Living With Lung Fibrosis: Knowledge is Power

Abstract

Lung fibrosis is a serious disease that takes a toll on patients and caregivers. The course of lung fibrosis is variable, but most patients experience breathlessness and fatigue and many are troubled by cough. In addition, the medications used to slow the worsening of lung fibrosis may cause side effects. The better that patients understand lung fibrosis, the more able they will be to manage its impact on their lives. Support is available to help patients manage the symptoms of their disease and the side effects of medications. In this article, we use our experiences as a patient with lung fibrosis, a wife and caregiver of a patient with lung fibrosis, and a clinician who cares for patients with lung fibrosis to provide information for patients on the diagnosis, course, and treatment of this disease.

Keywords::

• caregivers
• cough
• dyspnea
• interstitial
• lung disease
• patients
• pulmonary fibrosis
• pulmonary function tests

Lung fibrosis is a serious disease, but how it progresses and impacts a person’s life varies. Patients themselves are individuals, with distinct personalities, values and lifestyles. These differences make it challenging to provide information that is relevant to all patients. However, for all patients with lung fibrosis, a better understanding of the disease is the key to managing it. In this article, we use our experiences as a patient with idiopathic pulmonary fibrosis (IPF; and the founder of a support group for patients with lung fibrosis), a wife and caregiver of a patient with IPF, and a clinician with extensive experience in the management of lung fibrosis to provide information for patients with this disease. Our aim is to empower patients to take more control of the challenges of living with lung fibrosis.

What is lung fibrosis?

Many patients have not even heard of lung fibrosis at the time that they are diagnosed. The mechanisms behind the disease are very complicated, but in simple terms, lung fibrosis develops when the tissue around the air sacs in the lungs (known as the “interstitium”) becomes scarred (thickened) [1,2]. This makes it more difficult for the lungs to transfer oxygen into the blood and can lead to shortness of breath and a reduced tolerance for exercise (Figure 1).

Figure 1. Fibrosis of the lungs.

What causes lung fibrosis?

Patients often ask why they have developed lung fibrosis. “Why me?” For some patients, their doctor is able to identify a cause for their lung fibrosis. People with autoimmune diseases such as rheumatoid arthritis are at higher risk of lung fibrosis, as an overactive immune system can cause repeated damage to the lungs [2,3]. Lung fibrosis may also be caused by a bad reaction to an organic material such as mould or feathers [4]. Exposure to a poisonous material such as asbestos can lead to lung fibrosis [5]. Genetics also play a role; patients with parents or siblings with lung fibrosis are at greater risk of developing lung fibrosis themselves [2]. However, for many patients, the cause of their lung fibrosis is unknown. The most common type of lung fibrosis is a disease known as idiopathic pulmonary fibrosis or IPF. IPF has no known cause, but is much more common in people over the age of 60 years and in people with a history of smoking [6]. Other terms that you may see or hear used include “interstitial lung disease” or “ILD” (which is the group name for diseases of the tissue around the air sacs in the lungs that may lead to lung fibrosis) and “idiopathic interstitial pneumonia” (which refers to any type of lung fibrosis that does not have a known cause, including IPF).

How is lung fibrosis diagnosed?

Patients with lung fibrosis may remain undiagnosed for some time, as the symptoms of the disease are non-specific and patients may think they are simply due to ageing. A diagnosis of lung fibrosis is made based on a scan of the lungs known as a “high-resolution computed tomography” or “HRCT” scan [6]. This type of scan provides much more detail than an x-ray and gives a radiologist a clear picture of the damage to the lungs (Figure 2). In looking at the scan, the radiologist will be able to see how much of the lungs has been damaged and the pattern of fibrosis. This is important, as the speed at which lung function is lost is known to be greater in patients who have more of their lungs affected by fibrosis and in patients who have particular patterns of fibrosis [7].

Figure 2. Scans of healthy lungs and lungs affected by fibrosis.

(A) Healthy lungs. (B) Lungs affected by fibrosis.

Provided by and reproduced with permission from Professor Simon LF Walsh, National Heart and Lung Institute, Imperial College London, UK.

