To the Floor and Preparing to go Home

Happy to be down on the floor

With Lillian on the floor, most of our hospital stay was behind us. Now we just had a short period on the floor to make sure she was stable and to get us used to caring for her for the first time and getting used to her special needs. Lillian though, was clearly ready to be on her way out, as she was more alert every day.

Since she was finally stable enough to move, it was time to do the MRI that we had postponed so long to check on her brain bleeding. I was really viewing this test with apprehension, as the brain bleeding to me was almost a bigger issue than Lillian's heart. Her heart issue was serious yes, but it can be fixed and managed with relative ease. A mental issue though would be much harder to deal with, likely requiring a life time of special mental care. This MRI would tell us if Lillian would have to deal with that on top of her heart condition. A lesser worry too was that for the MRI to be useful, the patient has to be completely still for the procedure, a state that is almost impossible to get with young children. Thus, having an MRI involves being put on some sedative, and most infants are intubated on sedatives as a precaution. Since Lillian took a long time to come off the ventilator the first time, we were hoping to avoid the same thing again as a consequence of something as routine as an MRI.

Luckily for us, the radiologists happened to come at a time (on August 19th) when Lillian was dead asleep and completely still. They were able to do the procedure without having to intubate or give any sedative. The results from the MRI were also great, the bleeding had gone down and likely would not be an issue in the future. In all, exactly the results we were looking for.

 The next day on the 20th, we had to remove Lillian's PIV line due to that clogging as well. We were hoping to remove the PICC line first as that goes in farther, but we had to leave it in for a little while longer. In the mean time, we were focusing on teaching her how to eat, a reflex we had suppressed since birth. Lillian struggled with this at first, she simply wasn't used to having anything in her mouth. She continued to struggle on the 21st, but was enjoying the visitors that she had.

Annabelle was thrilled to spend time with her sister

Lillian's little cousin Robert also came to visit, but she was tired at this point.

On August 22nd, we finally took out all of the medicine and continuous drips, leaving the line in a couple more days just to be safe.  But seeing the pump rack finally leaving the room for good was a relieving sight.

Gone for good!

Lillian also had her great grandma and great aunt come to visit.

Since Lillian had done fine without any of the IV medications, it was finally time to take out the PICC line on the 23rd, leaving her feeding tube as the only tube in her, which doesn't even count since it goes through her nose. She had gone from 19 tubes (yes, I am counting all IV pump lines individually even though they consolidated before entering) to zero tubes, a massive milestone, even if it is only a mental one.

White patch is the last reminder of the lines.

We were starting to near the end of our journey at the hospital! There were only a few major milestones left, including removing Lillian's stitches and getting her back to oral feeding. The later is important, but is also something that we could do at home, so only the stitches remained. Kathryn and I thought it looked like it might need a little more time to heal, so we were surprised to hear that they were ready to be removed on the 24th, and removed they were.

Now all that was left was to make absolutely sure she was ready to go home by doing a car seat test and teach us how to place a feeding tube. We did both on the 24th. Lillian did great in her car seat test, and I did surprisingly well in my first time placing a feeding tube. If you ever find yourself in a position where you have to do this, it really isn't as bad as you would expect.

During the time since she had been on the floor, we had been trying to wean Lillian off of Morphine, which was proving difficult given that she had been on it for over half her life for this point. The day of the 25th was especially difficult, as Lillian was having a hard time with the wean and was extremely fussy. The 25th was also the first day she got to wear clothes, so you can imagine that this combination led to a less than happy child.

Finally, the time to go home had come! Lillian was stable, off all IV medicines, repaired, closed and stitched up and clothed. It was time to go home just after 4:00 PM on August 25th.

Getting Better - Recovery in the CICU

 

We finally had a good couple milestones under our belts! Lillian's swelling had gone down, she was off ECMO, and her chest was finally closed. But we weren't ready to leave the CICU yet, she still was on a ventilator, as well as medicines like Milrinone that can only be given in the CICU.

Our next step after closing her chest was to trial taking her off Lasix so we could remove the clogged PICC line in her arm before it caused any significant damage to her blood vessels. Lillian was also suffering from intermittent arrhythmia, a relatively minor issue, but just another thing that we had to deal with. Her medical team suspected that it might be an electrolyte issue, so we began labs on that to see. In the mean time, Lillian was more and more awake, in part realizing what was going on around her. She was on a ton of pain killers and intubated, but she was still awake enough to scream, but couldn't do it out loud because of the ventilator. Seeing her silent screaming was more difficult than seeing her immediately after surgery, but an extra dose of Morphine helped her feel better quickly.

