Worth 1,000 Words

I came back from the cafeteria and this is what was waiting for me. An empty bed and Eddie watching Polar Express in his La-Z-Boy. Didn't I say every day brings new and unexpected surprises? This is Eddie's first foray out of his hospital bed in almost four weeks!

. . . To Be Thankful For

Eddie is recovering in his room tonight after a successful pump change. He was under general anesthesia for the entire procedure and was clamped off for about four minutes. We are thrilled there were no complications.

When the Berlin Heart company sends their compressors and pumps to hospitals, they do so only when a specific patient is being treated. In other words, hospitals do not carry these items on their shelves for indiscriminate use. As a failsafe, they always send redundant units as backups should the originals fail. In this case, the compressor was (and still is) working fine, but the pump had to be replaced and will probably be shipped back to the manufacturer for analysis. That means we no longer have a backup pump so an additional unit was ordered today . . . should be on hand tomorrow. Phew!

On a separate topic, we are looking forward to LDS General Conference tomorrow and Sunday. For the uninitiated, these conferences are held twice a year (first weekend in April and October) and are broadcast via satellite to over 7,400 church buildings in 102 countries. In addition, the Church streams the meetings live on the LDS.org website so anyone who chooses can watch and/or listen. Whatever your faith tradition or religious leaning, I'm certain you'll find something to stimulate profound and existential conversation . . . at least more so than watching Louisville trounce Kentucky tomorrow!

Have a wonderful weekend!

There's Always Something . . .

Consistent with previous comments about how unpredictable things can be around here, Eddie will be going back into the OR this evening to replace his Berlin pump. Fibrin built up quickly in his pump overnight and, after talking to doctors at Berlin Heart (the company), we've decided to put a new pump in place to eliminate the risk of clotting and stroke.

This isn't necessarily unexpected, but it also isn't exactly normal. Typically, fibrin builds up over days and weeks rather than hours. These fibrin deposits sometimes dissolve or break free and float harmlessly through the bloodstream. However, they can eventually form embolisms (clots) and, if those end up going to the brain, may cause a stroke. Replacing the pump is the safest way to avoid these risks altogether.

Of course, pump replacement comes with its own risks. We haven't had the consult with surgery yet so we don't know the specific details of how they will proceed. We do know, however, that this will not be an invasive procedure and Eddie will not be required to go on bypass. Rather, the pump can be unfastened externally after the doctors clamp the valves shut. So in essence, while the pump is changed out, Eddie's blood will stop circulating through his body for 1-2 minutes which the doctors assure us is well within the safe zone.

So yet again, we're grateful for wonderful doctors, generous friends, family, and co-workers, and everyone else that is keeping Eddie in their thoughts and prayers. Procedure is scheduled for 5pm PDT.

Finally, in yesterday's post I mentioned that we're bound to run into "hiccups" from time to time considering Eddie's overall condition. Take a look at the video below for a more literal take on the subject :-) We love you all.

Thursday Volatility

It's really remarkable how quickly things can happen around here. Last night was rough for Eddie with a bout of diarrhea and, more seriously, pretty frightening arrhythmia through the early morning hours. Thankfully, he is resting very quietly now and the day ended on a much more upbeat note.

We spent several hours during the night worried about his irregular heart rhythm, but since his cardiac output seemed ok and his Berlin was filling well enough, it didn't qualify as an emergency. During early rounds at 8am (Surgery and ICU staff), there was discussion about needing to be prepared to go back on ECMO if his rhythms didn't come back in line so that was an unwelcome shock. Again, this was a case of very smart doctors not knowing exactly what was going on and trying to prepare for worst case scenarios.

During cardiology rounds later in the morning, our transplant cardiologist called into question any potential for going back to ECMO. He felt that Eddie's rhythms, while dangerous, could be managed through medication (amiodarone) or supplements (potassium) and that talking about ECMO was going a little overboard. That was definitely encouraging to hear, and he went on to emphasize how phenomenally Eddie has done on the Berlin pump and that no one should have expected this to be easy. There are bound to be hiccups along the way, but Eddie is in as good of a position as any previous patient.

Once the amiodarone and potassium kicked in after rounds, it was less than an hour later that he reestablished a normal sinus rhythm. So encouraging considering the frightening turn we seemed to be taking earlier.

