The ILD patient journey and what to expect

ILD

Navigating the journey of interstitial lung disease (ILD) can be overwhelming, filled with uncertainties, misdiagnoses, and emotional challenges. From the first signs of symptoms to receiving an accurate diagnosis and starting treatment, ILD patients often face a long and complex path. Understanding what to expect at each stage can help patients and their families make informed decisions, manage their health effectively, and find support throughout the process. In this blog, we’ll walk through the ILD patient journey, highlighting key milestones, challenges, and the available treatment options.

Typical Patient Journey 

The ILD patient journey usually begins with the patient visiting their primary care physician (PCP). Many ILD patients will need to visit their PCP multiple times and will receive numerous incorrect diagnoses and medications before ILD is suspected and they are referred. One notable study found that 55% of all ILD patients received at least one wrong diagnosis and 38% had two wrong diagnoses before they were correctly diagnosed [1].

The high levels of misdiagnosis in ILD can be ascribed to the highly non- specific symptoms of the condition which are often mistaken for other respiratory conditions e.g. asthma, pneumonia or bronchitis [1]. Other factors that can contribute to the ILD misdiagnosis include a lack of PCP knowledge of ILD symptoms, the poor sensitivity of available diagnostic tests in particular x-rays and the presence of comorbidities e.g. coronary artery disease or GERD that can potentially mask some ILD symptoms. When the patient shows no improvement in their symptoms, they are often referred to see a specialist. It is estimated that around 30% of ILD patients may visit their PCP at least four separate times before being referred. Following their referral, many patients will undergo more invasive diagnostic procedures e.g. lung biopsy to confirm their diagnosis. One study found that 61% of ILD patients undergo bronchoscopy or surgical lung biopsy [2]. 45% of ILD patients undergo surgical lung biopsy [2] and 21% of ILD patients may undergo both procedures [2].

These procedures can be stressful for patients and carry significant risks. The time to an accurate diagnosis of ILD may take as long as 4 to 5 years, imposing a significant emotional toll on sufferers as well as impacting their quality of life [3]. Delays in the diagnosis of ILD also represent a missed opportunity to intervene early and slow disease progression and put individuals at an increased risk of dying from ILD related complications [4]. The common presentation, repeated review by different doctors, and failure to recognise progression are hallmarks of a disease requiring early objective measurements which can be simply delivered.

Patient Diagnosis 

A confirmed diagnosis of ILD is important in order to start the appropriate pharmacological treatment (Table 1). Following the initiation of pharmacological treatment most ILD patients will experience some relief from ILD symptoms and improvements in their overall quality of life. The side effects associated with some of the medications can however be dose-limiting for some patients limiting their efficacy. Those common non-pharmacological options for ILD include pulmonary rehabilitation and oxygen therapy. One study found that over 40% of patients who attended a specialist ILD clinic were referred for pulmonary rehabilitation [5].

Often patients with ILD experience improvements in exercise capacity, quality of life and lung disease knowledge after undertaking pulmonary rehabilitation [5]. Another common treatment for ILD, oxygen therapy may also be prescribed in as many as 40% of all ILD patients [5]. ILD patients often report experiencing improvements in quality of life and exercise capacity following the initiation of oxygen therapy [5]. The careful monitoring of the disease parameters following initiation of treatment interventions may enable more optimal use.

As ILD progresses patients begin to experience declines in lung function, worsening of disease symptoms and a lower quality of life [6]. ILD patients may also struggle to perform normal daily activities and begin limiting social interactions which can lead to feelings of isolation and depression. In fact one recent study estimated the prevalence of depression in ILD patients to be as high as 14-49% [7]. As ILD progresses it also becomes important that ILD patients are closely monitored through regular follow-up appointments at a specialist clinic. These follow up appointments typically involve the patient undergoing repeat imaging and PFTs [8]. However, many patients with advanced ILD report challenges in being able to both travel to and attend clinic appointments and carrying out PFTs e.g. spirometry [9].

