Surgery day was hard. As hard as I thought it would be. Last night on preop day I managed to get our Christmas tree down and then fell into an exhausted sleep around 9pm. At 5:30am the alarm went off and it was time to drag myself out of bed and start hurriedly  getting ready for the day. Since we had fallen asleep so early we had not managed to pack our things nor given Julian a disinfecting bath using special sponges from the hospital. So the first order of business was waking Julian and getting him bathed. Next was packing between mouthfuls of cereal. But after forgetting only a handful of things we were off in record time and headed into Boston by 6:30. On the way in Julian let loose a hacking cough that he had acquired a couple days ago. It sounded as bad as ever. Would he be able to have surgery today?

At admitting we saw a room full of anxious parents clutching their small children and awaiting their turns for vital sign checks. Gretchen popped off to get some breakfast since she did not have a chance to eat before leaving and sure enough, as soon as she stepped out, Julian and I got called in for an initial checkup. The nurses listened to his chest and discussed the raspiness of his lungs, but in the end determined that it was upper respiratory and would not stop the surgery. But then they couldn’t get a good blood saturation level to come through because Julian kept wiggling. He was hungry after not eating since 8pm the night before. I wish I had woken up at 4am and given him some pedialyte. His blood saturation was hovering around 85% and had the nurses concerned. Dad to the rescue. I scooped Julian up and snuggled him into the nook on my neck that he loves so much. Instantly he calmed down and the nurses got an accurate reading of 94%.

After some more waiting we rode the elevator with two other families to the surgical floor. We stepped into the pod 7 holding area on the third floor of Children’s Boston and each family was ushered off into a curtained room. Here we took turns holding Julian and he was a bundle of joy. Waving his arms in the air, cooing and enjoying himself. Nurses discussed the procedures again with us and anesthesiology gave us another review. The best part was that Dr. Emani took the time to come down and speak with us. He did not look tired at all from his work the previous day, but he was all business. He ran through the procedure, which we had already heard many times before, and I suspect he then headed off to prep for surgery. Moments later our time was up. Julian was so well behaved that the nurses decided that he didn’t need to be sedated before leaving us. We bundled him into a warm blanket and he looked back at us with his big eyes and we said goodbye. And just like that the nurse turned around and walked away with him.

The waiting is the worst. I was so stressed. Except for my wife, everything anyone did bothered me. She had learned over the years what to do and say when I get overloaded. Joking about stuff bothered me, talking about the surgery bothered me, asking me if I wanted food bothered me. We walked to a nice waiting area filled with families awaiting the outcomes of their children’s surgeries. Here I sat in my chair and did my Facebook status updates and wrote a couple blog articles on the surgery. The writing filled the timeless void between Julian leaving us and periodic updates we received from the nursing staff. The updates were crucial, critical and so very much appreciated. I can’t imagine agonizing through the entire ordeal without knowing that he had successfully been sedated, successfully incised and moved to a heart lung machine. Each Facebook status update I posted would immediately begin blipping with activity. Words of encouragement and ‘likes’ coming through. I have never been a big Facebook status update person, but this is one case where I loved it. In real time I could see how many people were anxiously awaiting Julian’s progress.

When Dr. Emani finally appeared I didn’t know what to do with myself. Here was the man who had just stitched my son’s heart back together. The news was all good. Julian’s heart was beating on its own, blood flow was great and there were no conduction issues. I don’t even remember what he said. I don’t remember whether I thanked him or not. The whole group of us, Gretchen, my mother, my aunt Carol, my in-laws were all quiet. After Dr. Emani left I hugged Gretchen and both of us kind of fell apart. I was so scared that Julian was going to die. That he would be a still and lifeless corpse on an operating table with his chest cut open wide and a dead heart inside. Without a doubt the worst part was the waiting. Afterwards my emotions flooded out and in their wake I was whole again. Reinvigorated and anxious to see Julian in the recovery room.

The recovery room was not nearly as scary as I thought it would be. You hear that he will be hooked up to a million machines and have tubes coming out of everywhere, and indeed he did. But you don’t care. All you care about is that the surgery was a success and that he is okay and that he will be okay. I took my finger and slid it into the palm of Julian’s one hand that wasn’t covered with iv’s or tubes. I did not expect the cold and lifeless flesh against mine. I reached out with my other hand to stroke his hair and couldn’t help but feel that it would be coarse and hard like straw. But as my fingers ran through his fine silky threads I knew it was him. As his body regained its normal pre-operative temperature his body would regain its vibrance. Julian was on pain medication, sedation medication and paralytic medication. So Julian could not move, theoretically could not feel pain and was asleep. We set up his crib with stuffed animals, blankies and his favorite singing seahorse that lulls him to bed every night. And with that our big day was done. A friend very thoughtfully called Bertucci’s across the street and prepaid for our dinner. All we had to do was show up and the manager came over to make sure it was us. She personally served us our meals (very quickly) and overall it was a great experience.

