Pulmonary fibrosis treatment options

Preparing for your lung fibrosis treatment

Initiating treatment for pulmonary fibrosis can be daunting, yet thorough preparation plays a crucial role in effectively managing the process. Ensure each medical appointment is attended with a strategic approach. The presence of a friend or family member during these visits not only provides emotional backing, but also assists in remembering vital information shared by healthcare providers. Utilising resources, such as the Pulmonary Fibrosis Patient Passport checklist, designed specifically by Asthma and Lung UK for those with pulmonary fibrosis, is beneficial to compile your thoughts and confirm that all essential care points are covered.

Being prepared to address questions from healthcare practitioners improves the quality of the consultation and allows sufficient time to discuss key issues. This helps individuals make well-informed decisions about their health journey and gain a clearer understanding of what lies ahead.

Understanding medications

Treatment for pulmonary fibrosis, specifically idiopathic pulmonary fibrosis (IPF), focuses predominantly on limiting or slowing down the scarring (fibrosis) within the lungs.

The chief medications used are pirfenidone and nintedanib, both of which have demonstrated efficacy in decelerating fibrosis advancement in IPF. Although these drugs do not halt or reverse lung scarring, they play an essential role in improving life expectancy and preventing exacerbations, thereby significantly enhancing patients’ lives.

However, they may have side effects which should be balanced against their benefits.

Pirfenidone

Pirfenidone works by reducing the process of scarring (fibrosis) in the lungs. It does this by blocking certain chemicals in the body that cause inflammation and tissue scarring. This helps slow down the progression of the disease and can help preserve lung function for a longer time.

Main Side Effects of Pirfenidone:

1. Gastrointestinal issues: This includes nausea, diarrhoea and an upset stomach.

2. Skin reactions: Many people experience sensitivity to sunlight (photosensitivity), which can cause a rash or sunburn. It is recommended to use sunscreen and avoid direct sunlight while on pirfenidone.

3. Fatigue: Some people feel unusually tired while taking it.

4. Liver problems: In rare cases, it can affect liver function, so regular monitoring through blood tests is often required.

Nintedanib

Nintedanib works by blocking certain enzymes (called tyrosine kinases) that play a role in the process of scarring and inflammation in the lungs. By blocking these enzymes, it helps slow down the growth of scar tissue, which in turn can help preserve lung function and slow the progression of the disease.

Main Side Effects of Nintedanib:

1. Diarrhoea: This is one of the most common side effects, and some people may need to manage this with dietary changes or medication relief.

2. Nausea and vomiting: Some people feel sick or have an upset stomach.

3. Liver problems: Like pirfenidone, nintedanib can affect liver function, so regular blood tests are needed to monitor liver health.

4. Loss of appetite: Some patients may experience a decrease in appetite and unintentional weight loss.

5. High blood pressure: Nintedanib can increase blood pressure in some people, so monitoring is necessary.

Close supervision by your medical specialist team is necessary when undergoing pirfenidone or nintedanib to confirm its effectiveness and mitigate any adverse effects that may arise.

Quitting smoking

The individual is the only one who can take this action, but quitting smoking is a pivotal step in improving lung health and overall wellbeing. Smoking aggravates lung fibrosis, accelerating the disease’s progression and diminishing lung function. By quitting, they can significantly enhance both lung capabilities and general health. Various support mechanisms are available to assist with cessation, including free help from a GP or referral to an NHS stop-smoking adviser, ensuring that those attempting to quit have access to optimal support.

Quitting smoking provides substantial benefits. It not only slows the progression of lung fibrosis but also reduces the risk of developing other serious health complications. Taking this step may even improve respiratory function, reduce fatigue, and lead to an overall improvement in quality of life.

By taking this step, they can slow the worsening of the condition and manage symptoms more effectively in the long term.

Considering lung transplant

When pulmonary fibrosis progresses in spite of the above treatments, consideration may be given to a lung transplant. This is usually discussed when other therapies are unable to stop the advancement of the disease.

Several criteria must be met to qualify for a lung transplant, including good overall health status, absence or management of additional medical conditions, and appropriate body weight. Due to the risks and potential complications associated with the procedure, it is uncommon for people over 65 years old to undergo this surgery.

An extensive evaluation by a healthcare team at a specialised lung transplant centre, such as Royal Papworth Hospital in Cambridge, determines if someone is an appropriate candidate for such treatment.

