Happy Birthday Jacob

We are so proud of you Jacob. You have been through more in one year than many go through in a lifetime.

Make video montages at www.OneTrueMedia.com

Thank you Culloden Elementary!

I would like to say a big thank you to the kids at Culloden Elementary School. They recently participated in Jump rope for heart to raise money for the American Heart Association.

I would like to extend a special thank you to Hannah, Emily, Jacob and Ethan who raised $300 in Jacob's name.

Also thank you to Kelsy who also jumped in Jake's name at her school.

Thank you again to all of the kids who jumped and I am sorry if I left anyone out who may have jumped for Jake. It means so much to our family that you are helping fight to create a better future for everyone who is affected by heart disease.

Jacob Van Nostrand

Hannah & Emily Armstrong

Article in the Charleston Daily Mail

Monica Orosz of the Charleston Daily Mail did a wonderful article about our amazing little heart warrior. I couldn't believe how beautiful the print edition turned out. What a great keepsake for my little man. Thank you Monica, we love it!

Click here to read the article

Pajama holes

So I got a call from my dad the other day and he was saying he couldn't believe I have a picture of Jake on the blog with a hole in his pajamas. I started laughing and informed him that all of Jake's PJ's that have a zipper have holes in the foot. I cut the foot out so that I can hook u his pulse ox machine at night while he is sleeping. He also has a small hole on the side which we used to use for his feeding tube at night.

This started making me wonder how many other people think my poor kid is in ratty clothes lol. I thought I better post this to reassure everyone that the clothes are not falling apart... they are like that on purpose.

The things we take for granted...

When most parents go shopping for baby clothes they buy things because they are cute. When a parent of a sick child goes shopping we have to look for clothes that accommodate wires, tubes and medical equipment. I am now starting to be able to put Jake in two piece pj's which is better if I need to check his oxygen however I have to tuck in the shirt or he will try and pull out his g-tube.

The good thing is that now Jake is pretty much wireless! At one point in time he had 3 wires attached to him all night. He had the pulse ox, feeding pump and oxygen. This was so nerve racking because everyone knows you don't put things in a babies crib so imagine my fears with him having CORDS in his. He had to have it though. Now we check his pulse ox as needed so he never has cords in his bed at night (YAY!).

So my dad will be happy to know that I have stopped cutting holes in Jake's clothes!

Jake's visit to Cincinnati Children's Hospital

We left for Cincinnati Children's Hospital on Wednesday. The last few days have been an emotional roller coaster but I am glad we finally have the answer to Jake's "Unique" breathing. Warning: This will be long.

THURSDAY

We had an appointment at 8:15am with the ENT that would be doing one of Jacob's scopes the following day. We figured it would be a quick uneventful appointment and never imagined we would learn what we did. They wanted to look at Jake's vocal cords to see if they were paralyzed. He needed to be awake so they would be able to see if they were moving while he cried.

I was anxious to see what they found since there has always been the question about his vocal cords and whether they were paralyzed or not. I thought if they were then at most it would be partial since his voice was normal. I was wrong. The scope showed complete bilateral paralysis of the vocal cords.

When we breathe our vocal cords open, when we talk or eat they close. That is why Jake has never had aspiration issues and his voice is normal. However it does affect his airway. Jake only has a small opening for air to move through. The only movement in the vocal cords is when he exhales and that is just from the force of the air being pushed out. When he inhales he has to suck all of the air through the small opening. When he is active his breathing gets worse because he is trying to move air through. In 95% of the cases of bilateral vocal cord paralysis the kids need to get a tracheotomy immediately because they can't breathe at all. The fact that Jake is strong enough to breathe is amazing, add that to him only having half of a heart... I am just in awe of my son.

This explains why he has always had issues needing oxygen after his heart surgeries. His body was in a weakened state and could not breathe through that small hole until it was healed. Its amazing he was able to get off of the vent so quickly or even at all.

The doctor said that Jake won't need a tracheotomy because it would only be a temporary fix. In order to correct this Jake would need surgery. Right now Jake is doing great however as he gets older he will become more active and need to push more air through and he won't be able to do it. He also said that this could put more strain on his already weak heart so they may want to do the surgery sooner than later.

I know it sounds silly but I felt just like I did the day we learned about his heart. We left that day feeling like our son was just handed another life threatening diagnosis. Fearing even more the inevitable day when he gets his first cold. Fearing that if his airway swells even slightly that he would not be able to breathe at all. Happy and so proud that he is one of the 5% who don't need a tracheotomy but so very scared that one day he will stop breathing and I wouldn't be able to get him help in time. It was a very emotional day but we decided to make the best of it for the boys.

After getting the "OK" from the doctors we took them to the Cincinnati Zoo. We wanted Aidan to do something fun since he had to spend so much time at the hospital with us. We never take Jake in public so this was a big deal for us. However he was so bundled up that he slept most of the day lol. Aidan had a great time.

Friends of ours from back home were in Cincinnati because their son (also named Jacob) is having his 3rd open heart surgery on Tuesday. Their Jacob is missing the right side of his heart. His mom Michelle reached out to me after she read an article about awareness I did while pregnant with Jake. They came to the Zoo and we had a great time visiting with them. It was so nice seeing our Jacob's together.

Jacob Roth (Tricuspid Astresia)
Jacob Baker (Hypoplastic Left Heart Syndrome)

FRIDAY

We arrived at the hospital at 10:30am. Jake was having 2 seperate scopes, one from Pulmonary and one from ENT. They were also removing his skin tag and doing a heart echo while he was still under cardiac anesthesia.

