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I guess I should say the part that scares me the most, is the fluid around his

heart. I can't believe how sick he became and how quickly it happened. His

symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th he

had kidney damage and by the 24th the labs were showing liver damage and on the

27th he had a moderate peripcardial effusion, which was not there on the 22nd.

Now I sit here and watch and wonder what is going on in his body.

Amy - avery 17 sojra

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You know, I wonder.........

I read a study last month posted on here which got me thinking. It was on

cytomegalovirus. but it mentioned macrophage activation syndrome. It was from

Israel, and it said that some believe that up to 50% of systemics have occult

MAS. Occult means hidden. So it might show up on labs, but they might not have

the outward signs of it. Then I asked our ped rheumy about it. He said many

rheumys believe, and Carol Wallace at Seattle Children's is one, that many

systemics are right on the line of MAS. The labs get out of whack. The liver,

the kidneys, the platelets. My research has led me to believe that only about

7% of systemics get MAS, but some rheumys think it is much higher and may

approach that 50% mark.

When you mentioned Avery's liver and kidney, I started to think. n's

started to go when she had MAS. And for the first time ever in the disease she

got fluid around her heart. Now some systemics get fluid around the heart

without having MAS. The first line of defense of MAS is high dose steroids.

Specifically, pulsed steroids. n got 1000mg a day for 5 days. But that

isn't what pulled her out of her distress. It was cyclosporine. (n,

21, systemic)

On Oct 2, 2010, at 3:10 PM, Amy Spurger wrote:

> I guess I should say the part that scares me the most, is the fluid around his

> heart. I can't believe how sick he became and how quickly it happened. His

> symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th he

> had kidney damage and by the 24th the labs were showing liver damage and on

the

> 27th he had a moderate peripcardial effusion, which was not there on the 22nd.

> Now I sit here and watch and wonder what is going on in his body.

>

> Amy - avery 17 sojra

>

>

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Thanks I'm going to research and then ask the dr wednesday. We got pulse

for 3 days this week.

Amy - Avery 17 systemic

________________________________

From: Price <bc.price@...>

Sent: Sat, October 2, 2010 5:22:25 PM

Subject: Re: The part that scares me

You know, I wonder.........

I read a study last month posted on here which got me thinking. It was on

cytomegalovirus. but it mentioned macrophage activation syndrome. It was from

Israel, and it said that some believe that up to 50% of systemics have occult

MAS. Occult means hidden. So it might show up on labs, but they might not have

the outward signs of it. Then I asked our ped rheumy about it. He said many

rheumys believe, and Carol Wallace at Seattle Children's is one, that many

systemics are right on the line of MAS. The labs get out of whack. The liver,

the kidneys, the platelets. My research has led me to believe that only about

7% of systemics get MAS, but some rheumys think it is much higher and may

approach that 50% mark.

When you mentioned Avery's liver and kidney, I started to think. n's

started to go when she had MAS. And for the first time ever in the disease she

got fluid around her heart. Now some systemics get fluid around the heart

without having MAS. The first line of defense of MAS is high dose steroids.

Specifically, pulsed steroids. n got 1000mg a day for 5 days. But that

isn't what pulled her out of her distress. It was cyclosporine. (n,

21, systemic)

On Oct 2, 2010, at 3:10 PM, Amy Spurger wrote:

> I guess I should say the part that scares me the most, is the fluid around his

> heart. I can't believe how sick he became and how quickly it happened. His

> symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th he

> had kidney damage and by the 24th the labs were showing liver damage and on

the

>

> 27th he had a moderate peripcardial effusion, which was not there on the 22nd.

> Now I sit here and watch and wonder what is going on in his body.

>

> Amy - avery 17 sojra

>

>

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I am sure the doc would be on top of it. Steroids usually do the trick if he is

borderline of going into it. (n, 21, systemic and MAS survivor)

On Oct 2, 2010, at 3:34 PM, Amy Spurger wrote:

> Thanks I'm going to research and then ask the dr wednesday. We got pulse

> for 3 days this week.

