Sunday, June 24, 2012

Great Results...

We are back from Philly and excited to announce that overall we got Great News!


We left on Wednesday and headed back to our home away from home (CHOP). We had to be at the hospital at 6:45am on Thursday morning for Jacob's scope. We checked in at the Cardiac Prep and Recovery Unit and they took Jake back to prep him for his Broche. Jake was not a happy camper. He wouldn't let the nurse do anything without crying. We met with his surgeon and cardiac anesthesia and then they gave Jake Versed. For those who don't know much about this drug, it is the ultimate "Happy Medicine" lol. It basically calms them down and also has a bit of a amnesiac effect. Jacob had his head resting on my shoulder and I thought he was falling asleep. All of a sudden he popped up his head and started CRACKING UP. I couldn't help but to laugh at him. The medicine had taken effect. He didn't even mind the nurse checking his temp and other vitals which he usually hates.

The best part about Pre-Op was Jake's oxygen levels were 87 and above almost the whole time! This is great for him.  Before his airway surgery he would usually be between 78 and 83 but never high 80's. I have a pulse ox at home but only check it if I have reason to be concerned. I hadn't checked him since we first got home. I had hoped his oxygen level would go up after surgery but wasn't sure if it would. 78 to 85 is pretty typical for a Hypoplast but 87 is WONDERFUL!

Jake went back for his surgery shortly later. His surgeon Dr. Zur came back up less than an hour later to let us know that everything looked FANTASTIC. It took them longer to place the IV than to do the actual scope. His airway looked so great that he doesn't need another scope for 6 months!!!! That is great because they usually do another in 3 months but she said the airway has healed beautifully and she couldn't be happier. Dr. Zur said she doesn't expect any scar tissue to come at this point and thinks he will continue to do well.

I discussed with her what we should expect with his voice and function of his vocal cords. They are both paralyzed and it would be extremely rare for the function to come back at this point so most likely they will always be paralyzed. That being said he should still be able to talk. His vocal cords are still close together even though there is no movement. They basically opened them up enough to get air through but not too much that way he could still speak (Before it was covered scar tissue and only had a pinhole opening so he couldn't move enough air through). Jake was so vocal the past few days. He isn't saying words but he is making so many sounds. It is like he is babbling all of the time. He is starting to put words together as well. He will clap and say Yay! He will say Yeah and is mimicking us so much more now. It is amazing the progress he has made in the past month since he had his surgery. I am very hopeful and excited to see his progress.

I also talked to her about the strider we have heard since his surgery. She said there was some Laryngomalacia.
Laryngomalacia (literally, "soft larynx") is an unusual condition, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction. 
It was not bad but could cause a little noise when he is active. She said it should go away as he gets older and there is nothing they would do for it right now. His airway looked beautiful and the laryngomalacia was very small.

Dr. Zur said he may be able to stop his Prevacid in about 6 months (after the next scope). I could not have been happier with how everything went. We were brought back to see Jake and he was not the giggly boy we saw go off to the OR. He was PISSED! It took a while to calm him down but he did great and his oxygen and blood pressure looked perfect so we didn't have to stay long and he definitly didn't have to spend the night in the hospital. While waiting to be discharged Dr. Zur asked plastics to come take a look at the black blistered area on Jacob's head. I had asked her if they could since it was not found prior to being discharged after his surgery. I told them about the infiltration that happened with the central line. They looked at it and said it was a pretty big and bad blister but it has already started falling off. He said that it is also being held on by his hair and once the edges curl up more I will have to cut the hair behind it so it will come all the way off. I talked to them about how his hair was falling out. They said that with a blister that bad it is common for the hair in the area that was infiltrated to fall out all at once but it should all grow back. It will probably take at least 6 months before it grows back.

We were discharged from the hospital and made our way back to the hotel. It was kind of interesting because I got in the wrong lane and we ended up in Camden New Jersey lol. After fighting with the GPS on my phone we decided to do it the old fashioned way and my mom found a sign back to the Ben Franklin bridge. A few minutes later we finally were back at our Hotel. Jake was pretty fussy most of the night. He wanted to go home so bad. He kept bringing me his shoes and going to the door and waving bye-bye. It was so sad he didn't understand we had another whole day of testing.


