Long story short...(not really)

It's been too long since the last update, I know. Sorry about that, the last few days have been a whirlwind, so we haven't had a chance to put together an update. We have a lot of information, so we'll try to keep it short and sweet.

MONDAY

On Monday, Zoe was again promoted from the CICU to the CCU/Step-Down Unit. The Lovenox injections started, and Zoe is not a huge fan of being poked with a needle twice a day, but she tolerates it pretty well. I think she is growing accustomed to the torture of her lengthy hospital stay. Her least favorite thing, is when the nurses take her temperature under her armpits, that sends her into a rage. Actually, that might be #2, #1 is hunger. Other than that, she is pretty tolerable. Quite often we hear from the nurses, "she is a really good baby."

Monday night was Grandpa and Grandma Muenich's, and Emerson's, last night in Philly. They are off to Pittsburgh, PA for Grandma Muenich's Doctoral graduation from Pittsburgh Theological Seminary. Look at the big brain on grandma! In honor of their last night in town, we went for late night cheesesteaks at Jim's Steaks.

We already miss them all, but are so glad they were here to help. Without their sacrifice, picking up and spending three weeks in Philly, this journey would have been exponentially more difficult, if not impossible.

They certainly had some good times with Emmy, and Zoe, while they were here.













We even got one group photo, sort of, Ben Franklin substituted in for Zoe.



TUESDAY

Just another manic Tuesday. What a crazy day!

Tuesday was the day we had to check out of our rental home, however, we obviously need to remain in Philly. Before we left Arizona, we put our name on the Ronald McDonald House waiting list. The Ronald McDonald Houses, located across the country, provide housing to out of town families, whose children are hospitalized, generally for only $15 a night, which includes dinner. The Philadelphia Ronald McDonald house was the original location.

Unfortunately, the house does not take reservations, they have a wait list, but it's based on availability only. You have to call the morning of your desired stay, and hope they have a room.

We had to be out of our rental home by 11:00 a.m., but wouldn't learn our fate at the Ronald McDonald House until 12:00 p.m. So we rented a car, by the hour, loaded two months worth of possessions in it, and drove around around, homeless. Finally, we received a call from the social worker at the Ronald McDonald house. She said she had a room for us. The bad news, however, is that the room is tiny and does not have a full bathroom, only a sink and toilet. The shower is down the hall, in the restrooms - dorm style. Despite the downfalls to the room, we had no choice - we'll take it. So we immediately drove over, checked in, unloaded our bags, then trekked to the hospital in the pouring rain.

Once at the hospital, the doctors kept throwing around the word "discharge." It took us a second to process what that is, not really, but it seemed weird to finally approach an end to this chapter, this hospital stay.

As part of the discharge requirements, we attended a Lovenox class on Tuesday. Essentially, a nurse taught us how to use a needle to draw the medicine out of a vial and how to inject it. We were in a small classroom and we practiced our injections on rubber squares. I'm pretty sure we are Lovenox pros now. Zoe gets the shot twice a day. We were told that we need to give her the shots, until she is discharged. So, when 8:00 p.m. on Tuesday rolled around, we decided I would give the shot first. Stacey stood near Zoe's head and comforted her, while I gave her the shot in the leg. As usual, she was a champ. She has little marks all over her arms and legs from where the twice daily shots have been inserted.

Zoe also had to undergo a car seat test Tuesday. They put her in her car seat for several hours, to simulate long distance traveling. Apparently some kids, when seated in a car seat, for some reason their anatomy causes their respiratory system to be cut off or compromised. If that happens, then the child will need to be transported in a flat car seat. Fortunately, Zoe passed with flying colors.

While at the hospital, the doctors did one final check of Zoe and said she was ready to leave the hospital on Wednesday. Her primary cardiologist at CHOP, however, because we live so far away, wants us to spend a week with Zoe at the Ronald McDonald house. Next week, we will return to the hospital for an echo cardiogram and an office visit. If everything checks out, we will return to Phoenix!!!!

After hanging out with Zoe into the evening, we returned to the Ronald McDonald House to settle in.

Once we were at the house, I received a call from my mom's neighbor who reported that she stopped by her house and was unable to really speak clearly or communicate her thoughts and had stroke like symptoms. So apparently she called 911, and the paramedics came and took my mother to the hospital. She appears to be stable now, but is still in the hospital and I have way too many family members in the hospital right now for my liking.

WEDNESDAY

After a largely sleepless night, Wednesday morning we had to be at the hospital by 7:30 a.m., so Stacey could administer Zoe's Lovenox injection. She was nervous, in fact right as she almost had the needle in the thigh, she paused, but she gave the shot and Zoe even cried less than when I gave it to her. Good job ladies.

So, in between calling the hospital in Arizona, where my mom was admitted, and her doctors, we worked on the logistics for Zoe's discharge. The hospital was supposed to provide boxes and dry ice to ship three tubs of breast milk back to Arizona, however, they were out of boxes, so we had to rent a car and drive to South Philly, to a random dry ice store and buy the special boxes - necessary to ship dry ice. We then went to CVS to pick up Zoe's prescriptions, however, when we arrived they said they did not have any prescriptions for Zoe. So we dropped off the hourly rental car and returned to the hospital. After telling the nurse about CVS, she gave us the prescriptions, five in total, and suggested we run them over to CVS ourselves. After glancing at the prescriptions, Stacey noticed that the Lovenox was missing. The nurse seemed confused and paged case management. Case management, the department that assists with discharge and the related logistics, admitted that they dropped the ball. Because of the holiday weekend, we got lost in the shuffle and the medicine wasn't ordered. They arranged for all the medicines to be available on Thursday. Then will really take Zoe out of the hospital and "home" with us to the Ronny Mac house for the next week or so.

