"I love you and want for you all things that make you happiest; and I guess you, not I, are the one who knows best what those things are."

Sunday, September 29, 2013

Welcome to the Church!

On August 16, approximately 33 hours after his arrival, Charlie was baptized in Cardinal Glennon's NICU by our long time family friend, Father Charles Latour. It was a special and blessed day.

Today we were able to conclude Charlie's baptismal rite with Father John Beveridge at our home parish of Saints Peter and Paul. Charlie was presented and accepted into our parish and Church and "officially" received the light of Christ through his baptism candle.
Charlie's godparents were both present (we didn't have to FaceTime anyone in) and he got to wear a beautiful white suit.

Our parish has been so supportive throughout Charlie's journey. From prayers to financial support, babysitting to grocery shopping, providing clothes and assisting with life insurance - they have always been there to help. It felt so fitting to share him with the congregation and ask for their continued support in raising him in the Catholic faith.

Here's a picture from this morning! What a great day!!!


(Nathan is hiding somewhere behind us by the altar)

Friday, September 27, 2013

Sick Brother

Charlie continues to gain weight. Today he weighed in at 11 lbs 7.5 oz.

Weight gain is essential for Charlie. First, because of the anatomy of his heart he naturally burns more calories than most babies simply because his heart has to work harder to circulate blood. Second, Charlie needs to continue putting on weight to be able to better tolerate his second surgery, the Glenn. Typically the goal weight for the Glenn is 14-16 lbs.

Like everything in Charlie's world weight gain is a delicate balancing act. One of two things will happen in the future... If he continues to grow and thrive - we may be able to postpone the second surgery for a little while especially if his oxygen saturations remain relatively high. On the other hand, if he continues to grow and put on weight at this quick rate - his heart may not be able keep up with the growth and we may have to do the Glenn earlier than expected if his heart starts to struggle.

This morning his home nurse removed his last suture. He had one hanging on in his belly button that wouldn't dissolve. It had begun to get red and inflamed the past few days. Since it was stitched up after they removed umbilical catheters (IVs through his umbilical cord) it is naturally close to his blood line. Obviously the last thing we need is an infection in his blood stream so it was time to remove it. Charlie did great and his belly button immediately went from an "outie" to an "innie" once the stitch was out!

The only thing we're watching with Charlie this weekend is his brother. Our middle son, Nathan, has been running a fever, coughing and hacking, and generally not feeling well. Nathan is off to the pediatrician later today and we're trying to keep him at a distance from Charlie. However, Nathan loves Charlie immensely and it's hard to explain Charlie's immune-compromised state to a 3 year old. We just keep telling Nathan that if Charlie gets sick he'll have to go back in the hospital....

Today we would appreciate some prayers for Nathan. He's ready to be back at school, back to playing and back to feeling better. Plus - we want him healthy because on Sunday we're finishing up Charlie's baptismal rite at church! Yay!

Tuesday, September 24, 2013

The Norwood

What is the Norwood Procedure?
On his 6th day of life, Charlie underwent his first open heart surgery. The procedure performed was a modified Norwood with a Blalock-Taussig shunt. It is the first of three staged surgeries designed to help Charlie's heart function.

The most urgent problem with Charlie's birth heart was that the heart was unable to adequately pump blood to the to the body (systemic circulation). The goal of the Norwood procedure is to connect the single ventricle to the systemic circulation. To accomplish this, blood flow to the lungs is disrupted, and therefore an alternative path must be created to supply the lungs.

The heart is accessed through the sternum and Charlie was placed on complete heart and lung bypass throughout the duration of the procedure. The surgery can be broken down into two main components.

Providing Increased Circulation to the Body
In order to convert Charlie's left ventricle into the main pumping chamber the lower portion of the pulmonary artery was separated from the left and right portions of the pulmonary artery and joined with the aorta. This allows the blood, a mixture of oxygenated and deoxygenated, to be pumped to the body via the left ventricle through Charlie's pulmonary valve. The aorta was widened using a Gor-Tex graft to allow for greater blood flow out to the body.

