Some Not-So-Good news...

Lily's GI Tract seems to be shutting down.

she is barely tolerating 15ml/hr of gtube feeds.

She needs AT LEAST 45ml/hr to survive.

So we will be going home on TPN.

TPN is feeding a person intravenously, (IV) bypassing the usual process of eating and digestion. The person receives nutritional formulae that contain nutrients such as glucose, amino acids, lipids and added vitamins and dietary minerals.

You can read more by clicking here

I am VERY unhappy about this.

If we cant get her back onto g tube feeds this will destroy her gallbladder, liver, possibly kidneys and possibly cause more heart issues.

But at this point it is LITERALLY life or death.

So the good thing is TPN is gonna keep her alive

and it doesn't change HER quality of life all that much,

actually she is happier and has more energy when she is on it.

Its just The Long term effects of it that are really really bad...

 And her risk of infection has just increased drastically.

And then there are other added problems that come with having a port accessed 24/7.

Like what happened this morning for example:

We went up to the playroom this morning and Lily was VERY happy to be there

then she started grabbing her port and saying "OW"

I checked her port and the dressing was all wet,

which meant she had infiltrated

(the tpn and lipids had gone into her skin surrounding the port instead of into her bloodstream

I wrote a blog about this happening last time but it was only IV fluids not TPN you can read that post by clicking here)

I knew infiltrating TPN was BAD so I grabbed Lily and her IV pole and

all but ran back to her room.

The nurse saw me rushing and ran to ask whats wrong and 

all I said was "her port infiltrated, it's TPN"

That nurse called over to a bunch of other nurses as I got Lily back to her room.

By the time I had her in her bed and had gotten her shirt off there were 4 other nurses and 4 or 5 Dr's

and a couple techs all scrambling to help.

(I knew it was a big deal but had NO IDEA it was THAT big of a deal)

The good news was It had only just started to  infiltrate so there shouldn't be enough of the tpn

in her skin to kill all the tissue.

Yes, that's right ,TPN when it gets into the skin kills the tissue.

Then there was the issue of trying to save the port so she didn't have to have immediate surgery.

Luckily we were able to un-acccess her port (pull the dressing off and needle out)

and re-access (put a new sterile needle in and redress it) with only a few complications,

but after 3 or 4 scares her port was working again, so No surgery needed!

And the good news is her blood sugar stayed relatively stable the whole time!

The thing that really struck me was it took 4 people to un-access and re-access her

and they are sending us home with this for me and Shaun to do ourselves,

and that's assuming that Shaun isn't ever at work when there is an emergency...

They are planning on sending us home with nursing for 1 day a week

which helps but emergencies never happen on a schedule..

I am just a bit overwhelmed at everything I have to do for her medically now.

Her nurses here in the hospital get frazzled with all Lily has going on

(Her trach, g-tube, hypoglycemia, port, tpn, meds, therapies, Ivig, and such...)

 and they expect

me to go home and deal with it all by myself when Shaun is at work.

Yes, I am overwhelmed at the very thought...

But, it will all work out.

It just will.

God will make us equal to all that is expected of us,

all I have to do is lean on Him and He will get us through this.

I COULD NOT DO THIS with out my Heavenly Father.

It just would not be possible, nothing would.

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.

:'(

TPN vs Neocate THE BATTLE

Working Lily off of TPN 

was much slower than last time.

Her Tummy just didn't want to accept food AT ALL.

The large yellow bag is TPN the white syringe is Lipids

In order to sustain life Lily needed to get to a rate

of 45ml and hour for 24 hours a day,

7 days a week into her G-tube.

(45ml is equal to 1 1/2oz.)

When she goes on TPN,

NOTHING goes into her G-tube.

So after she has had a few days with nothing in her tummy we 

try introducing food (her specialized formula) again.

We have to take it painstakingly slow.

AFTER being on TPN ONLY for 72 hours,

she started at 5ml/hour of formula (via G-tube) for 24 hours,

then every 24 hours we would up it 5ml.

