Category: Bi-Directional Glenn

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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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???

Long overdue update.

So after being very confused about how Logan can be doing so great heart wise and yet still be blue and breathing heavy, I pushed Dr. Patel to do an echo sooner rather than later.  For my own piece of mind I just couldn’t wait another 2 weeks.  My biggest fear was that he had pulmonary vein stenosis once again.  This is what caused us to have the last pop-up open heart surgery.  unfortunately, if this does happen to Logan again we will most likely have to put him on the transplant list.   I was A WRECK waiting for this echo.  I mean really a complete nervous wreck. 

 Luckily Dr. Patel was able to squeeze us in fairly quickly.  Once we got there the nurses immediately commented that he looked bluer (thank you this is what I have been saying – Mom’s know these things) and once his pulse ox was taken it became very apparent, his oxygen is now around 72-73 instead of 80. 

(watching Elmo while getting his echo done)

Long story short Logan heart still looks good.  There is NO pulmonary vein stenosis that is visible.  His last sutureless repair on his pulmonary vein stenosis still looks excellent, his Glenn looks great, his valve is still holding up.  The only thing is that he still has relatively small pulmonary arteries.  So most likely because his pulmonary arteries are on the smaller side blood is finding alternative paths to travel down i.e. collaterals.  Heterotaxy kids are known to have abnormal vasculature so this crazy collateral growth is more common in kids with heterotaxy.   But keeping in mind the perspective that he could have had pulmonary vein stenosis, I was hugely relieved.  I mean collaterals….come on,  that’s just a cath fix. 

So the plan right now is to do nothing until Logan’s PO (pulse ox or oxygen saturations) drops further he is going to need a cath to coil more collaterals off.  This poor baby’s chest is just full of platinum (that is the metal used in coiling) his chest x-rays look really bizarre with over 25 metal coils in there right now.  I can’t imagine what some doctor one day is going to think when they do a chest x-ray and they don’t know about him. 

And then there was the G-Tube drama.  I just can’t get over how something that is typically no biggie in 99% of population quickly becomes so complicated and life threatening to a child with heterotaxy.  It was a one situation that clearly illustrates what Logan is going to have to struggle with for the rest of his life.  So to begin with, the pediatrician (who by the way- should we win the award for best pediatrician EVER!!!) had set up a conference call between Dr. Patel (cardiologist), our GI doc, myself, and him to discuss the big picture plan for Logan and to go over different contingencies (this phone call was one of the most helpful things we have had during Logan’s care and with a whole post in-an-of-itself) but one of the unanimous decisions that was made was that Logan needs a G-tube put it to help him gain weight. 

You would think that this would be a pretty straightforward and simple task to get accomplished.  Well, not for a heterotaxy child.  As it turns out his mixed up abdominal organs make placing a G-Tube much more risky.  Because Logan has a small misplaced stomach and a hiatal hernia he is at a very high risk for aspirated milk into his lungs. Because Logan is asplenic and any major infection could kill him the last thing we want is milk sitting in his lungs and causing an infection.  So to avoid this risk a fundiplication surgery would also have to be done. 

(fundiplication illustrated)

Basically wrapping the top half of the stomach around the the bottom half of the esophagus. 

*Excerpt taken from WebMD.com

How Well It Works

Studies show that laparoscopic fundoplication improves GERD symptoms in about 6 to 9 out of 10 people who have the surgery (depending on how experienced the surgeon is). But no studies have proven that laparoscopic fundoplication surgery is effective in maintaining healing of the esophagus over  the long term.1

  • A successful surgery does not guarantee that you will never have symptoms again. Some studies show that only about 1 out of 10 people who have fundoplication surgery done by an experienced surgeon have symptoms come back in the 2 years after surgery.2 But there isn’t much research on how many people have symptoms come back after more than 2 years.
  • About 2 or 3 out of 10 people who have surgery to relieve GERD symptoms have new problems (such as difficulty swallowing, intestinal gas, or bloating) after the surgery.2 These new symptoms may or may not respond to treatment with medicines.

Risks

Risks or complications following fundoplication surgery include:

  • Difficulty swallowing because the stomach is wrapped too high on the esophagus or is wrapped too tightly. This complication may be more likely to occur in people who receive fundoplication surgery using a laparoscopic surgical technique.4
  • The esophagus sliding out of the wrapped portion of the stomach so that the valve (lower esophageal sphincter) is no longer supported.
  • Heartburn that comes back.
  • Bloating and discomfort from gas buildup because the person is not able to burp.
  • Excess gas.
  • Risks of anesthesia.
  • Risks of major surgery (infection or bleeding).