Providing Alternative Circulation to the Lungs
Since the remainder of the pulmonary artery was disconnected from the heart a modified Blalock-Taussig Shunt (a kind of plastic tubing much like a straw) was used to connect the subclavian artery to the pulmonary artery. In Charlie's case, blood comes from the left ventricle, through the pulmonary valve, the reconstructed aorta, the subclavian artery, and the shunt, to the lungs.

Charlie - Norwood

The first successful Norwood procedure was performed in 1981. In the world of medicine it's still considered a fairly new procedure. While no one can tell us if Charlie will live to be 60, 70, or 80 having had this operation - they can tell us that some kids have graduated college and started successful careers.... And given the alternative, we'll take that!

Monday, September 23, 2013

FCI & NICU Reunion

This weekend has been filled with family activities! Charlie's grandparents watched him on Saturday so that the big boys could go to the ItalianFest parade with mom and dad. Everyone seemed to enjoy the time including great-grandma who got to feed Charlie a bottle that afternoon.

Then yesterday we packed up the whole family and headed to Laclede Park at St. Louis University. We had a great time celebrating our little Charlie at the Fetal Care Institute's and Cardinal Glennon NICU's annual reunion. They had an awesome set up complete with a picnic lunch, photo booths, face painting, storytelling and more. We had a chance to see several of Charlie's nurses and even saw the Rally Squirrel. We also spent some time catching up with my high-risk OB and the sonographer who saw Charlie weekly for nearly 4 months! Everyone was so impressed at how well he was doing, how much weight he had gained, how great his scar looked, etc.... It's so reassuring to know that the doctors and nurses think he's doing as well as mom and dad think he is doing!

Today was another nursing visit. I'm happy to report that we've broken the 11 lb mark! Charlie's heart rate was around 140 which is a little low for him, but still in the normal range. We'll continue to monitor it with the ever-so-finicky pulse-ox meter to make sure it is not dropping too much. His digoxin (heart med) slows the heart rate down so we'll need to be cautious when we administer that particular med.

Overall, we continue to thrive and we're slowly but surely getting out and about. As cold and flu season kick in we'll be somewhat quarantined due to Charlie's weakened immune system and lousy oxygen levels - so we're trying to spend a little time around the community now.

Tuesday, September 17, 2013

Progress is Progress

"Progress is progress" is our motto around here. Some days we can see that Charlie has made loads of progress. Other days it is slow moving - two steps forward, one step back - kind of progress.

The past few days have been funky when it comes to Charlie's eating habits. He refused the bottle a few times, skipped a couple feedings all together, and has been particularly pokey when it comes to eating. Last night (with the permission of the cardiologist) we let Charlie lead the way on his nighttime feedings. He slept one slightly longer stretch of about 4.5 hours. He polished off all his nighttime feedings after that and has consistently eaten every 3 hours all day. Yay! Maybe that one longer stretch helped even things out a little.... Plus we're trying to move him up to a larger size nipple on his bottle so he won't have to work as hard to eat (meaning he'll burn less calories and hopefully put on more weight).

Charlie started his new medicine today. We'll need to wait a few weeks to see how it works in regards to the leakage around his valves. It's good to know that we are on the road to addressing the issue though. Blood work and his echo in three weeks will tell us his progress on that front. Hopefully it works miracles!

Also by way of progress - we managed to get out of the house for a bit today. The older boys had an appointment a few towns over from us so we packed up after school and headed that direction. Afterwards we had lunch at a local farm and took a wagon ride out to the orchards to pick some apples fresh off the trees. Charlie slept through most of it only waking long enough to drink two bottles during the trip. Nonetheless, it was great to get out and about today and healthy for the big boys to see that life will return to normal.


Now, if I could just make progress on the mess that is my house these days or on some of the million thank you notes that need to go out - that would real progress!