The Apple Juice lady (aka the Speech therapist) came by in the morning to reassess Eddie's swallowing ability. Great news! Eddie is now approved to drink "1 oz. of honey-thick liquid via sippy cup with restricted flow valve" five or six times a day. If that doesn't sound delicious on paper, just swing by the hospital and we can arrange a free tasting . . . you'll be hooked at the first drop.

In other miscellaneous news . . . Eddie received his wish and was given the green light to brush his teeth with his special blue foam toothbrush soaked in water (see below). Ok, so the Apple Juice lady didn't actually say he could do this, but our nurse was awesome and turned a blind eye while we exercised our right to civil disobedience.

Other good news . . . while it's hard to tell from the photo below, Eddie completed his nitric oxide wean. Most importantly, this means that his nose and throat are clear except for his nasal feeding tube. Two down and one to go.

And finally, the handsome devil even got a shampoo before crashing hard. Sleep tight, little man.

Priceless

You've already seen the bear, but today we introduced him to Eddie . . .

And apparently the experience wiped him out . . . sleep tight, little guy.

Sarah managed to tear herself away from the hospital so I'm back to doing hospital duty. We had a good day today with some small victories and a few things to work on. The Speech team made their much anticipated return visit this morning and Eddie enjoyed 2 or 3 swallows of apple juice and a spoonful of apple sauce before decided he'd had enough. Not surprisingly, he started asking for warm chocolate (his version of hot chocolate) as soon as he saw his door of opportunity open a crack . . .

His Berlin pump hasn't been filling as well lately which generated a lot of discussion at rounds this morning. He is showing very strong signs of cardiac activity (warm toes, strong pulse, good color) which is good, but not necessarily what you would expect if the pump isn't filling. A lot of conversation as well about the quantity and quality of output from his chest tube . . . very bloody in appearance but very low hematocrit levels. If he was bleeding internally, we would expect to see much higher hematocrit levels . . . more comparable to his regular blood draws.

So Eddie continues "to boldly go" where few heart patients have gone before and our seasoned cardiologists and attending ICU doctors are at times left to "figure it out" through trial and error. Sounds scary, but really understandable since they are face-to-face with something they haven't seen before. In fact, while I can't speak for Sarah, I actually find these conversations fascinating and encouraging because you're seeing the decision-making process happen from start to finish . . . the scientific method in action. Some hypotheses are proven correct and others . . . not so much. But in every case, we have multiple doctors and nurses bringing hundreds of years of combined experience to the conversation. Difficult to arrive at consensus, but usually reaching a decision that everyone feels is the best way forward.

Respite

A genuinely quiet day for the most part. Eddie was very responsive this morning and seemed to have recovered very well from the previous day's surgery. He loved reading The Three Little Rigs and Olivia Saves the Circus, but then again, who doesn't love reading about precocious pigs that fill in for circus performers when they "get sick with ear infections"?

Biggest bummer of the day was that Eddie was asleep when the Speech folks came around so he didn't get his swallow test, so yet another day he'll have to wait for apple juice. Hang in there, Eddie! They'll be back again tomorrow . . .

Overall, the doctors are still trying to get a handle on how to balance his anticoagulation needs (to avoid clotting and potential for stroke) with his risk of bleeding. We have to keep his blood thin enough to prevent clotting as it passes through the tubing of his artificial heart. Basically, blood wants to coagulate the minute it touches a foreign surface and, in Eddie's case, this would be extremely dangerous since they could make their way to his brain causing a stroke. As a result, we and the nurses spend a lot of time monitoring his pump for fibrin buildup as well as watching his general behavior for signs of impairment. So far so good!

I found a very poignant New York Times article from a few years ago which outlines some of the specific issues we have dealt with and continue to face now that Eddie is on an artificial heart. The paragraph below is from that article and describes almost exactly what Eddie is confronting:

All patients with mechanical heart pumps face risks; the greatest are linked to bleeding, clots and infection. Whenever blood flows through foreign materials and artificial valves and chambers like the ones in a heart pump, it tends to form clots, which can be disabling or fatal if they reach the brain. Drugs can prevent clots, but also increase the risk of bleeding, and so doses must be fine-tuned to keep a patient on the thin line between two potential disasters.

