Dispatches from Boston

We had our 1 year post surgery check up in Boston at the end of September. Jake's echos had stayed stable following his last Cath in February, and he has just been soaring at home, so we were expecting good news. Still, there's always that brief moment of panic I feel just before they tell us the results. If Jake's behavior is any clue to how's he doing, I should have known we have nothing to worry about.

It's always nice to see Dr. Tworetzky. He just has a way at putting us at ease. Jake certainly seems relaxed with him.


Dr. T. had two fellows with him who were interested in the fetal intervention and we got a pretty thorough update on it from his explanations to them. Jake was the 14th two ventricular birth in some 5 years of doing the FI. They now have 20 successes. I'd say they're getting better at doing it and that number of successes is due to increase steeply as they do more and more FIs.

Dr. T. illustrated some key things that they learned specifically from Jake's case, including that they need to give these baby's time for their heart function to improve. We were just about ready to have the Stage 1 Norwood surgery which would have joined Jake's ventricles, giving him the traditional HLHS anatomy. However, that plan was thwarted when he came down with Sepsis (a blood infection) in the ICU. We needed to wait until the infection ran it's course and the antibiotics did their work before he could have surgery. Fortunately, by the time that happened, his left ventricle was pumping enough blood to indicate that it might be ok without the HLHS surgery. However, we would be back for the EFE surgery (see early posts).

Hearing Dr. T. explain Jake's case to these traveling fellows drove home the point that this treatment is still so new, so cutting edge, that they don't even have enough experience or successes to predict outcomes. This is trailblazing medicine and we are on the front lines with them.

But I digress...

Jake went to sleep for his echo, thanks to the sedative, so that they could get the most accurate pictures of his heart possible.

Dr. Lock and Dr. Del Nido made brief cameos in passing. Sadly, both Linda Zaccinini and Donna Morash had the day off, and we did not have the pleasure of bumping into Dr. Marshall.

We stayed at the Yawkey House - which is kind of like a Ronald McDonald house for families. It was nicer than most bed and breakfasts we've stayed at. We were also fortunate to run into Mary Beth and Anne Louise Atherton, Jake's roommates in the step down unit from his first hospital stay.

All in all, it was a good trip. Jake's heart has stayed stable since his catheterization from February 09 and we will not need to return to Boston for another cath in 6 months. We are so relieved and as long as everything stays stable, we can count on being home for a nice long while.

Binky woes

Right now: Jake crying inconsolably.
Reason: Removal of binky.

So we've decided once and for all to ban the binky. I know many may think that's a little harsh, that he's only 14 months and still needs it, and why don't we just relegate it to use only at bedtime. I agree one hundred percent. However, when both our pediatrician and cardiologist suggested that the binky may be related to a slight behavioral problem we have been experiencing, it was enough to convince us once and for all to ban the binky for good.

What behavioral issues could be caused by a binky? Try breath-holding spells. What is a a breath-holding spell? According to Wikipedia, breath holding spells are the occurrence of episodic apnea in children, possibly associated with loss of consciousness, and changes in postural tone that occur in 5% of the population. Basically, Jake wakes up in the middle of the night, sits up and begins to cry inconsolably, we rush in and pick him up, he cries so hard he can't catch his breath, he passes out, goes into a "seizure-posture", and looses consciousness for up to a minute. He's had about 15 of these such episodes since March. The episodes are terrifying and the hardest part is that they are benign. Despite that they have still resulted in me performing CPR, calling 911, and spending 3 days in the hospital.

When these episodes started to increase in frequency, our cardiologist became even more convinced that these were behavioral. She instructed us to put Jake back into his own room (we practically were sleeping in shifts with Jake in between us), and when he woke up crying, not to do anything. You mean like not even go into his room to make sure he's OK? I asked. Nothing.

It was incredibly difficult to stomach, but like clockwork, Jake woke up crying at 4am. Greg and I hovered outside his door, wringing our hands with concern and peaking through the open crack. Sure enough, when neither of came in to settle him, he lay back down and went to sleep. Amazing!

