Hospital Trips...again

Enzo didn't feel well during most of our California trip.  He had a fever off and on and wouldn't eat - anything - all week long.  He also had a cough and a lot of congestion - which always worries us with him.  The Sunday after we got back we were finally able to take him to the doctor.  Of course, his regular pediatrician wasn't there so we saw the nurse practitioner instead.  She tested him for RSV (negative), did a chest X-ray, and said his ears were fine.  But he sounded really "junky" in his chest, so she send us to the hospital to receive some sort of breathing treatment.  However, several hours time and $300 wasted later, we came home from the ER with nothing but an antibiotic.  I guess the NP didn't feel qualified to prescribe one, and Enzo apparently didn't need any breathing treatment either.  Needless to say, we won't ever see that NP again. 

Two days later the NP called saying that she got his results back from the chest X-ray and that she was really concerned.  She saw something on his X-rays that isn't normal in children.  She said she'd call his pediatrician, Dr. Lei.  Not 5 minutes later, Dr. Lei called and said he wanted to see Enzo right away.  With Enzo's history, I pretty much mentally prepared myself that he was going to end up in the hospital again.  However, Dr. Lei checked him out and said that Enzo didn't seem to be as sick as he thought he might be.  The doctor didn't know why the hospital had prescribed the specific antibiotic and told us not to give it to him anymore.  Enzo did, however, have a double ear infection, so we went home with a different antibiotic.  (It looks like our favorite NP couldn't even properly diagnose an ear infection.)

I was still quite worried about all of the congestion in his chest and was thankful that we were scheduled to see the cardiologist on Thursday.  When we drove to the doctor's appointment, we were again mentally prepared (and physically packed) for another emergency trip up to Primary's.  We were worried and knew that something just wasn't right.  At his appointment, Enzo had another routine chest X-ray done.  He wasn't scheduled for an Echo, but I planned on requesting one so that we could make sure everything was okay with his heart.  When we saw Dr. Mack, we explained Enzo's recent health history.  He said that what was seen on the X-ray was similar to what might be seen in adults when they had pneumonia.  He had us get an Echo right then and also sent us down to the lab to get blood work done to see if he had penumonia.  The Echo showed that there was some more narrowing in his pulmonary veins, causing some blood flow restrictions.  It wasn't nearly as bad as when he had to have surgery in April, so Dr. Mack said he would call Dr. Burch, Enzo's heart surgeon, and then get back to us.  We didn't hear back anything at all for several days.  I finally put in a few calls to Dr. Mack's office and heard back from him on Monday afternoon.  The pneumonia came back negative, but Enzo did need to have a cardiac cath done - a procedure where they insert a catheter up through a vein in his leg and into his heart to get a more accurate reading on what was going on in his heart.  They would measure the amount of narrowing in his veins, and if necessary, put a stent in to hold the veins open.  They would also be able to tell if another open-heart surgery would be necessary.  His cath was scheduled for November 8 at 11 am.

The hospital called us a couple days before and told us that his procedure was moved to 1 pm.  Enzo couldn't eat anything the morning of the surgery, so we woke him up in the middle of the night to feed him a bottle.  James gave him a blessing, and I know it was because of that that Enzo wasn't completely miserable having to wait all day without eating.  He did wake up a little grumpy, but wasn't too bad at all.

After we checked in, we got to wait in a waiting room full of toys.  We stuck Enzo in the "toy pit" and let him play.

