04 February 2012

A very brief post to say we've been cleared to leave tonight. Looking forward to sleeping in our own beds!

Hopefully we'll see many of you soon!

03 February 2012

Going Red for National Heart Month

How appropriate! I have a feeling we're going to be a lot more aware of these kinds of events soon . . . this is for you Eddie!

Picture This | Going red for National Heart Month | Seattle Times Newspaper

Marking Heart Health Month, the arches at the Pacific Science Center at the Seattle Center are lit in red lights with the Space Needle seen beyond on Thursday night, February 3..

The Bionic Boy

Eddie is back in his hospital room and recovering well from surgery this morning. They finished up with him around 2p and his surgeon (Dr. Permut, below) and electrophysiologist both said things went very smoothly.

Hard to believe our little boy has a piece of hardware the size of a pocketwatch in his abdomen, but we're thrilled to have the peace of mind that comes with the ICD. It is standard process to test the device before they close him up and testing went perfectly. For those that are interested in what "testing" looks like, the electrophysiologist basically induces arrhythmia in Eddie's heart and then they let the ICD do its work. Sounds a bit scary and this kind of testing does introduce a cetain level of risk, but it's the only way to verify whether the device is functioning properly. And to top it off, they ran the test twice and Eddie recovered both times. Phew!

One interesting note . . . since these devices are made for adults and there is basically no market for them in pediatric cases, our electrophysiologist and the ICD manufacturer need to petition the FDA for approval to re-program the device to shock at lower maximum values. Again, sounds a bit scary, but the risk that he would receive the full charge is extremely small and they should be able to have him back in a couple weeks to upload the new code. They actually have to enter custom software code since there really is no off-the-shelf solution for what they are doing. Eddie is a true pioneer!

Eddie's incision looks great. We have to be careful for the next few weeks . . . no strenuous activity . . . but otherwise it should heal up ok. Obviously he's not too happy about the pain and discomfort that comes with being opened up, but he's doing amazingly well with only Tylenol. They've approved morphine for him, but so far he hasn't needed it.

We had the Infectious Disease, Anesthesia, and Social Work teams come by today to do their transplant evaluations. I think he passed :-) They're really just asking questions to determine if there are any hidden risk factors before they conference later this week. I know we still need to talk to the Physical Therapy team and a few others before heading home. The great news is that it looks likely that they'll let Eddie go home tomorrow afternoon! We're looking forward to being back home together as an entire family.

If everything goes well, we won't have any more overnight hospital stays until the transplant operation. We'll do our best to enjoy our bionic boy in the meantime!

Big Thanks

This is a thank you post from Sarah . . .

Thank you for your phone calls . . . from near and far. Thanks for leaving me phone messages. Hearing your voice helps me really feel your love for us. I appreciate all the help with my kids. You've helped them before school and picked them up after school. When things have gone longer at the hospital, you've kept them hours longer than you expected . . . and were sweet about it.

Last night, the kids were so happy to see the hearts on our door, they had to stay up and make their own.

We've eaten your delicious cookies. We have some wonderful zucchini(?) bread with us at the hospital right now. The generous supply of sandwiches brought for dinner on Wednesday are still feeding us at the hospital.

Thanks to you, "Liz" is looking forward to her birthday rather than worrying about Eddie in the hospital.

All the cards.

We're in awe that the chaos in our life can be smoothed out so much by you . . . coordinating all of this help is a full-time job and we know none of you have that time to spare. We know you are sacrificing so much time on our behalf.

Eddie loves the pictures and notes that you left on our door. We have many of them with us at the hospital today.

Taking the initiative to come so far to visit us at the hospital so early in this journey . . . and bringing your calm presence.

You made brownies with my kids!

My stove top is still shining.

Sparkly pens.

Because you've offered to teach my lesson at church on Sunday, I can focus my thoughts on Eddie and his recovery.

We now have an accordion file to keep our huge volume of paperwork in order. Such an inspired gift . . . we can't thank you enough.

Your supportive emails have reminded me how much you care and offered help.

I look forward to sharing the bag of Valentine treats with the kids . . . thanks for thinking ahead for us!

Coloring books!

You've even offered to take the Christmas lights off our house (since we're so far behind!!!)

"Sean" Valjean (teddy bear) will soon have a sleeping bag for the hospital :-) Chances are, some of you were responsible for those bears being donated to Duvall Fire . . . never knowing that they would bless the life of our son in such an important way.

You tell us of temple visits and fasting on Eddie's behalf. We feel and appreciate your sacrifices.

Rides, playdates, and nurturing my children.

And the hugs . . .

