AMELIE DISASTER

January 1, 2006

Dear All

Awful night Amelie went down hill rapid, they got a xray couldnt

understand what was wrong sats low 70's working hard with breathing

back from 1.25 litres of o2 to 5 litres and still in the 70's etc,

they have told us they dont think she stopped refluxing 6 weeks ago,

probably the drug change just made it slightly better in that we

couldnt see it come out her nose, her left lung has stuff on already

when 36 hours ago it was clear.

They have said almost certainly she will need to go to theatre soon,

as they think its still refluxing into her lungs silently. in the

meantime her feeds have been suspended and she back on Iv's

They say either re do nissen one possibility, or they may do a dis

sociation i think thats the word something about taking the stomach

away from the oesophagus so she will never allow milk back up the

oesophagus. how unbearable does that mean she would never get the

chance to eat ever?

Please is their anyone who has had the latter of the two done I need

serious advice, not that it will change anything, because they said

if its to keep Amelie alive they will need to do which ever one they

all agree with on Tuesday, and they said she cant carry on the way

she is doing as we no this last two arrest were the worst,

Well heres to the new year I feel devastated angry had enough fed

up, tears of joy yesterday tears of desperation today.

Lesley

January 1, 2006

Lesley-

How sad today. I do know one family in IA whose daughter had the

disconnect. I will forward your email to her to see if she catches it.

She's off and on with the list. The fact that she's off right now may mean

that she's not having time for email in general. Her daughter is 18 and

very involved. They didn't have the knowledge and early intervention that

Amelie will get. So if you do talk to Sheryl about , keep all of

that in mind. Her education was sorely lacking all thru -- so much so that

they have won legal battles against the schools for not providing

appropriate services.

As far as medically -- yes, it would be sad to know your baby will never

eat. But if it saves her life, a life without food is better than no life.

We think food is so important, but the folks who are still using feeding

tubes will tell you that it really is ok. Just another loss for you, Mom.

But Amelie will be fine with it. Heartwrenching, I know. I used to agonize

over " why can't or won't my baby eat?? " Couldn't for the life of me

understand how she could be born without innate hunger.

I'm so sorry and frustrated for you and with you that Amelie did not manage

better these past hours. Hang in there, Mama.

Hugs

Michele W

Aubrie's mom

January 1, 2006

Not much to say to make things easier, just know we are thinking of you and

little Amelie. She is a fighter so keep holding on to that.

You will be in our thoughts.

Murray

mom to twin Chargers and and wife to Jay

January 1, 2006

Please, please remember what Michele wrote. is 21, not the 18 of

Michele's friend, and very little was known about CHARGE back then. I'm

not sure that the experiences of parents of older CHARGErs matter at all

in this. No one did much of anything for in the early days, years ---

just hearing aids, etc. Things have changed, and my guess is that us

older parents won't be much help; remember that as you hear from

people and know that I'm holding your hand as I write. My thoughts are

with you. Martha

January 2, 2006

Dear Lesley,

has what they call a g-j tube. She gets all of her feeds through her

J-tube,which drips Pediasure into her small intestine. I do not have any

personal experience with the dissociation, but I do know about the J-tube feeds.

has ripped out two nissens and she would just continue because of the

episodes of vomiting and pain. She now has another diaphragmal hernia and there

is no more surgery for the nissen or hernia, this is the safest way to feed

her. It was a huge setback for us, I know what it is like to think that

may never eat or never know hunger , I felt like someone had just dropped a bomb

in our lap, it basically took me to my knees, but she is actually growing and I

think she is doing so much better than she ever has as far as feeds. The

g-j-tube was a simple procedure, they just went through her existing stoma and

guided the tube through her stomach, into her intestines, she was awake the

whole time though and we had to basically keep her busy

and sort of held her in that position unitl it was done. I wish I could be more

of a help to you, if I can be of any assistance at all, or if you have any

question about J-tube feeds, please feel free to e-mail me, I will try to answer

your questions the best I can. Maybe you can ask the surgeon about the g-j-tube

procedure and see what they say. The main thing is that Amelie is kept safe, so

the G-J Tube may not be good for little Amelie, just something to keep in mind

and to ask about.

I am sure they will most probably give her the same type of machine and

backpack that has. Make sure you ask them for the backpack and they can

also put in a special high calorie powder in her formula or pediasure, here in

the U.S. we call it duocal this is just in case she is limited on the amount of

fluids. gets most of her feeds at night. We can still offer her a

little bit of food by mouth, and keep up with her oral stimulation program

because the G-J Tube actually lets us still provide her with tastes, just has to

get all of her caloric intake through her J-tube. You should see her running

around with her little backpack on, she plays and does whatever and doesn't seem

to mind the backpack too much.

