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HI Keely,

I don't believe I've introduced myself. I'm and I'm 20 yrs old. I became suddenly ill at the age of 15 and have gotten progressively worse in symptom presentation. I have a severe carnitine deficiency caused by a carnitine transporter defect in my renal tubes. I also am being evaluated for a mitochondrial depletion syndrome as I had fewer mitochondria in my fresh muscle biopsy than anyone else ever biopsied at CCF. My primary symptoms are severe muscle pain, weakness, fatigue (very sleepy all the time regardless of how much sleep I have had), intestinal dysmotility and pseudo obstruction, tension headaches, dysautonomia, severe osteoporosis, POTS, hypermobility of joints and reflexes, and frequent UTI's.

Tell me about yourself. What are your symptoms? Do you have a definitive Dx yet?

:)

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Hi !

its nice to meet someone my age! :)

I started having heart palpatations at 13, but they were basically

ignored by my doc. in fact nobody took me seriously at all until I

was 18... by that time I had severe scoliosis, recurrent hip

bursitis (to the point i needed crutches), asthma, severe allergies

(latex will kill me quite quickly - this came out of nowhere at 18),

chronic broncitis, my kidneys bled about 50% of the time (as in bled

enough that I could see it, no microscopic stuff here!), however

culture after culture came back negative, and antibiotics never

resolved it. (all of this happened while i was away from home at

college... nice) I dealt with it all relatively well, but in my

second year of college, I had a severe bout with pericarditis that

landed me in hospital for a week... at this point i made plans to

move home (I was halfway across the country with no family, living

in a dorm).

Since then I have developed dysautonomia (Also in the form of POTS),

peripheral neuropathy, my kidneys have their 'attacks' about twice a

year, and last christmas I had my first culture confirmed kidney

infection that took a good month and IV antibiotics to kick. I have

had recurring pericarditis, however since moving to a dryer climate,

my asthma, bronchitis, and bursitis have largely resolved. I have

an 'infarct' on my MRI (nobody can tell me what that means), I have

also developed a pinhole in my retina, that will eventually need

surgical treatment, however right now its small enough not to be an

issue...

Im a grab bag of medical literature... lol!

Amazingly, in the middle of all that, I managed to meet a guy that

didnt get scared away by the fact that our first date ended in an

ambulance ride, and a night in the ER (thought id get all my health

issues out on the table right at the start, see if he could handle

them... lol!) we got married, and have a 19 month old baby boy, who

is absolutely amazing.

Until January, I was told over and over my problems were autoimmune,

however in January my son got sick, and after all has been said and

done, were on our way in Sept to see the mito specialist, as he has

low carnatine, high lactic acid, abnormal urine organic acids, and a

whacky MRI, along with some rather random symptoms... I havent yet

been tested for anything associated with this, however we are

assuming its all the same root cause.

With all that, we also have gone through our family history, and I

have a cousin who died in the 80's of what now sounds like classic

Leighs syndrome (at the time they diagnosed atypical Reyes syndrome

for lack of a better diagnosis) I also have 3 other cousins whos

children are very sick, with random symptoms and no diagnosis, so i

suspect this has opened a door up that will benefit our entire

family...

You mentioned UTI's, are they confirmed by cultures all the time, or

do they go by symptoms? I will go to the ER, and teh initial urine

dip will show large amounts of blood, leukocytes, protein, nitrites,

and bacteria, however the cultured NEVER grow anything... its so

frustrating... and SO painful!

I had a cystoscopy (ugh) and it was negative, as well as xrays and

u/s of the kidneys, and my creatnine is always low, so theres no

signs of kidney damage. however I do have periodic protien in my

urine. NONE of that has ever been explained... Im hoping with time

it will be with a mito diagnosis...

I havent had a muscle biopsy, and my son hasnt either, however i

suspect its in the future for us both...

wow... sorry for babbling, there never is a short way to tell that

story....

Keely

> HI Keely,

>

> I don't believe I've introduced myself. I'm and I'm

20 yrs old.

> I became suddenly ill at the age of 15 and have gotten

progressively worse in

> symptom presentation. I have a severe carnitine deficiency caused

by a

> carnitine transporter defect in my renal tubes. I also am being

evaluated for a

> mitochondrial depletion syndrome as I had fewer mitochondria in

my fresh muscle

> biopsy than anyone else ever biopsied at CCF. My primary symptoms

are severe

> muscle pain, weakness, fatigue (very sleepy all the time

regardless of how

> much sleep I have had), intestinal dysmotility and pseudo

obstruction, tension

> headaches, dysautonomia, severe osteoporosis, POTS, hypermobility

of joints

> and reflexes, and frequent UTI's.

> Tell me about yourself. What are your symptoms? Do you have a

definitive

> Dx yet?

