L-TGA Refresher Course

L-TGA is known by many names, the most common being L-TGA, or levo-transposition of the great arteries, CC-TGA, or congenitally corrected transposition of the great arteries, Double Discordance, and Ventricular Inversion. L-TGA is thought to be the rarest of congenital heart defects. L-TGA is unique in that the ventricles form on the wrong side of the heart, and the great arteries rise up from the wrong ventricle. 

Approximately one out of every 100 babies will be born with some form of heart defect. L-TGA accounts for approximately 0.5% of all congenital heart defects. It is thought that L-TGA affects 1 out of every 33,000 live births. So approximately 120-200 babies are born with this heart defect in the U.S. each year. It is estimated that approximately 5,000-10,000 people are living in the U.S with L-TGA. Keep in mind that the population of the U.S. in 2014 was 318.9 million people. 

L-TGA need not be confused with D-TGA, which is the more common form of transposition. D-TGA only involves the great arteries, the pulmonary artery and aorta, and typically there will be a hole in the heart where the red and blue blood mixes. The left and right ventricles are positioned on the correct side of the heart. Babies born with D-TGA will be born blue, or cyanotic, and require heart surgery in the first days or weeks of birth, or else they will die. In this surgery the great arteries are switched back to the opposite ventricle. 

In L-TGA, the lower chambers of the heart, the ventricles, form on the opposite side of the heart, and the great arteries rise from the wrong ventricle. In other words, two wrongs made it right. If two wrongs made it right, what's the big deal? Each ventricle is designed to do a specific job. The typical left ventricle is round and thick. It pumps under high pressure and pumps oxygenated blood out through the aorta to the entire body. The typical right ventricle is crescent shaped and much thinner than the left ventricle. It pumps under lower pressure and sends de-oxygenated blood from the heart through the pulmonary artery to the lungs to pick up oxygen. The right and left ventricles have certain characteristics that are unique to them. 

L-TGA often presents with other defects. Up to 70% of people with L-TGA have a ventricular septal defect (VSD), or hole between the lower chambers of the heart. Approximately 50% of those with L-TGA will have pulmonary stenosis in which there is an obstruction on or near the pulmonary valve, in turn making it more difficult for blood to pass through the pulmonary valve. 20% of L-TGA people will have dextrocardia, where the heart sits to the right of the chest. 
Other common problems include tricuspid valve leakage and complete heart block. The tricuspid valve is the most delicate valve in the heart and since it is the valve connected to the right ventricle, the L-TGA systemic ventricle, it has a high likelihood of failing in the lifetime. This will contribute to right ventricular heart failure and can lead to the need for a heart transplant. If the L-TGA person has  complete heart block, the person will need a pacemaker.  

Home At Last!! March 29, 2017

We got home late on Wednesday, March 29, 2017. We spent the day, yesterday, Thursday, unpacking and settling back in at home. I am writing this post on Friday.

On Wednesday, Nicholas and I woke up to shift change with a new day nurse, and then we pretty much were left alone for most of the morning. The nurse weighed Nick and he dropped over 5 pounds since the pre-op appointment. She also checked his vitals, and then unhooked him from the monitor and said he didn't need to be monitored anymore. 

The doctors came in for rounds and said they were pretty sure Nicholas would be getting discharged today. It was dependent on the echo, which was happening simultaneously. The doctors said that we would need a follow up appointment with Dr. Wright in Sacramento within the next week or so. I asked how we would know if there were signs of progress or failure and they said that Dr. Wright would likely be following Nicholas closely with quarterly echos. They also gave us some signs to look for that would indicate Nick's LV is struggling. They want us to watch for swelling in his feet, shortness of breath, any indicators that he is self-limiting more than he was before. I feel like it will be hard to see some of these changes until he is more back to his normal self; he is still very tired and very much in the healing phase.

Nick had one final meeting with the physical therapist. They walked down the unit and she took him to the stairs where she wanted to see if he felt comfortable walking up and down, since we have stairs and his room is on the upper level of our house. He did really well, but she showed me how to spot him going up and then coming down, just to be safe. She said she thinks he will do great once he gets back home, and she doesn't think he will need more therapy after he is discharged. She did suggest that he do the exercises she gave him so that he can regain some strength he lost while hospitalized. 