Sarah's doing bedside duty tonight so be sure to include her in your nighttime prayers :-) Not easy to get a good night's rest on those old vinyl couches. I love you, Sarah!

At the End of the Day

It always seems to be so late by the time I get around to these updates . . . where does the time go? And apologies for leaving most of you hanging without much of an explanation in my previous entry. There really is no letting up these days . . .

So Eddie is doing ok after his procedure this afternoon. It was all a bit of a surprise for us since we had no idea he would be going into the operating room for the PICC line insertion. Since his central line was in his groin and thus much more susceptible to infection, we knew from the beginning we would need a more secure PICC line in his chest. However, with all of his ECMO and Berlin Heart excitement, the PICC line kept getting deprioritized (rightly so).

We discovered this morning that the IR team (Interventional Radiology) was taking Eddie to their operating room and he would need to be anesthetized for the duration. After enduring yet another discussion of risks for this procedure and signing the consent forms, Eddie rolled away. This time, however, we were allowed to tag along all the way to the OR . . . and Sarah even got to ride on the bed reading stories to Eddie.

When we arrived at the operating room, it looked something like the picture below. Basically, by using a combination of ultrasound and fluoroscopy, the doctors are able to get real-time, living images from multiple vantage points. Pretty amazing.

After a little more than an hour, the nurses brought Eddie back to the room for his daily dressing change and echocardiogram. There really is a lot of choreography that goes on in a hospital since everyone would prefer to minimize the number of times Eddie needs to be sedated, anesthetized, or otherwise drugged.

On the topic of sedation, Eddie has been receiving a continuous infusion of anti-anxiety and pain medications since he was admitted to the ICU three weeks ago. We are currently in the process of weaning him, but it will probably take two weeks until he can come off completely. Goal of moving so slowly is to avoid any withdrawal complications. While he has been a bit more lucid each day, we are happy to take this process step by step and are in no rush.

A final bit of good news . . . we heard Eddie's first real laugh in three weeks this morning. He was smiling quite a bit and really loved having Sarah in his bed reading to him. We are hoping he can rest up tonight and recover from today's operation so he can pass his "swallow" test tomorrow. If he does, he'll finally get some of the apple juice he has been begging for the past few weeks. Fingers crossed!

Back in Surgery

Eddie just went into the OR since he needs another PICC line in preparation for his eventual transplant. Beginning to feel the strain a bit of this ongoing saga. Hopefully will have an update soon.

Heart Transplants in the News

Some of you know that Dick Cheney had an LVAD implanted a few years ago. Well, it appears after waiting for 20 months, he finally got his heart today. I can't say I'm a big fan of our previous vice president, but it's wonderful to see the process work the way it should and his prominence didn't move him to the front of the line.

Note that the LVAD Mr. Cheney received is identical in function to Eddie's Berlin Heart. However, since research into adult heart disease is substantially ahead of pediatric research (and since adults are bigger than kids), he was lucky enough to have an implanted pump rather than an external one like Eddie.

Here's hoping that Eddie doesn't wait anywhere near 20 months!

Cardiac Health Awareness

Since it's a quiet Saturday morning, I thought it might be a good time for a little public service announcement. Heart health has obviously been on our mind lately, and we've wondered if we could have done anything to catch Eddie's condition prior to his first collapse in January. In hindsight, there were a few warning signs like poor appetite, chronic respiratory problems, and being underweight. But how do you make the leap from identifying isolated symptoms to engaging in a more earnest (and frightening) conversation with your pediatrician?

I thought some of you (especially those with children that actively participate in competitive athletics) would find the info from the following sources helpful. The first is a book that you can find at kcls.org: "The Heart of a Child: What Families Need to know About Heart Disorders in Children". Also the recent (and unfortunately titled) SeattleMamaDoc blog entry "How to Prevent Sudden Cardiac Death in Young Athletes."

Make sure you talk to your pediatricians about your child's heart health at every visit... and feel free to use our example as proof that you're not just being a paranoid parent!

And finally, if you're reading this in the Seattle area, get outside and enjoy the wonderful weather!

