Saturday, January 17, 2015

New Year... New Updates!

Happy New Year!

I hope everyone had a great holiday season. The boys had a wonderful Christmas and got spoiled by Santa. Their elf (Ralphie) came back to stay with us again and Jake LOVE it. Jake really got into the holiday spirit this year. He especially loved all of the decorations. It was so cute seeing how excited he was over Christmas.

We did have one sad thing happen over Christmas, we had to say good-bye to our sweet dog Mojo. He went into heart failure. He started coughing really bad so I brought him into the emergency clinic. The vet said she suspected that he was born with a Congenital Heart Defect. I couldn't believe my ears when she said that. His heart was barely beating and he most likely would not have made it the weekend. He lived a long life but it was still so hard to say good-bye. I stayed with him the whole time and hugged him and told him we loved him as he passed. Aidan took it pretty hard but is ok now. It took Jake about a week before he started questioning where Mojo was. He kept asking if he went to the North Pole. Mojo passed away 2 days after Christmas, so I think he figured Santa took him or he went back with their elf. He still mentions him about once a day. He says "I miss Mojo, Mojo up in the clouds". We all miss him but take comfort in knowing he lived a long life and that he is no longer suffering.

Our Sweet Mojo
I finally got some answers so I can update everyone about Jacob's upcoming appointments. On my last blog post I talked about how Jake's fenestration was still wide open. He had a heart echo in November and it did confirm that it is still open which is why he continues to have low oxygen levels. Thankfully his heart function continued to look good. It is on the lower side of normal but that is typical for him. He does not have any tricuspid regurgitation (Leakage in the tricuspid valve), so that is great news as well.

As with all things pertaining to Jacob, whether or not to close the fenestration is not an easy decision. He has a clotting disorder so we were not sure if it would be too risky for them to go in and close it. He is at a higher risk of forming a clot. His cardiologist wanted to talk to his cardio at CHOP and discuss their stance on closing the fenestration, in particular in a child that also has Factor IV Leiden.

Dr. Stines (Jake's local cardio), talked to CHOP this week and discussed the situation. They said that his factor IV leiden would not complicate the procedure. They are pretty sure that the reason he threw the clot during his airway recovery was because his IV infiltrated. Many people have 1 factor IV gene but live their whole life without knowing it. It becomes a problem if you are immobile for long periods of time and when you have lines in. Jake had been sedated for 10 days and the line in his jugular vein infiltrated causing a ton of fluid to compress on the vein. So he will need to remain on a heavy anticoagulant his entire life but they are not afraid he would have any issues during a cath because of it.

That being said, CHOP is choosing not to close fenestrations that do not close on their own, unless it is causing the child to become very tired, low energy or affecting their daily life. Jake has a ton of energy and is constantly on the go. His nails have been very purple which worries me and he does want me to carry him all of the time but I think that is just because he wants his mommy to hold him. I carry him a lot because I don't want to push him and because I am a sucker lol. But seriously all day long he yells "Mommy hold you (Meaning him)". How can I resist. Wanting me to hold him aside, he is always playing, climbing and getting into trouble. He never takes naps so energy is not a problem. His lips continue to stay pink, it is just those purple nails. His oxygen ranges from 85 to 89. Usually sits around 87.

I am not going to lie, I looked forward to being post-fontan and seeing those numbers get into the mid 90s. Jake has always had developmental delays and having low oxygen levels is a contributing factor. I have come to terms with the fact that he will most likely always have low oxygen levels.

His Cardio at CHOP said that one of the reasons they are choosing NOT to close the fenestration is that they have found that it puts the kids at a higher risk for Protein-losing enteropathy (PLE), a complication that occurs in some post-fontan kids. So their thought is to keep it open unless it causes issues. We most likely won't notice issues until he is older. As he gets older if he has a hard time keeping up with his peers or his oxygen decreases, lips turn purple etc. then we will revisit. Good news is we won't have to spend part of our summer in the hospital! And purple fingernails are all the rage now, haven't you heard?

As for all of the other testing we had planned, we have pushed it back. Jake was supposed to go this week to have his airway scoped and have part of his autism testing. Recently Cincinnati Children's has been hit hard with the flu. One of the nurses told me the week we spoke, they had 250 confirmed cases. His strider has not been noticeable so they were comfortable pushing the scope until late spring, after flu season. The autism also isn't testing we need right away. Even if it comes back that he has it, it won't change anything. He is already getting outpatient speech and OT. It will open doors for opportunities in school and help qualify him for services later on so we do still want to have it done but it can also wait until after flu season. There is no reason to expose him to the flu which could be deadly, for tests that we can do later on. So looks like we will be heading up to Cincinnati around May.

Now that things have calmed down with Jake, it looks like Aidan may need surgery (Yes, you read that correctly). We can't get a break lol. It is nothing major but he is most likely going to need his tonsils out. We have always been told his tonsils were huge but it wasn't something we had to deal with right away especially with all we were going through with Jake. Every time Aidan gets sick his tonsils are so big they press on his uvula. He also has a major gag reflex because of it, they can't even do x-rays at the dentist without him gagging so bad he almost throws up. We are going on Monday to meet with the ENT and see what they have to say about it, I am pretty sure he will tell us it is time. Hopefully that can wait until this summer as well so he doesn't have to miss school. I know it is nowhere near the same as the surgeries we have gone through with Jake but I am still nervous. It is never easy to hand your child over to be put under anesthesia and I don't want to see him in pain.

Please keep both of my awesome little guys in your prayers.


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For those not on Facebook, here are a few more pictures from Christmas.