IJLCD - 'Like going into a chocolate shop blindfolded': What do people with primary progressive aphasia want from speech and language therapy?

'Primary progressive aphasia (PPA)' is an umbrella term for a group of language-led dementias which leave people unable to communicate effectively with others. PPA can have a devastating impact on people's ability to function and socialise. Speech and language therapy offers a range of potential benefits to people with PPA. However, until now there has been little research asking them and their families about what help they would like from speech and language therapy.  In this podcast we chat with Chris Hardy, Anna Volkmer, Jason Warren about their research (*and that of colleagues) about their research to answer that question.

The paper is: 

‘Like going into a chocolate shop, blindfolded’: What do people with primary progressive aphasia want from speech and language therapy?

*Maria Loizidou, Emilie Brotherhood, Emma Harding, Sebastian Crutch, Jason D. Warren, Chris J.D. Hardy, Anna Volkmer

First Published: 
30 November 2022

Useful resources:

Access the paper here:
https://onlinelibrary.wiley.com/doi/full/10.1111/1460-6984.12818 

Further resources:

Rare dementia support
https://www.raredementiasupport.org/primary-progressive-aphasia/

A. Volkmer, J. Cartwright, L. Ruggero, A. Beales, J. Gallée, S. Grasso, M. Henry, R. Jokel, J. Kindell, R. Khayum, M. Pozzebon, E. Rochon, C. Taylor-Rubin, R. Townsend, F. Walker, S. Beeke & D. Hersh (2023) 'Principles and philosophies for speech and language therapists working with people with primary progressive aphasia: an international expert consensus' , Disability and Rehabilitation, 45:6, 1063-1078, DOI: 10.1080/09638288.2022.2051080 
https://www.tandfonline.com/doi/pdf/10.1080/09638288.2022.2051080?needAccess=true&role=button

Marshall, C.R., Hardy, C.J.D., Volkmer, A. et al. Primary progressive aphasia: a clinical approach. J Neurol 265, 1474–1490 (2018). https://doi.org/10.1007/s00415-018-8762-6
https://link.springer.com/article/10.1007/s00415-018-8762-

NOTES:
For RCSLT members, access this paper by navigating to the IJLCD website from our A-Z journals list here. Also, if you would like further information on the research terms used in the podcast, or many other aspects of research design, please navigate to the ‘Sage Research Methods’ collection from the Research Methods page of the RCSLT website’.

The interview is conducted by Jacques Strauss, freelance producer, on behalf of The Royal College of Speech and Language Therapists.

Please be aware that the views expressed are those of the guests and not the RCSLT.

Transcript

Transcript Name: 
ijlcd-like-going-into-a-chocolate-shop-blindfolded-what-do-people-with-primary-progressive-aphasia-want-from-speech-and-language-therapy (1) 

Transcript Date: 
14 July 2023 

Speaker Key:
HOST: JACQUES STRAUSS 
ANNA: ANNA VOLKMER
CHRIS: CHRIS HARDY 
JASON: JASON WARREN

 

MUSIC PLAYS: 0:00:00-0:00:09 

HOST: 0:00:09 Welcome to the RCSLT podcast. My name is Jacques Strauss. This is an IJLCD edition of the podcast, that is, the International Journal of Language and Communication Disorders, in which we talk to authors about research they’ve undertaken and articles they’ve published that we think may be of interest to the wider SLT community, other allied health professionals, and beyond. 

Today’s paper is about primary progressive aphasia – PPA – a rare dementia that affects a person’s speech and language. It’s complex and difficult to diagnose, and conventional models of aphasia and dementia treatment do not meet the needs of patients with PPA. 

However, I should add that our guests pointed out that this research is of interest to anyone who either works in the field of dementia or is affected by dementia generally.

The title of the paper we are talking about today is ‘Like going into a chocolate shop, blindfolded: What do people with primary progressive aphasia want from speech and language therapy?’

As always, links to the paper and other resources can be found in the show notes. 

We were joined by three of the paper’s authors, and I started by asking them to introduce themselves. 

ANNA: 0:01:16 My name is Anna, I’m a speech and language therapist by background, but I’m also a researcher now at UCL and I’m interested in primary progressive aphasia, which is the topic we’re going to be discussing today. 

