Autoimmune/Connective Tissue/Mito, Was Re: Anne and

[Guest guest]

Hi all :-)

This thread just got me to thinking and I am wondering how many see

some of the connections we're making over here. We have family history

of things like Fibromyalgia, Endometriosis, Lupus, Scleroderma, Dermatomyositis

on my (mom's) side, with Crohn's and MS on dad's side. I was told

at the conference in Dallas by a couple of the doctors there that they

are seeing more and more autoimmune components with the mito, though I've

not seen alot about it otherwise. We seem to like to have an inflammatory

response to everything!! Immune issues in general we're seeing with

Madison (just fights nothing off anymore) and my sister has always been

very sickly in this way. I don't think we've done any of the real

testing to see if there would be something there that would indicate a

true immune deficiency or not though.

Interestingly, looked into PANDAS ALOT when Chance got so bad .......his

tics increased dramatically and he certainly seemed to fit the profile,

all except there had been no signs of him being ill with strep beforehand

(course, he had strep earlier this year and NO ONE would have known had

his sister not been dx'd first).

Then we have this connective tissue thing becoming more and more prominent.

Chance and Madison both have a leaky mitral valve and regurgitation, both

are loose jointed, but especially Madison, and now their trachea's have

become floppy. Madison's skin biopsy was also very abnormal.

And finally, I just had to have all my teeth removed (at the ripe old age

of 35) and dentures placed because I apparently have Ectodermal Dysplasia

(our best guess for a name for it) and all my enamel on my teeth decided

to just disintegrate over the last few years.

Have no idea how it all fits together but keep thinking there must be

others out there like us and would love to understand it all better.

Not sure anyone has any thoughts for me here, but figured I'd toss it out

there and see.

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

McNair Family Home

Page

McNair Family Updates

United Mitochondrial Disease Foundation---

"I try to take one day at a time, but sometimes several days attack

me at once." Ashleigh Brilliant

Plant wrote:

Anne and ,

All three of my sons have evidence of autoimmunity. My son

with mito

has very high inflammatory markers and T-cell repsonses.

His brothers

both developed rheumatic fever post strep (even though the strep

was

treated with antibiotics). It is likely that they both had

a previous

mild strep infection that went undetected. In addition to

the

polyarthritis and chorea the boys also developed some PANDAS (Pediatric

Autoimmune Neuropsychiatric Disorders Associated with Streptococcal

infections) symptoms.

Anne, did you know that erythema nodosum has been linked to strep

infections and can occur in patients with rheumatic fever?

Studies at

the NIMH have shown some success in treating PANDAS and rheumatic

fever

with IVIG and plasmapheresis. I noticed that there is also

a link

between sarcoidosis and strep infections.

Here is some info on erythema nodosum, strep, and rheumatic fever:

Rheumatic fever, a streptococcal infection which

presents with fever,

erythema nodosum, patchy lung infiltrates, sometimes chorea and

myocardial symptoms. Rheumatic fever often occurs in

children after

the age of 3 years. Clinically there is a preceding sore throat,

accompanied by non-erosivearthritis of the large joints, fever

and skin

rashes. Cardiac manifestations include heart murmur as a mid-diastolic

mitral murmur (Carey Coombes murmur), cardiac enlargement (indicative

of

carditis), pericarditis and heart failure. A rising antistreptolysin

O

titre is a good indicator of streptococcal infection even though

the

organism may not be cultured.

http://www.medinfo.ufl.edu/year2/mmid/bms5300/bugs/strpyoge.html

Cutis. 1978 Jun;21(6):806-10.

Erythema nodosum. A review.

Soderstrom RM, Krull EA.

Erythema nodosum is not an uncommon dermatologic entity. Sarcoidosis

and

streptococcal infection have become the two most common causes,

while

tuberculosis was the predominant etiology prior to the use of isoniazid.

Histoplasmosis and coccidioidomycosis are two important geographic

considerations. Laboratory tests should include a PPD test, chest

x-ray,

throat culture for

beta-streptococcus, and ASO titer determination as a minimum.

Symptomatic treatment remains unsatisfactory in many cases, although

recent success has been reported with oral potassium iodide.

[Guest guest]

Kass, i was told i had a leaky mitral valve with regurgitation about 8 years ago. I had alot of palpitations too. The CoQ10 has improved this for me quite a bit.

has some hyperextendable joints - is this what you mean by flexible? My daughter and I can do some sitting positions most people find difficult.

As for teeth, I have had plenty of troubles. Not with the enamel, but i can't tell things are going wrong until it is too late. Last year, i got a clean bill of dental health on monday and tuesday was in severe pain and wednesday had a root canal! xrays on monday did not really show anything the regular dentist could see. Also, last summer my teeth were unexpectantly all loose. They firmed up, but the dentist was surprised because they were not usually like that. Haven't figured it out yet, but i have recently been having bruising problems again (from time to time this pops us, since college) and am waiting on test results. I've had several spontaneous bruises, and feel awful, and my gums are bleeding, yeah. On the mito oldies list, teeth problems were an issue we talked about and it seems pretty common.

I'm sorry to hear about your teeth and the problems your children are having. What is being done for the weak trachea? are they having trouble breathing?