 

All I can say to the above information is NO THANK YOU.  Logan has enough problems as it is.  And to do a surgical procedure for aspiration which may or may not even happen and potentially end up with a child who can’t swallow?  Not to mention the fact that some studies are showing only a 60% success rate?  And these risk are actually very minor in comparison to the major risk would be how Logan’s heart would handle such a large operation.  So needless to say,  Dr. Patel and I had a long talk about this and decided very quickly this is not the path we are ready to embark on.  So after countless hours on the phone talking and discussing and go back and forth between the pediatric general surgeon, the GI doc, Dr. Patel, the pediatrician, and myself.  We all settled on the fact that the once unanimous decision to have Logan get a G-Tube is now a resounding NO.  

  We are very lucky to have such an experienced and insightful pediatric general surgeon on our team.  He was a surgeon at CS Mott’s in Ann Arbor MI (one of the nation’s top Pediatric Heart Centers) before coming to the Cleveland Clinic and has tremendous experience with him.  He has experience with heteortaxy kids and has seen how quickly things can ‘get messy’ with them. 

A little side note here on second opinons……GET THEM.  This whole situation clearly illustrated the importance of finding a surgeon with actual experience with the type of illness you have.  I have spoken with 2 general surgeons and 3 GI docs to get a team I felt comfortable with. 

So after I had finally excepted that Logan was going to get a G-Tube I began picture him growing, and starting to sit up, and develop at a much increased pace and I started to get excited about the idea….until it was pulled out from under me. 

As Logan is quickly approaching the one year mark, I have been particularly emotional.  It is so much to take in, where we have been in this last year, and the road ahead as well.  The other issue with his 1 year mark  coming up, is that I have been avoiding calendars, timelines, charts, ect… in hopes of not having to worry about the comparison.  The comparison to where he is and where the average child is.  It is heartbreaking to see a baby almost a year old just start to have enough strength to hold his head up and for my own piece of mine I block out all the comparison charts and just accept him for him.  With his birthday looming it pulls me out of my world and into The 1 Year Old World.  The world of 1 year olds is crawling, walking, talking, waving, peek-a-boo, babbling, clapping, eating, sitting in a high chair, ect…. Logan can’t do any of those things.  In terms of physical strength Logan is a 3 month old.  This has been so tough on me that I have even considered canceling his B-Day party. 

 

I have come around though and accepted that Logan is very special and been thru a lot and it would be a shame not celebrate his life, because even though he is very small and weak, he has fought battles larger than most of us fight in our life. 

Logan’s birthday party is open to anyone in that would like to celebrate his life with us. If you are interested in coming on Logan B-Day Party which will be on Sunday June 5 please leave a comment with your email address and I will give you the details.  I look forward to meeting some of my fellow heart Mom friends that I only know thru words.

Echo Results.

 

So, yesterday we went back to see Dr. Patel.  I have been very worried about his respiratory rate  (this was how our last open heart surgery journey began afterall) so I was VERY anxious for this appointment.  First I have to start with the GREAT news.  Logan gained A POUND!!! I knew he gained weight but oh man, I didn’t realize I packed a pound on him in 2 weeks.  I don’t think Dr. Patel could even believe it.  I told him when I said I didn’t want a G-tube I DEAD serious. 

All my hard work and paid off and I have decided to do a separate post later today on exactly what I fed Logan because if it helped Logan maybe it could help another baby.  So look for that if you are also struggling weight gain!   I think that I have a pretty good food formula going!

Anyway, Dr. Patel did another echo, more for my piece of mind than anything else because he was  convinced that he wouldn’t find anything.  He was correct the echo looked perfect!  His pulmonary vein confluence (which was re-repaired during his last surgery) looked completely wide open with No narrowing.  I made him check all 4 pulmonary veins to make sure there was no stenosis there either.  There wasn’t!  His AV valve still looked good, his Bi-Directional Glenn looks great.  Dr. Patel said that he is very happy with echo. 

 

So then why does Logan have labored breathing??? Dr. Patel still thinks its from weakness.  I said ‘but he gained A POUND and his breathing has gotten worse.’  His response, ‘Well, now he has more energy to move around and do things so he is out of breath more.’

I am trying so hard to just accept this as the case.  I mean, this man has not been wrong about anything so far, and his pulse ox (oxygen saturations) are still good, and his echo looks good, ekg looks good……So. Maybe Logan is just out of breath because he is weak.  Dr. Patel thinks that if I can put another pound or so on him he will be a different baby in 2-3 weeks from now.  If I am going to be really honest, I will believe it when I see it.  I am not going to feel completely comfortable until Logan has a normal respiratory rate.  But for right now, I feel as though a couple of the 100 thousand bricks are off my shoulders.   So my goal right now is to put another pound of weight on Logan in the next couple weeks. 

(Logan eating Veggie sticks while waiting for Dr. Patel, he is a chow hound now!)

Also, this was GREAT news…..