>

> :)

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Hope you don't mind me jumping in here. Wnated to welcome you

to the group and also tell you an infarct is another fancy word for a

stroke. If you need more info, let me know where your infarct/stroke

was seen and I will try to help you. You probably had a stroke that

seemed to cause neurological symptoms, possibly durin or following a

virus, at some point. Did anyone tell you that you had a stroke or

is this news to you? Let me know if you need anything. :)

By the way, I know what it is like to be struggling with these

issues at your age. I really went downhill at the age of 20.

Darla: mommy to Asenath, Zipporrah, and the other 7 kiddos

> > HI Keely,

> >

> > I don't believe I've introduced myself. I'm and I'm

> 20 yrs old.

> > I became suddenly ill at the age of 15 and have gotten

> progressively worse in

> > symptom presentation. I have a severe carnitine deficiency

caused

> by a

> > carnitine transporter defect in my renal tubes. I also am being

> evaluated for a

> > mitochondrial depletion syndrome as I had fewer mitochondria in

> my fresh muscle

> > biopsy than anyone else ever biopsied at CCF. My primary

symptoms

> are severe

> > muscle pain, weakness, fatigue (very sleepy all the time

> regardless of how

> > much sleep I have had), intestinal dysmotility and pseudo

> obstruction, tension

> > headaches, dysautonomia, severe osteoporosis, POTS,

hypermobility

> of joints

> > and reflexes, and frequent UTI's.

> > Tell me about yourself. What are your symptoms? Do you have

a

> definitive

> > Dx yet?

> >

> > :)

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My 'infarct' (lol sounds so pretentious) is in the right frontal

lobe of my brain, and is 8mm in diameter... basically the size of a

small pea...

I have never been told I have had a stroke, however there have been

a few times in my life that I may well have had a mild one, I have

had periods of numbness in my feet, and one time last year where my

whole left arm went numb (however a CT scan at the time was

negative).

they kept saying it must have been a head injury, however I have

only ever had one concussion in my life, and that happened AFTER the

MRI was done (ironic... lol!).

They estimated my grandmother had a total of over 100 (mini)strokes

in the last 20 years of her life... she went from being completley

healthy to completely bedridden over 10 years... it was so scary,

but it makes sense in hindsite.. they couldnt figure out why she

kept on having stroke after stroke...

After I had my son, my neuro problems were extreme, so that may well

have been the trigger...

Keely

> > > HI Keely,

> > >

> > > I don't believe I've introduced myself. I'm and

I'm

> > 20 yrs old.

> > > I became suddenly ill at the age of 15 and have gotten

> > progressively worse in

> > > symptom presentation. I have a severe carnitine deficiency

> caused

> > by a

> > > carnitine transporter defect in my renal tubes. I also am

being

> > evaluated for a

> > > mitochondrial depletion syndrome as I had fewer mitochondria

in

> > my fresh muscle

> > > biopsy than anyone else ever biopsied at CCF. My primary

> symptoms

> > are severe

> > > muscle pain, weakness, fatigue (very sleepy all the time

> > regardless of how

> > > much sleep I have had), intestinal dysmotility and pseudo

> > obstruction, tension

> > > headaches, dysautonomia, severe osteoporosis, POTS,

> hypermobility

> > of joints

> > > and reflexes, and frequent UTI's.

> > > Tell me about yourself. What are your symptoms? Do you

have

> a

> > definitive

> > > Dx yet?

> > >

> > > :)

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My UTI's are confirmed by cultures. Infact it's just the opposite for me that my UA's are always neg. and then my cultures grow these weird things. When I was in the hospital my urine was thrown out twice after the UA because it was clear even though a culture had been ordered. Frustrating considering I was bleeding and symptomatic and nothing ever shows up in the UA's.

You are so fortunate to have found a caring and supportive husband inspite of your illness. Congrats on the precious little boy. I'm really sorry that he is ill but I hope that you will be able to find some answers for him and yourself as well.

Where do you live? I live in western KY, but would love to move closer to the eastern coast.

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The right side of your brain controls the left side of the body

so the numb arm could have been the time it happened. But we have

had tons of experioence, like your grandmother, with strokes in our

home. They can be as mild as a droop of the mouth or as large as

causing death or major impairment. Asenath has had over 11 strokes

that we can date and many that have been tiny TIA's. Zipporrah has

had two moderate strokes and several tiny ones. I myself have had

many episodes in the past which were most likely stroke activity.

Now my oldest son (16) is having stroke symptoms with major migraines

and we are thinking it is related to the Mito disorder. I can't say

exactly what the front temporal lobe controls (that is one web page I

would LOVE to find!-one that tells the location of all control

centers in the brain for what issue) but know that many times strokes

can be so minor that many do not know it has occured. I would

suggest you talk to your doc further about the issue.

My worse periods are always following giving birth and dealing

with the lack of sleep, so this isn't surprising that your problems

have gotten worse after the stress of a pregnancy, birth, and caring

for a child. Try to get as much rest as possible and that will help

some. I am also wondering if your grandma has/had Mito.

