It's Off to Stanford We Go!

I've been having funny feelings about Grace lately- call me crazy, but we've gone too long without major cardiac concern. I've spoken about these concerns with family, friends, other heart parents, our cardiologist, cardiac nursing friends, etc. I was starting to feel less concerned, but there was something that just didn't sit right.

I'm glad I followed my gut. I decided to seek a 2nd cardiology appointment with another practice. I adore our current cardiologist- he's youthful, optimistic, upbeat, kind, great with words... this was not personal in any way. When it comes to my kids, I just want to be sure I'm doing my best to get them what they need, so off I went.

Grace's most recent ECHO showed that the right side of her heart continued to be enlarged- working harder to compensate for issues. On a scale, she was a 120, with 150 beginning the point of no return (CT surgery imminent). This number doesn't typically change quickly, but it shows that her heart function isn't perfect, and will require intervention in the near future (1-2 years). We knew this, we are okay with this. Grace also has a 12mm conduit (homograft)- this is one of the SMALLEST conduits made. She's had this in place since July 2009.... there are kids that are smaller than her with larger conduits, and they're looking at replacement in the next 2 years. Again, we knew this, we're okay with this, but we don't want to wait until "too late".

We decided months ago that we would be going out of state for Grace's next heart surgery- and Stanford seemed like the best fit. I had been in contact with their surgeon, and we were waiting until Grace was deemed "ready" to be presented (post-cath or MRI here in Phoenix).

Today was our 2nd opinion- a 4 hour appointment! Grace was a champ. We left with MUCH information... where to begin?!

Apparently, we are all born with outlying pulmonary arteries that continue to grow for the first 6 years of our lives. If we reach the 6th year point, essentially we deal with what we have. We can balloon or stent, but no more true "growth". In Grace's case, she has SUPER tiny outlying PA's. September of 2010, Grace had her right side ballooned, and left side stented, because her heart pressures were approaching a bad range- as her heart was pumping against a "traffic jam", per se. The procedure helped. Today, we were told we need to be pro-active and take advantage of the next 3 years in growing larger and more PA's, to continue reducing the overworking of the heart, keeping us in a safer zone, and giving her a better long-term prognosis. Makes sense, right? A cath procedure- minimally invasive with potential long-term benefits? Yes, please.

In Grace's last OHS, the surgeon discovered multiple collateral arteries, or MAPCAS. Because none of them were of substantial size, they were coiled and killed off, instead of harnessing them for greater good in a unifocalization procedure. I was concerned that Grace's body was generating more collateral arteries, and that if we didn't see someone (surgeon) with MAPCAS experience, we were doing a disservice. Fortunately, (GOOD NEWS!) MAPCAS are not a concern at this time-there are no notable collateral arteries. Yippee!

Lastly, and here is where it get's confusing.... we have and have had, a valve issue. Grace's homograft (the 12mm one) is essentially a synthetic pulmonary artery, as she was born without one. The conduit looks decent- minimal narrowing. It could probably go another year or two. Unfortunately, the valve within the PA (typical of TOF kiddos) will begin to leak, wear out and need to be replaced. When TOF kids are born with their PA intact, this can potentially be replaced (after the 1st initial placement done OH) in a cath procedure, using a melody valve.

What does this all mean? Essentially a cath in the next 90 days @ Stanford. The cath will determine what outlying PA's can be ballooned, stented, grown, etc.; and if her conduit is big enough to accept a melody valve. Stanford needs to decide whether they'll: 1. do nothing; 2. stent/balloon/grow; 3. place a melody valve & determine replacement date of conduit; 4. do replacement of valve & conduit, or set a later date to do so.

Basically, we're going to Stanford blind. We could end up being there for a minor cath, or her complete repair (2nd). In a way, this is good for me. I don't have the time to panic about the what-if's.

Faxes have been sent. 16 DVD's are being fed-exed. Stay tuned....

PS- Grace's 1st Make-a-Wish visit is coming! Day after her 3rd birthday!!!

