We made it...

We are officially Post-Fontan!!!!

We have dreamed of this day since Jacob was in my belly and we found out he had HLHS. We knew he would need 3 open heart surgeries to survive. Back then it seemed so far away. The day we could be Post-Fontan, the day we would no longer have this surgery looming over us. That day is here. We did it, we are officially Post-Fontan!

Honestly, part of me is nervous because he is doing so well. I keep waiting for something to happen. Thankfully he has been stable and has done better then I could have imagined. 

We started off the day by running late. It never fails, our alarm clocks didn't go off and the iPad I set as a back up ran out of power and turned off. So we woke up 3 minutes before we were supposed to be there. I only woke up then because my mom happened to wake up and see what time it was. Thankfully we were right down the street at the Ronald McDonald House. We got dressed, parked and made it upstairs in the hospital in 20 minutes! He was having his MRI study first then going straight into his Fontan. They gave him versed to calm him down before we went and boy did it work. He was punch drunk and laughed the whole way down the hall as they were wheeling him to the OR. He kept saying "Weeeeeeeee" It was adorable! I was starting to feel the tears coming as I kissed him good-bye then I turned around and saw the doctors open the doors and Jake Pointed down the hall. It was like he was saying "Lets do this". I couldn't help but smile. Off he went, my amazing strong boy.

Everything went very well. Surgery went smoothly, Jake had no issues coming off of the bypass machine or the ventilator. He was extubated by the time we saw him. He only had one chest tube, a inter-cardiac line which goes directly into his heart, and an arterial line. They had to put the art line into his groin area because his his veins and arteries are so used up. They tried the hand but were afraid pushing it anymore could risk him not getting any blood flow to his hand which would be VERY BAD. He only needs the art line for the heperin they are giving him, once they get him to therapeutic levels they can switch him to lovenox. He is on the heparin because of his factor IV Leiden gene. He is at risk for clotting so we need to have him on anti-coagulants for a few months post-op. They have already gone down on his heart med (Milrinone) and he may not even need enalapril (Blood pressure med he was on at home) anymore once he goes off of the milrinone. He is getting some dex to help take the edge off. It is like a Valium but without the blood pressure side effects. He is also getting Fetinal for pain but we are transitioning to morphine. I know I am not spelling any of these meds right but forgive me I am too tired to look it up.

Dr. Spray, Jacob's Surgeon
 We are so blessed to have him as Jacob's Surgeon.
He is not only one of the best pediatric heart surgeons in the country, but he is a great guy too!

His oxygen levels are still a little low but he is still pretty sedated so that is normal. He needed a lot of fluid pushed earlier to keep his blood pressure where it needed to be but they said that is very common after the Fontan. It is a way of getting the volume in him to "Kick start" the new circulatory system. It has balanced out now and they actually are starting diuretics to help get rid of fluid now. It is a balancing act. He has not needed any blood transfusions since coming back. They do get blood in the OR on the bypass machine but he hasn't needed any transfusions.

He is not draining that much blood into the chest tube which is great. This may increase tomorrow as his body adjusts to the new pressure in his body. If he continues to do well they are going to shut off the sedation tomorrow, wake him up and get him moving more. The plan is to remove the inter-cardiac line tomorrow and possibly his art line. The chest tube will stay in for a while but it is really small and can be pinned to his pants so he can walk. They said they will get him up and walking around tomorrow or Saturday depending on if his art line can come out or not.

The plan is to keep him sedated and calm tonight. He is waking up once in a while but I can easily calm him right back to sleep.

Thank you so much for the continued prayers for our precious little warrior. He continues to amaze me with his strength and will to fight. The next few days will be rough but I will try and update when I can.

Back on Schedule...

We got the call we have been waiting for today. CHOP called to let us know that Jacob will be having his Fontan tomorrow morning. We also got into the Ronald McDonald House. Things are finally falling into place.

We need to be at CHOP at 5:30 tomorrow morning. Jacob is first on Dr. Spray's schedule. First he will have a MRI scan of his brain. This is a research study that we are participating in. They will look at the blood flow to his brain now and then compare it with the blood flow in 6 months after he has his Fontan. This will allow them to see if the difference contributes to developmental delays. We also wanted to participate because it will show us if Jacob has ever had a stroke or if there is anything else contributing to his speech delays.

