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

Ethical approval

The study was conducted in accordance with the Declaration of Helsinki.³³ 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 was appropriate to accomplish our research aim of exploring how persons with ALS and their family carers experience everyday close relations.^34–36 The reflexive evaluation approach of Stige et al.³⁷ emphasizes 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 process, influencing our methodological evaluation as described in the section of methodological considerations.³⁸

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 prognosis³⁹ between May 22, 2019, and June 01, 2021. This study sample comprises persons diagnosed with ALS and their family carers who were attending an ALS outpatient clinic in Western Norway. Included persons with ALS 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. Persons with ALS included for interviews acquired a total score of 92 or less on the Norwegian version of the Edinburgh Cognitive and Behavioural ALS screen (ECAS-N)⁴⁰; this cut-off score indicates cognitive impairment.⁴⁰ ECAS-N has its purpose for clinical use and is considered appropriate for research. The researchers obtained a copyright license.⁴⁰

Of a total of 16 eligible persons with ALS tested with the ECAS-N within the timeframe of this project, eight scored equal or below 92. Of these, one person with ALS 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 persons with a combination of ALS and FTD (ALS-FTD), the results of the ECAS-N indicated that one person with ALS met the criteria of ALSci because the person showed executive dysfunction, including dysfunction in social cognition. One person with ALS 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 persons with ALS met the criteria of FTD. Two of the persons with ALS relied on assistive communication aids because they had difficulty speaking. Characteristics of the included persons with ALS and their family 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. Persons with ALS and their family carers were interviewed at the same time, 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.⁴¹ 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), 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. Experiences regarding the healthcare was planned to be presented in another article. 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 Reflexive thematic analysis (TA) as set out by Braun and Clarke.^42–44 Data were coded and thematized using NVivo12 qualitative data analysis software.⁴⁵ Reflexive 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.⁴² 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 analysis, as we aimed to code the data without trying to fit it into a pre-existing coding frame or the researcher’s analytic preconceptions. We aimed to understand and theorize meaning in relation to the participants’ sociocultural environment and conditions. This choice was essential to theme generation and theorizing.

The first analysis step, familiarizing ourselves with the dataset, started already during data collection, considering to reach an overall understanding of the participants’ stories. Immediately after each interview, the interviewers 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 persons with ALS’ 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 persons with ALS 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 (persons with ALS and family 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, what was said and how it was said (e.g., expressed feelings), what triggered curiosity, and what was found surprising. This procedure generated data-driven themes 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,⁴⁶ which was partly identical to some of the metaphors used by the participants.⁴⁷ Goffmans’ perspectives contribute to a figurative understanding of how illness may influence the daily interaction between the persons with ALS and their family carers by describing how they perform various roles in relation to one another, family members and friends, and the public health care system. Performances can highlight or obscure various aspects, and the manner in which a role is performed is crucial for how the person perceived themselves and others.⁴⁶ The primary focus in this study is on the interaction that takes place between persons with ALS and their family carers. The dramaturgical metaphors provide a conceptual framework with rhetorical purpose. It is not a pure analogy.⁴⁶ Due to theoretical limitations on psychological and existential processes, like experience of grief, we found it relevant to add the dual process model of coping with bereavement (DPM),⁴⁸ 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.⁴⁸ DPM is central in the second section of the discussion in this manuscript, When the future becomes lost.

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 a daily life with limited and demanding verbal communication and social interactions due to ALS. All participants described unwanted changes in the way everyday roles became distributed. Moreover, they struggled to cope with an existential change about a ‘lost future’ together. Both persons with ALS and family carers suppressed difficult feelings bubbling up from loss and occupational limitations, leading to increased relational distance. Having different experiences and approaches to one’s own life situation seemed to challenge the foundation of their relationship. 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.