Zoe's discharge from the hospital is bittersweet. Obviously we want to grab her and run out of there with her. But given her condition, it's scary to take her out into the world for the first time, away from the caring and safe hands of the doctors and nurses at CHOP. But its time and we are lucky that Zoe won this battle and is doing as well as she is, despite a couple of set backs.

Tomorrow signifies the beginning of a new chapter in Zoe's journey.

Pesky clot

Zoe had another ultrasound this afternoon. As I indicated before, the clot in her left leg encompasses two different vessels. After nearly 48 hours on low dose tPA it cleared out of the smaller vessel, but is still occluding the larger artery, the one needed for future catheter interventions - which is why they are so motivated to get the clot out.



Because Zoe is on the tPA, they are taking blood for labs every six hours. Because Zoe is small, and her veins have a tendency to roll once the needle punctures the skin, it is very difficult to draw blood from her. To avoid the challenge of searching for blood and sticking her every six hours, on Wednesday morning they inserted an arterial line which allows them to regularly draw blood directly from the art line.

Late Friday night, the art line became kinked, and it was removed early Saturday morning, at about 4:00 a.m. I even put on a surgical mask and helped with the procedure. Once the line was removed, however, they were unable to obtain blood without sticking her again. So, they let the labs for today pass. In the interim, before they decided what to do, they wanted to review the results of today's leg ultrasound, so they can gauge the effectiveness of the tPA.

First, the talk was of reinserting the art line and continuing the tPA for another 24 hours, for a total of 72 hours. Physicians from hematology, the catheter lab, cardiology and neonatology all pow-wowed throughout the day on what to do. Since insertion of an art line, which would need to happen if the tPA would continue for another 24 hours, carries certain risks, including damage to the artery in the wrist, where the art line would be inserted, and for the second and third open heart surgeries, an art line will be needed, so damaging it now could possibly jeopardize its access for future surgeries.

One potential side effect of the tPA is excessive bleeding. After the tPA was started, the catheter site in her left groin started to develop a hematoma, which periodically oozed blood. Friday night, one of the critical care fellows had to hold pressure on the hematoma for 15 minutes, to stop the bleeding. Additionally, the hematoma continued to spread, which was a concern, as it was evidence that the tPA was causing some internal bleeding.

So, in the end, the decision was made to cease the tPA and Heparin Saturday night at 9:00 p.m. At 10:00 p.m., they will start Lovenox, which is a medicine, delivered via subcutaneous injection, which will prevent the clot from growing, or future clots from developing. They will also do a follow-up ultrasound on Sunday morning to determine if the tPA broke the clot down any further between the time of the last ultrasound and the stoppage of the tPA.

Our bodies naturally produce tPA, so often clots will reabsorb, on their own, over a course of several months. The hope is that Zoe's body will continue to reabsorb the clot over time, possibly even in its entirety.

Now, the plan is to start the Lovenox and monitor the blood to get the Lovenox dose to the appropriate level. We also have to take a class to learn to administer the Lovenox injections once we arrive home. Zoe will also start aspirin on Monday and will continue on Lasix (for fluid retention).

Saturday night, after the tPA ended, Zoe was able to get the IV out of her foot, which was wrapped up like a giant boot. The actual site of the IV, on the side of her foot, was pretty bruised so I can't imagine that felt good.

We gave her a bath tonight, put her in some clean clothes and Stacey held her for a long time.



Zoe looked great! Her hair has some natural waves and curls, that are really highlighted right after its washed. It was obvious on her face that she felt better.



She has been through so much in her first two weeks, you can see some old soul in her face. Ms. Zoe is physically strong too, several times while Stacey was holding her she was lifting her head and holding it up. She kept scanning all the machines that, in the last week, rendered her a prisoner in her crib. Now she could be held again, and she and momma both enjoyed it.



Tomorrow/Sunday (the days are all blending together) we will likely return to the CCU/step-down unit and we may be looking at a DISCHARGE sometime next week. Yes, I said discharge! Assuming no more set backs, we will be discharged next week.

This journey was, is, and will continue to be a roller coaster, so who knows where the next climb, drop, or turn will be. For now, however, it would be nice if we could just climb for awhile. I'm sure sweet Zoe would appreciate it. We'd all appreciate it.

The Lihns

Still clot busting...

Last night, we spent the night in Zoe's room again. Because of the potential side effects of the tPA we did not want to leave, nor sleep. We were each able to catnap for 2 hours, but otherwise we observed and/or comforted Zoe. She was pretty fussy last night, more so than usual, as she is generally pretty mild-mannered. It appeared she was uncomfortable, although with IV's in three of four appendages she had every right to be pissed.

They ran a blood test at 3:00 a.m. which appeared normal. Every hour the nurse checked Zoe's neurological status and the sites of all her IV's, for bleeding (a side effect of the tPA).

Zoe also had an ultrasound of her head to make sure there was no brain hemorrhaging as a result of the tPA. Fortunately, the head ultrasound was normal.