> Amy - Avery 17 systemic

>

> ________________________________

> From: Price <bc.price@...>

>

> Sent: Sat, October 2, 2010 5:22:25 PM

> Subject: Re: The part that scares me

>

> You know, I wonder.........

>

> I read a study last month posted on here which got me thinking. It was on

> cytomegalovirus. but it mentioned macrophage activation syndrome. It was from

> Israel, and it said that some believe that up to 50% of systemics have occult

> MAS. Occult means hidden. So it might show up on labs, but they might not have

> the outward signs of it. Then I asked our ped rheumy about it. He said many

> rheumys believe, and Carol Wallace at Seattle Children's is one, that many

> systemics are right on the line of MAS. The labs get out of whack. The liver,

> the kidneys, the platelets. My research has led me to believe that only about

> 7% of systemics get MAS, but some rheumys think it is much higher and may

> approach that 50% mark.

>

> When you mentioned Avery's liver and kidney, I started to think. n's

> started to go when she had MAS. And for the first time ever in the disease she

> got fluid around her heart. Now some systemics get fluid around the heart

> without having MAS. The first line of defense of MAS is high dose steroids.

> Specifically, pulsed steroids. n got 1000mg a day for 5 days. But that

> isn't what pulled her out of her distress. It was cyclosporine. (n,

> 21, systemic)

>

> On Oct 2, 2010, at 3:10 PM, Amy Spurger wrote:

>

> > I guess I should say the part that scares me the most, is the fluid around

his

>

> > heart. I can't believe how sick he became and how quickly it happened. His

> > symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th

he

>

> > had kidney damage and by the 24th the labs were showing liver damage and on

the

> >

> > 27th he had a moderate peripcardial effusion, which was not there on the

22nd.

>

> > Now I sit here and watch and wonder what is going on in his body.

> >

> > Amy - avery 17 sojra

> >

> >

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That might explain why they keep doing pt's and ptt's. lol It does sound a lot

like him and it also sounds like the dr's are watching for it.

Amy - avery 17 systemic

________________________________

From: Price <bc.price@...>

Sent: Sat, October 2, 2010 5:37:31 PM

Subject: Re: The part that scares me

I am sure the doc would be on top of it. Steroids usually do the trick if he is

borderline of going into it. (n, 21, systemic and MAS survivor)

On Oct 2, 2010, at 3:34 PM, Amy Spurger wrote:

> Thanks I'm going to research and then ask the dr wednesday. We got pulse

> for 3 days this week.

> Amy - Avery 17 systemic

>

> ________________________________

> From: Price <bc.price@...>

>

> Sent: Sat, October 2, 2010 5:22:25 PM

> Subject: Re: The part that scares me

>

> You know, I wonder.........

>

> I read a study last month posted on here which got me thinking. It was on

> cytomegalovirus. but it mentioned macrophage activation syndrome. It was from

> Israel, and it said that some believe that up to 50% of systemics have occult

> MAS. Occult means hidden. So it might show up on labs, but they might not have

> the outward signs of it. Then I asked our ped rheumy about it. He said many

> rheumys believe, and Carol Wallace at Seattle Children's is one, that many

> systemics are right on the line of MAS. The labs get out of whack. The liver,

> the kidneys, the platelets. My research has led me to believe that only about

> 7% of systemics get MAS, but some rheumys think it is much higher and may

> approach that 50% mark.

>

> When you mentioned Avery's liver and kidney, I started to think. n's

> started to go when she had MAS. And for the first time ever in the disease she

> got fluid around her heart. Now some systemics get fluid around the heart

> without having MAS. The first line of defense of MAS is high dose steroids.