We then took him down to the Cath Lab where they would do his surgery and left him with the nurses and doctors.  We were told that it would be a 3-4 hour procedure, give or take.  5 hours later, we finally got paged to go back to the lab and hear the results.  It was really neat because were able to see the video of exactly what they had done.  After the inserted the catheter into his heart, the injected a dye and we could see it move through his heart and veins.  We got both good news and bad news.  The good news was, they were able to put a stent into his upper right pulmonary vein to help it remain open.  The bad news was, his lower right vein had closed up completely.  I guess it had narrowed so much that it was completely gone.
We asked what this would mean long term.  Basically, as long as the upper stented-vein remained open and un-obstructed, Enzo could live a normal life, playing sports and doing everything a normal boy would do.  However, worst case scenario, if the stent did not work and his upper vein closed off as well, he would loose the use of his right lung and would have to live off of one lung.  This would mean a more limited life style, with no sports or strenuous activity.  It may sound bad, but to me it was happy news.  Limited activity was a worse case scenario that I could live with.  Up till that point, I thought that his worse case scenario was that they couldn't do anything about it and he would die - and that was always in the back of my mind.  Every time he got sick, wouldn't eat, wasn't gaining wait, etc. - I would think that my baby was going to die.  I know that in the big picture, this is still an option.  I know my Father in Heaven can take him back to live with him at any time.  I know his heart still isn't perfect and there's always a chance that he won't be with me his whole life.  But I'm always grateful for every minute with him that I have been given. And I was very grateful to hear that the most likely worse case scenario was a one-lung limited lifestyle.

After we talked to the doctors, we had to wait again in the waiting room until Enzo was ready to be taken to recovery.  Because it was so late in the evening, he was admitted for the night instead of staying in same day recovery.  It was a rough night.  Enzo was awake, but still very calm and sedated, for about 2 hours after surgery.

 

However, once the sedation started wearing off, it was a whole different story.  Once he started waking up, he wanted to move around.  But we had to keep his leg still and flat for 4 hours.  To make a long story short, he pretty much screamed and cried all night long.  It went from sleeping...to screaming...to crying...to whimpering...back to sleeping....about every 5 minutes or so.  During all of that, he had a reaction to the anesthesia that caused him to turn bright red.  The nurses thought it might be an allergic reaction.  That eventually went away and he finally drifted off to sleep at about 1:00 in the morning.  Then, his oxygen levels plummeted.  They had to wake him up again to put oxygen on him - which was a 3 person wrestling match to accomplish.  His oxygen levels stayed low, even with the oxygen on, so they took him down for x-rays at 4 am.  Everything worked itself out by morning, but it was one long night. He was almost back to his normal self and was climbing all over his crib by morning. 

He was discharged at about 10 in the morning, but had to go home on oxygen again.  That didn't stop him from moving around and having fun though!

We saw Dr. Mack again a week later, where they did another Echo to make sure everything was okay.  The stent looked great and he got to come off the oxygen.  Dr. Mack was pretty concerned, though, about the loss of his lower pulmonary vein.  He said that we need to be very careful and watch him closely because he is at a higher risk for infection in that right lung.  We now need to take him to the doctor if he ever gets a fever and be very careful that he doesn't get sick.  He also takes asprin daily to help prevent any kind of build-up from forming around the stent. 

Once again, he seems like a normal kid.  Unless you could see his scar, you'd never know about all he's been through.  Here's hoping he stays that way for good!

The Equipment

Enzo was hooked up to so many machines, tubes, and wires after surgery that it was a bit overwhelming.  It was like learning a whole new language - the nurses had to tell us MANY times what everything was and what it did.  I'm still not sure I have it all down, but here it is to the best of my memory:

 

1. Medications: At the beginning, Enzo had at least 10 lines of medication going at all times.  Obviously, this included heavy pain medications and medications to keep him sedated, as well as diuretics to help get fluid off of his lungs and make him pee, blood thinners to prevent clotting, and simple lipids and fluids to keep him hydrated.  

2.  This is the ventilator that was doing his breathing for him.  The monitor showed the number of breaths per minute, indicating whether they were done by the machine or were spontaneous breaths done on his own.  The machine did all of his breathing for home for several days.  It was only after they started weaning him off his sedation medications that he was able to breath at all on his own.  He came off of the ventilator a week after surgery

3.  This is a Nitric Oxide machine.  I'm not sure of it's exact purposes, but I believe Nitric Oxide is used to help the lungs heal and help the body absorb oxygen. Enzo was on this for several days along with the ventilator.

