Blech... Insurance Drama

First and foremost, Grace is doing well- not really any changes, but doing well. We're doing our best to stay quiet and keep her still, rested, and sleeping. Sleeping=healing. When Grace does stir (wake-up really isn't the proper term), she's pretty pissed about it and goes straight to pulling at her vent, her art lines, her IV, or whatever mischief she can get into while amped up on drugs. During rounds this morning, docs think her agitation is due to being vented, and having chest tubes, not pain, so we're weaning her meds. Dopamine is gone, Milrinone is weaning. TPN still running, but also getting a slow trickle of Elecare to keep her gut active. Still no signs of Chylo, but since she's literally being fed at a rate of 2 oz per DAY (yes, you read that right), it will take FOREVER to see chylo... Or, maybe another day or two. :) If you're a G Tube Heart mommy, you'll get my humor.

Vent settings are sticking with the APRV bizarre breath pattern- she's at a PHigh of 18, and to wean to traditional vent settings, PHigh ideally will be at 15 or 16. We'll get there. Slow and steady wins the race. As long as Ms. Sassy is vented, the sedation must continue.

We've officially been in California for 11 days now. We've seen the gamut of weather- sunny, windy, rainy, cold. I feel like I can drive the distance between Milbrae and Palo Alto in my sleep. I officially know my way around Millbrae, almost as well as I do around Surprise. We finally got a room at the Ronald McDonald House on this past Friday, but the bed is HORRIBLE. Literally, if one person rolls over, the other person flies off the bed (think camping air mattress), so we've been sleeping in a sleep room (twin bed) at the hospital, or going back to Millbrae. All of our food expenses and rental car expenses have been coming out of pocket and it's adding up FAST. With no return home in sight, I'm getting a bit weary. Not to mention that I miss my son like crazy! Gavin apparently cries out for us in the middle of the night, and is now at the point where he won't look at our picture. :( My heart aches for him- I know he has NO CLUE what is happening, and I wish I could make it better. Being a mom, my role is really up in the air right now. I'm feeling more out of sorts than ever before. Instead of being in charge of my daughter and son, right now I'm in charge of nobody, and have no control over either kid's environment. Talk about feeling helpless...

Insurance drama today doesn't help with things. 2 months ago I began calling both insurance companies (our private, and state MedicAid) to give them a heads-up of what was coming and to iron out any issues before our trip. Both stated that we were green-lighted, and nothing to worry about. NOT TRUE.... I received a call today from the MedicAid benefits coordinator. Nobody advised them from either Dr.'s clinic (Phoenix or Stanford) of the surgery. Since nobody there was notified, they didn't offer assistance. Apparently, in a perfect world, we would have had our airfare and lodging covered, and possibly food assistance. REALLY?!? I've been here 11 days and I'm just hearing about this now?!?! The lady was super nice and apologetic. Since there was no notification, payments weren't pre-authorized, but she told me not to worry about it, it'll be handled behind the scenes. I can't even imagine the bills we would have received.... OUCH! She's working her hardest to get us moved to the Stanford Guest House (on their tab) for the remainder of our stay. If we can move there, we can hand in our rental car (more savings!), and take the shuttle provided. She'll call me in a few hours with news, but I'm pretty hopeful for now. It would be AMAZING to be in a comfy environment 2 miles away from Grace. I'm not used to being away, considering in Phoenix we stay physically in the ICU. Here, we're lucky to have a seat! It's very different... but different, not bad.

Hopefully we'll get some help for meals- it's stupid expensive to eat here. $14 for lunch today! That's a chicken sandwich and 2 chicken enchiladas... I'd much rather do Chipotle for less!

Alright, I'm done venting. Off to hold Gracie's hand... Thanks for prayers, all!

The Long & Winding Road... to Recovery

I sit writing this by Grace's bedside, listening to the continual rhythmic beeping of her monitors. For the first time, post-operatively, I'm filled with peace.

Grace's surgery was a success! Dr. Hanley is truly an AMAZING man- he is humble, humorous, honest and just amazing. Grace's surgery started at about 7:30am Thursday morning, with her first dose of Versed. She left our sight laughing, smiling, and sticking her tongue out- that is the BEST way to say a goodbye to a kiddo going off to the surgical unit. I actually laughed, not cried! Well, okay... I cried when she left my sight, but I was super strong mama kissing her farewell. ;)

Dissection lasted a LONG time. Dr. Hanley met with us around 10 am, and he hadn't even headed into the OR suite at that point, letting the anesthesia team do their thing, and the fellows do the dissecting. Dr. Hanley estimated at that point, before going in, that he wouldn't be able to restore flow to the upper left lobe, but would reconstruct the upper right PA's leading to the upper right lobe, he'd attempt a 20mm conduit, and that the whole surgical portion would last until about 2 pm.