By midday on the 12th, Lillian's arrhythmia was gone, but was back by midnight. One of the nurses suggested that the cause might be one of the central PICC lines being in too far and actually sticking into the walls of her ventricle, irritating it, which would certainly cause arrhythmia. My impression of PICC lines so far is that they go to the heart, not actually in it, but this line may have been different. On top of the central line possibly being in to far, the arm line was now completely blocked and leaking, so it needed to be pulled. Ultimately, the planned MRI on the 13th to reassess her brain bleeding had to be postponed due to the arrhythmia and low blood pressure; and we instead went to look at getting a new line in to replace the clogged PICC line. Here is where there can be a major difference, as the first nurse could not get one in in 30 minutes, but another one got it in in less than 5 minutes.

Only 15 tubes left!

An added benefit of removing the clotted line was that we could also remove her central drainage tube, which you can see gone with the white bandage above. She still had the side drainage tube for the pneumothorax, but the chest one was the main drainage tube, meaning that most of her chest bleeding had gone down. We could also restart the Lasix through the new line and lower 2 of the medicines keeping her in the CICU. And the best news of the day (still the 13th for the record) was that her fevers were down, indicating that she likely either did not have an infection or had already fought it off.

On the 14th, the cultures confirmed that there really was not an infection, so the fever and white blood cell counts were likely just an immune response to the procedures they were doing after the surgery. We were revealed to find that out as we had been fearing a bad infection. We were also able to turn down her Dopamine and the ventilator, ultimately putting her on pressure support (to keep her lungs from collapsing, but not actually breathing for her) with the latter early on the 15th. This went well, as the trial off on the 15th went well enough for us to extubate her and take her off the ventilator. 

On the 16th, Lillian's recovery was really becoming apparent; not only did we take out the last drainage tube, but we were able to turn down the Milrinone again and hold her at long last. This meant that we could move to a less intense section of the ICU, and was also the day that I returned to work. Kathryn had the chance that day to take what is still one of my favorite pictures of Lillian:

We also turned off the Milrinone that night, which she tolerated well. This was the last thing keeping us in the ICU, so we were now able to go to the floor when there were spots available. Since there weren't though, they left in her arterial line, as that allowed us to continuously monitor her blood pressure. Even though this posed a slightly higher risk of infection, we were alright with leaving it in considering how recent the Milrinone shutoff was. We also took her from 8 IV pumps to 3, containing only flushes and foods (lipids/TPN).  With this switch, all medicines were now oral instead of over IV pumps.

Alas, our move to the floor on the 18th appeared to not be happening again. We did decide to take out the arterial line at this point, which was dramatic as something wasn't properly closed before removing it and blood began shooting everywhere until the nurse was able to clamp the line. With it out successfully, we also started her on Captopril, a drug which lowers blood pressure to help her heart be more efficient; and doubled her food intake. We also finally had a bed open up on the floor, finally taking us out of the ICU around 9 on August 18th. We had been there since July 23rd. Here's a last little collection of pictures from the CICU.

Our time in the ICU was very stressful, but it was made so much easier by the never ending support of tons of friends and family members. We especially want to single out the staff at Children's NICU and CICU, who have been nothing short of wonderful, giving us hope and pep talks when we needed it (Julie specifically), always ready to sit and answer all of my stupid questions, and doing a phenomenal job of caring, treating, and fixing Lillian. We would not have survived without you!

Touch and Go - Immediate Post Op Period

Lillian immediately after surgery

 Lillian's first real day in the CICU was on August 5th, as she came there out of surgery some time after 11:30 PM on the day before. Since she was post op and on ECMO, she was extremely fragile. The first day after surgery was immediately eventful, as even though she had been on the ECMO circuit for barely 12 hours, she had already developed a clot within the circuit and we needed to change the ECMO machine before the clot got into her system. Since she was in a small shared room with 3 ECMO machines in it (2 for her, one for the baby girl next to her), 2 ventilators, massive racks of IV pumps and 4 nurses, I had to leave, but you can see in the picture I snapped first just how crowded it was. It was so crowded I couldn't even get the ECMO machines in the picture, but they're at the bottom.

Before they had changed the ECMO machine, they had the Heparin (an anti-coagulant) levels in the lines fairly high to prevent them from clotting. This clearly didn't work, but we had to balance the risk of the clot against something else, as they had found some bleeding in her brain. This is extremely dangerous as blood in the brain ventricles (note that brain ventricles are completely different from heart ventricles that Lillian does have an issue with) can push against the brain tissue, causing brain damage and even death if it gets bad enough. We have to carefully balance that though, as using too little Heparin can cause the machine to clot as it did once before. Plus she would be at risk of a stroke and her heart has to work harder to push the blood, giving it less rest, completely counteracting even putting her on ECMO. If we over do the Heparin, we increase the bleeding on the brain, which could cause the aforementioned brain damage or death. It's an incredibly tight line to walk. However, despite having these complications, we still saw some hope from Lillian, as she was already stirring despite being on full bypass and hopped up on tons of pain medicine. Once they finished with the ECMO circuit, we finally saw Lillian's eyes open a bit.

