Category: Fontan

Fontan Update

We are officially 4 months out from the Fotan.  I had more anxiety about this fontan than all previous surgeries combined.  It is so much harder the older they get…..it does not get any easier, it gets way harder.  I remember thinking when we were singing Logan to sleep while his sedative took effect before they took him away for surgery, that I absolutely cannot go thru this again.

So many kids have the Fontan and then their oxygen sats are high 90’s and everything is near perfect for years.  I hear talks of familes that only have to check in with cardiology every 3-6 months.

Although, I did not expect Logan to have as smooth of a Fontan as other single ventricle kids, I didn’t expect him to come home and be satting 68% either.  Only a heterotaxy child goes into a fontan satting 85 and comes out satting 68.  Talk about a complete let down.  I have come to realize (an accept) that Logan may never get to check in with cardio every 3-6 months and it has taken me 4 months to get there.  After Logan being on around the clock Oxygen and now Sildenafil (Viagra) and his sats still are very low, I have realized that I have “buck up” (as my Dad would say) and keep moving forward.  I am not going to have the huge growth spurts, catching up, and just taking off periods that others have after the Fontan.

Logan is still very small weighing in at a whopping 21 Lbs…

Logan is still unable to walk or crawl on all fours.

Logan is still very blue. He honestly hasn’t been this blue since he was 6 months old.

Logan is still not out of the woods….

We had a cardiology appointment on Thursday that I have only been able to tell a handful of people about.  Everything went well…except his sats were 77-78%.  There was a new student nurse that did his EKG, Pulse Ox, height/weight, so I knew this was going to take a lot longer than normal….Because she was a new nurse she happily let me take the lead on trying to get Logan’s Pulse Ox.  I told her that we needed to take our time with this because Boston says that PO (pulse ox) in the 70’s means we have to come back to cath and 80’s means we are okay.

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(you can see in this pic that he is tingeing purple)

I spent almost 40 mn.  trying to get an 80%.  I tried both feet and toes, and his right hand.  I couldn’t get a damn 80%.  About two weeks ago he was looking bluer than normal and I POed him at home while he was watching a cartoon and it said 76-78%.  I just decided to bury my head in the sand and ignore it until his next cardio appointment.

There are so many things that could be going on I don’t want to speculate, hopefully he just grew some collaterals that can be coiled off.

I haven’t even told his doctors at Boston yet.  I don’t know if I have the stomach to go back yet.  And its winter, there could be RSV going around.

I have so many fears going thru my mind right now, that I am trying my hardest to drown out.  But I just can’t help but think, this is not the way Fontans are supposed to go.  And I highly doubt they will be able to close his fenestration this spring. Which is going to mean another whole year of low sats….worrying constantly about him getting a respiratory infection, and not being able to let either of my boys around many people 6 months out of the year.

So I guess now that I am making this post public, I guess I am now obligated to let his surgeon at Boston know what his sats are.  Don’t get me wrong, he knows we have been struggling and have been on oxygen and Sildenafil, but the last he heard Logan was low 80’s.

Taking a bit of a 180 here, there have been some positive outcomes of the Fontan that are hard to ignore.  First of all, Logan is much much happier.  He feels so much better.  I imagine this is because his heart is working so much less and he has mostly passive blood flow (as opposed to his heart having to pump it all out) and are you ready for this?  drum roll……..

He is cognitively ahead of his peers now! He knows the entire alphabet, counts to 12 forward and backward, knows all his colors and shapes, and is very adept at playing various puzzles on my iPhone!.  This is quite amazing to me, I had always thought that everything  he has been thru would undoubtedly cause learning disorders and cognitive delays.  The amount of narcotics, sedation, low oxygen, bypass, and severe heart failure, how could he not be cognitively behind?  Well, he isn’t! And a good friend of mine that teaches elementary school said he is ahead of many kindergardners.

I am so proud of little Logie.  I only hope that he stays happy being physically weak and mentally strong…I am dreading the day when I have to explain why he can’t be a baseball player.

Since I haven’t updated in so long I thought I would share some of my favorite pics of Logie since we have been home.

 

 

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This is Logan shortly after his new-found love for coloring and crayons.

 

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This picture makes me laugh for so many reasons.  Since his Fontan happened when he was a toddler everything that was done to him made him very angry.  I mean VERY angry.  So angry that going to the cardiologist’s office now really pisses him off.  I mean he just had a massive open heart surgery and now he has to go back to the cardio’s office every few weeks?  One time he threw my iPhone at the receptionists desk and shattered it.  But every time we go now, he doesn’t let anyone take his EKG stickers off.  And they do not come off in the bath.  I pick gross bits of sticky glue off his chest for days and days now.