I hope you can get some help for Amelie soon, the quality of her life will

improve greatly, I just know it. Once, you see her thriving on the nourishement

and how happy she will be because of no more reflux, I know you will feel so

much better. It is all worth it to see a happy face. Please take care, and know

that I am praying for little Amelie and for you everyday. Sending best wishes

and butterfly kisses to little Amelie.

May you find strength and peace this New Year.

n-mom to --3yrs. old(CHARGE) and her 3 siblings and wife to

Lester.

Lesley Chan wrote:

Dear All

Awful night Amelie went down hill rapid, they got a xray couldnt

understand what was wrong sats low 70's working hard with breathing

back from 1.25 litres of o2 to 5 litres and still in the 70's etc,

they have told us they dont think she stopped refluxing 6 weeks ago,

probably the drug change just made it slightly better in that we

couldnt see it come out her nose, her left lung has stuff on already

when 36 hours ago it was clear.

They have said almost certainly she will need to go to theatre soon,

as they think its still refluxing into her lungs silently. in the

meantime her feeds have been suspended and she back on Iv's

They say either re do nissen one possibility, or they may do a dis

sociation i think thats the word something about taking the stomach

away from the oesophagus so she will never allow milk back up the

oesophagus. how unbearable does that mean she would never get the

chance to eat ever?

Please is their anyone who has had the latter of the two done I need

serious advice, not that it will change anything, because they said

if its to keep Amelie alive they will need to do which ever one they

all agree with on Tuesday, and they said she cant carry on the way

she is doing as we no this last two arrest were the worst,

Well heres to the new year I feel devastated angry had enough fed

up, tears of joy yesterday tears of desperation today.

Lesley

Membership of this email support groups does not constitute membership in the

CHARGE Syndrome Foundation or CHARGE Syndrome Canada.

For information about the CHARGE Syndrome

Foundation or to become a member (and get the newsletter),

please contact marion@... or visit

the web site at http://www.chargesyndrome.org - for CHARGE Syndrome Canada

information and membership, please visit http://www.chargesyndrome.ca or email

info@... .

8th International

CHARGE Syndrome Conference, July, 2007. Information will be available at

www.chargesyndrome.org or by calling 1-.

January 2, 2006

Dear Kristy, U know that in the horrible world of fear and doubt-there's

nothing

more comforting than the voice of knowledge.

When came home at 3 mos age, she had 3 pede icu nurses that covered

the night shift each week. What a god send-circumstances I' never seen or

experienced before, much less imagined, come up out of the blue. As involved

as

I was with her care in-hospital, without the comprehensive knowledge that U

have, and they had, to put things into context, I honestly don't know how we

would have been able to avoid re-admissions. Not to short change myself or

any other Charge mother here, we're a dedicated bunch, but we have to spend

so much time looking, researching, learning, hoping, and at best its still a

guess, or an intuition, but not a fact-and that makes U such an invaluable

partner to the chat.

If U would, I would appreciate Ur consideration of a situation has

pending. After a full-spine MRI, Di was found to have a chiari I

malformation, with a 25 cm. protrusion. She has minimal symptoms, and the

neuro surgeon

said she could wait till summer for the correction.

He also said he would have to cut the neck muscles. She has no headaches,

no muscle pain (she had 4 episodes of vertigo- vomiting, drop in heart rate

and b.p. I've read that it could at any time be a much more serious reaction,

and I guess for that risk alone we must have the surgery. I'm wondering if

she will need a halo, and I'm wondering if the post surgical results might be

scarier than the position she is in now? Is this something U have

encountered? (this is not arnold chiari-no spinal bifida, or hydrocephalis).

Forgive me for taking advantange of U this way...but if U can comment I'd

be so very grateful. Thank you, We all need you nurses.

January 2, 2006

Dearest ,

You will have to forgive me, but I work weekends in the Peds Intensive Care and

I won't be on the listserv until Mondays (and it was a hellish weekend in our

unit too-- a 14 hour Saturday and 17 hour Sunday for me). Zombie woman today.

I hope I write logically.

Re-do the Nissen fundoplication!!!!!!!!!!!!!!!!!!!!!!!!!!! Tyler was a silent

aspirator. Sneaky little baby. He was one of those lucky CHARGErs that had a

Nissen that broke loose. The second one has NEVER come undone. It isn't worth

the risks to not have it repeated. I will tell you I was scared to have him go

under anesthesia again (Tyler has bradycardia as a side affect). I would tell

any parent to re-do it.

