'It’s become a theatre’: relational experiences of family carers and people with Amyotrophic lateral sclerosis (ALS) after cognitive impairment emerges

Ethical approval

The study was conducted in accordance with the Declaration of Helsinki.³¹ All procedures were evaluated and approved by the Regional Committee for Medical Research Ethics Western Norway, REK West (No. 2016/2187). All participants had received oral and written information about the study and signed a voluntary informed consent. Data was handled with confidentiality and stored in a secure research server. Participants are presented with pseudonyms in this manuscript.

Design

We sought to collect thick descriptions of individual experiences to reach an in-depth understanding of the participants’ situation. A qualitative design with a thematical analysis³² underpinning paradigm of occupational science^33,34 was appropriate to accomplish our research aim of exploring how people with ALS and their family carers experience everyday close relations.^35–37

Participants

Eligible participants were already enrolled in the overarching main registered clinical study, led by the last author (TT) of this manuscript, entitled: Cognitive impairment in ALS: screening tools, experiences and prognosis³⁸ between May 22, 2019, and June 01, 2021. This study sample comprises people diagnosed with ALS and their carers who were followed up at an ALS outpatient clinic in Western Norway. Included patients had to be native-Norwegian speakers so that they could understand the instructions and test situation, and to ensure accurate test-scores. Exclusion criteria included any non-ALS comorbidity in which a decline in cognitive function was the main feature and/or in which great difficulties in writing or reading were present. Patients included for interviews had to have a total score of 92 or less on the Norwegian version of the Edinburgh Cognitive and Behavioural ALS screen (ECAS-N)³⁹; this cut-off score indicates cognitive impairment.³⁹ ECAS-N has its purpose for clinical use and is considered appropriate for research. The researchers obtained a copyright license.³⁹

Of a total of 16 eligible people with ALS tested with the ECAS-N, eight scored equal or below 92. Of these, one patient declined to participate, and three were excluded because they had either no close family carers available to interview, or the recruiters (clinicians not involved in the research) evaluated that an interview situation could be demanding and increase their overall burden. According to the Strong-criteria¹² that classify frontotemporal dysfunction in ALS into the subgroups; ALS cognitive impairment (ALSci), ALS behavioural impairment (ALSbi), ALS cognitive- and behavioural impairment (ALScbi), and patients with a combination of ALS and FTD (ALS-FTD), the results of the ECAS-N indicated that one patient met the criteria of ALSci because the patient showed executive dysfunction, including dysfunction in social cognition. One patient met the criteria of ALSbi because of reported apathy in combination with other behavioural change. The data material does not entail enough information to determine if any of the patients met the criteria of FTD. On the basis of our experience, the rich and detailed interview data provided by the included four patients and four carers, we determined that our study sample was sufficient to answer our research question. Two of the patients had difficulty speaking, so they used a communication aid. Characteristics of the included people with ALS and their carers are presented in Table 1.

Data collection

Individual semi-structured, in-depth interviews were conducted from October 2019 to April 2021. All the participants were approached by telephone and chose to be interviewed at home. The participants were interviewed once, face-to-face. Patients and their carers were interviewed at the same time, but in separate rooms. Interviewers were the first (MSO) and last authors (TT). The relationship between the participants and interviewers were solely related to the research. TT had met some of the participants in the ALS clinic, once, prior to the interviews, but after informed consent were signed. MSO met the participants at first when conducting the interviews. Due to government restrictions related to the Covid-19 pandemic, the interviewers wore a facemask when recommended.

All interviews were audio recorded and lasted from 43 to 71 minutes. We followed a study protocol inspired by Creswell.⁴⁰ This comprised procedures for standardized data collection, safeguarding relevant information and transmission of information given to all participants. The protocol also included a lay introduction about the aim of the study, its purposes, and interviews; a pilot-tested semi-structured interview guide (supplement 2) developed by TT and the research group at Haukeland University Hospital (HUH); and a formal expression of gratitude to the participants’ participation and time spent. See supplement 3.

