Skip to main content

Palliative/ supportive care in CTD-ILDs

Taking a palliative/supportive care approach benefits patients with connective tissue disease-associated interstitial lung diseases (CTD-ILDs)1,2

PF360 Banner

NEED FOR A HOLISTIC CARE APPROACH FOR CTD-ILD PATIENTS

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 CTD-ILDs.2 For CTD patients with ILD, there is a broad range of approaches available to support ILD management from non-pharmacologic health maintenance to mindfulness.1–4 Patients with CTD-ILD should receive palliative/supportive care measures that may include pulmonary rehabilitation therapy, smoking cessation and supplemental oxygen.5


Effective implementation of palliative/supportive care for CTD-ILDs requires a multidisciplinary participation from clinicians, specialist nurses, psychologists, social workers, and, in some countries, non-governmental faith and community-based organizations with access to palliative care expertise.2

The needs of patients with progressive fibrosing CTD-ILD are similar to those with malignant disease2

HOLISTIC CARE AIMING TO OPTIMIZE QUALITY OF LIFE SHOULD INCLUDE:2

es

Both pharmacologic and non-pharmacologic therapies, including treatment of comorbidities

Icon of the person with magnifying glass, representing screening of the ILD symptoms.

Symptom-centered approaches around symptoms that reduce quality of life, as well as depression, anxiety, fatigue and deconditioning

cae

Patient- and caregiver-centered management, such as education and assistance for self-management

care

End-of-life strategies, such as advanced care planning

Holistic care should aim to optimize quality of life at any stage of ILD2

Optimized quality of care includes patient-centred approaches

Adapted from: Kreuter M, et al. Lancet Respir Med. 2017;5(12):968-980.

Holistic care should be provided throughout the CTD-ILD course, tailored to the individual needs of the patients with CTD-ILDs2,3

PULMONARY REHABILITATION CAN SIGNIFICANTLY IMPROVE FUNCTIONAL CAPACITY AND QUALITY OF LIFE IN PATIENTS WITH ILDs:1*

Icon representing improvement in SGRQ of life score for ILD patients

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

Icon representing improvement after pulmonary rehabilitation in patients with ILD

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

Dyspnea

65% of patients achieved MCID improvement in dyspnea

Fatigue

52% of patients achieved MCID improvement in depression score

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

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

Reproduced from Respiratory Medicine, Ryerson et al, 108, 203-210, ©2022, with permission from BMJ Publishing Group Ltd.
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.

Pulmonary rehabilitation can improve your CTD-ILD patients’ quality of life, dyspnea and depression1
– make sure you provide pulmonary rehabilitation to your patients with CTD-ILDs

SUPPLEMENTAL OXYGEN USE DURING ROUTINE DAILY ACTIVITIES IMPROVES THE QUALITY OF LIFE IN PATIENTS WITH FIBROTIC ILDs2

BREATHLESSNESS AND AMBULATORY OXYGEN

Patients with fibrotic ILDs 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 treatment6
Reprinted from The Lancet Respiratory Medicine, Vol: 6, Issue: 10, 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, 759-770, ©2022 with permission from Elsevier.

Ambulatory oxygen can significantly improve breathlessness in patients with exertional hypoxia6 – make sure you provide ambulatory oxygen when required to your patients with CTD-ILDs

QUALITY OF LIFE AND AMBULATORY OXYGEN

Ambulatory oxygen can significantly improve breathlessness in patients with exertional hypoxia6 – make sure you provide ambulatory oxygen when required to your patients with CTD-ILDs

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]6

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.
Reprinted from The Lancet Respiratory Medicine, Vol: 6, Issue: 10, 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, 759-770, © 2022 with permission from Elsevier.
* 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.

Ambulatory oxygen can significantly improve quality of life in patients with fibrotic ILDs with exertional hypoxia6 – make sure your CTD-ILD patients have access to it

EARLY PALLIATIVE CARE CAN AID SYMPTOM RELIEF AND HELP MAINTAIN PHYSICAL AND EMOTIONAL WELLBEING IN PATIENTS WITH FIBROTIC ILDs2

Almost half of the patients with fibrotic ILDs die in the hospital due to poor advance care planning.7 Consequently, it is valuable to offer advance care planning, including discussion of treatment limitations and preferences on dying, to all patients with fibrotic ILDs.2
Palliative care is sometimes incorrectly viewed as consisting solely of end-of-life care, which may explain the under-use 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.2Improved palliative care for patients with progressive fibrosing ILD is likely to improve symptom burden.7

WHAT ARE THE PALLIATIVE CARE NEEDS OF YOUR CTD 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

Reprinted from Lancet Respir Med, 5(12), Kreuter et al, Palliative care in interstitial lung disease: living well, 968-980, ©2022, with permission from Elsevier.

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 course2

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

Reprinted from Lancet Respir Med, 5(12), Kreuter et al, Palliative care in interstitial lung disease: living well, 968-980, ©2022, with permission from Elsevier.

How can you identify and manage ILDs as early possible in your patients with CTDs?

Footnotes
  • * Numbers based on a prospective cohort study.
    6MWD, six-minute walking distance; ABCDE, assess – backing – comfort-care, comorbidities – disease-modifying treatment – end-of-life care; CI, confidence interval; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; K-BILD, King’s Brief Interstitial Lung Disease; MCID, minimal important clinical difference; QoL, quality of life; SD, standard deviation; SGRQ, St. George’s Respiratory Questionnaire. 

  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. Geerts S, Wuyts W, Langhe ED, et al. Connective tissue disease associated interstitial pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Dif. 2017;34:326–335.

  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. 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.

  8. Wallace B, Vummidi D, Khanna D. Management of connective tissue diseases associated interstitial lung disease: a review of the published literature. Curr Opin Rheumatol. 2016;28(3):236–245.

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

Resources for patients