This morning Zoe had another ultrasound of her right leg, so the physicians could assess what effect, if any, the tPA had on the arterial clot. The ultrasound came back the same as the last one, but the doctors could feel, and hear, a stronger pulse on the foot of the clotted leg. Hematology and cardiology consulted and agreed that based upon the pulses and appearance of the leg, the tPA seems to be working, and it's not abnormal that after 18 hours of tPA the occluded clot is not altered. They agreed to leave the dose the same and obtain another ultrasound of the clotted leg tomorrow morning. Zoe also had another chest x-ray this afternoon, but the results are not yet back.

Overall, while the ultrasound was status quo, the doctors seem confident that the tPA is working away trying to bust the clot. Hopefully, we will see good progress on the Saturday morning ultrasound. Once the clot is dealt with, Zoe can have all three IV's removed, then we can lift her out of bed and hold her again. Plus she will be exponentially more comfortable.

We will probably stay in the room again tonight. We'll update again when we have more information.

Clot Busting

Today's ultrasound demonstrated that the clot, in two of the arteries in Zoe's left leg, did not reabsorb or shrink. Zoe's medical team is concerned enough about the clot, that they want to get more aggressive in its treatment.

Apparently the clot does not pose any immediate health risk to Zoe or her leg, as the blood flow is making its way down the extremity through other veins and vessels. Long term, however, the clot could create some big problems. One, if the clot is left untreated it will render the artery useless for future catheterizations, which she will need. The catheter team was apparently concerned enough about that possibility that they are pushing for aggressive treatment. The second potential long term effect of leaving the clot untreated is that the leg may not grow at the same rate as the un-clotted right leg, because the clot alters the level of blood flow. Therefore, her legs would be different lengths.

The treatment that was suggested to break up the clot is tPA. tPA is a clot busting drug, that is most often used in the treatment of strokes. It is a very strong medicine, with the potential for some serious side effects. We'd heard of tPA, but didn't previously know much about it. Just the mention of it, however, made us very nervous. We immediately started doing research on the drug and its use. Not surprisingly the medical team at CHOP did not take the decision to recommend the tPA lightly - Zoe's cardiologist, neo-natology team, catheter team, cardo-thoracic surgeon, and a pediatric hematologist, specializing in blood clots were all consulted and part of the decision making team. We met with the hematologist and peppered her with questions. She indicated that the recommendation is to run the tPA at the lowest dose for 48-72 hours. She said some hospitals run a larger dose, for a shorter period of time, but she said the recommended approach has less risks of complications.

The major complications that could result from tPA are significant bleeding and brain hemorrhages. After weighing everything, we decided to approve proceeding with administration of the tPA, in the hopes the clot will be busted, then we would no longer have to worry about the clot or the possibility of administering Lovenox (a blood thinner) injections for several months after we return to Arizona.

Before the tPA can begin, they have to administer a dose of platelets. That already happened. The tPA is hooked up and literally just started as I am writing this. Needless to say, because of the seriousness of the side effects, we are going to stay in Zoe's room and observe her all night. Right now, we are nervous, but hopeful that the tPA does its job and busts this stubborn clot.

Right now, because the tPA needs a dedicated line, Zoe has an IV in each arm and her right leg. Poor thing is getting so beat up.



Please send prayers and positive thoughts and energy that the medicine works and the clot is busted. If it does, then all three IVs can come out and we can get back on the road to discharge and a return to Arizona.

In the meantime, here's another family picture.



The Lihns

Exhaustion, Clot, and Reflux

As you know, last night was a very difficult night for Zoe; and her mommy and daddy.

After the last update, late last night, the physicians explained that Zoe had an occluded blood clot in her left leg. This is not an uncommon side effect from the catheterization procedure. As a result of the clot, a Heparin IV drip was started. Heparin is an anti-clotting agent, which will not necessarily dissipate the clot, but will prevent it from enlarging and ideally prevent additional clots from forming.

Because of the Heparin prescription, there was a need to obtain a blood sample to determine Zoe's PTT level, which is simply how fast the blood in your body clots. Ordinarily it will take 30 seconds or so, on Heparin, it should be double, or in the range of 60-80. With the medicine Zoe's was at 45. In order to continue to test this level, and thus determine if Zoe is receiving the proper amount of Heparin, regular blood tests must be run. This is why they were trying endlessly to get blood from Zoe last night. Throughout the night, and early morning, they continued to try, trying different spots on the body, trying different types of lines (IV versus arterial lines), several different people tried. At one point, when Zoe hadn't eaten in nearly 24 hours (she had to fast for the catheterization, which was delayed, then lie flat for 6 hours afterward) after they were trying to stick her for 10 minutes, Stacey finally stood up and said "No more, this is getting ridiculous. She needs to eat." Two nurses and a fellow, looked at Stacey with almost a sense of fear. The fellow explained why they needed the blood, but agreed to stop and said they would try again later. They quietly retreated from the room after receiving the rath of Stacey.

After Zoe was finally able to sit up, we fed her, and after fasting for nearly a day, she ate like a monster. We tried to feed her sooner, but as I said in an earlier update, because she was laying on her side she choked and had to have the liquid suctioned out. To try to hold her over, every hour or so, I would inject sugar water, through a syringe, into the corner of her mouth. As she gobbled on the end of the syringe, you could see the hunger on her face. It was tough to see her suffer all day.

We ended up staying with Zoe until 7:00 a.m., after arriving at 9:00 a.m the day before. We didn't have any sleep in that 22 hours. Despite our desires to remain with Zoe, we went home to sleep for five hours or so. Then we returned to the hospital this afternoon.

