06 April 2012


"It's like losing a wing at 35,000 feet, landing the airplane, and then calling it a great flight."

That was the analogy given to us this morning by the Director of Cardiac ICU about Eddie's operation yesterday. He was referring specifically to the release of the clot in his right ventricle on the operating table and the resulting pulmonary embolism (PE). We are just now getting the complete story of what happened and it appears the clot definitely did dislodge during the procedure and, with its significant size, should have been fatal. In fact, the same doctor told us he's never seen anything like it . . . the PE should have been "game, set, and match" for Eddie.

As you'd expect, this morning's rounds were about as celebratory as they get with an ICU staff. Eddie had virtually no bleeding overnight, and what little there was, was overwhelmingly serous. His blood pressure, after being elevated yesterday, dropped to normal levels. Lungs are clear, kidneys doing great, no pericardial effusion, no clots in the heart or Berlin pump, no fever, and as of a couple hours ago, no breathing tube. Lest we all get carried away with good news, he continues to have irregular heart rhythms so we are increasing his potassium levels and may need to put him back on amiodarone. No matter which way we slice it, however, we can't deny that we're thrilled with his progress and it's nothing short of remarkable.

Eddie's having a good morning and we're hoping to let him recover a bit from the craziness of the past few days. As I write, he's enjoying Sarah's dramatic interpretation of Horton Hears a Who!

We plan to leave the Heparin off today to limit any bleeding, but will watch his pump very carefully and will ECHO him this afternoon to make sure no clots are forming. If all goes well, we'll probably turn the Heparin back on tomorrow. As the ICU staff told us, this is definitely not a path they would ever follow for another patient (meaning actively turning off anti-coagulation treatment on an external heart pump), but as is clear, Eddie is a different kind of patient.

We are very conscious of the fact that any of these events (or numerous others) could happen again at any time and put Eddie in danger. However, we are celebrating the fact that he is with us today and know that he is being watched over.

05 April 2012

[Correction]: Auf Wiedersehen, Berlin

As Eddie was preparing to go to the OR, we told him the surgeons were going to take away his "other" heart. He gave us a funny look, screwing up his eyebrows, as if to tell us he didn't think that was such a good idea. As it turns out, he had the last laugh since he just came back to his room still attached to his Berlin heart!

So a couple surprises to note. First, after exploring with the transesophegeal ECHO, there were no signs of either left or right ventricular blood clots. We believe the LV clots dissolved or otherwise left the heart last night or this morning with no noticeable effect. The RV clot was clearly there during this morning's ECHO so we think it may have dislodged during today's procedure. But most importantly, there is no evidence of the kind of pulmonary distress associated with a pulmonary embolism.

Second, and perplexingly, we still found no bleeding in the pericardium. Since Eddie's bleeding seemed to have stopped about two hours before surgery (we thought it was probably due to a clot in his chest tube), Dr. Cohen was inclined to trust his eyes and leave good enough alone. That means the Berlin stays in place and we'll wait to see if the bleeding eventually stops.

Since the clots have disappeared, we're more comfortable turning off the Heparin for a couple days which should help the bleeding slow down. If we can get the bleeding to stop, then we'll turn the Heparin back on which should keep the Berlin pump clean. Regardless, we're back to playing the "wait and see" game and it's far too early to declare any definitive victory . . .

Sarah and I were treated to a bonus once Eddie came back to the room. Typically the nurses ask us to leave while they change his Berlin dressings (both for sterility reasons as well as some parents have been known to faint), but they thought it was about time we saw what they do each day. Neither of us fainted, but we can assure you it's not a sight for the "faint of heart" (pun intended). The combination of his ECMO and heart surgery incisions, two Berlin heart cannulae sites and two chest drainage tubes would have brought a smile to Victor Frankenstein's face.

So that's it . . . we'll keep the (non-alcoholic) champagne corked for now :-), but will celebrate each small victory as we find them.

Auf Wiedersehen, Berlin

It's hard to believe any three year-old could go through as much as our Eddie has, but here we are. As we speak, he's back in the OR and our surgeons plan to remove his Berlin heart. His bleeding continued for most of the morning and then suddenly slowed . . . his ECHO showed new pericardial effusion which obviously wasn't draining. So either his chest tube was blocked or he was developing pockets of fluid unreachable by the existing drain.