cara

Autoimmune/Connective Tissue/Mito, Was Re: Anne and

Hi all :-) This thread just got me to thinking and I am wondering how many see some of the connections we're making over here. We have family history of things like Fibromyalgia, Endometriosis, Lupus, Scleroderma, Dermatomyositis on my (mom's) side, with Crohn's and MS on dad's side. I was told at the conference in Dallas by a couple of the doctors there that they are seeing more and more autoimmune components with the mito, though I've not seen alot about it otherwise. We seem to like to have an inflammatory response to everything!! Immune issues in general we're seeing with Madison (just fights nothing off anymore) and my sister has always been very sickly in this way. I don't think we've done any of the real testing to see if there would be something there that would indicate a true immune deficiency or not though. Interestingly, looked into PANDAS ALOT when Chance got so bad .......his tics increased dramatically and he certainly seemed to fit the profile, all except there had been no signs of him being ill with strep beforehand (course, he had strep earlier this year and NO ONE would have known had his sister not been dx'd first). Then we have this connective tissue thing becoming more and more prominent. Chance and Madison both have a leaky mitral valve and regurgitation, both are loose jointed, but especially Madison, and now their trachea's have become floppy. Madison's skin biopsy was also very abnormal. And finally, I just had to have all my teeth removed (at the ripe old age of 35) and dentures placed because I apparently have Ectodermal Dysplasia (our best guess for a name for it) and all my enamel on my teeth decided to just disintegrate over the last few years. Have no idea how it all fits together but keep thinking there must be others out there like us and would love to understand it all better. Not sure anyone has any thoughts for me here, but figured I'd toss it out there and see. -- BIG hugs, Kass, proud mom to Chance(9-Mito) Our future Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?) Our Drama Queen McNair Family Home Page McNair Family Updates United Mitochondrial Disease Foundation--- "I try to take one day at a time, but sometimes several days attack me at once." Ashleigh Brilliant Plant wrote: Anne and , All three of my sons have evidence of autoimmunity. My son with mito has very high inflammatory markers and T-cell repsonses. His brothers both developed rheumatic fever post strep (even though the strep was treated with antibiotics). It is likely that they both had a previous mild strep infection that went undetected. In addition to the polyarthritis and chorea the boys also developed some PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) symptoms. Anne, did you know that erythema nodosum has been linked to strep infections and can occur in patients with rheumatic fever? Studies at the NIMH have shown some success in treating PANDAS and rheumatic fever with IVIG and plasmapheresis. I noticed that there is also a link between sarcoidosis and strep infections. Here is some info on erythema nodosum, strep, and rheumatic fever: Rheumatic fever, a streptococcal infection which presents with fever, erythema nodosum, patchy lung infiltrates, sometimes chorea and myocardial symptoms. Rheumatic fever often occurs in children after the age of 3 years. Clinically there is a preceding sore throat, accompanied by non-erosivearthritis of the large joints, fever and skin rashes. Cardiac manifestations include heart murmur as a mid-diastolic mitral murmur (Carey Coombes murmur), cardiac enlargement (indicative of carditis), pericarditis and heart failure. A rising antistreptolysin O titre is a good indicator of streptococcal infection even though the organism may not be cultured. http://www.medinfo.ufl.edu/year2/mmid/bms5300/bugs/strpyoge.html Cutis. 1978 Jun;21(6):806-10. Erythema nodosum. A review. Soderstrom RM, Krull EA. Erythema nodosum is not an uncommon dermatologic entity. Sarcoidosis and streptococcal infection have become the two most common causes, while tuberculosis was the predominant etiology prior to the use of isoniazid. Histoplasmosis and coccidioidomycosis are two important geographic considerations. Laboratory tests should include a PPD test, chest x-ray, throat culture for beta-streptococcus, and ASO titer determination as a minimum. Symptomatic treatment remains unsatisfactory in many cases, although recent success has been reported with oral potassium iodide. Please contact mito-owner with any problems or questions.

[Guest guest]

Kass,

I thought of you when I was researching PANDAS and chorea. So many of

the symptoms of chorea and PANDAS look like the regression that Chance

and Evan had. Also mitral valve problems are so common in post strep

autoimmunity. I can send some the info to you if you are interested. I

contacted Swedo at the NIH and her research assistant called back

a few days later. She sent a packet that I shared with the

pediatrician.

I had no reason to suspect that Colin had strep--he went to the doctor

for his increased asthma symptoms. His breathing treatments seemed to

be making things worse not better. I was afraid that he might be

showing signs of mito. His clinical presentation of post strep loss of

muscle strength, tone, and motor control along with the dreaded tremors

was enough to qualify for rheumatic fever. His OT eval report (in May 2

months post strep) clearly showed that what Colin had was a textbook

case of mild to moderate chorea. Post strep chorea is enough to meet

criteria for rheumatic fever even if there is no heart involvement.

Chorea and PANDAS are both later manifestations, in some of the cases

reported in the literature the strep infections had gone undetected.

Here is a list of rheumatic fever symptoms--

Fever

Joint pain, which often moves from joint to joint

Joint swelling, which may be accompanied by redness and a sensation of

heat

Nosebleeds

Abdominal pain

Vomiting

Skin rash: broad, pink to light-red patches that increase in size and do

not itch

Small lumps under otherwise normal-looking skin

Unusual, involuntary jerky movements

Muscle pain

Confusion

Decreased muscle tone

Shakiness of one or more parts of the body

Speech difficulties

Coughing

Fatigue and weakness

Heart palpitations

Chest pain

Here are a list of symptoms associate with exacerbations of PANDAs from

one article--

emotional lability 66%

change in school performance 60%

personality change 54%

bedtime fears/rituals 50%

fidgetiness 50%

separation anxiety 46%

irritability 40%

tactile/sensory defensiveness 40%

impulsivity/ distractibility 38%

deterioration in handwriting 36%

oppositional/defiant 32%

deterioration in math skills 26%

nightmares 18%

Other articles also mention clumsiness, loss of self help skills,

hyperactivity, and problems with speech (esp. due to dysarthria). One

article that was a report of the in-depth investigation of 11 children

with severe chorea mentioned that 100% of the children had dysarthria.

" Dysarthria was manifest as slurred or incoherent speech and was present

in all cases, with two children rendered mute. "

Here is an interesting quote from another one of the articles--

" The nature of the relationship between anti-neuronal antibodies and

neuropsychiatric symptoms is as yet unknown. However, the antibodies

appear to recognize cellular components within the basal ganglia and

basal ganglia dysfunction has been implicated in both Sydenham's chorea

and OCD. One working hypothesis is that when genetically vulnerable

(predisposed) children are exposed to GABHS (strep) infections,

antibodies are produced that mistakenly recognize cells within the basal

ganglia and cause an inflammatory response. This inflammation is

manifest by adventitious movements and (neuro)psychiatric symptoms.

Symptom expression--chorea, tics, and/or OCD --may depend on the extent

of inflammation, chronicity of the insult, developmental stage of the

child's immune system, inherited vulnerability, or a combination of

these factors. " (I believe it is possible that strep infections could

play a role in some cases regression in kids with PDD. So many of the

symptoms

are the same.)

Dr. Swedo (post strep expert from the NIMH) recommends considering

rheumatic fever in " any child presenting with an acute onset of

hyperkinesis, adventitial movements or behavioral change. "

There is also a form of chorea called chorea mollis that presents with

profound hypotonia and few choreatic movements. Out of 5 children three

had swallowing difficulties and one even needed tube feedings. This

type of chorea is typical longer lasting (many months).

[Guest guest]

Hi Cara and thanks for responding to my ramblings :-)

Glad to hear the CoQ10 has helped with the palpitations. I've

had them off and on now since they put me on the high dose prednisone a

couple of years ago and its, well, just very unpleasant at times to feel

that way!!! Makes me wonder if maybe the CoQ10 might help me too

(course, I've been saying I was going to try CoQ for a long time now and

just cannot find a way to afford it sadly). I know from a number

of exams that my mitral valve is not the problem, but as I mentioned, it

is in my kids. Its a tough thing to connect though, in large part

because its not all that uncommon in women at puberty and older, so may

be wholly unrelated. With my kids its most interesting because they

are so very young to be dx'd with it....but that really just means no one

has a real clue where we are headed with it ultimatly, which only frustrates

me. From what I have been able to find, because of them being so

young and because there is regurgitation involved (in addition to the prolapsing),

there is a higher chance they will need a valve replacement.....when is

an unknown. Of course....LOL. Just curious.....do they

have you on preophelactic antibiotics before surgury or dental work???