4.  Heat Lamp:  I'm not exactly sure what the official name for this is, but it's basically a heat lamp.  With no clothes and no blankets, Enzo needed something to keep him warm.  It came with some sort of sensor to monitor his exact temperature at all times.  

The pacer wires were connected directly to his heart and were attached to the external pacemaker.  Right after surgery, his heart was having a hard time beating regularly on its own, so they attached the pacemaker just in case it needed help beating.  As far as I can remember, he never ended up needing to use it.

These are the Ventilator tubes that helped him breath.  For the first couple of days, the machine did all of his breathing for him.  He gradually started taking breaths on his own and was able to come off of the ventilator at 7 days old.  

1.  With the breathing tube constantly in his mouth, he had a permanent binky.  He was able to suck on it whenever he wanted.

2.  The blue tube connected back to the machine which provided a steady supply of oxygen (and, for a few days, nitric oxide.)  They would frequently add a saline solution to it to keep the air and his mouth moist.

3.  This tube had some sort of pipe cleaner in it, which was inserted into his mouth several times a day to clean out mucous and saliva, which was then suctioned out.  He HATED it!

1. Chest Covering:  Because Enzo was so young and his chest was so small, there wasn't room inside his open chest cavity for his swollen heart and all of the fluid that would be draining off - so they left his chest open for several days.  They first covered it with a sheet of flexible plastic.  They then added several layers of medicated gauze to prevent any infection.  The medicated gauze was covered with several more layers of dry gauze and then finally with what looked like a little corset, which was taped to the sides and tied together in the middle.  This dressing was changed twice a day, during which they had to kick us out, close off his room, and put on complete medical gowns and masks for the sterile procedure.

2.  Drainage tubes:  3 tubes (one large and two small) were inserted into the chest to drain off blood and other fluids.  This was collected in containers on the floor at the foot of his bed, which the nurses measured and recorded every hour.  He also had a catheter and a bag to collect the urine, which they also measured hourly.  It was important to measure the amount of fluid output because he had a lot of fluid in his lungs (pulmonary edema).  They were giving him medicine to help get rid of that extra fluid - but at the same time they didn't want him to get dehydrated either.  Also, they had to measure the blood drainage to make sure he wasn't bleeding too much from the surgery.

1. EKG lines: These lines monitored his heart rate and respiratory rate.  A monitor above his bed displayed these readings, as well as a couple of other ones, and would beep anytime a line was disconnected or if his rates were above or below desired levels.  

2.  Pulse Oximeter:  This device measured the amount of oxygen in his blood.  It emitted a little light which shined through his foot or hand to a sensor on the other side.  Because the laser light could cause burns if left in one place for too long, they rotated this between his hands and feet.  The recordings were also displayed on the monitor above his bed. 

3. (and 7.)  IV's: There were countless IV's used to administer medications and/or draw blood.  He had them in his hands, feet, and who knows where else.  The first day, he even had one in his head.

4.  ART line:  This line in his neck went into an artery.  Also used to administer medication as well as take readings on blood pressure and other stuff.

5.  Belly Button line (RA line?): He had a line going into his belly button/umbilical cord..  I'm not sure what this was for.  I do know he also had a RA line, which went directly into the right atrium of his heart.  I'm not sure if this was the same line, or if there was an additional line hidden underneath bandages and such.  The RA line was used to measure the pressures directly in the heart.  Enzo did have pulmonary hypertension, which is high blood pressure in the pulmonary veins and chambers of the heart.  The RA line monitored these pressures and the readings were displayed on the monitor above his bed.  Because this line went directly into his heart, we were unable to hold him until they were removed (days after his chest was closed.)

6. Temperature sensor: Monitored Enzo's surface body temperature to make sure he wasn't getting too hot from the heat lamp.

7. IV's: see above

8. Medication lines: Several different medications would be administered into one line (whether it was IV, ART, or RA).  These tubes shown here would all connect to one line and then be clamped off when not being used.  They would connect back to the drip machines where the medication was administered.

