RE: Nadini IVIG cont.

[Guest guest]

I read your mail a little more in detail, I apologize for not doing

so previously. Rishabh has normal IgA levels , does that mean he cannot have

X linked Hyper - IgM Syndrome or should I still have it checked out.

Reading the discription clearly it sounded like my sone in his formative

years.

He is 12 now & it's been years since he's gotten a fever. He got many

respiratory infections & GI tract infections bet the age of 1 1/2 & 5 years,

all treated with antibiotics....ugh...

Unfortunately got all his shots. He was disgnosed with Autism at age 4 1/2.

At that time Drs just gave you the diagnosis & refused t look at the

physical manifestations of the disorder. Now of course there is a lot more

research out there.

Thaks for the information

>From: Schulman <dietdoc@...>

>Reply-

>

>Subject: Re: Nadini IVIG

>Date: Thu, 21 Sep 2006 15:10:32 -0700 (PDT)

>

>Nandini,

>

>Where are you located? It would help if you could tell me the name of your

>hospital and/or immunologist, but don't feel obligated.

>

>Most experienced immunologists, particularly those affiliated with Jeffery

>Modell Center's of Excellence, can run the needed tests. X-linked

>hyper-IgM is considered an immune disorder and blood tests for this

>condition should be run (e.g., CD40L and possibly others). If this

>condition is a possibility, I would get it evaluated soon.

>

>To learn about X-linked hyper-IgM syndrome, read the following:

>http://www.primaryimmune.org/pubs/book_pats/e_ch08.pdf

>

>Here is the Jeffery Modell's Expert Locater:

>http://www.info4pi.org/expertlocator/index.cfm?section=expertlocator & CFID=12515\

484 & CFTOKEN=20411676

>

>Finally, if you are not getting answers, I would urge you to call the IDF

>or have your doctor contact IDF for a free consult. Every patient deserves

>the benefit of early diagnosis.

>http://www.primaryimmune.org/med_programs/immunologist_prog.htm

>

>I think more people will chime in soon. It is helpful to sign your notes

>with your name, your child's name, diagnosis, age, or something that helps

>families know who they are talking to. = )

>

>

>mom to Dani (4) CVID

>

>

[Guest guest]

" Rishabh has normal IgA levels , does that mean he cannot have X linked Hyper -

IgM Syndrome or should I still have it checked out " ?

Nadini -

I believe that normal IgA might rule it out but I would definitely check with

your doc. Immunologists are finding mutations/variants of many classic primary

immune disorders. There is a mom on our board who might be facing an unusual

variation of hyper-IgM and she might chime in.

Most of the children with this disorder are much younger (or, perhaps,

undiagnosed).

So, my advice is to check with Dr. G.

mom to CVIDer

[Guest guest]

Nandini do you remember what his IgM is?

Ursula - mom to (13) and Macey (11, CVID)

http://www.primaryimmune.org

http://www.jmfworld.org

http://members.cox.net/maceyh

[Guest guest]

Ursula -

I have been referring people to the IDF materials and now wonder if they are

current.

IDF materials read that in hyper-IgM you get low IgA and low IgG. But, I just

read elsewhere that: " Hyper IgM syndrome (HIGM) is a congenital primary

immunodeficiency characterized by high serum IgM levels with normal to low

levels of serum IgG and IgA " .

Any insights?

[Guest guest]

I just thought I'd jump in and tell you that my son Conner (also 12 and on

the " spectrum " ) was diagnosed last week with NEMO a form of X-linked

Hyper-IgM (XHIM). In our case, the third time was a charm. We had seen two

other immunologists who missed the diagnosis, it was our third doc, Jack

Bleesing at Cincinnati Children's Hospital, who diagnosed the disorder.

I've done a lot of reading of XHIM in the last couple of months and did

learn that there have been a few documented cases of normal IgA with this

disorder (it's rare). In our case, Conner's IgA and IgG are very low, his

IgM has run low to normal and IgE has been normal to very elevated. From

what I've learned, this disorder can present very differently from one case

to the next.

