All you ever wanted to know about hearts…

I have written and deleted this post several times… mostly because it gets too long!  Andrew is scheduled for heart surgery tomorrow (Monday, July 11th), 7:30am at Phoenix Children’s Hospital (PCH).  The surgical procedure is called a Fontan and is the 3rd stage of Andrew’s “repair”.  Repair is not quite the right word because they can not fix Andrew’s heart.  Instead, they reroute his circulatory system to work with Andrew’s special heart.  To fully understand what will happen tomorrow (plus we have had a lot of questions), here is a recap of Andrew’s defect and past surgeries.  To begin (since for some of us, me included, high school biology was a long time ago!), here is how a normal heart works.

Andrew’s heart defect is called Hypoplastic Left Heart Syndrome (HLHS).  It is a fairly rare, but complex congenital heart defect (CHD).  It was discovered at our 20 week ultrasound (why you should always make sure that all 4 chambers of the heart can be seen during an ultrasound) and was not the reason that Andrew was a preemie (that was my own health issue, poor guy!).  Here is a great description of Andrew’s defect.

It took Andrew two surgeries to get through the first stage and showed us that each kid has their own path on the journey.  We have never done anything at the age that is recommended.  Andrew’s Norwood with Sano Modification was performed when he was 26 days old (usually performed by the time that they are a week old).  However, due to Andrew’s size (less than 3lbs) and a MRSA infection it was delayed.  The Sano was done instead of BT shunt because he was too small for the smallest BT shunt!  His second surgery (at 3 months old) replaced a clotted Sano (scary story) with a BT shunt.  Here is a great graphic that explains HLHS and the Norwood (doesn’t include the Sano, only the BT shunt).

It also took Andrew 2 surgeries to get through the second stage.  The first surgery (actually his 3rd surgery at 8 months old) was to fix a blocked left pulmonary artery (Unifocalization of the LPA).  A necessary step in order to proceed but has left lingering concerns that his left pulmonary artery is small and may narrow in the future (darn scar tissue!).  The next surgery was the actual second stage surgery, the Bidirectional Glenn or Hemi-Fontan and was done when Andrew was 11 months.  Here is a great explanation of this surgery.

Now we find ourselves at the Fontan, the third stage of the repair for HLHS.  This will give Andrew the closed loop circulation he needs to grow and thrive.  Here is some information on the Fontan and single ventricle hearts in general.  Here is also an animation look at how Andrew’s heart will function after surgery.  It walks through all 3 stages.  The last one is what Andrew will have done tomorrow.

Overall, the prognosis is good.  For the first time, Andrew is not considered a high risk going into surgery.  Mortality rates are less than 5% for this surgery.  There are some complications that Fontan kids can develop and he will be watched closely for these over the coming days and weeks.  The only additional concerns are with his small left pulmonary artery and narrowing in a small artery that comes off of his aorta.  This is the best scenario we have ever had going into surgery!

Here are the rest of the details.  We will check in at PCH at 5:30am.  Surgery is scheduled at 7:30am and is expected to be 4-6 hours.  One of the longest parts of the surgery is getting through all of the scar tissue.  Dr. Pearl does not have to stop Andrew’s heart, but he will be on a heart-lung bypass machine.  After surgery, we will be in the brand new CVICU on the 5th floor.  Typical hospital stay is 8-14 days but depends on how well Andrew adjusts to his new circulation.  We will be updating the blog and on Twitter (see the sidebar).  Thank you for all of the warm wishes and prayers.  They are greatly appreciated!

More details to the plan

Well, we have more of a definite plan for Andrew’s upcoming Fontan surgery. We actually left the hospital with this information but I needed to sit with it for a couple of days first before announcing.

