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Terri RE HIES (Job's)

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Terri,

I am so not the expert on labs... all I know is that 's IgE levels were

never outrageously high - that is why they dismissed HIES as a diagnosis

initially. We only received a confirmed diagnosis of HIES 2 years ago after his

STAT 3 mutation (DNA testing) came back confirming HIES (Job's Syndrome) He

always had the symptoms but IgE levels of about 1500 or so... and they always

told me that his IgE had to be about 10000 to confirm.

What confirmed it for me was the other issues of the disease - chronic mouth

sores, osteopenia and frequent fractures, no immune response to Prevnar or

Pneumovax, not maintaining immunity for tetanus, frequent pneumonias, lung

abcess, skin abcesses, candida infections, many herpes infections, skin

infections, ear infections, etc. I just needed the STAT 3 to confirm what I

thought was the best diagnosis of all know PIDs.

He has never had asthma but is allergic to CT contrast, plastic tape (for IVs),

and cephalosporins. He doesn't have any food allergies or environmental

allergies that we are aware of.

It sounds like your daughter is more complicated than Hyper IgE - has she had

any fractures ? ( has had 11)

I don't know if this helps...

Kim, mom to (8) with Hyper IgE (Job Syndrome)

From: tcerda@...

Date: Fri, 3 Sep 2010 12:31:32 +0000

Subject: Re: Intro

Good Morning Kim,

I am mom to a 10 yo dx'd w/ combined immune deficiency when she was 4. We

recently moved and re-establishing care has been nothing short of a nightmare

that is ongoing. One thing that continues to show up on her labs is an IgE that

is 4-5 times the high end of normal. In terms of allergies? She was tested with

68 allergens and came back postive to all but 2. She also has

hyperdermagraph-what-ya- ma-call-it, where we can write all over her with the

light touch of our nails. This is MUCH worse when she is not well (which is most

of the time). She is currently off all meds, including her Immunoglobin and is

taking nothing. I understand that severe allergies can be a secondary autoimmune

complication of PIDD. We have been told they cannot really treat her allergies

because there are too many. My understanding of Job's is that it is extremely

elevated IgE...into the tens thousands levels. Molly's levels are 980 on the

last test. There is no question that her Allergies/IgE issues are contributing

to her constant respiratory issues, including pan sinusitis and recurrent

pneumonias. She is also now colonized with pseudomonas. I wanted to ask your

take on the IgE issues.

Terri

Mom, Molly (10) and Maggie (8) CID

>

>

> Actually , sounds like you are on the right track. My son , who

is now 8, was also sick since birth with multiple pneumonias, lung abcesses,

RSV, etc. For the first 2 years of his life we were in the hospital with

infections for over 3 months in total... it is hard. But some of the testing

they need to do to determine if your one year old has an immune deficiency have

to wait until he is 2 so that your maternal antibodies in his system have

completely worn off.

>

>

>

> When he is 2 they will probably test him with pneumovax or prevnar to

determine his body's reaction and how it maintains immunity. This is a big tell

in the immune deficiency scale. And only through a history of immunizations and

titer checking will they see the pattern of how his B and T cells are

respoinding. Initially they thought only had a B cell issue, and 2 years

later found that it was a combined B and T cell problem.

>

>

>

> And even though we have a diagnosis now, and have had for about 2 years, the

way is treated hasn't really changed. We still have to deal with each

infection as it comes and try to keep him healthy. is now on IVIG every

4 weeks and has been for 2 years now. But each kid is different, even when they

have the same diagnosis.

>

>

>

> What are you in for ? - years of worrying, illnesses that come on very quickly

and ruin family plans, friends and family that think you are neurotic and

worrying too much, and growing an indepth knowledge of viruses and antibiotics.

But it gets better. This is a great group and a great resource. Hang in there

and trust your instincts with your son.

>

>

>

> Kim, mom to , 8, with Hyper IgE (Job Syndrome)

>

>

>

>

> From: amandaconstantin@...

> Date: Wed, 1 Sep 2010 20:20:39 -0500

> Subject: Intro

>

>

>

>

>

>

> Hi everyone! My name is and I have three kids. I have a six year old

son, a four year old daughter and a one year old son. My one year old has been

sick since he was three months old. He had RSV and the swine flu at 3 months.

Right after he recovered from that he got pneumonia. We are lucky if he goes

more than a week without being sick with something. He is on Prevacid,

Singulair, Flovent, Nasonex daily and Albuterol as needed. We have been seeing

the immunologist since he was 6 months old. His IgG levels are low but are

considered in the normal range. They have been checking his levels every three

months and they are slowly going up. My son got his one year immunizations and

one month later had his vaccine titers checked and they have all gone down from

5 months ago. They checked his T and B cell levels this time and his T cells CD3

and helper CD4 are low. I don't go back to the immunologist to go over the

results for 3 more weeks. I was wondering if anyone had any ideas of what any of

this means? Also has anyone else gone through this when there child was one? I

am just really looking for any sort of input on what I am in store for.