My brother Terry dropped in to say hi and drop off some things we had forgotten at home. As I explained how great everything was going medically I didn’t even notice that Terry had tears in his eyes until I turned around. I gave him a hug and it dawned on me that after such a big day I failed to realize all the emotion those who love us must have been going through. I had my moment to release all my tensions, to shed tears and to embrace the future, but others who love Julian maybe hadn’t. All those dozens of people sitting around their computer screens hitting the refresh button and anxiously awaiting the next status update must have had a collective sigh of relief and maybe even a shed a few tears when the good news finally came through. Here are those status messages again for all those who anxiously awaited them.

• 7:30 am – Waiting at inpatient admitting. Thank you for all the well wishes everyone!
• 8:30 am – We have moved up to the surgery holding area. Julian is waiting for the sedation team.
• 9:00 am – They took Julian away for the operation a few moments ago. We are now in the family waiting area. We are supposed to get updates from the nurses every ninety minutes or so. There were definitely tears shed as we watched them walk away with Julian bundled up in a warm blanket.
• 10:15 am – The nurse just gave us our first update. Julian has been sedated, had all his lines inserted and had his chest opened. All is well so far.
• 10:45 am – Julian has been successfully moved to a heart lung machine.
• 12:00 pm- Things are progressing. The surgeon is finishing up his work and they will start testing the effectiveness of the repair.
• 12:45 pm – Dr. Emani just came down and let us know that the surgery was a success. Julian’s heart is beating on its own and there are no conduction issues. In the next ninety minutes he will be transferred to ICU where we can see him.

I promised in my last post to get into what a surgeon, Dr. Craig, could detail of Julian’s heart procedure. When he came in and sat down Dr. Craig took on a conversational attitude and prepped us in a matter of fact way. Some things he said made my mind race with worry. For more complicated surgeries like this… Hundreds of stitches… There are several risk factors.

I am a man on a high speed training hurtling along the tracks. I know that most likely this train takes me to somewhere safe and beautiful, but I also know that this train can derail at any moment and destroy everything. In my arms I clutch Julian with his big blue eyes and wide-eyed stare. I look up and see the big red handle of an emergency brake and I imagine wrapping my fingers firmly around it and yanking it down. With a blazing screech of sparks and a momentous jolt forward that nearly throws me to the floor the entire train comes to rest. I look around and breathe a scary exhausted breath and everything is fine. Except I am not doing that. Instead I am holding tightly onto Julian and praying that this train slows to a stop where I can slide open the door with one hand while I clutch Julian in my other and let in the warm brightness of a blue day.

Let’s start with what is physically wrong with Julian’s heart. He has what is called a transitional atrioventricular septal defect(AVSD), a cleft mitral valve, a patent foramen ovale and a perforated foramen. The most serious of these is the AVSD which consists of a large hole between his atria (#1 in pic) and a small hole between his ventricles (#2 in pic.) If you look at the image below the ventricles are the big chambers are on the bottom and the atria are the small chambers on the top. To repair the smaller ventricular part they will rely on stitches, to repair the larger atrial part they will use a piece of tissue from the pericardium (sac that contains the heart) or if there is not enough pericardial tissue they will use a synthetic patch that Julian’s living tissue will grow over. An AVSD allows blood to shunt the wrong direction across the heart. This causes mixing of oxygenated and unoxygenated blood, making Julian’s heart work harder. In fact one side of his heart is already enlarged from this extra work. On average his heart is beating 150,000 times a day and working harder with each beat.

The blue is old blood entering the heart. It is then pumped to the lungs and comes back as red, oxygenated blood. Finally it is pumped back out to the body.

The cleft mitral valve is very typical with AVSD’s. It is found on the left side of the heart and controls blood flow from the atrium to the ventricle. When the valve was forming prenatally, it typically has two pieces that merge together. In Julian’s case they did not complete the merge and the result is a Y-shape. This can result in blood flowing backwards through the heart. To fix this the surgeon uses a few stitches to sew the Y shut. Typically they try and fix the problem 90% as opposed to completely fix the problem. This is because if they go too far it can actually impinge blood flow from the left atria down into the left ventricle. Here is a little graphic to give you an idea.

Mitral Valve

A patent foramen ovale is a flap that allows blood to openly flow between a fetus’ left and right side of the heart and bypass the lungs. This is because the mother’s lungs are doing the work of oxygenating blood. As the fetus matures this flap seals shut and neatly separates the chambers of the heart. In Julian’s case the flap did not seal completely. The surgeon will stitch it shut. Julian also has a secondary problem which is that the flap has small holes throughout it. These need to be repaired individually with a series of minute stitches. They cannot tell from the heart ultrasound, but if the flap is too thin then stitching these holes shut will not be possible because the tissue could tear. In that case a patch may be used instead.

