Living with IPF: Patient perspectives
Interstitial lung diseases (ILDs) include over 200 fibroinflammatory diseases that affect the alveolar interstitium of the lung1,2. The most common of these is idiopathic pulmonary fibrosis (IPF) which accounts for between 17–37% of ILDs1. There is no cure for IPF, which ultimately leads to severe respiratory failure and has a life expectancy of just 3–5 years3. Understanding the experiences of patients with IPF and other ILDs can help healthcare professionals improve their quality of life4.
A life-changing diagnosis
On average, the process of diagnosing IPF takes more than one year from when the patient first presents with symptoms1. This process in itself can be distressing and receiving a diagnosis of IPF is a life-changing and often traumatic experience for patients1,5,6. Appropriate and sensitive delivery of an IPF diagnosis is therefore paramount.
In one focus group study, patients called for a balance of honesty and hope from physicians and highlighted the importance of conveying the following messages at the point of diagnosis5:
- What IPF is
- That there is no cure for IPF
- That IPF is chronic and progressive
- That there are effective treatments to slow disease progression and manage symptoms
The importance of giving patients ample time to address their questions and concerns with their physician at diagnosis is also very apparent1,5. It has therefore been suggested that appointment times should be extended to reflect this need and ensure that patients receive adequate information and support during this consultation. In one study, it was found that 73% of patients and 60% of healthcare professionals felt that the time allocated for their appointment was not sufficient to discuss the diagnosis and treatment options fully1.
In the same study, 60% of patients reported a positive experience when discussing their diagnosis with their specialist physician, but one-fifth stated that they received no information about their disease at that time1.
Living with a terminal illness
Unsurprisingly, the process of coming to terms with a terminal diagnosis is not easy, and the impact of IPF on the lives of patients is huge6. Our understanding of the challenges faced by patients with IPF and other ILDs has improved significantly over the last decade, highlighting a need to establish strategies to improve quality of life for these individuals7.
For patients with IPF and other ILDs, cough and activity-limiting dyspnoea contribute to a substantial reduction in quality of life, with patients frequently reporting sleep disturbances as a result of persistent coughing6. Cough may also impact patients’ engagement in social activities, some of which they may choose to avoid completely, to the detriment of both their physical and emotional well-being6. Similarly, fatigue may contribute significantly to social isolation and a decline in physical and mental health6. Anxiety and depression are extremely common in patients with progressive fibrosing-ILD and many experience feelings of grief or anger in response to their diagnosis6.
Further, patients often feel frustrated by their physical limitations as their disease progresses, particularly when they reach a stage at which performing everyday tasks becomes challenging and they are faced with the loss of their independence4. As they become increasingly dependent upon others for care, patients often feel incompetent. This, combined with the reality of their diagnosis with an unpredictable disease that will ultimately prove fatal, can have a huge psychological impact7.
A lack of understanding of IPF among the general population may compound feelings of social isolation. Patients may feel stigmatised and concerned about how others may perceive them, which may lead to feelings of depression in some4. Similarly, patients may feel that, as their condition progresses and they lose the ability to take part in family activities, they grow increasingly distant from their loved ones6.
A sense of vulnerability has also been reported by patients. Importantly this can be attributed, at least in part, to a lack of knowledge about their condition4.
Knowledge is power
It is important to remember that patients and their family members are often unfamiliar with IPF at the time of diagnosis and that for many, their specialist physician and centre represent their main source of information about their disease4,5. Understanding their condition can help empower patients and improve their ability to cope with the reality of their diagnosis6.
Of note, patients have expressed a need to understand the terminology associated with their condition in terms of actual outcomes. For instance in one study, patients felt it was important that physicians took the time to explain what a diagnosis of mild, moderate, and severe disease really means in terms of survival5.
In recent years, a need for access to good quality information regarding IPF has emerged. When asked, many patients have expressed frustration at the lack of professional direction when trying to source appropriate material to help them better understand their condition4. In one study, patients suggested that a solution could be to involve patients in the development of educational materials targeted at their peers4. Alternative suggestions included allowing patients to educate healthcare professionals, giving them better insight into the lived experiences of patients4.
Feelings of confusion are not unique to patients with IPF. Indeed, patients with connective tissue disease related-ILD or cohorts of patients with rheumatoid arthritis- or systemic sclerosis-related (SSc)-ILD have reported a similar sense of confusion regarding the nature of their diagnosis6. For example, research into the experiences of patients with SSc-ILD revealed that some patients attribute their disease to exposure to vaccination, emotional or psychological shock, pregnancy or even spirituality8. This highlights a need for improved communication between patient and physician8.
It is important to bear in mind that patient information needs are heterogenous and may evolve as their disease progresses5. Therefore, provision of information should ideally be tailored to suit the individual. It is also worth considering that information needs may differ between patient and carer5.
