Immunoglobin Deficiency? Anyone else have this or been tested for this?

[rcq925]

My daughter Hayley, just turned 7 and is a double delta F508. She has been hospitalized 16 times over her short seven years including her diagnosis.

She just always seems to get sick, especially in fall and winter and usually requires inpatient hospitalizations 3-4 times per year with IV antibiotics, however, never really has anything major in her cultures. She has cultured PA twice, once in 2006 and once in 2009. She has had two bronchs in the last 4 years, neither of which came back with any bacteria.

Her PFT's range between the 80's and 100's, she is still getting the hang of the PFT technique and does not blow the air out fast enough.

Anyway she was just recently hospitalized and our pulmo wanted to check her immunoglobulin levels. Her Immunoglobulin G level is very low and he thinks this is the reason she keeps getting sick all the time.

On one hand this is devastating, because here is another condition on top of CF to deal with and on the other hand if we can now keep her from getting sick so much, it could be a good thing. Our doc says it is rare to have both CF and the Immunoglobulin Deficiency Disorder.

We still have more testing to do to in fact verify that she does have the Immunoglobulin Deficiency, which could mean infusions of immunoglobulin every 3 weeks for the rest of her life.

Just curious if anyone else has this or has been tested for it.

Thanks!

 

[rcq925]

My daughter Hayley, just turned 7 and is a double delta F508. She has been hospitalized 16 times over her short seven years including her diagnosis.

She just always seems to get sick, especially in fall and winter and usually requires inpatient hospitalizations 3-4 times per year with IV antibiotics, however, never really has anything major in her cultures. She has cultured PA twice, once in 2006 and once in 2009. She has had two bronchs in the last 4 years, neither of which came back with any bacteria.

Her PFT's range between the 80's and 100's, she is still getting the hang of the PFT technique and does not blow the air out fast enough.

Anyway she was just recently hospitalized and our pulmo wanted to check her immunoglobulin levels. Her Immunoglobulin G level is very low and he thinks this is the reason she keeps getting sick all the time.

On one hand this is devastating, because here is another condition on top of CF to deal with and on the other hand if we can now keep her from getting sick so much, it could be a good thing. Our doc says it is rare to have both CF and the Immunoglobulin Deficiency Disorder.

We still have more testing to do to in fact verify that she does have the Immunoglobulin Deficiency, which could mean infusions of immunoglobulin every 3 weeks for the rest of her life.

Just curious if anyone else has this or has been tested for it.

Thanks!

 

[rcq925]

My daughter Hayley, just turned 7 and is a double delta F508. She has been hospitalized 16 times over her short seven years including her diagnosis.
<br />
<br />She just always seems to get sick, especially in fall and winter and usually requires inpatient hospitalizations 3-4 times per year with IV antibiotics, however, never really has anything major in her cultures. She has cultured PA twice, once in 2006 and once in 2009. She has had two bronchs in the last 4 years, neither of which came back with any bacteria.
<br />
<br />Her PFT's range between the 80's and 100's, she is still getting the hang of the PFT technique and does not blow the air out fast enough.
<br />
<br />Anyway she was just recently hospitalized and our pulmo wanted to check her immunoglobulin levels. Her Immunoglobulin G level is very low and he thinks this is the reason she keeps getting sick all the time.
<br />
<br />On one hand this is devastating, because here is another condition on top of CF to deal with and on the other hand if we can now keep her from getting sick so much, it could be a good thing. Our doc says it is rare to have both CF and the Immunoglobulin Deficiency Disorder.
<br />
<br />We still have more testing to do to in fact verify that she does have the Immunoglobulin Deficiency, which could mean infusions of immunoglobulin every 3 weeks for the rest of her life.
<br />
<br />Just curious if anyone else has this or has been tested for it.
<br />
<br />Thanks!

 

[Ratatosk]

I do know of a child who has a rare cf gene variant as well as an immune disorder. I PM'd you a link to his caringbridge page.

 

[Ratatosk]

I do know of a child who has a rare cf gene variant as well as an immune disorder. I PM'd you a link to his caringbridge page.

 

[Ratatosk]

I do know of a child who has a rare cf gene variant as well as an immune disorder. I PM'd you a link to his caringbridge page.