Yes, hyperextensible and hyperflexible are the same thing.

I actually do remember teeth being discussed on Mito oldies a long time

back when I used to be one there. I was already heavily in the throws

of my problems, so may well have even instigated the initial conversation!!

Its always interesting to me to see how often there seems to be a link

between chronic disease (I've seen a similar response by other adults with

Dermatomyositis on my list for that) and dental problems, though am sad

to say there has been so very little research on the link and how it all

interconnects. Personally, I think there is some connection......may

not be the cause of the problems, but surely dental issues can affect your

health, so it should probably be looked at and more specifically addressed

one of these days.

Unfortunatly, there's not much to do for the floppy trachea's (tracheomalacia)

and its very very very very rare to happen suddenly in kids this age, so

we're all grasping in the dark for the most part. They are both on

inhalers (Advair and Pulmicort) daily and Chance takes a Foradil inhaler

right before exercise, though all of this has yet to really show any positive

impact on things. Ultimatly, we're waiting till things get bad enough

at this point, as bad as that sounds. Hoping maybe, just maybe, a

pulmo at Mayo might have some ideas, but this is a tough one and there

is just not a lot that can be done for it. I've found one woman with

acquired tracheomalacia (interestingly they think hers is caused by a connective

tissue disease) who started having problems when she was 30, was trached

and had a g-tube by 40 (and according to her, should have had both atleast

5 years earlier). She IS functioning pretty amazingly considering,

though of course, I am struggling with the idea of Chance and Madison being

trached. Hope every day we have a while longer before that decision

would have to be made. At the moment, both are just about 60%, so

still in the mild obstructive lung disease range, though their history

the last year shows them dropping about 4% every 4 months, which means

we might be into the moderate obstructive range now. Bad thing is,

there is only 10% points between mild and severe restriction, so there

is just not alot of room to play with. Unfortunatly, thus far, we've

not been able to stop or slow down the loss in function in either child.

Both kids present a little differently with this and in fact, Madison

really has no symptoms I have seen, but then she's a much more sedentary

child and never has been one to get out and really run around. Chance

was the first to show symptoms and those were a pain at the base of his

sternum anytime he'd run, and shortness of breath. It was VERY non-descript

and I honestly watched it for a long time, just not even sure what to do

with it or who to talk to. In the end, our cardiologist happened

to call and after about 5 months of watching him struggle in this way,

I was ready to figure it out. She ordered exercise testing, an Echo

following, and pulmonary function testing before and after. All looked

good except for the pulmonary functions and those showed a mild problem

before the exercise and a much more significant one afterwards, AND showed

he got worse after being given a breathing treatment. To make a long

story short, we saw the pulmo a month or so later and he realized that

instead of exercise induced asthma which was what the cardio had called

it, that the trachea was collapsing in on itself and that he gets worse

after a breathing treatment because bronchodilators further relax the trachea,

which only makes things worse. A bronchoscopy confirmed both tracheo

and bronchomalacia, and they determined he had inflammation in his lungs

that they suspected was aspiration of reflux related. pH probe confirmed

that (as much as it can be confirmed) and he's been on reflux meds for

a few months now, though he's honestly never really had any signs of reflux.

Madison too is a silent refluxer. As I mention, Madison really has

no symptoms, though we have full pulmonary function testing results from

just last May and at that time, her numbers were completely NORMAL.

We just took her back in for a yearly check in May and found she'd dropped

25+% since those tests were done last year, and she too worsened after

the bronchodilator like her brother. Since we'd already done the

other more invasive testing on her brother we have been, atleast thus far,

able to avoid them with her and are just assuming based on what we're seeing

she has the same things happening. They've both been losing muscle

mass and tone in their upper bodies since a little more than a year ago

and at the moment, thats the best guess as to why this is happening.

Thanks for asking and thanks for the feedback!!

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

McNair Family Home

Page

McNair Family Updates

United Mitochondrial Disease Foundation---

"I try to take one day at a time, but sometimes several days attack

me at once." Ashleigh Brilliant

cara waller wrote:

Kass,

i was told i had a leaky mitral valve with regurgitation about 8 years

ago. I had alot of palpitations too. The CoQ10 has improved

this for me quite a bit.

has some hyperextendable joints - is this what you mean by flexible?

My daughter and I can do some sitting positions most people find difficult. As

for teeth, I have had plenty of troubles. Not with the enamel, but

i can't tell things are going wrong until it is too late. Last year,

i got a clean bill of dental health on monday and tuesday was in severe

pain and wednesday had a root canal! xrays on monday did not really

show anything the regular dentist could see. Also,

last summer my teeth were unexpectantly all loose. They firmed up,

but the dentist was surprised because they were not usually like that.

Haven't figured it out yet, but i have recently been having bruising problems

again (from time to time this pops us, since college) and am waiting on

test results. I've had several spontaneous bruises, and feel awful,

and my gums are bleeding, yeah. On the mito oldies list, teeth problems

were an issue we talked about and it seems pretty common. I'm

sorry to hear about your teeth and the problems your children are having.

What is being done for the weak trachea? are they having trouble breathing? cara

Autoimmune/Connective

Tissue/Mito, Was Re: Anne and

Hi all :-)

This thread just got me to thinking and I am wondering how many see

some of the connections we're making over here. We have family history

of things like Fibromyalgia, Endometriosis, Lupus, Scleroderma, Dermatomyositis

on my (mom's) side, with Crohn's and MS on dad's side. I was told

at the conference in Dallas by a couple of the doctors there that they

are seeing more and more autoimmune components with the mito, though I've

not seen alot about it otherwise. We seem to like to have an inflammatory

response to everything!! Immune issues in general we're seeing with

Madison (just fights nothing off anymore) and my sister has always been

very sickly in this way. I don't think we've done any of the real

testing to see if there would be something there that would indicate a

true immune deficiency or not though.

Interestingly, looked into PANDAS ALOT when Chance got so bad .......his

tics increased dramatically and he certainly seemed to fit the profile,

all except there had been no signs of him being ill with strep beforehand

(course, he had strep earlier this year and NO ONE would have known had

his sister not been dx'd first).

Then we have this connective tissue thing becoming more and more prominent.

Chance and Madison both have a leaky mitral valve and regurgitation, both

are loose jointed, but especially Madison, and now their trachea's have

become floppy. Madison's skin biopsy was also very abnormal.

And finally, I just had to have all my teeth removed (at the ripe old age

of 35) and dentures placed because I apparently have Ectodermal Dysplasia

(our best guess for a name for it) and all my enamel on my teeth decided

to just disintegrate over the last few years.

Have no idea how it all fits together but keep thinking there must be

others out there like us and would love to understand it all better.

Not sure anyone has any thoughts for me here, but figured I'd toss it out

there and see.

