Pulmonary Fibrosis

The exchange of gas takes place in our lungs. We breathe in oxygen and we breathe out carbon dioxide. Our body needs a continuous supply of fresh oxygen. Carbon dioxide must be excreted promptly to prevent an imbalance of acids in the blood that can impair brain and heart functions and cause headache, drowsiness and fatigue (Pulmonary Fibrosis Foundation 7). The air that we breathe in goes through our windpipe or trachea and into the two bronchial tubes (bronchi) or airways. The bronchioles or the thinner tubes branch out from the bronchi. The bronchioles end in a cluster of tiny air sacs called alveoli.

There are 300 million alveoli in the lungs that are surrounded by small blood vessels called capillaries and this is where the gas exchange takes place (ibid. ). The blood flows into the larger veins and carries it to our heart. Our heart pumps the oxygen-rich blood to all our body organs to maintain its normal function. Causes In pulmonary fibrosis (PF), scarring begins in the air sac walls and the spaces around them resulting in the thickness of the tissue that makes it difficult for oxygen to get into the bloodstream (National Institute of Health 1).

In most cases, pulmonary fibrosis (PF) has no known cause; hence, it is called “idiopathic pulmonary fibrosis”. The word “idiopathic” means “of unknown cause”. The Coalition for Pulmonary Fibrosis stated that PF can be linked to a particular cause, such as exposure to metal dust, wood dust, gases, or fumes; chemotherapy or radiation therapy; residual infection; or a connective tissue disease, such as systemic lupus erythematosus or rheumatoid arthritis (1).

It also has been caused by drugs or certain treatments such as antibiotics (Nitrofurantoin, Sulfasalazine), antiarrythmics (Amiodarone, Propranolol), anticonvulsants (Phenytoin), chemotherapeutic agents (Methotrexate, Bleomycin, Erbital, Oxaliplatin) and therapeutic radiation (Pulmonary Fibrosis Foundation 12). Prevalence The Pulmonary Fibrosis Foundation estimated that more than 200,000 people in the United States suffer from PF and 50,000 new cases are diagnosed annually (13).

There are at least 5,000,000 cases worldwide affecting both men and women but there is a slightly higher incidence in men (ibid. Symptoms The usual symptom is shortness of breath that may initially manifest during exercise. As the condition progress, persons with PF may be short of breath even at rest. Other symptoms include a dry hacking cough without sputum, fatigue, weight loss, aching muscles and joints, inability to fight infection, frequent tiredness, enlargement and bulb-like development of the fingertips and nails known as clubbing. In severe and prolonged cases, heart failure with swelling of the legs may occur (American Lung Association 1; Pulmonary Fibrosis Foundation 15; Schoenstadt 3).

Diagnosis Taking a patient history will determine the environmental and occupational factors, drug use, arthritis, and risk factors for diseases that affect the immune system. A physician will also listen to the sound of the lungs and examine the patient’s fingertips (American Lung Association 1). Diagnostic tests for pulmonary fibrosis include CAT scan, echocardiogram, pulmonary function test, chest x-ray, bronchoscopy, and bronchoalveolar lavage (International Scleroderma Network 1).

Treatment There is no cure for pulmonary fibrosis. The scar tissue in the lung cannot be removed surgically or with medication. Treatment will be based on the patient’s age; health and medical history; extent of the disease; medications, procedures or therapy tolerance; and preference (Pulmonary Fibrosis Foundation, 21-23). Common drug treatment includes a combination of corticosteroid, e. g. prednisone to reduce inflammation and azathioprine or cyclophosphamide to suppress the body’s immune response (Coalition for Pulmonary Fibrosis 1).

Other options such as lung transplantation, oxygen management and pulmonary rehabilitation may be considered for people with chronic lung disease. Conclusion Pulmonary fibrosis is a disease characterized by scarring throughout the lungs. Although in most cases the cause of PF is unknown, the disease may be due to medications, environmental agents, infections, chronic conditions, and exposure to radiation. A person who has shortness of breathing, cough, and diminished exercise tolerance must consult a pulmonologist to properly diagnose, rule out or treat pulmonary fibrosis or any lung diseases.

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