Seven patients with diagnosed IBM were interviewed between July and November 2020. All patients were retired, married and lived together with their spouse. More characteristics shows Table 1.

Table 1 Patients' sociodemographic and disease characteristics

Six interviews were conducted face-to-face, five at the patients’ home, one at the Friedrich-Baur-Institute; one via telephone, due to social restrictions of the COVID-19 pandemic.

Two patients wanted their spouse to be present all the time or part-time (n = 4), respectively the grandson (n = 1). Two patients were interviewed on their own. A few field notes were sent to one patient to clarify ambiguities. The interviews lasted on average 1.30 h (min 0.54 h; max 2.06 h). Altogether, 10.34 h of audio material was collected.

Phases of HRQoL and care situation: perceived social support as overarching theme

All patients reported dynamic interdependences of physical, psychological, and social HRQoL during IBM progression (Fig. 1). Time-varying, but temporal constant, perceptions of the patients were described as phases. Thus, the results suggest four phases to structure the HRQoL and care situation during the IBM patient journey: (1) uncertainty about physical vulnerability until diagnosis, (2) promising treatment approaches, (3) self-management and dyadic coping and (4) weak body, busy mind and caregiver burden. Therefore, the in-vivo code and homophony “patience journey” explicates the reported necessary emotional patience to persevere slowly progressive or stable phases, as well as exacerbations with sudden deteriorations of HRQoL.

Fig. 1

Dimensions of HRQoL in IBM and suggested tendencies due to physical progression. Applied and adapted dynamic intrapersonal appraisal of HRQoL in IBM patients during progression, based on theoretical considerations of symptom network appraisal [35]. The codes of HRQoL appraisal are shown as bowls for psychological (D1), depressive (D1a), social (D2) and physical (D3) dimension. Intrapersonal appraisal factors summarise the codes: coping (emotional and problem-oriented), coherence, self-efficacy expectation, religiousness and spirituality. We explicitly used this coding and presentation to demonstrate the indicated time-dependent connections of the HRQoL dimensions (two sided arrows) found in the interview material. The grey filling of the bowls illustrates the extent of impairment of HRQoL dimensions at exemplary time point during the four phases

Moreover, our identified overarching theme in these phases is the perceived social support, specified as informational, practical, and emotional support (IS, PS, ES) (Table 2). Social support was defined comprehensively to encompass not only informal, but also professional input [42]. The phases of physical, psychological and social HRQoL and care situation seem to have an interindividual timing and to be impacted by perceived social support. Figure 2 illustrates the interpreted tendencies of perceived support during the IBM patient journey. The applied theoretical conceptualisation and coding framework is shown in Additional file 1.

Table 2 Main characteristics of the coded support categoriesFig. 2

Tendency of perceived support during the four phases. Due to the qualitative design of our study, the graphs should depict the suggested hypotheses for the tendencies of IBM patients’ perceived support in the German healthcare system during the four phases

Phase 1: Uncertainty about physical vulnerability until diagnosis

Overall, patients initially reported an increased physical vulnerability with an intangible onset. Compared to the almost stable social HRQoL, the most common psychological response was feeling uncertainty. The first phase seems to last several months or even years.

Physical behaviour adjustments, changed motion sequences while deterioration of walking ability and intermittent falls were eventually interpreted as pathological: “It got worse and worse with the falls, more often. It was just that the feet were lagging” (P06 (patient interview code), paragraph (para.) 5). Decreased physical HRQoL was minor underpinned with less grip strength or swallowing problems as initial symptoms.

The social HRQoL seems not to be limited in this phase. No one reported constraints in the activities of daily living (ADL) or employment status.