Incidently, we have also seen Conner's ability to mount a fever slowly

disappear over the last couple of years. I'm not sure why this happens, if

anyone has any insight, I'd love to learn more.

Take care,

Mom to Conner (12, unknown combined immune deficiency, lymphadenopathy,

asthma,

Hashimoto's disease and resolved adrenal insufficiency),

Hayden (12, unknown immune deficiency, IBS and moderate hearing loss/aided),

Evan (12, unknown immune deficiency, asthma and mild hearing loss/unaided),

and Kelsey - (10, unknown immune deficiency and asthma)

Please visit us at www.caringbridge.org/in/connersmith

[Guest guest]

Ursula his IgG was 844 ( 893 - 1893 )

IgA was 205 ( 70 - 432)

IgM was 87 (52 - 367 )

sorry it was IgE that was high 380 reference range <114

His WBC counts were below range in 1 test & then just about in range in the

next.

so I guess I mispoke & possibly the X Linked syndrome you mentioned is

probably not possible. Sorry for causing the confusion, I'm totally new to

all this.

Do these nos. however raise some red flags ?

And if someone could answer my original question, does IVIG help the gut,

getting rid of yeast & Bacteria ? and what side reactions should one expect.

Thanks you all, and again sorry for the confusion.

Nandini , mom to 12 yr old with Autism & IgG difficiency

>From: " Ursula Holleman " <uahollem1@...>

>Reply-

>< >

>Subject: Re: Nadini IVIG cont.

>Date: Fri, 22 Sep 2006 00:17:30 -0400

>

>Nandini do you remember what his IgM is?

>

>Ursula - mom to (13) and Macey (11, CVID)

>http://www.primaryimmune.org

>http://www.jmfworld.org

>http://members.cox.net/maceyh

>

>

>

[Guest guest]

Yeast is a t-cell problem. While some patients have seen improvement with

their t-cell illnesses and numbers while on IVIG, it's not the norm. His

numbers are encouraging. His IgE of course shows he's allergic to probably alot

of things. Has he ever had skin testing or any type of allergy blood work done?

The gut is coated with IgA and since there are only trace amounts in IVIG it's

not the best treatment. Some patients (pid and non-pid) have attempted oral IgG

medication. I think some BMT-SCID patients have used it for GVHD of the gut.

Mixed results. I may have asked before but have they tested any function of his

IgG. Vaccination titers are good for this. Pre and post levels help determine

if the immune system can mount a response. That way even if the IgG level is

normal you know whether it has any " kick " .

Ursula - mom to (13) and Macey (11, CVID)

http://www.primaryimmune.org

http://www.jmfworld.org

http://members.cox.net/maceyh

[Guest guest]

I think Dr. Gelfand at National Jewish is also doing some research on this.

When I spoke to him about jacob's case, he asked if anyone had mentioned

NEMO??? I said no, but then when I looked it up, I realized that that was

where Dr. K was headed in terms of wanting to know about his teeth....

's IGA is low normal, low IGG, IGM is low, IGE is normal; sub class 3

deficiency...NK cell defect and mod neutropenia and selective antibody

deficiency. I believe he has some complement deficiency as well that no one

has addressed. We are going to the heme in November and I will bring any

other questions there.

I can also ask at the IDF family meeting in OCT regarding some of this.

LINDA S

, hypogam, selective antibody deficiency with NK cell defect on SCIG and

doing well........

Quoting kristinsmith <kristinsmith@...>:

> I just thought I'd jump in and tell you that my son Conner (also 12 and on

> the " spectrum " ) was diagnosed last week with NEMO a form of X-linked

> Hyper-IgM (XHIM). In our case, the third time was a charm. We had seen two

>

> other immunologists who missed the diagnosis, it was our third doc, Jack

> Bleesing at Cincinnati Children's Hospital, who diagnosed the disorder.

