This is the story of our Little Heart Warrior, Joseph James Sbicca. Follow his journey as he comes into this world with a broken heart.
Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis.
Our little man is completely off of oxygen and most medications now. He also got the IV lines out of his neck today. He is only getting Tylenol when needed and he is being a champ!
Feedings aren't going super well. He is not latching on to the bottle and sucking like he needs to. They had to put the NG tube back in yesterday. He only gets about 10-20ml down at each feeding with the bottle and the goal is now over 40ml. What he doesn't take from the bottle he gets in his tube. Although we are a little frustrated, the nutritionist is very pleased with what he is doing now. She would rather him take what he can in 20-30 minutes, then get the rest through the tube so that he doesn't get frustrated, too tired, or associate feedings with being an uncomfortable time. So we are being patient... They have ordered a consult with the occupational therapist to see if they can assess any issues or gives us some tips.
If the feeding thing was his only issue, then we would be out of the ICU and into the step down unit now, but he still remains in heart block. So we are still here...
The heart rhythm specialist comes in to see him much more frequently now. She explained to us yesterday that the nerve that connects and regulates the pumping between the atrium and ventricles could have been damaged in surgery. They usually wait about 7 days to see if a heart block will work itself out or if the nerve will recover or repair itself. Thursday puts us at 7 days with heart block, so a pacemaker was brought up as a solution. Adding a pacemaker would be an additional open chest surgery, but not open heart, and they would go in through his current incision. They would place the wires on the heart and test which spots worked best, then close up his chest, and put the pacer box in his belly area. This will obviously require more recovery time, tubes that we just got out being put back in , and so on. Kind of a step backwards...
Today the same heart rhythm specialist spent quite a while playing with his current pacer to see if she could find a rhythm that would indicate that the nerve was working. She was really unsure, but she did measure what looked to be like two normal heart beats. Two is better than none, so she was slightly hopeful that this meant the nerve may not be completely dead. She is going to continue to monitor him frequently today to look for more signs.
We were scheduled for a pacemaker to go in tomorrow, but now she wants to give it a little more time and wait until at least Friday. But we are walking a rather thin line. We (the doctor and Jamie and I all agree) don't want to put a pacemaker in if he doesn't need one, obviously. But if he will need a pacemaker, we don't want to wait too long to put it in. His current wires will not really do well past the 10 day mark, so we can't wait forever. Plus, the sooner he gets the pacemaker, the sooner he can start recovery.
A pacemaker is obviously not what we want, but we do want what is best for him, I just wish we weren't down to the wire and the doctors didn't have to make this decision with the pressure of time looming over them.
It's a great sight to see the ventilator on STANDBY, but it's an even better feeling when the ventilator gets rolled out of the room! Yay! No more tube mean Joey now has audible cries and coughs and sneezes. I never thought I would love to hear our baby cry so much!
Joey had a couple of breathing trials this morning and he passed with flying colors so it was time to extubate. We knew that Joey had been congested and full of boogers, but the snot rocket that they suctioned out of his nose after the tube came out was one of the biggest all the doctors and nurses in the room had seen! It was a "man-sized booger" for sure. Joey immediately did great with the extubation and has continued to thrive thoughout the day. He now just has a nasal cannula pumping just a little bit of oxygen in, but they think they will wean that away by tonight or tomorrow. With the breathing tube, the NG tube (feeding tube) in his nose came out as well. He was given IV nutrition today before and after the extubation so that if he gagged and vomited it milk wouldn't aspirate into his lungs. So the goal was to try to start bottle feeding in the evening. And if he didn't take to it well enough they could easily put the NG tube back in.
Joey also came off of a lot of medicines today. They stopped the fentanyl completely, which was what kept him sedated and comfortable with the pain level. The good thing is that he is currently not showing any withdrawal symptoms. If a baby has been on fentanyl for 5 or more days they are automatically put on a withdrawal plan. But Joey had only been on for 4 days so they were just going to monitor him and see what happened. He remained pretty content until about 6pm and he was pretty fussy so the nurse gave him a tiny bit of morphine. Enough to calm him but not enough to make him go to sleep. Today was actually the most awake and alert he has ever been. They also stopped his dopamine and a couple of other medicines as well. Our day nurse thinks that he will get the IV in his arm to by tomorrow. Jamie got to hold him for quite a while today while he was awake and it was so nice to hold him without the breathing tube. Jamie also got to "Lion King" the baby which he has been dying to do since he was born! (If you know the Lion King, you know what we mean by this! Haha! Just think Raffiki and Simba!)
At 5 o'clock it was time for his first feeding! Mommy got to hold him and try his first bottle feeding. The goal was 21ml. He latched on really quickly at first and got about half of the bottle down, but then he lost interest and just played with the bottle. He only took 16 of the 21ml. But that was just enough to not need the tube out back in. His 8 o'clock feeding didn't go so well either. He really seemed disinterested with the bottle and it took him a long time to get it down. Now was the time that he wanted to try to go to sleep. With Daddy's help he got almost all of his 23ml, but it was a struggle and he didn't seem to enjoy it very much!