 

[hmw]

I'm sorry your daughter has had such a rough time of it and you now have that feeling of 'another thing' to deal with. I do understand that (Emily has other issues in addition to cf.) It is good though to be able to address this and hopefully be able to keep her healthier!

Emily had an immunology workup last summer after a bout of intermittent fevers lasting several months.

Her results came back indicating she had mildly low total igG as well as low levels in a couple specific subclasses of IgG. When they checked her vaccine titers, a couple of them were nearly nonexistent (there were only trace levels of Prevnar & Pneumovax and very low HIB as well as something else I can't think of right now, although others were completely normal. The fact that some were very low and others were normal is to be expected in subclass deficiency.)

It was upsetting to me as well- to have so little response to those particular vaccines and having low levels in those classes of IgG is NOT a good combination with CF due to the vulnerability to respiratory infection. Reduced total IgG is not so great either. We were told at this point her total IgG is not low enough to be extremely worrisome, and the vaccines she had not responded to can be redone as needed- so we will continue to monitor and see how things go. However, we were also told that if needed IVIG therapy could be a possibility at some point.

We were told that immune deficiency disorders were more common in the general population than CF, so while it's not common to have an individual present with both an immune disorder and CF, it's not unheard of.

Was just her total IgG checked (as is common in yearly labs) or did she have a full workup including vaccine titers and measuring levels of subclasses IgG1-4? If not I would ask for that.

Here is a good link discussing immune deficiency of various types... it will open up to discusssing subclass issues but you can find discussion on total IgG as well as other stuff if you look around. <img src="i/expressions/face-icon-small-smile.gif" border="0"> <a target=_blank class=ftalternatingbarlinklarge href="http://www.immunedisease.com/patients-and-families/about-pi/types-of-pi/igg-subclass-deficiency.html">ImmuneDisease.com</a>

 

[hmw]

I'm sorry your daughter has had such a rough time of it and you now have that feeling of 'another thing' to deal with. I do understand that (Emily has other issues in addition to cf.) It is good though to be able to address this and hopefully be able to keep her healthier!

Emily had an immunology workup last summer after a bout of intermittent fevers lasting several months.

Her results came back indicating she had mildly low total igG as well as low levels in a couple specific subclasses of IgG. When they checked her vaccine titers, a couple of them were nearly nonexistent (there were only trace levels of Prevnar & Pneumovax and very low HIB as well as something else I can't think of right now, although others were completely normal. The fact that some were very low and others were normal is to be expected in subclass deficiency.)

It was upsetting to me as well- to have so little response to those particular vaccines and having low levels in those classes of IgG is NOT a good combination with CF due to the vulnerability to respiratory infection. Reduced total IgG is not so great either. We were told at this point her total IgG is not low enough to be extremely worrisome, and the vaccines she had not responded to can be redone as needed- so we will continue to monitor and see how things go. However, we were also told that if needed IVIG therapy could be a possibility at some point.

We were told that immune deficiency disorders were more common in the general population than CF, so while it's not common to have an individual present with both an immune disorder and CF, it's not unheard of.

Was just her total IgG checked (as is common in yearly labs) or did she have a full workup including vaccine titers and measuring levels of subclasses IgG1-4? If not I would ask for that.

Here is a good link discussing immune deficiency of various types... it will open up to discusssing subclass issues but you can find discussion on total IgG as well as other stuff if you look around. <img src="i/expressions/face-icon-small-smile.gif" border="0"> <a target=_blank class=ftalternatingbarlinklarge href="http://www.immunedisease.com/patients-and-families/about-pi/types-of-pi/igg-subclass-deficiency.html">ImmuneDisease.com</a>

 

[hmw]