> Thanks,

>

>

>

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Hi Kim, after reading about hyper IgE, molly has ALL of the landmark problems

except skin infections. Her issues are nearly identical to your sons. She has

not had fractures, but has issues with her teeth and extren joint hypermobility

that is causing extreme issues for her. She also does not build antibodies and

get pneumonia every time she is exposed to a cold. She gets frequent herpes that

is often systemmic. She also has bronchiectesis for the numerous pneumonias and

is colonized as a result. It is all such a mixed bag of worms!

Terri

Sent from my Verizon Wireless BlackBerry

Intro

>

>

>

>

>

>

> Hi everyone! My name is and I have three kids. I have a six year old

son, a four year old daughter and a one year old son. My one year old has been

sick since he was three months old. He had RSV and the swine flu at 3 months.

Right after he recovered from that he got pneumonia. We are lucky if he goes

more than a week without being sick with something. He is on Prevacid,

Singulair, Flovent, Nasonex daily and Albuterol as needed. We have been seeing

the immunologist since he was 6 months old. His IgG levels are low but are

considered in the normal range. They have been checking his levels every three

months and they are slowly going up. My son got his one year immunizations and

one month later had his vaccine titers checked and they have all gone down from

5 months ago. They checked his T and B cell levels this time and his T cells CD3

and helper CD4 are low. I don't go back to the immunologist to go over the

results for 3 more weeks. I was wondering if anyone had any ideas of what any of

this means? Also has anyone else gone through this when there child was one? I

am just really looking for any sort of input on what I am in store for.

> Thanks,

>

>

>

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Share on other sites

Hi Terri.  I know you were specifically asking Kim some questions about HIES,

but I thought I would chime in since my daughter also has it and it is a

confirmed case of it.  She started showing symptoms at 2 months old with a huge

skin infection that covered her face and the entire back of her head.  She then

ended up with a lung infection, multiple outer and inner ear infections, and a

lymph node infection.  She only has allergies to dogs, eggs, and milk, and had

some asthma troubles when she was a baby.  When they tested her for a possible

immune disorder, they immediately thought of HIES after seeing the results. 

Everything pointed to HIES.  The only reason our immunologist wanted the

confirmed diagnosis was to know that this was definitely what she needed to be

treated for.  Her diagnosis was confirmed at the age of one.  Her Ige levels are

typically in the 1400-1500 range as well, however, when she was first diagnosed,

they ran

from 900-1100.  I do know that there are other reasons that a person can have

elevated Ige levels.  Now, I will also say that even though, our daughter has

HIES, her situation is very different from Kim's son so far, however, she is

only 3.  But the symptoms can vary from child to child just a little bit.  I

would agree with Kim, that there might be something else going on with your

daughter though.  I think the skin infections are pretty much the same across

the board, from what I have read.  Anyway, I know you didn't ask me

specifically, but I thought maybe any information could help you.

Thank you and have a great day.

Roach (mother of Hannah, 3, HIES)     

" When I don't measure up to much in this life, I'm a treasure in the arms of

Christ " --Sanctus Real

From: Kim MacEachern <kimmaceachern@...>

Subject: Terri RE HIES (Job's)

" Group " < >

Date: Friday, September 3, 2010, 4:50 PM

Terri,

I am so not the expert on labs...  all I know is that 's IgE levels were

never outrageously high - that is why they dismissed HIES as a diagnosis

initially.  We only received a confirmed diagnosis of HIES 2 years ago after his

STAT 3 mutation (DNA testing) came back confirming HIES (Job's Syndrome)  He

always had the symptoms but IgE levels of about 1500 or so... and they always

told me that his IgE had to be about 10000 to confirm.

What confirmed it for me was the other issues of the disease - chronic mouth

sores, osteopenia and frequent fractures, no immune response to Prevnar or

Pneumovax, not maintaining immunity for tetanus, frequent pneumonias, lung

abcess, skin abcesses, candida infections, many herpes infections, skin

infections, ear infections, etc.  I just needed the STAT 3 to confirm what I

thought was the best diagnosis of all know PIDs.

He has never had asthma but is allergic to CT contrast, plastic tape (for IVs),

and cephalosporins.  He doesn't have any food allergies or environmental

allergies that we are aware of.

It sounds like your daughter is more complicated than Hyper IgE - has she had

any fractures ? ( has had 11)

I don't know if this helps... 

Kim, mom to (8) with Hyper IgE (Job Syndrome)

From: tcerda@...