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, and different levels of power were experiences presented by all participants. These changes appeared to cause an imbalance in the relationship between persons with ALS and family carers, leading to more and more relational distance. Everyday roles as care receiver and care provider seemed taken for granted. The person with ALS reported that they were more dependent on others, mainly because of their physical limitations. All of them said they did not need much help from the health services, because their family carer was there to help. The family carers emphasized sayings about how they had been taken for granted in their caregiving role, hindering or ‘short-circuiting’ their own priorities. They depended on willingness from the person with ALS to accept their help to ease the caregiver burden. The person with ALS, however, often resisted help offered not to become a burden, to maintain their independence and/or because they had difficulties accepting or relating to their evolving disabilities. When comparing the participants’ narratives in pairs of persons with ALS and their family carers, they revealed that their knowledge about each other’s experiences was limited even though they were strongly tied to each other in daily life, like in following examples:

Lise and Ingrid demonstrate how they both, like other participants struggle to manage their roles in everyday life and to meet each other’s needs; the family carer did not come in position to help in the way that they wanted. The person with ALS acted as if they were not ill, or they did everything in their power not to become a burden without acknowledging that this strategy could make the situation more demanding to the family carer.

Keeping the façade of civility

Whether the persons with ALS acknowledged disease issues or not, most did not want to publicize the diagnosis. They seemed uncomfortable with disposing their evolving disabilities and pretended they were not really affected much. ‘I think he makes it a little better than it really is. […] It is almost like I feel that he feels a little ashamed to be sick. […]– “the neighbours must not see that I have gotten so ill” (he says) ‘Right? Because it is, it's embarrassing’, Irene, wife, said. One family carer explained how her husband in some situations did not even recognize his own disabilities; ‘So, when we enter the tram, then people bounce up to make space for him to sit. But he does not understand why they do that’ (Berit, spouse). She also explained that her husband did not want to tell the neighbours that he had ALS. She used much effort explaining to him why they should tell the close neighbours about the disease. Lise, daughter-in-law had similar experiences with her mother-in-law who did not want to talk about the disease and resisted using a walking aid or a wheelchair despite severe reduced balance. Lise 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 the persons with ALS avoided exposing their disabilities, their family carers’ efforts went unrecognized, leaving them alone with a heavy burden. However, although most of the family carers expressed high burden related to their effort with making daily life as best as possible to their person with ALS, many family carers did not actively involve others to ease their strain. ‘But for now, it's manageable. ‘Right? So, I get him dressed in the morning, and that's fine for now … And I manage to feed him dinner and things like that. And I don't want to involve others before... But I can't travel anywhere’, Berit said, expressing her ambivalence with wanting to manage the situation without homecare. Berit and others family carers resisted help from health care services before it was absolutely necessary. Some family carers rejected external help in loyalty to the person with ALS who did not want help from anybody else. Some expressed that they would not burden others. Others said they could not let go of the responsibility, because they did not trust that the person with ALS would get the best possible care if they were not there to make sure of it. They emphasized the importance of making sure the person with ALS was presented in a good matter when meeting others, with proper clothes and nice looking hear. Their stories demonstrated that it could be difficult for outsiders to fully understand the challenges they were experiencing in daily life, and to come in a position to help.

Demanding daily interaction and communication

Participants said that daily interactions became complicated, because communication was impaired, or challenging. The interviews revealed that persons with ALS’ ability to communicate were affected by impaired motor function, emotional lability, apathy or a lack of initiative or energy to speak. During the interviews, some persons with ALS had difficulty retrieving words or reflecting on context so that the interviewer had to formulate more concrete questions. One person with ALS sometimes gave answers that referred to something else than what the interviewer had asked, demonstrating how he easily misunderstood what was said. Some family carers said that persons with ALS had difficulties following discussions, and the family carers had to explain their rationale, repeat themselves, and sometimes talk on behalf of the person with ALS. Most persons with ALS expressed that it was difficult to accept being dependent on others to communicate. Some of them said that they were easily misinterpreted. Communication aids that two of the persons with ALS used were cumbersome and time consuming to use. They both said they were uncomfortable with its artificial-sounding voice. Losing their expressive ability seemed to be 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 the other person with ALS expressed being inadequate to participate socially in a satisfactory manner. Ester also explained how her ability to be a fine grandmother had evaporated:

