The emotional journey through the stages of primary progressive aphasia: seven co-produced care pathway recommendations for clinical practice
Anna Volkmer
A * , Yin Boll A # , Margaret Dibben A # , Vanessa Ward A # , Rosemary Townsend B , Jason D. Warren C , Chris J. D. Hardy C and the BCPPA Patient and Public Involvement group
A
B
C
Abstract
Primary progressive aphasia (PPA) describes a group of language-led dementias associated with frontotemporal dementia and Alzheimer’s disease. Speech and language therapy is the main intervention for PPA. Yet, there remains little guidance on the care requirements at the six stages of PPA (identified by Hardy et al. 2024a, 2024b). With the goal of generating care pathway recommendations, this co-produced study aimed to understand the opinions and perspectives of people affected by PPA (both people with PPA and their care partners). Informed by the People with Aphasia and Other Layperson Involvement framework for guiding patient and public involvement in aphasia research, this study used World Café methods to gather opinions and perspectives of people affected by PPA about a care pathway for PPA. Consequently, a survey was used to capture the emotional responses of care partners, also co-authors of the study. The World Café event highlighted the lack of awareness of PPA, and the need for timely access to relevant professionals from pre-diagnosis, during diagnosis, immediately after and then later post-diagnosis. The survey process enabled collaborators to reflect on their emotional responses to the six stages of PPA. From these, seven care pathway recommendations were synthesised. To better understand how we can meet the needs of people with PPA and their families it is essential that research is done with, rather than about them.
Keywords: care partners, care pathways, consensus methods, co-production, dementia, patient and public involvement, primary progressive aphasia, speech and language therapy.
Introduction
Primary progressive aphasia (PPA) describes a group of language-led dementias associated with frontotemporal dementia and Alzheimer’s disease. The term, primary progressive aphasia, was first used in the research literature in 2001 (Mesulam 2001), and the international diagnostic criteria were only agreed on as recently as 2011 (Gorno-Tempini et al. 2011). The consensus criteria describe three disease variants with differing speech and language profiles. Semantic variant PPA (svPPA) describes a gradual dissolution of semantic knowledge, with difficulties in naming objects and recognising word meanings. Nonfluent variant PPA (nfvPPA) is diagnosed based on the presence of either an apraxia and/or difficulties in understanding and using grammar – an agrammatism. Apraxia is a motor speech disorder, which causes difficulties in articulation resulting in effortful and distorted speech. Finally, people with the logopenic variant of PPA experience difficulties in word retrieval associated with a verbal working memory impairment. Of these, the first two – svPPA and nfvPPA – are associated with a frontotemporal dementia pathology, whereas lvPPA is associated with an Alzheimer’s pathology. Although described as a language-led dementia, PPA in not a language-only dementia, and nonverbal and behavioural symptoms are reported from an early stage in all major PPA syndromes (Hardy et al. 2024a, 2024b).
Speech and language therapy is the main treatment for communication difficulties for people with PPA, and referrals to speech and language therapists have been increasing (Taylor et al. 2009; Volkmer et al. 2019, 2020; Battista et al. 2023; Yaşa 2023). Although some speech and language interventions aim to maintain or improve speech and language difficulties, others provide compensatory strategies for the person themselves or address barriers in the environment. A recent systematic review identified 103 intervention studies, of which 73 focused on word retrieval interventions (Wauters et al. 2024). Although word retrieval interventions are most effective in the earliest stages of the disease trajectory (Cadório et al. 2017), beyond this there is little guidance on what the ‘ideal’ timing is for specific interventions for people with PPA and their families.
Current models of speech and language therapy for PPA have provided limited guidance for how to support people at specific stages of the disease journey. Early case studies by Murray (1998) and Rogers and Alarcon (1998) explore the longitudinal treatment of two different people with progressive aphasia (although not termed primary progressive aphasia in these papers, the studies are recognised as among the earliest treatment studies for PPA). They demonstrated the need for speech and language interventions to evolve over time as language deteriorated. A phased treatment model for PPA was proposed, with word retrieval interventions in the mild stages of the disease, compensatory strategies in the moderate stages and communication partner training in the severe stages (Hinshelwood et al. 2016). A more recent consensus study proposed a person-centred approach the named ‘Clock Model’ (Volkmer et al. 2023c). This study synthesised seven cogs that need to be considered and revisited throughout the disease trajectory, including knowing people deeply, preventing disasters, practical Issues, professional development, connectedness, barriers and limitations, and peer support and mentoring towards a shared understanding. The clock model emphasises the continuously shifting nature of the disease journey, the importance of personal preferences and the need to take a broader view, beyond speech and language symptoms to social participation, relationships, autonomy and identity. Connectedness advocates for speech and language therapists to facilitate referrals to peer support networks and other professionals, such as occupational therapists, psychologists and physiotherapists. Demonstrating that people with PPA require treatments beyond speech and language therapy, the model also promotes collaborating with other professionals to help them understand PPA to better support people. Yet, most importantly, none of these models consider the perspectives of the people directly affected by PPA, neither people living with a diagnosis of PPA nor those providing care for them.