The next day we had to be at the hospital at 7:30am. First we took Jake to have a ultrasound of his clot. He HATED this and screamed pretty much through the entire thing. After the ultrasound we went to hematology to discuss the clot in his jugular. This part was not the best news. Not what I was expecting. The clot hasn't changed at all. The GOOD news is that the clot has not gotten bigger. The bad news is his body has not absorbed any of the clot and at this point it is unlikely that it will. The hematologist said that after a certain point the body doesn't absorb it but turns the clot into a scar. Basically it fills in the jagged edges of the clot. It is the bodies way of "Fixing" what is wrong. It then hardens. The blood needs a way to move around since it can't go through that vein so it builds a bunch of smaller branches around it. She explained it a really great way. The juggular is like a super highway. When the highway is blocked the body created smaller backroads to move blood through. Those backroads are called collaterals.

They want us to go to Cincinnati in 6 weeks to do another ultrasound. If it is not changed at that point it most likely won't ever go away. I was concerned on if it could move and go to his brain and cause a stroke but they said that at this point he is not at risk for that. It is hardening and becoming a part of his body. The body basically looks at it like if it can't make it go away then it will just make it a part of it. So that was a big relief to hear. The shots (Lovenox) is doing it's job because it has been preventing more clots from forming in that area. She said if the next ultrasound shows that it is the same then they recommend going off Lovenox because the body won't be at risk for more clots forming at that point. It will be scarred over completely. Jacob's cardiologist at CHOP said that she wants him on the shots for at least 6 to 12 months. Probably because he will be having another open heart surgery in a year or two (Can you say vomit). Hematology is going to consult with her and see what she wants to do about the shots. He will at least be on them another month or two. Most likely he will continue to be on them longer but I am hoping we may be able to go to once a day instead of twice.


Our last test of the day was the Swallow Study. This was the one that we were really anxious about. Jacob didn't want to drink any of the barrium but they were able to see a few swallows. He did NOT aspirate at all, even with the super thin liquid that is like water consistancy! This is GREAT NEWS! ENT cleared him to eat any foods he wants. That was the biggest thing I had hoped for as far as eating. Just for him to be able to eat meals again and his cookies! So that was wonderful to hear. He is also allowed to start transitioning to thinner liquids. Because she was not able to see him gulp a ton of the liquid she was not comfortable with just giving him a cup and letting him chug at home. It makes sense that we need to transition. We have a good plan. I am going to start mixing his sippy cups to be necter thick. It is about half as thick as the "Honey thick" he was on. I am going to give him one to two of these a day and still use his tube about 2 times a day or to make up for whatever he doesn't drink from the cup. They told me how I can naturally thicken his liquids without buying the expensive thickeners. I can put apple sauce in apple juice, pudding in Chocolate milk etc. Then as long as he does well with the Necter thick then we can go back to thin liquids. I am also controlling his portions of food since he is a crammer (He shoves tons of food in his mouth to the point he can't chew well). I will also be controlling his sips from the sippy cup at first.

When we go for his scope in 6 months I am also going to see speech and if he is no longer using his tube we will discuss getting the g-tube REMOVED! It will probably take a month or two to get him back to his normal eating and transition the liquids but I am so excited we can start moving in that direction. Jake was super excited because he was able to have what he has been asking for all month...


After his appointments we went to the cafeteria and ate so we made sure to get him the biggest cookie we could find since he did so well! He of course started cramming it in his mouth so I had to break it up and give him small pieces.

We didn't want to watch Jake be sad all night at the hotel again so we decided to hit the road. Had the weather been cooler we may have stayed but it was over 100 degrees and there was not much we could do. So around 2pm we were packed and started our 9 hour journey back home. We got home around 11:30pm. It was a very long day but worth it to wake up in our own beds!

I had told Aidan that if Jake did well on his test we were going to throw him a COOKIE PARTY! I am true to my word so tonight I invited my parents, my sister and her kids over. Andy's mom is also in town so she was here to celebrate as well. I made some cookie monster cupcakes because what is a cookie party without the cookie monster! I had to improvise on the eyes because the store didn't have what I needed so i used coconut. I don't think they turned out too bad considering I threw it all together in a few hours. We all sang "Happy eating day to you..." Jake was glowing he was so happy.

