RE: New - Son with selective iga deficiency

July 7, 2004

Pam:

I think I have the story you're hoping for, at least how it turned out for

us. My dd got a tentative diagnosis of Selective IgA Def from our ped

(turned out to be an incomplete diagnosis) after having survived bacterial

meningitis at 5-1/2 months of age. We were referred to an Infectious Diseases

and

Immunology specialist at one year of age and that's when she was diagnosed

officially.

At that time, she had Absent IgA (zero, and when they did it on the low-titer

plate the " ring " was absent. I mean NO IgA). I'll skip ahead and tell you

that now at age 5 her IgA is normal!!!!!!! Anyway, read on.

So she had zero IgA, low IgG, low IgM, non-protective levels of antibodies to

three of her vaccines (polio, HiB and Prevnar), absent isohemagglutinins (a

marker for immune disorder, it's the basic function of reacting to foreign

blood types) and some partial T-cell deficits. Needless to say, we were

devastated.

Well, they re-vaccinated her for polio, HiB and Prevnar and retested, it took

two times for her HiB to come up but it has stayed and polio levels were fine

after an extra vaccine, but she still doesn't make appropriate antibodies to

Prevnar (after about eight vaccines!) and those she makes she drops in less

than a year.

At age two her IgA was still absent, her IgG was on the way up, the t-cells

were fine and isohemagglutinins were finally present. Better late than never!

At age three her IgG was low-normal, IgA PRESENT and NORMAL. HUH???!!!!! I

looked heavenward and thanked God profusely.

Now at age five she's in a holding pattern, all levels are normal except the

antibody response to Prevnar, which means she's susceptible to

sugar-encapsulated bacteria. That stinks because that's the kind of meningitis

she had

(pneumococcal). But how thrilled am I that everything else came up!!!!!!?????

Now, since she does have the antibody problem, there's always the thought

that this " normal " IgG level isn't doing her much good, after all, the numbers

being there don't help if the ones present are impotent, ya know? But we're

taking it a day at a time and waiting to see how she does. She certainly gets

sick

more than other kids, gets sicker with everything and has more bacterial

infections. But so far she's responding pretty well to antibiotics and we're

managing -- she hasn't been deathly ill by any stretch. My husband and I have

decided if she gets a third pneumonia this year we will go back to the

immunologist. See, they actually cut us loose when 's IgG came up! Can you

imagine?!!! Sigh.

But our ped is great and trusts my judgment.

The first thing I would tell you is YES, your IgA can improve. But there's no

guarantee of course! When had no IgA, we were told to consider a Medic

Alert bracelet in case she needed blood products after a car accident or

something. We didn't... through laziness and denial we put it off long enough

and

then her IgA appeared. That's not to say it's the right thing to do!

The next thing I wanted to ask you is did they do a full workup? Since IgA is

the most well-known disorder, it's all some docs know. For example, our ped

is the one who decided to test 's IgA, IgG and IgM after the meningitis.

All were low but since he didn't know anything about low IgG or IgM, he sort of

missed it!!!! In hindsight that's hard to believe but that's what happened.

So here I am looking up all this stuff about Selective IgA Deficiency, not

knowing her IgG was also deficient!! And we certainly didn't know about the

antibody deficiencies. I'll repeat, our ped is really good! And even he missed

it

due to ignorance of PIDs.

The final thing is that we did have another child and she is very healthy!

That's no guarantee of course, there are several families on the list with more

than one PID child. I was worried about that of course. I tested positive for

Group B Strep toward the end of my pregnancy (common but can put the baby at

risk during birth). You should have heard me reminding EVERY single health care

professional to come in my labor and delivery room about needing the

antibiotics for that!!!! It was sort of funny, they'd take two steps in the room

and

I'd mention the group B, 's PID and the chance the new baby could have a

PID, and again about the antibiotics. I had it down to a short and sweet

speech. Even after they infused the abx, I was like, " are you sure that will

cover it?!!! " !!!

And there was a time in dd #2 (Kate)'s infancy when she kept getting sick and

the ped basically said " one more illness and we'll have to test her immune

system " . But she did great. Now when a bug is making the rounds in my family,

Kate will get a little sniffle and will be knocked for a loop. It's odd to

see a baby be less sick than a big, athletic-looking five year old.