Dr. Patel doesn’t need to see Logan back for 3 WEEKS!!! I think in Logan’s life he has only gone that long 1 time before.

Echo Results.

So the results are in and Logan’s echo looks fantastic.  The Sutureless repair on the TAPVR still looks wide open and has great blood flow.  All four pulmonary veins appear to have great blood flow.  His Bi-directional Glenn looks good, his valve is actually stenotic (too tight) now and is causing some dialation because the heart is having to work hard to get blood flow up into the atrium now.  This is the opposite of the problem he had before, which is when the valve was pulled apart.  I am not sure if this is problematic or if they made it super tight in anticipation of it loosening up. 

 

Dr. Patel still thinks that the cause of Logan’s rapid breathing is from lack of calories.  He thinks that he has no reserves and that when he exerts himself he is immediately out of breath.  I don’t know what to think anymore.  I hope this is the case, it would be an easy fix that is for sure.  Since last Thurs.  I have stuffed Logan silly with food.  I have added a 1/2 TBLS of Olive Oil to his bottles (this adds an extra 75 calories) and also to any food that I gave. He also got constant buiscuts to chew on, rice ones, barley ones, wheat ones, you name the biscuit and Logan ate it over the weekend.  This weekend of gorging actually paid off and Logan gained 6oz in 4 days. This is amazing for him.  He is now weighing in at 5.8 K or 12.7 lbs. Yay!!!! Logan. If anyone has any good baby weight gain tips please leave a comment with them.  I could use all the tips I can get.

So I guess only time is going to tell us the exact cause of his labored breathing.  Dr. Patel is going to see him back in 2 weeks. 

Oh and also….Logan is saying Momma now!!! Not sure if he associates it with me but when he is upset he says amomma…mom….mmmmooom.  It is the most precious thing EVER.  Heheheheh…we are now 0 for 2 on Dadda being our children’s first word.

Collaterals.

So after a few episodes of very heavy strained breathing and a few weeks of a lot of fussiness, Scott and I decided to buy a Pulse Ox machine for our home.  I thought that if I could just see that his oxygen levels were OK, I could relax a little bit about whatever was going on with Logan.  The Pulse Ox device  is just a device that measures ones oxygen saturation (for adults it’s the little thing they put on your finger in the hospital.)  Well, instead of making me feel better the pulse ox machine registered that Logan had oxygen saturations of 65 (FOR DAYS),  we obviously got nervous and moved his cardiologist appointment up by one week. 

So inevitably,  I was very nervous about our appointment and expecting the worse and for Dr. Patel to find something really wrong with Logan during the echo. 

Well it turned out Logan (as Dr. Patel put it) was trying to prove me wrong because not only was Logan NOT FUSSY AT ALL, during our apt.  his O2 levels WERE 75.  I couldn’t believe it.  Now 75 is fantastic but it isn’t 65 either.  The ideal oxygen saturation after a Bi-Directional Glenn Shunt would be 85, so there is something that is causing the O2 levels to be less than that.  In Logan’s case it is because he is growing collaterals (tiny little veins) that are rerouting some oxygenated blood away from his lungs.  If you think of a hose with holes in it, and trying to fill up a pool with that hose.  That is sort of what is happening.  The blood is coming out the holes/veins and taking a different path instead of going to his lungs.  Since Logan’s oxygen is 75 we aren’t going to worry about trying to, plug those holes (coil those collaterals in the cath lab)  right now, but just give his body longer to adjust.  The reason he is growing the collaterals is because there is still some high pressure in his pulmonary artery so the blood is flowing down other smaller veins (because it is easier) and the very nature of this causes they veins to get bigger and then more blood flows thru them (you get the cycle here.) 

So we just have to wait and see if these collaterals are really going desaturate him A Lot (like into the 60s) or if he will continue to hang out in the 70s.  If he stays in the 70s we will wait until his next open heart surgery to deal with collaterals.

 

Logan is still a tiny little guy.  He is weighing only 11 pounds at 7 months old.  I think that officially has him completely fallen off the charts.  He has grown in length though and is now 24 inches long (although, this is only the 2-3%) for length as well.  He is still not able to hold his head up for more than a few seconds at a time and there is no sign of any sitting up or rolling over either.  Now, cognitively he seems right on track.  He is playing with toys, trying to drink out a cup, chewing on pieces of fruit, and he has Mommy completely wrapped around his finger.  Whenever I walk in the room, people tell me he starts behaving very differently (as to get my attention.) 

I do however, worry about him getting bored having to just lay there all the time, but at this point I am not sure about physical therapy.  I don’t really want to stress him out, and part of me feels that whatever he is doing right now is what his heart can support, and that he will catch up when he is ready.  Him staying on track cognitively is more important to me anyway, so if that is where all the energy is going right now, I am happy with that. 

HAPPY NEW YEAR TO EVERYONE!!!