> Specifically, pulsed steroids. n got 1000mg a day for 5 days. But that

> isn't what pulled her out of her distress. It was cyclosporine. (n,

> 21, systemic)

>

> On Oct 2, 2010, at 3:10 PM, Amy Spurger wrote:

>

> > I guess I should say the part that scares me the most, is the fluid around

>his

>

>

> > heart. I can't believe how sick he became and how quickly it happened. His

> > symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th

>he

>

>

> > had kidney damage and by the 24th the labs were showing liver damage and on

>the

>

> >

> > 27th he had a moderate peripcardial effusion, which was not there on the

>22nd.

>

>

> > Now I sit here and watch and wonder what is going on in his body.

> >

> > Amy - avery 17 sojra

> >

> >

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Let your rheumy know what is going through your head. I know it's a scary

process. I do not have systemic arthritis but I know enough about it to say that

its scary. I hope your little one gets better soon.

Prayers and best wishes

Xoxo

Shea

Sent from my iPad

On Oct 2, 2010, at 6:10 PM, Amy Spurger <mom2anz@...> wrote:

I guess I should say the part that scares me the most, is the fluid around his

heart. I can't believe how sick he became and how quickly it happened. His

symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th he

had kidney damage and by the 24th the labs were showing liver damage and on the

27th he had a moderate peripcardial effusion, which was not there on the 22nd.

Now I sit here and watch and wonder what is going on in his body.

Amy - avery 17 sojra

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I've got two standing lab orders for - the routine one (every 3-4 weeks)

for the CBC, ESR, and CRP. We also do a liver panel on that one (CMP). BUT

since they thought was borderline MAS a few months ago we also have the

second standing order for if she is ever really sick - it includes PT, PTT,

ferritin, and fibrinogen. Sometimes they watch D-dimer as well.

Good luck with the appointment! The pericarditis is scary, especially in how it

can sneak up quickly. It sounds like Avery's getting really great care Amy, I'm

so glad. Keep in touch.

nn (, 9, systemic)

> >

> > > I guess I should say the part that scares me the most, is the fluid around

> >his

> >

> >

> > > heart. I can't believe how sick he became and how quickly it happened. His

> > > symptoms started on Sept 5 and on the 6th his labs were normal. By the

13th

> >he

> >

> >

> > > had kidney damage and by the 24th the labs were showing liver damage and

on

> >the

> >

> > >

> > > 27th he had a moderate peripcardial effusion, which was not there on the

> >22nd.

> >

> >

> > > Now I sit here and watch and wonder what is going on in his body.

> > >

> > > Amy - avery 17 sojra

> > >

> > >

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Hi Amy-

 

I think that is really on the money (as usual) :)...... and i wanted to

add that in the original trials of Actemra (tocilizumab) in Japan, it worked

well on many kids who had suffered from MAS...... but of course high dose

steroids and cyclosporine are the number one defense.

 

And as a side note - I think  the $250 K for rilonacept is probably pretty

accurate, as crazy as that seems.  When we were in the trial... things weren't

going great, but they weren't terrible either.  We were sort of out of choices

at the time... and I would not have chosen to discontinue if given a choice. 

But the drug company decided to halt the trial for systemic kids, and pursue FDA

approval for periodic fever syndrome only.  They told me to come in and return

all of my remaining drug immediately..... and then I learned that they were

going to market the drug for over $200K.  Wow.......

 

That being said -  if it worked well, I wouldn't really care how much it

cost.....

 

Colleen

 

From: Amy Spurger <mom2anz@...>

Subject: The part that scares me

Date: Saturday, October 2, 2010, 3:10 PM

 

I guess I should say the part that scares me the most, is the fluid around his

heart. I can't believe how sick he became and how quickly it happened. His

symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th he

had kidney damage and by the 24th the labs were showing liver damage and on the

27th he had a moderate peripcardial effusion, which was not there on the 22nd.

Now I sit here and watch and wonder what is going on in his body.

Amy - avery 17 sojra

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I know about the PT and PTT and of the common ones.That is great they are

tracking those.The PT and PTT are blood clotting tests which you need to know in

case of surgery.In Avery's case keeping track of the Ferritan and Fibrinogen are

realy important.MASS is a parents worst nightmare.