Andrew’s surgery is scheduled for Monday, July 11 at Phoenix Children’s Hospital. The dust from the move into the new building and the merger with St. Joseph’s Hospital should have settled by then. There are a lot of pieces to put into place so I was so glad when Kristen, the nurse practitioner for Andrew’s surgeon stopped by our recovery room to set dates (thanks to a call from Andrew’s cardiologist who knows my need to plan!). There is a lot of arranging to do before July hits…

We will see Dr. Pearl, Andrew’s surgeon, on Friday, June 3. After that, we should have a good idea of what he will need to do surgically. We will then see the cardiologist on Monday, June 13 and Andrew’s pediatrician for his 4 year old well child check sometime in there as well. The good news is that we will have the whole month of May without a doctor appointment (knock on wood!).

We will keep everyone updated as we move forward. Hope everyone has a wonderful Easter!

Cath Lab Day

We are currently in recovery with Andrew from his trip to the Cath Lab at Phoenix Children’s Hospital. It was an early morning with the procedure starting at 7:30am and check in at 5:30am. Andrew flipped out when the doctors came by to talk. They gave him a dose of Versed to relax him. It made him pretty loopy for awhile. He was fine after that until we laid him on the table in the Cath Lab and they put the mask on him. Once he was asleep, we kissed him and went to sit in the waiting area. By 8:00, Andrew was tucked into the Cath Lab and we had made our usual trip to the cafeteria for breakfast and coffee.

Andrew was finished about 9:30am. The results are great! He has had issues with his left pulmonary artery and they are always fearful that it will narrow again. There was no narrowing, but it is smaller than the right. However, this shouldn’t be a problem. The squeeze of his heart is good and the blood pressure into his lungs is great! There were no extra vessels (collaterals) to coil and everything looked great. The only other issue is that one of the vessels that comes off of the aorta and goes to the right arm was narrow. The point where it narrows is where Andrew once had a shunt. But this is not an issue for the upcoming Fontan. The only thing that it will affect is that his blood pressure could be lower in that arm. Good to know for future use…

Andrew is sleeping off his 6 hours of laying flat with his leg straight. He has had some good drugs (Zofran, Demeral and Dex) and is resting comfortably. We should be headed home by 4:00 this afternoon. Good thing I put dinner in the crock pot this morning!

This was a big step in our journey towards Andrew’s next heart surgery called a Fontan. Our next step is to meet with Andrew’s surgeon, Dr. Pearl. He will then fill us in on what he feels is best (there are a couple of options) and schedule a surgery date for hopefully July.

Thank you to everyone who has kept us in prayer today. It is rare that Andrew has an uneventful cath lab result. He usually likes to throw in a surprise or two. So glad that everything is looking good!

Slices of Normal… Almost…

On this HLHS journey we are on, Andrew continually reminds us that he is also a 4 year old little boy.  Some slices of our “normal” life…

Andrew loves to play on our family computer. He has gotten quite good at it. However, he still needs practice to not fall out of the chair onto the tile floor

At the Mended Little Hearts Family Picnic, Andrew's favorite activity was the squirt guns.

Andrew's favorite target quickly became his cardiologist. Dr. Lindblade was such a good sport. Probably helped that it was 100 degrees that day!

 

Andrew had the opportunity to learn archery at Bumble Bee Ranch. Thanks Hope Kids!

 Since life has been so normal for the past several months and it is easy to move his heart stuff to the back of our minds.  It is never very far… I check his color when he is active, he takes Enalapril twice a day and a baby aspirin once a day, is on a special high calorie diet, has numerous doctor appointments, etc.  However, these have been so incorporated into our life, that they are now normal.

However, the heart stuff is crashing the party.  We have always known that he would need surgery when he was 3 or 4 and that time has arrived.  The steps had been laid out and we have been marking them off as they come and go.  However, now is the time for a pretty big step, the cath lab (similar process to angioplasty, except they only look around).  On Wednesday, April 20, Andrew has a date in the cath lab at Phoenix Children’s Hospital.  His procedure is scheduled at 7:30am that morning.  Since they are only having a look around, there is a high probability that we will be home for dinner (as long as the don’t have to fix anything).  It will be a long day, but should provide some useful information as we head towards his Fontan this summer.

