Wednesday, September 19, 2018

Honeymoon is over... time to be proactive

Hello Blog World!

It has been 3 and a half years since my last post... where have we been you ask? LIVING!!!

The past 3 and a half years we have been living pretty normal lives and it's been awesome! Jake has been growing like a weed and other than a cardio visit once a year and a baby aspirin every night, we really haven't had to deal with anything related to his medical issues.

I want to start by saying JAKE IS GREAT! Ok now breathe a sigh of relief.

That being said, the honeymoon is over and it's time to dive back in to the life of a heart mom. Over the past few months we have noticed Jake's breathing has increased in noise. Not as bad as before his airway surgery but definitely more noticeable and a little concerning. Today he had a checkup with both his doctor and his cardiologist. Here is the gyst of it...


His doctor and cardiologist both agree it is time for a scope. His airway was rebuilt about 6 years ago and he hasn't had any follow up scopes in years. With the increased sound in breathing, we need to make sure that the scar tissue isn't causing narrowing. It could be nothing but if it is something we need to know. We will be headed to Cincinnati soon for a sedated scope and to meet with the ENT team to discuss.


GOOD NEWS is his heart echo looked great. No valve leakage, good function. The fenestration is still wide open. At this point it is highly unlikely it will close. We have gone back and forth on closing it. Honestly these surgeries are so new that there is still so much that is not known. Jake is a pioneer and the oldest survivors are only in their early 30's if that. Some hospitals close it if it has not closed on it's own in a year but CHOP (Philly) tends to leave them open. Their thought process is that closing it will put him at risk for PLE and plastic bronchitis. BUT with it open Jake's oxygen levels are only around 85% at rest. We are pretty sure that when he is active they are dipping to low 80's which causes him to be tired and have labored breathing. Another issue with it remaining open is the fact that he is at a greater risk for a stroke. 

SIDE NOTE: For those that don't follow us on FB, my dad recently had a stroke and passed away. Writing this blog is actually so hard because it is my first post since he passed. My dad loved this blog. He used to get so excited to see how many followers Jake had and always pushed me to write more. I know he would be happy to see I am utilizing it again... Miss you so much dad. This one's for you!

Because of what we went through with my dad, the thought of Jake having a stroke scares me so bad. The increased risk plus his clotting risk from his factor IV Leiden gene is pretty scary. His cardiologist also said that all post Fontan patients are are at risk for PLE and Plastic Bronchitis. Its just a fine line. The thought of a stroke scares me but I am also so afraid to mess with his heart knowing his function is good and it scares me to mess with it.

IF we go ahead with closing the fenestration he will likely have to be placed on coumadin or the Lovenox injections again because he will be at risk for clotting after the surgery. Anytime a foreign object is inserted into the heart via cath there is a greater risk of clotting, especially for Jake.


Traveling, testing and discussion. First the airway scope to check on his airway. Waiting on that to be scheduled with Cincinnati.

November 27th we will head back to our home away from home.. Children's Hospital of Philadelphia. We will be participating in the Single Ventrical Survivorship program and Jake will have a ultrasound of his liver, heart echo, bone density scan, labs and a stress test. A team of doctors will review his results, discuss his case and meet with us to discus the findings and what they feel our next steps will be. We will discuss the fenestration at that time and if he will have a cath to close it or leave it alone. This will be a 3 or 4 day process and Jake is older and more aware now so pray that he will be able to handle all of the tests both physically and emotionally.

The Single Vent Survivorship program is actually one of the only clinics of it's kind so we are really lucky to be able to participate. They are on the cutting edge of HLHS treatment. There is no sugar coating that Jake may face a LOT of scary stuff in the future. I don't talk about it or think about it much because I swore a long time ago to not focus on the "What if's" and just give Jake the BEST quality of life I could. Truth is that Jake will most likely face liver problems including liver failure, he will most likely need a heart transplant one day, he could face PLE or plastic bronchitis. These are all issues that ALL fontan patients face. Their bodies are rewired to work in a way that they were not created to work. Their circulation is reversed and there is pressure on the liver and the heart is working harder than it should. The purpose of the Single Vent Program is to monitor the post fontan patients and stay ahead of the issues they know will arise so that we may begin treatment early and try to attack each issue head on if it arises.

We will also be participating in a study that will do a MRI of the brain. This study looks at neurological and developmental effects of HLHS. Many HLHSers have some level of ADHD or developmental issue. Jake has ADHD and mild autism so I think it is a great study for him and many other single vent kids. Ill talk about his developmental issues another time. In true Kathy fashion, I have already gone way longer than I intended.

In closing, we have loved living the past 3 years without focusing on any medical issues but the truth is, it is not something I can run from and the best thing for Jake is to be proactive. The testing is just a part of this life and staying ahead of it all. It won't change the way we raise Jake. He has no idea how serious his condition is because he is a kid and I want him to be a KID. This is my burden not his. When he is older, we will of course explain it all and help him navigate everything but for now, other than knowing he has a special heart, he has no idea he is any different than his friends. I would never want him to "Fear" life. What kind of life would that be? It wouldn't be good for him or for us. He is amazing, he is happy and as far as he knows.. he is just a wild 8 year old who just started 2nd grade!

I will try to keep everyone posted as we navigate through the next few months of testing.

This blog post is dedicated to Jake's buddy. My amazing, wonderful rock.  My dad who I miss more than words.  You are Jake's angel now dad and we need you to watch over our sweet miracle boy. We love you so much. <3 b="" nbsp="">

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