Category: AV Valve repair

I wear red for Logan.

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

I wear red for Logan.

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

I wear red for William.

I wear red for Noah.

I wear red for Elise.

I wear red for Pierce.

I wear red Ava.

I wear red for Rita.

I wear red for Jayson.

I wear red for EJ.

I wear red for Brookyn.

I wear red for Savannah.

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

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

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

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

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

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

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

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

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

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

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

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

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

That is why,

I wear red for Logan.

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Countdown to Surgery- The Fontan

I am a disaster.   We are leaving for Boston this weekend.  I would give anything in the world not to go back.  This life is so very unfair.  I can’t believe he has to go thru this again.  We have decided to rent an apartment in case we are there for a long period of time (if not…we’ll we will break the lease) but we have decided to take our other Son and our nanny because we think its important to keep the family together. We are leaving this Sun for Boston stopping along the way and finishing the journey on Monday.  The cardiac cath will be done late in the week and surgery is scheduled the following Wednesday. 

We had our last cardio appointment today with Dr. Patel.  A very good friend of mine who also has a child that has the odds stacked against him as well, gave us their good luck onesie!  J wore this onesie thru 3 surgeries and he is doing great.  I was honored to have his shirt passed down to us. 

He did a very thorough echo and everything looked as good as it can be. His Glenn and SVC look wide open with good flow.  All this pulmonary veins appear to have good flow.  His AV valve although slightly stenotic is working well with very minimal leakage.  His pulmonary artery in color appeared to have even grown a bit which is very reassuring.  The pulmonary artery is very important because the Fontan surgery is going to connect the IVC (Inferior Vena Cava) to the lower half of the body making pumping oxygen to the lower half much easier for Logan.  The Glenn connected the SVC (superior vena cava) to the PAs and once we have the IVC connected to he will have a Total Cavopulmonary Connection or a Fontan Circulation.

This is a very basic drawing of a Fontan that is done on a child with Hypoplastic Left Heart Syndrome.  If you take away the right 2 chambers from this drawing you would see what Logan has.  The Fontan is the “Tube Outside the heart” that connects the IVC. 

We had a much smoother appointment this week.  For one thing, Logan is very into counting right now albeit every number is ‘six’.  When he learned that EKG leads could be counted he decided they weren’t half bad…  “six, Six, Siiiix,  Siiiiiiix!” 

He weighed in at 9.7 K or 21 lbs and 33 inches in length.  Right now he is wearing 12-18 month tops and 6-12 months bottoms, well, and then 3-6 months socks and shoes.  Just to give you an idea about his size.

He is saying very random words right now….he says, ‘six’, ‘eat’, ‘hot’, ‘nuts’, and ‘hi.

He is really starting to enjoy Ethan.  They play together all the time and love taking baths together.

You can see clearly the oxygen difference between the two when they are together in the tube.

 

Logie is loves chasing balls.  He throws them, kicks them and then chases them all over the house and yard.

 

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I just want to thank everyone for their support thru all of this.  I literally couldn’t go thru this without it.  I know that this will not be the end for Logan.  He is going to need valve replacements, pacemakers, and eventually a heart transplant.  The transplant could be in 6 months, 6 years, or 16 years, we have no idea.  Living like this is an incredible blessing and curse at the same time. A curse for the obvious reasons but a blessing because it has given us so much clarity.  Clarity on what really matters.  I don’t care about fancy preschools, where he falls on arbitrary charts compared to his peers.  I don’t care about college and if Logan will get a high paying job.  No.  All I care is that both my boys are happy, healthy as they can be, and have a good quality of life. Nothing else matters. 

Life is about this moment right here.  As long as we have each other, we have everything.

While we are in the hospital I will update his Facebook group much more often than this blog.  If you would like to join please look up Heterotaxy Syndrome- Logan Spyker on FB.  I can quickly post updates there from my phone.

Surgery Time

So, I have not updated in a long time. Well, actually about 6 weeks.  I have been spending all of my free time in organizing the new Heterotaxy Network website and forums, and trying to just decompress a little and enjoy the non-surgical months a bit. 

I found out that Logan needs to have his next open heart surgery, the Fontan, next month and I feel as though someone has just kicked me in the gut.  I knew it was coming and we have had many talks about being this spring, but to “next month” was very hard to hear.  So hard to hear and process I have needed some time before I could even discuss it. 

Logan needs to have his fontan surgery next month for a number of reasons.  His oxygen sats are mid 70’s and having this low of oxygen levels does not give him much buffer in case of illness.  Currently, his top half of his body via the superior vena cava is better oxygenated, but his lower half via the inferior vena cava in not connected and is not oxygenating well.  This is important for several reasons; 1. being that having his superior and inferior vena cava’s connected to his oxygen circulation will greatly improve his over all oxygen levels, and because when the inferior vena cava (IVC) is connected in, blood will begin to travel thru his liver.  Liver blood is very important for his hemodynamic (blood flow) stability because it has been found that there is a unique function that the liver play in the blood by putting special enzymes in it that help regulate your body’s ability to stop the growth of collateral veins. 

Everyone has heard of those stories of a man or women having a blocked artery only to find out that the body actually compensated and grew a new path around the obstruction.  That new path is a collateral vessel.  In those situations collateral vessels are beneficial to the heart.  But how does the body know when to grow collaterals and when not too?  Well, that is not completely understood.  What is  known is that there is a special role the “liver blood” plays in this process which is it stops your body from just growing the “new paths” continuously. 

