"Time to Prepare and Pray"

Those are the words of our cardiologist from our appointment yesterday.  Do you see why we love him?  He is very straightforward about things (so I feel like I know exactly what is going on), but he his very loving at the same time.  I feel like that is a hard thing to find in a doctor.  I am SO grateful everyday that he is the doctor caring for Ethan.  (Because I want to keep this blog public, but would like to have some anonymity, we'll call him Dr. B.)

Yesterday's appointment was good... well as good as it can be these days.  We did an echocardiogram on Ethan's heart to see what his status is now.  The pressure in his decompressing vein has actually gone down a tiny bit.  (YAY!! Prayers are working!!)  It is still not good, but it hasn't gotten worse.  At this point, we'll take that as a blessing.  He still has hypertension in his left atrium and they can still see that this is causing blood to flow backwards to his lungs, which is not good.  But, to me, this is an answer to our prayers.  And your prayers.  And all the thousands of prayers that have been offered for us and for Ethan.  My mom was saying yesterday that there are SO many people praying for us right now.  People we know and love, and also people we don't even know.  That is amazing to me.  We are so blessed.  With the way Ethan's anatomy looked yesterday, Dr. B is hopeful that Ethan will come out with 50-60% oxygen levels, which means we can have a little more time with him.  He says that we will be intervening with urgency, but it won't have to be emergent.  This is comforting to me.  This means we will have the chance to hold him and be with him for a few minutes before they take him off to the cath lab.  I think I will feel a lot better sending him to surgery if I at least have had the chance to hold him.  We aren't going to act on that "hope" though.  Obviously we will still need to be prepared for what might have to happen if he comes out sicker than we think and things need to happen right away.

This brings me to the wrench that Dr. B threw into our plans yesterday.  Ethan's birth date... this requires a little bit of a back story... After our last cardiology appointment (two weeks ago), we came home knowing that at the next one we would be talking more about delivery dates for Ethan.  After thinking about that for a couple days, we realized that it was only about a month away and we needed to know sooner, rather than later, when this was all going to happen.  I had an appointment the next day with my OB and planned to meet with Megan (our hospital coordinator) afterwards too.  I planned to ask her to see if she could at least get a delivery date nailed down for us.  So that Friday, I went to my appointment and talked with Megan.  She had already come to the conclusion that we had (that we only had about a month left before everything happened) and she had planned a meeting that afternoon with all the doctors involved (our cardiologist, my OB doctor, one of the neonatologists from the hospital, the cardiac surgeon, and the cath lab doctor (or is he a surgeon?)).  She planned on talking through scenarios with the doctors, informing them of our wishes, and having them decide on what type/date of delivery would be best for Ethan.  I was so happy when she told me about this meeting.  She is wonderful.  I asked her to please call me and let me know what date they decided on so we could plan accordingly.  She called me that afternoon and said that they had all agreed on September 23.  On that date I would be 39 weeks, so Ethan would be a good size baby to operate on.  They would induce me to deliver vaginally.  (Babies delivered via c-section typically have more respiratory problems and that is the last thing Ethan needs.)  And my job is to keep him inside me, nice and cozy, until then.  We promptly called our families and let them know that if they wanted to meet Ethan, they would need to be here that day.  Flights were booked right away.
Now here's the wrench:  At our appointment yesterday, Dr. B tells us that he thinks the best day for Ethan to come is on September 30, my actual due date.  His opinion is that one more week means one more pound on Ethan and a better chance for his ability to make it through surgeries.  (Great, but where was that opinion two weeks ago?!)  It really does make sense, and I actually feel really good about that, but it is now a logistics issue.  Today, Megan is running around and making phone calls to see if all those doctors will agree to this new plan.  She has to check with my OB, the cardiac surgeon, and the cath lab doctor to make sure they are on board.  (We already talked to the neonatologist about it, more on that later.)  So I should get a phone call from her this afternoon and then we can have all our family members change their flights.  The small risk about this plan is that I could go into labor spontaneously.  But, since it's my first baby, I feel like that is less likely to happen before my due date.  And if it does happen, we will have several hours of labor to wait through before Ethan actually makes his appearance.  And really, if the doctors thought I'd make it to the 23rd, then there is really only one week of "risk" of me going into labor spontaneously.  So if that becomes the new plan, that last week we will be avoiding spicy food, castor oil, and walking of any kind.  :)  So I would be induced for a vaginal birth, the night of the 29th.  Dr. B said I'm going to be mad at him that whole last week, thinking that we could have gotten him out a week earlier, haha.  But really, I feel like Ethan's health is far more important than my comfort level.  And currently, at 35 weeks, I'm still relatively comfortable.  (Now all you who have made it to 40+ weeks in your pregnancy can tell me how bad it gets at the end... just kidding, please don't.)

