Re: Antibody def -polysaccharides

[Guest guest]

Ursula:

Okay, the ped is on vacation until Monday of course (drats!) so I don't have

the Hib #s yet. But I did some checking and came up with the fact that Hib is

a polysaccharide and the vaccine is a conjugate (I knew this but had

forgotten it). Where your t-cell info comes in is the fact that t-cell help

is needed to form and hold antibodies. Here's info from an article from USC:

" Polysaccharides without a protein component cannot attract T-cell help.

Although it is possible to some extent to generate T-cell independent

antibodies, high affinity antibodies and immunological memory require T-cell

help. Infants below the age of 2 years cannot make T-cell independent

antibodies. Even adults do not make high affinity anti-carbohydrate

antibodies very effectively. It is therefore necessary to couple

polysaccharides to protein carriers. "

Now. With that in mind, it makes sense that if doesn't make good

antibodies to Prevnar she might not make them to Hib or hold them or

whatever. But when I was reading the statistics on Hib disease, I started to

get REALLY MAD. It makes the little hairs on the back of my neck stand on end

to know she doesn't have protective antibodies to Hib or strep pneumo.

Fortunately, the Hib vaccine is pretty effective in kids with normal immune

systems, so I hope there's not much out there to catch.

Anyway, my question for the group is:

For those of you with a child with ONLY a polysaccharide antibody disorder

('s IgG is in low 500s and immuno was happy with that), are any of you

just being left to your own devices or is the child being treated in any way

(abx prophylaxis or IVIG).

Thank you -- I'm just a little ticked right now!

Some of you have kindly mentioned the possibility of a second opinion, but I

have to say I've hesitated because our ped is outstanding and he's the one

who recommended our immuno. Plus the fact that clinically, 's doing

pretty great! She's getting a ton of colds (but who isn't, this winter!) and

some secondary sinus or ear infections, but that's it. She attends one park

district class one day a week and if anybody seems sniffly, they try to keep

away from them. She always catches it anyway but it's only ever been a

URI. Thanks again - -

(mom to Kate, born 9/19/02, dairy intolerant; and , age 4, dairy

intolerant -- currently has polysaccharide antibody def, previously had

transient IgG, IgA, t-cell & other defs)

[Guest guest]

Hi

Don't know if I can be of any help, but I do have a specific antibody

deficiency to the polysaccharides. I also have IgA deficiency - BUT that is

not why they are treating me with IVIG (actually I get it subcutaneously). I

get the gamma b/c of the specific antibody deficiency. It has helped me

tremendously. I'm 36 now. I started IVIG when I was about 33 and I did get it

intravaneously for 2 years. I now take it subcutaneously which is simply more

convenient for me given that I work and am a mom. Prior to the gamma, I had

approximately 10-12 years of continuous sinus infections, sinus surgery (that

was horrid), and I was rarely off antibiotics for more than a week at a time.

Now, I still get sinus infections but only when I catch a cold first and I

can usually clear the infection with one course of antibiotics. So, I'm

probably on antibiotics 6x a year now - which is so much better than it used

to be! So, yes, gamma has been helpful for my polysaccharide antibody

deficiency.

Anne R. (IgA def., polysach. antibody def.) (mother to Sam - IgA def., IgG2

def.)

[Guest guest]

For those of you with a child with ONLY a polysaccharide antibody

disorder

('s IgG is in low 500s and immuno was happy with that), are any of

you

just being left to your own devices or is the child being treated in

any way

(abx prophylaxis or IVIG).

Plus the fact that clinically, 's

has Selective Antibody Deficiency..........polysaccharide antibody. But in

s case he has never clinically done well when not treated with IVIG. HE has

been receiving IVIG for 6 years. He had 6 surgerys in two years before being

diagnosed. When put on IVIG he has not had another surgery...........Well not

until we trailed him off IVIG in 6th grade within 6 months he had to have

another surgery. His Igg was in the normal range (always has been) but he has

no respose to the polysaccharide ........The doc put him back on IVIG. is

now in 7th grade doing fairly well on IVIG. He still battles with fatigue and

has flare ups in his joint but over all he is doing fantastic.