Ester said she felt that forming a personal relationship with her grandchildren was impossible. Lost interaction with her grandchildren seemed to amplify her grief and a sense of losing her social identity. Her experience was similar to the family 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 when explaining how Ingrid used to be a resourceful and engaged woman, but now she did not say much. The family carers also said that they were sad and frustrated that their stricken family member could not participate or engage in conversations as before. ‘He uses the kind of sayings that goes on repeat’, Berit said. This spouse also said she sometimes was infuriated because the person with ALS related to circumstances differently. Some family carers found it difficult to stay calm and be compassionate during arguments, and then felt guilty when they expressed frustration. Irene talked about how her husband could not discuss or handle disagreements like before, so she had to find other strategies to handle her frustration than to talk to him, like in the following excerpt:

Taken for granted changes in everyday roles

All the participants were grieving over lost occupational possibilities and meaningful roles they were expected to fill. Berit, for example, talked about spoiled plans to be proper grandparents together. Their capacity to watch the children was reduced because she had to take all the responsibility. ‘Grandpa, you are not supposed to sleep when you are babysitting’, Berit said, imitating one of their grandchildren. Persons with ALS also expressed that they could not meet the expectations of others or themselves. They talked about how they no longer could help their children with practical chores, like before. They described how they 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.’ (Irene). Even though Irene said that cognitive impairments did not impact on her husband’s ability to perform in daily activities, she and all family carers described that the person with ALS had changed behaviour or function that led to skewed responsibilities in daily life.

Spouses said they had lost their compatible partner, and most family carers said they felt ‘tied’ to the caregiving role, putting their own life on hold. Lise, daughter-in-law said she 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 mother-in-law with chores and personal activities, expending extra time and effort to maintain a positive atmosphere, all to no avail. One spouse, however, said he 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). Niklas had open and good social support during the days, which seemed to explain his positive experience. He also prioritized going for a walk alone in nature every day. In contrary, other family carers expressed that 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, like gardening or meeting friends. Everyday life was organized around what the persons with ALS needed. Family carers expressed that their role as a caregiver was taken for granted, and that they sometimes became invisible. ‘But when it’s about me, then, I am just a person in the house. That do everything’, Berit, wife, said. She also said in despair: ‘I had to ask for a hug’ when discussing how she was sick and needed support from her husband. This spouse also explained how her husband 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. Her husband, Tom and other persons with ALS deliberately leaned on their family carers, expecting that they were available to help them when needed, but often lacked the capacity to acknowledge those efforts. Yet, most persons with ALS often rejected unprompted help from family carers to maintain their independence. 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 (family carers say]” “No. Don't want any help [person with ALS says].”’. Lise said she found it hard to play a supportive role. Like other family carers, she wished the person with ALS would accept the disease and graciously accept help.

Coping with a lost future

Most persons with ALS and family 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. However, virtually all participants avoided mentioning anything about future aspects of life, especially the persons with ALS. Adjusting to the new existence with ALS and a lost future was described as difficult for both persons with ALS and their family carers. Despite somewhat different approaches, all participants tended to avoid conversations about the unpleasant and strived to think positively. Everybody emphasized their previous active and social life. Their narratives demonstrated that they identified with lived lives. They talked about what they could still do and what they had done in the past. Most participants focused on familiar practicalities of daily living. They concentrated on one thing and one day at a time. Participants seemed to find meaning in the normality and a sense of maintaining self-identity, by looking to the past.

