Re: Info, please

[Guest guest]

Dear ,

Your question is a good one. Determining the use of IVIG is usually based on

an individual basis. Over the past few years many top ranking immunologists

across the US and even around the world have recognized the over use and

misuse of IVIG, even with PIDS. The diagnosis of PID does not always mean

that a patient needs IVIG as the treatment. More so, physicians are now

recognizing the " symptoms " more then the lab numbers. Over time insurance

companies will begin to review these such case to " weed out " those that

perhaps really do not need IVIG as a therapy. This has been a " hot-topic "

among physicians for some time now. So your question is good since you will

just be beginning your process of trying to figure out why has so many

infections all the time and perhaps this explanation will help you understand

why some physicians are not as eager to begin IVIG right away anymore. My

first thought is this... has some low levels of certain immunoglobulins,

but, do they work? That is the key factor. Since you are wondering how we

came to the use of IVIG I will give you our " brief (LOL) story " of how we

began IVIG (some of you may want to stop reading at this time as this is a

bit long). With Mark, it was first noted at the age of 2 that he had a very

low level of immunoglobulins, a very low IgA and no response to polio. 7

injectable doses of polio over time still proved that he could not muster a

response (interestingly, polio is a protein not a polysaccharide). At the

age of 3 1/2 it was noted by the first allergist that we had seen that Mark

made very little response to DPT. A supportive 2nd and 3rd dose was given

and the numbers hardly budged. In 1994 we saw our first immunologist. More

tests revealed a reversed C4 and C8 (Tcells) and Mark's immunoglobulins were

low, but not that low. IgA was still low at this time. Hence the diagnosis

of Hypogammaglobulinemia came about (or so we thought). We decided to wait

and see. From 1994 to 1996 Mark's health continued to decline and the issue

of how to treat it arose again (I must tell you that I was very much against

the use of IVIG during all of this time). In April in 1996 Mark's first

pneummovax was given and the titer response was completely absent, meaning,

the test result indicated -0- as the number (not 1.8, 2.0, 3.5, or 4.1 for

example or any other low number non-protective number, just flat out ZEROS).

