First Week Home

We got home a week ago and it has been non-stop craziness since we walked through the door at 2 a.m. Not only was jet lag a problem, but we had to drive to Nevada to pick up Sienna, catch up on a huge stack of mail, pay bills, wash mountains of laundry, stock the refrigerator and pantry, and countless other chores. We have also had a few doctor's appointments, had to go to the kid's school to check in, and daily homework catching up has been very time consuming! I think some of you may be beginning to worry about us, and I apologize for that. I can assure you that all is well.

When we left Boston, we had several appointments the last few days we were there. Initially, Nicholas had a chest xray and an EKG. After all the tests were analyzed, Dr. Marx found that Nicholas had developed "pleural effusion", or water on the lungs on his left lobe. Because of this, Nicholas's Lasix dosage was tripled in an attempt to get the residual fluid out of his body. We had to go back two days later to check again with another chest xray and a blood draw. The fluid was still there so Nicholas's Lasix dosage was to stay pretty high. Nicholas had to have his blood chemistry checked that day, too, because when a person takes Lasix at a high dosage for a long time, it can mess up the electrolyte levels in the bloodstream, which can lead to cardiac arrhythmia's. Fortunately, Nicholas's blood panel looked good but our goal is (still) to decrease the Lasix to a point that he can stop taking it. 

Another thing that we determined in Boston at the end evaluation was that they would consider Nicholas to have borderline Long QT syndrome. Since I had sent them as much information prior to surgery as possible, they were able to compare his heart rhythm for several years before surgery, immediately after surgery, and the weeks after surgery while we were in Boston. Dr. Marx said that he thinks Nicholas might not have true Long QT, but that because of all the years of surgeries on his heart it might have caused his rhythm to perform differently than if he hadn't had all the surgeries. He recommended that six months to a year from now, we have Nicholas tested with a Holter monitor and exercise stress test to determine for sure if he has Long QT or not. Long QT syndrome is often-times the reason kids will die of sudden cardiac arrest during PE or while playing sports. In a true case of Long QT syndrome, a person's heart rhythm would lengthen, rather than shorten, during exercise. Obviously this is an important piece of information to be aware of, but we cannot really test for sure until Nicholas is all healed up. There are also certain drugs that are routinely prescribed for things like acid reflux and antibiotics for infections that are known to lengthen a person's QT and because Nicholas already has a "borderline long QT", Dr. Marx said he should avoid those certain medications for his lifetime. 

Nicholas has seen Dr. Wright here in Sacramento once already, and we go again this week. He had us take another chest xray before coming in and sure enough, the pleural effusion is still there on the left lung. We will go in for another xray and check up later this week. The really good news is that Dr. Wright said that Nicholas's heart function looks almost normal and that his Left Ventricle is performing like a normal LV. Dr. Wright was almost in tears seeing our boy after all these years of PA bandings and the heartache that came with those 4 attempts at getting Nicholas ready for the Double Switch. He said he couldn't be more pleased at the outcome and that he is so happy that everything has turned out as well as it is so far. 

Our heart warrior is getting stronger every day, and is looking more and more like himself. Nicholas is still healing, but daily activities are becoming easier to accomplish and he's closer to his old self every new morning. I am so grateful that he is eating well and looking so good. He has asked me a few times if he will be able to run as fast as the other kids as soon as he's healed, and I have told him that the only way to find out is to go for it! 

CHD doesn't go away once the surgery is complete, but I am hoping for a very long lull in heart issues for my boy. After he is done healing from the Double Switch, he will only need to see the cardiologist every 6-12 months for check ups. What a blessing this amazing and quick recovery has been! I am praying that the fluid in his lung goes away and he will be all healed up very soon.

Lastly, I want to thank anyone who is reading this who has been following Nicholas's story and praying for him and our family. We are so fortunate to have the best families, friends, co-workers, and community. Knowing that there are so many people who love and care about us is a very powerful thing. I hope that I have the opportunity to give back some of what has been given and shown to us these past few weeks and months. Thank you. I am forever grateful.

Recovery Mode

We have been here in Boston, still in recovery mode. After a couple days inside, we started to feel cabin fever and decided to rent a wheel chair so we can get around without pushing Nicholas's limits. Of course, Nicholas does not like sitting in a wheel chair and I think feels very self conscious when we go out. But there is no doubt that the fresh New England air is good for all of us. Nicholas looks better every day, and if you didn't know he had open heart surgery less than two weeks ago, you'd think we were just regular old tourists with a kid in a wheel chair.

Nicholas is supposed to take a few "walks" each day, so he mostly goes up and down the hallway of the apartment. He has gone up and down the stairs, and is getting stronger every day. I know he lost a few pounds in the hospital, and that will take some time to gain back. His muscles are not as strong as before, either, but I know he will be back to his regular self in no time. 

