Friday, July 20, 2012

And in an instant...everything changes

Dusk's mom died in a car accident when he was 9 years old. My mother has faced cancer 3 different times. We've had 5 miscarriages and a Down Syndrome Diagnosis. And then there was Bodie.

You'd think we'd be used to it by now. Used to a stranger, walking in and uttering a sentence that alters the course of your life. You'd think we'd never get too settled by this point, knowing our seemingly even-keeled existence could change at any point. But, no. It catches you off guard every.single.time. The world shifts ever so slightly, and the way you thought things were...just isn't.

Dusk has been battling hip pain for months now, ever since his pacemaker/ICD implant in February. Some thought it might be related to the ICD, that his chest cavity (which also still hurts) was too tight and pulling his hip out of joint. So his doctor did an x-ray 3 months ago (all looked fine) and referred him to an Orthopedist. It took us 2 months to see him. He decided that we should start with Physical Therapy and referred us out. Dusk saw a Physical Therapist for a month, doing all of the stretches they recommended...and the pain got worse. It has become excruciating. There are days it hurts just getting out of bed. He called his Orthopedist and (only after threatening to leave the medical group) were they able to move schedules around to get him in before 2013 (I only exaggerate slightly). He had scans done last Friday and met with his doctor Monday morning.

Left Hip Necrosis. Recommended total hip transplant.

At 39 years old.

Yes, we're reeling. Not what we expected to hear. Of course we're grateful for a diagnosis and not to just keep getting passed between doctors. And we're grateful it's something that can be "fixed". But we're worried. We're worried because his hip looked fine 3 months ago and now has massive necrosis. Even the doctor was surprised at the rapid change. We're worried because his other hip has started hurting in the same way. Monday, the right hip looked fine. But so did his left hip 3 months ago. We're worried because his medical team at UCLA doesn't communicate with one another, and he gets passed from doctor to doctor without any real cohesiveness or a holistic approach to his health. We're worried about what a hip replacement entails, the surgery, the recovery. More than that, we're worried that something else far more serious is going on, and the doctors are going to let it progress while they "sit and wait."

So that is the plan. We sit and wait and watch it for the next 3 weeks and then just "see what happens". Sometimes, very very rarely, it can get better. But it's unlikely. Necrosis like this is typically seen from either excessive alcohol use or significant steroid use, or occasionally by a fracture that doesn't heal properly. Now, for those of you who know Dusk, he does brew his own (mighty fine, by all accounts) beer and drinks his fair share. But he was very open with his doctor about exactly how much he drinks and his doctor was equally open that his alcohol use is nowhere near the levels required to produce this kind of result, that "excessive" alcohol use is typically referring to the levels seen in an alcoholic, which Dusk most certainly is not.

His doctor is assuming his necrosis is cause by steroid use. Last October, Dusk was diagnosed with Meniere's Disease, a rare inner ear imbalance that can be treated either by steroids or dieuretics. The ENT he was seeing at UCLA wanted to try steroids first, believing Dusk would be more responsive to it. He was responsive, but after 2 weeks of Prednisone, they had to stop it because it caused such massive hip pain. The general consensus was that there was no way it could cause hip necrosis that quickly, but that clearly he was very sensitive to it, so it wasn't a good idea. He obtained a second opinion from the House Ear Clinic, they recommended diuretics, which he started. Within weeks, the disease was under control and has been since. In any case, it seems to be his opinion that the steroids are currently the most likely suspect for what we're now seeing with Dusk's hip.

The final theory, a fracture that didn't heal properly, might also be coming into play. Apparently, there was a fracture in the hip. Although Dusk doesn't remember fracturing his hip, he does recall a particularly painful day golfing that might have been when it occurred. As an aside, anyone who has watched Dusk golf would totally believe he needs a hip replacement because of his golf swing!

The orthopedist (who was not his regular doc - he was on vacation) was very short and to the point - next step is hip replacement. We're not entirely comfortable with this as it happened very quickly, and there are other options which he didn't even discuss with Dusk. So we are in the process of obtaining a second consult from within the UCLA Medical Group (which Dusk is a part of) as well as a second opinion from outside the medical group. Additionally, due to how quickly this progressed and all of the open questions and concerns, he'll be seeing his regular Orthopedist, who is now back from vacation, Monday morning.

