Re: Thanks Betty and a Question

[Guest guest]

My son has Chronic Neutropenia..he was found to have a Bone Marrow Failure

syndrome also affecting the platelets.....his initial Bone Marrow Biopsy showed

the cellularity of his marrow at 20% (should have been 80-90%)...he also has

poor neutrophil function. Mouth ulcers and gingivitis are a manifestation of

chronic neutropenia...my son also had meningitis, sepsis and mastoiditis, as

well as several bouts of bacterial pneumonia before he was started on

Neupogen...it has forced his Bone Marrow to produce more neutrophils, he

receives it 3x/week....it has definitely helped with the bacterial

infections....while he is on IVIG also. This is the result of a Chromosome

trisomy he has.

Neupogen has been a life-saver, but it is not without risks...since it does

alter the bone marrow, it can cause cellular changes including myelodysplasia

and leukemic changes...that's why bone marrow biopsies are done regularly to

watch for these dangerous changes. The benefits must outweigh the risk.

The usual approach to Neutropenia is looking for the cause....by a Bone Marrow

Biopsy, to check the functioning of the marrow...Is it producing enough white

blood cells?  If so, what is happening to them after they are released into the

circulation?  So that's why an Anti-Neutrophil-Antibody test must be

done......also the hematologist should order the Neupogen...he/she should do at

least 6 weeks of CBCs twice or three times a week for the 6 weeks to look for

any pattern, cycling....that would distinguish if it is cyclic neutropenia vs

chronic. They are different. ...They also need to check the other cell line

precursors in the Marrow...the platelets and red blood cells....

How has her total white count been? and how low has her ANC gone down to? a

chronically low ANC should surely be checked into thoroughly...starting with a

Bone Marrow Biopsy.

I think she should have a Hematology referral soon...hope this is just

temporary, sometimes viral infections result in temporary neutropenia....that's

another reason the 6 weeks of CBCs is done...! good luck...sue

From: cerdaclan@... <cerdaclan@...>

Subject: Thanks Betty and a Question

Date: Thursday, February 2, 2012, 5:35 PM

 

The last of the 3 of us got the orders, officially to re-start SCiG. Our 9

year old, with combined deficiency, has a strange problem. She has low memory B

cells, low activated T cells, Selective AB defiency, has had an elevated ANA of

greater than 1:1280 since she was 4, and chronic neutropenia. Her neutrophil

count hovers around 1200 when she is well, and drops to critical range when she

is sick. She develops patches on her tongue that appear like " bald " spots.

This happens almost constantly now and will clear up for a few days, only to

return. When this happens, she is exhausted. Recently, I sent a picture of it

to her immunologist and he feels it is the result of her neutropenia. The other

ongoing issue is hair loss. For the past couple of years, we noticed that,

after a bath, the tub is filled with her hair. This issue has been discounted

in the past, but now, she has bald spots on the front of her hair line where it

looks like her

hairling is recessing and sh She has an area, about 2 inches square, where her

hair has broken off to be only about an inch long. At first, I thought she cut

her hair.....but she did not. I am baffled....docs cannot explain. I did some

reading this morning and saw that hair loss can happen with chronic neutropenia.

I have a feeling, if I ran this by her new immunologist, he would likely agree.

Does anyone else have a child with this issue? She will be starting back up on

immunoglobin next week, after the scripts are all processed, and if this does

not help her, in terms of energy levels and wellness, they are thinking about

adding neupogen to the mix.

[Guest guest]

My son has Chronic Neutropenia..he was found to have a Bone Marrow Failure

syndrome also affecting the platelets.....his initial Bone Marrow Biopsy showed

the cellularity of his marrow at 20% (should have been 80-90%)...he also has

poor neutrophil function. Mouth ulcers and gingivitis are a manifestation of

chronic neutropenia...my son also had meningitis, sepsis and mastoiditis, as

well as several bouts of bacterial pneumonia before he was started on

Neupogen...it has forced his Bone Marrow to produce more neutrophils, he

receives it 3x/week....it has definitely helped with the bacterial

infections....while he is on IVIG also. This is the result of a Chromosome

trisomy he has.

Neupogen has been a life-saver, but it is not without risks...since it does

alter the bone marrow, it can cause cellular changes including myelodysplasia

and leukemic changes...that's why bone marrow biopsies are done regularly to

watch for these dangerous changes. The benefits must outweigh the risk.

