Actor, comedian, and humanitarian, Jerry Lewis held his 44th Jerry Lewis Muscular Dystrophy Association Telethon this Labor Day weekend, and earned over $60.5 million for the organization. Lewis shared hosting duties for the 21-1/2 hour event as he fought his own battle with a debilitating disease, pulmonary fibrosis. He was diagnosed in May 2001 when he began to complain of shortness of breath. He is being treated the steroid prednisone, which caused him to gain over 50 lbs but has slowed the progression of his disease. A number of other celebrities have also suffered from pulmonary fibrosis, including actors Marlon Brando, James Doohan (“Scotty” from Star Trek), Gordon Jump (station manager, “Arthur Carlson” in WRKP in Cincinati) and singers Robert Goulet and Odetta.
The Respiratory System:
Figure A shows the location of the respiratory
structures in the body. Figure B is an enlarged image of airways,
alveoli, and the capillaries. Figure C shows the location of gas
exchange between the capillaries and alveoli. (Source: National Heart, Lung, and Blood Institute)
The function of the respiratory system (otherwise known as the pulmonary system) is to bring oxygen from the air into the blood stream and to release carbon dioxide (a product of the body’s metabolism) back into the air. Air drawn into the lungs is drawn into smaller and smaller airway tubes to the smallest units called alveoli. These tiny bubble-shaped sacs are very thin and are surrounded by the smallest blood vessels, called capillaries. This intimate relationship allows oxygen (O2) and carbon dioxide(CO2) gasses to pass freely back and forth from the airways to the blood system.
Pulmonary fibrosis (PF) is a disease where inflammation in the lungs causes thickening and scarring (fibrosis) of the delicate lung tissue. This lung tissue is no longer able to exchange oxygen. The primary symptoms are shortness of breath and dry, hacking cough, although with time, fatigue, weight loss, and rapid shallow breathing may appear. Although some causes of PF are known- environment exposure to silica and metal dusts, cigarette smoking, certain viral infections and chemotherapy drugs- most cases have no obvious cause and are called idiopathic pulmonary fibrosis (IPF). IPF is a progressive, irreversible disease, although there are some treatment modalities that can slow progression or improve symptoms. These include anti-inflammatory drugs such as prednisone, oxygen therapy (periods of continuous oxygen use by nasal prongs or mask), pulmonary therapy (exercises to increase stamina) and in some cases, lung transplantation.
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