To ensure care is relevant to the needs of people with PPA and their families, it is essential to understand the views and opinions of the people directly affected. A recent core outcome set study for PPA asked people with PPA and their families across 15 countries what they would most like to change about their communication, and how PPA affects their lives (Volkmer et al. 2024a, 2024b). The study recruited 82 people with PPA and 91 care partners, recruitment remained biased, and therefore only captured people living with milder stages of the disease; that is, at a stage when extra-linguistic skills are still relatively mild in relation to communication difficulties. Despite this, and in contrast to prior professional guidance, the top-rated construct, ‘Participating in conversations with family and friends’, was identified as more important than constructs relating to more restitutive intervention approaches, such as ‘getting words out’ and ‘being more fluent’. This underlines that research and professional opinion may not always align with priorities of patients and their care partners. Analysis also found that participants desired more certainty about their futures (Volkmer et al., unpubl. data), and care partners prioritised emotional and psychological support for additional behavioural symptoms, in addition to those that directly supported the communication needs of their partner with PPA (Volkmer et al. 2024b).
Qualitative research exploring the experiences of care partners has shown the ‘terrible, terrible journey’ (Pozzebon et al. 2017) that some of those affected by a PPA diagnosis experience. Alongside the lack of certainty (Davies and Howe 2020), this can take a toll on quality of life and well-being (Ruggero et al. 2019). Although PPA may begin as a language-led dementia, the additional cognitive and behavioural symptoms a person with PPA may develop often come as a shock (Loizidou et al. 2023). This is partly inherent to the illness evolution, but also because available educational resources in PPA (and indeed the current diagnostic criteria) generally emphasise communication exclusively – the Hardy staging (2024a, 2024b) does start to redress this lack of information about the PPA prognosis. Hardy et al. used survey methods, asking (154) care partners of people with PPA across the UK and Australia to identify the key stages of the disease. The study results identified six distinct stages within each PPA variant. This is the first study that has explored the prognosis of PPA in such detail. However, in response to this publication, several care partners of people with PPA, members of the Better Conversations with Primary Progressive Aphasia Patient and Public Involvement (BCPPA PPI) advisory group, identified the need to further extend the staging to understand the journey that care partners experience alongside. Prior to this, the preceding BCPPA PPI advisory group (Volkmer et al. 2023b) had advocated for research that could enhance access to specific evidence-based interventions at the right times.
With the ultimate goal of synthesising care pathway recommendations for caring for both people with PPA and their care partners, this co-produced study aimed to:
– Integrate the opinions and perspectives of people with PPA and their families into care pathway recommendations
– Examine and respond to the emotional responses of care partners of people with PPA to descriptions of symptoms of PPA over the six stages of the disease (Hardy et al. 2024a, 2024b).
Materials and methods
The idea of developing a care pathway for PPA was conceived by people with PPA and their families, all members of the BCPPA PPI advisory group. The BCPPA PPI advisory group comprised five couples, where one person had a diagnosis of PPA and the other was their partner. The advisory group participated in every stage of planning and analysing the results of the study, and are represented as a co-author on the paper. This paper was planned and written in partnership with four partners of people with PPA, three of whom are co-authors of the paper. Fig 1 provides an overview of the work undertaken in this study to develop the seven recommendations for a care pathway for people with PPA and their care partners.
Development of the seven recommendations for a care pathway for people with PPA and their care partners.
Informed by the People with Aphasia and Other Layperson Involvement (PAOLI) framework for guiding patient and public involvement (PPI) in aphasia research (Charalambous et al. 2023), the project was conceptualised during conversations in the PPI meetings. Although the PAOLI framework was developed for involvement of people with aphasia, the authors felt the model to be relevant to all those affected by aphasia, including care partners, as it provides a tool to equalise power dynamics, and maximise engagement of people regardless of their level of communication, education and other linguistic or social disparities. This is consistent with the ethics of the BCPPA advisory group, who have advocated for using the same accessible information, consent forms and assessment tools in all aspects of our research work.
Care pathways for people affected by PPA had been identified as a topic of mutual interest during the preparation and planning for the BCPPA project. The World Café event was co-planned to integrate ideas and perspectives from a wider group, to inform the care pathway development. Although the pathway for people with PPA was the main focus, care partners also raised the important issue of their own practical and emotional needs in response to communication and non-communication changes, the latter issues being considered potentially more potent. The PPI group nominated a representative to co-lead the development of a piece of work, using survey methods to examine their own responses to the staging research from care partners in the PPI group.