>

> I've done a lot of reading of XHIM in the last couple of months and did

> learn that there have been a few documented cases of normal IgA with this

> disorder (it's rare). In our case, Conner's IgA and IgG are very low, his

> IgM has run low to normal and IgE has been normal to very elevated. From

> what I've learned, this disorder can present very differently from one case

> to the next.

>

> Incidently, we have also seen Conner's ability to mount a fever slowly

> disappear over the last couple of years. I'm not sure why this happens, if

> anyone has any insight, I'd love to learn more.

>

> Take care,

>

> Mom to Conner (12, unknown combined immune deficiency, lymphadenopathy,

> asthma,

> Hashimoto's disease and resolved adrenal insufficiency),

> Hayden (12, unknown immune deficiency, IBS and moderate hearing loss/aided),

> Evan (12, unknown immune deficiency, asthma and mild hearing loss/unaided),

> and Kelsey - (10, unknown immune deficiency and asthma)

> Please visit us at www.caringbridge.org/in/connersmith

>

[Guest guest]

Hi Ursula,

Well my son has been on Nystatin for a few weeks so for no the yeast in

control, I am giving him probiotics. Does IVIG help with any bacterial

infections & reduction in allergies?

I do not have pre vaccine Titers, I do have vaccine titers down now but

that's at age 12. Really not sure how to read them. Any advice there?

Hope your surgery on Sunday goes well, my prayers are with you.

Thanlk you so much once again.

NAndini

>From: " Ursula Holleman " <uahollem1@...>

>Reply-

>< >

>Subject: Re: Nadini IVIG cont.

>Date: Fri, 22 Sep 2006 13:18:28 -0400

>

>Yeast is a t-cell problem. While some patients have seen improvement

>with their t-cell illnesses and numbers while on IVIG, it's not the norm.

>His numbers are encouraging. His IgE of course shows he's allergic to

>probably alot of things. Has he ever had skin testing or any type of

>allergy blood work done? The gut is coated with IgA and since there are

>only trace amounts in IVIG it's not the best treatment. Some patients (pid

>and non-pid) have attempted oral IgG medication. I think some BMT-SCID

>patients have used it for GVHD of the gut. Mixed results. I may have

>asked before but have they tested any function of his IgG. Vaccination

>titers are good for this. Pre and post levels help determine if the immune

>system can mount a response. That way even if the IgG level is normal you

>know whether it has any " kick " .

>

>Ursula - mom to (13) and Macey (11, CVID)

>http://www.primaryimmune.org

>http://www.jmfworld.org

>http://members.cox.net/maceyh

>

>

>

[Guest guest]

OK, Now I'm totally freaked out, please tell me what my next step should be?

Is there anyone of you who lives in the bay area CA or can I fax or email my

son's blood work to anyone of you? Please have a look & then tell me what I

should do?

Getting very worried here.........

Nadini

>From: lmschatz@...

>Reply-

> " " < >

>Subject: Re: Nadini IVIG cont.

>Date: Fri, 22 Sep 2006 13:12:36 -0500

>

>I think Dr. Gelfand at National Jewish is also doing some research on this.

>When I spoke to him about jacob's case, he asked if anyone had mentioned

>NEMO??? I said no, but then when I looked it up, I realized that that was

>where Dr. K was headed in terms of wanting to know about his teeth....

>

>'s IGA is low normal, low IGG, IGM is low, IGE is normal; sub class 3

>deficiency...NK cell defect and mod neutropenia and selective antibody

>deficiency. I believe he has some complement deficiency as well that no

>one

>has addressed. We are going to the heme in November and I will bring any

>other questions there.

>

>I can also ask at the IDF family meeting in OCT regarding some of this.

>

>LINDA S

>, hypogam, selective antibody deficiency with NK cell defect on SCIG

>and

>doing well........