Monday, September 16, 2013

First Cardiology Appointment

Charlie's cardiology appointment was this afternoon. After his echo we met with Dr. Peterson to go over the results as well as discuss Charlie's progress in general.

His echo showed a few things of minor concern. First, the two valves between the atriums and his left ventricle are showing signs of leakage. This is most likely a result of the increased pressure in his atriums that the Norwood operation causes. However, we don't want his only functioning ventricle to be overwhelmed with too much blood flow.... (everything in cardiology seems to be a delicate balancing act) ...so we've started Charlie on a new medication that will help his heart beat stronger, but a little slower. Dr. Peterson is hopeful this will help resolve the issue.

Second, she noticed that Charlie's aorta shows some narrowing. This could be one of two things. Either the narrowing is just the difference from the portion of the aorta Dr. Huddleston enlarged and where the enlargement ended or Charlie's aorta is narrowing on it's own. Right now Dr. Peterson is leaning towards the first one and wants to keep a close eye on it over the next couple months. Charlie will have a cardiac cath around 4 months old so they can get a better, more detailed look at the area then. And if he shows any signs of distress or things look like they are getting worse - we'll simply schedule the cath sooner than later. They should be able to balloon open the aorta during the cath if needed.

On a brighter note, Charlie's blood pressure, heart rate and pulse ox looked great today. In fact, his pulse ox was 91 which is pretty much unheard of in Norwood babies... And because he's continuing to put on weight, we've been cleared to let him sleep longer during the night between feedings. So tonight we are not setting an alarm clock for every three hours! Plus, Dr. Peterson said if we had another week of weight gain that we could move to nursing at night and pumping/fortifying/bottle-feeding during the day! Praise God! That means mommy will be getting a lot more sleep at night!

So for now we've added a new medicine and we're continuing to spot check his oxygen levels. We go back to the pediatrician later this week and return to the cardiologist and the echo lab in three weeks. Please pray that this new medicine does the trick and that we don't find any additional narrowing of the aorta in our next echo. We're in no rush to head back into the world of anesthesia and heart procedures....

Saturday, September 14, 2013

Aortic Issues

Wrapping up our mini-series titled "Charlie's Screwed Up Heart"

There are three main defects I haven't addressed yet. All three of these deal with Charlie's aorta which is the artery responsible for carrying oxygenated blood out of the heart and distributing it to the entire body. Without this oxygen-rich blood circulating the body faces organ failure and oxygen deprivation in the brain.

Let's breakdown the last three:

AORTIC HYPOPLASIA - a fancy medical term for a small, underdeveloped aorta. Because Charlie's right ventricle isn't functioning and pushing blood out through the aorta - the aorta didn't grow to the proper size. At birth his aorta was about 1/4 the size that would be expected in a normal heart. Very small aorta = very limited blood flow to the body.

AORTIC STENOSIS - in a normal heart all the veins and arteries that lead in and out of the heart have a small valve controlling blood flow. Think of these valves like one-way doors that open and close as the different chambers pump to allow a designated amount of blood to pass through. With aortic stenosis, the valve that connects the heart to the aorta is too small or fails to open properly and doesn't allow enough blood to flow through to the body. Again - too little blood flow through the valve to the aorta = lack of aortic growth.

COARCTATION OF THE AORTA - a doctor's way of saying there is a kink or narrowing of the aorta. Since Charlie's aorta is already ridiculously small any narrowing of the aorta can cause serious issues. It causes his heart to have to pump even harder to pass blood through the narrowed section.

So there you have it....Charlie's heart explained in three short days! Tomorrow we'll be back to our regularly scheduled programming complete with some medical updates and maybe even a picture...

Friday, September 13, 2013

Transposition Explained

Round 2 of explaining Charlie's Cherished Heart

Today I'm only going to tackle one of Charlie's defects:

TGA occurs when the two main arteries going out of the heart—the pulmonary artery and the aorta—are switched in position, or “transposed”.

