Background
Idiopathic pulmonary fibrosis (IPF) induces progressive damage and loss of healthy lung tissue, hence causing irreversible pulmonary function impairment [
1,
2]. Available anti-fibrotic drugs slow down the ultimately fatal disease progression but do not restore normal lung function, which is only possible with lung transplantation [
3]. Unfortunately, survival time after diagnosis is low with a median time between 2 and 5 years, if left untreated [
4].
Inevitably, affected persons and their caregivers might experience a high disease burden with various educational, supportive and palliative care needs [
5]. Patients have to live with physical symptoms such as shortness of breath and cough, resulting in a negative impact on daily physical activities. Also, living with IPF has an impact on the psychosocial dimensions of life as patients need to deal with this life-threatening disease with an unpredictable disease course and a complex pharmacological treatment [
6,
7]. Hence, providing care to the IPF population across the range of care continuum from diagnosis until end-of-life brings important challenges for healthcare providers and systems. Indeed, an inordinate load on the healthcare system is posed due to high costs of care, the chronicity of the disease and increasing incidence numbers. More specifically, high resource use and costs have been reported due to hospitalizations, outpatient care visits and the high cost of the pharmacological management of IPF [
8,
9]. Also, incidence numbers range between 3 and 9 cases per 100,000 individuals in Europe and, although still considered a rare disease, an increase in individuals diagnosed with IPF is reported [
10,
11]. Therefore, high-quality expertise is required to manage patients across the care trajectory. Consequently, it is crucial to establish care processes that maximize patient value and meet the patients’ individual needs. This is also highlighted in the European IPF charter, and the World Health Organization (WHO) advocates for integrated care [
12‐
15]. To strengthen the care processes, our research team has set up a project including a mixed-methods contextual analysis phase, aiming at assessing local practice patterns, identifying unmet care needs and discovering areas for improvement in the care trajectory of IPF patients at one of the three centres of excellence in Belgium [
16]. Stakeholder involvement is thereby of utmost importance to attain a shared view on needs and goals [
15]. The aim of this paper is to obtain a comprehensive understanding of the current IPF disease management program based on patients’ and healthcare professionals’ views.
The current disease management program and experiences of the care journey
From the moment of symptoms till diagnosis
All patients addressed at length difficulties to obtain an adequate recognition of their symptoms and associated diagnosis. Besides their local healthcare provider not recognizing the disease, some patients did not seek care as they never thought about the possibility of having a lung disease. They saw their symptoms of breathlessness as part of ageing. As a result, delayed referrals to centres of excellence as well as misdiagnoses and delayed diagnoses occurred. One patient even believed that the lack of knowledge about the disease has led local healthcare providers to make care decisions that posed a risk to his life (i.e. full anaesthesia without having received information about possible consequences). Once referred to a centre of excellence, patients felt grateful because of the available clinical expertise and the possibility to initiate anti-fibrotic treatment.
Diagnosis of IPF
HCPs considered the multidisciplinary team meetings as a highly valuable asset. More specifically, they mentioned the availability of an experienced team, the comprehensive assessment of the patient’s clinical data and the extensive discussion between team members as positive aspects. The opinions about involving a nurse specialized in ILD during the diagnostic meetings were mixed. Some participants did not see an added value of involving a nurse as they said the meeting aims to set a diagnosis, and the psychosocial needs of patients are not discussed at that time point. In contrast, other participants believed the nurse could give valuable insights regarding the further tailored management of the patient beyond medical or pharmacological treatment, as he/she potentially has a good understanding of the patient’s personal and social situation.
When asking patients how they experienced receiving their diagnosis, various stories and emotions were shared. Some patients indicated feelings of relief, but also mentioned difficulties to think about the future and stated words such as 'disbelief' and 'being frightened'. Only one patient felt well-aware of the severity of the disease as she had lost her sister due to IPF. All patients, except one, received their diagnosis from their local care provider, and some reported dissatisfaction regarding that moment, mainly due to a lack of information or because treatment options were not discussed.
Also, two participants indicated that the way the care provider communicates the diagnosis is crucial. More specifically, they shared negative experiences when the approach was focused on the prognosis, rather than on the pharmacological management. For instance, one patient had taken the 3–5 years survival expectation rather literally, resulting in life choices he had rather not taken. As an example, he communicated his diagnosis and survival expectation at work, which led him to be fired.
When we asked about online information sources on IPF, most patients mentioned not consulting them out of fear of misinformation and confrontation.