Off to U of M for our second opinion. U of M did their own testing and

hence, the diagnosis of Common Variable Immune Deficiency was given. I

should mention, that through all of this was Mark was also diagnosed with

asthma, fructose malabsorption deficiency, A1A, GERD, anemia, and at that

time questionable IBD (which as since been confirmed by colonoscopy). OK, I

was concerned with the prognoses that U of M was giving us so off we went for

a third opinion. We did not tell the third physician that we had been to U

of M....the result of our third opinion was CVID as well. We went back to

our first immunologist out of Children's Hospital in Detroit, who at that

time was Chief of Immunology. He told my husband and I that it was not a

question of " if " Mark needed IVIG, but " when. " Since I was still concerned

about the use of a blood product and was in for the most part " severe

denial, " I asked for a 4th opinion from Hopkin's very well known IDF

physician, named Lederman. Dr. Lederman, agreed with all of the above

and came up with questions of his own which did not sit well with me. I then

wrote to Dr. Geha out of Boston and he gave me some sound advice. I followed

it. I decided to take Mark to someone that strictly did immunology verses

allergy/immunology (which can be a different school of thought) and I

contacted the IDF for various names in states surrounding Michigan. Which is

how we ended up with Dr. Hostoffer in Cleveland. Dr. Hostoffer reviewed the

pages and pages of clinical notes, ER notes, hospital admissions, and lab

results. He told my husband and I that Mark clearly had CVID (so he thought

at the time) but wanted to return the pneumovax as he had NEVER seen titers

come back as -0- as the number. Many physicians will call a patient a

non-responder if the level too low and considered non-protective. Not only

was Mark a non-responder but he did not muster any response not even a

non-protective low number. So, off to the lab in Cleveland. Several weeks

later we were phoned and told that 12 out of 12 pneumococcal titers were

absent and we were asked to return to Cleveland as soon as we could and IVIG

was started immediately (I had to get out of denial at this point and face my

fears). HIB titers were drawn (after a supportive dose) and were 100%

absent. Mark's health continued to decline and the faces of autoimmune

diseases were starting to show up, something that usually does not happen in

CVID until the 2nd or 3rd decade of life. Dr. Hostoffer told us of one other

patient that he had that made -0- response and that this boy was similar to

Mark. He asked that we allow Mark to be admitted in to RB & C for week or

two and that we also get more lab work down that day. On the day of

admission (which was the following week) we were told by Dr.Hostoffer that

Mark had no b-cells, that all his b-cells were Cd5+ positive cells, premature

b-cells that do not offer antibody protection. Thus, the discovery of the

Cd5-Cd19 PID, a combinatin B-cell T-cell immune deficiency with a second

componet of Cd5+ cells that are destructive. The rest is in the medical

journals. Mark can measures immunoglobulins, but they are all Cd5+ bcells,

he has less then 2% circulating b-cells which is basically nothing. He makes

absolutely no response to pneumococcal, HIB, meningocaccol, very low to DPT

and very low to polio. He does not make " in-adequate responses/non-responses

but rather no responses (0) what so ever (again, I have to make this clear if

any one ever suspects they may have this) That is why this PID is twofold 1).

No B-cells and 2.) a cell that is destructive causing autoantibodies,

autoimmune disease and malignancies.

IVIG is going to be lifelong for Mark. Life expectancy is unknown but we are

told that it will be less then average from what they know at this time.

Quality of life will be poor. The Cd5-Cd19 PID is a moderate to severe PID

with many, many unanswered questions. I encourage you to ask many, many

questions and do not be afraid to do so. I realize that ours is an

exceptional story in as much as I went for many opinions for my own fear of

IVIG use ( I had to be certain) even afer being told by one physician after

another that Mark needed it. Our first immunologist works together with Dr.

Hostoffer and is known to be conservative in the use of IVIG, that helped to

ease some of my fears as I do realize that Mark survives because of it the

use of IVIG. It is important to look at the symptoms and if the numbers and

symptoms do not add up I would then recommend inquiring about an antibody

study to see if the immunglobulins/b-cells are working. Make sure that you

go to a good immunologist. Over the years I have heard stories of people

receiving a diagnosis and wanting their child to be placed on IVIG right

away. Make sure your child would need it. Many patients have terrible

numbers and no symptoms and then there are those that are the opposite. Good

example is who by physician standards presents worse then many PID/CF

patients and is not a PID or CF. ENT and pulmonary wanted him on IVIG a

long time ago and our immunologist said no way...we agreed. Unfortunately,

now does have a secondary immune deficiency as a result of being steroid

dependent so we keep a close eye on him. Keep an open mind and remember you

can always go for a second or third opininon. Good luck to you in your

pursuit.

Autumn mom to Mark Cd5-Cd19 PID, achalasia, GERD, ASA, A1A

[Guest guest]

- Macey had been treated as much as medically/surgically possible when

she was diagnosed. She was having her second sinus surgery in as many

months and they decided to put in her first PICC and then run tests. They

tested for immotile cilia from the sinus lavage samples and took blood to

test for the immune deficiency. That was on December 10, 1997, on December

15, 1997 we received a call from her pulmonologist with the term

" hypogammaglobulinemia " . He also had a surgery date for her port to be put

in. For a year before that they had been treating her symptoms and not what

was causing it. During her first sinus surgery (Oct 97) they cultured staph

A out of her sinuses and that I think started the ball rolling to find out

what was truly going on in her. Her initial level was 350 total, with

deficiency in subclass 1, 3 & 4. Plus a deficient IgA and IgM and low IgE.

Has been seeing the immunologist and is he established with him? Or is

it 's immunologist who is helping out. When Macey was diagnosed they

had and myself tested. It was a very simple blood test that insurance

had no problems covering. Since the test is so simple to do and the turn

around is usually only 3-5 days then I don't see where it wouldn't hurt to

run new levels on him. Again I'll say the same thing I tell all people,

also test his antibody levels. Remember there is a PID in itself called

" Selective Antibody Deficiency " . It is also treated by IVIG.

