Update on my family

[Guest guest]

I'll start with me since I saw the Doc today. He started giving me the cortisone

shots in the ribs. (he thought I had costochondritis) He asked me what I could

do to make it hurt now, so I moved my arm and I point to a spot, he'd shoot, I'd

move it again, he's shoot, this went on and on. 16 spots! Then he stops and

says, I don't think this is costochoditis at all. It's not following the rib

line, it's following the nerve line, so then he guesses maybe Shingles, without

the rash. The shots really weren't that bad. Kinda like little bee stings. He

said he doesn't really have a clue what this is (again) so if the shots helped

it has to be the inflammation in the rib, and if it doesn't help, maybe

shingles. Asked if I wanted Vicodine, I'm a light weight on pain meds, I said

no. I'd rather hurt and have a brain. It's not that bad. I'd have to say the

shots have helped. Ya know, over the last several years I've had rib pain, and

tests, they never know what I have. Only one time an ultra sound found an air

pocket under the ribs, but they didn't have a clue how it got there.

Dylan is better, the constipation is finally responding to the Miralax. The ole'

take on drug to counteract the affects of another. His constant pain level is

finally down to a 6 1/2. that is the best in over a year and a half for an

overall pain. Maybe he can go to school. The chronic constipation caused him to

loose 20 lbs. The loss has stopped and he is eating more, not allot, but more.

Brady's HA's are better. It's still there, but not nearly as bad. I've been

doing allot of reading on the net about Migraines. All the symptoms point to a

spinal fluid leak in the brain. These are caused by a blunt force or connective

tissue disorders. So... Ya know what I'm thinking. He had no blow to the head

for a year. And he does have (mild) stenosis (narrowing of the spinal column) in

the cervical and Lumbar spine. He see's neurology in 2 weeks again. Guess an MRI

or MRA is next.

So, all and all I'd have to say, we are still riding this rollercoaster ride,

but we are at a point of a straightaway. Things are calming down a bit.

Cinylouwho/Cindy C.

[Guest guest]

THANK YOU CINDY, for the update!!! I sure hope you don't have

shingles. My hubby had them, and his Mom as well. Those can be very

painful and take awhile to get over.

I'm tickled for Dylan! YEA!

Brady has stenosis???? Cindy, that was what was causing my horrible

headaches. Perhaps it's gotten worse?? I'll be anxious to see what

a MRI show. Please keep us posted!

Saying a prayer and sending healing thoughts to ALL of your's!!!

Love Lana

> I'll start with me since I saw the Doc today. He started giving me

the cortisone shots in the ribs. (he thought I had costochondritis)

He asked me what I could do to make it hurt now, so I moved my arm

and I point to a spot, he'd shoot, I'd move it again, he's shoot,

this went on and on. 16 spots! Then he stops and says, I don't think

this is costochoditis at all. It's not following the rib line, it's

following the nerve line, so then he guesses maybe Shingles, without

the rash. The shots really weren't that bad. Kinda like little bee

stings. He said he doesn't really have a clue what this is (again)

so if the shots helped it has to be the inflammation in the rib, and

if it doesn't help, maybe shingles. Asked if I wanted Vicodine, I'm

a light weight on pain meds, I said no. I'd rather hurt and have a

brain. It's not that bad. I'd have to say the shots have helped. Ya

know, over the last several years I've had rib pain, and tests, they

never know what I have. Only one time an ultra sound found an air

pocket under the ribs, but they didn't have a clue how it got there.

> Dylan is better, the constipation is finally responding to the

Miralax. The ole' take on drug to counteract the affects of another.

His constant pain level is finally down to a 6 1/2. that is the best

in over a year and a half for an overall pain. Maybe he can go to

school. The chronic constipation caused him to loose 20 lbs. The

loss has stopped and he is eating more, not allot, but more.

> Brady's HA's are better. It's still there, but not nearly as bad.

I've been doing allot of reading on the net about Migraines. All the

symptoms point to a spinal fluid leak in the brain. These are caused

by a blunt force or connective tissue disorders. So... Ya know what

I'm thinking. He had no blow to the head for a year. And he does

have (mild) stenosis (narrowing of the spinal column) in the

cervical and Lumbar spine. He see's neurology in 2 weeks again.

Guess an MRI or MRA is next.

