Fontan Talk

The meeting with Dr. Ilbawi went really well.  We got to see a lot of familiar faces from the staff and remind them that'd we would be seeing them in one short month.  He drew Olivia's current heart anatomy even including the the placement of the stent from the recent cath and then drew in the details that would be revised during the Fontan.  Mind you, he drew the entire thing anatomically correct upside down so we could review it as he drew - if that's not impressive I don't know what is!?!

A few things that were new to us:

• The Gortex conduit that they use to hook the Inferior Vena Cava (IVC) to the Pulmonary arteries is anywhere from 3/4"-1" in diameter.  Holy smokes, that's huge!  But their thought process behind it is make it as big as possible so that they wouldn't have to go in and do a revision later when Olivia's anatomy has grown larger
• Fenestration vs Non-Fenestration: this is something that will be decided at the end of the surgery depending on pressure readings throughout; if they are reading higher than they'd like in the new conduit and pressing on the right exterior portion of the heart, they will create a little hole to mix the blue blood with the red blood when the pressure gets too high: think little openings on the top of pot lids that allow for some steam to escape; this could naturally close on it's own if it's needed or it would get closed during a cath
• Pacemaker placement: about 50% of HLHS kids (figures are debatable) need a pacemaker sometime in their lives to help regulate their heartbeat - but because of their new re-plumbed anatomy, they cannot make that traditional cut below the collarbone to put the pacer in. Instead Olivia would need an incision made at the base of her sternum to put the pacer in and then the leads would be positioned directly on the heart instead of the vessels.  She doesn't currently need a pacemaker, but if she did, this is how it would go

General info:

• Post-op Medicine regimen: Lasix (dieuretics) to help her shed excess water will be needed for an undetermined period of time, 1/2 of 81mg aspirin, which she's currently on and Coumadin - for blood thinning.  There's a lot of debate between all the programs about the use of a blood thinner like Coumadin as a norm post-Fontan and Hope's team recommends it for at least the 1st year and then it's up to the parents. TBD on that one, Liv will drive that bus
• GI Issues: A known side effect from the Fontan can be ramifications on the GI tract, either temporary or permanent along the lines of constipation (hopefully short lived) all the way to more serious life changing but manageable conditions like Celiac Disease and Crohn's.  Dr. Ibawi's team feels that their incidence rate of these side effects are much lower in the patients at Hope because of the use of Coumadin post-op but was very quick to say that if we polled Cardiologist, General Practioners and Surgeons, we'd get even splits recommending different regimens anyway - so this is a play by ear and God willing irrelevant point for Livster
• Surgical Time & Stay: The surgery should take about four hours from IV placement to hopeful extubation at the end of the surgery but this could vary by a couple of hours depending on how much scarring Livvy has in her or if they see anything that they want to fix while they're in there...i.e. a collateral that they weren't able to get during her last cath.  Stay is still looking like 7-10 days pending her post-op drainage. She will come out with a medial and right chest tube to help with the drainage and those will be in until they're satisfied with the lack of fluid produced by her chest cavity

Holter was returned this past weekend and just awaiting those results.  All else is good to go and above all else Dr. Ilbawi stressed how well she is doing and how pleased he is with Olivia overall.  Music to a parents ears!

We Want Your Blood & Throw in Your Organs Too

As preparation for Olivia's upcoming surgery the hospital has asked us to have 4 units of blood allocated for her.  This means asking grandparents, aunts/uncles, extended family and friends to step up to the plate and roll up their sleeves for her cause.  She is A+ and can receive blood from anyone with A+, A- or O blood types.  Brian and I are not donating blood for her in the event that she may at some point need marrow or other tissue products; my understanding was if she previously received our blood it would make her reject anything transplanted later. I don't particularly understand that, but I'm willing to roll with it for now.  All four grandparents are donating for her or the hospital general blood bank - whichever it works.

If you're able to and looking for something to do for our families or Olivia, please go donate some blood - not that it'll go directly to her cause, but helping out the blood bank is something that's taken on a new importance to us given that we've had to use the supply a couple of times now during the course of her surgeries and recoveries.

While you're at it, please - make sure that you've legally consented to be an organ donor in whatever respective state you call home.  Legally consented means that you've given the authorization on your driver's license or state ID and also made sure to inform your family members your wishes should that dreadful day come that they need to make those decisions on your behalf.  There are approximately 75,000 people waiting on organ transplant lists at any given time.  Yes, I'll throw a little guilt out there - someday it may be Olivia on the list waiting for a new heart.  Hopefully it won't ever come to that, but there are HLHS families whose children needed new hearts and pediatric hearts are even harder to come by.  So please, at least consider it.