The plan is to open his chest and see if by chance there is any evidence of a single source of bleeding (highly unlikely since he was just in the OR on Tuesday and there was no evidence then). Assuming there will be no obvious bleeding source, they'll drain all the fluid they can and then close his chest. At that point, the Berlin pump will be disconnected and then replaced with a modified ECMO circuit. Since Eddie's right ventricle and lungs are working well, there will be no need for the oxygenation functionality of ECMO (the lung) so it will just be a closed circuit from the left ventricle to the centrifugal pump to the aorta.

A couple of the key benefits of this approach will be to eliminate the Berlin (to which Eddie seems to have some kind of inflammatory response) and stop the Heparin infusions Eddie currently needs to keep his pump clot free. Without the Heparin, his clotting function should improve and slow the bleeding. However, this also means he will be at risk of developing new clots in his heart, but this is a risk we are more comfortable taking . . . especially because we think the clots only formed in the first place because of his constricted heart during his tamponade on Sunday . . . an episode we hope to avoid in the future.

One piece of (apparent) good news is that the clots in the left ventricle seem to have vanished since yesterday. Nothing catastrophic has happened so we assume the clot dissolved . . . or ended up somewhere harmless.

Will share more details as we have them . . .

04 April 2012

After Rounds

In the words of the attending ICU physician, we need to put our eggs in one basket right now. In other words, we need to focus on the bleeding or the clots. Since the clots are a "potential" source of danger and the bleeding is actively putting Eddie in harm's way, we will spend our energy on the latter . . . his bleeding is definitely the priority

Right now, Eddie is resting quietly and seems comfortable. We are leaving him on the ventilator for now . . . at least until we can get the afternoon ECHOs.

Day 30

Good morning, everyone. I know many of you have been waiting patiently for an update, so here's what we know:

Yesterday's ECHO didn't show much of a change in Eddie's clots . . . still roughly the same size and in roughly the same place. This is good since we know they are still in his heart and haven't migrated somewhere more dangerous. Also, since clots tend to establish themselves more firmly over time, there is the possibility they will become more embedded and less mobile which, in the near term, may be a good thing since they don't seem to be obstructing blood flow through his heart. We will continue to monitor his clots through daily ECHOs.

We scheduled a care conference yesterday afternoon with Eddie's doctors to understand where things stood and get a comprehensive picture of his care options going forward. The net result was that we felt it best to take a watch-and-wait approach rather than take any interventional steps (surgery, specialized anti-clotting drugs). With constant monitoring and a bit of finger crossing, we would hope for the best.

Not long after the care conference, however, the team approached us and said they needed to take Eddie back to the operating room since the bleeding from his chest tube was increasing and was increasingly bloody. After much discussion with colleagues around the world, the doctors and surgeons felt it was getting to the point that we had to take the risks of surgery to identify the source of bleeding and try to stop it. This was around 6:00p last night.

About three hours later, we received word that Eddie was stable and recovering nicely. They had found one artery that was bleeding a bit, but mostly there was diffused and general bleeding and it was difficult to pinpoint the source. They closed him back up with the hope that the bleeding would slow down, but there was no smoking gun.

As of this morning, he is still intubated (on the ventilator with breathing tube) and he is still bleeding. He is awake and responding to us and we are so grateful to have him with us. We will have a more detailed discussion with the doctors in the next hour and will share news as we have it.

As you can tell, Eddie is very critical. We love him, and are so proud of his strength . . . he inspires us every day with his tenacity. Thank you for your prayers . . . we feel them every day.

03 April 2012

Fortunately / Unfortunately

Fortunately, Eddie's heart came back into rhythm about 1am yesterday and his heart has had some much needed rest over the past 24 hours. Unfortunately . . .

There are a lot of questions about Eddie's condition right now, but there are a few things we know. Most importantly, two blood clots have developed in both his left and right ventricles. Everyone is confused how this could have happened considering how heparinized he is right now . . . his blood is very thin to avoid clotting in his Berlin pump. Equally puzzling is how quickly these clots were able to form since no one saw them on previous ECHOs . . . we don't believe they were there.

Our best guess is that the clots developed during the pericardial effusion on Sunday, when his heart may have been constricted enough to give the clots time to grow. We continue to drain fluid out of his pericardial sac to prevent the problem from getting worse, but until we get good images this morning, we won't know if the clots are growing, shrinking, or staying the same size.