Strokes/regressions are SOOOOOO common with Mito. Was she diagnosed

with anything?

Darla

> > > > HI Keely,

> > > >

> > > > I don't believe I've introduced myself. I'm and

> I'm

> > > 20 yrs old.

> > > > I became suddenly ill at the age of 15 and have gotten

> > > progressively worse in

> > > > symptom presentation. I have a severe carnitine deficiency

> > caused

> > > by a

> > > > carnitine transporter defect in my renal tubes. I also am

> being

> > > evaluated for a

> > > > mitochondrial depletion syndrome as I had fewer mitochondria

> in

> > > my fresh muscle

> > > > biopsy than anyone else ever biopsied at CCF. My primary

> > symptoms

> > > are severe

> > > > muscle pain, weakness, fatigue (very sleepy all the time

> > > regardless of how

> > > > much sleep I have had), intestinal dysmotility and pseudo

> > > obstruction, tension

> > > > headaches, dysautonomia, severe osteoporosis, POTS,

> > hypermobility

> > > of joints

> > > > and reflexes, and frequent UTI's.

> > > > Tell me about yourself. What are your symptoms? Do you

> have

> > a

> > > definitive

> > > > Dx yet?

> > > >

> > > > :)

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Im actually up in Canada, I live in Manitoba (just above North

Dakota)...

thats bizarre about the UA's!! nothing normal huh??

Yea Im so thankful for my husband and my son... i cant imagine

doing this alone... (although I wish it was just me and not our son

as well..)

> My UTI's are confirmed by cultures. Infact it's just the opposite

for me

> that my UA's are always neg. and then my cultures grow these

weird things. When

> I was in the hospital my urine was thrown out twice after the UA

because it

> was clear even though a culture had been ordered. Frustrating

considering I

> was bleeding and symptomatic and nothing ever shows up in the

UA's.

>

> You are so fortunate to have found a caring and supportive

husband inspite

> of your illness. Congrats on the precious little boy. I'm really

sorry that he

> is ill but I hope that you will be able to find some answers for

him and

> yourself as well.

>

> Where do you live? I live in western KY, but would love to move

closer to

> the eastern coast.

>

>

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Just thought I would let you know that is the pregnant

one. I had a baby just 9 1/2 months ago and am trying to " abstain "

from getting pregnant in the future due to the medical issues the

last two have had. Thanks for the suggestions though.

Darla

> I wanted to mention after you have this baby, if your blood

pressure

> issues are still a major problem, it may be worth it to ask about

> metoprolol...

> Im gathering from your posts that its not only low bp, but high as

> well that you suffer with? and thats why their hesitant to give you

> anything for either... as it might make the other one worse?

>

> The reason i suggest asking about metoprolol is its a strange one.

> its a beta blocker, and is traditionally used for high blood

> pressure, and heart rhythm issues, as well as after a heart

attack.

> for me, when the first med i was on was working, but not enough to

> control my symptoms they started me on metoprolol. the specialist

i

> saw told me that in people with high bp it lowered it, and in

people

> with low bp it seemed to raise it... their not sure why, but in me

> it worked!!

> For me i find it just 'smoothes out the edges' of how im

feeling...

> regulates my heart rate (even when my bp is fine, i have a very

fast

> heart normally), and keeps my bp in check...

> might be worth asking about at least!

> I hear about SO many people that have trouble getting anyone to

take

> this problem seriously, but it really does cause a major disability

> in a lot of people... and just like a painful disorder, this one

> deserves treatment if its severe enough to interfere with normal

> life.

> Keely

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LOL! oops!!

sorry im still trying to keep track of every one... ill get it

figured out sooner or later! :)

> > I wanted to mention after you have this baby, if your blood

> pressure

> > issues are still a major problem, it may be worth it to ask

about

> > metoprolol...

> > Im gathering from your posts that its not only low bp, but high

as

> > well that you suffer with? and thats why their hesitant to give

you

> > anything for either... as it might make the other one worse?

> >

> > The reason i suggest asking about metoprolol is its a strange

one.

> > its a beta blocker, and is traditionally used for high blood

> > pressure, and heart rhythm issues, as well as after a heart

> attack.

> > for me, when the first med i was on was working, but not enough

to

> > control my symptoms they started me on metoprolol. the

specialist

> i

> > saw told me that in people with high bp it lowered it, and in

> people

> > with low bp it seemed to raise it... their not sure why, but in

me

> > it worked!!

> > For me i find it just 'smoothes out the edges' of how im

> feeling...

> > regulates my heart rate (even when my bp is fine, i have a very

> fast

> > heart normally), and keeps my bp in check...

> > might be worth asking about at least!

> > I hear about SO many people that have trouble getting anyone to

> take

> > this problem seriously, but it really does cause a major

disability

> > in a lot of people... and just like a painful disorder, this one

> > deserves treatment if its severe enough to interfere with normal

> > life.

> > Keely

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