Promise

I definitely followed through on my promise.  Friday, the 21st, the breathing tube came out and I made sure we got time together out of the bed.  It was a long time coming, 58 days to be exact.

The biggest concern when pulling the tube was her lungs and making sure the secretions we being coughed up.  We started with CPT vest therapy.  This involves an inflatable vest that is attached to a machine.  The machine mechanically performs chest physical therapy by vibrating at a high frequency.  The vibrations to the chest loosen and thin the mucus.  This vest treatment is most commonly used in patients with cystic fibrosis.

We scheduled vest treatments every two hours during the day and every fours hours overnight to make sure her lungs stayed open.  Surprisingly, the worst part for Lucy is getting the vest on.  After its on, she usually falls asleep.  However, having the respiratory therapist come in her room every two hours, along with other staff in and out all day, Lucy's anxiety is at an all time high.  So much so, that she started grinding her teeth.  Can you imagine sitting in a room with someone grinding their teeth for an hour?  Now do it for twelve hours?  I may need medication soon!
We are working hard to keep the traffic in her room down and some anxiety medication throughout the day.  At night, we are using melatonin to help her sleep through the night and it has allowed for a string of good ones.  Better sleep equals better days!

Last week we started prepping for home.  Don't get too excited, there's no official (or unofficial) date.  Home oxygen and feeding supplies were ordered.  All of her IV medications have been switched to oral.  Coumadin has been started to get her dosing just right and we'll look to stop heparin.  In order to get to floor two (step down) we still need a few things to happen.  We need to be off nitric completely and off high flow.  That means Lucy needs to keep her saturations above 75 with less than two liters of oxygen.  Right now we are on four liters and are experiencing some episodes where her saturations drop to the fifties.  The other issue is her stomach.  We are still draining a significant amount from the NG tube.  When we have tried to clamp it to see if she can handle it, she ends up throwing up the bile.  Unfortunately, being intubated put her as a high risk for aspiration.  Aspirating on vomit could have a huge impact on her lungs.  We don't quite have answers and will have an ENT evaluation in the next couple days.  With that risk, it means she also can't have food.  Luckily for us, she isn't very interested in food or water.  We will need a swallow study before we can proceed with oral feedings.  Whether that happens as in patient or out patient procedure will depend entirely on Lucy's desire to eat.

We have been working to get Lucy stronger and have been out of bed several times.  She can usually last about thirty minutes and can sit up on her own without support for at least ten minutes.  Each day she is getting a little stronger.  And each day we get some more big smiles.

Her favorite thing is to play with play doh. For those at home, that's a hot dog, banana, blueberries and a cupcake.

We have had some minor set backs with plural effusions and hazy x-rays, but we have fought off anything major with an extra dose of diuretics and keeping the CPT treatments throughout the day.

Praying specifically for the following:
Lucy's anxiety to subside
Lucy's oxygen saturation to stay about 75 with less than 2 liters of support
Lucy's stomach and vocal cords to heal
Lucy to gain strength
For us to be home soon

much love and thanks to our tremendous support system.

hindsight

I keep thinking if I had known that Lucy was going to be re-inutbated early Wednesday morning, I would have made sure I held her all day Monday and Tuesday after the chest tubes came out.  Hindsight is cruel.  I have had plenty of cruel thoughts.  What if we fenestrated initially?  What if we didn't go to the cath lab?  What if I had pushed harder for her breathing treatments?  Why didn't I hold her?  Would any changes have gotten us home sooner?  Unfortunately none of those 'ifs' change where we are today, so I am moving on and remember that the day this tube comes out we'll be sitting in the chair together for as long as she'll let me.

I know so many have been waiting since last Wednesday for an update.  I thank you for being patient.
To be honest, not much has changed in the last week.  Wednesday to Friday we were trying to understand the reason for her lung issues.  Cultures of blood, urine and breathing tube along with a viral panel were sent.  All came back negative.  She had been on antibiotics as a precaution, so we aren't sure if those masked anything going on in her lungs.  We continued her breathing treatments in hopes of breaking up lung secretions.  Over the weekend we made minimal changes.  Mike and I just hung out, helped with her treatments and watched movies with Lucy.

