much more reliant on their spouse/ partner.
Q: What type of benefits are available to me as someone with PPA?
A: There are a number of different benefits someone with PPA 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. If you are over State Pension age, you may be eligible to apply for the 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.
- It is important to note that there is no mobility element of AA. If you are under 65 then you should apply for PIP if you have mobility needs, rather than waiting to apply for AA, as payments will continue past state pension age if you are already receiving PIP.
The forms for PIP and AA can be difficult to complete. If you would like help completing these forms you can contact your local Citizens Advice Bureau. Depending on where you live, there may be other local organisations that can assist with the application as well.
Members have also recommended keeping a 2 week diary of ‘helping’ e.g. every time you get up in the night, how long for etc. to ensure you paint an accurate picture of how much assistance the person with PPA needs. 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 PPA 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.
- 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.
- 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.
- Vehicle tax exemption: if you get certain benefits you can apply for an exemption or reduction in vehicle tax.
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 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 here: https://www.alzheimers.org.uk/blog/how-apply-blue-badge-person-dementia.
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 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: How do I know when is the right time to exercise my power of attorney?
A: Members here have 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: Is there a way that I can record what I want to happen in the future if I have difficulty making decisions for myself?
A: 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. There is 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’. More information about Advance Decisions can be found on the NHS website.
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: My husband has started to have difficulties navigating recently and I’m worried that he might get lost. Is there an easy way that I could ‘track’ him if this happens?
A: There are several different devices and trackers that might be helpful in situations like this. One thing suggested in our discussion groups previously 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 us know – firstname.lastname@example.org. These are always available at our meetings 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: Is there anything we can do to make cooking safer?
A: Lots of people have said that people with PPA can find 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, follow instructions – and 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 locking cooker valve 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 their website at https://www.sgn.co.uk/LCV/
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 activities can people continue to enjoy after a diagnosis of PPA?
A: This can vary quite a lot, depending on what the person enjoyed before their diagnosis, and the specific subtype of PPA they have. Some people in the past have reported enjoying playing table tennis. Others have noted that people with a diagnosis of nfvPPA can sometimes sing better than they can speak, meaning that choirs and singing groups can be really helpful and enjoyable for some people. Walking is regarded as an enjoyable activity for some people, and 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 – although may not be so appropriate if the person has difficulties with movements. Other people have said that they 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 – but for other people, it can be really unnerving that 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 setting a routine, and ensuring that every day of the week has a focus, which works really well for them.
Q: I am having a lot of difficulties with reading and writing. What can I do to help manage this?
A: 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, sometimes audiobooks can 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 was developed at UCL for individuals with acquired reading problems (e.g. post-stroke aphasia), and research on 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 stroke and the researchers believe that it may be beneficial for people with primary progressive aphasia (PPA) who have particular difficulties with reading. 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 later in the Summer of 2019. More information is available at www.ucl.ac.uk/aphasia and if you would like to be notified when iReadMore is available on the Google Play store, please e-mail: email@example.com.
If reading is becoming very difficult and frustrating, however, it might be 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.
Q: Has anybody had experience of getting a dog or a cat after receiving a diagnosis?
A: Some members have found that having a dog and a cat can 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 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. 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 have agreements in place so that the person with a diagnosis will 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 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 lvPPA or nfvPPA while difficulty understanding road signs or signals or behavioural issues can be key in svPPA.
Q: My wife has become quite obsessed with knowing the time. Is this part of her PPA?
A: Yes – many people have reported that PPA has led to changes in attitude toward time. Some people do indeed seem to become more obsessed with time, needing to know the exact time, and wanting things to be done at the same times every day, or finding it very difficult to cope if something take place at a time other than what has been agreed. In others, the concept of time seems to be gone completely, and some people may have difficulties projecting themselves forward or backward in time to imagine a future situation or to remember the past. There currently isn’t much research into this – and one of Prof Warren’s PhD students, Mai-Carmen Requena-Komuro is studying time perception in PPA for her PhD. If you have any thoughts or observations about this, you could email Mai-Carmen directly at firstname.lastname@example.org
Q: Is hearing affected in PPA?
A: 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 really 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 – using a ball to regulate who is allowed to talk when – e.g. 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 said that 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: If someone already has problems with their hearing, are they more likely to develop PPA?
A: 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 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.
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 from seeing correctly. 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: Unfortunately, this is something that several of our members have reported. Dr Sobel spoke about apathy a little bit in her talk at our Support Group meeting on 6th September 2018 – 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, who spoke at our September 2018 meeting, gave a nice overview of some of these and you can see 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: Is there any evidence of a link between between PPA and long-term antidepressant use?
A: There is some evidence to suggest that certain types of antidepressants are associated with a slightly increased risk of dementia. However, it is important to note that there is no evidence to suggest that antidepressants cause dementia, only that there may be some sort of association. What is clear from research is that antidepressants can be really beneficial for some people living with different forms of dementia, so it is very important not to make any changes to a person’s medications without seeking advice from your GP and/or consultant.
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 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. 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. Speech and language therapy isn’t just for the person with PPA – communication partner training can also be really helpful in training communication partners (often the person’s husband/ wife/ other family member/ friend) to make their speech easier for the person with PPA to understand.
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 (https://www.assistiveware.com/products/proloquo2go), which they had been given access to via the NHS. Another option used by members is React2 (https://www.react2.com/) but there are many others and this should ideally be discussed with a speech and language therapist. Some members have noted that some of the technology in apps can be difficult for people with PPA – phones/ iPads 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 early in the course of the disease can be helpful later on.
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.
A: 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 alternatives, e.g. “hot” and “cold”) tends to be most common in nfvPPA, and can be incredibly frustrating. Some members have reported finding it helpful to try using ‘non-verbal’ responses, e.g. using thumbs up or down, or pointing up or down to indicate “yes” or “no” – but these strategies don’t work for everybody. A speech and language therapist may be able to help with alternative communication strategies. Some members have suggested avoiding using direct questions.
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