Changing of the Guard

After a week of non-stop hospital duty, Sarah is finally going home to spend the night in her own bed. The ICU is pretty crowded so we've been asked to vacate the honeymoon suite and move into Eddie's room...not the most comfortable place to sleep, but at least they won't need to page us the next time Eddie decides to create mischief for his nurses.

Today was a relatively quiet day as Eddie continues to recover and wean off the battery of medications he's been relying on to keep him comfortable (Dilaudid, Versed). The plan is to taper off over two weeks or so to prevent Eddie from suffering painful withdrawal...which means that the Eddie we know and love will be a little slow to return.

Now that his breathing tube is out (Thanks, Eddie!), he is speaking a little bit but continues very hoarse and, let's be honest, pretty grumpy. At least Sarah was glad to hear him say "Mommy" several times. No sign he remembers my name ;) Someone from Speech paid a visit this afternoon to check his throat and his ability to swallow, etc. The recommendation is to wait until Monday to start any oral nutrition since he isn't quite ready. Hopefully we can sneak a bit of apple juice into his gullet over the weekend :)

We haven't really talked much about the other kids, but they are holding up well. "Stefano" is on a camping trip this weekend, "Liz" is looking forward to a trip to the ballet store for her own pair of pointe shoes, and "Janey" the mover and shaker is busy with a birthday party and starting a write-in campaign to recognize hula hooping as a sanctioned event in the 2020 Olympics (fingers crossed that the fourth bid is the charm for Madrid, although their logo choice doesn't bode well)! That leaves "Phil" who undoubtedly will keep Grandma and Grandpa busy with some mischief or other...what would we ever do without him?

And a final piece of great news I forgot to mention...Eddie has been reactivated on the transplant list. We had to inactivate him immediately following his surgery and we thought it would be 4-5 days before he'd be back on. But his recovery has been so impressive that everyone agreed to reactivate him after only 48 hours. Woo-hoo!

Night of the Bear

All caregivers in ICU are given a pager so the doctors/nurses can get in touch quickly, usually to provide status during surgery, etc. So as you can imagine, if the pager goes off unexpectedly in the middle of the night and when your son isn't in surgery, parents can get a bit worried. Well, it was a bit after midnight when our pager went off last night. Standard protocol is to call the ICU front desk to find out what's going on . . . need to meet the surgeon in the conference room, should go to Eddie's room, etc. . . . so imagine our heightened anxiety when no one was answering the phone at the front desk. By now, we're imagining all kinds of crazy scenarios so we got dressed and went downstairs, looking like people do when woken up in the middle of the night!

We were met in Eddie's room by 7-8 doctors and nurses with all of the lights blazing . . . obviously not normal. Eddie was on the bed and he seemed to be breathing ok and no one was working on him, so a bit of head scratching on our part. Finally, one of the nurses broke the news . . . "Eddie decided it was about time for his breathing tube to come out." Yes, Eddie managed to pull a fast one on his night nurse and, when she wasn't looking, pull out his breathing tube entirely. And as you can see from the picture below, we're talking about 24cm of breathing tube (9-10 inches).

Apparently he had a good cough and the nurse was going to help him clear his lungs, but Eddie managed to get his hands on the tube and gave it a good yank. Most importantly, he is ok . . . probably suffering from a pretty sore throat both from two weeks on a breathing tube and from pulling it out so abruptly. The nurses attached a temporary breathing tube in his nose and the plan is to watch how he does over the next few hours. If he does well, they won't replace the ET and will let him start to recover on his own.

So in addition to that excitement, we also welcomed a couple new furry friends to our growing arsenal of stuffed animals. First, the Berlin Heart company (yes, the company that makes the Berlin Heart . . . ah, you Germans are so creative!) sends a stuffed bear to all of its patients.

And then we were met at the front desk with this surprise! Again, I'm not naming names, but the guilty parties know who they are. Wow! If you're creative, there are some fun things to do at the hospital . . . we've added another to our list . . . walking through the corridors carrying a 3 foot tall stuffed bear. Impossible for the many patients, their families, and even the doctors not to smile when they see Sarah walking down the hall with this bad boy. Thank you!



First 24 Hours

Sleeping quietly . . .

Eddie's constant companion . . . air compressor driving his heart pump . . . all the way from Germany!