> So, all and all I'd have to say, we are still riding this

rollercoaster ride, but we are at a point of a straightaway. Things

are calming down a bit.

> Cinylouwho/Cindy C.

>

>

[Guest guest]

From: buckysmom2000

To: ceda

Sent: Tuesday, August 10, 2004 10:50 PM

Subject: Re: Update on my family

THANK YOU CINDY, for the update!!! .........

Brady has stenosis???? ......... Cindy, that was what was causing my horrible

headaches. Perhaps it's gotten worse??

Love Lana

************************

Stenosis gets worse? I thought it was the way it is and stay's that way....?

When I mentioned Stenosis to the neuorologist, he just passed right by that

fact. Like it was not important. What the heck?

[Guest guest]

Re: Re: Update on my family

************************

Stenosis gets worse? I thought it was the way it is and stay's that way....?

When I mentioned Stenosis to the neuorologist, he just passed right by that

fact. Like it was not important. What the heck?

~~~~

Unfortunately, from all the posts of EDSers who have it, it DOES and can get

worse (

Sorry to hear that Brady has this... I have a friend who had it in his lower

spine and was " roto rooted out " when he had major back surgery to have rods and

screws put in his back. He was doing good until the screws broke off.... He's

on major pain meds because of it.

[Guest guest]

Thanks, Bonnie for answering this. Cindy, the bones in the neck/back

just keep getting worse until something usually is done. With me,

they offered the epidural cortisone steroid injection, which I've

refused so far. However, my shoulder is now hurting like H - - -,

and I believe it's from the stenosis. If it keeps up, I will be

considering more seriously, the injection. I also believe that once

you begin fusion, it eventually leads to more and more deterioration

and more fusions, just like what Sis is dealing with now! It's

really kind of odd that she and I have such similar problems. I

think it would at least be a start to have them do another MRI on

Brady to see if it's advanced. Please let us know what you find out!

Love Lana

> Unfortunately, from all the posts of EDSers who have it, it DOES

and can get worse (

>

> Sorry to hear that Brady has this... I have a friend who had it

in his lower spine and was " roto rooted out " when he had major back

surgery to have rods and screws put in his back. He was doing good

until the screws broke off.... He's on major pain meds because of it.

>

>

>

>

>

[Guest guest]

Thanks, Bonnie for answering this. Cindy, the bones in the neck/back

just keep getting worse until something usually is done. With me,

they offered the epidural cortisone steroid injection, which I've

refused so far. However, my shoulder is now hurting like H - - -,

and I believe it's from the stenosis. If it keeps up, I will be

considering more seriously, the injection. I also believe that once

you begin fusion, it eventually leads to more and more deterioration

and more fusions, just like what Sis is dealing with now! It's

really kind of odd that she and I have such similar problems. I

think it would at least be a start to have them do another MRI on

Brady to see if it's advanced. Please let us know what you find out!

Love Lana

> Unfortunately, from all the posts of EDSers who have it, it DOES

and can get worse (

>

> Sorry to hear that Brady has this... I have a friend who had it

in his lower spine and was " roto rooted out " when he had major back

surgery to have rods and screws put in his back. He was doing good

until the screws broke off.... He's on major pain meds because of it.

>

>

>

>

>

[Guest guest]

Cindy -- I'd be really intrested in the articles you've found /urls ect

discussing this - One of the " best " I knew off is no longer on the

web / discussed protacols ect for detecting small criptic leaks in

the brain == but here is one url that may offer insight now - a

Neurosurgical Focus journal issue focued on csf leaks in the

brain and spinal cord with a few good articles .

http://www.aans.org/education/journal/neurosurgical/july00/9-1-

NSF-toc.asp

( July 2000 issue if you have trouble pasting together this long url

)

'

I'll add some info below on three different aquired /or entwined

cases of chiari where Elhers danlos is known in two of them -

and csf leaks involved in the whole pic. too . Two of these are

folks with INTERMITTANT leaks - not always present or

manifesting to the same degree / and my current understanding

of a plan underway to better locate the leak ect .

You wrote -

.. I've been doing allot of reading on the net about Migraines. All

the symptoms point to a spinal fluid leak in the brain. These are

caused by a blunt force or connective tissue disorders. So... Ya

