What is Idiopathic Pulmonary Fibrosis?
Idiopathic Pulmonary Fibrosis (IPF) is a progressive lung condition where fibrosis or scarring causes the alveoli (air sacs) in the lungs to thicken and harden. The fibrosis interferes with the normal transfer of oxygen from the alveoli to the blood stream and results in low oxygen levels in your blood and shortness of breath.
The word ‘Idiopathic’ means ‘unknown’. The cause for IPF has not been identified yet.
What are the symptoms?
The most common symptom is breathlessness, particularly on exertion, for example when climbing the stairs or doing gentle exercise.
Shortness of breath can develop over time and as the fibrosis develops, falling oxygen levels in the blood can lead to fatigue (extreme tiredness), low energy levels and lack of concentration.
Sometimes patients delay in seeking medical advice as they consider their breathlessness to be a side-effect of getting older or perhaps a breathing-related side effect of smoking (if they smoke now or smoked in the past).
It is important to always seek medical advice if you notice any changes to your breathing. Patients who are diagosed early, tend to have better health as they are managed better and are closely monitored by their medical team.
Other symptoms of IPF include a dry, non-productive cough that can be very debilitating for patients. In addition, some patients may have clubbing (thickening) of their finger tips, excess stomach acid (acid reflux), and pulmonary hypertension (raised blood pressure in the lungs).
As IPF progresses, the symptoms will get worse with time.
Who can get the disease?
IPF is usually diagnosed in people aged over 50 years. It is more common in men and older adults.
IPF is common in patients who have previously smoked although some IPF patients have never smoked. If patients are smoking at the time of their diagnosis, they must stop.
IPF can occur in other family members in approximately 5% of cases. The risk of family members having IPF is increased if individuals have the MUC5B gene. Genetic testing is not currently recommended.
IPF is not contagious, and it is not a form of cancer.
How is the diagnosis made?
IPF can be difficult to diagnose and a number of tests are needed to confirm if you have IPF or another lung disease.
Normally you will be referred to a specialist respiratory centre where the doctor will take a detailed medical and employment (occupational) history to determine if you were exposed to dusts that might have triggered fibrosis.
You are likely to have lung function tests, an exercise stress test, chest X-rays, and a High Resolution CT scan to help the medical team decide if you have IPF.
In some cases, patients may need to undergo a bronchoscopy to wash some cells out of the lungs to look at them under a microscope. This involves passing a small flexible telescope into the breathing tubes and it is done under mild sedation. In a few cases, patients may need to have a lung biopsy to take a small sample of lung tissue; this is performed, by keyhole surgery, under general anaesthetic. Not all patients are suitable for this surgical procedure because of their age or underlying illness. You may be admitted to hospital for a few days to have these procedures performed.
What is the treatment?
The treatment of IPF is usually managed by specialist hospital doctors who are experts in respiratory medicine. Your treatment will depend on your level of fibrosis, your symptoms and overall health. Treatment for each IPF patient will be tailored to their own needs.
- There is no cure for IPF and the damage to the lungs cannot be reversed but the anti-fibrotic drugs Pirfenidone and Nintedanib can slow down the progression of the disease.Your hospital consultant will decide if this medication is suitable for you based on your level of fibrosis and the results of your blood tests and breathing tests.
- Your doctor will prescribe oxygen therapy if the oxygen levels in your blood are low. Oxygen is as important as your prescribed medicines and will help your body cope with breathlessness and fatigue.
- Exercise and pulmonary rehabilitation classes can help improve your breathlessness, exercise tolerance and quality of life.
Ask your doctor or physiotherapist if there is a pulmonary rehabilitation programme available.
The ILFA 2000 Steps a Day exercise challenge was designed specifically for IPF patients.
- 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.A patient’s prognosis is continually changing and will depend on how they respond to treatment and exercise programmes. Monitoring of disease progression is very important and you may need to have regular tests and investigations.
The Irish Thoracic Society (ITS) published guidelines for the treatment of IPF in 2012. The ITS statement can be viewed on the ILFA website www.ilfa.ie or www.irishthoracicsociety.ie
What about a lung transplant?
In Ireland, lung transplantation is carried out at the Mater Misericordiae University Hospital in Dublin.
Some IPF patients may be suitable for lung transplantation and will need to undergo a series of tests to ensure that they meet the strict criteria for lung transplantation to proceed and also that there are no other major health problems that would exclude them from the operation. Early referral to the transplant centre for assessment is essential.
Patients may need either a single lung or two lungs (double lung transplant) and this will be determined by the medical and surgical team caring for them.
A donor lung must be a good match in relation to size, blood group and antibody profile between the donor and the recipient patient.
Patients who are fortunate enough to receive a lung transplant generally have improved quality of life and increased life expectancy. Following the operation, patients must take life long medication to prevent organ rejection.
Glossary of Medical Terms --> A to C
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.
Glossary of Medical Terms --> D to L
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.
Glossary of Medical Terms --> O to S
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.
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.