Our first day out of the apartment on Friday, we mostly walked around Boston. We went to the finish line of the Boston Marathon, which takes place tomorrow. I am so inspired by the runners and hearing the stories of people who've overcome such trials in their lives. This year marks the 5th anniversary of the Boston Marathon bombing in 2013. There will be celebrations of survivors, first responders, and people stepping up to help those in need when this beautiful city was attacked by those low life terrorists. 

As some of you might know, I ran the California International Marathon in December as a way to acknowledge the heart journey Nicholas has been on his entire life. I wanted to have something to focus on while Nicholas's heart was getting ready for his Double Switch and keep myself from going crazy, thinking of all the possibilities of what could be...what was to come. I trained from April until the CIM in December, and it was therapeutic to my heart and soul. Being here for the Boston Marathon makes me want to do it again; and I would like to raise money for the Ethan Lindberg Foundation as thanks and a way to give back for this experience they have given us with this Home Away From Home. 

At the end of our day out on Friday, we ended up at our new favorite restaurant ever, Carlo's Cucina Italiana. We enjoyed their delicious homemade Italian food. We had the same waiter as the time before surgery, and when Nicholas and Lorenzo went to wash hands, I told him that Nicholas had had heart surgery since the week and a half ago when we were there. He could hardly believe it, but told the restaurant owner and she personally came over to us and told him that she thought he was such a strong and inspiring little boy. She told him to pick whatever he wanted for dessert and it would be on the house. Of course we were all stuffed, but we made room for a homemade cannoli and enjoyed it very much. Lorenzo had a seafood pasta plate, and one of the shells looked like a heart, so I couldn't resist taking a picture. 

On Saturday, we checked the price of Redsox tickets and it was totally affordable so we decided that we couldn't be in Boston without seeing a Redsox game. The high was 50 degrees, but with the wind, it felt like 30. We were completely frozen by the end of the game. We should have taken in the clues of the Bostonians, wearing hats, gloves, and heavy jackets with multiple layers. Even though we were under dressed for the weather, it was so much fun and we enjoyed every minute of it. Well, almost every minute. Hahaha...

Today, on Sunday, it was literally freezing outside, even snowing on and off. So we purposely stayed inside. The highlight of the day was officially meeting Jessica and Eric Lindberg and the marathon runners. The four runners are heart parents, a nurse in the cardiac ICU here at BCH, and a physical therapist whose life was touched by a heart child. We shared our stories and made new friends with people who have been on this journey before us. Maybe someday we will be the inspiration for a family in their heart journey.  

Post Op days 6 & 7

On Monday, post op day 6, we had another day of checking tasks off the list to get another day closer to getting discharged. The day consisted of taking walks around the unit, eating and keeping it down, drinking tons of fluids, checking Nicholas's heart function with an echo, and determining the next step based on how he did on all the tasks. Needless to say, Nicholas was able to not only walk around the unit, but he went downstairs (well, down the elevator 8 stories) and ate a piece of pizza from the cafeteria! He could check off a few boxes of things after that! 

He finally stopped vomiting on Day 6. I am absolutely certain that it was because he had his chest tubes out the day before and they stopped giving him hardcore pain medication. Nicholas has always been super sensitive with meds and I was so glad to see him out of pain and the cycle of sickness he felt to "keep him comfortable". He even stopped taking Tylenol on Day 6! No pain medication six days after open heart surgery....unbelievable to me! He is so strong!

Dr. Marx increased the Lasix (a diuretic) because he doesn't want Nicholas's heart to have to pump any extra fluid around his body. So that was one change. The goal is to keep everything in a perfect range with medication and that the Left Ventricle will "remodel" as it gets more comfortable with it's new job as a systemic ventricle. 

We waited much of Day 6 for an echo to physically see how the LV is performing. They said that it looks even better than it did in the CICU. I have not seen any reports to see the statistics, but Dr. Marx thinks Nicholas's heart is looking better each day. We may or may not have an echo next week at the follow-up appointment, depending on all the other tests we will do then. 

Yesterday, on Post Op Day 7, we were told first thing in the morning that we would be discharged by lunchtime! We packed up all our belongings, discarded the linens I had been using while I slept next to Nicholas's bedside, and got more "presentable" to go out in the real world. 