So with all of this going on, we are hoping that the feeding goes well tonight so that the NG tube doesn't need to go back in. And if his IV comes out soon then all he will have left are his pacer wires, PICC lines in his neck and leg and his nasal cannula. The less tubes and wires, the better. He even got to graduate to the big boy crib, or what I like to call the baby cage! His scar is looking great so far and tomorrow we might even be able to put a onesie on him!
Our big goals for the next couple days will be to get better at feeding and to try to get this heart block to self regulate. They aren't talking about a pacemaker yet, but that is the likely solution if it doesn't resolve. But they are going to give him a few more days. After those two things are doing better we could then move to the step down unit and we are one step closer to going home! We are so proud of his progress!
What we have learned about Joey: He is a typical boy! He is filled with boogers and slobber, likes to stick his tongue out at people when they are poking and prodding at him, and he blows some pretty awesome spit bubbles.
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Joey's road to recovery has begun! There have been a few hiccups, but the doctors tell us that none of it is really out of the ordinary and these are things that they see a lot with babies like Joey at this point in treatment. I think the best way to share everything is to break it down, timeline style, so you can get a better picture of what has been going on in the last few days.
Wednesday Night
Wednesday was pretty uneventful compared to Tuesday night and what was to come on surgery day. Except that Grandma and Grandpa got to come and visit! We were told after asking a couple nurses earlier in the week that they can make exceptions to the no visitors rule, but it really depended on who the charge nurse was and they recommended waiting until right before surgery to ask and that would give us the best shot at getting them up here. My parents still had not gotten to meet Joey, and they had only seen him very briefly in his transport carrier while leaving Huntington Hospital on the night he was born. Up until now we had tried our best to send pictures and videos and Facetime so that they could see him. So on Wednesday morning we asked the nurse if she could let the charge nurse know they we wanted to speak with him or her regarding having visitors. At about lunch time he came to do his rounds and we were able to ask. Because Joey was getting a new roommate that day, and because another patient on the floor had recently gotten a viral infection he had to get approval. He said it was about a 50/50 chance, so we waited. We went downstairs for lunch and he called us to tell us we could have them come for about 20-30 minutes if they came pretty soon. So they got in the car and were here in about an hour. They finally got to meet him in person, but in true Joey fashion, he slept the whole time! I still think he was excited to see them.
During the evening a doctor from the surgical team came to speak with us and get consent for the surgery the next day. Until now we had given consent for a few procedures like the PICC line, the balloon septostomy and a blood transfusion. It's always the same, they sit you down and ask you what you may already know and understand regarding the procedure, explain the process of the procedure in great detail and then go through ALL of the risks associated with it. That's always the hard part; knowing that your child needs to have this done, there are no other options, and have to sign that you understand that all of it could end in some catastrophic way. They do try to put you at ease and give you the likelihood of those things happening and with anything in life, you have to assume there is a risk.
This surgeon explained Joey's condition again and reviewed what the surgical plan was. He explained both the Arterial Switch and the Nikaidoh Slide and said that both were options. He also explained that if the slide was done they would use a homograft from a cadaveric donor, and that it would need to be replaced a few times during life as he grew, but maybe not always with an open heart procedure. We were slightly confused because, again, we were hearing different things from different people. Although Dr. Herrington had just told us that the Nikaidoh was what they were going to do, this surgeon explained that once Dr. Starnes got in there and saw what was going on, he would then choose which route to take, and that it may not even end up being a true Switch or Nikaidoh but he may just reconstruct and repair as he sees fit. He touted Dr. Starnes accolades and surgical abilities and let us know that Joey was in the best possible hands. (Which we already knew!)
Joey was the second surgery on the schedule for Thursday which meant that, depending on the first surgery, he would be prepped and taken to the OR between 11 and noon. We could plan that anesthesia would take about 30 minutes and the surgery, with both surgeons working on him at the same time, would take about 3 hours. He then would be switching rooms in the CTICU (finally to a private room!) for recovery.
After we signed consent, we got some more cuddle time and got to hold him for quite a while. He slept the whole time, but it was nice to be able to spend some time with him in our arms. We packed up for the night and went to the apartment close by to get some rest before getting back to the hospital early the next morning.
Thursday Pre Op
Jamie and I got to the hospital bright and early Thursday morning after a pretty restless night of sleep. (Plus, the bed at the apartment was SO uncomfortable, it didn't help). We spent most of the morning at his bedside. He was awake for a lot of the morning, just looking around at us, but he was also pretty cranky. The nurse checked him all over, she was concerned with one of his IVs, but checked it twice and it seemed to be okay. He had been congested a lot and she thought he just may be clogged up. He was doing a lot of rooting and so we grabbed the pacifier for some suckling practice. He loves it and is quite a professional now! This is a good sign for feeding later on!
At about 11 o'clock the nurse started his antibiotics and said that we had about a 30 minute wait. And at about noon the anesthesiologist came in to roll him to the OR. We were able to follow him through the maze of hallways until we reached the last set of doors. We said our "see you later"s and had the long walk back without him. (As I just proofread this last part, I realize I made it sound pretty easy and casual. Just know that it was not. I was a crying mess the whole morning. I was so scared and nervous and felt so bad for what our little boy was about to go through.)
