Member checking

Member checking is the process of presenting qualitative research findings to respondents or participants and inviting their feedback and/or checking for resonance of the findings with their own experiences.39 The advantages and disadvantages of member checking are much contested,40 41 and details of the debate are beyond the scope of this paper, but several issues bear particular relevance here. First, there is a concern that participants may inaccurately recall their original account, and this is perhaps more likely in the case of progressive cognitive decline. Second, developments since the participants gave their original accounts may have changed their perceptions or how they may now choose to respond to the same questions and this too may be of increased likelihood for those living with a progressive condition. For these potentially confounding reasons, we sought external validation of the current findings via two regional PCA support groups, made up of people with a diagnosis of PCA and their family carers (as in the study sample). Study findings were presented to both groups and comments invited, and there was a general consensus across both groups that the themes elicited here were compatible with support group members’ own experiences. Many support group members went on to share their experiences by way of demonstrating the overlaps and coherence with the results of the current study.

To further establish the quality of the current research project, the project was conducted in accordance with the COnsolidated criteria for REporting Qualitative research criteria (see online supplementary appendix 1).42

Neuropsychological assessment

All participants with a diagnosis of PCA had previously (within 6 months) completed a selection of neuropsychological tests of memory, language and visual processing skills (visuoperceptual and visuospatial). Descriptive data relative to normative data sets appropriate for the mean age of the group are presented in table 2. Mean participant scores on the Short Recognition Memory Test (words), fragmented letters (visuoperceptual) and dot counting (visuospatial) tasks were below the 5th percentile, with mean scores on the concrete synonyms test falling within a normal range. About half of participants’ individual scores on the tests of memory (Short Recognition Memory Test for words) and language (concrete synonyms test) fell below the 5th percentile (n=11 and n=9, respectively), whereas almost all participants’ individual scores on the tests of visuoperceptual (fragmented letters) and visuospatial (dot counting) processing skills fell below the 5th percentile (n=19 and n=18, respectively).

Qualitative interviews

Participants described a range of ways in which the diagnosis of PCA and the associated symptoms contributed to stress over time and various strategies they and close others employed in response. The findings comprise three central themes that highlight some of the diagnosis-specific characteristics of PCA: the journey to diagnosis; interacting with the physical environment including managing activities of daily living, navigating the outside world and use of aids and adaptations; and implications for the psychosocial environment such as maintaining independence and the adoption of a one day at a time approach to coping. Within these key themes, there were associated temporal variations due to transitions associated with stage of life and others more representative of living with a neurodegenerative illness. The illness experience was framed by numerous contextual and background factors (eg, existing relationship quality, personality factors (eg, being a gregarious/cautious/easy-going person) and life stage transitions).

The journey to diagnosis

Initial symptoms were often described as incongruous or hard to pinpoint but nevertheless were an indication that something was wrong. Often, struggles with very familiar activities were first noticed:

Everything was hard for about a year, and [I was] beginning to feel there’s something not right here, because I couldn’t work out sort of basic things. (Participant with PCA)

It was not uncommon for individuals to describe a problem that arose in their workplace, for example, reading financial accounts, or difficulties judging distance while driving. These challenges were often attributed to the health of their eyes and presented in stark contrast to the typical short-term memory problems first noticed in cases of tAD.43

With the everyday nature of tasks becoming difficult, this was inherently unsettling and a primary source of stress in and of itself. The majority (n=19) of individuals consulted eye health professionals (eg, optometrists) in the first instance and underwent various inconclusive eye health tests despite the cortical nature of their visual impairment. With hindsight, couples reflected on how the stress had been exacerbated and drawn out because of the lack of knowledge of the illness among the healthcare professionals they were consulting. For some (n=10), this was complicated by concurrent eye health issues, further delaying diagnosis. Experiences with general practitioners (GPs) (n=17) were similarly reported to be frustrating because of a lack of answers or appropriate and timely onwards referrals. For some, even a referral to a neurologist did not guarantee a diagnosis:

That would have saved me a lot of trouble if I’d believed myself. And it took ages, we went through about five or six neurologists… Nothing. It was just dreadful because I kept thinking if I tell them what’s wrong, what’s happening and the symptoms, they’re bound to know… And nobody knew. (Participant with PCA)

A minority of participants (n=3) reported a timely and efficient diagnostic process, and this was mostly attributed to their own efforts towards information seeking but for one male was attributed to the ‘pot-luck’ of his GP’s professional connections. The remaining dyads (n=17) described stress caused by having to persist in their search for a diagnosis for what they considered to be an unacceptably long period of time. This is consistent with existing literature that describes the benefits of and need to prioritise the early diagnosis of dementia for individuals, families and society44 45; however, this study highlights the particular barriers those with PCA face in receiving a timely diagnosis, owing to the rarity of the condition, associated lack of professional awareness and atypical symptom profile that lead them to exploring an eye health route. Consistent with this idea of the importance of having knowledge of the illness, many participants explicitly stated the relief they experienced when the diagnosis was provided (n=11).