Date: Fri, 3 Sep 2010 12:31:32 +0000

Subject: Re: Intro

 

Good Morning Kim,

I am mom to a 10 yo dx'd w/ combined immune deficiency when she was 4. We

recently moved and re-establishing care has been nothing short of a nightmare

that is ongoing. One thing that continues to show up on her labs is an IgE that

is 4-5 times the high end of normal. In terms of allergies? She was tested with

68 allergens and came back postive to all but 2. She also has

hyperdermagraph-what-ya- ma-call-it, where we can write all over her with the

light touch of our nails. This is MUCH worse when she is not well (which is most

of the time). She is currently off all meds, including her Immunoglobin and is

taking nothing. I understand that severe allergies can be a secondary autoimmune

complication of PIDD. We have been told they cannot really treat her allergies

because there are too many. My understanding of Job's is that it is extremely

elevated IgE...into the tens thousands levels. Molly's levels are 980 on the

last test. There is no question that her

Allergies/IgE issues are contributing to her constant respiratory issues,

including pan sinusitis and recurrent pneumonias. She is also now colonized with

pseudomonas. I wanted to ask your take on the IgE issues.

Terri

Mom, Molly (10) and Maggie (8) CID

>

>

> Actually , sounds like you are on the right track. My son , who

is now 8, was also sick since birth with multiple pneumonias, lung abcesses,

RSV, etc. For the first 2 years of his life we were in the hospital with

infections for over 3 months in total... it is hard. But some of the testing

they need to do to determine if your one year old has an immune deficiency have

to wait until he is 2 so that your maternal antibodies in his system have

completely worn off.

>

>

>

> When he is 2 they will probably test him with pneumovax or prevnar to

determine his body's reaction and how it maintains immunity. This is a big tell

in the immune deficiency scale. And only through a history of immunizations and

titer checking will they see the pattern of how his B and T cells are

respoinding. Initially they thought only had a B cell issue, and 2 years

later found that it was a combined B and T cell problem.

>

>

>

> And even though we have a diagnosis now, and have had for about 2 years, the

way is treated hasn't really changed. We still have to deal with each

infection as it comes and try to keep him healthy. is now on IVIG every

4 weeks and has been for 2 years now. But each kid is different, even when they

have the same diagnosis.

>

>

>

> What are you in for ? - years of worrying, illnesses that come on very quickly

and ruin family plans, friends and family that think you are neurotic and

worrying too much, and growing an indepth knowledge of viruses and antibiotics.

But it gets better. This is a great group and a great resource. Hang in there

and trust your instincts with your son.

>

>

>

> Kim, mom to , 8, with Hyper IgE (Job Syndrome)

>

>

>

>

> From: amandaconstantin@...

> Date: Wed, 1 Sep 2010 20:20:39 -0500

> Subject: Intro

>

>

>

>

>

>

> Hi everyone! My name is and I have three kids. I have a six year old

son, a four year old daughter and a one year old son. My one year old has been

sick since he was three months old. He had RSV and the swine flu at 3 months.

Right after he recovered from that he got pneumonia. We are lucky if he goes

more than a week without being sick with something. He is on Prevacid,

Singulair, Flovent, Nasonex daily and Albuterol as needed. We have been seeing

the immunologist since he was 6 months old. His IgG levels are low but are

considered in the normal range. They have been checking his levels every three

months and they are slowly going up. My son got his one year immunizations and

one month later had his vaccine titers checked and they have all gone down from

5 months ago. They checked his T and B cell levels this time and his T cells CD3

and helper CD4 are low. I don't go back to the immunologist to go over the

results for 3 more weeks. I was

wondering if anyone had any ideas of what any of this means? Also has anyone

else gone through this when there child was one? I am just really looking for

any sort of input on what I am in store for.

> Thanks,

>

>

>

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Share on other sites

Thanks ! I also read that IgE levels decrease and can even be normal as

children with hyper IgE get older. It does not mean they are over it. Thanks for

all the info!

Terri

Sent from my Verizon Wireless BlackBerry

Intro

>

>

>

>

>

>

> Hi everyone! My name is and I have three kids. I have a six year old

son, a four year old daughter and a one year old son. My one year old has been

sick since he was three months old. He had RSV and the swine flu at 3 months.

Right after he recovered from that he got pneumonia. We are lucky if he goes

more than a week without being sick with something. He is on Prevacid,

Singulair, Flovent, Nasonex daily and Albuterol as needed. We have been seeing

the immunologist since he was 6 months old. His IgG levels are low but are

considered in the normal range. They have been checking his levels every three

months and they are slowly going up. My son got his one year immunizations and

one month later had his vaccine titers checked and they have all gone down from

5 months ago. They checked his T and B cell levels this time and his T cells CD3

and helper CD4 are low. I don't go back to the immunologist to go over the

results for 3 more weeks. I was

wondering if anyone had any ideas of what any of this means? Also has anyone

else gone through this when there child was one? I am just really looking for

any sort of input on what I am in store for.

> Thanks,

>

>

>

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