Living with progressive neurological disease and breathing difficulties

Introduction

Persons diagnosed with progressive neurological disease, e.g., amyotrophic lateral sclerosis (1), multiple sclerosis (2,3), and muscular dystrophies (4,5), might in the later stage of their disease suffer from breathing difficulties with impaired cough function. Weak cough can be a consequence of one or more impairments, such as weak respiratory muscles, bulbar dysfunction, and reduced chest mobility (6). These impairments can lead to airway infections (7-11) and death (12). Breathing is fundamental for life and when it is threatened it can affect the whole person, including physical, psychosocial and mental wellbeing (13).

Aside from breathing difficulties, these persons also experience challenges with movement, communication, drinking, and eating (5,14-17). At this stage of the disease, some persons are near end-of-life and become dependent on others for performing daily activities (18-20). The multiple challenges that the persons are facing, makes the introduction of new treatments a potentially complex aspect of the care process. To better support it has been suggested that health care professionals need to understand how the patients and their family perceive and communicate their own situation (21).

To assist persons with the progressive neurological disease with a weakened cough, the use of a mechanical insufflation-exsufflation (MI-E) device is recommended (22). While evidence suggests that MI-E treatment can aid in mucus clearance, support deeper breathing, and reduce hospital admissions (23), there is limited knowledge regarding the broader impacts of breathing difficulties and MI-E treatment on the person’s overall life and wellbeing (24). Furthermore, there is also a complexity of whether treatment such as MI-E treatment is life-saving or a palliative relief for persons with progressive neurological disease. A few studies have described that living with progressive neurological disease is a multifaceted struggle and uncertainty (25-27), but there is a need to further understand care from a broader perspective, in order to meet the individual person’s wishes and needs.

There is a need to adopt a deeper and broader research approach that starts with the person who is being introduced to the new treatment. This can contribute to a better quality of care and improved communication about that care with the person and their families. Therefore, the aim of this study was to explore persons’ experiences of living with progressive neurological disease and breathing difficulties. We present this article in accordance with the COREQ reporting checklist (28) (available at https://apm.amegroups.com/article/view/10.21037/apm-24-139/rc).

Methods

Study design

A qualitative inductive approach was applied (29) using interviews. This was the initial study conducted in the larger research project understanding home-based MI-E treatment for persons with progressive neurological disease (30).

Research characteristics and reflexivity

The first author (A.A.W.), a female physiotherapist and PhD student, having clinical experience in rehabilitating and caring for persons with neurological diseases, trained in interview techniques and learning qualitative methodology from PhD courses and piloting the interviews, was involved in all the steps of the project. This was done with supervision from co-authors, with experiences in nursing, physiotherapy, neurology, respiration, person-centered care, palliative care, and qualitative and quantitative methods.

Setting and sample

Persons were recruited for the MI-E project from four clinics in three health care regions in the middle of Sweden. The inclusion criteria were persons over 18 years of age, diagnosed with progressive neurological disease and breathing difficulties with a weak cough who had been prescribed treatment with the MI-E device for use at home. For inclusion in the study, the person was also required to be able to communicate in some way and have enough energy to participate in an interview, either verbally and/or non-verbally, and/or together with a supportive person where necessary. The sampling was consecutive and the first contact with potential participators was left to the health care professionals at the clinics where MI-E was introduced. Hence, the researchers had no control over this, e.g., how many persons weren’t asked or how many refused. One speech therapist and ten physiotherapists at the clinics, identified 11 persons according to the inclusion criteria and asked the person whether the first author (A.A.W.) could contact them by phone for more information, e.g., aim and reasons for doing the research, and to answer any questions about participation. All 11 persons who were asked to participate in the project agreed and then the first data collection occasion was arranged. One of these persons suffered from severe fatigue and had difficulties with communicating, both verbally and non-verbally, and, even with support it was difficult for the person to participate in the interview, so, for this reason, this person was excluded. A total of 10 persons were therefore included (Table 1).

Data collection

Iterative semi-structured interviews (29), were collected in the larger project by the first author (A.A.W.). The interview started with the following question: “Please, tell me about your life with your illness”. Probing questions were used about breathing and about cough. Follow-up questions were used, such as “Please, can you tell me more about…” or “Can you explain what you mean with…”. Prompts were also used asking about difficulties and how to manage them. In addition, any experience of living with the disease that was identified in the follow-up interviews for the larger project was also collected. In total, the data comprised 15 interviews with 10 informants, of which 5 persons were interviewed twice, and 5 persons were interviewed once. The interviews lasted from between 14 to 53 min (mean 32 min). In five of the interviews, the informants chose to have support from another person. One of the persons wanted to be interviewed by telephone the first time and at the hospital the second time. Another person was an inpatient at the time of the first interview and at home for the second interview. The others were interviewed in their homes. Two informants died after the first interview and therefore a follow-up interview could not take place. In all the interviews field notes were taken and after, also all the interviews were audio recorded and transcribed verbatim by a professional transcriber. Transcripts were not returned to informants for comment and/or correction.