But remember was 7 when we finally were diagnosed and put on IVIG.

Hope that helps

le

[Guest guest]

" left to your own devices " in what way?

My son PJ is almost 5 and has just that " polysaccharide antibody

deficiency/IGA deficient. My daughter Meghan has that as well with some

other things going on with her that we don't know of yet, just had lots

of blood drawn with multiple tests being ran and we won't know until

April 21st. Our immunologist says she is very different than my son

because she can't come off of antibiotics for more that 3 days without

becoming infected.

On Thursday, April 3, 2003, at 10:23 AM, le wrote:

>

> For those of you with a child with ONLY a polysaccharide antibody

> disorder

> ('s IgG is in low 500s and immuno was happy with that), are any of

> you

> just being left to your own devices or is the child being treated in

> any way

> (abx prophylaxis or IVIG).

> Plus the fact that clinically, 's

>

> has Selective Antibody Deficiency..........polysaccharide

> antibody. But in s case he has never clinically done well when

> not treated with IVIG. HE has been receiving IVIG for 6 years. He

> had 6 surgerys in two years before being diagnosed. When put on IVIG

> he has not had another surgery...........Well not until we trailed him

> off IVIG in 6th grade within 6 months he had to have another surgery.

> His Igg was in the normal range (always has been) but he has no

> respose to the polysaccharide ........The doc put him back on IVIG.

> is now in 7th grade doing fairly well on IVIG. He still battles

> with fatigue and has flare ups in his joint but over all he is doing

> fantastic.

>

> But remember was 7 when we finally were diagnosed and put on IVIG.

>

> Hope that helps

>

> le

>

>

>

[Guest guest]

Has anyone out there ever researched on how our children got these

different kinds of deficiencies? I would just like to know if it is a

congenital/genetic thing did it come from the mother or fathers, not

that it matters to me, both of my kids have it and I don't care which

one of us " gave " it to them I am just curious how they have come about

this.

If anyone has any answers or reading material to look at I would love

to see it, I feel like I have looked almost anywhere.

Thanks, Peggy....Mom to PJ 5 IGA deficient and Meghan 16 months IGA

deficient/and other things pending blood test results.

On Thursday, April 3, 2003, at 11:58 AM, areckling@... wrote:

> Hi

>

> Don't know if I can be of any help, but I do have a specific antibody

> deficiency to the polysaccharides. I also have IgA deficiency - BUT

> that is

> not why they are treating me with IVIG (actually I get it

> subcutaneously). I

> get the gamma b/c of the specific antibody deficiency. It has helped me

> tremendously. I'm 36 now. I started IVIG when I was about 33 and I did

> get it

> intravaneously for 2 years. I now take it subcutaneously which is

> simply more

> convenient for me given that I work and am a mom. Prior to the gamma,

> I had

> approximately 10-12 years of continuous sinus infections, sinus

> surgery (that

> was horrid), and I was rarely off antibiotics for more than a week at

> a time.

> Now, I still get sinus infections but only when I catch a cold first

> and I

> can usually clear the infection with one course of antibiotics. So, I'm

> probably on antibiotics 6x a year now - which is so much better than

> it used

> to be! So, yes, gamma has been helpful for my polysaccharide antibody

> deficiency.

>

> Anne R. (IgA def., polysach. antibody def.) (mother to Sam - IgA def.,

> IgG2

> def.)

>

>

>

[Guest guest]

In a message dated 4/3/03 10:26:26 AM Central Standard Time,

bunneegirl@... writes:

> For those of you with a child with ONLY a polysaccharide antibody disorder

> ('s IgG is in low 500s and immuno was happy with that), are any of you

>

> just being left to your own devices or is the child being treated in any

> way

> (abx prophylaxis or IVIG).