It was a great night.

• Airway looks wonderful
• Clot hasn't changed
• Jake can eat solids and start transitioning back to thin liquids
• Follow-up with hematology in 6 weeks at Cincinnati Children's to check clot
• Follow-up with ENT in 6 months for another scope of his airway
• Follow-up with Speech in 6 months to evaluate getting his tube out
• Possibly getting off Prevacid in 6 months
• Unsure of when he will be done with Lovenox
I can't thank everyone enough for all of the prayers that were sent up for our little warrior. We couldn't be happier with all of the results. He is doing so well and I see progress daily with his speech and development. I am so hopeful and could not be more proud of my boys!

Here are some more pictures of our cookie party! 

Monday, June 11, 2012

Just when we get settled... it's time to head back.

I am SOOOO sorry I have not wrote in so long again. I promise I mean to but life happens and I just have been so busy lately that unfortunately the blog has been neglected. I think I take for granted that everyone sees my posts on FB and forget that not everyone is on facebook. So for those of you who have been waiting patiently for an update I apologize. I have been working on some pretty exciting projects and I can't wait to share them with everyone but this post will be long enough with Jake's update so I will do a separate post soon letting everyone know what has been keeping me so busy!

Now for the reason you come here... Jake Update! 

Jake is doing wonderfully! He has made really great progress since we have been home. The biggest change is he is interacting so much more with us. Before we were concerned because he had a blank expression on his face a lot of the time and didn't look if someone came or left. He pretty much could care less what was going on around him. Now he is engaging and waving and interacting with everyone which is so exciting for us. He is also signing appropriately which is so nice. Before he used to sign just to sign but didn't really use it to communicate. It was kind of like baby babble but with signs. Since we have been home he is using it to tell me his needs. His tantrums have pretty much gone away completely (Other than typical 2 year old fussiness). Tonight he even signed "Time Eat" which made me so excited because he put to words together appropriately. That is the next step in his signing. If you think about it like a child who learns to talk. First they babble, then they start using the words appropriately and then they start putting words together to make sentences. Jake is following the same progression just using signs. He is trying so hard to be able to communicate with us more and I am such a proud mama. He makes up his own signs sometimes which I don't always understand but I try to learn from him and eventually figure out what he is trying to tell me. He started signing Banana but he wasn't using the correct sign. I wasn't getting it so he walked me over to the counter and pointed lol. Then I knew that what he was telling me is his sign for banana. He is a pretty smart kid :) He made up a sign on his own for an iPad which is him holding up his hand flat and then using his other hand to look like he is pushing buttons on it.  I know most kids his age are talking a lot by now and he is delayed in speech but I have to say I am amazed by his ability to overcome his disability and find ways (His own way) to communicate with me. He is seriously my hero and I am beyond glowing with pride. 

I was concerned for a while when we got home that his strider was returning. After sending video's to his surgeon at CHOP and working weekly with his speech therapist I am not concerned anymore. CHOP said that there was nothing they heard that was concerning to them and that they agreed with our speech therapist here that Jake was just finding his voice. He likes to hear himself talk. He is starting to try and talk some. Jacob could possibly have Apraxia of Speech. It is commonly seen in children who have a rough start in life. Apraxia is basically when the child can understand what they want to say or what you are saying but they have difficulty communicating it. It is something that can be overcome but we basically have to retrain his brain. This starts at a very basic level where we first tried to get him to open his mouth when we asked. Now we are working on vowel sounds. He has started doing those but still keeps his lips pursed most of the time. 

The other day his speech therapist was here and I was telling him to draw a circle on the chalk board. He drew would then draw a circle (Which by the way is a 3 year old skill) and we would clap and say yay. One time we didn't say yay quick enough and he turned around and in a very deep crisp voice he looked at us and said "YAY!". Our mouths just dropped because the sound was so amazing. It was so crisp and not raspy like he does sometimes. Plus he was SPEAKING, not just making babble. That same day he was signing hat and tried to say it, he said "Haa". He is trying and that makes me so happy. I have always said that even if he never talks we will still be so proud of him and we would teach our whole family to sign for him. But seeing him try to communicate and seeing how happy it makes him really melts my heart. 