They closed up Enzo's chest on day 4 post-op and he was given a feeding tube.  This went from his nose to his stomach.  Up until this point, he had not been given anything to eat.  (He had been given lipids through an IV up till this point, which are basically liquids and fat for energy.) Once he got the feeding tube, they began giving him breast milk, which they fortified with formula to give him extra calories.  While we did begin giving him a bottle after he was taken off the ventilator, he still had the NG tube when he came home and used it until he was a month old. 

The Diagnosis

It took me a while to really learn everything that was hooked up to him, and it has taken me nearly a year to really understand his diagnosis.  When I first talked to James on the phone, it sounded to me like he said that Enzo had an extra chamber on his heart.  What the doctors explained to me (several times) turned out to be a little different.  What I soon learned was this:  Enzo was born with a congenital heart defect called Total Anomalous Pulmonary Venous Return, more easily referred to as TAPVR.  It took me several days to memorize that one.  To understand his defect, I had to re-learn some basic heart anatomy.  A normal heart looks like this:

The heart is divided into 4 chambers.  The blood (blue) is pumped from the heart to the lungs through two pulmonary arteries to pick up oxygen.  The oxygenated blood (red) then returns to the left atrium of the heart through 4 pulmonary veins (2 from each lung.)  The heart then sends the oxygenated red blood from the left ventricle to the rest of the body, which uses up the oxygen and sends it back to the right atrium of the heart. 

In TAPVR, none of the 4 pulmonary veins connect to properly to the left atrium.  They instead somehow connect to the right atrium, where the oxygenated blood mixes with the un-oxygenated blood.  In order for a baby to survive at all with TAPVR, he must also have another defect called an Atrial Septal Defect(ASD), which is a hole in the heart in between the left and right atrium.   It is through this hole that the mixed blood is able to travel to the left side of the heart and then throughout the body.

There are several subtypes of TAPVR, categorized based on where the Pulmonary Veins end up connecting to the heart.  Enzo was born with Infracardiac TAPVR, where the pulmonary veins join together behind the heart and drain downward, through the diaphragm and into the liver, where they connect to the hepatic (liver) vein, which drains back into the right atrium.  Enzo’s veins were also obstructed, or narrowed, which further restricted the blood flow and caused blood to fluid to build up inside his lungs.  

Here is a picture of what Enzo’s heart looked like when he was born:

(You can click on the picture to see a detailed description of the pictures.  Thank you Nanette at heartbabyhome.com for making this picture for me!)

So how rare is this?  About 1 out of every 100 babies born are born with some kind of congenital heart defect.  About 2% of these are born with TAPVR .  And 13% of all TAPVR cases are the Infracardiac subtype.  If I've done my math right (which is questionable), somewhere around 1 out of every 50,000 babies are born with Enzo's specific heart defect. 

Meanwhile...

Meanwhile,  I had taken a sleeping pill that night, while James went up to the hospital to be with our baby.  I went to sleep around 1:00 and woke up at 6:00 am to find out that my baby was in the middle of open-heart surgery.  Apparently James had called at 4am to tell me , but I was too asleep to take the phone call.  After I woke up at 6, I was told that my doctor would be coming in that morning to check on me.  If I was doing well enough, he would either discharge me, or give me some sort of “day pass” so that I could go to the other hospital and see my baby.  He came to see me around 10 and I was discharged from the hospital – less than 20 hours after giving birth. Melanie had come back that morning, and she drove me back to my house so that I could get changed. James met me back at our house and we drove up to the hospital together around 2 pm.

After checking in, getting my pink wrist band that proved I was Enzo’s mom, and getting the scrub down drill instructions (wash and dry, and then sanitize), I got to really see Enzo, and touch my baby for the first time.  

 

Dad's Story

I’m hoping that James will help me fill in what he went through that night, in his own words, but for now I’ll retell what he has told me as best I remember it. (I clearly don't do it justice - I'll make him tell it to me again soon so I can tell  it right.)