Data analysis

We used qualitative content analysis according to Graneheim and Lundman (31), adapted to the use of the software NVivo (2020). The first author (A.A.W.) was responsible for the analysis with peer review provided by the co-authors (E.B., M.S.). The analysis process, for each step, was also presented through regular meetings with all co-authors. The unit of analysis was transcripts from the 15 interviews described above. First, the transcripts were read iteratively to make a sense of the whole. From the sense of the whole, spontaneous preliminary subthemes were formulated but saved separately in the NVivo file. Second, the inductive analysis started by dividing the transcripts into meaning-units (parts of the text sharing similar content) and simultaneously formulating condensed meaning-units. The condensed meaning units were either formulated as descriptive words on manifest level, summarizing the content, according to Graneheim, Lindgren and Lundman (32,33) or when longer meaning-units interpretations were conducted (34). After each transcript, the condensed meaning-units sharing same content were sorted into preliminary sub-themes. This was also functions as a practical step to organize data in the software program to make it clearer and more manageable. The categorization was an iterative process of constantly alternating between parts (decontext) and the whole (recontext), resorting and moving, renaming, merging and splitting subthemes and themes (31,33). At the end of the analysis, the spontaneous sub-themes from the sense of the whole were tested against the inductively created sub-themes and themes. The condensed meaning units and spontaneous sub-themes, such as “no choice just accept”, “life changes”, “sadness”, “losses”, “adaptations” were used to facilitate and formulate the final sub-themes “growing awareness of the seriousness of the disease” and “learning to live with the disease in the present”. An iterative categorization process followed with varying levels of interpretation and abstraction with co-assessment from co-authors and continued while writing up the results. No participant checking on the findings was done.

The study was conducted in accordance with the Declaration of Helsinki (as revised in 2013). The study was approved by the Regional Ethical Review Board in Uppsala (No. 2017/417) and informed consent was obtained from all individual participants.

Results

Living with progressive neurological disease and breathing difficulties can be understood as a reconciliation with a difficult life, a powerlessness in the fight against deterioration, and a fluctuation between feelings of trust and mistrust (Table 2). Informants shared their narratives in different ways. Some began by describing their lives before the diagnosis, with a gradual onset of symptoms leading to the later symptom of breathing difficulties. Others started by focusing on their experience of breathing difficulties such as coughing difficulties. Living with a non-curable disease, and experiencing breathing difficulties, along with other impaired abilities, confirmed the seriousness of the disease, and deepened the sense of powerlessness.

Reconciliation with a difficult life

A growing awareness of the seriousness of the disease emerged as a worry over experiencing the deterioration of health associated with the disease without the recovery of impaired abilities, including an increased need of aids and support from others. The onset of the illness started as a single bodily problem, such as a drop foot, a weak hand, or an impaired ability, such as walking difficulties, or unclear speech, and then gradually spread to affect the whole body. Before even knowing about the diagnosis, informants felt a growing awareness of the seriousness of the disease.

“It’s been two years now. I went to a physiotherapist and was given an exercise program for my neck. But she probably knew all along that it was this because she referred me on ... it didn’t take long before I got to the neurologist … It took some time before I got these investigations. But before that, you’ve googled it, you know.” Woman, 62 years old.

Impaired breathing abilities, emerged as a particular concern, due to its existential threat. Breathing difficulties were described as energy demanding and a combination of multiple physical difficulties, e.g., taking deep breaths, coughing with sufficient force for mobilizing troublesome mucus and the effort keeping the body in a position to facilitate breathing since non-optimal positions could suddenly close the airway.

“It was not fun two years ago, I was feeling very tired and I was coughing for two weeks, and thinking how would this be like in the future. I felt that I used all my muscles when coughing. I was thinking what if I wouldn’t have this strength, I would be completely full of mucus, would not be able to take it out, because I felt how my muscles were working a lot. So, okay, even though I was getting really tired, still I was able to cough. So I was like, oh, what if I have no muscles to cough?” Woman, 35 years old.

Feelings of ambivalence were conveyed as a resignation and a sadness of having the disease, but also as the determination of trying to cope with its effects in everyday life. Learning to live with the disease in the present emerged from an acceptance and acknowledgement of life from the experience of having no choice and needing to make the best of the situation. There was a need to seek and gain knowledge about the disease, a need that could vary between persons and also over time for the individual. For example, a person could both express a need to ignore the future in order to cope with the present and at the same time express a need to understand the whole situation, including future deterioration, to accept and cope in daily life. An approach of learning more, not focusing too much on the negative in the near future and being in the present was expressed.

“I would like to know all about the disease, but I don’t think much about the really bad times. Right now, they are distant.” Man, 74 years old.

The experiences of living with the disease and its conditions, combined with the emotional strategies individuals adopted to manage its symptoms, revealed how they came to acceptance and made the best of the situation. The progressive decline in abilities, particularly the emergence of breathing difficulties, confirmed informants’ fear of life-threatening severity of the illness.

