Skip to main content

Intervention for fibrotic ILDs

Nurses have an important role to help patients understand their ILD treatment and manage their treatment expectations1–5

Intervention for fibrotic ILDs

HELP YOUR PATIENTS WITH FIBROTIC ILD UNDERSTAND THEIR TREATMENTS TO SUPPORT MEDICATION ADHERENCE2,5,6

Helping your patients understand their treatment options and managing their expectations of what the treatment can do may help increase their adherence with treatment.1,2,5

It is important that patients understand fibrotic ILD causes irreversible lung damage7 and that current treatments for IPF, for example, can help preserve lung function but cannot reverse the damage.8,9

Ensure your patients understand that current treatments for pulmonary fibrosis, for example for IPF, can help to slow disease progression, but they are not a cure10,11

EARLY TREATMENT IS ESSENTIAL IN IPF AND COULD POSITIVELY IMPACT PROGRESSION AND IMPROVE OUTCOMES FOR PATIENTS12–14

Antifibrotic treatment started immediately after diagnosis of IPF14 could help slow progression when there is the most left to preserve12,15,16

Antifibrotic treatment started immediately after diagnosis of IPF14 could help slow progression when there is the most left to preserve12,15,16

Consistent real-world evidence shows antifibrotic treatment significantly improves survival in patients with IPF17–20

Consistent real-world evidence shows antifibrotic treatment significantly improves survival in patients with IPF17–20

MANAGEMENT OF NON-IPF ILDs

Management approach may vary depending on the specific ILDs.21 There are recommendations for both pharmacologic and non-pharmacologic approaches.21 For example, for patients with hypersensitivity pneumonitis (HP), removal of the allergen responsible should be the first step in HP management.22

An expert consensus recommends that treatment for non-IPF progressive fibrosing ILDs should not wait.23

ADVICE AND SUPPORT FROM SPECIALIST NURSES ON SIDE EFFECTS CAN HELP IMPROVE PATIENT ADHERENCE TO TREATMENT2,5,6

nurses-affect-patient_0.png

 

Nurses have a key role in educating and supporting patients with ILD, including troubleshooting treatment side effects2

Patients might stop taking treatments when they experience side effects.24 It is therefore important for patients to be informed of potential side effects before starting treatment.6,25

Patients should know to promptly report side effects to their healthcare team, as managing side effects quickly and effectively may improve patient compliance and outcomes of treatment.6

Encourage your patients to discuss any side effects promptly with their healthcare team6

There is a range of approaches to help manage side effects

Patients should be made aware that there are strategies to help manage treatment side effects.25–27

For example, educating patients with IPF on how dose adjustment can manage possible side effects of antifibrotic treatment can also help reassure them about starting treatment and help prolong their treatment benefits.28

In patients with IPF, the majority of antifibrotic side effects are gastrointestinal in nature, which is why patients should take their medication with food.5

Lifestyle changes, such as dietary interventions* and attending support groups may help patients manage side effects. Staying active may improve their quality of life.25–27

Dietary interventions* may include adequate hydration at the first sign of diarrhea, avoidance or selection of certain food/drinks and the size and frequency of meals25

Dietary interventions* may include adequate hydration at the first sign of diarrhea, avoidance or selection of certain food/drinks and the size and frequency of meals25
Supportive medications can be used to manage diarrhea, nausea and vomiting associated with treatment5,25

Supportive medications can be used to manage diarrhea, nausea and vomiting associated with treatment5,25
If symptomatic treatment of side effects is ineffective, temporary ILD treatment interruption or dose reduction may be prescribed25

If symptomatic treatment of side effects is ineffective, temporary ILD treatment interruption or dose reduction may be prescribed25
Nurse-led telephone clinics and email communication can help patients have access to regular advice on what to expect, any required symptomatic treatment, and reduction/temporary interruption of treatment5,6,29–32

Nurse-led telephone clinics and email communication can help patients have access to regular advice on what to expect, any required symptomatic treatment, and reduction/temporary interruption of treatment5,6,29–32
Patients may also find support groups beneficial for emotional support, and practical guidance on symptom management27

Patients may also find support groups beneficial for emotional support, and practical guidance on symptom management27
Exercise training can lead to clinically meaningful improvements in ILD patients’ quality of life and exercise capacity26

Exercise training can lead to clinically meaningful improvements in ILD patients’ quality of life and exercise capacity26

*Dietary intervention recommendations, which come from consensus statements from an expert panel of pulmonologists. Consensus statements were developed for use in patients with IPF.25

Real-world evidence suggests that regular specialist nurse input to provide patient support and education improves adherence to/persistence with antifibrotic treatment in IPF5,6,29–32

What other resources can help you support your patients in their journey with fibrotic ILD?

Footnotes

  • HP, hypersensitivity pneumonitis; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis.