Happy Birthday Livvymeister - I think I can really start counting the months that go by but each one is better than the last!  You are officially 2 years old & 1 month.  Today your dada and I will be meeting with Dr. Ilbawi to talk next month's game plan.  Don't worry, I'm sure you'll get plenty of spoilage from your grandparents since  we thought it'd be best to keep you out of the hospital as much as possible.  I love you to the moon and back you amazing little girl!

Holter? Might as well be Holster

The 24 hour Holter Monitor test is underway.  It started off fabulously by me forgetting the order when I left for work in the morning, then forgetting to tell Nonna to bring it with her when we met.  So there we are - Olivia's already hip to what's going on and on the brink of hysterics and my mom runs back to the house to get the script to meet us back at the hospital. Luckily it was nearby but still, I felt dumb and bad for Olivia who's anxiety levels were already at a medium-high just being in a hospital setting.

Anyone who thought she would be fooled by a different hospital for the occasion got that one wrong.  There's a few things that tip her off: the big doors opening from one wing to another, the PA system with greetings or Codes, the sound of pulse ox alarms and of course scrubs on anyone and everyone.  Once my mom got back with the order they were great about rushing us through, getting the monitor setup and kicking Olivia out.

She on the other hand had a flair for the dramatic last night.  If you've ever seen a version of a Christmas Carole and can picture the ghost of Scrooge's partner Jacob Marley sitting there all morose in his chains and weights for eternity - that's kind of how Olivia looks with the wires and little monitor hanging off of her small frame. She doesn't want to walk anywhere or do anything and looks really pathetic while she's refusing to do just about everything.

I did bribe her and take her outside for a bit in the late afternoon tucking the monitor into the pocket of her hoodie and she enjoyed that. I'm sure that the weight of the monitor is enough to pull on electrodes a bit and be annoying - but to not want to do anything?  She does know how to milk the sympathy that's for sure.  Anyway back to clinical stuff around 4pm this afternoon test will be done, electrodes will be removed, and I need to drop the unit back off at the hospital for them to transmit the data back to Hope.

Meeting with Dr. Ilbawi on Friday so fingers crossed someone has read the test by then!

One Month To Go

It is very likely by this time next month Olivia will already be in the OR and doctors will be cutting open her sweet little chest once more.  Schedules have been cleared, vacations/trips cancelled or modified just so everyone can be around when she goes in.  During casual talks, everyone has been great about expressing their support, offering a lending hand for whatever needs may arise.  I hear all those things and am grateful but what hits me harder is how confident everyone is that this surgery will be just fine.  It's not ignorance because they realize the severity of her defect in saying it and they have the best of intentions, but it still rubs me the wrong way.

There are no guarantees in life and this is precisely one of those moments where I'm well aware of what hangs in the balance to a painstaking degree.  The thought of handing over this two year old with all sorts of personality, spunk and quick wits to get back anything less than the same post-op is extremely difficult.  The risk of death from the surgery is possible, but unlikely.  Still - what parent signing that consent form listing death as a possible risk associated with the procedure doesn't take pause?  I look at it from the perspective of there are % associated with the outcomes because someone has to be that minority - why wouldn't I consider Olivia to be in that small "unhappily ever"?  I know families in those small #'s, they had a child one day and didn't the next.  Heart families and healthy families alike - like birth, death is a part of life no matter how unnatural it may seem to grasp.

As I am writing this and delving deep, someone must have tipped Olivia off what's going on next month, she is running around screaming (quietly - you know vocal cords and all) throwing a huge tantrum to anything or anyone that will pay attention.  Duty calls!  And like that, life goes on...

Big Girl Bed & Updates

There is now a toddler bed in Miss Olivia's room as of Saturday morning.  By toddler bed in this particular case I mean, one crib rail was removed, mattress was lowered and now the partial toddler rail is there instead.  But still, she has a pillow, a blankie, her plush dog from Easter and to our dismay the transition was an easy one.  She hasn't quite come to realize that she can actually break out of her room now but I'm sure that day will be here before I know it.

I'm still going back and forth about trying potty training before next month.  I just think it would be unfair to get her all set and then put her back in diapers while she's bed ridden for a bit.  I'm probably one of the only moms out there who's child is giving signs to go potty and trying to delay the actual training part.

