RE: Need a little reassurance

June 1, 2006

Does Gwynn get ivig? Just curious. If not, maybe she needs it??

I am so sorry you are going through all this. I know how hard it is when my

kids are sick. Best wishes..Pam

Tim - cvid age 16

June 1, 2006

Hello Pam,

No Gwynn does not get IVIG, in his original diagnosis her immuno said that

IVIG may be helpful but that he hoped to not need it. I am wondering the same

thing but so far he has not suggested that.

mole1440@... wrote:

Does Gwynn get ivig? Just curious. If not, maybe she needs it??

I am so sorry you are going through all this. I know how hard it is when my

kids are sick. Best wishes..Pam

Tim - cvid age 16

June 1, 2006

Pattie-

No, I don't think this has ever been tested. At least in all the records I

have there is nothing like this. Do you think I should request this from her

immuno? Why would he have not tested this? You are a wealth of knowledge.

Pattie Curran <catholicmomof3@...> wrote:

Function of neutrophils, T Cells etc.not just the levels. People can have

normal levels and still be immune compromised because the cells they have do

not work properly.

~Pattie~

" What matters is not great deeds, but great love. " ~ St. Therese of Lisieux

Family website: www.shwachman.50megs.com

_____

From: [mailto: ] On Behalf Of

sarah larson

Sent: Thursday, June 01, 2006 1:22 PM

Subject: RE: Need a little reassurance

Pattie-

Thanks for the hugs....

I am so ignorant of the terminology, when you say tested the function of

her various immune cells do you mean igg levels?

This is what her immunologist wrote last year,

" I believe she has a primary immune deficiency charecterized by poor

specific antibody formation as demonstrated by the absence of chickenpox

titer despite 2 clinical episodes and vaccination, the low diptheria and

tetnaus antibodies , her absent isohemagglutinin titers, suggesting poor

ability to make antibodies against carbohydrates.

her original labs were as follows....

igg was normal 522,

igg1 was just above normal 387,

igg2 was just above normal 76,

igg3 was low 10,

igg4 was normal at 49

isohemagluttinin titers showed poor anti-carbohydrate capacity as she has

A positive blood, yet despite this the antibody against the b blood group is

only at 1-2 which is extremely low. cell marker studies show low absolute

lymphocyte count at 1284 normal is 3500-6400. The differential of

lymphocyte subsets shows adequate expression of helper and suppressor

phenotypes. Increased number of gamma delta T-cells, increased activated

T-cells at 5%, yet there is normal ration of helper to suppressor cells at 3

to 1.

Is that the kind of tests, if not I don't think so.

Pattie Curran <catholicmomof3@...> wrote:

,

(((HUGS))) you've had a rough go of it!

My boys have low WBCs all the time (abnormal) and we are happy if they eek

up to 6.0 when they are sick. Heck we get excited if they have a 5.0!

<smile> When J was hospitalized with the really bad cellulitis his WBC was

barely normal then. I forget the number-but it wasn't anything to write

home about... you'd think it would be a lot higher..

Have they tested the function of her various immune " cells " ?

Sorry to hear about your MIL, too. Aye yi yi.

~Pattie~

" What matters is not great deeds, but great love. " ~ St. Therese of Lisieux

Family website: www.shwachman.50megs.com

_____

From: [mailto: ] On Behalf Of

sarah larson

Sent: Thursday, June 01, 2006 12:55 AM

Subject: Need a little reassurance

Hello all,

It has been a few weeks since my last post, Gwynn just got out of the

hospital for the 3rd time in 8 weeks. I am looking for a little advice and a

little reassurance. Gwynn has an undefined primary immune deficiency. She

was diagnosed by her immunologist last March as being immune deficient but

he has yet to diagnose which one she specifically has. She got sick more

often then other kids from about 1 1/2 on(I breast fed for 13 months so that

may have helped), but she did not start having major trouble until Sept of

2004 when she entered a real daycare for the first time. She had her first

hospitilization in Dec 2004 when she began vomitting blood. Within 3 days

she became extremely swollen and had lost almost all of her albumin through

her stool. After 16 days in the hospital, a pic line, being fed with TPN

and a stomach biopsy it was determined that she had mono in her stomach.