>

,

You just described us to a T. Well, not exactly, but more or less the only

place Autumn showed any problems or deficiencies was with the pneumovax and

prevnar vaccines. For the most part, we just treat the illnesses as they

come and do long-term antibiotics during the winter months. The ped was

frustrated by others not willing to back us up, so he's just biding his time

to see if she really will outgrow all of this or until we gather more

ammunition to go back to the specialists.

Ray, mother to Tabitha (age 7), Autumn, age 5 (IgG def., asthma,

chronic sinusitis, and allergies), and Duncan (22 months)

[Guest guest]

In a message dated 4/3/2003 11:40:35 AM Central Standard Time,

psturgis@... writes:

> " left to your own devices " in what way?

>

Peggy:

The immuno basically set us adrift, her exact words were, " you've graduated " .

Since 's IgG is within normal range (low 500s) and her IgA has

remarkably made an appearance (not sure whether she has a mild IgA def or

not, as the secretory result was " less than 6 " and normal range is anything

above 3), she said she didn't need to see again unless she got a deadly

infection (then she'd get IVIG) or if she was sick a lot (her definition is

different than mine since was sick one of every two days in 2001 and

that didn't seem to impress her).

I don't feel I can complain too much because her clinical picture is not that

bad. But she catches seemingly everything she encounters, misses her park

district class about 40% of the time due to illness, and gets secondary

bacterial infections (sinus usually, ears sometimes) about every other cold.

Clinically she isn't terrible, but once in a while she also gets something

that causes a high white count and extreme left shift and she gets Rocephin

for three days and we never figure it out.

Mostly I just worry about the deadly infections. She's only had one and that

was in infancy, but I feel like she's obviously more vulnerable than the

average kid and the thought of getting something big scares me a lot. The

immuno said she wasn't allowed to go to preschool and should not start

kindergarten early due to exposure issues. So which is it, is she okay out

there in the world or is she not?

I DID sign her up for a little park district " preschool " class starting this

fall. It's only two days a week for two hours a day and I've arranged to

provide Purell for all the kids to use when they get there and before meals.

I figure the immuno meant all-day preschool or five days a week, that's why I

wasn't afraid to do this. We can always drop out if she gets sick too much

and it seems like a bad idea. I am trying to balance exposure with

socialization -- she's had some social issues from having not been able to

play with other children until the age of 2-1/2 (the immuno dictated that

because of the severity of her PID until then. Apparently it was serious

enough to isolate her from other children but not serious enough to treat. Do

I sound bitter).

I know I'm rambling, I'm just trying to work it out out loud in front of

people who would understand. I can't bounce this off many people because it

would take too much time to explain the medical side of it in order for them

to understand.

Anyway, thanks for letting me vent. Or complain. Or whatever it is that I

just did!

Okay, I got it. At long last! Here it is in black and white. I always felt

like could have benefitted from IVIG, especially back when she was

younger and her #s were so bad and she had so many deficits. Which is worse,

social isolation or IVIG? I don't know, so I never pushed. So now she's

improved on most fronts and that's absolutely wonderful, I relish every

improvement and am so grateful. And maybe treatment would have delayed those

improvements, I don't know. But strep-pneumo bacteria scares me more than

anything and that's what she's still vulnerable to. And my fear is that she

could get another deadly infection and end up on IVIG anyway and boy would I

be P*SSED beyond belief that we went through all this isolation and worry and

work just to end up in treatment anyway. I don't know whether such a rotten

medical procedure as IVIG would have been worse or better than some of the

illnesses she's suffered through that she *might* have been spared, but who

knows maybe she wouldn't have been spared any of those anyway. And do you

want to treat just " in case " she could someday maybe catch something bad? I

mean, what are the odds, right?

I drive myself nuts wanting that crystal ball.

Well, I guess this is a case where I should " let go and let God " since my

instincts are muddled on this one. I keep going around and around on it.

I'm sorry I dragged on so long on this. She's well right now so maybe I just

needed something to gripe or panic about! Plus I'll drive myself crazy until

I get those Hib #s on Tuesday anyway.

Okay, shut up already, Kel!

