Summer Time...

And the living has been relatively easy. We took Liam on our first family vacation to Sebago Lake, ME, over the July 4th holiday. Alex spent his summers there where his Grandma had a house. Its really beautiful, peaceful and undeveloped, which meant a true escape. And the house we rented had a helipad nearby so God forbid we needed it, we had it. I know, what a way to think on vacation but driving 6 hours north east away from life is scary too!

Two days before we left, Liam decided to take his first steps unassisted! Huge news. Our therapists think by the end of this month, he will be doing this more often, standing for 10-15 seconds at a time, take 2-3 steps, versus crawling full time. He cruises on everything, climbs, jumps, falls..without fear, so if he could start the steps soon, it would be pretty amazing. We recently started speech therapy and are already seeing little changes. He always has been a talker but he is making decisions, picking up the right flash card when asking for the dog for example, and trying to repeat everything we say- no more trucker Mom. I need to clean it up.

We have a CHOP day coming up in August with his immunology team. Its my hope that the bloodwork from this visit has improved since the last. We want to enroll Liam in a two mornings a week class and need the ok for him to be around the other kids. This class is unique- its called Stepping Stones and Liam and I made some good friends at a once a week playgroup it runs for kids his age with Down Syndrome. The two year old class meets for 2 hours twice a week where Liam will be receiving four extra hours a week of therapy, learn how to act in a classroom setting, and become more independent. During the class, unlike the group we were attending, I can't be there- that will be the hardest part! But there is a parent support group that meets at the same time which will be good for me too. Its a great way to learn from other wonderful parents and make friends.

Because his immune system is so low, we need to first ask the school if any other kids haven't had vaccines. If they haven't been updated, we either need to give Liam more meds to protect him or say no, which would frankly suck. We have to depend on the general public to protect our son and with the Jenny McCarthy followers (seriously, this is your Dr. Spock?), our son has a higher risk of being exposed to something that could be fatal for him. If his bloodwork looks good, maybe we can even start getting vaccinated. While its scary because its a risk, it would be a relief too.

Other than that, we are enjoying the cooler days (too hot\humid is on the no-no list because of the heart), yesterday we walked to the post office and ran a few errands rather than drive, which was great for me. Lots of time at the playground, baby pool, gardening. All with lots and lots of 100 SPF on for the two pasty white blonde kids we are :o)

Fly High Seth

One of the families I have followed online has been Seth's. He sadly lost his fight last week. Seth had the same diagnosis as Liam. His parents just posted this video they shared at his funeral. Please say a prayer for his family and Seth.

http://www.youtube.com/watch?v=OTs-I3gYujU

CHOP Cardiac Appointments

Pics Above: Liam happy to be in his big boy chair, 18 month pediatric appointment results- 32.5 inches, 50%, 26 lbs 12 oz, 75%. Typical chart.
Liam with JP and Reilly- two of his CHOP friends, survivors and miracles. Reilly was recently removed from the heart transplant list because her heart has begun to heal itself. We were there to celebrate JP's 2nd birthday!


Its been awhile since we've had medical updates but this past Wednesday, the three of us headed down to CHOP for a day full of cardiac tests and doctor visits. I have been nervous about this for a while- it was a big test, if he looked good, we would have a year between visits and we would be given an outlook on future surgeries. The word is possibly 20 years or more down the line. If the heart didn't look so good, we'd be visiting more often and discussing treatment (ie open heart surgery) plans.

I was also nervous because Liam is feisty- seriously, that NICU doctor who described our future as having a docile and nonspeaking son can babysit for a few hours and enjoy choking on his words as he stops the Troublemaker from bringing down the place. Anyway, having a baby lay down through an echo was hard but a kid who only wants to crawl, cruise, move constantly during his naptime...sigh.

Remarkably, his biggest fuss was when getting his blood pressure taken. He actually sat still for the doctor who examined him, did well for the EKG and then off to a 50 minute echo where he had to lay on his back and not move. Let me tell you, I was a dancing monkey. Alex restrained him because Liam was mostly fascinated by the equipment and wanted to give himself the echo. I danced, I signed songs, I sang, I blew bubbles. That poor technician. Oy.

After all the testing, we headed back to see the doctor. The first words out of her mouth- "Well...Liam's right ventricle is definitely enlarged." Right about there, my stomach dropped and it took everything inside of me to look her in the eyes without crying. But I also remembered the pressures of a tet heart cause the ventricle to be enlarged and some kids hold up well with this. She then spoke again and I was able to breathe. She told us it is a concern but not one for now, or even in the next year. Most tet kids have this and live for a few years before going into heart failure. Liam is healthy enough that he doesn't need to be seen for a year, which is positive. She wasn't going to worry and we were a few years at least from beginning the process of next steps. We are not looking at the 20 year outlook though, much closer to 10 if his heart stayed within this range.