Normally, blood returning to the heart from the body is pumped out of the heart to the lungs. There, it receives oxygen and returns to the heart where it is pumped back out to the body. In other words, blood in a normal heart follows this patterned route repeatedly: heart, body, heart, lungs, heart, body, heart, lungs....

In TGA, blood returning from the body is pumped back out to the body and blood returning from the lungs is pumped back to the lungs. This occurs because the main connections are reversed. Basically, the heart has two closed loops of blood flow. The result of transposition of these two vessels is that too little oxygen is in the blood that is pumped from the heart to the rest of the body because that loop of blood never goes to the lungs to pick up oxygen.

This means that TGA is a cyanotic (lacking oxygen) heart defect that leads to a bluish-purple coloring of the skin and shortness of breath. How bad the symptoms are depends on whether there is a way for the two separate blood circuits to mix, allowing some oxygen-rich blood to get out to the body. This mixing can occur through other defects, such as a hole between the bottom chambers of the heart (VSD), or through a shunt (ductus arteriosus) that normally is present at birth. Luckily (?), Charlie had both of these which is why he was remarkably pink at birth!

Also, because Charlie has a Double Inlet Left Ventricle all the blood coming back to Charlie's heart (oxygen-rich blood and oxygen-poor blood) is pumping into one ventricle and mixing together creating "partially oxygenated" blood. Then his left ventricle is pumping blood back to the lungs. Charlie's right ventricle is severely underdeveloped (HRHS) causing it to malfunction. Thus, the chamber that should be pumping oxygen rich blood to the body isn't doing it's job and Charlie has poor oxygen saturations compared with the general public.

After Charlie's birth, we relied on that ductus arteriosus mentioned above to keep some oxygenated blood circulating through his little body. This required him to be on special medications to keep that passageway open (it normally seals itself off shortly after delivery).

TGA may be the most confusing part of this whole diagnosis - if you managed to follow this post....tomorrow will be a breeze!

Thursday, September 12, 2013

Charlie’s Diagnosis

OK - so you've had a few days to digest the normal heart structure. Now let's talk about Charlie's heart.

First - the actual diagnosis
Double Inlet Left Ventricle
Ventricular Septal Defect
Hypoplastic Right Heart Syndrome
Transposition of the Great Arteries
Aortic Hypoplasia
Aortic Stenosis
Coarctation of the Aorta

Charlie - Birth heart

Trust me when I say - that's a lot to take in....so let's take it a little at a time for the next few days

DOUBLE INLET LEFT VENTRICLE (DILV) - both atriums dump blood into the left ventricle. In a normal heart the atriums dump blood into separate chambers keeping oxygenated blood from the lungs separate from de-oxygenated blood coming back from the body. In Charlie's heart the blood that is coming back to the heart from the lungs and body are mixing together in the left ventricle creating "partially oxygenated" blood that is circulating back out to his body.

VENTRICULAR SEPTAL DEFECT (VSD) - there is a hole between the left and right ventricle that is allowing blood to flow in both directions between the two. In a normal heart the two ventricles have a complete septal wall between them keeping the oxygenated blood from the lungs and the de-oxygenated blood from the body separate.
HYPOPLASTIC RIGHT HEART SYNDROME (HRHS) - this is simply the medical way of saying that the right side of Charlie's heart is underdeveloped. In utero the four chambers of the heart grow as blood flows through them. The DILV creates a situation where there is little blood flow through Charlie's right ventricle (only what is sneaking through the VSD). Therefore the right ventricle never grew to it's proper size.

OK - That's probably more than one can take in via a post. We'll tackle more tomorrow!

Monday, September 9, 2013

Normal Heart

This is a "normal" heart.


