Category: The Heterotaxy Network

Holiday Update

We had a terrific Christmas this year.  The last two years I wasn’t sure if Logan was going to live or die and it was pretty hard to really enjoy them.  This year I had a ton of Christmas spirit and really enjoyed the season.  I don’t know what is going to happen next year but this year I had my two boys and they were both feeling well and happy.

I will just share our holiday season with some pictures.

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Christmas Eve

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Ethan my 4 yr old Son.

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First year with our Elf which was fun.  Logan didn’t understand but Ethan loved it. 

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Playmobil Advent Calendars

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One evening when it was just Logan and I we had so much fun baking cookies together.  He filled and dumped this nut chopper probably twenty times.  I was a huge help 😉

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Christmas Eve was again overwhelming for Logan.  I am not sure if its trauma from all his hospitalizations, or some sensory processing issues, or if  its completely normal, but he gets over stimulated very easily and sort of checks out.  Last year he couldn’t stand to be in the same room as the chaos, this year he couldn’t stand the sound of wrapping paper being ripped and crumpled, he sat in this chair for most of the night with his half sister Stephanie and played a game on the iPhone.  He didn’t open anything and had no interest in any toys other than this iPhone and a deck of alphabet cards.

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Christmas morning was the same.  Logan had no interest and made a face and whined every time wrapping paper ripped.  He got some alphabet and counting books and he was set.  Ethan enjoyed the fact that he got to open more presents than Logan.  Yes, Ethan is 4 and yes that is a paci in his mouth.  I have nothing to say about this, other we have just had bigger fish to fry these last few years, and quite frankly I would rather Ethan be putting a paci in his mouth than his thumb which would be picking up many many more germs.

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Love these pictures of Logan looking thru his alphabet and number books.

People were asking me about the book with all the numbers so here is a link on Amazon if your interested.  Logan Loves it!

http://www.amazon.com/ABC-Sesame-Street-Treasury-Numbers/dp/0375800425/ref=sr_1_1?s=books&ie=UTF8&qid=1358020372&sr=1-1&keywords=sesame+street+alphabet

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It was a wonderful Christmas.  Hoping to start building more happy family memories.

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As far as Logan is doing medically he is the same.  His sats are lower than everyone would like and although his cardiologist at Boston is worried we have decided to not bring him to Boston during the flu season.  We are going to wait until spring and then he will have a cath done.  Maybe they will be able to close his fenestration (the large hole in his Fontan conduit, essentially a man made ASD) and his sats would then go up but they may not be able to either.  If they aren’t able these are just the sats we are gonig to have to accept.  The fenestration closure is an all or nothing thing, they can’t make it smaller, its close it or leave it open.

He is still not eating much.  This is becoming very tiresome.  He is hardly growing (at his last appointment is was 100 grams or something) and he is hardly eating.  He is still getting most nutrients from Similac Sensitive Formula which is utterly ridiculous for a 2.5 yr old.  Yes, if you are wondering, “have you tired?” I have tried everything.

I am very anti-feeding tubes and I have actually found myself wondering about a G-tube (stomach feeding tube) lately.  Only for brief moments and I am not really even close to that point. I would only do that if he was continually losing weight and it was a very last resort.

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This is one very finicky and stubborn child and food is the only source of control he has in his otherwise chaotic life.  I have been experimenting with some recipes that I have been having luck with and will post about that soon!

He is still only butt scooting, no crawling or walking, but he is tying! He gets on all fours but his upper body strength is just not there yet.  I believe crawling is going to happen soon.

He is still on Viagra or Sildenafil every 8 hours to help vasodialate his pulmonary arteries to reduce the shunting in his fenestration.  It is working okay, which it was working a tad bit better.  I think the O2 may have worked slightly better as a vasodialator on him but the couple of O2 pts isn’t worth the hassle.

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I also recently got in the mail some pictures that were taken of Ethan and Logan last Christmas. They are some of the most precious pictures I have!  Thank you to Felicia Lewis at FML Photo Design! If you live in the Cleveland/Akron area highly recommend her! More info at http://www.fmlphotodesign.com

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What a difference a year makes!

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2011                                                          2012

 

Also, if you are a heterotaxy survivor or parent please look up The Heterotaxy Network on Facebook.  There is a group of a couple hundred of us and the knowledge level is amazing.  Sharing stories with families from all over the world has truly been inspirational.  DSC03805

Me, Logan, and Logan’s Great Grandma

~Happy New Year!

