Impacts of pulmonary fibrosis
Nurses have an important role in supporting patients in managing the impact of their symptoms on their everyday life1–4
PROGRESSIVE FIBROSING ILDs HAVE A WIDE RANGE OF POTENTIAL EFFECTS ON YOUR PATIENTS5
As pulmonary fibrosis worsens in your ILD patients, their lungs become increasingly scarred resulting in continued lung function decline.5,6
Patients with progressive fibrosing ILDs may experience worsening of respiratory symptoms, lung function decline and reduced health-related quality of life5
DLco, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HRCT, high-resolution computed tomography; PFT, pulmonary function test.
THE BURDEN OF PROGRESSIVE FIBROSING ILD CAN SUBSTANTIALLY REDUCE A PATIENT’S HEALTH-RELATED QUALITY OF LIFE7
As fibrotic ILD progresses, there may be a negative impact on your patients’ day-to-day quality of life, independence and emotional wellbeing.8
In your patients with progressive fibrosing ILDs, cough, dyspnea and fatigue may limit their physical functional capacity, as well as their ability to perform basic activities and participate in everyday life.7–10
Decreased social activities
In addition to the functional impact, your patients with progressive fibrosing ILD may suffer emotional distress, including feelings of:7,8,10–12
Loss of independence
Patients with progressive fibrosing ILDs, such as IPF, HP and CTD-ILDs, have reported almost universal symptoms of anxiety and/or depression.10,12
NURSES HAVE AN IMPORTANT ROLE IN HELPING PATIENTS FEEL MORE IN CONTROL IN THEIR EVERYDAY LIFE4
Patients often express the (worsening of) fibrotic ILD symptoms by their ability to perform everyday activities or life priorities.13 Listen out for subtle changes in your patients’ daily activity level, as they may avoid doing things to compensate for worsening symptoms/lung functions.13,14
- Divide big tasks into several more manageable ones to make it easier to achieve17
- Set realistic goals to help your patients adjust18
- Encourage your patients to stay active with a light exercise routine19,20
- Avoid trigger factors for symptoms (e.g. smoking and second-hand smoke, perfume)21
- Suggest patients drink plenty of water throughout the day to keep their throat moist21
- Try breathing exercises that may help your patients control their breathing22
- Suggest mindfulness to help patients with their emotional challenges23
Jean explains how mindfulness really helps her deal with her pulmonary fibrosis and maintain a positive outlook
I was told the month before I retired aged 70 that I’d got IPF. My son had said to me: “Oh well mum, when you get the results, as long as it’s not IPF.” And of course it was IPF.
I mean it was just total disbelief. It’s incurable, it’s progressive, very few people have it and little is known about it.
If you look online the prognosis is very poor, but I’m not having any of that. I’m here for the duration. So you know….[Laugh]
So I do lots of things to help me holistically. I do mindfulness, previously I’d done a cognitive behavioural therapy course. Usually we work very much in the active side of our mind and when you do mindfulness you’re trying to get in touch with the being side of your mind, concentrating on your breathing. The idea being that instead of feeling the pressures of modern day life, thinking “I’ve got to do A, B, C and D”, you take a break from that and then when you come back to it you’ve got a much more relaxed, realistic approach. And also, I undertook it to help me come to terms with the prognosis and diagnosis.
I’ve always liked walking and have always have walked fairly fast, almost speed walking to be honest, for several years. To me, it’s just very good for my lungs. Because I walk fast, not just saunter along, I’m exercising my lungs to a good capacity.
When I say to my relatives and friends that I go to this support group they will say at some point, isn’t that a bit morbid for you.. And I say no, not at all; the people are so uplifting, comforting and I get so much from it. It’s really humbling actually.
I pride myself on having lots and lots of resilience, acquired from my mum and that sort of gets me by and helps me no end.
Be as positive as you possibly can be.
Perseverance, you will have days when you’re not feeling perhaps so good.
Optimism, hope, you know, there is a way forward.