I was worried about another PID, but I was also wanting the normalcy that

comes with having other children. With 's PID and all the associated

medical

drama, I feared that I'd really spoil her if she was an only child. Having

another child around really evens things up, you know?! I think it's good for

her not to be the total focus of everything, it will help her feel more normal

in the long run. And even with Kate being healthy, they still catch all the

same illnesses. was sort of a good barometer during Kate's infancy because

if Kate got sicker than , I knew Kate was really in trouble and needed to

go in!!! There were actually a few times that happened. But now she's really

healthy.

I feel so blessed with all 's improvements and also with finding this

group. What an amazing lifeline -- people who actually know exactly what you're

going through!!!! It's wonderful to have that because your friends and family

with healthy children usually just simply don't understand. On one side you'll

get people trying to make you feel guilty for complaining when there are

people in the world with bigger problems, and on the other hand you'll have

people

who are horrified at what you go through and really pity you. You don't want

either of those, you know? And I've learned sooooooo much here. I could go on

and on but right now I'm ignoring MY children to write this!!

Good luck, and feel free to ask lots of questions!

(mom to Kate, born 9/19/02, very dairy intolerant; and , age 5,

dairy-related GERD, mildly dairy intolerant -- currently has polysaccharide

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

July 7, 2004

from Dale, Mom to Katy, CVID, age 20

Pam, I'll try to answer some of your questions. First of all

everything is potentially transient until the immune system settles down

at age 3, again at age 5, and again at puberty. So don't ever give

up!!!!

The first thing you need to know is that PID is rare and a lot of

doctors don't have current research information. None of this is

covered in medical school -- although my son just completed an

undergraduate course in Immunology at Wheaton College that actually

contained some good stuff!!! So... it's getting better.

First of all -- there's a vast difference between no IgA and low IgA.

Only with NO IgA is there a possibility that the body will build

antibodies against IgA so that if you give him blood or blood products

(IVIG) he could have a reaction of his IgA antibodies trying to destroy

the new IgA found in the new blood or IVIG. That's not a pleasant

reaction! There are 2 courses of action that I know of: One is to

check his blood for anti-IgA antibodies (but if I remember right,

there's only a couple of labs in the US that do this test) so it's hard

to come by. The second, regarding IVIG, is to infuse using Gammaguard

from Baxter (it has the lowest IgA content that I know of) and infuse

VERY SLOWLY to make sure that there is no reaction and to stop infusion

at the first sign of problems! Most doctors chose the latter because of

the problem of getting the IgA antibody test run.

I need someone with IgA experience to explain what would be necessary if

he needed blood. I can't remember the procedure!

HELP! But the answer is YES. You need to tell the doctor that he is

IgA deficient if they ever need to give him blood.

Now, the second thing I would like to know is whether or not your

doctors checked the IgG and IgM levels and also the IgG subclass levels.

That would tell the doctors if there's something going on besides IgA

deficiency and at 3 his levels should be somewhat more stable than at a

younger age. They also need to check and see if he his body made

protective levels of antibodies to his vaccinations.

Inheritance -- most of this stuff just occurs randomly in the

population. The current theory is that it is caused by a transcription

error in the genes. But IgA deficiency is one that has a known history

of heridity. You might want to have yourself and your husband checked

and look around your family tree to see if there's anyone in your family

that was " always sick " or had unusual frequencies of sinus infecitons,

pneumonias, colds, asthma, etc. I recommend that you call the IDF

national office at 1-800-296-4433 and ask for , the genetic

counselor. She will talk to you about anything you want to know

genetically. She's a certified genetic counselor and it's a free service

provided by the Immune Deficiency Foundation.

The Immune Deficiency Foundation also keeps a file on Immunologists who

keep up with the latest research through their mail-outs. All you have

to do is call them and ask about the closest Immunologist. It may be

Duke in North Carolina or Memphis, Tenn though. I know those two places

have Immunologist, but I don't know the in-between area. I think it is

vitally important, especially in light of the thrombocytopenia. There

are some nasty syndromes that could tie this all together and I would

like for him to be checked out by an Immunologist.

Anyone who calls IDF should register on their data base and ask for

their free literature and get on their mailing list. None of it costs

a penny, and the literature is the best around. That number is

1-800-296-4433.

In His service,

Dale

July 7, 2004

Hi Pam,

I saw your post and wanted to welcome you and all the other new people. I have

been quiet for a while, things are nutty here. OK, your son has IgA def, a small

amount anyway, who knows if he would have a reaction or not since it is so tiny.

With IgA def, and blood, the blood needs to be either 1. from another IgA def

person, the blood bank in the hospital would have that info if the Dr. asked, or

2. washed 5 more times than it is usually done, I believe it is washed twice.

