Alpha-1 Lung Disease
Not everyone who has A1AD will develop lung problems and researchers are still trying to understand why some people do and some don’t. Factors are smoking, lung infections and workplace exposures to dust and fumes.
When to Suspect Alpha-1 Lung Disease
Alpha-1 Lung Disease should be suspected in people who have early onset Chronic Obstructive Pulmonary Disease (COPD); that is, in people in their forties or younger or in people with COPD who have not smoked or have smoked very little. It should also be suspected if there is a certain pattern of emphysema that shows up on a chest x-ray – basilar emphysema (emphysema in the bottom of the lungs) or a family history of emphysema. Alpha-1 should also be considered in asthma that’s tough to treat particularly if the lung function doesn’t return to normal with treatment. Recurring respiratory infections might be a sign of a different sort of Alpha-1 lung damage known to doctors as bronchiectasis.
How does the lung problem happen?
Imagine the lungs as a country. An enemy (cigarette smoke, including second-hand smoke, infection, or a variety of chemical or environmental pollutants) invades this country. These invaders are immediately detected by the home guard, otherwise known as white blood cells that spring automatically into action. The white blood cells release an enzyme, called neutrophil elastase (NE), which storms into battle. It has one mission and that is to destroy anything in its way – cigarette smoke, infection, etc. This is the good news. The bad news is that it doesn’t know when to stop. Once it has destroyed all enemy invaders, it will begin to attack and destroy healthy lung tissue.
It is the job of Alpha-1 Antitrypsin (AAT) to stop, or inactivate, the NE when it has done its job. People with Alpha-1 do not have enough AAT to protect them from the destructive tendencies of NE. It seems strange that NE, something that was initially so helpful, can become extremely harmful without proper controls. That proper control is sufficient AAT.