Colin's Fontan Surgerical Consult - Looking at Summer 2013

Yesterday we had Colin's pre-surgical consult with his surgeon at Texas Children's Hospital. A little back story to update all: In April we were told that Colin's next (and hopefully last) surgery (Fontan) was going to be this summer. After that appointment Colin had a heart cath in May and an echo in June to fully see what was really going on inside his little body.

Yesterday's appointment we were told that most likely Summer 2013 is our target date. We are pushing it back since Colin's heart looks so well and his function is good. Colin is still on the smaller side, so they want him to gain more pounds so they can use a bigger conduit (synthetic tube graft used to direct blood flow) in his surgery. They could use a smaller conduit now, but it does not grow with your body, so it is better for adulthood to have the bigger one.

But if his heart function starts to decrease, oxygen levels go down, or his leaky value starts to leak more, they will do the surgery sooner and have the smaller conduit. So we will continue our 6 month check-ups until they think he is either big enough or until he absolutely is in need of it, whichever comes first.  Yesterday we were told that Matt & I could pretty much plan it, so if all stays the same and he is doing so well, we think maybe late July 2013 when it gets too hot to do anything anyways.

Here are some of our questions we got answered, some might only make sense to the other heart parents following our blog, but I will try to speak to each point for all to understand:

-       Depending on how well Colin is doing, he might not need another cath before next summer. They feel as long as it's within a year of the surgery (or close to) they won't have to go in again. If they need more pictures, they can do an echo and/or MRI

-       Our hospital does the External Conduit Fontan (there is another version of the surgery.. Lateral Tunnel Fontan) which means the 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.

-       80% of their fontan's do not receive fenestration.. it is only done on a case by case basis. If Colin stays low risk he will not use fenestration – A fenestration is a hole that is created between the tube and the right collecting chamber (Right Atrium). As with the Internal Fontan there can be a rise in pressure in the lung arteries after surgery and the hole acts as a pressure valve.

-      Colin has a mild leaky valve. They worked on it during his first surgery. It has not changed since then, and they have been happy with that. As of right now, they are on the fence about trying to repair it or not. It is a mild leak, so they are going to do an echo in the operating room to make the call about it then. There is of course more risk with working on his valve, as you could make it worse. Also if they work on his valve, they will have to STOP his heart to work on it. And the risk of stopping the heart is that you can get the rhythm out of sync and might require a pacemaker to help with that.

-      We discussed possible complications: First is getting back in through his original incision and scar tissue, there is a chance of puncturing a hole in the heart, fluid can build up around the lungs (which will make your hospital stay longer).  And of course with all surgeries, chance of seizures and stroke.

-       This type of fontan was developed in the early 90's.. so most people are only about 18-20 years old. There is still so much they don't know about the future of having only half a functioning heart. What effects it has on the rest of the body. The liver, intestines, and kidneys see higher pressures due to this reroute of blood flow, so there is a chance later down the road to develop problems with other organs. Some have gone into heart failure and have had a heart transplant and some were not candidates and have passed away.

-      Normal hospital stay is about one and half to two weeks, depending on the fluid level.

-      He will have drainage tubes around his lungs and heart. To help with getting the fluid off, they are really going to restrict the fluids he takes in, which I heard is really hard when your child is begging for water and you have to tell him no, or only just a little sip.

-      They try hard to get the breathing tube out before leaving the operating room.

-      We will expect to spend about 2-3 days in the ICU, then move down to the regular floor. He will be able to move around and go to the play room with the tubes still in.

-      If Colin has developed fatty fluid around the lungs and is hard to get rid of, we will be sent home on a low fat strict diet for 3-6 months.

Okay so my thoughts and feelings:

-       Glad he is in great shape to wait and that they are so happy with his function and how well he has progressed. I am so grateful that he is a candidate for this surgery.

-       Once again weight gain is going to be a major stress in our house; he needs to gain about 3 more pounds by summer, which is not an easy task. He likes food, he eats.. but it is a lot of begging and pleading and sneaking calories in where I can. He has always been small and doesn't ever gain in huge amounts, but we will keep pushing food and more food, and more food.

-       The leaky valve scares me the most. I hate thinking they might have to work on it. I am distraught over the idea of them having to STOP my sweet little boy heart in that case. As a mother, I cannot even express the sadness I feel knowing the complications to that while looking at my blue eyed, white hair little man.

-       We are still going to live as life with the blessing we know Colin is right now doing well. We are going to soak up all we can out of life and each other, because one day Colin might not be as strong, he might develop other health issues, he might fly through these surgeries but at age 30, his heart might get too tired of only functioning on half a heart, and it might just give out. We will always live with the fear of the unknown.

-      I have so much HOPE for my son in the future. He is strong. He is a fighter. He is a superhero! He has done amazingly well thus far and has set the bar high to keep it up and I fully believe in him he can.

So it was a good appointment (expect they were running 3 hours behind) with lots of positive talk about how well Colin is doing, it also brings it back down to reality that he does have such a major heart defect. We have been so removed from the world of surgeries (his last one was 3 years ago) that is surreal to have to think about it again. But we now have about a year to live life to the fullest, to enjoy more trips to the beach & river, more play dates, more laughs and giggles, more bedtime stories, more snow cones & ice-cream, more road trips, more family movie nights, more of each other, more of everything really. However long my son is on this earth, whether it be 15 year or 80 years, time is not to be wasted. As one of my favorite quotes " Do not squander time; for that's the stuff life is made of". 

The photo collage that we gave Colin's surgeon.. he loved the peeing picture! We tried to capture the true love of life our son has and how active he is.