After our cardiology appointment, Megan took us over to the hospital to meet with one of the neonatologists.  He is one of the doctors who will be caring for Ethan in the NICU.  There are nine doctors on his team and while Ethan is in their care we will be in contact with one of them every day.  (Another reason why we decided on this hospital... we will not be talking to residents and fellows.  We will be talking with Ethan's actual doctors on a regular basis.)  He talked us through how Ethan's care will be handled after birth.  When I deliver Ethan there will be one or more of the neonatologists in the delivery room.  Right after he comes out they will intubate him and see if that helps his oxygen levels to go up.  This doctor has personally worked on two other cases (in his 11 years of practice) of babies with an intact atrial septum, like Ethan.  Both babies died.  He said that the problem is that the lungs fill up with fluid so fast, and there is no way to get it out.  Since Ethan has this anomalous decompressing vein, hopefully he has a little bit more of a chance.  But only time will tell.  There are so many question marks.  He also said that things change so much once the baby comes out and takes his first real breath.  After that, things can be very different from what they saw in utero.  So we will prepare and pray.

I think that is all the highlights from yesterday.  While writing this post, Megan called me and told me that everyone's on board for a September 30 delivery date.  Yay!  So that is the plan... for now.

A Harsh Reality

I don't even know where to begin...

I guess first of all, Jeff and I want to thank all of you for your support the past few weeks.  We have had so many prayers offered in our behalf and so many wonderful people who care about us and ask how we are doing everyday.  We really appreciate every kind word and positive thought you have sent our way.  It really helps us keep going.  So thank you.

Well, the past few weeks have been full of waiting.  Waiting for Boston to review our case.  Waiting for our cardiologist to call us and tell us what he's heard.  Waiting to have all the information necessary to make the next decision.  Over the past few weeks I had made calls to our cardiologist, but heard nothing back.  So Tuesday at my non-stress test (NST) I was able to visit with our hospital's "program coordinator."  (Her name is Megan and she has been so helpful for us.  She is basically a go-between for us and all the doctors and hospital staff.  She also sits in on the cardiology conference every week where they talk about cases/families like us.  She is in these meetings with cardiologists, neonatologists, the cardiac surgeons, etc. so she really knows what's going on.)  She told me that when she asked our cardiologist about Boston, he still hadn't heard anything back from them and he had been trying to get a hold of them with emails and phone calls daily.  Megan was really concerned for us because she knew this was time sensitive with me being 32 weeks along and them wanting to perform the surgery by 34 weeks.  Anyway, long story short, because she was so persistent, we were able to hear back from Boston on Friday.  I talked to our doctor over the phone Friday afternoon and get a little bit of an idea about what they thought.  He wanted to have us come in asap to get another good look at Ethan's heart and discuss our options.  We set up the appointment for Tuesday morning (yesterday).  [Side note:  I already had an ultrasound appointment later that morning and an NST that afternoon and I wasn't sure how long our cardiology appointment would be.  I called Megan to hear her thoughts about what I should do and her reaction was, "Don't worry, I'll take care of it."  See how wonderful she is?  She totally re-scheduled all my appointments for me and I didn't have to do anything.]