Leaving LPCH! 

We were finally discharged at about 3:00 on Wednesday. Of course we hit heavy traffic leaving Palo Alto, so our 2 1/2 hour drive took over 4 hours. We went out for a celebratory dinner and finally walked through our front door at almost 9:00 pm. It was sure good to be home. 

We kept Sienna home from school on Thursday and had a pretty easy going day, and now we are trying to get a routine started where Nicholas can start getting some school work done and have some structure to his days at home while his body heals and he gets his strength back. 

I will be praying that Nicholas's left ventricle does not struggle and that it starts regaining good function. I am working on a detailed description of what we are trying to accomplish with the PA banding procedure, and why this much tighter band is causing more concern this time around. Stay tuned....

CV-ICU Day 8, March 28, 2017

The team made rounds with us this morning. They think his LV is performing well enough that we don't need another echo today. They said the pacer wires can come out of his belly but first they want a baseline EKG to refer back to once Nick goes home and gets back to his normal routine. They said they think he looks great and that when we go home we will need to be on the lookout for minor changes in his activity level that might indicate his heart is struggling. 

Sometime later in the morning the EKG guy came in and hooked Nicholas up to all the slimy pads and wires. The EKG is probably the best cardiac test in terms of no pain or discomfort; even the little slimy stickers peel off without hurting.

We heard several times that we would be getting transferred out of CV-ICU today but each time someone came around they didn't have an answer as far as when the move to the step-down unit would occur. We played games, drew pictures, listened to The Beatles, and put a Lego together. We took a few laps around the unit and then we went and did a lap around the entire 2nd floor. 

The psychiatrist came back in at around 11:30 and said that she would like to spend some time talking to Nicholas, and she said Zo and I could stay or leave. Yesterday, Nick had told me he did not want to talk to anyone and was pretty upset that I thought it was a good idea. So today when she came in and said we could stay, we decided that was better for Nick to feel comfortable. She played a game with him to help identify how he was feeling and I was surprised that she was able to get him to open up. She said he is feeling the way most 10 year old kids would feel under these circumstances, and that having a support group of other kids in similar situations might help him be able to identify and cope with the emotions of what he's going through. So she said we should continue to have dialogue about what is going on with his heart in a meaningful way without making him feel like he's different, yet acknowledging that he is different, in the way of his heart. If that makes sense.

By about 4:00, the Physician Assistant came in and had Nicholas lay in bed so she could remove the pacer wires that were still attached to his belly. He had expressed earlier in the day to me that he was very nervous for the stitches to come out because they have to cut the knot right next to his skin. Needless to say, he was a champ, and barely winced. I asked when the lone remaining IV would be removed from his left wrist, and they said that it's almost superstitious to take out the IV before a patient is discharged, because it's Murphy's Law that they would need to give medication and then have to re-stick the patient. So as of this post, Nicholas has just one IV in his wrist and we hope that we will be discharged tomorrow and the darned thing can come out!

We waited a little while longer and at last they came in and said that we would be moving upstairs to the step-down unit. The step-down unit is a wing of the hospital where the cardiac patients go after they are no longer critical and are stable. The patients up there are still closely monitored by the nurses, but they are not watching them like a hawk. Our CV-ICU team gave report to the step-down cardiac team and Dr. Patel said Nicholas might possibly be her most favorite patient ever. She gave me and Nick each a hug and I felt like she really took good care of my boy. 

We arrived at our new room at around 5:30 and settled in. We have a double room with a bed for Nicholas and a couch like sleeping area for me.  Our roommate is an infant and the poor little guy has been crying non-stop. His monitors keep on going off and the nurse will come in and the dad will talk to the nurse about how the baby is in pain. As I started writing this post, I went out to the nurses station and asked if there is any other room available for us, because there is no way we will get any sleep tonight. Our nurse said he would talk to his supervisor to see if there is anything they can do for us. I hate being "that person", but my child is dealing with a lot of stuff, too, and we need to rest to heal. I feel a lot of compassion for the little baby and his parents. I have never had to deal with a crying infant post heart surgery, and I am sure they are completely frazzled. 