Ursula - & Macey (4 yr old w/CVID) mom

http://home.att.net/~maceyh/

[Guest guest]

In a message dated 01/05/2000 3:06:20 PM Eastern Standard Time, Autti@...

writes:

<< Mark can measures immunoglobulins, but they are all Cd5+ bcells,

he has less then 2% circulating b-cells which is basically nothing. He makes

absolutely no response to pneumococcal, HIB, meningocaccol, very low to DPT

and very low to polio. He does not make " in-adequate

responses/non-responses

but rather no responses (0) what so ever (again, I have to make this clear

if

any one ever suspects they may have this) >>

Autumn and I have talked about this issue many times. is an X-Linked

SCID, and after his BMT he reconstituted T-cells, but he never achieved

mature B-cells. He has his brother's T-cells, but regained his own

defective B-cells. has an adequate supply of B-cells, but they remain

naive (immature) cells, unable to mount an antibody response. Like Mark,

produces measurable immunoglobulin levels, and also like Mark, those

immunoglobulins are absolutely worthless. 's immunologists tried to

vaccinate with Hep B, HiB, polio, tetanus, diptheria, etc. No response

levels. Then, January of 1999, had the Bacteriophage Immunization study

done by Dr. Ochs in Seattle. This is the definitive test to determine if

B-cells are capable of mounting an antibody response. I think a few people on

this list have had the test done. I know Ursula's daughter Macey has.

(Ursula, I'm sorry I can't remember Macey's results.) Anyway, 's results

showed no antibody response at all. Zip. Zilch. Crash & Burn. In fact, the

results were so negative that Dr. Ochs called Dr. Starr ('s

immunologist) to find out what type of immune deficiency had, because

he seldom sees such a negative response. will need IVIG for the rest of

his life. Autumn and I have also talked about the autoimme correlations

between Mark and , despite the fact that they have different PIDs.

Just a little more info .....

Debbie, Mom to , 3.7 yrs old, Severe Combined Immunodeficiency post

Bone Marrow Transplant, IVIG dependant, Inflammatory Bowel Disease (IBD),

GERD with hiatal hernia, chronic hepatitis, G-tube, Hypotonic CP, non-verbal,

developmental delays, CVI, seizure disorder

[Guest guest]

, YOur is on almost the same meds as my daughter and she is on

IVIG. My immunologist goes by the rate of infections and not just by her

levels. It is worth investigating. I have learned in the past that a

mother's intuition is the best dx tool. Hope my two cents helped and hope

things improve with your pumpkins, I have a hard enought time taking care of

one I admire all the mothers that have more than just one sick kid to try

and manage. God Bless

annette mom to alissa

>

>Reply-To: PedPIDonelist

>To: " PEDpid " <PEDpidonelist>

>Subject: Info, please

>Date: Tue, 4 Jan 2000 17:00:32 -0500

>

> will be going to see his immunologist/allergist later this month and

>I have some questions and need a little info from the group. First,

>is my oldest--he's 8. He has had allergy symptoms since he was about 6

>months old, ear infections starting at around 3, and asthma diagnosed at

>age 5. Back in 97 when was initially diagnosed with

>Hypogammaglobulinemia we had tested. I was told that his tests came

>back normal only to find out last fall that his sub-class 1 was low--by

>approximatley 18%.

>

> has been increasingly gotten more " sickly " every year. He is on

>Flovent, Serevent, Vanconase, Singulair, and Albuteral daily. He takes

>Claritin when needed as well as using Elecon for exema. He has had a

>complete work up for allergies and shows positive to only the Hickory/Pecan

>family pollens. He " lost " his allergies to dustmites. There is no

>stomache upsets over food and his ear infections have recently

>stopped--none for over 1 year. He recently had a full culture

>smear--viral, fungal and bacterial which all come back negative. His CT

>scan of his sinuses showed a " bump " that requires monitoring, but is " not

>uncommon " in persons with chronic, untreated allergies.

>

>Now, here's where I need info/help. What were your child's levels when the

>doctors decided to try IVIG? What were the symptoms or reasons for it?

>Was this done as a test or as a last resort measure?

>

>'s immunologist is at a loss--when he saw him in September, he was

>baffled. Since then, has had more colds, viruses and problems than

>--who is immune defecient. I know his doctor doesn't want to really

>test for immune problems, but seems to me to maybe need them. Am I

>being over-reactive, or does it seem like I may asking for the right stuff?

> Any input would really help. Sorry about the novel and thanks in

>advance. BTW, this doctor told me a year ago that there is no way that

> could have the same problems has--yet the two kids are too

>simular to me.

