MUST READ!!!

Lily got together with her Tubie BFF"s last Friday

For a picture shoot.

Raya's Mom Brandis wrote about it on her blog 

And posted the AMAZING pictures she took!

Its AMAZING!

http://www.agirlandhertube.blogspot.com/2012/03/3-little-princesses.html

A story with a happy ending

It has been a very hard week or weeks actually,

"Logically" other weeks we have had have been worse,

much much worse...

but apparently my emotions don't respond to "logic"

(I am a woman  after-all)

I think it was a LOT of little things all building up.

And the anticipation of what was around the next corner was eating me up...

But, I am getting ahead of myself,

Let me just say now that

 this story has a happy ending! 

Lily has been on the edge of another admission for about two weeks.

 She has had a low grade fever for 2 weeks, 

It only has to raise .2 degrees for them to admit her.

yes that's only point 2 degrees.

Part of me thinks they might as well just admit her now and get it over with

but the other part of me (The larger part) is ECSTATIC 

We aren't there and maybe we wont get there this time...

 

Plus, that raging infection in her diaper area I wrote about last post

is still spreading,

everyday its creeping further down her legs and up her tummy.

The doctors don't know what to do about it because we are

on the last possible cream they know about to try on it.

If it hasn't significantly improved by Friday its probably an admission.

Shes refusing food more and more.

And shes waking up screaming multiple times a night again.

The past three nights Its taken four hours to get her to bed at night.

Half of which is spent with her screaming and crying,

While I do everything I can to calm her.

She has had FULL BLOWN meltdowns every time we take her anywhere

with lots of people.

So that brings me to yesterday.

One thing after another went wrong.

among about a dozen other things that will stay unmentioned,

we had a feeding therapy appointment which was missed due to

events out of my control,

 followed by an appointment with her

new developmental ped (Who we LOVE)

which we were a half hour late to, also because of

said events out of my control...

So there was a mix up in which doctor Lily was gonna see when we came in.

We saw a nice new doctor, who only fills in for the SN clinic when they are overbooked

She was unfamiliar with most of Lily's diagnosis'

and said she was sending us to the ER for labs and a probable admission.

She told me to wait for a few minutes and she's be back with the paperwork.

I sat there dejected, with tears brimming in my eyes threatening to spill over,

feeling completely overwhelmed and exhausted.

Then a  different doctor came in and said because of the time mix up 

he was actually the doctor we were supposed to see

and so we repeated the appointment with him.

At this point Lily started to perk up.

She started giggling instead of crying and her color was getting better.

This doctor was a little bit more familiar with some of her diagnosis,

and consulted with Lily's actual doctor

and came to the conclusion we could have four more days at home!

Unless her fever rose the .2 degrees of course...

Lily then started talking and kissing everything she could get her hands on

(Including the doctor)

And blowing kisses at everyone who wasn't within actual kissing distance.

She is really more aware than I give her credit for...

This raised my spirits.

I left PCH feeling better,

Lily took a nap on the way home,

which raised my spirits even more.

I went to the pharmacy and got her prescriptions.

As I drove home from the pharmacy,

I was feeling really really good.

I was feeling so capable and content.

In my own prideful way I started to pat myself on my back

And I was thinking how I could handle anything that was thrown my way.

Then I humbled myself as I realised the reason I was feeling so light

was because of the prayers that friends, family and perfect strangers 

are sending our way daily.

I COULD NOT do this without the Lord,

and without all the prayers.

I literally feel them. 

I desperately needed them yesterday and

They lifted me up.

Thank you for your prayers.

Thank you so so so much.

 

 Lily says "Thank You" too.

some good news and another diagnosis?

So I didn't post last week.

We had seven appointments in just three days.

It was a bit overwhelming.

This week we only have 4 appointments for Lily,

4 in a week is MUCH more manageable than 7 in 3 days...

So the good news is she is no longer delayed from a gross motor standpoint.

She will probably be released from physical therapy for a while so we can focus on ot and ft.

We are so so so pleased that she walks!!

The other good thing is we have IVIG tomorrow and

we went the

 whole three weeks

 without an ER visit or an admission since the last IVIG.

ITS A NEW RECORD!!

GO LILY!!!!

