They REALLY messed up this time...

Well Lilys tube change was a bit of a disaster.

I would be lying if I said I wasn't upset about it.

It should have been a simple quick 10 minute procedure.

Unfortunately, things went wrong...

That seems to happen a lot when Lily's health is involved...

What should have been a simple quick procedure turned into

45 minutes of torture for Lily,

leaving her writhing in pain and seriously traumatized.

When they tried to place her GJ

a couple of things happened.

First when they removed her G tube she bled A LOT,

which isn't unheard of, but isn't typical either.

Then when they tried to put the GJ tube in they realized that Lily's anatomy

was different in her bowels and instead of curving it takes a sharp right angle turn.

So that made placing it very difficult.

All of that couldn't be prevented and no one is to blame for any of that.

The part that went horribly wrong was

Lily was awake.

Like wide awake.

Now for a typical button change or a 

TYPICAL GJ change being awake is fine. 

there should only be minimal discomfort if any at all.

But this wasn't a typical button change, and 

Lily IS NOT a typical patient.

And where things went horribly wrong was instead of stopping

and realising because of her anatomy this was gonna be difficult 

and pulling out and putting her to sleep, 

They proceeded.

 They held her down for 45 minutes poking and prodding at her

until it finally slipped into place.

When they brought her to me her whole stomach was stained in blood she was dripping with sweat

And Shaking uncontrollably and

SCREAMING in pain.

And I mean SCREAMING.

I asked why she was hurting and

 they said she wasn't.

They said she was just traumatized.

JUST traumatized?!?!?

I asked why she was all bloody and they said 

"I don't know she just started bleeding when we took the tube out and tried to put the new one in"

I told them to give her some Tylenol for pain and they said 

no.

Then EVERYONE disappeared.

They left us in the recovery room

BY OURSELVES

for 30 minutes while Lily SCREAMED in pain.

We looked everywhere for someone to help us.

They were not to be found.

Until someone came to get us and take us back up to our room.

As soon as we got there I explained to the nurse what happened.

She looked up Lily's records about the procedure.

And THERE WERE NONE!

(By this time folks I was LIVID, like shaking, tears in my eyes, could hardly speak, angry

but I kept my calm exterior for Lily's sake. She had enough to worry about and didn't need an angry Mommy biting peoples heads off...

OOOHHH but I wanted to...)

I told our sweet nurse that Lily needed pain meds like pronto and she agreed.

The problem was it was the Dr who preformed the tube switch that had to order meds.

Well and hour and a half later that Dr came up to talk to me

(only because our AWESOME nurse DEMANDED it)

I calmly but firmly told her I was unhappy and extremely disappointed and confused with how things were handled

and that Lily needed pain meds NOW!!!

All I was asking for was Tylenol

Lily was still shaking uncontrollably and SCREAMING and CRYING

"OW OW OW"

The Dr saw this.

She had to ask me to repeat what I said because Lily was so loud.

And she still tried to  say she wasn't in pain.

I told her it didn't matter if SHE thought Lily was in pain or not

I told her this was our 20th hospital stay.

I have seen how my daughter reacts to trauma,

I have seen how she reacts to pain,

AND I have seen how she acts when it is BOTH trauma and pain.

And that in this case it was both.

I told her 

I AM THE PARENT AND I SAY SHE GETS MEDS,

IF I SAY SHE IS HURTING THEN SHE IS.

(It sure helps to know hospital policy and know that she was breaking policy by refusing pain meds)

She just said OK and left.

An hour and a half later

Lily was FINALLY approved for TYLENOL!

By this time the nurse was fighting back tears, and so was the head nurse.

I don't think I had any left.

I was kicking myself for taking the baby Tylenol out of my purse just 2 days ago.

If I had had any I would have given it to her in a moment.

The Tylenol ,when it finally came, calmed her and she stopped screaming enough to sleep.

But every 4 hours, like clockwork, her heart rate rises, she begins breathing fast and

wakes up crying "ow ow ow"

Today I finally convinced them to give her something a bit stronger, and that helped.

Her smile came back and she started playing softly and gingerly with her Elmo.

But still over 24 hours later she is still hurting SO bad.

Two nurses and I filed formal complaints.

Her normal Drs were either to mad to speak, or literally in tears when I told them what happened.

It turns out that during the 45 minutes that they were

messing around in her, her stoma closed

(stoma is the hole surgically place for the feeding tube to go in) 

Then they just forced it back open

and she felt it all

Her GI Dr said she basically had G tube surgery while she was awake to feel it all.
He WAS FURIOUS.