Reflux is nasty. It does not only terrible damage to the esophagus, but scar

tissue to the lungs too.

I am so sorry that this happened.

Now I know why I had a shi--y weekend, I wasn't listening to Abba at work. We

just might have had a descent weekend. I think I might have to take my Abba

Gold CD next weekend and tell her it will help

Kristy

PS I am not making light of your situation. You keep your faith. Smile where

you can. We are part of your family too. We are your CHARGE family.

AMELIE DISASTER

Dear All

Awful night Amelie went down hill rapid, they got a xray couldnt

understand what was wrong sats low 70's working hard with breathing

back from 1.25 litres of o2 to 5 litres and still in the 70's etc,

they have told us they dont think she stopped refluxing 6 weeks ago,

probably the drug change just made it slightly better in that we

couldnt see it come out her nose, her left lung has stuff on already

when 36 hours ago it was clear.

They have said almost certainly she will need to go to theatre soon,

as they think its still refluxing into her lungs silently. in the

meantime her feeds have been suspended and she back on Iv's

They say either re do nissen one possibility, or they may do a dis

sociation i think thats the word something about taking the stomach

away from the oesophagus so she will never allow milk back up the

oesophagus. how unbearable does that mean she would never get the

chance to eat ever?

Please is their anyone who has had the latter of the two done I need

serious advice, not that it will change anything, because they said

if its to keep Amelie alive they will need to do which ever one they

all agree with on Tuesday, and they said she cant carry on the way

she is doing as we no this last two arrest were the worst,

Well heres to the new year I feel devastated angry had enough fed

up, tears of joy yesterday tears of desperation today.

Lesley

Membership of this email support groups does not constitute membership in the

CHARGE Syndrome Foundation or CHARGE Syndrome Canada.

For information about the CHARGE Syndrome

Foundation or to become a member (and get the newsletter),

please contact marion@... or

visit

the web site at http://www.chargesyndrome.org<http://www.chargesyndrome.org/>

- for CHARGE Syndrome Canada information and membership, please visit

http://www.chargesyndrome.ca<http://www.chargesyndrome.ca/> or email

info@... .

8th International

CHARGE Syndrome Conference, July, 2007. Information will be available at

www.chargesyndrome.org<http://www.chargesyndrome.org/> or by calling

1-.

January 3, 2006

ive had both the nissen and the jtube the nissen never really worked they

say the balve dont know how its spet maybe kristy or kim could help me with

the spellign but they say that valvle is just loose and flaps around kristy

if ur reading its the oneat the top of the oesophigus im talking bout well

that falps and i ve ahd j tubes which has worked keeping you all in my

thoughts hugs and kisses ellen my new year was great stupid since so many on

here had bad ones i feel bad bout that lol

>

> Dear Kristy, U know that in the horrible world of fear and doubt-there's

> nothing

> more comforting than the voice of knowledge.

> When came home at 3 mos age, she had 3 pede icu nurses that covered

> the night shift each week. What a god send-circumstances I' never seen or

> experienced before, much less imagined, come up out of the

> blue. As involved as

> I was with her care in-hospital, without the comprehensive knowledge

> that U

> have, and they had, to put things into context, I honestly don't know how

> we

> would have been able to avoid re-admissions. Not to short change myself

> or

> any other Charge mother here, we're a dedicated bunch, but we have to

> spend

> so much time looking, researching, learning, hoping, and at best its

> still a

> guess, or an intuition, but not a fact-and that makes U such an

> invaluable

> partner to the chat.

> If U would, I would appreciate Ur consideration of a situation has

> pending. After a full-spine MRI, Di was found to have a chiari I

> malformation, with a 25 cm. protrusion. She has minimal symptoms, and

> the neuro surgeon

> said she could wait till summer for the correction.

> He also said he would have to cut the neck muscles. She has

> no headaches,

> no muscle pain (she had 4 episodes of vertigo- vomiting, drop in heart

> rate

> and b.p. I've read that it could at any time be a much more

> serious reaction,

> and I guess for that risk alone we must have the surgery. I'm wondering

> if

> she will need a halo, and I'm wondering if the post surgical results

> might be

> scarier than the position she is in now? Is this something U have

> encountered? (this is not arnold chiari-no spinal bifida, or

> hydrocephalis).

> Forgive me for taking advantange of U this way...but if U can comment

> I'd

> be so very grateful. Thank you, We all need you

> nurses.

