Countdown to Surgery- The Fontan

I am a disaster.   We are leaving for Boston this weekend.  I would give anything in the world not to go back.  This life is so very unfair.  I can’t believe he has to go thru this again.  We have decided to rent an apartment in case we are there for a long period of time (if not…we’ll we will break the lease) but we have decided to take our other Son and our nanny because we think its important to keep the family together. We are leaving this Sun for Boston stopping along the way and finishing the journey on Monday.  The cardiac cath will be done late in the week and surgery is scheduled the following Wednesday. 

We had our last cardio appointment today with Dr. Patel.  A very good friend of mine who also has a child that has the odds stacked against him as well, gave us their good luck onesie!  J wore this onesie thru 3 surgeries and he is doing great.  I was honored to have his shirt passed down to us. 

He did a very thorough echo and everything looked as good as it can be. His Glenn and SVC look wide open with good flow.  All this pulmonary veins appear to have good flow.  His AV valve although slightly stenotic is working well with very minimal leakage.  His pulmonary artery in color appeared to have even grown a bit which is very reassuring.  The pulmonary artery is very important because the Fontan surgery is going to connect the IVC (Inferior Vena Cava) to the lower half of the body making pumping oxygen to the lower half much easier for Logan.  The Glenn connected the SVC (superior vena cava) to the PAs and once we have the IVC connected to he will have a Total Cavopulmonary Connection or a Fontan Circulation.

This is a very basic drawing of a Fontan that is done on a child with Hypoplastic Left Heart Syndrome.  If you take away the right 2 chambers from this drawing you would see what Logan has.  The Fontan is the “Tube Outside the heart” that connects the IVC. 

We had a much smoother appointment this week.  For one thing, Logan is very into counting right now albeit every number is ‘six’.  When he learned that EKG leads could be counted he decided they weren’t half bad…  “six, Six, Siiiix,  Siiiiiiix!” 

He weighed in at 9.7 K or 21 lbs and 33 inches in length.  Right now he is wearing 12-18 month tops and 6-12 months bottoms, well, and then 3-6 months socks and shoes.  Just to give you an idea about his size.

He is saying very random words right now….he says, ‘six’, ‘eat’, ‘hot’, ‘nuts’, and ‘hi.

He is really starting to enjoy Ethan.  They play together all the time and love taking baths together.

You can see clearly the oxygen difference between the two when they are together in the tube.


Logie is loves chasing balls.  He throws them, kicks them and then chases them all over the house and yard.




I just want to thank everyone for their support thru all of this.  I literally couldn’t go thru this without it.  I know that this will not be the end for Logan.  He is going to need valve replacements, pacemakers, and eventually a heart transplant.  The transplant could be in 6 months, 6 years, or 16 years, we have no idea.  Living like this is an incredible blessing and curse at the same time. A curse for the obvious reasons but a blessing because it has given us so much clarity.  Clarity on what really matters.  I don’t care about fancy preschools, where he falls on arbitrary charts compared to his peers.  I don’t care about college and if Logan will get a high paying job.  No.  All I care is that both my boys are happy, healthy as they can be, and have a good quality of life. Nothing else matters. 

Life is about this moment right here.  As long as we have each other, we have everything.

While we are in the hospital I will update his Facebook group much more often than this blog.  If you would like to join please look up Heterotaxy Syndrome- Logan Spyker on FB.  I can quickly post updates there from my phone.


Big Week For Logan

So Logan had a huge developmental week! Just in one week, Logan started saying HI, clapping, and sitting up unsupported!  It is fantastic to see him developing and he is so ecstatic to be able to do these things.

He also tried a few new foods which is encouraging.  He typically only takes a few small bites but his willingness to at least try makes me very happy.

Trying some Ice cream…

Eating some french fries….

Logan continues to have his RSV shots monthly (fun times….nurses coming to the house with big needles!)

He had a cardiology appointment yesterday. 