>

>--mom to

>

>

>[Attachments have been removed from this message]

>

>------------------------------------------------------------------------

>This forum is open to parents and caregivers of children diagnosed with a

>Primary Immune Deficiency. Opinions or medical advice stated here are the

>sole responsibility of the poster and should not be taken as professional

>advice.

><< text3.html >>

______________________________________________________

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[Guest guest]

,

One more note....can't believe that I failed to mention this in my novel to

the group. Prior to IVIG Mark had chronic infections of the GI, lungs, ears,

sinuses, pneumonia's all the time. At one point he was on full course

antibiotics for 52 weeks straight without getting better. He cultured one

bacteria after another. I just wanted to clarify that Mark was indeed quite

ill at the time and was not placed on IVIG for lab alone numbers but for

symptoms and quality of life as well.

Autumn mom to Mark Cd5-Cd19 PID, ASA, A1A, GERD, Achalasia

[Guest guest]

Ursula:

Thanks for the input. and both see the same immunologist, the

same allergist, the same ENT, the same everything--sometimes I think I have

twins instead of boys 19 months apart! In fact, started seeing this

doctor before did because was being handled primarily through

Oncology at the time. I will be asking about the Titers-- has a memory

problem with his so this is a good question. Thanks for reminding me.

RE: Info, please

> - Macey had been treated as much as medically/surgically possible

when

> she was diagnosed. She was having her second sinus surgery in as many

> months and they decided to put in her first PICC and then run tests. They

> tested for immotile cilia from the sinus lavage samples and took blood to

> test for the immune deficiency. That was on December 10, 1997, on

December

> 15, 1997 we received a call from her pulmonologist with the term

> " hypogammaglobulinemia " . He also had a surgery date for her port to be

put

> in. For a year before that they had been treating her symptoms and not

what

> was causing it. During her first sinus surgery (Oct 97) they cultured

staph

> A out of her sinuses and that I think started the ball rolling to find out

> what was truly going on in her. Her initial level was 350 total, with

> deficiency in subclass 1, 3 & 4. Plus a deficient IgA and IgM and low

IgE.

>

> Has been seeing the immunologist and is he established with him? Or

is

> it 's immunologist who is helping out. When Macey was diagnosed they

> had and myself tested. It was a very simple blood test that

insurance

> had no problems covering. Since the test is so simple to do and the turn

> around is usually only 3-5 days then I don't see where it wouldn't hurt to

> run new levels on him. Again I'll say the same thing I tell all people,

> also test his antibody levels. Remember there is a PID in itself called

> " Selective Antibody Deficiency " . It is also treated by IVIG.

>

> Ursula - & Macey (4 yr old w/CVID) mom

> http://home.att.net/~maceyh/

>

>

>

> ---------------------------

[Guest guest]

Dear : I hope that your family is doing better and will keep them all

in our prayers, forgive the shortness of this have to go to work,GodBLess,

annette and alissa

>

>Reply-To: PedPIDonelist

>To: <PedPIDonelist>

>Subject: Re: Info, please

>Date: Fri, 7 Jan 2000 11:32:50 -0500

>

>To everyone who answered my questions, thank you. I now feel that my

>concerns regarding are enough to ask for--maybe even demand--some

>furhter testing. I would love to figure out a way to reduce his daily

>medications so this may be the way for us to proceed. Thanks again.

>

>Things here have been busy. My husband ended up in the E.R. on Wed. with a

>fever over 105. He has one of the flu bugs going around. He is still

>home,

>and I actually am getting a little tired of waiting on him--when I had the

>bug a few weeks ago, I had to do most everything for myself. Oh well, that

>comes with being the mommy. has a bad case of the runs which has led

>her bottom to the open, bleeding sores. So now I am administering my own

>advice about that to her. (Actually, I think it's a mild case of

>Rotovirus--smells like it to me, but I haven't taken her in to see her ped

>for conformation.) is struggling against something--he's looking

>anemic and he has no energy, but his hemoglobin is fine. I hope he's not

>getting this flu that's going around. is still fighting off yet

>another cold--or symptoms of one. Me, I'm doing fine. Tired, but fine.