After undergoing transplantation, patients will need medications indefinitely to hinder rejection of their new lungs. A lung transplant is not like changing batteries and everything is suddenly alright – it is a procedure with risks and, at the end of it, you will have changed one chronic lung disease for another, albeit we hope with a better quality of life overall.

Despite its challenges and hurdles, such as donor lung availability — which can impact individuals suffering from idiopathic pulmonary fibrosis especially hard — the opportunity for improved respiratory capability and better life quality makes lung transplants an important option in selected cases of pulmonary fibrosis.

Symptom management in pulmonary fibrosis

Managing symptoms is crucial for sustaining the quality of life in patients with lung fibrosis. The goal for treating idiopathic pulmonary fibrosis (IPF) is to alleviate symptoms and slow the advancement of the disease. Palliative care plays a significant role in controlling symptoms, such as shortness of breath, cough, fatigue and anxiety. Engaging specialists who are experts in palliative care ensures a holistic approach to symptom management.

One of the aims of Breathing Matters is to carry out research to ensure that the right treatment is given to the right patient at the right time. This means that we can avoid futile or inappropriate therapies. We call this stratified medicine or personalised medicine. At the moment, there is very little personalised medicine available for patients with lung fibrosis, but future work will focus on genetic variants that might be targeted by specific treatments. Our studies have shown that we can stratify patients using simple tools, such as the neutrophil-to-lymphocyte ratio, or NLR, that is derived from a simple full blood count test, or a more complex test, such as a nuclear medicine scan.

The hardening of lung tissue in pulmonary fibrosis is what leads to breathlessness, which severely affects routine activities. Using supplemental oxygen can enhance oxygen levels and therefore daily function. Not much is known about diet and PF, but it is thought that if you eat a healthy, antioxidant-rich diet, this may help diminish inflammation while promoting respiratory health. Read our article on staying strong through healthy eating.

Managing breathlessness

Pulmonary rehabilitation, or rehab, is an effective intervention for patients with lung fibrosis experiencing the challenging symptom of breathlessness. The primary goal of pulmonary rehab is to provide better strategies for managing your condition and to improve your ability to function normally in daily life.

These programs combine exercise training and educational components designed to enhance both lung function and overall physical wellbeing. Through customised exercise regimens, pulmonary rehabilitation works to increase breathing efficiency, reduce episodes of breathlessness, and build stamina and muscular strength. This helps make daily tasks easier to complete.

In addition, physiotherapists may recommend specific postures or body positions that can assist with easier breathing and better oxygen intake. Since pulmonary fibrosis can make even simple activities tiring, they can also teach energy conservation techniques, such as how to pace yourself during daily tasks to avoid becoming too short of breath.

It is important to note that, even people with conditions like pulmonary fibrosis or bronchiectasis, can improve their breathing. Experienced physiotherapists at UCLH have created a series of videos that provide techniques to help you enhance your breathing. You can view the videos here.

Oxygen therapy

Treatments for pulmonary fibrosis often involve oxygen therapy, which is vital for elevating the blood’s oxygen concentration and bolstering routine activities. In cases of idiopathic pulmonary fibrosis, using supplemental oxygen can improve your participation in physical activities as well as your overall quality of life.

Oxygen is administered via a mask or nasal cannula connected to a compact device, there are portable versions available to facilitate mobility. Depending on individual needs, some patients might only require this form of treatment while engaged in certain tasks, such as exercise or sleep; while continuous use may be necessary for others. This approach to care—referred to as supplemental oxygen—enables those with lung conditions like pulmonary fibrosis to sustain an active lifestyle. Many people do not want to use oxygen when out and about and this is a very personal choice which can be discussed with your medical team.

Dealing with cough

Patients suffering from lung fibrosis often have a continuous dry cough, which is both common and distressing. Cough can get worse due to conditions such as acid reflux, making it more difficult to control the intensity of the cough. By addressing root causes like acid reflux through pharmacological treatments or changes in diet, there may be a notable decrease in both how often and how severely you experience coughing. Physiotherapy can help with techniques to deal with your cough and there are medications available to suppress cough. The latter tend to be opiate based and may have side-effects, but new formulations are becoming available and are looking promising in clinical trials, for example Nalbuphine.

