Medication... Wow what a difference.

So sorry for the blog overload. I forgot to mention earlier that the pulmonologist in Cincinnati took Jake off of Bethanechol. It has not helped his breathing and he did not feel it was necessary for him. So that means that Jacob is down to ONLY 3 medications!

Look how far he has come just since August when he came home from his Glenn...

AUGUST 2010

8am: Lasix (0.6ml), Captopril (3 ml), Prevacid (2 ml), 1/2 Baby Aspirin, Bethanichol (0.6ml), Diuril (1.2 ml)
4pm: Lasix (0.6ml), Captopril (3 ml), Bethanichol (0.6ml)
8pm:Prevacid (2 ml), Diuril (1.2 ml), Sodium Suppliment (2.5 ml), Aldactone (1.2 ml)
Midnight: Lasix (0.6ml), Captopril (3 ml), Bethanichol (0.6ml)

He was also still on his overnight feeds.



NOVEMBER 2010

8am: Captopril (3 ml), Prevacid (3 ml),
4pm: Captopril (3 ml), 1/2 Baby Aspirin
8pm: Prevacid (3 ml)
Midnight: Captopril (3 ml)

No more overnight feeds.
Started baby food and day feeds increased to 190mls per feed

Have I mentioned how proud I am of Jacob. Wow! He has come so far in just a few months. Thank you Jacob for being so strong. I am so blessed to have you as my son. I love you more than you will ever know.

Who said cardiac kids don't eat?

I didn't think I gave you enough to read on the last one so here is one more lol.

Back Story:
When Jake was born he could not eat by mouth. With every tube feed I would dip his binki in breast milk so he would have something to taste and associate with eating. Some g-tube kids refuse anything by mouth and I wanted to prevent that. When he was hospitalized at our local hospital for c-diff they used apple jelly instead of sugar water to calm him. There was no going back after that he was hooked. He LOVES apple jelly. I know when I first saw it my initial reaction was "Where is the biscuit, seriously this can't be good for him" but I ran it by all of his docs and they all said it was harmless. Mind you I just coat the binki with it, its not clumped on there or anything. Just gives him some flavor since he did not eat by mouth at the time. Even his cardiologist uses it to calm them during EKGs.

Every night before bed we would put a small amount on a paper plate and put it at the end of his crib so if he got fussy we could give him a little dip and he would calm down and drift off to blissful sleep.

Last week
I woke up at 8am and realized Jake had not woken up at 5:30 for a diaper change like he usually did. I went into his room and he was all smiles with his binki in hand. Then I noticed that the paper plate was laying in the crib on the opposite side by his head. My guess is that he stuck his feet in it and it stuck. He then grabbed it and went to town. The plate looked like it had been chewed by a dog.

I grabbed Jake and ran him to the living room where I reached into his mouth to make sure there were no pieces that he could choke on. He seemed quite amused. He was perfectly fine. No paper cut, no paper pieces. Then I lifted all of the pieces of the plate and lined them up. There were no chunks missing. At most he would have gotten a few fibers. Good for constipation right?

After making sure Jake was completely ok I could finally laugh at the situation. How can you not when you see this plate? Cardiac kids and especially g-tube kids don't usually like to eat. Mine ate a paper plate lol. Can we say teething?

Needless to say we no longer put ANYTHING in Jacob's crib. He is getting too mobile.

Enjoy the pictures!

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!

Home Sweet Home

We decided to come home tonight so I am exhausted and will post a full update tomorrow. The short update is that Jake most likely has vocal cord paralysis but we won't know for sure until they do more testing in January. His heart looked great. Function had improved, no tricuspid regurgitation, arch wide open. It looked really good.

Ok I know that was a horrible update but I was at the hospital from 8:30 to 3:30 and then drove home tonight and just got home at 9pm so I need to sleep!

Thank you all for the support and prayers.

We Made It To Cincinnati

We are in Cincinnati and Jake has been a happy kid. He and Aidan were so good during the 3 hour trip (So much better than the 9 hour drive we usually take to Philly). Jake fussed for about 5 minutes when we were almost there but other than that they were both happy campers the whole time!

Our morning starts early tomorrow. I have to feed Jake at 6:45 because he can't have any food after 7:30 and I want to give him time to eat. He can have pedialyte at 11pm but nothing after that because of the sedation for the echo. That means he should be getting hungry around the time for his pulmonary visit.. hope he likes pedialyte.

We meet with pulmonology at 10am. They are not doing any testing because its his first visit with them but we may have to come back next month for that. I want to discuss his breathing because the bethanechol has not helped at all and he has been on it for 4 months. Jake will then have a sedated heart echo at 1:30pm. It is not cardiac anethesia just a twilight kind of drug to make him sleepy. Otherwise he would fight them so bad they wouldn't be able to see anything.

