17 March 2012

Weekend Miscellany

First, the important bits . . . Eddie is resting well today and is as comfortable as I have seen him since we've been in hospital. His nurses are wonderful and have done everything possible to help him relax . . . a very difficult task with multiple IV lines, heart and temperature monitors, and large cannulae running along one side of his head. Eddie really loves to have his teeth brushed since it is the only way he gets any water or moisture into his mouth. I can only imagine how hard it is for him since his breathing tube makes oral feeding impossible . . . and am not surprised that the liquid nutrition he receives via his nasogastric tube doesn't quite satisfy . . . diagram included to encourage sympathy :-)

After talking with our cardiologist and the CICU (Cardiac ICU) attending, it appears unlikely that we'll need to de-list Eddie as a result of his rhinovirus. They are going to ask the surgeon to confirm, but since there is no clinical evidence of the virus, level of concern is quite low. As a precaution, however, Eddie will stay in isolation for the next few days until cultures come back negative.

Overall, his improvement has been strong and his heart rate, blood pressure, oxygen levels, neuropsych function, etc. are in a good place. We are still concerned with his lungs since he continues to cough up fresh blood through his breathing tube, but overall, his AM x-rays look clearer and his chest sounds much looser than yesterday. Hopefully the blood will soon darken (dark brown/tan is much better than pink/red since it signifies old blood vs. new) and diminish. Also some concern about kidney function based on creatinine levels, etc., but he seems to be heading in the right direction.

For the rest of the weekend, our goal is to keep him relaxed and give his body the chance to recover from the stresses of the last week. Monday, when everyone is back in the hospital, we will assess his condition and determine when (not if) to implant the LVAD/Berlin Heart . . . most likely Tuesday or Wednesday.

On a more mundane note, I promised to share some pictures of our living quarters at the Waldorf Astoria @ Seattle Children's. As you can see, we're living quite comfortably and are in no rush to leave . . .

Ok, and when I wake up from my dream, this is actually what I see:

So while I don't have a relaxing pool at the foot of my bed, I can rest comfortably knowing that the door is only inches away. And then when the weekend comes, and I'm ready for some excitement, I can head down the hall to watch my clothes dry . . . who needs a vacation getaway?!

In all seriousness, the facilities here have made our stay incredibly comfortable. All laundry services are gratis, there are bathrooms and showers down the hall, and the fact that we have a twin bed in a private room to sleep in each night is far more than I originally expected . . . certainly much better than trying to lay down on the couch in Eddie's room. Makes me nostalgic for the 7th floor of V-Hall at DT (BYU dorms) . . . those wild and crazy days.

16 March 2012


Well, this is going to be a very brief update since I'm typing with my thumbs! The thrill of sleeping in my own bed last night was so overwhelming that I forgot to bring my laptop to the hospital. For those counting, that was nine consecutive nights sleeping in the ICU penthouse suite . . . will definitely post some pictures soon.

Eddie is definitely sad to be back on ECMO since it limits his movement and he requires more sedation. That said, I left him just a few minutes ago and he was resting peacefully. The nurses really did a great job cleaning him up earlier today and he looks so handsome considering the circumstances.

We've had a slight hiccup this evening since he tested positive for rhinovirus so is now in isolation. Basically means the nurses have to wear gowns, masks, and gloves and his door needs to remain closed. The virus certainly isn't dangerous, but it could impact his eligibility for a transplant if left untreated. He has no symptoms and is not outwardly sick, but the cultures came back positive so we have to take precautions.

Otherwise, it was a quiet day around here. Had a couple nice visits and were blessed with a few Trophy cupcakes from our guardian angels (you know who you are!)

Promise to share a more comprehensive update tomorrow once I can type with all eight fingers and two thumbs :-)

15 March 2012

To Be a Pioneer

Another quick update to let everyone know Eddie's surgery was successful. The actual cannulation only took 30 minutes or so and they were able to place the tubes in the same location (neck) since the artery and vein were still viable . . . all good news.

So here's the plan for now . . . Eddie will stay on ECMO until Monday at the earliest to give a few days for his lungs to clear up. At that point, if his lungs are strong, we'll make plans to transition to a Berlin Heart later in the week. If his lungs aren't 100% yet, we'll stay on ECMO and hope that a few more days will help. Since the Berlin Heart is purely a pumping device and doesn't do anything for the lungs, it is critical that Eddie's lungs are completely healthy before implantation.

During our physician conference today we discovered that only 30 restrictive cardiomyopathy patients in the world have had a Berlin Heart implanted so our team is essentially casting the mold with Eddie. With virtually no literature on the subject, this is more art than science . . . and our hope is that whatever our team learns from Eddie they can apply to help other patients in the future. Way to be a pioneer, Eddie!

Deja vu

Eddie's lungs have weakened enough that our cardiologists and surgeons agree he needs to go back on ECMO. Operation is scheduled for 3pm PDT and appreciate your thoughts and prayers. We love you, Eddie!