The charge nurse came in and said they could move the family with the baby to another room. She said that the doctor thinks there might be something going on with the baby and putting him in isolation might be beneficial, so the baby will be moving out, not us. The drawback is that sometime during the night tonight, Nick will get a new roommate. Apparently this new roommate is an older boy. I think we'll take it!

Tomorrow morning the team will do a complete survey with an echo and compare the LV and RV to check for any changes, good or bad. Pray for the LV to continue to hold steady and for the RV to stay stable, as well. If things look good, it is my understanding that we could be discharged to go home tomorrow.

CV-ICU Days 6 & 7, March 26-27, 2017

The last two days have felt a little like the movie, "Groundhog Day", where each day you wake up and repeat the prior day's events with a few minor twists until finally at the end, you get it just right and snap out of it and get back to reality. 

Sunday, March 26:  The day started out with the morning echo, which showed the LV is stable -- not better, but not worse. Nicholas was able to get out of bed and move around his room a little and in the afternoon he took his first walk from one side of the CV-ICU to the other side, where he became the roommate of another older boy. 

Prior to moving to the other side of the unit, Nick was in great spirits. He was playing the X-Box, racing cars and really starting to feel more like himself. Then the walk and room switch. The nurse said the older boy wanted to play the X-box and this totally dampened Nick's mood. He became very sullen and teary. The older boy coughs makes a lot of uncomfortable sounds and made Nicholas very uneasy. Thankfully that night, Nick slept hard, only waking up once to go to the bathroom and he got enough rest to give him the energy he needs to continue on the path to getting out of here.

Monday, March 27: Again, a morning echo. We didn't hear the results from the echo until late afternoon, which was that the LV is still performing at the same level as it has been for the past week. Nicholas took 2 1/2 laps around the unit, and he got to look at the flowering trees outside through the window. It was nice for him to see sunlight and get out of bed.  

Lorenzo and I met with two child psychiatrists in the afternoon to talk about how Nick is processing everything that he has gone through with his prior surgeries, this surgery, and the likely upcoming Double Switch surgery. Nicholas doesn't hardly ever complain and he seems to internalize most of his feelings. We worry that he is probably harboring emotions that he doesn't understand and that getting some of his feelings out to someone will help empower him now and in the future. We gave them a family history and told them how he only randomly ever talks about his heart, but the questions he asks are very deep and evident that he is thinking about his health and heart more than he verbalizes. There was not enough time for them to spend with Nicholas so they said they would be back tomorrow (which is now today) to talk with him. With that being said, yesterday when we got back to his room, he told me he did not want to talk to them. I asked why, and he said it's because he cries easily and gets very emotional. I told him that is okay, and that we think it will be nice to talk to someone who is impartial and can help him work through some things.

Nicholas finally got his "IJ" (intra-jugular) IV pulled from his neck and his "Art line" from his wrist. The art line required a splint-like contraption to keep it still and stable. Once that came out he was able to hold a pencil so he can start working on schoolwork and try to catch up on some of the material his teacher sent home with him. The only things left are the IV in his left wrist and his pacer wires in his belly. 

Day 5 CV-ICU March 25, 2017

Nicholas had a relatively uneventful night last night, only waking up to pee twice. He slept pretty hard until about 3:00 a.m. and then watched movies until I got up around 6:30. He had a chest x-ray at 5:00 a.m. and the cardiologists came in and said echo would be first thing this morning. Just like yesterday and the day before, we are hoping for the left ventricle to show signs of improvement to reassure the team (and US!!) that the left ventricle won't go into failure due to a too tight PA band. 

I also brought up this question during rounds this morning: If the left ventricle is struggling and the (septum) has shifted past the midline and into the right ventricle, does that mean that the right ventricle and therefore the tricuspid valve could be harmed by a too tight PA band? Remember, normally the right ventricle's job is to pump de-oxygenated blood at a low velocity into the lungs to pick up oxygen. Nicholas's right ventricle is on the left side of his heart and it pumps oxygenated blood out to his entire body under normal systemic pressure. The risk I referred to above is that the tricuspid valve normally works under low pressure, and if his systemic right ventricle has to work even harder than it has been for the last 10 years of his life, then it is also at risk for some degree of failure. So the team wants to be sure the band is not causing more problems on not just the left ventricle, but also the right ventricle. 