>

>Ursula--Here's to a good, speedy recovery to Macey.

>--Sorry to hear about Zach. I think we all have at one time or

>another looked back on a bad night and in hindsight think that we messed up

>by not getting our kids into the E.R. Please, keep us updated on Zach.

>

>--mom to -dysgammaglobulinemia; Leukemia survivor

> Re: Info, please

>

>

> > Dear ,

> >

> > Your question is a good one. Determining the use of IVIG is usually

>based

>on

> > an individual basis. Over the past few years many top ranking

>immunologists

> > across the US and even around the world have recognized the over use and

> > misuse of IVIG, even with PIDS. The diagnosis of PID does not always

>mean

> > that a patient needs IVIG as the treatment. More so, physicians are now

> > recognizing the " symptoms " more then the lab numbers. Over time

>insurance

> > companies will begin to review these such case to " weed out " those that

> > perhaps really do not need IVIG as a therapy. This has been a

> " hot-topic "

> > among physicians for some time now. So your question is good since you

>will

> > just be beginning your process of trying to figure out why has so

>many

> > infections all the time and perhaps this explanation will help you

>understand

> > why some physicians are not as eager to begin IVIG right away anymore.

>My

> > first thought is this... has some low levels of certain

>immunoglobulins,

> > but, do they work? That is the key factor. Since you are wondering how

>we

> > came to the use of IVIG I will give you our " brief (LOL) story " of how

>we

> > began IVIG (some of you may want to stop reading at this time as this is

>a

> > bit long). With Mark, it was first noted at the age of 2 that he had a

>very

> > low level of immunoglobulins, a very low IgA and no response to polio.

>7

> > injectable doses of polio over time still proved that he could not

>muster

>a

> > response (interestingly, polio is a protein not a polysaccharide). At

>the

> > age of 3 1/2 it was noted by the first allergist that we had seen that

>Mark

> > made very little response to DPT. A supportive 2nd and 3rd dose was

>given

> > and the numbers hardly budged. In 1994 we saw our first immunologist.

>More

> > tests revealed a reversed C4 and C8 (Tcells) and Mark's immunoglobulins

>were

> > low, but not that low. IgA was still low at this time. Hence the

>diagnosis

> > of Hypogammaglobulinemia came about (or so we thought). We decided to

>wait

> > and see. From 1994 to 1996 Mark's health continued to decline and the

>issue

> > of how to treat it arose again (I must tell you that I was very much

>against

> > the use of IVIG during all of this time). In April in 1996 Mark's first

> > pneummovax was given and the titer response was completely absent,

>meaning,

> > the test result indicated -0- as the number (not 1.8, 2.0, 3.5, or 4.1

>for

> > example or any other low number non-protective number, just flat out

>ZEROS).

> > Off to U of M for our second opinion. U of M did their own testing and

> > hence, the diagnosis of Common Variable Immune Deficiency was given. I

> > should mention, that through all of this was Mark was also diagnosed

>with

> > asthma, fructose malabsorption deficiency, A1A, GERD, anemia, and at

>that

> > time questionable IBD (which as since been confirmed by colonoscopy).

>OK,

>I

> > was concerned with the prognoses that U of M was giving us so off we

>went

>for

> > a third opinion. We did not tell the third physician that we had been

>to

>U

> > of M....the result of our third opinion was CVID as well. We went back

>to

> > our first immunologist out of Children's Hospital in Detroit, who at

>that

> > time was Chief of Immunology. He told my husband and I that it was not

>a

> > question of " if " Mark needed IVIG, but " when. " Since I was still

>concerned

> > about the use of a blood product and was in for the most part " severe

> > denial, " I asked for a 4th opinion from Hopkin's very well known

>IDF

> > physician, named Lederman. Dr. Lederman, agreed with all of the

>above

> > and came up with questions of his own which did not sit well with me. I

>then

> > wrote to Dr. Geha out of Boston and he gave me some sound advice. I

>followed

> > it. I decided to take Mark to someone that strictly did immunology

>verses

> > allergy/immunology (which can be a different school of thought) and I

> > contacted the IDF for various names in states surrounding Michigan.

