The Full Update

Jake and I had a long day yesterday so sorry I was unable to post this until now but I needed to regroup. Here is a breakdown of our day.

7am: Woke Jake up to have his bottle. He was not allowed to have any formula after 7:30am because he was being sedated at 1:30 for his heart echo. He could have pedialyte up until 11:30 but nothing after that.

My mom took Aidan to the Aquarium and I took Jake to the hospital. I decided I did not want Aidan stuck in a hospital all day. This way he had a good experience when we had to travel with Jake for appointments. This is important after the year he has had. Aidan made me promise to tell every doctor that he loved his little brother.... I kept my promise!

PULMONOLOGY
9am: Arrival at Pulmonary Registration

SATS: 80
WEIGHT: 18lbs 9oz

Warning: This may be confusing and really I can't give details until we go back in January and get some more answers.

First some Definitions...

Aspiration:
The entry of secretions or foreign material into the trachea and lungs.

Tracheomalacia:
A condition characterized by flaccidity of the tracheal support cartilage which leads tracheal collapse especially when increased airflow is demanded.

The trachea normally dilates slightly during inspiration and narrows slightly during expiration. These processes are exaggerated in tracheomalacia, leading to airway collapse on expiration. The usual symptom of tracheomalacia is expiratory stridor or laryngeal crow.

If the condition extends further to the bronchi (if there is also bronchomalacia), it is termed tracheobronchomalacia. The same condition can also affect the larynx, which is called laryngomalacia.

IN ENGLISH: HE HAS A FLOPPY TRACHEA AND VERY NOISY BREATHING

Vocal Cord Paralysis
Inability of one or both vocal folds (vocal cords) to move. The paralysis is usually due to damage to the nerves going to the vocal cords or due to damage to the brain itself.

Some causes of paresis include viral infection, cancer or tumor compressing the recurrent laryngeal nerve, intramuscular tumor limiting vocal fold movement, trauma, compression of the vocal cord nerve from intubation, or laryngopharyngeal reflux. Cardiac surgery represents a risk to normal voice function as the nerves serving the larynx are routed near the heart. Damage to this nerve during open heart surgery is not uncommon.

The vocal cords are open when we breath and closed when we speak. So when you eat it is supposed to close after you swallow and if it stays open like Jake's there is risk he can aspirate it into his lungs.

Now about our visit:
We met with the pulmonologist and talked about Jacob's history and his tracheobronchomalacia. After listening to Jake and talking with me he told me that he suspects that Jacob has partial paralysis of his vocal cords due to the high pitch in his breathing. The good news is that he said his lungs sound great. All of his noisy breathing is in his vocal cords.

I told him that this was a gray area when we were at CHOP and they said that his vocal cords were moving but not touching. CHOP did a swallow study, reflux study and ENT scoped him. ENT said they were not paralyzed but other people said they thought they were so we really never knew. He passed the swallow study with no aspiration. Due to severe reflux they did the g-tube/nissen surgery. If you remember from earlier blogs his vocal cords were so swollen and red that he had no sound for the first month of his life. He regained that sound after the nissen so it was though that they were not paralyzed only swollen from reflux. Warned you this was complicated.

Ok so that was all when Jake was only 4 weeks old. He has not had any scopes or studies since then. He continues to have noisy breathing. The pulmonologiist said he could be wrong about the paralysis but the squeak Jake has in his breathing and voice is an indicator. That being said if it is paralysis it is only partial. Jake makes noise and lots of it. He talks "Baby talk" and is saying all of his vowel sounds. He also has not had a problem with eating. The Pulmonologist said that it could be that Jake is so used to the feeling that he doesn't have that gag reflex and cough but he could still aspirate. However Jake does cough at times when he eats which means he does have sensitivity in the back of his throat so that is promising. He did not say we had to stop bottle feeding but they would like us to come back in January for further testing.

I will have to make one trip to meet with ENT and anesthesia. Then we will go back and Jake will get scoped by ENT and Pulmonology because they can look for different things. They talked about doing a GI scope to check the reflux but I WILL NOT put Jake through stomach surgery again to tighten the nissen so I told him I would rather not do that one. Then I will have to come back to meet with the pulmonologist to discuss the results. I am trying to arrange to do all of this bunched together so I don't have to make 3 trips but the pulmonologist is only there twice a month because he travels to satellite offices.