Following diagnosis, there was a widely reported lack of accessible information. This has previously also been reported by those adjusting to a diagnosis of tAD46 47 and young-onset dementia specifically,48 and the current findings therefore add to knowledge about the varied types of dementia for which advice and information are needed. The rarity of PCA had the potential to be an ongoing source of stress over time in that those living with the diagnosis repeatedly found themselves better informed on the condition than healthcare professionals they came into contact with, often having to re-explain the syndrome and their symptoms on multiple occasions.

Interacting with the physical environment

The nature of the symptoms (ie, predominantly visual) meant effectively interacting with the physical environment was the predominant issue. This included interactions within and outside the home environment and with activities both functional and ‘fun’.

Every participant described complications with completing self-care tasks, most prominently dressing and cooking. Difficulties with dressing included finding or selecting clothes or shoes, orienting them and using fastenings:

I do struggle a bit sometimes in working out which way round shirts go… If it’s all in a big heap, which it generally is, it’s just a question of I will perhaps turn it round, sort of, two or three times before I work out where the collar is. (Participant with PCA)

Dressing problems were exacerbated by distinguishing clean versus soiled clothes, seeing the closet and bedrooms being a shared space. Dressing assistance was frequently obtained from a family member or by decluttering, organising and simplifying the bedroom environment. In contrast to those with tAD, where problems with dressing may be attributable to problems with sequential task performance and attention,49 most of those with PCA articulately described clear visuopercpetual and visuospatial processing problems underpinning their difficulties. Those with PCA were able to be similarly articulate about their choices and preferences around clothing and remained motivated to initiate dressing activities, which may be in contrast to those with tAD whose dressing may more commonly be disrupted by temporal disorientation and lack of motivation.50

With cooking, typical problems were with locating ingredients/equipment in the kitchen, reading labels, following recipes, using appliances or confidently and safely handling hot materials. For cooking or other household tasks, some individuals attempted to use visually salient strategies such as labelling cupboards or putting a red dot on the start button of an appliance. The effectiveness of these strategies varied and more typically individuals retreated from these activities. As with dressing, when these difficulties are reported in the literature on tAD, they are usually attributable to declining executive function skills.51

Of particular importance to participants were difficulties they had in engaging with a wide range of hobbies and interests including reading, DIY, sports and arts activities. The impact of this seemed heightened by the typically young age of onset that saw all participants either approaching retirement age, having recently retired or reducing their working hours, and therefore allocating and looking forward to increasing time related to leisure activities:

I think we thought we’d be going out to theatre and travelling, and things more, whereas I’m planning in 2015 to make it the year I’m going to go to the matinees, try that. But, travelling has virtually stopped. We were going to take John’s mother to the Christmas markets, thinking, well, with her help, I can probably get John on and off the train, but it… with her breaking her hip, that’s another thing, another holiday went because of that, and so we thought, this is the time, this will be the time in our life that we need to travel, and this is the time in life, for one reason or another, we can’t… Yes, I think we thought this would be the golden years. (Family carer)

The stress at having to retreat from or renegotiate hobbies is consistent with a study that looked at aspects that are important for quality of life but challenged by dementia.52 However, the barriers here were largely due to the specific visuospatial and visuoperceptual deficits, which is in contrast to a study by Giebel et al 53 in which carers described those with tAD as having difficulties with the initiation rather than performance of such activities.

Beyond the home environment, all participants had difficulties navigating the external environment either on foot, by car or on public transport. Particular challenges were wayfinding through crowds, reading signs or maps, general orientation and using stairs/escalators:

I’m usually quite okay here but the minute I step outside the door it all goes mad… Life goes a bit strange, yes… even inside the village can be a bit strange but definitely when I go catching buses and dealing with… interacting with people, in general, no, it’s not great, not great… I don’t know what happens but it goes mad, yes. Not all the time and not every time but I’m much less comfortable and avoid, now, going unless I really need to go into town. (Participant with PCA)

Participants commonly stated they relied on routine responses to the environment and cues within it (eg, using the same underground line or bus) or environmental cues (eg, recognising a street by a particular shop or church), but naturally the external environment is not a stable one. In the case of (for example) a disruption in public transportation or a lorry blocking the view of an environmental cue, problems arose such as the person with PCA getting lost or disoriented. It is important to note that, for most, this was not due to forgetfulness, distractibility or other executive function deficits as might be expected in cases of tAD54 55 but because of problems accurately perceiving visual information about the environment that would help individuals to work out where they were in relation to their target destination.