Oh yes, and to cap my great day off? My hubby ran a red light tonight and T-boned someone, totaling his company car. Awesome...

Changes Ahead....

Grace has been looking duskier and duskier as of late. I've tried to chalk it up to mommy worry, but now friends and family have been commenting on it. It was time to make the dreaded call to cardiology. Grace was seen last week by Dr. Shah. He agreed her color was a bit off, but he was most concerned about her behavior. Grace has always been a calm kiddo- but at 2 1/2 years old, she should be FAR more active and energetic than she is. Grace is the type of kiddo that's content to watch Dora (I don't think she can ever get enough of Dora!!), color, lay in bed with mama, or cuddle on the couch. Don't get me wrong, there are times where she's running with her brother- strangling him, hitting him, hugging him, chasing him; the calm times are greater than her energy spurts. Most recently, Dr. Shah thought we'd be able to get to age 5 without surgery, but my gut has been telling me differently- and I think my gut is right, sadly. Mark my words, people-it's the one time in life I would love NOT to be right! :)

Grace's O2 sats hovered around 94 (her norm is 99), and while crying dropped down to the low 80's. Not a HUGE concern, but with the other issues, it's enough of a concern. Her EKHO looked decent. There is definitely regurgitation- but without an MRI or more invasive procedure, it's impossible to know what percentage of regurgitation there is. The thought is, she's not as active or as pink as usual because the regurgitation may be at a more severe range than expected. I'm not sure that her pressures have stayed down either, but time will tell. Dr. Shah ordered an MRI with anesthesia. He had hoped to get it done within the month, but with the merger of the two hospitals, scheduling may not have been ideal.

Thankfully, we were able to get in pretty quickly. Grace will have her MRI on June 22nd at 10:30 am. We'll check in at 9, with nothing to eat or drink beyond 4 am. If there is something that can be potentially fixed in the Cath lab, they'll potentially take her there. Unfortunately, it doesn't appear to be a Cath fix.

The day of cardiology, we started talking surgery. I wasn't ready for it, but knowing my gut, I've kind of known all along- my gut tells me we'll be doing CT surgery within the next 6 months. After talking with the cardiac team, my family, and my God, we've decided to have surgery out of state. Dr. Shah supported our decision, and recommended that we make our hospital decision now- versus waiting until an emergent situation. He suggested Baylor, Stanford, Boston and CHOP as options, among others. Financially and emotionally, Stanford makes the most sense. We are originally from the Bay Area, and still have many friends and family that reside in that general outlying area. I'll be scared to be away from home- but comforted by the familiarity during the uncertain time.

To complicate my worries, we saw GI yesterday with mixed results. The GI asked if we had seen cardiology, as they're noticing a change in her color. UGH! Seriously?!? I explained that yes, we had, and gave them cardiology's plan. GI is a bit concerned that it may be time for cardiac surgery as well, as for the first time in over a year, Grace lost weight, despite the extra feeding therapy sessions. The thought is, if there is less positive blood flow (regurgitation), her gut would be the last organ to receive blood support- thus making it difficult for her. She hasn't been vomiting much, but it's increased above her normal range. Maybe it's because of this? Maybe not... Either way, we're bumping up her caloric intake primarily through tube feedings to give her a better buffer for weight loss due to either CT surgery, or admission to an inpatient feeding program (more to follow on that). Also, because of her daily aspirin dosage, the concern is that there is damage being done to her gut, that would only get worse during CT surgery. Grace will now be taking Omeperazole to soothe her gut lining. Not sure we'll see the outward benefits of this, but hopefully we'll avoid any internal gut bleeds post-op.

Prior to all this cardiac craziness, we had been admitted into Phoenix Children's outpatient intensive feeding program (we had been on a waitlist since she was 3 months!!). We were super hopeful about this being the answer to Grace's behavioral issues when eating. It was helpful, but not nearly enough. GI recommended looking into a behavioral inpatient program. Unfortunately, there is no such program in the state of Arizona. We discussed both Milwaukee Children's and John Hopkins (Kennedy Krieger). The cost of airfare is bound to kill us, but we'll make it work, even if I have to max-out credit cards. After communication with both programs, Kennedy Krieger seems to be the better option. Prior to the cardiac appointment, we scheduled an eval appointment for July 14th.

