Table 4 Joint display of key quantitative findings for each PACIC subscale and interrelated qualitative theme

PACIC dimension: Delivery system design/Decision support

Definition: Actions that organize care and provide information to patients to enhance their understanding of care

PACIC dimension: Patient activation

Definition: Actions that solicit patient input and involvement in decision-making

Delivery system design/Decision support (Item 4–6)

*Theme 1: Experiencing organized care with limited participation

Describes SSc patients’ experiences with care delivery and shared decisions

Most patients were satisfied with overall organisation of care

(Item 5: \({\overline{x}}\) = 3.9, 95% CI 3.7–4.1)

Nevertheless, only 29% of patients (always/most of the time) received a written list of things they should do to improve their health

(Item 4: \({\overline{x}}\) = 2.6, 95% CI 2.3–2.8)

Only 37% were (always/most of the time) shown how their self-management strategies influenced their condition

(Item 6: \({\overline{x}}\) = 3.1, 95% CI 2.8–3.3)

Differences in reported PACIC levels were found according to patient comorbidities. Patients with lung problems reported the highest mean PACIC levels (\({\overline{x}}\) = 3.2, CI: 2.9–3.5), while lower levels were found in those with more than 2 comorbidities (\({\overline{x}}\) = 2.8, CI: 2.5–3.0)

Participants appreciated regular medical check-ups (approx. every 1–2 years), but feared negative results. Those with an early/mild form of SSc sometimes doubted the necessity of such expensive examinations

«The examinations were really stressful. The organization was super, but I thought, hopefully they won’t find anything. The more examinations there are, the sicker you feel. I thought, I just have to hang in there. But I asked myself later if that was really necessary. If they don’t find anything, fine—but it still costs a lot. It’s contradictory, or..?!» (Patient 4, interview)

Participants reported limited own participation during consultations/check-ups. They all reported to be able to ask questions, but in their perception, it was the healthcare team that made the decisions

«Later, the (health care professionals) talked among themselves and reached an agreement, then I was asked to join them and was informed. That’s good in principle, then I can ask questions or learn what’s going to be done next.» (Patient 1, interview)

Participants experienced that their own self-management strategies were not specifically valued or integrated by healthcare professionals (HPs). Some reported being afraid to inform physicians about strategies such as complementary therapies or felt not taken seriously if they did

«I noticed myself that it’s hard to contribute during the examination. For about 10 years I didn’t dare to say I take Vitamin D. And now I also take B-Vitamins.» (Patient focus group)

One patient described her experiences with one-to-one peer support as very supportive in the decision-making process for a possible lung transplantation

«Lung transplantation was being considered for me. I could talk to two people who had been through that. With one person in a one-to-one telephone call and I met the other personally. I got a lot out of it because it was possible to talk about personal problems and ask questions. You don’t dare do that in a group.» (Patient 2, interview)

Patient activation (item 1–3)

*Theme 2: Dealing with the illness in tailored measure

Describes patients being overwhelmed and protecting themselves from constant confrontation with their disease

Quantitative results:

27–31% of patients with SSc were never/generally not asked for their ideas when a treatment plan was made nor given choices about treatment to think about

(Item 1: \({\overline{x}}\) = 3.6, 95% CI 3.3–3.8, Item 2: \({\overline{x}}\) = 3.2, 95% CI 2.9–3.4)

Participants reported being overwhelmed with the disease information they received just after diagnosis, especially when it came in a written form without much explanation and the possibility to ask questions

«I had questions (about the disease) and since I knew nothing about it, he (doctor) just gave me a brochure. I knew I have a limited system sclerosis, but [the brochure] described really everything horrible and that scared me even more. It was terrible. I felt awful for a while and didn’t read anything more. I had first to get over the shock.» (Patient 2, interview)

Only half of patients were (always/most of the time) asked to talk about their problems with medicines or their effects

(Item 3: \({\overline{x}}\) = 3.4, 95% CI 3.1–3.6)

