Refractory

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• Norway 2012

I have Alpha-1-antitrypsin deficiency

Alpha-1-antitrypsin deficiency is an inherited disorder which usually affects the lungs.

Alpha-1-antitrypsin is a protein which is produced by the liver and enters the blood stream. Its main role is to protect the lungs from destruction by other proteins called enzymes.

Alpha-1-antitrypsin deficiency is present when there is less than the normal amount of this protein in the blood. It becomes important only when the concentration in the blood is less than 20-30% of what we would normally expect. When this deficiency occurs, the lung is poorly protected from destructive enzymes and loss of lung tissue occurs, leading to a condition called emphysema.

Once in every 2500 people in Australia has severe alpha-1-antitrypsin deficiency (levels below 20% of normal). This translates to nearly 7000 Australians with alpha-1-antitrypsin deficiency.

What genes are related to alpha-1 antitrypsin deficiency?

Mutations in the SERPINA1 gene cause alpha-1 antitrypsin deficiency. This gene provides instructions for making a protein called alpha-1 antitrypsin, which protects the body from a powerful enzyme called neutrophil elastase. Neutrophil elastase is released from white blood cells to fight infection, but it can attack normal tissues (especially the lungs) if not tightly controlled by alpha-1 antitrypsin.

Mutations in the SERPINA1 gene can lead to a shortage of alpha-1 antitrypsin or an abnormal form of the protein that cannot control neutrophil elastase. Without enough functional alpha-1 antitrypsin, neutrophil elastase destroys alveoli and causes lung disease. Abnormal alpha-1 antitrypsin can also accumulate in the liver and damage this organ.

I also have mild Neutropenia:

Neutropenia is a condition in which the number of neutrophils in the bloodstream is decreased. Neutrophils are a type of white blood cell.
These Neutrophils contain enzymes that help the cell kill and digest microorganisms . Neutropenia affects the body's ability to fight off infections.

So, perhaps my mild neutropenia compensates for the reduced alpha-1 antitrypsin?

Or maybe I just don't need to produce as much alpha-1antitrypsin, because I don't have as much of the neutrophil elastase to have to protect my lungs from?

What is interesting is that I have no problem fighting off infection/bacteria,  never get B.O. and so have never had to use deodorant. Perhaps the bacteria in my sweat is either non-existant or is killed off?