The hardest decision of my life this far...

where to start...

 

Well, the GJ failed.

Completely.

We tried EVERYTHING

to make it work.

 

When the Drs ran out of ideas,

I told them a few things I thought they should try

 

We tried them all.

Nothing worked,

She isn't even tolerating pedialyte through the GJ now,

We had to stop using it COMPLETELY

She is only on TPN right now

(TPN is IV nutrition and only to be used as a last resort)

So remember that post back in August about how this GJ HAD to work?

(You can read it by clicking here)

 Well since it didn't work

We are faced with a choice

either Long term TPN at home

or

try going back to G tube feeds and trach her.

With Long term TPN:

Her port will be accessed 24/7

It almost guarantees a line infection,

(a line infection is extremely serious and often deadly)

most peoples gallbladders are shot in 14 weeks,

and they have liver failure within 1-4 years.

A person cannot live without a liver...

But with TPN she would get the necessary nutrition to survive and

probably wouldn't

need a trach,

or so we thought...

With a Trach:

she will have a tube sticking out of her throat,

She wont be able to speak unless she gets a special speaking valve

and there is NO guarantee that the speaking valve will work

and she wont qualify for one for the first month or so...

(so we wont hear her precious voice or cry or laugh at all)

She will be at a higher risk for lung infections.

She could have damage to her wind pipe.

She will have A LOT more medical equipment that goes everywhere with us

But with a trach

when her airway collapses

she wouldn't turn blue and pass out she could still breathe

She might be able to start eating food orally again

and we could get the GJ tube OUT of her and try to go back to a G.

She shouldn't develop brain damage from lack of air

She should be able to Fly again and go see the out of state Drs she needs to see...

With the two options we have We feel the OBVIOUS choice is the Trach.

Its really kinda sad when going with a trach is the option that will give the best quality of life

So after much prayer and research.

Lily is getting a Trach.

We meet with the surgeons tomorrow.

Then last night almost as if it was ANOTHER confirmation that we were doing the right thing

She had THREE spells.

Her airway collapsed,

She was trying to breath but couldn't

She didn't pass out

but  alarms were going off and her mouth was blue.

The thing was she was on TPN when they happened.

So OBVIOUSLY keeping her stomach empty wasn't going to stop these events

No these events weren't as bad as the others

 but they were bad enough.

I will update more as I learn more.

But I am sad to say my baby girl is getting a trach.

:'(

"Life Threatening Events"

Ok, so long time no post.

Sorry about that but things got pretty busy

and I have struggled to find the time.

Things were going pretty well for a while!

We went two WHOLE months without a hospital visit.

Lily was/is still struggling with her rash,

but she made HUGE progress in eating!

About six weeks ago we started her on the new medicine for

the pain associated with her V.H.(Visceral Hyperalgesia)

And after two weeks of being on it she 

started eating and eating and eating!

She made progress in leaps and bounds!!

She was eating up to 2oz of puree's

 in ONE SITTING!!!

Plus she has been doing the signs for

"more" "food" "please" 

and stringing them together!

And she was asking for more of her bottle when it was empty!!!

She even was accepting some puffs and happily eating 10-15 of them!!!!!!!!!!!!!!!

I got so excited that I started imagining her weaning off the g-tube in a matter of months! 

Ha ha ha

What a vivid imagination I have!!!!

Silly silly me.

Then we decided to go to walmart.

Dun, dun, dun!!!!

Now its not exactly
Walmart's fault

its just happened to be where we were and I now associate Walmart with the following event.

She started acting fussy.

weird fussy.

Lily was with Shaun and I had gone to another isle.

Then she started crying/wimpering a weird cry so I made my way back over to 

Shaun and her.

He had just set her down in the shopping cart when I walked up

but I picked her up cause she was acting odd.

Then out of nowhere she arched backward so forcefully and stayed bent backwards.

She went rigid and her arms bent backwards in an odd way.

Her mouth was opening and closing like she was trying to breath

And

SHE WASN'T BREATHING.

I watched her panicked eyes as

 her lips, then mouth, then face turned blue.

Her eyes then rolled back in her head

 and she passed out.

I started yelling "she's unconscious shes not breathing!"

I shook her and smacked her back and was just about to lay her down for CPR

When she took a breath and woke back up.

The whole thing was only about a minute but it felt like 20! 

It was only then that I became aware of the shoppers standing there staring...

We noticed her legs were blue and stayed that way for 10 minutes or so afterwards.

We went to the hospital.

They said it was most likely a seizure

And they weren't gonna worry about it and sent us home.

(not worry about a seizure?!?!?)

We were there barely an hour!

The next night my brother and I went out to Sushi

and Lily was happily playing and eating her puffs in the industrial wooden highchair they provided.