Box 1 Quotes regarding the diagnostic process
"[…] it would be nice to involve an advanced practice nurse specialized in ILD, but I actually think that it would not add any value to clinical practice. Because you do not have the time at that moment (multidisciplinary meeting to set a diagnosis) to discuss psychosocial issues, and also because often at that moment the psychosocial status (of a patient) is not yet clear.” HCP7 |
"[…] and that was something like, I was just told that my life would be shorter than I thought it would be, of lesser quality, and here I am on the street uhm that….is rather direct indeed […]." Patient3 |
File needed to request reimbursement of the anti-fibrotic drug
One participant reported a high administrative burden to assemble all necessary documents for the reimbursement file and this high workload was confirmed by other HCPs. In Belgium, a national platform for patients’ medical files is not yet in place, making it difficult to get access to all tests and their results. Also, the time needed to prepare the file and to wait for approval resulted in a delay in treatment initiation, which was perceived as unfavourably for patients.
All patients reported having received information regarding their treatment from a nurse at UZ Leuven and were satisfied with the information provided as it was given in a clear and understandable way. Furthermore, patients and their caregivers found the session interesting and were satisfied with the amount and depth of the information. For those attending a group session, patients were not bothered by the fact that other patients and their caregivers were present. One couple was interested in knowing how their peers were doing. Only, one participant and his caregiver reported the session to be overwhelming as they reported difficulties accepting the diagnosis and its fatal prognosis.
In the interviews with HCPs, HCPs believed the group session to be highly valuable as it allows for a structured information provision to patients.
IPF follow-up care after treatment initiation
Patients had adequate knowledge regarding the organisation and content of the follow-up care and were mostly satisfied with how care was organised and how HCPs communicate with them. Some patients wished to be more involved in their care while others preferred that HCPs instructed them on what to do. Information sharing was mentioned as an important aspect of a consultation. Indeed, several patients reported being dissatisfied when results of lung function tests were not shared as patients consider those results as a reference for disease progression. Getting reassurance that the disease seems stable was highly valued, although patients acknowledged the fact that HCPs are not able to provide accurate information on their prognosis. Although some patients reported a lack of information-sharing, they believed the HCPs would notify them if results were bad.
When addressing the topic of patient involvement in the interviews with HCPs, most of them questioned the instructive approach and wished patients to be more actively involved in their care.
Also, some HCPs reported a need for the care to be more pro-active as they would like to react before certain issues occur.
Several patients find it bothering to see a different HCP every outpatient consultation. Some said it impacts the establishment of a relationship of trust and reduces individual contact with the care provider. Therefore, all patients and caregivers were grateful to have a nurse specialized in ILD as the point of contact for questions and issues. Only one patient reported a need for the IPF team to be more reachable. Some HCPs argued the delay in time that patients contact the nurse specialized in ILD, hereby questioning whether patients know who and when to contact. HCPs also mentioned difficulties to establish a bond of trust with the patient since they don’t see the same patients every time. In addition to the difficulties gaining patients’ trust, HCPs also believed this way of working resulted in barriers for long-term follow-up of patient-tailored conversations and could cause misunderstandings as a result.
Box 2 Quotes regarding IPF follow-up care after treatment initiation
"[…] I am not involved in that, they do… they do their job and I am the direct object. Do I have to tell them what to do? Well, it doesn't work like that, does it?” Patient6 |
“That (the consultation) is usually one-way traffic, a doctor sits there saying everything, the patient assimilates […].” HCP8 |
"[…] each time, I end up in front of a stranger, they might read my file, but besides that, there is no personal uhm contact". Patient9 |
“Patients attend the consultation, always see someone else, are not always given an equal opportunity to speak to a nurse and that, of course, frequently causes problems. Hence patients with a more difficult situation or those with more side effects, sometimes slip through the net. We don't screen for that. The next time they will see another assistant who will make notes, but yes then it does get a bit lost". HCP1 |
Pharmacological treatment
All interviewed patients were on anti-fibrotic treatment and all suffered from side effects, such as gastrointestinal issues and phototoxicity, which resulted in a decrease in quality of life for some patients. More specifically, two patients said they did not feel free or felt dependent due to the occurrence of diarrhoea and both admitted they therefore intentionally skipped a dose of their anti-fibrotic drug when going out. Another barrier challenging therapy adherence were disruptions or changes in patients’ routine. Also, some patients mentioned difficulties with the fact that they do not feel the impact of the treatment. Nonetheless, all patients stated to be persistent on their drug regimen. Most patients were well-informed regarding the management of their side effects, however, some patients felt unsure about how to implement some of these pieces of advice, for instance about the intake of anti-diarrhoea medication. Furthermore, some patients decided to implement a change in their diet to diminish gastrointestinal issues and diet advice was also provided by the nurse when needed.
