Tag: Heterotaxy

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 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.

Baby Pierce Update

Just got off the phone with Jessamyn and Pierce is doing astoundingly well.  For a family that was told surgery would most likely be a death sentence things have really turned around since Pierce was in Boston.  Pierce had surgery on Monday afternoon, went in around 1pm and came out around 8pm and has since, he had a TAPVR repair, AV canal repair, and other repairs with no name because Pierce is so complex that there are no standard repairs. 

 Critical 48 hours after open heart surgery have been completely uneventful with no codes or desats.  He is following his post op recovery to a T.  

I have to say this…Children’s Hospital of Boston never gives up on kids.  They bring hope to kids that others have given up on.  Not just in Pierce’s case but I personally know of 5 other families in the last 6 months whose children were sent home to die by another hospital only to be giving the tremendous gift of life by the Cardiac team at Boston.  CHB is in a league all its own.  Thank you, thank you, thank you, CHB for never giving up…as a Mom with a child who carries an 80% mortality rate you have brought me nothing but hope, and you are doing that every single day for so many families.  Not only do they have the best outcomes but their outcomes are on the absolute sickest children in the world. 

As Pierce recovers beautifully from his extremely complex surgery another little boy named William is set to go to step-down at CHB.  William was another Heterotaxy boy who was sent home to die by another hospital.  Just 2 short months at CHB William is thriving for the first time in his life! For the first time in his life he is all smiles and off a ventilator.  Children’s Hospital of Boston has given these two Heterotaxy families Christmas Miracles!  And from the very very very bottom of my heart and the Heterotaxy Community THANK YOU BOSTON! 

 

The Heterotaxy Network

First, I am sorry for my lack of updating this blog lately.  I have been focusing much of my attention on getting our new Heterotaxy Network site up and running.  This is incredibly important to me.  This syndrome is so rare that in order to really understand what is working for them and what isn’t we need a website to be able to share information with each other.  We have run into some hiccups…( 3 web designers in row committed to the job and then backed out) but we still have a developer and a fabulous user experience expert (thank you Maria!) and we are determined to get this site up.  The site is going to include a memorial page for our Heterotaxy friends that have passed away, as well as inspiring stories of hope.  We are going to get our own forum up so that we can talk and ask each other questions, and have a collective (and current) source for all Heterotaxy related research and publications. 

When I was given the diagnosis of Right Atrial Isomerism/Heterotaxy Syndrome at my 20 week scan, I immediately went home to do as much research as I could about this defect.  I know enough about the medical community to know that not all doctors, surgeons, and hospitals are created equal and that I was going to find the best team I could for Logan.  There is so much that I can’t control in this world but I knew I could take some of the control back by finding the best team I could. 

When I first googled Heterotaxy Syndrome I was stunned. There was NOTHING.  There was obscure research articles and publications using abbreviations that I didn’t understand.  There was no statistics, no answers, almost no information at all.   So I did what any stressed out, neurotic woman would do…..I went to www.amazon.com and searched for books about pediatric heart surgery, pediatric heart defects, congenital heart defects, open heart surgery, and many more.  I ordered a stack of books….and I read….and read. 

FOR MONTHS.  I studied all things heart related.  And then I read this book….

Walk on Water: The Miracle of Saving Children’s Lives

 

http://www.amazon.com/Walk-Water-Miracle-Saving-Childrens/dp/0142004111/ref=sr_1_2?ie=UTF8&qid=1320894829&sr=8-2

If you do one small thing that will greatly improve the outcome of your child’s care it is this….

READ THIS BOOK. 

 I know that all of this is scary and I know its easy to bury your head in the sand, but it turns out….we have every reason in the world to be terrified, scared, and to question our doctors.  This is a very hard book to read.  It is so real, it is exactly what we go thru…the only difference here is that you get a glimpse of the other side.  What the doctors think…what they are not telling us.  

And what they are not telling us is frightening. 

We must educate ourselves so we can advocate for our children.  I the last few months I witnessed heroic heart repairs from surgeons and heart centers that fight till the very end for our kids. And then I have seen the exact opposite.  Heart centers that just give up.  They don’t try….they say it’s not worth it, it will be too hard on your family, it will be too much for your baby to go thru……

 Logan has the worst heart defect you can have and has had all the even worse ‘Risk Factors For Death” as stated in all the publications….and he is alive and he is happy.  Was this luck? NO Was it Fate? NO  It was months and months and months of obsessive reading, studying, and analyzing what would be the very best care for Logan.  I sought opinions from multiple hospitals, surgeons, and pediatric cardiologists.  Everything in the literature said that RAI/Heterotaxy with Severe AV valve regurgitation was a death sentence.  So I took him to the best pediatric heart valve surgeon in the country (Dr. Del Nido – Boston).  Logan now has NO regurg in his valve.  He also has small pulmonary arteries (the tube that brings oxygen to the lungs) and acquired Pulmonary Vein Stenosis ( all “Risk Factors For Death” in RAI/Heteortaxy). 

There are experts out there for various defects.  Like Dr. Del Nido being the foremost expert on pediatric heart valves, and Dr. Frank Hanley (Stanford) being the world expert on correcting MAPCAs (when you don’t have one tube taking oxygen to your lungs but a bunch of very tiny ones)  but how do you find this information?  What do you do when you get the diagnosis of Right Atrial Isomerism & Heterotaxy Syndrome and google it and there is nothing there???

This is why The Heterotaxy Network is so important.  Not just for families here in the US but for families from all over the world.  There is hope.  And by bringing us together and sharing stories, and resources, we can help each other, we can help the doctors.  There is so little they even know about heterotaxy.  I can’t tell you how many times a family has shared that their pediatric cardiologist had to GOOGLE heterotaxy before having a family conference.  And what good is googling heterotaxy when there is nothing there? 

But none of that is even slightly as upsetting as the discrepancies between pediatric heart centers.  In a world of sadness, loss, grief, and tremendous responsibility that we hand over to our trusted doctors, it turns out that many times our precious child is not the priority. In many cases politics, ego, money, resume building, paper writing, conference speaking, and administration pleasing are all the priorities that come before our children. 

We need to be their voice. 

Many, many, many pediatric cardiologists have come out of the woodwork to agree with what I am about to tell you right now.  There are less than a handful of surgeons that they would let come near their own child if they had a diagnosis as complex as Heterotaxy Syndrome. 

Can they tell you that? NO

Will they lie to your face? YES

When you ask them…”what would you do if it were your child?”

9 times out of 10 they are not being completely honest.

But it It is not all about the surgeon anyway.  Was it extremely important to find Dr. Del Nido to fix Logan’s AV valve? YES But equally important was the skill set of the nursing and CICU team that cared for him afterwards.  Heterotaxy is incredibly rare and unless you have a team of nurses and doctors that are versed in all things heterotaxy your child will likely get into big trouble in a variety of areas….(sepsis, vent dependence, feeding issues, low nitric production, just to name a few…) Heterotaxy kids are so much more complex than just a heart defect, you need to find a center that has a good pulmonology, gastrointestinal, CICU nurses, infectious disease, and a hospital that has made a (real) concerted effort to reduce their infection rates (infection is the second leading cause of death for our kids….)

My goals for The Heteortaxy Network are very simple.

1. Connect Families all over the world with this illness

2. To provide a comprehensive resource of all things related to Heterotaxy for families as well as clinicians.

3. To help facilitate a change in the outcomes for our children.

Please stay tuned for more information about The Heterotaxy Network, how you join, how you can help, and how you can connect with other families in your country.