When we arrived at the hospital Grandma and Grandpa Muenich were already there with Emerson. Emerson even borrowed a bike from the play room.



Zoe was much better today, however, she still has the clot. Sometime this morning, another fellow was able to insert an arterial line in Zoe within 5 minutes. Now, they can get whatever blood they need from that line and no longer have to stab her incessantly for blood. Based upon the PTT levels they drew this afternoon they increased the Heparin.

Thursday morning they will do another ultrasound on Zoe's leg. The hope is that the clot will be smaller or in the process of breaking down. So, please send positive thoughts and prayers that Zoe's body is beating away the clot. Once they run the Heparin IV for a day or so, they may be able to switch to a blood thinning regiment of aspirin and perhaps Lovenox, which is given subcutaneously through a needle, similar to insulin. We will know more about the prognosis and treatment of the clot over the next couple of days.

Zoe's appetite was very good most of the day today. Especially when mommy was feeding her.



During an evening feed, for the first time, Zoe arched her back, kicked her legs and screamed out in pain. The nurse, Stacey, and I immediately recognized that Zoe was having acid reflux, which is not uncommon in HLHS babies. It was clear she was still hungry but the reflux was making her miserable. As is common knowledge by now, she is a tough cookie and does not usually scream or cry too much, even when she became the human pin cushion last night, but this was causing her some real discomfort. So the nurse ran out and got an order for Zantac. By the time the prescription was filled, however, Zoe's discomfort subsided and she fell asleep. So, the next time she wakes up tonight, and before her next feeding, the nurse will give her a small dose of Zantac. Hopefully, that will relieve her reflux symptoms and allow her to eat in peace.

Poor girl is already uncomfortable, with all the IV's and wires on her, but still cute as hell. Hopefully her reflux was isolated and doesn't require prolonged medication or even additional treatment.



We will update more tomorrow.

The Lihns

We could all learn something from her.

So, things did not turn out as we'd hoped. Once the doctors were inside the heart and took a close look at the shunt, they immediately noticed some pronounced narrowing at the top of the shunt, the same location where the shunt originally kinked, during the Norwood. Where there's smoke, there's fire and apparently Dr. Spray smelled smoke when he ordered the cath. We were told this evening that "Dr. Spray hardly ever orders caths." The original kinking of the shunt apparently made him nervous. Good thing, because the shunt was pretty narrow. the cardiologist told us we were lucky, because all her numbers/vitals were good, such that you wouldn't expect narrowing to be present, but it obviously was, so had the cath not been done, at any point the shunt could have failed or become compromised.

In order to fix the narrowing in the shunt, the doctors placed two coronary stents, the exact same stents used in adult hearts. At the time of the Glenn surgery (the second of the three required open heart surgeries for HLHS), in approximately 3-4 months, the shunt and the stens will be removed. In the interim, however, the stents traditionally do a good job of holding open the shunt, but with that comes the increased risk for blood clots, also a potentially fatal side effect.

Tonight, Zoe will go on a round of Heparin, a blood thinner, then tomorrow through the Glenn, she will be on a daily dose of aspirin and Plavix, both thinning agents to aid in the prevention of clots.

To make the night harder, after we were able to go see Zoe in the CICU, the nurse said Stacey could try feeding Zoe, since she hadn't eaten since 5:30 this morning. She has to remain flat, however, for six hours post cath, so we had to feed her on her side. She immediately latched on to the bottle and started eating with a fury. After taking about 8 ml she starting gurgling. I immediately recognized that she was choking, but our nurse was in another room. Zoe's gurgling and gasping grew in intensity and volume and it was quite clear there was a problem. I ran and grabbed the first nurse I could find and said "get in here now!" She came in and as she examined Zoe, we saw her oxygen sats drop into the 50s, a dangerously low level. The nurse could not see anything in the airway, but I was not shy in pointing out that she was still choking. The nurse then grabbed suction to vacuum the liquid out of Zoe's throat, then they put a oxygen mask over her face. The blue in her lips started to fade, her gasping began to subside, her sats started to rise, but none of this did anything to eliminate the panic we felt. I was immediately overcome with anger. Not at the nurses, but in wondering why does Zoe have to go through all of this.

Minutes later, on rounds, the physicians needed to obtain some blood for labs. For some reason they were having trouble and they stuck her in both arms, multiple times, then went to her groin. After spending the day in a cath, then choking, she was being poked and prodded an inordinate amount of times. Just seeing her go through this brings tears to our eyes. While they were taking blood, the charge nurse turned to me and said, "Dad, you really have a brave one here. We could all learn something from her." "Damn right," I said. "I've already learned a ton from her."

Zoe is resting right now, after a very hard day.

To anyone reading, please send positive thoughts and prayers that Zoe's stents do their job and she has no complications or clots and nothing but smooth sailing until her Glenn procedure. Then ask people you know to send the same thoughts and prayers. Zoe is tough as nails, but she can use all the help she can get.

The "I" word! Intervention.

We just received another update. Unfortunately, because the shunt is either narrowing or kinking, the cardiologists deemed it necessary to place a stent in the shunt. They are just beginning that process now. After the stent is implanted, they will take some more pictures, pressures, and measurements from the various heart chambers. Because intervention was required, the procedure could last another two or three hours.

This likely explains Zoe's low oxygen sats yesterday. We told several nurses and at least one physician that's what we thought, but they didn't seem so sure. Well now we know. It's a good thing that cath was already scheduled, so any larger problems could be avoided.