Box 3 Quotes regarding the pharmacological treatment
“yes, I lost control over myself and it (diarrhoea) came up and I had to go to the bathroom” Patient7 “yes, during a whole time, we didn't dare to go anywhere, and if we had to be somewhere, for example a funeral, then we wouldn’t take any (anti-fibrotic drug). Yes, that was, that was not bearable” Informal caregiver7 “if we had to be somewhere the next day, then I would not take them (anti-fibrotic drug) in the evening and then it was solved” Patient7 |
"[…] No, sometimes you forget it (to take in Nintedanib) and most of the time you forget it when you have to do something unusual. You have to go away or uhm we go away for a couple of days (laughs) then I think I can take them with me, but then I think oh I forgot. That is normal.” Patient5 |
Self-management and supportive care
HCPs appeared to have no or limited knowledge regarding existing self-management strategies or interventions. They mentioned a lack of education regarding self-management as a potential reason and some believed that self-management interventions in a mostly elderly population are challenging. Some HCPs reported that advice is given to patients (for instance on physical activity), but limited consistent changes are seen in patients’ behaviour. Regarding psychosocial topics, HCPs indicated to poorly address these issues and a wish for a psychologist or social worker to be included in the ILD team was mentioned, although they acknowledged the lack of resources.
During patient interviews, all patients mentioned trying to be as physically active as possible and one patient wished for more tailored advice of the healthcare team. Another patient reported high satisfaction with the pulmonary rehabilitation program at a local general hospital, although the program he was involved in was not designed for IPF patients specifically. One patient reported difficulties managing his coexisting medical conditions and treatments, hereby requesting care coordination.
Box 4 Quotes regarding self-management and supportive care
“I think that we are still too much saying what patients need to do, hence curtailing self-management a bit, and I am now trying more often to ask, ‘what are you suggesting’, ‘how are you going to tackle that’, uhm ‘do you have ideas yourself’. And if people come up with an idea, they are going to implement it more easily and I think we don’t, I can’t say concretely that we have a plan for self-management uhm.” HCP9 |
"[…] well, it would indeed be better to approach it (care) in an interdisciplinary way, because now we focus too much on those pills (anti-fibrotic drug).” HCP3 |
Research in UZ Leuven
Overall, patients reported a wish to be involved in research to help others. Most of the interviewed patients did not recall why they are asked to fill out patient-reported outcome measures (PROMs), but no issue filling them out was mentioned. However, some patients believed they would not fill out the PROMs once they feel sicker, mainly to avoid being confronted with their worsening disease status. One patient even stated she expects she would not answer psychosocial questions truthfully. Some patients indicated that a lot of questions refer to the same topic, were not stated clearly or did not apply to IPF. When asked whether patients would like to have feedback on the results of the PROMs, some, but not all replied positively.
During HCP interviews, the question of whether the results of the PROMs should be integrated into the routine care of patients resulted in diverse views. Some HCPs stated the loss of one-to-one contact between patient and care professional as a barrier. Also, some HCPs questioned the impact filling in PROMs has on the patient. More specifically, some of the HCPs believed psychological questions could harm patients. Another mentioned barrier is the processing and integration of all data to receive results in time. Furthermore, the clinical relevance and usability of using PROMs in routine care were questioned. On the other hand, opportunities to integrate PROMs in routine care were also mentioned, for instance the use of PROMs as a method to screen for patients’ needs.
Box 5 Quotes regarding research in UZ Leuven
“I think there are possibilities…. uhm… to lower the threshold for patients to report things, or to do things, or they could keep track of things more easily, for instance ‘I have not taken a pill at that moment’, or that they can indicate ‘I had that problem that day’, uhm that it would be easier to keep track of things, but I personally, I prefer personal contact.” HCP3 |
eHealth
Regarding eHealth, currently, no tools are used to manage patients at distance and various opinions were given by HCPs when asked whether they would see options in using eHealth in routine care. For some HCPs, it is not clear what the possibilities would be for using eHealth in the care management of IPF patients. Also, several HCPs believed eHealth could result in a loss of one-to-one contact between patient and care provider. Others questioned the possibility to use eHealth in a mostly elderly population. Another reported barrier was the need for resources to be able to adequately follow-up the eHealth tool and associated patients’ responses and needs.