14 March 2012


Good morning, everyone. Eddie had a pretty good night and is more alert than ever . . . which also means he is more frustrated with his breathing tube and his inability to talk. He has been pointing at things all morning trying to tell us what's on his mind, but we have to stumble around asking Yes or No questions until we figure it out.

During morning rounds the doctors agreed he is doing well, but his cardiac functions are a little weaker than they would like. He has a fair amount of fresh blood in his lungs and his renal (kidney) functions aren't great. The plan is to monitor him over the next 12-24 hours to see if he gets any worse. If he does, we may need to consider alternate support.

All things considered, it's wonderful to see him looking more like our Eddie!

More soon . . .

13 March 2012

Afternoon Update

As planned, Eddie's cannulae came out around noon. No complications. He is still sedated so we haven't been able to talk to him yet, but he seems to be doing really well. He will continue to be on the ventilator to help him breathe for at least several more days so we won't be leaving ICU anytime soon. We really just need to continue observing him for any signs of trouble . . . rhythm problems, bleeding, etc.

It's so nice to have almost full access to Eddie now that the ECMO is out of the room. We're looking forward to that first hug!

New News

So today was supposed to be a rest day for Eddie before clamping off ECMO again tomorrow. However, after this morning's x-ray, it became clear that his venous cannula was dangerously high and would need to be addressed one way or the other. Since re-establishing him on ECMO after repositioning the cannula carries significant risks, we began to discuss taking him off ECMO entirely rather than waiting for tomorrow.

So about an hour ago we turned off the machine and Eddie has been doing all of his own work. In spite of a period of junctional rhythm (not good) during the trial off, he has recovered and is in good sinus rhythm with good heart rate and blood pressure. We are going to watch him for the hour or so to see how well he does before the surgeons come in to remove the cannula.

Most likely, his vein is going to be in pretty bad shape since the cannula has been in place for a week and he has been a very "active" patient. The surgeons will try to repair the vein, but it's possible they won't be able to suture it and will need to creatively close the vein.

Once again, we are on a roller coaster ride and every day brings new surprises. If all goes well, this could be a great step for Eddie . . . his body and heart will certainly let us know what he needs. If he struggles, there are a variety of options available to us from implanting the VAD (Berlin Heart) to recannulizing via his femoral artery, but we'll cross those bridges when we get to them.

Recap and Thoughts

This is John. Today was a very emotional day for me and I’m struggling to find the right words . . . but that’s not uncommon, right? Many of you are used to my rambling, but I hope I can express at least a few clear facts before I get too personal.

So according to plan, we began to wean Eddie off his ECMO flow around 4:00am. Sarah and I arrived around 7:30am while the surgeons, attendings, and cardiologists were conferencing. At this point, Eddie was sitting at approximately 60 cc/min flow, down from 110 the night before.

In the meantime, one of our cardiologists left the conference and stopped by to put hands on Eddie, to get a more personal look at how he was doing before making a final recommendation. I can’t describe how much this small visit meant . . . not just because he was focusing on our son, Eddie, the boy of flesh, blood, bones, and malfunctioning heart muscle . . . but because he took such unique care in preparing for his examination.

If you’ve been in a hospital lately, you’ll know that very few people actually “scrub in” with soap and water anymore. Rather, a healthy dose of hand sanitizer seems to do the trick and is probably totally appropriate. However, this doctor not only took the time to scrub in the old-fashioned way, but also thoroughly sanitized his stethoscope with alcohol . . . something I’ve not often seen. This was a simple act and probably just unconscious habit, but it made the examination much more intimate . . . definitely respectful and gracious . . . demonstrating a level of reverence I didn’t expect.

As the rest of the doctors gathered around Eddie’s bed, it was clear we would need to open the ICU doors all the way to allow for the many nurses and specialists that were needed in case the trial went wrong . . . not to mention the many people who were simply anxious to find out if Eddie would respond as we hoped. It all happened so quickly. The ECMO nurse began to dial down the flow . . . to 50 . . . 40 . . . 30 . . . and then suddenly it was off and the circuit was clamped. It all happened so fast and the room was absolutely silent, with all eyes fixated on the overhead screen. Heartbeat, EKG, blood pressure, atrial pressure, oxygen saturation . . . some numbers advancing, others retreating . . . I thought it felt a bit like watching the stock market . . . or a casino . . . just waiting to see where the wheel would stop.

However, this wasn’t Wall Street or Las Vegas . . . not a speculative exercise at all, but also not free of serious risks. Everyone had taken abundant precautions should Eddie need help . . . I’ll never forget the image of 15-20 syringes at the foot of Eddie’s bed, ready to inject him with a cocktail of dopamine, fentanyl, and whatever other frighteningly labeled drugs were deemed necessary. The ECMO nurse was ready to unclamp and resume blood flow. But the miracle was that Eddie seemed ok. While everyone was watching the screen, I looked at his little chest . . . saw it rise and fall peacefully . . . seeing no outward change. He was calm (definitely sedated) and seemed completely unfazed by the fact that the machine that had been supporting him almost entirely for the past six days was now turned off. Minutes went by and soon everyone was breathing again . . . there were even a few smiles . . . tears for us.