The echo tech was in the room at around 9:00. She took a bunch of images and the doctors said they would let us know as soon as all the eyes that needed to see them saw them. By noon we still had not heard anything. We were really hoping for improvement, not only because it means the LV is handling the new requirements imposed upon it, but also because it meant Nicholas could have a few  of the lines removed from his chest and finally get out of bed. He cried to us this morning that he wants to go home. I don't blame him, this has been really hard on him. He is brave to a fault; he never complains or cries in front of a doctor or nurse. I really worry that the biggest scar he'll come out of this won't necessarily be from his wounds on his chest. It might be from the emotional trauma he has been dealt. 

Dr. Patel came in at about 1:00 and said that on echo the LV looked ever so slightly better today than yesterday. So she sent one of the Physician Assistants to come in and pull the LV cardiac line. We had to wait for about an hour to make sure no fresh amounts of blood started draining from Nick's chest tube before it could be pulled. About an hour later, she was back to pull the chest tube. Holy moley, I will never get used to seeing that sight. The chest tube is about the circumference of my finger and wraps around the heart inside the chest. It is probably in there about a foot long. It drains out the tube into a calibrated box, which measures how much fluid the patient has drained from around their heart. Nicholas has not had much drainage for about the past 24 hours so they knew they wanted to pull the chest tube, but not until the LV cardiac line was out first. So thankfully both of those came out today. 

**A note about the left ventricle: Yesterday, March 24, someone had raised the amount of dopamine from 3 to 5. So when Nicholas had the echo this morning, the LV was pumping with more help from the dopamine than had been measured the previous mornings when the dopamine was at a 3. Dr. Patel said they reduced the dosage right before the echo so she felt that the heart was being measured consistently, but I can't help but worry that the "ever so slight improvement" today was actually residual dopamine helping the left ventricle just slightly more than with the lower dose.**

The nurse also removed 2 IV's from Nicholas's left wrist. That was a big relief to him, although he still has one more in there. 

Nick ate really good today, drank really good too. They weaned him completely off of oxygen and he is staying in the mid 80's to low 90's on room air. This will probably improve even more once he starts moving around more. His lungs are not working too hard just laying in bed and not even being upright. 

This afternoon, since all the lines had been pulled and he was doing well, the nurse helped him out of bed and into a chair, where he got to bring a t.v. and x box into the room to play games. He was so happy. He stayed in that chair for a couple of hours and we even saw him smile a bit!

At the end of the night, he took a lap around his room to test out his legs. He did great and I think tomorrow he will be walking around the CV-ICU and getting more exercise and getting some more of his strength back. 

Day 4 CV-ICU March 24, 2017

Last night after the shift change we watched movies until about 10:30 when our night nurse turned down the lights and told Nicholas and our neighbor boy, Samuel, that it was time for bed. I must have crashed pretty hard. I woke up several times throughout the night and Nicholas was awake, quietly watching movies. The night was uneventful. 

I woke up at about 6:20 and Nicholas had to go pee, so I helped him go. I asked him why he didn't sleep much and he said it was because he had to pee and I was asleep so he just held it. I told him to please wake me up if that happens again because it isn't good to hold it like that and that I am here to take care of you! 

I left from 7:00 to 7:30 like I have to during shift change. I went down to the cafeteria and bought Nicholas some scrambled eggs with cheese and a banana. When I got back to the room, I made sure it was ok with the nurse if I gave him some real food, and she said it was fine. He cautiously ate his banana and half the eggs! I think that really helped give him some much needed energy!

The echo tech came in around 8:30. She called down to the radiologist who reads the echo and then she called the head cardiologist to make sure they liked the images she got. They did not. She tried again and called again. She said that the head cardiologist would have to come up here himself because that was the best she could get. Right then, the cardiac team came by for rounds and one of them talked to the head cardiologist downstairs. While she was on the phone with him, Dr Hanley called her and said that the LV looks about the same as yesterday and therefore, Nicholas would have to wait at least until tomorrow to have the LV cardiac line removed. This means that all the other tubes and IV's and "lines" coming out of his body must stay as well. If that is not bad enough, it also means that he cannot get out of bed.   