Jamie and I gathered up our things from our room so that we could move into Joey's new room down the hall once he was in recovery. Since arriving at CHLA, he was placed in a double room because they were so filled up in the CTICU that they reserved most of the single rooms for post operative patients. He was finally moving to his own room, but they were still sure of what room he would be moving to, so Jamie and I took all of our things to the second floor waiting room and parked ourselves there. The nurse said that the surgeons would look for us there to give us the after surgery update. They said that they typically don't give updates throughout the surgery because it moves so fast. We could then expect to be called by the nurse about an hour after that to let us know that he was settled back in his room and we could join him. So we had about 4-5 hours to sit and wait. The nurse suggested we go downstairs or out to eat, but I couldn't leave that waiting room. Luckily, because of the visitor restrictions we had the whole family waiting room to ourselves the majority of the time. We ate the lunch that we had packed, and I even was able to take a few little naps. But I think I woke up to look at the clock about every 15 minutes or so. Time couldn't have moved any slower.
At 3:15 the door to the room opened and I jumped up to see Dr. Starnes with a pleased look on his face walking over to us. He then proceeded to give us the best news ever! Joey's surgery had gone VERY well. He was able to repair the holes, do a simple switch of the arteries and coronaries and remove some muscle tissue around the pulmonary valve to unblock it. He was confident that the pulmonary valve would be able to sustain Joey, even as he grew, so a homograft or valve replacement was not needed! We breathed a HUGE sigh of relief. This meant that, unless there were some unplanned circumstances that came up later on, Joey would not need any follow up open heart surgeries! To know that we would not have to go through this again 2-3 more times was the biggest weight off of our shoulders.
Dr. Starnes told us that Joey was off the heart-lung machine at that point and that Dr. Herrington was finishing up the procedure. Joey's chest would be left open to allow for swelling and would probably be closed on Friday or Saturday.
For the rest of the day we had doctors and surgeons (some of whom we didn't even know) come up to us and tell us that they saw or heard about the surgery and how impressed they were with what Dr. Starnes did and the result. I didn't know that a surgery could be described as "beautiful" but that is how most of them described it.
After Dr. Starnes left us, Jamie and I waited patiently for a full hour and a half before getting too antsy and calling the nurse. She said that they had about 20 more minutes to get him all settled and then we could come in the room to see him. So we waited...
Thursday Post Op
We were prepped ahead of time by the surgeon that did our consent and by our nurse on what to expect in terms of tubes and wires and what Joey would look like post surgery. I guess that I was just so relieved to see him that I didn't care too much about what I saw. But it was still very hard to see him in such a state. When we entered the room, the nurse asked if we would like her to cover up his chest with gauze, but it was too late, we had already seen it. His sternum was open about and inch wide and about 3-4 inches long. It was covered by mesh and a yellow sterile tape/patch. You could see his little heart beating, and it oddly gave me comfort to see it working like that. (It wasn't as gruesome as you might be imagining, you could see the movements from the beats, but not really the heart itself.) Jamie had a harder time seeing him like that because he was worried that he was in pain or suffering, but with all of the anesthesia, sedation and pain medicine we knew he wasn't feeling any of it. We had to be strong for our little man.
The nurse was great at going through and explaining what everything else on him was. He had a main line IV in his neck, a sensor on his forehead, they moved his breathing tube from his mouth to his nose, he had a feeding tube through his nose, a drain tube coming from his chest, two wires coming from his chest that were connected to the heart and worked like a temporary pacemaker so that they could control his heart rate, a urine catheter, his normal sticker sensors and two of the three other IVs that he had before surgery. The nurse told us that once they got him in there they found that the IV in his left hand (the same one she was suspicious of and checked twice earlier) had gone bad and was leaking. His hand was super swollen because of it. No wonder he was so fussy throughout the morning... She apologized for missing it. Bad IVs seem to be the story of his life.
The rest of the day on Thursday he was pretty sedated and they were just trying to keep him comfortable. They were concerned with a low blood pressure and the nurse said the first 12 hours usually had a lot of ups and downs and were the most critical. But they thought he was in pretty good shape.
Friday
During morning rounds we got an update on the night. Everyone said it went very well and had many more highs than lows. His lung collapse was doing better since they moved his breathing tube from his mouth to his nose. The only things that were negative so far was that his blood pressure had been high and he was very puffy and holding on to a lot of fluid, which they would try to fix with more diuretics. The cardiologists came in after rounds to check on his heart rhythm and found that he was in a heart block. His heart rate was junctional, which means that it was beating from the center of the heart rather than the atrium and ventricles communicating and working together to form a regular "lub-dub" rhythm (thanks Bill Nye the Science Guy). Joey's atrium was beating much faster than the ventricles and they are disassociated and just out of synch. They examined it further with an EKG and the immediate plan was to just monitor because this is something that is not uncommon and is known to self regulate with the help of the pacemaker to "train" the heart to beat regularly. There is a more serious intervention for this problem that includes a medication to slow the atrial rate down and then speed it back up with the pacer to match the ventricle rate. But using a cardiac suppressant like that is not something wanted to do unless they had to.