So, Tuesday we headed to the cardiologist.  My mom met us there to give me a ride home, since Jeff was going straight to work after the appointment.  We are so grateful that she was willing to drop everything and be there.  After talking to our doctor Friday afternoon, I kind of knew we would not be having an easy conversation and the more ears hearing the information, the better.  We had a fetal-echo that was actually only about 40 minutes long this time!  I think they were really just focusing on a few aspects of his heart this time.  The sonographer was able to take some cute pictures of Ethan for us and show us the "hair" on his head, his cute chubby cheeks, and we were even able to see him playing with his toes.  :)  He was moving around a ton... she commented that he was being a "wild man" in there.  It was so fun to see him doing so well.

After that it was time to have "the conversation."  Megan came in to see us and actually offered to stay to hear all the same information we were hearing.  That was such a blessing.  She would hear the information otherwise, but this way she heard it first-hand without me and/or the doctors having to relay everything over again.  Our cardiologist first told us that Ethan's anatomy is the same as what they saw last month.  The pressure in his decompressing vein has not gotten any worse, but it is still bad.  He still has hypertension in the left atrium, which is causing damage to his lungs.  He said that this is what is causing the most problems and what will ultimately determine how long Ethan will be able to live.  (Kind of ironic that his lungs are the real problem, not his heart.)

We talked about Boston.  Their success rate for doing this fetal intervention surgery is not good.  It's 50% at best, and they use the term "success" very loosely.  What it means to them is that they baby didn't die right away.  If the baby made it through the surgery okay, only to live a month or so longer, that was still considered "successful."  Also, in some cases they were able to make a hole in the heart and place the stent successfully, in other cases the stent did not get placed correctly, and it really didn't make a difference in the overall health of the baby.  Some babies with successful stent placements died, while other babies with un-successful stent placements lived longer.  All things considered, it really isn't a good option for us.  Jeff asked some great questions that I wasn't thinking of like, "Would it at least stop more damage from happening to the lungs between now and then?"  Our doctor says that they have tried to study that, but there is no real solid evidence that it even does that.  The hard part is that this is so rare, that there are not enough statistics to really know anything conclusive.  Boston is the #1 hospital doing this fetal intervention and their most recent study only had 9 cases to look at.  We talked about every one.  None are very hopeful.  We decided that Boston isn't for us.  It would require moving there for several months (leaving next week) while we did the intervention, made sure I didn't go into labor early, deliver there, and stay (for who knows how long) until Ethan is stable enough to come home.  It just wouldn't be the best situation for us.

So then we talked about Ethan's overall prognosis.  It is not good.  Other babies that are born with this type of anatomy and lung damage do not live long.  Their best guess is that Ethan has a 5-10% chance of living to the age of 5.  That is our best-case scenario.  Even then, his life will be filled with constant hospital stays, numerous medications, and very few "healthy" moments.  This makes me so sad for him.  I wish I could give him a better life.  Most likely we will have him for a few days? weeks? months?  We really don't know.  It really comes down to how healthy or sick he is when he is born. So, the state of his lungs and his oxygen levels.  Good oxygen levels for Ethan would be 50-60%.  At this level, they would actually consider some cath lab or surgical options.  But he may come out with only 30% oxygen levels, in which case, he would be too sick to operate on.  So the better his oxygen levels, the more time we can have with him.

Our after-birth options really come down to deciding between giving him comfort care and letting his body pass on its own, or trying to do anything and everything possible to prolong his life.  To say this is a difficult decision is an understatement.  How do you even choose?  Jeff and I have talked about our initial feelings on this decision and have discussed them with the doctors and with our families.  But please forgive me for not writing them publicly on the internet or sharing them with everyone who asks.  It's just too personal.  It's not easy.  Things can change.  Nothing is set in stone at this point and our feelings may change.  For now we are discussing it as a couple, listening closely to the Holy Ghost and constantly praying to know if what we are thinking is right for Ethan.

