What is Laryngomalacia?

FACTS vs. FICTION
Things you should KNOW about your child's laryngomalacia!

What is Laryngomalacia?
Laryngomalacia is floppy tissue or under developed cartilage. The tissues involved are the epiglottis and arytenoid cartilages. These are located above the vocal cords in the airway. When a person has laryngomalacia the floppy tissue falls into the airway blocking or constricting airflow upon inspiration. Usually when this happens it produces a sound known as stridor.

What is Tracheomalacia?
Tracheomalacia is a condition characterized by flaccidity of the tracheal support cartilage which leads to the collapse of the trachea when increased airflow is demanded. In a normal airway, the trachea dilates slightly upon inspiration and narrows slightly during expiration, when a person has tracheomalacia, however, these processes are exaggerated causing the airway to collapse upon expiration.

What is Bronchomalacia?
Like Tracheomalacia and Laryngomalacia, Bronchomalacia is the weakness of the bronchial tubes causing a collapse of the airway and a wheezing/stridor upon expiration. Bronchomalacia is due to a deficiency in the cartilaginous rings of the bronchial tubes.

What is Stridor?
Stridor is a high pitched wheezing sound resulting from turbulent air flow in the upper airway. Stridor is a physical sign which is produced by narrow or obstructed airway path. It can be inspiratory (upon inhale), expiratory (upon exhale), or biphasic (both). Inspiratory stridor is the most common (and is associated with Laryngomalacia). Expiratory stridor happens with Tracheomalacia and Bronchomalacia. Stridor can be indicative of a serious airway obstruction from severe conditions of the epiglottitis, a foreign body in the airway, or a laryngeal tumor. Stridor is indicative of a potential medical emergency and should always command attention.

What Dr's don't want you to know...don't let this happen to you....
For some strange reason many Dr's seem to try to ease the minds of laryngomalacia parents by saying things like "they will grow out of it" or "it's just noisy breathing." These statements are not true, first of all, the noisy breathing is stridor which is very dangerous. What they mean by that statement is your child's episodes are not as significant as some more severe children and therefore require less intervention, but the bottomline is every time you hear that sound which I'm guessing in many cases of "mild laryngomalacia" is frequent, your child's airway is being restricted and that is a BIG deal. And how do they determine it's mild? I was told Declan had mild LM, but a year later it was SEVERE?? It's all relative to what the doctor sees in the five second scope they do in office? I mean that's just ridiculous and I have seen too many "mild" cases turn south to believe this crap about 90% of these kids growing out of it or only having mild LM.

Another problem is it's all one dimensional to many of these Dr's, it's only noisy breathing, but laryngomalacia has many layers and leads to other problems, such as frequent ear infections, low immune system, failure to thrive, and reflux. Lets touch on a few of those side effects, failure to thrive for instence. Has it ever occurred to anyone that the reason so many laryngomalacia babies have failure to thrive is because they are having to work over time to breathe? Well apparently it's not a big deal to have stridor, ha, yeah right. Failure to thrive is very prominent in these children and it's because their airways are not being protected by their doctors and their parents are not educated enough to speak up about it. I had to fight for Sawyer otherwise he would be a statistic (a SIDS baby). Declan I didn't fight for because I didn't know what to do...lesson learned, now I have one child that has serious medical issues (probably for life) and another that is starting to thrive. You are your child's advocate never forget this and remember these Dr's work for YOU! Low immune system led to Declan picking up a mycobacterium (most likely from our water source), which led to a granuloma (similar to a tumor) in his lymph node near his parotid gland...these things happen guys! I'm not trying to scare you I'm just giving you the information you need to be a better advocate for your child's condition.

As I continue to stumble across things that have to do with LM, TM, BM, I will update on here to give more information, but this is a good start...goodnight all.

Sawyer's Bi-Pap Machine!!!

I am so happy to report that after having his Bi-Pap machine for 1 month Sawyer went from having almost 200 sleep apnea events a NIGHT to 1 event the entire month!!!! I mean seriously? That's just phenomenal! I've posted a video of Sawyer doing our nightly routine. He USUALLY doesn't even fight me but you see him decide to put on a little show for the camera! LOL! Stinker, but not too bad for having a mask on his face every night right? So happy we have a good pulmonologist that got us this machine! <3 <3 <3

