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.

Down syndrome and Windpipes

Sophie had her first meeting with our area pediatric lung specialist. He's our new ologist, pulmonologist that is. He listened to Sophie's chest as well as encouraged her to talk so that he could hear her voice. She coughed a few times and although my ear hears congestion, he still hears a bit of the bark. We played our recording of the latest battle with croup and he listened closely, calling her cough a seal bark. He spoke of stridor (the sound of the struggle to inhale).

Then we got a science lesson that went a little like this: Children with Down syndrome have midface hypoplasia, macroglossia, a narrow nasopharynx, and a shortened palate. (Translation = little face, little parts) These anatomical abnormalities along with generalized hypotonia (low muscle tone), an immature immune system, and a tendency to obesity predispose children with Down syndrome to upper airway obstruction. Gastroesophageal reflux disease (GERD) and chronic lung disease are also common in these children and may worsen airway problems. He went on to discuss the problem of sleep apnea, especially as she gets older.

Asking how Sophie's cough tends to come on, we described symptoms of a cold for a day - 1.5 days, then her cough changes from congestion to the seal bark, getting worse over the next day, croup for 3-4 ish days with a cold remaining for a week or more after croup. We discussed her meds, first a tablet, Dexamethasone 4 MG crushed, to cover 72 hours. Then Predisolone SOD PHOS 5MG/5ML SOL, 9 ML twice daily (liquid). We asked about a nebulizer with Pulmicort. We won't yet be making any decisions on treatment for future cases of croup.

Our next lesson was a mathematical formula about breathing, pressure, and radius of the windpipe that I won't even try and repeat. The message is simple; a very, very small change to the size of the windpipe can have dramatic changes to breathing. A typical adult's windpipe radius, is 16 times greater at moving air than a small child's. When you add the possibility of an already smaller than typical windpipe, anything that restricts the size can make a big difference. Inflammation from a cold virus, trunk hypotonia (that low tone business again) or 'floppiness' of the cartilage or airway itself (malacia), you can have problems. I asked if it was common to have the malacia when symptoms only present with a virus (meaning the cough only comes with a cold, not all the time) and he said, yes. My own research has lead to learning about laryngomalacia, pharyngomalacia, supraglottoplasty, and tracheotomies, but I'm getting way ahead of myself.

Dr. Lung needs to see what we're dealing with, so the next step is a pediatric flexible bronchoscopy which will be done in a week at our local hospital. I overheard him with his scheduler who informed him that he didn't have an opening next week and he said, make one. Move the conflict to my lunch hour. She's had 4 significant episodes in 2 months time.

So it goes, after midnight, no food or water. Check in at 8:30am, procedure at 10am lasting about an hour plus wake up time. Sophie will have her feet and hands numbed with a topical solution so that they can place the IV. The IV will keep her hydrated as well as deliver meds (I forgot to write down what he listed). She will be deeply sedated but not put completely out, meaning she will be doing her own breathing, some moving around or even crying with no memory. He reviewed her heart issues (small PDA a year ago and echo scheduled for next month) and is unconcerned as he cannot hear a murmur, which hopefully means it has closed on it's own. They will monitor HB, breathing, BP, O2 and give extra oxygen. They will also numb her airways.

A small instrument will go down her nose or in her mouth to look at Sophie's throat, voice box, windpipe, and smaller breathing passages of the lungs. There is no incision, this is commonly done, and is quite safe. He will be looking for any anomalies such as inhaled foreign object, tumors, and shape and size of her breathing parts. He told us to expect her waking up time to be an hour and likely she'll be sent home the same day. If she doesn't come off the sedation well or if he sees something he doesn't like, she'll stay overnight.

Once we know what we're dealing with, then we'll come up with a treatment plan. Mark's mom is coming up to hang with Alexander. It's the first week of classes so Mark can't be there. If you're local and want to hang with me in the waiting area, let me know.

As I reminded Mark, nothing has changed because of this consult or the procedure. We'll simply have more information in a week with a better idea of how to treat her when the coughing starts. And then we move forward.

Croup Be - GONE!

I'll take a kick to the head over croup any day. So, I did. Last night Soph slept (SLEPT!) all night with minimal coughing next to me and laying down!!! Most of the time she had her little feet in my chest or even in my face, but I didn't mind. I did have a strange dream though. I have always been a vivid dreamer and can remember at least one usually per night. Pregnancy has added a level of bizarre to them. I was at my parent's houe and the family was all there having a pool party in summer. (It was not their house in real life). We watched in horror when an air raid began as war on American soil, in a big way, took us all by suprise. Stress much?

My conversation with Sophie's ped this morning went well. As Soph is not barking so much today as she is coughing up wet ickiness, we decided to stop the steroid after today and not continue for the full 5 days of meds, totalling 3.5 days of Prednisalone. It's not like needing to finish antibiotics for effectiveness and I don't want her taking any more than she needs.