 They also caused damage to her intestines.

She is barely tolerating feeds at a rate of 15 ml an hour.

She has to be at least at 45ml and hour before she can go home.

If she is still hurting in the morning they will have to take more pictures and may have to remove the tube and do it again (Under anesthesia and WITH pain meds)
  or she might not even be able to have one at all...

so that's that I guess,

We just have to wait and hope the damage isn't severe and will correct itself quickly.

I don't really know what else to say

except thank you all for your thoughts and prayers!

Stay #20 at Hotel PCH

OK here's the latest update on our sweet Little Lily Bug.

The week after she was released from the last hospital stay 

we had a ton of follow up appointments

(9 in 5 days)

At her IVIG appointment her port looked really red and she had a slight fever

and was acting odd so they wouldn't access her port to give the IVIG

until we had been checked out at the ER .

We went to the ER and spent 3 hours waiting for test results 

and to make sure she didn't have an infection in her port

(which is potentially deadly in a person with a REGULAR immune system)

 and for once nothing was wrong.

:D

So we headed back over to her IVIG.

IVIG went well and no other complications arose until Friday Morning.

In my last post I wrote how they finally sent us home with a monitor to tell me if 

she stops breathing in her sleep.

Well its a good thing they did.

Friday morning around 6ish her alarm went off.

I ran in and she was grey and not breathing.

I touched her and she opened her eyes and took a big breath and cried.

It seemed like it wasn't a bad event at all

because she wasn't even blue yet,

just all over grey,

plus she was incredibly easy to pull out of it.

But she hasn't had one of these events while she was sleeping yet..

(at least not as far as I know) 

and it concerned me.

Luckily we had an appointment with her Pulmologist (lung/airway Dr)

that morning so we made the hour long drive to the dr.

She was in a great mood happily talking and singing to me the whole way.

We got to our appointment and went back into the room.

Lily was standing happily coloring when she slammed her crayon down on the 

table and started yelling in a terrified voice

"NO NO NO NO!" 

Her voice got raspy and she went ridged and fell straight as a board backwards into my arms.

She was gasping for breath.

I started to yell for help. 

She turned blue

stopped breathing and passed out.

She had felt it coming...

I don't know how, but she somehow knew it was gonna happen.

The Dr came running in and found her unconscious in my arms.

I put her down on the table

and let the Dr take over.

She woke back up and stared at nothing for about a minute then

burst into tears and cried for 20 minutes.

After actually seeing one of the events her Dr was even more concerned than before.

He sat down and we had to talk about what these events meant for Lily.

He had mentioned a couple of times before that he thought Lily might be a candidate for a Trach.

A basic picture of a Trach

I had always listened to what he said but thought he was 

overreacting and a Trach seems SO EXTREME.

Well, it is extreme,

Very extreme.

But these events are getting more frequent and are getting worse.

He now was/is fully suggesting a Trach for Lily.

And it just FELT WRONG.

My brain was scrambling to think of something we hadn't tried.

Anything at all.

Yes the idea of a trach scared me,

but so did the g tube originally, and the port and the Fundo etc...

But with those they felt right despite of my fear.

This seemed to hasty.

Then I remembered someone, I don't remember who,

(possibly my friend Brandis)

Had mentioned trying a GJ tube with continuous venting.

I mentioned it to her Dr and he paused for a moment and thought

then looked surprised and said

"Well that might just work, Its definitely worth a try."

Now why in the world would a feeding tube make any difference in weather lily can breathe or not?

Well, The results of the last bronchoscopy 

(lung scope)

Did show large amounts of stomach acids all throughout her airway and in her lungs.

And during her last EGD (Stomach scope) they witnessed her refluxing past her Fundo.

Somehow even though her Fundo is perfectly intact and almost too tight, 

She is still refluxing.

That leads us to believe

that it is still the stomach acids that are causing the

laryngospasms

So, If we remove anything and everything from the stomach,

That should stop these events.

So Today Lily is being admitted to the hospital for a GJ tube.

In a GJ feeding tube, her formula will be fed into her intestines

bypassing her stomach completely.

Since all her feeds will be going into her intestines we will then be draining her stomach

of all gastric acids 24/7.

If there is nothing in her stomach she cant reflux right?

 This is our last ditch effort to prevent a Trach.

A Trach means lung infections for an already immune compromised little girl.

It means no swimming, EVER.

It means possible permanent damage to her windpipe, and vocal chords.

If she does have another "event" after getting the GJ tube

we will have to trach her.

This GJ tube MUST to work.

It simply MUST work.