It would be a lie to say that I am not nervous.  Anytime Andrew has to have anesthesia it is a little scary.  He always does well with this procedure (this will be #5) and it is not as invasive as surgery.  However, I am feeling that this is when that proverbial “other shoe” will drop.  Andrew likes to throw curve balls into plans and this would be a prime time for him to do it.  I am hoping that I am being nervous for no reason, but will still be prepared.  We will be packed as if we are spending the night.  It is an automatic jinx to go into a procedure not prepared to stay the night!

Please keep Andrew in prayer on Wednesday.  He is old enough to understand that he doesn’t like this kind of stuff, but not old enough to understand why it is necessary.  He will have to lay flat and still for 6 hours after the procedure in the recovery area.  It makes for a very long day for a normally very active little boy.  As always, we appreciate everyones support.  Thank you!

Where we are and where we are going

It has been way to long since my last Andrew update.  So first up is where we are now…

Andrew looking sweet.

Andrew is doing really well.  He is in special ed preschool and LOVES it.  His highlight of the day is riding the school bus.  We just had his IEP review and he hit most of his goals.  So now we have a whole new set.  His vocabulary has exploded in the last 9 months and he talks in full sentences.  He is very direct and precise in his speech, especially when he thinks we are not paying enough attention .  His motor skills and core strength are also getting better.  He still has some strength issues, especially when climbing stairs, but all in all is starting to really catch up on his milestones.  He will continue at EMECEC (special ed preschool) in the fall, but most likely in a mixed classroom.  He is in a self-contained class right now (all special ed kids) but next year will be with peers who are regular preschoolers.  Next spring we will make a decision about kindergarten (WOW!?!).

Healthwise, he had a good winter.  After our little bout with pneumonia for Halloween, he has been amazingly healthy.  They put him on Flowvent (inhaler) to help with his wheezing.  Andrew is still the best weather forecaster… everytime our weather would turn from nice to stormy, he would get a gunky cough and wheeze.  Luckily, we seem to be done with the stormy weather. 

We saw Andrew’s cardiologist today for a regular follow-up visit.  He wanted to take a look at Andrew, check on his weight and talk about the next surgery.  Andrew did really great at the appointment and was our first one ever that he did not fuss about anything.  Such a big boy!!!  No echo, just EKG, blood pressure and sats.  He now weighs 25.4 lbs and is just over 3 ft tall.  His blood pressures were all good and his EKG was great.  The only issue was his sats (oxygen levels).  We couldn’t get a reading above 80% in the office, which pretty much matches what we are seeing at home.  He wants to stay between 75% and 80% which is lower than the 85% he used to be and where they would like him.

Andrew sitting for his EKG

The issue of the sats leads into where we are going.  We have been watching his sats slowly drift down.  Over the last few weeks, he is very rarely above 79% and when he is running around will drop to the low to mid 60s (don’t recommend checking active HLHS kids when they are running!).  When he is in the 60s, his sats will recover after sitting for a few minutes.  He also is getting out of breath a lot more.  Just climbing up on our bed has him huffing and puffing.

All of these are signs that his body needs the next stage, the Fontan.  I will post more about the nature of this surgery in another post.  We have always known that this was coming, however, it is surreal to have it right in front of us.  We have decided that the ideal time for our family to have this surgery is in July.  So the next few months will be busy.  We have to see ENTin April to follow up about Andrew’s paralyzed vocal cord so they have a baseline before surgery (just in case there is an issue).  Then we will have a date with the Cath Lab in early May to make sure that Andrew is a candidate for the Fontan (his pulmonary arteries are of particular concern).  After that, we will meet with Dr. Pearl, Andrew’s surgeon in late May.  We will head back to cardiology on June 13 to make final plans for surgery sometime in July.  Wow!!! What a schedule!

