This is the first study exploring how couples live and cope with MD1. It offers new insights into the self-management strategies used by couples from the perspective of persons with MD1, their partner and as a couple. Persons with MD1 experienced physical, cognitive and social barriers in daily life. Their strategies included postponing, avoiding or giving up activities, but also adjusting expectations and finding alternative ways to engage in less demanding activities. Partners reported an increasing burden and felt that if they did not do things, nothing happened. Strategies used by partners included taking over activities and giving instructions and prompts. As a couple they faced challenges in finding a mode together with renegotiation of tasks and individual and shared activities. Giving each other freedom, respecting each other's differences in pace and giving scope for their own interests were ways in which couples managed together. Couples experienced lack of understanding from family, friends, society and health care. This lack of understanding contributed to further withdrawal and avoidance of social situations. Couples said they preferred to help each other, rather than asking for help or understanding. Health care was experienced as many islands, with each speciality looking at one part of the disease. Little attention was paid to the consequences of this systemic, neuromuscular disease for their daily life and marital relationship.
Strengths and weaknesses of the study
Although a small, purposive sample (three men and two women with MD1), the strength of the study was that three in depth interviews, lasting between 60-90 minutes, were held with each couple. This resulted in 15 interviews with rich information from three perspectives: persons with MD1, their partners and as a couple. The codes saturated the four themes and resulted in "theme saturation" as well as "theoretical saturation". There were no obvious deviant or negative cases, but categories were saturated with a variety of positive and negative experiences. The trustworthiness of the study credibility was promoted through triangulation [25, 29].
There was homogeneity in age and the type of MD with the onset of symptoms in adulthood. All participants were Caucasian. There is no information on how MD1 affects the lives of persons from other than western cultures. Younger persons with MD1 might have different experiences, especially since this disease has the characteristic of anticipation, meaning that children of couples with MD1 may have a more severe type of MD revealing problems at an earlier age. The findings give an impression of the lives of middle aged couples with one partner having MD1. These middle aged couples may have found a way to live with MD1, which may not be the case in younger couples, who are in a different phase in their lives. Therefore these findings cannot easily be generalised beyond older couples.
Besides, it is to be expected that there is a considerable group of men with MD1 without a partner. Men with MD1 appear to have a decline in marriage eligibility whereas women continue to marry at a young age and in a proportion almost equal to that of the unaffected population . In a study of personality patterns of 15 people with MD1, 11 lived alone after divorce or were unmarried . Our research shows the support needs of individuals with MD1 and support strategies provided by their partners. This support need probably also applies for single persons with MD1. This often causes an additional burden for the healthy parent who also takes on the responsibility of taking care of and encouraging their child(ren) with MD1.
This study also included participants with communication difficulties due to dysarthria in contrast to a previous qualitative study . In some participants intelligibility was limited. The researcher repeated the participants' answers in order to make sure she understood; this repetition also facilitated the transcription.
Although MD1 is the most prevalent adult type of neuromuscular disease, the incidence is approximately 1 in 20.000; these findings may be relevant for couples with other complex chronic illnesses facing physical, cognitive and social impairments which limit everyday activities and social participation.
Meaning of the study: possible explanations and comparison with other studies
Previous studies aiming to get insight in the consequences of MD1 used quantitative approaches [[9, 31–33]], interviewed a heterogeneous group of persons with muscular dystrophies or their partners separately [[8, 12, 34]] or focused on living with the hereditary aspect of a muscular disease . This study is the first to combine clients' and partners' perspectives on their daily life experiences and how they cope as couple with MD1.
Lack of understanding of family, friends, society and health care was a major theme which was described in other studies in muscular dystrophy [[8, 11, 12, 35, 36]]. The following factors may be possible explanations: lack of communication, wrong expectations, ignorance and avoidance. Communication was not only an issue because of dysarthria, decreased intelligibility and limited facial expression in persons with MD1. More crucial is the lack of communication about the illness experience of persons living with MD1. How can friends, family and health care professionals understand if they do not communicate about the impact of the disease on daily life? Although patient-centred care is being promoted , participants in current study still experienced illness-centred care with many health care professionals focusing on part of the disease and its management and not on the consequences for their daily life. A recent review on the management of MD1 confirmed the concentration on impaired functions and structures in medical subsystems .