>Which

>is

> > how we ended up with Dr. Hostoffer in Cleveland. Dr. Hostoffer reviewed

>the

> > pages and pages of clinical notes, ER notes, hospital admissions, and

>lab

> > results. He told my husband and I that Mark clearly had CVID (so he

>thought

> > at the time) but wanted to return the pneumovax as he had NEVER seen

>titers

> > come back as -0- as the number. Many physicians will call a patient a

> > non-responder if the level too low and considered non-protective. Not

>only

> > was Mark a non-responder but he did not muster any response not even a

> > non-protective low number. So, off to the lab in Cleveland. Several

>weeks

> > later we were phoned and told that 12 out of 12 pneumococcal titers were

> > absent and we were asked to return to Cleveland as soon as we could and

>IVIG

> > was started immediately (I had to get out of denial at this point and

>face

>my

> > fears). HIB titers were drawn (after a supportive dose) and were 100%

> > absent. Mark's health continued to decline and the faces of autoimmune

> > diseases were starting to show up, something that usually does not

>happen

>in

> > CVID until the 2nd or 3rd decade of life. Dr. Hostoffer told us of one

>other

> > patient that he had that made -0- response and that this boy was similar

>to

> > Mark. He asked that we allow Mark to be admitted in to RB & C for week

>or

> > two and that we also get more lab work down that day. On the day of

> > admission (which was the following week) we were told by Dr.Hostoffer

>that

> > Mark had no b-cells, that all his b-cells were Cd5+ positive cells,

>premature

> > b-cells that do not offer antibody protection. Thus, the discovery of

>the

> > Cd5-Cd19 PID, a combinatin B-cell T-cell immune deficiency with a second

> > componet of Cd5+ cells that are destructive. The rest is in the medical

> > journals. Mark can measures immunoglobulins, but they are all Cd5+

>bcells,

> > he has less then 2% circulating b-cells which is basically nothing. He

>makes

> > absolutely no response to pneumococcal, HIB, meningocaccol, very low to

>DPT

> > and very low to polio. He does not make " in-adequate

>responses/non-responses

> > but rather no responses (0) what so ever (again, I have to make this

>clear

>if

> > any one ever suspects they may have this) That is why this PID is

>twofold

>1).

> > No B-cells and 2.) a cell that is destructive causing autoantibodies,

> > autoimmune disease and malignancies.

> > IVIG is going to be lifelong for Mark. Life expectancy is unknown but

>we

>are

> > told that it will be less then average from what they know at this time.

> > Quality of life will be poor. The Cd5-Cd19 PID is a moderate to severe

>PID

> > with many, many unanswered questions. I encourage you to ask many, many

> > questions and do not be afraid to do so. I realize that ours is an

> > exceptional story in as much as I went for many opinions for my own fear

>of

> > IVIG use ( I had to be certain) even afer being told by one physician

>after

> > another that Mark needed it. Our first immunologist works together with

>Dr.

> > Hostoffer and is known to be conservative in the use of IVIG, that

>helped

>to

> > ease some of my fears as I do realize that Mark survives because of it

>the

> > use of IVIG. It is important to look at the symptoms and if the numbers

>and

> > symptoms do not add up I would then recommend inquiring about an

>antibody

> > study to see if the immunglobulins/b-cells are working. Make sure that

>you

> > go to a good immunologist. Over the years I have heard stories of

>people

> > receiving a diagnosis and wanting their child to be placed on IVIG right

> > away. Make sure your child would need it. Many patients have terrible

> > numbers and no symptoms and then there are those that are the opposite.

>Good

> > example is who by physician standards presents worse then many

>PID/CF

> > patients and is not a PID or CF. ENT and pulmonary wanted him on IVIG

>a

> > long time ago and our immunologist said no way...we agreed.

>Unfortunately,

> > now does have a secondary immune deficiency as a result of being

>steroid

> > dependent so we keep a close eye on him. Keep an open mind and remember

>you

> > can always go for a second or third opininon. Good luck to you in your

> > pursuit.

> >

> > Autumn mom to Mark Cd5-Cd19 PID, achalasia, GERD, ASA, A1A

> >

> > ---------------------------

[Guest guest]

To everyone who answered my questions, thank you. I now feel that my

concerns regarding are enough to ask for--maybe even demand--some

furhter testing. I would love to figure out a way to reduce his daily

medications so this may be the way for us to proceed. Thanks again.