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

McNair Family Home

Page

McNair Family Updates

United Mitochondrial Disease Foundation---

"I try to take one day at a time, but sometimes several days attack

me at once." Ashleigh Brilliant

Plant wrote:

Anne and ,

All three of my sons have evidence of autoimmunity. My son

with mito

has very high inflammatory markers and T-cell repsonses.

His brothers

both developed rheumatic fever post strep (even though the strep

was

treated with antibiotics). It is likely that they both had

a previous

mild strep infection that went undetected. In addition to

the

polyarthritis and chorea the boys also developed some PANDAS (Pediatric

Autoimmune Neuropsychiatric Disorders Associated with Streptococcal

infections) symptoms.

Anne, did you know that erythema nodosum has been linked to strep

infections and can occur in patients with rheumatic fever?

Studies at

the NIMH have shown some success in treating PANDAS and rheumatic

fever

with IVIG and plasmapheresis. I noticed that there is also

a link

between sarcoidosis and strep infections.

Here is some info on erythema nodosum, strep, and rheumatic fever:

Rheumatic fever, a streptococcal infection which

presents with fever,

erythema nodosum, patchy lung infiltrates, sometimes chorea and

myocardial symptoms. Rheumatic fever often occurs in

children after

the age of 3 years. Clinically there is a preceding sore throat,

accompanied by non-erosivearthritis of the large joints, fever

and skin

rashes. Cardiac manifestations include heart murmur as a mid-diastolic

mitral murmur (Carey Coombes murmur), cardiac enlargement (indicative

of

carditis), pericarditis and heart failure. A rising antistreptolysin

O

titre is a good indicator of streptococcal infection even though

the

organism may not be cultured.

http://www.medinfo.ufl.edu/year2/mmid/bms5300/bugs/strpyoge.html

Cutis. 1978 Jun;21(6):806-10.

Erythema nodosum. A review.

Soderstrom RM, Krull EA.

Erythema nodosum is not an uncommon dermatologic entity. Sarcoidosis

and

streptococcal infection have become the two most common causes,

while

tuberculosis was the predominant etiology prior to the use of isoniazid.

Histoplasmosis and coccidioidomycosis are two important geographic

considerations. Laboratory tests should include a PPD test, chest

x-ray,

throat culture for

beta-streptococcus, and ASO titer determination as a minimum.

Symptomatic treatment remains unsatisfactory in many cases, although

recent success has been reported with oral potassium iodide.

[Guest guest]

Kass, they used to put me on prophyllactic antibiotic prior to dental work, etc. but now that i don't feel the palpitations and am on CoQ10, I don't mention it, so we have not the last few times. I had a root canal and some other work without antibiotics and all has gone fine. I rejected the one type of crown material and had to have it replaced within several weeks, but not sure how that figures in.

I hope they come up with some way to help Chance and Madison's breathing problems soon.

cara

Autoimmune/Connective Tissue/Mito, Was Re: Anne and Hi all :-) This thread just got me to thinking and I am wondering how many see some of the connections we're making over here. We have family history of things like Fibromyalgia, Endometriosis, Lupus, Scleroderma, Dermatomyositis on my (mom's) side, with Crohn's and MS on dad's side. I was told at the conference in Dallas by a couple of the doctors there that they are seeing more and more autoimmune components with the mito, though I've not seen alot about it otherwise. We seem to like to have an inflammatory response to everything!! Immune issues in general we're seeing with Madison (just fights nothing off anymore) and my sister has always been very sickly in this way. I don't think we've done any of the real testing to see if there would be something there that would indicate a true immune deficiency or not though. Interestingly, looked into PANDAS ALOT when Chance got so bad .......his tics increased dramatically and he certainly seemed to fit the profile, all except there had been no signs of him being ill with strep beforehand (course, he had strep earlier this year and NO ONE would have known had his sister not been dx'd first). Then we have this connective tissue thing becoming more and more prominent. Chance and Madison both have a leaky mitral valve and regurgitation, both are loose jointed, but especially Madison, and now their trachea's have become floppy. Madison's skin biopsy was also very abnormal. And finally, I just had to have all my teeth removed (at the ripe old age of 35) and dentures placed because I apparently have Ectodermal Dysplasia (our best guess for a name for it) and all my enamel on my teeth decided to just disintegrate over the last few years. Have no idea how it all fits together but keep thinking there must be others out there like us and would love to understand it all better. Not sure anyone has any thoughts for me here, but figured I'd toss it out there and see. -- BIG hugs, Kass, proud mom to Chance(9-Mito) Our future Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?) Our Drama Queen McNair Family Home Page McNair Family Updates United Mitochondrial Disease Foundation--- "I try to take one day at a time, but sometimes several days attack me at once." Ashleigh Brilliant Plant wrote: Anne and , All three of my sons have evidence of autoimmunity. My son with mito has very high inflammatory markers and T-cell repsonses. His brothers both developed rheumatic fever post strep (even though the strep was treated with antibiotics). It is likely that they both had a previous mild strep infection that went undetected. In addition to the polyarthritis and chorea the boys also developed some PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) symptoms. Anne, did you know that erythema nodosum has been linked to strep infections and can occur in patients with rheumatic fever? Studies at the NIMH have shown some success in treating PANDAS and rheumatic fever with IVIG and plasmapheresis. I noticed that there is also a link between sarcoidosis and strep infections. Here is some info on erythema nodosum, strep, and rheumatic fever: Rheumatic fever, a streptococcal infection which presents with fever, erythema nodosum, patchy lung infiltrates, sometimes chorea and myocardial symptoms. Rheumatic fever often occurs in children after the age of 3 years. Clinically there is a preceding sore throat, accompanied by non-erosivearthritis of the large joints, fever and skin rashes. Cardiac manifestations include heart murmur as a mid-diastolic mitral murmur (Carey Coombes murmur), cardiac enlargement (indicative of carditis), pericarditis and heart failure. A rising antistreptolysin O titre is a good indicator of streptococcal infection even though the organism may not be cultured. http://www.medinfo.ufl.edu/year2/mmid/bms5300/bugs/strpyoge.html Cutis. 1978 Jun;21(6):806-10. Erythema nodosum. A review. Soderstrom RM, Krull EA. Erythema nodosum is not an uncommon dermatologic entity. Sarcoidosis and streptococcal infection have become the two most common causes, while tuberculosis was the predominant etiology prior to the use of isoniazid. Histoplasmosis and coccidioidomycosis are two important geographic considerations. Laboratory tests should include a PPD test, chest x-ray, throat culture for beta-streptococcus, and ASO titer determination as a minimum. Symptomatic treatment remains unsatisfactory in many cases, although recent success has been reported with oral potassium iodide. Please contact mito-owner with any problems or questions.

[Guest guest]

Just an FYI ~ even if the chances are very small.