Now, for some not so great news.

Two weeks ago Lily had stopped tolerating her bottles and her g tube feeds as well, 

She went from tolerating 5oz in a bottle at one time back to 3oz at a time

if she accepted at all.

It took us 9 months to get her to accept more than 3 oz at a time and it

was incredibly frustrating to have her regress back.

Plus, she had crazy amounts of gas and pain

and had severe diarrhea

which burned her diaper area.

The Doctor said she had a bacterial overgrowth in her gut

 due to the huge amounts of antacid meds she is on

 combined with the iv antibiotics she has to get every so often.

So, They put her on Flagyl (a strong antibiotic) for 7 days

To kill the excess bacteria.

It seemed to work.

but her diarrhea never ceased.

About 18 hours after the last dose of Flagyl,

She spiked a fever.

It has come and gone for the past week.

It never stays long though so they don't feel she has to be admitted

(yay)

Then her gas came back

 and this time she has been vomiting during the night.

We saw the GI today

She said it was the overgrowth coming back so we will be doing 

a week of flagyl once a month now.

She said it was common in mito kids.

She also looked at the burn/rash she had from the diarrhea and

said it had turned into a raging infection and we had to start steroids right away on it.

She kept emphasizing it was bad and to watch it closely because of her immune deficiency.

Its treated with a good (painful) cleaning and steroids 4 times a day for a week.

Then the part I wasn't expecting..

Another diagnosis?!?

Yup, 

She said she had been suspecting for a while

 but had just confirmed that Lily has 

Visceral Hyperalgesia.

She then sat me down and told me that just from a GI standpoint alone

Lily was gonna have a tough road.

Things were gonna keep coming up and we were gonna have to follow her very closely.

Treating her will be tricky and difficult.

It will unfortunately take some trial and error.

This new diagnosis is just one of many to come as the mito progresses

And that's just from a GI standpoint.

Now she wants to see us every 4 weeks instead of every 6.

During this whole appointment,

 by the way,

Lily was having a total meltdown like she has every day this week

and was 

SCREAMING and CRYING

the whole time.

The GI did a fantastic job of speaking over the screaming.

That brings me back to her new diagnosis.

"Visceral Hyperalgesia,

 which may also be called Visceral Hypersensitivity,

 is a term that simply means an individual has increased sensitivity to pain

 in the visceral system of internal organs

 like the stomach, intestines, or pancreas.

 Normally, when one eats or drinks, 

the stomach and intestines stretch

 to accommodate the meal with no discomfort whatsoever.
But in a child with Visceral Hyperalgesia,

 the mere act of filling the stomach or intestine
with a small amount of fluid or food triggers the nerves in the gut to respond as if a
painful stimulus has been introduced.

What is painless to most children

 feels excruciatingly painful to children with Visceral Hyperalgesia.

 Children with this diagnosis

 commonly have pain responses to one or more types of agents: 

pain due to digestive processes such as food entering the gut 

or liquid stretching the gut; 

significantly increased pain due to infections, viruses, 

or other external insults on the gut;

 and a pain response to psychological events such as anxiety or
fear."-Complex child emagazine

So not only is Lily in pain whenever food hits her tummy

(which we kinda already knew)

She has a pain response to anxiety or fear.

This makes so much sense

 and breaks my heart.

She has a sensory processing disorder.

Fear and anxiety, are her constant companions.

She is getting overwhelmed almost every time we go to a store.

Sometimes, (Saturday morning or at church Sunday)
 she just shuts down and its not to obvious, 

other times like Saturday afternoon or Monday, or today at the Dr's,

She has full on screaming, crying, kicking, hitting, tearing at her skin, head banging fits.

Then she starts screaming "ow, ow ow!"

And cries more.

I knever knew what to make of it.

Now I understand.

Her body has a pain responce to fear and anxiety.

And I feel helpless.

I can only pray that God will help her.

The "good news" is she will now be starting a 

low dose pain medication.

At least she will get some releif.

Also, next month we will be starting her on pancreatic enzymes

To try to preserve her pancreus.

And we will be monitoring her Liver very closely now.

Pancreatitis, and or Liver disease are very common with kids with this 

AND

with mito kids...

Sometimes it feels like the deck is so stacked against us.

