My late FIL had this, which was discovered when he was very ill in hospital before he died duet to a very hard to kill bacterial infection that got into his heart.
He did had bronchectasy (sp) but he apparently smoked for ?10 years (gave it up i believe around 20 years before i met DH), and they always thought it was damage from that, but it could have also been from the alpha 1, which is implicated in higher rates of lung related problems. He had no liver problems that were known of.
My DH decided to refuse genetic testing - the rest of his siblings had it done but kept their results private - but as my DS has asthma I requested the test done when he was 3 to know if it was asthma and whether the alpha 1 was implicated in making it worse. DS was negative, so although this doesn't ensure DH is (i am assuming - which is bad as my degree is in genetics and i'm just to lazy to google) that it is recessive, so he has a 50% chance that he is negative.
My readings up on it is that it is rare (although then again how many people who show persistent problems with liver or lungs are not put down to environmental influences - smoking & drinking, and actual are tested for it is another question), and even those who do have it, most have no symptoms what so ever (with genetics you can have a 'disease' but due to other genes your body really has very few symptoms of problems - even for more serious ones like Down Syndrome and Cystic Fibrosis).
You can encourage your child (which everyone should anyway) that due to their genetic loading (and we all have mutant genes, we just don't know about it yet) they should look after themselves more than usual in relation to lungs/liver, and becoming a full time smoker and drinker might cause extra problems than those vices would normally bring (not that they are pleasant in and of themselves anyway)
Was there a reason why the doctors thought to do this test?
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