Because he was squirmy, the technician didn't get the best pressure readings and pictures so when he gets closer to 3 or 4, she is going to begin MRIs. He will need to go under general anesthesia for this, which we don't like but it does mean she will get the best picture possible of his heart which we need. At the earliest, he will hopefully be at least 10 or so before his next surgery. It would entail replacing his pulmonary valve, which right now is typically done with animal tissue versus human tissue, which he received through organ donation last year.

The good news for us is by this time, the odds are in our favor of Liam NOT needing an open heart surgery and instead, being able to replace the valve with a cardiac catherization. Europe is doing it, some hospitals in the US are experimenting with it now, Johns Hopkins Children's has had success. While still a dangerous procedure, he would not need bypass time, no invasive surgery, breaking the ribs, etc. It would be a vein insertion in the groin and the repair would go on the end of a stent. Liam had this procedure last year when they couldn't get a good picture of what was happening in his heart and it was discovered his patch had dehisced. We know he can handle this procedure, he did at his very sickest already. Hey Bill Clinton did it, Liam's got this. It gives us hope that even though he will need another surgery, modern medicine can make it a much easier process next time. How lucky we are that he is born now- its likely he wouldn't have survived ten years ago.

Here is a great article on how another kid had this, and was at this hs graduation within 48 hours-
http://www.hopkinschildrens.org/a-less-invasive-pulmonary-valve-repair.aspx

Sometimes I read this back and think to myself- when did I become this calm and positive outlook person? How can I be calm when speaking about my son's heart being taken out of his chest and repaired, yet again? I have no idea how this happened but it has. And it is what it is. What a different life than we imagined when we first got pregnant and discussed Senator Kuhns future. Hey who knows? Maybe there will be a district who is able to recognize how amazing a kid like Liam is and what they can learn from him.

More Pics from DC

Haven't had our computer at home but Alex is home for the weekend so here are some more favorite shots from our trip!

Visiting DC

Alex, Liam and I spent a lovely 6 hour car drive last Friday down to DC (thanks random bear on the NJ Turnpike) and will be here for a few weeks as Alex goes to class for work. Liam and I are spending our days visiting friends and seeing old favorites of mine. Over the weekend, we hit up Eastern Market where we bought some fruit, walked to the Capital and then down to the Mall. We took a ride on the carousel and looked at planes. Well, Alex and I did, by then, Liam passed out. However, he still had some astronaut ice cream when we got back to the hotel.

We are staying in Old Town, where our wedding guests stayed, so the memories have been nice. We are walking down King Street every day, just getting fresh air, checking out everyone. Since we've been here, Liam has been sleeping very long nights, which has been nice for us! We all are fighting allergies but so far, a little Claritin and Dayquil have done the job.

I was able to speak with CHOP yesterday about Liam's latest lab work. They tested his blood against a control in three situations to see how his immune system did. In January, it functioned at 55% level. This time...wow. The first test, 85%! Second, 90% and the third....100%. His IGG levels have also increased, from 491 to 719. Anything over 450 is normal so we are officially at a stable level for his IGG. The not so great but not unexpected news is that his t-cells, which fights and kills viruses, and also teaches other cells to do the same, decreased slightly. We are at 481 overall when a normal immune system is up around 1200. Within the t-cells, his cd4 dropped to 275. The goal is at least 700. So while his immune system is doing great with what he has, he still doesn't have much. We are going to stay on Bactrim and still hold off on vaccines. The immune team is very encouraged that his system functions normally despite the low number of cells. So when he has recovered and his system has gotten to the normal level of t-cells, he is going to be one seriously healthy kid!

That's all for now, happy spring and soon, happy Easter!

Superboy!

On Tuesday, Liam and I spent a long rainy day at CHOP. We finally got off the wait list for their Trisomy 21 Clinic after over a year wait! Unfortunately, I wasn't that happy with it so I'm hoping with the next visit, I will see its worth. So many other parents speak so highly of it, maybe it was just a gloomy day.

We met with a doctor and a team of therapists- OT, PT and ST who evaluated and played with Liam on a small mat as the doctor asked me questions about our daily life. He was a little overwhelmed and didn't have much room to move but he still played, and seemed to hit it off with the PT. We received individual evals from each therapist and then the doctor with an overall one. It started off good- he is super social, very strong in his tone and core strength, independent and knows what he wants.

But, there's always a but, the areas we were told to work on were the complete opposite of every other evaluation he has had in the last 16 months. They wanted him more vocal (seriously people?) and less independent- huh. They would like Liam to begin to tell us what he wants versus going and getting it himself. I was a little taken back by that but understood he should communicate more, especially if he needs us specifically but thought being independent was good! I was also advised we needed a lot more "real" words versus mama, dada, baba, moo, etc etc. Again, surprised because he has been babbling and talking since a very early age. When she said most 16 month old have 50 real words and start putting sentences together, I did ask if that was the Harvard track? No smile. Yikes.

