Re: Re: Blue nose??

[Guest guest]

A little off topic, but I remember when my son was young he was ALWAYS sick, he caught everything before his autism. Now he rarely gets ill or at least shows overt signs and when he does it goes fast. He is more of a respiratory illness person or allergenic now.

Now What is frightening the hell out of me is my daughter. SHE IS ALWAYS ILL AND VIOLENTLY, we were in the hospital since 700 last night, she had started on MONDAY with a throat thing and loss of voice, With a cough, by wednesday fevers, wednesday night and thursday she vomited once i thinkby Friday Morning she had started vomiting at 6 am and did every 15 minutes non stop until she was hospitalized at 700 pm for dehydration severe and potassium loss I have never been so frightened she finally stopped vomiting after rehydration for now at least. I keep hearing things are going around but my daughter gets everything and BAD and LONG last illess started with hairloss and then a month bronchitis that would nto go away with two different antibiotics one which we found out she had a VERY severe sulfur allergy that almost killed her. I am at a loss, nothing helps, TF nothing. I am stupidly enough pregnant again, by accident and petrified. I can truly say that if anything happens to my daughter or new son I probably will go insane. I would appreciate any help as doctors have been NONE., any ideas about mito issues.

crystal

[Guest guest]

Hi :-)

Thanks a ton for the feedback. Madison is very fare skinned also, so we

have always chalked her unusual color changes in part to this....being

thin/fare skinned makes sense it might be easier to see on her than

another child. I am with you though on when it gets BLUER what does

that mean???

Thanks for the feedback also. What would be the best way for me

to be able to check circulation here at home?? My thinking had been

either its the chronic dehydration or something autonomic happening with

her, or maybe a little of both....with her temp regulation out of whack

yesterday too, autonomic seems to be a good possibility. Oh, a side

question for you since you have been dealing with the autonomic stuff

for a while......I know that these kids sometimes run fevers more

frequently, but with Madison it seems as though she NEVER runs a fever,

even when we know she is sick and likely should be. Okay, I cannot say

never, but in her whole life, she has probably run a fever all of three

times, and while we have been blessed with a good immune system on her

so she has not been sick much, there have been plenty of times where one

would expect her to have had a fever when she has not. If anything, her

temp tends to run on the low side of the norm. Does this fit into the

autonomic stuff?? Thanks for helping hun!!

Thanks for responding too!! Yes, Madison looks AWFUL when she

starts to slip, and this happens numerous times a day. She'll be

running around looking so...healthy!!...one minute, and then suddenly

just sink, lay down somewhere, try to zone out completely, and those

dark circles are just suddenly there where they had NOT been moments

before!! We can literally watch it happen. She also gets even paler

during these times and SO limp. Sometimes she can lay down and within

half an hour or so get back on track, and then other times she is down

and out for the rest of the day. Lately its been an up and down thing

over and over all day long and its not something I like seeing happening

so much. Thanks again for sharing that you have seen a similar thing.

At what age did your sons nose stop doing this if you recall??

Thanks friends for the feedback on this one!!! Only place I can go and

ask something like this...others would think I was crazy!! LOL

BIG hugs,

Kass, proud mom to Chance(8) and Madison(4 1/2)-both unspec mito, and

Abby(3 1/2)

[Guest guest]

wow that is scary,

I have the big blue veins on the side of my head, so i think those are hereditary, but one thing i noticed about both my kids, is that sometimes they have huge pronounced veins in the chest and abdomen they dont have to be during any time specific.

Crystal

[Guest guest]

>It was so interesting reading about the blue veins on the forehead.

My daughter Leah has prominent veins on either side of her forehead.

I finally asked her neuro about it because I thought it was so

strange. Of course, when they need to put in an I.V. they all

practically salivate at those good veins to put in an I.V. but know

that they can't. Leah is a very hard stick when it comes to finding

her veins in her arms and such for not only drawing blood but to put

in an I.V. But the neuro stated she just has fair skin and they stand

out more. Do you feel that is something all mito kids have? Fair

skin? Seems like there are several in this post alone that stated

that fact.

mom to Leah

Ronan often gets a " blueish " color around his eyes, lips and nose.

> he seems to have good O2 levels depsite his color. We have chalked

> it up to his very fair, very thin skin. I'm not quite sure

sometimes

> he is " bluer " than other times though. He also have very prominent

> veins in the center of his forehead.

>

>

>

>

>

[Guest guest]

do you mind me asking what the disorder is and how you found out about it and what symptoms they have

crystal

[Guest guest]

My little boy has extremely prominent veins all over his body. It becomes

much more noticeable when he is ill - probably because that is when his bone

marrow fails and he needs blood.