I just remind myself that

GOD is in control.

Its my new mantra.

"God is in control."

SHE WALKS!!!!!!

OH MY GOODNESS!!!!

SHE CAN WALK!!!!!!!!!!!!!!!!!!!!

We honestly didn't know if she would be able too!!

And She can!!!! 

This afternoon she was crawling, and then she just stood up

and started walking.

she was a bit shaky at first, but then she gets the hang of it.

She walked back and forth across our living room nonstop for 45 minutes!
We are so so so excited!!!!
AND SO WAS SHE!!!
She kept clapping and saying "yay"!!!
(Sorry the videos are sideways, they were taken on a phone and we are unable to rotate them)

Some days

Some days,

I look around my house at all the necessary medical supplies and I cry.

Some days,

Things seem to be going so well that I almost forget Lily is sick...almost.

 

Some days,

I feel so worn out I think I must be transparent.

Some days,

I just cant stop smiling because we are so blessed.

Some days,

the very sight of her tube makes me want to scream out loud in defiance of it all.

Some days,

her laughter is so infectious that I laugh till my sides hurt.

Some days,

I feel so tense from the anticipation of her "next symptom", 

that my heart might pound out of my chest.

Some days,

its hard to keep the happy tears from flowing all day long.

Some days,

Its hard to keep the sorrow tears from flowing all day long.

Some days,

I rejoice in my motherhood, and joy in every little task.

Some days,

 my heart feels icy with the fear of what the next moment will bring.

Some days,

I look at how far she has come and I marvel!

Some days,

I cannot do it on my own and I know my Savior is carrying me and Lily.

EVERY DAY,

I give thanks to my Lord and Savior for the blessing of Eternal Families.

EVERY DAY,

I LOVE my family.

EVERY DAY,

I find comfort in the phrase

"BE STILL and know that I am God"-Psalms 46:10

 

EVERYDAY,

I trust in God's will,

 with every stitch of my soul,

And I know I am nothing, and would have nothing without HIM.

Your questions answered!

Q: How long will Lily have the tube?

A: Unfortunately there isn't an exact answer. She will have the tube as long as she needs it to survive. At this point she isn't improving at all in her dysphagia, actually she is getting worse. If she keeps getting worse they are gonna take oral feeding completely away. And over the past couple weeks she has started refusing again. It took 9 months to get her to accept more than 3 oz at a time, and now she is back to 3 again. So, as of right now there is no end in sight as far as the tube feeding goes.

Q: How long do her feeds take/can she move around while being fed?

A: She gets fed for 10-12 hours continuously every night while she is sleeping, and for a while after she wakes up in the morning. She is on a relatively slow rate of 55ml(approx. 1.8 oz) an hour. During the day she has a goal of 12oz by mouth, I try and give her 3 bottles of 4oz each, but as she refuses I then have to bolus feed her (put the formula in a giant syringe and push it slowly through her tube). Occasionally it isn't safe for her to eat by mouth at all (like when she is super sick) So she sometimes has to go on continuous 24 hour feeds. She can move around while being fed I just have to follow her around holding the pump. As she gets older and hopefully stronger there is a backpack she can wear to make the pump more mobile.


Q: Can she be potty trained when she is old enough?

A: Maybe...kinda. It all depends on how weak her core muscles are and  weather she will have the ability to strengthen her muscles enough to be potty trained. Then there is the feeding tube. If she is strong enough there is still the issue of her being fed continuously while she is sleeping. From what I understand it is sometimes possible to potty train tube fed kids during the day but not a realistic goal for over night potty training. She will need diapers at least overnight as long as she is fed overnight...

Q: Does her tube hurt her/is it sensitive?

A:The tube doesn't hurt her unless she tugs on it or it gets caught on something. I have to spin it daily (which she doesn't even notice) and We have recently discovered she is super ticklish all around it and will actually laugh out loud when we poke her skin around it (laughing out loud is exceedingly rare for her).


Q: What is it that makes her able to handle some foods and not others?

