Fontan Pre-op Day



Today was Teagan's long pre-op day for her big 'Fontan' surgery tomorrow. It's been quite awhile since I've updated the blog, but it's the easiest way to document and update everyone on what's happening when we are at the hospital, so here we are again. I haven't even visited the site in almost two years because when Teag is doing good and things are 'normal', it's nice to take a break from the 'heart world' and focus on normal kid/family stuff.  This blog has been a source of connection for me in the early days of Teag's diagnosis- a way for me to find other families who also had a child with a congenital heart defect when we felt so alone, and for that I'm so grateful!

The surgery that Teag is having tomorrow is called the 'Fontan' and is the 3rd part of the 3 stage repair that was started at birth when she was diagnosed with Hypo-Plastic Left Heart Syndrome. (HLHS) http://www.cincinnatichildrens.org/health/h/hlhs/

 

When Teagan was born, we discovered that her left ventricle (the main pumping chamber that sends oxygenated blood to your body) had not formed correctly. She quickly went in to heart failure as no blood was able to exit her aorta to support her body. In a normal heart, your right ventricle receives 'blue blood' from your body and pumps it to your lunges for oxygen. Then oxygenated blood enters your heart to your left ventricle and is pumped out to your body via your aorta. Her first surgery called the 'Norwood' was performed at 6 days old. Her aorta and pulmonary artery were fused to both lead to her right ventricle (her only functioning chamber) and a shunt was put in to allow blood flow back out to her body. All of the 'blue' and oxygenated blood was mixing in her heart so her oxygen levels were extremely low at this point, but it kept her alive until she grew bigger. She was so weak during this time that she was unable to eat so we fed her via an ng tube through her nose to her tummy controlled by a feeding pump every 2 hours. She was on 9 meds rotating every 2 hours and required constant care.

 

At around 6 months she was big enough and ready for her 2nd surgery called the 'Glenn'. This surgery connected her 'SVC' (the main artery that sends all of your upper body blood flow to your right ventricle to be pumped to your lunges for oxygen) to her pulmonary bypassing her heart. Now all of her upper body 'blue' blood is going directly to her lungs, picking up oxygen and entering her heart fully oxygenated. This really helped raise Teag's oxygen levels (from the low 70s to the mid 80s), gave her more energy and allowed her to begin to eat a bit by mouth slowly weaning off of the feeding tube. She learned to crawl, walk and eventually eat on her own and has since thrived doing many 'normal' kid things which we're so thankful for! As she has gotten bigger however, physical activity has gotten really hard with oxygen levels 20% lower than most kids her age. She turns blue, gets winded and has chest pains any time she does any physical activity for more than a few minutes. Her fingers and toes are blue/purple all the time and show signs of clubbing. These are all signs that she is ready for the third and final stage of her repair the 'Fontan'. Most kids need this surgery at age 2-4 and she's managed to make it to 7 1/2, giving her time to get bigger and stronger. She has had 6 heart catheterization surgeries over the years to check pressures and balloon/dilate certain areas to keep her one ventricle working its best. In April of this year, she had her most recent heart cath and it was decided that she's ready for this final stage of the repair and we began planning for this summer surgery.

 

Here's a bit more about tomorrow's surgery the Fontan:

 



The final stage of surgery will be performed as the child starts to show that they need more blood flow to the lungs. They may become more breathless on exercise or their growth may slow down. For some children this will occur before they start school. Others may be able to wait a little longer.

The Fontan procedure aims to separate the blue (deoxygenated) blood supply and the red (oxygenated) blood supply. Although this does not make the heart function normal, it does allow the children to grow and enjoy more physical activity.

This operation is done by attaching a tube of a special plastic (a conduit of Gore-Tex) from the lower body vein (inferior vena cava) to the base of the lung artery (pulmonary artery) diverting blue (deoxygenated) blood away from the heart straight to the lungs.
A hole (fenestration) may be created between the tube and the right collecting chamber (right atrium).  There can be a rise in pressure in the lung arteries after surgery and the hole acts as a pressure valve.
Children may be in hospital for some weeks after the third operation as it is important to give them time to adjust to their new circulation.

Tomorrow's surgery will greatly increase Teagan's oxygen saturations and she should be close to or above 90% O2 for the first time in her life! Her nails, lips and cheeks will finally be pink! It is however a major shift for her body, rerouting all of her lower body blood flow directly to her pulmonary artery to her lungs for oxygen and all the blood going in to her heart will now be fully oxygenated! Her one single pumping chamber will now only have one job- pumping fully oxygenated blood to her sweet little body. She will have a fenestration- a small hole in her shunt that will act as a pop off valve in case pressure builds up in her system so we don't anticipate 100% sats, but they should be in the 90s. The main complication (other than normal heart surgery issues) is the drainage she will deal with from her 3 chest tubes placed during surgery. We expect these to drain for 1-3 weeks until her system adapts to her new circulation. This will be the longest part of her recovery and we just don't know how long this will take. The Dr.'s have told us to expect a 4-6 week stay in the hospital but we're praying for much much less!