Especially with how fragile she was after surgery, it was great seeing signs of life from her.



And despite the fact that she was functionally unconscious with all the medicine, Lillian's natural reflexes were in full view, as she worked on an imaginary pacifier.



One of the side effects of changing the ECMO machine was that Lillian had to have a flood of Heparin to prevent the lines from clogging during the switch. As you might expect, this caused the bleeding in her brain to get worse. This we unfortunately just had to deal with as not changing the clogged ECMO machine would have been fatal eventually.

Lillian's second day in the CICU was not as eventful as the first, but had its own ups and downs regardless. On the good side, we could see that Lillian's blood pressure had started to pulse on its own (the atrial lines she has allow for continuous blood pressure monitoring as opposed to having to use a cuff), which was a sign that her heart was getting stronger and starting to beat on it's own initiative, something that it had not been doing yesterday. To compare, immediately after the surgery, her heart was so tired that her blood pressure line was flat when it should have been pulsing similar to a heart beat or oxygen levels. On the bad side, it looked like Lillian had a minor seizure. It was small and we don't think it did any damage, but Neurology still wanted to start her on a anti seizure medicine just in case it was the start of a trend.

There was more good news though, as Lillian's chest bleeding had gone down, and she reacted well to a scale down in ECMO, exactly the type of thing we needed to take her off of ECMO.

On August 7th, we were ready to try to take Lillian off of ECMO. This started with a trial phase where the circuits were clamped shut, but she was still connected so if she still needed it, it would be quick to put her back on it. The trial was about 2 hours, after which they decided Lillian was doing well enough off it to take her off ECMO entirely, which was a major step in her recovery. Once they took her off of it entirely, the surgeons told us that her heart looked good and there was no bleeding as a side effect of de-cannulating her.

On August 8th, Lillian's condition took a turn for the worse even without ECMO as she started developing a fever (at 38.4c). This is normally the first sign of infection, which is especially worrisome given that Lillian still had her chest open and was still in the immediate post operation time period. Whether it was from an actual infection though or just a standard immune response from having them change things around in her chest, we weren't sure. The next day, August 9th, the fever got worse (39c or 102.2f) and her white blood cell counts were up. Since we now had two signs of infection (but no sure confirmation from early labs) we began treatment with a wide spectrum antibiotic and ran much more extensive labs to confirm any possible infection.

Later in the evening on the 9th, we were able to get Lillian's fever down to 38c, allowing us to start using Lasix again, which was important as she needed Lasix to get rid of fluids, which in turn would allow us to close her chest. However, we decided the next day that switching the PICC line from her arm to her leg was more important than closing her chest, as the PICC line in her arm looked like it was clogging, which would have severely damaged one of her vessels to the point of making her next surgery much more difficult. In the end though, after waiting most of the day for the PICC line, it was delayed due to an emergency with another patient.

Changing course from a daily recap for just a minute, I took these pictures to try to emphasize how many IVs she had going in. Some had been removed already (especially the ones relating to ECMO), but there will still 10 or so, all consolidating to the PICC line eventually.

On August 11th, we were able to get a new PICC line in her leg after taking her to Interventional Radiology to have it placed. Although a trip to Interventional Radiology is not normally required to place a PICC line as it can normally be done at the bedside, Lillian's vessels were extremely thin, requiring the more advanced placement. This timing was very good as her other PICC line in her arm had in fact clogged at this point. Even with the clog, we had to leave it in for the Lasix as that requires a separate line as it does not interact with other medicines well. More importantly than the PICC line though was that they were finally able to close Lillian's chest after having it open for 7 days. Having a chest left open that long is not normal, and was putting Lillian at increased risk for infection. Unfortunately, it looked like the closure also created a pneumothorax (basically a small air pocket) in her chest. This required a new drainage tube in her side, but was a relatively minor issue.

Finally, chest closure!

The Norwood Procedure - The Surgeon's Perspective

Note: The following post is an excerpt from Dr. Michael McMullan's (the surgeon that did Lillian's surgery) account of the surgery. As such, it is heavy on the medical terms. I've tried to insert some of my own comments [they look like this] when appropriate to translate things into English when I can personally for those that are not medical professionals.This is an exact copy of what he wrote aside from my comments. Dr. McMullan, if you happen upon this and would prefer this not be up, please contact me.

Also, I'm not sure why it looks like the black is changing colors in some areas. It is all the same color when I post it, it may be some weird visual brain trick.

OPERATION:

1. Modified Norwood procedure with 6 mm Sano shunt.

2. Central ECMO (extracorporeal membrane oxygenation) cannulation.

SURGEONS: David McMullan, MD, Attending; Lester Permut, MD, Assistant.