The interview topics mainly focused on three areas: (1) everyday life before and after the ALS diagnosis; (2) everyday situations, which are described as being challenging when suffering from ALS-specific cognitive impairment; and (3) healthcare in early phases following diagnosis. To obtain rich, unprejudiced, and descriptive responses, we used open-ended questions while always nudging the discussion toward the topic in question. The interviews began with one of the following opening questions: “How do you see your own situation?” “Can you describe a typical day?” To encourage more descriptive stories, we used follow-up questions like: “Can you describe a specific situation when … ?”. To encourage participants to relate any other experiences, we asked closing questions like: “Do you have any additional experiences of importance that we haven’t talked about?”

Data analysis

We used thematic analysis (TA) as set out by Braun and Clarke.^32,41 Data were coded and thematized using NVivo12 qualitative data analysis software.⁴² TA uses a six-step analysis structure guide (described in the following paragraphs), permitting great flexibility regarding theoretical and epidemiological positioning, and individual choices for coding.³² During analysis, the researchers attempted to set aside their field-specific (e.g. occupational science) preconceptions and ALS expertise in trying to understand the participants’ experiences. This was important in our TA, as its inductive nature aimed to code the data without trying to fit it into a pre-existing coding frame or the researcher’s analytic preconceptions; it was ‘data-driven’.⁴³

The first analysis step, familiarizing ourselves with the dataset, focused on reaching an overall understanding of the participants’ stories, and started already during data collection. Immediately after each interview, the interviewer wrote global reflection notes to characterize the setting, feelings that emerged, and early general thoughts about what was said. The interviewers consecutively transcribed the audio recording verbatim, eliminating names of people and locations to protect participants’ anonymity. The interviews contain potentially identifiable sensitive data about the patients’ cognitive function and relational issues within the participants family. Because ALS is a relatively rare diagnosis with large variation in disease trajectories, and because the hospital identified in our dataset treats a limited number of patients a year, full de-identification of the transcripts was not possible without removing information necessary to analyse the data.

Transcripts were read at three levels: (1) holistically across all participants, (2) individually for each participant, and (3) in pairs (patient and carer together). Additional reflection notes, keywords, and candidate topics were created. The transcripts were not returned to the participants for comments and/or corrections.

Before moving to step two, two authors (MSO and TT) discussed and agreed on preliminary keywords (e.g., communication) that best characterized the dataset and made decisions that ensured the given theme captured the pattern of importance in relation to our research question. For example, we decided what was important based on interpretations on what was said and how it was said (e.g., expressed feelings), what triggered curiosity, and what was found surprising. This procedure and the rich data generated data-driven themes (inductive analysis) at a latent (interpretive) level.

Step two is generating initial codes. The first author (MSO) started the coding process of step two after clarifications important to moving the analysis forward were agreed on. Examples of initial codes are presented in Table 2.

Codes generated for the entire dataset were discussed and agreed upon by all the authors. In the third step, searching for themes, relevant coded data were sorted into candidate topics. Some codes formed main themes, like Challenging relations. Others, like absent communication, formed sub-themes.

By step four, reviewing the themes, the topics were all processed. All proposed themes appeared in relation to what emerged to be the core theme, Increased distance of close relations.

In step five, defining and naming the themes, the themes were precisely defined, and names finalized. A detailed analysis of each theme’s core content was documented before a theory was connected to the data. To better understand how daily interactions might affect the participants’ close relations, we were inspired by the dramaturgical metaphors Erving Goffman uses to describe face to face interaction in everyday life,⁴⁴ and his perspectives of stigma.⁴⁵ Goffmans’ perspectives in relation to our findings are described in the first section of the discussion, Burden of disrupted roles and interaction. Due to theoretical limitations on experience of grief, we found it relevant to add the dual process model of coping with bereavement (DPM),⁴⁶ and Bury’s theory of a biographical disruption as presented in the introduction.⁴ DPM has its foundation in theory on attachment and coping, and may help to better understand the normal process of grief and to recognise prolonged or complicated grief.⁴⁶ DPM is central in the second section of the discussion in this manuscript, When the future becomes ‘lost,’ a well-lived life provides hope for the present.

In step 6, reporting the findings, analyses were completed by presenting the findings of the study here. The participants have not provided feedback on the findings.

