Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are atypical Parkinsonian disorders with extended morbidity and reduced lifespan, known to have marked and early impact upon quality of life (QoL). This study aimed to address the lack of studies in the literature regarding personal perspectives on QoL in MSA and PSP in both patients and carers. Participants took part in qualitative, in-depth interviews in the North East of England, exploring what impacts their QoL and their experiences of living with these complex conditions. Connection to others was found to be a prevailing theme, encompassing difficulty communicating, social isolation, impact on personal relationships, and stigma. This work is helpful in that it emphasises the personal experiences of these patients and carers, which can provide insights into important areas for clinical service planning and best clinical management of individual patients as well as considerations for future research into QoL in these rare disorders.
Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are sporadic atypical Parkinsonian disorders (AP) which have poor response to symptomatic treatment, rapid and relentless progression, and reduced life expectancy compared with Parkinson’s disease (PD) [
This was an exploratory, qualitative project using semistructured interviews.
The study was approved by the Leeds-Bradford Research Ethics Committee. Participants were recruited from specialist atypical Parkinsonism clinics across three sites in the North East of England. All participants provided written informed consent. Participants were approached in clinic and provided with detailed information sheets informing them of the study aims, that participation was voluntary, that they could withdraw at any time, and that data would be treated confidentially. This qualitative study was part of a larger project exploring QoL in MSA and PSP using both quantitative and qualitative methods. Recruits to the project gave written consent if they wished to be approached to provide an interview. Any identifiable information was removed from transcripts, such as first names or surnames mentioned during interviews, to ensure the anonymity of interviewee and confidentiality was protected by the use of pseudonyms.
Patients had a diagnosis of MSA or PSP and carers were unpaid and voluntary. Purposive, pragmatic sampling was used to achieve a range and richness of experience with a balance of male, female, MSA, and PSP patients and a range of severities to give a more complete picture of living with AP. Particular effort was made to facilitate interviewing of participants with poor or negligible speech who used communication aids, as theirs is a poorly heard voice in clinical and research terms. In many articles in which interviews are performed with patients with Parkinsonian conditions, inability to communicate clearly is an exclusion criteria for participation, even in work on advanced disease. Indeed, few publications could be found by the authors (one included one patient with MSA and a mixture of neurodegenerative conditions and the other PD and stroke) describing the significant communication problems encountered in advanced neurological disease and allowing their participation [
The interview schedule was produced with reference to the literature on MSA and PSP and QoL as well as qualitative work in PD (due to the lack of work looking at QoL and experience of living with MSA and PSP) [
Basic semistructured interview schedule. Structure derived from Patton [
Semistructured interview topics |
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Background of life before diagnosis |
Process of diagnosis |
How have things changed |
Discussion of relationships |
How is MSA/PSP challenging |
Experiences with medical teams |
What is your understanding of Palliative Care |
Interviews were carried out by LW as a one-to-one meeting either in a clinical research setting or in participant’s homes, depending on which environment they were most comfortable in. Interviews were recorded on a digital device and transcribed verbatim. The interview transcripts were analysed using thematic analysis based upon the system described by Braun and Clarke [
Stages of thematic analysis from Braun and Clarke 2006.
In qualitative work, it is important in terms of transparency and rigour to describe the background of the researchers due to the influence this can have on their analysis. LW, the interviewer and primary researcher, is a training doctor in Neurology specialising in movement disorder with a particular interest in MSA and PSP and is an MD student looking at QoL and Palliative Care need in MSA and PSP. She undertook the main study design, participant recruitment, interviewing, analysis, and production of the manuscript. RD is a Geriatrics training doctor and movement disorder specialist who critiqued the analysis and contributed to the writing of the manuscript. ML is an advisor to the project and is a Consultant Physician and researcher in Palliative Care with an interest in movement disorder and was an advisor in the design of the project, the analysis process, and writing/review of the article. KB is an Associate Professor of Ageing & Health and is a Social Gerontologist. She advised on project design, reviewed and provided feedback on the coding process, and advised on project analysis and writing/review of the article.
QSR International NVIVO version 11 was used as an aid to analysis and data retrieval in thematic analysis. Coding was carried out as interviews were completed and were integrated into overarching themes; both data collection and analysis were an iterative process, whereby interviewing ceased when saturation took place, that is, when both LW and KB agreed that no more new, meaningful codes were being generated [
Rigour was ensured by referring to qualitative guidelines such as COREQ and Yardley’s criteria [
Two criteria used to demonstrate quality and validity in qualitative work and how they apply to this study. From Tong et al. [
COREQ domain checklist summary | Yardley’s qualitative validity criteria |
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Sampling method: purposive, pragmatic sampling | Sensitivity: study designed with respect to known literature with respect to patient input (patient/carer group consulted in design) |
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Setting: participant’s homes or clinical research facility (choice given). One-to-one interviewing | Commitment and rigour: LW experienced in movement disorder and specialising in AP. Extensive review of literature, training in interviewing methods, and analysis overseen by KB, an academic with many years of experience in qualitative research |
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Method: thematic analysis. NVivo v. 11.0 used as analysis aid | Transparency: methods described in methods section, process used shown in Figure |
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How was data recorded: recorded on digital device and transcribed verbatim | Impact and importance: having implications for planning of future services for AP and for improving best practice. Potential to impact QoL in a rare, underresearched group of conditions. Demonstrating the need for more work in the future |
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Description of themes: themes derived from data, not preselected then imposed | |
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Supporting extracts: quotations used throughout report |
Nineteen interviews were carried out in total, ten with patient participants and nine with carers. Four patients had MSA and there were four carers of individuals with MSA. There were six patients with PSP and five PSP-carers. Sixteen of the participants were patient-carer spouses and their relationships are described in Table
Participant demographics and relationships.
Pseudonym | Sex | Condition | Role | Age | Profession | Marital status |
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Matthew (MP1) | Male | MSA | Patient | 64 | Retired lawyer | Married to Sally |
Emma (CM1) | Female | MSA | Carer | 61 | Retired charity worker | Married to Matthew |
Sally (CP1) | Female | PSP | Carer | 70 | Retired dental nurse | Married |
Bryce (PP3) | Male | PSP | Patient | 76 | Retired technician | Single |
Doris (MP2) | Female | MSA | Patient | 59 | Retired librarian | Married to Bill |
Bill (CM2) | Male | MSA | Carer | 57 | Director | Married to Doris |
Rose (MP3) | Female | MSA | Patient | 71 | Retired teacher | Married to Jackie |
Jackie (CM3) | Male | MSA | Carer | 73 | Retired head teacher | Married to Rose |
Julia (MP7) | Female | MSA | Patient | 62 | Retired hotelier | Married to Tiberius |
Tiberius (CM7) | Male | MSA | Carer | 66 | Retired hotelier | Married to Julia |
Sarah |
Female | PSP | Patient | 67 | Retired teacher | Married to Tom |
Tom (CP3) | Male | PSP | Carer | 70 | Retired oil chemist | Married to Sarah |
Helen (PP18) | Female | PSP | Patient | 68 | Retired newsagent | Married to Earl |
Earl (Cp17) | Male | PSP | Carer | 70 | Retired chartered accountant | Married to Helen |
Mary |
Female | PSP | Patient | 69 | Retired newsagent | Married to Bob |
Bob (CP23) | Male | PSP | Carer | 69 | Retired newsagent | Married to Mary |
Gary (PP20) | Male | PSP | Patient | 58 | Retired project manager | Married to Pat |
Pat (CP19) | Female | PSP | Carer | 62 | Analyst | Married to Gary |
Jack (PP19) | Male | PSP | Patient | 71 | Retired HGV manager | Married |
A prevailing theme which was found in analysis was that of “connection to others.” Other themes which emerged from the interviews using the topics covered in Table
All prevailing themes from this project and their relationships to one another shown on the left. The theme “connection to others” and its associated subthemes are discussed in this article and are expanded on the right.
In order to relate to others and have meaningful interactions, communication is vital. The patient participants in this study had variable speech difficulty. Some were profound, necessitating the use of electronic communication devices (one participant used an adapted iPad and another a light-writer), whilst some remained intelligible. However, the majority of patients found that their ability to communicate with others was impaired. A recurrent subtheme was patients finding reassurance that their speech or fluency was not severe, frustrating, as they felt their struggles to communicate were being dismissed. (Doris, age 59, participant with MSA)
(Gary, age 58, participant with PSP)
Gary, a participant with PSP, referred to a comment from LW prior to interview where the intent had been to encourage him that his voice would be clear when recording. This feedback is valuable in that it informs us that a social nicety of trying to give confidence to patients that their voice “isn’t bad” may dismiss real concerns and distress which impact upon quality of life.
When the range of speech difficulty is considered, other participants were scarcely able to communicate verbally and needed to use electronic typing devices to “speak” for them. Interviewer: Sarah: Interviewer: Sarah: Interviewer: Sarah: (Sarah, age 67, participant with PSP (bold text indicates “spoken” via light-writer device))
(Bob, 69, carer of Mary who has PSP)
Although these devices can enable people otherwise rendered unable to speak to make meaningful contact with others producing improvement in relationships and well-being as a consequence, the reality is that motor slowness, stiffness, and possible cognitive problems make communication devices increasingly difficult to use as the disease progresses. This can be frightening for patients and carers as they consider that their new “voice” may not be useable forever. This can be seen from Bob’s statement above, as his wife Mary finds her light-writer more and more difficult to use and in Sarah who had profound slowness and rigidity, needing several minutes at a time to type short sentences or single words. This led to frustration where she tried to communicate with monosyllabic sounds when she was unable to type her thoughts.
All participants in the study, across disease types, both patients and carers, described the negative impact of MSA or PSP on their ability to maintain a social life. This seemed to manifest in different ways. One frequent concern of patients with Parkinsonism is maintaining the volume of their voice. Work has suggested that patients with PD may have impaired ability to detect low volumes in their own voice, feeling that they are shouting when they are in fact difficult to hear. This likely adds to social awkwardness and feelings of effort or struggle in conversation [ (Doris, age 59, participant with MSA)
(Rose, age 71, participant with MSA)
Therefore, patients may be experiencing distress due to being less able to connect to others by speech, even if families and medical staff are not aware of any problem. The change may be innocuous, such as Rose’s gradual shift during dinners to sitting back and letting the conversation flow around her as she realized her voice was becoming less able to cut through many voices in a loud social setting. This comparison with former selves and reducing abilities can produce a bereavement reaction for what has been lost.
From the carer’s perspective, the burden of care and responsibility, especially the 24-hour nature of it, had an effect on social networks. Emma discussed the rare times she was able to meet with friends but found that she was unable to confide in them and she was afraid she would be unable to “click back” into a mindset which she uses to cope. This suggests that some barriers between carers and their old relationships and social lives grow because of their new responsibilities and experiences: perhaps not feeling that they share enough in common to confide in them. This produces a disconnect from society for the carer. (Emma, age 61, carer of Matthew who has MSA) (Earl, age 70, carer of Helen who has PSP)
(Sally, age 70, carer of partner with PSP)
Earl and Sally discussed a very common subtheme in carers of both groups but most prominently in carers for people with PSP, the constant vigilance due to fear of their partner falling and injuring themselves. This seemed to encompass the full-time nature of caring and the emotional as well as physical pressures it exerts. This need to protect their spouse leads to an ever-shrinking social circle as their partner becomes less able to leave the home, resulting in social networks reduced to care-giver, patient, and occasional visitors to the home.
The ability to connect with others is influenced not only by the willingness and ability to communicate but also in how others perceive you (or possibly more importantly, how you believe others perceive you). Patients with MSA and PSP perceived stigma from others on the basis of their ability to interact. This was largely based on fear of perception from other from conversation, such as Gary feeling that his impaired fluency caused others to dismiss him or Rose’s concern that her motor slowing would be seen as cognitive decline. Gary: LW: Gary: (Gary, age 58, participant with PSP)
(Rose, age 71, participant with MSA)
Mary, who used a light-writer to communicate, felt her inability to speak and the time she needed (due to a combination of bradykinesia and bradyphrenia) to type her responses caused her to believe herself judged by those around her. She felt that her combination of disabilities led to others believing that she was stupid and ignoring her, a powerful and profound insight into how these patients’ symptoms impact upon their identity and well-being. Mary: LW: Mary: Mary: LW: Mary: LW: Mary: (Mary, age 69, participant with PSP (bold text indicates “spoken” via light-writer device))
Relationships and how they succeed or fail beyond a diagnosis of AP were a frequent theme. Relationships with others were frequently discussed within interviews. These relationships did not only include that between patient and carer (in this study, spouses) but also with other family members and friends.
As communication is so fundamental to relationships, the ability to have meaningful interactions with a partner is very important in maintaining that relationship through a diagnosis of a Parkinsonian disorder [ (Jackie, age 73, carer of Rose who has MSA)
(Sally, age 70, carer of husband with PSP)
Julia, a participant with MSA, spoke positively of the impact her marriage and the relationships with her family had on maintaining home life with a degenerative disease. The use of the term “rock” is an interesting one, suggesting her husband keeps her tethered or connected despite her illness. (Julia, age 62, participant with MSA)
(Tiberius, age 66, carer of Julia who has MSA)
Similarly, Julia’s husband Tiberius, as he simply put it, did not feel that the quality of his relationship with his wife had changed, despite the change in her health and abilities and that they still loved each other. This support for people with chronic conditions is very valuable and may trigger medical and social teams to consider patients and carers more as a pair, supporting both, as the well-being of one is so fundamental to the other.
Interviewed participants discussed the difficulties in maintaining friendships which seemed to be multifactorial. The nature of friendship and even the friends themselves seemed to shift or change with diagnosis and increasing symptoms. Bryce, a participant with PSP, felt that certain friends were only interested in socializing when he was well enough to do so and lost interest in him when he became less physically able. From the statement below he feels discarded by his former peers. Bryce: LW: Bryce: (Bryce, age 76, participant with PSP)
Bryce was the only participant within the cohort who did not have a partner or spouse. Therefore, the impact of this loss of interaction with friends was especially profound for him. Friendship is often built upon mutual interests, and being less able to participate may mean less contact with friends as those activities become less accessible.
This qualitative study suggests that maintaining connection and appreciating the possible barriers that MSA and PSP patients and carers face in this challenge are vital in enhancing QoL. There is evidence in the literature that connection to others is key to QoL in other chronic, life-limiting illnesses [
Being able to communicate, verbally or otherwise, is integral to being able to connect to others, individuals, and communities. It has been shown that people with PD feel excluded from conversations as speech becomes more effortful, impacting upon their relationships, personal identity, and dignity; they, like some participants in this study, felt that their voices were impaired even if others said they were not. These perceived communication problems were correlated with depression [
Stigma has been described in PD patients not just from physical changes in appearance but from decline in the ability to speak clearly and fluently [
Finally, AP affects the care-giver’s ability to connect to others. This is borne of the increasing support their relative needs, both physical and emotional, and the constant burden of vigilance and worry in keeping the patient safe. This can cut carers off from outside social life from the demands on their time and limited freedom [
This study is qualitative and based upon a group of participants in the North East of England; it is therefore not possible to conclude that its findings are generalisable. However, the strengths of this work include the subjective and personal insight into this group which is lacking in the literature; this is particularly meaningful when it is considered that QoL has a very personal and self-reflective element to it which may not be fully captured by quantitative survey data collection. Future work should consider QoL of patients and carers with AP, taking into account communication and relationships. The aim of this paper was to explore personal perspectives on QoL and how disease impacting connection to others can give clinicians insight into how AP affects patients and carers beyond a simple description of symptoms. The implications for practice are to emphasise the patient’s need to communicate and feel connected to others, their carer, families, and friends and to feel they are involved in clinical decision-making. This is of paramount importance in maintaining QoL and allowing holistic assessment in these conditions for which we, as yet, have no curative treatment. Acknowledging the impact of speech issues on the QoL of patients and carers is key, giving time for patients to try and convey their concerns (even when very slow or needing many attempts to do so) and good, early speech and language input to maximise their ability to communicate by whatever means they have available. These findings also have wider societal implications. It is difficult for medical services to address these far-ranging issues of a disconnect from others in patients and carers alone. In the future, incorporating social care, the volunteer sector and even technological advancements to enhance communication and independence should complement medical services. QoL is often assessed using quantitative measures and whilst this is valuable, work with patient groups using qualitative methods has the potential to shine a light on concerns that we may never have known existed.
The authors declare that there are no conflicts of interest regarding the publication of this paper.