Verbally, Liv's saying a lot of new words now.  Bye Bye, Nana (banana), Up, Off, Yep, WaWa (water) - the only problem with all these new strides she's making is that she doesn't love to share her progress with her speech therapist which is kind of amusing.  I haven't made the appointment with the ENT yet, her problems with swallowing water seemed to go away on their own mid-week but her sweet little voice is still only partially there.  At this rate by the time it comes back it'll be time to take her back in for the Fontan. Boo

This week I'm going to get the holter monitor test done.  Should be quite interesting to see if she pulls leads or removes the electrodes to throw off all the results.  Yippee!

Fontanning

What I thought would be a midsummer's night is really a Mid-May gig now.  Olivia is officially booked for the Fontan on May 21st.  I have all sorts of emotions about the whole process, what it means, what needs to happen and where she goes from here.

It's the moment that I've been waiting for since that 20 week diagnosis and yet now that it's here, I'd really like to go back to bed and pull the covers over my head for a minute.  Pre-Op registration and testing needs to be done May 17th and before that still, a meeting with the surgeon needs to happen (for peace of mind) and even before that I need to get Little Miss in for the 24 hour holter monitor next week.

This is the next step, no avoiding it, no hiding from it and no denying it.  It's here and we are going to face it the way we do everything else - Head On.

Waiting on the Call

We've all been teenagers, crazy over someone or other and remember the days of waiting by the phone (or cell in this day and age) just waiting and agonizing over them to call - because heaven forbid you call them first.  Waiting on the hospital to call to schedule Olivia's Fontan is like all of that and more.  You want the Central Booking to call so badly, just to know the DAY but you don't because you really want to ignore the fact that this surgery is going to happen.  The Fontan is set for Olivia and it's going to happen within the next three months.

Her recovery from the cath is going amazing.  Her left thigh had virtually no bruising while her right which was occluded has bruising that runs up into her abdomen but doesn't seem to be bothering her in the least.  Her left arm is almost completely healed and the right carotid entry is barely noticeable.  Trying to keep her from her normal daredevil ways is already proving to be a challenge, but thankfully her sleep habits have almost gone back to normal so I can still consider it a victory.  The medicine regimen is the same as pre-cath .7ml of Enalapril - 2x  and 1/2 of an 81mg aspirin daily.

One complication I will cite is that I think there was some damage or irritation done to one of her vocal cords during intubation of this last procedure.  Liv's voice is still only a fraction of what she had pre-cath and she's having trouble with thin liquids like water without coughing or gagging.  She's also realized that if she really wants to she can make herself throw up.  I definitely could have done without her learning that little dramatic effect.  At this point I'm willing to wait this out and see if it'll get better on it's own before I call up an ENT to give her a look.  The less medical intervention the better for Miss Liv from our collective prospective.

"The Call" should come this week - hopefully someone will be there to field it or the office will call Brian and/or my cells to make sure they get in touch with a parent.  I've got an entire list of birthdays and anniversaries for all the family to make sure that we stick with tradition and now it's just a matter of hanging tight.

I Am in Awe

Children are just so resilient.  When do we lose that along the way growing up?  The ability to fall down at the playground, scrape a knee and get back up again to continue playing.

Olivia was discharged yesterday morning around 11 and props to the hospital staff for really working on the discharge process over the past year to get patients out when they actually say the words "discharge".  It's a big deal, it used to take a whole day sometimes which was just all the more aggravating if you're a parent with a month's worth of stuff trying to figure out how to time packing up, med refills, etc.  Once she was back in her own comfortable surroundings you could see how she just relaxed and went around kissing everything and anything that she missed.  It was super cute.

She is doing far better than I think anyone expected at this point with any residual pain.  The doctors said we could give ibuprofen or tylenol if she needed it, but she hasn't so we haven't given her a dose yet.  All the sites look really good and the band aids need to be changed at least three times per day which isn't her fave but she tolerates.  I'm fairly certain if I'd been poked and prodded as much as her in the past 48 hours that I'd want to be curled up and sleep it all off undisturbed by anyone and everyone.  Yet she continues to really get more pleasant as the minutes pass and this "low activity" that we're supposed to live by the for the next month or so due to the stent is going to be quite a challenge.

Sleeping however has been hard to come by as Little Missy has gotten very spoiled first by sleeping with me while we were in FL and now by having Mommy or Daddy around in the hospital to cuddle with.  She wants to be with someone for naps or nighttime and while I can't say I blame her the first night back in her own room from the hospital, this is looking to be a big battle in the near future.

For now I sit here so appreciative of all the texts, emails, facebook posts, messages, phone calls and everything else that we received with well wishes of good things for Liv.  Thank you all for thinking of her!

