Pulmonary fibrosis

Diagnosis

  • Physical examination
    • Initial assessment: Your healthcare provider will conduct a physical examination, review your medical and family history, and discuss your symptoms and medications.
    • Environmental exposure: Questions about exposure to chemicals, dusts, fumes, or similar materials, especially at work.
    • Listening to lungs: During the exam, the provider listens for a crackling sound at the base of the lungs, a common indicator of pulmonary fibrosis.
  • Blood tests
    • Organ function: Tests to assess the health of your kidneys and liver.
    • Screening for conditions: Blood tests to rule out or screen for other medical conditions.
    • Disease monitoring: Lab tests to monitor the progression of the condition over time.
  • Imaging tests
    • Chest X-ray: Helps identify scar tissue associated with pulmonary fibrosis.
    • Computed tomography (CT) scan: A high-resolution CT scan provides detailed images to diagnose the extent of lung damage.
    • Echocardiogram: Uses sound waves to create images and videos of the heart, measuring pressure in the arteries to the right side of the heart and lungs.
  • Lung function tests
    • Spirometry: Measures lung capacity and airflow.
    • Lung volume test: Assesses the total volume of air the lungs can hold.
    • Lung diffusion test: Evaluates the efficiency of gas exchange between the lungs and the bloodstream.
    • Pulse oximetry: Monitors blood oxygen levels with a device placed on a finger.
    • Exercise stress test: Assesses heart and lung function during physical activity on a stationary bike or treadmill.
    • Arterial blood gas test: Analyzes oxygen and carbon dioxide levels in a blood sample from a wrist artery.
  • Tissue sample
    • Surgical biopsy:
      • Video-assisted thoracoscopic surgery (VATS): A minimally invasive method using a tiny camera and surgical tools inserted through small incisions between the ribs.
      • Thoracotomy: An open surgery that involves a larger incision to access the lungs.
    • Bronchoscopy: A less invasive technique where tissue samples are taken using a bronchoscope inserted through the nose or mouth.

These diagnostic procedures help not only in diagnosing pulmonary fibrosis but also in monitoring the disease’s progression and the effectiveness of treatment over time.

Treatment

The primary aim of most treatments for pulmonary fibrosis is to enhance quality of life and alleviate symptoms. Unfortunately, the lung thickening and scarring caused by pulmonary fibrosis are irreversible, and there is currently no known medication capable of halting its progression. However, certain treatments may temporarily alleviate symptoms or slow down the rate of progression, while others can improve quality of life. The approach to treating pulmonary fibrosis depends on its underlying cause, assessed severity, and is typically determined collaboratively by healthcare providers and patients.

  • Medication: Your healthcare provider could recommend taking pirfenidone or nintedanib if you have idiopathic pulmonary fibrosis.  Additionally, nitedanib is authorized for additional forms of rapidly worsening pulmonary fibrosis. These medications may help avoid episodes in which symptoms develop quickly and may also help decrease the progression of pulmonary fibrosis.

Nausea and diarrhea are among the adverse reactions of nitedanib that might occur. Nausea, lack of appetite, and sunburn are some of the side effects of pirfenidone. Your healthcare provider will utilize routine blood tests to monitor liver function while on either medication.

In cases when you exhibit symptoms of gastroesophageal reflux disease (GERD), healthcare providers may prescribe anti-acid medications. GERD is a digestive disorder that is frequently experienced by patients who have idiopathic pulmonary fibrosis.

  • Oxygen therapy: Adding supplemental oxygen to your body allows easier breathing and exercise. It could also give you additional energy and power. This could also lessen any complications due to low blood oxygen level.

When you workout or go to sleep, you could utilize oxygen. However, certain people are always in need of oxygen. Being more mobile can be facilitated by using a portable oxygen concentrator or carrying a small oxygen tank.

An acute exacerbation is a quick worsening of symptoms. Patient may require greater oxygen support. You could require hospitalized mechanical ventilation in specific circumstances. During this procedure, a breathing assistance machine is connected to a tube that is inserted into the lungs. When symptoms suddenly worsen, your healthcare provider may prescribe antibiotics, corticosteroids, or other medications.

  • Pulmonary rehabilitation: Pulmonary rehab helps you feel better and do more every day. It includes exercises to boost your strength, breathing techniques for better oxygen use, advice on healthy eating, emotional support, and learning about your condition.
  • Lung transplant: A lung transplant replaces a healthy lung (or lungs) from a donor for one or both of the damaged lungs. It has the capacity to enhance your well-being and quality of living. Not everyone is a candidate for a lung transplant since it is a major procedure.

For certain patients with pulmonary fibrosis, a lung transplant may be a recommended. However, problems from lung transplants, such rejection and infection, can occur. You need to take medication for the remainder of your life after a lung transplant. If your healthcare provider determines that a lung transplant is the best course of action for treating your condition, you and your healthcare team may discuss about it.