Our feelings at this time are very tender.  We are very sensitive.  These are the kind of conversations and decisions you never thought you'd have to think about.  Yet somehow, here we are.  This is our reality.  We know Heavenly Father performs miracles.  Last night we were talking that Ethan's decompressing vein is a miracle.  They have no idea why/how his body grew that vein, but it is his one small chance at life right now.  They are hoping that they can stent it after birth in the cath lab, if he is born with good enough oxygen levels.  But... we also know that we are a forever family, no matter what.  Jeff and I have been sealed in the temple and we know that no matter how much or how little time we get to spend with Ethan, that he will be ours forever after this life.

We understand that you may not know what to say.  I wouldn't know what to say if I was on the other side of this either.  Please know that just saying something is better than nothing.  You can't say the wrong thing.  And really, right now all we need to hear is that we are loved.  Please don't hesitate to send a note, text, call, etc.  It really helps to keep us going.  Thank you.  We love you.

A Quick Update

Just wanted to write a quick note for those who are checking the blog regularly.  I haven't posted since our last appointments because we still don't know much more.

We still haven't heard back from Boston.  The more time that passes, the less likely we are to go that route.

I will also start seeing my maternal fetal specialist twice a week for non-stress tests.  I go in this afternoon for my first one.  This will monitor the baby and check for signs of fetal distress.  I'm actually pretty excited to have some peace of mind.  Although it's a good thing I don't have a job... How would that even work?!

That's all for now.  We are feeling your prayers!  Thank you so much and keep them coming!!

Not What We Expected

Monday did not go as expected.  We planned on going to another cardiology appointment.  Then we planned on meeting with the cardiac surgeon who will perform the surgeries on our baby boy's heart.  Then we planned on meeting with the hospital family coordinator who would give us a tour of the hospital where we are planning on delivering.

Things kind of fell apart at the cardiology appointment.  We had a (nearly) 2 hour long fetal echocardiogram.  By the time it was over we should have been meeting with the surgeon.  My parents came to meet the surgeon and tour the hospital, but since they were already there they came in to meet with the cardiologist too.  I'm glad they were there to hear the news too, because Jeff and I were having a hard time.  It always helps to have multiple ears when you are hearing bad news.

This is what we learned about Baby Ethan's heart:  (You can click on this picture to make it bigger.)

1. They could actually see his left atrium today, which seems like a good thing, but it's not.  Since they've never been able to see it before, this meant that there was blood flow going to it that wasn't there before.  They learned that his pulmonary veins were actually attached to this side, so blood is going in, but there is a lot of pressure in this chamber and the blood is not able to get out as well as it should.  This hypertension in the left atrium indicates a problem.

2.  Most (94% or more) HLHS kids have another defect which is a hole between the right and left atrium.  This is called an Atrial Septal Defect (ASD).  Ethan's heart does not have this.  (Who would've thought that we'd actually WANT him to have another defect?)  If his heart had this hole it would relieve this hypertension in the left atrium and would make his life a little easier.  As I've researched this a little more, I've found that they call this an Intact Atrial Septum (IAS) or Restricted Atrial Septum (RAS).  

3.  Remember how I said before that HLHS kind of starts a chain reaction of problems in babies?  Well since Ethan's heart has no ASD, his body tried to make up for that by growing a new vein going from the left atrium to the Superior Vena Cava.  This "decompressing vein" was kind of Ethan's life line.  It should have solved the problem by draining the blood from the left atrium back into his system.  The problem is that this "decompressing vein" is obstructed and can't do it's job.  They looked closely at this vein last month and measured the pressure of blood going through it.  This month the amount of pressure has more than doubled.

4.  All of this extra blood pressure building up is also starting to cause problems for Ethan's lungs.  Because all this blood in his left atrium cannot drain fast enough, it has started to flow backwards through the pulmonary veins toward his lungs.  This can easily and quickly damage his lungs.  

