10 March 2012

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.

08 March 2012

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 . . .

07 March 2012

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.

Out of Surgery

Eddie is out of surgery and stable. The stent was placed successfully and his left atrial pressure has dropped dramatically which is very important to avoid pressure building up in his lungs.

He is still showing very poor heart function so the bypass is basically doing all of the work. Where his left ventricle used to pump well but struggled to relax, it is now showing significant weakness in the "squeeze" or pumping function. This has not changed after surgery and we can only hope he will regain some independent pumping ability in the days ahead.

This was a very high-risk procedure and we are thankful he came through ok. He is still very heavily sedated so we're hoping to see more responsiveness tomorrow after he recovers a bit more.

Sarah and I are staying at the hospital tonight and will share more tomorrow as we have news.

06 March 2012

Familiar Faces at Seattle Children's

We will intentionally keep this post short on details, but we thought it would be important to let everyone know what is happening with Eddie. So, in bullet point format, here it is:
  1. This morning, Eddie woke up, had breakfast, got dressed, and was otherwise on track for a great day
  2. Without warning, around 8am, he said he felt sick and said he wanted to go to bed. It was clear something was wrong so we called 911 immediately and worked to keep him awake and alert
  3. Medics arrived and took Eddie and Sarah to Seattle Children's via ambulance
  4. En route, Eddie threw up but seemed to be doing a bit better
  5. Upon arrival at the ER, however, he took a turn for the worse and his heart definitely was beating very irregularly. A code call went out and the ER was soon full with 20+ doctors, nurses, and specialists working to keep him stable
  6. Our surgeons made the decision that Eddie needed to be put on an ECMO machine (Extracorporeal Membrane Oxygenation) since his left ventricle was no longer squeezing sufficiently to support him on its own
  7. He spent most of the day resting from surgery and the doctors/nurses were busy monitoring his vitals for any signs of worsening/improvement
  8. While Eddie was heavily sedated through this process, we had several happy moments when he was alert enough to nod his head in response to our questions and to squeeze our fingers when we asked him to
  9. Around 5:30p, shortly after the kids arrived to say hello, our doctors told us Eddie needed to go back to surgery because there were signs of pulmonary and cardiac distress that needed to be addressed quickly. Basically, there was some blood coming up through his breathing tube and the fear was that the pressure building up in his left ventricle and atrium would soon spread to the lungs which could eventually make him ineligible for a transplant
  10. So as of this moment, we are waiting to hear back from our surgeons. This procedure requires inserting a balloon stent between the left and right atria to relieve the pressure in his left atria/ventricle. This is not a simple procedure and carries significant risk
At this point, it appears certain Eddie will be in the hospital until a heart becomes available for transplant. Due to his current condition, our transplant surgeon has expanded the available geographic area to cover virtually the entire country and will now look at possible using larger hearts that we wouldn't have previously considered.

We are extremely grateful to those that helped at the hospital today and at home with the kids (you know who you are!) We're sorry we haven't been able to reply to your many texts, voicemails, phone calls, and emails. Know that we love you and feel the impact of your thoughts and prayers.