Increased distance of close relations

Participants reported experiencing tension between ‘losing self’ and ‘losing each other.’ They all described unwanted changes in the way everyday roles became distributed, as well as experiencing limited and demanding verbal communication and social interactions. Moreover, they struggled to cope with an existential change about a ‘lost future’ together. Increased relational distance was to both patients and carers caused by suppressing difficult feelings bubbling up from loss and occupational limitations. Having different experiences and approaches to one’s own life situation challenged the foundation of their relationship.

Everyday life together but apart: a demanding role to play

All participants described being increasingly dependent and losing freedom to engage in meaningful occupations. Skewed responsibilities, challenging communication, different levels of power and ability to respond to one another caused an imbalance in the relationship, leading to more and more relational distance. Everyday roles as patients and carers were taken for granted. They seemed to know little about each other’s experiences. The patients reported being more dependent on others, mainly because of their physical limitations. The carers had been taken for granted in their caregiving role, hindering or ‘short-circuiting’ their own priorities. They depended on the patients’ willingness to accept their help to make them feel important and ease the caregiver burden. The patients, however, had difficulties accepting disease progression, affecting their desire to maintain their independence.

Daily interactions in social settings between carers, patients, and others became complicated, because communication was missing, impaired, or challenging. Patients’ communication abilities were affected by impaired motor function, emotional lability, apathy or a lack of initiative or energy to speak. During the interviews, some patients had difficulty retrieving words or reflecting on context, and some tended to communicate ambiguously, using words that did not match expressed feelings. Patients had difficulties following discussions, and their carers had to explain their rationale, repeat themselves, and sometimes talk on behalf of the patient. Communication aids that two of the patients used were cumbersome, and they were uncomfortable with its artificial-sounding voice. Losing their expressive ability was like losing an important part of themselves; they could no longer say what they wanted in the way they wanted. ‘It is very sad, because it [communication aid] doesn’t say what I mean,’ Ester said. She and other patients often felt excluded or misinterpreted. Depending on others to help them communicate was impossible for most to accept. The carers were also sad and frustrated that their stricken family member could not participate in conversations as before. One carer said they were infuriated because the patient related to circumstances differently. Some carers found it difficult to stay calm and be compassionate during arguments, and then felt guilty when they expressed frustration.

All the participants were grieving over lost occupational possibilities and meaningful roles they were expected to fill. Spouses felt they had lost their compatible partner, while patients felt they could not meet the expectations of others or themselves. Some patients experienced severe limitations when doing familiar daily activities, like Hans who had difficulty with the recipe steps when making dinner. These difficulties were, however, partly invisible to his wife, who said: ‘No [his short-term memory loss] doesn’t affect him …. no, like, he does everything he is supposed to do.’ Ester, who communicated with a communication aid, explained how her ability to be a fine grandmother had evaporated:

Ester felt that forming a personal relationship with her grandchildren was impossible. Lost contact with her grandchildren amplified her grief and made her feel she was losing her social identity. Her experience was similar to the carers’ experience of watching their loved ones ‘disappear,’ and in some cases, ‘turn into someone unrecognizable.’ ‘It’s just like a flower that’s withering,’ Lise, daughter-in-law, said. The carers, however, also felt that they sometimes had become invisible. Everyday life was organized around the patient’s needs, causing some carers to feel little emotional support. Berit, a spouse, said in despair: ‘I had to ask for a hug’ when discussing how she was sick and needed support. This spouse also explained how the patient lacked initiative and interest, in contrast to before his cognitive impairment emerged. If she did not organize activities, he just lied on the sofa or walked around doing nothing. The patient, Tom, however, denied having any difficulties, explaining occupational limitations with natural causations (e.g. retirement). He said: ‘You know that thing with the disease […], it doesn’t affect my daily life at all. I don’t worry … […] so far, so good.’ He trivialized symptoms and expressed unrealistic thoughts about the future.