>

January 3, 2006

Wow,

First off. Ellen. I am so sorry that your nissen didn't work for you. That

sucks. The J-tube is bulkier--Tyler used to have problems with granulation

tissue around the stoma site. I hope you don't have that problem too. Now for

the Nissen fundoplication. This is where they take the fundus of the stomach

(the upper curve) and wrap it around the lower part of the esophagus and they

sew in place so that there small portion of esophagus going through the stomach

muscle. What is supposed to happen: there is a sphincter called the lower

esophageal sphincter (this is what is supposed to prevent reflux) it will

strengthen while allowing the esophagus to heal. It also helps some of our

CHARGErs from aspirating (like Tyler used to do--he had reflux that bad).

The j-tube goes into the jejunum which is the second part of the intestine. The

duodenum is very short and begins and the end of the stomach and ends at the

jejunum (have I confused you yet)!

I have to get to bed.

Kristy

Re: AMELIE DISASTER

ive had both the nissen and the jtube the nissen never really worked they

say the balve dont know how its spet maybe kristy or kim could help me with

the spellign but they say that valvle is just loose and flaps around kristy

if ur reading its the oneat the top of the oesophigus im talking bout well

that falps and i ve ahd j tubes which has worked keeping you all in my

thoughts hugs and kisses ellen my new year was great stupid since so many on

here had bad ones i feel bad bout that lol

On 03/01/06, mdlinda@...

> wrote:

>

> Dear Kristy, U know that in the horrible world of fear and doubt-there's

> nothing

> more comforting than the voice of knowledge.

> When came home at 3 mos age, she had 3 pede icu nurses that covered

> the night shift each week. What a god send-circumstances I' never seen or

> experienced before, much less imagined, come up out of the

> blue. As involved as

> I was with her care in-hospital, without the comprehensive knowledge

> that U

> have, and they had, to put things into context, I honestly don't know how

> we

> would have been able to avoid re-admissions. Not to short change myself

> or

> any other Charge mother here, we're a dedicated bunch, but we have to

> spend

> so much time looking, researching, learning, hoping, and at best its

> still a

> guess, or an intuition, but not a fact-and that makes U such an

> invaluable

> partner to the chat.

> If U would, I would appreciate Ur consideration of a situation has

> pending. After a full-spine MRI, Di was found to have a chiari I

> malformation, with a 25 cm. protrusion. She has minimal symptoms, and

> the neuro surgeon

> said she could wait till summer for the correction.

> He also said he would have to cut the neck muscles. She has

> no headaches,

> no muscle pain (she had 4 episodes of vertigo- vomiting, drop in heart

> rate

> and b.p. I've read that it could at any time be a much more

> serious reaction,

> and I guess for that risk alone we must have the surgery. I'm wondering

> if

> she will need a halo, and I'm wondering if the post surgical results

> might be

> scarier than the position she is in now? Is this something U have

> encountered? (this is not arnold chiari-no spinal bifida, or

> hydrocephalis).

> Forgive me for taking advantange of U this way...but if U can comment

> I'd

> be so very grateful. Thank you, We all need you

> nurses.

>

January 3, 2006

,

I am by no means an expert. I am by no means " the smartest nurse " either. The

only thing I possess over my most of my co-workers is empathy. I have BEEN

THERE. True compassion is deep. I went back to the hospital yesterday morning

at 9:20 AM (on my time) to give a pt with muscular dystrophy a bed bath and wash

his hair. I REALLY wanted to do it Sunday. It was just the day from Hell. I

was exhausted, but I promised him Sunday night/Monday morning @12:30 AM I would

be there between 8:30-9 AM--I was a little late. He can't even get his hands up

to his face anymore. He just recently got a trach and a peg tube to prolong his

life anymore. I cried on the way to work, partially out of exhaustion, the

Christian song on the radio, and because I know he will die before long. I had

to do this for him. I am not the smartest nurse, but I care and I will love

until I leave this Earth.

Now to you. I am not an expert in this field at all. We get very few of these

kids a year. She will not get a halo! The bradycardia and vomiting aren't

usual side effects that I am aware of---the others are. Again, I am not a

doctor, only a nurse. I am pondering if she if having a vagal response.

One thing that occur is scarring as the kids get older. How old is ? If

she is older, that is something that I might put a fire under their butts

(meaning an adult CHARGEr, but if she is younger you may be okay to wait).

I have a doctors appt, I will check back later.

Kristy

Re: AMELIE DISASTER

Dear Kristy, U know that in the horrible world of fear and doubt-there's

nothing

more comforting than the voice of knowledge.

When came home at 3 mos age, she had 3 pede icu nurses that covered

the night shift each week. What a god send-circumstances I' never seen or

experienced before, much less imagined, come up out of the blue. As

involved as