IPF is a devastating disorder and the findings of this qualitative survey of UK, German and Italian patients with IPF add to the existing body of knowledge regarding the substantial impact IPF has on the lives of patients and their caregivers [6–11]. In agreement with recent literature [12, 22], these findings also identify the need to more adequately support patients with IPF in terms of disease awareness, lifestyle management, psychological support and management of medication side effects, as well as emphasising the important role played by the HCP in having primary contact with the patient and their family.
Approximately one-third of patients in the survey were identified as being unaware of and/or uninformed about IPF, and most patients wanted a better understanding of the disease. However, sources of information on IPF were frequently of poor quality, out of date or not available in the patients’ native language. In the UK and Germany, patients predominantly used online sources when looking for further information about their condition, while Italian patients reported a preference for printed materials; however, it was unclear whether this was actually due to preference or to a lack of web-based IPF information in Italian. In general, however, when diagnosed with a chronic disease such as IPF, many patients now turn to online sources of health information as this allows them a degree of autonomy, and the ability to examine and digest information at their leisure [23]. The findings of this study support the need for good-quality, freely available, online information on IPF in multiple languages, to support patients in learning more about the practical management of their disease. It is also interesting to note that many patients expressed a desire for better understanding of IPF among the general public. The recent US EXPLORE survey found that patients with IPF often felt isolated, embarrassed and stigmatised [24], and patients with other chronic respiratory diseases, such as chronic obstructive pulmonary disease, often also report feeling stigmatised [25].
The findings of this survey also emphasise the important role played by the HCP who is the primary contact for a patient, whether they are a physician or a specialist nurse. In the UK, specialist nurses were reported to play an important role in supporting patients with many aspects of disease management and patients clearly felt this was beneficial. However, the UK is quite unique in having advanced ILD specialist nurses who are able to play a significant role in helping patients understand the disease and coordinate their care [22]. In a recent report by the BLF, 50 % of English healthcare trusts reported allocating an ILD specialist nurse within 6 months of a patient’s diagnosis [22]. Furthermore, UK clinical guidelines state that all patients with IPF should have an ILD specialist nurse allocated to them and that any centre that wants specialist status must employ a specialist ILD nurse [26]. UK clinical guidelines also recommend that the ILD specialist nurse should provide accurate and clear information to patients and their families, regarding diagnosis and management of IPF, and support the patient at all stages of the care pathway to facilitate transitions in care and advise on symptom management [26, 27]. In Germany and Italy, nurses were less involved in patient care. Specialist nurses for IPF care may warrant consideration by German and Italian healthcare authorities due to their ability to provide a holistic programme of care.
Caregivers were present in 80 % of the interviews conducted for this survey and provided a useful source of information. However, it should be acknowledged that the presence of caregivers during the interview may have inhibited responses from some patients. Nevertheless, the findings of this survey support the difficulties faced by caregivers in looking after patients with IPF and are in agreement with the literature. The caregivers of patients with IPF have reported hardships throughout the course of a loved one’s disease, including emotional devastation at the initial diagnosis and difficulties living with a loved one because of their limitations [28, 29]. Furthermore, providing additional support to caregivers of patients with IPF has been demonstrated to provide some positive benefits. For example, caregivers who attended a 6-week nurse-led group intervention programme on IPF management, alongside their loved ones with IPF, reported significantly lower stress levels post-intervention compared with those who did not attend the intervention programme [29].
Pirfenidone was the first medication approved for the treatment of European patients with IPF, and the positive feedback reported in this survey suggests it provides hope and reassurance to patients. However, the findings of this survey also highlight the need for guidance on how to identify and mitigate the side effects experienced by some patients during treatment with pirfenidone, such as the nausea and loss of appetite reported as symptoms of IPF in this study, which may have been related to pirfenidone therapy [16, 17] rather than to IPF itself. It should be noted that this survey was conducted in 2012, prior to the launch of pirfenidone in some European countries (including the UK). At this time, HCPs would have been less familiar with pirfenidone and its side-effect profile than they are at present. Recent reports from real-world studies suggest that the tolerability issues with pirfenidone can be well managed, with no new safety signals identified versus the clinical trial programme [16, 17, 30, 31]. However, there remains a need to provide patients with lifestyle guidance when they are taking pirfenidone to minimise the risk of treatment side effects. Some strategies that have proved successful include using sun protection to avoid photosensitive skin reactions and taking pirfenidone during or after a meal to alleviate effects on gastric motility [32].
