Category: pulmonary vein stenosis

I wear red for Logan.

The Month of February is Congenital Heart Disease Awareness Month. Today had me thinking about the last 3 years and everything we have been thru. Honestly, I don’t know how I got thru it. From getting the devastating diagnosis that kept getting worse every single time we saw a new doctor, to having to rock him in my arms for 8-10 hours a day just to keep him calm while he was in severe congestive heart failure, to his first surgery in which we spent 6 weeks in an intensive care unit wondering if he would ever come home. During his second surgery (his ‘sutureless’) which was a complete shock in and of itself, we thought we were having a cath, but 4 days later we were having his 2nd open heart surgery. The most traumatic moment was when we came the closest to losing him. I remember his heart rate was getting up to 285 and his BP was dropping to almost 30….
His surgeon, looking very concerned said (which is forever ingrained in my mind) “I have to get in there right now, there is no time for anesthesia to come, I need to operate now.” We left the room shaking….I asked the nurse, “no anesthesia?” I was told, “well he has morphine in his system.”
These moments are the nightmares I live with every day. I know that I will never be the same.

I wear red for Logan.

Today had me thinking about all the children and their parents that I have gotten to know. The children that have passed on from this life are the ones that leave me with a burning desire to advocate and improve the outcomes for our kids.

I wear red for William.

I wear red for Noah.

I wear red for Elise.

I wear red for Pierce.

I wear red Ava.

I wear red for Rita.

I wear red for Jayson.

I wear red for EJ.

I wear red for Brookyn.

I wear red for Savannah.

I wear red for the countless heterotaxy children that I never met but whom I will forever be connected to by heart.

I don’t think its possible to describe the life we lead as parents to these children. The ups then downs, the unknowns, the waiting, the questions that can’t even be answered.
“there just aren’t enough kids alive, with what Logan has to have any real answers.” I have been told that one countless times.
If you just look at Logan’s first year of life, you will get a glimpse, of what our life has been like.

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As far from any dream about ‘ideal birth plans’ as one could get

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He was taken from me immediately, and I was not able to see him till the next day.  Excruciating is an understatement.

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Staring at my boys the last night before Logan’s first and most risky surgery, wondering if this was the last time Ethan would see his brother. At this point even though Logan looks like a normal baby….he really only had weeks to live.

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His bidirectional Glenn, AV valve repair, TAPVR repair, and SVC ligation.

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Yes, this was Logan’s post-Glenn oxygen sats.

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See how very blue he was in this picture?  It looks like he is holding his breath or something but in fact the path that blood goes after getting oxygenated in the lungs was blocked and he had close to only have of the oxygen in his blood that a healthy child should have.

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At this appointment I was told that Logan’s only chance of survival was a bilateral lung and heart transplant, because that blockage was not ‘fixable.’  This was the point that I decided that the only team I was going to listen to was Boston Children’s Hospital.  They disagreed, and this is when he had his ‘sutureless’ repair.

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Despite Dr. Jim Lock doing an amazing cardiac cath and bringing Logan’s sats up 20 points, it was decided that he needed to have the obstruction surgically removed.

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Despite the surgery opening up the pulmonary veins he still settled out in the low to mid 70’s post Glenn.  His heart however, was not having to work as hard, and for the first time, he didn’t become drenched in sweat while eating or crying.

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Logan on his first birthday.  At 12 months old, Logan could not sit, crawl, or roll, Logan wasn’t even strong enough to hold his head up yet.  He was medically anorexic and wearing 3-6 month clothing.

This was just Logan’s first 12 months of life. 

That is why,

I wear red for Logan.

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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.

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. 

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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???

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