A: Eosinophilic Gastroenteritis is the main culprit for her being so sensitive to so many foods. Because of her Eoe, she has many many allergies/intolerance's. Then there is also the issue of her dysphagia/silent aspiration, For things that are safe to eat from an allergy stand point, they still aren't safe for her to swallow. Like water for example, every bit of water that is swallowed goes into her lungs, so if I were to give her a 6 oz bottle of water she would drink it and would drown. On days when she is teething and has excessive amounts of drool we have to constantly listen to her lungs because it goes to her lungs and she doesn't consistently cough it up (silent aspiration).


Q:Will Lily be able to attend regular school?


A: Honestly, probably not. Her immune deficiency makes public school way to dangerous. Plus the nature of Mito is, it is progressive, and so by then she most likely will be sicker than she is now. Arizona's special education programs (in my opinion) are sorely lacking. She will either be home-schooled, or preferably she will go to a private school where they are equipped to handle medically complex children. So far it looks like she is cognitively fine, even gifted (YAY!) as far as intelligence goes, so that also poses a challenge for schooling her because finding a program that can tailor to her physical needs but still challenge her intellectually is hard to come by.


Q: Is Lily a million dollar baby? (Do her medical bills before insurance equal one million yet)?

A: If my calculations are correct, then yes. Just between her hospital stays and her IVIG alone equal 1 million. That doesn't even include all her doctor appointments, medicines, equipments, therapies, etc. 


Q:Are we considering having more kids?


A: This is a tough one. We definitely will have more kids there is not question in our minds about that, but as of right now it looks like we will be adopting. The percentage of having another child with Mito is extremely high, and well, lets just say it is not something we are taking lightly. The decision will be made through much prayer and meditation on the subject and ultimately we will do our absolute best to do God's will. 
 


Q:What does the future hold in store for Lily.


A: This is what my nightmares are made of now. Mito is progressive that much we know. But the thing we do not know is how fast it will progress or what will be affected next. Most common symptoms include:

• brain: confusion, memory loss, headaches, seizures, developmental delays, and stroke-like episodes
• nerves: pain caused by nerve abnormalities (neuropathic pain), gastrointestinal problems linked to nerve abnormalities, abnormal sweating, and fainting
• skeletal muscles: muscle weakness, muscle cramping, muscle pain, loss of coordination, exercise intolerance, and poor growth
• liver: liver failure and low blood sugar (hypoglycemia)
• heart: heart muscle weakness and disturbed electrical signals in the heart (called heart block)
• kidneys: abnormalities that cause difficulty with absorbing nutrients and electrolytes back into the body (called Fanconi syndrome)
• ears: hearing loss
• eyes: eye muscle paralysis, progressive loss of vision
• pancreas: diabetes (a group of conditions characterized by excessive urine excretion and persistent thirst) and pancreatic failure
• Sensory processing disorders, or autism

Other symptoms include failure to thrive in infants, poor growth, short stature, fatigue, respiratory disorders, swallowing difficulties, and increased risk of infection.

Read more: http://www.answers.com/topic/mitochondrial-disorders#ixzz1mV1wSD4r

Only 20% of children with infant onset Mito will make it to adulthood, and out of those 20% very very few will see their 30th birthday. It is a cruel and sinister disease.

Not two people get mito exactly the same way, and just because somethings aren't listed here doesn't mean it can't be caused by mito. Mito is a guessing game at best, and since there is not cure and no real treatment we just take one day at a time and we and her doctors are forced to watch every little change closely and decide if it is a symptom or a personality quirk or a "typical baby thing". Kids with mito can go from acting and seeming fine to complete system shutdown within hours. That is why everything is watched so closely. The sooner we catch a new symptom the sooner we can stop it or at least slow it down, if possible.

The future terrifies me. I am doing my best to live here and now, because that is where we are and that is where I have her safe in my arms. We have to just keep swimming. And we will accept God's will in ALL things. He is in control and He knows whats best in EVERYTHING. I trust him with my life, and soul. And I trust him With Lily's as well. I will have her as my daughter forever. That I know. And no matter how long any of us are on this Earth we will be together forever and ever in Heaven. 

Feeding tube awareness week - a day in the life & what I want clinicians to know

Today's topic is:   

What do you want clinicians to know about the day-to-day life with a feeding tube?  

This post for me is gonna be pretty short because

basically yesterday's post covered the majority of it.