(mom to Kate, born 9/19/02, dairy intolerant; and , age 4, dairy

intolerant -- currently has polysaccharide antibody def, previously had

transient IgG, IgA, t-cell & other defs)

[Guest guest]

Hi ~~

I can really understand how you're feeling. Back in 1999, Lacey's pulmonary

doctor did some tests on her, (gave vaccines, drew titers, etc) and told me

that she had an IgG subclass 2 deficiency. He said he was consulting with the

immunologists at Children's about what tests to give and what course of

action to take. They decided at that time to put her on a prophylactic

antibiotic. They mentioned the IVIG then, but said they didn't want to go

that route. Lacey has had SO many ear infections, sinusitis, pneumonia,

bronchitis, etc.. She's my adopted daughter, (we finalized 3 years ago today,

by the way, but she's been with us for 8 years), but I remember reading a

hospital consultation report from when she was 3 years old. It said " This is

the TENTH hospital admission for this child who has a long history of chronic

lung disease and recurrent pneumonia " . In that same report it said that her

IgG was slightly low, she had low IgA, low IgE, and normal IgM.

It wasn't until this last November when she was hospitalized with a horrible

strep pneumonia that they started her on IVIG, (I just happened to mention to

the infectious disease doctor that she had had some suspected immune

deficiency that he may want to check on. He told me, " Oh thank you, I'd not

heard that " )! I shudder to think what may have happened if I hadn't thought

to tell him that, because quite honestly I didn't understand the full

consequences of what an immune deficiency could mean. I had asked her

pulmonary doctor many, many times since he first told me about the subclass

deficiency if there was anything else we should be checking into with that,

but he really just sort of put it aside each time.

I wish so much that they'd been more aggressive with her treatment earlier

on. It could have saved her lungs several pneumonias. I know it's not good to

focus on the " what-ifs " , but sometimes those thoughts just creep in. All I

can say is if you're not sure the right thing is being done, keep pushing

until you feel satisfied. I wish I had~~

Belinda

Lacey's mom, (15, CdLS, immune deficiency)

Pittsburg, CA

<A HREF= " http://www.cdls-support.org/family/aug2001/index.html " >CdLS OSG:

Featured Family of August 2001</A>

[Guest guest]

Hi,

Just thought I let you know that my immunologist put me on IVIG so that my

son and I would not have to become " hermits " (in his words)! Prior to IVIG, I

was ready to isolate myself and my son so that we would be healthy!

Anne R.

[Guest guest]