CHRIS: 0:01:30 Hi there. I’m Chris Hardy. I’m a senior research fellow at University College London, where I’ve worked with Jason Warren for about ten years now, and I’ve also worked with Anna for probably eight or nine of those ten years as well, also working in the area of primary progressive aphasia. And I’m not a clinician, but I’m a research psychologist with a special interest in these diseases. 

JASON: 0:01:53 I’m Jason. I’m a clinical neurologist running a cognitive clinic in young onset dementias, and progressive aphasia as being one of those key interests, and also the research group that includes Chris as senior postdoc for about the past 20 years looking at physiological biomarkers of dementia, particularly hearing and communication, and I’m a collaborator of Anna’s. 

HOST: 0:02:18 Jason, I wonder if you could tell us what primary progressive aphasia is? 

JASON: Yes. So, primary progressive aphasia is a group of rare language-led dementias, so they are neurodegenerative language disorders. They’ve been recognised for well over 100 years, actually, but were rediscovered about 40 years ago and since then had a real explosion of interest, led by clinical neurologists. The consensus diagnostic criteria defining the different forms of PPA was published in 2011, so particularly since that time they’ve attracted huge interest, clinical and research interests.

Three major varieties of PPA are recognised, and they are defined by their clinical features and also their anatomical changes, which we can see on brain scans and, to some extent, by the underlying brain pathology, which are all within the neurodegenerative disease spectrum. 

So, the nonfluent/agrammatic variant of PPA is led by a problem with speech production, either the articulation of speech sounds or a agrammatism or varying combinations of those, and that’s due to atrophy, usually centred on the left inferior frontal gyrus, and usually underlying brain protein tower accumulation. 

The semantic variant of PPA, which is led by problems with vocabulary but part of a wider problem with semantic knowledge, so word meaning and word-finding is affected very early, but the syndrome then encompasses other changes with visual auditory and other information as the disease advances. This is due to focal atrophy of the left temporal lobe, usually due to one particular form of brain protein, TDP-43 protein. 

And the last of the major syndromes is logopenic aphasia, which is characterised by problems with word-finding, and on testing particularly problems with reduced and verbal short-term memory or phonological memory. And this is, in most cases, but not always, a form of Alzheimer’s disease. 

About 30% of patients with PPA do not fall into one of those three categories, which makes for really quite a difficult clinical situation. And also, even within the major categories things are not as simple as I’ve just painted them, in that we find a lot of variation between patients, different combinations of problems, and also variations in the underlying brain pathology. 

So overall, PPA has been a very, very complicated and confused area of neurology in terms of the classification of diagnostic systems and it’s also presented major problems of treatment, which we’ll be focusing on for the large part of today, to some extent because it’s so different in its challenges from the vascular aphasias of classical aphasiology. 

HOST: 0:05:34 So, just for the layman like me, am I correct in saying we haven’t really made terrific progress in terms of drugs when it comes to the treatment of dementia? 

JASON: 0:05:44 Yes, that’s right. And so, like all dementias at the moment, they are not curable. We’ve just – just – within the last year had the first inkling of a genuinely treatable dementia in Alzheimer’s disease, where we actually have what seem to be effective disease-modifying treatments. So, it’s not clear whether, for example, the logopenic variant of PPA will benefit as a form of Alzheimer’s from that. 

But at the moment, the treatment is symptomatic treatment, both drug treatment and also supportive interventions. 

HOST: 0:06:19 Anna, I wonder if you could just give us an explanation of what clinical question you were trying to answer. 

ANNA: 0:06:24 Of course. So, as Professor Warren was describing, there aren’t many available… well, options for people with primary progressive aphasia, and speech and language therapy is the main support that we can provide. But the research literature on speech and language therapy interventions for PPA is mainly focused on what we call impairment-based intervention – interventions that aim to make the weakness better, really target the area of difficulty and try and… and I shouldn’t have used the word ‘better’, really; it’s about maintenance for this group, because we’re not going to reverse the progression of the disease, we’re really looking at maintenance. 

But in reality, what we found is, actually, speech and language therapists spend a lot more time giving advice and strategies to get around the problem than they perhaps do providing some of these practice strategies. 

