Fontan Talk….

So.  The echo looked good from what you can see on an echo…ventricular function looked good, his ‘sutureless repair’ (for his pulmonary vein stenosis) was wide open with no obvious narrowing, and his Glenn was working well.  Things that we don’t know right now are the severity of his collateral growth and what his pressures are.  Pressures is the dreaded P word for us heart families.  Everything is always dependant on pressures.  Pressures too high…pressures too low.  All of our children’s surgeries require certain parts of their heart to have pressures within a range.  Lung pressures, heart pressures, blood pressures…..and its the one thing that we don’t know unless a cardiac cath is done, which you don’t want to do unless you have to. 

So in discussing “The Fontan” it is always told me that all of this is dependant on what his pressures are, when they do a cath, 1-2 days before surgery.  It’s really honestly a cruel and unusual situation to be in because we can never count on anything.  Most heart Mom’s that I talk with have a lot of anxiety, nervousness, depression, and much of the time post traumatic stress.  But how can you blame us?  The doctors tell you that in order for your son/daughter  TO LIVE he/she needs _____ surgery, BUT we don’t know if we will be able to perform it when the time comes because the pressures may be too high.  Seriously, think about that.  That is what we live with day in and day out.  That is what we think about when we tuck our children into bed and kiss their head.  Not that I hope this next surgery is sucessful….OH NO, that would be too easy. 

We are thinking, I hope they are able to attempt surgery.   

It’s awful to live this way and I don’t think anyone will ever fully get what its like until you are in those shoes. 

Even though Logan’s cardiologist knows me very well (ie how much time our appointments take because of my laundry list of questions) I don’t think even he was prepared for how many questions I had regarding the fontan. 

The biggest questions that I have surrounding the fontan are…

1. Do we really have to do it?  (and I don’t say this cavileir, I have done my homework )

2. Timing

I watched this video about the life of an adult fontan patient and it scared the %$#* out of me…..

Warning to all heart families this is not easy information to hear, but knowledge is power and education is everything when it comes to our kids.  I want to know everything, and I do NOT like surprises.

The Adult Fontan Patient from ACHA on Vimeo.

This video is long but the points that I took away from this video are what follows…

1. The Fontan is no joke.  The idea that our children are going to have 1,2,3 surgeries and then be fine is just not true. 

2. They are finding that adults with Fontans are wearing out quicker than they anticipated.  Some in their 20’s, some in their 30’s, and if you are very very very lucky your 40’s. 

3. As of RIGHT NOW, statistically the odds of our adult fontan children receiving a heart transplant once their fontan starts to fail is (and I quote from this video) Slim To None.

A friend of mine and fellow heterotaxy Mom asks a question in this video to which the response was…”enjoy the good years.”

I am having a very hard time accepting this. 

 My Son is going to have more than 20-30 “good years”,  I am going to question everything, and I am going to fight so damn hard to try to give him more than 20-30 “good years.”

I have done a lot of reading, research, question asking  and I will let you know what I find out but I am really starting to wonder about the fontan.  The biggest reason being is that typically the reason for Fontan failure is not the heart.  It is the (here we go again) the pressure it puts on your liver and other organs.  There are so many complications of the fontan it is just scary.  AND of course, heterotaxy kids are more prone to all of them.  My biggest fear is doing anything to Logan that will damage his other organs.  One damaged organ is enough for my son…thank you. 

There are so many breakthroughs coming for our kids.  Stem cell hearts, total artificial hearts, ventricular pump devices and I want to make sure that Logan will be in a place to utilize them when they are here.  This is not science fiction.  Currently there are 13 adults in the world right now walking around with total artificial hearts.  There are ventricular pump devices being used right now.  They are growing organs with stem cells.  These things will one day be here  but if Logan has ruined lungs, a ruined liver, or other major organ problems he won’t be eligible for them. 

It’s a lot to think about, a lot to take in, and a lot of worry.  His surgeon thinks its important to do the Fontan between 18-20 months old.  There is a controversy over the timing of the Fontan.  Too early, too late, when is the best time.  And we are talking about a surgery that 1 in 10 die from, so there is a lot to think about….just another day in the life of a heart Mom. 