As for the nebulizer, let's just say I didn't have to bring out mama bear to get Doc to see things my way. He's on board for next time with the steroid (Pulmicort) through a nebulizer and saline/distilled water with the neb only as a secondary comfort measure (as in during an attack at night versus going to the ER). Since she is on the mend and already has an appointment with Dr. D (ped lung specialist) next week, we'll either get the new script from him, or the ped agreed to write it.

Thanks to all who have supplied articles, support, and strength. Momologists Unite! On another note, Maybe seems to love my morning glass of orange juice and is moving like crazy!

It's as Easy as Breathing

Except that sometimes breathing is definitely NOT easy. When it's your baby struggling with breathing, it's downright scary.

Monday Night: Tale tell signs of a cold appear in Sophie.

Tuesday Morning: Yep a cold, but will we avoid the 4th croup since November?

Tuesday mid-afternoon: No we won't and I'm not waiting to start the oral steroid, Prednisolone 5MG/5ML. She'll only get one of the 2 doses today, 9 ML each, as we started so late.

Tuesday Night: Not good. Coughing increases. Sleep is pitiful.

Wednesday Day: No fever (she never has a fever with croup). Nap time fails. Can't lay down to sleep, even propped up as coughing increases.

Wednesday Night: 2 vaporizers in our room. Still, we hit a new level. The cough is not good and that it so obviously hurts her, hurts me. What makes the hair stand up on the back of my neck is her attempt to inhale during a coughing spell. Attempt. Try. Not happening. Choking, gasping, her expression and my inability to stop it - pain. We've got a new sound with this inhale and that sound is fear. It passes. My fear does not.

We sat in the bathroom with the shower on hot for our Poor Man's Sauna (to breathe in the steamy air) while Mark talked to the nurse. A trip to the ER tonight? It could go either way and so the ER dance begins. Do we or don't we? Ran out of hot water and transitioned to where we would be for the night. The living room rocker/recliner. She's upright and doing better. Call a friend in "the know." ER? It could go either way.

11pm we record this for the docs. The room was dark so you can't see anything. The inhale is better and the sound of fear isn't there. Still, I pack the diaper bag and start the car. She settles down. She's sleeping soundly on her daddy's lap in the crux of his arm and breathing is good. No coughing. I turn off the car and Mark begins the first shift. He makes me lay down after midnight and begins reading another book about Russia.

4:15am her coughing wakes me up. She's been fully upright and asleep for 4 hours. On and off for 10 minutes she coughs. I bundle us up and we dance outside on the front porch for a few minutes. We call this Shock the Monkey, as in breath in cold air to calm down the inflamed airway, like icing a swollen ankle. It works or she stops on her own. Who knows?

My shift begins. Mark is to riled to sleep until 5am. I assume the position in the chair. Since there is no way I can keep up the fully upright position, we settle for Soph on my chest as inclined as I can stand. It occurs to me that we are three, me, Maybe, and Soph, snuggled in and breathing together. Soph's breath effects mine which effects Maybe's oxygen. I try not to watch the clock.

Thursday: Another Poor Man's Sauna as we clean up. Dr. sees us near 11. Weight: 21 lbs. Temp: 99.1 O2: 97 Lungs: clear. We play the recording of her cough. Doc agrees it's croup. Duh. Ask for a new remedy, up the steroid dosage, nebulizer, something. Yet, we are given No change in treatment. Doc says a neb is used to help the lungs and Soph's issue is in in her breathing tube (obviously not in these terms - give me a break I'm tired). He says a neb could do more harm than good. I think he meant a neb with epinephrine, which we haven't asked for or even Albuterol. Yes I know she doesn't have asthma or pneumonia. I've heard and read that a neb with steroid (and we're already giving oral steroids) or even a neb with just with saline can be quite helpful.

We get an appt. with Dr. Lung next week so this ped lung specialist can have a look. We're tired, frustrated, expected Something to change and nothing did. Doc says the ER probably wouldn't do much either (give oxygen and monitor her) but go in tonight if we feel it's needed. ? Not letting the nebulizer decision go quite yet. Any info is appreciated.

Refill meds and ask Mr. Bojangles (pharmacist) why I can't find the BabyLax suppositories. They stopped making it. Now they only have Pedialax for ages 2-5 and it's a liquid suppository vs. gel glycerin. We have to have it on hand as 1 missed dose of Miralax (given near bedtime as part of our routine that occasionally isn't done before she conks out) causes Painful constipation within 1.5-3 days. Sometimes introducing new foods or illness causes it too. Fine, I settle for Pedialax.

At Culver's for lunch we sit in a booth with giant window next to the train tracks and sure enough Alexander gets to see a freight train. He also, out of the blue informs me, "Mom you're not fat." I'm quite sure we don't talk much about being fat so I thank him and ask him what fat means. I got a jumbled reply, something about being different? I'll take the compliment anyway.

Soph is still sick and snot is coming out of her nose and right eye but nap time yielded a short and much needed rest. She's also saying her favorite new word a lot this evening, Uh-oh.

So tonight? ER? Poor Man's Sauna or Shock the Monkey? We'll see. Tomorrow I'm speaking to her ped to give an update and I plan to forward on some info about nebulizers for croup. Hope mama bear doesn't have to come out.