So, as we face yet another challenge, we're asking for prayers for our family. Prayers for Dusk's body, that it would miraculously heal. Prayers that a second opinion would guide us to a doctor that might have a better solution. Prayers for a more holistic approach to Dusk's care, that we might find that at UCLA (or, that somehow another medical group might take him - UCLA will release him, but Cedars (where we hoped to go) won't take him mid-course of treatment; we'll have to wait until the next plan year, which begins in January). Prayers that he would stop being pulled from one doctor to another, waiting months between appointments. Prayers for significantly diminished pain for him. Prayers that this is not simply a sign of something much more serious underneath. Please blanket our family in prayers.

And, if you have a recommendation for an Orthopedist within the UCLA group that you like, who is open to more holistic treatments, please send their information our way. Also anyone outside of UCLA who you might recommend for a second opinion. Thank you so much!

Saturday, July 14, 2012


When Bodie first came home from the hospital, he came home on 6 different medications. By the time he came home from his unplanned pacemaker surgery 4 weeks later, the med count had increased to 8 (Aspirin, Digoxin, Enalapril, Hydrocodone, Lasix, Propranolol, Reglan and Sildenafil), given multiple times a day, for a total of 17 syringes. Because of his paralyzed vocal cord, every single one of those syringes had to be thickened. That means I had to draw up each dose, shoot it into a little medicine cup, mix it with a little bit of thickener and then draw the mixture back up into the syringe. It was one of the many reasons interstage was so overwhelming for me.

I'm going to let you in on a little secret - Bodie is just a touch impatient. As in "I am totally not willing to wait for you to heat that bottle up, so just give it to me cold" impatient. And "I'm going to scream so loudly and persistently that you won't be able to concentrate on a single other thing until you give me what I want" impatient. So, faced with a kiddo who was so demanding and medicines and bottles that had to be very precisely drawn up and calculated, I came up with a system very quickly.

Every night, once the kids were in bed and the house was quiet, I would pump and then prep the next 24 hours worth of bottles and meds. I would put pumped breastmilk, the requisite amount of formula (to fortify for extra calories) and the requisite amount of thickener (all determined by a pretty complex formula calculated by someone who understands this stuff waaaaay better than me!) into the blender and let it go. When it was fully mixed, I'd pour it into a large lidded jar in the fridge and then bottle off whatever I needed throughout the day. That way, when Bodie was hungry, it would literally take me 5 seconds to go to the fridge, pour a bottle and feed him. Saved me MUCH frustration and listening to him scream.

I took the same approach with his meds. I would take all 8 bottles of meds and 17 syringes, a medicine cup and a few packets of thickener. I'd sit at our little round table in front of the tv, catching up on my shows and mixing meds. Once they were prepped, the syringes were labeled and put in either a divider in the fridge or on the counter (depending on whether they were room temperature or fridge meds). The entire process took about 30 minutes a night. Then, the next day, when it was time to give meds, it was as simple as grabbing a prepped syringe and popping it in his mouth. It was super easy to look and see whether a particular med had been given (you'd be amazed how hard it is to remember anything at that stage in the game) and I wasn't having to worry about human error caused by prepping meds with 2 kids needing your attention at the same time.

Over the past 2 years, we've slowly worked our way down on those meds, both on the frequency and the dosages. It got a little easier as we slowly dropped meds off the schedule entirely. It got a lot easier once we were able to lose the thickener. By the time we got to this summer, we were down to meds only 3x a day -

1/2 Baby Aspirin + 1 syringe (Enalapril/Sildenafil/Propranalol mixed together)
After nap: 
1 syringe (Sildenafil/Propranalol)
Late night:
 1 syringe (Enalapril/Sildenafil/Propranolol with a 1/2 a crushed Amiodarone tablet mixed in)  

Although the med count is much lower and I could probably prep and dispense meds on the fly, I never got out of the habit of prepping them at night. I still prep them once the kids are down - it takes me a few minutes now, but it's still an ingrained part of my evening routine. And then I always give Bodie his last syringe right before I go to bed. I give it as a "dream feed" - without even waking up, he sucks it right down. Most nights, I can also sneak a toothbrush in to try and get the sugary meds off of his teeth!

I'll be honest, this is still a hefty load of meds for your typical hypoplast - most that I know are only on Enalapril and aspirin at this stage in the game, and maybe a reflux med. But we all know Bodie likes to be challenging. :-)

So why I am I giving you this really detailed history? Because today was a BIG BIG day for us. And you can't understand how big until you see how far we've come.

As I mentioned earlier this week, we lost the Sildenafil (which he seems to have done great with, thankfully). Today, we took the next step - we switched him from Propranolol to Atenolol for his fast heart rate. This is big for 2 reasons - the first is that Propranolol was 3x a day, but Atenelol is only 2x a day. This means that all of his meds are now 2x a day or less. The second is that Atenelol isn't available in a liquid form, so we had to get it in tablet form. I had anticipated that I'd have to crush it and mix it with his Enalapril, his last remaining liquid med at this point.