Striving to live in the ‘normal’ present

Most participants emphasized the everyday hopes of ‘normal’ days. 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. In conversations the participants talked about concrete, familiar things in daily life: what one could see, touch, and sense. Hans described how a normal evening could look like to him and his wife:

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. ‘I don't notice any difference. I do everything as I did before … I get tired faster, but then I must sit down and knit’, Ester said when describing her daily life after the diagnosis. Her husband also emphasized that their daily life looked almost the same; ‘If you don’t look at the parts with the voice and the eating, then much of the days work as before’ (Niklas). Ingrid resisted becoming a burden by focusing on positivity; ‘Nothing good comes from being negative. Then life becomes miserable both for me and those around me’ (Ingrid). However, her daughter-in-law, Lise, described this avoidance of the future and emotional conversations 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. Contrary to Lise, most participants expressed that they 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 all the persons with ALS, she and most family carers did not want to ‘meet trouble halfway.’ Tom, for example, said he wanted to live ‘blissfully ignorant.’ Future aspects seemed unachievable. He 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.’ In the interview, he trivialized symptoms and expressed unrealistic thoughts about the future. 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.’ Other participants said that by avoiding the future, as it were, they could try to live as normally as possible.

Holdin on to self

All participants said that their history became purposeful in creating meaning for the present. The persons with ALS 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. Persons with ALS reported the need to maintain self-identity by telling their story. Spouses focused on who they were as a couple by remembering shared experiences. All family carers emphasized who the persons with ALS were prior to the ALS diagnosis, and by helping them engage in activities valuable to them, they solidified their identity. They also safeguarded the person with ALS'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. Niklas explained how he did his best to involve his wife in conversations, so that she could still participate and maintain her social personality;

Niklas and the other family carers facilitated activities for the person with ALS that they knew they valued; ‘he loves quizzes’, Berit said, and explained how she organized the quiz so that her husband, Tom, could ask the questions rather than answering them. Despite significant loss of function, some persons with ALS also tried to maintain their usual activities, for example, by going from reading articles as they did before to reading poetry. However, some activities could not be facilitated or 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 going for a walk 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 meaning to the present and contributed to persons with ALS’ ability to hold on to self. Like Hans, all participants demonstrated that by cherishing memories, they could try to hold 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 persons with ALS tended to avoid conversations about their disease or felt uneasy when meeting others. Similarly, we believe that one reason why family carers struggled to comprehend the situation living with the person with ALS was because they sacrificed their own valuable interests in favour of caring for the stricken. Their experience of biographical disruption might also explain why they struggled to communicate with compassion when the person with ALS expressed themselves or behaved differently than they used to before ALS. Disruption in personal roles and relational changes was also reported in a recent study of persons with ALS,¹ but in contrast to our findings, some persons with ALS experienced improved relationships with their family carers. In contrast to a study that identified caregiving between persons with ALS and their family as bi-directional —leading to the person with ALS also experiencing carer burden¹⁹ —we observed that one-way support was overwhelmingly from family carers toward the persons with ALS. Possible differences in person with ALS’ cognitive impairments in the two studies may underlie this difference.

Our results correspond with previous research, suggesting that persons with ALS’ changed behaviour and dependence on family could lead to interpersonal conflicts.¹⁹ Findings in the present study is also supported by Johnson et al.⁵⁰ who suggest that misunderstandings and miscommunication between persons with ALS and their family carers can trigger potential stressors that may influence one’s well-being. In relationships, differences in opinions may arise from misunderstandings. A divergence in quality-of-life perspectives between persons with ALS and their family carers sometimes occurs because of misunderstanding each other’s experiences.⁵⁰ These findings prompt clinicians to more prominently emphasize better communication between persons with ALS and family carers. Our study, however, indicates that mutual dialogue exchanges about issues affecting their well-being might be difficult when the person with ALS suffers from cognitive impairments. Persons with ALS’ impaired ability to reflect on, to recognize others’ state of mind, or to comprehend complexities may make conversations more difficult. Aligned with our results, impaired cognitive abilities in certain domains may affect the capability that persons with ALS have to act loyally, disciplined, and with care, which Goffman⁴⁶ emphasizes as essential qualities for team members to feel united.⁴⁶ In ALS-related cognitive impairment, we observed that persons with ALS lost this ability to apply strategies that are essential for interacting with and to understand i.e. body language from family carers and others. When, due to cognitive impairments, like decreased executive functions, the person with ALS may lack the prerequisites to play along with their partner in interaction. Social cognition, for instance, involves multiple cognitive processes that allows a person to recognize how others may feel, and to understand their intentions in a situation.^51,52 Hence a person who has impaired social cognition may have difficulties engaging in discussions and participate in decision making. Social cognition is also considered essential in the ability to understand oneself in relation to others, which may explain why some of our persons with ALS had difficulties with recognizing, admit or talk about their losses.^51–53 This decreased ability persons with ALS may have to interpret own feelings and behaviour in relation to others is coincide with the narratives that some of the family carers in our study described.