Everytime we upped her formula rate we would lower the TPN a bit.

So to go from 0 to 45,

without any complications would take 

9 days...

And this is Lily we are talking about so

OF COURSE there were some complications.

Somewhere around 35ml

she started backing up stomach contents into her g-tube,

and then when I vented her some blood came up.

Luckily the blood only came up once so it looks like it was from the scope.

Then when we got her to 45,

She stopped tolerating completely.

Her stomach stopped emptying,

and her stomach became hard and extended again.

She was grunting and moaning in pain and her pupils were HUGE from pain.

So we had to back off a bit and turn the feed down.

Then she started having lock jaw.

About three times a day her jaw just locks open.

It doesn't seem to hurt her but

she does get frustrated with it and she has to physically

push it

( with my help)

 to get it to close.

Its only stuck like that for a few seconds

but when it does close there is a LOUD "Click"

The Doctors wanted me to take a picture of it

And I was fortunate enough to actually capture TWO. :)

We will be seeing an oral surgeon soon to see what (if anything) needs to be done

There is a possibility this will get worse over time, 

but hopefully its just a weird thing that will go away on its own as she grows...

OK, so back to her feeds.

When she stopped tolerating her feeds again,

The Dr came in to talk to me about 

Sending her home 

ON TPN.

I was NOT OK with that.

That would mean her port would be accessed 24/7

MAJORLY increasing the risk for infection.

An infection in the PORT 

IS BAD NEWS.

It would mean more trauma of dressing changes and

accessing and re-accessing the site.

It would mean home nurses.

Plus, I was against prolonged TPN use because of the damage it can do to the organs.

I just felt like sending her home on TPN was a bit drastic and 

while I am glad it is there when necessary

I don't think it was completely necessary to send her HOME on it YET.

So I told the Dr all this.

He was annoyed but agreed (after much persuasion) to consult with another Dr on it and also

give Lily a bit more time to tolerate her feeds so

we could get her on her needed rate of 45ml/hr.

Well later that day, she began tolerating much better,

and she did so well that we eventually were sent home

without TPN.

YAY!!!!

Yes her diarrhea is still there though...

She has had diarrhea for 70 days now

(with the exception of 1 or 2 days last month)

 I don't know what they are gonna do about it,

but we have many appointments over the next week so

hopefully we can get some more answers there.

So this hospital stay was just a bunch of

"waiting and seeing"

And I had to get very CREATIVE to keep Lily occupied.

 My friend Brandis brought Lily an awesome bag of little toys

and glow sticks and things to keep a toddler occupied while in the hospital.

She also brought us a red and white checkered plastic table cloth from the dollar store

to place on the ground to make a "sanitary" play place for lily on the hospital room floor.

 then I had Shaun bring two bags of beans to put into a bin for Lily to play with.

SHE LOVED them!

Here is the beautiful view from our room 

Phoenix

Helicopter???

this is our lovely prison...oops I mean Hospital room

PCH 6th floor

Happy girl

We had lots of visitors and watched ALOT

of Disney movies.

Tangled, Monsters INC, Finding Nemo, and Tinkerbell are her favorite

watching Tangled

We set up a bunch of pillows and toys in a wagon. And set the laptop so she could watch Backyardigans.

We also had the Joy of having Lily's Grandma-ish and Aunt-ish visit.

(Shaun's Aunt and cousin, but we call them Grandma-ish and Aunt-ish)

They had a trip planned for months and unfortunately we were in the hospital for almost

their whole visit.

We still had fun and Lily LOVES Her Grandma-ish!!! 

Lily is home now and very weak and clingy from spending 2 weeks in the hospital

but VERY VERY happy to be home.

Hotel P.C.H.

So last Friday Lily was admitted to the hospital again.

This is stay #17

We spend so much time here it feels

"normal" to be in the hospital.