5.  With all this going on inside me, they are a little bit concerned about Ethan's health inside the womb.  Before they said that he would be okay until his due date, but now they aren't so sure about that.  I will now have to be monitored more frequently to make sure the baby is not in fetal distress.  And I am now more paranoid if it's been more than 10 minutes since I've felt him move.  Great.

This unique set of problems is very rare.  HLHS itself is rare, occurring in about .04% of births.  IAS occurs in only about 1% of all HLHS cases.  And honestly, it's a very lethal combination.  The outlook is not good for our baby boy.  We have been presented with three options to chose from to try and fix this problem and give our baby boy a chance at life.  All of these three options are considered "high risk."  None of these options are good, but they are the only chance we have.  

1.  Our cardiologist recommended sending our case to Boston Children's Hospital where they have the #1 children's heart center in the U.S.  There they are performing fetal intervention for cases like ours where the baby does not have an Atrial Septal Defect.  They would stick a needle in my belly and through the baby's chest to get to his heart.  They would then be able to create a hole between the right and left atria.  This surgery is high risk to me, and to the baby.  It can also sometimes cause pre-term labor, and HLHS babies born premature have almost no chance at life.   By sending our case to Boston, our cardiologist is hoping that they can shed some light on other cases they've seen like ours and what research they have gathered.  He's a little concerned that since this surgery is still in the research phase and a baby with this specific anatomy is so rare, that they will be very enthusiastic about having us come to Boston and give it a shot.  (In his words, "If the only tool you have is a hammer, you're going to want to use it." Even if a hammer is not the best tool to use.)  He said he should hear back from them within a couple days and then he would get in contact with us to tell us what they said.

2.  The second option he presented to us is an emergency Norwood procedure.  This is the first open heart surgery that all HLHS babies have.  They typically like to wait as long as possible to do this surgery (about a week after birth) so the baby has at least a few days to gain some strength and get stable.  This would not be the case with Ethan.  If we choose this option, he would be taken immediately after birth, have an echocardiogram to see what shape his heart is in, then the surgery would be performed immediately.  This surgery includes removing the atrial septum between the left and right atria and enlarging the aorta by connecting it to the large main pulmonary artery.  This seems like a great option.  The cardiac surgeon came and visited with us and explained the whole procedure as well.  But he said in his 26 years of experience, when he has performed and emergency Norwood, the babies have not done well.  They don't recover well and typically have other complications that arise from it.

3.  Our third option is to have a different emergency surgery.  This would mean that Ethan would have two open heart surgeries within the first week of his life (this one and then the Norwood).  In this surgery they would create a whole between the left and right atria and also place a couple bands around his pulmonary veins to keep the blood flowing to his body and not as much toward his lungs.  When the surgeon talked us through the different surgery options, it seemed like this one was the "best" option.  It is still high risk, but maybe a little bit less-so than the others.

With the last two options, we are most likely going to have to have an induced labor in a very controlled setting so that "all hands are on deck."  That means I will likely be induced about a week early, so September 23.

We need your prayers.  We need your prayers so that Ethan will be safe inside me for 9 more weeks and won't go into fetal distress.  We need your prayers so that Jeff and I can be strengthened and have a clear mind to make the right decision for our sweet baby boy.  We need your prayers so that we can have hope and faith that whatever outcome is in our future we can be okay with.  We need lots of encouragement and kind words.  It's hard to describe what this feels like for first-time parents.  We love you all and are go grateful for all the support we've gotten from you so far.  Thank you.  

June Doctor Appointments

Each month we visit two different doctors: a maternal fetal specialist and a pediatric cardiologist.  We had our two appointments last week.  Here's what we learned. 