After he is taken back we will go meet with Dr. Spray to discuss the surgery and ask him any questions we may have. Then he will go down and once again operate on our beautiful baby boys heart. This is the third time we have handed Jake over for open heart surgery, 5th surgery he has had (g-tube, airway). It never gets easier to hand him over but we know he is in the best hands possible.

The past few days have been crazy but I am glad we are back on track. The boys had a great night tonight playing and exploring the Ronald McDonald House. Jake was in LOVE with the trains. 
 

This is the first ever Ronald McDonald House. The architecture is beautiful!

Aidan met a new friend, Perry. They built a really cool dragon out of legos.

Please pray for our baby boy as he enters this next stage in his journey. I will try and post as soon as I can to let everyone know how it went.

The waiting game...

I would love to update you by saying we are finally Post-Fontan but plans have taken a different turn. Jacob's surgeon went home sick yesterday with an acute viral illness and his surgery was cancelled. It has been a crazy couple of days but here is an update about what has happened so far.

We arrived in Philadelphia Sunday evening. Yesterday our day began at 8am at the Cardiac Prep and Recover Unit. As soon as we were taken back and Jacob saw the hospital gown he started crying and yelling to go home. We finally got him changed and calmed down but he was not a happy camper. Thankfully we had the iPad so he could watch his favorite movie "The little Engine that could".

Child life came by and gave him some toys. They had a police car he could get in and ride so he cruised the halls for a while. I was excited to see that his oxygen levels were 86/87 the whole time we were there! That is awesome for Jake.

While waiting to go for his cardiac MRI we had our surgical consult and learned step by step what they would be doing in the surgery. We signed all of the paper work giving them consent. We also met with anesthesia to go over risks and sign those forms. Jacob's nurse gave him a cute train pillowcase and a "Shadow Buddy". The Shadow buddy is an adorable little doll with a heart line on his chest and a heart patch. He comes wearing a gown and with a bag full of items for the kids to use to "Treat" him. It is a way to teach kids about the surgery and what may happen to them. We named him J.J for Jacob's Journey. The boys (especially Aidan) loved playing doctor with him and fixing him up.

When it was time for his MRI Jacob was given versed (His happy juice) to help take the edge off. Unfortunately it did not work as well as it has in the past and he was still giving us a look like he didn't trust any of us. I went back with him while they put the mask on and put him to sleep. I hate that part, he fought them the whole way. He was not going down without a fight. The MRI took a few hours, while they had him back there and sedated they went ahead and did his echo and took all of his labs. I was so grateful for that because getting blood from Jacob is very difficult. Once Jacob was awake and drinking juice he was taken down for a chest x-ray. They also did an ekg and then it was time to get discharged!

Sleeping after MRI

Before we got discharged we learned that Jacob's surgeon Dr. Spray went home sick. Any heart mom can tell you that if you find out your surgeon is not there you kind of panic. We were told to go back to the hotel and plan on being back at the hospital at 5:30 am unless they call and tell us otherwise. Later that night we got a call that his surgery was cancelled because Dr. Spray was too sick to come in. They had no idea when we would be rescheduled and said they would call and let us know.

As you can see Jacob was really sad that his surgery was Cancelled lol.

I spent all morning today on the phone with people trying to figure out what is going on or where we could stay. The Ronald McDonald House in Philadelphia and New Jersey were both full and wouldn't even consider letting us in if they had room because Jacob was not admitted yet and there was no plan as to when he would be back on the schedule. I tried talking to the hospital and the RMH social workers but there was nothing they could do. We were already in our second hotel but it would be expensive to continue to stay there. We decided to come to Holiday Inn, in Cherry Hill New Jersey because they had a hospital rate of $63/night which was about half of the price of what we were paying in Philadelphia and there was no parking fee. In Philadelphia you have to pay $20 to $40 in parking fees (CRaZy)!

The GOOD NEWS of the day is that Jacob's heart echo and the preliminary MRI results showed that his heart function looks great. Clinically he could wait longer for his Fontan if he had to. Although that makes us very happy to hear, we don't want to have to wait until later this year for his Fontan because we are already here. My husband has used vacation time, we drove 9 hours and have already invested in this trip. If we were pushed back until the fall Aidan would be in school and Andy's vacation time would be used so he couldn't come either. We tend to think of the surgeons as super heroes but they are human and these things happen. I understand that but I also feel that Jacob should get priority since he was scheduled back in February and we have come all this way.