At the level of GPs and physicians in community hospitals, promoting better understanding of IPF and other ILDs could facilitate earlier diagnosis and referral1. This could, in turn, help avoid the negative impact on quality of life that is associated with a lengthy diagnostic process9.
The importance of shared-decision making is increasingly recognised. This requires health care professionals to communicate with their patients and ensure that they have a good understanding of the treatment options available to them1.
In a survey of 1448 patients with IPF, 63.5% felt that they were not well informed about disease management10.
The availability of nitedanib and pirfenidone that slow the decline in lung function in patients with IPF, has sparked a renewed sense of hope in the ILD community11,12. However, although these drugs represent progress, a cure for fibrotic ILDs remains elusive7.
Nevertheless, some patients may believe that they are able to slow the progression of their disease or even reverse the damage through lifestyle alterations8. It is important that patients understand that this is not the case, as such misconceptions have the potential to impact adherence to treatment8.
Nonpharmacological treatments including lung transplantation, pulmonary rehabilitation and supplemental oxygen may also be available to help improve exercise tolerance and quality of life1. Lung transplantation offers the possibility of restored lung function, improved quality of life and extended patient survival13. Though effective, the eligibility criteria for this therapeutic option are only met by certain patients7. In one study, investigating patient perspectives in IPF, it was noted that patients want to understand the therapeutic options available to them, including why they might not be eligible for lung transplant5.
Qualitative studies have revealed that in addition to information regarding specific treatment options, there is also a need for improved access to specialist centres and nurses, as well as resources explaining palliative care options to patients14.
Note on COVID-19
An international multicentre study investigating the impact of COVID-19 on patients with ILD found that these patients, particularly those with poor lung function and obesity, are at increased risk of death from the virus2.
Many patients will have been made aware of their elevated risk status and may look to their physician for guidance. It is therefore important to communicate with patients about ways to minimise risk and direct them to resources that might help address their concerns and alleviate their anxiety15. In patients who have been advised to shield, additional efforts should be made to preserve mental well-being via provision of appropriate support.
It was also reported that anti-fibrotic, or immunosuppressive drugs did not appear to affect the outcomes among patients with ILD2. This suggests that it is not necessary to withhold these medications during the pandemic, though further research is required to confirm this.
- Moor CC, Wijsenbeek MS, Balestro E, Biondini D, Bondue B, Cottin V, et al. Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey. ERJ Open Res. 2019;5(4):00124–02019.
- Drake TM, Docherty AB, Harrison EM, Quint JK, Adamali H, Agnew S, et al. Outcome of hospitalisation for COVID-19 in patients with Interstitial Lung Disease: An international multicentre study. Iain Stewart.45:43.
- Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic pulmonary fibrosis: Evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824.
- Senanayake S, Harrison K, Lewis M, McNarry M, Hudson J. Patients’ experiences of coping with idiopathic pulmonary fibrosis and their recommendations for its clinical management. PLoS One. 2018;13(5). doi:10.1371/journal.pone.0197660.
- Masefield S, Cassidy N, Ross D, Powell P, Wells A. Communication difficulties reported by patients diagnosed with idiopathic pulmonary fibrosis and their carers: a European focus group study. ERJ Open Res. 2019;5(2):00055–02019.
- Swigris JJ, Brown KK, Abdulqawi R, Buch K, Dilling DF, Koschel D, et al. Patients’ perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. European Respiratory Review. 2018;27(150). doi:10.1183/16000617.0075-2018.
- Kreuter M, Bendstrup E, Russell AM, Bajwah S, Lindell K, Adir Y, et al. Palliative care in interstitial lung disease: living well. The Lancet Respiratory Medicine. 2017;5(12):968–980.
- Cheema TJ, Young M, Rabold E, Barbieri AN, Baldwin N, Steen VD. Patient and physician perspectives on systemic sclerosis–associated interstitial lung disease. Clin Med Insights Circ Respir Pulm Med. 2020;14. doi:10.1177/1179548420913281.
- Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: A cohort study. Am J Respir Crit Care Med. 2011;184(7):842–847.
- Collard HR, Tino G, Noble PW, Shreve MA, Michaels M, Carlson B, et al. Patient experiences with pulmonary fibrosis. Respir Med. 2007;101(6):1350–1354.
- Richeldi L, Du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis. N Engl J Med. 2014;22:2071–82.
- King, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis. N Engl J Med. 2014;22:2083–92.
- Laporta Hernandez R, Aguilar Perez M, Lázaro Carrasco M, Ussetti Gil P. Lung transplantation in idiopathic pulmonary fibrosis. Med Sci. 2018;6(3):68.
- Moor CC, Heukels P, Kool M, Wijsenbeek MS. Integrating patient perspectives into personalized medicine in idiopathic pulmonary fibrosis. Frontiers in Medicine. 2017;4(DEC):226.
- NICE. COVID-19 rapid guideline: interstitial lung disease. 2020 https://www.nice.org.uk/guidance/ng177. Accessed 12 October 2020.
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