What’s the biggest risk to an Alpha’s lung health? Cigarette smoke. It’s not good for anyone, but it’s especially bad for Alphas – adults and children. Smoking and secondhand smoke damage your lungs, along with your heart and other organs. And the damage happens even faster when you have Alpha-1. The good news is that you can quit. Read on to learn more.

First among the controllable risk factors associated with development of lung disease in Alpha-1 is exposure to cigarette smoke. Cigarette smoke has been undeniably shown to exert the greatest risk.

For individuals with Alpha-1, smoking is the greatest risk to your health, leading to the development of lung disease and premature death.

For the smoker, inhaling cigarette smoke is known to accelerate the destructive processes at work in the lungs and puts the development of lung disease on the “fast-track.” For Alphas who smoke, that fast-track becomes an “express train.” This train begins rolling faster after 100 cigarettes have been smoked in a lifetime.

Secondhand smoking is defined as exposure to tobacco products generated by others. Large population-based studies have shown that secondhand smoke increases the risk of respiratory infections, particularly in children. In addition, children of smokers appear to have a much higher incidence of asthma than children of non-smokers. Although there aren’t any studies that show a direct impact of secondhand smoke in Alphas, the linkage of secondhand smoking with more respiratory infections implies that secondhand smoke is almost certainly of harm to Alphas. This impact is expected to be more important in childhood.

If your child is either an Alpha or an Alpha-1 carrier, the risk of developing lung disease later in life will be increased by exposure to secondhand smoke. The risks associated with short durations of smoking and small numbers of cigarettes, especially in the presence of Alpha-1, are clear and profound.

Although cigarette smoking is recognized as a controllable risk factor, it is clear that, in many cases, it is not an easily avoidable one. Most individuals with Alpha-1-related lung disease are not identified until they have already become smokers and are addicted to nicotine or are former smokers. Some Alphas live with smokers who are unwilling to quit. Although some Alphas never take another puff of a cigarette after they are diagnosed, this is certainly not always the case. It is important to understand that dealing with a cigarette addiction, as with any addiction, may be very difficult. Successful change takes knowledge, help from others and a long-term commitment to health.

The facts about smoking

Nearly 500,000 people in the United States die each year from smoking cigarettes. Smoking is a well-known cause of cancer, stroke, heart disease, COPD and peripheral vascular disease, and exerts harmful effects on many other systems in the body. Recent long-term studies indicate that approximately one-half of all regular cigarette smokers will eventually die from their addiction.

Since having Alpha-1 already puts you at an increased risk for developing lung disease, the damage inflicted by smoking will almost guarantee it. Therefore, your first priority for managing the risk factors associated with developing lung disease in Alpha-1 should be the elimination of exposure to all forms of tobacco smoke.

The effects of smoking in Alpha-1 Antitrypsin Deficiency

Smoking affects every part of the body. Cigarette smokers inhale about 7,000 chemicals that include over 400 toxins and 69 known carcinogens every time they puff. As smoke enters the respiratory tree and lungs, it causes irritation and triggers inflammation. This inflammation causes the body’s defenses to send white blood cells to the area. While performing their normal function, the white blood cells release powerful enzymes, one of which is neutrophil elastase. Neutrophil elastase is destructive to unprotected lung tissue. As you may have already learned, alpha-1 antitrypsin (AAT) is the protein in our bodies that provides protection to the lungs by neutralizing this powerful enzyme before it can attack normal lung tissue.

We know the chemicals created from burning tobacco destroy alpha-1 antitrypsin’s ability to block neutrophil elastase. In fact, a single cigarette destroys most of the alpha-1 antitrypsin in normal lungs. Because each cigarette smoked results in a repeated episode of irritation and inflammation, this process may therefore account for a significant portion of the lung disease seen even in non-Alphas. Among individuals with normal AAT levels, this damaging process generally occurs gradually. The symptoms of lung disease in these individuals, if they occur, tend to develop after the age of 50 or 60.

For Alphas, where protection against neutrophil elastase is already compromised by reduced AAT levels, the exposure to cigarette smoke increases the risk that lung damage will occur and will result in significant symptoms of lung disease. As opposed to “normal” individuals, this damaging process is accelerated in Alphas and their symptoms may develop as early as in their 20s.

In view of the relationship between smoking and AAT levels, individuals preparing to receive augmentation therapy for Alpha-1 should be smoke-free and successful in maintaining smoking cessation before therapy is initiated. This makes sense if you remember that “augmentation” therapy is simply giving you a “boost” of AAT. If you smoke, the AAT boost will be destroyed just as your own AAT is destroyed by cigarette smoke.

While almost everyone is aware of the links between smoking and heart and lung disease, few people think about the effects of smoking on other parts of the body. When the various toxins and products of cigarette smoke enter the lungs, they subsequently enter the bloodstream, where they circulate to the remainder of the body. One of the liver’s primary functions is the processing of drugs, alcohol, chemicals, and other toxins to remove them from the body. Evidence suggests that smoking alters the ability of the liver to handle and “detoxify” such substances. Some research also suggests smoking can aggravate the course of liver disease caused by excessive alcohol intake or hepatitis. Smoking can also increase the risk of liver cancer. Despite this damage, additional research has shown that the harmful effects of smoking on the liver are temporary and that most problems can be reversed when the individual stops smoking.

E-Cigarettes Are Not a Solution

E-cigarettes contain nicotine in a carrier solution that is heated and inhaled into the lung. At the current time, these carriers remain different between vendors and contain a variety of flavorings, propylene glycol, vegetable glycerin, and other ingredients. In 2016, the FDA was given authority to regulate these products and assure that they are as safe as possible. Just the same, at this writing, the ingredients of many e-cigarette cartridges have not been fully disclosed.

Since e-cigarettes contain nicotine, which is highly addictive and can harm fetuses and adolescent brain development, how the FDA will manage this responsibility is not clear.

Learn more about the risks of cigarette smoke and find ways to quit when you visit the Big Fat Reference Guide (BFRG) via bfrg.alphanet.org or the Subscriber Portal