Maternal Fetal Specialist

He is basically a high risk OB.  I went to this appointment with my mom so Jeff wouldn't have to take time off work, but I needed someone there in case something was not normal.  At the appointment they did an ultrasound (if you are counting, that would be #7 this pregnancy) to make sure the baby is growing normally.  Afterwards we talked to the doctor and asked a few questions.  To be honest, we really aren't impressed with this doctor.  I'm really not sure he knows much about HLHS.  Some of the things he's said are not really true and we kind of get the impression that he really thinks we should terminate the pregnancy.  He never actually asks us what we want to do, but he says things like, "and then we need to decide what you guys want to do... how far you want to try and go with this.". He kind of just has a "gloom and doom" kind of attitude about it.  I don't think we will be seeing him again.  I have to switch over to Jeff's insurance this month because I lose mine since I'm not going back to teaching.  We'll see what next month brings.  We did learn that the baby is growing normally (yay!) and he said that there isn't any reason to induce labor early, so I will probably deliver around my actual due date.  The hospital situation is still up in the air due to the switch in insurance. 

Pediatric Cardiologist

We LOVE him!  If you ever find yourself in need of a pediatric cardiologist (and I hope you never do), let me know.  I can recommend the best one to you.  He just has the best attitude about everything.  He makes us feel like, "It's okay.  We've done this before.  You don't need to worry about things."  Not that we know everything will turn out the way we want, but worrying about things that we can't control is useless.  We'll deal with the complications when they come, but for now things look okay.  

The technician did another fetal echocardiogram (basically a fancier ultrasound, so #8).  As she was looking at the baby we were asking her a few questions about TAPVR and Heterotaxy, but she had to stop us because she said she didn't know about all that.  She was impressed with the knowledge that Jeff and I have gained already about this.  That reassured us that we are on the right track with what we have learned so far.  

After the echo, we met with our cardiologist.  He sat down and answered every single question we had.  Some of the things we talked about were:

-The baby's anatomy looks the same, which is good.  I was really worried that another complication was going to pop up.

-The TAPVR is probably unobstructed, but they are going to keep watching it.  These are the veins that go from his lungs to his heart.  They are not connected in the right place, so eventually they will need to be fixed.  They are measuring the pressure (isn't that crazy... that they can measure the pressure of the veins in utero?!) to see if it goes up or down before he is born.  That will determine when they will need to address these veins.  

-Our baby does not have Heterotaxy.  We've heard that Heterotaxy is a common complication with the diagnosis our baby has.  That is where all of his internal organs are jumbled up and not positioned correctly.  The technician said it's kind of like a chain reaction, when one thing goes wrong, more things follow.  But the cardiologist told us that he sees the liver, stomach, etc. in the correct positions, so yay!

-We have narrowed our hospital options down to two.  

     *Hospital #1 - located downtown (45-60 min. away from  home), heart surgeon has worked there for 30+ years and is very good, has good accommodations for me and for the baby

     *Hospital #2 - located about 4 miles away (10 min. from home), is an academic hospital so we would be working with a lot of residents which may add frustration to an already high-stress situation, has ok accommodations for me and great for the baby, this pediatric cardiology unit is ranked #12 in the nation

Obviously there are pros and cons to both.  We've been assured that either place we go will have the same outcome for our baby.  The 2nd hospital is actually a Primary Children's hospital, but they have added on a section where women can deliver babies there.  We've been warned though, that if there was some sort of complication with delivery, there are only pediatricians there to assist.  Our doctor said that 99% of the time it is fine, but he worries about the 1% of the time when it's not.  

Next month, in conjunction with our cardiology appointment we will visit each hospital, meet the surgeons, and tour the facilities.  I hope that helps us make our decision.  

-We also asked our cardiologist about this case study at the Mayo Clinic.  He actually did his residency there and said it is an amazing hospital.  But he also said that he needed more information about this particular study before he would recommend it to us.  He said that one of his reservations about it is that it would be very difficult to determine if it is effective.  Some HLHS babies have very strong heart beats, and others have weaker heart beats.  He wants to know more about how they would actually measure if the stem cells did any good, or not.  He doesn't want us to do it just to do it.  He said that there isn't really any added risk, but his gut feeling is that it probably won't do any good.  He has emailed the hospital to get more information and we'll talk about it more once he hears back from them.  