The difficulties that participants described in interacting with their physical environments are compatible with the sorts of neuropsychological deficits documented in people with PCA in the literature. Deficits like visual crowding, simultanagnosia, spatial navigational problems and apraxia4 9 14 corroborate with the issues people described with locating and manipulating objects, reading and dressing in this study.

As described above, the tendency towards simplification and familiarity meant withdrawing from certain activities. In addition though, almost all participants described ongoing uncertainty and unpredictability associated with the disease profile, commenting that the difficulties were not reliably ever-present (n=16). This uncertainty was once again exacerbated by a reported lack of disease-specific provision and guidance. A minority of participants opted for off-the-shelf adaptations for those with eye health problems, for example, a symbol cane (n=3), or had been in touch with the Royal National Institute for the Blind regarding visual aids (n=5) but most took a self-initiated, largely trial-and-error approach owing to the unusual, unpredictable and continually changing nature of the symptoms. This process was rife with uncertainty regarding if things would work, why they might not and over time, how long they would continue to work:

Camilla can still read, so if it’s just one word it’s okay… but we’ve done different colour coding [on shampoo/conditioner bottles] and this sort of stuff but then she forgets which colour’s which. So it’s not, you know, it seems to be simple… but then there’ll be some other obstacle along the way. (Family carer)

This family carer provides an example of the complexity of progressive cognitive decline where strategies to compensate for the dominant visual symptoms may rely on other cognitive capacities (eg, memory) that may also be affected to an extent. This was in contrast to those without memory impairment at the time of interview (n=9) who frequently relied on the familiarity of their environment to help them find their way or something they needed, often closing their eyes or feeling their way to minimise any confusing visual information.

Allen et al 56 recently described a similar trial-and-error approach to environmental adaptations being employed by community-dwelling people with tAD and their carers, but these were more often triggered by—and designed to ameliorate—difficulties associated with dominant memory problems (eg, using labels as reminders).

Acknowledging the temporal context, the participant above like many others referred to what his wife could ‘still’ do—appearing to demonstrate an anticipation of continuing decline over time. This is something commonly reported throughout the dementia literature in general.57 58 In the case of PCA though, this was coupled with a relative paucity of accessible knowledge or professional guidance as to how the particular course of disease would progress.

Implications within the psychosocial environment

Overall, there were broader psychosocial ramifications arising from these day-to-day difficulties such as maintaining independence and contemplating an uncertain short-term and long-term future.

The symptoms themselves and resulting difficulties in interactions within the physical environment naturally impacted individuals’ ability to perform daily activities independently:

I think that is the worst thing I can do nothing for myself so all the time you’ve got to ask somebody. (Participant with PCA)

Psychosocial strategies used to mediate the stress associated with performance of these daily activities were largely the provision of physical assistance and reallocation of responsibilities within the dyad or family. Over time, this often resulted in increasing feelings of dependence:

One of my big problems is frustration that I can’t do things I want to do. I don’t need to do them but because I’ve always been able to do them it really irks me to have to phone my son up and say, come and put this together for me. (Participant with PCA)

This example illustrates a common phenomenon reported in the interviews—the way this perceived dependency resulted in changing roles and responsibilities within families. The extent to which this caused secondary strain for individuals seemed to be mediated by contextual factors including the age of onset, personality factors and previous relationship quality. Interestingly, the one couple who were recently married (<5 years) described their biggest challenges as being with organising the household responsibilities, though they were able to emotionally support each other well. This was perhaps owing to their less well-established household roles and responsibilities but their relatively recently established and continuing affection for each other.

With age of onset, individuals had often been at the peak of their careers and/or in a critical position within the family system (eg, looking after both children and elderly parents). Such activities and the roles they represented were often defining in terms of individuals’ senses of self and identity:

…he does all the ironing and everything now. And I say, no, that’s my job… I’d always done it, you know. [He] was always working and I had the children and everything, and just did it… And I must admit, I sit here now and I think, I can’t do anything. (Participant with PCA)

The threat that dementia and the associated decline in functioning can pose for a person’s sense of identity and independence is well documented throughout the existing literature, even when functional capacity is disrupted by memory problems58 59 rather than predominant cortical visual deficits as seen here.