The study was a co-produced work, as defined by the National Institute for Health and Care Research (NIHR) in the UK – ‘an approach in which researchers, practitioners and the public work together, sharing power and responsibility from the start to the end of the project’ (NIHR 2024). Importantly, the NIHR flags that co-production includes all necessary views and perspectives. This work qualified as patient and public involvement, meaning that no ethical approval was required or sought, although ethical issues were considered, in line with the PAOLI framework.
Procedures
The PAOLI framework outlines four phases. The first two aims of the PAOLI framework are to encourage patient-centred priorities by involving people as partners in identifying research questions and equalising power through co-designing research. The following description of the study development addresses each of the four phases of the PAOLI framework, to explain how the BCPPA PPI Advisory Group developed this research study:
The BCPPA PPI advisory group was formed in late 2022 by the lead author (AV) working on the BCPPA and rare dementia pilot study. Group members were recruited via an email invitation circulated to members of the Rare Dementia Support Group (https://www.raredementiasupport.org/) and other support networks, including Dyscover (https://dyscover.org.uk/) and a Manchester Frontotemporal dementia support group known to AV. A total of 11 people with dementia and a relevant partner responded. AV met online via a video conferencing platform (Zoom) with all 11 people and their partners, five of whom were keen to participate as members of the advisory group. Prior to the first meeting, the researcher drafted a term of reference for the group (based on NIHR guidance) and met with each couple individually. The individual meetings focused on building a relationship between the researcher and the group members, ensuring the researcher could meet the communication needs of the person with PPA, and sharing information about the research plans made to date (including information about reimbursement and honorariums). An initial BCPPA PPI Advisory Group meeting was organised with the aim of getting to know one another and setting group rules. This included exploring formatting for aphasia-accessible agendas and minutes with the agreement that an accessible document using photos, images and aphasia-friendly written information would be accompanied by a video recording (see example accessible agenda in Supplementary File S2). Meeting times and formats were agreed on, and communication supports for discussions (such as stop & go cards, and a general rule that people with PPA should be given the opportunity to respond first).
The remit of the BCPPA PPI Advisory Group was over three work packages comprising: (1) development of an international core outcome set for PPA (Volkmer et al. 2024a, 2024b); (2) development of a care pathway for PPA; (3) delivery of a National Health Service-based randomised waiting list-controlled trial of BCPPA (Volkmer et al.; protocol currently in preparation). At each PPI meeting, different methodological approaches were used to support participation, including voting, brainstorming and drawing ideas. These were based on AV's previous experience of working with people with PPA and their family members to undertake PPI work (Volkmer and Broomfield 2022).
The focus of this paper is work undertaken as part of the WP2 care pathway. Plans to develop a care pathway for PPA had been identified as a priority for further grant funding by the PPI advisory group on a prior study undertaken by the lead author (Volkmer and Broomfield 2022). Consistent with the PPI-led approach, plans to develop a care pathway were explored using a World Café task methodology (Löhr et al. 2020) at a large PPA event attended by 30 attendees with PPA and their family care partners, where participants were asked what they felt a care pathway should look like. The 2-h PPA event is held annually by the lead author, AV, in collaboration with RT, a service lead at a charity that supports people with PPA. Information about the event is disseminated via the charity’s mailing list and clinical networks, inviting people to RSVP to RT if they wish to attend. The event enables people with PPA and their families to come together for peer support, and to hear about different aspects of current speech and language therapy practice. Given this, and the purpose of the discussion to collate opinions and perspectives in a way that reduces inequities in power (in line with NIHR guidance on co-production), it was not felt to be appropriate to collate demographic data from attendees.
A World Café methodology was selected, because it provided a structured conversation process that encouraged people to share ideas and perspectives in a relaxed setting. This also allows for use of supported communication techniques, as described in previous qualitative co-production studies with people with dementia (Bates et al. 2022). The method is designed to promote open and intimate discussion, which in turn promotes equal relationships, thus aiming to reduce both barriers to participation and power imbalances. Five elements of the World Café method (Bates et al. 2022) were considered, including: (1) setting, (2) welcome and introduction, (3) small group rounds, (4) questions, and (5) harvest. AV and RT gave a presentation to introduce participants to the PPA event, and provided an informational talk about speech and language therapy before inviting them to participate in peer networking and/or the World Café discussion.