The good news was that Joey was on the list for chest closer that day! We waited until about 1 o'clock and then were asked to leave the room for an hour. Although they did the chest closure in his room, it was still a surgery and they had to create a sterile environment. Plus, with the anesthesiologists, doctors, nurses and surgeons in the room it got crowded very quickly. Jamie and I went to eat lunch in the waiting room and returned about an hour and a half later and he was all closed up. They explained that the breast bone is pulled closed with wire that remains in the body and the bone continues to grow around it. All of the stitches are internal so there are no sutures to remove later on and the skin surface has a tape that will eventually flake off like dried glue. This makes for a smoother scar, which is exactly what we are hoping for!
Joey was pretty sedated for the remainder of the day again, and the only concern seemed to be around his heart rhythm. He was still in heart block and the cardiologists had a specialist in electrophysiology come to look at him. Even the specialist said that the best thing was to just wait and monitor. Joey had been on so many different meds since surgery, that they just wanted to see if he would level out on his own.
Saturday
Saturday was interesting because along with our nurse, we had a second nurse that was cross training, so our nurse was explaining EVERYTHING to her in great detail so Jamie and I got a lot of free lessons!
Early on Saturday morning Joey was more awake and aware than he had been since surgery. And he was having a hard time. They had to readjust his pain meds and were trying I figure out what he needed. Because of the diuretics he was given to get some of the fluids out, he was peeing a lot more and therefore passing more of his meds through than when he was retaining a lot of fluid. So they upped his Fentanyl. He was also so congested. They worked their hardest to get his lungs to loosen up and to get him to cough up the gunk enough so that they could suction it out. And just when they thought they had gotten most of it, he would have more congestion in his chest. All of this pain management and coughing was causing him to desaturated, which he hadn't done at all since surgery, and his blood pressure and heart rate kept climbing. They tried a nitric trial to see if the desaturation was coming from pressure in his lungs, but it proved not to be the case. Instead, they just made him more comfortable with meds, suctioned him more frequently and rolled him over to his left side. He seemed to respond well to all of this. He also had bloody secretions coming up when they suctioned him, so they x-rayed his chest and saw that his breathing tube was too far down in his lungs, so they pulled it up and the bloody secretions began to clear up.
This was all before morning rounds that day, but when the doctors came around their main goal was to get all of his excess fluid out. He was still so puffy in his feet and hands, and his urine output was not where they wanted it to be. As far as weaning from the ventilator, they would not focus on that until later, but when he was feeling better, they could back off of the ventilator as the Respitory Therapist saw fit. We were also excited to hear that they were going to take his chest tube and catheter out today as well as give him breast milk through his feeding tube today!
Since the late morning he turned into a different baby! He was much more content, comfortable and the plan that the doctors put in play for the day seemed to be working well. We played with him while he was awake and gave him a little pacifier practice. When Jamie and I left for the night, only his feet were a little puffy, he still had congestion, but everything seemed to be loosening up, he had two less tubes coming out of him and they lowered his pressure support on his ventilator!
The plan for the near future is to get the breathing tube out, continue breast milk through the feeding tube, get his heart rhythm under control, do more pacifier play, and possibly try feeding with a bottle.
(This will be a quick update and I will tell you all about Joey's big day this weekend when my mind and heart have settled down a bit and I am able to process everything fully.)
Joey's surgeon Dr. Starnes just met us in the waiting room and brought probably the best news we have ever heard in our lives. After three hours of surgery Joey is just fine, off the heart-lung machine and the surgery was being finished up by Dr. Herrington. After going in, he was able to repair the holes, do the arterial switch, switch the coronary arteries and not have to remove or replace the valve! He was able to remove tissue around and inside the pulmonary valve and thinks that it will be a sufficient valve to carry him through his life. Which means no more surgeries as he gets older!! (At least nothing planned, fingers crossed.)
His breast plate remains open to allow for swelling to go down and that will be closed either tomorrow or Saturday and then the real recovery can begin.
Both Jamie and I are so grateful for the team here at Children's Hospital, and that Dr. Starnes and Dr. Herrington were the ones that took on his case. We were in the best of hands.
And thank you to everyone who was thinking of and praying for Joey today! We really felt the love!
So, we came into Tuesday with good news, everything was pretty much the same with medications and trials of breathing on his own, and the only news that we got from morning rounds was that current treatment was going to stay the same and they were going to add a breathing treatment today to loosen up some junk in his lungs. He weighs almost 8 pounds now, but all of the weight gain is still retention of fluid.
They have been doing pressure support breathing trials for the last couple days, which means that they back the ventilator off for a period of time and allow him to breathe more on his own. Before, this was to see if we could get him off the ventilator and extubated before surgery, but now, because we are pretty close to surgery, they will not be extubating him and the pressure support trials are simply to give him some practice and exercise in breathing on his own. He has been able to go anywhere from 20 minutes to one hour on his trials and today they tried to go for two hours. He did fine during the trial, but after that and after a breathing treatment, he had a hard time recovering and his oxygen levels had been desaturating pretty frequently. They tried a few things out to stabilize him and to figure out what may be causing it.
