There is a broad range of approaches available to support patients with ILDs from pulmonary rehabilitation to mindfulness.1–4
The needs of patients with progressive fibrosing ILD are similar to those with malignant disease.2
Patients with ILD need timely diagnosis, comprehensive information, early treatment access, and holistic care2
Holistic care aiming to optimize quality of life should include:2
Disease-stabilizing care with both pharmacologic and non-pharmacologic therapies, including treatment of comorbidities
Symptom-centered approaches around quality-of-life-debilitating symptoms, as well as depression, anxiety, fatigue and deconditioning
Patient- and caregiver-centered management, such as education and assistance for self-management
End-of-life strategies, such as advanced care planning
Holistic care should aim to optimize quality of life at any stage of ILD2†
Holistic care should be provided throughout the ILD course, tailored to the individual needs of the patients with ILDs.2,3
An early holistic therapeutic approach could help preserve lung function in patients with IPF.5
PULMONARY REHABILITATION CAN SIGNIFICANTLY IMPROVE FUNCTIONAL CAPACITY AND QUALITY OF LIFE IN PATIENTS WITH FIBROTIC ILDs:1
6.1-point improvement in SGRQ quality of life score (95% CI 3.7–8.6, p<0.0005); 51% of patients achieved ≥5-point improvement in SGRQ
57.6 m improvement in 6MWD immediately after rehabilitation (95% CI 40.2–75.1 m, p<0.0005)
65% of patients achieved MCID improvement in dyspnea
52% of patients achieved MCID improvement in depression score
Pulmonary rehabilitation significantly improves quality of life, dyspnea and depression in patients with fibrotic ILDs1
Box plots for (A) quality of life, (B) dyspnoea, and (C) depression, comparing values pre-pulmonary rehabilitation to post pulmonary rehabilitation and 6-month follow-up.
* 6-month follow-up data refer to measurements recorded six months after initiation of pulmonary rehabilitation.
SUPPLEMENTAL OXYGEN USE DURING ROUTINE DAILY ACTIVITIES IMPROVES THE QUALITY OF LIFE OF PATIENTS WITH PROGRESSIVE FIBROSING ILD2
BREATHLESSNESS AND AMBULATORY OXYGEN
Patients with progressive fibrosing ILD with isolated exertional hypoxia reported that breathlessness was significantly reduced after 2 weeks of ambulatory oxygen vs. no oxygen treatment (p<0.0001)6
Number of patients reporting improved, same, or worse breathlessness after two weeks on ambulatory oxygen or no oxygen treatment.
QUALITY OF LIFE AND AMBULATORY OXYGEN
Ambulatory oxygen significantly improved total K-BILD score compared to no oxygen treatment in patients with progressive fibrosing ILD with isolated exertional hypoxia6
Mean difference in K-BILD scores between ambulatory oxygen and no treatment, adjusted for order of treatment. Ambulatory oxygen used during daily activities for 2 weeks was associated with significant improvement in total K-BILD scores vs. no oxygen treatment [55.5 (SD 13.8) on oxygen vs 51.8 (SD 13.6) without oxygen; p<0.0001].
* Higher scores reflect better quality of life, minimal clinically important difference estimates for K-BILD scores are 4 (range 3.7–4.2) for total score, 6 (5.6–6.5) for breathlessness and activities score, 5.4 (4.6–6.9) for psychological symptoms score, and 0.5 (SD 8.9) for chest symptoms score.
PATIENT AND PARTNER EMPOWERMENT PROGRAMME FOR IPF (PPEPP)
PPEPP is a short multidisciplinary empowerment programme that was co-developed with patients and multidisciplinary experts.7 PPEPP involves the IPF patient and their partner (spouse, partner, relative or friend) engaging with small groups that stimulate personal interaction, tailor discussion and balance participation of intervention for the patient
PPEPP, involving engagement with small groups, has been shown to improve quality of life for patients with IPF and their partners2
PPEPP has been shown to reduce stress and demonstrate a positive effect on the wellbeing of patients with IPF and their partners,7 indicating that potentially distressing information should be carefully tailored for patients and partners.
EARLY PALLIATIVE CARE CAN HELP MAINTAIN PHYSICAL AND EMOTIONAL WELLBEING IN PATIENTS WITH PROGRESSIVE FIBROSING ILD2
Almost half of the patients with progressive fibrosing ILD die in the hospital due to poor advance care planning.8 Consequently, it is valuable to offer advance care planning, including discussion of treatment limitations and preferences on dying, to all patients with progressive fibrosing ILD.2
Palliative care is sometimes incorrectly viewed as consisting solely of end-of-life care, which may explain the underuse of palliative care expertise for patients with fibrotic ILDs.2 Palliative care has a valuable role earlier in the ILD course – there is a rapidly growing consensus that palliative care in ILD should be initiated early and not delayed until ILD is advanced, which can help avoid the misconception that palliation is synonymous with imminent death.2 An ILD behavior-based management strategy might be helpful in identifying patients’ need for palliation due to the unpredictable course of most fibrotic ILDs.2
Palliative care involving pharmacologic management complemented with non-pharmacologic management has been shown to improve symptom-related quality of life.2 Early palliative care should be implemented in patients whose ILD has become progressive, despite optimal medical management, where overt distress is experienced, or symptoms are burdensome.2 Improved palliative care for patients with progressive fibrosing ILD is likely to improve symptom burden.8
WHAT ARE THE PALLIATIVE CARE NEEDS OF YOUR PATIENTS WITH ILDs?
ILD behavior-based algorithm to help clinicians assess the immediate need of palliative care for patients with fibrotic ILDs2
Palliative care should be provided throughout the ILD course in patients with fibrotic ILDs2
The ABCDE model shows a possible structured approach to comprehensive care, including palliative care throughout the ILD course.
* Fibrotic drugs are licensed to use only for IPF.
† Non-IPF ILDs.
How can you identify and manage ILDs as early possible in your patients?
ILD patient cases for evaluation, diagnosis and monitoring
- * Numbers based on a prospective cohort study.
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Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980.
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Sgalla G, Cerri S, Ferrari R, et al. Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy. BMJ Open Respir Res. 2015;2(1):e000065.
Molina-Molina M, Aburto M, Acosta O, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Exp Rev Resp Med. 2018;12(7):537–539.
Visca D, Mori L, Tsipouri V, et al. Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial. Lancet Respir Med. 2018;6(10):759-770.
van Manen M, Kreuter M, van den Blink B, et al. What patients with pulmonary fibrosis and their partners think: a live, educative survey in the Netherlands and Germany. ERJ Open Res. 2017;3(1):00065-2016.
Ahmadi Z, Wysham N, Lundström S, et al. End-of-life care in oxygen-dependent ILD compared with lung cancer: a national population-based study. Thorax. 2016;71(6):510-516.
Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: A double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.
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Hoffmann-Vold AM, Fretheim H, Halse AK, et al. Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med. 2019;200:1258–1266.
Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. The Lancet Rheumatology. 2020b;2: e71–e83.
Asano Y, Jinnin M, Kawaguchi Y, et al. Diagnostic criteria, severity classification and guidelines of systemic sclerosis: Guideline of SSc. J Dermatol. 2018;45, 633–691.
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