Tuesday, October 28, 2014

Long overdue update...

Hello Blog World!

Wow, I can't believe how long it has been since I have updated Jake's blog. I haven't written in over a year! I apologize for the lack of updates, so much has happened over the last year. One big and exciting event was Jacob's Make-a-Wish trip! Our family went to Disney World in April and it was amazing. I promise I will get to work on a separate blog post about the trip with lots of pictures. For now I thought I would just fill you in on some medical things he has going on right now.

The last time I blogged Jake had just come home from having his Fontan (Last open heart surgery). I am happy to report that heart wise he has been doing great! He weened off of all medication pretty quickly and is now just on the Lovenox injections twice a day and Enalapril (Blood pressure med) twice a day. He will be on a strong anti-coagulant for the rest of his life. We have chosen to keep him on the shots for now because he only needs to have blood work to check the levels every 4 to 6 weeks. When we switch to Coumadin he will need it checked more often and his veins are so scarred over that it is very difficult and traumatic for him to get his blood drawn. We will consider switching in a few years but for now he is doing well on the shots.

Getting his EKG, such a big boy now.
Jacob's oxygen levels continue to stay in the 80s which means his fenestration is still wide open. After the Fontan children can have trouble with fluid building up around the lungs, like Jake did. To help lower this risk some surgeons make a fenestration from the venous circulation into the atrium. When the pressure in the veins is high, some of the oxygen-poor blood can escape through the fenestration to relieve the pressure. Normally the fenestration will close over time, separating the Blue and Red blood which will increase their oxygen levels to the 90s, sometimes high 90s. Occasionally it will not close on its own and will have to be closed in the cath lab. It has been over a year and Jake's is still open. He will have a heart echo next month to check the Fontan and they will look at the fenestration as well. Jacob's cardiologist is going to consult with Jake's doctors at CHOP and discuss if he should have his closed. Normally it would not be that big of a deal but Jake has a clotting disorder. Any foreign object has the possibility of causing a clot. A clot in his heart could be deadly. If we don't close it then he will continue to have low oxygen levels which could contribute to his developmental delays. We need to weigh the risk vs. benefits and then will move forward from there. As of now the lower oxygen is not effecting his energy levels which is good. His nail beds are dusky/purple in color but the rest of his body stays pretty pink.

We went to Cincinnati Children's hospital a few months ago for Jake's annual appointment with the neuro cardiac clinic. This is where he meets with a team of specialists who evaluate his delays and give recommendations. As usual Jake is pretty delayed on paper which is extremely depressing especially because I can see how far he has come and how well he is doing compared to a year ago. They also suspect that he could have autism. This was a huge blow. I have always suspected it but it still hurt to hear. To know that he could have another label and more testing just makes me sad for him. It took me awhile to adjust to the news but I am ok with it now. In the end he is the same amazing boy who a year ago was barely talking and is now communicating all of his needs and talking in sentences. 

1st day of Pre-K
We get so many mixed messages, first they thought he had speech apraxia now possible autism. His local therapists said he doesn't seem autistic especially with how he is progressing but each case is so different. Cincinnati did say that if he does have it then they think he is very high functioning so he would be more like Aspergers. They said he is already progressing so fast which is a great sign. It is hard because they see him in a limited time span. They gave him a train set and then didn't understand why he didn't want to do their tests lol. Maybe because you gave him a new shiny toy! There were things he wouldn't do for them and then we came back home and he did it for the therapist here no problem. He tested high on all of the high functioning advanced skills but didn't want to do some basic things. Some of the tests were from lack of experience like "standing on one foot for so many seconds without falling over" (I can't even do that lol).

I am not denying he is delayed. He is and I am aware of the struggles we face and how "different" he is than a typical 4 year old. Communication is extremely hard. Although he can say his needs or tell me if he wants something, if I say "How was your day"... crickets. He is talking more and more everyday and is starting to talk in sentences now which is huge for him. He has a long way to go but for a kid with paralyzed vocal cords I think he is doing pretty amazing. We still struggle with the talking on inhalation and getting sound out loudly. This is common with kids that had his airway surgery and will most likely be a struggle for years to come. He does have traits of autism, he is very repetative and does play in his "Own little world" sometimes. Lack of eye contact is a big one. On the flip side he is very loving and gives me hugs all day long. I could go on and on but the truth is we won't know until he is tested and even then it won't change how we are approaching his therapy. He is already getting Speech and OT (occupational therapy) outpatient once a week and is also getting Speech and OT at school.