I am having massive anxiety about his next steps.  I am terrified of the Fontan surgery.  This surgery is going to connect his IVC (major blood supply to the lower half of the body) directly to his pulmonary arteries.  This way he will have better oxygenation to his lower half of his body.  Currently his top half (waist up) is oxygenating at about 80%.  His lower half is oxygenating at about 72%.  The other benefit to having the lower half connected is that blood that flows thru the liver gets into circulation.  This is important because there are enzymes in the liver blood called “hepatic factor” that help keep collateral growth in check.  We know Logan has the ability to grow very extensive and invasive collaterals if his vasculature is stressed so it is important to get this “hepatic factor” into circulation sooner rather than later.

(getting ready for his EKG, weight check, Pulse ox, and blood pressure)

Really angry about the EKG leads…he is starting to hate them. Typically he hates having the stickers taken off but now he just hates the whole process.

Took a while to get them all on because he pulled them back off so quickly.  We went with the stick’em on quick, cover with blanket, and distract with IPhone method!

Logan’s echo went well.  His PVS has not returned! (yay!!!) his Glenn is working well, his heart is squeezing well, his valve has no regurgitation (thanks to Pedro Del Nido, it had been severely regurgitating), and his pulmonary arteries grew.  although his PAs grew they are still small for his age.  They are considered mildly hypoplastic.  Not great news for his Fontan.  Dr. Patel wants Logan to have a cardiac cath done in early spring to check pressures and collateral growth.  We will have that done in Boston and then based on that data we will determine whether he is going to have his Fontan then.  More than likely he will.  BIG SIGH….hard to even think about him having another OHS.  The only positive aspect is that he is healthier than ever before.  He got thru tough surgeries while in severe heart failure. He is so healthy right now, so that can only make surgery go smoother. 

I will leave you with some more cute Logie pics….

Another fantastic Silly hair pic!!!

Getting ready for a bath…

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.

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


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???

No Echo….

SO.  Burnt out, fried, and extremely nervous Mom got the appt. dates wrong.  UGH………. Logan’s app was on Tues., not on Thurs. so I had missed it.  Did the heart center call me? NO.  argggg…

Anyway, echo is rescheduled for Mon.  So I get to worry about this all weekend now, great!

I have good news to report though.  Logan ate half a cookie.  And it was one of those really high calorie cookies from Cheryl’s!!!! 

Here is him playing after eating his cookie…(notice all the crumbs in my bed) Oh AND also notice how good he is with his feet.  He has been on his back for so long he has really gotten use to doing things with his feet.  I always picture him being one of those adults that can chop vegetables with their feet! Haha!!

Untitled from kristen spyker on Vimeo.

Logan really wants to be part of everything right now, so even while baking with Ethan he sits up on the counter and watches.  I love that he wants to be with us. For so long he just wanted to be left alone in his swing with no one touching him. 

Just another reason the fontan will be so hard.  It will be so difficult to see him transition back to the scared, afraid, anxiety ridden baby again.

I will post after our echo on Mon.  If you aren’t already member of Logan’s FB update page you can look that up.  That usually gets much quicker and more frequent updates.  Just look up Heterotaxy Syndrom Logan Spyker. 

Also, we had professional pics taken of both the boys this morning and I am dying to see the results.  These pics are super important to me, we don’t have any of the two of them together.  Logan is a little scared of Ethan because Ethan is unpredictable in his movements (clumsy, takes his paci, makes loads noises, ect…) so I have had a heck of a time getting them to sit by each other for a picture.  We got Logan to sit in this really soft rocking lamb toy and he was SO cute.  Can’t wait to share them!  XXX

I have no idea what the title of this post should be!

I have been avoiding updating on here and I am not exactly even sure why.  Everything is so up and down and all over the place that it never seems like a good time to update.  I tell myself that I will update once a decision is made, or we have a better plan.  But maybe this is it.  Maybe things are always going to be up and down, maybe plans our going to always change.  That is a very hard concept for me to accept but I am working very hard at trying to be present vs.  constantly worrying about the future. 

Logan’s sats continually seem to be all over the place.  After his sats being being low 70’s and having the fontan discussion, we had a cardiology appointment last week and his sats were 87.  I talked with Dr. Del Nido on the phone (seriously this man is an angel) and he said that he does not want to do the fontan this fall unless his sats are really low or his valve is giving out.  He said recovery would be a very very long time (I imagine in my mind that this means months).  So this is terrific news.  I am waiting for Dr. Del Nido and Dr. Patel to officially have a conversation about this, but it is now looking like we can wait until the spring for the Fontan.  I am very excited about that news!!!