Family carers (n=13) also described concerns they had about their competencies in taking on the new role of caregiver and reported the strain of uncertainties in knowing how and when to provide help. Many noted trying to strike a balance between getting things done, preventing their family member’s distress or frustration and encouraging or facilitating their ongoing independence.

Any stress and strain for participants who had a diagnosis of PCA regarding the impact on identity, role and independence were arguably emphasised by their relatively intact long-term memory functions and abilities and inclination to reflect on and compare their previous experiences with their current situations. One way this caused stress was that the person with PCA could reflect on themselves and their declining abilities, and in doing so most (n=15) were concerned that they were becoming a burden to their partner or wider family. However, these relatively intact capacities were also able to contribute to relieving stress for the majority of couples who took an interdependent or ‘teamwork’ (n=16) approach to managing the difficulties, via continued collaboration and joint problem solving. Those dyads who did not adopt such a team-based approach (n=4) appeared not to because of either factors relating to: their previous relationship quality (eg, living fairly independent lives); personality (eg, pride and stubbornness); or the cognitive decline of the person with PCA (eg, poor memory for shared events). The dominant ‘teamwork’ approach or sense of ‘being on the same page’ and navigating the illness experience together was made evident in shared story-telling during the joint interviews and in the corresponding and complimentary accounts given in the individual interviews. However, there were also some instances of discrepancies in the accounts of the person with PCA and their family carer. Sometimes differing recollections of events (eg, a family occasion, first symptom onset) would seem to be easily explained by the person with PCA’s concurrent memory impairment, but in other cases, the discrepancies were harder to unpick. Several dyads offered differing accounts of the person with PCA’s functional ability. In one case, a daughter attested that her mother was trying to not let us, the researchers, know how impaired she was by insisting she was still doing household chores and that the discrepancy could be explained as an exercise in self-presentation. In another case, a man with PCA and his wife disagreed over whether he could safely go for a walk unattended—she considered his wayfinding abilities to be too compromised and he considered her to be too closely monitoring him and disproportionately concerned. Day-to-day difficulties were also attributed to different underlying symptoms, for example, one man with PCA put his problem with navigating the stairwell at a relative’s house down to the area being ‘dim’ and ‘dark’, whereas his wife put this down to him not remembering where he was correctly:

…you still get lost on that landing…So it’s a square area with four closed doors where does he go? And he never knows…like where is it, where on earth is it? So he can’t retain the information…this is like every single time…he is walking down those steps for the first time. (Family carer)

These discrepancies—though not the common pattern in the current study—clearly highlight the importance of acknowledging potential differences in perspectives and the challenges these could pose in the day-to-day understanding of and responses to the symptom profile and its impact.

Despite these occasional discrepancies, many individuals with PCA (n=11) expressed extensive gratitude or feeling ‘lucky’ for their spouse/family carer while also normalising any symptom-specific dependency as one of many ways in which they and their family member worked together to manage life’s challenges:

If you get married, sickness and in health, you have to keep to these things… I’m old-fashioned enough to think… you know, if it were me, he would look after me, I have no doubt…So, you know, what I think, and this is what I say to him, we’re married, we’re two parts of a whole, so in many ways it affects me, because then when you are supposed to be as one, as a whole…then you have to look after the other half of you, and, you know, by keeping one half healthy, helps the other half. (Family carer)

There was a suggestion that a relatively preserved insight and ability to plan in people with PCA promoted a continuation of closeness and collaboration between dyads, potentially contrasting with previous studies involving participants with young-onset but memory-led dementias. Baikie60 reported a loss of joint decision making in marital relationships, while separateness made up part of an overarching theme in a study by O’Shaughnessy et al 61 about the impact of dementia on the marital relationship. Similarly, Wright62 described how a lack of awareness contributed to discrepancies in the accounts of people with dementia and their spouses regarding their experiences of tension within the relationship and also an overall reduction in shared meanings made about the illness experience. However, there are also reports of couples living with memory-led dementias taking a continued teamwork approach46 and sustained reciprocity in consideration of the others’ needs,63 both of which serve as a useful reminder of our need to interpret with caution. An example of this within our own study was one of the male participants who demonstrated concurrent memory problems along with his dominant visual processing deficits, whose wife commented:

Everything’s more flat, yes. And he’s got no sense of time, so whereas before… he would go…oh yes…this is the year that she said was our silver wedding anniversary…now he’s got to rely on the children to say…we’d better do something about it…I found it painful. (Family carer)

Two points seem salient here. First, the dementias progress in different ways for different people, and second, given the variation, states of separateness or connectedness, or teamwork versus independence, do not apply discretely and exclusively to a group of people with one diagnosis and not to another. The participants with PCA interviewed here will likely progress to have more memory challenges over time, potentially posing additional challenges to shared meaning-making, and those in the early stages of more typical dementias are increasingly shown to be able to reflect reliably and accurately on their own experiences and abilities,63 64 something essential for joint problem solving and shared decision making.