Improving the way that a persistent cough is handled can significantly improve quality of life whilst dealing with lung fibrosis. Employing methods like breathing exercises and utilising supportive care practices and appropriate medication provides relief from symptoms, contributes to better wellbeing.

Clinical trials and research

Participating in clinical trials grants patients access to novel treatments that are not yet available. A significant number of Phase 2 and combined Phase 2b/3 trials are actively being conducted with the aim of discovering new interventions for idiopathic pulmonary fibrosis (IPF). These studies offer optimism for improving control of IPF, particularly because there is a constant need for therapies capable of completely stopping the advancement of this disease.

Over thirty-five current studies related to idiopathic pulmonary fibrosis can be found listed on ClinicalTrials.gov. These involve active recruitment or preparations to begin soon and feature potential therapeutic agents, such as Bexotegrast, TTI-101, and BBT-877. The orphan drug status awarded by the FDA (Federal US Food and Drug Administration) to several drugs under investigation underscores their anticipated importance.

Involvement in these research initiatives aids scientific advancements which may prove advantageous to those with idiopathic pulmonary fibrosis now and in the future.

Let your clinician know if you are interested in knowing more about ongoing trials or indeed taking part.

Self-care and lifestyle adjustments

It is essential to be diligent and engaged in the management of pulmonary fibrosis. To improve quality of life, the patient and their family need to actively participate in the care regimen and understand the symptom burden. It is very important for everyone to be confident in handling shortness of breath and other associated symptoms.

Maintaining a proper diet is key in the control of your pulmonary fibrosis. A diet low in sodium and fat can aid symptom management while promoting better lung function and overall health.

Home oxygen therapy plays an important role by increasing mobility and providing critical assistance that may allow you to lead a more active life.

Staying active

It is very important to engage in regular exercise for as long as possible as this plays a crucial role in improving the life quality of individuals suffering from lung fibrosis. Programmes for pulmonary rehabilitation combine physical training and educational components to improve lung function and general body strength. Typically, these initiatives encompass exercise routines, breathing techniques and dietary recommendations.

Personalised exercise regimens will help enhance your stamina and muscular strength, and Breathing Matters is committed to understanding muscle strength and how it may be reduced in patients with pulmonary fibrosis. Read our article on sarcopenia and interstitial lung disease here.

Nutritional support

Making modifications to diet is also essential for general well-being. Keeping a healthy weight and eating a diet that is healthy, replete with antioxidants and well-balanced can diminish inflammation while strengthening the health of the lungs. Breathing Matters is looking more closely at nutrition and hope to do some work on this in the future.

Dealing effectively with a chronic cough may require tackling concurrent conditions, such as acid reflux, and relief might be found by using medications designed to suppress stomach acid in order to control symptoms of GERD (gastroesophageal reflux disease), such as proton pump inhibitors. There is a clinical trial, TIPAL, that is looking at whether routine use of these drugs can improve life quality and life expectancy in idiopathic pulmonary fibrosis.

Emotional wellbeing

Emotional distress, such as anxiety, depression and stress can arise in patients with lung fibrosis due to the impact of lung disease on your wellbeing. To address these emotional struggles, employing coping mechanisms and seeking mental health assistance is beneficial. Please talk to your medical team about this.

Engagement in support groups offers solace by giving individuals a platform to exchange stories and receive encouragement from those dealing with comparable hurdles. Check out support groups from Asthma and Lung UK and Action for Pulmonary Fibrosis.

It is essential that, if you are experiencing the psychological impact of lung fibrosis, you also focus on your mental health in order to manage these challenges effectively.

Palliative care and end-of-life planning

The objective of palliative care is to improve the quality of life for patients by easing the symptoms and stress that come with severe diseases, including pulmonary fibrosis. This type of care concentrates on providing comfort and managing symptoms when treatment options can not cure the disease.

It is essential that preferences are taken into account when designing a palliative care plan, which should be developed in conjunction with healthcare providers. Early integration of palliative care can offer robust support from experts in symptom management.

Everyone will eventually face death, and it’s beneficial for each of us to consider where we would prefer to die, who we want to have by our side and what kind of supportive care we would like to receive. Are there things that we want to do beforehand, do we dream of taking our grandchildren to Disney or taking our dog for one last walk? Think carefully of what a good day and a good life means and share this information with the clinical team, so that they can help fulfil those dreams.