My sister in law had an undetected heart murmur, she had dental work

done and within a few months was ill. Due to her PCP not running

a simple blood culture and passing her fevers and illness off for months

and months she ended up having Endocarditis. This was about 1 yr

after her dental work and she had to have a heart valve replaced @ 27 yrs

old.

Personally I would take the antibiotics before any

dental work, but that's just my opinion.

Kerry

(PS my post take 1-2 days to show up)

cara waller wrote:

Kass, they

used to put me on prophyllactic antibiotic prior to dental work, etc. but

now that i don't feel the palpitations and am on CoQ10, I don't mention

it, so we have not the last few times. I had a root canal and some

other work without antibiotics and all has gone fine. I rejected

the one type of crown material and had to have it replaced within several

weeks, but not sure how that figures in. I

hope they come up with some way to help Chance and Madison's breathing

problems soon. cara

Autoimmune/Connective

Tissue/Mito, Was Re: Anne and

Hi all :-)

This thread just got me to thinking and I am wondering how many see

some of the connections we're making over here. We have family history

of things like Fibromyalgia, Endometriosis, Lupus, Scleroderma, Dermatomyositis

on my (mom's) side, with Crohn's and MS on dad's side. I was told

at the conference in Dallas by a couple of the doctors there that they

are seeing more and more autoimmune components with the mito, though I've

not seen alot about it otherwise. We seem to like to have an inflammatory

response to everything!! Immune issues in general we're seeing with

Madison (just fights nothing off anymore) and my sister has always been

very sickly in this way. I don't think we've done any of the real

testing to see if there would be something there that would indicate a

true immune deficiency or not though.

Interestingly, looked into PANDAS ALOT when Chance got so bad .......his

tics increased dramatically and he certainly seemed to fit the profile,

all except there had been no signs of him being ill with strep beforehand

(course, he had strep earlier this year and NO ONE would have known had

his sister not been dx'd first).

Then we have this connective tissue thing becoming more and more prominent.

Chance and Madison both have a leaky mitral valve and regurgitation, both

are loose jointed, but especially Madison, and now their trachea's have

become floppy. Madison's skin biopsy was also very abnormal.

And finally, I just had to have all my teeth removed (at the ripe old age

of 35) and dentures placed because I apparently have Ectodermal Dysplasia

(our best guess for a name for it) and all my enamel on my teeth decided

to just disintegrate over the last few years.

Have no idea how it all fits together but keep thinking there must be

others out there like us and would love to understand it all better.

Not sure anyone has any thoughts for me here, but figured I'd toss it out

there and see.

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

McNair Family Home

Page

McNair Family Updates

United Mitochondrial Disease Foundation---

"I try to take one day at a time, but sometimes several days attack

me at once." Ashleigh Brilliant

Plant wrote:

Anne and ,

All three of my sons have evidence of autoimmunity. My son

with mito

has very high inflammatory markers and T-cell repsonses.

His brothers

both developed rheumatic fever post strep (even though the strep

was

treated with antibiotics). It is likely that they both had

a previous

mild strep infection that went undetected. In addition to

the

polyarthritis and chorea the boys also developed some PANDAS (Pediatric

Autoimmune Neuropsychiatric Disorders Associated with Streptococcal

infections) symptoms.

Anne, did you know that erythema nodosum has been linked to strep

infections and can occur in patients with rheumatic fever?

Studies at

the NIMH have shown some success in treating PANDAS and rheumatic

fever

with IVIG and plasmapheresis. I noticed that there is also

a link

between sarcoidosis and strep infections.

Here is some info on erythema nodosum, strep, and rheumatic fever:

Rheumatic fever, a streptococcal infection which

presents with fever,

erythema nodosum, patchy lung infiltrates, sometimes chorea and

myocardial symptoms. Rheumatic fever often occurs in

children after

the age of 3 years. Clinically there is a preceding sore throat,

accompanied by non-erosivearthritis of the large joints, fever

and skin

rashes. Cardiac manifestations include heart murmur as a mid-diastolic

mitral murmur (Carey Coombes murmur), cardiac enlargement (indicative

of

carditis), pericarditis and heart failure. A rising antistreptolysin

O

titre is a good indicator of streptococcal infection even though

the

organism may not be cultured.

http://www.medinfo.ufl.edu/year2/mmid/bms5300/bugs/strpyoge.html

Cutis. 1978 Jun;21(6):806-10.

Erythema nodosum. A review.

Soderstrom RM, Krull EA.

Erythema nodosum is not an uncommon dermatologic entity. Sarcoidosis

and

streptococcal infection have become the two most common causes,

while

tuberculosis was the predominant etiology prior to the use of isoniazid.

Histoplasmosis and coccidioidomycosis are two important geographic

considerations. Laboratory tests should include a PPD test, chest

x-ray,

throat culture for

beta-streptococcus, and ASO titer determination as a minimum.

Symptomatic treatment remains unsatisfactory in many cases, although

recent success has been reported with oral potassium iodide.

Please contact mito-owner with any problems or questions.

[Guest guest]

Hi

I noticed that you were mentioning MVP. When I was diagnosed with it--I

think in the 80's, I was told then that it is common in women and

children.

Shirley, mom to and jordan

> Hi Cara and thanks for responding to my ramblings  :-)

>

> Glad to hear the CoQ10 has helped with the palpitations.  I've had them

> off and on now since they put me on the high dose prednisone a couple

> of years ago and its, well, just very unpleasant at times to feel that

> way!!!  Makes me wonder if maybe the CoQ10 might help me too (course,

> I've been saying I was going to try CoQ for a long time now and just

> cannot find a way to afford it sadly).  I know from a number of exams

> that my mitral valve is not the problem, but as I mentioned, it is in

> my kids.  Its a tough thing to connect though, in large part because

> its not all that uncommon in women at puberty and older, so may be

> wholly unrelated.  With my kids its most interesting because they are

> so very young to be dx'd with it....but that really just means no one

> has a real clue where we are headed with it ultimatly, which only

> frustrates me.  From what I have been able to find, because of them

> being so young and because there is regurgitation involved (in addition

> to the prolapsing), there is a higher chance they will need a valve

> replacement.....when is an unknown.  Of course....LOL.   Just

> curious.....do they have you on preophelactic antibiotics before

> surgury or dental work???

>

> Yes, hyperextensible and hyperflexible are the same thing.

>

> I actually do remember teeth being discussed on Mito oldies a long time

> back when I used to be one there.  I was already heavily in the throws

> of my problems, so may well have even instigated the initial

> conversation!!  Its always interesting to me to see how often there

> seems to be a link between chronic disease (I've seen a similar

> response by other adults with Dermatomyositis on my list for that) and

> dental problems, though am sad to say there has been so very little

> research on the link and how it all interconnects.  Personally, I think

> there is some connection......may not be the cause of the problems, but

> surely dental issues can affect your health, so it should probably be

> looked at and more specifically addressed one of these days.