Monday morning, the infectious disease doctor came to review Lucy's case to see if we could change or adjust her antibiotic regimen.  The first question she asked was, "who has been visiting?".  This is exactly why we are so strict about visitors.   Unfortunately you don't always know what you have been exposed to and for kids like Lucy, especially when her immune system is down, the littlest things can be devastating.  Her current regimen, they felt covered all her bases and that morning her x-ray looked better than it had since intubation.  Tuesday morning, her right lung had collapsed again.
The next step would be a rigid bronchoscopy.  This procedure is done in the OR under general anesthesia and would remove the large secretions blocking her airway.  Yesterday afternoon, Dr. Sherman (ENT) came to do a bedside evaluation and put her on the schedule as an add on.  She wasn't able to go Tuesday afternoon because you have to be NPO to go under anesthesia.
Can you guess what happened?  You can't, come on.  This morning, her large airways cleared up and x-ray looked better.  All you have to do is schedule something......and it appears that Lucy will then fix it.  With clear airways, a rigid bronchoscopy would not provide any benefit.  Here we are again in another vicious cycle.  Kids do better off the breathing tube after the fontan, to stay off the breathing tube she has to be able to cough up the secretions to keep her lungs open, she is having trouble coughing up secretions because they are thick, her secretions are thick because she is dehydrated, she needs to be dry because of her fontan physiology.  If her x-ray is the same tomorrow morning or even better, the team will start to weigh the risks and benefits of extubating.

Again, we're asking for prayers of strength.
-For Lucy to be strong enough to be extubated for good
-For Mike and I to continue to handle the uncertainty

I'm going to brag a little bit.  Miss Lucy has handled this intubation like a champ.  Usually kids need to be on moderate to heavy sedation in order to not pull the tube out.  But THIS GIRL has been on minimal sedation since Sunday.  She hates the breathing treatments and being deep suction (where they shove a tube down your throat to suction out secretions).  Yet, through every treatment, she follows your directions even as she cries.  Open your mouth...done, cough as hard as you can....done, one more time...done.  This is probably the hardest thing we've been through and she has been polite, respectful and a model patient.  We couldn't be more proud of how she has handled herself!

Only a true hospital kid falls asleep holding her breathing tube.

She was able to get a little play time in with water marbles.  These things are pretty neat.  The more water you add the bigger they get!

42

42.  FORTY TWO, you guys!!!!!  You'll understand my excitement better when I tell you early last week her chest output was roughly 1700 milliliters.  Since pulling the breathing tube, we have seen a steady decline, starting Friday (the day we submitted all our paperwork to CHOP).

Here are how the numbers looked each day.  This is the total chest tube output from all five of her chest tubes.

1100 mL - Friday
650 mL - Saturday
496 mL - Sunday
400 mL - Monday
250 mL - Tuesday
220 mL - Wednesday
42  mL - Thursday

I believe breathing on her own, coupled with some small medication changes had a big impact on the decrease.  With starting feeds, we are also seeing improvements in her skin.  It's hard to see the feeding tubes and I am coming to the realization, that the possibility we go home on it is high.  Right now she is getting continuous feeds at 36 mL an hour.  Talk about flashbacks.  It was a little over 3 years to the date that we switched her to continuous feeds to leave the hospital for the first time.
Wednesday, the team decided that it was time to start diuretics again  We saw her urine output increase.  Then yesterday we went up on the dose and holy cow could you see a difference.  It looks like her eyes are resting on the back of her skull.  And as expected, she begged for water.  We were able to please her and give her as much as she wanted because the NG tube is sucking all the stuff out of her belly.  Overnight they stopped the lasix drip in order to not dry out her too much.  We have been there (week 2) and it did not go well!  It will be a fine balance.

Our next issue to tackle is the sludge that is resting in her stomach.  The bleeding has subsided and now we are seeing mostly bile.  Last night we flushed dye down her NG tube, then took x-rays twenty minutes apart.  We were looking for an obstruction in her GI track.  Usually they would do an upper GI, but in Lucy's fragile mental state right now, this was our best option.  The dye made it's way through no problem.  We'll begin to clamp the NG suction for an hour at a time to see if she can handle it.  If she does we'll increase time clamped, until we can remove it.

With the help of some anxiety medication, Lucy has been better able to handle the commotion that is ICU.  It helps a ton that her nurses are so diligent about who is let into her room.  We have also tried to have minimal procedures this week.

If I said it once, I'll say it a million times, we can't thank our prayer warriors enough for their diligence.  I truly believe our prayers were answered as our trip to Philadelphia has been put on hold.  We will continue to keep CHOP informed on Lucy's progress and if the need changes we will reassess, but for now we are staying put.