Nicholas and his friend, Samuel

I tried to keep Nicholas happy, to look on the bright side, he could eat and order anything he wants from the menu. So we ordered a brunchish feast, with some breakfast and lunch. He ate a few bites of this and a few bites of that. His little neighbor guy, Samuel, even gave him a slice of chocolate cake which his mom brought him to celebrate him leaving the CV-ICU to go upstairs to general recovery. Nick's surprise of the morning was when Lorenzo walked through the door with balloons, cards, gifts, and lots of hugs for our boy. Nicholas cried some happy tears to see his daddy and it was obvious he was so happy to see him. (Lorenzo left on Wednesday after the chest tube was pulled so he could spend a few days with Sienna before more family could take care of her.)

The day went mostly well, with periods of being content and periods of sadness. I suspect that Nicholas is in discomfort, to say the least, if not pain. His blood pressure seems higher today and that leads me to think it's because he's agitated from all the tubes and lines coming out of his body. I remember the depression he dealt with after the last surgery and I'm sure it's partly PTSD and trauma coupled with the effects of all the drugs and anesthesia. He has a pump for pain and he uses it very sparingly along with a dose of Tylenol every 6 hours. He is not good about communicating details of how he feels so I'm not convinced that he doesn't hurt more than he leads us to believe. 

The physical therapist and occupational therapist came by to give Nicholas some exercises to work on over the weekend to keep his strength up. Poor guy just looks at those as pouring salt on his open wound. He wants to get out of bed and walk, not lay there and do leg pumps. He is really having a hard time. 

Later in the evening, I could tell that he was really getting tired so I encouraged him to take a nap. He slept hard and deeply, so that was good. He woke up again around shift change at 7:00 and stayed up until about 8:30, when his blood pressure started rising again. A few times it was really up there and the night nurse said the team called from the nurses station and said they wanted to lower his dopamine down, and I said I thought it was already at the lowest dose? I do not know when it was ordered to be raised up because I thought it was nice and stable yesterday. I even told the nurse today that I thought it seemed higher than it had been and it bothers me that I wasn't told that it was raised. 

Nicholas ordered a good dinner, which he never ate. He seemed more and more agitated and upset. Finally he fell asleep around 9:30 and he is sleeping soundly now. I am sure he's very tired from all of this and his body needs lots of time to heal. 

Day 3 CV-ICU March 23, 2017

Today started out better after the shift change at 7:00 when I came back and saw that we had the same nurse as yesterday, Cora. She is so good with Nicholas and she takes really good care of him. She was very happy to hear that Nicholas started peeing regularly and did not need to be re-catheterized. She was also sad to hear that he had such a rough night with all the nausea and vomiting. 

The blood pressure issue seems to have worked itself out for now, both the right and left ventricles are pumping efficiently and at normal measurements. The thing that has been challenging is the upset stomach and lack of food. My boy is very hungry, and so far has just had ice chips and water and he feels like he's starving. 

When Cora asked how the night went, I broke down. I couldn't sugar coat the way I felt about the nurse and I told her I do not want him to care for Nicholas again. She validated my feelings and said she would talk to the charge nurse tonight at shift change to make sure Nicholas gets a different nurse. 

The radiology department came by early this morning and took an x-ray of Nicholas's chest, and the respiratory therapist said that we could move Nicholas off of the high flow nasal canula and on to a regular canula that is lower velocity. Nicholas's oxygen saturations have stayed in the low to mid 90's all day today. He has also stopped the albuterol treatments. 

The doctors wanted to wait to allow Nick to eat or drink anything but clears until after the morning echo. The echo was at around 11 and I was told that the LV looked as good as it did yesterday; no better, no worse. At around 1:00 our nurse got permission to give Nicholas some water and saltine crackers. Shortly after a few small sips and 1/2 a cracker, he started heaving again. So they gave him benedryl to try to calm his stomach and to rest. They also gave him a patch that is used for nausea that lasts for 3 days. Simultaneously they put him on a pain medication that is pump style and Nicholas has the button which he can push when he feels he needs pain control. The dosage had to be modified after the first pump, as it made him very loopy and he started feeling really strange and it seemed to have an exaggerated effect.