,

I guess my answer would be " no " being left to our own devices. My

children's immuno is fabulous and PJ and Meghan just love him. PJ, when

he was born threw up constantly and several formula changes later and

several hospital stays, pneumonia, more ear infections than I can

count, we changed pediatricians. I actually managed this ones office,

he started to see PJ and listened to me so I thought and ran some blood

work and said " oh, he has hardly any IGA's " at the time, I had no clue

what it was and what it ment and when I asked his pediatrician, I was

told it's nothing we can fix anyway Peggy, it just means that he will

get lots of infections. So, onto the ENT we went, (another fabulous

doctor) we had surgery, sinus,turbinates,and ears. We were well for 1

1/2 years. Boom, he starts to get sick again, we had moved across town

since that time and started seeing a new pediatrician (he's fabulous

too) he said have you ever been to an immuno before, we said no. We

went. ANA screenings were done at the rheumatoid doctor and all types

of blood work at the immuno as well. It came back that he had 3 IGA's,

and the others came back that he needed some immunizations to boost his

immune system and if that didn't bring up his scores he would go on

IVIG. We prayed. We won! So he is just straight IGA deficient and gets

sicker more often than most. Okay, here goes Meghan. (Yeah this does

make it nice to know someone will read this, not get board and

understand, it makes me feel great) Okay, during the first few immuno

visits I was pregnant with Meghan, the question was asked, is she at

risk, the answer was yes quite possibly. There wasn't anything we could

do until she was 1 or closer to 1 because of inaccuracy of numbers and

kids not getting their IGA's really till then. She was 8 1/2 months

when I quit breastfeeding her and mind you had already has RSV by 3 1/2

months and on breathing treatments. Anyway, a week after stopping the

nursing she got her first ear infection in the left ear, then the right

then RSV again and a sinus infection all in the same week, they started

the amox, then the zithromax,then the augmentin, then the omnicef then

we went to the hospital, rounds of Rocephin later and 10 days of high

doses of Omnicef, the infection went away but not the fluid, so ever

since she was 8 mths. she has been chronically sick. Oral drugs do not

work for her she either gets 3 days on and 3 days off and then 3 days

on of Rocephin and 10 days of high Omnicef or we go to the hospital for

IV antibiotics. So I had enough of it and we to the immuno anyway at 11

months, he said close enough, the tests were ran and sure enough 0

IGA's. Surgery was scheduled for her by the same ENT that my son PJ

had. She just had her first surgery on Feb 3rd this year, turbinates,

sinus, and tubes. 10 days after surgery and the next day after her

antibiotics were finished, what do you think, this time is was a

bi-lateral ear infection, and pneuomonia. Back in the hospital.

Basically since then it's non stop again, we finally went to the immuno

again on March 31st and he ran some more stuff I couldn't even spell.

He says Meghan is definatly different than PJ there is something more

deeper going on with her than just straight IGA deficient. That scares

the hell out of me wondering what it is and the worst part is that I

have to wait now until April 21st before we go and see him again

because these blood tests take 2 weeks to get back they have to be sent

off to California. Some of them anyway. PJ has had his sinus done 3x's

turbinates 1x now and since he is 3 1/2 he's only had occassional

infections. Meghan, I am still waiting. Oh by the way the Friday before

the dr visit to the immuno she came off of antibiotics and by Monday of

the dr visit, yes, another ear infection. This is crazy. So the person

I am bitter with is the pediatrician he said I couldn't do anything

about it. And didn't tell me that gastro problems were part of

IGA/M/E/G deficiencies. Meghan is also on Zantac because she throws up

alot too.

Talk about going on and on that was me but I sure feel better, thanks!

Peggy, mom to PJ almost 5 and Meghan 17 months tomorrow.

On Friday, April 4, 2003, at 02:59 PM, bunneegirl@... wrote:

> In a message dated 4/3/2003 11:40:35 AM Central Standard Time,

> psturgis@... writes:

>

>

>> " left to your own devices " in what way?

>>

>

> Peggy:

>

> The immuno basically set us adrift, her exact words were, " you've

> graduated " .

> Since 's IgG is within normal range (low 500s) and her IgA has

> remarkably made an appearance (not sure whether she has a mild IgA def

> or

> not, as the secretory result was " less than 6 " and normal range is

> anything

> above 3), she said she didn't need to see again unless she got a

> deadly

> infection (then she'd get IVIG) or if she was sick a lot (her

> definition is

> different than mine since was sick one of every two days in 2001

> and

> that didn't seem to impress her).

>

> I don't feel I can complain too much because her clinical picture is

> not that

> bad. But she catches seemingly everything she encounters, misses her

> park

> district class about 40% of the time due to illness, and gets secondary

> bacterial infections (sinus usually, ears sometimes) about every other

> cold.

> Clinically she isn't terrible, but once in a while she also gets

> something

> that causes a high white count and extreme left shift and she gets

> Rocephin

> for three days and we never figure it out.

>

> Mostly I just worry about the deadly infections. She's only had one

> and that

> was in infancy, but I feel like she's obviously more vulnerable than

> the

> average kid and the thought of getting something big scares me a lot.

> The

> immuno said she wasn't allowed to go to preschool and should not start

> kindergarten early due to exposure issues. So which is it, is she okay

> out

> there in the world or is she not?

>

> I DID sign her up for a little park district " preschool " class

> starting this

> fall. It's only two days a week for two hours a day and I've arranged

> to

> provide Purell for all the kids to use when they get there and before

> meals.