Today we had a full day of appointments at the hospital to get little miss ready for her big day tomorrow. She is always so brave and had the best attitude about everything! Here she is relaxing during her EKG and blood pressure checks, and a few other pictures from our day:

 
 
 
 
 
 
 
 
 
 
 
 
 

The last part of our day is always a visit to the lab for blood draws. We had prepared her and told her just one quick draw and we'd be out of there and she was so brave! Then after we finished the first draw, the nurse looked at us and said we had to do two! I felt so bad for Teag, she was heartbroken about doing the whole thing over again but she handled it like a champ. Here she is with both arms taped up and still smiling.



 

During some of our appointments, Nancy from child life joined us to talk with Teag about how she was feeling, brought her a few toys and medical supplies to play with and gave us a tour of the hospital to show Teag what her room would look like and ask questions. This was really helpful because Teagan has been really nervous not knowing what to expect... At the end of the day, Teag hugged Nancy and was excited to see her tomorrow and during our hospital stay to play and help her through the painful parts of her recovery.

 

We left the hospital around 6 after a quick visit with one of our heart buddies in town from Alaska for a quick heart cath procedure. It's so nice when Teag can visit with her friends who are going through the same thing as her. They are great friends and share a bond that's pretty unique!

 

Teag requested Italian food for dinner tonight and we stuffed ourselves before heading to the hotel to finish prepping for surgery. We had to wash her with special Hibiclens soap for a long time in the shower and then put antibacterial ointment in her nostrils to prevent her from inhaling MRSA and other bacteria tomorrow. Then she enjoyed playing 'surgery' with the medical supplies and bear that she was given today and we watched her re enact placing an iv, doing blood draws and putting bear to sleep with the mask. We talked about what it will be like when she wakes up from surgery tomorrow, about her tubes/lines/oxygen and a bit about the pain. She told us some things she nervous about and we talked through how we'll handle all of that too. We snuggled her, said prayers and watched her drift off to sleep. We check in at the hospital at 7am tomorrow and she is scheduled for the OR at 8:30am. Surgery is supposed to take 4-5 hours and then another 2 before she is settled in to the CICU and we can see her. Please pray for Teag and also the Dr.'s, nurses and staff caring for our sweet girl tomorrow. We love her so, so much and can't wait to see her pink little face tomorrow afternoon when she's all done! I'll be updating throughout the day with the latest. 



Bode's appointment

 Last Monday after our fun Easter at home, we packed up the car and headed over the pass to Seattle for Bode's CT scan and appt. with the surgeon. We made it in time to grab a quick bite in the hospital cafeteria and meet up with Nana and Auntie Deni who played with Gav and Teag so we could focus on Bode.

 

We headed down to radiology and Bode had fun playing the 'Endless Alphabet' app on my phone (its pretty awesome) while we waited. Radiology told us that the scan would be quick, but that most kids Bode's age are sedated for it. I wanted no part of that and asked ahead of time if we could just go for it and see. Thankfully, the tech was super nice and amazing and Bode didn't seem too scared as we walked back there. Trent and I got to be right there with him in the room and I laid Bode in a little cradle lined with warm sheets on the CT scan bed. The tech gently wrapped him up and secured his arms while pointing out all the cool stickers on the dome of the machine. Bode was nervous when the machine turned on and kept saying,"Um, guys?" But he didn't move or cry! The scan took 20 seconds or so and then we were done! I was so proud of him for being brave and so happy to have him skip the sedation!

 

We headed back upstairs to Cranio/facial for our follow-up with the surgeon and ended up waiting for over an hour in our little room. Here is what the guys did to pass the time- you can tell Bode is thrilled to have another pic taken, ha.

 

 When the surgeon came to talked to us, he showed us a few images from Bode's scan. In the one below, you can see the indentation where the cyst has affected his skull. (the small dent above the ear area) Dr. G didn't seem surprised since Bode has had the cyst for most of his life, but he does want to remove it so that the cyst doesn't continue to grow inward and cause more damage to the skull. He explained that the procedure is 'simple' in the cranio/facial world and shouldn't take more than an hour.



Surgery is scheduled for this coming Friday, and Bode is 1st case right now. We're headed back to Seattle on Thursday with Nana along for the ride to help with Gav and Teag. If all goes well, Bode should have a few removable stitches and his skull indentation should repair itself over the next few years. The cyst will be sent to pathology but Dr. G is confident that it is nothing to worry about long term. Bode shouldn't need further treatment once surgery is done! The thought of our sweet little man in surgery is killing me, but I'm trying to stay positive! We're praying for smooth sailing and a quick recovery.

 

These guys got spoiled by Nana and Auntie while we were at the hospital with Bode. They went for ice cream and picked out a book and a Lego project at the toy store!

After we left the hospital, we went out to an early dinner at the Ram and headed back to our hotel. We are trying to avoid germs as much as possible, so we rented a redbox and planned a movie night! We checked in around 6 and the kids jumped on the beds and danced to kidz bop for at least an hour before winding down. We had a great time just playing with them and being goofy!

Our hotel window looked out at the space needle (in the distance) and lots of city traffic. The kids took a break from jumping long enough to enjoy the sunset and watch the action. I love these guys so much!

