Pulmonary Fibrosis

1 What is Pulmonary Fibrosis?

Pulmonary fibrosis is a disorder in which lung tissue becomes  scarred and thickened. This makes breathing difficult because the lungs are unable to expand as a person breaths in. The damage of the lung tissue is irreversible a and cannot be repaired.

However,medications and therapies that relieve symptoms exist. In severe cases, a lung transplant might be recommended.

Most cases of pulmonaryfibrosis are idiopathic, meaning the exact cause is not known.

2 Symptoms

The signs and symptoms of pulmonary fibrosis include the following:

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3 Causes

The cause of pulmonary fibrosis in most cases remains unknown and these cases are called idiopathic. The damage of the lung tissue in pulmonary fibrosis can be caused by airborne toxins in the environment, some lung diseases, exposure to radiation and some medications.

Occupational and environmental factors

Long-term exposure to toxins and pollutants in the workplace, like mines or farms, can damage the lungs.

Toxins and pollutants that can cause lung damage include:

  • silica dust,
  • asbestos fibers,
  • grain dust,
  • bird and animal droppings.

Radiation therapy can also damage  the lungs.

This depends on the dose of radiation used, the duration of treatment, presence of an underlying lung disease and whether the patient is also receiving chemotherapy.

Medications that can damege the lung tissue include:

  • chemotherapy drugs like methotrexate ( Trexall) and cyclophosphamide (Cytoxan),
  • antiarrhythmic drugs like propranolol ( Inderol,Innopran) and amiodarone (Cordarone, Nexterone, Pacerone),
  • certain antibiotics like Nitrofurazone.

Other diseases like tberculosis,pneumonia, systemic lupus erythematosus and others can lead to pulmonary fibrosis.                                                                                                                                                                                                                                                                                                                                                       

4 Making a Diagnosis

The tests that can be used to diagnose pulmonary fibrosis include:

  • a physical examination,
  • imaging tests,
  • lung function tests,
  • biopsy of the lung tissues for lab analysis.

Imaging tests used include:

  • Chest X-ray that shows the scar tissue of pulmonary fibrosis and can be useful in following the course of the illness and treatment.
  • Computerized tomography (CT) scan is used to view the cross-sectional images of the lungs.
  • Echocardiogram, a sonogram for the heart that can help doctors to evaluate the amount of pressure on the right side of the heart.

Lung function tests used include:

  • Pulmonary function testing. Patients exhale quickly and forcefully into a machine that measures the amount of air the  lungs can hold and how quickly the air can move in and out of the lungs.
  • Oximetry. A small device is placed on the fingers to measure the amount of oxygen in the blood.
  • Exercise and stress test in which a patient exercises on a treadmill or stationary bike. During this period the lung function is also monitored.

A biopsy of the lung tissue can be taken by :

  • Bronchoscopy, in which a tube is passed to the lungs through the nose.
  • Bronchoalveolar lavage, a procedure in which salt water is injected through a bronchoscopy the a section of the lungs and immediately sucked out. The drawn solution contains cells from the lungs.
  • Surgical biopsy. In this invasive procedure, surgical instruments ans a small camera are inserted into the thorax through  small holes made between the ribs. General anesthesia is required in this procedure.                                                                                                                                                                                                                                                                                                                                                                                                                                                            

5 Treatment

There is no cure for pulmonary fibrosis, however, several treatments that can help to alleviate symptoms exist. They include:

  • Medications like corticosteroids (Prednisone) which are usually combined with drugs like methotrexate, which suppress the immune system.
  • Oxygen therapy, which makes breathing easier and improves the levels of oxygen in the blood.
  • Pulmonary rehabilitation which improves the daily functioning of the lungs which in turn helps patients to live satisfying lives.
  • Lung transplantation is required in severe cases of pulmonary fibrosis where other treatments have proven to be ineffective.                                                       

6 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with pulmonary fibrosis.

It is essential for people living with pulmonary fibrosis to be involved in their own treatment. Living a healthy lifestyle can also prove to be beneficial. This can be achieved by:

  • Not smoking.
  • Eating healthy foods especially enough calories since more energy is needed for breathing.
  • Getting vaccinated taking into account that some respiratory infections can aggravate pulmonary fibrosis.

Because pulmonary fibrosis worsens with time, it is important for patients to attend pulmonary rehabilitation which can help to improve the quality of life.

7 Risks and Complications

There are several risks and complications associated with pulmonary fibrosis.

Risk factors for having pulmonary fibrosis include:

  • Being an adult,
  • Smoking,
  • Working in an environment where there is a constant exposure to toxins or pollutants like mines or farms,
  • Having radiation therapy and chemotherapy for cancer treatment,
  • Having a  family history of pulmonary fibrosis since it appears to run in families.

Some of the complications that occur as a result of pulmonary fibrosis include:

  • Pulmonary hypertension. It occurs when the smallest arteries and capillaries in the lungs are compressed by scar tissue causing increased resistance to blood flow in the lungs. This will further lead to an increase in the pressure in the pulmonary arteries and the right ventricle.
  • Right-sided heart failure ( cor pulmonale). This occurs because the right ventricle has to pump harder than usual to pump blood the the lungs.
  • Respiratory failure. It occurs the lung tissue becomes so stiff that oxygen fails to diffuse into the blood. Thus the levels of oxygen can dangerously decrease.
  • Lung cancer can also occur as a result of chronic pulmonary fibrosis.
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