The usual approach to Neutropenia is looking for the cause....by a Bone Marrow

Biopsy, to check the functioning of the marrow...Is it producing enough white

blood cells?  If so, what is happening to them after they are released into the

circulation?  So that's why an Anti-Neutrophil-Antibody test must be

done......also the hematologist should order the Neupogen...he/she should do at

least 6 weeks of CBCs twice or three times a week for the 6 weeks to look for

any pattern, cycling....that would distinguish if it is cyclic neutropenia vs

chronic. They are different. ...They also need to check the other cell line

precursors in the Marrow...the platelets and red blood cells....

How has her total white count been? and how low has her ANC gone down to? a

chronically low ANC should surely be checked into thoroughly...starting with a

Bone Marrow Biopsy.

I think she should have a Hematology referral soon...hope this is just

temporary, sometimes viral infections result in temporary neutropenia....that's

another reason the 6 weeks of CBCs is done...! good luck...sue

From: cerdaclan@... <cerdaclan@...>

Subject: Thanks Betty and a Question

Date: Thursday, February 2, 2012, 5:35 PM

 

The last of the 3 of us got the orders, officially to re-start SCiG. Our 9

year old, with combined deficiency, has a strange problem. She has low memory B

cells, low activated T cells, Selective AB defiency, has had an elevated ANA of

greater than 1:1280 since she was 4, and chronic neutropenia. Her neutrophil

count hovers around 1200 when she is well, and drops to critical range when she

is sick. She develops patches on her tongue that appear like " bald " spots.

This happens almost constantly now and will clear up for a few days, only to

return. When this happens, she is exhausted. Recently, I sent a picture of it

to her immunologist and he feels it is the result of her neutropenia. The other

ongoing issue is hair loss. For the past couple of years, we noticed that,

after a bath, the tub is filled with her hair. This issue has been discounted

in the past, but now, she has bald spots on the front of her hair line where it

looks like her

hairling is recessing and sh She has an area, about 2 inches square, where her

hair has broken off to be only about an inch long. At first, I thought she cut

her hair.....but she did not. I am baffled....docs cannot explain. I did some

reading this morning and saw that hair loss can happen with chronic neutropenia.

I have a feeling, if I ran this by her new immunologist, he would likely agree.

Does anyone else have a child with this issue? She will be starting back up on

immunoglobin next week, after the scripts are all processed, and if this does

not help her, in terms of energy levels and wellness, they are thinking about

adding neupogen to the mix.

[Guest guest]

Sue, thanks for the response.

Her neutrophil count has dropped to as low as 200 when she has an infection.

This has been true every time it has been tested during illness. I find the

part about viruses interesting. We have been told that her T cell function is

the big culprit of her immune disease and that she (and all of us since we have

the same for CID) leaves her highly vulnerable to primary viral infection and

that her SAD leaves her vulnerable to secondary bacterial infection. Another

think I did not mention is that she has extremely low NK cell count....it is 40,

with normal being 210. She is always neutropenic, but the levels fluctuate

significantly with illness. She also has chronically enlarged cervical nodes

and tested postitive for GI lymphoid hyperplasia. She had a hemoc following her

for years, but no one has ever recommended a bone marrow biopsy OR a node

biopsy. Once, when he was out of town, there was another doc covering for him.

She was very concerned about the size of her nodes, when she was not sick, that

she said she would biopsy them if she were her patient. She has an extremely

good immunologist following her now....we fly across the country to him. I

trust that he will make appropriate recommendations at this point. We ran the

hair loss issue by him this am and he is well aware of all of her other issues.

My guess is that he would run all the appropriate tests prior to jumping into

things like neupogen and I definately appreciate your feedback as it helps us

know what we need to address with him. Although he is a top notch immunologist,

he said he has never seen an immune disease like ours....his assessment reads

" it is a combined cellular and humoral combined immune deficiency that fits

into no defined immune deficiency " .......gotta love it.

>

> From: cerdaclan@... <cerdaclan@...>

> Subject: Thanks Betty and a Question

>

> Date: Thursday, February 2, 2012, 5:35 PM

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> The last of the 3 of us got the orders, officially to re-start SCiG. Our

9 year old, with combined deficiency, has a strange problem. She has low memory

B cells, low activated T cells, Selective AB defiency, has had an elevated ANA

of greater than 1:1280 since she was 4, and chronic neutropenia. Her neutrophil

count hovers around 1200 when she is well, and drops to critical range when she

is sick. She develops patches on her tongue that appear like " bald " spots.