Things here have been busy. My husband ended up in the E.R. on Wed. with a

fever over 105. He has one of the flu bugs going around. He is still home,

and I actually am getting a little tired of waiting on him--when I had the

bug a few weeks ago, I had to do most everything for myself. Oh well, that

comes with being the mommy. has a bad case of the runs which has led

her bottom to the open, bleeding sores. So now I am administering my own

advice about that to her. (Actually, I think it's a mild case of

Rotovirus--smells like it to me, but I haven't taken her in to see her ped

for conformation.) is struggling against something--he's looking

anemic and he has no energy, but his hemoglobin is fine. I hope he's not

getting this flu that's going around. is still fighting off yet

another cold--or symptoms of one. Me, I'm doing fine. Tired, but fine.

Ursula--Here's to a good, speedy recovery to Macey.

--Sorry to hear about Zach. I think we all have at one time or

another looked back on a bad night and in hindsight think that we messed up

by not getting our kids into the E.R. Please, keep us updated on Zach.

--mom to -dysgammaglobulinemia; Leukemia survivor

Re: Info, please

> Dear ,

>

> Your question is a good one. Determining the use of IVIG is usually based

on

> an individual basis. Over the past few years many top ranking

immunologists

> across the US and even around the world have recognized the over use and

> misuse of IVIG, even with PIDS. The diagnosis of PID does not always mean

> that a patient needs IVIG as the treatment. More so, physicians are now

> recognizing the " symptoms " more then the lab numbers. Over time insurance

> companies will begin to review these such case to " weed out " those that

> perhaps really do not need IVIG as a therapy. This has been a " hot-topic "

> among physicians for some time now. So your question is good since you

will

> just be beginning your process of trying to figure out why has so

many

> infections all the time and perhaps this explanation will help you

understand

> why some physicians are not as eager to begin IVIG right away anymore. My

> first thought is this... has some low levels of certain

immunoglobulins,

> but, do they work? That is the key factor. Since you are wondering how we

> came to the use of IVIG I will give you our " brief (LOL) story " of how we

> began IVIG (some of you may want to stop reading at this time as this is a

> bit long). With Mark, it was first noted at the age of 2 that he had a

very

> low level of immunoglobulins, a very low IgA and no response to polio. 7

> injectable doses of polio over time still proved that he could not muster

a

> response (interestingly, polio is a protein not a polysaccharide). At the

> age of 3 1/2 it was noted by the first allergist that we had seen that

Mark

> made very little response to DPT. A supportive 2nd and 3rd dose was given

> and the numbers hardly budged. In 1994 we saw our first immunologist.

More

> tests revealed a reversed C4 and C8 (Tcells) and Mark's immunoglobulins

were

> low, but not that low. IgA was still low at this time. Hence the

diagnosis

> of Hypogammaglobulinemia came about (or so we thought). We decided to wait

> and see. From 1994 to 1996 Mark's health continued to decline and the

issue

> of how to treat it arose again (I must tell you that I was very much

against

> the use of IVIG during all of this time). In April in 1996 Mark's first

> pneummovax was given and the titer response was completely absent,

meaning,

> the test result indicated -0- as the number (not 1.8, 2.0, 3.5, or 4.1 for

> example or any other low number non-protective number, just flat out

ZEROS).