The pump itself . . . Eddie's temporary new heart.

Surgery Update, part 2

Wow . . . that's about all we can say right now. We just had the chance to see and touch Eddie for a few minutes before they had to do some more work on his dressings. Words can't begin to describe the awe and amazement we feel right now . . . our little boy has been through so much and to watch his chest rising and falling feels like a miracle. He has a long way to go, he continues to bleed quite a bit, and looking at him from the outside, he looks pretty rough . . . but he is stable and his surgeons/nurses are allowing themselves a few smiles right now.

Need to compose ourselves over the next few hours and will write more then. Thank you again for all your support!

Surgery Update

Sarah and I sat down with the surgeon shortly after noon to get an update. Overall, things are going well and Eddie is now connected to the Berlin heart . . . it has been operating for about two hours and no signs of trouble. There has been a lot of bleeding, which was expected, and the rest of the surgical team is trying to get that under control. Bleeding will continue to be a significant risk over the next 3-4 days while the Gore-Tex connectors on his left ventricle and aorta get used to the pressure.

We hope to see Eddie back in his room in the next couple hours.

To the OR

Eddie is on his way into surgery...we're expecting them to be done in the early afternoon. Thank you for all of your thoughts and prayers. We love you all!

Ich bin ein Berliner

It's official . . . Eddie will be rolling into the OR tomorrow morning around 7:30 for open heart surgery. While we wish he were getting his new heart tomorrow, we are encouraged that our doctors feel that the Berlin heart is our best option at this stage and that Eddie is stable enough to undergo this significant procedure.

In summary, during surgery Dr. Cohen will 1) remove the atrial stent placed on 3/6, 2) close the resulting hole in the atrial septum, and then 3) attach the LVAD/Berlin heart. We are optimistic we can avoid a bi-VAD scenario where both sides of his heart would need to be connected. All of the clinical research clearly demonstrates that LVAD success rates are much higher than bi-VAD, so our fingers are crossed. Dr. Cohen will make the final decision either while Eddie is on the operating table or, more likely, after he's had a few days on the LVAD. Some children don't respond well to the LVAD at first, but then recover good function after a few days and thus avoid an unnecessary RVAD. For more information on LVADs and the Berlin heart, see my earlier posts here and here . . . or Bing it!

More to follow!

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.

TGIF

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 :-)

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!

Recovering

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 . . .

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.

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.

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.

Dancing with ECMO

Today was relatively quiet with Eddie getting some good rest. We were able to read him a few stories which he clearly enjoyed and was even mouthing many of the words in spite of his breathing tube.

Our cardiologist and the attending CICU doctor discussed decreasing the flow on the ECMO today to determine how much stress Eddie's heart could handle. We did a bit of this yesterday, but Dr. Mazor (attending) wanted to take it a bit further today. While our cardiologist was dubious about the possibility of weaning Eddie off of ECMO altogether, she agreed it was worth a try . . . and if Eddie didn't respond well, we could simply increase the flow again and start preparations for a Berlin Heart.

So through the late morning and early afternoon, our ECMO nurse took Eddie's flow from 130 cc/min down to 50 cc/min . . . a 60% decrease. In general, his heart performed very well. His toe temperatures dropped and his kidneys struggled a bit, but overall, everyone was very pleasantly surprised. As a result, Dr. Mazor asked to take Eddie back up to 100-110 cc/min flow to give him some rest and then we'll begin taking him down again around 4am. By early morning rounds, he should be at 50 again at which point they'll run an ECHO. If things look encouraging, they will ramp him down even further, possibly turning off the machine altogether. While we're not letting ourselves get too excited, it's gratifying to see him doing so well.

On a more personal note, we had some wonderful visits today. Sarah and I have been buoyed by your kindness and generosity. It's definitely therapeutic to step away from the ICU for awhile and talk with friends about what's going on in the outside world. Thank you.

For your viewing pleasure, I've included a few snapshots of our day . . .

Every four days, the nurses have to completely transition Eddie's meds and leads. It took our nurse the better part of two hours to change all of the lines and set up a new rack of medications. This photo should give you a feel for how crazy this process is. Even better, our nurse forgot that this was an automatic faucet so each time she reached in the sink to sort/discard, she got a surprise :-)

Still life with tennis shoes

Finding time for hospital shenanigans . . .