know what I'm thinking. He had no blow to the head for a year.

And he does have (mild) stenosis (narrowing of the spinal

column) in the cervical and Lumbar spine. He see's neurology in

2 weeks again. Guess an MRI or MRA is next.

Two of us currently under the care of Dr Bolonese and Dr

Milhorat at the NY chiari instute have known intermittant leaks

involved in our chiari situation - and concominant EDS -- but let

me start with what I can remember of a different case /and what I

learned sharing the woman's journey to better diagnosis and

care .

A nice gal arrived at the chiari suport group a few years ago

--with an unusual /intresting case that neurosurgeons at NIH got

involved with when I read her story - sugested she seek their

insight as someone in an unusual chiari situation .

Basically -- she'd had skull surgery for ANOTHER condition with

MRI " S showing NO chiari prior to that surgery --THEN developed

chiari symtpoms and new MRI's showed an aquired chiari .

NIH ( Dr Heiss ) offered care ( her local doctors had been

bewildered for a couple years at that point --and had NOT found

the chairi quickly depite it being evident on MRI's taken three

months post the first surgery ) -- and she accepted the offer to go

to NIH . Dr Heiss determined that the chiari had developed as a

result of a csf leak in her brain following the first surgery --and

felt because of the closed hydrodynamic's involved in sustaining

the herniation / ONLY decompresion of the chiari would have a

hope of alleviation of the symtpoms . NO leak could be seen on

MRI's with gadolinium ect as remaining - but once sucked down

- those tonsils REMAIN down with a suction action keeping them

in the spinal cannal too .

He carried out decompresion -being unable to find any

continuing leak -- but surgery to decompress the chiari DID Stop

the symtpoms of compression /cranial nerve dysfunction and

headaches ect .

In my situation -- I had a prior know acm of 7 mm or so --stable

montiored over a full 10 year time frame - then a hard blow to the

head --and my tonsils started decending ( they'd hit 13 mm

herniation by a year later ) -- MRI with gadolinium enhancement

did NOT show any leak of csf in my brain they could detect .

My symtpoms included three considered lifethreatening AND an

odd " unusual " positional impact where sitting up awhile lead to

greater presure in my head - but laying down WITH just the

" Right " angle of head /neck positioning would help bring the

presure headache aspect back to a lower level . At the same

time I could FEEL an odd constant nasel type drainage into the

back of my mouth /throat -- and my regualr doctor had noticed

what she thought was a constant " post nasel " secretion drip or

drainage as well from the eustasian tube .

In my situation -- a very conservative chiari decompresion did

stop the worst life threatening symtpoms -- others remained and

continue a problem now INCLUDING that same variation of

intensity of presure /headache / impacts to some cranial nerve

functions --that VARY " S in intensity . When I goof past my own

limmits -- the headache blows sky high ( as well as muscle

spasms /spacticity ect ) BUT -- IF I get my head /neck in JUST the

right slightly hyperextended position ( VERY slight - a greater

degree causes this to WORSEN -) -- VERY slight suddenly

INCREASES that sensation of fluid draining down into the back

of my throat -- I've got to CONSTANTLY swollow gulps of fluid -

not saliva - and the headache preseure sensation GOES BACK

DOWN - other symtpoms lessen again rapidly ( 15-20 mins of

laying with just that RIGHT degree of hyperextension usually will

help enormously again ) ==

In discussing this with the neurosurgeon --it's probably that

when my presure ( ICP ) does go way up -- a small SLIGHT leak

somewhere springs open --and drains to the eustaisan tube .

I've chosen at this point--to leave well enough alone while

working through the possible /proabable additional problem of a

type of " pseudotumor cerebri " issue where my body

overproduces CSF for the amount it actually absorbs --and

causes intermittant high ICP -- shunting may be my best option

ot help this in the long run -- Ehlers danlos makes greater

decompresion /with the use of a plate a HAZZARDOUS way to fix

the skull shape strutural issue like would be done if I didn't have

EDS . ( I have a congenitally cause form of chiari where the

posterior fossa is sloped in to too great an angle not allowing

the room for my full cerebellem ) -- BUT --

what I want to try and get across is -- this was a problem

BEFORE a blow to the head -- that BLOW caused the chiari itself

to worsen AND SEEMS to have caused a tiny intermittant criptic

csf leak to open as well . IT acts as an escape valve reopening