Fast-forward to now- WOW! So, there are a few ways this can go... 1. Grace will have her MRI and need surgery right away & we'll be sent to Stanford. 2. Grace will have her MRI and need surgery, but may be able to wait a few months. 3. Grace will have her MRI and not need surgery for a year. The big question for our family is- do we risk paying tons of money in airfare to get the eval @ Baltimore, only to have to postpone treatment for surgery, or worse, she succeeds treatment, then has surgery, and loses her skill. UGH! What to do, what to do?!? At this point we've decided to wait it out. We should have some cardiac answers at the end of this month, and we'll make the rest of the tough decisions at that point.

Gavin is doing AMAZING!!! He's been taking his budesonide slurry (mixed with 4 splendas)- he drinks 1/2 in the morning, and 1/2 in the evening- and it's been working!! His latest biopsy shows partial remission. He is clear in his upper esophagus and middle esophagus, but his lower esophagus still has numbers in the 60's. Our GI thinks that by adding Omeperazole we'll be able to combat any reflux that's occuring, and achieve full remission. He'll have another biopsy in 2-3 months, and we're hoping it will be our last for some time. Praying for remission!!

On a brighter note, he's eating EVERYTHING! Falafel, Pad-Thai, milk, applesauce, etc. You name it, he eats it. He gained an average of 12 grams a day- we were shooting for a goal of 8! That's my little overachieving man! He's still having his sensory melt-downs, but we're coming up with creative methods to combat the screamies. Our swim lessons with him are GOLD- he's a true fish! He's already swimming underwater and able to grab the wall, and climb out- all by himself!!

I feel bad for Gav during this time of chaos with his sister- I don't want him to feel my stress and tension, nor do I want him to feel less important. It's a trying time- and I'm finding myself unable to sleep at night, but needing a nap by 10am. I'm hopeful that I'm just in a funk and that I'll work my way through it.

We'll soon have TONS of family around- Felix's parents are moving here this week, and his sister her husband and daughter will be here next month. His brother and wife will be here this winter. I'm stoked to have the kid's relatives around and to have more family time! My mom has lived here for a while now, also Mimi (my other mother). My dad and family live in Wisconsin, but we'll be able to visit more often now that we have less places across the US to vacation.

I think that's it for now. I'm long winded... and tired, but grateful for my babies and content to start over again tomorrow.

As Grace now says, "I You Mama" for I love you- that is ALL I need to get by! XOXO

Tired of Playing Catch-Up... the Title of My New Book: Procrastination

I am really bad at this. No really, I mean it. I'm REALLY bad at updating this. So many nights I spend thinking about what I could be updating and making sure I remember, or thinking about how I really should be posting about where we're at medically, but time manages to escape me. It's official- I am a procrastinator.

The kids are well- healthy and continuing therapies as normal. I get scared during these "coasting" times- I'm always anxious about when it will be "time" again for Grace's next OHS. Every day I scrutinize her lip color, nail color, etc. I hate to say it, but going to cardiology once every 6 months is far more anxiety inducing than every other month. Sad, but true.

Both kids are walking and running EVERYWHERE! They're literally into everything. Grace is talking up a storm, Gavin's speaking in spurts- it's going WAY too fast!

If nothing else, this post can be a journal entry for me to remember their "language".

We say: Grace says:

YESSSSS! Ssssaaawwwhhhh!

I'm ready I'm wettty

I'm hungry I num-num (while touching her nose) or I knee

Brother Boo-Boo

I love you TU-TU

I love you too TU

Bubbles Pop-Pop

I'm two I Two!

Private Parts Butt

Ketchup/Ranch I want Dip-Dip

She's adorable! I love her mmmmm's the most after she eats something really yummy. I also really like her aaaaah! after she drinks something refreshing.