She's Maaaaad

Anesthesia has officially run it's course.  There's nothing from keeping Olivia letting everyone know exactly how mad and unhappy she is in her current environment.  Her voice is still super soft and scratchy from intubation, but her sats and all other vitals jump all over the board whenever people of the medical profession cross the threshold of her room for any reason and she lets them know.  She spent the night in either Brian or my arms in the lovely recliner in her room.

Progress was made in the way of fluids and around 1am she even started eating some macaroni and cheese. After her EKG this morning she decided that she wanted out of the room, so we detached all the wires and monitors and sprung her loose.  That might have been a mistake though as now she wants nothing but to see the hospital behind her.  Cardiology has to do rounds and we still need a chest xray before there's going to be any talk of hitting the road.

Until then, Liv is her firecracker self.  All dressings on sites have been changed but she's still a hot sticky mess in dire need of a shower (since she can't bathe for the next week).  Sats are still in the high 80's low 90's, resting heart rate is around 100-110.  Everything looks great from what I can tell - hopefully the doctors agree.

The View from Floor 2

Writing from room 2036, we've been here since approximately 4 this afternoon.  Yes - all that other time was spent in a recovery room not much bigger than the crib Olivia was in.  Now, we're in a beautiful private room with it's own full bathroom and plenty of space to have grandparents come and visit complete with dinner.  Olivia dozed throughout most of the visit.  She's still nauseated and more anti-nausea meds have been ordered up.  All of her fluids are still being delivered via IV as she doesn't want to drink anything.  Makes sense to me, I don't like drinking or eating when my tummy's upset either.

Unfortunately there is no easy way to get around this vicious cycle.  No discharge without liquid and food intake but Olivia still hasn't worked the drugs through her system enough to where she feels like her old self yet.  Vitals look great, there's been no additional bleeding from the cath sites and she's really just sleeping it off like a twenty-first birthday celebration.  Hopefully tonight can be as non-eventful.

Recovery

Sitting here in recovery with Miss Liv. They went in through her arm, neck and both legs. Declared ready for the Fontan, she looks like a fighter that went many rounds.

Stented, coiled and ballooned were her vessels and arteries. She is here and healthy but angry and I am thankful

Waiting on Dr

Word is they're done. She's been poked and prodded more than we expected. I will update once we go over the findings with the doc

Cath Update #2

The lab called to speak with me and give a live update.  All of Olivia's vitals look great, she went to sleep well and was intubated without trouble.  They were able to go in successfully through her arm but as it turns out that was for naught as they need to put a stent in through the LPA.  Shucky darns!  What this means is that even though they got in through the arm, they will have to also go in through the neck to place the stent in the LPA.

There was no mention of coiling or ballooning yet, but that doesn't mean anything at this point.  It's definitely an overnight stay now for monitoring and administering preventative antibiotics.  Looks like it'll be another hour or two potentially before she's out in recovery.

Cath Update - Non Update?

The general procedure nurse just came around giving families status updates.  Liv is under and they have begun the procedure - it seems like that took longer than I was expecting, but who knows.  I'm still hoping they went in through her arm instead of her neck but it doesn't really matter so long as she comes back safely and as healthy as she was when they took her in to the lab.

Check-ins with the grandparents prove that they are having a hard time concentrating and keeping busy to pass the time.  My mom is cleaning her house, so please note that whenever Olivia has a procedure you should really get her booked up to come over and clean your houses.  The more worried she is, the more effectively she cleans.  I'll have to keep it in mind the next few weeks while Olivia's immune system may be a little weak she can go hang out in Nonna's sterile environment.  :)

Cath Day Update #1

I'm here in the waiting room of the outpatient procedure area here at Christ/Hope Hospital in Oak Lawn.  Olivia's been with the medical staff now for about 30 minutes.  It seems like their process has gotten a lot smoother since the last time we had to do this 18 months ago.  They gave her some Versed orally to relax her before they took her back seeing how ferocious she was becoming and she still fought.

The outlook right now is an overnight stay.  For all you heart parents, they are going to try to go in through her arm and use the neck as a last resort entry point.  They want to review and coil off any collaterals they can reach, balloon her LPA and they mentioned some narrowing on the Aortic Arch.  Triple blah if you ask me.  It's not unexpected news but I was hoping for a same day discharge and now she's going to have to endure the overnight stay and up the anxiety a bit.

The doctors set the expectation that this will be about a three hour deal, so we should hear something in another hour or so.  I'll update as information becomes available.  Thanks for hanging in there with us!