Der Himmel über Berlin

Interesting statistic of the day. There are currently 13 patients in the Cardiac ICU and 7.5 nurses (I don't know what a half a nurse looks like, but I'm glad he/she isn't in Eddie's room!) Since Eddie has two dedicated nurses, that means there are 5.5 to cover the remaining 12 patients. So while I suppose Eddie's nurse-to-patient coverage ratio means he is one of the needier patients, I like to think it also means he's one of the cutest. All of the nurses have fallen for him . . . especially his thin blond hair. Who wouldn't?




During rounds this morning, there was some discussion about Eddie's good progress . . . his increased pulsatility, healthy lungs, and improved right ventricle function. Unfortunately, based on today's ECHO, his left ventricle continued to be significantly depressed. Our cardiologist and the attending physician agreed to decrease the ECMO blood flow slightly to test Eddie's response. Ideally, reducing his reliance on ECMO would generate some additional stress on his heart and could trigger increased function. So far the jury is still out. At first, his heart rate dropped lower and his hands/feet cooled noticeably (signs that his heart wasn't responding with as much strength as we would like), but later this evening his heart rate was back up to previous levels and his extremities (fingers/toes) were warming up. They will continue to decrease flows gradually through the night so we'll wait until morning to see how he has handled.

We had a conference with the Berlin Heart coordinator today and plan to meet with Dr. Cohen on Monday to discuss. In a nutshell, Seattle Children's has implanted eight Berlin Hearts in the past six years so Eddie would be number nine. Believe it or not, this is one of the higher numbers in the country since prior to December 2011, the device wasn't formally approved by the FDA. A couple tricky aspects to this procedure . . . Eddie will probably need to be inactivated on the transplant wait list while he recovers from his implantation surgery. Considering that the alternative is to stay on ECMO while the clock ticks and run the risk of end organ failure, a couple weeks of delay would be a small price to pay.

Speaking of price, the coordinator said the cost of the device can run anywhere between $50,000 and $120,000, but she was also quick to point out that Seattle Children's will never turn anyone away based on their ability to pay. Thank you, Microsoft, for one last year of 100% coverage!

So as things stand today, our best option is that a donor heart becomes available before we implant the Berlin Heart. This eliminates the need for two major open heart surgeries and reduces the risk of anything going wrong in the operating room. Our next best option is to transition to a Berlin Heart for the remainder of Eddie's wait (probably a matter of weeks) which buys us critical time until transplant. Until just a few years ago, Eddie wouldn't have had any option other than to remain on ECMO. If a heart didn't materialize, he probably wouldn't have made it since his current heart function simply isn't strong enough to circulate blood effectively.

I thought this diagram was good for showing how the Berlin Heart works. FYI, it appears likely Eddie will only need one pump (for the left ventricle) since his right ventricle appears to be functioning relatively well. So based on the diagram, Eddie's pump would manage the process as defined in steps 5 and 6. His body is healthy enough to take care of steps 1-4.

Thursday Rounds

As always, morning rounds are one of the best ways to get calibrated with the doctors and to get consensus decisions about how to manage Eddie's treatment. This morning, with Eddie continuing to stabilize, we had a good discussion about what to expect in the next few days.

Everyone is in agreement that Eddie will stay on ECMO through the weekend and we'll use Monday as the decision day for transferring him to a Berlin Heart. If we decide to go to a Berlin Heart, it will take a few more days to get a pump here so most likely, we'd be looking at going into surgery mid- to late-week next week.

Obviously, this plan is entirely dependent on availability of a donor heart. If at any time an acceptable donor heart comes into the picture, we will immediately prep Eddie for surgery and everything else goes out the window. Near-term transplantation continues to be the best available option.

Eddie has been resting very well this morning. He was much more alert today and was clearly responding to our voices and touch. The nurses are head over heels about Eddie and have remarked many times about how gratifying it is to see him responding so well . . . a strong sign that his neurological functions are intact.