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We FINALLY got to meet with the surgeon today. This is where we didn't quite get the news that we were hoping for. She said that the team reviewed his case and his echoes and made the decision to do Switch with the Nikaidoh Slide procedure rather than just the Switch itself. Although last week we got news that his stenosis was minor, the combination of the stenosis, the bicuspid valve, the small size of his Pulmonary Artery, and the location of his VSD, they have opted to do the Switch with the Nikaidoh Slide. Although we were hoping for the "one and done" Switch procedure, we trust in their decision that this is the best procedure for him to have. It just breaks our hearts that this will not be his only open heart surgery in his lifetime. He will need follow up surgeries at least two more times as he grows to full size to replace the conduit that is put in during the Nikaidoh procedure.
Surgery is scheduled for Thursday, March 24th (his original due date) at either 8am or 10am and will be performed by Dr. Vaughn Starnes and Dr. Cynthia Herrington. This is it the first time Dr. Starnes has operated on a Sbicca! He also performed Jamie's Grandfather's heart surgery years ago. Please keep Joey in your thoughts and we will update you on his recovery as soon as we can.
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Later on in the day, his oxygen levels were still very low and everything that they were trying was not fixing it. The attending doctor decided to order another echocardiogram and chest x-ray. The x-ray was to check on his lungs to see if the tube had moved or if his partial collapse had gotten worse. The echo was to look at the ductus arteriosus and the small holes between his atriums. He has been taking medicine to keep the ductus open (normally this closes soon after birth) and the holes in his atrium septal wall (ASDs) sometimes begin to close on their own as well. The echo showed that his ductus was beginning to close and this was probably the reason for the desaturated oxygen levels all day, because they even tried to double the dose of medicine they were using to keep the ductus open (prostaglandin) and that didn't work. They decided pretty immediately that it was time to do the balloon septostomy procedure. They had held off until then because he was doing alright without it, but with the ductus closing and the VSD not mixing enough blood, it was time. Within 30 minutes he was sedated and they began the procedure. The septostomy is a catheterization procedure in which they go into an artery in his groin, lead a catheter up and inside the heart and through one of the holes in the atrium, inflate a balloon and pull it back through to tear the hole open more and to keep it open. This allows for more oxygenated blood to go through the heart to the body. They will fix the hole when in surgery. The doctors said that the procedure went really well. His heart rate has gone down and his oxygen saturation levels have greatly improved!
The last thing that they will do tonight will be a blood transfusion. He did have some bleeding at the catheter site in his groin after the septostomy, but the transfusion is mostly just to get his red blood cell count up. Red blood cells carry oxygen through the body, so they want to maximize that as much as possible before the surgery on Thursday.
Jamie and I are doing fine. It does make it harder that they have the no visitor rule still in play. We would love for our families to get to meet him, but hopefully it will be lifted soon, or hopefully they will make an exception to get grandma and grandpas up here before surgery. It is nice having an apartment close by this week. We stocked up on food so that we can just bring our breakfasts and lunches to the hospital. And whatever sleep we don't get at night we try to make up for with frequent naps at the hospital. Either the little window bench is comfier than it looks or we are just tired enough to be able to sleep on it every day! My milk has finally started to come in and boy does that 2-3 hours in between pumping sessions go really fast! Joey still won't be able to get any of my milk until sometime after surgery, but at least we will have it ready for him when the time comes! Although today was hard (not to mention these postpartum hormones have started to kick in), we are hoping to get to hold Joey again tomorrow, so that we can get a little more cuddle time before surgery. We were planning on doing it today also, but things didn't end up going that way. We continue to hope that the transfusion goes well and that he continues to be stable so that tomorrow goes as planned.
I don't think I will have any updates for the blog before his surgery, so please keep Joey in your thoughts the next couple days. I will hopefully be able to jump on here on Thursday during recovery time to give you a quick update on the surgery. And you can expect a longer post about the surgery on the weekend!
Yesterday and this morning were very exciting for Jamie and I!
We got to hold little Joey for the first time! It was amazing to not have to just stand at his bedside and poke and play with him, we got to poke and play with him while in our arms! I can't get enough of his fluffy hair, soft skin and tiny toes.
Last night they also started giving him something for his adema, he has been so puffy and swollen up since birth that he hasn't been able to open his eyes. I remember seeing them open a tiny bit right after birth, but since then he has had the swollen face of a boxer after a fight. But the treatment worked so well that he is now able to open his eyes and see his mommy and daddy. Jamie and I stood around his bedside forever this morning, just looking at him look at us! (Sorry I didn't get any pictures yet! It was took dark and we were too excited!) He was also doing a little bit of rooting so we were able to try and give him a pacifier. He licked it and sucked a little bit but didn't seem too interested. Sometimes we see him sucking on his breathing tube, so that is good. The sooner we can encourage suckling the better for feeding and breastfeeding after his surgery, so we will keep trying!
It is looking more and more like surgery will be Thursday, we are waiting for confirmation from the surgical team themselves to confirm the date, the type of procedure and the surgeons that will be performing it.