So problem solved, right? Wrong. It's incredibly hard not to run to your child when they are crying, regardless of the cause. So somewhere along the way, we started running back into his room to stick the binky in his mouth as soon as we heard a peep. It was a gradual progression but a few short weeks later he was back to his breath holding episodes again.

This time, our pediatrician concurred. Get rid of the binky. So here we are again, though this time we're sticking to our guns. So far, it kinda sucks, but as I finish typing I hear the cries softening and am hopeful that as I peak in, he will be sound asleep and stay that way until morning.

Jake and Binky: A History

Hey You With the Face

My son has the uncanny ability to make the most ridiculous faces, and by ridiculous, I mean ridiculously cute.

Check it:

Poopy Face


Happy Face


Mischievous Face


Crazy Face


Sleepy Face


I can't believe I'm sitting Face


I'm in the tub and having fun Face


Whoa! Weee! Face


I blink for pictures Face


Please stop taking my picture Face


I'm the man Face

Nature Boy

We've finally had a chance to spend some time outside these past few weeks. It seems like this year winter didn't go on forever as much as summer took so long to come. Some people might call that "spring", but it was far too dark and dismal for spring. Some might call it seasonal purgatory, or rather, San Francisco.

Luckily the rain has stayed away on the weekends and we're spending tons of time enjoying our new yard. Jake seems to enjoy it, as long as a blanket is underfoot. He's not quite sure what to do with this thing we call grass, besides rip it up and stick it in his mouth, but then he does that with everything. Here's some pictures from his first outing.

Would the real Jake Maxwell please stand up?

Jake Maxwell Casden meets Jake Maxell Shufro.



Greg's brother's good friend Lee Shufro had a baby about a week or so before us. His name... Jake Maxwell. Fortunately, we didn't find this out until after Jake was born, not that we would change the name we had picked out for months. Jake got a chance to meet the other Jake Maxwell at his cousin Nicky's 3rd birthday party. I don't think either one was too phased by sharing a name.

Mother Nature got one sick sense of humor

Nature has played yet another cruel joke on this family. As if it is not bad enough that the events of Jake's cardiac arrest are forever be etched into my mind to relive over and over again in my imagination, we will now have to suffer through a set of very similar events as a part of a benign condition Jake apparently has called "Breath Holding Spells."

We're in the hospital again, this time after Jake's fourth such spell in a three week time period. Jake had an echo on April 14th. The results were good, not normal, but still good, with no change from his last catheterization in February - overall, positive news. That night Jake awoke about an hour after we put him down crying. As I picked him up to comfort him, his crying became more intense. After a particularly hard sob, Jake could not get his breath back, and proceeded to hold his breath of close to 20 seconds. After he finally took that breath he went stiff, the color drained from his face, his eyes were open but rolled back. He was out for close to a minute.Then, just as quickly he snapped out of it with a couple more cries and went back to acting completely normal.

We placed him on the pulse/ox immediately and his vitals were normal. His coloring came back immediately, and he sucked down 5 ounces of milk like nobody's business. It was as if that event never happened, which is why the cardiology fellow on call wasn't too concerned when I called him to tell him what happened.

Fast-forward a couple days to Friday Night: We have a repeat of the exact same sequence of events as I describe above, right down to the timing - about an hour after we put him down. Only this time when I get to him he looks like he's having a seizure. His back is arched, eyes rolled back, hands and arms twisted up by his head and feet curled in. Then he slumps into unconsciousness again. We were terrified and called 911. By the time the ambulance arrived Jake was back to acting totally normal. We decided to take him in anyways so here we are - all of Jake's vitals are normal and he's no worse for the wear. It's hard to say the same for his parents, however. Quite frankly, I just don't know how much more we can take.

The making of a tear

I've got wings, but I can't fly...

I'm talking about Jake's hair. I always knew I'd have a little rugrat running around rocking some badass hair.

"I Love Presents!"

If I ever get around to updating the blog to recap our Christmas, you'll see the adorable onesie I got Jake that has a mischievous looking Christmas Tree on it with the voice bubble saying, "I Love Presents".