Tom and other patients deliberately leaned on their carers but lacked the capacity to acknowledge those efforts. Most carers felt ‘tied’ to the caregiving role, putting their own life on hold. Stress arose from worrying, having to be vigilant, and disregarding their own needs. ‘I feel like an executioner sometimes,’ Irene, spouse, said, referring to her guilt when trying to prioritize her own needs and desires. Lise, daughter-in-law felt bad for her mother-in-law, who ‘is in a prison,’ but also felt being ‘chain [ed]’ to her. She described spending hours helping her with chores and personal activities, expending extra time and effort to maintain a positive atmosphere, all to no avail. However, one spouse, Niklas, felt that the caring role was normal, not burdensome: ‘So, we try to make the best possible out of it [..], and for me, I don't feel that it's any bother to support her … for her to have the best situation possible.’ Niklas had open and good social support during the days, which seemed to explain his positive experience.

Whether the patients acknowledged disease issues or not, most did not want to publicize the diagnosis. They felt ashamed and pretended they were not really affected much. Lise, daughter-in-law explained how they tried to maintain a façade of civility in everyday life: ‘It has become a theatre, like wearing a mask. That hurts. It’s the worst!’ When patients avoided exposing their disabilities, their carers’ efforts went unrecognized, leaving them feel alone with a heavy burden. Most patients, however, were afraid of becoming a burden to others. They resisted ‘whining,’ and often rejected unprompted help from carers to maintain their independence and sense of self-sufficiency and autonomy. Yet, most patients expected that their carers were available to help them when needed. Some carers felt they had to guard the patients, treating them like children. Lise, daughter-in-law said ‘because we are standing there with widespread hands (pulls her arms out to demonstrate) just like an octopus. “What do you want me to do? [we carers say]” “No. Don't want any help [patient says].”’. Lise found it hard to play a supportive role. Like other carers, she wished the patient would accept the disease and graciously accept help. For most participants, daily life became a tug-of-war between trying to hold on to oneself and doing what they thought was best for the other.

Coping with a lost future: living in the ‘normal’ present and searching for hope through a well-lived life

All participants emphasized their previous active and social life. Adjusting to the new existence with ALS combined with a lost future was difficult for both patients and their carers. Despite somewhat different approaches, all participants tended to avoid the unpleasant and strived to think positively. They identified with lived lives and held onto what they could still do. Most focused on familiar practicalities of daily living and concentrated on one thing, one day at a time. They found meaning and hope in the past.

Most participants found the future frightening and avoided talking about it. ‘I know how it goes, and I know how it looks, […] but I don’t dare to think ahead,’ Berit, spouse, said. Like the patients, she and most carers did not want to ‘meet trouble halfway.’ Tom, for example, said he wanted to live ‘blissfully ignorant.’ Future aspects seemed unachievable. He tended to relate only to what was concrete and manageable at the moment, like taking his pills and dealing with requests like ‘do as [he] is told.’ By avoiding the future, as it were, the participants could live as normally as possible. Irene, spouse, said: ‘I try to keep daily life as it used to be.’ Similarly, several participants talked about how everyday life is mostly the same. However, to Lise, daughter-in-law this avoidance of the future and emotional conversations was as a ‘double burden.’ She expressed hopelessness: ‘It’s just like I’m in a tunnel … There is not much light over there,’ and highlighted an unrealized longing to talk about death and to be able to share the negative feelings of grief and fear. Her mother-in-law, Ingrid, however, resisted becoming a burden by focusing on positivity.

In conversations the participants talked about concrete, familiar things in daily life: what one could see, touch, and sense. Despite experiencing severe loss and limitations, most participants recalled remaining shared activities. They could sit on the porch together, enjoy the view, and watch the grandchildren play. They all clung to the hope of what was normal. Patients found meaning in what they could still do for others. Some tried to maintain their usual activities, for example, by going from reading articles as they did before to reading poetry now. However, some activities could not be replaced. Hans said: ‘To walk with a four-wheeler on the pavement … like 50 meters … That’s not a true experience for me.’ Experiencing nature had been so important to him that he now found it impossible to enjoy and perceived it only as a ‘defeat’ when trying to adjust. He replaced these adventures with daydreaming, as shown in the following excerpt:

Hans’ reimagining his adventures gave him joy; remembering how it used to be allowed him to joyfully indulge in nostalgia. Nature experiences and the companionship he felt became real in his imagination. It seems like the past created hope for the present and contributed to patients’ ability to hold on to self.