Does lung fibrosis always get worse?

Most forms of lung fibrosis are progressive, which means that the amount of scar tissue in the lungs increases over time [7]. Once lung fibrosis has started to progress, it is not possible to stop it. However, the speed at which lung fibrosis worsens is different in different people (Figure 3). Some patients have periods of time in which their disease remains largely stable. In other patients, the disease gets gradually worse over time. A small proportion of patients have sudden, severe worsenings of their disease, which usually result in an admission to hospital [8].

Figure 3. Possible patterns of progression of lung fibrosis.

How is lung fibrosis monitored?

Patients with lung fibrosis need regular follow-ups so that their doctor can check whether their disease has got worse, assess symptoms, and consider whether therapy needs to be increased or changed. Doctors usually assess the severity of lung fibrosis by measuring a patient’s lung function [2]. The two most commonly used tests in patients with lung fibrosis are known as “forced vital capacity” or “FVC” and “diffusing capacity for carbon monoxide” or “DLco” [2]:

• FVC is the maximum volume of air that the individual can breathe out. To measure FVC, a patient is asked to take a deep breath and then blow as hard as they can into a mouthpiece attached to a small machine known as a spirometer. The spirometer then provides a measurement of the volume of air that the patient blew out.

• DLco is a measure of how well the lungs are able to transfer oxygen into the blood. To measure DL_CO, a patient is asked to put on a mask and take in a deep breath of gases, hold their breath, and then exhale. The mixture of gases that the patient exhales are analysed to give a measure of how well gases were exchanged between the patient’s lungs and blood.

A doctor may also look at how well a patient is able to exercise by measuring how far they can walk in 6 minutes and whether this walk results in the oxygen levels in their blood becoming too low. This is known as a 6-minute walk test [9].

In patients with lung fibrosis, measures of lung function and exercise tolerance get worse over time. However, these measures vary from day to day and may be affected by other medical conditions such as infections (even the common cold), emphysema, or tiredness. It is important not to put too much weight on a single value. It is the pattern of change over time, taking account of other factors, that provides the best measure of how a patient’s lung fibrosis is progressing.

How is lung fibrosis treated?

Drugs known as “antifibrotics” (nintedanib [OFEV] and pirfenidone [Esbriet]) [10,11] are available that slow the progression of lung fibrosis and extend life, but these do not provide an improvement in symptoms [6]. It can be difficult to manage the symptoms of lung fibrosis, particularly in patients who have had the disease for a long time. Below you will find some advice on managing the most common symptoms of lung fibrosis (Box 1). Remember that every patient is different. If one approach does not work for you, try an alternative [2,12].

Box 1. Advice on managing the most common symptoms of lung fibrosis.

Shortness of breath

• Call your doctor right away if you have tightness in your chest, trouble breathing, pain or fever

• Practise breathing techniques

• Ask your doctor about medications that might help, such as inhalers

• Stay active, if possible, find a local pulmonary rehabilitation programme [13]

• If you are having problems using supplemental oxygen, make sure you are using it properly and talk to your doctor about alternative methods for oxygen delivery [14]

• Ask your doctor about purchasing a pulse oximeter so that you can monitor your oxygen levels at home [14]

Cough

• Treat the cause of your cough when possible; for example, use medications for asthma, acid reflux, or post-nasal drip

• Drink fluids, such as hot tea, with honey

• Buy cough drops or cough medicine from a pharmacy

• Ask your doctor about prescription medications

• Cough may be triggered by talking or eating. Ask your doctor about whether a therapist could help you learn how to talk without triggering a cough

Fatigue

• Get plenty of rest and take short naps if you need to

• Stay active. Enrol in a pulmonary rehabilitation programme if possible [13]

• Ask others to help with tasks such as grocery shopping or cleaning

• If you have oxygen, use it as directed [14]

• Eat well

Gastrointestinal problems

For loss of appetite:

• Eat several small meals throughout the day

• Add olive oil, milk, or yoghurt to increase calories and protein in meals

• Consider nutritional supplements like milkshakes

• Take a walk before you eat

For nausea or vomiting:

• Eat bland foods, such as toast or rice

• Drink peppermint tea, ginger tea or ginger ale

• Ask your doctor about prescription medications that might help

For diarrhea:

• Avoid high-fibre foods, such as beans, pulses and fruit

• Eat bland foods, such as toast or rice

• Eat small meals

• Drink plenty of water between meals

• Ask your doctor about prescription medications that might help

How can the side effects of medications be managed?