On the other hand, some possibilities for eHealth options were also mentioned. For instance, one HCP believed that it could give structure and focus on the patient’s management and that it could lead to targeted individual patient care. eConsultations were also mentioned as an opportunity, although the fact that eConsultations are not reimbursed nor technically feasible in Belgium at the time of the interviews was highlighted.
Patients mostly were not in favour of using eHealth, mainly to avoid disease confrontation.
Box 6 Quotes regarding the use of eHealth
“So I find telemonitoring particularly interesting, but I think that this can only be done in a responsible manner if you also have budget to allocate people who can monitor it and who can intervene very quickly if something goes wrong, because otherwise I think it is pointless and even dangerous.” HCP6 |
Link with local healthcare providers and the community
All patients mentioned being satisfied with the communication and involvement of their general practitioner (GP) regarding their care. They indicated that their GP acquired knowledge on their disease to be able to better support them, as IPF was an unknown disease for them. HCPs considered collaboration and coordination with external parties necessary and positively viewed the existing collaboration and communication between UZ Leuven and the satellite centres.
When asking patients about their experience with the patient advocacy group, only one patient mentioned to be actively involved and was highly satisfied with the information sessions it organizes. Another patient did not wish to be involved mainly due to the confrontation with the disease and one patient decided not to become a member due to travel distances. When asking to HCPs how they perceive the patient advocacy group, they provided positive feedback as they see their activities as an important way for patients to access easy-to-understand information. One HCP mentioned the possibility to work closely with the patient advocacy group to gain deeper insights into patients’ unmet needs.
Advanced care planning and palliative care
HCPs mentioned addressing advanced care planning rather intuitively or at certain ‘key’ moments only, such as during hospitalization or when they noted a worsening disease progression. They believed advanced care planning was often addressed too late and they raised questions regarding who, when and how to address advanced care planning. Examples of barriers to initiating conversations included a lack of time and inadequate ways to follow-up on conversations.
Some HCPs were also reluctant to talk about advanced care planning as they did not want to take away patients’ hopes and induce anxiety. It appeared that nurses are more pro-active in initiating such conversations. Furthermore, HCPs mentioned having only limited knowledge regarding possible local organisations to support patients and therefore referred the patient to their general practitioner. During the patient interviews, one patient said that all arrangements are made with the GP, whilst others did not want to address this topic. One patient even reported a negative experience with the IPF/ILD team on having this discussion as he felt he has not accepted his disease yet and is therefore not ready to talk about end-of-life.
Box 7 Quotes regarding advanced care planning and palliative care
“[..] often at a certain moment you are confronted with this (advanced care planning or palliative care) anyway, then you have a patient who suddenly feels very ill, but then it is actually too late, then it is not advanced care planning, but rather late care planning.” HCP7 |
“For some people, that (the current support regarding palliative care) is enough, but others wonder, ‘where were you in the whole process’?” HCP1 |
Living and coping with IPF according to patients and their caregivers
All patients and caregivers reported a change in lifestyle and tried to adapt as much as possible to the disease. Most patients reported trying to stay as independent as possible, and others reported an increasing dependency from their caregivers. Also, they indicated a continuous confrontation with their disease and feelings of powerlessness. One participant wished it all to stop and reported feelings of social isolation. Others felt a less severe impact on their daily life as they did not have significant disease symptoms or side effects. One patient was on oxygen treatment and reported anxiety of running out of oxygen. Therefore, the couple decided not to go on holidays anymore and to go out only for short walks. Some patients reported a lack of understanding from their environment as the disease is not well-known and patients do not look sick. One patient even reported feelings of shame and a negative social perception when walking in the streets. When talking about family and friends, patients shared several stories. Some patients actively involved their family and friends in their disease, whilst others choose not to. Some patients indicated that they did not want to be a burden to their friends or children as everybody has his or her life problems.
The interviewed patients mentioned several coping strategies. Some patients said they tried to stay positive as complaining is not helping anyone. Other patients tried to avoid confrontation with being ill as much as possible and one patient even found the interview confrontational. One patient stated that acceptance is necessary, as there is no other option, whilst one couple reported having a hard time coping with the impact of the disease.