Obviously we have many questions, and won't have a chance to get those answered until after the procedure, when the doctors come talk to us.

Keep your fingers crossed that the rest of the procedure goes well and the stent does the trick for the next several months, in terms of maintaining the integrity of the shunt.

We will receive another update in 30 minutes or so.

Cath update

We received our first update on the cath. Zoe tolerated the anesthesia well and is fully sedated and intubated. The cardiologists inserted the catheter and are currently measuring and taking pictures of the inside of Zoe's heart. It is not yet clear if any intervention will be required. Let's hope not!

So far so good!

We should receive our next update in 30-45 minutes.

Cath, where are you?!?!

So, we are still sitting in the CPRU pre-op room, waiting for Zoe's cath, which was scheduled at 11:00 a.m. Fortunately, Zoe has been a trooper and has slept in our arms most of the day.

We just learned from the nurse, that they still want to proceed with the cath, and anesthesia is on the way up to get Zoe now. Finally!!

See how patiently Zoe is waiting.



We will update when we can.

Waiting...

We're sitting and waiting in the prep room. Zoe's doc came in and announced there may be an emergency "case" which will take precedence. They may push her cath back, or reschedule it. Let's hope they tell us soon, since Zoe is STILL starving. Luckily she's sleeping in daddy's arms right now and is content. Will update when we get word.

The path to the cath

Zoe's catheterization is still on pace to happen at 11:00 a.m. this morning. Zoe is anxious to get it over with, because she is not allowed to eat before the procedure and she is starving and is not keeping that a secret.

In a few moments, staff from the Cardiac Preparation and Recovery Unit (CPRU) will come get Zoe to begin prepping her for the procedure, including administering the anesthetic. That process will take about an hour. Once the catheterization gets underway, the procedure will take an hour or so, unless additional intervention is required.

Once the procedure is completed, Zoe will return to the CPRU for another 5 hours or so, as she has to lie flat and can't move her legs for that period of time. After that, she will either return to the Cardiac Intensive Care Unit or the Cardiac Care Unit/Step-Down unit, depending on how she is doing after the procedure.

We will obviously know more later and will update as we receive new information.

Alarms, good timing, and presents

Zoe continues to eat like a champ, generally exceeding her minimum requirement at every feeding. Today was no different, however, her oxygen saturation levels were very low, the lowest they've been since her surgery. For a normal infant, you would expect to see oxygen sats in the neighborhood of 95% to 99%. In HLHS babies, post-Norwood, the sats can range anywhere from the 70s to the 90s. Every child is a bit different, but you learn where their range is and monitor it closely. If they vary from their normal range it can signal a greater issue, such as a problem with the shunt, which was surgically implanted during the Norwood.

Since her Norwood, Zoe's sats have consistently ranged from 80% to 88%, sometimes they creep into the 90s and occasionally they dip into the 70s. Today, however, they were in the lower 70s for significant periods and even dropped into the upper 60s a few times. Her monitor is set up to sound an alarm every time her sats drop below 75%. Needless to say, her alarm went off quite a bit today. In fact, on our walk home from the hospital, which meanders us through the tranquil campus of The University of Pennsylvania, Stacey turned to me and said "I can't get the sound of that alarm out of my head." I knew exactly what she meant, we exchanged a look, and without saying a word to one another, we knew this was another dip on the roller coaster of having an HLHS child.

Now, this could be a benign finding, but one of the primary indicators of inter-stage (the period of time between two of the three operations) problems with HLHS children is a decrease in their sats. Occasionally, a shunt fails, and the blood which was to be redirected from an artery to the lung vessels is not redirected as intended, and therefore the lungs are not receiving the appropriate amount of oxygen. This can be fatal.

If there is time to repair a faulty shunt, an interventional cardiologist can perform a cardiac catheterization and use a balloon procedure to open the shunt or implant a stent to hold it open.

In a stroke of good timing, Zoe was already scheduled to undergo a cardiac catheterization on Tuesday morning. Since our journey home is lengthy, Dr. Spray ordered an investigatory cardiac catherterization to be performed approximately 10 days post-surgery. His thinking, wisely, was if there is a problem with the shunt, better to find out and deal with it prospectively, while we are still in Philly. There is also some heightened concern in Zoe's case, because her shunt had to be revised during surgery, when it became kinked.

We spoke to Zoe's nurse moments ago (it's about 11:00 p.m. here) and she indicated that the sats seemed to balance out a bit, and were holding in the low 80s. That's definitely good news, but we're thankful that the catheterization is scheduled for tomorrow morning at 11:00 a.m. Earlier today, we spoke to the interventional cardiologist, who will perform the procedure. He desrcibed that they will sedate Zoe, and insert a breathing tube in her. Once she is fully sedated, they will insert a thin tube, or catheter, in her groin through a blood vessel or artery. The tube will then be guided, with the assistance of an interventional radiologist, through the torso up to the heart. The catheter will first enter the right atrium - the top right-hand chamber of the heart that receives oxygen-poor (blue) blood from the body. Eventually, the catheter will be guided into the right ventricle, the pulmonary artery and potentially the right and/or left pulmonary artery branches.

While inside the heart, the physicians can use the catheter to obtain blood samples from each chamber of the heart, check the oxygen content of each chamber of the heart, measure the blood pressure from all chambers, and inject dye, to see how it flows through the heart. Lastly, as I said before, if a problem with the shunt is discovered, a stent can be placed to support or reinforce the shunt. The catheterization will tell us if today's low sats were correlated to a problem with the shunt.

