Re: IVIG therapy and Immunodeficiency (Laurel)

[Guest guest]

Laurel,

Thank you for the information. We are just in the beginning stages

of determining if he has an immune deficiency. The yeast in the

esophogus is the tip off that something is wrong. We will be having

another biopsy next month. After reading your response I have some

more questions. I have very little knowledge about the immune

system. Is IVIG the only thing they can do to help the immune

system function properly? At what point do they determine the

immune system is not working efficiently enough and you have to

start IVIG? And what exactly does IVIG stand for?

Thanks for your help.

Geri-Anne and Wyatt, Complex I

> Gerri Anne and Dawn,

>

> Rather than explain in two seperate emails I figured I'd combine

responses

> since your questions are related.

>

> There is a group of us on the list whose children have an

immunodeficiency

> and mito. Our doc feels that it is definitely related. son

has a B Cell

> deficiency. He at one point had both a T cell and B cell but since

we removed his

> port, it is just a B Cell. When they test his various IGG

subclasses, he is

> deficient in 4 of the 5 subclasses. The only one he is not

deficient in is

> IGG-A.

>

> receives IVIG every 21 days for the deficiency. He receives

50 grams of

> Gammimune N, which is a low concentration brand. has had bad

reactions

> in the past to IVIG - including the mennig. reaction. We received

a lot of

> help from another Mom on the list which our doc has used to meet

's needs.

> His infusions have to be inpatient because of his history of

severe reactions.

>

> Typically, infusions can be done over 4 - 8 hours at home or in

the hospital

> or infusion center. In our case, his infusion takes 24 hours

because his heart

> does not tolerate the fluids and also has reactions to the IVIG if

it is not

> done slowly.

>

> 's reactions have included the following: severe drops in

blood

> preassure; increased bleeding and brusing (not sure why although

he has Von

> Willebrands Type 1 and a Platelet Aggregation Defect); Rash; High

temps; menningitis

> like symptoms and flu like symptoms.

>

> gets medicated with Tylenol, Solumedrol and Benedryl pre-

IVIG, post

> IVIG and half way thru. His infusions start at 2 cc per hour, then

4 cc per hour,

> then 8 per hour and finally 16 cc an hour. If we go any higher his

BP

> crashes.

>

> Some people have no problem tolerating the IVIG and only get flu

like

> symptoms and headaches.

>

> All this aside, when comparing our life before IVIG and now, I

wouldn't trade

> it for anything. 's motility has improved because his body is

now using

> the natural bacteria in his gut properly. His overall health has

improved and

> this year for the first time in three years, he has made it

through the winter

> without being placed on home instruction. Before the IVIG, he

would get sick

> in November and be too sick to return until March or April.

>

> His B cell deficiency is still there and he still gets sick often,

but the

> severity is less than before.

>

> I hope this helps. Good luck to both of you.

>

> Laurel