Powerlessness in the struggle against deterioration

A fear of losing control of breathing emerged from feelings of uncertainty, worry, and anxiety, and the panic of not being able to cope with breathing difficulties in stressful situations. In situations when breathing abruptly stopped working, the person felt paralyzed and feelings of panic arose, such as when lying flat, eating, drinking, or exercising. Feelings of muscle weakness were conveyed in situations where persons were affected by exhausting coughing, for example, when having a cold, and breathlessness while experiencing premenstrual syndrome. Breathing difficulties, for example, shortness of breath, were experienced as worsening daily life in situations such as when dressing, eating, talking, and climbing stairs. In these situations, when the disease progression became obvious, feelings of not being in control were expressed, as well as a sense of fear of not being able to cope with even more severe breathing difficulties ahead. The will to fight this was expressed as doing everything they could to not end up in these difficult situations, for example, by performing various breathing techniques and optimizing bodily positions.

“[quiet cry]… It’s really difficult to cough when I have a little phlegm and stuff like that, you have to make several attempts before you can sort of manage to get it in your mouth ... the trouble is, I have noticed ... last three months there is a change … but so far it works so to speak ... so the problem is that it’s getting worse, that’s the crux.” Woman, 48 years old.

Bodily expressions, such as sighing, looking down, silence and/or crying were expressed during the interview, over a constantly changing everyday life with increased dependence. Loss of one’s control and integrity in life was present in their descriptions of experiences of becoming more and more dependent on others in everyday activities. Need for assistance could include grocery shopping cooking, feeding, personal hygiene, and transfer between bed and wheelchair or turning in bed. A wish for co-determination was expressed, but, along with the physical deterioration, there were no alternatives, they just had to accept the descending process. For example, experiences of being dependent on others in mealtime situations, such as needing help to adjust the consistency of food and drink or getting help to connect to an alternative source of nutrition via a percutaneous endoscopic gastrostomy (PEG) tube and pump when the ability to swallow no longer worked. In continuously losing one’s independence and autonomy of these elementary abilities, a feeling of powerlessness in life emerged. Existential contemplations were conveyed about losing control of the ability to make decisions about one’s life.

“I have to have help with everything, I can’t eat [quiet cry] ... I have no choice but to accept ... I’ve had many thoughts … for a long time … but then it’s too late.” Woman, 76 years old.

Giving up social life emerged through feelings of inadequacy and uncertainty in social situations. Experiences of inadequacy were expressed as arising out of physical and mental tiredness. Moreover, the uncertainty of being in social contexts, for example, concerns about being exposed to respiratory infections, were expressed. There was a sense of being limited in their own ability to control and independently handle these situations. Giving up their social life also gave rise to feelings of guilt towards relatives because plans for joint trips and events could no longer be carried out as before. Work or pleasure trips with colleagues and relatives were no longer possible as a matter of course. Any outings were tiring due to the cost of the physical and mental energy that entailed. Along with this, much more planning was required, as the person often had difficulties moving independently and may need others to assist as well as bringing various aids to make the trip possible. As the deterioration progressed being a burden to others needs of social life was expressed.

“I used to be able to go out and mow the lawn and go up on the roof or whatever. Now I can’t do a shit, my wife gets to do everything and this time of the year it’s a lot... She would like us to go away, but I think it’s so difficult, so I don’t want to, but I don’t want to say it straight out…” Man, 74 years old.

Giving up a social life was expressed with gradually deteriorating functional abilities. A lack of ability to communicate was experienced as being a challenge when engaging in social gatherings, described by the informants as having limited vocal strength or unclear speech required a greater effort in social contexts such as large groups. The energy required to raise or strain the voice was felt to be insufficient. Participation in social situations was considered to be something positive for one’s well-being, but decisions about participation were considered together with the negative consequences. Moreover, the choice to leave one’s home to live in a nursing home was expressed with sadness of giving up on having a social life, but at the same time as being the only option with considerations for the well-being of relatives.

The ongoing struggle against deterioration, experienced as feelings of uncertainty, inadequacy, fear, and anxiety, gives an understanding of a powerlessness that encompasses entire life.

Fluctuation between feeling trust and mistrust

In the everyday experiences of difficulties, it was expressed that relationships and contact with health care services could both bring feelings of gratitude for getting help and disappointment for not always getting help on time, or that care was not always provided.

Feelings of trust were implied through experiences of care for symptom relief, such as giving help with reducing burdensome symptoms and having practical support when bodily abilities deteriorate. A great gratitude was expressed over being understood and in safe hands, by being provided with concrete help. Good concrete help was described as the alleviation of breathing difficulties through medical treatment against saliva production and airway obstruction and the facilitation of breathing by physiotherapists, such as being given aids to facilitate deep breathing. Other good support could be exercised for trunk balance, mobility, or cardio training. Receiving this concrete and individualised adapted care was experienced as being listened to, also given a sense of security and crucial for managing life. When more support and help were brought in, a sense of relief was conveyed. In addition, having care and support provided opportunities to save energy and facilitate other aspects of life, such as working, spending time with family and friends, and engaging in leisure activities. There were experiences of using an electric wheelchair to make it easier to accompany the family, for example, to the shopping mall, and of being provided with aids to operate the computer to continue working.