-We also discussed more about the "inter-stage" between the baby's first and second surgeries (when the baby is 0-6 months old).  This is the most fragile time for HLHS babies and we've heard lots of information about how to keep the baby healthy during this time.  Our doctor is a member of the same church as us, so he confirmed that we should not be taking him to church during this time or any other public place.  Since he will probably be coming home from the hospital right when flu season starts he will need monthly injections to protect him from RSV.  I forget what he called them, but basically it's like a monthly flu shot, but it costs $3000!!  Good thing we have insurance that will pay for it!

I think that is everything.  Probably pretty boring for most of you, but I wanted to write it all down before I forgot what we talked about.  I'll update more after we visit the hospitals on July 22.

Sisters By Heart Care Package

In the past month I have done more googling of HLHS than anything else.  It has been so helpful to read other people's experiences with this.  Every situation is so different, and every family has different experiences, but it is so helpful to know what we may expect.  No one writes the book of "What to Expect When You're Expecting a Baby With HLHS."  But I guess that's because things can go so many different ways.

Anyway, one wonderful thing I found through all my internet research (What would I do without the internet??) is the website Sisters By Heart.  They are a group of HLHS heart moms who keep a blog and send care packages to support families with HLHS babies.  I sent in a request for a care package a few weeks ago and didn't tell Jeff.  I wanted to surprise him with something thoughtful, I guess.  The package came on Friday and it was so much fun to open.  They did a wonderful thing and put a small note on each item explaining why it may be helpful in the future.  We loved it.  It was wonderful!



Here's a list of what was inside...

• Aden + Anais Swaddle Blankets - I've heard amazing things about these.  I'm so excited to start using them!
• A homemade flannel blanket - Super cute and very thoughtful.
• Hand sanitizer and moisturizer - Necessary to keep germs away.
• Car seat sign - It says, "Please wash your hands before touching mine."  This can be used once we finally get to take him out of the hospital and/or house.  
• Sisters By Heart Pen
• a heart ring - for mom
• a deck of cards - for dad to keep busy during long hours in the hospital
• ZoLi bottles - I don't know much about these, but apparetly they are environmentally friendly.  
• canvas bag - A huge, nice bag that everything came in.
• Sisters By Heart Notebook
• Baby Legs - I've heard these are great for keeping your baby "dressed" in the hospital, but not interfering with all the cords, tubes, and diaper changes. 
• Pacifier
• EZ AXS Hospital Gown - This has snaps in front and on each shoulder which can be opened to allow easy access to the baby's chest.
• Zutano Side-Snap Onesie - I've heard these are great because the snaps allow tubes to easily fit through (in case he has to come home on oxygen or a feeding tube) and the snaps are on the sides instead of in front so it does not irritate chest scars.  Plus, this one is super cute!

Thanks so much Sisters By Heart!!!

Where Do We Go From Here?

The Pregnancy

According to our doctors this pregnancy should go normally.  I say should because if there is an exception to that rule, I now feel like it's me.  With HLHS occurring in approximately 4 in 10,000 births, Jeff and I are contemplating buying a lottery ticket.  Instead of seeing a normal OB doctor, I now see a Maternal Fetal Specialist for my appointments.  (Hello 45 minute drive and $50 co-pay.  ugh...)  I have my second appointment with him this week.  I'm not sure exactly what will happen at each appointment, but I assume I will be doing an ultrasound at each appointment with him.  When I called the office today to see what they would be doing at my upcoming appointment, the nurse (or receptionist?) said they would be "looking at the heart."  I had to laugh and hold back a sarcastic comment.  So I guess I'll update after that appointment.