Nicholas fell asleep until about 2:30. When he woke up he looked like he was in pain. He didn't answer me. I was under the impression that the doctor was going to come to remove that cardiac line that is taped to the outside of his chest and wraps around his heart inside. I knew he was going to need pain control for that so I asked him again if he needed pain control and that even if he wasn't in pain, he would need pain control when they came in to remove that line. I pushed the button myself in anticipation. Almost immediately he was completely unable to speak or make eye contact. Cora, the nurse, called the attending doctor into the room 911 style and the doctor ordered Cora to remove the patch from behind Nicholas's ear. Within like 30 seconds he was coming out of the drug induced stupor he was in. Again, it reminded me of the hallucinations from 5 years ago, only this time it looked like he was having a stroke or a seizure or something. The doctor said we should probably include that patch on his allergy list, but she said she wonders if a smaller dose would do the same thing. It appears that Nicholas is very sensitive to drug dosages and he doesn't need the usual amount. 

While I had the doctor in the room, I asked when the cardiac wire was going to be removed. She said that they actually changed their minds, that they were going to wait at least another day or two to see if the left ventricle was getting any better. This came as news to me, as the day before they gave me the impression that his LV looked even better than it did in surgery. I asked the doctor to explain to me what that meant, because I felt confused since they told me the LV looked good, just like yesterday. She said that the left ventricle struggled somewhat after surgery and that they always give dopamine to help the heart contract, and that yesterday during the echo it appeared that the contractions were better than they had been in surgery. Needless to say, the LV has not improved since then. So I asked what would happen if it doesn't improve? She said that they might just need another day or two to see more improvement or there is a possibility that they would have to go back in and loosen the band. 

Lord, please give me strength. 

Day 2 CV-ICU March 22, 2017

I am writing this entry a day late as we had a busy day and a rough night....but for the sake of sharing---

Yesterday morning started off with the team making rounds and letting us know that they were pleased with the way things looked on all the monitors. Nicholas had a few episodes of nausea from gagging on the tube and they determined that the tube could come out as soon as the echo confirmed that his heart was still holding up as well as the day before.

Echo to check LV function

 

After the echo determined that Nicholas was holding steady, the respiratory therapist came in and started prepping for removal of the ventilator tube. This tube goes all the way into the lungs and breathes for Nicholas with 100% oxygen pumping in. After the tube was pulled, Nick's voice was very raspy and he could only really whisper. After about a half hour, he started having "stridor", which is kind of like croup. It might have had something to do with the cough he had before surgery, or the air tube might have caused some laryngeal inflammation. At any rate, breathing became more labored and his oxygen saturations dropped dramatically into the 70's and 80's, even with a nasal canula. So the respiratory therapist ordered albuterol treatments to open up his airway and that helped raise his oxygen levels back to higher 80's and low 90's. The unfortunate thing about using albuterol is that it causes the heart rate to speed up and the blood pressure to rise. So in order to reduce those back to a safer level, the doctors prescribed morphine to follow the breathing treatments. The morphine lowers the heart rate and pressure, and this was the routine all day. As soon as the albuterol opened the airway, the blood pressure elevated, the morphine was administered, the blood pressure lowered, the airway tightened, again and again and again...

Yay, no more ventilator

During the stable periods of time, Nicholas had his rectal temp tube pulled along with his Foley catheter in his bladder. They followed this with a dose of lasix to get some of the excess fluids out of his system. Nicholas didn't feel the urge to pee for at least 3 hours and our nurse said he was not allowed to get out of bed until one specific monitor was pulled from his heart. This monitor is inside his chest sending information to the outside regarding the function of his left ventricle. Our nurse explained that it is a really great tool to have for the real-time information, but the patient cannot move out of the bed while it is in because it could dislodge and cause severe internal bleeding. 

Back to the pee issue. Our nurse was off duty after 6 hours of the Foley catheter coming out. She explained that if a patient has not urinated in 4-6 hours, they would have to re-catheter to get the urine out of the bladder. She had already been dealing with the blood pressure thing all day and did not want to cause Nicholas more stress which would further elevate his blood pressure. At the fourth hour, she tried to get Nicholas to pee. He could not get anything to come out so his nurse sprinkled warm water on his belly, we played a waterfall sound on my phone, we fed him some ice chips to see if the cold going down his throat would start the flow. But nothing worked. After 15 minutes or so, she suggested he try to relax and try again in an hour or so. It was nearing end of her shift, and she was worried the night nurse would have to re-catheter him so just before she left, she let him dangle his legs over the edge of the bed to see if getting him verticle would get that urine flowing. It did not work. She expressed her concern to the night docs and got permission from the team to give him few extra hours to get the urge to pee. Meanwhile his little belly was getting bigger and harder with fluid that had nowhere to go. This is when the shifts changed. 