I should have known that Murphy's Law is always applicable at any given time in life. Our nurse came in at around 11:30 and started removing all the remaining band-aids, tape, and adhesive coverings from Nicholas's skin. She took off a gauze from his groin, which was covered with a clear, plastic cover. As soon as it came off, I noticed a very large bruise in his groin which reached almost up to his belly button. I never saw it at any point while helping him use the bathroom. He had complained several times over the past few days that when he coughed he felt pressure down by his groin. So we really don't know how long the bruise was there, whether it was "new" or if it had been there for a while. Our Nurse Practitioner had to go investigate what type of "line" had been in that vessel, to determine if it was safe for us to leave. 

Meanwhile, the physical therapist came in and did a few tests with Nicholas and she said his strength was much better than the day she came into the CICU. She said the only other thing she wanted to have him do was walk up and down stairs since his bedroom is upstairs at home. He went up a flight of stairs and back down with flying colors!

Dr. Marx stopped by to see us while we were finishing up with PT, and he reiterated that Nicholas's LV is performing so well. He gave me a big hug and I expressed my appreciation to him for reviewing Nicholas's case all these years without any compensation and with nothing to gain from giving us his expert opinion. He is truly an angel to me. I don't think I can ever thank him enough. He said the greatest thanks would be to receive a high school graduation announcement and to be included in all of Nicholas's life's accomplishments. I think that is very doable! 

Back to the bruise. By 1:30 we hadn't heard anything so we ordered lunch for Nicholas because he was getting hungry and it didn't seem like we would be leaving any time soon. They came in around 3:00 and said they wanted Nicholas to have an ultrasound downstairs to make sure there wasn't any damage where the A-line had been in his groin during surgery. (An A-line is a wire-like thing that is inserted into an artery and attaches to the heart muscle to measure pressure. It is much more accurate than measuring blood pressure with a cuff on the arm.) We didn't head down for the ultrasound until 4:30. It was 5:30 before we got back upstairs, and we then had to wait for the radiologist to read the images to give us the green light to leave. After more time hurrying up and waiting, it was determined that the artery was healthy and the bruise was not due to a damaged artery. We have to watch it and make sure it doesn't get darker, and it should start to fade with each passing day. We have to stay in Boston for at least a week so they said to let them know if we have any concerns with it. 

We finally headed down the elevator to the ground level at about 7:30 pm. I feel such disbelief that we were walking out of the hospital a week to the day after major, complex open heart surgery. It is still a miracle to me!







We grabbed a pizza from the cafeteria since it was already late and we knew Nicholas would enjoy it. We called a Lyft and got a ride back to the apartment, walking through the door of our Home Away From Home at 8:02 pm. 

Nicholas had a few packages to open from friends and family, and after eating three, yes THREE huge pieces of cheese pizza, went to bed well on the road to a full recovery!

Overwhelming Gratitude

I hope I can write the feelings I have spilling over from my heart. I feel like I have witnessed a miracle. I came back to the apartment and I immediately felt a sense of peace and gratitude for this trip to Boston. Let me explain.

Back in November 2017, Nicholas had a cardiac MRI which showed that his Left Ventricle was functioning at 38%. Normal function would be anything over 55%. Prior to his last PA Band, his function was 69%. Immediately after his PA Band, the LV was functioning at 58%, which was not too surprising due to the very tight band which was attached to his Pulmonary Artery. 

Nicholas's LV was being trained for the Double Switch, to become his systemic ventricle. At the time of his MRI, his LV was pumping at pressures higher than his systemic Right Ventricle. The LV had increased in mass. But the actual squeezing of the blood out of the ventricle had decreased to 38%. Anything lower than 35% is considered heart failure. 

We reached out to our surgeon at Stanford and he felt like leaving the LV in this state was not going to cause permanent damage. He wanted to wait until summer 2018 to look at the LV with an MRI. He felt like if the LV was within a few percentage points +/- 38%, that it would indicate that the LV would be able to take over systemically. But he wanted enough time to pass between collecting data to determine if the LV stabilized. 

Meanwhile, we reached out to the experts in Boston. They have reviewed Nicholas's heart data multiple times throughout the years. Long story short, after gathering reports, images, phone calls, emails, and such from Sacramento and Stanford, we were able to get an appointment in the beginning of June to come for an in person consultation and later a potential Double Switch. 

June felt safe enough to me. It gave me enough time to plan and to prepare. Until the phone rang one Friday afternoon in mid-March when they called from Boston and said that they felt like the situation was more urgent. Not an "emergency" but "urgent". We had 10 days to plan to fly across the country to a place we've never been to; to figure out what to do with our other kids; to arrange for our absence with our jobs; to figure out how we are going to afford it all. 

I didn't purposely wait until the last minute to find a place to stay, but some of the other details were first to be dealt with. We needed airfare. We needed to arrange to be gone from work. We needed to arrange care for our younger daughter and pets. We had to get the kids excused from school for who knows how long. 