But, no. I figured I'd let him try and take the tablets as-is. He tried and told me it tasted "yucky." I told him to drink his milk afterwards, that it would take the taste away. So he did. And did it again at dinner like he'd been doing it forever. Bodie, our little amazing 29 month old fighter, is now taking tablet meds no problem whatsoever. I am floored. These aren't even "chewable" - they're small, but they're adult tablets, designed to be swallowed. I haven't tried them, but c'mon, they can't taste good. But he took them! This kid is so amazing.

So, our med schedule now?

1/2 tablet baby aspirin + 1ml syringe (little tiny amount) of Enalapril + 1/2 tablet Atenelol.
 1ml syringe of Enalapril + 1/2 tablet Amiodarone + 1/2 tablet Atenelol.

And that's it! 4 meds. 2 syringes. 4 little half tablets. That's it. Seriously, people. This is HUGE. He takes it all (including his syringe meds) by himself. I don't have to prep meds anymore!!! (I think I can handle drawing up 1ml of Enalapril with him stealing my attention - and it sounds like we'll be switching him to tablet form of that before too long as well.) Seriously - no med preparation at 10pm at night! And no remembering to give him his meds before I go to bed at night! Can you say FREEDOM?!? Seriously. First time in almost 2 1/2 years that I'm not prepping meds - or administering them before I go to bed. Whatever will I do with my time now?!? Maybe I'll actually let this little guy sleep at night now. :-)

So proud of him...and of us...for making it to this point. (Yep, we know this will all change come-Fontan, so for now, we're going to enjoy this little bit of normalcy!)


Monday, July 9, 2012

The Breakup

Yesterday, Bodie ended a long (2 year +) and tumultuous (on again, off again, back on again and desperately needed) relationship with his beloved Sildenafil, the pulmonary hypertension drug better known as Viagra. 
Bodie giving himself his last dose.

Mommy is thrilled (no more battles with the insurance company - you'd be amazed at how hard it is to get something approved for your 2-year old son to be able to breathe properly, considering most of the male population over the age of 60 has no issues obtaining it).

The cardiologist is happy (no sense in keeping him on a med he doesn't need anymore).

Let's hope Bodie's body is as happy as the rest of us.

Big day for our little man. 

Sunday, July 8, 2012

There is HOPE...part 2

If my last post was directed at parents finding themselves trying to vacation for the first time with multiple kiddos, this post is directed at parents finding themselves facing a new diagnosis of HLHS. When we received our diagnosis almost 2 years ago (wow, can't believe it's been that long), Dusk and I grieved for the loss of the life we had envisioned for ourselves, for our children. It's just kind of what every parent goes through as you face a diagnosis of a special needs or medically fragile child. I think every parent has something they hold onto, that kind of represents their "before" life, the life they lived before they received the diagnosis. For Dusk and I, we held onto Tahoe, the place where Dusk had vacationed as a child and we had taken Sierra as a toddler. When we received our diagnosis, we figured our dreams of family vacationing in Tahoe were just a part of our "before" life. Like everything else, we would adjust. But that didn't mean we weren't sad about it. We knew the likelihood of taking a child with a half a working heart to a high elevation was probably pretty slim. But we still held hope - an outside hope that maybe our unborn child would fight this HLHS thing with all he or she had, and that maybe, just maybe, we would make it up to Tahoe someday.

So, when we made it up to Tahoe last year, with oxygen tanks, a pulse ox machine, and nerves all in tow, it was amazing and incredible, a true dream coming true. We tried to take advantage of every moment (even the trying ones - which, as it turned out, were most of them), knowing that there was a good chance we wouldn't make it back up this summer, being that we'd either be too close before Bodie's Fontan that his sats wouldn't be high enough, or we'd be too close post-op to push him at elevation. So what an absolutely mindblowing experience it was for us to be back up at Tahoe again this summer, having even more fun than we did last summer. What a ride it was. And perhaps made even more amazing just in knowing what a miracle it was to have gotten there at all, and in knowing each year we spend there might be our last. So we make every moment count.

So, to newly diagnosed parents, I offer you HOPE. Every child's journey is different and the fact that we've made it to Tahoe 2 years in a row doesn't mean every child will. But hold onto whatever dream you have for your child and your family. Because you just never know what God has in store for these special kids we're privileged to parent. No matter how long we get to parent them for, they're special and they're incredible gifts.