Psychologically, the inexplicable vulnerability and laboratory findings were perceived as uncertainty. It appeared, that patients developed a sense of hazard cognition to manage resources or prevent falls: “I said: "I'm also thinking about the way back. Let's not go too far."” (P05, para. 53). Continuous informal ES mostly promoted patience to persevere this first phase of the total IBM “patience journey” and actively seek professional support until the patients receive a final diagnosis. Interestingly, a confirmed diagnosis of IBM by neuromuscular specialists (NMS) was vital for patients to satisfy an informational need. The negative emotional coping turned into more positive with time: “It pulls your feet away; And then there's just one thing: you have a wife, you have a family, and you just have to look ahead” (P06, para. 265). At the time of diagnosis, empathetical and honest professional ES was appreciated just as IS in the form of individual recommendations. The evidence that IBM does not lead to premature death promoted optimism in most patients.

Phase 2: Promising treatment approaches

A prominent theme in the second phase was to pinpoint various treatment approaches. Patients described slow but steady physical deterioration and social constraints. Positive coping abilities and emotional responses were minor underpinned by professional support. The data also indicated a varying duration of phase 2, mainly depending on how long patients perceive professional support as most helpful.

More specifically, professional PS (e.g., physical-, occupational therapy) and independently performed exercises by patients seem to enhance the physical HRQoL. Most patients aimed at muscle training, while they felt slightly impaired functioning. Some patients reported side effects of IVIG (thrombosis, hypertension, paravasat, dermatologic problems) and “warn [ed] about being too careless with immunoglobulins” (P01, para. 89). Hair loss was mentioned once, while participating in a pharmacological trial. For some, naturopathy mitigated pain or promoted muscular strength. Improvements or stabilisation of physical HRQoL due to pharmacotherapy was rarely reported; placebo effects were suspected twice. Most patients still walked independently, used crutches or canes occasionally. Walking and carrying objects simultaneously were challenging. Diminutive adaptions appeared important: “Whether you hold on to the banister just for safety or for fear is also important […] or […] to get upstairs at all […]. That's quite a big difference” (P02, para. 891–893). Swallowing problems were characterized as being less communicative during dinner or as experiencing embarrassment in public while choking.

Problem-oriented coping was common as “doing well with close relatives, with friends, with travel” (P04, para. 210). It appeared beneficial, “to have the time” (P02, para. 755) and “be relaxed” (P02, para. 758) at home and to afford mostly self-paid adjustments (e.g., banisters, higher toilets). Behaviour adjustments (e.g., changing hobbies) and commodity items (e.g., shoulder bags) served as assistive devices (AD), enabling social independency and flexibility.

AD were dominantly assessed positively as self-determination and security (psychological). Thereby, professional IS and ES “saved the mood” (P03, para. 595) and promoted acceptance and accurately customisation of AD. In contrast, anxiety, hopelessness or impaired self-efficacy expectations arose with minor empathetic and need-oriented professional IS and ES: “She [logopaedic] first explained to me all the things that happen to me with this dysphagia. She ended with a feeding tube. […] This frightened me a lot” (P04, para. 112–115). Information on available healthcare services provided security, whereas the cost coverage of IVIG distressed. Most perceived ES and IS from self-help groups as too general or scaring “steppage gait, polyneuropathy, […] ALS and all that shit” (P06, para. 145). Downward comparisons as “it is this [IBM] disease” and not “Pompe or other possibilities” (P02, para. 447) were mentioned. Anger, frustration, or shame were reported as thinking about visible vulnerability, physical ageing and barriers in the environment.

Phase 3: Self-management and dyadic coping

The transition from phase 2 to phase 3 could be characterized by intensified physical and social burden and the perceived run out of professional support as patients and caregiver predominantly cope dyadic. Psychological deteriorations appeared to be more prominent.

Precisely, the progression was more experienced as subphases, where “the disease strikes again and again” (P04, para. 139). Exacerbations were tremendous, relating to physical and psychological HRQoL: “I felt good, strong and then I get this hit of fracture and I don't even know how to get out of it” (P07, para. 16), or to social HRQoL: “I can't drive the boat through the harbour anymore, [so] I can't drive it at all.” (P03, para. 142).