He gets quite blue on the nose and around the mouth . When he was younger

the cardiologist and nurses were concerned about this. They did continuous

pulse oximetry monitoring and EKG's and echocardiograms and 48 hr holter

monitors on him . What we ended up saying was that it was autonomic and not

anything to really fret over. Often it was triggered by reflux and or just

plain old nausea without vomiting. With autonomic dysfunction this caused

sinus bradycardia (low heart rate but normal rhythm) and this was felt to be

why he would get that cyanotic.He continues to do it with nausea even at the

age of 4.

The veins in the abdomen and upper chest and arms are especially prominent

and that is due to liver failure. He has what is called portal hypertension.

Someone asked about ability to run a fever with autonomic dysfunction.

Sometimes there is not that ability or you might see it go up slightly for

just a short time and then actually drop to below normal. This can also be

caused by problems with the adrenal glands or the pituitary gland and the

body can not make enough of its own steroids to generate an immune response.

If my little boys temperature suddenly drops below 96 we do blood cultures

and a CBC, chem panel and lactate to look for infection and sepsis

syndrome - we treat it the same as if he had a temp over 101. This is also

seen in newborns in the ICU - when they are in septic shock they will often

drop their temperatures rather than run a fever.

Anne - mom to Brittany, Zachary, Abby and Sammy

Re: Re: Blue nose??

> >It was so interesting reading about the blue veins on the forehead.

> My daughter Leah has prominent veins on either side of her forehead.

> I finally asked her neuro about it because I thought it was so

> strange. Of course, when they need to put in an I.V. they all

> practically salivate at those good veins to put in an I.V. but know

> that they can't. Leah is a very hard stick when it comes to finding

> her veins in her arms and such for not only drawing blood but to put

> in an I.V. But the neuro stated she just has fair skin and they stand

> out more. Do you feel that is something all mito kids have? Fair

> skin? Seems like there are several in this post alone that stated

> that fact.

>

> mom to Leah

>

>

>

> Ronan often gets a " blueish " color around his eyes, lips and nose.

> > he seems to have good O2 levels depsite his color. We have chalked

> > it up to his very fair, very thin skin. I'm not quite sure

> sometimes

> > he is " bluer " than other times though. He also have very prominent

> > veins in the center of his forehead.

> >

> >

> >

> >

> >

[Guest guest]

Crystal,

Just a note of encouragement and understanding to you. We have found out that our both of our two children (1 confirmed, 1 presumed) have a mito disorder. It is a heartbreak and it is terrifying. Perhaps this will not be the case with your other two children but how can we not wonder.... Just wanted to reach out to you.

, Mom to Seth (10) and Ben (5)

Crystal wrote:

I can truly say that if anything happens to my daughter or new son I probably will go insane. I would appreciate any help as doctors have been NONE., any ideas about mito issues. crystal

[Guest guest]

In a message dated 09/22/2001 10:39:06 AM Eastern Daylight Time,

kass@... writes:

<< At what age did your sons nose stop doing this if you recall?? >>

Kass:

I wish I could recall but he is 6.5 now and it is very subtle but I see it in

his school picture taken in May.

I also see the dark circles form under his eyes in a matter of minutes. I

think it may have to do with gi/vagus nerve involvement.

Michele Sullivan

Mom to Nolan (6) and Alana (5)

[Guest guest]

Thanks Anne SO much for the feedback!!!!

We just happen to be seeing a new cardiologist this next week and I

think we will discuss things a little more and see if there is any need

for further testing on her. We know she has Mitral Valve Prolapse

with mild to moderate regurgitation and this is what we are currently monitoring

with the cardio, but maybe there are a few other things we can do to be

sure that what we are seeing is autonomic and not cardiac in nature.

New doc is supposed to be VERY good, so we'll see what she has to say.

I personally think its the autonomic stuff so am not pushing to do more

testing right now, but we'll see what she has to say and go from there.

Thanks a ton for the help with understanding the fever and autonomic

components to all of this. We have learned over the years now to

not depend on fever in her to know she is really sick, although there are

still those that just cannot accept that she isn't running one and still

needs some help.....grrrr. Just wondering which type of thermometer

you have found to be the most accurate in monitoring this??? We really

are gonna have to start getting a bit more proactive in checking her blood

work so we can get some handle on whats happening to her....something to

discuss with the new ped this week. Thanks again for the feedback

on this hun!!!

BIG hugs,

Kass

PS...oh yes, and we are gonna be doing some more liver function testing

on her too this week as her PT and PTT came back mildly off in some previous

blood work and we need to do atleast the bleeding time test before we head

into her g-tube surgury. Hoping its nothing, but better safe than

sorry!!

Anne K Juhlmann wrote:

My little boy has extremely prominent veins all

over his body. It becomes

much more noticeable when he is ill - probably because that is

when his bone

marrow fails and he needs blood.

