Update on

[Guest guest]

,

We started monthly IVIG in October and it is really helping, however, it is

always hair raising. We were going every 21 days which really takes its toll.

This month we are starting with every 28 days and hopefully it will work out

better for us.

I am still a novice at this and have learned all my knowledge from another

Mother on the list.

If I recall, Sam has had IVIG before. Does she tolerate it well?

It seems from the little bit I know, that its an either or type of thing.

Some people tolerate it well and are in and out in 8 - 12 hours. Some people

even get it at home.

Unfortunately, that is not our case. always has to be inpatient as he

had a bad reaction to his very first infusion. We will never know if this was

because he was sick when he got it or if it was something in the IVIG.

has only had one problem free infusion. We premedicate him with

Tylenol, Benedryl and Solumdedrol and then every 6 hours and at the end of

the infusion. Many times he ends up with a rash and when that happens he

comes home on Prednisone for 3 days after the infusion.

son also has issues where his blood preassure drops low and they have to

slow and/or stop the infusion. We titrate it up very slow - first hour 4 than

8 than 12 than 16 than 22 ml per hour for the rest of the infusion. For him,

this seems to be the thing that works the best.

Does Sam have a central line?

no longer has a central line because last spring he had septic shock

and we almost lost him. His immunologist and neuro will not allow another one

to be put in because of his poor immune system, We use a regular IV (which of

course never lasts the entire infusion) and he is a hard stick so it takes

longer for us than if you have a central line.

I hope that this helps. Feel free to email me if you want more info. I would

be happy to share.

Laurel

[Guest guest]

She caught another cold and had some

small bleeding from gtube site, but did not escalate past that.

's gtube bleeds quite frequently...he has had his november...I would have thought it would have healed by now. When they changed tubes he bled and bled and bled. He now has the button and it still bleeds but not quite as much.

He also get this mysterous orange oil (think it looks like CoQ but he isn't on that) in his tube...some have suggested that might be blood or unprocessed fats?

so how much bleeding are you talking about and how did they diagnose the bleeding problems? has prolonged bleeding time and frequently has off readings on HmG and Hematocrit...

thanks

deb

[Guest guest]

----- Original Message -----

To: <Mito >

Sent: Saturday, March 01, 2003 7:43 PM

> > >anne,> Thank you so much for the info. Sam did extremely well with her > infusion. How will I know if it helps?

Well when we first started it we asked ourselves questions about what would improve and then figured out objective ways to answer it. This we did because really we had no clue what it would help. We were advised to use the autoimmune sized dose of 2g/kg and so we figured it should do more than help their immune systems.

I would ask questions that have to do with her issues to see if it helps or not.

For example for Sam we asked:

will IVIG improve bone marrow function?

We decided to assess his cbc 2x a week and that way we could see what it was right before the IVIG, immediately after and then 1, 2 and 3 weeks later. After 6 months we felt we would say yes - it does improve bone marrow function temporarily but the effect wears off after 2-3 weeks.

We asked if it would improve GI function.

We decided to assess pain and rate of feeds.

For Sam it did not help.

For Zach he went from tolerating 5 cc an hour to 50 cc an hour. His pain decreased as evidenced by number of times/day he needed pain meds and he did not need mag citrate as much to treat constipation.

We asked if it would improve renal tubular function.

To assess this we measured the tubular reabsorbtion of phosphate and also looked at how much bicarb was needed in their IV fluids.

This improved with IVIG

WE asked if it would make their mitochondrial function better and looked at lactate levels as a sign of cellular happiness.

etc etc etc. I hope that helps - just ask yourself any question you want to answer and figure out reproducible, objective ways to answer it . The benefit here is that this info can be used to justify the treatment to insurance companies. Also, when I feel like I just can't do one more admission for IVIg (Ours are 5- 6 days every month) I look at the data and it reminds me why I am doing it.

hasn't shaken the cold and it will be 11 days since she got it. > Will the IVIG help her get over it?

Sometimes but it definitely should keep her from getting sick so much if she has a pre existing immune deficiency.

trying. Did you say that Sams coq10 level is ok?

My Sam's level is too low and I think your Sam's was low but can't remember now.

She wieghs > 27kgs so her dose really can go up more right?

Many MD's feel the dose can be as high as 10 mg/kg/day but I would do this in steps and not all at once. I would also split the dose into 3 x/day if you are not already doing this. It will facilitate absorption.

Hope this helps.