There are risks to a catheterization, as there are risks to any surgical procedure. Indeed, the risks increase if some type of intervention is required. The hope is obviously that the procedure provides evidence that the shunt is not compromised and today's decreased sats were simply an aberration, and therefore no intervention will be required. If intervention is required, however, than we are lucky that Dr. Spray ordered the procedure to occur while we were still in Philly.

In other Lihn news tonight, Ms. Emerson turned 2 today! We did not match the gala we threw for her first birthday, but we made sure she had fun. She opened presents.











She ate a birthday brownie.



And she broke in the highlight of her birthday, her new keyboard with a built-in microphone.





Thus far, Zoe has been receiving the lion's share of the credit for demonstrating strength and resolve, every bit of which she deserves. Emerson, however, has also shown herself to be one hell of a trooper. We suddenly whisked her away from the comforts of her home, crib, and playroom and brought her to a weird city, and took up residence in a weird house. Then when Grandma and Grandpa Muenich joined us and Zoe was born, Emerson all of a sudden started spending nearly all her time away from mommy and daddy, while we spend all our time with Zoe. Emerson has seen each of us hold Zoe, and has to know that Zoe is the strange baby responsible for cutting into her time with mom and dad. Through all of that, she has been a champ. I can count on one hand how many tantrums she has thrown since we've been here. She has shown nothing but compassion and interest towards her new baby sister and nothing but patience with her mom and dad. I was already prouder than words can say of Emerson, but her behavior on this journey has been nothing short of remarkable. We are damn lucky to have Emerson and Zoe in our lives.

By the time many of you read this, Zoe will be in the midst of her cardiac catherterization or already done with it. In any event, please send positive thoughts and prayers that Ms. Zoe's procedure goes smoothly and that no intervention is needed. If it is needed, that the doctors perform at the top of their game and the intervention turns out as desired.

Obviously, we will provide updates, when we receive them. Thanks again for all your thoughts, prayers, and supporting words.



The Lihns

Zoe has Firepower

Sorry for the delayed update, however, better late than never.

On Friday, much like the day before, our focus with Zoe was on her eating. The dietitian, nutritionist, and physicians decided to adjust Zoe's feedings a bit, taking them from 50 ml a feeding to 45 ml a feeding, however, they want to fortify her milk with additional calories, to help her gain weight. As before, if Zoe doesn't meet her goal at each feeding, then the remaining milk, up to her quota is delivered through her NG tube.

As has become her pattern, Zoe rose to the challenge. Since the new goal was set, Zoe has taken all she needed, plus more, via her bottle!! She has been drinking 55, 60, 55, 50, 60, etc...way more than required. Her performance provided hope that the feeding tube could soon be removed.

Friday was also mine and Stacey's 11th anniversary. Under the circumstances, we weren't up for a traditional celebration, but I scheduled a massage for Stacey and we went out for dinner. Dinner was excellent, but we couldn't stop thinking about Zoe and her feedings, so we decided to return to the hospital for a late night visit and just in time for her 9:00 p.m. feeding.

When we arrived, just before 9:00 p.m., Zoe was sound asleep. We woke her up to start her feeding. As usual, before we started the feeding, we told her what her goal was and that we knew she could do it. I started feeding her and she was drinking like a mad woman. Every time she feeds, I feel like I'm holding my breath, and nervously watching the liquid in the bottle slowly pass the measurement marks. Then I do the math in my head and start to calculate how far she is from her goal, at every moment of her feeding. Her feedings are stressful. If she hits her goal, I immediately breathe a sigh of relief. So, back to the 9:00 p.m. feeding, Zoe starts off zipping along. We started at 60, she quickly hits 50 ml, then 40 ml, then down to 38, then she starts to slow down. Often, when she slows and starts to doze off, we will change her diaper, to get her riled up in hopes that she'll take the remainder of her bottle.

The diaper change started off like any other. I had the old diaper down, then started to clean her up, then...SQUIRT!!! A cannon of bowel movement erupted from tiny, sweet Zoe!! The launch was audible and the contents were propelled with velocity, so much so, that when the launch occurred, I immediately yelled "Oh shit!" (How appropriate.) After I quickly recovered from the shock of what happened, I thought, we need to change the sheets on this bed, so I panned down to see how bad the damage was. As I panned down, however, I quickly realized that Zoe's propulsion sprayed all over my shirt. My immediate reaction - laughter. The nurse heard me yell, so she walked in the room, then she saw my shirt and started laughing and said she'd never seen that before. I'm glad I could be a pioneer of something. Stacey was turned around so she had not yet seen the damage, so I turned around and showed her, she immediately started laughing so hard that she spilled a bottle of milk all over her shirt. The whole thing played out like a scene from a National Lampoon movie. After we all stopped laughing, the nurse said you must have another child, because a first time parent would not react like this. Although, even if Zoe were our first child, after what she's been through, and will go through, I can't imagine reacting any different, even is she poops all over my shirt again.

Oh, I know what you're thinking, of course I took photos of the poo stained shirt.



I even got one of Stacey's shirt after she spilled the bottle of milk.



One more thing, after I cleaned up Zoe, and myself, we resumed her feeding and the all star took 60 ml.

On Saturday morning, I called Zoe's nurse to find out how her overnight feedings went, both feedings were 60 ml! The girl is on fire.