The decline of strength, functioning, digestive disorders and chill was perceived as impaired physical HRQoL. Immobilisation and the need to rest happened faster than expected. The appropriate utilisation of AD was challenging. Many “always exaggerate it until” they “dropped” (P03, para. 110) painfully. More acute PS after falls was needed. Patients with severe dysphagia avoided certain foods and increased fluid intake. With professional PS (esp. physiotherapy), the patients now aimed more at maintaining mobility and functioning. Effects of IVIG > 6 months (n = 2) fluctuated between “no longer measurable” (P01, para. 28) or improvement. “Occasional examinations” at follow-ups with NMS were mainly perceived as “useless” (P01, para. 192). From patients’ views, clinical appointments documented physical deterioration, influenced work and leisure time but for some also promoted hope.

Rising PS was noticed for personal hygiene, domestic care or social participation. Self-paid services and multifunctional AD beyond statutory claims indicated a pleasing social HRQoL. Without appropriate information, “adventurous” (P03, para. 165) AD were customised to maintain mobility or lower the fear of falls. Most “got used to the visible disability” (P05, para. 75) and spend their time at home: “[Friends] always arrive with their cake, so that we are here in my familiar surroundings, where I have everything” (P05, para. 250). Loneliness was rarely reported.

The psychological burden was highlighted, especially symptoms of depression: “At least half of the strength that is lost goes out of the body, but also out of the mind. […] Sometimes even much more from the mind than from the body” (P01, para. 363–365).

The need of IS, how the progression “concretely affects the ability to move […] in the end” (P05, para. 216), was common. Patients were disappointed if they noticed physical exacerbations: “I thought it could not progress any further” (P07, para. 5). Lower perceived professional support after phase 2 and thus more self-management was mainly coped with negative emotions and distrust in healthcare. Two patients refused follow-ups with NMS, while others valued the “exchange of experience” (P01, para. 15). Patients felt distress by the rising costs of AD and the justification of their necessity to health insurances. Therefore, the support by physicians or therapists was mainly perceived as helpful, especially after tremendous exacerbations “when professionals say: "Go there, you're entitled to this, you need that"” (P06, para. 45). Hidden support let thereby patients “forget again that something is not possible” (P07, para. 133) indicating self-efficacy and positive coping. Self-help groups, ongoing treatment and religion served solely for some patients as most helpful coping strategy. But PS and vast ES from spouses were frequently a foundation for high HRQoL. The important issue of dyadic coping in the long-term was consistent in all interviews.

Phase 4: Weak body, busy mind and caregiver burden

Overall, IBM was in the fourth phase experienced as being “enclosed” in the body. Patients aimed at staying mentally healthy and active. The patients’ attitude towards support was now more to decrease the primary caregivers’ burden and postponing their own needs.

In detail, physically “the worst thing [was] this complete inability to move” (P03, para. 102) and the reliance on AD like electric wheelchairs. Inadequate positioning caused pain (e.g., joint pain). Swallowing problems increased, but no one needed special nutrition or tube feeding. Ageing seems to intensify physical vulnerability. Especially during the treatment of comorbidities, patients perceived professional PS as inappropriate or too little, mentioning “pressure sore on the back” (P03, para. 759) or too much “feeding” (P04, para. 67).

The social “circle [got] smaller and smaller” (P03, para. 208). Some features of AD like “toilet showers, which also have a blow dryer ((laughs))” (P04, para. 300) created intimate personal hygiene more pleasant for both caregiver and patient. IS was needed to arrange home care. “Care work is not really the right thing for an 83-year-old [wife]” (P03, para. 447) but nursing homes were predominantly “no alternatives” (P03, para. 793), as expecting lower HRQoL. Professional PS was valued if it relieved the rising caregiver burden, “to make it as comfortable as possible for them [caregiver]” (P043, para. 65).

A key theme was the gratitude being mentally healthy, like “not suffering from dementia” (P06, para. 61). Negative emotions appeared mainly from inappropriate professional support concerning the common close patient-partner-dyad: “365 days, 24 h, no old sow works for 728 euros. But the relatives.” (P06, para. 344).