And there’s actually only been a small number of case studies that have gone and asked what people want. So, we know what the research literature says; we have an idea of what speech and language therapists are doing in the UK, but we don’t really know what the experiences are of people living with PPA. 

So, this study was about trying to really understand the needs of people with PPA and their family members, so that we can understand their experience and, I guess, produce interventions and prioritise service delivery that actually meets the needs of people’s day-to-day lives.

HOST: 0:08:01 There isn’t much, but of the literature that is around, what does it say?

ANNA: 0:08:06 As I mentioned, there’s only a small number of studies. One of those studies was actually something I did! I developed an intervention called Better Conversations with PPA, which is what we call a communication partner training intervention. In part of the coproduction of that intervention I asked people what they wanted that to look like. It was very specific questions that I was asking. 

Separately, there’s a researcher, a speech and language therapy researcher, who I know very well, Margaret [P-o-s-i-b-o-n 0:08:39] over in Australia in Melbourne. She’s published several case studies about the experiences of spouses in particular. One of those papers is called ‘It was a terrible, terrible journey’, and it really describes the experience of the partner of a person with semantic variant PPA. Essentially, one of the key components was how she became more isolated, how the person with PPA became more dependent on them as a spouse. 

There is another paper that was actually written by somebody with nonfluent variant PPA themselves. This paper really emphasises the importance of personalised interventions. So, this was a person who lived over in the States, and they were talking about the fact that they didn’t want off the shelf interventions, they wanted interventions that were refined and personalised and bespoke to their needs. 

But if we look at the broader dementia literature, you can look at that wider literature – even there, there’s very little, and most of the research there has actually looked at what the opinions and experiences are of the professionals who work around the people with dementia, or the family caregivers. 

So, asking people themselves is not something that has been undertaken very much.

HOST: 0:10:00 Chris, I wonder if you could tell us a little bit more about how you undertook the study. 

CHRIS: 0:10:06 Starting at the beginning. This work was part of a larger study called the Rare Dementia Support Impact Study. Rare Dementia Support is a UK-based organisation which focuses on empowering, guiding, and informing people diagnosed with rare dementias, including PPA. And actually, Jason, Anna and myself work together as the joint leads of the PPA support group that falls under the auspices of Rare Dementia Support. 

We recruited people with PPA and their conversation partners from members of that support group. We sent an email out with a brief description of the study, asking people to let us know if they’re interested. 

One important thing to note was that we ran these groups, the focus groups, using video conferencing technology, so people had to be able to use that, which we might come back to a bit later. And then based on the response that we had, we allocated people into a number of different groups, which we tried vaguely to assign based on the subtype of PPA that the person had themselves or the subtype that the conversation partner was most familiar with. We made sure there was no more than eight people in any one group. 

Then the focus groups themselves were led by Maria, who was the first author of the paper, and was quite a brilliant master’s student at the time working with Anna and myself. Maria asked the participants a variety of different questions about their communication difficulties, how speech and language therapy might address those needs, and she used some planned prompts that she discussed with Anna myself in advance, and made reference to a slide presentation that she’d made again in advance with accessible imagery to make sure that the participants in the groups could understand the questions that she was asking and to help guide them through exactly what she was trying to ask. 

We recorded all of the groups then we use something called reflexive thematic analysis when analysing the data. 

HOST: 0:12:13 What were the key findings?

CHRIS: 0:12:16 We identified four key themes, which comprised, firstly, conversation [recording clipped 0:12:19], secondly, adjusting to the diagnosis, thirdly, communication abilities and difficulties, and fourthly, a theme which we called beyond language. 

Within those themes we had a number of different sub-themes. So, conversation partners [burden 0:12:33] comprised two additional sub-themes where people talked about the responsibility of maintaining communication, and also the shifting roles and responsibilities. 

The second theme, adjusting to the diagnosis, comprised three additional sub-themes where people spoke about challenges in navigating healthcare support, they talked about coping strategies, and also a general need for education about PPA. 

The third theme, communication abilities and difficulties was made up of three additional sub-themes, which included developing techniques to manage language symptoms, the skills of the communication partners, and experiences of speech and language therapy. 

And then the fourth theme, which we called beyond language, had one sub-theme, which was personally relevant speech and language therapy. 