This is the plan for now… as I get dates and we see doctors, I will post.  I have a feeling that as we move through the coming weeks and months, it will feel a little like waiting for the other shoe to drop.  Andrew always has his own plan and his own way of doing things.  We will just have to wait and see what comes.  For now we will just continue to enjoy Andrew and continue to get him to gain weight.  As seen below, this should be a fairly easy task!

Andrew eating spaghetti... notice the lack of clothing and glasses!

Thank you for all of the ongoing thoughts and prayers… they are greatly appreciated!

A Plan of Action

Wow!  I have severely neglected Andrew’s blog.  Mostly because things have been so good.  So good, that we have been really busy with all things work and kids.  Andrew has been well other than another run in with that strange pneumonia on Halloween.  This time it landed us an overnight hospital stay, mostly because he spiked the fever (103) and dropped his sats (down below 60%) on a Sunday morning.  Our only option was the ER at Phoenix Children’s and that usually means a hospital stay.

Our only other issue has been Andrew’s very stunningly slow weight gain.  The kid only weighs 23 1/2 lbs at 3 1/2 years of age.  He has been put on a 1300 calorie/day diet and most days he is closer to 1500.  All this seems to do is give him more energy to run around.  The GI doc seems perplexed on his lack of weight gain.  However, we know what it is… constant motion!  Combine that with only 1/2 of a heart and you get a very high metabolism.  Anyway, they have been talking about placing a feeding tube in order for us to feed him at night.  We are not impressed or sold on the idea.

The issue with weight gain is that Andrew needs to have his Fontan (3rd stage surgery) soon, perferably before he outgrows his current anatomy and becomes ill.  And for the Fontan, they would like him to be 15 kg (about 33lbs), 12 kg (26lbs) minimum.  GI is afraid that we will get backed into a corner where he absolutely is in immediate need for surgery and not big enough.  While I can understand this point of view, we don’t agree with placing a feeding tube in a kid that can eat and drink over 1300 calories per day just fine.

We decided to let Andrew’s cardiologist make the call.  If he felt like it was important, then we would consider doing it.  Andrew had his 6 month check up on Monday, January 10.  His echo looked great… no problems but they did notice a slight “turbulence” in his aortic arch.  Dr. L is not sure what it is, but is pretty sure that Andrew has had it for awhile (since last surgery) and that it is the source of the very loud murmur.  Anyway, a lot of time was spent discussing Andrew’s immediate future, necessary surgery (Fontan) and his weight issues.

So we now have a plan!  We will go back to see cardiology in 3 months (April).  He will listen to Andrew and check on his sats and weight gain.  As long as he is gaining some weight and holding his sats (low 80s), we will schedule a cath lab visit (necessary procedure prior to surgery) in May or early June.  If all checks out in the cath lab, then his surgery will be scheduled for sometime in July.  And the best news is that cardiology and surgery are ok with Andrew being on the small side for surgery (when has he ever not been small for surgery!) and don’t think a feeding tube is necessary at this time!

While it is scary to think about another round of hospital visits and surgery, it is nice to have a plan and not just have it looming in the distance.  We will see his GI doc on Tuesday and break the news that there will be no feeding tube.  Should be an interesting appointment since they think we are going to be talking about putting one in!

Of course, right now Andrew is eating like a champ and weighed 25lbs (fully clothed) at the cardiologist’s office.  He always gets sick right before we see GI and loses weight.  He ate constantly today, but was cranky and pulling at his ears tonight.  Right on schedule!

I promise to update on Andrew further… there is so much that he is doing.  However, this post is now long enough.  I even promise pictures next time!

Bumps in the Road

I am definitely a slacker when it comes to blogging. It is unintentional, I always mean to write an update a week but it always ends up on the bottom of the to do list. Not sure why, but it happens.

This summer has been full of mostly good things but a few bumps in the road. To start… the good news…

From a cardiology standpoint Andrew is doing great. The good cath report in May continued with our quarterly checkup. Since we are in a holding pattern until he is ready for the next surgery (Fontan), our cardiology appointments are now every 6 months. This is the longest we have ever gone between appointments… yay!!!