Other than that, we are still just giving him a little practice each day with breathing more on his own. He doesn't like it much, but the team here is really persistent on letting him continue to try, which we like. His jaundice is looking a lot better today, but until he begins to get food through a tube to his gut, he will continue to have mild jaundice.
Joey has now been in the Cardio Thoracic Intensive Care Unit (CTICU) for three days. We got to change diapers today and got to take our masks off for a quick family picture!
Everything is going well and nothing has been too different from what we expected pre-birth. His condition is good and he is very stable. All the nurses say he is an easy baby because he is so calm and likes to sleep. He has actually put on some weight and now weighs about 3 ounces above his birth weight. The nurse said that most of that is simply retention, and he will start to lose weight pretty soon. He has been making lots of poops and he has nice, fluffy brown hair! The nurses and doctors at CHLA have all been great so far. Joey is currently in a large shared room with another infant girl, as the entire CTICU is filled up. He will, more than likely, get a private room during surgery recovery. Jamie and I spend our days in the room with him. We try to make it to the hospital each morning for morning rounds so that we can check in with the night nurse and get his full update from the CTICU medical team. The cardiologists don't have set rounds but we have been able to catch some of their check-ins.
He is still on the ventilator and after being sedated for an MRI yesterday, there has been only a little progress towards being able to wean him off. He was even sedated today a couple more times for various procedures, one being getting a PICC line so that they don't have to rely on his IVs for everything. They will continue to try to wean. It would be nice to get him extubated because then we would be able to hold him and finally see his little face unobstructed. (We do know that he definitely has his mommy's nose!) It will, eventually, get to a point where we will be too close to his surgery date, the extubation just wouldn't make sense (because it would go right back in for the surgery), so we are hoping the weaning takes a turn for the better. Today they did pull the tube out by half a centimeter. They did this because the top lobe of his right lung is slightly collapsed which may be caused by the tube being in just a little to far. They gave him a little sedation and pulled it up today, we will see if that changes anything.
He has had two echos and the diagnosis of his heart is currently better than expected! They are still in agreement that the balloon septostomy is not needed at this time. He has two small atrial holes that seem to be staying open for now, and with those and his VSD enough mixing is happening that his oxygen levels aren't too low. The septostomy is not fully off the table, and although it is a somewhat minor procedure (compared to everything else), it is still one less procedure that he has to have! During the fetal echos, he was diagnosed with Transposition of the Great Arteries and a VSD, those diagnoses were spot on. However, it seems like the Pulmonary Stenosis that they believed he had could be something different. If he has stenosis at all, it is minimal. But they have found that his pulmonary valve is bicuspid instead of tricuspid (two flaps that open and close instead of three). And while this is pretty rare, it should not cause any major issues, or change the surgery plan. So this is good news! It seems as though he may only need the Arterial Switch Operation, and not other of the alternatives that would require follow up open heart surgeries later in life. We are keeping our fingers crossed!
It is also good news that the possible malrotation of his intestines that they saw in his fetal MRI is not present. He is currently being fed via IV, and if he is able to get extubated before surgery he already has a tube in place for him to be fed some of my breast milk (which is finally beginning to come in!) We are hoping that he will get some nutrition to his gut before surgery. If not, our hope is to be able to at least swab some of my colostrum in his mouth.
Besides just keeping up with the things mentioned above, the only thing we are truly waiting on is a set surgery date and a definite answer as to which operation he will have. The Monday date that was originally mentioned by the surgeon last week will not be the day... our surgeon has unfortunately not been here this week, so all we have heard from other doctors is that the surgery is tentatively set for Thursday. That would make Joey eight days old. Since we haven't really been able to discuss this with anyone, I am keeping my hopes high in thinking that they can wait longer because he is doing well and his condition is not as dire as they had previously thought. This also gives him a few extra days to get big and strong before surgery. We were just hoping to get a jump on the procedure so that we can go home that much earlier, but a big, strong boy going in to surgery will hopefully have a very speedy recovery.
As for me, I am recovering very well. But I think it feels easier for me because I have such a great motivation from Joey to be there for him and great support from Jamie. Next week we are renting an apartment about one mile from the hospital so that our commute is easier and we can have a place closer to shower, nap and eat. It should make things a lot easier. Thank you to everyone for the messages, texts and calls, we hope that the hospital lifts the visiting ban soon so that our family and friends can come meet Baby Joey!
All went as planned for labor. They started my pitocin at about 11:30pm on Tuesday. Contractions started very slowly so both Jamie and I were able to get some sleep. At about 7am, contractions started to be stronger and more consistent and my water broke that morning as well. Things progressed pretty well and I got an epidural at about 11am when I was about 4cm dilated. And just as my doctor predicted I dilated about a centimeter an hour, I started pushing at 5:30, and he was here about 40 minutes later.
We only got to spend a minute with Joey before he went to the NICU and then we got to go into the NICU to see him transferred into his transport carrier. Jamie was able to ride in the ambulance and stay with him the whole time. Joey is stable at the CTICU at Children's Hospital Los Angeles and they are monitoring him. He has IVs, feeding tube and is intubated. They are really trying to see if the septostomy is necessary, but as of right now they have not ordered one. They are trying to wean him off the ventilator, but he isn't likely my that too much, yet.