Have a Heart

The CHD community is pretty amazing.  I have met some great heart parents along the way that have offered advice, a night out or a shoulder to cry on.  We are so very fortunate that our community extends far past just those in the CHD 'club'.  We knew we wanted to give back!

LJ's Healing Hearts is our way to make an impact on the lives of the CHD community.

Here's how you can help!

1.  Talk about us!  The more people that know about what we do, the more heart families we can help.

2.  Attend an event

3.  Donate items for an upcoming event (our next event is in August-details below)

4.  Donate money

5.  Hold a fundraiser

6.  Volunteer

trip to philly .....maybe?

I am always amazed that days in the hospital can go by so quickly and yet seem so long.  There are days when I look at the clock thinking, how can it already be 5 pm?  I've been in this room all day and it feels like I have done nothing.  But tending to a three year who has developed some major anxiety can keep you really busy!

It has gotten to the point that any time we pull out detachol (it's magic in a bottle that gets sticky tape off skin) or put on masks, she immediately starts to cry.  Masks are worn for all dressing changes.  So when you have five chest tubes and two IV's, it seems all the day consists of is dressing changes.  Her anxiety has also expanded to loud voices and crowds.  Unfortunately, those are also not easily escaped, especially when you're so cute.  We are trying to manage it by keeping her nurses the same, explaining everything that is happening and trying when possible to keep extra 'friends' out of her room.

This past week has been filled with small setbacks.  Monday afternoon we were prepared to bring her off the vent but the team was still very concerned about her drainage.  They decided to postpone extubation.  They reached out to a handful of other hospitals to see if any were able to do lymphatic imaging.  We were trying to gather any data possible to send to Children's Hospital of Philadelphia.  Any procedure would require her to be intubated, so the team thought it best to wait a day and find out if any facility could help.  Since none had the capabilities, we removed Lucy from the vent Tuesday morning.  After coming off the ventilator, she was put on high flow oxygen, so her nose is completely obstructed.  She has her NJ tube in the right nostril, the NG tube in her left nostril and the oxygen cannula over both of them.  Luckily none of it seems to bother her all that much.

She was still displaying signs of major discomfort, which was due to constipation.  Her x-ray showed a mass that was not moving.  Two days of enemas and Mira LAX and she was a different girl.  As we started talking about feeds, the NG tube that was sucking out blood and bile from her stomach started to consist of mostly blood.  They called in the GI doctor and they think she developed a stomach ulcer, coupled with the heparin, the bleed was not clotting.  The heparin was stopped but we are still seeing a lot of stomach bile.  We are not sure what the next steps will be except to continue to give medication to help heal.  Then we saw a partial collapse of her upper right lung, we started treatment and with in a day it was showing improvement.  Because of the aforementioned anxiety, we ended up stopping treatments as soon as we could.  When she is grabbing and crying for them to stop, sometimes you just have to throw in the towel.

On Thursday, Dr. Penk came to talk to us about the likelihood of going to CHOP.  It is the only hospital that does lymphatic system imaging and intervention.  At the end of last week, her drainage was still pretty high, so we decided to start all the paperwork for the procedure and transfer.  Since it was nearing the weekend and a holiday weekend at that, we don't have a lot of information.  But here's the gist, the doctors use dynamic contrast MR lymphangiography to identify abnormally leaky vessels and then occlude them.  This stops the drainage and then she can heal.  We know transport will be out of Midway and one of us can go with her.  That means one of us will need to book a flight last minute to get out there.  We also have no idea of recovery time or if we know the lymphangiography works, that we can come back to Advocate to finish the rest of her recovery. I really don't want to leave Advocate, it scares me to death...new doctors, nurses, new everything.  But I also know, if they are telling us this is our best option, we have to try it.   The earliest we would go out to Philadelphia is Thursday, however, if the team feels that the drainage is slowing down, we can postpone.

On top of being extubated another big accomplishment for Lucy is she sat all by herself.  She was so proud, she gave me a smile.  It has been over 3 weeks since has sat without any support, so we applaud even the littlest of milestones

 And she got some of her friends to join in on the fun.

Another milestone we hit was first haircut.  Although not planned, as you can see from the picture below it was a MUST.  A big thank you to our friend Katie for her help in making it a great experience!

We have a little time to let the back grow out, but at least she is not laying on a dreadlock.

 We are continuing to pray that her drainage decreases and ask that new prayers for Lucy's anxiety be added.  We are doing everything we can to try to ease her fears!
Just to reiterate my first point....I started this blog post at 8 am this morning, it's now almost 9 pm and I am just finishing it up!