Another factor to consider is the sociocultural context within which research questions are framed and studies are carried out. As the value put on quality of life surpasses that of quantity and as cures for dementias remain elusive, attention has shifted to ideas of preserving personhood of those with dementia and more recently still, the couplehood of dyads living with a diagnosis. Acknowledging the person and not just their disease has also meant a shift in focus from seeking to document deficits, loss and failings and attempts to capture the whole breadth of experience as attempted here: the positives, strengths, closeness and resilience.65 66

One day at a time

Beyond diagnosis and in terms of longer term ongoing coping with the diagnosis, uncertainty and a lack of knowledge persisted, this time with regards to what to expect and how the disease would progress:

There’s little point in thinking about the future because, in that sense, one would have to have a model of what the future may hold and therein lies part of the difficulty, that I can’t map that future and make any choices. (Participant with PCA)

This impacted how able many dyads felt to effectively plan for the future and many described taking a conscious decision to not think about it, given that there seemed to be little purpose or pleasure in doing so because of the incurable and progressive nature of the diagnosis:

Every now and again I get down, mainly because if I think too long about what the future holds then… it’s counter-productive…It’s going to happen. There’s nothing you can do about it. You know, it’s like one of those things. It’s nothing… it’s all… you can give a problem a lot of thought if there’s an answer; right, do we do this or do we do that? Right, think about it a long time, perhaps worry about it for a couple of days. Right, let’s do that. With this, there isn’t… What’s Plan B? You haven’t got a Plan B. And that’s this situation. There isn’t a Plan B. (Participant with PCA)

In shifting the focus away from an uncertain future, many described their approach as being centred around ideas of ‘keeping going’ and ‘getting on with things’, with almost all dyads describing efforts to maintain normality as far as possible (n=17):

When she [my wife] got her head around what she had, she said, there’s nothing I can do about it, we’ve just got to get on with it. And we just carried on as normal…When it crops up, I deal with it, but 99% of the time, we just carry on as normal…I mean, obviously you gradually get worse and worse, but, you know. (Family carer)

This family carer’s comment clearly demonstrates the complexity and significance of temporality in describing the simultaneous day-by-day approach that can exist in combination with broader acknowledgement and anticipation of ongoing decline over the long term.

There were several reports of professionals endorsing or echoing this approach of living in the moment:

She [doctor] just looked at him and… put her hands on his legs and said, just live your life…just go on and live your life, that’s all you can do. (Family carer)

This comment also seemed to address the inevitability of the progression of the disease and the absence of a cure, in describing continuing to ’go on' and to ’live your life' as the only options open to participants in facing the diagnosis. Perhaps it also hints at the lack of published guidance and knowledge about progression of the disease and what to expect that may have enabled or assisted longer term care planning and management. This is corroborated by a recent paper that identified the challenges families and practitioners face in finding tailored, disease-specific information and practical advice about PCA  that is evidence based.67

Taking a day-by-day approach was preferred and required due to the ever-changing nature of the symptom profile as the disease progressed. Individuals and families were continually responsive, and the majority described being attuned to the necessary ongoing adjustments and adaptations required by the continual change that is characteristic of the disease profile (n=16):

Yes, as I say, if I let my mind go there [the future], I will probably collapse in a heap, so I find it’s best just to deal with things as they present, and just try and think one step ahead, and not too far, because, as I keep being told, every individual with the disease is different, and they can make no… they’ve got no crystal balls to see into the future, about exactly how it’s going to pan [out] for any… one person. (Family carer)

The sense of needing to balance the maintenance of normality in the face of diagnosis-related changes that require ongoing adjustment is also commonly reported throughout the qualitative literature on dementia.46 61 64 66 A decision to focus on the ‘here and now’ is also widely reported, owing to the uncertainty or discomfort associated with thoughts about the future, in line with what was reported here.46 61 These similarities with existing literature perhaps highlight the progressive nature that is common to all variations of AD and dementias more broadly.