If it doesn't... well We will tackle that hurdle if it presents itself.

As for now, we are just getting a GJ tube.

I CAN do hard things with the help of The Lord.

I know The Lord is mindful of Lily. 

I know things will go according to his will.

He is in control and I take comfort in that.

This life was not meant to be a walk in the park.

It is the refiners fire.

We are being shaped into better people through every trial.

And our suffering will be but a moment

• Alma 33:11

  "11 And thou didst hear me because of mine afflictions and my sincerity; and it is because of thy Son that thou hast been thus merciful unto me, therefore I will cry unto thee in all mine afflictions, for in thee is my joy; for thou hast turned thy judgments away from me, because of thy Son."

So NOW they finally take me seriously...stays #18 and 19

So I haven't blogged for a while.

Things have been...busy...

So let me catch everyone up on the past month.

On June 30th we were finally approved by Lily's Dr's to fly so we flew up to Utah.

Shaun's sweet Grandma was dying and she wanted to meet Lily before she died.

So We jumped on a plane and made the short hour long flight to SLC.

Lily did great on the plane, and I was very excited about it.

We drove straight to Grandmas so we could see everyone.

And it happened again.

 

She stopped breathing.

 

Turned blue and passed out in my arms.

 

Shaun took her laid her on a bed and started CPR.

But the rescue breaths couldn't get passed her throat.

Something seemed to be blocking her airway.

So he did some chest compressions 

Which did the trick. 

She started breathing and woke up.

She cried while I just cried and held her.

(I am so glad Shaun had JUST renewed his CPR certification the week before)

We called the Drs back in Phoenix and they decided it was probably just an isolated incident

due to the change in pressure and altitude from the flight.

And once she stopped crying she was fine.

(I must admit, I started to second guess myself and wonder

if this was just a "breath holding spell" and not laryngospasms despite the fact that her airway seemed to be blocked when Shaun was giving CPR...

but more on that a bit later)

We had a nice visit and it was GREAT to get to see family.

Although it was heartbreaking to see Grandma dying, 

We were so glad Lily got to meet her, 

and everyone else she hadn't had a chance to meet yet!

On the 4th of July we went to a park for a BBQ

and Lily had her first taste of playing in the sprinklers.

The next day We went to a birthday party for Lily's adorable cousin.

At this party her diarrhea became much much much worse.

In the next 28 hours she had more than 20 diapers.

She was hurting. 

We had to take her to the ER.

Luckily there is a Children's hospital in SLC.

Most typical hospitals aren't equipped to deal with 

Lily's "complex medical nature" even those with pediatric wings

And so we were very fortunate to be near a children's hospital 

where they had the tools and knowledge to be able to accommodate

to her needs

(IE: the pediatric sized port, and g-tube)

We got to the ER and she was dehydrated

( No surprise there, who wouldn't be after diarrhea like she had?)

But she was also significantly anemic, 

and her blood sugar was 50,

which is low but not horribly low.

An ideal blood sugar for her would be between 80-110.

(remember that for later)

So they admitted her.

Primary Children's Medical Center is a great facility.

Even though it is TONS smaller than PCH,

I almost liked it better.

The Doctors were very respectful and it was nice to

get some fresh eyes to look at her and find some things we had been missing

(like the anemia, and possibly the blood sugar)

We were there for only 3 days.

Just long enough to get her stable enough and filled up with fluids

 for us to make the drive back to Phoenix.

We had to drive back because they said Lily shouldn't fly again because she had stopped breathing

just hours after getting off the plane.

On the way back to Phoenix

we stayed the night at Shaun's old friends house in Saint George.

That night Lily started holding her tummy and saying ow

So I vented her like I always do when she does that

and I got this out of her tummy

yup that's blood mixed with her formula

And this picture only shows part of it.

There was more all along the tubing and I had to fill 2 syringes because there was so much just sitting in her stomach.

All in all, I probably vented almost 4 ounces out of her tummy all mixed with blood.

The good news was after we talked to the Dr's they thought it was safe enough to bring her to Phoenix the next day and get checked out.

So that's what we did. 

We drove the rest of the way the next morning and Lily did great!

 And even better I didn't see anymore blood all the way home!

 

The Dr's back at PCH didn't seem to concerned when they saw her and they ended up sending us home

without having to admit us.

 

About a week later I vented blood from her stomach again.

I called the Dr, who called another Dr, 

who then called me and said they wanted to admit her for a BUNCH of testing.

I must admit, I was in a Circle K holding Lily when they called me.

I had just left the funeral of an old dear friend so I was already emotional,

But I totally embarrassed myself by bursting into tears in the middle of the store.