Aidan was not allowed in the pre-surgery prep area so he and daddy stayed in the waiting room while I went in with Jake. He was not happy about not being able to eat and let everyone know it. We answered many questions and I also was able to speak to the pulmonary doctor who was doing his scope. He was so great and talked with me for almost an hour about the vocal cords and what would happen if he needed surgery. He also told me that it is common to paralyze the left cord during heart surgery but very rare for them both to be paralyzed.

I walked with Jacob into the Operating Room where I told him I would see him soon and then began crying as soon as I left him. I know that it is nothing compared to everything else he has gone through but it is just so hard as a mother to hand your child over for any procedure especially when their anatomy is so fragile.

We waited almost 2 hours before I was finally able to go see my sweet baby. We then went to a conference room to talk to the ENT and Pulmonary Doctor. They told us that they were able to see that the vocal cords are being held together by scar tissue. Most likely this is due to him having to be intubated so quickly when he crashed right after birth. There was not much time because his oxygen was down to 30% so they had to act fast. He was then intubated for 2 weeks after. This explains why when CHOP looked they were moving some, he was only 4 weeks old so the scar tissue must have just started growing over them. Now they are completely tied down by it.

The good news is that there is a possibility that Jake's vocal cords are not paralyzed. The problem is we won't know until we get rid of the scar tissue. They explained that they could go in through his mouth and cut the scar tissue out and once he heals they can look at his vocal cords and see if it worked. They said it has not been very successful in the past. Maybe 1 in 10 cases are successful. They said that if Jake were a healthy child they would say to just go ahead with the vocal cord surgery however since he is not they think he should do the least invasive route possible. If it doesn't work then we can schedule surgery. Most likely it would be in a few months.

They also said that he does not have tracheomalacia. All of his breathing issues are from the vocal cords.

SKIN TAG
They were able to remove the skin tags on Jacob's left ear. It did bleed quite a bit but they stitched it up with dissolving stitches.

HEART ECHO
Jake's heart echo looked great! They were very pleased with his heart function and there was very little Tricuspid regurgitation. The Glenn shunt looked great. YAY for good news!

RECOVERY
Jacob had a very difficult night and I am so glad I asked them to keep him for monitoring. He did not like the feeling of the anesthesia and I am pretty sure it gave him a headache. He screamed and thrashed for 2 straight hours.

He kept trying to rip out the IV's so they took one out and just left the one on his foot. He also was very gassy so I vented him a few times and was shocked at how much air came out. I think this was also a big reason he was so uncomfortable. Many kids vomit after they first eat when coming out of anethesia, Jake has a Nissen so he does not throw up. I suspect instead the air sat in his belly and caused him a ton of pain. He also kept grabbing the ear where they cut off the tags.

Dr. Heydarian's daughter is a cardiologist at CCH and she stopped by during this time and had them order him Toradol because he was so miserable. She stayed for an hour and a half to make sure Jake was ok. I told her she did not have to stay but she was so sweet and wanted to make sure that he was ok. We are so lucky to have the Heydarians in our life!

He woke up throughout the night but each time it was easier to get him to sleep. I also realized that Jake has already started fearing doctors and nurses. Every time one would come in he would start crying. The staff were all extremely nice and tried not to bother him except when they had to take his vitals. It broke my heart to see him hurting so bad. I started crying myself. He would just look up at me and say ma-ma. It was so hard to not be able to make it better for him. He also has started saying na-na anytime he doesn't like something.. his word for No. We didn't get much sleep but by morning he seemed to be back to normal. THANK THE LORD!

The Surgery

If Jake needs to have the vocal cord surgery they said that he would be in the hospital at least a month. He would be on the ventilator and sedated for a week. They basically would have to take a small piece from his vocal cords and see how that works and if its not enough then they do it again. They can't take a huge chunk out because as you remove some of the cord you also lose voice quality. They want to find a good median. They also would put a graft in using part of his rib. It sounds like a very difficult surgery/recovery so I am praying we hit the crazy odds once again and cutting the scar tissue works.

If Jacob needs the surgery his voice will not be great quality no matter what. He will have a voice but because the cords won't be able to shut completely, his voice will become hoarse or wraspy. He also may have trouble drinking think liquids. The goal is to cut the least amount as possible but also allowing him to have more airway. As much as I don't want his quality of his voice affected I also know that his airway is more important.

WHAT'S NEXT?

I have been keeping Jake's cardiologist at CHOP informed every step of the way. Cincinnati is sending them the video and images from the scopes so she can show them to the ENT team at CHOP. Cincinnati is #1 for ENT however if Jake is going to be intubated for a long period of time and have such a difficult recovery I would feel more comfortable knowing that he is in CHOP with his cardiac team who knows all of his curve balls and history.

CHOP will most likely need to do their own scopes. I had hoped they wouldn't need to but Cincinnati told me that CHOP would need to do their own because images/video is not enough. You need to be looking with your own eyes to really see whats going on. This means we will probably have to go to CHOP soon for scopes. Then their ENT dept. will talk to Jake's cardiologist and surgeon and they will decide the plan of action.



We had hoped that Jake would not need surgery this year but as we know life with a hypoplast is very unpredictable. Right now we are just so happy to be home and so blessed that Jake is doing so well despite his challenges. He is my hero.

Thank you so much for all of the prayers and well wishes.

In the OR

Jacob is in the OR. Pulmonary and ENT are each doing a scope, they are removing his skin tag and he is getting a heart echo.

Yesterday they looked at his vocal cords and it looks like they are both paralyzed. 95% of the kids that have this need a tracheotomy immediately after they are paralyzed it is amazing he is doing so well. He will most likely need surgery to open up his airway in the near future.

We will learn much more after today's scopes. I will do a very long update with everything tomorrow. I am staying the night with him in the hospital tonight so will not be able to.

Please keep him in your prayers as he always has trouble with SATs after Cardiac Anethesia and yesterday it was found that his airway is very very small.

Thank you!