Anyone heard of Congenital lobar emphysema?

A friend's little boy has just been "maybe" diagnosed with it. They thought he had asthma for a while, then thought he had some sort of foreign body in his lung, so did exploratory surgery last week, and found nothing, except that he might have this condition which is causing the problems.

He only just turned one a couple of weeks ago, and they spent 6 days in hospital last week with him, and have now gone to Melbourne (they're in Australia) to see more specialists in the hope the docs there will know more than at the hospital they were at last week in Canberra (which doesn't have as many good specialists).

I Googled for it and the first thing I found was this:

Congenital lobar emphysema (CLE) is a potentially reversible though possibly life-threatening cause of respiratory distress in the neonate.

Click to expand...

It sounds as though it's rare to start with, and normally diagnosed at birth, rather than in 1 year olds. The article also says:

Infants with CLE who are not clinically in respiratory distress and who are able to feed and grow do not necessarily need surgery.

Click to expand...

He is able to feed, and he is growing, but he's definitely in some sort of "respiratory distress" because they thought he had asthma.

I'm struggling to find a lot of good info, and it's hard to talk with my friend since they're constantly seeing specialists, and they're staying with her parents, so if anyone knows anything about it so I can better understand what this problem is, that'd be great.

Awww, I have never heard of it...but wanted to send vibes for the little guy I hope it turns out okay

I wish i could offer some advice, but i've never heard of this condition. Please let your friend know i will keep her family in my prayers though I hope they are able to safely help the little guy!

Awwwwwww I've never heard of it either.Many prayers coming that way for him and his family.

It's pretty rare. We didn't learn about it in med school.

I did a quick PubMed search, and it seems like the severity & outcome are variable. This may depend on the location of the affected lung segment. Some infants have no symptoms or get better w/o much treatment, while others require surgery. (It seems that surgery used to be the only treatment, but now they're finding some infants can be managed more conservatively.) Also, sometimes the condition is associated with heart defects. The condition can occur spontaneously or be inherited (autosomal dominant).

Here's a link to an article that describes the condition in more detail, but it's written really for medical professionals:
http://www.emedicine.com/radio/topic188.htm

Healing vibes

for your friend's baby & kudos

to you for being a good, supportive friend.

P.S. In general what happens in emphysema is that the lung's small air sacs lose their elasticity, and therefore, cannot breathe out air properly (i.e. think of a stretched out rubber balloon -- air can go in, but the lung's air sac doesn't have that elasticity of "snap" it needs to get the air back out for proper air exchange, so the air sac gets bigger & bigger -- hyperinflation, which is not good ).

Originally Posted by whiskerynature

It's pretty rare. We didn't learn about it in med school.

I did a quick PubMed search, and it seems like the severity & outcome are variable. This may depend on the location of the affected lung segment. Some infants have no symptoms or get better w/o much treatment, while others require surgery. (It seems that surgery used to be the only treatment, but now they're finding some infants can be managed more conservatively.) Also, sometimes the condition is associated with heart defects. The condition can occur spontaneously or be inherited (autosomal dominant).

Here's a link to an article that describes the condition in more detail, but it's written really for medical professionals:
http://www.emedicine.com/radio/topic188.htm

Healing vibes

for your friend's baby & kudos

to you for being a good, supportive friend.

P.S. In general what happens in emphysema is that the lung's small air sacs lose their elasticity, and therefore, cannot breathe out air properly (i.e. think of a stretched out rubber balloon -- air can go in, but the lung's air sac doesn't have that elasticity of "snap" it needs to get the air back out for proper air exchange, so the air sac gets bigger & bigger -- hyperinflation, which is not good

).

Click to expand...

Thanks SOOOO much for the information, I really appreciate it!

I just feel so bad for them, it must be horrible when they have to go through that sort of thing when they're so little!