Later in the afternoon we were told that Nicholas was changing rooms. So I packed up all our stuff and the nurses brought him into a shared room. There is a little boy a smidge older than Nicholas on the other side of the room. He initially had a breathing tube but it was pulled and now he's very vocal; quite the opposite of our Nicholas. 

The nurses change shifts every 12 hours and the parents are not allowed in the room during shift change. When I came back from the shift change, our night nurse was a male. He did not look like a nurse, more like a skateboarder with funky hair and noticeable scent of cigarettes. I felt like there is no way that Nicholas is going to communicate with a guy who looks like this, so I stayed in the room all night long. I was not very happy with the way he interacted with Nicholas and I felt like if Nicholas needed something, the nurse might intimidate him. He was unlike all the female nurses we've had, who are very nurturing and explain everything they are doing. At one point I had drifted off to sleep in my chair and next thing I know, the nurse is sitting Nicholas up to STAND up to pee. I knew that he was not supposed to get out of bed yet because he has that cardiac line in his heart that is taped to the outside of his chest. Nicholas lost all his color and looked like he was about to pass out or crumble to the ground. The guy nurse called the nurse in the other side of the room to help get Nick back in bed. They got him back in and thankfully the cord stayed put, This was the start of all the nausea. 

After Nicholas was back in bed, he started heaving and then vomited. He was nauseous all night long and threw up 3 or 4 times (I lost track because I would wake up from dozing off). The guy nurse kept giving him doses of anti-nausea medication but it didn't work, apparently. Poor Nicholas has not felt well pretty much the entire time he has been off sedation. 

  

Surgery Day / Day 1 CV-ICU

Pre surgery family selfie

We got to the hospital at about 6:30 this morning. Nicholas was acting very brave, like he always does. They took him back to the OR prep room, which is a huge open space with like 15 beds just separated by curtains lining all four walls of the room. We updated his medical history with one nurse, then the cardiac team started filtering in. His nurse anesthesiologist was very nice and she verified that his blood work came back normal, no infection with the lingering cough. 

The team took him back pretty quickly; we walked with him to the door and then he was out of our sight. I was feeling mostly nervous and still a little unsure that we just did the right thing. It is not any easier than it was the first time we did this over 6 years ago. Those first three surgeries were so stressful and we didn't really have any time to heal our emotions between them. But this time around we have had 5 years of "normalcy". Nicholas is also older and capable of figuring some of the things out, like the silly juice is not to make him silly but to prepare him to get IV's and then anesthesia for a heart cath, or in this case, cardiothoracic surgery. He has also asked questions like, "will I be dead when they open me up?" and "how do they cut through my skin and bones?". Those have been really hard.  

LPCH is really centered around making the families of the kids having procedures feel included in making decisions and let the families know what's going on behind the scenes. We had a case worker walk us to the all too familiar waiting room and we settled in for the next few hours. Lorenzo stayed with me for about an hour, then he left to grab our suitcases from the hotel since we left before 6 a.m. and didn't want the hassle of loading up the car. I stayed in the waiting room and a nurse called me twice to let me know that things were going well in the OR. I have to admit that I was afraid to update anyone until I knew our boy was safely out of the OR and on the road to recovery. 

Dr. Hanley came down to talk to us around 12:30. Every time I talk to him I feel an amazing sense of peace and calm. I had to ask him (again) if we were doing the right thing here, this 4th surgery. He said there really is not a "right" answer, but that making his left ventricle his systemic chamber when we do the double switch should provide Nicholas a brighter future than if his right ventricle gives up and starts to fail. He also added that if Nick's LV is "trained" in the future, we don't have to do the double switch right away, but having a strong left ventricle in the event that we want to proceed with the DS is better than a failing right ventricle and an untrained left ventricle. He added that the 3rd band was still in the same position as when it was placed almost 5 years ago and that it made the pulmonary artery "hour glass shaped" and looked really good. He explained that the inner layer of our vessels have ripples, which helps the blood as it squeezes through the arteries and veins. He speculates that Nick's inner layer of his pulmonary artery "remodeled" and smoothed out, which resulted in the physiologic result of decreasing the amount of pressure needed from the left ventricle to get the blood out to the lungs (because the inner layer got smoother).