All drugs have side effects in some patients. Drugs used to treat lung fibrosis may cause diarrhoea, nausea and loss of appetite [10,11]. Some patients lose weight. Taking these drugs with meals helps to reduce their side effects [10,11]. Patients who are struggling to tolerate drug side effects should speak to their doctor. Reducing the dose, or taking a short break from treatment, can help to reduce side effects [10,11]. Ideally the dose can be increased back to the original dose once the side effects are under control. Over the counter products, such as anti-diarrhoeal medications, can also be helpful.

Patients taking drugs for lung fibrosis need regular blood tests so that their doctor can check the effects that the drugs are having on their liver and kidneys. It may be necessary for a patient to reduce dose, take a break from treatment, or even stop treatment based on the results of these tests [10,11].

What about other conditions?

Patients with lung fibrosis often have other medical conditions (known as “comorbidities”) that cause symptoms and affect quality of life (e.g. emphysema/COPD, acid reflux, pulmonary hypertension, sleep apnea, autoimmune diseases) [15]. Respiratory infections may make the symptoms of lung fibrosis worse. It is important not to assume that symptoms of breathlessness, cough or fatigue are entirely due to lung fibrosis. It may be possible for symptoms to be relieved, at least in part, by managing other conditions.

Many patients with lung fibrosis feel depressed or anxious as a result of their symptoms or fears about the future. Patients should not be afraid to talk to their doctor about how they are feeling emotionally as well as physically. “Talking to your doctor about how you feel is not only a way of expressing your concerns, but also a way of building trust and understanding. Your doctor can listen to your emotions, empathize with your situation, and offer you the best advice for your health and well-being”, says Bill Vick.

Is a lung transplant an option?

Some, but not all, patients with lung fibrosis are eligible for a lung transplant [2]. A patient’s care team should be able to provide them with information on evaluation for lung transplant and what that might mean for their life before and after surgery, or refer them to another medical professional who can provide this information. Haley Hoy, an experienced lung transplant nurse practitioner, says “While lung transplantation is not an ideal remedy, it can add years to the life of someone who has advanced lung disease but relatively few other medical problems. A comprehensive assessment of the patient’s health is needed, as well as a discussion with the patient about what a lung transplant could mean for their life”.

Get your questions answered!

Knowledge is power. The better that patients and caregivers understand lung fibrosis, the more able they will be to manage its impact on their lives.

There is a lot of information about lung fibrosis available on-line. Some of it is useful, but some of it is inaccurate, out-of-date, or does not apply to a particular patient [16,17]. If seeking information on-line, make sure it comes from a trustworthy source such as a patient organisation or an expert healthcare professional. Information read on-line should be checked with a doctor or nurse practitioner experienced in managing lung fibrosis. Further advice on getting more information about your disease is given in Box 2. Good sources of information are listed in Box 3.

Box 2. Getting more information about your disease: advice from a patient.

• Remember that all patients are different. Your experience of lung fibrosis may not be the same as the experience you have read about on-line or heard about from another patient.

• Write down your questions and take the list to your next appointment with your doctor or nurse practitioner.

• You might not be able to get all your questions answered in one go, or you might receive too much information to take it all in at one-time. Feel free to ask more questions later and repeat questions if you need to.

• Tell your doctor or nurse practitioner about any problems you are having with your symptoms or medications. Help them to help you.

• Don’t be afraid of the term “palliative care”. Palliative care is a medical specialty dedicated to symptom management and can be helpful to patients at all stages of disease.

Box 3. Good sources of information.

