Happy Thanksgiving

¬†Although, Logan didn’t eat any turkey he ate about a bazillion bread and muffin crumbs ūüôā

Happy Thanksgiving from Scott, Kristen, Ethan, and Logan.

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Fontan Talk….

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

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

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

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

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

The biggest questions that I have surrounding the fontan are…

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

2. Timing

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

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

The Adult Fontan Patient from ACHA on Vimeo.

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

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

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

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

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

I am having a very hard time accepting this. 

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

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

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

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

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

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

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

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

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

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

Logan Update

 

I have not been meaning to keep everyone in the dark about Logan and his progress.¬† I don’t really know what is going on with me.¬† I think after 18 months of this being my reality its still¬†really tough to write about him and his progress (or lack their of).¬† When I found out that his oxygen levels were a bit higher and that we could hold off on the Fontan¬†for the winter, it was the biggest gift….I thought.¬† But it has just given me more time to worry about the Fontan.¬† It is going to be here in a blink of an eye and everyday that Logan gets bigger and stronger and shows more and more personality his next OHS is going to just get tougher and tougher and tougher.¬†

It literally makes me sick.¬† It makes me sick to think about them having to re-cut open his chest (his nearly perfect¬†incision¬†is barely visible right now)¬† It makes me sick that he will have to spend hours and hours getting IVs, lines, and blood draws.¬† About 4 weeks ago I had to make an ER trip for myself, I was having a horrible asthma attack (thanks autumn…) and had to get an IV of steroids.¬† When the nurse went to go get the stuff to start the line I held my breath and prepared myself for a big ordeal.¬† It wasn’t…it took 2 mn.¬† and it hit me how different the perspective is.¬† For those of you that haven’t experienced this getting an IV, or PICC¬†line in a baby with a complex heart condition (bad veins) it HORRIBLE.¬† It takes hours.¬† It takes 3-4 people holding your baby down while they stick him over and over and over again trying to get access.¬† Most of the time with Logan we would go thru 2-4 people before someone could “get him” many times they would have to use an ultrasound machine just to find his small frail veins.¬†

I just cannot believe that we have to go thru this again. 

But right now…Logan is so happy.¬† He is getting less and less scared of people every day.¬† He even lets other people hold him now!¬† He jibber¬†jabbers¬†constantly which is the sweetest sound to my ears.¬† He still cannot sit up unsupported or crawl, but he has compensated and scoots on his back.¬† He gets around quite well this way.¬† He loves to sit in his high chair and “eat” dinner with us.¬† He more or less pushes a few crackers around but loves feeling like a part of the family.¬†

His brother Ethan just turned 3 this last week.¬† He is an amazing little boy with an amazing talent for guitar playing.¬† I know what your thinking…but I am serious he actually plays.¬† Scott is teaching him notes!¬† Ethan is started to understand more and more about Logan as well.¬† Last week we had a new babysitter here and one of the first things he did was lift up his shirt, point to his chest, and tell the sitter that Logan “has boo boo¬†on chest”¬† I had to explain to Ethan that tomorrow a doctor has to come (really a nurse) to give Logan a boo boo¬†on his leg (his RSV shots)¬† Ethan is very upset by all this and doesn’t want Logan to get anymore boo boos.¬† Its just heartbreaking.¬†

I wish too…that Logan didn’t have to get anymore boo boos.

Ethan with his prized Green Guitar that he got from my parents. 

And Ethan taking a break after a  jam session with Dad!!!

Now for Logie Pics….highlights from the last 2 months

 

Loves that he can now sit up in the Bumbo. It has given him a whole new perspective on life. 

He loves to sit at the table.¬† This is him “eating” he maybe eats 3-4 tiny bites of a cracker.¬† I have found that he will eat a handful of goldfish though.¬† I have tried all different types of foods and he still is being stubborn about the food being dry and crumbly.¬†

About 4 weeks ago he grabbed a sippy cup from the couch and just started drinking it.  Luckily I had my handy IPhone near by and actually caught the moment!!! His first time drinking from a sippy cup.  Even with the new sippy cup he will only drink apple juice at room temperature.  Nothing cold nothing warm.  Nothing too sweet, nothing thick (tried smoothies and milk) he is VERY finicky when it comes to textures, flavors, and pretty much anything new.  Starting to wonder about Sensory Processing Disorder. 

 

 

 

In turns of sleeping…that has gotten a little better.¬† As he is starting to eat more at a time (10 goldfish instead of 1) he will go a little longer in the night without getting hungry.¬† I think we are just stuck in the newborn eating/sleeping schedule because he doesn’t eat food.¬† I mean that is why we start our babies on cereal and food is to help them sleep thru the night…please Logan just eat!!!

In terms of physical development he is still like an infant.  But mentally he has turned into a little boy and it is so exciting to see his personality come out.  He loves music like his brother and gets so excited and moves his head back and forth whenever something comes on. 

Logan has an echo on Thurs…PLEASE let there be no surprises….please… oh and almost forgot to mention.¬† He weighs in at 18.5 pounds now.¬† Not sure if that is good or bad.¬† We will find out on Thurs.

 

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.