Logan decided that he was going to eat a cookie while waiting for his cardiologist to come in the room.  Amazing! Another cookie!  This is fantastic…any food he eats is going to get him bigger and stronger for our next surgery. 

http://vimeo.com/moogaloop.swf?clip_id=32562009&server=vimeo.com&show_title=0&show_byline=0&show_portrait=0&color=00adef&fullscreen=1&autoplay=0&loop=0

Logan deciding to kick the TV instead of watch it during his echo.

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At home is trying so hard to figure out his baby walker.  On the lowest setting his feet barely touch the floor (he is only 29.5 inches long) but he manages to sort of push the walker in a circle with one foot.  Once he gets used to this though I think he will really enjoy being able to move around.  I mean he is 1.5 years old.  How frustrating it must be to not be able to get around yet.  I can’t wait to see him scooting around in this walker confidently! 

I hope everyone has a pleasant holiday.  Happy Thanksgiving.  Maybe Logan will decide to try some turkey this year???

7 thoughts on “Fontan Talk….”

  1. Heavy post today! I know how you feel and spend a lot of time researching, questioning, thinking, wondering, and crying about the Fontan. We, like most single ventricle families have been told that the Fontan is it. Sadly, I don’t think it will end at the Fontan, but who knows. They have changed how they do the Fontan and I don’t know if the video takes that into account (of course I haven’t watched it yet), but I know it is different than the earlier Fontan. I also don’t think having Glenn physiology is a great alternative either for a long time. So much to think about. Hopefully you can hold off for a while befoe contemplating the Fontan.

  2. Hi Kristen-

    My name is Jesse and my son is also a single ventricle (most closely diagnosed as double inlet left ventricle). He is almost 5 and we are still waiting on the Fontan. Our cardiologist is a big believer in only doing the Fontan when indications point to needing the surgery. Luke’s sats are relatively high (85%) and his heart function is excellent. So we wait. It is a very interesting place to be for sure and I really resonated with your post. Our card. feels like once you do the Fontan, the clock to liver failure starts. Horrible. But, I too, have hope in where medicine is going and I pray that better options are just around the corner! Have a happy thanksgiving … Logan is adorable!!! You can follow our road to the Fontan on our blog if you’d like: http://smithfamilyjourney.blogspot.com

    Jesse

  3. Hi… I have followedyour blogs for a while now and I’m just wondering how much he weighs now. I know going into a surgery they want them “as big as possible”. I saw you sad he is 29.5 in long so I was wondering what his weigt was… have a wonderful holiday.

    1. SO FUNNY! you just asked that because I was just thinking that I forgot to say how much he weighs now. He is 18.7 pounds. Really tiny. Way under the 1%. What I was told was it wasn’t really his size that matter

      1. Yes… Those are I have yet to hear for my daughter. She is 16 months weighting only 14.7 pounds and 27 1/2 inches tall… I like to ask and compair with her size…. also I tried to find his page or yours on facebook and coulnt find it. What was the link again?

  4. “enjoy the good years,” really got to me. I am mother of 22 year old single ventricle with failing fontan. This is tougher than I ever dreamed (nightmared) over the years wondering what it would be like when the other shoe dropped. It did drop and suddenly, without warning, and with no real idea of what happened to cause the change.
    We did enjoy the good years, but it always seemed that they would be able to detect changes that would lead to decisions about maybe a fontan revision or maybe some meds to help with the first signs of problems. No, it just became time to use oxygen, and eventually a cath that showed “distrubingly high pressures,” and connections that had sprung leaks all over. We were to be thankful for the leaks as they are helping now.
    Yes, we are being directed to the transplant team, and while that might be full of hope, it is not at all the clear route for him – he may not be eligible.
    So for now, we are still trying to enjoy the good years.

  5. Hi there, I just found your blog, and I know these posts are super old, but I am hoping you still are around…:-) We just adopted a little girl with single ventricle, single atrium, heterotaxy syndrome, pulmonary stenosis, and asplenia. She just turned two, and had the Glenn before she was adopted. Anyways, if you are around and are willing, I would love to ask you some questions…

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