> I figure the immuno meant all-day preschool or five days a week,

> that's why I

> wasn't afraid to do this. We can always drop out if she gets sick too

> much

> and it seems like a bad idea. I am trying to balance exposure with

> socialization -- she's had some social issues from having not been

> able to

> play with other children until the age of 2-1/2 (the immuno dictated

> that

> because of the severity of her PID until then. Apparently it was

> serious

> enough to isolate her from other children but not serious enough to

> treat. Do

> I sound bitter).

>

> I know I'm rambling, I'm just trying to work it out out loud in front

> of

> people who would understand. I can't bounce this off many people

> because it

> would take too much time to explain the medical side of it in order

> for them

> to understand.

>

> Anyway, thanks for letting me vent. Or complain. Or whatever it is

> that I

> just did!

>

> Okay, I got it. At long last! Here it is in black and white. I always

> felt

> like could have benefitted from IVIG, especially back when she

> was

> younger and her #s were so bad and she had so many deficits. Which is

> worse,

> social isolation or IVIG? I don't know, so I never pushed. So now she's

> improved on most fronts and that's absolutely wonderful, I relish every

> improvement and am so grateful. And maybe treatment would have delayed

> those

> improvements, I don't know. But strep-pneumo bacteria scares me more

> than

> anything and that's what she's still vulnerable to. And my fear is

> that she

> could get another deadly infection and end up on IVIG anyway and boy

> would I

> be P*SSED beyond belief that we went through all this isolation and

> worry and

> work just to end up in treatment anyway. I don't know whether such a

> rotten

> medical procedure as IVIG would have been worse or better than some of

> the

> illnesses she's suffered through that she *might* have been spared,

> but who

> knows maybe she wouldn't have been spared any of those anyway. And do

> you

> want to treat just " in case " she could someday maybe catch something

> bad? I

> mean, what are the odds, right?

>

> I drive myself nuts wanting that crystal ball.

>

> Well, I guess this is a case where I should " let go and let God " since

> my

> instincts are muddled on this one. I keep going around and around on

> it.

>

> I'm sorry I dragged on so long on this. She's well right now so maybe

> I just

> needed something to gripe or panic about! Plus I'll drive myself crazy

> until

> I get those Hib #s on Tuesday anyway.

>

> Okay, shut up already, Kel!

>

> (mom to Kate, born 9/19/02, dairy intolerant; and , age 4,

> dairy

> intolerant -- currently has polysaccharide antibody def, previously had

> transient IgG, IgA, t-cell & other defs)

>

>

>

[Guest guest]

In a message dated 4/4/2003 6:56:43 PM Central Standard Time,

Lacey449@... writes:

> I wish so much that they'd been more aggressive with her treatment earlier

> on. It could have saved her lungs several pneumonias

Belinda:

Do NOT beat yourself up about that -- that's what the docs were getting paid

for!!! We have all been taught to rely on the advice of experts and how are

we to know that sometimes the docs don't know everything or don't care quite

as much as we do? You are doing a great job, you are a great advocate for

your child, just focus on that. You're doing the best you can and you did the

best you could at the beginning too, you simply couldn't possibly know what

you know now!

(mom to Kate, born 9/19/02, dairy intolerant; and , age 4, dairy

intolerant -- currently has polysaccharide antibody def, previously had

transient IgG, IgA, t-cell & other defs)

[Guest guest]

In a message dated 4/15/2003 7:51:52 AM Pacific Standard Time,

bunneegirl@... writes:

> You're doing the best you can and you did the

> best you could at the beginning too, you simply couldn't possibly know what

>

> you know now!

>

~~

I know you're right........it's just the nature of motherhood, I

guess........especially mothering one with so many complicated challenges!!

Thank you for the pep talk!!

Belinda

Lacey's mom, (15, CdLS, IgG deficient, IVIG)

<A HREF= " http://www.cdls-support.org/family/aug2001/index.html " >CdLS OSG:

Featured Family of August 2001</A>