>

>Quoting kristinsmith <kristinsmith@...>:

>

> > I just thought I'd jump in and tell you that my son Conner (also 12 and

>on

> > the " spectrum " ) was diagnosed last week with NEMO a form of X-linked

> > Hyper-IgM (XHIM). In our case, the third time was a charm. We had seen

>two

> >

> > other immunologists who missed the diagnosis, it was our third doc, Jack

> > Bleesing at Cincinnati Children's Hospital, who diagnosed the disorder.

> >

> > I've done a lot of reading of XHIM in the last couple of months and did

> > learn that there have been a few documented cases of normal IgA with

>this

> > disorder (it's rare). In our case, Conner's IgA and IgG are very low,

>his

> > IgM has run low to normal and IgE has been normal to very elevated.

>From

> > what I've learned, this disorder can present very differently from one

>case

> > to the next.

> >

> > Incidently, we have also seen Conner's ability to mount a fever slowly

> > disappear over the last couple of years. I'm not sure why this happens,

>if

> > anyone has any insight, I'd love to learn more.

> >

> > Take care,

> >

> > Mom to Conner (12, unknown combined immune deficiency, lymphadenopathy,

> > asthma,

> > Hashimoto's disease and resolved adrenal insufficiency),

> > Hayden (12, unknown immune deficiency, IBS and moderate hearing

>loss/aided),

> > Evan (12, unknown immune deficiency, asthma and mild hearing

>loss/unaided),

> > and Kelsey - (10, unknown immune deficiency and asthma)

> > Please visit us at www.caringbridge.org/in/connersmith

> >

>

>

>

>

>This forum is open to parents and caregivers of children diagnosed with a

>Primary Immune Deficiency. Opinions or medical advice stated here are the

>sole responsibility of the poster and should not be taken as professional

>advice.

>

>To unsubscribe -unsubscribegroups (DOT)

>To search group archives go to:

>/messages

>

[Guest guest]

" OK, Now I'm totally freaked out... "

Nadini,

Don't fret. Everyone was responding to the information that you posted the

other day, when you said your son had high IgM. = )

Dr. Gupta should have answers for you as questions arise. Be sure to educate

yourself (go to the Jeffery Modell Foundation and Immune Deficiency Foundation

websites to read the info about PIDDs).

You are welcome to post your sons labs here to hear reactions. Obviously, we

can't provide medical advice but we might be able to help you to ask the right

questions to your immunologist. = )

Also, when referred to Dr. Gelfand, she may have thought that you were

already seeing him. Often, we use the first initial of the doc " Dr. G " and it

can be confusing when more than one doc has same initial. Sorry about that..

Let us know how we can support you.

[Guest guest]

I find it very frustrating- I went to Dr. Gupta for my child, and really

liked him, and am 100% glad I took him there; my problem is Dr. Gupta does

not have time to answer questions as they arise. I have a very sick child,

and have questions, and I have been informed that he is busy, and does not

have time to answer ANY questions, and if I want to ask questions I must

make an appointment to see him again. I live in Arizona, and it was not

easy to drive my son to Irvine in the first place (he has autism and

multiple illnesses-SCAD a metabolic disorder, common variable immune

deficiency, seizures, colitis gastritis ect.) I absolutely " get " that he is

busy, but I did take the time to travel to him for my appointment, and so it

is just frustrating to not be able to contact them now when questions OR

concerns do come up.

_____

From: [mailto: ] On Behalf Of

Schulman

Sent: Saturday, September 23, 2006 9:16 PM

Subject: Re: Nadini IVIG cont.

" OK, Now I'm totally freaked out... "

Nadini,

Don't fret. Everyone was responding to the information that you posted the

other day, when you said your son had high IgM. = )

Dr. Gupta should have answers for you as questions arise. Be sure to educate

yourself (go to the Jeffery Modell Foundation and Immune Deficiency

Foundation websites to read the info about PIDDs).