After James and his dad left to go up to the hospital, I think they decided to stop and get something to eat since James hadn’t eaten for 24 hours or so.  They grabbed something from the drive through and were headed to the hospital.  It was now about 10:30 or so at night and the two hospitals were about an hour drive apart.  They were still about 15 minutes away when they got a phone call from the hospital, asking them if they were almost there.  James says he drove like a maniac, running through every red light on Foothill Avenue, because he was worried that his baby was dying. I don’t know the details well enough, but I know that Enzo was in the NICU at Primary’s and the doctors were doing many tests to see what was wrong.   

If you know James at all, you know that he is not an emotional person.  Up until Enzo was born, I could count on one hand the number of times James had gotten tears in his eyes - and that was what he called crying.  But if you look in that last picture, you can see that it all changed the minute our baby was born.  James cried more that night, and in the following couple of days, than he had probably cried in his whole 20-something other years of his life combined.  He cried more than I did, and I was supposed to be some emotional postpartum wreck or something. But I love this picture, because you can see how much he loves his baby - and how much he worried about him that very long night. 

James and Alan waited in a waiting room until 1 or 2:00 when they were finally shown to a small room and told to get some sleep and that someone would come tell them when they knew something.  I don’t think they’d hardly fallen asleep when they were woken up and told that Enzo had a heart defect and would need emergency open-heart surgery in order to survive.  They got shown to a different waiting room, where a Nurse Practitioner came and updated them on the progress of the surgery every hour or so.

After Enzo was out of surgery, James and his dad got to go in and see him.  I guess the surgeon had come and talked to them after the surgery was over, around 11:00 or so.  They were then told that they would get to see him in 20 minutes or so.  20 minutes came and went, and James checked if they were ready yet – they were told to wait another 20 minutes.  Another 20 minutes came and went – they checked again, told to wait again.  Another 20 minutes went by and they were finally able to go in and see him.   

A Very Sick Baby

It was two or three hours later before we heard anything else. We were waiting in my room when a doctor came in and introduced herself as the head of the NICU.  She basically explained that our baby was very, very sick.  They had done everything they could to figure out what was wrong, but they still didn’t know.  They didn’t have the proper tools there at the hospital to figure out what was going on, so they were going to send him up to Primary Children’s Hospital.  Life Flight was already on the way. 

James had been able to go out of the room and get a glimpse or two of the baby while he was in the NICU – he and his Dad had even been able to give him a blessing – but all I had seen was the quick snap shot from across the room.  I was so grateful when the doctor told us that they would bring him into my room so I could see him before he left.  It took the Life Flight doctors a good half an hour to 45 minutes to get him stabilized and ready for the flight, and then they wheeled him down so I could see him.  The first time I really got to see my baby, he was inside of an incubator all hooked up to tubes and wires. 

I wanted to touch him, but didn’t ask because I didn’t know if I could, and didn’t want to be told no.  So I got to sit in my bed and look at my baby for a few moments before they took him away again.

James followed them out to the helicopter.  He said that the baby was so sick, he couldn’t even handle hitting any bumps on the way down – so the Life Flight doctors had to lift up his entire bed – incubator, cart and all – whenever they came to a tiny bump in the floor so that he wouldn’t be jostled.

After the Life Flight team left to fly the baby 30 or so miles up to Primary Children’s Hopsital, James came back to my room for a few minutes before he and his dad would follow them up there in the car.  We had everyone leave the room, and James climbed beside me in my bed and we just held each other and cried. 