I called the scheduling department tonight but they said they still couldn't tell us anything. A short while later I got a call from the Chief of Anesthesia, Dr. Nicolson. She was so nice and apologized that we were not contacted earlier today. She said that Dr. Spray has only cancelled surgeries because of illness two times, this being one of them so he is really sick. He spoke to Dr. Nicolson earlier and told her that he would see how he feels tomorrow and IF he feels better then he will come in tomorrow. If he comes in they will meet and discuss if he is well enough to do surgery on Thursday. If so, Jacob will have his Fontan then. Everything depends on how Dr. Spray feels. Obviously we don't want him operating on Jake if he doesn't feel 100%. This is heart surgery on a child so he needs to be at his best. We also can not risk Jacob getting any illness which could hurt his recovery or worse. We spent weeks trying to protect Jacob from any illness so we can't risk him getting sick in the OR. Dr. Spray is an amazing doctor and I know he will not do anything that could risk Jacob getting sick. If we have to wait then we will but hopefully it is not too long!

We discussed the possibility of using a different surgeon. CHOP has three caridac surgeons. One of them is not in town, she is on vacation until late July. The other one is booked this week. So we are pretty much just in a waiting game. We need to take it day by day and see what happens with Dr. Spray. I was very concerned but I feel much better now that I spoke with Dr. Nicolson. She is a very good person to have in your corner when needing to get on the schedule. She is going to call me tomorrow after she talks to Dr. Spray and give me another update. We should have a plan then (I hope).

So 3 days, 3 hotels and a whole lot of stress but thankfully Jacob's heart looks awesome and that is the only thing that matters. He has also been talking like crazy since we have been here. He has been responding to questions and talking in sentences which is awesome for Jake. Thank you so much to everyone who has reached out to us. Your generosity and kindness has been greatly appreciated. Please pray that Dr. Spray is feeling much better soon and we get back on track.  Until then we will be hanging out in Jersey...

Entering the next stage in our journey...

Ok so I have been avoiding writing this blog post like the plague. Seriously, I have sat down to write this countless times in the past few months but just couldn't do it. There is no more avoiding it. 2 weeks from now we will be in Philadelphia for Jacob's third and final planned open heart surgery (The Fontan).

Before I get into that let me catch you up on some other pretty big things that have happened since I last blogged (which was months ago!).

Jake and Aidan had their birthdays. Jake is 3 and Aidan is now 6. My boys are getting so big so fast! We partied hard this year lol. Each of the boys had small family parties and then we had a big combo party at pump up the fun.

Jake started pre-school. He is actually on summer break now but he had 2 months in a special needs pre-school class. We love his teacher Ms. Jessie. He did so well. I know it is going to be really good for him to be around other kids his age and have that interaction. He will also get speech twice a week. That is in addition to the weekly outpatient speech he gets.

Jacob's local cardilogist retired. Dr. Heydarian is like family to us. He took care of Jacob like he would his own son. He would call us when we were out of state for surgery to check on his "Buddy". I am very happy for him that he got to retire but we will miss him.

Aidan graduated Kindergarten! I can't believe that I now have a first grader. I am so proud of Aidan. His did so awesome this year. We were so blessed to have an amazing teacher. We love Mrs. Clark and she set the bar really high for any future teachers. Aidan is so smart, he got all M's on his last report card. M stands for mastery and is the best grade you can get!

 
Aidan also lost 2 teeth over the past two months. He is getting so big!

 

THE FONTAN

The Fontan is the third in a series of open heart surgeries that Jacob needs to survive. There are two different methods, Jacob will be having the extracardiac fontan.

The Children's Hospital of Philadelphia has a great video series which describes the surgeries for Hypoplastic Left Heart Syndrome.
Click here to watch the video and learn more about the Fontan.

The phrase Post-Fontan is music to the ears of single ventricle parents. It is what we look forward to, it is a huge milestone for single ventricle kids. Their oxygen levels increase and they have a lot more energy (I find that hard to Believe because Jake is constantly going lol). We know from the get go that our kids need 3 open heart surgeries and we dream of the day that we can say they are Post-Fontan.

Although I can't wait to be post-fontan, I have to go through the darkness to get to the light. I am not looking forward to the next few weeks or the next few months for that matter. It makes me physically ill to think of having to hand Jacob over again for another major surgery. The recovery after his airway surgery was so rough on him. I hate the thought of him being scared and in pain. Despite the fear I know that Jacob needs this. I know he can't survive without this. I know I am strong enough to get him through this. And I know he will rock it. He has to, I can't let myself think any other way. It will be hard, it will be hell for a few days (Maybe weeks) but I am just dreaming of the day we get to come home with our amazing little boy and celebrate being Post-Fontan!