One of the bumps in the road continues to be Andrew’s growth, especially his weight gain. Or more like the stunning lack of it. In May we saw a GI doctor and a nutritionist who put Andrew on a 1300 calorie per day diet. The goal was an average gain of about 100 grams per week (about 3.5 oz). At his weight check a month later (June), he had only gained 100 grams .

However, with some tweaking to his diet, at his July appointment he had shown a monthly weight gain of 500 grams (over 1lb) ! This is more than they hoped for. So we are under orders to keep it up. If we can, then he should easily hit the 30lb goal for the Fontan by next summer.

The next bump in the road came this last week. Monday, Andrew spiked a fever and was very mopey with no other apparent symptoms. As the day wore on, he began to breathe faster and so we put him on the oximeter to check his oxygen levels. His normal 83% was now 75%. So off to the after hours clinic at our pediatrician’s office.

Luckily, Andrew’s doctor was staffing the clinic that night. He was able to rule out the “easy” things like an ear infection or strep throat. After listening to Andrew for several minutes, he became concerned with the fast breathing. He didn’t hear anything concerning, but didn’t like the high respiratory rate with lower oxygen levels. In light of Andrew’s history, he sent us off with antibiotics and a referral for a chest xray.

Needless to say, it came back as mild pneumonia. So yes, you can get it when it is 110 degrees outside and show absolutely no obvious symptoms. No cough, no runny nose, only a fever and symptoms the monitor can pick up. Just one more reason that the home health care company is not getting their oximeter back.

Well, almost a week has gone by and he is getting better. His fever is gone and his energy is back. His sats are still low but very slowly getting better. I will be glaf when they come all the way back up.

To end on a good note, Andrew is talking a ton. He even strings 3-4 words together. He is also excited to start school on Thursday. I think it has more to do with the new Blues Clues backpack than school, but we will take it. Check back at the end of the week for the First Day of School update! Until then, here is a pic of Andrew learning to fish from Grandpa and this is at 7500 feet in elevation! Not bad for a kid witg half a heart!

A Day at the Lab

Well… the results are in from our day at the lab (cath lab)…. and the verdict is…. there is nothing wrong with Andrew’s heart (except for the missing half and rebuilt plumbing!). They did not have to fix anything so we are home. Very tired, a little loopy from the drugs, but home.

For once the surprise Andrew had was a good one. Everyone was sure that there was a problem with his left pulmonary artery (carries blood to the left lung). But they found nothing. In fact, it is about the same size as the right one. So 6 hours in recovery and we headed home.

Hint for other parents of heart toddlers… ask for meds to keep your child sedated… they put Andrew on a drip of Dex and he slept all afternoon! Easiest 6 hours of hospital time ever. Andrew can not even sit still for a minute let alone lie still with his leg straight for 6 hours. They even left it on long enough for us to go home groggy.

The funniest part of the whole thing is that they did an echo while he was still sedated. The echo showed the lwft pulmonary artery as narrowing. The thought now is that the echo is not seeing this artery in full. It is probably a fluke of his anatomy.

They did get really good data from the carh. Andrew’s heart is strong and his Glenn pressures and lung pressures are in the range they would like them to be. There is no pressing need to do his next surgery until later which will allow him to get bigger. It is always nice to have actual data to back what we see every day.

So we rest tonight with the knowledge that he is doing well. I am sure I am leaving something out, but it has been a long day! Thank you for all of the prayers and support. They are much appreciated!

Early Morning

Tomorrow is our big day in the Cath Lab. We will leave our house at 4:45am so that we will have plenty of time to check in at the hospital by 5:30am. The procedure is scheduled for 7:30am. So we will begin starving Andrew at midnight.