She was released and the next month included 4 ER visits, a case of the

chicken pox(she has had c. pox twice and been immunized), influenza and a

couple other undiagnosable viruses. This continued into Feb and in March she

was hospitilized with a kidney infection. (she has grade 1 kidney reflux),

We began amoxicillin profolactic and she had a relatively easy summer with

the exception of a couple viruses and 1 case of bronchitis, the fall and

winter were about the same.

Now starting at the end of March she has been in and out of the hospital.

She had another gastoenteritis which caused some vomitting of blood( we were

in for 5 days), then home for almost a month with a couple urgent care

visits, once because she fainted and had awful black marks under her eyes

and once because of stomach pain, and then she was hospitilized with what

appeared to be the mumps, swollen face, high fever etc but then turned

repiratory and almost required her to be moved to ICU because her o2 stats

dropped so low, that was a 4 day stay and then this time she had another

kidney infection.

I feel like we just can not get her stable. Her most recent lab work

outside of the hospital was in early April and her doc said she had

significant improvement from her previous lab work so I am wondering why we

are having such a bad run and what we can do about it. Her doc says the same

thing, she does not have a normal immune system and that is why she keeps

getting so ill and requring hospitilization. That is great advice but that

does not help me keep her healthy. She will be entering kindergarten in the

fall and if we are having this much of a problem now what happens when she

is exposed to 25 kids at a time 5 days a week?

we know that my 6 month old had the same viral illness at the beginning of

May and rode it out pretty uneventfully and she almost had respiratory

failure so it is obvious she is struggling.

My little one is now coughing and has 101 fever, I'm sure something he

picked up at the hospital....Gwynn just started coughing tonight. I am

praying we don't have to go back in right away.

Any suggestions? I am a bit burned out and know that you all know exactly

what I am talking about....it is so great to be able to communicate with

people that know what you are going through. My mother-in-law keeps telling

me Gwynn must be sickly because I don't use a strong enough disinfectant

when I clean or maybe she needs to make sure to teach me how to get all the

germs, it is so hard to explain any of this to someone who does not know

anything about it.

also does anyone else have a little one whose white blood cell count does

not go above normal limits even when they have an infection? Gwynn's was

only 9.2? Her CRP was 5.3 but the WBC never went out of normal limits.

~~

---------------------------------

Do you ?

Everyone is raving about the all-new Beta.

June 1, 2006

In a message dated 5/31/2006 9:56:00 PM Pacific Daylight Time,

mom2gwynn@... writes:

I am looking for a little advice and a little reassurance. Gwynn has an

undefined primary immune deficiency.

_____________________________________________________________________________

Hi ,

My son's PID was undefined for five or more years. This, in retrospect,

was a huge mistake on my part. I trusted his Ped at the time, but all he did

was give antibiotics. What he needed was IVIG.

The infusions have made such a difference in the quality of Bri's life.

Before IVIG, he was sick constantly. Ear, sinus and strep infections.

Sometimes all three at once--but always two to three weeks apart. It was not

going

well.

IVIG has not only allowed him to get well (except for some sinus

infections) he has returned to school, which is the love of his life!! So,

coming from

someone who has been there, I would pursue an exact diagnosis to see if there

is something further that can be done.

Sandi, Mom to --age 13--CVID

June 1, 2006

It can't hurt to test the function.. Better to be safe than sorry, right?

My boys have neutrophil function problems. If you go to

www.shwachmandiamondamerica.org <http://www.shwachmandiamondamerica.org/>

and click on " community " (pop up blocker off!) and then on the blood forum,

you can see a bit of info on some of the function tests there are. I have

not yet added everything back since the hacker crashed it-but am getting

there!

I cannot say for sure why he did not test function. We have run into people

who don't think there is a function problem to be had. if you've got the

numbers on paper, you must be okay. When my boys had their neutrophil

chemotaxis studied, only 17% of J's worked properly and 33% of 's, the

control had 100% of hers work properly.

~Pattie~

" What matters is not great deeds, but great love. " ~ St. Therese of Lisieux

Family website: www.shwachman.50megs.com

_____

From: [mailto: ] On Behalf Of

sarah larson

Sent: Thursday, June 01, 2006 1:37 PM

Subject: RE: Need a little reassurance

Pattie-

No, I don't think this has ever been tested. At least in all the records I

have there is nothing like this. Do you think I should request this from her

immuno? Why would he have not tested this? You are a wealth of knowledge.

..