Much love and many prayers,

Becki and 12 systemic onset

________________________________

From: nn <rosannwinn@...>

Sent: Sat, October 2, 2010 7:13:43 PM

Subject: Re: The part that scares me

I've got two standing lab orders for - the routine one (every 3-4 weeks)

for the CBC, ESR, and CRP. We also do a liver panel on that one (CMP). BUT

since they thought was borderline MAS a few months ago we also have the

second standing order for if she is ever really sick - it includes PT, PTT,

ferritin, and fibrinogen. Sometimes they watch D-dimer as well.

Good luck with the appointment! The pericarditis is scary, especially in how it

can sneak up quickly. It sounds like Avery's getting really great care Amy, I'm

so glad. Keep in touch.

nn (, 9, systemic)

> >

> > > I guess I should say the part that scares me the most, is the fluid around

> >his

> >

> >

> > > heart. I can't believe how sick he became and how quickly it happened. His

> > > symptoms started on Sept 5 and on the 6th his labs were normal. By the

13th

>

> >he

> >

> >

> > > had kidney damage and by the 24th the labs were showing liver damage and

on

>

> >the

> >

> > >

> > > 27th he had a moderate peripcardial effusion, which was not there on the

> >22nd.

> >

> >

> > > Now I sit here and watch and wonder what is going on in his body.

> > >

> > > Amy - avery 17 sojra

> > >

> > >

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Yep, it is scary when your child goes from ok to MAJOR pain,fever and rash

within a weeks time.Sounds like we got a bit luckier then you as far as within

21 days no major organs were involved but also got to start high doses of

pred. I tried staying away from this list because my son is doing so well but I

keep being drawn back.There is a realy GREAT chance of a combo of meds that will

bring relief and ultimately remisssion. AS Much as it may scare you slam Artrhur

hard and fast.You can't mix Biologicals but you can gradualy max out on sub q

injections of 25mg Methotrexate and take the full adult dose of Kiniret and take

300mg of Plaquenil a day.Once things settle down you can drain and inject the

stubborn joints with steroids.

From: Amy Spurger <mom2anz@...>

Sent: Sat, October 2, 2010 5:10:00 PM

Subject: The part that scares me

I guess I should say the part that scares me the most, is the fluid around his

heart. I can't believe how sick he became and how quickly it happened. His

symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th he

had kidney damage and by the 24th the labs were showing liver damage and on the

27th he had a moderate peripcardial effusion, which was not there on the 22nd.

Now I sit here and watch and wonder what is going on in his body.

Amy - avery 17 sojra

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And we are glad you keep being drawn back. It gives us hope for our

children. ( n,21, systemic)

Sent from my iPod

On Oct 2, 2010, at 9:17 PM, Becki Larson <sojramom@...> wrote:

> Yep, it is scary when your child goes from ok to MAJOR pain,fever

> and rash

> within a weeks time.Sounds like we got a bit luckier then you as far

> as within

> 21 days no major organs were involved but also got to start

> high doses of

> pred. I tried staying away from this list because my son is doing so

> well but I

> keep being drawn back.There is a realy GREAT chance of a combo of

> meds that will

> bring relief and ultimately remisssion. AS Much as it may scare you

> slam Artrhur

> hard and fast.You can't mix Biologicals but you can gradualy max out

> on sub q

> injections of 25mg Methotrexate and take the full adult dose of

> Kiniret and take

> 300mg of Plaquenil a day.Once things settle down you can drain and

> inject the

> stubborn joints with steroids.

> From: Amy Spurger <mom2anz@...>

>

> Sent: Sat, October 2, 2010 5:10:00 PM

> Subject: The part that scares me

>

> I guess I should say the part that scares me the most, is the fluid

> around his

> heart. I can't believe how sick he became and how quickly it

> happened. His

> symptoms started on Sept 5 and on the 6th his labs were normal. By

> the 13th he

> had kidney damage and by the 24th the labs were showing liver damage

> and on the

> 27th he had a moderate peripcardial effusion, which was not there on

> the 22nd.