You are welcome to post your sons labs here to hear reactions. Obviously, we

can't provide medical advice but we might be able to help you to ask the

right questions to your immunologist. = )

Also, when referred to Dr. Gelfand, she may have thought that you were

already seeing him. Often, we use the first initial of the doc " Dr. G " and

it can be confusing when more than one doc has same initial. Sorry about

that..

Let us know how we can support you.

[Guest guest]

" my problem is Dr. Gupta does not have time to answer questions as they arise "

Ewww...that's not so great.

Cari,

Dr. Gelfand will be speaking at the University of Arizona on November 27-28th

with the IDF's LeBien Visiting Professor Program. Dr. Gelfand is a strong

immunologist researcher and physician. I am not sure if this is public or not

but you might be interested in calling Tamara at the IDF for details:

410-321-6647

mom to CVIDer

[Guest guest]

Is this just me, or have other's ran into this problem with Dr. Gupta? (I

wrote the original email)

_____

From: [mailto: ] On Behalf Of

Schulman

Sent: Saturday, September 23, 2006 9:51 PM

Subject: Re: Nadini IVIG cont.

" my problem is Dr. Gupta does not have time to answer questions as they

arise "

Ewww...that's not so great.

Cari,

Dr. Gelfand will be speaking at the University of Arizona on November

27-28th with the IDF's LeBien Visiting Professor Program. Dr. Gelfand is a

strong immunologist researcher and physician. I am not sure if this is

public or not but you might be interested in calling Tamara at the IDF for

details: 410-321-6647

mom to CVIDer

[Guest guest]

You should ask the doctor if you can email questions with the assurance that

you will get timely answers. BUT if you have urgent questions, you need to

be able to reach him asap.

Good luck

Pam

(TIM -CVID age 16)

[Guest guest]

No he emailed me back and said he did not have time to answer questions

because he is a professor that is busy teaching, and is only in the clinic ½

a day a week. He told me to make an appointment if I had questions.

_____

From: [mailto: ] On Behalf Of

mole1440@...

Sent: Sunday, September 24, 2006 7:19 AM

Subject: Re: Nadini IVIG cont.

You should ask the doctor if you can email questions with the assurance that

you will get timely answers. BUT if you have urgent questions, you need to

be able to reach him asap.

Good luck

Pam

(TIM -CVID age 16)

[Guest guest]

In a message dated 9/24/2006 10:01:28 AM Central Standard Time,

itsallaboutkreed@... writes:

> No he emailed me back and said he did not have time to answer questions

> because he is a professor that is busy teaching, and is only in the clinic ½

> a day a week. He told me to make an appointment if I had questions.

>

>

>

I just wanted to let you know we had a doc like this. She was the head of

Infectious Diseases and Immunology at a local children's hospital and I never

did

like her, she was too blunt and too practical and cold as ice. I found out

all I could from her ONLY during the three or so appointments we had with her, I

wrote out all the questions and she answered them all during each

appointment, but nothing in between. My ped was clueless about PIDs so I had a

lot of

gaps in knowledge I needed filled in, and I have to say much of that happened

here. Remember nobody here is a doctor, but boy do these ladies have a wealth of

experience. I did a lot of research on the internet, read books on immunology,

asked questions here. It takes some time to get a diagnosis, lots of tests

and lots of waiting. Oh, I hated that. And in the end they didn't do anything

for my DD anyway, but that's our story, and it was because our DD had some

transient defects and we were able to manage her infections " well enough " that

the

doc wouldn't consider any treatment.

That's another story, my point was we had a doc who could only answer

questions during appointments. I feel your pain!! The problem is you have

questions,

then you have some tests, then you get results, then you have more

questions.... the info seems ever-changing so you always have more questions. I

can't

help much, I'm just saying many of us understand and it does STINK!!!

(mom to , age 7, dairy intolerant-related GERD (resolving?) --

currently has polysaccharide antibody def, previously had transient IgG, IgA,

t-cell & other defs... and also to Kate, age 4!, more dairy intolerant but very

healthy!)