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!

almost breathing on her own

Saturday and Sunday were very uneventful.  A much needed calm break from the craziness that has been the last 30 days.  It's hard to believe it has already been a month and we've packed three visits to the operating room and two visits to the cath lab.  Lucy is a super trooper.

The team made very minimal changes over the weekend except for her ventilator setting.  Over the course of 3 days she was able to move to CPAP pressure support.  This means Lucy is initiating every breath and the ventilator is giving support at a preset pressure value.  Once there, the only thing to do is ween her sedation and remove the tube.

The team gathered this morning to discuss what are Lucy's next steps.  Above all, is to get her off the ventilator, but a bigger concern is the amount she is draining out of her chest tubes and the implication that her lymphatic system is not healing.  The return to the OR twice last week were obvious stressors on her system.  However, the team is looking ahead, in the case we see no change. There were three options that were discussed the first (I can't remember the name) but it has to do with 'scrubbing' the lungs to stop some of the leakage.  The second is a fontan take down.  The third, a procedure where they technically glue the holes that are leaking in the lymphatic system.  The third option doesn't seem so bad until you know that this is done at Children's Hospital of Philadelphia (CHOP).  Yikes, right!  Anyway, all of these options are on the back burner until we've given Lucy a chance to breathe on her own.

After contacting CHOP, the team wants to see if we can find a doctor in the Chicagoland area that can shed some light on her lymphatic system.  There may be a diagnostic test that we are able to do in the hospital which would provide initial information we can then send to CHOP.  Any diagnostic test, would require Lucy to be intubated, so we want to see if that can happen by tomorrow and then we would pull the tube.

We know you are all praying, so here is our specific prayer request:
~Lucy can tolerate breathing on her own
~Lucy's lymphatic system can start to heal once she is off the ventilator and there is a drastic decrease in her chest tube drainage
~Lucy's blood pressures and oxygen saturation remain stable through any changes we make

Then they cried to the LORD in their trouble, and he delivered them from their distress.  He made the storm be still, and the waves of the sea were hushed.  Then they were glad that the waters were quiet, and he brought them to their desired haven.  Psalm 107: 28-30

a little scare

The worst part about the breathing tube other than it being there and for so long is the pure frustration I have from trying to communicate with Lucy.  I can see in her eyes she is trying to talk to me but without words it is nearly impossible to understand exactly what she needs.  We have been using the squeeze method.  I asked her question and if they answer is yes, she will squeeze my finger.  Unfortunately, I don't always know all the questions to ask.

Case in point, last night (I'll get to the scary stuff in a minute).  After all was said and done and Lucy was stable, she was showing much discomfort.  She was able to communicate that she was not in pain and did not want water, but I really didn't know what to ask.  Late last night she threw up sludge.  Pretty much the stress to her body created a back up of old blood, bile and other stuff that was just sitting in her stomach.  After she threw up, a tube was placed in her other nostril and they suctioned out more of the sludge.  She seems to really settle after her stomach was clear.  I have since added, 'is your stomach icky' to my list of questions.

Back to Friday, this week has left Lucy sensitive and very highly stressed.  I mean who could blame her, two sternotomies in three days and her skin has taken a beating from all the dressing changes on her chest tubes.  Early afternoon the PICC team came to change yet another dressing and I think that was the straw that set her over the edge.  Shortly after Mike came back in the room and her saturation began to drop, followed by her blood pressure.  The room started to fill up with nurses and doctors, then her heart rate dropped and they started chest compressions.  She stabilized quickly, but had no other reason then stress and agitation for her code.  They pulled all her pain and sedation medication to wake her up to ensure no other damage (like stroke) had happened.  By 5:30, they had given a dose of phenobarbital and she was calm again.

Since the fenestration stenting, her oxygen has been sitting in the 65 to 70 range.  With lower oxygen saturation, her belly is not getting optimal blood and that's probably a factor in the aforementioned sludge.  This morning her blood pressure and saturations have looked great.  The team has been able to ween the vent settings back to before the code.  We are keeping her sedated today (and probably tomorrow) to heal, but are hoping that was rock bottom and we can only move up from here.

I can't tell you the comfort I feel from those that have continuously been lifting Lucy, our family and her medical team up in prayer.  We are eternally grateful for your diligence.  We love you!

fenestration

This stay is eerily starting to feel like our first time here.  Breathing tubes for weeks, multiple procedures, unexpected setbacks.  We made home and thrived then.  We can definitely do it again!

Sunday was a low key day, we kept Lucy comfortable and didn't make many changes.  Dr Nater decided not to pull the breathing tube because going back to the OR was a high possibility and we did not want to take it out, only to place it again.
Monday morning, the team gathered and decided to do the fenestration.  The fenestration is a hole that is placed in the right atrium and the fontan, which is then sutured together.  The hole allows for blood to flow over to the atrium when the lung pressure gets to high.  Although this will lower her 02 saturtaions, it hopefully will stop all the drainage.

#1 is where the fontan (conduit) is connected to the pulmonary artery
#2 is the fenestration

They were debating on when to take Lucy to the OR and placed her on the schedule Monday afternoon.  I signed all the consent forms (I feel like I am signing one a day at this pace).  Around 3 pm they had her mobile and ready to roll on down to the operating room.  I think normally, they would place Lucy on bypass in order to get into the heart and have a good look at where to place the fenestration, however with the previous week, I believe they felt it was too risky for her body to handle.  What they did is clamp off the space, then go in to find the best part of the atrium to place the hole.  The surgeons were in there longer than expected because after all was said and done, they expected her saturation to drop.  However, they remained in the mid to high nineties.  Lucy was back in her room by 8 pm and we were with her shortly after.  During surgery, on top of her three existing chest tubes, they placed one more and then a peritoneal port in belly.  Usually used for dialysis, but this was going to be used to only drain fluid as needed.  The fenestration ended up taking longer than the whole fontan surgery.  Only Lucy, I swear!

With no drop in oxygen saturations, the doctor's wanted to confirm that the hole was still open.  All of Tuesday was spent doing tests.  First an echocardiogram.  The tech was unable to get a visual of blood flow.  Next, they pushed saline through her PICC line.  This was actually cool to see.  When that happens a rush of bubbles flood through and pass through the fenestration.  They thought it would be easier to see this way.  Still no!  Our last effort was an TEE (transesophageal echocardiogram).  This is where an echo transducer is attached to a thin tube that passes through your mouth, down your throat and into your esophagus.  Because the esophagus is so close to the upper chambers of the heart, the images can be more clear.  I will say, after seeing the tube, thin is an understatement!  Dr. Roberson was unable to identify if the hole was still in place.  I could barely believe it.  Did Lucy really close the hole on her own less than 24 hours after surgery.

The team reconvened yesterday afternoon to discuss and decided that placing a stent in the fenestration would be the next step.  Our biggest concern about going back to the lab was damage to her kidneys.  We paged the nephrologist to ensure that Lucy was prepared and her kidneys were protected,  She was given extra fluid and a medication called mucomyst.  This medication is known to help protect kidneys from harm caused by contrast dye.  The goal is to try to place the stent using as minimally invasive procedure as possible.  They will try first with a catheter, then if they are unable they will go back through the sternum, but will only need to open up the lower part.
So we will wait to see if this (hopefully our last) procedure will do the trick.

Sometimes in hard times like these, all you can do is lean.  Lean on your faith, your family, your support and your prayer.

Rejoice and exult in hope; be steadfast and patient in suffering and tribulation; be constant in prayer.
Romans 22:13