10 February 2012

All Clear

We heard from our cardiologist today confirming that the kids' ECHOs are clear. That is one weight, at least, lifted from our mind.

Overall, it was a good day at the hospital. Thankfully Whitney spent the week with us and helped chaperone the girls through their tests while I was with the boys. And Sarah hung out with Eddie in NeuroPsych while they ran him through a series of IQ and motor skills testing.

Our consult with Nutrition was really about how we can prepare Eddie for surgery . . . gaining another pound or so to be at optimal weight. So we've welcomed whole milk back into our kitchen and we'll be slathering some extra butter on his toast each morning! Not very impressive to Eddie apparently . . . since he fell asleep almost immediately :-)


We had a great meeting with Dr. Cohen (surgery) and he walked us through the organ selection process, surgery, and post-op recovery. We were surprised to hear that he'll begin to get calls from hospitals offering organs almost immediately. It turns out that there are quite a few organ offers, but the real challenge is finding an ideal match. Luckily in Eddie's case, Dr. Cohen can be more selective since his heart isn't in late stage decline. With a defibrillator giving him some level of protection from arrhythmia, he is not in immediate danger since pulmonary hypertension and general organ failure can be monitored over time. If he begins to take a turn for the worse, we'll have to consider accepting a "good enough" offer . . .

Finally, we met with Physical Therapy and Eddie basically got to throw a football, run, jump, kick a soccer ball, balance on one leg, and walk on a balance beam. Needless to say, he loved it! The purpose of the PT consult is to establish a baseline of physical performance so the therapists can help Eddie get back to normal after surgery. Doesn't hurt that he had some fun after a long day.

A big thank you to the wonderful staff at Seattle Children's. Can't say it enough, but we are so fortunate to have them in our backyard. Happy to pay the 520 toll and negotiate Montlake traffic if those are the biggest inconveniences.

09 February 2012

ECHOs and Evaluations

Tomorrow is a busy day for us at the hospital as we have echocardiograms for each of the kids and Eddie's final transplant consultations. Anyone interested in learning more about how echocardiograms are administered and how they are used should watch this video and read this article . . . pretty good introductions. This is what the kids will be doing tomorrow instead of going to school! (BTW, the ECHO below does not belong to anyone in our family, but it does represent a straight forward example of RCM . . . note the atria (on bottom of screen) ballooning as they clearly face resistance due to the poor relaxation of the ventricles (top).



There is always the possibility that Eddie's RCM diagnosis is hereditary so each of us will be evaluated via face-to-face consultation and echocardiogram. Several doctors have asked if we have any family members that have suffered "sudden death" at a young age . . . and so far, we think the answer is no. Any Harper or Powell relatives out there that know otherwise? Regardless, it's important for each of us to be tested and the kids are on deck first.

There are some specific gene mutations that occasionally characterize familial restrictive cardiomyopathy. It appears that these mutations cause production of defective cardiac proteins which interfere with the heart muscle's ability to relax. This, in turn, means not enough blood enters the left ventricle to be pumped to the rest of the body. And logically, this means blood begins to back up in the left atrium and lungs causing pulmonary hypertension and potential heart failure . . . more or less, a textbook definition of what RCM is. Once again, if any of you are interested in further clinical reading, feel free to browse here or here.

For Eddie, he will be meeting with neuropsychology, nutrition, occupational and physical therapy, and finally, with the lead transplant surgeon himself, Dr. Cohen. Once these evaluations are complete, we are on track to have Eddie added to the transplant waitlist which should be no later than next Friday (2/17).

Hope to post a follow-up entry by tomorrow evening . . .

05 February 2012

We're Home . . . What's Next?

Well, after four days and two surgeries, Eddie is home and resting well. The kids were thrilled to see him, but I think he was more interested in sleeping than hanging out with his siblings :-)

He'll be taking a bunch of meds for the first five days or so but then will taper off. We're excited for the incision to heal and to get our Eddie back without the pain killers!



So what's next? We wish these surgeries marked the end of the battle, but unfortunately, we are only at the very beginning of a long and difficult journey. As with most organ recipients, Eddie will gain a healthy heart at the expense of his immune system. He'll be on immunosuppresants for the rest of his life to avoid organ rejection, and will need ongoing monitoring to ensure that his heart remains healthy and that he doesn't fall victim to all kinds of other nasty illnesses due to his weakened immune system. So this is just the opening round of what's sure to be a long, no-holds-barred, knock-down, drag-out fight.

As it stands now, we'll need to go back to the hospital this week to finish the transplant evaluations with Occupational/Physical Therapy, Neuropsychology, and Cardiology since they weren't able to complete before we left the hospital. Only when those evaluations are complete can they conference and formally submit Eddie's name to the transplant waiting list and assign a priority level. Based on initial conversations with the transplant team, they believe they will be able to list Eddie as a 1a status which is the highest priority. To qualify as 1a, a recipient typically needs to be hospitalized and have a life expectancy of fewer than 14 days without medical intervention, but because of Eddie's "aborted sudden cardiac death" episode a few weeks ago, he may jump to the top.

Once he is waitlisted, there is no guarantee how long it will take to find a matching donor. This is a pretty morbid topic so I'll leave it to you to decide how much you would like to learn about the process. A couple very good sites are the United Network for Organ Sharing and the Organ Procurement and Transplantation Network. Also, for those of you that are statistically-minded, Milliman Research publishes a triennial "U.S. Organ and Tissue Transplant Cost Estimates and Discussion" report with all kinds of interesting information including overall transplant costs, survival rates, and average wait times.

I'll talk about what happens after transplantation another time. I'm exhausted and really trying to stay focused on the here-and-now rather than getting too far ahead of ourselves. We are simply grateful to have another day with our wonderful boy and the joy that we feel now having him with us fortifies us for the difficult road ahead.

Thanks for your many thoughts and prayers on our behalf!