We also are very fortunate to get to visit with a Pediatric Cardiologist throughout my pregnancy.  I consider this such a blessing because he is awesome.  And he can answer so many questions for us.  He is the one who cares for these babies after they are born, so he knows a little bit more about what our future holds.  At our last appointment with him, he told us that our baby boy has another anomaly on top of HLHS, which is Total Anomalous Pulmonary Venous Return (TAPVR).  This means that his pulmonary veins, which connect the lungs to the heart are not formed properly.  At my next appointment with him he will be able to determine if these veins are obstructed or unobstructed, which will determine when our baby will need his first surgery.  

Delivery

We don't know much about this day.  When I asked the doctors about it at my first appointments, they said we could discuss it more in the future.  I've heard that during week 25 of my pregnancy is the best time to see the baby's heart and what we're dealing with.  Right now I am 24 weeks, so hopefully after next week we'll have some more answers.

What we do know is that heart babies recover better from vaginal births as opposed to c-section births.  So I will hopefully be able to have a normal birth.  We don't know yet where it will be or when.  From what I've read about other people's experiences with this, I would assume that I will be induced so that all the right people are in all the right places when he is born.  Depending on his TAPVR, he may need surgery immediately, but they are hoping to wait until day 7 or so if possible.  He will go on medication right away to keep his ductus arteriosus open.  This is a hole in every infant's heart that helps with circulation in the womb. After babies are born this hole closes naturally, but keeping this hole open will keep our baby boy alive.  (The more I learn about this whole thing, the more amazed I am that someone knew all this and figured out how to make it work!)

So until delivery day I am a ticking time bomb.  Just counting down the days until our life changes in a huge way!

After Delivery

After the baby is born he will be in the hospital for a while.  His first open heart surgery (the Norwood) will take place within the first week.  After that, he will be in the hospital recovering and learning to eat.  This can last anywhere from 4-8 weeks.  Some babies are put on feeding tubes (in fact, that used to be the norm), but our cardiologist said that we will really try and avoid that as much as possible.  It causes more problems down the road.  He said that kids on feeding tubes will often lose their sucking reflex and the ability to close off their wind pipe when eating.  So we want to avoid that by giving him a binky as much as possible and trying to nurse him right away, even if it's just for practice.

Going Home

This is what we are aspiring to!  Not every HLHS baby makes it home.  I know we need to prepare ourselves for that realization, but I am also so hopeful that we will make it this far.

From what we've heard, this is the most fragile time in these babies lives.  From this point until his second open heart surgery (the Glenn) which should take place 4-6 months after the baby is born, we will be the super-paranoid parents.  But for good reason.  A simple cold can put him back in the hospital and cause numerous complications including death.  From what we've heard of other's experiences we can expect that he will not be allowed in public (church, grocery store, park, etc.).  We have also heard that anytime we are in public, after we get home we should change our clothes and wash our hands before interacting with the baby.  I've heard others say that they are to limit the number of times they open their front door and be very, very cautious of how many visitors they allow inside.  And we will be doing all of this from November-February... right in the heart of cold and flu season.  Yay.

According to our cardiologist, our baby boy should not have to be on oxygen or on a feeding tube when he comes home.  But I've heard from many others that there is a good chance he might be.  Especially because we are at such a high altitude.  We have heard that we will be monitoring oxygen levels constantly and weight.  We will need a hospital-grade scale that can measure super small increments to see if the baby is gaining or losing even a tiny amount of weight.  As scary as it sounds, our doctors have assured us that all of this will give us peace of mind.  We will know exactly how he is doing and exactly when something is not quite right.  I guess that's a good thing.  After his second surgery, life should be a bit more stable.

The third open heart surgery (the Fontan) will take place anywhere between 3-5 years old.  After this surgery, life should go on "normally."  It's very difficult to say what life will be like after this point, but other HLHS kids that I've read about are doing very well.  They are able to run around like normal kids and play sports with their friends.  They may tire more easily than their peers, but they don't have to be afraid to push themselves.  They don't have to live in a bubble.  We are hoping for the best for our baby boy's future.