His last echo showed that his pulmonary artery is still very small.  This means that before he has a fontan they will need to do a cardiac cath to determine if his PA will even allow the extra blood flow that it would be getting after the connect the SVC directly to the PA.  So not being a canidate for a fontan is still a very real possibility for Logan.  I was looking up the statistics and many papers were reporting that only 25% of RAI (right atrial isomerism) babies are canidates for the fontan after the glenn surgery.  Not great statistics but Logan does have a few things in his favor right now.  Fairly good sats, no AV valve regurgitation, and no reoccurrence of PVS (pulmonary vein stenosis).  But his biggest obstacle is going to be his small PA.  I am trying very hard not to think about life if we can’t get the fontan…..I am imagining that this would mean transplant much sooner. 

Logan has been in a great mood for the past few weeks.  Even at his last cardiology exam everyone exclaimed that this was the happiest he has ever been! 

For the first time in his life he didn’t scream bloody murder when putting on and taking off the EKG leads….

He just played!!! He is obsessed with these little foam letters and numbers from Alex Jr.  They stick to the side of the bathtub when wet.  He loves them and even falls aspleep grasping them. 

He has been interested in things and has started letting other people hold him.  He still spends the majority of the day sitting in his swing but he wants to do more now.  Oh, and he officially has rolled onto his stomach all by himself.  Not a full roll yet but we are getting there.  He is now 14 months.  He weighs about 16 pounds and finally is in 6-9 month and some 6-12 month clothes fit him. 

I was shocked that he let me put him in this swing.  Typically that would be waaaay too much stimulation for him, but on this day, he loved it.  Because he can’t sit up on his own I had to stuff his blanket in there to prop him up, but he really really liked that baby swing that day!

He is still on his feeding strike.  Occasionally, we can get him to take 2-4 bites of chocolate pudding or something else irresistible but then he is done.  He wants nothing to do with food.  Nothing. Because of his very abnormal organ arrangement feeding tube placement was deemed too risky by his general surgeon.  So right now it just is what it is….

We did have a bit of a scare this past weekend.  Logan started vomiting on Fri.  and then for the first time I had ever seen he vomited up what appeared to me to be bile.  It was bright almost flouresent yellow.  I got very nervous and called his pediatrician.  Between calling the his ped, his ped card, and the on-call general surgeon they all agreed that it would be safer to have him checked out at the ER.  BIG UGH here becuase, although I have a tremendous appreciation for everything that has been medically done for Logan, the ER has never been a very good place for him to be.  The ER tends to be a place where none of the doctors know anything about him and they tend to love doing intervention after intervention in there. 

(Waiting in the X-ray room.  He always scrunches his legs like that when he is scared. )

(Labs that I was refusing…)

After the x-ray they determined that Logan didn’t have an intestinal blockage but they still wanted to do labs.  I didn’t understand why?  We were there to see if he had an intestinal blockage, once that was ruled out we should be able to go home.  I asked the attending why he would want to do labs now and he looked at me and said….’well, we always order labs.’

Ummm…I need a much more compelling argument than that.  Getting vials of blood from Logan is about as traumatic of an experience that you can have.  His veins are tiny and fragile, and it is very hard to get very much blood from him.  To fill up a bunch of those vials would take hours, probably 15+ pokes, sometimes ultrasound machines, and usually only the pediatric intensive care nurses or the ambulance transport team can even ever get a vein. 

Anyway, we ended up having to just refuse them.  They told me they wanted to check and see if Logan’s electrolytes were off.  REALLY??? He has been throwing up since Fri.!!! I can tell you his electrolytes ARE off.  Well, we want to know exactly how off (they said) my response was, his eyes and his Fontanelle aren’t sunken, and he just had HUGE crocodile tears in the X-ray room. 

My issue with labs beyond how traumatic and painful it is for Logan is that the doctors always find something ‘outside the normal range’ with his labs.  Then they get all worked up and start chasing those numbers.