Brynn (Jake's speech therapist) and I have talked a lot about the fact that Jake probably could not hear before his surgery. His breathing was so noisy it was probably hard for him to hear anything but his own sound. We had a hearing screen done and they said he could hear loud noises but it was inconsistent with the quieter noises because of his breathing. The hearing specialist also told me that for kids who have to work so hard to breath like Jake did can have their ears pop a lot because of it so that can also affect hearing. So he may not have interacted much before because he couldn't hear us. When I would get frustrated because I would call his name 10 times and he wouldn't turn around, maybe he didn't hear me. Since we have been home he does "Hear" me. I call him and he comes. When I say we are going bye-bye he gets up and goes to the door and follows me to the car instead of running the opposite direction. The other night I told him it was time to get out of the bath and he stood up and held his arms up for me to get him out. This may sound like small stuff to everyone else but for us it is HUGE. To be able to know that Jake understands me and see him progressing so much it just is such a relief for me. I feel like we can connect so much better now. I know we have a long way to go but just in the past month Jake has come so far. 

Physically Jake is back to normal. He is climbing on everything. He is running and playing better than ever. The black spot on his head where the skin died from the infiltration is still there but has started to lift on the edges. I am kind of afraid for it to fall off. I have no idea what it looks like under there. I also noticed the other day that he is starting to lose his hair across the bottom of his head where all of the fluid was. I got upset when I noticed the bald spots. I know it is very minor compared to everything else. It is hard to explain but when you have a child with a life threatening illness you are on edge all of the time. You question everything worrying that even the little things can be big things. I don't dwell on his illness, many days I don't even think about it. But when you notice something your mind starts racing, after everything he has been through how can it not. Then you question is it something, is it nothing, should you call the doctor or just wait and see. So there he is playing in the bathtub and I am staring at the shape of his head worrying. Thinking his head looks big in that area and maybe he still has fluid under his skin and that is why the hair is falling out. Then I started comparing his head shape to Aidan's which is silly since everyone has a different shaped head (Yea, I probably need therapy lol). After a few moments of panic I convinced myself that his head shape is fine and there is no fluid, the hair is falling out because of the infiltration and the damage the fluid must have caused to his hair folicals when it was swollen. I just pray that the hair comes back eventually because Jake has had to deal with so much. Yes this is minor but there are so many things that they add up and I don't want this to be yet another thing for him to have to deal with. 

We finally have gotten settled and now we are planning to head back to Philadelphia. Andy and my mom have to work so Jake and I will be heading to Philly on our own this time. This will be my first time driving there by myself so I am a little nervous about not having anyone to help entertain Jake in the car but we can do it lol. Aidan will have to stay in WV though because he is not allowed to be back in the Cardiac Prep and Recovery Unit and I have to be back there with Jake before and after the scope. My parents and my sister will watch Aidan while Andy is at work. I have to head up on Wednesday. Jake will have his bronche on Thursday. He should be outpatient and be able to go back to the hotel that night. Friday morning we will head back to CHOP and meet with Hematology. They will do a ultrasound of the clot to see if it has shrunk any. Then we will go for a Swallow Study to see if he can start eating by mouth again. He is doing good with the purees but still misses getting to eat like he used to. Please pray everything looks good! 

Provided everything goes as planned we will be coming back on Saturday. Then we will have to go to Cincinnati every 2 months for follow up bronches and ultrasounds of the clot. It is going to be a very long year! I really hope the clot is shrinking but even if it does shrink he has to remain on the lovenox shots for at least 6 months to a year and they are talking about keeping him on it until his Fontan which may even be 2 years away. His poor little arms and legs are so bruised up from the shots. I can't wait until he no longer needs them but it will be a while. I think I took for granted how "Normal" our life had become. Now I feel like we have been thrown back into the pre-glenn phase where he is on more meds and tube feeds. Worrying about him aspirating. I can't tell parents of healthy children enough to never take anything for granted. I wouldn't trade any of it for the world though. Jake is worth all of this and more. He is amazing, my miracle. My hero. 

Please continue to keep my little warrior in your prayers. I promise to give updates on Thursday and let everyone know how he does. 


Aidan and Grandpa planted Aidan his first garden!