Being antsy, my hubby suggested we take a walk in the fresh air and sunshine- I was hesitant, but I'm super glad I listened and went along. Stanford University is BEAUTIFUL! We went to the Hoover Tower and took in the beauty and silence- amazing! After, we walked to the Memorial Church. We were lucky enough to hear the organ play (I hear this is a VERY unusual occurence, and only happens during church services). The organ boomed out a jubiliant tune, and literally seconds later, we got a call that she was doing amazing, and things were going better than expected. I truly felt peace- what a spiritual moment!

Dr. Hanley met with us around 4pm- he was able to get a 22mm conduit in place, and restore flow to BOTH sides (remarkable!!). Dr. Hanley sees no reason why this size conduit shouldn't last a good 10 years, and now she'd be a candidate for the Melody Valve procedure if/when the valve becomes faulty. The right side of her heart will slowly reduce in size, now that the pressures are down. There was a fair bit more work on her lungs than expected, so she's been recovering from a re-perfusion injury.

Originally we were told that we'd be out of ICU in about 24-48 hours post-op. Unfortunately, due to Grace's history of sick lungs, added to the complexity of the surgery repair to her PA's, we're far OFF that estimate. Grace will likely be intubated (sedated) throughout this week, then hopefully to a high flow nasal canula. The floor won't take her unless she is on 3 liters of O2 or less- so that could potentially be a few weeks off. It's easy to become overwhelmed under these circumstances- Grace is completely nonresponsive, and we literally are just sitting, watching her breathe for 12 hours/day, only to go to bed, exhausted, at night.

I'm coming to terms with the longer hospital stay, but I'm human, and had my breakdown. Today, I'm filled with more peace- I can see small changes, they're giving me hope and I'm seeing the light at the end of the tunnel, granted a LONG tunnel, but we'll get there. Thankful for the prayers, the blessing of friends, and the gift of family!

We made it to Stanford!! We also went a on Grace's wish trip, but since I'm typing this from out of state, I'll post wish trip details when we get home.

Yesterday Grace had a heart cath with Dr. Perry. The cath lasted about 3 hours. Here's the info for surgery that will take place Thursday with Dr. Frank Hanley.

Dissection is planned to take 2-4 hours. They'll dissect a bit more than originally planned to be able to get to her upper lobe and outlying PA's. Conduit change as planned, hopefully 15mm or bigger. She has a 12mm now. 14 or bigger makes her qualify for the melody valve in the future if her valve fails but conduit still open.

Cath showed that her upper left lung lobe has no blood low. Lung perfusion test completed to see more in depth. Still no results. Unsure if upper lobe of left lung hasn't had flow long-term, or if this is a recent issue. The degree to which the lobe and arteries are damaged will give them an idea of timeline.. If Dr. Hanley is able to find a viable artery it's possible he can rehab and restore. Unfortunately, upper lobe on right has severe stenosis and is headed to the same plight. Thankfully, Hanley can rehab and fix, and also ensure no future lobe flow loss since he is somewhat of a pioneer in this situation.

The PA's are quite close to the Frenetic nerve. If the frenetic nerve is messed with, it causes a diaphragm issue. We won't know if there is an issue until extubation, and will be apparent if she struggles with breathing. Apparently, the frenetic nerve controls the up and down mechanism of the diaphragm, so the issue is that the diaphragm could be "stuck" in the up position, requiring another small surgery to stitch the diaphragm down.

Each PA repair takes about an hour and the conduit change also takes about an hour. Actual operation time will be 6-8 hours, but we won't see her for probably 10ish. Bypass time is expected to last 3 hours, and goal is to keep heart beating, since the VSD fenestration healed itself.

There is a concern about chylothorax since she's had it twice before. If it happens, well wait until she dries up. I asked about the JP drain that PCH sent us home with in the past only to receive stares. Their response? It's not in our medical practice safety guidelines to do that.

Also asked why we didn't know about the lobe flow issue before. Their politically correct reply? PCH probably only sees surgeries with her complexities maybe a few times a year, Hanley sees this several times a week. Its his life's specialty- he knows EXACTLY what to look for. I feel so comfortable with my decision to come here. Only upset I didn't know before birth so we could have avoided some of the complications that we've seen since operating at Phoenix Children's.

Grace is now running a low grade temp, I'm assuming because of anesthesia? Also now super junkie because of being intubated. Worried that surgery could be affected... Pray for health.

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...