ANESTHESIA: General

FINDINGS:

1. Severely hypoplastic ascending aorta with focal narrowing of the transverse aortic arch. [her aorta is very small and only gets smaller]

2. Globally depressed ventricular function. [What she does have doesn't work well]

SPECIMEN REMOVED:

1. Thymus.

2. Ductal tissue. 

3. Atrial septum.

4. Right ventricular muscle.

INDICATIONS: This infant was born with hypoplastic left heart syndrome and was stabilized in the Neonatal ICU. Her diagnostic studies and clinical history were presented at our multidisciplinary conference and it was the consensus of those present that she would benefit from a Norwood procedure at this time. By echocardiography, she has a very small ascending aorta. I discussed the risks and benefits of the surgical procedure with the patient's parents in detail. They asked appropriate questions, expressed their understanding, and provided written and informed consent. 

DESCRIPTION OF PROCEDURE: After obtaining Informed consent, the patient was taken to the operating room and underwent general anesthesia. Her chest and abdomen were prepped and draped. A transesophageal echocardiogram [an echo where the probe is passed down the esophagus as opposed to being on the outside of the chest to get better pictures] was performed. A midline sternotomy [this is the opening and cracking of her chest] was performed. The thymus [an organ that helps regulate the immune system] and anterior portion of the anterior pericardium were resected. [they were removed] The pericardium [the double walled sack that contains the heart and the roots of the blood vessels] was treated in glutaraldehyde [a medical grade disinfectant]. Inspection of the mediastinal [the group of structures containing contains the heart, the great vessels of the heart, esophagus, trachea, phrenic nerve, cardiac nerve, thoracic duct, thymus, and lymph nodes of the central chest] contents revealed a dilated right ventricle, severely hypoplastic ascending aorta, and focal narrowing of the mid transverse arch. The right subclavian artery and innominate artery [arteries that supply the right side of the body with blood] were mobilized. While attempting to prepare the ductus arteriosus [the shunt that kept her alive] for cannulation, the patient developed hypotension and bradycardia [extremely low blood pressure and heart rate, respectively]. In light of what was perceived to be acute coronary ischemia [i.e., severe restriction of blood flow to the heart] related to inadequate blood flow through the hypoplastic ascending aorta, a brief period of direct cardiac massage was initiated [that is, they were manually pumping her heart with their hands]. Cardiopulmonary bypass was initiated utilizing ductus arteriosus and right atrial cannulation. Upon initiation of bypass, the branch pulmonary arteries were mobilized and occluded using vessel loops to prevent pulmonary over circulation. Using vascular clamps to isolate the distal innominate artery, a longitudinal arteriotomy [the cutting open of an artery] was performed at this site and a Gore-Tex tube graft was anastomosed [or connected] to the site using running Prolene suture. The vascular clamp was removed to establish flow through the graft. An arterial cannula was then inserted into the graft and secured with Nurolon suture. This was then connected to the arterial limb of the cardiopulmonary bypass circuit to establish a second source of arterial blood flow. As the patient was being cooled to deep hypothermia [done to protect her brain], the transverse aortic arch, ductus arteriosus, left subclavian artery, and left common carotid artery were mobilized [the left subclavian artery supplies blood to the left arm, the left common carotid artery supplies the head and neck with oxygenated blood]. A vascular clamp was placed across the distal ascending aorta and the ascending aorta was transected proximally [they cut into the aorta]. Cold crystalloid cardioplegia [the root cardio is heart and plegia is paralysis, fairly straight forward from there] was delivered directly into the ascending aorta to achieve diastolic arrest. Cardioplegia was readministered at 30-minute intervals by direct aortic cannulation. A tourniquet was placed around the ductus arteriosus to exclude the pulmonary circulation [they tied off the main source of blood to the arteries that carry blood to the lungs]. The pulmonary artery was then divided at its bifurcation. An incision was made in the ascending aorta towards the sinotubular junction. A similar incision was made in the base of the pulmonary artery in the two sites were anastomosed [they joined a portion of her aorta with her pulmonary artery] with running 7-0 Prolene suture to create the root of the neoaorta. The ductus arteriosus was then separated from the pulmonary artery [this is no loner needed as blood can now flow to the lungs through the hybrid aorta/pulmonary artery they created]. The resulting defect of the pulmonary artery was closed primarily. A 6 mm Gore-Tex graft was then anastomosed to a portion of autologous pericardium [donated tissue from herself] after creating a circular defect with a punch. The pericardial skirt was then anastomosed to the distal main pulmonary artery. When the patient had adequately cooled, cardiopulmonary bypass flow was reduced [they turned down the bypass machine] and vessel loops were used to exclude the left common carotid and subclavian arteries. The base of the innominate artery was excluded with a vascular clamp to establish low-flow antegrade cerebral perfusion [they stopped blood flow to certain parts of her body when she was cool enough to maintain blood flow to the brain]. The ductus arteriosus cannula was clamped and removed. An occlusive clamp was placed across the descending thoracic aorta distal to the insertion of the ductus arteriosus. The ductus arteriosus was sharply resected. A longitudinal aortotomy was made from the transected end of the ascending aorta to the level of ductal insertion [they made a large cut in her aorta]. The midportion of the transverse arch appeared to be severely hypoplastic, measuring approximately 0.5 mm in diameter. The arteriotomy was then extended distally beyond the insertion of the ductus arteriosus for approximately 1 cm. A piece of pulmonary homograft was trimmed to the appropriate dimensions and then used to reconstruct the undersurface of the transverse arch with running 7-0 Prolene suture [I think his wording is pretty clear, but to make sure you understand, they used donor tissue to rebuild and expand her aorta]. A brief period of deep hypothermic circulatory arrest was initiated [her entire body's circulation was stopped]. During this period, a limited right atriotomy was performed and a portion of the atrial septum was excised to create an unobstructed inter-atrial pathway [they created a defect that allows blood to flow between the right and left atriums, most HLHS children have this defect already]. The right atrium was then closed with running Prolene suture and low-flow antegrade cerebral perfusion was reinitiated [they restarted blood flow to her brain]. Advancing a right angle clamp through the neoaortic valve, an appropriate site on the right ventricular free wall was selected for the origin of the Sano shunt. A circular punch was used to create a defect and the surrounding muscle was undermined sharply [i.e., they made a hole in her heart wall for the shunt]. The reconstructed ascending aorta/transverse arch was anastomosed to the reconstructed aortic root [they reconnected the aorta and and it's root after they were repaired] using running 6-0 Prolene suture. The proximal Sano shunt was then anastomosed to the right ventriculotomy [they connected the shunt to the hole they made in the right ventricle] using interrupted pledgeted mattress Prolene sutures. The vascular clamp on the descending thoracic aorta was removed, allowing the arch to fill with blood. The vascular clamp on the base of the innominate was then removed to establish antegrade blood flow through the aortic arch and cardiopulmonary bypass flow was increased [they were reopening and increasing blood flow to the heart after creating the shunt]. The patient was fully rewarmed. Vessel loops around the left common carotid artery and left subclavian artery were then released. The patient was weaned from cardiopulmonary bypass. Although ventricular function initially appeared to be adequate, global myocardial dysfunction [her heart was not beating well enough on its own] was evident on echocardiography. Visual inspection on the surface of the heart revealed appropriate filling of the left and right coronary distribution. Cardiopulmonary bypass was reinitiated to rest myocardium. After a period of approximately 30 minutes, we again weaned cardiopulmonary bypass. The patient achieved a sustainable perfusion pressure for a number of minutes. However, she subsequently began to demonstrate progressive myocardial dysfunction.

