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Hi, Well Im not sure I forward this right as a few have said they didn't

recieve this part of the message. So I'll try again........This is a

response to an e mail my husband sent (its at the end of the letter) this

response is from s Immuologist at Stanford.

Dear Dale and le

It is true that we don't really know whether 's immune system will

improve at some point. However, the IVIG we are giving is to replace what

he lacks and will not impact his ability to ever produce functional

antibodies on his own. We cannot change the interval of dosing since the

infusion lasts only about 3-4 weeks so anything less frequent than that

would leave him without effective antibodies after that time. The only

way to know whether his immune system has changed is to take him off the

IVIG for 3 or 4 months and repeat the studies (as we have done previously).

The new vaccine is a pneumococcal vaccine intended primarily for infants

and toddlers. Since it is a pneumococcal vaccine it is unlikely could

mount an effective immune response to benefit from it.

It is true we are learning more and more about subtypes of immune

deficiencies (such as the letter you included). (By the way, has a

partial response to pneumovax). However, at this point most of the

information is descriptive since not enough patients have yet been

characterized. But we certainly follow these developments with interest!

I will not be available during your next visit but Dr. Tepas will be

there. So I look forward to seeing you the following visit!

Rich Shames

Ddpenne@... writes:

>Dr. Shames:

>

>I am writing to inquire regarding 3 things:

>

>1. It is my understanding that we were hoping that 's body would

>someday

>begin to produce his now-missing antibodies, maybe during adolescence. I

>am

>fearful that if we are artificially supplying these antibodies, that his

>body

>may not get the signal to produce them itself, since its need is being

>met.

>Instead of taking him off when he is at the 3-week interval, suppose we

>try

>4-weeks for a while, then 5 weeks and then stop. You probably have a

>reason

>that this might not work, probably because the antibodies are out of his

>system in 3-4 weeks? However, I think that since he is soon to enter

>puberty, now would be the time to give his body another try at doing its

>job.

>

>2. le has heard of a different vaccine named " Prevnar " . Might this

>be

>helpful. Any thoughts?

>

>3. I have included below, the text of a letter that we received regarding

>a

>possibly related PID case. It would only take 3 minutes to read, and

>I'd

>appreciate it if you would just tell us, " No, this is not relevant " , or

> " Great, we'll check into it " .

>

>

>le is coming down to Stanford with on July 6th and 7th for

>'s

>IV on Friday the 7th. She can arrange her schedule to meet with you on

>Thursday, if it would serve a purpose beyond our correspondence in this

>matter. Please let us know.

>

>Thanks, Dale Penne

>

>Email that we received....

>My name is Autumn Austin and I am a subscriber to the PEDPID list. I

>read

>the mail daily but seldom write in. I hope you do not mind me responding

>to

>your recent post personally. I have a son with a very rare Primary Immune

>Deficiency. You may be interested in looking at my Webpage and having

>you

>son's physician review the Journal Abstract or have him or her review my

>Webpage themselves. The PID named Cd5-Cd19 PID can often times be

>misunderstood or confusing to physicians for the mere fact that some of

>these

>kids can measure within normal levels of IgG. My son measure below

>normal,

>but this is not always the case. What is astonishing is that these boys

>make

>zero (some physicians call an inadequate response to the pneummovax a

>non-response) to all 12 sero types of the pneummovax. It is as if they

>never

>had the shot at all. What makes this PID even more amazing is that these

>boys have 100% expression of a b-cell called Cd5+. Hence, what actually

>is

>being measured with the IgG is the Cd5+ bcells. Cd5+ bcells are

>premature

>Bcells, they do not mature and they are responsible for making

>autoantibodies

>that in turn can attack the body in various ways, including malignancies,

>usually Chronic Lymphatic Leukemia. With the Cd5-Cd19 PID the amount of

>good

>b-cells or antibodies is actually less then 2%( which is very bad), the

>test

>can often times be mistaken for normal or below normal levels of IgG to a

>physician when actually the Cd5+ bcells are what are showing up as normal

>levels of Bcells...does that make sense? In this PID the level of Cd5+

>bcells is so high that it often times stumps physicians when they first

>hear

>of this...most have a hard time comprehending that a person could make

>this

>much Cd5+ bcells. You should not have any Cd5+ bcells after infancy and

>if

>you do it is often associated with leukemia, cancers or autoimmune

>disease...even then, the amount measured is ususally less then 50%, with

>these boys it is 100% which is so incredibly scary. If your son is

>measuring

>normal or near normal levels of IgG and truly has all zero's to the

>pneummovax, I strongly encourage you to have your physician contact my

>sons

>physician (who not only discovered the defect but is also a consulting

>immunologist for the IDF) to discuss how to go about checking your son

>for

>the Cd5-Cd19 PID. The Cd5-Cd19 is also referred to as Severe

>Anti-Polysacchride Antibody Deficiency with Cd5+ B-cell Predominance. It

>would be very important for you to know if you son has this as it can be

>a

>very serious PID that is different from CVID. The Autoimmune and or

>malagnancy component of the PID is very, very serious.

>

>My web site is http://www.scid.net/cd5cd19pid/

>

>Please feel free to contact me personally if you have any questions or if

>I

>can be of further help.

>

>Good luck to you.

>

>Autumn Austin

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le,

My email did not include the reponse from 's doc or the emila that

your husband wrote to the doc. I am very interested since it seems that

& my son, Wade have some similarities & they are almost the same age.

I think that there may be significant findings when these kids become

teenagers & go through puberty...just my opinion. I would really appreciate

it if you could forward those emails to me. Thanks!

Hope is feeling as well as can be & enjoying the summer!

Regards,

Beth

At 10:16 AM 6/27/00 EDT, you wrote:

>Hi Everyone,

>

>Well my husband e mailed the doc, see bottom of page. was wondering if

>he could go longer between ivig or stop for awhile. Here is the response.

>Thank you to Autti who shared about the new developments in cd5. Also to

all

>who shared news about the prevnar.

>

>Thanks to all for sharing

>

>le

>

>

>

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