when I mannage to blow my ICP up high --but isn't ALWAYS

Anoticably present to the same extent --and is clearly

POSITIONAL as well ---but MRI's with gadolinium don't

demonstrate any leak either .

ANOTHER chiarian with ehlers danlos syndrome currently in the

midst of WORSE problems - and facing a couple different

uncomming surgerys with the NY chiari instute has a VERY

similar situation to mine in that constant esutaisan tube

drainage --BUT --when HER presure goes way up -- she spouts

csf through her NOSE as well . ( there are reports of these

intermittant leaks draining out the ears as well in medical

literature ) -- AGAIN her pattern of leaking is intermittant -

positionally influanced --but a GREATER risk of infection entering

her brain through the pathway too ---and they are in the midst of

a workup to try and find the source of her leak now .

Some things that are considered -- MRI " s with VERY FINE

scaning debth of slices may demonstrate this sort of leak -- one

radiologist wrote an indebth article I can't find on the web

anymore saying 3 mm slices . Gadiolinium contrast can be

helpful in this .

A cysternogram can show this sort of leak /source --

You need a VERY experienced team of doctors to truly track

down the very small leaks -- it's an area of specialisation FEW

have much experience with .

I'd sugest you consider taking him to the NY chiari instute for

consultation with Dr Bolognese EVEN if chiari doesn't seem to

be a part of the whole picture -- He does have an understanding

of this unusual problem AND a grasp on how surgerys to work

on fixing such a leak CAN be done safely in someone with

Ehlers Danlos -- at the least do write to him /discuss the

situation /and ask who else he'd recomend .

The reality is this ISN " T an easy sort of thing to track down and

find -- regualr MRI won't demonstrate this sort of leak at all --at

the least gadolinium will need to be used /and those VERY thin

slices will have a better hope of detecting a leak .

Cisternogram can be helpful as well -- I don't remember much of

the detail discussed in doing this test -- I can't have it done due

to an allergy to the type of Dye used in this or in mylogram either

-- and once I'd FOUND that way I can help GET the presure back

down through my own esacape hatch shunting sort of body

positioning allowing better drainage of the leak -- I'm in a holding

pattern while the full shunt evaluation plan unfolds now .

That doesn't mean I don't still need better eval of this too -- just

that it's lower down on the plan / and I'm mannaging it through

body positioning right now with less risk of brain infection that

someone with an intermittant leak through the nose or ears ect .

Hmmm ---VERY tricky stuff -- if a leak is found in your son like

this --then I think Dr Heiss might be intrested in taking him into

study at NIH outside the acm /sm studies underway ( he can

accept UNUSUal cases where science can learn outside the

studies too ) --and he does occationally take peds patients with

unusual problems -- I'd totally trust him as a neurosrugeon as

well -and be glad to send his email addy ect .

This ISN " T a very common problem EVEN within those with

chiari malformations Cindy ---but that EDS overlay IS a factor that

could lead to this sort of situation -- and I'm amoung two I know

off who've been found to have chiari and eds out of the 100 or so

they've identified this year at TCI . It's a NEW finding that there is

an association -- so I'd suspect this ADDITONAL Possible

problem of a criptic csf leak /dura tear is going to be one of those

things BETTER understood as they gain a larger pool of those

affected to study more closely .

Whew -- I hope that's somehow a slight bit of help -at least in

validating your research may well be on the right track AND

offering a couple doctors names /locations with an

understanding -grasp that this can happen and some

experience in treating those with both chiari and eds too .

Sending hugs --and glad to discuss it more /try to help with

finding better info /articles ect too .

in Paradise ( New to your group - new to the EDS

diagnosis --but veteran chiari advocate /retired neuropsych

nurse . )

[Guest guest]

Cindy -- I'd be really intrested in the articles you've found /urls ect

discussing this - One of the " best " I knew off is no longer on the

web / discussed protacols ect for detecting small criptic leaks in

the brain == but here is one url that may offer insight now - a

Neurosurgical Focus journal issue focued on csf leaks in the

brain and spinal cord with a few good articles .

http://www.aans.org/education/journal/neurosurgical/july00/9-1-

NSF-toc.asp

( July 2000 issue if you have trouble pasting together this long url

)

'