Also, spoken language is central for maintaining communication and a thriving relationship.⁵⁴ When people with neurological diseases lose the ability to speak, as in ALS, family-patient relationship is challenged. Qualitative studies of family carers’ experiences with persons with ALS who develop language or speech difficulties, are to our knowledge limited. Pozzebon et al.²⁵ found that when spouses of people with primary progressive aphasia became disconnected in their spousal relationship, they had to readjust their sense of self. These findings support our findings that when family carers are unable to speak or communicate with the person with ALS, it may effectively ad a second burden on top of the disease burden. It is unclear whether lack of communication was also a ‘double’ burden to the persons with ALS. Maintaining close relationships is described as critical for persons with ALS’ well-being,¹ and this point is well established in applied and social psychology.^55,56 However, our results do not allow us to determine whether the extent or content of communication between persons with ALS and family carers defines how persons with ALS feel about the quality of their relationship.

Nonetheless, communication skills, whether due to cognitive qualities or the motor ability to speak, is essential to participate in decision making and own health care planning.^57,58 Coinciding with our findings that persons with ALS experienced lost ability to express themselves like before and were easily misinterpreted in conversations, Paynter et al.⁵⁷ found that communication impairment in ALS caused limitations in persons with ALS’s ability to communicate needs and preferences with healthcare professionals. As for participants in our study, many persons with ALS depended on their family carer to participate in health care follow-up.⁵⁷ They also found that communication impairment in ALS may reduce the individuals’ experiences of receiving proper health care. It is, however, interesting that both persons with ALS and family carer participants in our study often refused receiving public health care or camouflaged their overall situation. Our results imply that it may be difficult for health care professionals to recognize their overall burden and needs in terms of health care. The reported differences in experiences and needs from the persons with ALS and their family carers in this study, accompanied by their misunderstandings of each other’s experiences, may also generate a hindrance when health care professionals try to tailor health care follow-up. We argue that the awareness of this tendency should encourage health care professionals to reflect on how to approach both persons with ALS and their family carers individually to discover “where the shoe pinches”.

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 persons with ALS gave to family carers in this study, and due to the importance of emotional support for family carers when coping with grief,⁵⁹ clinicians could encourage family carers of persons with ALS to seek social support outside of the patient-carer dyad and to prioritize important social relations. Tailored information, based on cognitive test results, may help family carers to better understand subtle cognitive impairment and behavioural changes of persons with ALS, which may ease caregiver burden. Family carers’ increased understanding might have a positive influence on persons with ALS, leading to fewer relational conflicts. Similarly, appropriately tailored information for persons with ALS may help them understand and participate in interactions and decision making.

When the future becomes lost

Participants’ experiences of lost future, 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 Coinciding with our results, Ozanne and Graneheim⁶⁰ found that ALS caused persons with ALS and their spouses ‘lose their foothold.’ As with our participants, they found that fear of the unknown caused persons with ALS and spouses to struggle with comprehending the diagnosis.⁶⁰ Shifting focus to familiar things, like positive memories and still accessible and meaningful activities, seemed to ease this feeling, like it did for most of our participants. This process resonates with the concept of doing in occupational science, where engagement in meaningful activities provide a sense of purpose and connection.^30,31 A tendency to find meaning and resilience by reflecting on the past has also been described in persons with primary progressive aphasia,²⁵ a neurodegenerative disease similar to ALS in certain respects.^61,62 The focus on positive aspects of lived life can also be understood through the lens of being – a state of existing authentically and maintaining a connection to one’s sense of self.^30,31