What I can say unequivocally is that, the day we received our initial diagnosis, or the better part of 5 months we spent by Bodie's bedside in the hospital, worrying whether he would make it another day, we never in our wildest dreams imagined we'd be up at Lake Tahoe 2 years later.

And yet, there we were, seeing a lot of dreams come true...

We played in a park at 6,000ft

We went on a hike at 8,500ft (this was SUCH a dream of ours, I can't even tell you)
...and apparently, my kids interpreted "Say cheese" as "strike the most ridiculous pose you can"

We took the kids to Circus Circus, where apparently they weren't as bothered as we were by the fact that the "acrobats" were a couple of way-too-old-to-be-in-spandex jugglers...

We got to spend SO MUCH time with grandparents who live 8+ hours away. The first 2 times they met Bodie, he was inpatient, literally fighting for his life. The third time, he was recovering from his 3rd major open-heart surgery. Being able to spend time with them outside of any hospital setting is NOT something we take lightly. 

We got so dirty we got to take a bath in a bucket in Popo's garage.

We got to go out with Gram Jan and Popo Alan to eat at my favorite - a casino buffet (hey, we all have things weren't not proud of - my love affair with casino buffets happens to be one of mine...)
(yep, Bodie definitely was blue at times during this trip - but in all fairness, he had just eaten ice cream)...

And finally, we got to take pic of each of their kids with their namesakes...
(Ok, we didn't really name Sierra after the road the Circus Circus was built on, but it was a cool pic)...

But we did really name Bodie after the town...
And I LOVE how they're both pointing at the sign...

So, to families facing a new HLHS diagnosis, what I can say to you is yes, grieve you must, because your life will be very different than you imagined. But, when you're done grieving, take heart, and have HOPE. Dream big. Because our amazing kids deserve nothing less than our greatest dreams for them.

Thursday, July 5, 2012

There is HOPE

Last year, we embarked on our first family vacation to Tahoe with both kids. It was fun, yes. But it was also extremely stressful. It involved 3 of the 4 of us being hit with the stomach flu, a whole lot of crying and whining (from everyone, but I'll be honest - Bodie started it) and a lot of stress. My exact description of it (from this blogpost, if you need a laugh) was "I don't know if parents of more than 1 child have been lying to us this whole time or if we just have bad luck, because this seriously has been the worst.vacation. ever. We're trying to find the humor in it (and remember that someday, we will look back and laugh at this vacation), but it's been tough."

Well, I am here to tell you parents of multiple little's that it does indeed get better. Because we're a little over halfway through this year's Tahoe trip and it has ROCKED. I mean, it has been! A few highlights of the trip (or, I don't know, "a few thousand pictures" - sorry, couldn't decide - that's how much fun we've had!!)

We got ready for the 4th in our best patriotic gear...

We watched lizards run around, in complete awe...

 We colored with Popo...a lot...

We ate at our favorite Mexican restaurant (our first night in Tahoe family tradition!)...
 ...where we spent most of dinner giving mommy "the look"...
 We hiked...
We dipped our feet in our favorite creek...
 ...and goofed off with mommy...
 ...and hiked with Gram Jan and Popo Alan
 ...We found a tennis racket and practiced playing...
...and then hit mom in the temple with it (at full force) - no pic of it since mom was running around crying in pain (funny story at least, right?!? Right???)

 We spent a lot of time at the beach...
 ...and shamelessly plugged Sisters by Heart...
 We played on the playground...
 ...and had a BBQ...
 We ate at our most favorite ice cream place on the planet!

On the 3rd, Gram Jan (as Bodie can coined her) came and picked the kiddos up and brought them back to spend the night with her and Popo Alan, while Daddy and I stayed up in Tahoe by ourselves. It was the first time we've ever left the kids overnight - so we were pretty nervous, but they did great! They had so much fun! And so did we - we got to go to a non kid-friendly restaurant and just relax! So nice to know the kids can make it without us for a night!  A huge thank-you to Gram Jan and Popo Alan!

Then, we joined up with them on the 4th to visit the Carson City Railroad museum - and we got to ride on real steam trains (one of which was brought out only for July 4th!). The kids had a blast...

And Sierra even got to go up to the front with Daddy and Popo to ride (no dice on allowing Bodie up there - something about him having to able to sit still. Yeah, right).

As you can see, this has been a stellar vacation - and we're not even done yet! So, all you parents struggling with that first family vacay, where kids are screaming and it's more of a "change in location" than a true vacation - take hope. Because it gets so.much.better!!!