While this survey reports the impact IPF has on patients’ lives and supports the need to address the unmet needs of patients’ and their caregivers, several limitations must be addressed. The survey was not designed to exclusively evaluate patients’ experiences of IPF. No formal measure of quality of life was implemented and patients were not randomly selected for participation in the survey. Furthermore, the sample size was small, although this is typical of other surveys that have been conducted in patients with IPF [2, 6, 11].
Developments in IPF management and disease awareness
As a result of surveys such as this one, many aspects of care for patients with IPF in Europe have changed since 2012. In 2013, the patient support initiative ‘IPF Care’ was established in eight European countries, to provide help and education for patients treated with pirfenidone through frequent discussions with ILD specialist nurses. The benefits of the programme are already receiving recognition, such as in the UK where 66 % of patients who reported an adverse event during the ‘IPF Care’ programme remained on maintenance therapy [13], and in Austria, where 96 % of patients who started pirfenidone with the support of ‘IPF Care’ remained on treatment for ≥3 months compared with only 64 % of patients who were not enrolled in the programme [13]. The success of ‘IPF Care’ demonstrates the value that patient support programmes can offer as a tool for complementing and enhancing the support provided by healthcare systems, and as a forum within which to discuss patients’ worries. Other initiatives include IPF World Week, established in 2012, to raise awareness of the disease through its ‘Blowing Bubbles’ campaign in several European countries [33], and the European IPF Patient Charter, established to support equal access to IPF treatment and care standards in Europe [34].
In the UK specifically, there have been many advances in IPF, including the launch of two patient advocacy groups (Pulmonary Fibrosis Trust in 2011 [35] and Action for Pulmonary Fibrosis in 2013 [36]), which provide information and support to patients, and the launch of a clinical pathway for IPF in early 2015 [37]. Furthermore, the BLF has supported the development of many projects, including an IPF Patient Charter (supported by multidisciplinary IPF experts, patients and caregivers) [38], a survey evaluating patients' experiences in England [22] and numerous sources of patient information. The BLF is also contemplating the development testing the feasability of a personal organiser for patients with IPF in the UK.
In Italy, general awareness of IPF is slowly improving; for example, there are several patient associations, including RespiRARE in Sicily, dedicated to rare lung diseases [39] and Ama Fuori dal Buio, which is very active in supporting patients and providing them with disease information [40]. The Observatory for Rare Disease conducted a successful social-media campaign inviting people to post pictures or videos of them trying not to breathe, to raise awareness of IPF [41], and in 2013, a patient- and caregiver-specific website was launched with disease information in Italian [42]. Italian clinical guidelines for IPF were also recently published [43]. In Germany, the patient association Lungenfibrose e.V is also very active in supporting patients and providing disease information [44], and clinical guidelines for IPF were launched in 2013 [45].
Future directions in IPF management and disease awareness
Nevertheless, more work is still needed to improve the diagnosis and management of IPF. In this study, most patients reported a delayed diagnosis and this is not uncommon for IPF and other rare diseases [46], as demonstrated by the results of the BLF’s recent survey where many patients with IPF in England reported struggling to obtain a diagnosis [22]. Patients in the BLF survey also reported not having access to information about IPF that they could understand, having to navigate their own care pathway, and a lack of access to an ILD specialist nurse. We should, therefore, continue to seek each patient’s perspective on their disease and treatment, so that the information and care they receive can be tailored to their specific needs, thereby providing the support they and their families require to live with IPF.