The World Café event took place in a large open room at the university (1. setting), where participants could enter or leave to an atrium for refreshments whenever they wished. Participants who selected to participate in the World Café event were welcomed to the room (2. welcome and introduction), and further detail was provided on the ultimate aim to develop a care pathway. Consequently, small groups of up to five people were asked to circulate around four tables (3. small group rounds), discussing their experiences and opinions on key priorities for each stage of a care pathway guidance. They were given pens and asked to note down recommendations on flip charts at each table. The first author, AV, provided support for communication, occasionally facilitating the discussion by re-explaining the task using simplified language, writing key words or acting as scribe when participants identified recommendations. When participants had been at a table for approximately 15 min, they were encouraged to move onto the next table. Each table focused on a new stage (4. question), ‘What should care look like at a different stage of the pathway?’: (1) at pre-diagnosis when seeking help, (2) at diagnosis, (3) just after diagnosis, and (4) after that. These stages had been previously selected by the BCPPA PPI advisory group as important stages on their own disease journey. Finally, the ideas were briefly summarised to the wider group (5. harvest) by AV – using accessible language (key words, emphasis and gesture) – at the end of the wider meeting. The only adjustments made to meet the needs of the people with PPA were through the tasks fulfilled by AV, a trained speech and language therapist.
The opinions and perspectives from the World Café task were consequently explored by the BCPPA PPI Advisory Group and integrated with their own experiences. During this process, several of the PPI members – care partners – highlighted specific concerns about the experiences of the partners, who felt a great weight of responsibility, yet often lacked guidance on their role in the journey. It was agreed that there must be a care pathway that includes guidance for the care partners, and that this should be synthesised using any current evidence on the stages of the disease progression as a framework.
At the time of the study, the PPI care partners who participated in this co-produced work were all female spouses or partners, representing experiences of mixed logopenic and nonfluent PPA, ranging in symptom onset from 2 to 6 years. The PPI care partners (MD, XX1 and YB) nominated VW as a representative to support the lead author in planning how to address the issue. The lead author shared several papers about prognosis (Tastevin et al. 2021) and stages of PPA (Hardy et al. 2024a, 2024b) with VW. Through a process of reflexive discussion, the lead author and VW explored how care partners have or may respond to the proposed disease stages outlined by Hardy et al., and what might be done to support and guide families at each stage. To reflect a wider range of experiences, and establish whether a consensus existed across care partners, AV and VW generated a brief survey, inviting the three other partners to share their reflections (see survey questions in Supplementary File S1).
A survey methodology was selected at this stage, as AV and VW felt that it might ensure the most honest and open responses from the care partners if they were able to complete their responses in the privacy of their own homes, due to the often personal nature of reflecting on their emotional responses (Braun et al. 2021). Carefully crafted qualitative surveys can still provide rich, in-depth insights, accessing their language and terminology (Braun et al. 2021). This survey comprised 24 questions hosted on the Qualtrics platform, asking care partners to reflect on their emotional reaction to specific symptoms described as associated with the six stages of PPA (taken from Hardy et al. 2024a, 2024b). A subset of 24 symptoms were selected from Hardy et al. (2024a, 2024b)’s staging models – to reduce the burden of response – as it was felt to be a difficult exercise to address all symptoms. VW and AV jointly selected symptoms through a process of verbal discussion, aiming to include symptoms that spanned disorders and were highly prevalent within the stages outlined by Hardy et al. (2024a, 2024b). Additionally, VW and AV endeavoured to ensure inclusion of one verbal communication symptom, one non-verbal thinking and personality symptom, and one personal care and well-being symptom for every stage where possible (the 6 stages of PPA include predominantly verbal communication and personality/thinking symptoms in stage 1 vs predominantly verbal communication and personal care symptoms in stage 6). VW and AV then reworded the symptoms to form accessible, plain English questions, and where symptoms overlapped across variants, they were combined; for example, difficulty finding names – stage 1, logopenic PPA and difficulties remembering specific familiar vocabulary – stage 1 svPPA, were combined to ‘Your partner has difficulty finding the right word’. Bodily complaints with no apparent cause – stage 3 svPPA was reworded as ‘Your partner has stopped recognising normal bodily functions; for example, doesn’t know to blow nose if it is running’
First, VW, XX, MD and YB responded to each symptom with a statement that best reflected their emotional reaction – how would they feel (or how did they feel) if they have experienced this Second, they were also asked to identify what would be helpful for them when dealing with this situation. AV collated responses, and in a process of triangulation, presented the results verbally to two care partners and members of the PPI group, XX and MD, at a consequent PPI meeting for further reflection and discussion. Results from the study were presented as a written analysis to VW, XX, MD and YB, who reviewed and commented on them.