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First time at the Zoo.  He loved playing with the other kids.  This is a first for him.  He has never previously felt good enough to enjoy kids.

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Ethan and Logan waking up after sleeping together on their “floor bed.”  I am completely okay with the fact that my children love to sleep on the floor next to us, in fact I love it.  That is Logan’s oxygen concentrated in the background.

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First time at the pumpkin patch. He was terrified of the wagon so I carried him in my ergo carrier.  He would live in that thing if I let him.  He is very attached to me.

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More coloring….pretty much the only thing that distracts him at the doctor’s office now.

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Logan dressed up as a pumpkin for Boo At The Zoo.  The Mended Hearts Of NE Ohio got tickets donated to them and we all met up there.  A fellow Heterotaxy Mother started the chapter here and it has been great to meet more heart Moms.  You can see how insanely happy Logan was to be out and about.  Most were joking that they never thought I would let Logan come to something like this (germs) but we had some fluke 80 degree weather so I decided to go last-minute.  It was still a risk, but the joy he had was worth it all.

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How cute did he look?!?

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Tried so hard to get a pic of them together.  Tried for 45 mn. and then gave up.  This was the closest I got.

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On of my favorite pics.

This was taken on our drive home from Boston.  We stopped for the night and seeing my two boys in bed together, was one of the best sights I have ever witnessed.

Home From The Fontan

Most people follow Logan on Facebook and they know that we have indeed been home from the Fontan surgery since July.  It went well.  We ended up only being inpatient for 2 weeks, which, for this big of a surgery is amazing for Logan.  Ironically, he came home with much lower sats than he went in with.  Typically a Fontan surgery increases your sats, but for Logan it lowered it.  Dr. Pedro Del Nido decided to do a fenestrated Lateral Tunnel Fontan.  Pedro put a larger than normal fenestration in to make sure that Logan could get out of the hospital quickly.  Many times kids drain fluid thru chest tubes for days to weeks to months after a Fontan, but having the large fenestration reduced the risk of pleural effusions, however it left him with lower sats than pre-fontan.  The fenestration is essentially an ASD to allow some blue/red blood mixing if his pressures get high (this is called shunting.)  This residual shunting made Logan’s sats acutally level out at 68-72% once we got home.  This is obviously not exceptable and we went around and around and back and forth about what to do and if we should cath him.  We decided to be conservative first and try oxygen at home (to vasodiatlate the pulmonary artery) and then we experimented with Sildenafil as well.  His pulmonary arteries were in fact reactive to both and we are now on around the clock Sildenafil (Viagra) and his oxygen saturations are now 78-82%.  This is much better.  Ideally, everyone would like Logan to be 85%, but, it just isn’t happening.

I have not blogged in a long time.  There were a lot of mixed emotions about the Fontan and coming home from it.  For 3 years now I have been told that for Logan to live he would need a Fontan.  I have been anticipating this Fontan for so very long.  The past 3 years have been excruciatingly hard and overwhelming, to be done with the Fontan now…..it feels wonderful! He isn’t out of the woods, we still need to get his O2 levels up, this spring they will attempt to close the fenestion in the cath lab.  If his pressures are too high and they are not able to, he will be stuck with these sats for another 1-2 years, until they try again.

Since we have been home and a million bricks have been lifted off my shoulders I have disconnected from this blog, FB, my phone, and I have been just trying to enjoy my family.

Thank you for being patient with my updates, I really needed a break from all this for the time being.  I do however post more on Facebook and if you would like to follow not only Logan but other kids like him, please look up The Heterotaxy Network on Facebook.

~Kristen

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.

 

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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.

Logie in the Pool!

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Just wanted to share a quick video of Logie playing in his little pool!

He has been having so much fun with the nice weather we have been having.All of this exercise has really built him a little appetite too.  He is eating foods that he normally wouldn’t even try, he is loving mashed potatoes right now! We have a cardiology appointment this afternoon, just a check up, I don’t anticipate anything to have changed.  We are still looking at his Fontan around June 13th.  It is coming up really fast and I am trying my hardest to not dwell on it, but to just enjoy these last few weeks we have before surgery.  I anticipate that going thru this surgery will revert him back to the scared and timid boy he was just 6 months ago, so I am trying to get him outside and playing as much as possible. 