We arrived at the hospital a few hours later and immediately noticed that her NG tube was removed. We asked the nurse when the doctors decided to remove the tube. She said, "they didn't, she did" as she pointed to Zoe. This girl never ceases to amaze me. She told us that rarely does an infant remove her own NG tube; usually 3-4 month olds do this, not an infant. Obviously, she doesn't know our little Zoe is feisty, just like her mom.

Zoe, sans NG tube (and preparing to ask for one million dollars a la Austin Powers).



About an hour later, one of the attending cardiologists, from the CCU, came in and reiterated how well Zoe is doing. She said because of how much she is eating on her own, at this point, there is no reason to reinsert the NG tube. She also confirmed, pursuant to Dr. Spray's request, that a cardiac catheterization was scheduled for Tuesday. This will allow the physicians to have a better look at the anatomy of the heart and to prospectively determine if there is a problem or a potential problem, before we return to Arizona.

We were also scheduled for an NG tube training class on Tuesday, but it appears that Zoe will get us out of that. Good Girl!

After spending most of Saturday morning with Zoe. We, along with Grandpa and Grandma Muenich, took Emerson to Healthy Kids Day. The event included performances from Hip Hop Harry and the Laurie Berkner Band. As always Emerson loved the music.



She also got to meet Hip Hop Harry.



Oh, and they were passing out plenty of healthy foods and beverages, so we stocked up. Emerson even took inventory at the end.



Saturday evening, we returned to Zoe's bedside and discovered that she continued to take as much or more than she needed at all of her feedings. We stuck around for two more feedings and she continued to meet or beat her quota.

Overall, she is doing phenomenal. She is only on two medicines - lasix and aspirin - and the only wires she is connected to are simply to measure and record her vital signs.

Thanks again to all who have Zoe in your thoughts and prayers. Even though she is doing very well, she still has a long road in front of her, including a second, of three, open heart surgeries which will likely occur in August or September. So, please continue sending positive thoughts and prayers to Zoe and our family.

Love,
The Lihns

CCU, Eating, and Emmy

Zoe is now in the CCU. Which comes complete with a larger bed and a canopy that looks like something from a Hazmat training exercise.





We spent a lot of time with Zoe today working on her feedings. He goal is 50 ml per feeding. Her feedings are scheduled at 12, 3, 6, 9, 12, etc. If she doesn't take at least 50 in a sitting, she is given the difference, up to 50, through her NG tube. So, the goal at the beginning of every feeding is getting to at least 50. If we get a few days in a row strung together, without the need for the NG tube, then they will likely remove it, before we return to AZ.

When we arrived at the hospital today, we were told that Zoe finished both her bottles on the overnight shift. Then, in the morning, she had one, which she only took about half of. We were there for the next feeding. We filled the bottle up and started feeding, 50, 40, come on Zoe, 30, 20, almost there Zoe...you can do it, 10, 5, 4, 3, and no more. Zoe hit the wall, but she gave it a valiant effort, so she had a measly 2 ml's pumped into her NG tube. Not too shabby.

Before the next feeding, I laid her in my lap, and we gave her a pep talk. She probably understood about, oh 0% of it, but maybe she heard the desperation in our voices. Because we are optimists, we filled the bottle up to 73 ml's. She must have taken something away from our pep talk, because she stepped up and delivered a personal best 63 ml's! Nice work! Poor girl was fighting to stay awake, but she delivered, as she's been doing since she was born.

Time for the 9:00 p.m. feeding. She was still passed out from the end of the last feeding, but we woke her up, changed her diaper, she received her lasix (to help with fluid retention), and we put her in Stacey's lap. Stacey gave her another pep talk and said she was going to meet her goal on this feeding. We filled the bottle up to 70 ml's. After spending a half hour, and nudging her awake once or twice, Ms. Zoe delivered another solid performance and took down 55 ml's. Thus, no need for the NG on the second feeding in a row.

We left before the midnight feeding, but we told the nurse we would be disappointed if he didn't get Zoe to break her personal best of 63 during his shift. We need to keep the string of NG free feedings going.

The ultimate goal of the CCU is to prepare the patient, and parents for discharge. So we were signed up for an infant CPR class tomorrow and an NG class on Tuesday, although we are holding out hope that the NG class will be moot and not necessary. We didn't see the cardiologist today, but we heard a rumor through the nurses that there may be some concern about flying home with Zoe on a commercial plane. Listen here doc, if you can get Blue Cross/Blue Shield to foot the bill for a private plane, I'll pass out snacks and drinks. Something tells me, however, that such an extreme measure won't be required.

While we spend time at the hospital with Zoe, Grandma and Grandpa Muenich (Granny and Papa) spend time with Emmy, both at the rental house and taking her around Philadelphia. Thanks Granny and Papa! Emmy even got her own seat on the bus yesterday.



Emmy's playtime yesterday included her first formal meeting of her new baby sister. She was apprehensive at first, but as I held Zoe in my arms, Emerson slowly approached. She pointed to her and said "Soe." At one point, Zoe started crying and Emerson's face quickly changed to a look of worry and concern. She said "hurt" then grabbed a blanket and lightly set it on Zoe. She will be an awesome big sister.

Since Emerson just met Zoe, it provided a great opportunity for our first family picture.

We don't need your stinking oxygen & thanks for the tissue.

Ms. Zoe is not yet in the Cardiac Care Unit (CCU) or step down unit from the Cardiac Intensive Care Unit (CICU). She is physically ready to go, in fact she was de-intensified (no longer requiring intensive care) on the 17th, however, there was not a bed available. So, she will remain in the CICU for one more night.