ANNA: 0:13:21 I think one of the things with talking about the results verbally is that it’s really hard to convey the words of the people who we actually worked with, and the actual paper itself is supported with heaps of direct quotes, which, short of reading them out, I think is tricky to convey! I would recommend people to go and have a look. 

But they’re really, really inspiring quotes. We even used one of the quotes as the title of the paper, of course. So, the title is ‘Like going into a chocolate shop blindfolded’, which one of the care partners… it was actually a spouse used to describe this experience of going to a speech and language therapist not knowing not knowing anything about what we do for people with PPA, thinking, it must all be really good, but not really knowing. This idea that it could all be absolutely wonderful and fantastic, but just having absolutely no idea what that looked like. 

I think that there’s lots of other quotes, which I would recommend people go and read. I’ve certainly use some of these quotes when I’ve been talking to clients directly, and people have really resonated with some of these quotes and some of the metaphorical language as well. In fact, I should confess we’re writing another paper! Chris has been part of that, but with some other data looking at the metaphorical language used by people in these focus groups because it’s quite powerful, some of the analogies, similes, the figurative language that people use. So, I’d urge people to go ahead and actually read it themselves. 

HOST: 0:15:08 I would certainly recommend reading the paper to get a more visceral sense of the experience of both patients and their conversation partners. 

In the shifting roles and responsibilities theme, one conversation partner is quoted as saying, I just don’t have the patience a carer needs and that makes me a very, very bad carer. And then the adjusting to diagnosis theme, a patient says, a complete loss of confidence because you knew you couldn’t speak. And social withdrawal, if you withdraw and you shut down then you almost don’t want to try anything. 

And finally, in negotiating healthcare, a conversation partner says the following: If it’s a degenerative disease, you’re never going to have a positive outcome, are you? And I don’t know how you convince somebody that putting money into something that is declining is worth it. 

This sense of futility is, I’m sure, a problem in many degenerative and terminal diseases, but we need to resist this trap. Because the provision of healthcare is about the best wellbeing we can manage at all phases of our lives. 

I next asked whether the researcher changed the way the panel thought about the disease. 

ANNA: 0:16:18 I think so. Jason’s nodding. When we’re working in clinic in particular, we often talk about the feedback we get, and we use the information that people tell us. And one of the key points here, I guess, is that we need to meet people’s needs. Perhaps leading on from the comment I made, I think one of the key things it’s influenced us more broadly, also Chris in terms of the support group, is actually the value of education, information sharing. If people don’t know what they can ask for then they can’t ask for it. It’s really hard to advocate for yourself unless you know what you need! 

And so, we know that we need to empower people with the information they need in order to advocate through their GPs, through their neurologists, through their medical team, through their speech and language therapists to ask what they need. 

I know, for example, Chris is actually doing another study – and I should let Chris talk about that – that’s looking at the stages of PPA, and we don’t really understand… previously, we haven’t understood much about that, and this has been a question that’s arisen from people with PPA. And so, Chris addressed that. 

Alongside that, and the information we’ve collected from this piece of work, we’re also looking forward to how this could influence work around developing care pathways. Can we use the information we gather from people, from the work that Chris is doing helping us understand what the trajectory looks like to actually develop a schema, a map, so people can advocate? 

And I guess I feel like I’m going into the next question you were going to ask me, Jacques, but maybe, I guess, what it does tell us also is what speech therapists aren’t doing. One of the things that people with PPA and their families were saying was they were saying when they got speech therapy it was really supportive, really helpful. But lots of people basically said it just wasn’t always available, and they really wanted it. 

At the end of the day, if we can advocate for speech and language therapy because people are saying they really want it, that’s going to be really valuable to commissioners, to policy writers, to the Royal College of Speech and Language Therapy, hopefully. 

HOST: 0:18:43 On the one hand, there’s the problem that the general public often don’t understand what SLTs do, then there’s the problem of access to services, and people need to have a better understanding of what they can get from those services. But if you’re an SLT, and I suppose not a huge number of SLT’s work in this area, but what advice do you have for those that do? 

ANNA: 0:19:08 I’m going to take this question, and actually, I’m going to go back to the way you phrased the question, because actually, we know that speech and language therapists in general practice do see these kinds of clients occasionally. Not all of them. As I said, we’ve got this postcode lottery in the UK, but there are speech therapists who perhaps work in more specialised settings and there are people work in generalised practice who will come across people with PPA a couple of times a year or a couple of times every couple of years. 