Unfortunately in an emergency they may not have time to rewash the blood, but

should be able to see if there was any of the IgA def blood in the bank, if it

is not an emergency than it gets washed again. The Dr. needs to know, any Dr,

really if he gets hurt or anything so they can watch and prepare for

anaphylaxis, although they are very careful with blood transfusions anyway,

vital signs every 5 minutes for 15 minutes than every 1/2 hour for an hour than

every hour until the blood is done. Any sign of a reaction and they stop the

blood, treat the reaction. A reaction can be as mild as an increased temperature

or as bad as anaphylaxis. But they are prepared for anything.

As for other children, there are plenty of other parents who have healthy

children and one with a deficiency, or all have the deficiency.. We were told at

s Hopkins that if we were going to have other children we should go to a

genetic counselor and have everything looked at. The chance of having another

child with an immune def, was higher if you already have one. I don't want that

to scare you off, but getting into a genetic counselor is a good idea.

As to your other question, as Dale and someone else has referred to, the immune

system is a tricky thing, our dtr had Selective IgA deficiency, but now has

CVID. That in itself is very rare, I wouldn't worry about that, has

had 3 different immunodeficiencies 1. Hypogammaglobulinanemia 2. that changed to

Selective IgA def around 2 1/2 -3 years and now at 8 almost 9 she has CVID. The

Dr's at s Hopkins were concerned that this might happen because of the way

her immune system developed. Anyway things have a way of working themselves out.

It is very frustrating and scary, but you get through it, I believe it has made

us all stronger as a family and we deal with each thing as it comes.I have many

times wrote to this group when things go wrong, or I feel like I can't take it

anymore, the support is here and we go on. Love your little one, they are truly

special children.

mom to New - Son with selective iga deficiency

Hi all - It was nice to find this group tonight. I have just read a

few of the posts...and my heart goes out to all of you.

My son - Michal - was diagnosed with selective IGA deficiency at

about 15 months old (he will be 3 in a few weeks). He was sick all

of the time from about 4 months - 18 months, but is really doing well

right now. His levels are <3. I have been told that <3 means he

does not make any IGA and also that it means he only makes a little

bit??? I have read that children with selective IGa deficiency can

have a bad reaction to IVIG or to other blood products containing

IGA. Does anyone know anything about this? I am currious if he were

to ever need a transfusion if I need to inform the doctors of

anything special. I ask this because when we went to the local

childrens hospital a week or so ago (just for stitches) I was telling

the doctor (resident) his medical history and when I said he had

selective IGA deficiency, the doc asked me if there was anything they

needed to know about it????

Also, I am curious what the recurrence rate is in other children as

my husband and I are considering another child.

also had a condition called neonatal alloimmune

thrombocytopenia when he as born (they tell me it is not related).

We have been told that there is a 100% chance that the neonatal

alloimmune thrombocytopenia will repeat itself. The treatment for

this condition is IVIG treatments and platelet transfussion before

and after birth. My concern is that if another child gets both the

above condition and selective IGA that he/she could not get the IVIG

treatment.

Also, did anyone have a child who was dianossed with selective IGA

deficincy that subsequently " grew out " of the condition?

How have subsequent pregnancies gone and was there anything special

done or special tests needed a birth?

Also, does anyone know an immunologist in Alabama

Sorry for all the questions,,It is late so I am kind-of rambling.

Pam Moran

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July 9, 2004

Hi, Pam,

I'm Pam from Minnesota! My dd Rebekah was diagnosed at 29 months with no

IgA and low IgG and a complement disorder. She is now infused every 21 days

with gammaguard. To our surprise, she actually does make IgA...but only

when infused. Before infusing regularly, she had no detectable IgA and we

were worried, like you are, that any blood transfusions would be terrifying

events. Our ped, who admits that he isn't an expert in immunology, told us

that IgM is immature antibodies and since Rebekah has more need for IgG,

they all converted to IgG's. Now, that her IgG totals are up because of the

infusions, she converts some of the IgM to IgA. She is still deficient in

IgA and we've never determined whether her IgA is secretory or only

circulating. Now, whether that all if true, I don't know. But what is true

is that her IgA levels became detectable (I even made them send her blood to

three different labs for confirmation....I know...psycho,

obsessive/compulsive mommy! LOL) after we were regularly infusing

antibodies.

Pam in MN

mom to 4

Rebekah, 4, CVID and ??

July 10, 2004