About Lung Fibrosis

The first symptom you may notice is shortness of breath especially when you are climbing the stairs, walking, exercising or taking part in physical activity. Your breathlessness can worsen as your disease progresses. Other symptoms can include a dry cough, low energy and fatigue, changes in the shape of your fingertips (they may get bigger and more rounded – this is called ‘clubbing’), stomach acid and reflux (indigestion). These are the most common symptoms and it’s important to note that not everyone gets all of the symptoms. Some ILD’s like IPF are progressive which means symptoms get worse over time. The rate of disease progression is different for everyone though and can be improved with medication, other therapies, and lifestyle choices. Some patients remain stable with a gradual change in symptoms over many years. Other patients have a steady decline in their health and their symptoms get worse. Some patients may experience a sudden and serious worsening of their symptoms. This is called an acute exacerbation.

It can be challenging for doctors to know what causes lung fibrosis. It’s believed that anywhere from 3 to 20% of people with the disease can have another family member with the disease, but that doesn’t mean there is a genetic cause. The most common form of lung fibrosis is Idiopathic Pulmonary Fibrosis or IPF. Idiopathic means there is no known cause and a diagnosis of IPF is generally made after all other potential causes are ruled out. There are five main categories of identifiable causes of lung fibrosis. 

• Drug-Induced: which includes medications such as amiodarone (a heart medicine), nitrofurantoin (an antibiotic), and methotrexate, bleomycin and bisulfan (all used for chemotherapy). 

• Radiation-Induced: radiation treatment to the chest. 

• Occupational: breathing in dust, fibres, or fumes in the workplace that contain tiny particles (such as asbestos, silica, wood, metal, or textiles). 

• Environmental: breathing in air or dust contaminated with bacteria, viruses or animal material such as bird droppings 

• Autoimmune: medical conditions that affect the joints and connective tissue such as arthritis, scleroderma or sarcoidosis. 

IPF, in particular, is linked to smoking and so if you currently smoke, you should stop. Help can be found on the HSE Smoking Cessation page: https://www2.hse.ie/living-well/quit-smoking/

Your doctor will review your medical and family history and discuss your symptoms with you. You may be asked about any continuous or repeated contact with dusts, gases, chemicals or similar substances.  

Your doctor will also examine your lungs by listening with a stethoscope. During this, they may detect sounds known as “crackles,” or “rales,” which resemble the noise of Velcro being pulled apart. 

Your doctor may perform an additional breathing test called a pulmonary function test to measure how much air your lungs can hold. Scar tissue and inflammation can make your lung stiff which means you have to work extra hard to breathe. Your brain senses this extra work and lets you know there’s a problem by triggering a feeling of breathlessness. The more scar tissue, the less air the lungs can hold. 

Scar tissue also blocks the movement of oxygen from the inside of your air sacs into your bloodstream. This reduces your oxygen level, particularly during exertion or exercise. Oxygen levels can be assessed to see if they decrease after walking by using a six-minute walk test. 

These tests will provide clues but if pulmonary fibrosis is suspected then you should be referred to a specialist centre for additional imaging (X-ray or High Resolution CT scan), lung function tests, or a biopsy. 

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 lung fibrosis, including medications, oxygen therapy, supportive strategies such as exercise and nutrition, and, in some cases, lung transplantation. 

Here are a few 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 for lung fibrosis is often specific for the type of fibrosis the patient has. Some common therapies include:

• Antifibrotics – Pirfenidone (Esbriet) or Nintedanib (Ofev) may help slow the progression and may prevent bouts when symptoms suddenly get worse. 
• Steroids, also called corticosteroids, are 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 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. 

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. 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. Ask your doctor or physiotherapist if there is a pulmonary rehabilitation programme available. ILFA provides online exercise classes as well free to all members. Contact us for more information.

 You may also be prescribed medicine to help with your cough, acid reflux (excess stomach acid), antibiotics, steroids, calcium supplements, anti-depressants and steroids depending on your symptoms. Monitoring of disease progression is very important and you may need to have regular tests and investigations.

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.

ABGs, Arterial Blood Gases: Blood tests that measure the amount of oxygen and carbon dioxide in the blood.

Activities of Daily Living: A term used to describe the functional status of a person and how they cope with mobility, dressing, feeding, personal hygiene and getting dressed, and independent living.

Aetiology: The causes or origin of a disease.

Alveoli: Tiny air sacs found in the lung. Alveoli have a thin membrane that facilitiates the movement of oxygen from the lung to the bloodstream.

Biopsy: The surgical removal of a small piece of tissue for examination undera microsope.

Bronchoscopy: A test to view the airways and diagnose lung disease.

Clubbing: An abnormal enlargement of the fingertips or toes.

Corticosteroids, Steroids: A type of anti-inflammatory medicine.

Crypotogenic Fibrosing Alveolitis, Idiopathic Pulmonary Fibrosis, Lung Fibrosis, IPF: Scarring or thickening of the lungs without a known cause.

CT scan, Computed Axial Tomography (CAT) scan: A series of high resolution and detailed x-rays  taken to create cross-sectional pictures of the body.

Cyanosis: The skin may be discoloured blue due to a lack of oxygen in the blood.

Dyspnoea: Shortness of breath, breathlessness.

ECG, Electrocardiogram: This is a diagnostic test that records the electrical activity of the heart (the rate and regularity of heartbeats).

Electrocardiogram, ECG: This is a diagnostic test that records the electrical activity of the heart (the rate and regularity of heartbeats).

Fibrosis: Scarring or thickening.

Hypertension: A rise in blood pressure.

Idiopathic: Of no known cause or origin. Unknown cause.

Idiopathic Pulmonary Fibrosis, Crypotogenic Fibrosing Alveolitis, Lung Fibrosis: Scarring or thickening of the lungs without a known cause.

Lung Fibrosis, Idiopathic Pulmonary Fibrosis, Crypotogenic Fibrosing Alveolitis: Scarring or thickening of the lungs without a known cause.

Oedema: Swelling due to fluid retention.

Pneumonia: A type of lung infection caused by bacteria, viruses, or fungi.

Pulmonary: Relating to the lungs.

Pulmonary Function Tests, PFT, Lung Function Tests, Spirometry: This is a set of tests that measure how well the lungs work by measuring the volume of air taken in and released and how well oxygen moves into the blood.

Pulmonary Hypertension: High blood pressure in the arteries of the lungs due to narrowing of the small arteries in the lung.

Respiration: Breathing.

Respiratory: Relating to the lungs.

Spirometry, Lung function tests, Pulmonary Function Tests: This is a set of tests that measure how well the lungs work by measuring the volume of air taken in and released and how well oxygen moves into the blood.

Steroids, Corticosteroids: A type of anti-inflammatory medicine.