 

Lily has a reputation as the "adorable sassy one",

And usually the first thing a new doctor says is

"OH, I have heard ALL about you Lily!"

And they proceed to tell  us about some cute story someone told them about

from one of our past visits.

 

I remember the first time we were transferred to PCH.

This place felt SOOOO BIG!

I remember thinking 

"how can there be this many sick kids in one city at one time???"

 

PCH doesn't seem so big now. 

 

Its funny how something so large and overwhelming

can become so regular and unimpressive.

Not that I am not SOOOO grateful it exists

and that we have access to it.

PCH is a HUGE blessing in our lives!

So, the reason we are back here again is:

Her diarrhea actually got even worse.

And she stopped tolerating ANYTHING in her stomach at all.

 She was in such pain that she just screamed and screamed.

When she would actually fall asleep she was breathing fast and 

whimpering the whole time.

AND  her stomach stopped digesting and emptying properly.

So, back to the hospital and back on TPN.

We started TPN on Saturday and they did more stool studies.

Of course the studies came back positive for C. Diff

But I informed the nurse it was my belief (and the Infectious disease Dr's belief) that

it was a false positive.

The Dr didn't agree and came to "convince" me that Lily needed

yet another round of Vancomyacin.

I told him it could wait until the scope (the next morning) because I did not think she had C. Diff.

And giving antibiotics unnecessarily is a bad idea and often does more harm than good.

He didn't like that, but he had no choice because I am the Mom and

I can refuse treatment.

 

They did the scope and took some stool from

inside the colon and took a bunch of tissue biopsies.

Everything came back normal!!!

which meant no C. Diff!!!!

I was so happy for about 15 minutes that

I had made the right choice in refusing the Vanco again.

I was proud of myself for saying "NO" right to a Dr's face.

 

But as the pride faded I realized...

 

this meant we had NO IDEA why she was having this diarrhea

and why she wasn't tolerating feeds.

 

She did have a UTI however, 

but that was secondary to everything else and a few rounds of 

yet another antibiotic cleared that right up.

 

So we added two new medications to try to see if that helps the diarrhea.

We are also slowly weaning off TPN as we are verrrrrry slowly putting her back on formula.

She isn't tolerating it very well though and every time they raise the rate on her feeding pump,

She becomes more agitated and fussy

And her tummy gets slightly more tender and more extended,

And the diarrhea worsens.

She had an ultrasound of her kidneys today just to make sure

that the UTI hadn't reached them and it was normal 

so that's good.

At this point its feeling like a guessing game.

One Dr said she may just have to "live with the diarrhea, and rashes."

Yeah... I am not ready to accept that.

This isn't your average lose diaper and rash.

This is watery, putrid stuff

that comes so violently and in such huge amounts that it fills the diaper and 

then shoots up her back sometimes reaching her hair...

If I hadn't seen it happen I wouldn't believe it.

And Its not just once or twice a day(unless shes on TPN of course)

If she is on her formula it is 10-12 times a day.

No, I do not believe I can just accept that there is nothing to be done...

 I know of a few things we haven't tried yet

and I'm not even a Dr...

But, I will not quit until we have exhausted every possibility.

 

That's part of the problem with the health care system.

The Doctors only hear reports or see test results.

They cant be there to witness everything like the nurses can.

So when the Dr hears about

"severe diarrhea, and a child in pain"

they don't get a clear picture of how it actually is.

And I feel like they are inclined to believe the parent is exaggerating.

Hearing is very different from seeing.

 

Any of the nurses that have seen the diapers,

 helped clean up the blow outs,

changed her sheets multiple times a day,

seen her screaming, 

watched her skin get worse and worse as the diarrhea returns as she is weaned off TPN...

Any of the nurses that have been there to actually witness it agree

 that it is severe and we CANNOT just give up.

But nurses only have so much sway with Dr's.

Luckily, I learned early in Lily's life

how to be an advocate for her 

and I surely wont stop now.