“It will be a little calmer when the home care comes in ... she [the wife] can’t handle it... she wants to help. Both for me and for my wife, I think this can be good ... and you might have time for something else, too.” Man, 78 years old.

Besides the importance of receiving care for symptom relief, the idea of trust also emerged from easing of life through the caring presence of others. This care, with warm goodwill of others, characterized by being engaged, generous, understanding, careful, receptive, energetic, confident, and physically close was expressed as being supportive when challenging emotional and practical situations in everyday life. In experiencing such an easing of life through the presence of others, an openness to receiving help and having confidence and trust in others emerged.

“… I met some physiotherapist, some are better than others… they feel more confident and closer, like … how to say, closeness? I feel like someone that is taking care of my body, like when they touch… And I think if you, you’re not scared, I think … I could like feeling more your body … and also the person feels more your body and you can connect more…” Woman, 35 years old.

In contrast, feelings of mistrust were implied through experiences of being neglected as a whole person from a broader time perspective and delays in receiving care. This was expressed as being disappointing and was said to lead to misdiagnoses, unnecessary examinations or unsuitable transitions between different levels of care. A sense of not being able to trust the information received from healthcare providers or their knowledge was conveyed. This could relate to staff having insufficient knowledge about the disease at the health care center, for instance, not understanding how serious a cold could be in the shadow of their underlying illness. Experiences of being badly treated were expressed as being devastating and a sense of being abandoned by health care services emerged and contributed to this sense of mistrust.

“That’s what’s bad about care, if you seek help for a drop foot, then they only focus on that instead of looking at the whole and listening in. That you don’t connect things better and faster and listen ... but it was just like ... [laughter] I sat there and made up a lot ... the more I told ... [crying].” Woman, 48 years old.

Other issues in the context of care were experienced, such as missed or delayed care arising out of a lack of access to follow-up care by specialists and specific examinations within specialist health care. This was described as having major consequences for them as individuals. Disappointment with specific health care professionals or clinics was described, as well as general mistrust for the health-care system in general.

“… it’s probably primarily at X [the neurology department] that their responsibility lies, that, that’s where I have my doctor and nurse, so they’re the ones who have to give me information if no one else does ... I don’t like them too much, because I was at X [an intensive rehabilitation center], a year ago for a month and trained maybe five hours a day and then I came home and was well trained, but I didn’t get a follow-up, not a word.” Man, 41 years old.

There were also experiences that staff were too busy and there was a risk that things that had previously been decided were falling through. A sense of being abandoned and having to manage on your own emerged. Not having access to the appropriate care, which they felt they had a right to, created the feeling that they were taking part in a lottery. Living with this, while at the same time being totally dependent on the care of others, was experienced as a sense of insecurity.

“Now I was very surprised, because they had just walked out on me and said, ‘You only need to raise the alarm and we will come,’ and now it took quite a while... I did raise the alarm. So I feel this is not good. You never know what I want.” Woman, 76 years old.

Fluctuations of trust and mistrust, implied from experience of being respected and not respected as a whole and competent person. The sense of being safe and secure and having reliance in others revealed the importance of feeling goodwill and caring presence of others, also being listen to and receiving symptom-relief care.

Discussion

Persons’ experience of living with progressive neurological disease, breathing difficulties with impaired cough emerged as a gradual reconciliation with the difficult situation, a sense of powerlessness in the struggle against deterioration and a fluctuation between trust and mistrust. These notions of reconciliation, powerlessness, and trust align with the idea of loss of dignity of identity explained by Nordenfelt (2004). The dignity of identity is changeable, influenced by one’s own and other persons’ actions and attitudes, and by the illness and its impairments, and, when this threatens the person’s autonomy and integrity, it leads to consequences for their identity and thereby their dignity (35). To treat a person with respect for his or her dignity means to respect the person’s standards and values and this is fundamental for respecting his or her equal status (36) and worth as a person (37).

Our findings indicate that living with progressive neurological disease encompasses a process of moving towards a changed identity along with a threat to dignity. The person becomes gradually reconciled with a new identity from a growing awareness of the seriousness of the disease and learning to live with the disease in the present. The sense of powerlessness that arises from the fear of losing control of breathing, losing one’s independence, and giving up one’s social life indicate a threat to the person’s identity and dignity. Similarities with other qualitative studies in the field have been described, such as balancing between acceptance and fighting spirit (38), feeling violated in not being taken seriously and treated as a thing (39), fighting a losing battle but also gradually conquering oneself (27), having concerns for the future (26), and having to constantly adapt due to the disease and its changing influence on bodily functions (40). The sense of trust was implied from a sense of ease that evolved from the goodwill and the caring presence of others and from situations of experiences of symptom-relieving care. This element of our findings, experiencing trust, security, confidence, respect and kindness, is in line with the provision of dignified care (41). The experiences of caregivers who had the time and the will to see and listen to the person have, in earlier studies, been described as empowering the person to live (38,42) and maintain their dignity (41). In contrast, a sense of mistrust emerged from experiences of care and health care situations that brought feelings of insecurity, ignorance, disrespect and misunderstanding. The findings in our study are in line with previous research regarding the importance of respecting the person’s own standards and values. In contrast, not respecting these can be humiliating and shaming, and can promote a loss of the sense of being a human (35).

