Patients reported that their overall experience of living with IPF was one of coming to terms with a complex condition requiring a complicated and often protracted diagnostic work-up, with an uncertain but limited prognosis. They felt they received insufficient information on the clinical and practical management of their disease and would have welcomed more advice on pragmatic interventions at key points to help manage an unpredictable future. Although several common themes emerged for all patients, there was evidence of variation in the impact of IPF on patients from different disease trajectories.
Additional file 2: Box 1 summarises the key themes, common to patients and carers from all four trajectories, where IPF had significant impact on daily life.
Communication and information
Communication skills
Patients and carers reported that their key needs were structured around context, timing, content and format of information. They described a need to balance honesty and hope, whilst dealing with a terminal prognosis but searched for positive ways to manage and live with an uncertain disease trajectory (Additional file 3: Box 2).
Context
The specialist IPF clinic was seen as a trustworthy source of information. However, focus of the consultation was often perceived as disconnected from participants’ experience of the disease and its impact on everyday life. They felt unable to interpret the relevance of disease-focused assessments such as lung function tests to future exercise capacity or overall prognosis (Additional file 4: Box 3). Carers felt their role in clinical consultations was ambiguous and passive and this compounded the difficulty of accessing and interpreting information. Carer needs for independent information and advice were overshadowed by their desire to avoid distress to their partners in the clinic setting. Outside the specialist clinic the relative lack of knowledge about IPF in Primary Care added to the burden of uncertainty about future management.
Timing
The complexity of the diagnostic process often resulted in prolonged investigations and delays in referral to the appropriate specialist. Initial relief that the diagnosis was not cancer was replaced by shock at the likely prognosis. Patients and carers in the Limited Stable trajectory in particular struggled with uncertainties around the possible course of IPF. Their need to receive information at an appropriate pace triggered by changes they perceived in health status didn’t always coincide with the timing of clinic appointments. Paradoxically, they recalled feeling greatest concern about attending clinics where they had observed fellow patients with more severe IPF.
The specialist nurse was perceived as a key resource, enabling patients and carers to access help on practical management and triggering medical intervention when required. Despite this, patients and carers still struggled with uncertainty around key turning points if they occurred between clinic visits.
Content and format
Patients and carers appeared to show a good understanding of the overall prognosis for IPF but had difficulty translating this to their own particular disease progression and the management/support options available to them. They declared a need for specific information relating to oxygen therapy, nutrition, exercise, management of cough and breathlessness and disease management towards the end of life. Patients living alone were most direct in their need for information about future care planning.
Written information was most often suggested as useful. The internet was sometimes cited as portraying ‘worst case scenarios’ and there were mixed responses to the utility of a peer support group.
Changes in health status
Monitoring disease progression
As patients and carers had difficulty interpreting objective, clinic-based disease assessments, they described how they developed their own subjective strategies for monitoring IPF. These included looking for clues to health status by observing alterations to the frequency of clinic appointments and comparison with other patients encountered in the clinic or support groups. They also associated deterioration of IPF with intercurrent illnesses such as chest infections, initiation or increased use of use of oxygen, and changes in functional activity (Additional file 5: Box 4).
Negotiating disease progression
Sustained deterioration in health status led subjects to re-evaluate the uncertainty of their future (Additional file 6: Box 5). For those in the progressive trajectories, these shifts were associated with emerging differences in the information needs and coping styles of their carers who sought frank, objective information about functional deterioration and prognosis to help plan future care. This contrasted with patient reluctance to receive such detailed information. Perhaps unsurprisingly, the clinic environment struggled to meet these differing needs.
Functional activity
Diminished possibilities
Deterioration in health increased risk of social isolation for patients and carers, who experienced loss in relinquishing valued activities and responsibilities. Both groups adapted to a lifestyle of gradually declining choices rather than seeking assistance to optimise their functional activities (Additional file 7: Box 6). This was particularly apparent in patients living alone.
Most patients and carers felt unable to identify potential sources of help due to their uncertainty about the IPF course. Declining health status increased the emotional and domestic burden on carers, while patients expressed fears about employment loss, finance and how their partner would cope in future.
Understanding of symptoms and medical interventions
Specific concerns
There were specific concerns, especially for carers, regarding symptom monitoring in relation to breathing, cough and use of oxygen. Differing expectations between patients and carers were particularly evident around oxygen use (Additional file 8: Box 7). Carers viewed it positively as facilitating daily living. However patients often interpreted continual use of oxygen as a failure on their part which undermined the carers’ attempts to improve functional activity inside and outside the home. Oxygen therapy highlighted the perceived disparity between objective assessment of health status in IPF and functional ability of patients in everyday life.
Medical management
Patients viewed pulmonary rehabilitation as positive intervention, enabling them to participate in the management of their own disease and improve everyday living through increased awareness of coping strategies like energy conservation and better task management. Carers felt these strategies were more readily accepted if offered by a professional rather than by themselves.
Palliative care
Patients and carers frequently compared their situation unfavourably with cancer patients whom they considered to have “help coming from every direction”. In this context, common misconceptions about palliative care emerged in relation to reasons for referral as did concerns about being referred onto “that path to death.”
Two patients had received palliative care, one in a hospice out-patient programme and the other through home visits. They reported significant improvement to quality of life through peer support, and expert advice on sleep problems, palliative medication and home adaptations.
Roles and coping strategies of patients and carers
Patients
This study discovered significant differences in the range of coping strategies adopted by patients and carers. Core coping strategies for patients across all four trajectories included acceptance and adapting to change (Additional file 9: Box 8). The most significant factor for patients was whether they lived alone or had a partner. Patients living alone expressed their need for information about the future course of the disease and available sources of support.
Patients with partners expressed concern about how their partner would cope as the disease progressed.
Carers
Carers provided motivation and emotional support, placing the needs of the patient above their own (Additional file 10: Box 9). They were less likely to express positivity as a coping strategy for themselves and expressed feelings of not being prioritised and feeling unsure how to help. However, they did help implement strategies such as adaptation of lifestyle and task management to conserve energy, or using oxygen. The supportive role of the carer was particularly marked in the Extensive Progressive group, but carers across all trajectories expressed fears about managing their domestic situation in the future. Carers expressed living with feelings of guilt in all trajectories. The majority of carers in the study were female but male carers discussed how they had adapted gender roles to cope with the changing domestic situation as their partner deteriorated.