>

> Unfortunatly, there's not much to do for the floppy trachea's

> (tracheomalacia) and its very very very very rare to happen suddenly in

> kids this age, so we're all grasping in the dark for the most part. 

> They are both on inhalers (Advair and Pulmicort) daily and Chance takes

> a Foradil inhaler right before exercise, though all of this has yet to

> really show any positive impact on things.  Ultimatly, we're waiting

> till things get bad enough at this point, as bad as that sounds. 

> Hoping maybe, just maybe, a pulmo at Mayo might have some ideas, but

> this is a tough one and there is just not a lot that can be done for

> it.  I've found one woman with acquired tracheomalacia (interestingly

> they think hers is caused by a connective tissue disease) who started

> having problems when she was 30, was trached and had a g-tube by 40

> (and according to her, should have had both atleast 5 years earlier). 

> She IS functioning pretty amazingly considering, though of course, I am

> struggling with the idea of Chance and Madison being trached.  Hope

> every day we have a while longer before that decision would have to be

> made.  At the moment, both are just about 60%, so still in the mild

> obstructive lung disease range, though their history the last year

> shows them dropping about 4% every 4 months, which means we might be

> into the moderate obstructive range now.  Bad thing is, there is only

> 10% points between mild and severe restriction, so there is just not

> alot of room to play with.  Unfortunatly, thus far, we've not been able

> to stop or slow down the loss in function in either child.

>

> Both kids present a little differently with this and in fact, Madison

> really has no symptoms I have seen, but then she's a much more

> sedentary child and never has been one to get out and really run

> around.  Chance was the first to show symptoms and those were a pain at

> the base of his sternum anytime he'd run, and shortness of breath.  It

> was VERY non-descript and I honestly watched it for a long time, just

> not even sure what to do with it or who to talk to.  In the end, our

> cardiologist happened to call and after about 5 months of watching him

> struggle in this way, I was ready to figure it out.  She ordered

> exercise testing, an Echo following, and pulmonary function testing

> before and after.  All looked good except for the pulmonary functions

> and those showed a mild problem before the exercise and a much more

> significant one afterwards, AND showed he got worse after being given a

> breathing treatment.  To make a long story short, we saw the pulmo a

> month or so later and he realized that instead of exercise induced

> asthma which was what the cardio had called it, that the trachea was

> collapsing in on itself and that he gets worse after a breathing

> treatment because bronchodilators further relax the trachea, which only

> makes things worse.  A bronchoscopy confirmed both tracheo and

> bronchomalacia, and they determined he had inflammation in his lungs

> that they suspected was aspiration of reflux related.  pH probe

> confirmed that (as much as it can be confirmed) and he's been on reflux

> meds for a few months now, though he's honestly never really had any

> signs of reflux.  Madison too is a silent refluxer.  As I mention,

> Madison really has no symptoms, though we have full pulmonary function

> testing results from just last May and at that time, her numbers were

> completely NORMAL.  We just took her back in for a yearly check in May

> and found she'd dropped 25+% since those tests were done last year, and

> she too worsened after the bronchodilator like her brother.  Since we'd

> already done the other more invasive testing on her brother we have

> been, atleast thus far, able to avoid them with her and are just

> assuming based on what we're seeing she has the same things happening. 

> They've both been losing muscle mass and tone in their upper bodies

> since a little more than a year ago and at the moment, thats the best

> guess as to why this is happening.

>

> Thanks for asking and thanks for the feedback!!

>

> -- BIG hugs,

> Kass, proud mom to Chance(9-Mito) Our future Scientist, Madison(6-Mito)

> Our Purple Butterfly Princess, and Abby(5-?) Our Drama Queen

> McNair Family Home Page

> McNair Family Updates

> United Mitochondrial Disease Foundation---

>

> " I try to take one day at a time, but sometimes several days attack me

> at once. " Ashleigh Brilliant

>

> cara waller wrote:

>

> Kass, i was told i had a leaky mitral valve with regurgitation about 8

> years ago.  I had alot of palpitations too.  The CoQ10 has improved

> this for me quite a bit.  has some hyperextendable joints - is

> this what you mean by flexible?  My daughter and I can do some sitting

> positions most people find difficult. As for teeth, I have had plenty

> of troubles.  Not with the enamel, but i can't tell things are going

> wrong until it is too late.  Last year, i got a clean bill of dental

> health on monday and tuesday was in severe pain and wednesday had a

> root canal!  xrays on monday did not really show anything the regular

> dentist could see.     Also, last summer my teeth were unexpectantly

> all loose.  They firmed up, but the dentist was surprised because they

> were not usually like that.  Haven't figured it out yet, but i have

> recently been having bruising problems again (from time to time this

> pops us, since college) and am waiting on test results.  I've had

> several spontaneous bruises, and feel awful, and my gums are bleeding,

> yeah.  On the mito oldies list, teeth problems were an issue we talked

> about and it seems pretty common. I'm sorry to hear about your teeth

> and the problems your children are having.  What is being done for the

> weak trachea? are they having trouble breathing? cara 

>

> Autoimmune/Connective Tissue/Mito, Was Re: Anne and

>

>  Hi all  :-)

>

> This thread just got me to thinking and I am wondering how many see

> some of the connections we're making over here.  We have family history

> of things like Fibromyalgia, Endometriosis, Lupus, Scleroderma,

> Dermatomyositis on my (mom's) side, with Crohn's and MS on dad's side. 

> I was told at the conference in Dallas by a couple of the doctors there

> that they are seeing more and more autoimmune components with the mito,

> though I've not seen alot about it otherwise.  We seem to like to have

> an inflammatory response to everything!!  Immune issues in general

> we're seeing with Madison (just fights nothing off anymore) and my

> sister has always been very sickly in this way.  I don't think we've

> done any of the real testing to see if there would be something there

> that would indicate a true immune deficiency or not though.

>

> Interestingly, looked into PANDAS ALOT when Chance got so bad

> .......his tics increased dramatically and he certainly seemed

> to fit the profile, all except there had been no signs of him being ill

> with strep beforehand (course, he had strep earlier this year and NO

> ONE would have known had his sister not been dx'd first).

>

> Then we have this connective tissue thing becoming more and more

> prominent.  Chance and Madison both have a leaky mitral valve and

> regurgitation, both are loose jointed, but especially Madison, and now

> their trachea's have become floppy.  Madison's skin biopsy was also

> very abnormal.  And finally, I just had to have all my teeth removed

> (at the ripe old age of 35) and dentures placed because I apparently

> have Ectodermal Dysplasia (our best guess for a name for it) and all my

> enamel on my teeth decided to just disintegrate over the last few years.

>

> Have no idea how it all fits together but keep thinking there must be

> others out there like us and would love to understand it all better. 

> Not sure anyone has any thoughts for me here, but figured I'd toss it

> out there and see.