I just am tired of having 

SO MANY HOSPITAL STAYS.

And I hate that this is so hard on my sweet little Lily.

We got admitted around 4 on a wed, which meant nothing would really happen until the

next day except for xrays and some blood work.

Thursday also brought more sitting and waiting.

Friday morning we were scheduled for three different scopes and 

ANOTHER EEG.

Lily had been acting odd all morning on Friday but during the EEG she really started to scare me.

She was pale, and couldn't even sit up on her own, I had to hold her up.

Her eyes kinda glazed over and she stopped responding to my voice.

Here eyes were open but it was like she wasn't there.

 

I told the lady I thought something was wrong, but she said she was  just tired.

I then told another person that Lily was acting weird and was really scaring me

and they ignored me as well.

It wasn't until we got back to our room,

and her AWESOME nurse came in that someone listened to me

The nurse immediately took her blood sugar and she was 21!!

She called the Dr's and started giving her some emergency meds.

They were saying they didn;t know how she was still consious,

that she could slip into a coma or a seizure at any moment.

 

she had only been given 10% of the recommended dose of medicine

when they checked her blood sugar again to make sure it was rising...

It had jumped up to 385!!!

So they brought in another specialist.

Endocrinologists.

The regular Doctors also came in and sat down with me and told me that they were 

sorry I wasn't listened to before and they have it in my chart that

I am to be listened to, that I (as her mother) am good at reading the subtle signs of distress 

and that I will be taken seriously from now on!

 

BOO-YAH!!!

Anyways,

They now had to do a bunch of tests to figure out her new blood sugar issue before we could even think of going home,

but in order to do the tests her blood sugar had to drop again.

So We had to take her off her feeds and check her blood sugar every 1/2 hour until it dropped.

After a while she became dehydrated and stopped bleeding as much so they had to poke her 2-3 times every 1/2 hour.

By the next morning she had had 35 pokes throughout the night 

and her poor feet were stained in blood.

I HATED allowing them to do it, but we had to know how to treat the blood sugar issue

before we could go home.

Blood sugar issues aren't something to mess with.

Then because of the blood sugar issues we had to postpone the scopes that she had scheduled

until the following Monday.

 

She had three scopes planned.

An EGD(stomach scope with biopsies),

a sigmoidoscopy (rectum scope with biopsies),

and a bronchoscopy (lung and airway scope with biopsies).

 

So apparently during the procedure

(after she was asleep but before they had begun anything else)

She stopped breathing again.

Because they saw it they confirmed it was a laryngospasm.

The anesthesiologist left claw marks on her chin from where he had to

hold the oxygen mask on so tight and FORCE her airway open.

He said while it does happen while people are under anesthesia,

It was the quickest one he had seen,

They now have NO doubt that these events she has been having 

ARE LARYNGOSPASMS.

And I don't have to second guess myself either.

They said her larynx is incredibly touchy and it just likes to spasm shut at the slightest irritation.

They then proceeded with the scope and found out that she had

"large amounts of frothy liquid" all throughout her airway and into her lungs as well.

The tests on this "frothy liquid" are still pending, however they believe it is stomach acids

and that she is refluxing past the fundo

(which isn't supposed to be able to happen)

Anyway, she has a lot of liquid in areas it shouldn't be and that's a big concern.

 Then after we got her safely back in my arms and back into the room last night, she spiked a fever

then her heart rate rose to the 200's and her oxygen kept dropping.

She finally stabilized after a few hours and has been stable ever since

So that's where we are now,

just sitting in the hospital 

waiting for test results so

they can know how to treat her and where to go from here.

I do know we will now be checking her blood sugar at home regularly,

and we get to have her back on our "good friend" the apnea monitor while she is sleeping.

(she was on an apnea monitor until she was 11 months old)

Tomorrow we should start getting some test results.

They believe that she was/is in a "metabolic crisis"

which is unfortunately a part of Mito,

But it often means the disease is progressing.

I hope it never happens again...

Honestly I hope she never ever ever has another symptom again.

I wish it would just STOP.

It hurts my heart that she is only 18 months old

and has had 19 hospital stays.

But she is a fighter.

She has such a happy spirit and she is so strong willed.

She LOVES life and it shows.

playing peek a boo from her princess castle hospital bed

happy girl minutes before going back for the scopes

You would never know she had stopped breathing on an operating table less than 24 hours before this was taken

 You would never know from just looking at her

that this little angel is fighting for her life.

She is so strong.

I admire and respect her strength, her spirit.

I LOVE HER!