I am really praying everything looks well on the echo. It has been a while since he has had one so I always get nervous. They will check his tricuspid regurgitation and hopefully it has not gotten any worse. This is leakage around the tricuspid valve. It happens because the right ventricle is enlarged so the valve doesn't quite match up. I am also hoping his heart function has improved. He is on Captopril because he has always been on the lower side of normal.

It will be a long day at the hospital so we decided that my mom and Aidan would not go and I would take Jake alone. My mom is taking Aidan to the aquarium.

The last time I was here I got the worst news of my life. I found out my baby had the most severe heart defect there was and that we needed to decide if we would have him or not. We couldn't terminate (If Jake was not going to make it I wanted to tell him I loved him and know I did everything possible for him) but we had to prepare that our baby may not survive. Thank GOD he did and we have much to be thankful for now a year later. I am hopeful we will get only GOOD news this trip. Please say a little prayer.

I will update tomorrow.

Love,
The Bakers

Tis the season!

Ok so I know that many of you are going to want to yell at me but we decorated for Christmas. We usually wait until Thanksgiving but its been a long year and I thought we should start the celebration early!

Every year we get a real tree. We hike up the hill, cut it down and bring it home. It's really a fun tradition but we changed it up a bit this year. Honestly the thought of cleaning up pine needles and having yet another thing to take care of was not appealing to me this year. So I went out and got an artificial tree. I was not sure if I would like it but I am happy to say I love it!

The best part was that Aidan got to help me put it together. He was so excited, I have never seen him more excited. He organized the pieces into piles and handed them to me quicker than I could put them up. He could not wait to show daddy all of his hard work when he got home from working. We waited until daddy got home to do the lights and ornaments. We had such a good time.

Jake was asleep while Aidan and I "built" the tree but he woke up after the lights were on. His face was priceless. He looked at the lights and started laughing and kicking. His mouth opened up and he grinned from ear to ear. Both boys were so happy.

So I know it was early but you know what after the year we have had we deserved some Christmas joy and the smiles on the boys faces were priceless!

Merry Christmas!

I love how these look like boxing gloves with hearts.
Perfect for my little fighter.

Here are some more cute pictures I took of the boys rocking out!

Got Synagis? We do!

I am so sorry that it has taken me so long to post an update. But no news is good news in our house. Between two kids and working from home its hard to find the time to blog.

Here is what's been going on lately...

SPEECH/NUTRITION
Jake continues to do very well with eating. He has been averaging 80 to 100mls by bottle and the rest goes in his tube. Amy and Bryn (Nutritionist/Speech therapist) are both happy with his volume right now so we are not going to push it anymore right now. Instead we are focusing on him taking more by bottle. I am giving him more breaks and trying to see if he takes more by mouth. Jake tends to drink 80mls real fast and then he is done and wants nothing more to do with it. The longer we wait between feeds the more he drinks by bottle.

The good thing is that since he is no longer on a feeding pump I don't have to be so structured on when he gets his feeds. Its blissfully normal now so I just take his cues on when he is hungry. I have to give him the rest of the food because he needs the calories. It is hard to tell if he stops because he is not hungry or because he is tired. He doesn't cry when I put the rest through his tube so that tells us that its because he is tired not full.

We are increasing his baby food intake to 3 times a day. Again, I go by Jake on this. If he doesn't seem interested then I don't push it. He has never had a problem eating baby food. He even smacks his little lips, its really cute. He usually eats about one stage two container (3.5oz) and then he is done. If we get 3 in a day that adds another 10.5oz to his calorie intake which is great.

Both ladies were very impressed with all of Jake's new "Baby Talk". His favorite thing to do right now is to scream lol. Mommy of course loves this stage. He is also experimenting with blowing bubbles and other baby sounds. Still no ma-ma or da-da but I am working on that with him. He is interacting so much more these days. His favorite game is peek-a-boo. He laughs so hard. It is so fun to see him light up and try to play.

PHYSICAL THERAPY
We are in the process of switching physical therapists. I loved Jake's physical therapist, she was great with him. Unfortunately her kids have been sick a lot lately and we have not been able to have a visit because its to risky for Jake. Its been over a month and I really want someone to come and start working with him more frequently. I work with Jake everyday on sitting, tummy time, and we even practice walking (with mommy holding under our arms). Jake can sit for a little wile but still topples to the side after a minute or two. He rolls from side to side and rolls over to his back every time I put him on his belly.


Jake is not crawling yet but Aidan did not crawl until he was 9 months old and Jake spent a few months in the hospital which could cause a delay in his gross motor skills. That is why its really important to me that we have someone come in more frequently to work with Jake.

SYNAGIS
For the past few months I have been on the phone trying to get Synagis shots for Jake. RSV season is coming up and for children with a complex heart condition RSV can be deadly. Synagis is a shot which is given to prevent RSV. It is an antibody that will help protect Jake if he were to get it. The problem is that Synagis is very expensive so its hard to get approval for it. Jake's doctors all agreed that he has to have the monthly shots through March. First Medco called and told me it was going to cost me $700/month. That is just what my portion was going to be. I told them NO WAY! After calling my insurance company and about 4 other numbers I finally figured out who we needed to order specialty drugs from. They called me a few days ago and told me that my insurance would cover 100%... AMAZING NEWS!

