What happens in the lungs and liver?

Alpha1 Germany eV.

The lungs are an organ you need to breathe, or inhale air. The lungs consist of many tubes filled with air. These branch out and become progressively smaller until they are just tiny air sacs (alveoli).

The following happens when you inhale/take in air:

• Air flows into the lungs through the nose, mouth, and trachea.
• Air contains oxygen.
• Oxygen enters the blood through the air bubbles.
• The blood then carries the oxygen to all organs in your body (vital nourishment for the whole body).
• At the same time, carbon dioxide („waste“ from the „used oxygen“) is released from the blood into the air bubbles, which the body no longer needs.
• The carbon dioxide is then exhaled.

The liver is an organ with various functions; for example, the liver is the body's "purification plant":

• The body constantly produces toxic substances (waste).
• Some toxic substances also come from external sources, such as alcohol and medications.
• The body cannot use these toxic substances.
• The liver breaks them down, rendering them harmless so that they can be excreted from the body, for example in urine.

In addition, the liver also produces its own substances, such as bile.

Alpha1-antitrypsin is also produced in the liver.

What does the liver have to do with the lungs?

• The liver releases the alpha1-antitrypsin it produces into the bloodstream.
• Alpha1-antitrypsin flows through the bloodstream throughout the entire body and into/through all organs.
• Alpha1-antitrypsin also reaches the lungs via the bloodstream.
• At the same time, air also enters the lungs.
• Unfortunately, the air we breathe doesn't only contain "good" things like oxygen. It also carries substances into our lungs that can damage them, such as cigarette smoke, very fine dust, and pathogens.
• Alpha-1 antitrypsin is a great "protector" of the lungs. It ensures that the lungs remain healthy, even when pollutants such as smoke enter them.

What is different if you have an Alpha1 antitrypsin deficiency?

• The liver produces alpha-1 antitrypsin.
• If you have an alpha-1 antitrypsin deficiency, your alpha-1 antitrypsin will look different. Individual "alpha-1 antitrypsin 'bricks'" fit together nicely, like Lego bricks. They practically interlock, just like when you assemble several Lego bricks.
• The liver has difficulty releasing these aggregated "alpha-1 antitrypsin stones" into the bloodstream. As a result, some of them simply remain in the liver, and only a small portion makes it into the blood.
• Alpha-1 antitrypsin is then lacking in the blood, hence the name alpha-1 antitrypsin deficiency.

And what happens then?

• Clustered "alpha-1 antitrypsin stones" remain in the liver. This makes it harder for the liver to function properly.
• Because there is less alpha1-antitrypsin in the blood, less of it reaches the lungs via the bloodstream.
• The lungs therefore have to cope with fewer "protectors" and are less protected against harmful substances such as smoke.
• Because the protective barrier is missing, the harmful substances can gradually damage the lungs over many years.

And now?

To stay healthy for a long time, you need to take a little care of your liver and lungs. It's not difficult at all; you just need to keep the following in mind:

• Very important: You must protect your own lungs.

Don't smoke, and ask smokers not to smoke when you are there.

• Don't drink alcohol, then your liver won't have to work so hard.
• Discuss with your doctor which vaccinations against diseases are advisable.
• Discuss all medications you intend to take with your doctor.
• Discuss with your doctor when or how often he needs to check whether your lungs and liver are working properly.

By the way:

• You can't help having an Alpha1 antitrypsin deficiency.
• Alpha1 antitrypsin deficiency is inherited; you have had it since birth.
• Alpha1 antitrypsin deficiency is not contagious.