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...

AIRWAY

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.

HEART

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.

WHAT'S NEXT

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="">

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.

Love,



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

RALPHIE!

 POLAR EXPRESS!

CHRISTMAS!

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.

HEART

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.

AUTISM?

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.

 

AIRWAY

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.  

HAPPY HALLOWEEN! 

Jake and Aidan as characters from Minecraft

CHEESE!!!!!

"Our little Minecraft Family"

Home and LOVING IT!

THANK YOU FOR ALL OF THE PRAYERS!
 As you can see, they worked.

I am sorry I have not updated the blog since we have been home but we have been busy. Jake is doing AMAZING. He is pretty much back to normal. Happy, playing and pretty much pushing every limit and restriction he was sent home on lol.

As soon as we got home I set up my command center. 

Anyone who knows me also knows that I do better when I am in control (I am a serious control freak). Jake being sent home on all of those medications made me feel pretty uneasy but as soon as I set my board back up I felt so much better. I only really needed it for the first day or two but still it makes me feel better to have it lol.

We have been pretty busy with appointments since we have been home. Last week we had 2 chest x-rays, blood draw and two cardiology appointments. When we left CHOP Jake still had some fluid on his right lung. They increased his oral diuretics and we were able to come home. By that Monday the chest X-ray showed almost no fluid! It could be the added diuretic but I think it is because he was happy to be home and out of that hospital bed.

The X-ray on Friday looked even better. There was no fluid! Yay, now lets hope it doesn't come back. We went back to the Cardiologist today and the X-ray still looked awesome so we were able to get rid of one of his meds! Diuril is now cut off that long list pictured above. If things look good on the X-ray next week then he can drop the Aldactone and Sodium and the Lasix can be cut down to two times a day. That would mean we would be down to only 4 medications a day. We are headed in the right direction! We have to slowly ween off of the diuretics to make sure the fluid doesn't start building back up.

Jacob's oxygen levels are still hanging out around 85. This is normal. It could take 6 months to a year for his fenestration to close and the oxygen levels to get up into the 90s. If in a year they are still low then he will need to have the fenestration closed through a Cath. Hopefully that won't be necessary and those numbers will start to go up on their own. Either way it is not something we need to worry about right now.

Jake is back to his crazy 3 year old self. He is still technically healing yet you would never know it. He is climbing on everything despite me telling him he isn't supposed to yet lol. I woke up the other day and he was in my bed. He climbed up on the table next to my bed and climbed in bed with me while I was sleeping. SERIOUSLY JAKE. A few days ago I looked over and he was trying to go down his little slide on his belly. I ran over and grabbed him and he started laughing at me. He may be driving me crazy with worry but it is so awesome to see him back to normal. A smiling, happy rambunctious three year old boy. He amazes me daily.

I will try to write again soon but as always no news is good news. If I am not posting that just means we are living life and enjoying every minute!