When we arrived at the hospital this morning, we immediately noticed that Zoe was no longer on oxygen. They took her off the oxygen at 4:00 a.m., and her oxygen saturation levels were very good, even up until when we left at 10:00 p.m. tonight.

We were able to hold Zoe for a long time today. Stacey held her for almost three hours, I held her for another two hours, and Grandma Muenich held her about an hour and a half.









Really the only thing left for Zoe to improve on is her feedings. When she is bottle fed, she eats anywhere from 50% to 100% of what she is supposed to eat. What she doesn't finish from the bottle, she is given through her NG tube. In an HLHS baby, eating can be difficult for a variety of reasons, primarily because it takes energy to eat and these babies often tire easily because of their modified cardio-pulmonary system. Many kids are discharged from the hospital and sent home with an NG tube. We would like to avoid that. Everyone is now working to help Zoe build up the energy to finish her entire feedings, on a consistent basis, without the need for the NG tube. Once the NG tube comes out, Zoe will be close to discharge.

We learned today that part of the materials used to reconstruct Zoe's aorta were from donor tissue from an organ/tissue donor. Organ donation is often one of those things that you don't think about until you or a loved one need it. Stacey and I are both organ donors, but I've never had any real exposure to it. Frankly, I became an organ donor simply because it seemed like the right thing to do. We were given the information to write a thank you letter to the family of the donor who provided the tissue for Zoe's heart. We definitely plan to do that. The decision to donate, whether made by the donor or their family, was done in the hope of helping others in need. Most families, however, don't generally learn the outcome of the donated organs or tissue. If I were on the other side of this equation, I would like to learn the specific ways in which my loved one's donation saved others. Accordingly, we will send a letter, this week, to the family of the donor who provided the necessary tissue, thanking them for their contribution toward saving Zoe's heart and giving her a chance to live.

Tomorrow, Zoe should be transferred to the CCU and we will continue to work with Zoe, in conjunction with her nurses, to improve her feeding, taking us one step closer to returning to AZ.

Zoe's graduating!!

Ms. Zoe had an excellent day today! She is really recovering like a champ from her Norwood operation.

Zoe continues to be weaned from her oxygen, she is down to 50% oxygen and with that level her oxygen saturation is even better than what the cardiologist wanted. She is also doing better with her feedings. They are trying bottle feedings first, then what she doesn't finish from the bottle, she gets through her NG tube. She is doing well with the bottle feeding, so well one of the nurses suggested the NG tube may come out soon. Also, Stacey was able to breastfeed Zoe for the first time today and Zoe performed like a natural!

All in all, we couldn't ask for a better recovery. The professionals agree, that's why Zoe will be transferred to the Cardiac Care Unit (CCU) tomorrow morning, which is considered the step-down unit from the intensive care. Once in the CCU she won't get the nursing attention she received in the intensive care unit, but she also doesn't need it. The step down unit is where we will start to receive hands-on training on HLHS related issues, things we will need to know once we get Zoe home.

Today, we spent a long time hanging out with Zoe. Stacey held her for awhile and I held her for two and a half hours straight! She only cried for about 5 minutes of that time. Not bad dad.





Zoe and her pod mates were also entertained by musician Woody Wolfe. Once a week he tours the cardiac intensive care unit and plays his guitar and sings for the kids. Zoe seemed to like the music.



In other firsts, Zoe graced us with her first smile today. Granted, I think the smile was prompted by an episode of gas.





We will update tomorrow from our new digs in the CCU.

No shirt, but how about a mohawk?

Today we took a bag full of preemie and infant clothes to the hospital, as we were told Zoe may be able to wear clothes today. She was sleeping so much today, we never got a chance to get a shirt on her, she did, however, get a new hairstyle, compliments of Nurse Jay.

MOHAWK!!



Zoe was weaned off her nitric oxide today, which means her heart and lungs are working together and her body is really improving its efforts with breathing. She is still on oxygen, as a supplement, but her cardiologist said today that Zoe's oxygen saturation levels are very good, therefore she is confident that Zoe will do just fine with breathing, once the oxygen is ceased.

She also continues to receive feedings through her NG tube, while receiving bottle feedings as a supplement. The goal is to increase the bottle feedings and simultaneously decrease the NG tube feedings, until it can be removed completely. One of her nurses today said that there is improvement with every feeding and Zoe will be just fine with her feedings.



Zoe received visits today from the cardiologist following her at CHOP, a neo-natology fellow, and a speech therapist. The speech therapist said there are no speech issues, but suggested a slow flow nipple when feeding from a bottle. Her cardiologist said she is doing "great!" She said her chest x-ray from today looked really good. Indicated she is confident about the respiratory situation, and suggested all she needs to do is work on her feeding.

All in all, our little warrior is doing very, very well under the circumstances. We were able to hold her for two extended periods today, once for about 20 minutes, then a second time for about 40 minutes. Finally getting to hold her, after all the time on sedation and hooked up to wires, for a moment, makes things feel totally normal, like nothing else is wrong.



Stacey even gave Zoe her first nickname today, "monkey toes." As you can see from her foot, it is a fitting name.



Hopefully, we will we have pictures of Ms. Zoe in clothes very soon. I'm sure she is tired of being naked all the time and would like some warm baby clothes.

Your positive thoughts and prayers are clearly helping, please continue to send them for Zoe's ongoing recovery.