The last detail to deal with was a place to stay. Initially we made arrangements at the Holiday Inn, which was relatively close in proximity to the hospital. It ran between $155-$215 per night. I put out my plea to the housing people at the hospital and they sent me a few links to foundations that help families with housing when they do not live within the immediate area. I was very skeptical that anything would work out because of the super-short notice. I literally filled out the request sitting in my car before my last day of work. It was a Wednesday morning. We were to arrive the next day, on Thursday. We had our Holiday Inn reservations to get us through until the following Monday when we took Nicholas in for testing. 

Everything literally fell seamlessly into place.

We have been so fortunate to have an apartment that has been granted to us at a fraction of the cost of the Holiday Inn, by the Ethan Lindberg Foundation. This is part of the reason for my overwhelming emotions when I walked in today. I have been at the hospital for most of the last 7 days, only coming to the apartment to shower and change for a few hours. When I walked through the doors this afternoon, I could feel the love that this foundation has for us heart families. This is not an easy road to be on. We have this little piece of home that is ours while we get the best care in the world. There is a good chance that I will bring my boy "home" to this apartment tomorrow. He is doing so well. But the Lindberg family did not get to bring their boy home from the very same place. He lived much of his little life in that hospital, and didn't survive to go home to his real home. 

We have been so blessed and I feel like we witnessed a miracle. It is a miracle because Nicholas made it through the most complex open heart surgery. He has healed in record time. His heart is functioning well. Everything fell into place for us to be here. 

I have been so blessed to work for an office that understands the importance of family. I have been told that I shouldn't worry about anything while I am gone. What a relief it is to know that I have their support. They even pulled together and sent us with a cash donation on my last day.

We were gifted our airfare by my dad. When I called my dad to tell him that we were leaving sooner than later, I heard my dad cry and tell me it would be ok and he would help any way he could. I am so grateful that he got us here on such short notice.

My sister and sister-in-law both came up with a plan on how they could help take care of Sienna. Mind you, they both live in different states than us. Either plan was a good plan and knowing that they both would step out of their responsibilities to take care of her meant so much to us. 

Our older daughter agreed to stay at our house and hold down the fort. It is a great comfort to know that someone is there keeping an eye on things. 

Both my mom and Lorenzo's mom offered to help with Sienna. My mom sent a sweet package to Nicholas, which arrived at the hospital before we did. She is very thoughtful and hasn't gone a day without checking in or letting us know she's praying for us. I know that Lorenzo's mom is there to help us if needed and that she has also been praying non-stop for his health and recovery.

Lorenzo's sister made up a super sweet and thoughtful set of letters and pictures for Nicholas to keep him entertained and for him to know how much he's loved. Those letters will be so fun and inspirational for him to look back on when he's older.

The school PTA president heard what we were going through and she personally brought over a cash donation to help with our expenses while we are away from home. 

We are so grateful for Dr. Marx and Dr. del Nido here in Boston. Dr. Marx called us multiple times in California to discuss his feelings about the next step for Nicholas and has been very prompt to be there when we have questions now and going forward. Dr. del Nido is so gifted in what he does - pediatric heart surgery - and we are truly blessed that he crafted a better version of our boy's heart. One of his associates said that it's like Dr. del Nido can see what others cannot see for each patient he works on, and we are so blessed and fortunate that he did such an amazing job for our boy. 

There are so many people across the country, and even other parts of the world that have been praying for us. I believe those prayers are what have helped get us through this part of our journey. Thank you from the bottom of my heart for those prayers. 

~Brenna 

Post Op Day 4 & 5

On post op days 4 and 5, Nicholas has had multiple things to check off during the day. He has been working really hard to clear a bunch of mucous out of his lungs, and that entails using an incentive spirometer, walking, and coughing. Because he has had a lot of mucous trapped deep in his lungs, he has been coughing, which leads to gagging, which leads to heaving and vomiting, which leads to a rapid heart rate, and that has been the biggest challenge for the moment. 

Every step he takes on his own, his body gets stronger and the mucous loosens up and he can breathe better. His goal is to get out of the room and walk around the unit 4 times per day. So far he has reached that goal and he's looking more like his old self with each new day.

Nicholas has lost some weight, much of that is due to the gagging and dehydration. He doesn't have a lot of wiggle room to begin with, so he's looking really thin. He is afraid to take his medicine, thinking that it will induce the gagging, and so it has been somewhat harder to get him to drink and eat and take the meds. Hopefully he is starting to get over that cycle and he will start keeping everything down.