All participants said that their history became purposeful for creating meaning for the present. The patients emphasized what they had done and talked about what they had accomplished in life with pride: ‘I have been to all the mountains in the area’ (Tom). ‘I worked until I was 75’ (Ingrid). They perked up when talking about hobbies they had engaged in and how they had been a helpful resource to others. Patients reported the need to maintain self-identity by telling their story. Spouses held onto who they were as a couple by remembering shared experiences. All carers emphasized who the patients were prior to the ALS diagnosis, and by helping them engage in activities valuable to them, they solidified their identity. They also safeguarded the patient's identity throughout the course of the disease by telling their story to others and by communicating on their behalf so that they felt included in social settings. All participants stuck to the everyday hopes of ‘normal’ days, and by cherishing memories, they held onto their identity.

Burden of disrupted roles and interaction

The participants’ stories in the present study are consistent with the characterization of living with ALS as a ‘biographical disruption’.⁴ Experiences like lost ability to perform valued occupations strongly associated with self-worth and identity, might explain why our patients tended to avoid conversations about their disease or felt uneasy when meeting others. The participants’ everyday roles that became disrupted because of the disease and the ensuing loss of autonomy degraded the interactions between them. Tense feelings tended to cause patients and carers to avoid talking to each other. This disengagement in conversations was partly motivated by the idea that talking about negativities would cause more burden to their significant other. Patients also may have been incapable of handling the complexities of the disease, which manifested as silence. The patients were heavily burdened and felt that they were losing their sense of self, especially after becoming anarthric. The latter also seemed to be why they tended to avoid or complicate conversations and one of the main reasons behind participants’ lost roles and social possibilities. This is consistent with another study that showed patients with bulbar ALS losing power and independence.¹ The narratives in our study also suggest that losing the ability to participate in conversations due to language (cognitive) difficulties or executive impairment, amplified the experience of lost control in daily interaction, and lead to a feeling of outsider-ness – but possibly without the ability to recognize, admit or talk about this feeling.

Spoken language is central for maintaining communication and a thriving relationship.⁴⁸ When people with neurological diseases lose the ability to speak, as in ALS, family-patient relationship is challenged. Qualitative studies of carers’ experiences with people with ALS who develop language or speech difficulties, have not been done, to our knowledge. Pozzebon et al.²⁵ found that when spouses of people with primary progressive aphasia, became disconnected in their spousal relationship, and they had to readjust their sense of self. Similarly, carers in the present study reported that being unable to speak with the patient effectively added a second burden on top of the disease burden. It is unclear whether lack of communication was also a ‘double’ burden to the patients. Maintaining relationships is critical for patients’ well-being.¹ However, our results do not allow us to determine whether the extent or content of communication between patients and carers defines how patients feel about the quality of their relationship. Patients may feel excluded, because their communicative abilities were reduced compared to others, effectively leading to little social participation. The patients’ increased burden due to anarthria demonstrates how important language and speech are to forming and maintaining identity. Alienation and loss of identity may explain why the patients hesitated using communication aids with an artificial-sounding voice. According to Goffman,⁴⁵ functional disease-related loss forces a person to reorganize their biographical past. As with the patients in our study, if patients do not recognize themselves anymore, they may express ‘disapproval of self.’ Goffman⁴⁵ argues that impaired speech is a ‘stigma symbol,’ one highly visible to others. Every time patients try to speak, they are reminded of their stigma. Patients in this study had difficulties speaking, in addition to having impaired non-verbal communication; these may have intensified this stigmatization and contributed to a loss of social identity.⁴⁵ Additionally, when the patients have cognitive impairments that reduce their ability to effectively learn and use technical aids, this complicates their possibilities to participate in conversations.