Box 8 Quotes regarding living and coping with IPF
“I want to be confronted with it (disease) as little as possible. I know what I have, I know what is heading towards me and that's it.” Patient2 |
“It is difficult, it is very difficult, difficult to accept. Yes, I get a lot of support from my wife, from her children, from everyone who says, ‘come on, let’s move on’, but I can’t go on anymore, it’s over, I'm finished. Yes, we talk about going on a trip again this year, but where to? To nowhere (laughs).”Patient 9 |
[…] it’s getting difficult to handle. Yes, and then you say sometimes, ‘let it be over soon, I want it to be over, then other people won’t be bothered anymore, then I won't bother anybody anymore. (sir is emotional) yes we will continue moving one […].” Patient 9 |
“You can always start whining and complaining, but you don’t help anybody with that, you don’t help yourself and you don’t help your loved ones, so I have no reason to complain.” Patient 8 |
“[…] yes, everyone you know actually lives with it too.” Patient 6 |
[…] with their daily worries and, uhm, yes, and you shouldn’t be an additional burden to them (family and friends) in any way.” Informal Caregiver5 |
Strengths, weaknesses, opportunities and threats according to HCPs and patients
Overall, HCPs mentioned both the extensive expertise of the team members as well as the availability of nurses specialized in ILD as the most important strengths of the care program. Also, the access to clinical trials and the comprehensive assessment of the patient to make an adequate diagnosis were mentioned as strengths. In contrast, the most important threat as mentioned by some HCPs is the increase of patients diagnosed with IPF, as resources remain scarce. All patients need lifelong follow-up care at the centre of expertise, resulting in a high workload and administrative burden. Various topics were already mentioned in previous sections, but we reflect here further on the opportunities that were discussed during the interviews.
An opportunity proposed by most of the HCPs is the expansion of the role of the nurse specialized in ILD. More specifically, some HCPs argued that a nurse-led consultation in addition to the routine consultation with the physician would be of interest so that topics such as psychosocial needs, therapy adherence or self-management strategies could be discussed. Also, some HCPs wanted to be proactive and able to track improvements and collaborate with patients on care needs. Most patients also expressed a need to be followed by the same HCP over time.
Furthermore, HCPs mentioned the need to involve a psychologist, a social worker and a physiotherapist in the ILD team. Yet, HCPs believe insufficient resources hamper the expansion of the team. Also, an HCP believed a case manager to be valuable to prepare the patients’ files and assure consistent follow-up and coordination of care decisions.
Regarding eHealth, some HCPs suggested creating a platform to follow-up certain parameters or to share data with patients or external parties. Examples of parameters that could be followed-up are the prevalence of side-effects, social issues and general well-being of patients. The need for a user-friendly national platform was also suggested to improve the process of the reimbursement application. Another suggestion involved the screening of patients before consultations, resulting in triage of patients to align individual needs with tailored follow-up care. For instance, screening for psychological or self-management needs was suggested. Furthermore, HCPs reported a need to be able to address advanced care planning on time and all participants saw room for improvement.
Travel distances to get to the centre of excellence seem to be an important complaint amongst patients. One couple even shared their concerns about not being able to get to the hospital anymore if the caregiver is no longer able to drive. During the interviews with HCPs, some also questioned the accessibility of care for patients due to travel distances, hereby preventing initiation and persistence of pharmacological treatment. Also, some HCPs mentioned the possibility to use telecommunication/virtual tools for the multidisciplinary team meetings at the phase of diagnosing. This would also allow other care professionals to join the meeting, should this be needed. Besides advantages, HCPs, however, were also aware of potential disadvantages of such virtual meetings, such as the need for equipment and the potential impact on the efficiency of the discussions between team members.
Although clinical guidelines are available to manage patients, one HCP suggested discussing insights gained at conferences to further improve routine care. Furthermore, some HCPs mentioned they actively looked for courses and believed training on motivational interviewing, communication during discussions on advanced care planning and oxygen therapy would be valuable.
All HCPs and patients acknowledged the lack of awareness regarding the disease and its management in the community. They suggested informing their partners and working towards a stronger collaboration with professionals working in primary or secondary care. Increased involvement of the general practitioner into patient follow-up care was stated as well, although some HCPs mentioned a need to centralize IPF care as education of general practitioners was seen as unfeasible. Also, further strengthening the collaboration with satellite centres was believed by some HCPs to hold great potential.
Box 9 Quotes regarding the strengths, weaknesses, opportunities and threats
"[…] I find that a failure of the system if you can’t make it available to such an extent that they (patients) have to stop because they can’t access it (care) anymore.” HCP5 |
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