>

> -- BIG hugs,

> Kass, proud mom to Chance(9-Mito) Our future Scientist, Madison(6-Mito)

> Our Purple Butterfly Princess, and Abby(5-?) Our Drama Queen

> McNair Family Home Page

> McNair Family Updates

> United Mitochondrial Disease Foundation---

>

> " I try to take one day at a time, but sometimes several days attack me

> at once. " Ashleigh Brilliant

>

> Plant wrote:

>

>  Anne and ,

>

> All three of my sons have evidence of autoimmunity.  My son with mito

> has very high inflammatory markers and T-cell repsonses.  His brothers

> both developed rheumatic fever post strep (even though the strep was

> treated with antibiotics).  It is likely that they both had a previous

> mild strep infection that went undetected.  In addition to the

> polyarthritis and chorea the boys also developed some PANDAS (Pediatric

> Autoimmune Neuropsychiatric Disorders Associated with Streptococcal

> infections) symptoms.

>

> Anne, did you know that erythema nodosum has been linked to strep

> infections and can occur in patients with rheumatic fever?  Studies at

> the NIMH have shown some success in treating PANDAS and rheumatic fever

> with IVIG and plasmapheresis.  I noticed that there is also a link

> between sarcoidosis and strep infections.

>

> Here is some info on erythema nodosum, strep, and rheumatic fever:

>

> Rheumatic fever,    a streptococcal infection which presents with fever,

> erythema nodosum, patchy lung infiltrates, sometimes chorea and

> myocardial symptoms.   Rheumatic fever often occurs in children after

> the age of 3 years. Clinically there is a preceding sore throat,

> accompanied by non-erosivearthritis of the large joints, fever and skin

> rashes. Cardiac manifestations include heart murmur as a mid-diastolic

> mitral murmur (Carey Coombes murmur), cardiac enlargement (indicative of

> carditis), pericarditis and heart failure. A rising antistreptolysin O

> titre is a good indicator of streptococcal infection even though the

> organism may not be cultured.

>

> http://www.medinfo.ufl.edu/year2/mmid/bms5300/bugs/strpyoge.html

>

> Cutis. 1978 Jun;21(6):806-10.

> Erythema nodosum. A review.

> Soderstrom RM, Krull EA.

>

> Erythema nodosum is not an uncommon dermatologic entity. Sarcoidosis and

> streptococcal infection have become the two most common causes, while

> tuberculosis was the predominant etiology prior to the use of isoniazid.

> Histoplasmosis and coccidioidomycosis are two important geographic

> considerations. Laboratory tests should include a PPD test, chest x-ray,

> throat culture for

> beta-streptococcus, and ASO titer determination as a minimum.

> Symptomatic treatment remains unsatisfactory in many cases, although

> recent success has been reported with oral potassium iodide.

>

>

>

>

>

>

> Please contact mito-owner with any problems or

> questions.

>

>

[Guest guest]

Thanks for sharing Shirley :-)

Mitral Valve Prolapse (a floppy valve) and Mitral Valave regurgitation

(leaking of the blood back into the chamber it just came from) are related

obviously (cannot have one without the other) though are actually pretty

different. MVP IS a relatively common finding in women and girls

over the age of 12 in particular, though is very very very rare in kids

under the age of 12. Its seen, but so seldom there's been all of

maybe one study on them, so information is limited. We've seen a

total of 4 cardiologist the last few years and none have ever had patients

as young as my kids. The regurgitation aspect makes them much more

suseptable to infection of the heart if bacteria is introduced to the blood

stream, but otherwise, for the most part atleast at this time, there are

no other issues with it. It is something that can be seen in Marfan's

patients, thus there is a real connective tissue component to it, which

was why it was mentioned. With time the regurgitation can get more

severe and begin to cause other problems with the heart which is when replacing

the valve might become something that would need to be considered.

The thing that was happening in the 80's was that there were a TON of

people being dx'd with MVP with little or no testing. If they were having

heart palpitations or feeling odd and there were no other obvious answers,

docs we're giving them the MVP dx. In reality, without an ECHO its

impossible to diagnose and alot of women have now had the dx taken away

once adequate testing was done. All said to say that back in the

80's it was assumed that alot more people had MVP than actually did.

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

McNair Family Home

Page

McNair Family Updates

United Mitochondrial Disease Foundation---

"I try to take one day at a time, but sometimes several days attack

me at once." Ashleigh Brilliant

Shirley Cabaldon wrote:

Hi

I noticed that you were mentioning MVP. When I was diagnosed with it--I

think in the 80's, I was told then that it is common in women and children.

Shirley, mom to and jordan

[Guest guest]

Hi Cara....thanks again for the support. I too hope there are some

options we've yet to explore out there (we see our pulmo here on the 4th

again and then should be getting to Mayo sometime next month, so hopefully

we'll atleast know one way or the other by then).

The reason they have someone with MVR take prophelactic antibiotics

is because when you have a regurgitating valve your at much higher risk

of developing an infection in the heart and surgury and dental work are

easy ways to introduce bacteria to the blood stream. A person with

normal valves has little problem fighting off the infection (may go somewhere

else but won't settle in the heart) while someone with regurg is very suseptable

to it finding a place to settle in the heart. If you've been on it

in the past (thus have a very confirmed case of MVP with Regurg.....there

were lots of false dx's of this in the past though it sounds like yours

was confirmed by Echo) you really probably should do it now too, even with

the CoQ and lack of palpatations. The palpatations may well have

been related to the MVR, but its not something that gets better, so even

though the palpitations are under control, you very likely still have the

regurg happening (my kids have no palpitations or other symptoms that we

can see). Getting a bacterial infection of the heart can be very

dangerous and life threatening, so definatly not something to mess with!!!!

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

McNair Family Home

Page

McNair Family Updates

United Mitochondrial Disease Foundation---

"I try to take one day at a time, but sometimes several days attack

me at once." Ashleigh Brilliant

cara waller wrote:

Kass, they

used to put me on prophyllactic antibiotic prior to dental work, etc. but

now that i don't feel the palpitations and am on CoQ10, I don't mention

it, so we have not the last few times. I had a root canal and some

other work without antibiotics and all has gone fine. I rejected

the one type of crown material and had to have it replaced within several

weeks, but not sure how that figures in. I

hope they come up with some way to help Chance and Madison's breathing

problems soon. cara

[Guest guest]

Thanks for the feedback!!! Definatly seems to be a possibility when

all is said and done. I need to go and see if I can find that article!!!

-- BIG hugs,

Kass, proud mom to Chance(9-Mito) Our future

Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?)

Our Drama Queen

McNair Family Home

Page

McNair Family Updates

United Mitochondrial Disease Foundation---

"I try to take one day at a time, but sometimes several days attack

me at once." Ashleigh Brilliant

Hanna wrote:

Hi Kass. I was diagnosed with lupus 4 years

ago and mito only a few months ago. Looking back I think I had signs of

mito when I was a child. My theory is that the autoimmune disease (lupus)

was triggered by the mito. Someone here posted an article about a connection

a while ago but can't remember who it was. But it does make sense that

if the body isn't working properly like in mito then the immune system

won't work right either.