Now that you know what is wrong and how they will fix it I can get into the details of what the surgery entails. Julian will be sedated via a mask and then prepped for surgery. This includes insertion of a catheter, arm and leg iv’s, and a breathing tube through the nose. His body temperature will be lowered to slow blood flow. Next they make an incision down his sternum and use a bone saw to separate the sternum down the middle. Retractors are used to hold his chest cavity open to access the heart. If Julian’s thalamus is obstructing access to the heart then it will be removed. The thalamus is similar to your thyroid, but is temporary and goes away by adulthood anyways. His thalamus may be used to make medicine for many other people.

Next they will sever his aorta and transfer his blood flow to a heart lung machine. This machine will be responsible for keeping Julian alive until the surgeons can finish the repair of his heart. Next an incision is made in his left atrium through which the surgeons will do all their stitch work. Dr. Craig said that they expect to use hundreds of needles and make hundreds of stitches during the course of the surgery. Once the stitches are complete they will begin to warm Julian’s body back up and test the effectiveness of the repairs. If anything does not work quite correctly they will make the fixes now. Next Julian will be taken off the heart lung machine. Finally his breastbone will be wired together (no MRI’s for Julian) and his chest closed up.

There are three major dangers Julian faces from the operation. The first is infection. Typically 1-2% of patients will have some type of infection. Hopefully these are infections of the skin or subcutaneous fat layer as opposed to an infection in the heart.

The next major danger is stroke. The clamping of the aorta can release tissue into the bloodstream which can travel to the brain. Strokes can be anywhere from minor to terminal. Because Julian is so young and should not have built up material in his arteries, this should be of minimal risk.

The greatest problem that Julian faces is conduction problems which occurs in about 5% of surgeries. The heart’s beating is controlled by an electrical system that flows around Julian’s AVSD (the holes in his heart). As Dr. Emani seals these holes he must be careful not to pierce the conduction system while still creating an effective stitch. The electrical system is completely unidentifiable from surrounding tissue, so Dr. Emani will need to rely on his experience and skill to effectively place each stitch.

Yesterday was a big day. We had Julian’s preop appointment where he was analyzed to ensure he was healthy and ready for his surgery. We started out at 7:30am in admitting and then moved up to a small office where a coordination team was prepped and ready to get things moving. I thought that like most doctor’s visits we would spend the majority of the time sitting around, but it was a whirlwind trip through the hospital.

We started by visiting radiology for a chest x-ray. I had to hold Julian’s arms up against his head and keep him very still for the x-rays. He cried a bit because it was so uncomfortable. Fortunately moments later he was his happy usual self.

Next he was brought to an exam room for vitals, height and weight. Julian had just finished a bottle so he puttered off to sleep and didn’t wake up through the entire procedure. He must have been having some pretty cool dreams. His blood oxygenation sat at a very good 99% and his blood pressure and temperature were normal. He weighed in at 14lbs and 5oz and was 26in long at seven months old. He is still a cute little button! Next up was the squid which consisted of a dozen or so wires with little stickies on the end. Unfortunately I don’t have pictures or videos of this because Gretchen had left to get some breakfast and coffee. By the time the squid was all stuck to him his little torso was nearly covered in the things.

Next we headed down to what would be the worst part of the day. Blood analysis. The last time we took Julian down to have his blood drawn he screamed bloody murder while Gretchen and I kept him pinned to the table so the phlebotomist could work. I had never seen him cry so hard or so loud in his entire life. Apparently dear old dad’s habits of rough housing with Julian have paid off though, because this time he was like a UFC fighter. The needle went in and squiggled around looking for a vein, but Julian manned up and hardly let out a peep. I was so proud of him.

From there we headed out to have a tour of the ICU and postop recuperating areas. The ICU was pretty intense. The monitoring equipment was enormous and took up three quarters of the room. Off by a window a thin and narrow mattress was where was one of us parents could sleep. For the other parent there is a family suite in another building with showering facilities and beds. After ICU he will go to a shared room in another wing of the hospital. I don’t plan on leaving the hospital for the next week so I am going to get very familiar with Children’s.

After a short lunch break (the food is decent, the selection is good and the price is great by the way) we headed back to a waiting room where we met with a nurse practitioner, anesthesiologist and a surgeon (but not our surgeron). The nurse practitioner went over Julian’s history and we could ask her questions about what the following day would be like. The surgeon went over the details of the surgery which I will cover in more detail in my next post. The anesthesiologist went over how Julian had strict food intake requirements until the next day and things like that.

We still weren’t done yet! We met with two nurses and chose to opt in for two studies. The first was strictly a monitoring study called TXA. The second was a study on whether treating blood sugar levels through insulin injection immediately post-surgery prevents infection. A similar study in adults found this to be true. They expect this to also be true for babies, but at the worst neither helpful nor hurtful.

Unfortunately we were unable to meet with Julian’s surgeon, Dr. Sitaram Emani, because he had an unexpected surgery and was tied up in it. My only thought after hearing that was I hoped he had enough time to get home and get some rest. I don’t want our surgeon to be overtired from a long day of work and then have to get up the next morning and do two more.