On day 4 we had the Chinese family on the other side of the room I mentioned before. Part of the reason I haven't updated is because the little heart warrior was literally up. all. night. long. talking to himself. From what I could gather, he was hallucinating and manic and not making sense to his parents. Sadly, one of the hardest obstacles for them was the language barrier. The hospital doesn't have a nurse or practitioner who speaks Mandarin, so communicating was next to futile. They could call remote translators on the computer monitor, which is good for them, but for us, it was very disruptive and loud and hard to be on the other side of the curtain.  I haven't felt so exhausted up to that point. I have a lot of compassion for them because he reminded me of when Nicholas had the paradoxical reaction to Ativan after his 3rd surgery. Only the poor little boy next door did not come back to being his normal self and after multiple satellite translators and different teams trying to figure out what was going on with him, they moved the family in the middle of the second night. 

On day 5, it was very similar to the previous day, except that the plan was to take out Nicholas's chest tubes at the end of the day if he was able to get rid of some excess fluid. Nicholas was very anxious for this to happen because it hurts extremely bad, but the amount of pain he feels will be reduced to almost nothing. They gave him a cocktail of Versed to give him amnesia and Morphine to help ease the pain of the removal. The time came for this to happen, and they took Nicholas into a 'treatment room'. Lorenzo helped the Child Life Specialist keep Nicholas distracted, and I watched them first remove the pacer wires; then they removed the chest tubes. 

The chest tubes were probably each about 15 inches long, and they wrap around inside the abdominal cavity, providing an exit for all the fluid used during surgery and all the excess fluid a person retains after surgery. Nicholas has never had two tubes before; so I stand to reason that he has been twice as uncomfortable this time around. When the NP got ready to pull them out, she literally wrapped the outside ends around her hands and got right over the top of him and pulled, almost like doing an exercise machine. She had to pull hard to get them out of there. Our nurse was standing by, keeping the holes closed as the NP pulled, so that air did not get inside his abdomen. They sutured the holes up, covered them with sterile gauze, and covered it with a dressing. Needless to say, Nicholas feels much better since they've come out. 

After the chest tube removal, we had to have another chest xray to confirm there is no air in the abdomen. A few more walks around the unit and that concluded the day. 

Nicholas is making such good progress. We continue to pray that he will have good heart function and that he will continue to feel more energetic with each day.   

Post Op Day 3

Today has been a busy day, to say the least. It started with me waking up to Nicholas having had several "lines" removed overnight. (I guess sleeping on a hard bench-like cubby in the corner of the room is good for me, I've been sleeping like a baby, haha.) When I opened my eyes he only had his left and right side peripheral lines, which are regular old IV lines which they take blood for lab work and if they need to give IV medication. He still has the two chest drainage tubes (which are by far the most uncomfortable things), and his pacing wires coming out of his belly. 

During rounds, the doctors and nurses discussed the plan for the day, just like every day before. Much to my surprise, they suggested that today the goal was to transfer to the step down unit in another wing of the hospital. They said that it would be dependent on the echo, which is the first one Nicholas has had since surgery, and if everything looked good, he would move out of Cardiac ICU. They also wanted to see him drinking more fluids, get out of bed and sit in a chair, and go for a walk around the unit. His heart meds were all weaned by the end of the night shift, but they decided to add a medication to help lower his blood pressure. His blood pressure is within the normal range, but is slightly higher than they'd like to see, so it will probably be temporary, we hope. 

Nicholas got out of bed twice to use the restroom and instead of going to bed, he sat in the chair for breakfast. He ate a little bit better than yesterday, and Physical Therapy came in and tested his abilities and gave him some strength exercises to do. Shortly after, Occupational Therapy came in and asked him to show her that he could take his socks on and off, and that he could track her fingers with his fingers, things like that. She said she would like him doing as many of his daily activities by himself as possible, brushing teeth, combing hair, pulling his pants up and down in the bathroom. She said it is likely that he'll need some help because the chest tubes make range of motion very uncomfortable, but to try to have him do more and more. 

We took our first stroll around the Cardiac ICU today. It was really hard on my boy, but he did so great. He was pretty pooped when we got back, but he is getting better each day. I am simply amazed how good he looks. We have even seen a few little smiles here and there. 

The echo was later in the afternoon. We watched as the images popped up on the screen. After all these years watching so many echos, I have been able to semi-understand what we are looking at. It was amazing to see his new anatomy. It was clear to me that the ventricle we were looking at was squeezing differently than it has been for the past year, since the last PA band was placed. Dr. Marx came by just as the images were done being captured, and he asked for 3-D images as well. So the echo tech took a few more pictures and Dr. Marx said that it looks perfect. I have such a feeling of relief, knowing that the echo looks as good as can be, and the proof is in the pudding--Nicholas looks as good as can be, as well. 