Our results, describing trust in different care situations, share similarities with the empathetic and compassionate care described by Sinclair et al. (43). Empathetic characteristics can be defined by an acknowledgment of suffering and promoting an understanding of the person by providing an effective response, such as “Can you help me to understand your situation?” (43). Compassionate characteristics also involve an action-oriented state, presenting understandings such as, “I know you are suffering, but there are things I can do to help it be better” (43). In contrast, sympathetic care comprises understandings that include approaches such as “This must be awful”, and are considered to be unwanted pity-based response, a shallow and superficial emotion based on self-preservation, and an unhelpful and misguided reaction to suffering (43). It has been suggested previously that professionals should acknowledge the expertise of the individual person and their relatives and collaborate with them in setting shared goals (40). A care context characterized by trust, where making the decisions for the person takes much energy, could be left to the health care professionals where the individual is confident that they will act in their best interests (44). Also, knowledgeable health care professionals could provide the person with theoretical understanding and explanations of their illness and the symptoms they are likely to experience, which can be significant for them in understanding how to manage the illness (40). This person-centered care approach, including a co-creation process, sharing experiences and learning from each other, is described as being a multiple parallel process, unique to each individual person, can be useful to facilitating each patient’s vital goals (45) and to facilitating an individualized introduction of the MI-E device for persons diagnosed with progressive neurological disease (30).

Our study revealed suffering from living with progressive neurological disease, implied by informants’ experiences from reconciliation with difficulties in life and from powerlessness in the struggle against deterioration. This underscores the importance of integrating a palliative person-centered care approach early on for individuals diagnosed with progressive neurological disease, addressing both their suffering and the need for comprehensive support before and under the terminal stages of the disease (46-49). Radbruch et al. (2020) (50) defined palliative care as an “active holistic care of individuals across all ages with serious health-related suffering due to severe illness and especially of those near the end of life. It aims to improve the quality of life of patients, their families and their caregivers.” (42).

In the literature, from a palliative care perspective, holistic care was defined as “care addressing psychosocial, spiritual, emotional, social and/or existential domains” (50,51). These domains were all present in our subthemes to some extent, taking in the seriousness of the disease, living with the sadness of the disease, losing control of breathing, losing one’s independence, giving up one’s social life, receiving symptom-relief care, a sense of easing life through the caring presence of others, and managing on your own while feeling abandoned by healthcare. Touzel and Shadd (2018) describe that a palliative care approach exists when care simultaneously addresses a form of holistic care, improved quality of life, and the acknowledgement of mortality (51). In our study, we described that there might be some variation in the ways in which persons wish to receive information and make decisions: some of our informants preferred to ‘take each day as it comes’ rather than focusing on the difficult future, while others wished to plan extensively to allow them to adapt for future deterioration. Being perceptive to the patient’s wishes has been suggested to be a key approach in preserving dignity in a palliative care context (42). Through perceptiveness and with a respectful dialogue about death, dying and a limited prognosis, an opportunity is opened up for the person to express their wishes and needs for the last time in their life (51).

From our study, both symptom-relief and curative care was important for the person, such as receiving curative care to manage a cold and relief care to manage symptoms related to their illness. Our findings, feelings of managing on your own and being abandoned by health care in not receiving care are in line with those of Chochinov et al. (2002), who describe the relationship between symptom distress and dignity (52). Living with a fatal illness and not being relieved from, for example, breathing difficulties, presents a sense of fear of not having control of one’s own body, and the uncertainty of not coping with the suffering, poses a threat to one’s dignity. For persons suffering with severe illness, symptom management is important for maintaining dignity (52) and is also recognized as a core aspect of palliative care in order to improve quality of life (50). The complexity of symptoms and symptom relief have already been described by Cicely Saunders in the 1950s and 1960s in hospice care in England (53). Saunders developed the concept of “Total pain”, which includes the physiological, psychological, social, emotional and spiritual aspects of the symptoms of pain and pain relief for people diagnosed with cancer at the end of life (53). Saunders’ holistic view of her patients, taking the person’s story as a starting point, is considered to be the basis for today’s view of palliative care (54). The person’s breathing difficulties, where the cause is related to a gradual weakening of the respiratory muscles, can be difficult to deal with, as there is no cure for the disease. However, within a palliative person-centered care approach, seeing symptoms from a holistic perspective, there may be interventions to support the person to manage other symptoms that catalyse breathing difficulties. Moreover, dignity-conserving care activities, such as targeting the symptoms holistically (38) encouraging patients to live in the moment and giving the patient support for maintaining normalcy and sharing discussions about existential matters (42), is suggested to enable the person to engage in continued participation in various social contexts, and has also been suggested to maintain their autonomy and dignity (38,42).

