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.

Friday, January 26, 2018

The Truth Hurts Sometimes

Echo at Dr Wright's Office in Sacramento
It has been a while since I've posted on my blog and there is much to update. I have "written" many posts in my mind but putting my thoughts on paper make them permanent and there's no denying I've had them once they are out. 

The last time I posted, Nicholas was home - recovering from his last PA banding surgery - and I was home with him, tutoring him from the school he was missing and trying to keep his spirits up. Truth be told, I found myself kind of sinking into a black hole and really suffering watching him suffer and knowing that there's nothing I can do except love him and advocate for him as we try to 'fix' what's broken with his heart. It was during this time while I was away from work, and it was mostly just him and me, that I had one of those ding,ding,ding! moments. I realized that unlike other diseases, such as some cancers or type 2 diabetes, there is no "we're going to beat this!" or "I'm changing my diet and exercising more so I can beat this!". I had a very defining moment where I realized that congenital heart disease could win this battle and I could lose my son. 

It is something that I acknowledge now...but I cannot wrap my head around it. Knowing that Nicholas having an eventual Double Switch will not actually FIX his heart. Knowing that everything we have tried to do to prepare him could backfire and he could struggle even more than he already does. Knowing that there are random complications in the operating room. Knowing that his heart could give up and he could need a heart transplant. Knowing that he might not have a match if he needs a heart transplant. Knowing that he could enter the gates of heaven before me and that's not how things are supposed to be.

I am going to start from where I left off and attempt to explain everything that has transpired since April of 2017. Here goes:

Wednesday, April 5, 2017

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.  

Friday, March 31, 2017

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....

Tuesday, March 28, 2017

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. 

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.