THE PLAN

July 14th
We will be driving 9 hours to Philadelphia (Our home away from home). We are on the list for the Ronald McDonald House but it usually takes days sometimes a week or longer to get in there. Please pray we get in because staying at a hotel is really expensive and we can't afford that.

July 15th
We have a long day planned for pre-op at the hospital. Here is the plan.

• Cardiac Echo- ultrasound of the heart
• EKG
• Bloodwork- If you have followed this blog for a while you know how difficult it is to get Jacob's blood. His veins are all scarred over. They usually have to get him by using an ultrasound guided IV placement. Please pray they get him without hours of screaming and poking. It breaks my heart. 
• Sedated Cardiac MRI- Jacob's echo looked so good the last time they didn't think he needed a heart cath (YAY!). Many hospitals are now doing Cardiac MRIs instead of caths. This is a 3D rendering of his heart. I am actually kind of looking forward to seeing this. 
• Sedated MRI of the Brain- CHOP is currently doing a study which I am actually really interested in. They are doing a study to see if the difference in blood flow to the brain Pre and Post-Fontan has anything to do with developmental delays in single ventricle patients. They are going to do a MRI of his brain before his Fontan and another 6 months after and see if the difference in circulation has any effect on brain development. Another reason I am interested in this scan is when we went to the neuro-cardiac clinic in Cincinnati they recommended we have one done because of his speech and developmental delays. The MRI can show us if Jacob has ever suffered a stroke without us knowing about it. He also had a bad fall a few years ago and it will show if there is any scarring which cause damage to his brain that could be causing delays. It won't change anything in the course of therapy and there is no reversing any damage if it occurred from either the fall or a stroke but it would help give us more pieces to the puzzle. I told them we didn't want the MRI done at the time of the clinic because we knew they would be doing one before his surgery. They agreed that there is no urgent medical reason that we couldn't wait so that we wouldn't have to sedate him twice. This way he can get it done while already under anesthesia.
• Meet with Anesthesia- Sign lots of forms saying I understand all of the risks
• Cardiology Appointment- Meeting with his Cardiologist at CHOP to go over results and surgery.
• Hematology Appointment-  We need to meet with the hematology team to discuss his post-op treatment. Jacob got a blood clot while recovering from his airway surgery. They did bloodwork after that and found out he has a rare factor IV leiden gene mutation. This gene puts him more at risk for clotting if he is immobile for a long time or after major surgeries. Because of this he will need to be on a stronger blood thinner after his surgery. They will be giving him Heparin. He will also need to go back on the lovenox injections. Remember those shots we had to give him for months after his airway surgery. How can I forget, we hated them. Well he will need them again. The good news is it should be a prophylactic dose. That means he may not need to travel to Cincinnati every month to check his levels etc.

July 16th
SURGERY. Jacob will undergo his third open heart surgery. We yet again hand over our baby to the man with the magic hands, Dr. Spray. It never gets easier handing our baby over for surgery but we are really lucky to have such a talented surgeon. Dr. Spray is world re-known. I know Jacob is in the best hands possible.

We were told to expect 2 to 4 weeks for recovery.  Andy, my mom and Aidan are staying for 2 weeks. If we are not home by then, they will head back to WV and I will stay with Jake at the hospital. My mom will watch Aidan while Andy is working and sleeping. I am praying we can all come home together after a week but I always say.. I plan for a few months and pray for a few weeks. I learned a long time ago not to get my hopes up about when we will get to go home. We will be home when Jacob is ready. We are on his body's time table. The bad thing about the Fontan is that the kids tend to have a lot of fluid build up around their lungs. They are put on a lot of diuretics post-op but sometimes they end up needing to be re-admitted for a chest tube. I don't want to rush home and find out he needs to go back. If we do get home and something like that happens then we can always go to Cincinnati for a chest tube but I would rather stay there longer and make sure he is good to go before we go home so we don't have to go back at all.

We wanted to do something fun for the boys before having to spend our summer at the hospital so we recently took a trip up to Cass Railroad. Jake loves trains and talks about them all day long.  I emailed Cass and told them about Jake and wanted to see if he would be able to see the inside of the engine and see the train up close. The superintendent scott wrote me back and gave Jake a trip to remember. He not only gave him a full tour of the train shed but he gave us 5 tickets on the 2 hour train ride and let us stay in one of their company houses the night before. It was such an amazing mini-vacation. The boys had a blast and it was just what we needed. If you read this Scott, thank you so much. I can't tell you how much this meant to Jake and our family.

