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-F.A.Q: What is Idiopathic Pulmonary Fibrosis?
-F.A.Q: What are the symptoms?
-F.A.Q: Who can get the disease?
-F.A.Q: How is the diagnosis made?
-F.A.Q: What is the treatment?
-F.A.Q: What about a lung transplant?
-F.A.Q: Glossary of Medical Terms --> A to C
-F.A.Q: Glossary of Medical Terms --> D to L
-F.A.Q: Glossary of Medical Terms --> O to S
-What is Idiopathic Pulmonary Fibrosis?
Idiopathic or Interstitial lung fibrosis is a scarring condition that affects the alveoli (air sacs) in the lungs, interfering with the normal function of taking oxygen out of the air and removing carbon-dioxide from the blood. Scarring of the lung tissue occurs as a result of injury to the lungs.
The most common symptom is breathlessness, particularly on exercise, which, at first, is often attributable to middle age, heart disease or asthma. The other major symptom is a dry cough. Other symptoms may include painful hands and fingers that are susceptible to the cold, and painful joints.
It can affect anyone of any age, although usually it occurs in people over 50 years. It is not contagious, and it is not a form of cancer. It is not caused by smoking, although if you do smoke, it is advisable to stop, to prevent additional lung damage. Occasionally it is familial.
Normally you will be referred to a specialist centre where a detailed medical and occupational history will be taken. Investigations are likely to include lung function tests, an exercise test, special x-rays of your lungs called C.T. scans, and another special x-ray called a D.T.P.A. scan. This measures the inflammation in the alveoli. All these investigations are painless.
It may also be necessary to do a bronchoscopy to wash some cells out of the lungs to look at them in the laboratory. This involves passing a small flexible telescope into the breathing tubes and it is done under a mild sedation.
You may be admitted to hospital for a few days to do these investigations. This is usually all that is necessary to make a diagnosis and plan appropriate treatment.
In rare circumstances it may be necessary to take a small sample of lung tissue; this is performed, by keyhole surgery, under general anaesthetic.
If, following all the investigations, the disease process is thought to be dormant, you won’t be put on any treatment but you will be followed up frequently and have lung function tests and repeat C.T. scans at regular intervals.
The aim of treatment is to prevent any further lung scarring, therefore it is essential that treatment be started early at the first signs of inflammation. The need for treatment is usually lifelong, although, once suppression has occurred treatment can be reduced.
Anti-inflamatory drugs can cause side-effects in some people and therefore a balance has to be found between side-effects and a response to treatment. People who are taking these drugs need to have their blood checked regularly to ensure they are not receiving too much treatment. Patients receiving treatment will need to be seen at regular intervals, where their response to treatment will be assessed by changes in symptoms and in some of the above tests which will be repeated.
Unfortunately, however, anti-inflammatory therapy has largely been a failure in this condition. In particular, steroids have not been shown to be beneficial and there is a major need for new more effective treatments.
For more information on the management and treatment of IPF see the Scientific Resources Section.
-What about a lung transplant?
In patients with IPF the lung becomes hardened like a dried out sponge and the lungs can't move. Once the process of fibrosis starts the progression is fairly inexorable. However, with dry lung failure, were there is no productive cough and no sputum, a single lung transplant can have a very satisfactory outcome.
The survival rate for patients who are fortunate enough to be offered a lung transplant is 90% to 95%. The outcome in terms of quality of life is superb and the average length of survival after a single lung transplant is 7 to 9 years, but it can range anywhere from 4 to 16 years.
The rate of survival depends on whether rejection occurs or not.
To be accepted for transplantation a person must have no other major health problems apart from lung failure.
The rate of transplantation depends on the amount of organs donated. In Ireland the donor rate is 22 per million, which is good, given that we have a volunteer system. Ireland has the fifth highest donation rate in Europe. Spain has the highest at 35 per million.
A donor lung has to be matched for size, blood group and it also has to be a cross match between the donor and the recipient. This is because 75% of the population have allergies and antibodies that could cause a lung to be rejected.
-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 by Occupational Therapists used to describe the functional status of a person and how they cope with mobility, dressing, feeding, personal hygiene and grooming, and independent living.
Aetiology: The causes or origin of a disease.
Alveoli: Tiny air sacs found in the lung. Alveloi enable oxygen to enter the lung.
Angiogram: Arterial Blood Gases, ABGs: Blood tests that measure the amount of oxygen and carbon dioxide in the blood.
Biopsy: The removal of a small piece of tissue for microscopic analysis.
Bronchoscopy: A test to view the airways and diagnose lung disease.
Clubbing: Term used to describe an abnormal enlargement of the base of the fingernails.
Corticosteroids, Steroids: A type of anti-inflammatory medication.
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 x-rays are 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 which can be caused by bacteria, viruses, or fungi.
Pulmonary: Relating to the lungs.
Pulmonary Functions 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 medication.