Everything you've done helps so much . . . moment to moment . . . along this journey. We feel your prayers lifting us. And every kind thought reaches our heart.

Thank you. We love you.

Eddie in Surgery and Happy Friends

Eddie rolled into surgery around 9am and, if all goes well, should be done around 11. Fingers crossed!

On a lighter note, Sarah went home last night and found some surprises on our front porch :-) Thanks to all for your love, smiles, and care.

02 February 2012

Recuperation and Drinking from the Fire Hose

So today was a big day for all of us. Eddie was able to rest up a bit and recover from yesterday's procedure while Sarah and I were introduced to the wild and daunting world of organ transplantation. But before I say anything about what happened at the hospital, I want to take a minute to say thank you to EVERYONE who has helped our family over the past few days. I can't bring myself to name names because I know I would miss so many of you, so instead, I'll simply say "You know who you are and we love you!!!" We've have kind visitors, an abundance of loving emails and comments on this blog, families inviting our kids into their homes, and innumerable prayers and well wishes. We are truly blessed to know each of you and hope to return your kindnesses with our own soon.

Meanwhile, back at the ranch . . .

Eddie slept great last night and woke up to a breakfast of eggs and oatmeal (Seattle Children's may be a Tier 1 pediatric cardiology hospital, but they could work on their menus a bit). Anyone who knows Eddie won't be surprised to hear he gobbled down the oatmeal. Sarah arrived around 9:30am and walked into a room crammed with what appeared to be well-dressed high school students . . . a quick reminder that we're in a teaching hospital!

Basically, our attending physician, Dr. Law, would pepper the residents with questions about Eddie's condition and they would do their best to mask their fear and give a decent answer. It was fascinating to watch and I have to admit it felt like I had slipped into an old episode of St. Elsewhere.

After rounds, Dr. Permut (Eddie's ICD surgeon) stopped by to discuss implanting an ICD in pediatric patients and its attending risks and benefits. He was also kind enough to bring a couple samples so we could get an idea of what kind of hardware they wanted to put inside our little boy. As you can see from the picture below, these are relatively small devices, but not insignificant when you consider Eddie's size.

We are going to move ahead with the ICD and surgery is scheduled for tomorrow morning at 9:00am. It's hard to think of Eddie going into the OR again so soon after his cath procedure, but we feel much better knowing that the ICD will minimize the risk associated with arrhythmia which was a likely cause for Eddie's previous cardiac fainting episode. The surgery should last about two hours after which Eddie will probably need a couple days to recover. We hope to leave the hospital early next week, but that may depend on . . .

The transplantation evaluations . . .

We had an hour-long conversation with Dr. Law and one of the transplant coordinators about the waitlisting process and some of the realities of heart transplantation. First, Dr. Law informed us that all members of our cardiology team agreed that, considering the results from Eddie's cath, we should kick off the transplant process immediately. Yesterday, there was some discussion about waiting for genetic testing results to come back, but Eddie's pressures were high enough that waiting for another six weeks didn't seem wise.

So the first step in adding Eddie to the transplant waiting list is to complete a battery of evaluations from various teams: cardiology, infectious disease, physical therapy, even social work. The goal of these evaluations is to determine if Eddie is a viable candidate for transplantation. Basically, the thinking is that if Eddie were too sick (or too well), he wouldn't qualify and this could only be teased out by having several different groups assess his condition and then conference to make a final decision.

Happily, Dr. Law felt that Eddie was in a very favorable position at this stage . . . that he could potentially qualify for a high status on the waitlist due to his restrictive diagnosis and pulmonary hypertension, but not sick enough to require hospitalization during the wait period.

We have agreed to proceed with the evaluations since they can perform most of them while Eddie is in the hospital for his ICD surgery. Once the evaluations are complete and a decision is reached about his viability as a candidate, then we are faced with the real decision about actually adding Eddie to the transplant list. It goes without saying that we are taking this process very seriously and will make a final decision when that time comes . . . probably in the next 7-10 days.

Needless to say, Sarah and I are in awe of the patients and their families that have navigated these waters before us. In fact, we are working with the transplant coordinators to connect with families of other young transplant patients and are excited to meet several in the weeks ahead. It sounds like this is a pretty tight knit group and we are anxious to learn from them and come to grips with many of the difficult realities of living with a new heart.

So in all, we continue to feel overwhelmed and intimidated by the volume of information being thrown at us every day. In spite of that, we also feel quietly confident we are headed down the right path and are certain that we'll be guided as we make such important decisions in the coming days. And finally, it was wonderful to see the day end the same way it began . . . with a dose of welcome sun flooding into the room. We love you.