We hadn’t decided on a name yet, but knew that we needed to right away.  We already had the middle name Lamar picked out, after a close friend and neighbor who lived behind James growing up, but we didn’t have a first name picked out yet. We really liked the name Enzo (we had occasionally called him Enzo before we even knew he was a boy), but hadn’t decided between Vincenzo and Lorenzo.  We also liked the nickname Vinnie, so if we named the first kid Lorenzo and called him Enzo, we could later have a Vincenzo and call him Vinnie.  We had been leaning towards Vincenzo, but it was a mouth full.  (Who in this English speaking country was going to ever know that the c in his name was pronounced “ch” because it was Italian?)  However, we had to decide quickly.  We didn’t know what was going on, how sick he was, or how long he would be here with us.  So we went with the one we had been leaning towards, and Baby Boy LeFevre became Vincenzo Lamar LeFevre.  (I know, we doomed him forever.  It took Grandpa LeFevre a week before he could say it right, and Grandpa Steve would ask, “What is his name again?” and when I would tell him, I’d hear a long pause and then,”….Oh.” I still don’t know if he can say Vincenzo.  As I say to many, we just call him Enzo.)

James left with his dad to go up to the hospital with Enzo, and James’ mom stayed with me until my cousin Cassie came later to spend the night. Melanie left when Cassie got there, and Cassie got to spend what I’m sure was a very uncomfortable night in a chair by my bed.  I’m very grateful to both of them for being with me that night so I wouldn’t have to be alone.  

It was around midnight when I was finally able to try and get some sleep.  My baby had been taken from me before I ever got to touch him.  It was sad, and it was hard, but the entire time I was filled with a peace and assurance that it would be okay.  I know that words won’t do justice to explain how I felt, but I know that I was being strengthened and supported by my Heavenly Father.  I just knew it would all be okay. As I lay there in bed, I prayed for my baby. I prayed that Heavenly Father would watch over him and keep him safe.  It came to me then that the feeling I had, that everything would be okay, might not be the same kind of “okay” that I would like.   Okay might be a healthy baby (like I wanted, of course), okay could be a minor sickness, or okay could be Heavenly Father taking my baby back home with him.  But I knew it would be okay, even if Enzo didn’t make it.  I cried as I prayed, knowing that I would be okay whatever happened, but please, just let me hold my baby first. 

I’m so grateful to the answer to my prayers that night.  Things are okay, and I did get to hold my baby.  And I don’t know if it’s a mom thing, or if it is because of my experiences with Enzo’s rough start to life, but I still can’t get enough of holding my baby.  I feel like that story, “I’ll Love You Forever” – I too go in at night, after my baby is fast asleep, and rock him back and forth, back and forth.  But unlike that story, where she says, “As long as I’m living, my baby you’ll be,” I get to know that he’ll be my baby not just while he or I are living, but for eternity.

He's here!

The long anticipated moment was finally here – and little baby boy LeFevre arrived at 5:29 pm on December 16, 2009.  They had warned me that I wouldn’t get to hold him right away – so I was prepared when they whisked him across the room before I even got to see him.  After cleaning him up and whatever else it is they do, they decided to take him to the NICU for observation to make sure everything was okay.  Thankfully, James asked if they could hold him up so that we could see him and get a quick picture.  I got to see this quick view from across the room:

Finally on his way

I had had some heart problems during the pregnancy, but the baby was always fine – and doctors didn’t really think there was much to be concerned about with my 150 beats-per-minute resting heart rate. I mean, who needs to walk across the room and breath at the same time, right? No big deal - it just made for a fun last 4 months of pregnancy. Needless to say, I was more than ready for this baby to get here. However, he had different plans. He probably knew what he was getting himself into and just wasn’t quite ready to face it. (Who could blame him, look who his parents are, right?)

I was 5 days overdue and the baby still showed no signs of coming when I was finally able to go to the hospital to be induced. I went in the night of December 15 at 8:00 at night. I had to stay over night so that they could get things moving and get the baby to come. They used some medication to speed things up, but then had to lay off when the baby’s heart rate started freaking out. I had to stay pretty still in a certain position and was put on oxygen a couple of times to keep the baby’s heart rate stable – but it didn’t seem to me like anyone was too concerned about that.

When the doctor finally broke my water, there was meconium in the water. There was a risk that the baby could have swallowed some of it, so they had a respiratory team standing by just in case. Overall though, it didn’t seem like anything was out of the ordinary. Not that I would know what was ordinary when it comes to having babies.