We go for his testing in January. It will be a two day event. They will be doing the ADOS testing and also an IQ test. No matter what I am so proud of him and I know we will overcome any obstacle that comes our way.


Now to the newest concern. Recently I have noticed Jacob's strider has been coming back. Since his airway reconstruction it has been normal for him to have a strider (Noisy breathing) when he is very active and exerting himself. At rest he is usual quiet. Lately I have been noticing he has been having the noisy breathing even at rest. It is not as bad as it was pre-surgery and not constant but it is noticeable and has been happening more often. I first noticed it when he was in bed falling asleep. At rest he should not be breathing like he is working out. I also notice it when he is coloring or doing other non energetic activities. His oxygen saturations are in the normal range for him and his heart rate is normal. He is not sick at all and lungs sound crystal clear. The good news is he doesn't seem to have Tachypnea. His breathing is from the belly not pulling from his ribs as if he were in distress like it used to be. It also is not effecting his energy or upsetting him at all. It worries me but he doesn't seem to notice. At this point it is just the strider.

I trusted my mama gut and talked with his pediatrician this morning and sent her a video. She agreed that it wasn't an emergency but was concerning and he should probably have a bronch to check things out. I then emailed his ENT doctor at Cincinnati Children's Medical Center. I sent him the video and asked if he wanted us to come in to discuss the concerns and possibly do a scope. He wrote back within an hour, and what was even more amazing is that he was at the airport about to fly out of the country.  I was so impressed but not surprised. The physicians and surgeons I have dealt with at different Children's Hospitals have time and time again shown they make time for patients and go above and beyond. They truly have a passion for what they do. This surgeon has seen Jake one time for a scope over a year ago, yet he took the time to write me while at the airport when he could have easily said "Call the office".

He told me that he did hear the strider and does want us to come to Cincinnati for a follow up. ENT and Pulmonology will scope him at the same time so he will only need to be put under once. He said it was not urgent but could be a arytenoid prolapse or may still be issues from the vocal cords. I won't lie, I am worried. The airway reconstruction was the hardest surgical recovery Jake has faced. His hands had to be tied to the bed for 10 days while he was given enough sedation meds to kill a horse. He went through horrible withdrawal, couldn't eat food and was on a liquid diet for months and had 3 IV infiltrations, one causing a clot in his Jugular and the start to the Lovenox injections. It is not something I ever want to see him relive again. That being said, if there is something going on then we need to know about it. I am praying that if he does have an obstruction to his airway that it can be fixed via bronch and he won't need surgery again. He will need cardiac anesthesia for the bronch which always comes with it's own risks especially with his airway issues. This trip will be basically to see what they find and come up with a plan. If they need to do intervention they will do that during another visit either by bronch or surgery. Until we get some answers I am going to TRY and not worry about that.

As a mom, I desperately want my baby to catch a break. He has hit so many rare odds I can barely keep track... HLHS, intact septum, paralyzed vocal cords, clotting disorder, fenestration not closing, possible autism and now his airway strider coming back. I want to throw my hands up and just say "NO MORE!" but that is not how this life works. I know that Jacob's life will be a constant struggle and there will always be obstacles to overcome. As much as I hate it for him, it is our reality. I can only hope and pray that this time, it ends up being nothing and we can go home and back to living our version of a "normal life". Anytime I start having a "woe, is me" moment I remember how lucky I am to have him here to deal with these issues. It could always be worse.

So that is the update. Right now we are working on figuring out what to do with the fenestration, putting the autism pieces together and most importantly following up with ENT and Pulmonology to make sure his airway is not becoming obstructed again. Jake's heart echo is in a few weeks. I will update when we start getting some more answers. Until then, if you could say a little prayer for Jake I would appreciate it. He is the strongest, most amazing little boy I know.  


Jake and Aidan as characters from Minecraft
"Our little Minecraft Family"

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