Tag: Heterotaxy Syndrome

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.

Logan's Birth 011

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

Logan's Birth 029

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

8.10 162

Blue Feet

Boston 869

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.

Boston 950Boston 951Boston 986

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

12.10 035

2.11 049

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

2.11 052

2.11 006

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.

2.11 016

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.

2.11 209 2.11 214

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.

3.11 023

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.

6.11 032

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.

CZ_TinyTemplateE

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.

12.25 129

Christmas Eve

12.14 012

Ethan my 4 yr old Son.

12.14 014

12.7 007

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

12.24 018

Playmobil Advent Calendars

12.28 004

12.24 024

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 😉

12.28 007

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.

12.27 00412.27 001

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.

12.27 01112.27 026

12.27 03312.27 024

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

12.24 016

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

12.24 010

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.

12.17 003

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.

12.17 005

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

01_3  01_8901_47   00201_41

What a difference a year makes!

001IMG_1965

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!

 

 

 

 

 

 

 

 

 

 

 

 

 

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.

12.1 1037 (2)

(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.

 

 

12.1 379

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

 

12.1 427

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.

12.1 537

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.

12.1 636

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.

12.1 773

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.

12.1 413

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

12.1 839

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.

12.1 872

How cute did he look?!?

12.1 780

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

20120605-102526.jpg

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.

Logie in the Pool!

\

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…..

What is Logie up to?

Logan has made so many strides lately!  He is doing just terrific.  One of the greatest things that we ended up doing was hiring a nanny.  This was tough to do because its hard to turn over control, and it takes a lot for us heart Mom’s to trust anyone with our kids but it turned out to be the exact thing that everyone in our family needed.  It is allowing me to work more, which has been very good for my mental health, and it has introduced someone to Logan that he doesn’t associate with any of his medical issues.  Our new nanny has never given him meds, never seen him in the hospital, and never taken him to the doctor.  Logan trusts her! And the best thing of all is that he has started to eat for her!  it is very interesting because he still will not eat for me and he actually stops eating if I am in the room. 

He is verifying that this is all psychological and not a physical issue. So I am making a point of not asking this nanny to ever give him medicines or be involved the his health issues, because he trusts her!

I think that is has been fantastic for him to be around a caring person that he feels 100% confident won’t try to force him to take medicine, force him to put on a pulse ox, or take him to get boo boos at the doctor.  He has made so many strides since the arrival of our new nanny that it has made me realize how this whole journey has really affected him mentally. 

(love being read too)

Now that he is getting more nutrition he is able to do physically advance as well.  For the past few months he had been extremely frustrated because although he was mentally like a 20 month old, he was stuck physically in a 6 month old’s body.  Him being able to move and explore has really opened up a whole new world for him and he is so happy about it.

He is sitting up very confidently and has figured out how to go from sitting to laying and vice versa.  He is so so so very happy at this new ability.  He feels like a real person now!

With his new-found independence and strength, going to the cardiologist has gotten to be a bit more of a struggle.

(EKG and Blood pressure)

Last check up was good.  O2 is is decent (mid to upper 70’s) sometimes hitting 80 sometimes hitting 70.  He definitely looks blue when he is moving around and playing.  His feet get very blue, and the area around his mouth and eyes as well. 

He currently weighs 9.1 kilo (20 pounds) and is 30 inches in length.  He is the sweetest little thing you could ever meet.  I wish everyone could meet him. 

The last week we had a stretch of weather out of the blue where it was 75-80 degrees.  Logie had the time of his life.  Last summer his body couldn’t handle heat and he would just sweat like mad if he was in the sun. 

(he didn’t eat these apples btw….)

(we actually had to bring this picnic table inside because we couldn’t get him to come in without it)

Ethan is also enjoying Logan now that he is able to interact with him more.  Ethan almost has a bit of a playmate!

Still working on figuring out a surgery date.  Couldn’t tell you if its going to be April or June at this point.  I am hearing something different from everyone that I talk to.  Trying my hardest to figure all that out now.

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.