Anne

[Guest guest]

Hi All,

A quick update on - He had a very rough day on Monday - in and out of awareness and unresponsive at times. Tuesday he was more awake & alert, but was hyper/upset/off-the-wall, which caused him to crash and sleep a lot afterward. He had a lot of seizure activity last night, and today he has been awake but more subdued, laying around a lot. Hospice feels that though he seems a bit better than on Monday, overall his brain function is deteriorating and he doesn't have much time left.

Deb and Fred remain strong in their faith, but are struggling to know what to do to help him, and it is also hard for them to try to prepare and the other children for what's to come. Please keep the prayers coming!

[Guest guest]

,

Thank you for the update. I have been praying for the family, but mostly that find peace and comfort. It must be so difficult for them all, but for him to have to endure much pain would be sooooo unfair! is such a lucky boy to have been chosen by his parents.

mary b

Connor's mom - 9 1/2 years old - Leigh's/COX

Update on

Hi All,

A quick update on - He had a very rough day on Monday - in and out of awareness and unresponsive at times. Tuesday he was more awake & alert, but was hyper/upset/off-the-wall, which caused him to crash and sleep a lot afterward. He had a lot of seizure activity last night, and today he has been awake but more subdued, laying around a lot. Hospice feels that though he seems a bit better than on Monday, overall his brain function is deteriorating and he doesn't have much time left.

Deb and Fred remain strong in their faith, but are struggling to know what to do to help him, and it is also hard for them to try to prepare and the other children for what's to come. Please keep the prayers coming!

Please contact mito-owner with any problems or questions.

[Guest guest]

Oh my gosh-life is so difficult at times. How do you tell your child that

they are dying, that their time of living here is over very soon..., that

they will find peace where they journey to, that he will no longer have

to suffer. But what child wouldn't be terrified of the thought of being

with out Mom & Dad. This blasted disease..., may Deb, Fred, and

family find some peace, peace of mind, peace of heart. Someday........,

someday......, my prayers are with them. I wish I could do much more.

Mom to Red, Ada, Angel Lil

I recently found a poem about dying that I like. The author is unknown.

A ship sails and I stand,

watching til she fades on the horizon.

Someone at my sides says, " She is gone. "

Gone where?

Gone from my sight that is all.

She is just as large as when I saw her.

The diminished size, and total loss of sight is in me,

not her,

and just at that moment when someone at my side says, " She is gone " ,

there are others who are watching for her coming,

and other voices take up a good shout,

" There she comes! "