  1. Barbera T, Davila L, Patel NM. Management and support of patients with fibrosing interstitial lung diseases. Nurse Pract. 2021;46(7):39–44.
  2. Grewal JS, Ryerson CJ. A closer look at the multidisciplinary interstitial lung disease clinic: Who, what and how. Respirology. 2021;26:12–13.
  3. McLean AEB, Webster SE, Fry M, et al. Priorities and expectations of patients attending a multidisciplinary interstitial lung disease clinic. Respirology. 2021;26:80–86.
  4. Russell AM, Olive S, Lines S, et al. Contemporary challenges for specialist nursing in interstitial lung disease. Breathe. 2018;14:36–41.
  5. Shaw J, Marshall T, Morris H, et al. Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age. J Thorac Dis. 2017;9:4700–4707.
  6. Bendstrup E, Wuyts W, Alfaro T, et al. Nintedanib in idiopathic pulmonary fibrosis: Practical management recommendations for potential adverse events. Respiration. 2019;97:173–84.
  7. Wells AU, Brown KK, Flaherty KR, et al. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5):1800692.
  8. Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.
  9. Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
  10. Borie R, Le Guen P, Ghanem M, et al. The genetics of interstitial lung diseases. Eur Respir Rev. 2019;28(153):190053.
  11. Cottin V. Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases. Eur Respir Rev. 2019;28(153):190109.
  12. Maher TM, Molina-Molina M, Russell AM, et al. Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries. BMC Pulm Med. 2017;17(1):124.
  13. 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.
  14. Robalo-Cordeiro C, Campos P, Carvalho L, et al. Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence. Rev Port Pneumol. 2017;23(5):287–293.
  15. Torrisi SE, Pavone M, Vancheri A, Vancheri C. When to start and when to stop antifibrotic therapies. Eur Respir Rev. 2017;26(145):170053.
  16. Kolb M, Richeldi L, Behr J, et al. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. Thorax. 2017;72:340–346.
  17. Guenther A, Krauss E, Tello S, et al. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19(1):141.
  18. Jo HE, Glaspole I, Grainge C, et al. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 2017;49:1601592.
  19. Kaunisto J, Salomaa E, Hodgson U. Demographics and survival of patients with idiopathic pulmonary fibrosis in the Finnish IPF registry. ERJ Open Res. 2019;5:00170-2018.
  20. Behr J, Prasse A, Wirtz H, et al. Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry. Eur Respir J. 2020;56(2):1902279.
  21. Meyer KC. Diagnosis and management of interstitial lung disease. Transl Respir Med. 2014;2:4.
  22. Varone F, Iovene B, Sgalla G, et al. Fibrotic hypersensitivity pneumonitis: diagnosis and management. Lung. 2020;198(3):429–440.
  23. George PM, Spagnolo P, Kreuter M, et al. Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities. Lancet Respir Med. 2020;8:925–934.
  24. Nantz E, Lui-Seifert H, Skljarevski V. Predictors of premature discontinuation of treatment in multiple disease states. Patient Prefer Adherence. 2009;3:31–43.
  25. Rahaghi F, Belperio JA, Fitzgerald J, et al. Delphi consensus recommendations on management of dosing, adverse events, and comorbidities in the treatment of idiopathic pulmonary fibrosis with nintedanib. Clin Med Insights Circ Respir Pulm Med. 2021;15:11795484211006050.
  26. Dowman LM, McDonald CF, Hill CJ, et al. The evidence of benefits of exercise training in interstitial lung disease: a randomised controlled trial. Thorax. 2017;72(7):610–619.
  27. Magnani D, Lenoci G, Balduzzi S, et al. Effectiveness of support groups to improve quality of life of people with idiopathic pulmonary fibrosis a pre-post test pilot study. Acta Biomed. 2017;88:5–12.
  28. Maher TM, Wuyts W. Management of fibrosing interstitial lung diseases. Adv Ther. 2019;36:1518–1531.
  29. Hughes G, Toellner H, Morris H, et al. Real world experiences: Pirfenidone and nintedanib are effective and well tolerated treatments for idiopathic pulmonary fibrosis. J Clin Med. 2016;5(9):78.
  30. Duck A, Spencer LG, Bailey S, et al. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis. J Adv Nurs. 2015;71(5):1055–1065.
  31. Russell AM, Ripamonti E, Vancheri C. Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients' perspectives of the disease and treatment. BMC Pulm Med. 2016;16:10.
  32. Harris E, Harding K, McLellan T, et al. Antifibrotic medications for idiopathic pulmonary fibrosis (IPF): A real world single centre experience of 447 patients over a 6 year period. Thorax. 2019;74(Suppl2):A243–A244. Abstract M15.
  33. Kreuter M, Bendstrup E, Russell A, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017;5(12):968–980.
  34. Denton CP, Laird B, Moros L, Flores JLL. Things left unsaid: important topics that are not discussed between patients with systemic sclerosis, their carers and their healthcare professionals–a discourse analysis. Clin Rheumatol. 2021;40:1399–1407.
  35. Baile WF, Buckman R, Lenzi R, et al. SPIKES – A six-step protocol for delivering bad news: application to the patient with cancer. Oncologist. 2000;5:302–311.
  36. Wijsenbeek MS, Bonella F, Orsatti L, et al. Communicating with patients with idiopathic pulmonary fibrosis: can we do it better? ERJ Open Res. 2022;7:00422-2021.

Resources for patients