In relationships, differences in opinions may arise from misunderstandings. A divergence in quality-of-life perspectives between people with ALS and their carers sometimes occurs because of misunderstanding each other’s experiences.⁴⁹ These findings prompt clinicians to more prominently emphasize better communication between patients and carers. Our study, however, indicates that mutual dialogue exchanges about issues affecting their well-being might be difficult when patients suffer from cognitive and/or language impairments. Patients’ impaired ability to reflect on, to recognize others’ state of mind, or to comprehend complexities may make conversations more difficult. In ALS-related cognitive impairment, we observed that patients lost the ability to apply formerly effective strategies that are essential for interacting with others. They also lost the ability to interpret covert signals from the carers when interacting. According to Goffman,⁴⁴ this loss will lead to disrupted ‘team-play’. Impaired cognitive abilities may affect patients' capability to act loyally, disciplined, and with care, which Goffman⁴⁴ emphasizes as essential qualities for team members to feel united.⁴⁴

The increased relational distance between participants in the study increased the burden to both patients, who felt excluded when losing control, and to their carers, who had more responsibilities and felt lonely. Our results correspond with previous research, suggesting that patients’ dependence on family could lead to interpersonal conflicts¹⁹ and that patients rated themselves more of a burden than did their carers.⁴⁹ Findings in the present study is supported by Johnson et al.⁴⁹ who suggest that misunderstandings and miscommunication between patients and their carers can trigger potential stressors that may influence one’s well-being. Disruption in personal roles and relational changes was also reported in a recent study of people with ALS,¹ but in contrast to our findings, some patients experienced improved relationships with their carers. In contrast to a study in which identified caregiving between people with ALS and their family as bi-directional —leading to the patient also experiencing carer burden¹⁹—we observed that one-way support was overwhelmingly from carers toward patients. Possible differences in patients’ cognitive impairments in the two studies may underlie this difference.

The effects of carers’ worry about their own health that we observed are in line with what has been reported previously.¹⁸ However, when patient and carer have a strong and close relationship, we noted that the burden and disruption overall was eased. The importance of maintaining close relationships when coping with ALS have also been described by others,⁵⁰ and this point is well established in applied and social psychology.^51,52 Also, the quality of the carer-patient relationship appears to have a direct impact on the carers’ grief intensity after the patient dies, suggesting that difficult relationships might increase the risk of complicated bereavement.⁵³ By contrast, good quality relationships can ease the bereavement.¹⁶ Our findings suggest that healthcare teams should focus on helping patients and their carers maintain a close relationship as the disease progresses. Stroebe et al.¹⁶ argue that former research on attachment^54–56 shows that good relational quality between a dying patient and a bereaved importantly influences the outcome for the bereaved. Since good relations, high quality of life, effective coping, and resilience in severe illness²⁰ are important, healthcare professionals need to provide helpful preventive and supportive procedures to address relational issues at an early stage. How to effectively handle relational challenges in ALS is unclear. Given the limited support patients gave to carers in this study, and due to the importance of emotional support for carers when coping with grief,⁵⁷ clinicians could encourage carers of people with ALS to seek social support outside of the patient-carer dyad and to prioritize important social relations. Our findings suggest that some carers will need professional support. Their stories about how they struggled because they de-prioritized themselves in favour of the patients’ needs demonstrate that carers could benefit from occupational therapy intervention involving strategies to increase occupational balance and prioritize occupations with high personal value. Moreover, tailored information, based on cognitive test results, may help carers to better understand subtle cognitive impairment and behavioural changes of patients, which may ease caregiver burden. Carers’ increased understanding might have a positive influence on patients, leading to fewer relational conflicts. Similarly, appropriately tailored information for patients may help them understand and participate in interactions.

When the future becomes ‘lost,’ a well-lived life provides hope for the present

Virtually all participants avoided mentioning anything about future aspects of life, especially the patients. This denial and avoidance is in line with research showing that cognitive impairment amplifies this tendency.¹ Patients’ avoidance of the abstract future might be due to faulty reasoning resulting from their cognitive impairment, which is consistent with the cognitive profile of ALS.^11,12 Alternatively, it might represent a survival strategy, a way to avoid the unpleasant.

