Palliative/ supportive care for ILDs

Taking a palliative/supportive care approach benefits patients with pulmonary fibrosis1,2

ILD patients with the HRCT scans in their hands.

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

ILD patients need timely diagnosis, comprehensive information, early treatment access, and holistic care

 

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†

Optimised quality of care includes end of life care, patient centred approaches and disease stabilising care

 

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

Checklist icon.

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

Walking person icon.

57.6 m improvement in 6MWD immediately after rehabilitation (95% CI 40.2–75.1 m, p<0.0005)

Dyspnea / breathlessness icon.

65% of patients achieved MCID improvement in dyspnea

Worried person, sitting on the chair icon.

52% of patients achieved MCID improvement in depression score

Pulmonary rehabilitation significantly improves quality of life, dyspnea and depression in patients with fibrotic ILDs1

Pulmonary rehabilitation significantly improves quality of life, dyspnea and depression in patients with ILDs

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

Reduction of isolated exertional hypoxia progressive fibrosing ILD patients breathlessness significantly

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

Isolated exertional hypoxia progressive fibrosing ILD patients total K-BILD score significantly improved

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

Scheme introducing our patient and multidisciplinary expert empowerment program (PPEPP).

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.

Proactively collaborate for ILD diagnosis

 

 

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

ILD behaviour-based algorithm to assess the need for palliative care
 

Palliative care should be provided throughout the ILD course in patients with fibrotic ILDs2

Palliative care should be provided throughout ILD course as per ABCDE model of ILD care

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?

Footnotes
  • * Numbers based on a prospective cohort study.

  1. Ryerson CJ, Cayou C, Topp F, et al. Pulmonary rehabilitation improves long-term outcomes in interstitial lung disease: a prospective cohort study. Respir Med. 2014;108(1):203-210.

  2. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968-980.

  3. Maher TM, Wuyts W. Management of Fibrosing Interstitial Lung Diseases. Adv Ther. 2019;doi:10.1007/s12325-019-00992-9. [Epub ahead of print].

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. Theodore AC, Tseng C-H, Li N, Elashoff RM, Tashkin DP. Correlation of cough with disease activity and treatment with cyclophosphamide in scleroderma interstitial lung disease: findings from the Scleroderma Lung Study. Chest. 2012;142(3):614–621.

  11. 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.

  12. 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.

  13. 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.

  14. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383:958–968.

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