This happens almost constantly now and will clear up for a few days, only to

return. When this happens, she is exhausted. Recently, I sent a picture of it

to her immunologist and he feels it is the result of her neutropenia. The other

ongoing issue is hair loss. For the past couple of years, we noticed that,

after a bath, the tub is filled with her hair. This issue has been discounted

in the past, but now, she has bald spots on the front of her hair line where it

looks like her

> hairling is recessing and sh She has an area, about 2 inches square, where

her hair has broken off to be only about an inch long. At first, I thought she

cut her hair.....but she did not. I am baffled....docs cannot explain. I did

some reading this morning and saw that hair loss can happen with chronic

neutropenia. I have a feeling, if I ran this by her new immunologist, he would

likely agree. Does anyone else have a child with this issue? She will be

starting back up on immunoglobin next week, after the scripts are all processed,

and if this does not help her, in terms of energy levels and wellness, they are

thinking about adding neupogen to the mix.

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[Guest guest]

Sue, thanks for the response.

Her neutrophil count has dropped to as low as 200 when she has an infection.

This has been true every time it has been tested during illness. I find the

part about viruses interesting. We have been told that her T cell function is

the big culprit of her immune disease and that she (and all of us since we have

the same for CID) leaves her highly vulnerable to primary viral infection and

that her SAD leaves her vulnerable to secondary bacterial infection. Another

think I did not mention is that she has extremely low NK cell count....it is 40,

with normal being 210. She is always neutropenic, but the levels fluctuate

significantly with illness. She also has chronically enlarged cervical nodes

and tested postitive for GI lymphoid hyperplasia. She had a hemoc following her

for years, but no one has ever recommended a bone marrow biopsy OR a node

biopsy. Once, when he was out of town, there was another doc covering for him.

She was very concerned about the size of her nodes, when she was not sick, that

she said she would biopsy them if she were her patient. She has an extremely

good immunologist following her now....we fly across the country to him. I

trust that he will make appropriate recommendations at this point. We ran the

hair loss issue by him this am and he is well aware of all of her other issues.

My guess is that he would run all the appropriate tests prior to jumping into

things like neupogen and I definately appreciate your feedback as it helps us

know what we need to address with him. Although he is a top notch immunologist,

he said he has never seen an immune disease like ours....his assessment reads

" it is a combined cellular and humoral combined immune deficiency that fits

into no defined immune deficiency " .......gotta love it.

>

> From: cerdaclan@... <cerdaclan@...>

> Subject: Thanks Betty and a Question

>

> Date: Thursday, February 2, 2012, 5:35 PM

>

>

>

>

>

>

>

>

>  

>

>

>

>

>

>

>

>

>

> The last of the 3 of us got the orders, officially to re-start SCiG. Our

9 year old, with combined deficiency, has a strange problem. She has low memory

B cells, low activated T cells, Selective AB defiency, has had an elevated ANA

of greater than 1:1280 since she was 4, and chronic neutropenia. Her neutrophil

count hovers around 1200 when she is well, and drops to critical range when she

is sick. She develops patches on her tongue that appear like " bald " spots.

This happens almost constantly now and will clear up for a few days, only to

return. When this happens, she is exhausted. Recently, I sent a picture of it

to her immunologist and he feels it is the result of her neutropenia. The other

ongoing issue is hair loss. For the past couple of years, we noticed that,

after a bath, the tub is filled with her hair. This issue has been discounted

in the past, but now, she has bald spots on the front of her hair line where it

looks like her

> hairling is recessing and sh She has an area, about 2 inches square, where

her hair has broken off to be only about an inch long. At first, I thought she

cut her hair.....but she did not. I am baffled....docs cannot explain. I did

some reading this morning and saw that hair loss can happen with chronic

neutropenia. I have a feeling, if I ran this by her new immunologist, he would

likely agree. Does anyone else have a child with this issue? She will be

starting back up on immunoglobin next week, after the scripts are all processed,

and if this does not help her, in terms of energy levels and wellness, they are

thinking about adding neupogen to the mix.