I'll add some info below on three different aquired /or entwined

cases of chiari where Elhers danlos is known in two of them -

and csf leaks involved in the whole pic. too . Two of these are

folks with INTERMITTANT leaks - not always present or

manifesting to the same degree / and my current understanding

of a plan underway to better locate the leak ect .

You wrote -

.. I've been doing allot of reading on the net about Migraines. All

the symptoms point to a spinal fluid leak in the brain. These are

caused by a blunt force or connective tissue disorders. So... Ya

know what I'm thinking. He had no blow to the head for a year.

And he does have (mild) stenosis (narrowing of the spinal

column) in the cervical and Lumbar spine. He see's neurology in

2 weeks again. Guess an MRI or MRA is next.

Two of us currently under the care of Dr Bolonese and Dr

Milhorat at the NY chiari instute have known intermittant leaks

involved in our chiari situation - and concominant EDS -- but let

me start with what I can remember of a different case /and what I

learned sharing the woman's journey to better diagnosis and

care .

A nice gal arrived at the chiari suport group a few years ago

--with an unusual /intresting case that neurosurgeons at NIH got

involved with when I read her story - sugested she seek their

insight as someone in an unusual chiari situation .

Basically -- she'd had skull surgery for ANOTHER condition with

MRI " S showing NO chiari prior to that surgery --THEN developed

chiari symtpoms and new MRI's showed an aquired chiari .

NIH ( Dr Heiss ) offered care ( her local doctors had been

bewildered for a couple years at that point --and had NOT found

the chairi quickly depite it being evident on MRI's taken three

months post the first surgery ) -- and she accepted the offer to go

to NIH . Dr Heiss determined that the chiari had developed as a

result of a csf leak in her brain following the first surgery --and

felt because of the closed hydrodynamic's involved in sustaining

the herniation / ONLY decompresion of the chiari would have a

hope of alleviation of the symtpoms . NO leak could be seen on

MRI's with gadolinium ect as remaining - but once sucked down

- those tonsils REMAIN down with a suction action keeping them

in the spinal cannal too .

He carried out decompresion -being unable to find any

continuing leak -- but surgery to decompress the chiari DID Stop

the symtpoms of compression /cranial nerve dysfunction and

headaches ect .

In my situation -- I had a prior know acm of 7 mm or so --stable

montiored over a full 10 year time frame - then a hard blow to the

head --and my tonsils started decending ( they'd hit 13 mm

herniation by a year later ) -- MRI with gadolinium enhancement

did NOT show any leak of csf in my brain they could detect .

My symtpoms included three considered lifethreatening AND an

odd " unusual " positional impact where sitting up awhile lead to

greater presure in my head - but laying down WITH just the

" Right " angle of head /neck positioning would help bring the

presure headache aspect back to a lower level . At the same

time I could FEEL an odd constant nasel type drainage into the

back of my mouth /throat -- and my regualr doctor had noticed

what she thought was a constant " post nasel " secretion drip or

drainage as well from the eustasian tube .

In my situation -- a very conservative chiari decompresion did

stop the worst life threatening symtpoms -- others remained and

continue a problem now INCLUDING that same variation of

intensity of presure /headache / impacts to some cranial nerve

functions --that VARY " S in intensity . When I goof past my own

limmits -- the headache blows sky high ( as well as muscle

spasms /spacticity ect ) BUT -- IF I get my head /neck in JUST the

right slightly hyperextended position ( VERY slight - a greater

degree causes this to WORSEN -) -- VERY slight suddenly

INCREASES that sensation of fluid draining down into the back

of my throat -- I've got to CONSTANTLY swollow gulps of fluid -

not saliva - and the headache preseure sensation GOES BACK

DOWN - other symtpoms lessen again rapidly ( 15-20 mins of

laying with just that RIGHT degree of hyperextension usually will

help enormously again ) ==

In discussing this with the neurosurgeon --it's probably that

when my presure ( ICP ) does go way up -- a small SLIGHT leak

somewhere springs open --and drains to the eustaisan tube .

I've chosen at this point--to leave well enough alone while

working through the possible /proabable additional problem of a

type of " pseudotumor cerebri " issue where my body

overproduces CSF for the amount it actually absorbs --and

causes intermittant high ICP -- shunting may be my best option

ot help this in the long run -- Ehlers danlos makes greater