Given what appeared to be appropriate coronary artery filling and improvement of myocardial function after a period of additional cardiopulmonary bypass support, we elected to proceed with ECMO [went over this before, but to recap, a portable full heart/lung bypass machine] cannulation to support the heart during the early postoperative period. An ECMO circuit was introduced to the field. The innominate artery cannula was disconnected and then reconnected to the arterial limb of the ECMO circuit during a brief period of cessation of cardiopulmonary bypass. The right atrial cannula was then connected to the venous limb of the ECMO circuit in a similar fashion. ECMO flow was initiated. Protamine was given [Protamine counteracts Heparin, it increase pulmonary artery pressure and decreases peripheral blood pressure, myocardial oxygen consumption, cardiac output, and heart rate]. Hemostasis was obtained [they contained her bleeding to a set area]. Chest tubes were placed and secured with suture [to drain said bleeding].

The surgical site was then closed with an Esmarch dressing and an occlusive dressing was placed over this. The patient tolerated the procedure well and was taken to the Cardiac ICU in critical condition. We plan to perform ECMO decannulation once myocardial recovery has been demonstrated by echocardiography.

An appropriately trained resident was not available to assist with this procedure. I have asked the ICU team to assist in the postoperative management of this patient, including establishing hemodynamic stability, ECMO management, and ventilator management.

The Norwood - Lillian's First Open Heart Surgery

 A Basic Overview of the Norwood Procedure

 04/24/13 Update - This post is probably one of the most popular on this blog. Due to this, it gets a fair amount of comments with irrelevant/spam links that do not get through the moderator and do. Because of this, comments on this post that do not link to anything directly connected to SCH or that add peer-reviewed information that I have left out or omitted will be removed. Thanks for your co-operation.