What we know from what we found is that different strategies are useful depending on the diagnosis and the disease journey, as well as the personal preferences of the individual. As a speech and language therapist, we need to juggle both the diagnostic differences, so planning different interventions where they’ve got a semantic impairment or a phonological issue, but also think about the progression, so where they are in their disease journey. 

And one of the things we know from this study, and we suspected from the research – we thought this was the case – is that people want early intervention. People were saying it was really important to them that as early as possible that they were linked with a speech and language therapist, and that they could work on impairment-based interventions. So, they wanted to work on strengthening their deficits, but also some people advocated on perhaps practising less used skills in an anticipatory approach, so that perhaps when they might need them in the future they could have practised them in advance. Things like practising gesture and practising the use of writing embedded into interaction, I think, is a good example of that. 

We also recognised from this, and we’ve seen this in the literature, so we know from the literature that the most successful interventions that work on strategies, compensatory stuff, are the ones that work on strategies people are already using, and people expressed a preference for that. They don’t want us to suggest some fancy new strategy, they want us to look at what they’re already doing and refine it. 

This study also flagged that speech and language therapists must use goal-setting and do it collaboratively, so speak to the person and jointly decide what to work on and to connect them. Often people with PPA are quite isolated – I mentioned that at the beginning – so speech and language therapy can’t just focus on the motor speech impairment or the word-finding issue, it needs to focus on interactions, social interactions, social connection. For example, linking people back to things like the PPA support group that Chris, myself, and Professor Warren run is absolutely essential for these people who often don’t know anybody else, who’ve never met anybody else, might not have even heard of PPA. So, it’s really important to people that they get connected. 

JASON: 0:22:03 As a neurologist, I think it’s tremendously important because it’s very, very nihilistic otherwise for people not offering them really anything beyond a diagnosis. And I think this is really transformative in that it focuses on real-world things, actual natural communication, not the sometimes quite artificial techniques that can be used in therapy. It also focuses on capacity, which is very important, again, outside the usual model of dementia, which tends to be very nihilistic and focused on what are you losing? This is this is focusing on what have you got, and how can we maximise what you’ve got? 

The other thing it does is it absolutely integrally brings in the care partner, which is vitally important psychologically as well as therapeutically for the patient because, of course, there’s a lot of frustration and impotence from the care partner often with PPPA, who see this happening and don’t feel they can do much. This is really putting them quite central. 

Just from the standard old neurological model of diagnosis, it’s also potentially very exciting because you come out from an intervention like this with a whole set of new markers for language function – any one of which could turn out to be quite a useful way of tracking that disease, particularly if we do get effective treatments, or even diagnosing it early. Because the ways these sorts of diseases come to light is they don’t come to light with a reduced score on a psychology test, they come to light where people are having problems with their everyday communication, so you want something that measures that.

I’d say it’s really important, and I really hope that we can draw this work to the attention of neurologists, not just speech and language therapists!

CHRIS: 0:23:48 Just to add to what Anna and Jason have said there. I’m not a clinician, so I can’t really comment on the utility for clinicians, but from a research perspective, I think something that’s really powerful about the way that this research, and actually pretty much all of the research that Anna leads, is that this is [inaudible 0:24:07] the traditional model of research it’s very top-down. You have scientists or clinicians who might be quite far removed from the patients that they’re studying, and they impose their theories and ask questions. 

I think the power of this this kind of approach with focus groups where you treat the participants as the real experts, which they absolutely are… Jason and Anna are the two leading clinical authorities on PPA internationally, but of course neither of them knows what it’s like to live with PPA every day, or to live with and care for somebody with PPA every day. And so, I think that sort of humility and being prepared to learn from the real experts by lived experience I think is really important and allows for a greater understanding that could possibly be achieved if we just had that that top-down approach. I think that’s a really important strength to this type of work. 

HOST: 0:25:06 Given that there are very significant challenges within the NHS, and that this research is calling for more resource, more SLT support for these patients, how do we go about building a case for this? 