I recently taught her the Gaga paw... Bad Romance came on in the car, and I thought it only appropriate to teach her how to "put her paws up" for Lady Gaga. She's really got it down!

Gavin is saying Bottle (BaBa), Water (WaWa), and Mama and Dada.

More updates to follow.... sometime this year (maybe).

My Life with Gavin....

I want to explain to you why my child is
having such a rough time today…
What you are seeing when you look at my
child is not a child who is "out of control" but
rather a child who is "out of sync." My child
has Sensory Processing Disorder (SPD),
which is the inability of the brain to properly
and/or efficiently process and organize
sensory information. When my child's brain
receives information through any of his
senses - sight, smell, hearing, taste, touch,
vestibular or proprioception -- he doesn't
always know what to do with that information
and can become very disorganized,
overwhelmed, frightened and confused. You
can read more about SPD at
www.spdnetwork.org.
My child has good days and bad days. You
must have run into us on a bad day. I
apologize if we have made you uncomfortable
or if we have been disruptive. My child is
currently involved in many types of
interventions, working on these sensory
issues, so that he can feel normal like you
and so that when we go places, he feels safe
and comfortable in his own body. It is going
to be a long and difficult journey, but in the
meantime, please know that inside him is a
beautiful and magnificent little person
waiting, hoping and trying to emerge!
Think about how difficult my child's SPD was
for you today, then try to imagine how hard it is for him.
The next time you see us
out somewhere, please understand...

Inspired!

Such a great evening tonight! After much debate, I managed to get out of the house and attend the Eller Family Heart Council meeting. What a group!! The families were all so friendly, so supportive, and so energized by one another. I left feeling part of a group, finally! Super excited to get more involved.

Today started with Gav's biopsy. Good news, so far. His furrowing (imagine tunnels running down his esophagus) has decreased, but the biopsy will tell us the real results. In a week or so, we'll know if he's in full "remission". If he's in remission, we can safely
assume his allergies are all food-triggered, and we can start introducing 1 new food at a time, with a biopsy between each addition. Sounds long and painful, and it will be, but at least our little big man can start eating something other than Elecare (*hopefully*)! Speaking of my little big man, he cracked me up yesterday trying on mommy's glasses! Check out these silly pictures!

Grace is doing well, she's a full-fledged toddler FOR SURE! We have days where she loves on her brother, giving him continual hugs and grabbing his bottle when he's upset, to days where she's running him over with her Little Tikes buggy, his motorized quad, or hitting him and pushing him away from their toys. She often throws food, but this week we've managed to get more into her belly than on the floor. We're hoping for a BIG growth spurt by the next GI appointment. She's still hovering around the 21 pound mark. We're slowly introducing the potty, although we've yet to have success. Grace likes to sit on her potty, pants around her ankles. Then she stands, wipes, throws toilet paper in the bowl, and waves goodbye to it. CRACKS me up! Hopefully someday soon, we'll have some actual results- no rush, though. I'm on the fence on transitioning her out of her crib. She seems okay with it, still, but she likes to sleep in our bed for naps, or even for fun. She's started jumping up and down in her crib, and I'm starting to worry about injuries. We'll see... anyone out there have any reccomendations?

We got GREAT news at our last cardiac clinic visit. Grace's pressures have improved so significantly they are not even viewable on the ECHO any longer! Woo hoo!! Dr. Shah estimates them to be in the teens/twenties. Considering we were in the 50's/60's pre-cath, I'm stoked! No more viagra for my girl! If we continue on the path we've been going, Grace may be able to hold off on her next surgery until closer to age 5. Let me say that again, because I'm still in shock over it, age 5!!! AGE 5!!! That is AMAZING! I won't be shocked if we don't get there, but at least we've made it a year and a half with no major "zipper" surgeries. ;)

Grace and Gavin's birthday party was a success. Since Grace was sick during her "scheduled party" we decided to do a combo party. Such a fun day! Here's the pics!

We also fed the ducks this past week- Grace said "duck" for the 1st time. Such an experience!! Too bad her favorite word this week is "butt".... :( Oh well, we can't pick our battles. LOL!