I'm sorry your daughter has had such a rough time of it and you now have that feeling of 'another thing' to deal with. I do understand that (Emily has other issues in addition to cf.) It is good though to be able to address this and hopefully be able to keep her healthier!
<br />
<br />Emily had an immunology workup last summer after a bout of intermittent fevers lasting several months.
<br />
<br />Her results came back indicating she had mildly low total igG as well as low levels in a couple specific subclasses of IgG. When they checked her vaccine titers, a couple of them were nearly nonexistent (there were only trace levels of Prevnar & Pneumovax and very low HIB as well as something else I can't think of right now, although others were completely normal. The fact that some were very low and others were normal is to be expected in subclass deficiency.)
<br />
<br />It was upsetting to me as well- to have so little response to those particular vaccines and having low levels in those classes of IgG is NOT a good combination with CF due to the vulnerability to respiratory infection. Reduced total IgG is not so great either. We were told at this point her total IgG is not low enough to be extremely worrisome, and the vaccines she had not responded to can be redone as needed- so we will continue to monitor and see how things go. However, we were also told that if needed IVIG therapy could be a possibility at some point.
<br />
<br />We were told that immune deficiency disorders were more common in the general population than CF, so while it's not common to have an individual present with both an immune disorder and CF, it's not unheard of.
<br />
<br />Was just her total IgG checked (as is common in yearly labs) or did she have a full workup including vaccine titers and measuring levels of subclasses IgG1-4? If not I would ask for that.
<br />
<br />Here is a good link discussing immune deficiency of various types... it will open up to discusssing subclass issues but you can find discussion on total IgG as well as other stuff if you look around. <img src="i/expressions/face-icon-small-smile.gif" border="0"> <a target=_blank class=ftalternatingbarlinklarge href="http://www.immunedisease.com/patients-and-families/about-pi/types-of-pi/igg-subclass-deficiency.html">ImmuneDisease.com</a>

 

[lilywing]

I don't have an immunoglobulin deficiency, but I do have an auto-immune disorder called ITP (idiopathic thrombocytopenia purpura) wherein my body destroys my platelets. To help manage my ITP, I receive IVIG (intravenous immunoglobulin) infusions, 2 days every 2 weeks (52 times per year). I've been having these infusions since September 2005.

 

[lilywing]

I don't have an immunoglobulin deficiency, but I do have an auto-immune disorder called ITP (idiopathic thrombocytopenia purpura) wherein my body destroys my platelets. To help manage my ITP, I receive IVIG (intravenous immunoglobulin) infusions, 2 days every 2 weeks (52 times per year). I've been having these infusions since September 2005.

 

[lilywing]

I don't have an immunoglobulin deficiency, but I do have an auto-immune disorder called ITP (idiopathic thrombocytopenia purpura) wherein my body destroys my platelets. To help manage my ITP, I receive IVIG (intravenous immunoglobulin) infusions, 2 days every 2 weeks (52 times per year). I've been having these infusions since September 2005.

 

[rcq925]

Harriett,

Thank you so much for your reply. They have just run the initial immunoglobins blood screen,which showed her igG levels to be low, I believe that hers was around 200 and it should be anywhere from 400-1500 for her age. It also checked the other levels, but those were okay.

Our doctor just wants to get her through this infection and then run more blood work and tests. He did mention doing the checks for vaccine titers as well. So we will just have to wait and see.

Also thank you for the link!

 

[rcq925]

Harriett,

Thank you so much for your reply. They have just run the initial immunoglobins blood screen,which showed her igG levels to be low, I believe that hers was around 200 and it should be anywhere from 400-1500 for her age. It also checked the other levels, but those were okay.

Our doctor just wants to get her through this infection and then run more blood work and tests. He did mention doing the checks for vaccine titers as well. So we will just have to wait and see.

Also thank you for the link!

 

[rcq925]

Harriett,
<br />
<br />Thank you so much for your reply. They have just run the initial immunoglobins blood screen,which showed her igG levels to be low, I believe that hers was around 200 and it should be anywhere from 400-1500 for her age. It also checked the other levels, but those were okay.
<br />
<br />Our doctor just wants to get her through this infection and then run more blood work and tests. He did mention doing the checks for vaccine titers as well. So we will just have to wait and see.
<br />
<br />Also thank you for the link!

 

[hmw]

Emily's total IgG was something like 270, so not as low as your dd but still quite low. I'm glad my post and link were helpful! Like your child, her IgA, IgE, etc were normal too- it was just the IgG that came back a problem.

 

[hmw]

Emily's total IgG was something like 270, so not as low as your dd but still quite low. I'm glad my post and link were helpful! Like your child, her IgA, IgE, etc were normal too- it was just the IgG that came back a problem.

 

[hmw]

Emily's total IgG was something like 270, so not as low as your dd but still quite low. I'm glad my post and link were helpful! Like your child, her IgA, IgE, etc were normal too- it was just the IgG that came back a problem.