So I know this is full of contradictions. Welcome to my world. This is why I really am on board for the scopes. He is older now and we can get a better understanding about what is going on with his vocal cords and hopefully a definitive answer! I am not worried though. Jake has continued to do well with eating and yes he is noisy but he is making more and more sounds and "Talking" without a problem. I just want to know one way or another because if there is risk for aspiration or anything that could lead to speech problems I want to be able to work on that for his safety and development.

After our appointment they sent us down for a chest x-ray so they have a baseline on file for Jake.

Next on to...
CARDIOLOGY

By this time I had tried to give Jake his pedialyte so he had something in his belly but he was not having it at all. HATED the stuff. I even had the fruit flavor... no go. So it all went in his tube. We got the chest xray done which he hated as well and then made our way up to cardiology.

Jake was supposed to get a sedated heart echo but we still had 2 hours. I checked us in hoping he could go early because at this point he was irate that he had not had formula yet. He was hungry, tired and pissed off from being messed with. The heart echo lab was full so no chance on going early.

I decided to walk Jake around in hopes he would fall asleep. Everyone kept telling me how cute he was. One girl came up to us and asked if she could touch him. I quickly relied NO! I explained his condition and she understood and told me how special he was. At least she asked, I HATE when strangers come up and want to touch your kid. This is why I don't bring him in public!

Jake finally fell asleep but was not able to get the echo for 40 minutes so he woke up and still needed to be sedated for the echo.

I was able to meet Dr. Heydarian's wonderful daughter. She is a cardiologist at Cincinnati Children's. It was so nice seeing someone who knew us (by name anyway). I felt so lost there all day. She held him until he passed out from the sedation. She jokes with the echo tech that she would have to give him the echo in her arms because she was not letting him go. Love the Heydarian's! I am so glad she was able to meet Jake while we were there. She also gave me her information so that I can have her involved when they sedate Jake for the scopes since he is so high risk.

Echo went smoothly since Jake was knocked out. It was odd because we were in the room next to where I was a year ago finding out about Jake's condition. Today marks the anniversary of when I heard those words that changed my life forever..."There is something wrong with your babies heart". Wow we have come so far in the past year. It definitely has been a roller coaster but I am so blessed to be here a year later with a beautiful baby who has taught me so much.

Dr. Heydarian's daughter called me a few hours after we left with the results of his heart echo. His heart looked great. No significant tricuspid regurgitation which is very good news (For those not familiar with hearts this means that the tricuspid valve is not leaking). Leaking can occur because the right side of the heart is enlarged due to the extra strain on the heart. Therefore the valve does not meet up correctly. Too much leakage can be fatal. The arch and pulmonary veins were wide open. This is also great. The best news I have had in a long time was that his heart function looked very good! Jake used to be on the lower side of normal. Now it looks great so his function improved!

The rest of the night Jake was pretty out of it. He would wake up but you could see in his eyes he was all drugged up. He scared me once because his lips looked blue and I hooked up to the pulse ox and he got down to the high 60s. He had just eaten and acted like he was retching so I think the food was to much with the sedation. Throwing up is the most common side effect. Since Jake can't throw up he retches. I put oxygen on him for a second. He freaked out and ripped it off. His SATs went back to mid 80s and he remained there.

I decided since he was so out of it we should just head home that night. I kept Jake's pulse ox on him so I could monitor him the whole way. I also had the oxygen in the back just in case. He did great. He got fussy towards the end when he was hungry but slept most of the way and his SATs stayed in the mid 80s. Aidan slept the WHOLE time. We got home around 9pm. It was a LONG day!

Jake has been fine with the rest of his feeds and has been chugging down bottles like a champ! Overall I think it was all good news. His heart and lungs look great which is the main thing. The vocal cords... we will figure out but I am confident that Jake will overcome this obstacle just as he has in the past.

Love,
The Bakers

BTW... Where can I mail off for my nursing degree? I really think I should have one by now!