I tried for the quickest recovery possible and got discharged 19 hours after giving birth. Joey, Jamie and I are now reunited at CHLA! Because of flu season, no one but the parents of the babies are allowed to visit. We will let our family know when they open the floor back up to visitors! Hopefully it is soon!
It's been an eventful first 24 hours of life for baby Joey.
Thank you all for the good thoughts you sent to Joey, Jamie and I in the last couple of days. Keep the good vibes coming!
The plan (and I say that loosely as many things can change) is to go to the hospital late tonight to be induced. They will start the pitocin as soon as I get there, and I will try to get a little sleep. We will shoot for a Wednesday afternoon delivery time as best we can. Joey will be stabilized in the NICU at Huntington Hospital, and then be transported to CHLA. Jamie will be with the baby as I recover as quickly as humanly possible back in Pasadena. The septostomy, or balloon procedure will happen once at CHLA (not sure of the exact timing), echos and other tests will be done, but the doctors seem hopeful that after that, he will be a pretty stable boy. We will be able to hold him, but probably not be able to feed him, as taking milk from a bottle or breast would be too exhausting for him. Throughout the next few days they will continue to monitor him and make a decision as to which procedure he will have. It is currently between the Arterial Switch or the Nikaidoh, depending on the severity of his pulmonary stenosis. The surgery is tentatively scheduled for Monday. But we will definitely keep you updated until then!
Yesterday Jamie and I celebrated our one year wedding anniversary. Yes, we ate the cake (some bites) that had been tucked away in the freezer for the last year. Our first year of marriage was exciting, to say the least. It had a lot of ups and downs. At the very beginning I would tell people that being married it didn't seem very different, and because we had been together for 10 years, the only thing that really changed was my last name. Then August came around...
Within one week we found out that we were pregnant, told our families the great news, and also got the news that my mom was diagnosed with breast cancer. Talk about highs and lows. After the summer ended, Mom had her lumpectomy, which seemed to have removed the cancer, and had plans to do radiation after the new year. Good news. Then November came around...
That's when Joey's diagnoses started pouring in. It's hard to enjoy your pregnancy, when you get bad news at every doctor's appointment. But we tried our best to stay positive. The more we talked about it, shared our story and learned of other's stories, the better we felt. It's amazing how many people we have met that have had heart issues from birth, survived open heart surgery, or had a baby with a similar diagnosis.
The new year came, Mom finished her radiation and continues to show no cancer. Jamie and I remained positive and had as much of a plan as we could.
Now, the time has come. It still doesn't feel real. As I sit on the couch, pack our final bags, and drink as much water and raspberry leaf tea and eat as much fresh pineapple as possible, it STILL does not feel like I will be popping out a baby tomorrow! This has to be the perfect combination of excited, nervous, scared, and happy that I have ever been. Wish us luck!
Everyone has been asking to see Joey's nursery, and it is finally done! This boy is so spoiled! He has the nicest room in the whole house! A big thanks to my parents, who helped us put it all together!
CHD. d-TGA. VSD. PS. OHS. It's been an alphabet soup of sorts!
I never in a million years thought I would learn so much about the human heart, let alone the fetal heart. But since that day in November when we got Joey's first diagnosis, Jamie and I have tried our best to learn all that we can so that we can get the most out of our conversations with our doctors and truly understand our son's condition. Unfortunately, since November we have learned of four different heart defects as well as a possible intestinal issue. There was a long stretch of time in which each appointment brought more bad news.
Not many people know that heart defects are the most common birth defects. A Congenital Heart Defect, or CHD, is a heart defect that is present at birth. CHDs effect about 1 in 100 babies born today. There are approximately 35 different types of congenital heart defects that can combine and manifest into thousands of different situations! Many heart defects don't need treatment or can be fixed quite easily, however there are some that require very invasive treatments and can cause serious health problems or death.
Currently, Joey has been diagnosed with several heart defects which include:
Ventricular Septal Defect
Transposition of the Great Arteries
Pulmonary Stenosis
Retrograde Blood Flow Through The Ductus Arteriosus
And during an MRI at about 24 weeks, they also noted a possible malrotation of the intestines. However, this is something that cannot be officially diagnosed until birth, so it is something that is still on the table, but that we are not currently focused on.
In case you were curious of Joey's conditions and possible treatments, I thought I would share some of the details and science-y stuff. It is such a miracle that the doctors were able to diagnose so early, but there are also a lot of things that we will not know until he is born.
Ventricular Septal Defect (VSD)
At our 20 week anatomy scan, the Perinatologist was able to identify a large VSD, or hole in between the heart's ventricles. The size of his hole was classified as moderate to large and measured about 5mm.
At this appointment we discussed a lot of possible outcomes of the VSD. These included the hole possibly closing on its own, being able to live with a hole of a certain size without treatment, invasive surgery and noninvasive surgery. It really all depended on the condition at birth. But we were also informed that this type of heart defect is sometimes correlated with certain genetic disorders. We weighed the risks of amniocentesis with the facts that my previous non-invasive prenatal testing showed little to no risk of a genetic disorder and that the baby didn't show any other physical markers of any common genetic disorders. We decided to not go forward with any other genetic testing.