I just want the doctors that don't understand feeding tubes to attend a seminar or something,

and for the ones that do,

like our fantastic GI,

Or our new developmental pediatrician,

I feel like they already understand

what day to day life with a tube is like.

well, as best as they can without  actually living it. :)

I am fine with the doctors not totally understanding being a parent to

a medically complex child.

Its not their job to be a parent that's my job.

I just need them to understand enough that they can do their job as a doctor.

That's all I ask.

And luckily we finally have a team of 

16 doctors and specialists

Who all understand their specialty

and are in fact fantastic at whatever their specialty is.  

Its only taken us a year to build this team, 

And that is a relatively short amount of time.

A LOT of families with medically complex kids look for years

before finding "the right mix of doctors"

if they ever do...

We have been truly blessed to have this after only a year.

********************************

The topic I missed was: 

Understanding Life with a Feeding Tube.

Explaining a day in the life/daily routine of tube feeding... and all it requires.

Well the biggest thing about
Life with Lily is she has different symptoms day to day

some days she acts almost completely healthy

most days she has just a few big issues

and other days she can hardly move

This really makes a difference in our daily routine.

I will try to explain our day as best I can...

8:00am-hopefully Lily is just waking up, with coo's and babbles,

if she wakes up screaming it is a sure thing it will be a VERY BAD DAY.

And we almost always will end up in the ER within 24 hours.

I turn off her pump unless I had to add more than her normal 18 oz to her night feed

I change her soaking diaper

check her temp

clean up any vomit

and if she fed the bed, clean that up.

I give her her morning meds then take off her extension

change her g tube dressing 

put ointment on her eczema spots and on her scars

and get her dressed

and lots of kisses and loves :)

Most weekdays we have a morning appointment

so we go to that

otherwise we go for a walk

which usually brings us to;

11:00-I give her more meds and

change her diaper.

If she feels warm I take her temp

11:10-She gets to have her first 4 oz bottle

if she doesn't finish at least 3 oz

I put it through the tube

Which entails attaching the extension, making more "non-thickened formula"

putting it into a 60cc syringe and slowly pushing it in

then unhook the extension, rinse it all out, and pray she doesn't puke.

11:40-1:00-floor time, or "therapy homework"

1:00-we can try to give her solid food if she is physically able to have it that day

2:00-Diaper change then Next bottle 

Whatever she doesn't finish goes through the tube

2:30-I try desperately to get her to take a nap

(It usually doesn't happen, but when it does she sleeps from 1-3 hours)

3:30-If she hasn't gone to bed by now she isn't going to...

So, if she is awake we go for a walk

or she gets play time, or whatever

This is the most unstructured part of the day

I LOVE this time of day

And if she IS sleeping I catch up on chores and if time blog,

or maybe just maybe read.

5:00 -time for her next bottle with special vitamins added

check temp and change diaper.,

5:30- work on "therapy homework"

6:30-bath time, lotion massage,

attach extension tape it down, and get in pj's

7:00-8:00- start putting her to bed depending how the day has gone will determine

weather its closer to 7 or 8 that I start trying.

Putting her to bed is a challenge.

Usually I rock her and pat her for 1-3 hours.

Sometimes I get lucky and she goes down really fast

(That happened three times this past week!!!)

But usually, It takes a lot of calming and rocking.

I would love to just put her in her crib and let her

"cry it out"

but because of her health issues I cannot.

I have tried it quite a few times and this is what happens:

Her heart rate rises way too much

and she screams until she vomits

and then screams BECAUSE she vomited

and then Vomits more and more

until her screaming causes an asthma attack.

Which needs an albuterol treatment which makes her scream,

then she becomes jittery and hyper...

Once she is asleep

(Usually about 10, but can often be 11 or 12)

I make 18 oz of formula, put it in her feed bag

attach the bag to the pump, prime the feed,

Put an ice pack in the bag with the pump,

give her her last med, attach the extension to the bag and start her night feed.

If she is coughing, and wheezing a lot I give her a breathing treatment

I then do any housework that needs to be done, 

and finally go to bed.

On most nights her pump will alarm, or she will wake up fussing or coughing or choking

and so I usually get up at least once a night.

That about sums up the AVERAGE day for us.