Declan A-Typical TB Story

I'm not sure if I wrote about them on here or not but just to recap after Declan's surgery he had some complications. First immediately after the surgery he was parallyzed on the left side near his mouth which made his smile droopy. They assured us this was only temporary and because the nerve was weak after surgery. They were right and after almost 5 months it seems to be completely gone. After two days in the hospital the intern working with us decided to remove the tube from his neck. This tube was draining the excess fluid that was building up in the spot that used to have his parotid gland. The removal of the tube was not what I wanted nor what had been discussed the day before by the main Doctor, but it was done and we were sent home from the hospital. As an effect his face swelled severely to the point that he could hardly see out of his left eye. We rushed him back to Arkansas Children's and the determination was to "watch and wait" to see if it went down. It did go down but in the process it made a fistula. A fistula is an abnormal space created in between two organs. In our case it was preventing his skin from fusing right at the seam where the skin was still a little "iffy" from stretching due to the granuloma. This space was collection saliva which is a common complication of the parotid gland removal surgery. His face would fill up like a pimple and you could press on it and it would squirt out saliva. I was told to drain this whenever I saw it filled up. The Dr. once again wanted to continue doing the "watch and wait" but decided to send us to the Infectous Disease clinic to see if he could do anything different with medications. I forgot to mention he had been on Biaxin since August. This went on for a few months until around mid-December, when I finally said I had researched fistulas and that it was a fistula that needed to be corrected with surgery or a feeding tube (to prevent the saliva from building up). At this point the ENT Dr and I had different perspectives on what Declan had. He felt Declan had A-Typical TB (but had nothing to back it up...all the test results came back negative) and I felt that Declan just had a benign tumor because they had nothing to prove it was A-Typical TB. The reason why the results were negative were because they never tested the samples with DNA analysis, this is something you MUST do in order to find out whether a person has A-Typical TB or not. The Infectious Disease Dr was somewhat smarter than our ENT Dr and got the right tests ordered and put him on another antibotic (Rifampin) in conjunction with Biaxin that he thought would help. After only a week of waiting we received word back from his clinic that Declan did indeed have A-Typical TB originally and that a granuloma had formed within his lymphnode near his parotid gland. His other lymphnodes were swollen as well and the Dr was concerned about them so he tested for cancer to be sure, but the test was negative, it is possible that A-Typical TB can spread throughout the body into other places, but they said it's "unlikely." After two months of this treatment of 2 antibotics at a time, his saliva slowed down long enough to close off the hole and fuse, it's not completely fixed but it is getting better. So we have hope. He will be on the double antibotics for 6-12 months to try to prevent the A-Typical TB from spreading throughout his body. A-Typical TB is not contagious and is usually contracted from the environment. Many of the mycobacteria that cause A-Typical TB are found in drinking water, but people with suppressed or low immune systems (Declan) are more susceptible to getting sick from contact with bacteria like this. Moral of the story...purify your kid's water source? I think so...

In the beginning

As the Granuloma Grew...

Last few days...before surgery...almost bursting through the skin

Another look at it...

Sweet boy before his surgery...

 

After surgery with all his presents for being brave!

Pretty shocking moment when we unveiled this baby!

Paralysis of the facial nerve near his mouth

Declan's face swollen a week after surgery (it got worse from here)

Spot that wouldn't heal

After 5 months of antibotics

(Dec is in the green shirt) You can see his smile is weird here it was pretty significant.

Back to Normal Smile

UPDATE ON SAWYER

Ok. Lets start with Sawyer. The last I updated his MRI came back normal, which I rejoiced about, but the MRI results might not be accurate according to the neurologist that graded his sleep study on 10-19-2011. First, the entire reason we had a sleep study was because I was "claiming" that Sawyer was still having breathing problems in the night even after multiple surgeries to correct his condition. Since no one believed me they asked if I was willing to do another sleep study to prove he was in need of a CPAP and O2 monitor which I have been asking for since Sept. 2010! I agreed and a month later we were seen in the sleep center. The results were worse than the first sleep study which constituted his first surgery (supraglotoplasty) on 2-16-2011.

Results: Sleep Study 10-19-11
Cent. Apnea
6-9 secs: 8
10-14 secs: 5

Obst. Apnea
>20 secs: 1

Obst. Hypopnea
6-9 secs: 31
10-14 secs: 46
15-20 secs: 39
>20 secs: 62

"His lowest oxygen saturation was 76% and was <92% for about 3.5 minutes of the total night. Although his oxygenation was ABNORMAL he did maintain his baseline avg. 97% oxygenation 94.1% of the the sleep time. The drops came with sequential events in REM sleep (particularly of a CENTRAL nature)."

"Actually, as the child fell asleep, he began to demonstrate periodic breathing, which was a mixture of central apneas, and central and obstructive hypopneas, but which were so irregular and difficult to identify that it was quite hard to score with numerous events lasting less than 6 seconds. Of those that were less than 6 seconds, only those which were most clear cut were scored. Thus the child's breathing pattern particularly in the first part of sleep was much worse than these number reflect."

"Sleep was disrupted by these numerous events. The great bulk of the central events were very short, but the hypopneas ranged as long as 53.2 seconds in duration. Overall, this study is more abnormal than the child's first study."

Recommendations:
"Consider reviewing with neuro-radiology this September's MRI to look at the cranio-cervical junction to evaluate for a Chiari I malformation. The report comments that the brain stem looked normal so that issue has likely been address."

May L. Griebel - Neurologist in the Sleep Study Lab - Arkansas Children's Hospital

So after months of asking for a CO2 monitor (still don't know if they will give me one or not) and a CPAP machine. They are finally getting us a CPAP machine and evaluation. This will happen on December 20, 2011. A full year from the time of his first sleep study and 2 surgeries later. He has nothing left to be blocking his airway (tonsils gone, adenoids gone, tubes in ears, & supraglotoplasty).