As part of the disease course, many ILD patients also experience a sudden worsening of symptoms termed acute exacerbations. ILD exacerbations are characterised by the rapid onset of breathlessness, coughing, fatigue and sometimes a fever. Acute exacerbations may occur as a result of disease progression or may be triggered by infections, GERD, air pollution, anaesthesia or certain medications. An estimated 4% of IPF patients may experience an acute exacerbation each year [10]. Often patients who experience exacerbations are admitted to hospital where they are given antibiotics and supportive care measures. Approximately half of patients who suffer an acute exacerbation requiring hospitalisation will not survive to discharge [10]. The closer and real-time monitoring of key indicators may allow smart devices to predict the onset of these exacerbations and to rationalise these to an individuals pathophysiology.

As part of their journey some ILD patients may undergo lung transplantation of either one or both lungs. Lung transplants can be life saving for many patients with ILD with reported median post-transplant survivals of 6.7 years [11]. However, the procedure itself is not without risk with an estimated 20% of patients not surviving one year post transplant [12]. Notably the long-term prognosis for ILD patients who do not undergo lung transplantation is significantly poorer with a median survival of 3-4 years post-diagnosis [13]. The end of the journey for many ILD patients is characterised by a significant escalation in disease symptoms and declines in functional capacity. As a consequence many ILD patients will spend the last few months of their life in a hospital or hospice facility [14].

While the ILD journey is often difficult, early diagnosis, proper treatment, and ongoing care can improve quality of life and slow disease progression. By staying informed and proactive, patients can navigate their condition with greater confidence and support. Whether newly diagnosed or caring for a loved one with ILD, there are resources, medical advancements, and a community ready to help. Stay engaged, ask questions, and advocate for the best possible care.

References:

[1] Cosgrove GP, Bianchi P, Danese S, et al. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med. 2018;18:9.

[2] Lederer DJ, Bianchi P, Loboda J, et al. AB017. Interstitial lung disease patient diagnostic journey (intensity). J Thorac Dis. 2016;8:AB017–AB017.

[3] Lamas DJ, Kawut SM, Bagiella E, et al. Delayed Access and Survival in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2011;184:842–847.

[4] Lamas DJ, Kawut SM, Bagiella E, et al. Delayed Access and Survival in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2011;184:842–847.

[5] Hoffman M, Mellerick C, Symons K, et al. Pulmonary rehabilitation for interstitial lung disease: Referral and patient experiences. Chron Respir Dis. 2021;18:147997312110460.

[6] Maqhuzu PN, Szentes BL, Kreuter M, et al. Determinants of health-related quality of life decline in interstitial lung disease. Health Qual Life Outcomes. 2020;18:334.

[7] Yohannes AM. Depression and anxiety in patients with interstitial lung disease. Expert Rev Respir Med. 2020;14:859–862.

[8] National Institute for Health and Care Excellence (NICE). Idiopathic pulmonary fibrosis in adults: diagnosis and management Clinical guideline [Internet]. 2013. Available from: www.nice.org.uk/guidance/cg163.

[9] Kumar D. Assessment and follow-up of interstitial lung disease. Indian J Rheumatol. 2021;16:69.

[10] Collard HR, Ryerson CJ, Corte TJ, et al. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. An International Working Group Report. Am J Respir Crit Care Med. 2016;194:265–275.

[11] Chambers DC, Cherikh WS, Harhay MO, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult lung and heart–lung transplantation Report—2019; Focus theme: Donor and recipient size match. The Journal of Heart and Lung Transplantation. 2019;38:1042–1055.

[12] NHS Blood and Transplant. Benefits and Risks of Lung Transplants [Internet]. [cited 2024 May 12]. Available from: https://www.nhsbt.nhs.uk/organ-transplantation/lung/benefits-and-risks-of-a-lung-transplant/.

[13] Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.

[13] Cross SH, Ely EW, Kavalieratos D, et al. Place of Death for Individuals With Chronic Lung Disease. Chest. 2020;158:670–680.

[14] National Institute for Health and Care Excellence (NICE). Idiopathic pulmonary fibrosis in adults: diagnosis and management Clinical guideline [Internet]. 2013. Available from: www.nice.org.uk/guidance/cg163.



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