But we succeeded in leaving the ER with the labs still sitting on the edge of the bed as we walked out…

(Haha Labs…you lose!)

Currently, Logan is still throwing up and not eating much.  Curiously, he has no other symptoms.  No one else in the family is sick, he has no fever, and beyond throwing up he seems to be in a great mood. 

To quote Logan’s cardiologist at Boston Children’s Hospital, “Logan ALWAYS has something up his sleeves for us….”

Next ped card appt. is next week. Most likely just a clinical exam.


“The Fontan Talk”

So I have not updated in a while.  I am not sure why exactly.  I do update on facebook, but writing these posts is emotionally exhausting sometimes, I think that is the real reason.  Logan has had some scary symptoms, some crazy oxygen readings, and is really throwing his doctors thru a loop.  I believe that last time I updated I had mentioned that Logan’s oxygen had been drifting downwards…Well it had been in the low 70’s for some time (about 4-6 weeks.)  Dr. Patel did an echo and determined that the reason for these lower sats are the fact that he is growing venous collaterals that are taking oxygenated blood away from the lungs.  Then we had a period where his sats jumped up to +85.  This was very bizarre right after his Glenn (4 months) he wasn’t even 85%.  And to be 8 months post Glenn with a tremendous amount of venous collaterals, for his sats to be going up??? Doesn’t make any sense to me.  Then last week Logan had a period of about 5 days straight where he was sweating profusely all day. 

Sweating profusely while sleeping + child with a CHD= Mom FLIPPING OUT!!!

I of course had all these theories running thru my head about these change in sats and the sweating.  I called Dr. Patel numerous times and even asked his heart surgeon if he knew what could be happening.  Everyone determined that Logan was okay, because although he was sweating, his respiratory rate was fine and he wasn’t retracting (breathing in so hard that your ribs show.) Then just like it started the sweating stopped. 

That sweating episode was about 2 weeks ago.  Since then he has been occasionally sweaty but nothing like those few days. 

Although, I love Logan’s doctors right now, Dr. Patel, Dr. Del Nido, and Dr. Lavin, I have to vent about something.  When I call his a doctor and tell them that my son has been sweating for days and I am concerned, WHY do I have to be asked if it is hot in the house???

Seriously??? Do you think I would urgently call doctors if my child was sweating do to a hot house? I can’t even tell you how many times I was asked that. Or, here is a good one….When he is sweating is he outside?

Hmmm…..yep! I took my son outside into 90 degree whether and it is making me nervous that he is sweating….Come On???

This past Thurs.  I we saw Dr. Patel again.  Just for a clinical exam.  And once again Logan had some very bizarre oxygen reads.  Now, after 3-4 weeks of being in the 80’s he is now back to reading 71.  Dr. Patel asked the nurse to bring the machine into the room because he wanted to see with his own eyes what was going on and to try on different places (hand and foot).  On his foot he was reading 71-72 and on his hand he was 85-87.  It is just strange.  Logan has NEVER had any oxygen readings above 75-80%.  And typically after the Glenn your sats slowly drift downwards as you grow. 

And then we had “The Talk.”

The Fontan talk…..

Dr. Patel said that we need to start getting ready for the fontan.  That Logan is going to need to have the fontan sometime before it starts to get chilly.  He doesn’t know if Logan would be able to make it thru a flu season.  If Logan gets a respiratory infection he would probably really really struggle.  Typically, children have fontans at 2-4 years old.  Logan will be about 15 months old which is very young and is going to make recovery much harder. 


I have been asking around in different CHD communities and I have yet to find anyone whose child had a fontan at such a young age.  Most children after the Glenn keep their oxygen level up for a longer period of time, they don’t typically have the abnormal collateral growth (I was told this is ‘a heterotaxy thing.’) There are many surgeons that require children to be 30 pounds before they will do the Glenn.  Logan is only 16 pounds. 


The Fontan completes his new circulation.  And this new circulation can be very hard for a little babies body to adjust to. 

I found this video about the fontan procedure.  Here they talk about it being used in HLHS (Hypoplastic Left Heart syndrome) but this is also the surgery used in Logan’s heart defect.