Sometimes patients struggle to accept that their fibrotic ILD is getting worse and that it is becoming harder for them to go about their daily life.24 In these instances, caregivers have an important role in providing physical and emotional support, as well as motivating them to try simple tips to support their everyday lives.25–28 It is important to note that caregivers’ responsibilities can increase and change as their loved ones’ needs increase, and they may also need support.29
HELPING PATIENTS MANAGE THEIR BODY WEIGHT
When patients with progressive fibrosing ILD are underweight or overweight, it can have a negative impact on their lung function, which is why it is important to help them manage their diet.30
- If you struggle to eat large meals, try instead to eat six to eight small dishes which are high in calories and nutrients32,33
- Try not to have drinks just before or during meals, as this can make you feel too full to eat31,33
- Drink lots of water especially when you are exercising34
- Make sure you eat enough high-protein foods. Meat and dairy products like yoghurt and cheese have lots of protein to give you strength. High-protein foods should be included in your diet several times a day31,33
- Avoid low fat or diet versions of foods and drinks (e.g. use full-fat milk or cream rather than skimmed or semi-skimmed milk)31–33
- If you have diarrhea, avoid spicy and sour foods34
- Eat more high-fat foods like cheeses, cream-based foods and yoghurts, or high-calorie drinks like fresh fruit juice or yoghurt drinks32,33
Simple tips that may help your obese patients
Fibrotic ILD patients who are obese may find it harder to breathe and exercise.30
Motivate patients to stay active32
Suggest patients find a distraction if they are eating for comfort32
NURSES CAN HELP PATIENTS ACCESS OTHER HEALTHCARE SPECIALIST TO SUPPORT THEIR FIBROTIC ILD MANAGEMENT4,15,35
Your patients with fibrotic ILDs may also benefit from supportive care measures that may include pulmonary rehabilitation therapy, smoking cessation and supplemental oxygen.35
Pulmonary rehabilitation can improve the quality of life, dyspnea and depression of patients with fibrotic ILDs36 and ambulatory oxygen can significantly improve breathlessness and quality of life in patients with fibrotic ILDs and exertional hypoxia.37
Different healthcare multidisciplinary team members can combine to help you deliver supportive care for your patients with fibrotic ILDs.15
As your patients’ ILD progresses, it is important to help them and their caregivers understand their end-of-life care options, as they may need to consider a referral to hospice care.38
Peter talks about how his ILD specialist nurse helped him with managing his symptoms.*
Peter was diagnosed with a fibrotic ILD and he is already under the care of the multidisciplinary team (MDT). At a routine check-up, he mentioned that they are going to convert a downstairs room into a bedroom for him. On further enquiry, Peter volunteered that in addition to feeling tired generally and struggling with his cough, he also doesn’t like going up the stairs because he has recently been finding it difficult to catch his breath when he tries. After hearing this, the ILD nurse decided to speak with the pulmonologist on the MDT and they agreed that he should be referred for pulmonary rehabilitation to help manage his breathlessness, cough and fatigue.
Peter attended his pulmonary rehabilitation sessions for 2 hours, twice a week, for 6 weeks. The exercises really helped him feel stronger. However, when the program finished, Peter struggled to establish a regular exercise routine. He discussed this with his ILD nurse and she helped him create an exercise progress chart, with achievable goals and milestones, which really motivated Peter to exercise more regularly.
*Hypothetical patient. Patient case study for illustrative purposes only.
What other resources can help you support your patients in their journey with fibrotic ILD?
Interventions for fibrotic ILDs
- CTD-ILD, connective tissue disease-associated interstitial lung disease; DLco, diffusing capacity of the lung for carbon monoxide; FVC, forced vital capacity; HP, hypersensitivity pneumonitis; HRCT, high-resolution computed tomography; HRQoL, health-related quality of life; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; MDT, multidisciplinary team; PFT, pulmonary function test.
- 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.
- Grewal JS, Ryerson CJ. A closer look at the multidisciplinary interstitial lung disease clinic: Who, what and how. Respirology. 2021;26:12–13.
- 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.
- Vega-Olivo M, Criner GJ. Idiopathic pulmonary fibrosis: A guide for nurse practitioners. Nurse Pract. 2018;43:48–54.
- Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.
- Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
- 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 Respir Res. 2017;4:e000212.
- Swigris JJ, Brown KK, Abdulqawi R, et al. Patients’ perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180075.
- Olson AL, Brown KK, Swigris JJ. Understanding and optimizing health-related quality of life and physical functional capacity in idiopathic pulmonary fibrosis. Patient Relat Outcome Meas. 2016;7:29–35.
- Morisset J, Dubé B, Garvey C, et al. The unmet educational needs of patients with interstitial lung disease: setting the stage for tailored pulmonary rehabilitation. Ann Am Thorac Soc. 2016;13:1026–1033.
- Duck A, Spencer LG, Bailey S, et al. Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis. J Adv Nurs. 2014;71(5):1055–1065.
- Hinz A, Brähler E, Möde R, et al. Anxiety and depression in sarcoidosis: the influence of age, gender, affected organs, concomitant diseases and dyspnoea. Sarcoidosis Vasc Diffuse Lung Dis. 2012;29(2):139–146.
- Mittoo S, Frankel S, LeSage D, et al. Patient perspectives in OMERACT provide an anchor for future metric development and improved approaches to healthcare delivery in connective tissue disease related interstitial lung disease (CTD-ILD). Curr Respir Med Rev. 2015;11(2):175–183.
- Birring SS, Bushnell DM, Cutts K, et al. King’s Brief Interstitial Lung Disease (K-BILD) questionnaire: content relevance in progressive fibrosing ILD (PF-ILD). Eur Respir J. 2020;56 (suppl 64):1813.
- 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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- Action for pulmonary fibrosis. Managing breathlessness when bending. Available at: https://www.actionpf.org/information-support/managing-breathlessness-when-bending. [Accessed January 2023].
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- 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.
- American Lung Association. Physical activity and pulmonary fibrosis. Available at: https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/living-well-with-pulmonary-fibrosis/physical-activity.
- Action for pulmonary fibrosis. Top tips: Other ways to manage the pulmonary fibrosis cough. Available at: https://www.actionpf.org/information-support/managing-a-cough. [Accessed January 2023].
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- Belkin A, Albright K, Swigris JJ. A qualitative study of informal caregivers’ perspectives on the effects of idiopathic pulmonary fibrosis. BMJ Open Respir Res. 2014;1:e000007.
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