This post is separate from the others as it is intended to be an overview of what the Norwood surgery is. This will be much easier to understand if you've previously read my post on HLHS and heart function itself located here. To help recap though, here's the picture of a normal heart vs a pre operative HLHS heart.

The first surgery of a three surgery track, the Norwood procedure is a long and complicated surgery, taking anywhere from 4 to 10 hours on average. It has a 85% survival rate in children with a textbook case of HLHS; for Lillian this is 80% or lower due to her aorta being far smaller than normal. I think the easiest way to describe the surgery is as a re-plumbing of the heart.

As the most pressing issue with HLHS babies is an inability to pump blood the the body, the goal of the Norwood is to connect the right ventricle of the heart to the systemic circulation (i.e., the blood flow that carries blood with oxygen to the body). The systemic circulation is normally connected to the left ventricle, but since that is not able to pump, it must be switched to the right. Consequently, the blood flow that is normally present in the right ventricle will be disrupted, so part of the surgery has to create a new way for that blood to flow back to the lungs. Since HLHS babies also have small aortas, part of the surgery involves rebuilding the aorta using donated tissue so it is large enough to sustain blood flow to the systemic circulation. Finally, a Norwood procedure must include a shunt as a way to help blood flow back to the lungs. For Lillian this with be a Sano shunt, which connects the right ventricle to the pulmonary arteries through a Gore-Tex conduit. The end result of the surgery is a heart that looks like something like this.

Children's does deviate from other hospitals in how they do the Norwood procedure. Since it's a directly invasive surgery on the heart itself, all patients must be on full heart/lung bypass and typically have their bodies cooled to a near death state to protect the brain since they do not send blood to the brain during surgery. Children's differs in that even though they put the body in a near death state, they keep blood flow to the brain. Kathryn and I were very happy to hear that, as we (and Children's does as well) that no good can come from depriving the brain of blood that long, even in the cooled near death state.

Lillian's Norwood Surgery

Lillian's Norwood procedure took place on August 4th. Since the Norwood procedure usually takes from 4 to 10 hours, they want to start them early. Lillian was no exception, the surgical prep team arrived just after 8 AM to take her to surgery.This was an incredibly stressful moment knowing that there's always the chance that your baby doesn't come back. Thankfully, we were able to walk with them a portion of the way to the operating room until we had to stay behind. We had one final chance to say goodbye, and then she was gone.

Not feeling like sitting and waiting for up to 10 hours, Kathryn and I left the hospital to go spend time with Annabelle during the surgery. The surgical team kept us up to date with pages on an actual pager throughout the surgery, which is where the following information came from.

After taking Lillian back into the OR at 8:37 AM, they started by putting Lillian under general anesthesia, and prepping and draping her chest. and then conducted a pre operative echocardiogram at 10:35 AM. This is standard procedure, as even though they have already done echocardiograms as leadup to the surgery, they want to know as much as possible before they actually open the chest and start the surgery.

At 11:15 AM we were informed that they had started the actual surgery itself. By 11:45 AM, she was on full heart/lung bypass and they had started the actual repair described above. The repair took until around 3:00 PM, at which point they finished and started bringing Lillian's temperature back up, so they could take her off the bypass machine. At 3:55 PM, they succeeded in taking her off bypass, with her own heart finally beating well enough to sustain her life. At this point, we were told to come back to the hospital despite that we would not be able to see her right away.

Once we arrived back at Children's at 5:00 PM, we had a little of a surprise, and not in a good way. The front desk staff informed us that they had not actually taken her off life support, and the surgeons wanted us there, and I quote, "just in case". This is all that they could/would tell us, which was extremely stressful. We found a waiting room and waited for them to tell us more information. We were informed at 5:30 PM that they were going to be putting Lillian on what is called ECMO, short for extracorporeal membrane oxygenation. ECMO is a full heart/lung bypass machine that is portable enough that a baby can be on it outside of the operating room and in the ICU. We were informed at a later time that about 10 to 15% of the Norwood surgery patients came out on ECMO so it isn't common, but isn't unexpected either. It isn't idea to have to be on ECMO, but it is better to need it and be on it than to not be on it. This apparently caused some problems though, as they were still trying to put her on the ECMO machine at 8:30 PM, the delay caused by bleeding issues. Around this time, Kathryn left to take care of Annabelle, so I was at the hospital by myself waiting for them to finish.

At around 10:15 PM, we were told that they were back in Lillian's chest working again, which is never a good sign. Whatever they were doing, it appeared to be working as at 11:00 PM the charge nurse told us that it looked like they were applying the patch to Lillian's chest. Finally, around 11:45 PM she was out of surgery and was wheeled back into the CICU. She had been in surgery for over 15 hours, one of the longest surgeries they've done for this procedure. Keep in mind that this procedure has a range of 4 to 10 hours, with an average of 5, so Lillian was far above average. Even though she was out of surgery, they still had to stabilize her, so I was unable to see her. During this time, the main surgeon, Dr. Michael McMullan, came and discussed the surgery with me.