> Now I sit here and watch and wonder what is going on in his body.

>

> Amy - avery 17 sojra

>

>

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they told me his ferriton was up to 30,000 at one point but now down to 5,000.

I am sure one day I will know what all this means. lol

amy - avery 17 systemic

________________________________

From: nn <rosannwinn@...>

Sent: Sat, October 2, 2010 7:13:43 PM

Subject: Re: The part that scares me

I've got two standing lab orders for - the routine one (every 3-4 weeks)

for the CBC, ESR, and CRP. We also do a liver panel on that one (CMP). BUT

since they thought was borderline MAS a few months ago we also have the

second standing order for if she is ever really sick - it includes PT, PTT,

ferritin, and fibrinogen. Sometimes they watch D-dimer as well.

Good luck with the appointment! The pericarditis is scary, especially in how it

can sneak up quickly. It sounds like Avery's getting really great care Amy, I'm

so glad. Keep in touch.

nn (, 9, systemic)

> >

> > > I guess I should say the part that scares me the most, is the fluid around

> >his

> >

> >

> > > heart. I can't believe how sick he became and how quickly it happened. His

> > > symptoms started on Sept 5 and on the 6th his labs were normal. By the

13th

>

> >he

> >

> >

> > > had kidney damage and by the 24th the labs were showing liver damage and

on

>

> >the

> >

> > >

> > > 27th he had a moderate peripcardial effusion, which was not there on the

> >22nd.

> >

> >

> > > Now I sit here and watch and wonder what is going on in his body.

> > >

> > > Amy - avery 17 sojra

> > >

> > >

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Bless you , Your experience and ordeal with your own precious child may

save a life.When I joined I know she was older then .Either way you did a

fine job and have a smart,beautiful, intelligent daughter who will make a big

impact on our world.

________________________________

From: Amy Spurger <mom2anz@...>

Sent: Sat, October 2, 2010 5:34:55 PM

Subject: Re: The part that scares me

Thanks I'm going to research and then ask the dr wednesday. We got pulse

for 3 days this week.

Amy - Avery 17 systemic

________________________________

From: Price <bc.price@...>

Sent: Sat, October 2, 2010 5:22:25 PM

Subject: Re: The part that scares me

You know, I wonder.........

I read a study last month posted on here which got me thinking. It was on

cytomegalovirus. but it mentioned macrophage activation syndrome. It was from

Israel, and it said that some believe that up to 50% of systemics have occult

MAS. Occult means hidden. So it might show up on labs, but they might not have

the outward signs of it. Then I asked our ped rheumy about it. He said many

rheumys believe, and Carol Wallace at Seattle Children's is one, that many

systemics are right on the line of MAS. The labs get out of whack. The liver,

the kidneys, the platelets. My research has led me to believe that only about

7% of systemics get MAS, but some rheumys think it is much higher and may

approach that 50% mark.

When you mentioned Avery's liver and kidney, I started to think. n's

started to go when she had MAS. And for the first time ever in the disease she

got fluid around her heart. Now some systemics get fluid around the heart

without having MAS. The first line of defense of MAS is high dose steroids.

Specifically, pulsed steroids. n got 1000mg a day for 5 days. But that

isn't what pulled her out of her distress. It was cyclosporine. (n,

21, systemic)

On Oct 2, 2010, at 3:10 PM, Amy Spurger wrote:

> I guess I should say the part that scares me the most, is the fluid around his

> heart. I can't believe how sick he became and how quickly it happened. His

> symptoms started on Sept 5 and on the 6th his labs were normal. By the 13th he

> had kidney damage and by the 24th the labs were showing liver damage and on

the

>

>

> 27th he had a moderate peripcardial effusion, which was not there on the 22nd.

> Now I sit here and watch and wonder what is going on in his body.

>

> Amy - avery 17 sojra

>

>

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