To adapt to a holistic care, a multidisciplinary team is recommended to facilitate a broad perspective of care, with the aim of maintaining and improving both physical and psychosocial functions, including respiratory function (55). Supporting a person through their disease can be challenging, particularly where the person has been diagnosed with an incurable disease, and where the consequence of the progressive respiratory failure is death (56). Nurses have expressed uncertainty in caring for persons diagnosed with ALS, due to the person’s individual and unpredictable illness trajectory, and also their difficulties in identifying changes in the deterioration of bodily functions, especially breathing difficulties (57). A need for improved education programs and resources to provide better quality of care has been expressed (57). A multidisciplinary team can also provide support to the health care professionals within a team. Physiotherapists have expressed that having peer support, in sharing knowledge within the team, and emotional support, reflecting on one’s own emotions while caring for these persons, provided assurance in their interactions with the patient (30).

Methodological considerations

A major strength of our study was the qualitative design starting with a broad question about life. This gave depth and width of the informants’ narrations about the phenomenon, combined with a large variety of informants. However, some informants had difficulty in communicating and were exhausted by their disease, thus difficulties obtaining interviews of good quality. Here, the supporting person contributed by interpreting the person’s speech. The interviewer also repeated what she had perceived from the conversation, and the person could confirm or oppose through body language and/or by providing a short answer. Even if many of the persons were tired afterwards, they expressed that it felt good to share their stories, in knowing that the sharing of their experiences might help another person in a similar situation.

Conclusions

This study has contributed to a deeper and broader knowledge of the experiences of progressive neurological disease and breathing difficulties from the person’s perspective of the illness, and how health care professionals can support these persons. Our findings highlight the need of a person-centered palliative approach, which is holistic, dignity-preserving, empathetic, and compassionate. This approach should prioritize symptom relief and involve a co-creation process to support each patient’s vital goals. It underscores the need to integrate palliative care early in the disease trajectory, well before the terminal stages. A palliative approach also requires a multidisciplinary team and educational efforts to clarify the implications of palliative care for this patient group, alongside knowledge exchange between different professions.

Acknowledgments

The authors thank all the patients who gave their time by taking part in this study. The authors also thank Aileen Ireland for excellent language review.

Funding: This work was supported by the Uppsala-Örebro Regional Research Council (RFR-842271 to A.A.W.); Agreement Concerning Research and Education of Doctors (OLL-935347, OLL-960580, OLL-973020 to A.A.W.), and Örebro Research Committee (OLL-781231, OLL-869781 to A.A.W.) and NEURO Sweden (to A.A.W.). The funders played no role in the design, conduct, or reporting of this study.

Footnote

Reporting Checklist: The authors have completed the COREQ reporting checklist. Available at https://apm.amegroups.com/article/view/10.21037/apm-24-139/rc

Data Sharing Statement: Available at https://apm.amegroups.com/article/view/10.21037/apm-24-139/dss

Peer Review File: Available at https://apm.amegroups.com/article/view/10.21037/apm-24-139/prf

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://apm.amegroups.com/article/view/10.21037/apm-24-139/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki (as revised in 2013). The study was approved by the Regional Ethical Review Board in Uppsala (No. 2017/417) and informed consent was obtained from all individual participants.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