They are going in to check on his left pulmonary artery (LPA). His last echo showed some narrowing and he has beem showing some symptoms that may be related. They repaired his LPA when he was 8 months old and the area of concern is in the same place as the repair. The thought is that scar tissue may be causing it. The hope is that they will be able to place a stent and be done.

Not sure is it will be an overnight stay or not. We always prepare to stay so tonight is filled with packing and making meals for the family at home. Also, trying to figure out how to keep Andrew entertained at the hospital.

Please keep us in prayer tomorrow. It has been awhile since we have had to go back to PCH for more than a doctor appointment. His Glenn, our last hospital stay, was exactly 2 years ago today. We are praying for no surprises and will give Andrew the all important talk about following the rules. Will update tomorrow as we get information.

First Day of School

Our baby officially started school yesterday! 

First Day of School

Ok, it is only preschool, but still there were days that we thought that we would never get to see this day.  His backpack may be almost as big as he is, but he is ready.  It was great that it happened on my furlough week, so it was easy for both of us to take him.  School starts at 9:30am.

My school is in there!

Walking to school with Dad

Walking up the stairs to his classroom

Once inside the classroom, there were no tears.  We had the opportunity to visit his teacher and classroom the day before, so he made a beeline for his favorite toys.  We had to call him back to say goodbye.

Kissing Dad Goodbye

The teacher offered for us to stay if we wanted, but since he hadn’t quite realized that he was being left, we decided to go.  It was nice to leave before the screaming started.   We went to Crackers and Co for our usual First Day of School Celebratory Breakfast.

At noon, it was time to pick Andrew up.  His teacher said that he didn’t cry at all until he saw us at the top of the walkway.

First tears of the day

He does this at the babysitter’s as well.  It’s like all of the sudden he remembers that we left him and he isn’t supposed to like it.  However, he quickly forgives and forgets.

Along with preschool and related services, Andrew qualified for transportation services.  A school bus will pick him at the sitter’s and take him to school and then will bring him home.  The staff in transportation services is a little nervous about Andrew and his issues.  One of the major ones is his size, so the decision was made that he will ride on the bus in a car seat.  Today, two very nice gentlemen came to fit Andrew to his “bus car seat”.  He thoroughly enjoyed testing out a new seat!

Getting fitted for his bus seat!

The drivers and monitors that will be transporting Andrew are going to come on Tuesday to “train” on Andrew.  They will get the opportunity to ask us questions about Andrew’s heart condition and any other concerns we may have.  It is great that they take the time to get to know all of their kids!  We are hoping that they will start transporting him next Thursday.  He is very excited to ride on a school bus!

Overall, the transition from AZEIP (our state early intervention program) to Mesa Public Schools (our school district) has gone extremely well.  The school district has been extremely helpful, much more so than the state program. 

We have been asked a few times why we chose to start him now, close to the end of the school year.  This was done in consultation with his current speech, physical and occupational therapists.  All 3 felt that the experience of preschool, even if only for 6-7 weeks, would be a great one for him.  Also, it will give us some insight on what issues he is going to have outside of our home.  Either way, we were not going to have services through the summer.  AZEIP and DDD will stop paying for services on his 3rd birthday (June 6) or the first day of preschool, whichever comes first.  School gets out on May 26, so it is somewhat of a wash.

In light of this, we have chosen to blend Andrew’s care.  We are lucky enough to have good insurance.  They will help pay for his therapy services outside of school.  Therefore, we will continue with our current therapists in addition to Andrew starting school.  With starting preschool, issues that we have never seen before may surface.  His current therapists will work with Andrew through the summer and can help address some of these.  We will re-evaluate the situation when school starts again.

Overall, we are all excited about preschool and what it can bring to Andrew.  He seems to like it, although we will have to see if Monday is a tear free day.  He may have figured out that we are going to leave him.  If so, I am sure it will be short-lived!  How can you be sad when you know your favorite lunch (hotdogs, mandarin oranges and Doritos) will be waiting for you at home?!?

Andrew's favorite lunch!