Wednesday, July 15, 2015

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)
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. 


Tina Hayden said...

Love and hugs to the entire Maxwell clan. <3 You all are in our thoughts and prayers! Thank you for the updates, and sharing this journey. Go Teag!

xo Miss Tina, Miss Mari, Miss Meg and Kai

Katie Allred said...

Of course I've already checked this a few times today...praying for you all! I so wish I was there with you in that waiting room but really not quite sure how much support I'd be! This brings back so many memories, PTSD!!! Lol! Love you all and will be praying steady throughout the day and the coming days. Teagan is so amazing and is going to do great. Keep your HOPE in HIM! Love you and hugs Sus, Trent, Gavin, Bode and of course, Teagie!

Janie and Gene said...

Trent, Susie...we love you, the boys and Teagan so much. Praying constantly through this day and all throughout this portion of your journey. Praying for the Dr.s during surgery and the entire staff during recovery. Praying for wonderful success and for God's comfort to your nerves this week. Hoping you get sleep. Will avidly watch your blog. Luvs, Janie and Gene

Cayle Maxwell said...

Hmmm...My first comment seems to have been lost to the Internet. Just want to tell you guys we love you and we've been sending prayers your way. We'll be over Friday night to help out with anything we can.