She was blowing kisses and saying "Hi" to EVERYONE in the restaurant. 

The waitress was commenting on how she was the cutest kid she had ever seen.

We had just finished eating when she

 arched back (with enough force that she broke the industrial high chair straps)

I couldn't straighten her and it was difficult to get her out of the highchair

And she was trying to breathe again but couldn't.

I stood up

and started crying

All I could say was 

"Not again, please not again"

She went blue and passed out.

I ran to the car as My brother payed the bill

She woke up and cried and cried and screamed

She was completely disoriented and didn't seem to recognize me at all.

 We drove to the ER which was directly across the street from where we were.

(It would have taken longer to call 911 and wait for the paramedics)

 As we pulled into the ER she stopped crying

We went inside and she started 

giggling and babbling at the fish in the tank!!!

The ER attendant looked at me like I was crazy bringing this

Happy giggling girl in there

(until i explained of course)

We then got to have our second ambulance ride

It was a LONG ride there and she was stable and sleeping so

I had the presence of mind to take a picture

But ambulances are SUPER bumpy

And my new phone camera isn't that great so this is the best I could get...

 we were transported to PCH

They did the "normal" labs they always do for her.

The good news was her white count was normal!!!

(for once)

She had an EEG,

  it was normal,

 Which rules out most kinds of seizures.

She then had an MRI.

It has to be done under anesthesia

which is very dangerous for her

but It went beautifully!!

We had an AWESOME anesthesiologist

and she woke up happy and giggling!!!

In this last one she was "singing" and trying to dance but was 

super loopy and couldn't even lift her head

It was hilarious!!!

The MRI was also normal.

So, they monitored us a bit longer in the hospital

and then sent us home.

We were there only 3 days.

After talking with the Neurologist, Pulmologist, And Gastroenterologist they are confident

they know what happened.

They believe Lily's sandifers syndrome/GERD

has become so severe that she is

aspirating the stomach contents

which is aggravating her already problematic

larynx and esophagus

and causing Laryngeal spasms .

Meaning her vocal chords are freezing up and she has to pass out in order

for them to work again...

If a persons vocal cords freeze,

It is impossible to breathe. 

After talking with her GI today

who spoke with 2 pulmologists, her SN PCP,

 and another specialist that I wasn't familiar with the title for...

They all agree that because of these

"life threatening events"

(that will only continue and probably get worse)

Lily needs another surgery.

A Nissan Fundoplication or "Fundo".

There is one more extensive test they will do first

where they will put a probe down into her stomach and monitor

her for 23 hours in the hospital.

Then depending on that test we will be having the surgery.

The "Fundo"  is when the top of the stomach is wrapped

around the bottom of the esophagus

making vomiting impossible.

You can see how its done here...

If  nothing can come back up out of her stomach this should stop 

these laryngeal spasms.

Our GI said she has only recommended a Fundo to 4 other 

patients in her 15 year career.

Fundo's have widely become thought of as

"outdated and unnecessary UNLESS severe aspiration is causing life threatening events"

which in Lily's case it is...

This also means she will

ALWAYS need her G Tube.

It Means she will probably significantly regress in her eating skills

as she will have to not eat anything by mouth for quite some time.

Because of her EoE,

She is also HIGH RISK for many complications like 

tearing the esophagus or stomach ,

internal bleeding, etc..

I am discouraged about it,

but at the same time I  am grateful that this technology

is available,

because holding my daughter in my arms while she is blue and unconscious is

HORRIFIC!

And I hope it NEVER happens again.

So, with that said...

Bring on the Fundo!!

Not sure what to make of all this...

So I have so many many emotions going through me right now

relief that we didn't have to have surgery

but frustration cause we still might have to,

Anger at the Jerk doctor that just came in here an made me feel 1 inch tall and an

Overwhelming desire to get that doctor to see and listen

or maybe JUST READ HER CHART!

Guilt for feeling that anger.

Fear, so so much fear for what lies ahead.

Physical and emotional exhaustion,

Sorrow, for my sweet daughter and her pain and suffering and fear

And OVERWHELMING GRATITUDE AND AWE

at the incredible out pouring of service, kindness and love.

So many many people have reached out to us.

I feel so undeserving.

I am amazed by how many people love and care about Lily.

Ashamed that I can't do more to help her and others.

Overpowering LOVE 

for 

My Savior and Redeemer Jesus Christ

And For My sweet daughter, and husband and family and friends.

Mostly I want to scream, cry and laugh.

I don't really know how I SHOULD feel,

but this is how I feel.

I also wanted to give an update.

The surgery was set for 11:30

It was pushed up to 9:30

then pushed back to 10:45

Then to 11:30

Finally Shaun told a nurse that SOMEONE needed to tell us 

WHAT WAS GOING ON???