He gets quite blue on the nose and around the mouth . When

he was younger

the cardiologist and nurses were concerned about this. They did

continuous

pulse oximetry monitoring and EKG's and echocardiograms and 48

hr holter

monitors on him . What we ended up saying was that it was

autonomic and not

anything to really fret over. Often it was triggered by reflux

and or just

plain old nausea without vomiting. With autonomic dysfunction this

caused

sinus bradycardia (low heart rate but normal rhythm) and this was

felt to be

why he would get that cyanotic.He continues to do it with nausea

even at the

age of 4.

The veins in the abdomen and upper chest and arms are especially

prominent

and that is due to liver failure. He has what is called portal

hypertension.

Someone asked about ability to run a fever with autonomic dysfunction.

Sometimes there is not that ability or you might see it go up slightly

for

just a short time and then actually drop to below normal. This

can also be

caused by problems with the adrenal glands or the pituitary gland

and the

body can not make enough of its own steroids to generate an immune

response.

If my little boys temperature suddenly drops below 96 we do blood

cultures

and a CBC, chem panel and lactate to look for infection and sepsis

syndrome - we treat it the same as if he had a temp over 101.

This is also

seen in newborns in the ICU - when they are in septic shock they

will often

drop their temperatures rather than run a fever.

Anne - mom to Brittany, Zachary, Abby and Sammy

[Guest guest]

Thanks Michele!!!

Thanks for trying to think back for me. Would love to know more

about the GI/vagus nerve involvement if you have a chance to share....this

sounds very interesting to me, and I'd love to understand better how the

dark circles come on so quickly and are so dramatic when they do.

BIG hugs,

Kass

PS..interestingly, we see this alot with my older child with mito too......he

is not as physically affected by it, but they come on suddenly and we are

taking them much more seriously now than we ever did before.

michsull@... wrote:

In a message dated 09/22/2001 10:39:06 AM Eastern

Daylight Time,

kass@... writes:

<< At what age did your sons nose stop doing this if you recall??

>>

Kass:

I wish I could recall but he is 6.5 now and it is very subtle but

I see it in

his school picture taken in May.

I also see the dark circles form under his eyes in a matter of

minutes. I

think it may have to do with gi/vagus nerve involvement.

Michele Sullivan

Mom to Nolan (6) and Alana (5)

[Guest guest]

Hi, Crystal...

I think this question is for me so I will answer. We believe the disorder is a COQ10 deficiency which prior to supplementation caused a deficiency in Complex III. Since we have supplemented the COQ10 and Complex III levels were normal in the muscle, as in the blood. This is a new area of testing (COQ10) and I don't think it is tested routinely.

Seth's symptoms include problems with fine and gross motor skills, speech disorder, global developmental delays, fatigue, GI problems and a seizure disorder. When he started losing muscle strength at age 6, the neuro decided to do a muscle biopsy. That showed the complex III deficiency and COQ10 deficiency in the muscle. We then started supplementation of COQ10 and Carnitor. His endurance, muscle tone and strength improved. I think without them Seth would be in a wheelchair at least part time now. The later biopsy and further tests showed improvements but has not solved all problems. He still has the same problems and sometimes adds a new one. Ben, our second child is 5 years younger. At 3 he started having seizures, too. He has a speech disorder and some minor motor delays. He has been found to have some processing delays, too. He is presumed to have the same thing.

When Ben had his first seizure, it broke my heart and I was crying, "No - not a seizure! My baby! This can't be happening!" The paramedic who was there said, "Maam, we see seizures all the time. This is just a seizure!" I said, "I do not see seizures all the time. My other son has what may be a fatal disease and this seizure probably means he has the same thing so don't insult me by saying this is just a seizure!" After that, they went on to have to bag him and the seizure lasted nearly a half hour, grand mal.

I was devastated. Most of the time, my boys do well compared to many others with this disease, but it affects them, and us, in every way. We have to accept over and over again that our lives are different from others'. It is hard.

, mom to Seth, 10, and Ben, 5

-----Original Message-----From: csacco29@... Sent: Saturday, September 22, 2001 8:31 PMTo: Mito Subject: Re: Re: Blue nose??

do you mind me asking what the disorder is and how you found out about it and what symptoms they have crystal Please contact mito-owner with any problems or questions.

[Guest guest]

We just use a cheap old electronic one from the drug store. I think it is less than 10 dollars. I have taken their temps with it and then with the hospital therms and they seem to be about the same.

anne

Re: Re: Blue nose??

.. Just wondering which type of thermometer you have found to be the most accurate in monitoring this??? We really are gonna have to start getting a bit more proactive in checking her blood work so we can get some handle on whats happening to her....something to discuss with the new ped this week. Thanks again for the feedback on this hun!!!

BIG hugs, Kass