Given the aim of this co-produced study was to influence clinical practice, specific recommendations were extracted from the results and agreed with all co-authors. Similarly, research recommendations were generated and agreed in the same way. These are reported in the current paper, alongside reflections on how people affected by PPA can contribute to new knowledge. Although the PAOLI framework is designed for people with non-progressive aphasia, this study reports on extending this to those affected by PPA and their care partners. All PPI members involved in this work, VW, XX, MD and YB, were invited as co-authors on this peer-reviewed scientific journal paper that reported on the co-produced study.
Members of the BCPPA PPI advisory group were invited to provide frequent feedback throughout the PPA process. The progressive nature of PPA was an importantly and regularly discussed aspect of reflection. Ensuring frequent rest and fatigue breaks were planned from the outset, group Zoom meetings were avoided unless necessary due to the increased auditory processing difficulties making this more difficult for group members with PPA and leading to a preference for less frequent, but longer, in-person meetings. Additionally, group membership has changed over time, as group members experienced disease progression. Taking time to establish good relationships with all individuals at the outset made it easier for AV to raise and discuss stepping down when group members were no longer able to participate effectively; for example, due to significant comprehension problems, cognitive or behaviour change.
Results
Stage 1: World Café care pathway recommendations
During the World Café event, awareness of PPA and access to ongoing support for both the person with PPA and the care partner were raised as significant areas of concern. This included awareness among gatekeepers – GPs, access to information for people who might have or do have PPA, and specific information about the stages of PPA, and how monitor and address each stage with support from a ‘pool’ of experienced healthcare professionals. Table 1 presents four stages prediagnosis, at diagnosis, immediately post-diagnosis and follow-up post-diagnosis alongside recommendations data generated during the World Café task.
| Stage of pathway – process stages identified as important by the BCPPA PPI advisory group. | Care pathway recommendations generated by the World Café participants. | |
|---|---|---|
| Prediagnosis | GPs to be educated on PPA, so they can be aware of and make patient aware of potential diagnosis of PPA no matter what age they are. | |
| For people with a possible PPA diagnosis and their care partners to be able to access information that explains the symptoms of PPA – such as through appropriate internet websites. | ||
| At diagnosis | To be provided with a clear explanation of what PPA is | |
| Provision of ‘mental care’ at the time of diagnosis (psychological support) to both patient and care partner | ||
| Support for carer | ||
| Immediately post-diagnosis | Connection to someone with experience who can clearly explain what the diagnosis of PPA means and can journey with you continually throughout. | |
| Provision of speech and language therapy with a focus on quality of life, empathy and positivity. | ||
| Provision of a pool of therapists, including speech and language therapy, occupational therapy, physiotherapy and specialist nurse. | ||
| Lifestyle advice to optimise health, and enable patient to live their best life, including dietary and exercise advice | ||
| Being put in touch with peers for support such as offered by Rare Dementia Support | ||
| Access to participation in clinical trials. | ||
| Educate the GP about PPA – empowering carers to be more proactive with medical and paramedical professionals. | ||
| For partner – access to information about the stages of PPA that we should anticipate | ||
| Follow-up support post-diagnosis | Continued support from an expert speech and language therapist who can monitor how we are doing, and provide tailored guidance and strategies. | |
| Regular opportunities to meet with others with PPA. | ||
| Support to continue to do the things we enjoy. | ||
| For partners – access to information about the stages and prognosis of PPA, and how my partner is progressing through the stages from an expert (although it is recognised that some partners might find this overwhelming, the emphasis from people at the World Café event was that this information should be publicly accessible to provide choice). | ||
| For partners – advice on what to do and say to my partner with PPA | ||
| For partners – advice on how to tell family and friends, so they understand | ||
| For partners – advice on how to plan for the future, including communication aid options, future decision-making and crisis planning for emergencies. |
NB: Grey, italicised font has been used to highlight recommendations specific to care partners, whereas black font is used to highlight recommendations for both the person with PPA and their care partner.
Stage 2: synthesis of care partners responses to stages of PPA
The research group, comprising AV and VW, reflected that although PPA is a heterogeneous disease, that care partners nevertheless tend to measure the progression of their partners disease in milestones. This map of the emotional responses to the progression of speech, language and other symptoms of PPA described in Table 2 demonstrates how stress, frustration, sadness and loss reoccur as new symptoms develop. The proposed actions map back to the identified care pathway recommendations outlined in Table 1, highlighting the need for access to a network of support for both the person with PPA and the people around them. Fig. 2 provides a synthesis of specific recommendations for clinical practice generated from this data.