So a surgeon came in and told us that there were 3 surgeons

each had a differing opinion about this surgery

and they were arguing about who was gonna do it

It basically all came down to whether 

her position in the x ray could effect the position of the cath in her heart.

So they did another x ray

It turns out it did...

kinda...

So they determined her heart wasn't in immediate danger from the port

so It was gonna stay,

one of the surgeons disagreed because they think she may be septic.

we should know by the morning if she is.

That would be very bad.

I am not thinking about that unless it actually happens.

so we are just back to waiting

and just to make things even more pleasant the doctor

requested an RSV test

which means we are on precautions.

She has no symptoms of RSV and the nurse called to tell him that

but he insisted...

So that's where we are...

more waiting...

Again?!?

So here we are again!

Lily is back in the hospital and

She is having surprise emergency surgery tomorrow.

This is her 11th admission.

I am still a bit in shock it all kept happening so fast. 

This morning she woke up crying

(not a good sign)

She had fed the bed yet again...

but this time she was FREEZING and her legs were blue...

I put her in a warmish bath to warm her up like I always do

when she has fed the bed, but this time she didn't warm up.

I put her in our bed between Shaun and I and she finally started warming up after we massaged her legs and feet for a long time.

About an hour later she was ice cold on her arms and chest and was uncomfortably warm on her legs

She had weird splotches all over her legs and she had a temp rectally

but her head was really cold.

Her temp fluctuated but she had an appointment for tomorrow so 

I just put in a call to her doc to see if they wanted to see her today instead.

We were on our way to our first feeding therapy when

the doc called.

She said to skip FT and go straight to the ER.

So much for our first feeding therapy appointment..

We waited 8 months for this one so one more week wont hurt I guess...

The First doc we saw asked why we were even here cause

"she looks fine to him"

I told him how she "looks" is deceiving and he kinda rolled his eyes.

So I told him to call her doc who sent us there

And ask HER why we were here

He did.

He came back and said

The Doctor wanted some blood work then he was sure we could go home.

Because She didn't LOOK sick.

Within minutes of taking her blood the doctor came back in

He said her white count was EXTREMELY HIGH

and "He didn't see that coming cause she doesn't look sick"

I totally pulled my best I told you so look

and rolled my eyes...

Then reiterated (in an exasperated tone) that

how she "LOOKS" has nothing to do with how sick she is.

Yes it was rude I know, but he wasn't nice and kinda deserved it...

Anyways then her heart rate started to go high and her blood pressure rose

and her temp started going kinda crazy.

One minute she is bright red and sweating and the next she is way too cold

She started swelling all over for no reason

They sent her for a chest x ray to make sure her lungs were clear

AND GUESS WHAT

her port is back in her heart!!!!

how does that even happen??

Was it not fixed last time this happened?

They have to pull the whole thing tomorrow.

They also know she has a raging infection somewhere but they don't know where.

They are afraid its in her port...

that would be bad!

All I know is she is sick and they don't really know why

And she has to have surgery tomorrow

And Anesthesia is incredibly dangerous for kids with Mito

She has had bad reactions to it in the past.

she could really use some prayers

and if I'm being honest... so could I and Shaun too.

Feeding Tube Part Three

By October Lily had developed complications from the NG tube.

She was gaining weight great however,

and was no longer FTT!

But She had laryngomalagia,

and the tube was making it worse

causing her to have episodes of not being able to breathe,

Her voice was non existent or hoarse all the time,

and she was getting nose bleeds.

Plus,

the emotional trauma from me holding her down

and shoving a tube in her while

she screamed and cried

was taking a serious toll on her and me

and on her trust in me.

Plus we had a meeting with her SLP(speech language pathologist)

and with her GI, and with nutrition

and her dysphagia had gotten worse.

She HAD to have a feeding tube still for medical reasons

and also because she refused/didn't have the energy to drink more than 12 oz by mouth a day.

(she needs to get 32 oz in a 24 hour period 

to sustain herself.)

We also knew she needed a med port, and a muscle biopsy

so we thought we would do all three at once and reduce

the anesthesia exposure.

So the date was set.

It doesn't matter how 

"simple or routine" the surgery is

when your baby goes under for surgery its terrifying.

We arrived at the hospital at 8 am for a 11 o clock surgery

why they make you show up hours early to just sit in the waiting room 

and get more and more nervous is beyond me!

She was in a fantastic mood that morning and it broke my heart that she was gonna be in 

so much pain later that day.

 

 

The surgery started 2 hours late

cause they had to coordinate between 3 different specialties.

A jittery male nurse came in to take her back.

He took her out of my arms.

She screamed and reached for me as he carried her down the hall.