However, in the dual-process model of bereavement by Stroebe and Schut,⁴⁸ a single focus on positive aspects may relate to maladaptive coping.⁴⁸ The oscillation between loss-orientation and focusing on practical normalities, that seemed to be the tendency for most of our family carer participants, is according to Stroebe and Schut⁴⁸ the ‘normal’ way of coping and most likely a healthier way of dealing with bereavement. Yet, it appears to be little evidence describing ‘normal’ coping in grieving with 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. The denial and avoidance of future aspects of life that our participants revealed, could represent a survival strategy, a way to avoid the unpleasant. However, our findings are in line with research indicating that cognitive impairment amplifies this tendency of denial.¹ Persons with ALS’ avoidance of the abstract future might be due to faulty reasoning or conceptualizing, which is consistent with the cognitive profile of ALS.^11,12 For instance, the ability to visualize how life events will impact on future situation, depend on cognitive abilities that may be affected when having ALS, like abstract thinking and reasoning about context.^9,12,63

Nonetheless, how one person in a relationship cope will influence the other, and when they have different coping styles, relationships can become challenging.⁴⁸ The quality of the carer-patient relationship also appears to have a direct impact on the family carers’ grief intensity after the person with ALS dies, suggesting that difficult relationships might increase the risk of complicated bereavement.⁶⁴ By contrast, good quality relationships can ease the bereavement.¹⁶ Stroebe et al.¹⁶ argue that former research on attachment^65–67 shows that good relational quality between a dying patient and a bereaved importantly influences the outcome for the bereaved. The hypothesis that different coping styles intensify relational challenges between persons with ALS and their family carers⁴⁸ strengthens our argument that healthcare professionals have a responsibility to provide early support to persons with ALS and their family 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 persons with ALS’ and family 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 persons with ALS and their family 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.⁶⁸ Persons with ALS and family carers shared the approach of finding resilience by recalling good memories while aiming to ‘normalize’ their days. Aligned with conceptions of occupational science,^30,31 they reported how everyday things can have great impact, creating good days in the present. As Hitch^30,31 discusses, resilience and identity are preserved not only by action and the process of becoming through adaptation, but also through maintaining a sense of self in relation to others. It may be in the relational and emotional dimensions of belonging and being, where individuals find meaning and continuity through their relationships and experiences.^30,31 With a transactional understanding of occupation,^69–71 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. Persons with ALS’ experiences and behaviour in daily life affect the family carers’ and vice versa. Thus, professionals should encourage persons with ALS and family carers to reflect on their history together. Occupational therapists may highlight their occupational identity, and like Hammell⁵ argues, focus on meaningful more willingly than purposeful occupations when aiming to help persons with ALS and family carers to regain a sense of meaning in everyday life.

Methodological considerations

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 Reflexive thematic analysis to analyse the content of the interviews provided a systematic and rigorous, yet flexible thematic analysis.^42,43,44 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 person with ALS’ impaired cognitive ability to reflect on their situation, person with ALS’ 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 persons with ALS had indications of cognitive impairment, and some were with severe speech impediments, rarely addressed in ALS research. Our study presented perspectives from both persons with ALS with cognitive impairment and family carers, an underrepresented design that provided new insights. Due to fewer included participants than expected, the recruiting time was extended. Because we only included persons with ALS who had an ECAS-N-score indicating cognitive impairment, and with participants only from one region, the sample size is limited, representing 4 dyads. However, participants provided descriptive narratives, permitting sufficient data to identify themes. We lend support to Malterud,⁷² who emphasize that the information power within the sample determines how many participants is sufficient. The author report that if the data has high information power, between four and seven participants may provide sufficient rich material.⁷² On the basis of our experience, the interview data provided by the included four persons with ALS and four family carers, we determined that our study sample was sufficient to illuminate our research question, and to contribute with knowledge about an underrepresented research field. Larger studies that may underpin our findings is, however, needed.