Friday, January 23, 2009

Laryngomalacia, Tracheomalacia & Down syndrome

The day of the pediatric flexible bronchoscopy went something like this: register at 8:25am, go to 3rd floor and wait 3 minutes for room assignment on 2nd floor, get room (closet sized) and wait over an hour for the numbing cream for both hands and wrists for placing the IV, wait for scope to be cleaned from previous patient, sit in dark room (closet) and try to get Sophie to sleep as she hasn't eaten since 10:45pm the previous night, converse with Cathy, update Twitter (geek), finally go to the procedure room and calm daughter down as they place the IV, BP cuff, O2, EKG wires, watch as doc pushes Versaid and Ketamine, get sent back to room (closet) to wait - 1 hour and a half after we were originally scheduled to begin (not bitter), get report and photos from Dr. Lung MUCH quicker than anticipated, comfort sedated girl as she wakes up, feed and dress her, home by 2, nap till 4:30, still slightly goofy -but good.

So what did we learn? Sophia has Laryngomalacia (la-Ringo-mal-A-sha) and Tracheomalacia. The cartilage of her voice box is soft and floppy as is part of her windpipe. As hypotonia (low muscle tone and floppiness) commonly goes along with Ds, this is no surprise. The sound called stridor (upon inhale) and cough that sounds like a bark or like croup (on exhale) is due to the soft tissues. Just like with croup, colds can cause inflammation and the slightest bit of swelling reduces the size of her airways enough to be problematic and noisy. However, unlike with croup, steroids will not help. The only treatment is comfort measures, Dr. Lung prefers cold vapor vs. steam, sitting up, getting older and growing bigger (AKA time). Typically this appears in younger babies and goes away on its own by 18-24 months, but in the case of children with Ds, it can take a little longer for the airways to mature and stiffen.

So, I take it as good news, albeit frustrating, that we basically just have to live with it until she gets older and cold/flu season passes. I was able to get a nebulizer with just saline and a neat pediatric sized mask with a purple dinosaur on it. I had to persuade her ped for this as both he and Dr. Lung feel that its just a semi-expensive vaporizer. Since we have 2 vaporizers in her room as it is and nothing else will help her condition (except 'shock the monkey' - dance outside in the cold air), I insisted we have it on hand. I heard from both docs about how there is no evidence that saline would provide anything more than temporary relief. I (and the other mom's in-the-know) am happy to have it as an option when it gets really bad in the middle of the night.

Here are some photos of the day including from the actual bronchoscopy. As always, click on the pictures to enlarge. If you are a visitor (or anyone really), please request permission to use the bronchoscopy photos by leaving contact information in comments.

Thanks for the good thoughts and prayers everyone. As for now, I'm removing twitter from the sidebar and going to bed.

Funny girl in purple hospital diggs.
She was pretty happy until her hunger increased while waiting.
After the procedure, getting foot IV removed in our room. Still very 'out of it.'

Coming out of sedation with her daddy, in her 'streets.'

Photos from Sophie's bronchoscopy.


Bethany said...

Oh no. I wonder how common this is in our kids actually. I guess you'd never know unless you did the tests. Interesting! I hope Sophie grows out of it fast!

Michelle said...

Well, I'm glad the testing went well, but too bad they can't do more for her... hopefully winter will be short (Maybe?) and better weather will help, too?

Lacey said...

I just found your blog through Sarahs. your kidos are so cute, and I'm glad the procedures went smooth. Lacey and Jax

tawanda28 said...

As a mom...I know what you go through with your daughter. I have a 17 yr. old with dS and he has tracheomalacia since he was born. Things that could cause other kids to get a cold can cause our kid's airway to swell and get really sick. Our insurance just bought our boy a hospital to keep him upright at night and that has helped. I will say a pray for your daughter at night too...the pictures on your sight are adorable! They grow up soo fast! Have a good day! Blessings, Cory

Amy said...

I am a physician assistant student working on a report on tracheomalacia. Your images are fantastic. Would you mind if I shared them with my fellow students? Please contact me via email

Anonymous said...

My son is now 5 years and still is struggling with laryghomalacia. I have several sleepless nights nabulising wilth saline relives him a lot - temporary as they may say but it is an option for us mothers with children with DS. I wish your daughter well. Doreen

Paul and Gayle said...

As a new mom of a Colby with DS, we have been experiencing some breathing problems more pronpouced when feeding and with congestion. Went for a swallow study and they questioned the possibility of tracheomalacia. Colby was doing better and now seems to e worse again, so back to the Doctor to discover if he truly has tracheomalacia. Glad they grow out of it but hope it's sooner than 18 months (he is only 3 months)!

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