and that is dying.

~~~~~~~~~~`

All I can add is that surprisingly, there is " good life " still after

one's child dies. I find I can laugh just as heartedly as before. Yet I

do miss her so. Tough times are ahead for Deb, Fred & family. I so wish I

could do something to prevent them from entering the black abyss from

which I am emerging.

SAL

________________________________________________________________

The best thing to hit the internet in years - Juno SpeedBand!

Surf the web up to FIVE TIMES FASTER!

Only $14.95/ month - visit www.juno.com to sign up today!

[Guest guest]

Thanks so much for the update . I will continue to pray for the family.

[Guest guest]

my thoughts/prayers will continue for andrew and his family.

bethany

> Hi All,

>

> A quick update on - He had a very rough day on Monday - in

and out of awareness and unresponsive at times. Tuesday he was more

awake & alert, but was hyper/upset/off-the-wall, which caused him to

crash and sleep a lot afterward. He had a lot of seizure activity

last night, and today he has been awake but more subdued, laying

around a lot. Hospice feels that though he seems a bit better than

on Monday, overall his brain function is deteriorating and he

doesn't have much time left.

>

> Deb and Fred remain strong in their faith, but are struggling to

know what to do to help him, and it is also hard for them to try to

prepare and the other children for what's to come. Please

keep the prayers coming!

>

>

[Guest guest]

I remember when I met Deb at the UMDF conference last summer, she was

waiting for 's " official " diagnosis. She is such a strong and

neat gal.

I will continue to pray for Deb, , and Fred (and Gaige & Bliss,

too).

P.S. Anne, my kids also like Someday Heaven

[Guest guest]

:

My heart goes out to Deb and Fred and to the children. I believe

from a post that Deb had earlier I feel that knows what is happening.

It is amazing what not only kids feels, but adults when they are closer

to the end. It is the seizure activity that would have me worry about

how to help him. Just being there as parents for is what Deb

and Fred can do. I know when my dad was near the end just sitting

with him, reading the Bible to him ( he was a pastor) and singing his favorite

hymns to him just seemed to calm him. I even saw a tear roll down

his cheek the evening before he died as I was singing him songs.

Even though he was in a coma like state I knew he could hear me.

What is hard here is that besides being with Deb and Fred need to

be there for the children. They will know how to tell the siblings,

I just remember Bliss's name and not the other boy, about what is

going on. They will need a major hug time as they are doing the talk

and a box of kleenex and not to worry about crying because they need to

see mom and dad cry and that this is hard on them too. I truly believe

they are a strong family and have thought about this; but like every one

else when the time is actually here, it is quite hard to deal with.

Thank you soooo much for letting us know what is going on. My heart

stops a beat when I read about knowing what is going on. Thank

you for being there for Deb and Fred and their family.

Nerenhausen

mom to Leah

and Peabody wrote:

Hi

All, A quick update

on - He had a very rough day on Monday - in and out of awareness

and unresponsive at times. Tuesday he was more awake & alert,

but was hyper/upset/off-the-wall, which caused him to crash and sleep a

lot afterward. He had a lot of seizure activity last night, and today

he has been awake but more subdued, laying around a lot. Hospice

feels that though he seems a bit better than on Monday, overall his brain

function is deteriorating and he doesn't have much time left. Deb

and Fred remain strong in their faith, but are struggling to know what

to do to help him, and it is also hard for them to try to prepare

and the other children for what's to come. Please keep the prayers

coming!

Please contact mito-owner with any problems or

questions.

[Guest guest]

For those who believe in God and Jesus there is a wonderful comforting book

I would like to recommend for children:

Someday Heaven by Larry Libby.

A dear friend of mine sent it to me a few months ago and my children love

it, it is comforting but truthful and answers many questions that a child

might have about heaven and dying in child language.

Anne

Re: Update on

> Oh my gosh-life is so difficult at times. How do you tell your child that

> they are dying, that their time of living here is over very soon..., that

> they will find peace where they journey to, that he will no longer have

> to suffer. But what child wouldn't be terrified of the thought of being

> with out Mom & Dad. This blasted disease..., may Deb, Fred, and

> family find some peace, peace of mind, peace of heart. Someday........,

> someday......, my prayers are with them. I wish I could do much more.

>

>

> Mom to Red, Ada, Angel Lil

>

> I recently found a poem about dying that I like. The author is unknown.

>

> A ship sails and I stand,

> watching til she fades on the horizon.

> Someone at my sides says, " She is gone. "

> Gone where?

> Gone from my sight that is all.

> She is just as large as when I saw her.

> The diminished size, and total loss of sight is in me,

> not her,

> and just at that moment when someone at my side says, " She is gone " ,

> there are others who are watching for her coming,

> and other voices take up a good shout,

> " There she comes! "

> and that is dying.

>

> ~~~~~~~~~~`

> All I can add is that surprisingly, there is " good life " still after

> one's child dies. I find I can laugh just as heartedly as before. Yet I

> do miss her so. Tough times are ahead for Deb, Fred & family. I so wish I

> could do something to prevent them from entering the black abyss from

> which I am emerging.

>

> SAL

>

>

>

> ________________________________________________________________

> The best thing to hit the internet in years - Juno SpeedBand!

> Surf the web up to FIVE TIMES FASTER!

> Only $14.95/ month - visit www.juno.com to sign up today!

>

>

> Please contact mito-owner with any problems or questions.

>

>

[Guest guest]

Thanks again for the update. This is so hard to hear. I know the absolute sadness and even fear that must be going on in their family. I think there is also a part of me that fears facing these issues some day with my own daughter. It is just so hard some days to believe what is before my eyes. I say, she is just fine, and then she shows me she isn't. But it hurts so deeply to know one of our friends, who has been a vital part of our group, is sufferring so greatly. Please let their family know that we will continue to pray, askiing that the presence of God would surround them giving them peace and wisdom of speech to prepare the children for what lies ahead. Darla: mommy to Asenath (3) MNGIE Mitochondrial disorder...strokes, bi-hemi, seizures, migraines, g-tube fed, metabolic acidosis, carnitine deficiency, bi-carb/ketone issues... plus Luke, Leah, , Isaac, Tirzah, Kezia, and Marquis (and hubby-Dean) Update on Hi All, A quick update on - He had a very rough day on Monday - in and out of awareness and unresponsive at times. Tuesday he was more awake & alert, but was hyper/upset/off-the-wall, which caused him to crash and sleep a lot afterward. He had a lot of seizure activity last night, and today he has been awake but more subdued, laying around a lot. Hospice feels that though he seems a bit better than on Monday, overall his brain function is deteriorating and he doesn't have much time left. Deb and Fred remain strong in their faith, but are struggling to know what to do to help him, and it is also hard for them to try to prepare and the other children for what's to come. Please keep the prayers coming! Please contact mito-owner with any problems or questions.