Now it would be wonderful to say that Eddie responded perfectly . . . but he didn’t. His heart performed fabulously with his left ventricle showing significantly improved function since Saturday. However, he also began showing quite a lot of blood in his urine and his core and toe temperatures dipped more than we would have liked. After watching him for a few hours completely off ECMO support, the motley crew of doctors, surgeons, and cardiologists once again huddled in a corner and sent a brave attending to give us the word . . . that we were going to restart ECMO support and try again Wednesday.

While in some ways disappointing, we believe this is actually the right decision. Overall, Eddie performed very well, but there are still a few questions about whether he would be able to support himself if we took him off ECMO today. Giving him two more days will allow his heart to rest and potentially strengthen. The ECMO circuit is still clot-free so we should be able to get another few days use before it would need to be changed. If Eddie trials successfully or shows little to no improvement, we’ll go ahead and take him off ECMO Wednesday. If he performs worse, then we’ll need to go back to discussing a VAD (Berlin Heart) . . . an option we are now optimistic we can avoid.

So after all of the drama in the morning, the afternoon was remarkably uneventful. Sarah went home to be with the kids and I made a nuisance of myself in the cafeteria. I can feel a follow-up post coming on, but it will have to wait until another time because it is so late. But I can say with some confidence that a heart patient’s father should never take to the cafeteria a book to read that contains first-person narratives of children living with heart disease. In case that was too cryptic, I’ll be clear: I bawled my eyes out when I began to read My Heart vs. the Real World and I'm sure there were a lot of sympathy stares in my direction :-). I am only beginning to imagine the pain these children suffer along with their families, but was even more touched by the tenacity and grace with which these kids cling to life. (FYI, the embedded video on the site is awesome . . . just be warned there is some colorful language).

It’s definitely time for bed, but wanted to share one quote from Patty Folgar, one of the kids from the book. She was diagnosed with hypertrophic cardiomyopathy at age 10. She had two siblings, a brother and a sister, who were undiagnosed until they died . . . both at age 10. At 18, she was asked to talk about how living with heart disease had affected her and I think her words are beautiful:

I thought at first that it was just bad luck having heart disease and I used to wish I didn’t have it. I wished I could just run around like everyone else, just doing everything other kids were doing . . . I guess I considered them lucky. But I don’t see it that way anymore. A healthy person might think “I’m so healthy, I’m never going to die, and I can do whatever I want.” And people don’t appreciate the things they have. If you just appreciate what you have every single day then . . . You never know what’s going to happen to you the next hour or minute. You could just – sorry to say this – but just drop dead. And then what if you didn’t enjoy the day you just had, or say “I love you, Mom.” What if you didn’t get the chance? I guess I consider myself lucky.

12 March 2012

ECMO Trial Update

Some encouraging news this morning as they clamped off Eddie's ECMO circuit about two hours ago. So far the trial is going well. He is still hooked up to the pump so if he begins to struggle, the nurses can turn the flow back on . . . but for now, he is responding well. Good heart rate and blood pressure and this morning's ECHO is definitely showing some improvement in his left ventricle function, i.e. much better squeeze than last week. Picture of the ECMO device is below . . . the blue cylinder with the red lid is the pump (heart) and the white diamond-shaped unit to its left is the oxygenator. You can see the inbound/outbound tubes on the far left side of the image.

The plan for the day is to continue monitoring his progress and make a decision this afternoon about whether or not to decannulate (remove the ECMO tubes from his neck). We'll share updates as we have them, but right now we are just happy to see him make it this far.

Thanks for all of your thoughts and prayers . . . they truly make a difference.

11 March 2012

Sunday Rounds

Eddie handled the ECMO trial last night very well . . . good temperature and good kidney function, but his blood pressure did spike a bit more than expected. Regardless, when we met with Dr. Mazor early this morning and then Dr. Law during regular rounds, we all thought it best to trial again tomorrow morning once the surgeons and transplant team are back in the hospital. They conference at 7am and then we'd look at clamping the ECMO flow around 9am if Eddie continues to do well enough.

As a result, today is becoming a day of rest, appropriately enough. We had a visit from two members of the Seattle 3rd Ward who were kind enough to administer the Sacrament (similar to receiving the Eucharist or Communion) as well as help me give Eddie another blessing. Sarah left this morning to attend church meetings in Redmond and spend some time with the kids.

Key decisions now if Eddie does well on reduced flows are:
  1. Do we take Eddie off ECMO tomorrow?
  2. If yes, do we still put him on a VAD (Berlin Heart) or leave him on his own?
  3. If no, how long do we wait before putting him on VAD?
  4. If we move to a VAD, do we make changes to the donor criteria to make the process more selective again? In other words, since the VAD is viable over a longer term, do we make a play for a higher-quality heart or do we still believe time is of the essence and take whatever we can get?
We may have answers to #1-3 tomorrow, but #4 might take a little bit longer.