Collagen in the lungs

Oops managed to post twice - clearly too tired for this!

[KateMoss]

Can you elaborate??

Kate

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Nooooooooo! It posted twice and the wrong one's been deleted...

Haven't got time to get into as much detail but does anyone have any ideas about how having faulty collagen might affect the lungs? I have a genetic defect that means I don't make collagen properly - Ehlers-Danlos Syndrome.

Despite 10 years of specialist respiratory care, there's still no definitive explanation for my lung problems. My asthma is not entirely typical as I don't swell enough to audibly wheeze (don't swell generally despite frequent joint injuries including dislocations) but it's still definitely there! I also have frequent infections and when these are not being aggressively treated I have a really horrible productive cough all the time.

What effect would laxity of the lung tissue have? Might it result in above average maximum lung capacity (very, very occasionally - I rarely hit my best...) but at the same time make breathing that little bit harder? I don't really understand enough to work it out - I was hoping someone out there might...

I'm seeing my latest consultant for the second time next week so it'd be good to have some ideas to throw back if they try to say there's not a problem...

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Hi sparkles,

I'm sorry to hear that you've got Ehlers-Danlos syndrome as well as problematic asthma. It's a very interesting question that you ask, and I'm afraid I don't really know the answer. I've come across one or two people with Ehlers-Danlos, but I don't recall them having respiratory problems.

In terms of your frequent infections and your constant productive cough, have you had a CT scan to exclude bronchiectasis? This is thickening and scarring of the lungs which can make infection much more likely. Your doctors may also want to check out your immunoglobulin levels, which are part of your immune system, to see if you have an immunoglobulin deficiency which is making you more prone to infection.

Hope you get some answers soon.

Take care

Em H

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Thanks Em.

Before my last consultant decided I really was making it up he did a fair amount of testing (although only after my GP wrote a very, very angry letter and practically forced him to...)

Anyways here's the results. CT was clear. Cilial biopsy was fine. It's not mild CF. I'm not hyperventilating. Not sure what my blood results showed but something did show up at one point. No idea what that was as he never explained anything!

I've asked my new consultant to get the scan double checked. I had whooping cough and had problems from that before I was asthmatic. As I have fragile tissues from the EDS it's even more possible that I've got some underlying lung damage. I'm also concerned that as my infections get more severe (keep getting into pneumonia territory...) the more chance there is of (further) permanent damage to my lungs. Infection almost always sits in the same spot...

Anyone else got any insight?

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After being diagnosed with hypermobility and reading up on it, I came across this:

Management of the joint hypermobility syndrome: the rheumatologist's approach

from Howard A Bird MD, Clinical Pharmacology Unit, University of Leeds, Leeds, UK

""The increased frequency of asthma likely reflects altered collagen structure in the lungs.""

This is the URL doctor.medscape.com/viewart...

I've not managed to find any more detailed info but it looks like there is a connection.