Here are some more cute pics…

First time in the sandbox.  At first he wasn’t so sure about the sand! Now he loves it and whines to go in.

First trip down a slide with Mommy of course! He loved it!

He looooooves to flip the pages of books and to be read to.  Ethan doesn’t have the patience to sit and read books, so I am loving being able to cuddle Logie and read books.  His favorite are Dora The Explorer books.  He is always so surprised to see Dora in books, he looks up at the TV and then back at the book, it’s too cute.

Still drinking out of a little baby bottle and scrunches his feet up like a newborn.  I am completely okay with this.  The bottle issue is not even on my radar screen as issues to worry to about.  It also is how I “secretly” give him his heart meds 🙂

 

I also wanted to remember Sweet Elise.  She passes away 2 weeks ago, and her loss was just devastating to me.  She has the same diagnosis as Logan and was born a couple of months after him.  Her Mom became one of my closest Heart Mom friends, and we watched in amazement as our two kids followed such similar paths.  This is a family that I have been in constant contact with for over 1.5 years.  Elise’s Mom Tessa, has provided me with so much support and encouragement thru Logan’s battles.  My heart is broken for Tessa and her family.

Elise went in to have a cardiac cath and her Fontan (third stage surgery), and never got extubated (breathing tube taken out) after the cath.  Her heart was too weak to go one to the Fontan.  Elise is Logan’s heterotaxy sister and she is greatly missed.  Elise is forever in our hearts….

                                       Remembering Elise

Surgery Date.

So we got the call today.  The call from….scheduling.  I knew this day was coming.  But to hear a date.  An actual pre-op, cath, and surgery date has literally made me sick to my stomach.  The worst part…we have to be there June, 8th….his birthday.  It’s like a sick joke.  Having a very very hard day today.

Not to mention he has been very very blue this entire week.  I thought to myself thank goodness we are going to be in Boston in a few weeks.  Then to find out this afternoon it’s not till June.

I haven’t wanted to Pulse Ox him (check his O2) because honestly, I don’t want to know.  But I thought since now it was going to be so long before his surgery I would look.  Ugh.Ugh.Ugh.  It said he was 65-70.  I haven’t seen the 60’s in so very long.  I feel sick.  I rarely have a pity party for myself because Logan is the one that bares everything, what do I have to complain about? But today, I have fallen into self-pity, anger, frustration, and sadness.  I am so pissed off that Logan has to be cut open again.  It’s so unfair.  So very very very unfair.  I am at the same time fully aware of how lucky we are to live in a time and place where he can have access to the best care, I know all that.  I just look at his sweet little face and his silly hair, and I just want to take his place so bad.  I wish I could just go thru this for him.

I told our pediatric cardiologist when I was still pregnant that my absolute worst nightmare was to get him to be 2-3 years of age and then lose him.  We can’t lose him now.  I am so scared.  Absolutely terrified.  The only relief that I feel in all this is that we Dr. Pedro Del Nido.  He could not be in any better hands.  That is what I hold on to.

There are so many scary things about the Fontan.  I don’t even have the stomach to get into right now, but it’s not just the surgery, there are all sorts of complications that can happen from the Fontan that are awful.

Just a really crap day.  I had no clue it would be so upsetting to get an actual date.

The emotional stamina it takes to get thru all this is just staggering.  I feel so beaten down.  I feel like I have been punched, kicked, thrown in the street, drove over repeatedly by a dump truck…and now someone is saying to me, ‘hey, get up, we have to go climb mount everest now.’

I rarely do posts like this, I try to keep strictly to the facts about what is going on in our life.  But this is the facts of our life.  I was explaining to a friend of my how precarious Logan’s life is.  I said its like being an Olympic Ice Skater.  I feel like for the past 2 years we have landed triple axles, and now we are coming up to the last one….

The amount of time, research, and ABSOLUTELY meticulous care it took to get Logie to wear he is right now would really surprise most everyone.  Logie didn’t get lucky, I fought like a crazed lunatic, to get him to the best surgeon/hospital that I could find that could fix him.  I was told twice Logan needed surgery at a different hospital and only because of the meticulous research I had done, did I know to say NO to those surgeries.    It’s because of me that he is alive.  I know that and everyone tells me that.  Well, what if I am missing something now?  What if I didn’t read some new research?  What if there is actually a center in Asia that could do better?  What if we waiting a year or two more and there is some new technology and he wouldn’t need the Fontan?  What if that surgeon in California that is growing stem cell arteries could do something for him?  It is so much pressure.  So so so much crushing pressure.  I feel at my absolute very weakest and I am being asked to perform my last triple axle…. I am skating around that turn, slowing down to prepare…..