 

 

 

 

 

 

 

 

 

 

 

 

 

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

All is Calm, All is Bright

Merry Christmas to all! and a very special Christmas wish to all the children celebrating Christmas in the hospital this year.  Santa Claus was very very thoughtful this year and left a plane ticket to Boston in my stocking!  He was thinking it would be nice for me to make a trip up there to meet Jessamyn and Baby Pierce!  I am leaving tomorrow morning bright and early.  I am looking forward to meeting them and other Heterotaxy families at CHB. 

We had a wonderful Christmas at home.  Logie was a little overwhelmed on Christmas Eve, and super tired all day on Christmas.  Logie’s favorite present was 2 brand new wubanubs (so easy to please!)

I will post pictures when I get back from Boston. 

I hope that everyone had a peaceful Christmas and will soon have a joyful New Year!

Pierce featured on CHB’s Website

Social media helps bring very sick patient to

Children’s

by Tripp Underwood on December 21, 2011 (taken from CHB’s blog)

Pierce Heilinger recently underwent a complex surgery at Children’s Hospital Boston that may have saved his life. The young patient’s story has resonated deeply with parents who use social media, and even though many of those people had never met the child or his family, that online support system was instrumental in bringing him to Boston.

Pierce has heterotaxy syndrome, a birth defect that involves the heart and other organs. Normally the human body has organs that grow on both sides, like the lungs or kidneys, and others that develop on a specific side, like the stomach or liver. But with cases of heterotaxy one or more of those organs may be reversed, including the heart.

In researching her son’s condition, Pierce’s mother Jessamyn learned that despite being an extremely rare condition Children’s Hospital Boston has performed over 100 surgeries to correct heterotaxy syndrome in the past few years.

In her search she also came upon a group of internet-savvy parents—many who have children with heart defects— including several whose kids were treated at Children’s. Collectively these moms tapped into their individual social networks and through forums like Facebook, Twitter and blogs they were able to raise enough money and awareness around Pierce’s situation to bring him to Boston.

Baby Pierce’s condition may be rare, but the strength and passion his mother showed in arranging his care is not. There are tens of thousands of parents whose children are battling illness, and like Jessamyn many are using social media to educate people about their conditions or support others facing similar situations. Individually these outlets represent a small portion of the Internet population, but together they have a powerful voice that can be heard by millions.

The movement that brought baby Pierce to Children’s is proof of their collective strength.

As the online experience becomes more personalized, this type of interactive communication will become more and more common. And for parents dealing with the stress of childhood illness that deeper connection to others who share their fears and frustrations can be very comforting. But like with all online medical information, these forums should be approached with a buyer beware mentality; health information is only as valuable as the source providing it. With so many medical sites and forums competing for digital readership, more than a few inaccurate pages have attracted followers.

Fortunately for parents interested in pediatric heart conditions, there’s The Heart Center at Children’s Hospital Boston’s Facebook page. Our page offers families a secure place to interact with each other and get plenty of factual information on pediatric heart health. It currently connects over 2,000 families and is monitored by a pediatric cardiology specialist who can direct people with specific treatment questions to the proper channels.
If information on heart health and treatment is important to you, or you are looking to connect with other families who have been touched by a pediatric heart condition, please join our page and help us grow the conversation online.

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! 

 

Meet Baby Pierce

Meet Baby Pierce.  He has a very similar type of Heterotaxy that Logan has.  Jessamyn (Pierce’s Mother), myself, and many others fought like mad to get him transferred to a heart center that has more experience with  these types of children.  That was going to be his only shot at living.  Pierce is so sick that even in the best hands his recovery is going to be very hard.

For a Preview of the CNN piece please veiw the short promo piece on the below link (takes a little time to load…) 
http://i.cdn.turner.com/cnn/.element/apps/cvp/3.0/swf/cnn_416x234_embed.swf?context=embed&videoId=health/2011/12/16/pkg-saving-baby-pierce.cnn
Finally, after the fight of our lives, Pierce arrived safely in Boston last Fri. night.   He is going into surgery today.  Today is the day that Pierce’s heart will get fixed.  Please keep this family in your hearts today as Pierce under goes a long long long surgery.

For the full story on Pierce please tune in to Anderson Cooper 360 on CNN tonight.  This is a very important story.  This is a story not only about just Pierce but a story about how difficult our current medical system makes it to get our children the best care we can.  This is a story that affects us all.

And please remember to check out The Heterotaxy Network on FB we have a small but mighty group of heterotaxy families.   I can guarantee you that there is no group on the planet more knowledgable about heterotaxy than our humble group of families.

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.