An hour or so later, we got news that we were being transferred to the step down cardiac unit. The ICU has 32 beds, which is a huge wing; the step down has 44 beds, and is even bigger. We got settled into our new room and it seemed like we had the double-bed room to ourselves. Nicholas wanted to try to play a video game, but was sorely disappointed that they didn't have any games he liked or knew how to play. He tried a soccer game, but it just didn't do much for him. Next thing you know, we are getting a roommate. They try to match patients with kids the same age and gender. Our roommate is a little boy in diapers from China. I'm guessing he's about 4. His parents are both here and none of them speak english. It is somewhat of a distraction listening to their language as they converse, but it also reaffirms the fact that coming to Boston Children's is what you do when you want the best care in the United States - the world, even - for your child.

We took another 2 walks tonight, and have settled in nicely in our new room. I feel so much at peace being here. I feel like we made the right choice coming here and I have no regrets. Lorenzo was talking on the phone tonight and he made the comment that maybe our bad experience at Stanford last year was part of God's plan to get us here for our rare, complex Double Switch operation. I am in disbelief at how seamless this has gone so far. Nicholas is not out of the woods yet, but he is doing so well I wouldn't believe it if I didn't see it with my own eyes.  

I also have to say thanks to all those who have been praying for our boy. I think we have an army of prayer warriors out there and there is no doubt that it has had an effect on the way this has gone. So, from both Lorenzo and me - thank you. 

Post Op Day 2

Today has been a good day, for the most part. The morning started out with several bouts of nausea and vomiting; but by tonight it seems to be old business. Morphine is a drug of choice for pain, but it results in more of the upset tummy. They tried changing up the pain meds and the nausea has definitely decreased.

Nicholas has had a some pain issues today. He is not one to speak up and demand things, so by the time he is in pain it is like we are chasing it and it's hard to catch up to it. He has complained that his rear end hurts from being in a bed for three days now, and he absolutely hates the chest tube drains that are sewn into his abdomen. The nurse tried to transfer him to a chair to sit in for a change, but that caused a lot of pain from the tubes. He spent a lot of the day blinking back tears because he couldn't communicate what he needed. He is much more alert today and is aware of where he is and what he's been through. 

The chest tubes are probably one of the last pieces of discomfort that will come off of him. This is because they are actively draining the fluid from inside his chest post surgery and if there is any residual bleeding, that comes out of these tubes, too. They "pulled" his LA-line, which is a monitor that is sutured to the atrium above his left ventricle. It measures the pressure as the blood dumps into the ventricle below it. The sweet spot is in the high single digits or low double digits, around 8-12. They suspected that the line was not functioning quite right and that it was safe to pull it, but it could cause internal bleeding because it is attached to the atrium with the sutures. The chest tube output did not drastically increase after this procedure was done, which indicates minimal bleeding. As all the wires and tubes come off, Nicholas is one step closer to leaving.  

I would attach a photo of my boy, how good he looks, but he is watching Netflix on my phone and I don't want to bother him. He deserves to have any gadget that distracts him from everything else going on. 

Post Op Day 1

Lorenzo and I went back to our apartment late last night and purposely did not set our alarms, waking up when we felt rested. Thankfully, our phones never rang, and we slept hard, waking up much later than I had anticipated. We got ready quickly and walked back to the hospital, arriving around 10:30 am. 

Our nurse explained to us that Nicholas had some relatively unstable blood chemistry and some arrhythmias in the beginning of the night before but that now he was pretty stable and his blood chemistry looked to be improving. Apparently his glucose was very high, which required them to give him insulin, and he also had a very high level of lactic acid, which is treated with potassium, calcium, and magnesium, and it had started to improve by the time we got there. 

We missed the morning rounds, but we were told by our nurse that Nicholas would be extubated (breathing tube removed) over night or tomorrow morning. Throughout the day, Nicholas had good numbers, good pulses, and lots of other good signs that he was progressing better than anticipated. They started unparalyzing him shortly after we arrived. It was a beautiful sight when I saw his little toes twitch and his body move. They didn't want him to go from being paralyzed to fully conscious, so they put him on a medication that helps with pain, amnesia, and moderate sedation. After a few hours they could see that he was doing well and took him off of it so that when we were repositioning his body, his eyes shot open and we looked at each other for the first time since we left each other for surgery. Of course, he fell right back to sleep, but it was another encouraging sign that he is doing well. 