_____

June 1, 2006

" ...has a primary immune deficiency charecterized by poor specific antibody

formation as demonstrated by the absence of chickenpox titer despite 2 clinical

episodes and vaccination, the low diptheria and tetnaus antibodies, her absent

isohemagglutinin titers, suggesting poor ability to make antibodies against

carbohydrates... "

-

Based on what you wrote, it seems that the clinical picture reflects what is

going on in her blood. If the antibiotic trial was not a success and she is

still suffering, IVIG is a generally considered. Some PIDers survive okay

with antibiotic only. However, I do not believe that avoiding mortality is the

goal here -- the children's quality of life matters and, usually, kiddos with

compromised immune sympoms with clinical signs significantly benefit from IVIG.

If your doc is leaving your daughter with antibiotics only ... given everything

she has been through/going through...it makes me wonder of the quality of care

you are getting...

There are a lot of well meaning docs that practice in different camps -- some

are more or less conservative than others when it comes to antibiotics, testing,

or IVIG. Some only initiate IVIG if the child has had > 1+ near death

experience and are essentially acting as gatekeepers. Other docs care about

quality of life issues and will place a child on IVIG when the immune system is

dyfunctional -- and the child is suffering.

Many times, the docs only see a snapshot of a beautiful child coming through

the office and just can't wrap their head around the daily suffering. You

really have a battle to fight here. My heart goes out to you.

Keep in mind that there are often no identifiable/precise defect before

treatment -- it is often just a description of what is going on and not a

cause/effect explanation (e.g., hypogam, low titers, T-cell dysfunction, CVID,

etc.).

In our case, my daughter did not get an diagnosis until many months after IVIG

-- and the exact defect is still not confirmed. However, she fits the criteria

for CVIDand likely has an enzymatic defect -- resulting in an undeveloped immune

system.

If your daughter is suffering, tests show odd function, and she is not living

a " normal " life with antibiotics then you need more help. If you can't get what

your daughter needs from her current immuno (and I see that you are have been

trying to work through stuff) then you have to consider another opinion. I

hope that conducting more blood studies will get your immuno to do the right

thing...but it is not clear that he/she is paying close attention to what is

already going on...I just don't know...

mom to CVIDer

June 1, 2006

" ...does anyone else have a little one whose white blood cell count does not go

above normal limits even when they have an infection? "

-

I am finally getting through all of the messages and finally read your entire

post.

The answer is, yes, many PIDers have irregular levels of WBCs -- some spike

too high for too long (lymph nodes are swollen much of the time) and others run

low all of the time. In our case, my daughter will only get to " normal " range

with a serious infection. She can't mount a " normal " response.

Also, I am really fuzzy about your daughter's history and don't understand why

she is not on IVIG or SCIG. What state do you live in? Maybe there are some

mom's in this group that can support you in getting answers -- or at least

getting your daughter the best possible care.

mom to CVIDer

June 2, 2006

Hello -

When her doc first diagnosed her as immune deficient he said he thought she

may need IVIG. However on her third set of labs 1 year later it appeared that

she was starting to build titers after her last round of innoculations so we

were hoping things were getting better. I no longer think this is the case and

would like further blood work as we are obviously not controlling it if she is

in the hospital 3 times in 8 weeks. We live in MN and her doc is well known and

frequently speaks at the IDF functions. So I never have second guessed him, and

I am not neccesarily now but I don't feel comfortable just waiting to see what

happens next!

He is out of town and so I have not been able to talk to him but I think I am

going to voice my concerns as soon as possible.

What would you do? Any one else in MN that can suggest a doc. Gwynn sees Dr.

Shapiro. He is well known isn't he? Anyone had any problems with him?

Thanks,

Schulman <dietdoc@...> wrote:

" ...does anyone else have a little one whose white blood cell count does not

go above normal limits even when they have an infection? "

-

I am finally getting through all of the messages and finally read your entire

post.

The answer is, yes, many PIDers have irregular levels of WBCs -- some spike

too high for too long (lymph nodes are swollen much of the time) and others run

low all of the time. In our case, my daughter will only get to " normal " range

with a serious infection. She can't mount a " normal " response.

Also, I am really fuzzy about your daughter's history and don't understand why

she is not on IVIG or SCIG. What state do you live in? Maybe there are some

mom's in this group that can support you in getting answers -- or at least

getting your daughter the best possible care.

mom to CVIDer

June 2, 2006

My dd sees Dr. Shapiro. He's wonderful. I would call for an appointment

and give them a bit of your child's most recent hospitalizations. I bet

that can get you in earlier than you expect.