Other factors contributing to lack of understanding include wrong expectations and ignorance of other people regarding the potential or limitations of persons with MD1. As MD1 is classified as a neuromuscular disease, muscle weakness and fatigue are expected and treatment is aimed at improving body functions like aerobic capacity, breathing or hand function [[14, 17, 18, 39, 40]]. However, the additional cognitive and social barriers may explain why adherence to exercise programs is limited . Breaking down disabling social practices against people with MD1 might be as important, if not more so, than seeking treatment for physical impairments . This is in accordance with Boström and Ahlström who state that: many issues in the management of long-term chronic illness can better be interpreted from a social perspective rather than a biomedical perspective .
Professionals and others primarily focus on the needs of the person with MD1 and overlook the support needs of partners or other family members . Health professionals usually perceive partners as caregivers rather than care recipients. Also partners do not express their need for support, although they might experience a large caregiver burden, feeling no scope for own interests outside the home which can be a source of despair . This study showed how frustrating and annoying the lack of initiative and avoidant behaviour can be for partners. This is in agreement with findings from Timman et al. who found that for partners, worse general well-being and more anxiety was associated with a lack of initiative of the their partner with MD1 and less marital satisfaction. Partners experience difficulties in interpreting whether the need for support is related to MD1 or to lack of will or laziness . Not all partners were aware that lack of initiative and avoidant behaviour are part of MD1. Current study showed that insight and awareness that these problems are related to MD1 helped to more consciously apply compensation strategies like prompts or reminders.
Current study also showed the resilience of couples to manage together. Supporting each other and giving each other freedom were described as vital couple management strategies. This finding replicates the management strategies used by couples facing other multiple chronic illnesses . Two life philosophies were found: 1) staying positive; and 2) being married means supporting each other whatever happens and coping with what comes along. The combination of individual and shared coping strategies was considered indicative of a healthy balance . A continuum was described between positive support like a helpful reminder and problematic support like nagging or pushing a partner . A balance was promoted in which the person with the disease manages themselves and the partner provides support to this managing. How assistance is given and received as well as marital interactions that accompany this support, appeared to impact both marital quality and health . These findings support a couple-centred approach in couples with complex chronic diseases like MD1.
A couple management approach has shown to be effective for patients with dementia and their caregivers [44, 45]. The problems experienced by these couples in relation to coping with loss of abilities, initiative and participation in social activities, decreased quality of life and pressure on family relations and friendships  are strikingly similar to the problems experienced in MD1. The consequences of MD1 have been described as comparable to those of the aging population . This is in line with insights that MD1 is considered a progeroid syndrome with accelerated emergence of features of senescence including symptoms of dementia . There is evidence that a community approach aimed at increasing abilities of older people with dementia to engage in meaningful daily occupations and interventions to increase support giving skills of partners increases participation of the person with dementia and reduces caregiver burden [44, 45]. Further studies evaluating couple management for couples with other progressive, complex diseases are warranted.
Implications for clinicians and policymakers
Although there is mounting evidence for effective system changes that improve chronic care, such as the Chronic Care Model [[47–49]], health care often remains with basic structures and practices designed for acute diseases . The traditional medical care model mainly focuses on treatment of impairments , ignoring the illness experience and its impact on the system, including partners. Our recommendations for clinicians therefore include:
1. A shift from the focus on physical functions to a person-, or even a couple-centred approach aiming to understand not only the disease, but also the illness experience of persons with a complex chronic illness and their partners. This shift involves:
- A more narrative approach besides the traditional biomedical analytical approach; and
- Referral of patients and their partners to appropriate interdisciplinary medical, rehabilitation and community services to address barriers from medical as well as psychological and social perspectives.
2. A shift from self-management to couple management in complex chronic conditions, in which self-management skills of both partners are supported in order to:
- Maximize participation for persons with a complex chronic disease such as MD1;
- Reduce caregiver burden by enhancing support giving skills; and
- Find a healthy balance together in shared and individual activities.
Challenges for the future include further development and evaluation of the acceptability and effectiveness of a couple-centred approach for complex chronic illnesses. A couple-centred approach includes the three aspects of self-management: medical management, role management and emotional management  and should also address disabling social practices. As this can be considered a complex intervention including several components [52, 53], it is recommended to use a stepwise approach as described in the framework for design and evaluation of complex interventions .