Suffice it to say - Charlie's birth heart looked NOTHING like this. His arteries and ventricles were switched, one chamber of his heart was practically non-existent, he had a kink in his underdeveloped aorta and a valve that didn't open properly (to name a few of his issues). Several followers have asked for details regarding his diagnosis, long term plan, life expectancy, etc.

Over the next few days I'm going to attempt to put some of this in perspective for those of you who are following Charlie's story. I'll try to explain the differences in Charlie's heart and the impact they have one him now and in the long run. Some issues will be remedied along the way, others will remain unfixed, more will be manipulated to a 'working' yet abnormal state.

I'm far from a medical guru - but I've come a long way in heart anatomy since April when we received Charlie's diagnosis. Hopefully, together we can map some of this out!

In the meantime - study this normal heart.....you'll need to understand it before you'll be able to understand the mess that is Charlie's heart.

Friday, September 6, 2013

A Pediatrician

It is hard to believe that Charlie has been home for a week!

Today, he met his pediatrician for the first time. Not a neonatologist, not a surgeon, not a cardiologist, not an anesthesiologist - a pediatrician. You know, the doctor that 'normal' babies go see...

After a long and detailed recap of Charlie's first three weeks, Dr. Herman gave Charlie a once over. Turns out that our little man is still thriving despite all his early setbacks. He was in the 85% for height and 60% for weight (which is particularly impressive when you consider her didn't eat for the first 12 days of life). We are going to continue doing some weight checks along the way to monitor his growth. Because of the anatomy of his heart, Charlie burns more calories than the average baby so his caloric intake is vital.

Dr. Herman is submitting the paperwork for Charlie to get the RSV shots. The shots are monthly and cost $1200 per dose. Insurance companies notoriously don't like paying for the shots so say some prayers that our insurance will cover the shots for Charlie. He really cannot afford to get RSV with his oxygen saturations only being in the lower 80's on a regular basis.

Charlie's only bump in the road today was being diagnosed with thrush. He started medication today to clear it up. I've been on an anti fungal since Tuesday so with both of us being treated we are hoping to get past this quickly.

On the other hand, this mama is a little worse for the wear... After waking up from an afternoon nap with the chills and 102* fever, I've been diagnosed with mastitis. So now I'm on an antibiotic to clear up that infection. The plan is to sleep as much as possible, pump as much as possible and take my medicine so that I can get back to taking care of my baby....

Thursday, September 5, 2013

Surgical Follow Up

We were back at Cardinal Glennon today for Charlie's cardio-thoracic surgical follow-up. Walking in the front door of the hospital felt almost surreal. It was the first time I carried Charlie through the doors of the hospital. It was the first time I checked in at the front desk for an appointment as opposed to flashing my "we're here every day indefinitely" ID badge that I had for the last few weeks. It was the first time Charlie was seen in the Dallas Heart Center.

Dr. Huddleston was very happy with Charlie's progress since he's been home. Charlie has gained some weight and showed off his stellar bottle drinking skills. His chest X-ray looked good from all angles and his blood pressure was spot on. His oxygen saturation and pulse looked as good as can be expected with the current anatomical set up of Charlie's heart. Dr. Huddleston even decided to discontinue Charlie's diuretic. The remainder of Charlie's meds will most likely be long term/lifetime meds.

We wrestled with the pulse oximeter as a group in an attempt to come up with a battle plan that doesn't make us want to throw it out the window. Ultimately we (myself, Dr. H, and the CTS nurse) decided it was best if we switched to spot checks for his pulse/ox readings as opposed to continuous monitoring. At night we'll do a spot check before bed and one first thing in the morning...then we'll only use continuous monitoring at night if Charlie is sick or we have other concerns. Praise God!

Dr. H's only concern was the scar tissue building up at the surgical site. But after close examination it was determined that it is just that...scar tissue. So we'll watch it and hope it doesn't build up too much as it will make the subsequent surgeries a little harder to access and the new sites won heal as nicely. A small price to pay for keeping our little man's ticker ticking!