Good sources of information

• PF Warriors: https://pfwarriors.com or https://facebook.com/pfwarrior

• Pulmonary Fibrosis Foundation (PFF): https://pulmonaryfibrosis.org

• American Lung Association: https://lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis

• Your doctor or nurse practitioner

• Check out patient/caregiver support groups in your area

• A podcast of advice for patients with lung fibrosis is available at: https://www.usscicomms.com/respiratory/Hoy/LivingWithLungFibrosis

Ask for help

Family and friends are a vital source of support for patients with lung fibrosis, but they might also need help in understanding the disease and coming to terms with its impact. Sharing health information with family and friends can be helpful, but patients shouldn’t feel compelled to share everything they know about their health with others. Decide how much you want to share and when [18].

Patient support groups (in person or on-line) can be really helpful for information-sharing and support. Nothing is more powerful than patients helping patients.

Conclusion

Living with lung fibrosis brings physical and emotional challenges. Patients can help equip themselves to meet these challenges by becoming more knowledgeable about their condition and how its impacts can be managed. A patient’s clinical care team and patient organisations are excellent sources of information and advice.

Future perspective

Over the next few years, it is likely that additional drugs for the treatment of lung fibrosis will become available. It is hoped that these will be more effective at slowing the worsening of the disease, and have less impactful side effects, than existing therapies. Combinations of drugs that work in different ways are likely to provide the best outcomes for patients. More research is needed to develop therapies (both drugs and non-pharmacological therapies) that alleviate the symptoms of lung fibrosis. The participation of patients in clinical trials and other studies will be vital for the development of better treatments. By collaborating with researchers, regulators, and industry, patient-led organisations can ensure that research questions are meaningful to the people who are most affected by the disease. Further, as explained by Bill Vick, “Patient-led organisations can also play a crucial role in communicating the findings of new research to patients and caregivers, using their networks and platforms to raise awareness, educate, and empower them to make informed decisions about their health and their care”.

Executive summary

Objective

• This article provides information to empower patients with lung fibrosis to take more control of living with their disease.

What is lung fibrosis?

• Lung fibrosis occurs when the tissue around the air sacs in the lungs becomes scarred (thickened). This impairs the exchange of oxygen between the lungs and the blood and leads to shortness of breath.

What causes lung fibrosis?

• Some cases of lung fibrosis have a known cause, e.g., an autoimmune disease, but in other cases, no cause can be identified.

How is lung fibrosis diagnosed?

• A diagnosis of lung fibrosis is made based on a detailed scan of the lungs known as a “high-resolution computed tomography” or “HRCT” scan.

Does lung fibrosis always get worse?

• Most forms of lung fibrosis progress (worsen) over time, but the speed at which lung fibrosis progresses is variable.

How is lung fibrosis monitored?

• Regular lung function tests to check whether lung fibrosis is worsening.

What about other conditions?

• Patients with lung fibrosis often have other medical conditions that cause similar symptoms, so it is important not to assume that all their symptoms are due to lung fibrosis.

How is lung fibrosis treated?

• Drugs known as “antifibrotics” can be used to slow the progression of lung fibrosis and extend life, but these drugs do not relieve symptoms.

How can the side effects of medications be managed?

• Side effects of the drugs used to treat lung fibrosis may need to be managed by reducing the dose or taking a short break from treatment.

Is a lung transplant an option?

• Lung transplant is an option for a minority of patients with lung fibrosis.

Get your questions answered!

• Patients with lung fibrosis can obtain more information about their condition from their doctor or nurse practitioner and from reliable on-line sources, such as patient support groups.

• The better that patients understand lung fibrosis, the more able they will be to manage its impact on their lives.

Future perspective

• Over the next five to ten years, it is likely that new therapies for lung fibrosis will become available, which will improve the lives of patients living with this disease.

Author contributions

The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE).

Financial disclosure

The authors did not receive any payment for their work on this article. The authors have no financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Writing disclosure

Editorial support was provided by Wendy Morris of FleishmanHillard, London, UK, which was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. Boehringer Ingelheim was given the opportunity to review the article for medical and scientific accuracy as well as intellectual property considerations.

Competing interests disclosure

The authors have no competing interests or relevant affiliations with any organization or entity with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.