Jake was flush with presents for his birthday, too. Check him out on his new ride-on toys:

A new hot-rod from mom and dad.




And a "Race Day" ride-on from Cousin Nicky and his parents




Jake loves to ride around on both of them!

Psycho-Mommy Photo Shoot

An overzealous photo shoot the morning of Jake's birthday produced 80+ images. Here are some of the best ones:

One Bad Bug is One Year Old

Jake is one! He turned one four weeks ago. Make no mistake, I now suck at blogging. But rather than explain, I'm just going to share. Below is a letter that I've written to all of his doctors, and to anyone who has involved in his care over the past year. It sums up the emotion this occasion brings up for us.

March 26, 2009

Today is our son Jake Maxwell Casden’s first birthday and it's been an unbelievable first year. He's had more harrowing experiences than most people ever go through in a lifetime and has made it to this huge milestone no worse for the wear.

Jake was diagnosed with aortic stenosis evolving into Hypoplastic Left Heart Syndrome at 22 weeks gestation. We underwent a fetal cardiac intervention at Boston Children’s Hospital. Jake was born in Boston on March 26th and had a catheterization at six hours old to dilate is aortic valve and create an ASD. Jake had a rough start but with time and medical management, he was able to leave the hospital without needing surgery.

We eased into life at home with the knowledge that at some point surgery to repair or replace his valves and to remove scar tissue from his ventricle was probable. Eating, reflux, and weight gain presented problems, but overall, life was grand. We were so happy to finally have our little boy home.

On the evening of August 3rd, Jake awoke from a nap with an intense cry. When he settled, he just didn’t look right to us so we brought him to the emergency room at Columbia/Presbyterian. An echo showed he was in heart failure. The next day, while awaiting transport to Boston, Jake went into full cardiac arrest. It took thirty minutes of CPR and several defibrillator shocks to get his heart rhythm back to normal. Fortunately no neurological, tissue or organ damage was sustained and we set about on the road to recovery. Another month in the hospital and Jake was able to make the trip to Boston.

On September 9th, coincidentally our wedding anniversary, Jake underwent open-heart surgery at Boston Children’s Hospital for EFE resectioning, aortic and mitral valve repair and an ASD fenestration. There’s been no looking back since!

Today, Jake is absolutely thriving. In January, he turned a corner. At that time, he was barely able to sit supported. Now, just three months later, he’s cruising and climbing! His reflux issues have also subsided and mealtimes have become much less stressful and he is finally starting to gain a little weight. At just about 17 lbs, he is small but mighty and we find it difficult to keep up with his unrelenting energy. He's commando crawling, getting his teeth, babbling, pulling up to standing, cruising and learning more and more each day. He loves his books and toys and can entertain himself for what seems like hours without fussing. All in all, we’d say Jake has quite a normal, not to mention wonderful, life.

As for his parents, well, we’ve learned a lot about who we are and what we’re capable over the past year. Strength and love go a long way but you have to arm yourself with knowledge, too. We’ve learned to believe in the power of science and medicine and to trust doctors who do not always have the answers. And we’ve been inspired to be better people by the remarkable example shown to us by our little fighter.

As we reflect upon the events of the past year, we find it hard to find the words to thank you for the role you’ve played in helping Jake reach this milestone. Sometimes words are not enough; therefore, we have made a donation to the pediatric cardiology programs at both Boston Children’s Hospital and Morgan Stanley Children’s Hospital of New York. Please know that you have played a very important role in this family’s happiness and our gratitude to all involved is unending.

Sincerely,

Amy, Greg, and Jake Casden


We had an awesome party to celebrate with at least fifty people at our new house. It was the first time many of our friends had seen Jake since before his arrest last summer. I was so busy entertaining and showing Jake and the house off that I didn't get too many pictures. Here's a few...

Bad Bug cupcakes decorated by the lovely Laurie Rudnick


Make a wish!


Jake's first taste of sugar!


Cheers!