References

1. Sancho J, Servera E, Díaz J, et al. Predictors of ineffective cough during a chest infection in patients with stable amyotrophic lateral sclerosis. Am J Respir Crit Care Med 2007;175:1266-71. [Crossref] [PubMed]
2. Levy J, Bensmail D, Brotier-Chomienne A, et al. Respiratory impairment in multiple sclerosis: a study of respiratory function in wheelchair-bound patients. Eur J Neurol 2017;24:497-502. [Crossref] [PubMed]
3. Tzelepis GE, McCool FD. Respiratory dysfunction in multiple sclerosis. Respir Med 2015;109:671-9. [Crossref] [PubMed]
4. Muni-Lofra R, Juanola-Mayos E, Schiava M, et al. Longitudinal Analysis of Respiratory Function of Different Types of Limb Girdle Muscular Dystrophies Reveals Independent Trajectories. Neurol Genet 2023;9:e200084. [Crossref] [PubMed]
5. Statland JM, Tawil R. Facioscapulohumeral Muscular Dystrophy. Continuum (Minneap Minn) 2016;22:1916-31. [Crossref] [PubMed]
6. Mamarabadi M, Mauney S, Li Y, et al. Evaluation and management of dyspnea as the dominant presenting feature in neuromuscular disorders. Muscle Nerve 2024;70:916-28. [Crossref] [PubMed]
7. Wenninger S, Jones HN. Hypoventilation syndrome in neuromuscular disorders. Curr Opin Neurol 2021;34:686-96. [Crossref] [PubMed]
8. Benditt JO. Pathophysiology of Neuromuscular Respiratory Diseases. Clin Chest Med 2018;39:297-308. [Crossref] [PubMed]
9. Bourke SC. Respiratory involvement in neuromuscular disease. Clin Med (Lond) 2014;14:72-5. [Crossref] [PubMed]
10. Sheers NL, Berlowitz DJ, Dirago RK, et al. Rapidly and slowly progressive neuromuscular disease: differences in pulmonary function, respiratory tract infections and response to lung volume recruitment therapy (LVR). BMJ Open Respir Res 2022;9:e001241. [Crossref] [PubMed]
11. Rose L, McKim D, Leasa D, et al. Patterns of healthcare utilisation for respiratory complications of adults with neuromuscular disease: a population study. Eur Respir J 2018;52:1800754. [Crossref] [PubMed]
12. Perrin C, Unterborn JN, Ambrosio CD, et al. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve 2004;29:5-27. [Crossref] [PubMed]
13. Laviolette L, Laveneziana P. Dyspnoea: a multidimensional and multidisciplinary approach. Eur Respir J 2014;43:1750-62. [Crossref] [PubMed]
14. McGinley MP, Goldschmidt CH, Rae-Grant AD. Diagnosis and Treatment of Multiple Sclerosis: A Review. JAMA 2021;325:765-79. [Crossref] [PubMed]
15. Rowe JB, Holland N, Rittman T. Progressive supranuclear palsy: diagnosis and management. Pract Neurol 2021;21:376-83. [Crossref] [PubMed]
16. Mercuri E, Muntoni F. Muscular dystrophies. Lancet 2013;381:845-60. [Crossref] [PubMed]
17. Galvin M, Madden C, Maguire S, et al. Patient journey to a specialist amyotrophic lateral sclerosis multidisciplinary clinic: an exploratory study. BMC Health Serv Res 2015;15:571. [Crossref] [PubMed]
18. Prell T, Gaur N, Stubendorff B, et al. Disease progression impacts health-related quality of life in amyotrophic lateral sclerosis. J Neurol Sci 2019;397:92-5. [Crossref] [PubMed]
19. Mioshi E, Lillo P, Kiernan M, et al. Activities of daily living in motor neuron disease: role of behavioural and motor changes. J Clin Neurosci 2012;19:552-6. [Crossref] [PubMed]
20. Bass AD, Van Wijmeersch B, Mayer L, et al. Effect of Multiple Sclerosis on Daily Activities, Emotional Well-being, and Relationships: The Global vsMS Survey. Int J MS Care 2020;22:158-64. [Crossref] [PubMed]
21. Clarke G, Fistein E, Holland A, et al. Planning for an uncertain future in progressive neurological disease: a qualitative study of patient and family decision-making with a focus on eating and drinking. BMC Neurol 2018;18:115. [Crossref] [PubMed]
22. Chatwin M, Toussaint M, Gonçalves MR, et al. Airway clearance techniques in neuromuscular disorders: A state of the art review. Respir Med 2018;136:98-110. [Crossref] [PubMed]
23. Mahede T, Davis G, Rutkay A, et al. Use of mechanical airway clearance devices in the home by people with neuromuscular disorders: effects on health service use and lifestyle benefits. Orphanet J Rare Dis 2015;10:54. [Crossref] [PubMed]
24. Morrow B, Zampoli M, van Aswegen H, et al. Mechanical insufflation-exsufflation for people with neuromuscular disorders. Cochrane Database Syst Rev 2013;CD010044. [Crossref] [PubMed]
25. Hughes RA, Sinha A, Higginson I, et al. Living with motor neurone disease: lives, experiences of services and suggestions for change. Health Soc Care Community 2005;13:64-74. [Crossref] [PubMed]
26. Harris DA, Jack K, Wibberley C. The meaning of living with uncertainty for people with motor neurone disease. J Clin Nurs 2018;27:2062-71. [Crossref] [PubMed]
27. Wenneberg S, Isaksson AK. Living with multiple sclerosis: The impact of chronic illness. Vård i Norden 2014;34:23-7. [Crossref]
28. Tong A, Sainsbury P, Craig J. Consolidated criteria for reporting qualitative research (COREQ): a 32-item checklist for interviews and focus groups. Int J Qual Health Care 2007;19:349-57. [Crossref] [PubMed]
29. Kvale S. InterViews: an introduction to qualitive research interviewing. Thousand Oaks, CA: Sage; 1996.
30. Andersson-Watz A, Nygren-Bonnier M, Bergdahl E, et al. Introducing Mechanically Assisted Cough for Patients With Progressive Neurological Disease: Patient-Physical Therapist Interaction and Physical Therapist Perspective. Phys Ther 2024;104:pzae012. [Crossref] [PubMed]