For heterotaxy kids this type of “checks and balances” is essential because heterotaxy kids (for an unknown reason) tend to grow these “new paths” much quicker.  Again there are some instances where they could help your heart, but more times than not these news paths end up taking oxygenated blood away from the lungs.  The sooner that we can get his blood circulating thru his liver, the sooner he will get this “hepatic factor” into circulation. 

Lastly, the sooner we can get his bottom half into circulation his heart won’t have to work as hard.  When his heart isn’t working so hard there is a better shot at keeping his AV valve functioning well for a longer period of time.  I always knew that the repair that Dr. Del Nido on Logan’s heart was amazing, but the more I learn the more I realize how spectacular the repair actually is.  When Logan went in to have his first AV valve surgery his valve was a 4+ Severely regurgitating, and just barely functioning.  Currently, 12 months after his last AV valve repair (he has had 2) there is ABSOLUTELY NO REGURGITATION.  NONE. PERIOD. I am saying this in all caps because I want to emphasize this point.  I have heard many cardiologists and surgeons say that it is impossible to fix these valves. That no one (worldwide) is having luck repairing these AV valves.  Well, I just want to shout it from the roof tops.  My son is living proof that Dr. Del Nido at Boston Children’s Hospital is doing what the rest of the world is claiming is impossible.  He is repairing these valves, and not just to a manageable level, he is repairing them to a level of ABSOLUTE ZERO regurgitation.  I am even temped to post his echo online because so many doctors do not believe it.  If you have a child with an “unrepairable” AV valve, please seek the opinion of Dr. Del Nido at CHB. 

Anyway, sorry for the rant but I am so sick of hearing that it isn’t possible to fix them. It is.  But please…don’t think that just because Dr. Del Nido can do this repair, that any surgeon can if they just tried.  This is not the case.  This is a very specialized surgery that should be done by someone with  experience. 

Beyond the fact that Logan will need to have open heart surgery next month….he is doing fantastic.  He is so happy.  Seriously, this kid is SO very happy.  He is now 20 pounds (9.1 kilo) and is 30 inches in length.  He is over most of his trauma from the last surgery, and is willing to let people hold him, and his is willing to touch and explore new things. He isn’t terrified of every sound and movement anymore.  He is just loving life….which is one of the hardest things about this next surgery.  He was so traumatized for so long…the thought of him going back to being scared of everyone and everything is utterly heartbreaking.  Honestly, I have to just end this post right now, because just forcing myself to write this post is making me feel sick.  I did however, want to update everyone.  I will make an effort to do so more often, it was just nice to take a break from writing about all of this. 

Logan is not on the surgical schedule yet, but most likely it will be the 2-3 week of April.

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.

Post Op Results.

First, I should say that Monday night Logan started to feel better.  It was the first night that we saw symptoms of the flu starting to reside.  Tues. morning he was back to his smiley self.  This was a HUGE relief to me because I was starting to wonder if he just wasn’t going to be himself again. 

Tuesday after we went to Dr. Patel office to for a post op. check up and echo.

 

It was the best appointment I think he has ever had.  Dr. Patel said his hemodynamics (blood flow) look GREAT!  He said the surgery made a huge difference and his Pulmonary arteries look good his blood flow thru the Glenn looks even better, his ventricular function looks great, his valve is greatly improved.  He said he looks fantastic.  I felt a huge sigh of relief…….for about 10 seconds….. and then I wanted to know why scar tissue won’t grow at the site that Dr. Del Nido just did surgery on.  The response was what I expected which is that scare tissue can grow there and we could be in the exact same spot all over again. Although, this isn’t true the situation would be worse because the scare tissue would now be even closer to the pulmonary veins which would be the situation that we have been trying to avoid since Logan was 18 weeks in utero.  Dr. Patel said that he is optimistic that this won’t be the case but we need to keep our fingers crossed. 

Logan weighed in at 5.5 Kilo or 12.2 pounds.  Pretty tiny for a 9 month old.  It was so funny because this time when we were in Boston everyone kept commenting on how big he got.  I was couldn’t believe people were saying that.  When we were there at 4.5 months he was 5 k and now he is 5.5 k.  Not much difference if you ask me although he is longer.  And when you think about it 1 pound of weight gain in 4 months is pretty sad.  Dr. Patel said that he really thinks that Logan will start catching up now.  He should have the energy to start eating a bit more (although heterotaxy kids tend to have smaller stomachs than normal so he may never have normal) and once he gets more calories he may have energy to start doing baby things like holding his head up, sitting up, ect…

Yes, I did say that Logan at just 9 months can’t even hold his head up.  I feel much relief to see him holding his head up. 

I have to say that this last open heart surgery really shook me up.  It has created an uneasiness in me that is really hard to shake.  Its one thing when I know its coming and I can prepare and emotionally for all the possibilities but having it sprung on me out of the blue was just devastating.  And maybe now I am just starting to process what all really happened.  Now I feel like I have to hold on to that rug really tight because I can’t handle someone pulling out from under me again right now.

I am also very much looking forward to the spring and summer when I can relax a little about germs and Logan getting sick.