I won't walk you through the detailed risks, but at a high level we're concerned about a major respiratory event (if the clot in the right ventricle moves to the lungs) or a major stroke event (if the clot in the left ventricle moves to the brain). Any surgical removal is extremely risky due to the thinness of his blood. Using clot-busting drugs is very risky since they can cause spontaneous bleeding. So overnight, we were simply waiting and hoping that the clots dissolve since they are relatively new and unhardened. Once we are able to get a new ECHO this morning, we'll have more information on which to base our next step decisions.

We hate to share this kind of news . . . especially after Eddie had such a great day on Saturday. We feel it is important to keep everyone up to date, however, and know that he will be in your thoughts and prayers. More to follow . . .

02 April 2012

Happy April Fools Day!

I just now realized that it is April Fools' Day . . . it has been that kind of day . . . so I hope everyone was able to enjoy a bit of relaxing humor. On a fairly random note, I remember a story I wrote in elementary school that had to follow a "fortunately / unfortunately" narrative structure. For example, if I wrote, "Fortunately, I received a bicycle for my birthday" then I had to follow that line with something like, "But unfortunately, my handlebars fell off", which could then be followed up with "Fortunately, there was a hospital nearby." You get the picture. Well, that's the pattern that predominated at the hospital today . . . it seemed like every positive/negative development was followed in short order by its opposite.

Consistent with the developing pattern, Eddie's great day yesterday was followed by a pretty rough one today. He had a hard time sleeping last night and his Berlin pump struggled to fill properly throughout the morning. As time went on and he was showing signs of confusion and disorientation, we noticed that his chest tube wasn't putting out as much fluid, either. Once our nurse began stripping the tube and seemed to dislodge a blockage of some sort, Eddie's color quickly improved and his pump begin to fill quite nicely.

During the next hour or so, we were able to get very nice pictures of his heart via ECHO which have been next to impossible to get since his Berlin placement almost two weeks ago. We saw that there were a couple significant pockets of fluid in the pericardial sac that didn't seem to be draining through the chest tube. This is a concern since fluid buildup restricts heart function, leads to poor perfusion, and in Eddie's case, limits the effectiveness of his Berlin pump.

We tried positioning Eddie in a variety of ways, but we couldn't get any more fluid to drain through his existing tube. Either a blockage of some sort was making it impossible for the fluid to drain, or the tube was simply not accessing the parts of the pericardium where the fluid was sitting. Within a couple of hours, Eddie's perfusion was poor and his pump was again not filling well.

At that point, the best option was pericardiocentesis, or basically, using a large needle and catheter to access the non-draining portion of the pericardial sac. This is a relatively straight forward procedure, but as always, there are risks. One of the interventional radiologists did the dirty work with the help of ultrasound . . . need to know when the needle is in the sac and not the heart. As a precaution, Eddie was intubated.

Thankfully, the procedure went relatively smoothly and drained over 180 ml . . . that's more than 6 oz. of fluid from a sac that should normally have only 15-50 ml . . . for an adult! Our goal now, of course, is to keep that fluid draining consistently and monitor closely via ECHO/ultrasound. At this point, since the fluid appears primarily serous, we aren't too concerned about internal bleeding. Rather, we think this is an immunological response to the Berlin cannulae. Not much we can do other than to keep draining . . .

As we had hoped, Eddie was much stronger after the procedure and was extubated almost immediately. He was quickly asking for apple juice again :-)

Just an hour or so later, however, his heart starting beating out of rhythm again. This has happened a few times before, but always under slightly different circumstances. Just a few days ago, a good dose of potassium was all it took to bring him back into rhythm. We are hoping that a good night's rest along with doses of amiodarone will see him back in sinus rhythm tomorrow morning.

We are constantly reminded that Eddie's battle is no skirmish, but rather a drawn out campaign. As we look back over the past two months and add up the number of events, surgeries, and procedures . . . not to mention the difficult conversations . . . we are humbled by how little that is happening is within our control. In spite of this, we know that we are at our strongest when we respond to these challenges with hope and optimism rather than questioning, complaining, or giving into despair. As Montaigne puts it: "Not being able to govern events, I govern myself." And we also know that we aren't alone in this trial . . . each of you have offered to bear a share of our burden for which we are very grateful. Thank you.