Participants who did not experience their symptoms as part of a severe disease, especially if the symptoms were only mild, did not want to deal with examinations and possible disease consequences, which have not yet occurred. Nevertheless, several emphasized the need for a step-wise learning process oriented toward progression of their own illness

«I try to live as normally as possible. That’s why I don’t do some things that make me feel sick (for example, examinations) even if that overtaxes me sometimes. I like it when I can solve something myself or can call in if something is wrong.» (Patient 1, interview)

Participants in a later disease stage described how they protect themselves from a constant confrontation with the disease by a reduction of medical consultations and examinations or by limiting them to a certain period of time. Those with extensive expertise reflected that for optimal patient activation, health care professionals have to tailor information and provide support according to an individual patient’s disease stage and readiness

«At the beginning, you really need support from other people. Later there’s a phase where you can manage the situation yourself. But later, you reach another point where you just can’t cope, because this happens or that turns up. I am now at a point where I have lost the orientation. I don’t know any more what I should do, or which doctor I should see. Then you need support again. What you need differs and it’s really hard for the health care professionals. The only solution is talk to one another and find out what is difficult for the person and what can be done about it. Just general strategies don’t work.» (Patient focus group)

PACIC dimension: Goal setting/Tailoring

Definition: Acquiring information for and setting of specific, collaborative goals

PACIC dimension: Problem-solving/Contextual Counselling

Definition: Considering potential barriers and the patient’s social and cultural environment in making treatment plans

Goal setting/Tailoring (Item 7–11)

*Theme 3: Not knowing which strategies are effective or harmful

Describes the lack of guidance for independent self-management by patients

Quantitative results:

73% of patients never/generally not received a copy of their treatment plan

(Item 9: \({\overline{x}}\) = 2.0, 95% CI 1.7–2.3)

60% were never/generally not encouraged to go to a patient support group or class

(Item 10: \({\overline{x}}\) = 2.3, 95% CI 2.0–2.6)

Almost half of patients were never/generally not asked to talk about their self-management goals (43%) or helped to set specific goals to improve their eating or exercise (47%)

(Item 7: \({\overline{x}}\) = 2.8, 95% CI 2.5–3.0, Item 8: \({\overline{x}}\) = 2.6, 95% CI 2.4–2.9)

22% were never/generally not asked questions about their health habits (e.g., risk factors such as smoking)

(Item 11: \({\overline{x}}\) = 3.6, 95% CI 3.3–3.9)

Problem-solving/Contextual counselling (Item 12–15)

Quantitative results:

57% of patients were (always/most of the time) sure that their doctor or nurse thought about their values and traditions when recommending a treatment

(Item 12: \({\overline{x}}\) = 3.5, 95% CI 3.3–3.8)

55% were never/generally not helped to plan ahead to take care of their condition(s) even in hard times (Item 14: \({\overline{x}}\) = 2.5, 95% CI 2.2–2.8), nor were they helped to make a treatment plan that for their daily life (48%, Item 13: \({\overline{x}}\) = 2.7, 95% CI 2.4–3.0)

15% (n = 15) reported having a depression in the last year

Participants described that they lacked guidance and exchange with health care professionals on self-management strategies to maintain their health and well-being, not only during and after diagnosis, but also later when they were already considered ‘experienced’ patients

«I always had the feeling that the answer (from the doctor about what one can do oneself) was that the course of the disease is very individual and for that reason, there is no general answer. That doesn’t really help and I said to myself: OK, I’ll just leave it.» (Patient 2, interview)

Participants explained that even when self-management strategies were recommended by professionals, as for example to improve physical activity, they often felt lost with respect to planning and evaluating these activities. Even participants spending time searching for appropriate information reported difficulties in adapting the information they found

«They never told me what I really should do with the crosstrainer or the bicycle in reference to me specifically, how long, at what level, at what watt setting. I’m sorry about that, because I really don’t know whether I do too much or too little. It would also help as motivation to training. I don’t even know if it would be noticed, if I didn’t go there.» (Patient 1, interview)