Just realized that I should post the video of his Glenn also.  That way you can really understand what they are trying to do.  The ultimate goal is to have the SVC (artery to head) and the IVC (artery to lower half of the body) by passing the heart and being directly connected to the pulmonary arteries.



While Logan has turned one years old it has left me with much time to think about the last year.  When we got the diagnosis, how devasted we were.  I think back to that period and I can’t believe how little I understood and how little I knew about what life was really going to be like.  And then I think back to our very ugly NICU experience which really opened my eyes to massive problems our medical system has.  It opened my eyes to the massive discrepancies between heart centers, cardiologist’s, and other clinicians.  It has changed my world view so much.  Things that were once important are no longer important, things that would’ve seemed silly to worry about just a year ago, worries me constantly every day.  People that you thought would always be there for you….disappear.  And complete strangers become your family.  The journey our family has been on in the last year is about as far off the trail that we set out on as one can go…but I am okay with that. 

The statistics for right atrial isomerism (the specific type of heterotaxy that Logan has) are beyond freightening.  I have read studies ranging from 20-40% survival rate at one year old.  And at five years this number dwindles even further….and statistically the amount of these children that make it to adulthood is painful to even think about.  I try not to dwell on these numbers.  Logan has had every single risk factor for death even among heterotaxy kids (right isomerism, asplenia, severe AV valve regurgitation, obstructed pulmonary veins) and he beat the odds.  I have so much hope for Logan because I believe we have the right team of doctors.  I think we have a brilliant cardiolgoist that has done everything under the sun to keep Logan out of the hospital and to make sure that thru all this he has a good quality of life.  And we have arguably one of the best pediatric heart surgeons in the world, who beyond being exceptionally talented, has the biggest heart and is incredibly compassionate.  I really feel like we can do this.  I really feel in my gut that Logan is going to be one that grows up to be an adult.

 The next surgery is going to be a big one and very risky.  The surgery is going to attach his IVC (inferior vena cava  – the artery that goes to the lower half of your body) to his pulmonary artery.  This direct connection will make it much easier on his single ventricle.  His first surgery (the Glenn) is when they connected the SVC (superior vena cava – the artery that runs up your neck to your brain) to his pulmonary artery, once the SVC and the IVC are connected directly to the pulmonary arteries he will have complete fontan circulation.  This is the ultimate goal.  This is so far the best possible way for single ventricle kids to live.  But all of this is dependant on one thing.  Your pulmonary arteries.  Fontan circulation requires children to have a lot of blood flow thru the PAs and Logan’s PAs are small.  It is going to be difficult for his body to adjust to the fontan circulation having small pulmonary arteries. 

Logan saw Dr. Patel last Tuesday and had an echo done.  He has grown a lot of collaterals because his pulmonary arteries are small and blood is finding other routes to go down.  He is going to need a cath in the relative near future to coil more of these collaterals off so that all that oxygenated blood is forced back into the PAs and reaches his lungs instead of  just getting lost in collateral viens.  His O2 right now is low 70’s.  He will most likely need a cath in the next 6-8 weeks unless his oxygen levels drop futher sooner.  We will be going to Boston for that cath and more discussions will happen at that time about the timing of the fontan.  We have heard that most likely he will have a very early fontan.  Most kids have this surgery between 2-4 years old.  Logan will have his at the latest 18 months.  This is because his heart will not be able to function well on just the Glenn connection for that long.  If we do the fontan too early we deal with having small pulmonary arteries and the procedure is risky….if we wait to long there could be too much resistance and his pressures in his heart and lungs could be so out of whack that it wouldn’t take to a fontan either.  Timing is going to be everything here….and just like with life, every person with a set up lips has a different opinion.  Dr. Patel is going to see Logan in two weeks from last Tuesday. 


I would also like to wish a Happy Father’s Day to daddys around the world and especially the ones dealing with children of special needs.  Logan’s Dad said his biggest fear about having a baby was that he would have one with severe medical problems.  It is so interesting because even though his worst fear came true, I think if you saw the way he cuddles Logans, and watches him sleep you would see that his worst fear has grown into the biggest love of his life.