Re: IVIG therapy and Immunodeficiency (Gerri-Anne and Dawn)

[Guest guest]

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

[Guest guest]

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

[Guest guest]

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

[Guest guest]

Laurel,

Thanks very much for the answer but now I am more concerned.

's doc said it would take about 3 hrs. the first time and then

it could go faster the next. I am very concerned about a reaction.

She has blood transfusion reactions. When she is transfused (every 3

wks) she has a meningitis like reaction starting 24 hrs. post.

Sometimes it is bad sometimes not too bad. This scares me. I do not

want her to suffer. OUr goal with her is comfort. If we can treat

her and have her remain comfortable then we do it. I am afraid if

she has a blood trans. reaction wouldn't it be likely that she would

react the same to the IVIG? It is a blood product right?

Thanks for the help.

Dawn