Some participants questioned the strong focus on medical outcomes, such as lab or examination results, to evaluate their health and well-being. In particular experienced patients emphasized the importance of patient- experienced outcomes as a focus of care, to prevent anxiety and insufficient self-management in patients.«The question is also: "Who defines the outcomes?" When the doctor looks at the values and says: "Super! It’s stayed the same, the blood value is good! That’s good, I am satisfied." But the patient feels worse. Yeah, what happens then?» (Patient focus group)

*Theme 4: Feeling left alone with disease and psychosocial consequences

Describes the difficulties of dealing with disease consequences that are rarely addressed by HPs

Participants reflected on their difficulties to address negative emotions and social problems with health professionals. They reported feelings of shame or just did not expect support from professionals. Especially patients in the early stages of the disease described how they isolated and tried to cope with negative emotions by themselves

«You isolate yourself in shock and in fear of what can happen. And then there is self-stigmatization. You think you’re alone in the world with this disease. And the professionals can’t help you, although they know everything.» (Patient focus group)

Participants revealed that psychosocial consequences such as depressive symptoms or financial problems were not systematically addressed by professionals. Several patients reported that they suffered from problems of feeling down, sleeplessness or financial worries over longer time periods and had not been asked about these by professionals

«What was my experience? Well, definitely not thorough, that someone (professional) said, so let’s sit down and talk about you and your situation, what effects this might have (..) In fact, my husband was the one who suffered most, who had to put up with everything I couldn’t explain. This despair: Now I am really sick and there is no cure and that’s terrible. And the sleeplessness and all these things, he had to put up with them. It certainly wasn’t an easy time for him.» (Patient 2, interview)

Participants emphasised the importance of having at least one trustworthy person to talk to about their daily concerns related to their disease. Some did not want to burden their relatives with these worries or did not feel understood by them and sought professional help themselves by consulting a psychologist or psychotherapist

«I can’t really talk about this at home. They don’t understand. That’s why I think psychological help is important, to get this off my chest. It’s not about finding new strategies to do things better, it’s about the everyday burden, everyday worries.» (Patient 1, interview)

Participants did not perceive self-help groups as a source of support for problem solving. Whereas some felt they were still 'too healthy' to join such a group, others experienced participation as additional emotional strain on them. For example, because of moaning by others. Given the variety of disease representations and experiences, focus group participants discussed the limitations of traditional self-help groups for people with SSc

«It doesn’t help. I come out of there and say, how can one ever be so taken by yourself, by the disease, that that’s the main topic in life. It is often very extreme, when you always hear the same thing at every meeting, I just have to say, that’s too much for me.» (Patient 1, interview)

PACIC dimension: Follow-up/Coordination

Definition: Arranging care that extends and reinforces office-based treatment, and making proactive contact with patients to assess progress and coordinate care

Follow-up/Coordination (Item 16–20)

Quantitative results:

69% were never/generally not encouraged to attend programs in the community

(Item 17: \({\overline{x}}\) = 1.9, 95% CI 1.7–2.1)

64% were never/generally not contacted after a visit to see how things were going

(Item 16: \({\overline{x}}\) = 2.1, 95% CI 1.8–2.4)

55% were never/generally not referred to a dietitian, specialist nurse/health professional

(Item 18: \({\overline{x}}\) = 2.5, 95% CI 2.2–2.8)

42% were never/generally not asked how their visits with other doctors were going

(Item 20: \({\overline{x}}\) = 3.0, 95% CI 2.6–3.3)

24% were never/generally not told how their visits with doctors like heart or vascular specialist helped their treatment

(Item 19: \({\overline{x}}\) = 3.6, 95% CI 3.3–3.8)

**Theme 5: Taking over complex coordination of care

Describes how difficult it is for patients to obtain a problem/therapy synthesis and to coordinate their own care

Participants emphasized that living with a rare disease such as SSc requires consultations with many specialized medical providers. Timewise this required coordinating work by themselves. Overall, they perceived these services as highly competent with some variety in professional’s ability to summarize results in plain understandable language. This was important for them, to make sense out of the examination results in a specific organ-oriented field