Pam

mom to 4

Rebekah, 6, CVID and ??

What would you do? Any one else in MN that can suggest a doc. Gwynn sees

Dr. Shapiro. He is well known isn't he? Anyone had any problems with him?

Thanks,

Schulman <dietdoc@...> wrote:

" ...does anyone else have a little one whose white blood cell count does

not go above normal limits even when they have an infection? "

-

June 2, 2006

In a message dated 6/2/2006 10:52:13 AM Central Standard Time,

mom2gwynn@... writes:

> However on her third set of labs 1 year later it appeared that she was

> starting to build titers after her last round of innoculations so we were

hoping

> things were getting better. I no longer think this is the case

:

Maybe it's possible that she's building the titers after a time, then

dropping off again? can make blips here and there after vaccination (but

never

normal level of response), but those blips can build upon one another for a

time until you start to think maybe enough titers are protective. But then over

the course of about a year, they all drop off. Maybe look at the trend overall

-- I graphed 's responses and it was illuminating.

(mom to , age 7, dairy intolerant-related GERD -- currently has

polysaccharide antibody def, previously had transient IgG, IgA, t-cell & other

defs... and also to Kate, age 3!, more dairy intolerant but very healthy!)

June 2, 2006

:

I lost the original post, but weren't you the one whose MIL was saying your

housekeeping may be to blame for Gwynn's illnesses?

I just wanted to share the fact that many, many of us here have had

well-meaning-but-ignorant family members or friends, as well as

oddly-malicious-and-accusing family members and friends, say the wrong thing at

every possible

juncture in this journey. I have a SIL who seems to be jealous at the

" attention "

that 's PID gives us and makes biting little comments here or there, I have

a MIL who is always pointing out other kids who have it worse (You think I

don't know there are worse things out there than what has? But other kids

suffering doesn't make me feel better!!!), and trying to blow sunshine up my

skirt by raving on and on about some little thing that has right with her

PID and whitewashing over any of her suffering.... and neither of the chicks

can get any of the details straight. They misunderstand certain facts or just

plain don't listen, then they " repeat " this info to other people -- it drives

me nuts.

I finally learned just not to give anybody too much info. It was too

frustrating having to continually educate them and they never got it no matter

how

many times I tried. I simply don't discuss 's health or situation with the

SIL now, and I don't discuss the particulars of the testing or the indepth

stuff with anybody really, except this group. Nobody else understands it. It may

help to have some pat responses at the ready. So if she comes at you about the

housekeeping you can simply reply, " No, her doctor says she was born with it "

or something like that. I know, it's really aggravating to have to put up with

ignorance when you are already dealing with so much. But it seems to be a

theme!

(mom to , age 7, dairy intolerant-related GERD -- currently has

polysaccharide antibody def, previously had transient IgG, IgA, t-cell & other

defs... and also to Kate, age 3!, more dairy intolerant but very healthy!)

June 2, 2006

In a message dated 6/2/2006 11:57:47 PM Central Standard Time,

mom2gwynn@... writes:

> she loves the attention so she starts the phone game

Oh, I hear ya. We were at a Mc's and MIL told the cashier she wanted

ketchup packets (instead of using the ketchup pumps) " ...because SHE (pointing

at ) has an immune disorder. "

After we sat down I told her I didn't want her talking about the PID in front

of . Fortunately was only like 3 at the time and didn't know what

was going on. I told her you can ASK for ketchup without explaining why, after

all. Oh, brother.

(mom to , age 7, dairy intolerant-related GERD -- currently has

polysaccharide antibody def, previously had transient IgG, IgA, t-cell & other

defs... and also to Kate, age 3!, more dairy intolerant but very healthy!)

June 2, 2006

,

It is nice to hear that I am not alone. I have a SIL like that as well but my

MIL is the worst. She passes on misinformation to everyone, especially her

parents. They are fairly elderly and I don't notify everyone every time we are

at the hospital but she loves the attention so she starts the phone game. Gwynn

had a kidney infection this week and when I checked my messages from the

hospital it had gotten around that she had kidney failure. She does not

understand the immune deficiency and keeps saying how can it be genetic?....I

don't know anyone that has it. And god forbid we both be recessive because that

would mean it may have come from her side as well. When she is misinformed I try

to correct her but this is a woman that once told me her car did not have spark

plugs...

bunneegirl@... wrote: