Did you know that 1 in 100 babies in the U.S. are born with a congenital heart defect? CHD's are the leading cause of infant death, and the #1 birth defect of live infants. The cause of most congenital heart defects are unknown, yet funding for research vastly lags behind childhood cancer and other genetic diseases.

Has your child been diagnosed with l-tga? I would love to talk with you. Please enjoy my blog and email me if you would like to join a network of parents of children with l-tga and other CHD's.




























Saturday, March 25, 2017

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. 

Thursday, March 23, 2017

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. 

  


Tuesday, March 21, 2017

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. 

 







Monday, March 20, 2017

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.


Wednesday, April 11, 2012

Home at last!!!


Nicholas leaving LPCH
Yesterday was a bit of a whirlwind....we were still in the CV-ICU until afternoon, when they informed us there were still no beds to transfer to upstairs.  So we were kind of in a waiting mode, not sure what was going to happen next.  Then before we knew it, they were getting us ready to DISCHARGE for good!  We left the hospital at dinner time, so by the time we stopped for a bite to eat and gased up the car, it was pretty late.  It was really nice to get home and sleep in our own beds.  Nicholas looks really good, for having just had open heart surgery a week ago.  I feel very grateful that everything went smoothly and we can get our family all home and start his healing together.