Lung Fibrosis Treatments

Although there have been significant advances in recent years, including the development of new and promising medications, current treatments may help relieve symptoms and slow disease progression, but they cannot reverse lung damage or cure the condition. 

There are several approaches to managing pulmonary fibrosis, including medications, oxygen therapy, supportive strategies such as exercise and nutrition, and, in some cases, lung transplantation. 

The following information provides a general overview of some of the common treatments that doctors offer pulmonary fibrosis patients. This information is not medical advice. Some treatments may be right for some people, but not for others, and no one treatment is right for everyone. You should speak with your doctor before starting, changing or stopping any medical treatment. 

Drug Therapy 

Drug therapy for pulmonary fibrosis is often specific for the type of fibrosis the patient has. Some common therapies (but not all potential therapies) include: 

  • Antifibrotics – Pirfenidone (Esbriet) or Nintedanib (Ofev) may help slow the worsening of pulmonary fibrosis and may prevent bouts when symptoms suddenly get worse. 
  • Steroids, also called corticosteroids – medications that help control inflammation in the lungs by suppressing or weaking the immune system.  
  • Other medications, including those for pulmonary hypertension, a common complication with pulmonary fibrosis 

Your doctor may prescribe other medications to help you manage your symptoms and any other health problems you may have.  

Oxygen Therapy 

Your oxygen levels can be measured in different circumstances like at rest, while walking, or during sleep. If your oxygen levels are low, your doctor may prescribe supplementary oxygen.  

While it can be frustrating and inconvenient to use supplementary oxygen, it can help you manage your breathlessness and improve your quality of life. Many people report that they have less breathlessness and fatigue and are better able to live an active lifestyle when using oxygen.  

Your oxygen needs may change over time so tell your doctor, nurse or physiotherapist if you are more breathless. If you’re having problems using supplementary oxygen, have an open conversation with your doctor about your concerns. 

Pulmonary Rehabilitation 

Pulmonary rehabilitation consists of classes delivered by healthcare professionals that include exercises, breathing techniques, ways to manage anxiety and stress, nutritional counselling, education, and other supportive components. A course of rehabilitation is generally 6 – 8 weeks with two classes per week. The goal of pulmonary rehabilitation is to improve your ability to function without extreme breathlessness, to increase your exercise capacity and to improve your quality of life.  

To get the most out of pulmonary rehabilitation, it’s best to attend the entire course. It’s also important to do the exercises you learned during the course as recommended, especially once the course has finished. 

Community based pulmonary rehabilitation programmes are somewhat limited in Ireland for pulmonary fibrosis patients, but you may have access to a rehabilitation programme through your ILD Specialist Centre, or your GP might be able to refer you to a programme in your community.  

In addition, ILFA offers exercise classes several times per week and regular patient information sessions that, while not a structured pulmonary rehabilitation programme, contain many of the same elements of a structured programme.  

Lung Transplantation 

A lung transplant – surgery to remove a damaged or diseased lung and replace it with a healthy lung from a deceased donor – is the only effective treatment for people with late state pulmonary fibrosis, but this is not a suitable option for everyone. It involves a major operation and depends on disease severity and your general health.  

If you are being considered for a lung transplant, you will be extensively evaluated by the National Lung Transplant Centre in the Mater Misericordiae University Hospital in Dublin to determine whether you meet the candidacy requirements for lung transplant.  

  • To be considered a strong candidate for a lung transplant, it’s important to keep your vaccinations and overall medical care current. If you have additional health conditions alongside your lung disease, take your medications as directed, attend all follow-up appointments, and adhere to any recommended dietary plans. 
  • You must also meet weight requirements. Studies demonstrate that outcomes are better when patients are at an ideal weight, as measured by Body Mass Index (BMI). Follow nutrition and exercise guidance provided by your doctor to ensure you remain at the optimal weight and BMI. 
  • You must not use tobacco, alcohol, or any illicit drugs to be considered for transplant. Tobacco use includes nicotine replacement therapies like patches, gum and vaping.  
  • You must have a strong support system with two or three caregivers who can provide continued support during the pre-and post-transplant period.  

Even if you’re found not to be a candidate for transplant or decide not to pursue one, your health and wellbeing will be better if you follow these guidelines. 

In Ireland, lung transplant waiting times can vary, and how long a patient waits depends on the severity of their disease when they are placed on the list as well as when a suitable donor becomes available. 

If your blood type is less common, you are very tall or short, or you have high levels of antibodies against tissues received from others, it may take longer to find a suitable match. 

Being in the best possible physical shape at the time of your surgery will help with your recovery and transition to a more active life. If you are on the waiting list for a transplant you will receive pulmonary rehabilitation and be expected to continue with exercise programmes after your transplant. 

Symptom Management 

Breathlessness and cough are the major symptoms experienced by people living with pulmonary fibrosis. You may also experience fatigue, anxiety, and depression. Contact your doctor for help managing your symptoms.  

  • Breathlessness – programmes such as pulmonary rehabilitation can enhance fitness levels and provide training in breathing techniques, along with other strategies like using fans to help ease shortness of breath. Your doctor may recommend the use of supplemental oxygen as low oxygen can be a cause of breathlessness. 
  • Cough – There are many causes of cough in addition to pulmonary fibrosis. Post-nasal drip from allergies, a cold, or gastroesophageal reflux (GORD) are examples that could contribute to cough. Low oxygen levels can trigger cough in some people as well. Your doctor can help identify the cause of the cough and provide treatment. There are also special exercises you can do to help manage cough. Physiotherapists with expertise in cough suppression can teach you these. 
  • Medication Side Effects – Managing medication side effects, particularly with antifibrotics like pirfenidone and nintedanib, focuses primarily on managing gastrointestinal issues and skin sensitivity. Key strategies include taking medication with food, staying hydrated, using high-SPF sunscreen, and consulting your doctor for dose adjustments.
  • Anxiety and Depression – it’s normal for everyone to experience a range of different emotions. You might feel frightened, worried, angry or confused. You might also feel isolated or lonely at times. If you find that your mood has been low for more than a few weeks, or that anxiety is affecting your daily life, and these feelings aren’t going away, these could be signs that you have anxiety or depression. It’s important to talk to your doctor. It might also be helpful to join a pulmonary fibrosis support group where there are other people with similar experiences who can help. Learning some relaxation techniques and how to use mindfulness may also help you. Make sure you draw on the support of your healthcare team and family and friends. They’ll want to help you.  
  • Acute Exacerbation – it’s important to contact your doctor right away if you develop serious difficulty breathing, fever or flu-like symptoms, or your cough gets worse, as you may be experiencing what is called an acute exacerbation. Not everyone will experience an acute exacerbation but if you do it can be serious and you may have to go to hospital. 

A Note on Palliative Care: Palliative (or supportive) care is meant to help people living with life-limiting conditions have the best possible quality of life. It’s focused on relieving and preventing symptoms that are bothersome or distressing. Palliative care can also help you address advanced care planning, which allows you and your family to decide your goals for care as your disease progresses.   

Palliative care is not the same as hospice or end-of-life care which is generally focused on the last months of life. Hospice care is focused on comfort, dignity, and ensuring that the person’s remaining time is as peaceful as possible. It can include treatments to control pain and other symptoms and provides bereavement support for those important to the person after death.