On another note. Sawyer received the RSV shot on 11-15-2011. So far so good. Everyone else in the family has been sick and he hasn't been sick yet! Really hoping this lasts and he's protected this winter!

MRI & CT SCANS

Sawyer's MRI has come back NORMAL!! YAY!! He's definitely not out of the woods for typical laryngomalacia, tracheomalacia, and bronchomalacia complications BUT he does not have any related neurological problems and we are very thankful for that! <3

Declan's CT Scan was a less exciting revelation. The Dr. believes that Declan has something called atypical TB. This is something that can form if you have a compromised immune system (which he does) and TB germs infest a certain part of your body. It is not contagious and the cure is immediate surgery (or it can spread to other organs) on the area affected, in this case it would be Declan's parotid gland. They don't know if it's in other organs already but he said that was rare... My first instinct is I'm freaking out! I don't dislike our ENT doctor as a person, I just believe him to be a very busy man that sometimes rushes through things and perhaps he doesn't pay enough attention to all the details. For a situation like this surgery that cannot happen and I feel like it's a big risk using our same Dr. for this surgery, however, it is the opinion of relatives, this Dr., and our PCP that this is something that cannot wait too long on and going out of state for a second opinion is not really an option. My fear is the facial nerve that can be damaged in this situation and the fact that he is leaning toward the riskier surgery (possibly leaving cells behind causing regrowth and having a higher risk toward the facial nerve damage to occur). He believes this thing is sitting on top of the gland. He also noted that it could be a cyst or granuloma. We have had blood tests done and it is not an infection or cancer! Thank GOD! He has said he can change his mind once he gets in there and take out the whole gland, but I will re-address my concerns on surgery day and probably try to push for the least risky surgery...I don't want to remove the gland unless necessary but he tends to forget and just do whatever is on the paper instead of making decisions in the OR...at least he's never changed his mind before during a surgery. I don't feel comfortable with this situation...I feel rushed and trapped but there is nothing I can really do...my husband, family, and Dr's are pushing me to do this...I want him better...just so scared for him...so much is at risk...just very stressed...

Declan's Tumor (Benign or Cancerous?)

Well...I've been sitting on this for almost two weeks...but I think it's pretty firm that we're dealing with a serious issue with Declan (my first LM baby). On Friday, Sept 2nd I noticed a "bump/growth" on his face from his jaw to the middle part of his ear on his face. It's a little larger than a quarter in width and sticks out a little but if you didn't know to look you might not see it...however if you see it then you're freaked. I called immediately after seeing it but it was around lunch time and it was the friday before Labor Day weekend. I wanted to know if I needed to go to the ER & the ENT nurse said it was non-emergent and we should wait until Tues. morning. The doctor looks at him on Tuesday and says there is a 90% chance it's a benign "mass" that is as large as his entire parotid/paratid (sp?) gland he also tells me I have the worst of luck with some of the rarest things a child can have. He then decides he's going to give him 7.5 ml of bactrim for the next 10 days to see if it's by some reason an infection and not a tumor and then we were to come back on the 27th if it's not gone and they will do scans. So we've gone a full 7 days of antibotics and no change in the gland it's huge. I read that fast growing "masses" are most likely cancerous! So I've been totally freaking out. Scrambling trying to get a second opinion and nothing is working out. We're trapped here in Arkansas unless we want to lose our house to get another opinion because Tricare won't cover anything out of state?? RUDE! Even if it's not cancerous I don't understand waiting so long to find out what it is. I get trying antibotics for 10 days but why wait until the 27th? I think that's too long. Another thing that freaks me out is he has growth problems and 90% of the cancerous tumors in this gland stem from the thyroid gland...what if he's had this his whole life?? I'm just worried about him. I want answers and I have to at least finish out the antibotics before they will see us. If it's benign he's still not out of the clear because his facial nerves run directly through the gland and it's a very risky surgery...I don't trust our current Dr. but I'm trapt with my other Laryngomalacia baby that needs the RSV shot here in Arkansas, I feel torn between the two. I dont' know how much more of this kind of stuff I can take. I can't lose him. And the stupid/mean people that have been talking to me today during this situation I just wish they could understand what it's like for me on a daily basis because maybe they wouldn't be so mean about me calling worried about my sick baby. I'm just praying he's going to be fine. There is more to this story but I'm just too tired to even write it all out...

Declan the night he almost died in the hospital.

Sleeping during his time in the hospital

Finally feeling better Day 5

Declan's Surgery Feb. 2010

One Type of Sawyer's Stridor

Sleep Study - Dec. 2010

IV in the head...never seen that before for his 5 day hospital stay in Jan. 2011

Happy Baby

Sawyer's First Surgery - Supraglottoplasty 2-16-2011

Bronchomalacia diagnosis bottom right 7-11-2011