[Guest guest]

Thanks for the info, Kass. I think I will try to get another Echo to see if it actually has gone away. Until then, like you say, I should still be careful.

I read the other post about MVP being common and that many were diagnosed without proper testing. I have to wonder, too, that just because something is 'common' does not mean it is 'normal'. Hasn't some MVP been connected to all the unnecessary, unhealthful, synthetic additives in the food supply these days? Just watching TV cereal commercial gives me the creeps! Rainbows! Blue bananas! Ugh. (Common can just mean that the population is being exposed commonly to the same toxin/substance/etc. Mercury in fish comes to mind.)

cara

Re: Autoimmune/Connective Tissue/Mito, Was Re: Anne and

Hi Cara....thanks again for the support. I too hope there are some options we've yet to explore out there (we see our pulmo here on the 4th again and then should be getting to Mayo sometime next month, so hopefully we'll atleast know one way or the other by then). The reason they have someone with MVR take prophelactic antibiotics is because when you have a regurgitating valve your at much higher risk of developing an infection in the heart and surgury and dental work are easy ways to introduce bacteria to the blood stream. A person with normal valves has little problem fighting off the infection (may go somewhere else but won't settle in the heart) while someone with regurg is very suseptable to it finding a place to settle in the heart. If you've been on it in the past (thus have a very confirmed case of MVP with Regurg.....there were lots of false dx's of this in the past though it sounds like yours was confirmed by Echo) you really probably should do it now too, even with the CoQ and lack of palpatations. The palpatations may well have been related to the MVR, but its not something that gets better, so even though the palpitations are under control, you very likely still have the regurg happening (my kids have no palpitations or other symptoms that we can see). Getting a bacterial infection of the heart can be very dangerous and life threatening, so definatly not something to mess with!!!! -- BIG hugs, Kass, proud mom to Chance(9-Mito) Our future Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?) Our Drama Queen McNair Family Home Page McNair Family Updates United Mitochondrial Disease Foundation--- "I try to take one day at a time, but sometimes several days attack me at once." Ashleigh Brilliant cara waller wrote: Kass, they used to put me on prophyllactic antibiotic prior to dental work, etc. but now that i don't feel the palpitations and am on CoQ10, I don't mention it, so we have not the last few times. I had a root canal and some other work without antibiotics and all has gone fine. I rejected the one type of crown material and had to have it replaced within several weeks, but not sure how that figures in. I hope they come up with some way to help Chance and Madison's breathing problems soon. caraPlease contact mito-owner with any problems or questions.

[Guest guest]

Kerry, thanks for mentioning this. I would not have suspected that there could be such a time lapse. Wow. Thanks again for the heads up!

cara

Autoimmune/Connective Tissue/Mito, Was Re: Anne and Hi all :-) This thread just got me to thinking and I am wondering how many see some of the connections we're making over here. We have family history of things like Fibromyalgia, Endometriosis, Lupus, Scleroderma, Dermatomyositis on my (mom's) side, with Crohn's and MS on dad's side. I was told at the conference in Dallas by a couple of the doctors there that they are seeing more and more autoimmune components with the mito, though I've not seen alot about it otherwise. We seem to like to have an inflammatory response to everything!! Immune issues in general we're seeing with Madison (just fights nothing off anymore) and my sister has always been very sickly in this way. I don't think we've done any of the real testing to see if there would be something there that would indicate a true immune deficiency or not though. Interestingly, looked into PANDAS ALOT when Chance got so bad .......his tics increased dramatically and he certainly seemed to fit the profile, all except there had been no signs of him being ill with strep beforehand (course, he had strep earlier this year and NO ONE would have known had his sister not been dx'd first). Then we have this connective tissue thing becoming more and more prominent. Chance and Madison both have a leaky mitral valve and regurgitation, both are loose jointed, but especially Madison, and now their trachea's have become floppy. Madison's skin biopsy was also very abnormal. And finally, I just had to have all my teeth removed (at the ripe old age of 35) and dentures placed because I apparently have Ectodermal Dysplasia (our best guess for a name for it) and all my enamel on my teeth decided to just disintegrate over the last few years. Have no idea how it all fits together but keep thinking there must be others out there like us and would love to understand it all better. Not sure anyone has any thoughts for me here, but figured I'd toss it out there and see. -- BIG hugs, Kass, proud mom to Chance(9-Mito) Our future Scientist, Madison(6-Mito) Our Purple Butterfly Princess, and Abby(5-?) Our Drama Queen McNair Family Home Page McNair Family Updates United Mitochondrial Disease Foundation--- "I try to take one day at a time, but sometimes several days attack me at once." Ashleigh Brilliant Plant wrote: Anne and , All three of my sons have evidence of autoimmunity. My son with mito has very high inflammatory markers and T-cell repsonses. His brothers both developed rheumatic fever post strep (even though the strep was treated with antibiotics). It is likely that they both had a previous mild strep infection that went undetected. In addition to the polyarthritis and chorea the boys also developed some PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections) symptoms. Anne, did you know that erythema nodosum has been linked to strep infections and can occur in patients with rheumatic fever? Studies at the NIMH have shown some success in treating PANDAS and rheumatic fever with IVIG and plasmapheresis. I noticed that there is also a link between sarcoidosis and strep infections. Here is some info on erythema nodosum, strep, and rheumatic fever: Rheumatic fever, a streptococcal infection which presents with fever, erythema nodosum, patchy lung infiltrates, sometimes chorea and myocardial symptoms. Rheumatic fever often occurs in children after the age of 3 years. Clinically there is a preceding sore throat, accompanied by non-erosivearthritis of the large joints, fever and skin rashes. Cardiac manifestations include heart murmur as a mid-diastolic mitral murmur (Carey Coombes murmur), cardiac enlargement (indicative of carditis), pericarditis and heart failure. A rising antistreptolysin O titre is a good indicator of streptococcal infection even though the organism may not be cultured. http://www.medinfo.ufl.edu/year2/mmid/bms5300/bugs/strpyoge.html Cutis. 1978 Jun;21(6):806-10. Erythema nodosum. A review. Soderstrom RM, Krull EA. Erythema nodosum is not an uncommon dermatologic entity. Sarcoidosis and streptococcal infection have become the two most common causes, while tuberculosis was the predominant etiology prior to the use of isoniazid. Histoplasmosis and coccidioidomycosis are two important geographic considerations. Laboratory tests should include a PPD test, chest x-ray, throat culture for beta-streptococcus, and ASO titer determination as a minimum. Symptomatic treatment remains unsatisfactory in many cases, although recent success has been reported with oral potassium iodide.

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