According to Dr. McMullan, the surgery overall went as expected, but they did have some major surprises. One of the biggest was the fact that her aorta was on average .5mm, the smallest the other surgeon, Dr. Lester Permut had seen in 20 years. The size of the aorta also explains why we were never able to see it on earlier echos. The size of the aorta was due in part to the valve to it being basically closed off entirely. On top of having a tiny aorta, Lillian's coronary arteries (of which there are normally two) actually consisted of one coronary artery which had mutated (for lack of a better word) into going down its side as expected but then coming back up the other side of the heart and serving that as well. Additionally, this is when we discovered that her heart muscle was extremely thick, which makes actually getting blood to the heart itself much more difficult.

One of the reasons for the 15+ hour surgery was the difficulty they had taking her off bypass. It is expected after the surgery that the heart should be able to resume activity and support the body on its own even directly after surgery. This is true for about 90% of patients, but the other 10% have to stay on heart/lung bypass. Before they put her on ECMO, they tried to take her off the bypass machine. Her heart was pumping on its own, but not at the level they expected, so they put her on bypass again and gave her heart a chance to rest. After 30 minutes of rest, they tried again. This time they noticed increased function, but not sufficient, so they gave her a 2 hour break and tried again. And again, they saw increased function, but again, it was insufficient. They finally decided that putting her on ECMO would give her heart the best chance it needed to recover. At this point, she was stable, but touch and go considering all that she had gone through. She still had blood oozing from her heart, and she had 12 hours to stem the tide of bleeding or she would have gotten a second surgery. Dr McMullen informed me at this time that our goals at this time were, in order of significance: getting bleeding under control, getting swelling down, getting her off ECMO and closing her chest.

After explaining all this to me, he left to check on her and write his report (which I've copied in the following post for those interested). As they were still stabilizing her, I had to wait a little longer to see her. During that time, they did an x-ray, where you can see the shunt in her heart.

Finally, at around 12:30, 16 hours after entering surgery, I was able to see Lillian.

This picture shows most everything  that was used to support her. To the right, you can see the top of the ECMO machine (gray panel), to the far left you can see 10 of the 13 IV pumps she was on, with the ventilator being directly behind the nurse. And in the middle, almost impossible to see among the machines is Lillian. 

On ECMO (2 thick red tubes, they're actually clear), the ventilator and 2 drainage tubes (thinner red tubes). She has the PICC line on her left arm, and a general IV line on her right hand. As you can see from the picture, her chest was left open, as there wasn't enough space to close it. They also typically do not close chests while on ECMO.

Just a couple extra angles to see just how she looked. I was fully expecting to be a little light headed when she had her chest open, but it wasn't that bad in person. Perhaps it was the exhaustion and emotions at the time, but it was relieving to see her there. Even though she was on so many machines, she was still there, and that was reason enough to celebrate.

A closer look at the ECMO machine.

Lillian's surgery was one of the hardest days of my life. It was full of extremely trying moments, especially while waiting in the waiting room of the hospital with no word on her status. Even seeing her in the shape she was in, it was so good to see Lillian again, and with a heart that could actually sustain her.

Waiting for Surgery - Lillian's Stay in the NICU

Lillian's first twelve days of life were spent in the NICU at Children's Hospital. For a prenatal diagnosis this is fairly standard, as it gives the doctors a chance to figure out the specifics of a child's anatomy and finalize a plan that is specific for the child. The last thing they want to do is rush into surgery unprepared, so we were more than alright with allowing them the time to prepare. During this time, she had to be on a constant drip of prostaglandin. It's an interesting feeling, seeing a single IV pump that is responsible for keeping your child alive. You can see it in the picture below, middle pump on the right. The other ones were important, but that one alone kept her alive.

The first day that Lillian was in the NICU was the day she was born, July 23rd. On July 24th, our biggest concern was getting a PICC line in as her lines through her umbilical cord were not steady. I don't remember if I explained this already, but a PICC line is a type of IV that is used in some situations where you need more stable access than a normal IV or if you need to be able to draw labs as well. PICC stands for peripherally inserted central catheter, and is inserted from the entrance point and then drawn through the veins until it reaches one of the major vessels just before the heart. According to the nurses who have tried many times to insert the lines (she's been pricked around 20 times in the foot trying to get a line in, so she's not exactly fond of her feet being touched any more) she has rolly veins, so that was an issue for quite a while until they took her to Interventional Radiology, where they can insert a PICC line with the patient asleep (normally the procedure is done at the bedside with minimal pain management) and with the use of imaging technology so they can see exactly how the catheter needs to be inserted.