This week we're catching up on some homeschool lessons, playing outside and trying to stay healthy before leaving on Thursday.

With so much going on in our lives and so much that I've missed writing about this last year, I decided it was finally time to update the blog! I figured I'd just start with today and add in some recent birthday posts and other things soon!

 

Celebrating Easter today at home with our little family was so great! Spring is finally officially here and the weather was beautiful! We are supposed to be quarantining ourselves for some upcoming surgeries the kids are having (more on this later) so church was out, and we enjoyed a quiet morning reading the story of the resurrection with the kids, eating candy with breakfast and riding bikes in the garage. 



 I wanted to make a big lunch to celebrate the day and Gav helped me in the kitchen chopping the potatoes and setting the table. He turned 7 in the fall and is quickly becoming quite the little man. He loves to cook and is always up for a project! We are still homeschooling and using a co-op 2 days a week. He's almost finished with 1st grade and doing great! His favorite things are math, science and history- but he's also quite the artist and is always making a craft in our schoolroom. We pulled lunch together around 1 and had Auntie Loren over to join us for our feast!

After lunch, we headed outside for an egg hunt and to enjoy the warm sunny weather! The kids love looking for eggs, and eating the candy even more. Teagan had candy or gum in her mouth for most of the afternoon and was thrilled. She turned 5 last week (will post on her bday soon!) and is a little ray of sunshine! She is really into 'tricking' us lately with a funny joke or telling us something that's not true and then quickly saying,"I'm just kiddin!" Today she had a funny smile on her face and handed a tootsie roll to Trent urging him to open it and eat it. He took it and put it in his pocket, but she insisted that he eat it right then. He obliged and when he unwrapped it, it wasn't a tootsie roll inside but a piece of her chewed gum. When she saw that he opened it, she laughed so hard and ran away, ha! Trent likes to tease the kids with gum wrappers, or other empty candy wrappers that he's refolded to make it look like its a new piece. She's quickly catching on to the trickery...

Bode loved the egg hunt too! He would open each egg he found and get distracted wanting to eat the candy right away while the other kids ran all over. He had a birthday last week also and is now a big boy 3 year old! He always has some funny question or face he's making- and we're always laughing at what comes out of his mouth. Today he fell in the dirt while running and he jumped right up and said,"that was wicked!" (I have no idea where he heard this?) He is our little firecracker and always wants to play 'fight' or 'tackle you'.

Checking out their stash...

 After our egg hunt, we went inside to color eggs and make cookies while Trent worked on a project. When we were done with that, we took a long family jog to try and run off some of our lunch! There's an area near our house where a new road is being built, so there is lots of room to explore and no cars yet! The kids were loving the shadows cast on the road below and were instructing Trent on what position to do. The top right pic below is a move they called 'The Goat'?! We had Teagan and Bode in the jogger, but when they saw the dirt field they wanted out. We ended up being gone for a few hours and found a fun trail along a nearby creek at the park. We threw rocks, got dirty and just spent time as a family. I love days like today! I try to soak in every second of the times like these we have and don't ever take them for granted. We have some big decisions to make in the next few months regarding Teag's plan for the future, and today was one of those days where I could just enjoy being together and not thinking about all that stuff. For that I am so grateful!

Tomorrow we head over the pass to Seattle for Bode's appointment at Seattle Childrens. He is having a CT scan on his head/left temple and also an appointment with the cranio/facial surgeon. Bode has had a small mass on his left temple since just after he was born. We had it checked out back then and were told it was nothing to worry about, but now that its still there the Dr. was more concerned. We saw the Cranio/Facial surgeon a few months ago and he thinks it is a dermoid cyst that will need surgery, but he wants a CT scan done first to make sure. So tomorrow is the scan and then we will get the results/make a plan. Bode is already scheduled for surgery on April the 12th, and the scan should show the surgeon if its only superficial or if the cyst goes deeper into his head. I am trying to keep my anxiety at bay and praying that its superficial and easily removed!

Teagan was supposed to go in for a heart catheterization surgery on Tues. (in two days), but that was postponed last week after a trip to the ER for a double ear infection and low SATS. After a few hours of O2 and some strong antibiotics, she's doing great again. But her cath has been rescheduled for May 1st and the surgeon wantes her illness free for 3-4 weeks before hand. The cath will let us know if she is a Fontan candidate or if we need to look at other alternatives... (the dreaded 'T' word) Last year in April she had a heart catheterization that showed high pressures in her right ventricle (17 I think) and also her tricuspid valve was moderately regurgitating. Those two combined do not make for a successful fontan candidate, so we've spent this last year adding and increasing meds to try and make an impact on the pressures/regurge. I pray nightly that all the medicines we give her each day are making a difference, but also know that God's perfect plan is being played out in her life each and every day! She is doing pretty well overall and her O2 levels are usually in the low 80s when resting. She asks to be carried after walking for a bit or going up stairs, her lips are frequently blue and she is easily winded- but it doesn't stop her!

I will update again with the results of Bode's scans and also the kids birthday posts soon!