JASON: 0:25:21 This intervention that Anna’s developed, there is a genuine potential to ultimately conserve resources for the NHS, because if you can get people happier, more functional, less likely to call on other much more expensive interventions, pharmacological and other things for as long as possible, then that makes good economic sense as well as keeping people happier. So, I’d say that there is an important message for the NHS, which is these sorts of specialist interventions can make quite a lot of sense economically. 

HOST: 0:26:01 Chris, are there any limitations to the study that the listeners should be aware of? 

CHRIS: 0:26:06 Yes, sure. I mentioned earlier that we had to run the focus groups using video conferencing because of COVID-19, and so there’s obviously some bias introduced there because we could, by definition, only recruit people who were able to use video conferencing. 

I think more generally, there’s always the possibility of recruitment bias, selection bias. And I think here, as I said, we recruited people from the membership of the PPA support group, but that’s potentially not a very representative sample of the wider UK PPA population. It’s entirely possible that the people who are members of that support group might have more time or more energy, more capacity to seek out that information, those groups, and actually, we might not have sampled from the whole cross-section of PPA across the UK. 

I guess one other potential bias, and we acknowledge all of these in the paper, is one other potential [bias 0:27:17] the fact that… and Anna is, as I said, a highly, highly specialist, highly qualified internationally recognised speech and language therapists. But the fact that some of these emails were coming with Anna’s name in them. Anna was present in the background of the first couple of focus groups to supervise Maria, but that might have introduced some ascertainment bias, in that people might have wanted to respond to please. So, we did try to minimise that by making it very clear that Maria was leading, and Maria made is clear that we were very interested in having positive and negative feedback, but it’s just possible that that would have introduced some bias as well. 

HOST: 0:28:00 It’s great to hear that the reputation of an SLT has to be flagged as a potential limitation in research. 

Finally, are there any key takeaways for listeners?

ANNA: 0:28:17 Probably the biggest take-home message would be that people with PPA and their family members want speech and language therapy at every stage of their disease journey as early as possible. So, that’s the message to neurologists, to speech and language therapists, to GPs, to anybody who meets a person with PPA; if they’re not connected to speech and language therapist, check, and connect them if you can. 

I guess the other thing for me links with what we were saying earlier is that the number of people with dementia in the world are on the increase, and PPA is… we often describe it as an extreme phenotype of dementia. So, people with PPA are a smaller group of people with dementia, but actually, people with other types of dementia have really similar difficulties with speech, language and communication. They’re perhaps just the second or third or they’re another symptom of their dementia. And what we’re doing can be transferable – it is transferrable. With that in mind, we need to plan for an advocate for speech and language therapy for people with PPA. This essentially comes back to this being… I think David Cameron or some politician a few years ago said that dementia is a tidal wave, and I think it is for speech and language therapy. It’s the biggest expanding caseload for speech and language therapists, so we cannot deprioritise it. Even if we’re pressured to. I think we actually need to stand up and shout very loudly and say this is a client group who absolutely need our support, they want our support, and we can hear that; they’re telling us we need to take a person-centred approach and do what they need. And often, what they need isn’t reams and reams of our time. Often what we can do is dip in and dip out, providing them with the right supports that allow them to, I guess, self-manage their… and especially if we’re focusing on things like empowering people to do their own exercises, or have better conversations, supporting family members to think about their own communication techniques, anticipatory work. They’re all things that we can do, where we don’t have to be consistently seeing them, we can dip in and dip out. In other words, advocate for people with PPA and take a person-centred approach. 

HOST: 0:30:37 This is kind of at the pointy end of dementia, and there’s lots of stuff that can be applied to people who suffer from other kinds of dementia. 

JASON: 0:30:46 I think I’d endorse anything Anna has said, and she’s said it much more eloquently. I think very important that neurologists are also involved and feel that they are involved, and they should be recommending and looking to access speech and language therapy for their patients. They need to educate patients, also other medics, other professionals about PPA and its needs. And then when it comes to treatment interventions, it’s don’t just focus on deficits, focus on capacity, and involve the care partner in the process. 

HOST: 0:31:22 A very big thank you to Chris, Jason, and Anna for their time today. Today’s podcast is a good reminder of the importance of finding ways to collaborate and talk across disciplines, and sharing research to help improve the wellbeing of all patients in the NHS. 

Until next time, keep well. 

MUSIC PLAYS: 0:31:38
Ends