From this evaluation, we had agreed to participate in a NIH study called Growing Up and its to help create new DS charts. Liam hasn't fit on those charts since about 9 months old and is 50% on the typical kids chart for both height and weight. We will be followed for 3 years every 6 months. The doctor at the study did tell us that he was one of the biggest so far they have seen at his age, taller and heavier (but proportionate). Then they did a neurological study, checking tone, cognitive skills etc. And funnily enough, she marked him as typical, non DS in every category. I made a comment that she should go talk to the other doctor who told us Liam was only functioning at 8-10 month level, despite all the other groups saying he was on track with his age. Sigh.

Unfortunately, we headed to the labs next to get our immune levels checked next. We have not heard back on this yet, it usually takes a week to two. His blood is tested against a control group as it reproduces, etc. Its very neat to hear about, even better when it does the right thing!

So the entire way home I freaked. Thanks Maureen for calming me down. I know a lot of 2 and 3 year olds. I don't really hear them speak with that many words or sentences and to think that we had been told his communication was his highest and most advance area. I should point out that as soon as we got home, I redid some of the tests with Liam and he did it perfectly for me, whereas at the doctors, he turned away and wanted nothing to do with her.

And the next morning, after a night of being sick, upset, tossing and turning, I went to ShopRite to get some groceries and walked past a familiar looking couple. They looked at me too and we figured it out- our kids were at CHOP at the same time. And as soon as I asked how their daughter was doing, their faces fell and I knew. She passed a week or so after we were discharged last March. It hit me like a ton of bricks and I cried the entire ride home. And then some. I was sad but mostly felt guilt. Here I was worried about one not so great evaluation and they lost their child, a 16 month old little girl who was being taken off breathing vent and it was too much. Do you know how many vents Liam was off and on? It is frightening.

We saw two of our three therapists this week and I explained my concerns and let them read the evals. They all had the same advice- we see him week after week, and have worked with him for a few months- CHOP saw him for 45 minutes in a setting he isn't used to. Trust us, he is fine. And I trust them wholeheartedly. Mr. Mike and Ms. Melinda are Liam's besties- he may cry half the time they are working him out but he exceeds each week and pushes through the PT. And he always gives kisses, high fives and waves goodbye as we leave happy as can be.

That couple has been through more than I personally know and we definitely thought we were headed in that direction- we haven't had it easy but they have had the worst any parent can have. No parent should bury a child, its not the natural cycle. So as I look at my entertaining, expressive, loud and happy Superboy loving life, I can't help but look up and say thank you. He is on his own pace and things can always be worse. Some people have been saying we are so lucky, we aren't Japan, or another disaster but there are disasters close to home too. When a young couple has to bury their 16 month old because she couldn't just catch her breath, that's a disaster. You don't need to look far in life to see reality- via Facebook statuses, blogs like this, the kid down the street. Be thankful for what we have and reach out to neighbors, family, see how you can be of service to them now. You would be surprised how much the question alone can actually help.

Officially Moved

Pictures: Liam and I at the park on the 70 degree weather two weeks ago and Liam with his friend Isla from our weekly group class. He is a bit of a flirt!

So we moved to Cranford, NJ in early February and closed on our house two weeks ago- its been a hectic few weeks, but we love the house and are having fun making it a home. Liam loves it too, if at all possible, he is a happier more energetic kid! Most of our days are spent playing, unpacking, therapy and just being a family again- its been amazing.

This past Saturday night, Alex and I attended a fundraiser for CHOP's CCU unit. We didn't stay long, it had been a long day already and we had an hour ride to get home, but we ran into someone we knew as we were getting ready to leave. As we walked past a table of other couples, someone grabbed my arm and said, "You're Liams parents right?" I recognized her but forgot her name. She knew Liam's story, who was discharged a little over a year ago. Karen was the PA in charge of Dr. Sprays surgical team. The fact that she remembered us, our son and his specifics, out of his 1200 cases a year means we made quite the impression..oy!

We are seeing Liam making some advances lately, getting a few new words in and pulling to stand on anything, getting into the crawling position quite a bit and trying to figure it out. Our therapists goal is to have him cruising along furniture and taking a few unassisted steps by June, which she thinks he will beat. On the other hand, he also is fearless. This means diving head first off couches, beds, anything to give me a brief heart attack and thankful I played sports as I grab a leg to stop him from crashing. We have gotten into hand stand position several times, which he cracks up in as he looks around. Trouble with a capital T.

While we finally just moved and got our stuff back, I'm also excited for an upcoming 2 week trip to DC. Alex has a class he needs to take and will be gone for 5 weeks so Liam and I will head down for the first few. We don't want to miss too much therapy though and will stay through Alex's birthday in early May then come home.

We are hoping for spring soon so Liam and I can start our garden soon! Best wishes to you and your families!