Overview
Idiopathic Pulmonary Fibrosis (IPF) is a progressive lung condition which causes scarring of the tissues (fibrosis) around the air sacs in the lungs. This scarring reduces the efficiency of oxygen and carbon dioxide exchange within the lungs and causes difficulty in breathing.
Causes
Who is Likely to Get IPF?
The exact cause of IPF is unknown. However, the risk of IPF is greater for:
- People who smoke or used to smoke
- People who are above the age of 50 years old
- Members of the same family (occasionally)
In the early stages of IPF, patients may experience mild or no symptoms. However, symptoms may worsen with increased scarring of lungs.
Symptoms of IPF are not unique to this disease. While the symptoms may be different for each IPF patient, common symptoms associated with IPF include:
- Breathlessness, particularly when climbing up the stairs or walking uphill
- Persistent dry cough
- Tiredness
Detection & Treatment
How is IPF Diagnosed?
Your doctor may ask you about your existing medical conditions, medications and occupation. He or she may also check for abnormal breathing sounds using a stethoscope.
If you are suspected of having IPF, the following tests may be done:
- Blood Test: To exclude other possible causes of lung scarring
- Breathing/Lung Function Test: To measure how well your lungs are working
- Computer Tomography (CT) Scan: To obtain a detailed imaging of your chest
- Bronchoscopy: To obtain cell samples from your lungs
- Lung Biopsy: To obtain tissue samples from your lungs
How is IPF Treated?
You can play your part through the following:
- Quit smoking to prevent further damage to your lungs to ensure effectiveness of your medications.
- Attend pulmonary rehabilitation sessions to learn how to manage your symptoms and improve your physical strength.
- Stay active and healthy by working out, eating well, and maintaining social activities and hobbies.
- Get vaccinated to prevent infections from affecting your lungs.
- Seek help if you are facing emotional or financial difficulties. Speak to your doctor for referrals to support services (e.g. medical social workers).
Your doctor may prescribe the following treatments:
Medications that may help slow down lung damage:
- Nintedanib
- Pirfenidone
- Oxygen therapy to be used when you are feeling breathless, or throughout the day, depending on your condition.
- Lung transplantation to replace one or both of your lungs with a healthy one from a donor. If this is an option, you will be referred to a transplant centre for evaluation.
Care Management
Palliative Care and Advanced Planning
Palliative care is care for patients with moderate to severe symptoms that significantly affect their well-being.
Palliative care aims to improve a patient's quality of life by managing thier symptoms such as breathlessness, cough, and fatigue. This will allow people to continue leading meaningful lives and it also provides support to family members and caregivers.
Depending on your condition, your doctor may refer you to a multi-disciplinary team that specialises in providing palliative care.
Advanced Care Planning (ACP) involves discussing your personal values, beliefs, and care preferences with your family and healthcare professionals. ACP allows you to give instructions regarding the type of treatment you may or may not want, should you face a critical illness. It would relieve your family members from making difficult decisions for you, should you encounter a medical emergency.
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