PEDIATRICIAN VISIT
Today we went to Jake's new pediatrician. She was really nice and great with Jake. Aidan came with us today. Its great having all of Jake's doctors in one area. Bert (Dr. Heydarian's nurse) made Aidan's day by giving him stickers and crayons to color with while we were there. Thank you Bert!

Jake weighed 18lbs 9oz! He is getting so big. Jake was all smiles for his new doctors... until the shots came. Aidan was really sweet. I asked him if he wanted to hold Jacob's hand and he replied... of course! He held Jake's hand the whole time. Although I am sure Aidan made him feel better Jake still screamed bloody murder. He was calm by the time we left and all smiles again. Jake has been great all night, no side affects from the shots. His pediatrician said there rarely are any because its more of an antibody than a vaccine. He was a tiny bit fussy tonight but he has been teething a lot.

PEDIATRICIAN TAKE TWO
On our way home from Jake's appointment Aidan told me his ears were hurting. He said it a few more times and when I asked where he pointed inside of his ear. I figured I better take him to his pediatrician just in case he had an ear infection. They were just about to go to lunch but said to come on in so they could check it out. We got there and I rushed the boys in so he could be checked out. When we got there I asked Aidan again and he said he was pretending but then when I said we would cancel the appointment he said no it does hurt mommy. He told me that it hurt really bad and he was not pretending. I had my doubts but decided we were there anyway so we went in. After Dr. Harris said his ears looked perfect Aidan said "I told you I was just pretending!" He was so proud of himself. KIDS!!!!!

I told him that his little prank cost me $20 bucks and its not good to pretend about health matters, especially in our house! I couldn't be mad at him though because I know why he did it. Jake goes to the doctor all of the time and he was wanting some of the attention even though its not good attention. When we were at Jake's new pediatrician Aidan mentioned that he wanted Dr. Harris. He loves Dr. Harris and I think it confused him that we were at a different doctor. I know it may be easier to have one pediatrician for both kids but I am going to keep Aidan with Dr. Harris because he really loves him and I think some stability is good for him right now.

So taking Aidan today was great for him to feel bonded with his brother and feel included. It also backfired on me with the prank illness lol. I have to say I love my boys more than life itself. Life would be so boring without them.

VISIT TO CINCINNATI
Next week we will be heading to Cincinnati for a follow up pulmonary appointment and a fetal heart echo. They won't be doing any testing during the pulmonary visit but if they feel it is necessary than we will have to go back for that. Jake will be sedated for the heart echo so he won't be able to eat anything for 6 hours prior. Jake HATES heart echos. The last one took us 2 hours to get him calmed down. The sedation will just be oral sedation and won't be anything strong. They said you could pinch him and he would wake up. It will just help him sleep so they can get good pictures. It has been a while since he has had a echo so I am anxious but also very glad to know how he is doing internally. I am excited to meet Dr. Heydarian's daughter, she is a cardiologist at Cincinnati Children's. She will be there when we get Jake's echo.

It is always nerve racking to travel and go to a hospital with Jake. Next week will be especially hard because his appointment is exactly one year from the day we were told about Jake's heart condition when I was pregnant. Last year at this time I was traveling to Cincinnati for a fetal echo and I was told I had to decide if my baby would live or not. I was told I had to decide if I would terminate, have the baby and let him die or choose to do 3 open heart surgeries which won't ever fix his heart but would give him a chance at life. I know that it is going to be emotional for me to be there again. On one hand I am so blessed that I have him and he is going with me. On the other hand in the back of my head I am scared about the results and what they will say. I got such horrible news last year there. I left thinking we were going to lose our baby. I am praying that we get very good news this time.

We will be leaving here on Tuesday and Jake's appointments are on Wednesday. My mom and Aidan will be coming with us. Daddy has to work. I am bringing Aidan because although he did so well with everything we have been through this year it has still affected him. Almost daily he tells me "Mommy I am going to miss you when you go to the hospital" or he tells me that I hurt his feelings when I left him to go to Philadelphia with Jake. It does not bother him much but its there and that is completely understandable with us having to be separated the first few months of Jake's life. I think it will be good for him to come and see that I am not going away and I will be back. I don't want him to resent Jake for taking me away from him.

I have decided to stay Wednesday night there as well. I don't want to put Jacob through a long car ride after the long day he will be having. I will update the blog next week and let everyone know what happened.

Please pray that everything goes well for Jake next week.

Thank you so much for all of the prayers for our beautiful miracle.

Love,
The Bakers

Jake's Blankie with a Cause. Created by Shannon in honor of her angel
Lorenzo who lost his battle with HLHS at 13 months old.