> Off to U of M for our second opinion. U of M did their own testing and

> hence, the diagnosis of Common Variable Immune Deficiency was given. I

> should mention, that through all of this was Mark was also diagnosed with

> asthma, fructose malabsorption deficiency, A1A, GERD, anemia, and at that

> time questionable IBD (which as since been confirmed by colonoscopy). OK,

I

> was concerned with the prognoses that U of M was giving us so off we went

for

> a third opinion. We did not tell the third physician that we had been to

U

> of M....the result of our third opinion was CVID as well. We went back to

> our first immunologist out of Children's Hospital in Detroit, who at that

> time was Chief of Immunology. He told my husband and I that it was not a

> question of " if " Mark needed IVIG, but " when. " Since I was still

concerned

> about the use of a blood product and was in for the most part " severe

> denial, " I asked for a 4th opinion from Hopkin's very well known IDF

> physician, named Lederman. Dr. Lederman, agreed with all of the

above

> and came up with questions of his own which did not sit well with me. I

then

> wrote to Dr. Geha out of Boston and he gave me some sound advice. I

followed

> it. I decided to take Mark to someone that strictly did immunology verses

> allergy/immunology (which can be a different school of thought) and I

> contacted the IDF for various names in states surrounding Michigan. Which

is

> how we ended up with Dr. Hostoffer in Cleveland. Dr. Hostoffer reviewed

the

> pages and pages of clinical notes, ER notes, hospital admissions, and lab

> results. He told my husband and I that Mark clearly had CVID (so he

thought

> at the time) but wanted to return the pneumovax as he had NEVER seen

titers

> come back as -0- as the number. Many physicians will call a patient a

> non-responder if the level too low and considered non-protective. Not

only

> was Mark a non-responder but he did not muster any response not even a

> non-protective low number. So, off to the lab in Cleveland. Several

weeks

> later we were phoned and told that 12 out of 12 pneumococcal titers were

> absent and we were asked to return to Cleveland as soon as we could and

IVIG

> was started immediately (I had to get out of denial at this point and face

my

> fears). HIB titers were drawn (after a supportive dose) and were 100%

> absent. Mark's health continued to decline and the faces of autoimmune

> diseases were starting to show up, something that usually does not happen

in

> CVID until the 2nd or 3rd decade of life. Dr. Hostoffer told us of one

other

> patient that he had that made -0- response and that this boy was similar

to

> Mark. He asked that we allow Mark to be admitted in to RB & C for week

or

> two and that we also get more lab work down that day. On the day of

> admission (which was the following week) we were told by Dr.Hostoffer that

> Mark had no b-cells, that all his b-cells were Cd5+ positive cells,

premature

> b-cells that do not offer antibody protection. Thus, the discovery of the

> Cd5-Cd19 PID, a combinatin B-cell T-cell immune deficiency with a second

> componet of Cd5+ cells that are destructive. The rest is in the medical

> journals. Mark can measures immunoglobulins, but they are all Cd5+

bcells,

> he has less then 2% circulating b-cells which is basically nothing. He

makes

> absolutely no response to pneumococcal, HIB, meningocaccol, very low to

DPT

> and very low to polio. He does not make " in-adequate

responses/non-responses

> but rather no responses (0) what so ever (again, I have to make this clear

if

> any one ever suspects they may have this) That is why this PID is twofold

1).

> No B-cells and 2.) a cell that is destructive causing autoantibodies,

> autoimmune disease and malignancies.

> IVIG is going to be lifelong for Mark. Life expectancy is unknown but we

are

> told that it will be less then average from what they know at this time.

> Quality of life will be poor. The Cd5-Cd19 PID is a moderate to severe

PID

> with many, many unanswered questions. I encourage you to ask many, many

> questions and do not be afraid to do so. I realize that ours is an

> exceptional story in as much as I went for many opinions for my own fear

of

> IVIG use ( I had to be certain) even afer being told by one physician

after

> another that Mark needed it. Our first immunologist works together with

Dr.

> Hostoffer and is known to be conservative in the use of IVIG, that helped

to

> ease some of my fears as I do realize that Mark survives because of it the

> use of IVIG. It is important to look at the symptoms and if the numbers

and

> symptoms do not add up I would then recommend inquiring about an antibody

> study to see if the immunglobulins/b-cells are working. Make sure that

you

> go to a good immunologist. Over the years I have heard stories of people

> receiving a diagnosis and wanting their child to be placed on IVIG right

> away. Make sure your child would need it. Many patients have terrible

> numbers and no symptoms and then there are those that are the opposite.

Good

> example is who by physician standards presents worse then many

PID/CF

> patients and is not a PID or CF. ENT and pulmonary wanted him on IVIG a

> long time ago and our immunologist said no way...we agreed.

Unfortunately,

> now does have a secondary immune deficiency as a result of being

steroid

> dependent so we keep a close eye on him. Keep an open mind and remember

you

> can always go for a second or third opininon. Good luck to you in your

> pursuit.

>

> Autumn mom to Mark Cd5-Cd19 PID, achalasia, GERD, ASA, A1A

>

> ---------------------------