Seattle Sunshine and Work in Progress

Thought this view from Eddie's hospital room was an apt metaphor for today . . . lots of work ahead, but hey, if we can get sun in Seattle, anything is possible.

01 February 2012

A Few Answers, More Questions

Well, Eddie came back to us and we are so thrilled to see him. He is resting now . . . watching a documentary about wolverines before going to sleep :-). Sarah headed home to be with the kids and I'll stay at the hospital tonight.

To the results . . .

This looks to be one of those scenarios where the good news and the bad news are all mixed up together. It certainly does look like Eddie is suffering from restrictive cardiomyopathy (RCM) based on the pressure readings from his heart and lungs. His results were 2-4x higher than normal which is obviously cause for concern, but consistent with the original diagnosis. The scariest findings were in his lungs since non-reactive pulmonary hypertension would disqualify Eddie as a transplant candidate. We do not want to consider our options if transplantation is off the table.

So Dr. Rubio decided to perform a "challenge" to determine whether or not the blood vessels in Eddie's lungs would respond favorably to applied oxygen. Basically, his pulmonary blood pressure dropping in response to oxygen would be a very favorable result and demonstrate sufficient resilience to be considered for a transplant. If the oxygen challenge didn't produce good results, he would conduct another challenge where Eddie would inhale nitric oxide to promote pulmonary dilation. Thankfully, Eddie responded favorably enough to the oxygen that Dr. Rubio didn't bother with the nitric oxide.

So our immediate dose of bad/good news is that our doctors are virtually certain that this is a case of RCM (bad), but that Eddie is very much a viable transplantation candidate (good). Our doctors are also sending blood samples to a lab at Harvard to conduct genetic testing to look for the particular gene mutations that define RCM and hypertrophic cardiomyopathy (HCM). If the results come back positive, then we proceed with absolute certainty about the diagnosis. Regardless of the root cause, however, we have very limited options for treating this condition.

Finally, an obvious concern we have in the intermediate term is how we can prevent Eddie from having another cardiac "event." There is one option we are discussing with the team which would involve inserting an implantable cardioverter-defibrillator (ICD) that would kick start Eddie's heart automatically in case of cardiac arrest. The ICD would be in place until transplantation at which point it would become unnecessary and need to be removed. We still have several questions about this option and will be discussing it in further detail with our doctors tomorrow morning.

So as we go to sleep tonight, we are comforted to know that Eddie is resting peacefully and that he came through his cath procedure well. We have a few more answers and know that transplantation is still a viable option. And finally, we are glad to have options on the table for addressing the very real concern of cardiac episodes in the near future. We'll take any victory, large or small.

More tomorrow . . .

Out of Cath Lab and Doing Well

Just a brief update to let everyone know that Eddie is out of the cath lab and in the recovery room. We haven't seem him yet, but have had several doctors and nurses tell us he is doing great. We expect him to join us in his room by 5p tonight.

He has been admitted and we will be staying at least through the night. We have preliminary outcomes, but will wait until later tonight to talk about details. In all reality, it appears likely he will be in hospital for a few more days.

So thank you for your thoughts and prayers . . . they make a huge difference!

I've attached a video of Eddie immediately before he went into surgery . . . but after they administered some pre-meds. The anesthesiologist warned they would make him silly, but even we were surprised at how effective they were :-)

Love to all.

31 January 2012


We'll take Eddie to the hospital at 9:15 tomorrow morning to be prepped for the catheterization. The cardiologist will be Dr. Rubio, and I had the chance to talk to him yesterday over the phone. Basically, he will conduct a right and left cardiac catheterization to measure the oxygen and pressure inside his heart.

He also plans to perform a myocardial biopsy which should help confirm the diagnosis and rule out other potential conditions such as constrictive pericarditis. Based on what he sees, Dr. Rubio may also perform a couple challenges on Eddie's heart and lungs to see how they respond to fluid and oxygen. For the latter test, it sounds like he would introduce nitric oxide through the respiratory tract (inhaled) to dilate Eddie's pulmonary blood vessels. He mentioned he would only perform this last challenge if Eddie's pressure measurements were high.

Based on his initial viewings of Eddie's EKG and echocardiogram, he doesn't see any telltale signs of high immediate risk. He was very clear that if the RCM diagnosis is confirmed by the catheterization, then transplantation will be the only option, but he's not ready to make that call yet. He reiterated that heart transplantation is not a cure, but rather a life saving option that introduces entirely new challenges. I assured him that we don't expect any quick fixes, but that we have faith and confidence that by counseling together we can arrive at the best treatment options and make the necessary life adjustments. We are prepared to accept any potential outcome . . .