| Symptoms by stage (selected behaviours from across PPA variants Hardy et al. 2024a, 2024b) | Care partner's emotional reaction | Care partner's suggestions for what would or has helped them deal with this situation | ||
|---|---|---|---|---|
| 1 | Your partner has difficulty finding the right word (sv, lv) | Unsafe when he gives incorrect information. Frustrated, as he refuses to tell people. Upset and distressed. Want to be supportive and understand. | If the GP understood. If my partner explained to people. To wait for words. I need strategies to know how to help my partner find the word. | |
| Your partner has difficulty speaking in stressful situations (nfv/lv) | I feel stressed too. We’ve missed out on our best years. Anxious. I want them to make a good impression on others. Downhearted. | Don’t dwell on the future. Live day to day. Having a phone line to talk to someone who understands. If my partner indicates whether or not they want help, this helps me to know what to do. We talk about what my partner wants me to do to help. Anticipating stressful situations and planning for them. | ||
| Your partner doesn’t want to join in social situations (lv) | Excluded. Sad. Resentful. Frustrated. Upset. | Having someone else to help with being with my partner. I go out on my own, because my partner wants me to have a social life. If the community understood more – this would help us. | ||
| Your partner loses the thread of a sentence in conversation (lv) | I haven’t got a clue. I feel lost. Frustrated. Concerned. I feel I need to help. | Patience. Putting myself in their position. Having tools and strategies to help my partner, such as pictures. | ||
| 2 | Your partner doesn’t enjoy activities that involve thinking e.g. reading (sv, lv) | Upset and fearful of future decline. Longing for the things we used to enjoy together. It’s lonely. | I need specialist support to understand and encourage me to continue my activities with other people. Arrange for him to spend time with other people. Help to find activities that we can enjoy together that don’t involve language. | |
| Your partner has difficulty finding their way on a route (lv) | Fearful, this signals further decline. Scared and worried he will get lost if he’s on his own. | Use tools, such as a sat nav or a phone tracker. | ||
| Your partner’s mood is changeable (nfv, lv) | Frustrated, irritated and embarrassed. The feeling that my partner’s personality has changed and they are no longer the person they used to be! | Guidance and tools to manage their behaviours. Support to plan a routine to reduce the risk of this being an issue. A person to offload to. | ||
| Your partner becomes rigidly focused on a topic or activity e.g. a particular subject in conversation or cleaning (sv) | Agitated. Very frustrated and irritated. | Tools and techniques to provide distraction. Respite. | ||
| Speaking requires a lot of effort for your partner (nfv) | I feel sorry for them. Upset for them. Sad to see this happening to my partner. | Communication strategies that do not rely on memory, and that create a positive experience for us both. | ||
| Your partner’s conversation rambles (sv) | Confused. Frustrated and irritated. | Other people to lighten the load. Strategies to keep them on topic in conversation | ||
| 3 | Your partner has difficulty understanding longer sentences (lv) | Fearful for their safety. Upset on their behalf. Feel a responsibility to change the way I speak. | Constant opportunities to talk to someone to help me understand how PPA is affecting my partner. Training to use shorter sentences. Advice on how I can help other people understand that too. | |
| Your partner has difficulty judging distances when driving (lv, nfv) | Apprehensive. Terrified. | Advice from a healthcare practitioner, formal assessment of driving skills and support to remove car keys. | ||
| Your partner cannot manage tasks that have multiple stages like cooking or DIY (lv) | Frustrated. Annoyed. Dejected. | Support to find and plan other activities, and accept these changes. | ||
| Your partner lacks empathy (sv) | Hurtful. Exasperated. Sometimes it is infuriating when I feel I do so much and not feel any response! Never getting a hug is dreadful to deal with. You take it personally. | Support to understand PPA, and know it isn’t personal. Peer support from others in the same situation, and who know my partner. | ||
| Your partner is forgetful (nfv, sv) | Frustrated and annoyed, but also something I might foresee. | Think ahead and plan for him, with support from other people. | ||
| Your partner walks slowly (nfv) | It is heartbreaking to see my partner losing his agility. It is annoying too. | Information about the fact that physical symptoms are in fact part of PPA. Physiotherapy. | ||
| Your partner has stopped recognising normal bodily functions e.g. doesn’t know to blow nose if it is running (sv) | Heartbroken. This is such a fundamental thing. | Expert advice. | ||
| 4 | FOR ALL nfvPPA/lvPPA/svPPA | |||
| Your partner has difficulty understanding questions | Stressed in case they answer important things incorrectly. | Power of attorney. Recognition from healthcare professionals that I can help them understand and help with decision-making even before they lose capacity. Advice to try alternative ways of communicating. | ||
| Your partner needs help dressing | Despondent, frustrated and increasingly helpless. | If it gets beyond trousers and tops we’d need a carer. Occupational therapy guidance. | ||
| Your partner has difficulty recognising familiar people | Hurt, especially if it’s me! Heartbroken and helpless. It is such a loss. | Support to understand PPA, and know it isn’t personal. Peer support from others in the same situation, and who know my partner. | ||
| Moving requires effort for your partner | I’d feel useless and despondent. | Physiotherapy and occupational therapy advice and a carer, as well as tools to help keep them as independent as possible. | ||
| 5 | FOR ALL nfvPPA/lvPPA/svPPA | |||