A week after this diagnosis, we made a follow up appointment with a Pediatric Cardiologist from Children's Hospital Los Angeles and she performed a fetal echo cardiogram. This is where we first learned that the VSD was not the only thing we had to worry about.
Transposition of the Great Arteries (TGA)
This is where everything really changed. The cardiologist performed a very long, silent, ultrasound/echo cardiogram when I was at 21 weeks. After she was done, she broke the news that she had found the TGA. She explained it to us by drawing on a diagram. Basically, TGA involves the two main arteries of the heart, the Aorta and the Pulmonary Artery, being switched and connected to the opposite sides of the heart.
But all I heard while my head was spinning in that doctor's office was that this was a very serious condition and would require an open heart surgery. When we returned home, we wanted to educate ourselves even more on this condition. Of course, the first place we turned to was You Tube! But I found a really great video that explains TGA very well. I have sent this video to anyone that has wanted to learn more because I think she does a very good job in demonstrating this concept.
Luckily, neither the VSD or TGA has any effect on Joey while in utero. However, once born and he takes his first breath, the TGA will basically make it so that he has two separate loops of blood flowing in his body, one oxygenated and one deoxygenated.
Joey's VSD will actually be a help to him when he is born because there will be some mixing of oxygenated blood. But it will not be enough. This is where the amazing aspects of the fetal heart will come into play. Everyone is actually born with a small hole between the two atria of the heart and with an extra duct that extends off of the pulmonary artery. These aspects of the fetal heart are what allow blood and oxygen to flow through the baby's heart while in utero and not breathing air. Both of these parts of the heart close shortly after birth. Part of the treatment of TGA is to keep these mixing locations open. The Ductus Arteriosus is kept open with a medicine called prostaglandin and the hole between the atria of the heart may be kept opened or enlarged by a balloon catheterization procedure called a Septostomy, done shortly after birth.
Most of the time, the solution to d-TGA is the Arterial Switch Procedure about 3-4 days after birth. This is an open heart surgery in which the two arteries are cut and switched back to their rightful places and the coronary arteries are attached to the correct vessel as well. This was the plan that we had for a few months, until a follow up echo cardiogram revealed what we hope are the last two heart defects. Depending on the severity of one of these defects, and Arterial Switch may not be the way to go.
Pulmonary Stenosis
At about 30 weeks, we had another echo cardiogram at Children's Hospital LA. This was another 90 minute ultrasound because the tech was having a hard time figuring out what she was looking for. She seemed pretty new and both Jamie and I were very frustrated with her bedside manner, which didn't make this appointment any easier. Once the cardiologist came in she drew on more diagrams to show us what she saw in Joey's heart. (We have so many heart diagrams with red and blue scribbles all over them, I should start a collage!) She explained that the pulmonary valve had some narrowing. Now, obviously we will not know the true extent of the stenosis until he has an echo after birth, they can only see so much while in utero.
This diagnosis could potentially change the treatment/surgery plan we have been preparing for. My understanding is that because the Arterial Switch Procedure switches the arteries, but not the valves, what is now his pulmonary valve (with the stenosis) will become his "neoaortic" valve. The aortic valve is the most important valve in the body as it pumps blood from the heart into the body, so if the stenosis is more than minor, it could have a greater effect on the blood flow in his body. The answer to this may be a different procedure all together that would not involve a switch, but more of a "rerouting" and using a conduit to relieve pulmonary obstruction (Rastelli Procedure) or an aortic translocation (Nikaidoh Procedure). We REALLY don't have a good grasp on what either of these procedures mean, or do, however, they both would required additional surgeries later in life as he grows as well.
Our next step is to learn more about the Rastelli and Nikaidoh procedures and know when and why they would use one over the other and when they would use one of those versus doing the Arterial Switch. There is still a lot of learn! And we definitely won't know which procedure is best until he is born and can be evaluated, but we were also told that the surgeon may make a decision based on what she sees once the open heart surgery begins! So we wait...
Retrograde Flow in the Ductus Arteriosus
This is really what we know the least about. The cardiologist doesn't ever seem too worried about it when she mentions it, so I guess we shouldn't worry too much either. But to have blood flowing in the opposite way through a duct seems weird. However, this Ductus Arteriosus is the duct that is present in a fetal heart but then closes shortly after birth, so maybe that has something to do with it. We will keep asking questions.
Wow! Once I lay it all out like that makes my heart ache for my little man. The good news is that we have a plan, we trust all of the doctors and other professionals that we are working with through USC, Huntington Hospital and Children's Hospital Los Angeles and we have come to the realization that will need to wait a couple more weeks to truly know which path to take. Little ones are so resilient and the current surgeries and medicines are so amazing with what they can achieve, our hopes are high for our little heart warrior. Jamie and I will keep you all updated on his condition before and after birth, the plans for surgery and recovery and we just ask that you keep our family in your thoughts and hope for the best.