Sleeping soundly

Nicholas has always had blood pressure on the lower side of normal. Dr. Hanley said that the gradient between the pulmonary artery and the left ventricle was around 40mmHg. In order for the left ventricle to take over systemic function, the gradient would need to be around 90mmHg and 100mmHg would be even better. In the operating room, Dr. Hanley was able to tighten the pulmonary artery band to about 100mmHg. He said Nicholas's LV was able to tolerate that load and that the next 24-48 hours will let him know if the LV is keeping up with the job that has been asked of it from the band tightening. He added that the ASD (the small hole between the upper chambers) has been passing (shunting) oxygenated blood to the unoxygenated side, but with the added pressure to the left ventricle, the blood started shunting deoxygenated blood to the oxygenated side. This is not that big of a deal, except that when Nicholas is very active or worked up and his blood pressure rises he might get a little bluish in color. 

We have a private parent's sleeping room tonight, not sure about tomorrow, but we'll take it one day at a time. I will most likely stay with Nicholas all night in his room anyway. 

A little tear escaped his eye

We have been with Nicholas since about 3:00 p.m. and so far everything has been holding steady, for the most part. The cough that was lingering has reared it's ugly head a few times while we have been here with him, and twice he has coughed so badly that all the alarms on the respirator were chiming and he vomited because he was gagging on the tube while trying to cough. Thankfully the suction is right near his head and we were ready to suck it out of his mouth. His eyes looked at us like he was suffocating and that was a bit scary. Twice. The coughing fits made one of the attending doctors suggest that they more heavily sedate him so he doesn't get worked up again if he coughs, but she wanted to give him Versed and I worried that because it is in the same family as Ativan, Nicholas would have the same type of scary hallucinations like he did once before. So we were able to calm him down with music and holding his hand and he slept soundly for a few more hours. After a third coughing attack, the nurse gave him more morphine and he has been asleep for about an hour now.

The plan for the night is to keep him comfortable and calm. He will stay sedated at least until tomorrow when they can do an echo and check the pressures to make sure that the gradient has stayed relatively the same and to make sure the left ventricle is handling the load and not showing signs of stress. 

Nicholas is seriously my very own Hero. 

 

4th PA Banding Pre-op

Here we are again.... almost 5 years to the day. We had a busy day of  preoperative testing today.  We got to the hospital at about 7:15 this morning and headed straight to the heart center. They got Nicholas' vitals: blood pressure, pulse ox, height, and weight.   First up, the echocardiogram, which both an echo tech and a cardiologist had to come in to try to get good pictures of Nicholas' heart. The cardiologist (whom we have never met before) said that the PA to LV gradient is about 40 mmHg. She also said that his PA band is very hard to see on the echo, so it is not necessarily the most accurate data. After that, Nicholas had an EKG. I asked the EKG tech if his EKG appeared normal and he said it appeared that his right ventricle might show some signs of dilation, but that is "normal" in L-TGA. 
Then we headed to the lab where Nicholas had 4 vials of blood drawn. He barely even flinched. We then went to xray and they took two views of his heart in his chest. Then it was back to the heart center to meet with the cardiac and anesthesia teams. 
The PA-C from the cardiology team was new to us today. She was charmed by our boy and said he had great hair and a charming smile. She went over with us the surgical risks and explained that this time around since there is more scar tissue each time they go in through his sternum, that they will have by-pass ready in case they need it. That really freaks me out. 
The anesthesiologist NP was the same one we saw in March and she was optimistic that all would go smoothly anesthesia-wise. 
The only thing that had them minorly worried is that Nicholas has a cough that he's had for a few weeks now, and they wanted to make sure it isn't an actual sickness. I think it is probably allergies, but they said they will check his bloodwork and verify that he is healthy before they make the final call that he is good to go for surgery in the morning. So far we have not heard from them.