One thing that was unsettling today was that even while fully sedated, he went into SVT, which stands for supra-ventricular tachycardia. This means that there is a faulty electrical connection or abnormal areas of electrical activity starting in the upper chamber of the heart and a normal rhythm cannot be sustained. When this happens, the heart rate accelerates too quickly and does not allow enough time for the heart to fill with blood before pumping again. This episode lasted for about 10 minutes and it came on rather quickly. They had been talking about extubation when all of a sudden his monitor started chiming and next thing you know there are 5 doctors and nurses in the room trying to break the arrhythmia. They hooked his pacing wires up to this machine and tried to break the speeding heart rate, but it didn't work. So they tried again, and it didn't work. They were about to intervene with medication, but luckily he came out of it on his own. The medication they would have used would be a beta-blocker, and the MD explained that it would actually decrease the left ventricular function -- which is exactly opposite of what we want to do, now that the double switch is done and Nicholas has a left ventricle that pumps to his entire body. We want it to prove that it can do the job; not give it a free pass to be lazy. So we are praying that the arrhythmias work themselves out as the swelling and fluid output continues to decrease.  

About a half hour after the arrhythmia episode, the respiratory therapist came in and started prepping Nicholas to get the breathing tube out. Nicholas had started trying to communicate and of course couldn't. They had weaned him off of the ventilator and he was basically breathing entirely on his own. So they came in and removed the tube. Initially his throat kind of hurt, but he is now able to talk and he feels much better already. 

One of the biggest hurdles after his last surgery was his nausea. They are being extra careful not to do anything too quickly here so that we can avoid him starting a cycle of throwing up and not feeling well, which means no food or water for 4 hours after the breathing tube is removed. He literally just got to eat 2 little ice chips, and much to our relief, they stayed down without any heaves or nausea. After the second chip, he said he wanted to rest. I guess begging for water or ice for 4 hours wore him right out! He has been sleeping soundly for an hour now. 

We ran into Dr. Marx downstairs when we went to grab a bite to eat. He said he is pleased with how things are progressing. He seems cautiously optimistic. As soon as we returned upstairs, Dr. del Nido was making rounds and he also thought Nicholas looked very good. He wants him to rest and we will continue taking things very slowly. 

A funny side note is that all the nurses have commented on his great hair. Even Dr. Marx said something about it downstairs. For just having had a major open heart surgery, his hair DOES look amazing. <3 

Double Switch Operation

We got to the hospital at 10:00 this morning. We went straight to the 3rd floor, which is the surgical floor, and they got right to it. We changed Nicholas into his gown and they gave him premed to help him relax. He was quite emotional at the start of it all, but by the time they wheeled him out of the prep area, he was "fine", relatively speaking. 

The family waiting area is quite large, and we had plenty of space to get comfortable for the long haul. The surgical assistant, Dr. Kwan, came and talked to us and explained that the operative preparation will take about 1-1.5 hours. This is when they will place all the lines in his arteries to monitor him during the surgery, place the incision and get through all the scar tissue from his previous surgeries, cool his body temperature, start the IV's, etc. 

Dr. del Nido said that he expected the surgery portion to last about 5 hours. Nicholas was on bypass for that entire time. He said that the first part of the Double Switch is to do the atrial switch. This means that he re-routes the INCOMING blood from the veins to the opposite atria. He does this by using "native tissue" from Nicholas's pericardium, the tissue that surrounds the heart, and creates a "baffle" - which is like a tunnel - that takes the blood to the opposite side of the upper chambers of the heart. 

After that is complete, he starts the arterial switch. This is the part where the aorta and the pulmonary artery are switched to the opposite ventricle. He was able to switch the two arteries without any real issues, but when they "started" his heart again, the left ventricle struggled more than he would have liked to see it struggle. He said we kind of expected that to happen to some degree because of the super high pressure the left ventricle was pumping to get blood out through the PA band, and now that the PA band is no longer there, it kind of lost some of the oomph it had been used to pumping against. Also, the right ventricle had been pumping at a systemic pressure beside the left ventricle (causing a "stiff" septum, the wall between the lower chambers) and once the pulmonary artery was attached to the RV, the pressure automatically went down to a normal pulmonary pressure so the septum remodeled and the LV has encroached into the right ventriclular space. 

Dr. del Nido said that he saw some distortion of Nicholas's aortic tissue above the aortic valve, and that he would fix that part of the vessel as well when he did the switch. He said that he feels like the technical part of the surgery was very successful, and the first 48 hours will tell if the LV will be able to do it's job.  Because of all of this, they are "supporting" his left ventricle with quite a few meds and keeping Nicholas paralyzed and heavily sedated. They don't want his heart to do anything but get used to the new job that's been asked of it. They are planning on keeping him like this for the next two days or so. 

It is really hard to walk in and see your big boy on a bed surrounded by wires, tubes, monitors, and laying on a cold pack as big as he is. His body was cold to the touch and they said the heart beats better when it doesn't have to keep the extremities warm. 