It LITERALLY took everything in me not to tackle him to the ground

grab her from his arms and flee the country.

They were gonna cut her.

They were gonna HURT her.

They were gonna put things in her.

I didn't like it at all.

Logically I knew she would be under for the procedure

and they would give her meds for the pain.

But I still wanted to "save her"

The waiting was hard. 

After it was over they came to get me and took me back to the recovery area

as soon as they opened the doors I heard this eerie scream/moan

I thought "wow whoever that is sounds bad".

they took me too her and I realized that noise was coming from her.

She was really out of it from the anesthesia and I could tell she was hurting.

once she was stable they took us to our room.

 she was really outta it all day

she wouldn't sleep

and for the most part didn't want me to touch her

The night of the surgery she spiked a fever. 

Her stats were all over the place.

The next few days were touch and go.

She finally stabilized enough to go home 8 days later.

 It wasn't that big of an adjustment to go from an NG to a g tube.

Its really a neat lil invention.

Its just a tube with a balloon.

 

this is what the g tube looks like with the balloon deflated.

The first time I saw it I thought to myself

"That's it?"

I expected it to be a bit more complicated

This is the g tube with the balloon inflated.

That balloon is all that keeps it inside her.

There were also a few more supplies We had to have now

 

Two more kinds of syringes

 

split 2x2 gauze

And the extension set

This is what plugs into her g tube and attaches to her feed bag.

Well that pretty much sums up the feeding tube chronicles. :)

Iv port adventures

Lily was fitted with an Iv port on Nov 1st. Here is what it looks like:





She needed it for her ivig every three weeks and for her frequent IVs for dehydration. Also her veins are oddly deep and they roll and collapse. So every blood draw, every stick required her being held down for 45 min being stuck in every vein they could find until they found one that wouldn't collapse. It was traumatic for everyone involved.  The last iv she was given before her port surgery was the worst. I watched as three nurses held her down and stuck her over and over and over and over. she screamed and fought with every ounce of her strength. she had just started saying mama that week and screamed "mama mama" until she lost her voice. After they were done i picked her up and she just sunk into my arms, and stared at nothing. She barely took notice of toys, of people passing. She just stared. For a day and a half when she wasn't sleeping she was staring into nothing. Any question we had about weather the port  was a good idea had been settled. So she had the surgery. She was already going under for a g tube and a muscle biopsy so adding a port wasn't too big of a deal.However the port comes with complications. For every fever she has to be hospitalized for at least 48 hours to make sure the port isn't sending an infection into her heart.

 Th red arrow is pointing to the bump of the port that is visible just under the skin the purple arrow is pointing to the catheter that runs up her vein. This was taken a few hours after surgery.

 My finger is pointing to what her port looks like now that the swelling is gone.

 in order to place it they also had to put a small slit in her neck.

this is what the port looks like when its accessed. they use a special 1/2 inch Huber non boring needle.

  And then there was the unexpected complication. She had a staph infection that wouldn't go away. On DEC. 8th she spiked a high fever. They admitted her for iv antibiotics and to be monitored. with in 12 hours her fever was gone...
I had noticed the few weeks previous she kept grabbing her chest and moaning. She was more fussy than normal and would sometimes even fall over screaming and grabbing her chest. I told the ER doc and they didn't care. I told the admitting doc, he didn't care. I told her first day nurse...she didn't care. We finally got an awesome nurse the day they were gonna release Lily from the hospital. I told her and she said she had noticed it earlier when she was watching her so i could take a bathroom break. Then Lily did it again in front of her.

You can see the pain in her eyes in the pic as she holds her chest. The Nurse called the resident doc (who is AWESOME! she hasn't been a doctor long enough to get jaded and stop caring as much...). She ordered an x-ray. The x-ray showed the cath from the port had slipped far into her heart. She needed surgery. They booked the OR for the next possible opening. The moved us down to the cardiac ward to monitor her heart. They said they wouldn't know if any damage had been done until they got in there. There was a possibility it had fused to her heart. We asked a friend to come that night to help give her a blessing. The blessing she was given brought so much peace. The spirit was so strong. I knew she'd be fine and things would go smoothly. Early the next morning they sent her off to surgery. It went smoothly and quickly. The surgeon was able to go in through the old slit in her neck and correct it from there. She was monitored for another 24 hours to make sure her heart was ok and then sent home. We were so blessed. It could have been so so much worse. at the very least, the surgeon said he was sure she would need a whole new port. He said he didn't know how she got so "lucky". It wasn't Luck, it was the blessing. The Lord heard our prayers and granted peace and healing. I give Thanks to My Heavenly Father for blessing her that day and every day. He knows our desires and answers our prayers. I am so so so grateful!