Our path over the next few months will be dictated by Charlie. We are basically waiting for his oxygen saturation levels to start declining. His cardiologist, Dr. Peterson, will be responsible for monitoring this and will help determine when to schedule Charlie for surgery #2, the Glenn. He'll have a cath somewhere around 4 months old if not before to prep for the Glenn.

Our new goal is to keep Charlie as germ free and healthy as possible as we navigate cold and flu season....

A positive appointment overall! Thank you for the continue prayers...

Wednesday, September 4, 2013

I Loathe Pulse Oximeters

We continue to wrestle with the pulse ox machine. We've tried multiple different locations for the sensors, different types of tape, extra tape, etc. we called the medical supply company that provided the equipment only to be told...the sensors are temperamental. We may end up buying some other type of sensor to see if that makes any difference. *sigh*

Charlie is really settling into a routine he...re at home. Granted, that routine consists of eating, sleeping and pooping, but he's adjusting to life outside the hospital walls well. He's even starting to sleep through some of the 800 kisses his brothers lay on him.

Because Charlie is gaining weight they've cleared him to nurse 1 or 2 times a day. He's been doing pretty well although we've found that he gets hungry about 30 minutes earlier than normal after nursing. It's probably a sign that he burns more calories nursing than using a bottle, he doesn't get quite as much volume, and he's not getting those extra 48 calories he gets when we fortify the milk in the bottle. Still - progress is progress...

We've learned lots of little things along the way with Charlie. For instance, we had to register our home medical equipment with the power company. This makes us a priority for restoring power in an outage since no power means no functioning equipment. Also, we've provided the local EMTs and 911dispatch with details on Charlie's medical status. That way in the event of an emergency they'll be better prepared by the time they arrive at our house. Small things that can make big differences with medically fragile kiddos!

Monday, September 2, 2013

Our Little Warrior

Ironically, we may be getting less sleep now than when Charlie was in the hospital. At the hospital only one of us was up with him at night and if he monitors decided to be finicky a nurse would deal with it. Now both of us are up (Matt feeds Charlie while I pump) and we both wrangle the pulse oximeter at different points in the night. But we're trading mornings of sleeping in and trying to get naps in during the day.

Charlie's feeding went better yesterday evening and last night! He's back up to his 80-90 mL goal thankfully.

Unfortunately his somewhat awkwardly timed 'suck, swallow, breathe' results in lots of hiccups. As you can probably imagine, hiccups are an unpleasant experience for someone who has their breastbone wired shut recently. So lots of writhing and crying come with what is a normal baby thing...

His scars are airing out this morning before we doctor them up with betadine, neosporin, gauze and band aids. So here's a look at our little warrior's battle wound... He doesn't seem bothered by it based on that goofy grin he's got going...

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Sunday, September 1, 2013

A New Normal?

Last night we managed to get the pulse oximeter set up so that it only alarmed twice and both showed saturations in the upper 60'a (not uncommon for Charlie when he sleeps). This resulted in mommy getting *a little* more sleep than the night before.

Today however Charlie is letting us know that he was just taking it easy on us yesterday because it was his first day home. His feed volume is way down today and he's been far more irritable. Maybe cause he's not eating as much? (You just can't reason with kids these days!)

We did manage to get all his medications in him this morning and get his chest wounds cleaned and re-bandaged. But that was much like wrestling a screaming, crying octopus. But all in all - his incision site looks good. What little redness there was when we left the hospital seems to be fading.

Most of this morning was spent trying to put the house back together after dropping everything in piles when we came home from the hospital. We now have places for most of Charlie's equipment and cleared space in a cabinet for bottles/feeding supplies. We've washed loads of dishes, wiped down counters, and sorted laundry. There is still lots to-do but we are slowly catching up.

We're trying to figure out how we'll get three boys out of the house on time for anything. And how we will be able to carry all of Charlie's equipment with us when we do leave... It's going to be an interesting first few trips out of the house...