A 3 day stay turned into 10....

So what was supposed to be a short 3 day stay turned into 10 days.

She was admitted for TOTAL gut rest meaning they were going do TPN.

 So we got there and OF COURSE they didn't have the TPN ready yet

So we waited and got comfortable in our room for about ten hours

Until the TPN was ready.

She LOVES to play with foam tape to pass the time...

And what kid doesn't LOVE stickers?

 

They did a bunch of blood work and stool studies and

She tested positive for C. Diff.

 

"Clostridium difficile (klos-TRID-e-uhm dif-uh-SEEL), 

often called C. difficile or C. diff, is a bacterium that can cause symptoms ranging from diarrhea to life-threatening inflammation of the colon."-MayoClinic.com

The problem was she was ALREADY on the medicine

most commonly used to treat it (Flagyl) and was on her THIRD cycle of that med.

The doctors started her on Flagyl a FOURTH time.

They also sent in an Infectious Disease Dr. (ID)

After speaking with her ID, GI, and Attending

They all came to the conclusion

(after I suggested it)

That since we had already tried  Flagyl with little to no effect

they wanted to stop the Flagyl and switch to 

a much more potent antibiotic

Vancomyacin.

But an odd thing happened.

As soon as she went on TPN,

Her diarrhea stopped completely.

Now, if it was C. Diff it wouldn't have just stopped like that.

The ID docs AND GI BOTH agree it was probably a false positive.

But just in case they wanted us to finish out the 10 day antibiotic.

From there it was just a LOT of waiting.

She was on COMPLETE TPN for 3 days

Then 1/2 strength formula and half TPN for 24 hours

Then 3/4 strength formula and partial TPN for almost 36 hours,

Then they boosted her up to Normal formula at the slowest rate possible and weaned her off TPN.

As soon as they started her back onto the 3/4 strength formula the diarrhea came back.

It wasn't as bad, but bad enough.

We also found out she had lost almost 2 lbs.

She had been 22lbs 14oz

and was now 21lbs .

They wouldn't let us leave until she gained weight.

Oh and somewhere during the stay she developed a G tube infection.

Which meant 3 rounds of ANOTHER antibiotic.

I took this picture so I could remember how "fun"

it was to have a VERY MOBILE toddler attached to 

so many cords.

Now when she was younger there were times when she was attached to

just as many if not more.

And it was annoying.

But she moves SOOOO MUCH now.

There are seven or eight cords/lines in this picture.

I must admit I am so so glad we only have ONE at home now ;)

Oh and they also had her attached to TWO different IV poles.

It was a HUGE ordeal just to walk across the room.

I have never been more grateful for our laptop and Netflix than I was then!

She typically sleeps like this

Usually only if she is SUPER sick does she sleep on her back.
Unfortunately when she is all scrunched up the nurses think

 they have to turn her over to give her her meds.

When they do She wakes up.

When she wakes up she takes 2-3 hours to go back to sleep. 

Then she sleeps for an hour and its time for them to give meds again...

So after 5 nights in a row of this she slept a whole night like this

Sorry Its so blurry I didn't dare take another one for fear of waking her

For the most part we just tried to keep Lily entertained.

which was a bit of a challenge because we weren't allowed to leave the room.

 We made diaper hats...

  and tutu Lions...

 We set up a fun toy area in her crib 

and set up the laptop so she could watch her shows and Mommy could

take an HGTV break ;)

 

Oh, and we learned to fly by watching

The land before time...

When we finally did get to go home they sent her home on a 24/7 continuous drip.

That means she is ALWAYS attached to her feeding pump.

AND it means NOTHING by mouth.

I am sad that she no longer is able to have anything by mouth.

Not even her formula...

It is so hard to have her asking for food and trying to sneak food

when we can't let her have any.

If she sees us eating she opens her mouth wide and signs "Please"

it breaks my heart.

                                                   Hopefully this will only be temporary.