Surgery Time

So, I have not updated in a long time. Well, actually about 6 weeks.  I have been spending all of my free time in organizing the new Heterotaxy Network website and forums, and trying to just decompress a little and enjoy the non-surgical months a bit. 

I found out that Logan needs to have his next open heart surgery, the Fontan, next month and I feel as though someone has just kicked me in the gut.  I knew it was coming and we have had many talks about being this spring, but to “next month” was very hard to hear.  So hard to hear and process I have needed some time before I could even discuss it. 

Logan needs to have his fontan surgery next month for a number of reasons.  His oxygen sats are mid 70’s and having this low of oxygen levels does not give him much buffer in case of illness.  Currently, his top half of his body via the superior vena cava is better oxygenated, but his lower half via the inferior vena cava in not connected and is not oxygenating well.  This is important for several reasons; 1. being that having his superior and inferior vena cava’s connected to his oxygen circulation will greatly improve his over all oxygen levels, and because when the inferior vena cava (IVC) is connected in, blood will begin to travel thru his liver.  Liver blood is very important for his hemodynamic (blood flow) stability because it has been found that there is a unique function that the liver play in the blood by putting special enzymes in it that help regulate your body’s ability to stop the growth of collateral veins. 

Everyone has heard of those stories of a man or women having a blocked artery only to find out that the body actually compensated and grew a new path around the obstruction.  That new path is a collateral vessel.  In those situations collateral vessels are beneficial to the heart.  But how does the body know when to grow collaterals and when not too?  Well, that is not completely understood.  What is  known is that there is a special role the “liver blood” plays in this process which is it stops your body from just growing the “new paths” continuously. 

For heterotaxy kids this type of “checks and balances” is essential because heterotaxy kids (for an unknown reason) tend to grow these “new paths” much quicker.  Again there are some instances where they could help your heart, but more times than not these news paths end up taking oxygenated blood away from the lungs.  The sooner that we can get his blood circulating thru his liver, the sooner he will get this “hepatic factor” into circulation. 

Lastly, the sooner we can get his bottom half into circulation his heart won’t have to work as hard.  When his heart isn’t working so hard there is a better shot at keeping his AV valve functioning well for a longer period of time.  I always knew that the repair that Dr. Del Nido on Logan’s heart was amazing, but the more I learn the more I realize how spectacular the repair actually is.  When Logan went in to have his first AV valve surgery his valve was a 4+ Severely regurgitating, and just barely functioning.  Currently, 12 months after his last AV valve repair (he has had 2) there is ABSOLUTELY NO REGURGITATION.  NONE. PERIOD. I am saying this in all caps because I want to emphasize this point.  I have heard many cardiologists and surgeons say that it is impossible to fix these valves. That no one (worldwide) is having luck repairing these AV valves.  Well, I just want to shout it from the roof tops.  My son is living proof that Dr. Del Nido at Boston Children’s Hospital is doing what the rest of the world is claiming is impossible.  He is repairing these valves, and not just to a manageable level, he is repairing them to a level of ABSOLUTE ZERO regurgitation.  I am even temped to post his echo online because so many doctors do not believe it.  If you have a child with an “unrepairable” AV valve, please seek the opinion of Dr. Del Nido at CHB. 

Anyway, sorry for the rant but I am so sick of hearing that it isn’t possible to fix them. It is.  But please…don’t think that just because Dr. Del Nido can do this repair, that any surgeon can if they just tried.  This is not the case.  This is a very specialized surgery that should be done by someone with  experience. 

Beyond the fact that Logan will need to have open heart surgery next month….he is doing fantastic.  He is so happy.  Seriously, this kid is SO very happy.  He is now 20 pounds (9.1 kilo) and is 30 inches in length.  He is over most of his trauma from the last surgery, and is willing to let people hold him, and his is willing to touch and explore new things. He isn’t terrified of every sound and movement anymore.  He is just loving life….which is one of the hardest things about this next surgery.  He was so traumatized for so long…the thought of him going back to being scared of everyone and everything is utterly heartbreaking.  Honestly, I have to just end this post right now, because just forcing myself to write this post is making me feel sick.  I did however, want to update everyone.  I will make an effort to do so more often, it was just nice to take a break from writing about all of this. 

Logan is not on the surgical schedule yet, but most likely it will be the 2-3 week of April.

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…