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[Guest guest]

Sue, I missed a couple of things.....she has been neutropenic for years.....it

is not a new thing. We have labs from 3 and 4 years ago where her counts were

200-300. Her WBC runs a little low all the time (3.9 or there abouts) and also

drops to low range (not critical) with active infections. She NEVER raises her

white cell count during infections. I believe it was around 1.7 last it was

checked. She has hers labs done only every 6 months or so. She has had one of

her " non-sick " labs come back with a neutrophil count in critical range and a

WBC in very low range (1.9)...she slept all day long during this period......She

is always tired, but I can tell when her counts are down because she is

exhausted and her mouth is a mess with almost the entire surface of her tongue

gone and mouth sores. The problem is she also has chronic mucotaneous candiasis

and I always thought that was the cause until the immuno said he thought it was

the neutropenia, not the CMC.....tough and confusing to say the least.

> >

> > From: cerdaclan@ <cerdaclan@>

> > Subject: Thanks Betty and a Question

> >

> > Date: Thursday, February 2, 2012, 5:35 PM

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> > The last of the 3 of us got the orders, officially to re-start SCiG.

Our 9 year old, with combined deficiency, has a strange problem. She has low

memory B cells, low activated T cells, Selective AB defiency, has had an

elevated ANA of greater than 1:1280 since she was 4, and chronic neutropenia.

Her neutrophil count hovers around 1200 when she is well, and drops to critical

range when she is sick. She develops patches on her tongue that appear like

" bald " spots. This happens almost constantly now and will clear up for a few

days, only to return. When this happens, she is exhausted. Recently, I sent a

picture of it to her immunologist and he feels it is the result of her

neutropenia. The other ongoing issue is hair loss. For the past couple of

years, we noticed that, after a bath, the tub is filled with her hair. This

issue has been discounted in the past, but now, she has bald spots on the front

of her hair line where it looks like her

> > hairling is recessing and sh She has an area, about 2 inches square, where

her hair has broken off to be only about an inch long. At first, I thought she

cut her hair.....but she did not. I am baffled....docs cannot explain. I did

some reading this morning and saw that hair loss can happen with chronic

neutropenia. I have a feeling, if I ran this by her new immunologist, he would

likely agree. Does anyone else have a child with this issue? She will be

starting back up on immunoglobin next week, after the scripts are all processed,

and if this does not help her, in terms of energy levels and wellness, they are

thinking about adding neupogen to the mix.

> >

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[Guest guest]

Macey's had cyclic neutropenia in the past. She has always had a problem

with losing hair. They attribute it to poor nutrition (crappy gut) and

chronic disease state of her body. She's also had geographic tongue in the

past but i'm not sure that's what you are describing.

p.s. don't forget to sign your posts.

Ursula

mom to macey (16,CVID)

On Thu, Feb 2, 2012 at 12:35 PM, cerdaclan@... <

cerdaclan@...> wrote:

> **

>

>

> The last of the 3 of us got the orders, officially to re-start SCiG. Our 9

> year old, with combined deficiency, has a strange problem. She has low

> memory B cells, low activated T cells, Selective AB defiency, has had an

> elevated ANA of greater than 1:1280 since she was 4, and chronic

> neutropenia. Her neutrophil count hovers around 1200 when she is well, and

> drops to critical range when she is sick. She develops patches on her

> tongue that appear like " bald " spots. This happens almost constantly now

> and will clear up for a few days, only to return. When this happens, she is

> exhausted. Recently, I sent a picture of it to her immunologist and he

> feels it is the result of her neutropenia. The other ongoing issue is hair

> loss. For the past couple of years, we noticed that, after a bath, the tub

> is filled with her hair. This issue has been discounted in the past, but

> now, she has bald spots on the front of her hair line where it looks like

> her hairling is recessing and sh She has an area, about 2 inches square,

> where her hair has broken off to be only about an inch long. At first, I

> thought she cut her hair.....but she did not. I am baffled....docs cannot

> explain. I did some reading this morning and saw that hair loss can happen

> with chronic neutropenia. I have a feeling, if I ran this by her new

> immunologist, he would likely agree. Does anyone else have a child with

> this issue? She will be starting back up on immunoglobin next week, after

> the scripts are all processed, and if this does not help her, in terms of

> energy levels and wellness, they are thinking about adding neupogen to the

> mix.

>

>

>