Jacob with Scott, the man who made this trip possible.

 

So there it is, the countdown has begun.  I will post frequent updates on here while at the hospital. Until then, we will be taking in every precious moment and getting lots of snuggles with our little guy. Please continue to keep Jacob and our family in your prayers as we embark on this next stage in our journey.

Three years ago...

The first time I saw my beautiful warrior... 3 years ago.

Three years ago I was sitting in a hotel room scared beyond belief, unsure if my child would survive. Three years ago I knew my life would never be the same but I had no idea I would find my purpose in life. Three years ago today I was preparing for the birth of my heart baby. I thought I knew what to expect but I really had no clue. I was supposed to be anticipating one of the greatest joys of my life. Instead I was scared to death of the possibility that I may have to say good-bye to him forever.

This journey has been a roller coaster full of emotions. We hit so many different odds and had many curve balls thrown at us throughout the past three years. It has also been the greatest three years of my life. Jacob is such a blessing to our entire family. He has taught us all what life is truly about. We have met so many amazing families throughout our journey who we never would have met had it not been for Jacob. We have been able to help others through his story and find strength in theirs. What I had thought was my biggest fear has turned into our biggest blessing. Jacob and Aidan are my greatest gifts and I am so grateful I was chosen to be their mother.

Tomorrow my beautiful little heart warrior will turn three years old. A day I wasn't sure I would ever see. A day I celebrate with all of my heart and soul. I am blessed beyond words. 

Jacob,
You continue to amaze me by your strength, inspire me to be a better person and teach me that together we can overcome any obstacle that we face. Happy birthday beautiful boy. I love you more than life itself and I am so very proud of you!
 

Spreading Awareness!

It is that time of year again. The time of year when heart moms from all over the world blow up facebook and the web with posts about Congenital Heart Defects. It is Heart Month and this week (February 7 thru 14) is Congenital Heart Defect Awareness week.

I plan on doing a longer post with details about our recent trip to Cincinnati but today I wanted to focus on CHD Awareness. To hold you over until then I will let you know that Jacob's tests went great. His airway looks good, he won't need another scope until after his Fontan (Last planned open heart surgery) this summer. His hearing test went great and showed his hearing is 100% NORMAL!

JUMP ROPE for HEART

This year Aidan is participating in Jump Rope for Heart. It is a fundraiser his school is participating in that raises money for the American Heart Association. Aidan was so excited when he found out about the event, he told his class all about his brother's special heart. He told me he wanted to collect money to help babies like Jacob! I am so proud of Aidan, he is such a good big brother. Jacob gets a lot of attention because of his heart. I try to involve Aidan as much as I can but there is not much he can do. This fundraiser is so great for Aidan because it is a way that he can help a great cause that is so close to our hearts. He gets to make a difference and I want to make this a huge success for him.

I knew immediately that I wanted to help out at the school so I talked to his coach and asked if they needed any information about congenital heart defects. They didn't have any so I offered to create a video about CHDs for them to show the kids. Timing was perfect because I was trying to think of something I could do for CHD awareness week.

I created two versions of the video, one for Jump Rope for Heart and one to share online for CHD Awareness Week. I am really happy with how the video turned out. Every child in the video was born with a congenital heart defect, many were born with the same defect as Jacob. Each of these kids holds a special place in my heart and I am so grateful to their parents for allowing me to use their photos in the video. I am posting the videos below, please feel free to share them and help us spread awareness!

I am also going to go to the school on the day of the jump and help out at the event. I will take lots of pictures and post them on here after the jump. It will be at the end of this month. I plan on making Aidan a shirt with this design:

 

Looking for a way to contribute to CHD Awareness Week? Please help support Aidan by sponsoring him in his mission to help fight heart disease. Make a tax deductible donation in honor of Jacob by clicking on the link below. Online donations can be made until February 21st. Thank you!

CLICK HERE TO DONATE!

CHD AWARENESS

 CHD AWARENESS- JUMP ROPE FOR HEART VERSION




Happy Heart Week! Please feel free to share this video and continue to spread awareness for all of the children fighting this battle and for those who were taken from this earth way too soon!