Pray for our boy, that his heart does the job it has been asked to do, and that he does not have any unnecessary traumatic setbacks along the way. We decided to come back to the apartment because I don't think I would be able to sleep with all the commotion with them caring for our boy. That was really hard, to leave him. I pray that everything goes smoothly tonight until we get there in the morning. And I hope that he is truly sedated and does not know I left him. 

(Leading up to) SURGERY DAY

So this is the day we've been anticipating for years. YEARS. 

Now that it is finally here, I find myself in a state of disbelief, grief, hope, fear, love, faith, and did I say fear? 

Ever since Nicholas was diagnosed with L-TGA all those years ago, I have been consumed and obsessed with learning about the defect, studying it and teaching myself and learning from others, so that I could help make the best decisions for my boy. I have done my due diligence to be an expert in this defect and I understand the benefit of this scary surgery, which will hopefully provide my sweet boy a chance at a long and happy life. 

This has been literally one of the scariest points in my life. I have been trying to be strong so he won't see me cry, but I break down often. He has been very quiet and reserved. He cries tender tears at night. He is so brave. I know he tries to protect us by not showing how scared he is. But I know he is afraid. He knows his body will be open to the hands of his surgeon and his body will be kept alive by machines today. He knows he will feel pain and hunger and confusion that he can't control. He knows he will have a long road ahead of him to get back to the happy, sweet, carefree boy he is. This will change him. Inside and out. 

We are blessed and fortunate to be in Boston. Dr. del Nido is probably the best pediatric cardiothorasic surgeon in the world. He looks unassuming, but the people here at Boston Children's Hospital sing his praises. He does twice as many Double Switch surgeries every year as they do at Stanford. He is the one we feel is the very best to finish up this journey we have taken to make Nicholas's heart anatomically correct. 

Yesterday, we were at the hospital all day. Our day started with check in at Pre Op, where we filled out paperwork, signed forms, etc. We went straight to labs, which Nicholas hates, and they drew his blood to have baseline chemistry and to make sure he is not harboring sickness. We went to x-ray, where they checked to make sure his lungs were clear.  We had a consultation with the cardiac nurse, who showed us around and took vitals. We had an EKG. The Child Life Specialist pulled us aside and Nicholas got to transport the Stanley Cup from the basement up to the exit of the hospital (which was funny because he has never so much as watched a single minute of hockey in his entire life!). We got to take a break for lunch and then we had the. longest. echo. ever. Their machine took such clear images that even an untrained eye could probably decipher what they were looking at. It also took 3-D images, which was pretty cool. The echo was almost 2 hours long and they took over 200 images and videos. We then waited for a bit and officially met Dr. del Nido. He was very informed and answered all of our questions. He indicated that he felt confident that this was a window of opportunity. I'll explain more about that in a bit. 

We then met Dr. Marx, who has discussed Nicholas with me and Dr. del Nido all of these years. He is very experienced in L-TGA and has a wealth of knowledge that he shares and helped us feel some peace in the looming surgery. He is a very kind and empathetic man.

The last stop of the long day was the MRI with gadolinium contrast. The gadolinium is an important piece of data that would let us know if there was any damage to Nicholas's left ventricle from the very tight PA Band. This was almost a nightmare. Lorenzo and I had both decided and agreed that we would not consent to doing the Double Switch if the MRI wasn't similar to last November's and if the gadolinium showed damage. Nicholas hates needles and his only "good" vein had already been used for the blood draw earlier in the day. Not one, not two, not three, but seven attempts later were they able to get a vein for the dye contrast injection for the MRI. At one point in the fiasco, the MRI tech told us we did not even need the contrast and tried to get us to agree to do the MRI without it. I almost lost it. I told him we came all the way from California for this particular piece of information and we weren't leaving this hospital without a gadolinium contrast MRI. I would not have been able to sign the paperwork without 100% assurance that Nicholas's left ventricle wasn't as healthy as possible for this surgery. They ended up calling in a fourth nurse who got an IV in for the contrast. The MRI itself took almost 2 hours and they got the images they needed to assure the team and us that Nicholas is as ready as he can be for the Double Switch. 

Nicholas had to stop eating at midnight, so we went out for a real Italian feast at Carlo's Cucina Italiana. It was delicious.

And here we are.

A Looonng day at BCH pre-op

We were at the hospital today from 7:30 am until about 6:45 pm. We had every test done known to man today, and we have the green light for Nicholas to have the Double Switch to "correct" his anatomy. We have to get him a bountiful dinner right away, because he cannot eat after midnight tonight, but I promise I will update the blog when we get back. 

Thank you to all who have texted and sent messages today. Keep those prayers and good thoughts coming!