Eddie and the EEG

Today Eddie went to Seattle Children's in Bellevue for an EEG. This was to discover whether there might have been a neurological cause for his collapse January 8th. It is also great to have a baseline for his brain activity as he begins treatment for his heart condition.

We kept him up 3.5 hours later than normal last night, and he didn't take a nap yesterday, so he was extra tired. He was quiet and still as the tech attached the electrodes to his scalp and a couple to his chest. I was so glad Eddie was able to fall asleep on demand so patterns during sleep could be measured.

We dropped John off for a day's work after the test, and Eddie and I bought milk and a few things at the grocery store. He was still in his green striped sleeper and had really crazy hair from the goo and the stuff that took the goo off. We discovered we could make it to story time at the Duvall library, which Eddie hates to miss. After that we got Eddie dressed in the car and he played at the park by our house, pushing his bear in the swing . . . he had a great time. It was so nice to see him active and happy.

We had so much help and compassionate visits from friends today. The kids were taken care of before and after school, we had a fabulous hot dinner brought in, and we got lots of hugs and even some yummy cookies! Eddie and I actually took a short nap together too, once all the excitement was over.

Thanks to all who helped in so many ways today--we couldn't make it through without you all! Keep us in your prayers tomorrow. We hope for the best possible results after his catheterization.

Setting Out . . .

Hello dear family and friends.

We've set up this site to keep everyone up to date on Eddie's progress as well as giving you the opportunity to share your thoughts and well wishes. We are so thankful for each of you and, in spite of the challenges we'll undoubtedly face in the days/weeks/months/years ahead, we face the future with faith and confidence knowing that we have such a wonderful network of support and love.

Take a minute to read Eddie's background story and diagnosis. Also, please feel free to leave a few comments. Know that we read and cherish everything you write even if we can't always respond personally.

We love you all and thank you for your prayers!

Background Story

We wish we could talk with you in person to tell you about this and to let you know what is going on in our lives and to ask for your support and patience. We're going to need it! We don't have a problem talking about these things or answering questions in person, but felt it would be overwhelming to seek out each of you and let you know the basics.

Cause for Concern

On the evening of Sunday, January 8th, Eddie (who turned 3 in October) passed out without any apparent cause and John could not detect breathing or heartbeat for about 3 minutes. Since then, doctors have said there is a good chance he was faintly breathing and his heart was faintly beating, but it was an incredibly shocking episode for our whole family. "Stefano" actually picked Eddie up and felt how limp he was, and the entire family was present while Sarah was on the phone with the 9-1-1 dispatcher and while John applied CPR. By the time the Duvall EMTs arrived about four minutes later, Eddie had started breathing and was slowly regaining consciousness. Eddie was taken to Evergreen ER in Kirkland for tests and released at 11:30 that same night.

Looking for Answers

On Tuesday (1/24), Eddie went for his cardiology appointment at Seattle Children's to see if they could tease out any possible causes for his collapse. After an EKG and echocardiogram (basically an ultrasound of the heart), Eddie was diagnosed with Restrictive Cardiomyopathy. His left atrium was described by Dr. McQuinn, head of the department, as "huge." This is an extremely rare disease . . . especially in children . . . and there is very little research available to our doctors. Of the three types of cardiomyopathy, restrictive cardiomyopathy is the rarest and least understood.

Next Steps

On Wednesday (1/31), Eddie will undergo a procedure called catheterization to determine the pressures of the blood as it moves through and out of his heart muscle. After this procedure, the doctors will have a better idea of what will be needed. As we understand it, there is no treatment or cure for this disease and Eddie will need a heart transplant. The catheterization will help determine where he belongs on the transplant list.

Looking Forward

We are really glad to have a diagnosis. It is a blessing that through his collapse earlier in the month we have been led to know what can be done to help Eddie. We are very optimistic and look forward to this opportunity to become closer as a family and closer to those who will reach out to support us. We believe that whatever the short term or long term outcomes, Eddie and our family will be okay. We believe this life is a time to grow and learn and make choices that help us become more like Jesus Christ. We know that just as we are with Eddie now, he will be a part of our family after our time in this life ends. We know that if he doesn't get to stay with us now, he will be in a wonderful place until we get to see him again.

What You Can Do?

We appreciate any positive thoughts, prayers, moral support, and love in the coming months. Thank you for your love and support and for caring about our family. It truly makes all the difference to have good people cradling us in this "best of times and worst of times."

We love you.

Sarah, John, "Stefano", "Liz", "Phil", "Janey", and EDDIE!