decompresion /with the use of a plate a HAZZARDOUS way to fix

the skull shape strutural issue like would be done if I didn't have

EDS . ( I have a congenitally cause form of chiari where the

posterior fossa is sloped in to too great an angle not allowing

the room for my full cerebellem ) -- BUT --

what I want to try and get across is -- this was a problem

BEFORE a blow to the head -- that BLOW caused the chiari itself

to worsen AND SEEMS to have caused a tiny intermittant criptic

csf leak to open as well . IT acts as an escape valve reopening

when I mannage to blow my ICP up high --but isn't ALWAYS

Anoticably present to the same extent --and is clearly

POSITIONAL as well ---but MRI's with gadolinium don't

demonstrate any leak either .

ANOTHER chiarian with ehlers danlos syndrome currently in the

midst of WORSE problems - and facing a couple different

uncomming surgerys with the NY chiari instute has a VERY

similar situation to mine in that constant esutaisan tube

drainage --BUT --when HER presure goes way up -- she spouts

csf through her NOSE as well . ( there are reports of these

intermittant leaks draining out the ears as well in medical

literature ) -- AGAIN her pattern of leaking is intermittant -

positionally influanced --but a GREATER risk of infection entering

her brain through the pathway too ---and they are in the midst of

a workup to try and find the source of her leak now .

Some things that are considered -- MRI " s with VERY FINE

scaning debth of slices may demonstrate this sort of leak -- one

radiologist wrote an indebth article I can't find on the web

anymore saying 3 mm slices . Gadiolinium contrast can be

helpful in this .

A cysternogram can show this sort of leak /source --

You need a VERY experienced team of doctors to truly track

down the very small leaks -- it's an area of specialisation FEW

have much experience with .

I'd sugest you consider taking him to the NY chiari instute for

consultation with Dr Bolognese EVEN if chiari doesn't seem to

be a part of the whole picture -- He does have an understanding

of this unusual problem AND a grasp on how surgerys to work

on fixing such a leak CAN be done safely in someone with

Ehlers Danlos -- at the least do write to him /discuss the

situation /and ask who else he'd recomend .

The reality is this ISN " T an easy sort of thing to track down and

find -- regualr MRI won't demonstrate this sort of leak at all --at

the least gadolinium will need to be used /and those VERY thin

slices will have a better hope of detecting a leak .

Cisternogram can be helpful as well -- I don't remember much of

the detail discussed in doing this test -- I can't have it done due

to an allergy to the type of Dye used in this or in mylogram either

-- and once I'd FOUND that way I can help GET the presure back

down through my own esacape hatch shunting sort of body

positioning allowing better drainage of the leak -- I'm in a holding

pattern while the full shunt evaluation plan unfolds now .

That doesn't mean I don't still need better eval of this too -- just

that it's lower down on the plan / and I'm mannaging it through

body positioning right now with less risk of brain infection that

someone with an intermittant leak through the nose or ears ect .

Hmmm ---VERY tricky stuff -- if a leak is found in your son like

this --then I think Dr Heiss might be intrested in taking him into

study at NIH outside the acm /sm studies underway ( he can

accept UNUSUal cases where science can learn outside the

studies too ) --and he does occationally take peds patients with

unusual problems -- I'd totally trust him as a neurosrugeon as

well -and be glad to send his email addy ect .

This ISN " T a very common problem EVEN within those with

chiari malformations Cindy ---but that EDS overlay IS a factor that

could lead to this sort of situation -- and I'm amoung two I know

off who've been found to have chiari and eds out of the 100 or so

they've identified this year at TCI . It's a NEW finding that there is

an association -- so I'd suspect this ADDITONAL Possible

problem of a criptic csf leak /dura tear is going to be one of those

things BETTER understood as they gain a larger pool of those

affected to study more closely .

Whew -- I hope that's somehow a slight bit of help -at least in

validating your research may well be on the right track AND

offering a couple doctors names /locations with an

understanding -grasp that this can happen and some

experience in treating those with both chiari and eds too .

Sending hugs --and glad to discuss it more /try to help with

finding better info /articles ect too .

in Paradise ( New to your group - new to the EDS

diagnosis --but veteran chiari advocate /retired neuropsych

nurse . )