But this means we got a new pump!!!

The previous pump was temperamental and heavy and bulky.

The new pump is small and works upside down or on its side

and it comes with a backpack so she is completely

independently mobile! 

 

Here she is dancing to Pandora's "toddler radio" wearing her new pump.

(once again sorry it is sideways my phones camera is old and doesn't let me switch it :/ )

Hospital stay #16 TPN

I am sad.

Very sad.

I try very hard to count our blessings and

look on the bright side,

but sometimes I just have to feel sad.

Tonight I am allowing the tears to fall.

Call it weak if you may,

But I think it is appropriate and 

actually healthy for me to feel

sorrow occasionally given the circumstances.

Yes I am fully aware that things could be 

Much much much  worse.

I tell myself that daily.

But we all have our own individual trials

And for me,tonight, this one is difficult.

That does not mean I am going to wallow for days.

Tomorrow morning I will get up, 

put on my brave face and smile until the smile becomes genuine.

But tonight I have to get all this out

or I cannot be the Mommy and wife that

My loves deserve.

And I wont be able to be strong

for the days ahead.

I am sad because

Once again Lily is going into the hospital.

This is her 

16th admittance!

Tomorrow morning they are admitting her

for at least 3 days.

They are giving her complete Gut rest,

which means no food or liquid into her stomach or bowels

AT ALL.

In order to accomplish this they will be putting her on TPN

" Parenteral nutrition (PN) is feeding a person intravenously, bypassing the usual process of eating and digestion. The person receives nutritional formula that contain nutrients such as glucose, amino acids, lipids and added vitamins and dietary minerals. It is called total parenteral nutrition (TPN) or total nutrient admixture (TNA) when no food is given by other routes.

TPN is an artificial method of feeding, fully by-passing the GI tract. This unnatural way of feeding the body is far from perfect and comes with several significant complications"-Wikipedia

As you know from my last few posts Lily has been having severe diarrhea,

And has been acting odd.

She is increasingly fussy and clingy.

 She is having a hard time standing up straight and 

seems to have severe discomfort in her tummy especially her g tube area.

Oh, and I think she has lost weight.

She was completely into 18 mo size shorts

but they are falling off her and she is back into shorts

and pants she had previously outgrown, and her ribs are poking out again.

Now it is possible that she just grew in height therefore stretching

her out a bit,

But she doesn't seem much taller...

We have already tried partial gut rest 3 times since the Fundo.

(just pedialite for 24 hours, then half strength formula for 24 hours)  

The idea of a gut rest is to give the stomach and bowels time to 

"rest" and recharge so when food enters them again they can work properly.

Its the same idea as when a cell phone is acting weird,

 so you shut it off and turn it back on again.

Well that's the idea I guess 

But there is a problem,

It works for a few days but then it just keeps coming back.

And of course her RASH!

It is STILL THERE

and getting much worse now.

Now instead of a nice big rash she just looks raw.

This morning when I was trying to change her diaper

She was in such pain she was screaming

"Ow OW! Why Mommy Why why why why??"

I could only cry and tell her I was sorry and was trying to

figure out how to help her.

They  will also be doing lots of blood work and stool studies of  course.

 Now, I knew the moment I heard the Mito test results on the phone, those many months ago,

that our life would be FULL of hospital stays, Dr visits, therapy's,

 and one new symptom after another.

But to actually live it...

To have it actually happen and keep happening...

I don't really have words for it.

I feel like I am constantly holding my breath for the next hospital stay.

For the next "symptom"

and the knowledge that it is not going to get better anytime soon

is always staring me in the face.

 

This is Life with Lily.

However painful it is,

No matter how many tears I shed.

I am made better just by knowing her.

She is INCREDIBLE

She is like a shooting star

lighting up my whole sky.

I marvel at her strength, beauty and intelligence.

And I am so grateful and HONORED

that The Lord trusted us

to be her parents.

I am forever changed because I know her.