31. Graneheim UH, Lundman B. Qualitative content analysis in nursing research: concepts, procedures and measures to achieve trustworthiness. Nurse Educ Today 2004;24:105-12. [Crossref] [PubMed]
32. Graneheim UH, Lindgren BM, Lundman B. Methodological challenges in qualitative content analysis: A discussion paper. Nurse Educ Today 2017;56:29-34. [Crossref] [PubMed]
33. Lindgren BM, Lundman B, Graneheim UH. Abstraction and interpretation during the qualitative content analysis process. Int J Nurs Stud 2020;108:103632. [Crossref] [PubMed]
34. Svantesson M, Durnell L, Hammarström E, et al. Moral and exhausting distress working in the frontline of COVID-19: a Swedish survey during the first wave in four healthcare settings. BMJ Open 2022;12:e055726. [Crossref] [PubMed]
35. Nordenfelt L. The varieties of dignity. Health Care Anal 2004;12:69-81; discussion 83-89. [Crossref] [PubMed]
36. Barclay L. In sickness and in dignity: A philosophical account of the meaning of dignity in health care. Int J Nurs Stud 2016;61:136-41. [Crossref] [PubMed]
37. Gallagher A, Li S, Wainwright P, et al. Dignity in the care of older people - a review of the theoretical and empirical literature. BMC Nurs 2008;7:11. [Crossref] [PubMed]
38. Gysels M, Reilly CC, Jolley CJ, et al. Dignity Through Integrated Symptom Management: Lessons From the Breathlessness Support Service. J Pain Symptom Manage 2016;52:515-24. [Crossref] [PubMed]
39. Lohne V, Aasgaard T, Caspari S, et al. The lonely battle for dignity: individuals struggling with multiple sclerosis. Nurs Ethics 2010;17:301-11. [Crossref] [PubMed]
40. Handberg C, Werlauff U. People with neuromuscular diseases and their relatives' perspectives on challenges in everyday life and healthcare. Neurodegener Dis Manag 2023;13:289-302. [Crossref] [PubMed]
41. Lindwall L, Lohne V. Human dignity research in clinical practice - a systematic literature review. Scand J Caring Sci 2021;35:1038-49. [Crossref] [PubMed]
42. Harstäde CW, Blomberg K, Benzein E, et al. Dignity-conserving care actions in palliative care: an integrative review of Swedish research. Scand J Caring Sci 2018;32:8-23. [Crossref] [PubMed]
43. Sinclair S, Beamer K, Hack TF, et al. Sympathy, empathy, and compassion: A grounded theory study of palliative care patients' understandings, experiences, and preferences. Palliat Med 2017;31:437-47. [Crossref] [PubMed]
44. Lindberg E, Hörberg U, Persson E, et al. "It made me feel human"-a phenomenological study of older patients' experiences of participating in a team meeting. Int J Qual Stud Health Well-being 2013;8:20714. [Crossref] [PubMed]
45. Bergdahl E, Ternestedt BM, Berterö C, et al. The theory of a co-creative process in advanced palliative home care nursing encounters: A qualitative deductive approach over time. Nurs Open 2018;6:175-88. [Crossref] [PubMed]
46. Bužgová R, Kozáková R, Bar M. The effect of neuropalliative care on quality of life and satisfaction with quality of care in patients with progressive neurological disease and their family caregivers: an interventional control study. BMC Palliat Care 2020;19:143. [Crossref] [PubMed]
47. Tarolli CG, Holloway RG. Palliative care and Parkinson's disease: outpatient needs and models of care over the disease trajectory. Ann Palliat Med 2020;9:S44-51. [Crossref] [PubMed]
48. Kluger BM, Miyasaki J, Katz M, et al. Comparison of Integrated Outpatient Palliative Care With Standard Care in Patients With Parkinson Disease and Related Disorders: A Randomized Clinical Trial. JAMA Neurol 2020;77:551-60. [Crossref] [PubMed]
49. Oliver DJ, Borasio GD, Caraceni A, et al. A consensus review on the development of palliative care for patients with chronic and progressive neurological disease. Eur J Neurol 2016;23:30-8. [Crossref] [PubMed]
50. Radbruch L, De Lima L, Knaul F, et al. Redefining Palliative Care-A New Consensus-Based Definition. J Pain Symptom Manage 2020;60:754-64. [Crossref] [PubMed]
51. Touzel M, Shadd J. Content Validity of a Conceptual Model of a Palliative Approach. J Palliat Med 2018;21:1627-35. [Crossref] [PubMed]
52. Chochinov HM, Hack T, McClement S, et al. Dignity in the terminally ill: a developing empirical model. Soc Sci Med 2002;54:433-43. [Crossref] [PubMed]
53. Clark D. 'Total pain', disciplinary power and the body in the work of Cicely Saunders, 1958-1967. Soc Sci Med 1999;49:727-36. [Crossref] [PubMed]
54. Clark D. From margins to centre: a review of the history of palliative care in cancer. Lancet Oncol 2007;8:430-8. [Crossref] [PubMed]
55. Berlowitz DJ, Mathers S, Hutchinson K, et al. The complexity of multidisciplinary respiratory care in amyotrophic lateral sclerosis. Breathe (Sheff) 2023;19:220269. [Crossref] [PubMed]
56. Cheng HWB, Chan KY, Chung YKJ, et al. Supportive & palliative interventions in motor neurone disease: what we know from current literature? Ann Palliat Med 2018;7:320-31. [Crossref] [PubMed]
57. Daneau S, Bourbonnais A, Allard É, et al. 'Intensive palliative care': a qualitative study of issues related to nurses' care of people with amyotrophic lateral sclerosis at end-of-life. Palliat Care Soc Pract 2023;17:26323524231170881. [Crossref] [PubMed]