I also thought I'd include a bit more information about Eddie's surgery Tuesday night. For the doctors in the audience, Eddie was showing evidence of pulmonary edema as a result of his earlier heart failure. This was manifesting primarily as bloody froth in his breathing tube. In order to reduce the left atrial pressure buildup, our doctors decided to proceed with a blade and balloon atrial septostomy, or cutting a small hole between the right and left atria. This would allow the pressure in the left atrium to come down and balance with the normal pressures in the right atrium. As is clearly shown in the diagram above :-), a metal stent was placed in the hole to keep the channel open. So far so good . . . his pressures immediately dropped to normal levels and the immediate risk of pulmonary hypertension has diminished significantly.

More later . . .

Day 2: Drawing to a Close

Knew I'd need to sit down and start this entry early in the evening (it's 7:50p) since we are pretty exhausted. Eddie continues in stable condition and his irregular heart rhythms are slowly, but steadily improving.

He has been completely sedated all day so no chance to see his smile or feel his little hands squeezing our fingers. That is hard, but we know it is super important for him to be as relaxed as possible while he is on ECMO . . . otherwise he would be pulling at the bypass tubes which would be a problem. While on the subject, I thought it might be helpful to post a diagram of an ECMO setup which is pretty consistent with what is going on in Eddie's case (except he is much more handsome than the kid in the picture below!):

Basically, the blue canula is taking the blood from the body and sending it into a centrifuge (blood pump) which is spinning at roughly 2,000 RPM and sends the still deoxygenated blood to the oxygenator (I know, it sounds like a bad Arnold Schwarzenegger movie). There, the CO2 is removed and oxygen is introduced into the blood simulating Eddie's normal lung function. The now oxygenated blood continues on its way back to Eddie and is pumped throughout his body in support of his weakened heart. All the while, Heparin (anticoagulant) is put into his bloodstream to keep the blood thin enough not to clot in the plastic tubing. There is a full-time nurse (we call her the Nurse of the Pump) who's entire job is to monitor ECMO performance at bedside. If she needs to get a drink or go to the bathroom, she must call another nurse to replace her . . . this is serious stuff.

So what did happen today? Well, we learned a lot and much of it was technical so won't bore you with too many details. Most importantly, we know that Eddie can't stay on ECMO for long (1-2 weeks tops) and our doctors want to take him off by early next week at the latest. Ideally, he will come off ECMO because his own heart recovers enough to take over independently. Realistically, he may need another bridging option to get him to transplant . . . a Berlin Heart. Good video introduction here.

We are just learning about the Berlin Heart, which is basically an assist device rather than a full-blown artificial heart. It is relatively new and was just approved by the FDA for pediatric use a few months ago. While not an ideal option because it requires more surgery and isn't perfectly suited for restrictive patients, it is our best course should a donor heart not come available in the next week.

On the transplant front, since the geographic, size, and blood type criteria have all been loosened, we are cautiously optimistic about finding a suitable match for Eddie in the coming days.

We also received a little more insight into what happened in the ER yesterday when Eddie's heart stopped. I spent time with one of the electrophysiologists today and he let me know that the ICD did indeed fire within milliseconds of the external defibrillator. Our surgeons, who were standing over Eddie when his heart stopped, were quite upset because the ICD wasn't firing when it should have . . . that's why they pulled out the paddles and went to work.

As a result, the electrophysiologist made three changes to Eddie's ICD to ensure the delayed firing doesn't happen again. First, he lowered the beats per minute threshold from 200 to 180 . . . so that the ICD would trigger at a slightly lower heart rate since Eddie tends to peak at lower levels than most children. Second, he reduced the number of beats above 180 required to fire from five to three. To prevent accidental firing, Eddie's heart has to stay above 180 BPM for three consecutive beats . . . it was previously five consecutive beats above 200 BPM. And third, he increased the initial "shock" from three joules to seven. When the ICD was originally implanted, our doctors were very conservative in the settings which seemed appropriate at that point. However, this is a perfect example of how each patient's unique characteristics need to be taken into account when calibrating such a sensitive device. Hopefully we'll never need the ICD again, but we feel better knowing that we've made custom adjustments to match Eddie's specific profile.

Finally, as a special gift, here is Eddie's admittance film from yesterday. Reminds me of the old Sesame Street game where you have to figure out which of the things in the picture don't belong . . . hmmm.