Idiopathic Pulmonary Fibrosis (IPF)


Idiopathic pulmonary fibrosis (IPF) is a rare, progressive, life-shortening interstitial lung disease. The median survival of patients with IPF is 3 to 5 years after diagnosis.1 The main symptoms of IPF, chronic cough and exertional dyspnea, are nonspecific, and a more common disorder, such as chronic obstructive pulmonary disease, gastroesophageal reflux disease, or heart disease, may initially be diagnosed.1 Because the differential diagnosis frequently requires a multidisciplinary discussion and extensive testing to rule out other diseases,1 patients are often left feeling frustrated, anxious, and uncertain before their diagnosis is determined.2 Accurate and timely education and support for patients are critical to helping them deal with the implications of this disease.1

Patient Educational Needs

Surveys of patients with IPF have found that information about disease progression and what to expect is at the top of the list of their educational needs.2,3 Patients also indicated that they want to be able to do their own research and use preferred websites as their primary means of acquiring disease-related information.2,3 They expressed concerns about where to find trustworthy information,2 which is important because recent studies have found that most sources of information on the Internet, including websites and YouTube videos, contain information that is inaccurate, incomplete, and outdated.4,5 

The importance that patients placed on information about disease progression is most likely due to the highly variable natural history of IPF; some experience a slow decline, others have a rapid decline, and still others experience periods of relative stability with intermittent acute exacerbations.1,3 Patients also wanted information about how to live with and manage the disease; topics mentioned ranged from new developments in medications and disease research to more day-to-day matters, such as how to travel with supplemental oxygen.3 

Importance of Patient Education

As previously mentioned, many of the resources available to patients online contain outdated and inaccurate information, and it is critical to provide them with reliable information.4,5 Education is important to help patients understand and deal with the consequences of living with IPF.1 Information should be provided at the time of diagnosis, but it may be helpful to adjust how often information is presented according to the pace of disease progression and the changing needs of the patient.6 

Patients should be informed that IPF is progressive and that lung function may decline rapidly, such as during acute exacerbations, even if it has previously been stable.6 Because the progression of IPF can be rapid in some cases, it is critical that information about disease-slowing therapies, such as pirfenidone and nintedanib, and their advantages and disadvantages be provided early and in an understandable form so that patients can make informed decisions.6 It is important that patients understand that these treatments will slow disease progression only modestly and most likely will have no beneficial effect of relieving symptoms.2 Patients who may be candidates for a lung transplant should be referred to a transplant unit early because the mortality rates among persons on waiting lists can be high.7

Information about the effects of IPF on daily life and about how to manage symptoms can also be important so that patients will be better equipped to deal with future challenges as the disease progresses. For example, the need for supplemental oxygen can create logistical issues when patients leave the house, but more subtle effects also come into play, such as the psychological consequences of having their disease made apparent to others and feeling like a burden on family and society.8

Patients should receive information about support groups and palliative care soon after their diagnosis to improve quality of life.6 Early referral to palliative care can help to optimize symptom management with such interventions as pulmonary rehabilitation, supplemental oxygen, and pharmacologic symptom relief.9 The early implementation of palliative care can also help foster patient-provider relationships by facilitating discussions about advanced care and end-of-life planning based on the patient’s wishes.9 

Patient Education Resources

Several nonprofit organizations work to improve patient education. The largest is the Pulmonary Fibrosis Foundation (PFF). Although its activities are not limited to persons with IPF, the PFF offers patient support programs, advocates for legislation on issues that affect patients with IPF, funds research on IPF treatments, and offers educational resources for patients, caregivers, family members, and healthcare providers.10

To help patients find additional resources, the PFF also offers free access to a PFF Help Center through email or phone calls. Some of these resources include information about the locations of PFF Care Center hospitals where physicians specialize in the care of patients with pulmonary fibrosis, about how to start or join a support group, and about clinical trials in which patients may wish to enroll. The PFF also manages the PFF Registry, whose purpose is to gather information from patients, caregivers, and family members that may be useful for research on the diagnosis and treatment of IPF.10

To help raise awareness of pulmonary fibrosis, the PFF hosts a national PFF Walk Day each year in September. The PFF also coordinates awareness events every September as part of Pulmonary Fibrosis Awareness Month.10

References

  1. Quinn C, Wisse A, Manns ST. Clinical course and management of idiopathic pulmonary fibrosis. Multidiscip Respir Med. 2019;14(1):35. doi:10.1186/s40248-019-0197-0
  2. Ramadurai D, Corder S, Churney T, et al. Understanding the informational needs of patients with IPF and their caregivers: ‘You get diagnosed, and you ask this question right away, what does this mean?’ BMJ Open Qual. 2018;7(1):e000207. doi:10.1136/bmj oq-2017-000207
  3. Ramadurai D, Corder S, Churney T, et al. Idiopathic pulmonary fibrosis: educational needs of health-care providers, patients, and caregivers. Chron Respir Dis. 2019;16:1479973119858961. doi:10.1177/1479973119858961
  4. Fisher JH, O’Connor D, Flexman AM, Shapera S, Ryerson CJ. Accuracy and reliability of Internet resources for information on idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;194(2):218-225. doi:10.1164/rccm.201512-2393OC
  5. Lashari BH, Chan V, Shoukat U, et al. YouTube as a source of patient education in idiopathic pulmonary fibrosis: a media content analysis. J Community Hosp Intern Med Perspect. 2019;9(2):98-102. doi:10:1080/20009666.2019.1593779
  6. Wuyts WA, Wijsenbeek M, Bondue B, et al. Idiopathic pulmonary fibrosis: best practice in monitoring and managing a relentless fibrotic disease. Respiration. 2020;99(1):73-82. doi:10.1159/000504763
  7. Laporta Hernandez R, Aguilar Perez M, Lázaro Carrasco MT, Ussetti Gil P. Lung transplantation in idiopathic pulmonary fibrosis. Med Sci (Basel). 2018;6(3):68. doi:10.3390/medsci6030068
  8. Wuyts WA, Peccatori FA, Russell A-M. Patient-centred management in idiopathic pulmonary fibrosis: similar themes in three communication models. Eur Respir Rev. 2014;23(132):231-238. doi:10.1183/09059180.00001614
  9. Zou RH, Kass DJ, Gibson KF, Lindell KO. The role of palliative care in reducing symptoms and improving quality of life for patients with idiopathic pulmonary fibrosis: a review. Pulm Ther. 2020;6:35-46. doi:10.1007/s41030-019-00108-2
  10. Pulmonary Fibrosis Foundation. Pulmonaryfibrosis.org. Accessed June 26, 2021.

Reviewed by Harshi Dhingra, MD, on 7/1/2021.

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