Focusing on positive aspects of life and recalling good memories seemed to help participants regain meaning and instil renewed hope. Ozanne and Graneheim⁵⁸ found that ALS caused patients and their spouses ‘lose their foothold.’ As with our participants, they found that fear of the unknown caused patients and spouses to struggle with comprehending the diagnosis.⁵⁸ Shifting focus to familiar things, like positive memories and still accessible and meaningful activities, seemed to ease this feeling. A tendency to find meaning and resilience by reflecting on the past has also been described in patients with primary progressive aphasia,²⁵ a neurodegenerative disease similar to ALS in certain respects.^59,60

Most patients and carers in the present study had somewhat opposing perspectives of their life situation, grieving and coping differently. While some appeared totally unaware of their loss, only focusing on positive aspects and daily practicalities, others tended to oscillate between hurting and orientating towards aspects that ‘normalized’ the days. In the dual-process model of Stroebe and Schut,⁴⁶ this oscillation is the ‘normal’ way of coping and most likely a healthier way of dealing with bereavement. Participants’ experiences of anticipatory grief and grief over a ‘lost’ self and lost fellowship can be understood in terms of theories of bereavement. Bereavement is associated with a higher risk of physical and psychological health issues.¹⁶ The dual-process model of bereavement may relate to adaptive versus maladaptive coping.⁴⁶ However, there appears to be little evidence describing ‘normal’ coping in grieving people with a fatal disease and cognitive impairment, as in ALS. When a person who has cognitive impairments lack the capacity to process abstract and complex information, their singular focus on pleasant things may represent a necessary coping strategy. Nonetheless, how one person in a relationship copes will influence the other, and when they have different coping styles, relationships can become challenging.⁴⁶ The hypothesis that different coping styles intensify relational challenges between patients and their carers⁴⁶ strengthens our argument that healthcare professionals have a responsibility to provide early support to patients and their carers, if necessary providing separate and individual support. Healthcare professionals need to identify who might be at risk of a complicated bereavement arising from prolonged intense grieving. Strada¹⁵ stresses the importance to recognize early on both patients’ and carers’ grief and psychosocial issues to ease the burden throughout the disease trajectory, which is a relevant application consistent with our observations.

Our findings indicate that patients and their carers may have separate needs when it comes to follow-up help during disease progression. However, all participants in the present study revealed a strong sense of occupational identity.⁶¹ Patients and carers shared the approach of finding resilience by recalling good memories while aiming to ‘normalize’ their days. They reported how everyday things can have great impact, creating hope for the present. With a transactional understanding of occupation,^62–64 participants’ experiences and behaviours occur in the dynamic context of their past and present, all intertwined in a holistic relationship with objective conditions and subjective experiences. Their history impacts the creation of meaning in the present, and patients’ experiences and behaviour in daily life affect the carers’ and vice versa. Thus, professionals should encourage patients and carers to reflect on their history together. Occupational therapists may highlight their occupational identity, and like Hammel⁵ argues, focus on meaningful more willingly than purposeful occupations when aiming to help patients and carers to regain a sense of meaning in everyday life.

Methodological considerations

The reflexive evaluation approach of Stige et al.⁶⁵ emphasize researchers engagement, processing, interpretation, critique related to materials and respective sociocultural and academic discipline, the usefulness and relevance of the research and ethical considerations. The approach encouraged us to provide a reflexive dialogue throughout the research and influences our methodological evaluation as followed. The researchers who carried out the study are occupational therapists with ALS-specific clinical experience. Hence, the course and presentation of this study may reflect the researchers’ engagement with the ALS clinic. First author (MSO) was inexperienced as researcher and conducted the study as part of a master’s degree. The second author (US) is professor with PhD and multiple qualitative publications, and last author (TT) has PhD and extended experience as a researcher within the field of ALS. Throughout the study, discussions within the research group helped contextualize and minimize the undue influence of preconceptions and clarify the research focus. Using TA to analyse the content of the interviews provided a systematic and rigorous, yet flexible thematic analysis.^32,41 The complexity of ALS challenged the analysis. Interpretations of possible motor- or cognitive causations in relation to the data, may not always be distinguished. Misinterpretations may have occurred because of patients’ impaired cognitive ability to reflect on their situation, patients’ communication difficulties, and/or potential emotional stress related to a worsening crisis. Participants may have been reserved about revealing all relevant information because of familial fealty or fear of losing or damaging their necessary relationship with the hospital involved in the research. On the other hand, a strength of our study is that all patient-participants had indications of cognitive impairment, and some were with severe speech impediments, rarely addressed in ALS research. Our study presented perspectives from both patients and carers, an underrepresented design that provided new insights. Finally, participants provided rich, descriptive narratives, permitting sufficient data to identify themes.