| Your partner has difficulty understanding simple messages | Stressed. Frustrated. Irritated. Worried about my reactions. Helpless. | Tools and strategies for my partner to communicate feelings and needs, such as symbols for verbs. | ||
| Your partner needs help with basic life activities e.g. eating and washing | Stressed. Worried I couldn’t cope. | Outside help and occupational therapy to provide tools and equipment. | ||
| 6 | FOR ALL nfvPPA/lvPPA/svPPA | |||
| Your partner’s talking is impossible to understand | Heartbroken. At the end of my tether. Lonely. Tearful. Tense. Exasperated. | Advice from the start that he needs to do home practice to reduce the risk of this happening. Communication aids. Outside help. | ||
| Your partner is immobile | Depressing, but anticipated. Lonely. Stressed and extremely difficult. | Outside help such as home nursing or get ready for care home | ||
Recommendations for a clinical care pathway for people with PPA and their care partners. These initial recommendations do not align with specific process or disease stages, rather comprising key components of care to be considered throughout the disease journey from prediagnosis. HCPs = health care professionals; ‘Should’ is used when a recommendation has been made based on qualitative research data and recommendations from people affected by PPA in this study. ‘Must’ is used when the ‘should’ criteria has been fulfilled AND there is strong research evidence demonstrating the positive outcome of these treatments.
Discussion
To better understand how we can meet the needs of people with PPA and their families, it is essential that research is done with, rather than about them. This novel co-produced study methodology demonstrates how people with PPA and their families can be actively involved with driving research agendas that are meaningful and relevant to their lives. This work was born out of guidance from people affected by PPA, advocating for research that also prioritised their needs through the development of care pathways to enable them access to the right supports at the right times – starting from pre-diagnosis, at diagnosis and following them throughout the disease journey. This co-produced work found there is an overwhelming need for awareness raising across health care and the public to ensure timely diagnosis of PPA. Access to information about what PPA is will enable equitable access to care and support for people affected by PPA.
Participants’ experiences in this study highlight a general lack of awareness about PPA among health professionals and the public. As with many rare dementias, this is known to contribute to a delay in diagnosis, often of several years (Davies and Howe 2020; Mouton et al. 2022). The lack of awareness of PPA, and the additional lack of awareness of the role of the speech and language therapist, contribute to a further delay in access to appropriate speech and language therapy interventions (Ho et al. 2023; Beber et al. 2024; Davies et al. 2024a). Family members have reported searching for guidance on communication strategies when their partners have been diagnosed with PPA (Loizidou et al. 2023; Davies et al. 2024a). Yet, nihilistic views of such nonpharmacological interventions, perpetuated by a lack of evidence-based knowledge among the healthcare community, create barriers rather than gateways to care (Loizidou et al. 2023; Ho et al. 2023). This study emphasises a significant need for more collaborative and co-produced work in the shape of a strategic PPA awareness campaign, highlighting both PPA and the available treatments.
We know that families are trying to find meaning in the communication and behaviours of their loved ones with dementia (Volkmer et al. 2023a; Davies et al. 2024a). This co-produced work extends that, demonstrating that care partners are trying to track the progression of their family members with PPA, searching to pin down milestones – comparing the communication and non-communication symptoms they see in their partners with PPA with what they see in other published PPA stages and other literature – and trying to understand how to prepare for and best support their partners. Although having access to information about the physical and emotional stages of PPA may seem overwhelming, the research team identified a need to provide access to information that could also empower people to shape the narrative of their journeys. Importantly, symptom balance changes as the PPA syndromes evolve – meaning care and support needs are not only complex, they are also dynamic. Given the frequency of people with PPA and their family members feeling lost or abandoned on the disease journey (Pozzebon et al. 2017; Davies and Howe 2020; Loizidou et al. 2023; Ho et al. 2023), there is an urgent need for guidance that could prompt referrals to relevant professionals and prevent people from falling through the gaps.
Care partners report a burden of responsibility in the care of their partners with PPA (Davies et al. 2024b). Partners of people with PPA in this study, who co-led the research, have identified emotional vulnerabilities in this role as care partner, for which they need outside help. Care partners are unable to meet all the needs of people with PPA without the right support from peers, professionals and carers. As recognised previously by clinical academic speech and language therapists who developed a consensus set of practice principles for working with people with PPA, the professional support extends beyond speech and language therapy (Volkmer et al. 2023c). Partners of people with PPA have previously highlighted a need for psychological and peer support (Volkmer et al. 2024b; Davies et al. 2024b). This study emphasises that as physical symptoms develop alongside speech, language and communication difficulties in the later stages (4, 5 and 6), this support must come from a much wider range of professionals, including physiotherapists, occupational therapists and, most importantly, in home care support.