«I had to take a lung function test every year and the information there was very good. I also thought highly of that doctor. He spent about half an hour with me after the test and explained what it means and what I have to expect, or maybe expect, in the future.» (Patient 2, interview)

Participants reported that the communication of an overall synthesis (i.e., of the many specialists’ examination results and decisions) to gain an overview of individual disease manifestations and outcomes was lacking. They highlighted their need and attempts for a coordinated decision-making, by bringing information from other services into discussions. However, all emphasized that these coordination tasks cannot be conducted equally well by all patients at all time points as it needs expertise, self-empowerment and depends on disease-complexity

«That’s how it is in the hospital, I understand that, they have a lot to do, but then someone comes about blood vessels, then someone about the lungs, and then there’s someone about the heart, totally different things. And there is no summery at the end. I am no professional and I can’t put the puzzle together from these pieces.» (Patient 2, interview)

Remarkably, some highly experienced patients reported that they took over the coordination of the involved professionals (e.g., organ specialists, other HPs) and made them aware of their multiple problems to be considered in the overall decision making. This gave them a sense of control

«It was important to me to realize that the disease affects the whole system, everything, physical, emotional, family, material. And our health system is fragmented and organ-related. There is no Care Management or Case Management, at least not in Switzerland. And I had to learn on my own to do that, to see how I can deal with the specialists. These specialists who pounce on one organ system and know a whole lot about that, but don’t see the connection or only want maximum therapy for one part. Who pulls this all together if I don’t? After all, we can’t change the system so quickly. We can only emancipate ourselves.» (Patient focus group)

Additionally, some participants highlighted the problem of short-coming economic incentives in our fragmented healthcare system, which prevents care coordination and long-term savings

«I need 3 × 50 mg per day. And then I found out that there is also Sildenafil from Sandoz, 3 × 50 and I figured out what that costs for a year. The Revatio, 20 mg, or Sandoz 50 mg. Sandoz 50 mg costs almost 10,000 Franken less per year. I blame the whole system a little. There are things that could be optimized using these doses or brands. I would think looking at such things would be part of the medication check the pharmacies make.» (Patient 3, interview)

**Theme 6: Relying on an accessible and trustworthy team

describes patients’ initiatives to find reliable professionals and peers to support them on an ongoing basis

Participants highlighted that the ‘rigid’ annual monitoring appointments have the advantage that examinations with a range of relevant specialists are bundled and coordinated within one centre of expertise. This enables easy and reliable access to a defined range of providers once a year. However, individual adaptations to this plan (e.g., meetings with additional professionals for specific problems) were only possible to a limited extent

«I like having an assessment where there is a location once a year. I don’t live directly near the hospital and it’s really great for me if I don’t always have to make the trip. But what isn’t so good, you can’t always introduce additional things. Even if you said you wanted to when the date was set.» (Patient 1, interview)

Building their own trustworthy team of professionals was a major point raised by participants to improve care. Having confidence in their expertise and being cared for continuously over a long period of time was crucial for them. They even paid for consultations and therapies not reimbursed by the health insurance out of their own pockets to collaborate with professionals of their preference and receive the therapies they needed

«With respect to Physio, I organized something special for myself. I think the person is very competent, so the relationship is very good. It is not only Physio, but sometimes a bit psychological as well. We’ve known each other for quite a while. But that’s it, when you know each other for some time—now with that one person it’s not the problem, with the other it is the problem: it becomes routine. And then the same thing is done, whether you need it or not.» (Patient 1, interview)

Participants in a later disease stage described how they had learned to educate professionals who were not yet familiar with the disease and its consequences. In this context, they highlighted the importance of self-management support to reach expertise. Trustworthiness of an individual provider was finally also judged on their ability to collaborate with them

«I see it as a tipping point, a kind of transition where you are quasi the expert and you get asked and you’re "empowered" in the sense: I now look after myself. And if I have to go somewhere else, like to a new dentist or a new gynaecologist, then I tell the doctor that I have this disease and that I have dry mouth, for example. The doctor maybe never heard of sclerodermatitis and I can explain it…» (Patient focus group)