An arm PICC, this one didn't stay in long as surgeons do not want them in the arms.

Before they attempted to do a PICC line through the legs or arms the first time, they attempted to retry a PICC line through her umbilical cord, as that is the easiest way if it is still an option. Establishing a PICC through her umbilical cord was successful, but the nurse gave her a dose of morphine during the procedure. It appears that she might have gotten too much morphine as she spent the next five hours forgetting to breath constantly. The nurse also never put in an order for the Morphine until five hours afterwards, so we never will know exactly how much she got and if it was too much. This is the one time I have ever been upset with Children's staff, but they were able to fix the apnea by giving her a drug that counteracts morphine. This drug woks instantly, so seeing her go from not breathing to screaming her head off in less than 7 seconds was quite an experience. However, getting that PICC line in allowed us to pull an IV line from her hand and a different PICC from her arm on the 28th. On top of getting the lines pulled, she also had two pre-surgery ultrasounds on the 28th to help the surgeons get extra information.

Our plan from the beginning was to have Annabelle see Lillian as soon as possible, and she was able to do so for the first time on the 25th, although to be honest, Annabelle seemed more interested in Captain Jack Sparrow, who happened to be visiting that day.

Annabelle was thrilled to see Lillian and hold her hand, but for the most part was thoroughly upset at being unable to do whatever she wanted in the hospital room. She did visit several more times, including getting to hold Lillian before the surgery happened.

Aside from having to constantly put lines in, one of our biggest concerns while Lillian was in the NICU was managing her oxygen levels. For a person with normal anatomy, we would expect to see oxygen saturations anywhere from 95% to 100%. Since Lillian is mixing her oxygenated blood with her unoxygenated blood because of her ductus, we would not expect her to be at this level. In fact, we want her to be lower than that, ideally between 75% and 85%. This is because higher saturations in a Hypoplastic child means there's lower blood pressure in the lungs, prioritizing blood flow there at the expense of the rest of her body actually getting oxygen. So not only does her body not actually get enough oxygen with high saturations, but the increased blood flow to the lungs causes them to swell, making the small space the surgeons have to deal with even smaller. All around, not exactly ideal.

To try and accomplish the lower oxygen levels, we had to put Lillian in a sub ambient hood.

In the hood we can regulate oxygen levels, and in Lillian's hood the oxygen levels were about 15% to 16% (normal air is 21%). Because she needed to be breathing in the tent most of the time, we were only able to hold her for 30 minutes at a time, 3 times a day, with several hours (3 to 4) between each time. When we did have her out, she either needed to have a nasal cannula or have her special air blown on her to keep her from getting her saturations too high. In the end, none of these techniques were particularly successful, no matter what we did to lower her levels, she would just breath faster and get her saturations right back where she instinctively wanted them. Aside from being one more piece of evidence for the redhead pile, this exasperated a couple of other issues that she had, mainly poor circulation. We could tell that this was happening simply because of temperature changes across her body, as her core temperature is normally around 36.5 C (97.7 F), while the probe on her foot recorded around 30.2 C (86.4 F). We were able to get her circulation up by increasing her blood pressure by giving her blood transfusions, but never to be at an equal level with her core.

Since we arrived at the hospital on a Friday (7/23) , we had to wait to the next Monday (7/26) to learn anything about the plan for Lillian, as Children's only holds cardiac surgical conferences every Monday. On the 26th, she was presented at the conference along with the information they had gathered through the ECHO. About the only thing we were told from the first conference was that there was unanimous agreement that she needed surgery, and a Norwood to be specific (some HLHS children cannot have Norwoods due to other complications). One blessing about her specific case is that she doesn't have other complications. It is quite normal for HLHS to be accompanied by other defects, none of which Lillian has. She does have severe hypoplasia of her ventricle and her aorta, but she has a text book case of the condition besides that.

From the conference, we knew the date of the surgery was tentatively scheduled for August 3rd. As we approached the date of the surgery, we had to make some changes to prepare. First of all, we needed to change her to a NG feeding tube as we couldn't have her eating by mouth and spitting up, as this would postpone the surgery, even though she was only getting 5ml every hour or so. This was placed on July 30th. Also as mentioned earlier, we had to pull out her umbilical cord lines due to that falling off, and were only able to get a PICC line in her arm after failing multiple times with her foot and arm, and even then only at IR. That took quite some time to get right as they started trying on July 30th, and were only able to get it in on August 2nd.

Even though the surgery was scheduled for the 3rd, it was pushed back due to 2 heart transplants in the same night. Finally, on August 4th, it was time for the biggest day in Lillian's life, and what will likely be the longest and most complicated surgery she will ever have, even though she will only be 12 days old. We knew in advance that we would not return to the NICU after surgery, but we were very fond of the nurses who were there.