Strengths and limitations
This study presents entirely co-produced work, meaning it may be biased by the people who have inspired the study, who are predominantly from well-educated, English speaking, higher socio-economic demographic backgrounds in England. Relatedly, there is the potential issue of selection bias for people who have had a potentially atypically good journey engaging with research in the first place. Although the World Café task captured the views of 30 people with PPA and their partners’ survey study, the survey study only captured the views of four care partners. Additionally, the care partners who participated in the survey study only had experience of caring for their partners with logopenic and non-fluent PPA, all with incomplete experiences of the PPA disease journey. This co-produced study highlights the need for a really important piece of research to be conducted staging caregiver burden (psychological, emotional, practical, physical and spiritual) using surveys or interviews with a larger sample of carers who have lived the full journey, affected by the full range of PPA syndromes. Consequently, the lead author, AV, directly addressed some of these issues, collaborating with several other colleagues to pursue a research program to explore barriers and limitations to accessing support for people with PPA from ethnically and linguistically diverse backgrounds (currently in preparation).
The research team also employed continuous reflexivity, as befits qualitative research work, taking opportunities to consider the implications of their role and relationships. Importantly, acknowledging the power dynamic, with the lead author being a leading speech and language therapist and researcher within the field of PPA, who has worked with people with PPA and their families who collaborated in the study in a clinical role. This familiarity can also be considered a strength, given it enabled AV to provide appropriate communication supports through PPI, and strategies to people with PPA throughout advisory meetings and the communication-heavy World Café tasks.
Implications
Although this study used the PAOLI framework, a tool designed to support involvement and engagement of people with aphasia in research, this is the first to apply this to involvement of people with PPA. This study evidences that this model is applicable to the involvement of people with PPA, but highlights future additional recommendations, such as taking account of the progressive nature of the disease when planning for participant involvement; making provision for people to step down and new people to be recruited where necessary are suggested. Being mindful of the progression of disease also means that clinicians need to be able to constantly adapt and adjust to disease progression over the course of the study, thus meeting with PPI collaborators individually to develop and refine support requirements for each new group meeting is vital.
This study has implications for clinical care of both people with PPA and their care partners. The care pathway recommendations build on previous models of practice for speech and language therapy for PPA (Hinshelwood et al. 2016; Volkmer et al. 2023c), and takes into account the recent research identifying six stages of PPA. We anticipate the seven practice recommendations outlined in this paper will provide a tool for people with PPA, their care partners and other healthcare professionals. They also highlight future priorities to develop PPA awareness campaigns, improve early diagnosis of PPA and access to support, such as speech and language therapy for PPA. Future research in this area should test whether the recommendations proposed resonate with people with PPA, their care partners and health professionals internationally.
Declaration of funding
AV has been funded by an NIHR Advanced Fellowship NIHR302240. JW has received grant support from the Alzheimer’s Society, Alzheimer’s Research UK, the Royal National Institute for Deaf People (Discovery Grant G105_WARREN), the National Institute for Health Research University College London Hospitals Biomedical Research Centre and the National Brain Appeal (Frontotemporal Dementia Research Studentship in Memory of David Blechner). CJDH was supported by a Fellowship award from Alzheimer’s Society, UK (grant number 627). The views expressed are those of the authors, and not necessarily those of the NIHR or the Department of Health and Social Care.
Ethics
The study was a co-produced work, as defined by the National Institute for Health and Care Research (NIHR) in the UK – ‘an approach in which researchers, practitioners and the public work together, sharing power and responsibility from the start to the end of the project’ (NIHR 2024, accessed January 2025). Importantly, the NIHR flags that co-production includes all necessary views and perspectives. This work qualified as patient and public involvement, meaning that no ethical approval was required or sought, although ethical issues were considered, in line with the PAOLI framework.
Acknowledgements
The research team acknowledges all the people with PPA and their care partners who participated in the World Café event in July 2023, and all the people with PPA and their families who shared their journeys with us. We also thank all the members of the Better Conversations with Primary Progressive Aphasia Patient and Public Involvement advisory group, the BCPPA steering committee – Professor Catherine Mummery, Professor Jonathon Rohrer, Dr Paul Conroy, Hannah Luff, Dr Samantha Stern and, additionally, Professor Seb Crutch and Nikki Zimmerman.
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