High-dose IVIG frequency

[mdmom]

We are preparing for DS15 to get his first IVIG treatment after the holidays. Doc has ordered 2mg/kg (PANDAS dose). He also has a primary immune deficiency. I believe doc wants to do high dose every 4 weeks. Is this too frequent? Mind you, docs speculate that he has had PANDAS since age 4 and was only diagnosed at age 13. Aggressive treatment for infections began 20 months ago, so we seem to have those pretty well under control (he's been on IV abx, IV antifungals for 10 months). He is not currently in a PANDAS flare.

 

Thoughts?

[peglem]

My daughter gets 1.5g/kg every 3 weeks. We can tell when she's due for another one. It does seem to help her get sick less, but has not ended flares. Monday after Christmas we will be trying low dose methotrexate to decrease her own immune response to infection. I think if you're not getting good immune system control with what you're doing now, it's worth a try. You don't have to continue any treatment that is not working.

[Jtsmama]

My son also has Pidd, is doing Scig weekly, Started Hdivig monthly, just had his 6th dose last week. We have to do both because of complications, but it works for him. A whole new kid!! Definitely a miracle!!

[mdmom]

Jtsmom -

What dose of High dose IVIG is your son getting? And why do you have to do the SCIg too? you can PM me if you'd like.

 

Thanks in advance.

[mdmom]

Bumping up hoping for more responses. Thx.

[Hayley]

I'm sorry I didn't respond to this sooner! I was away and for some reason couldn't log in to reply on my iphone!

 

My DD is younger and likely has CVID which I'm not sure is exactly the same as PID, but here's what I know based on our case if it's helpful...

 

My DD7 had HD ivig 2/kg December 6th and 7th. We have approval to do monthly, but the plan as of now is to do it this one time and wait and see. Her IgG/IgA numbers are indicative of a CVID, but her clinical presentation doesn't support it (she's not been a kid who is always getting sick, she seems to fight illness ok, etc.). When Dr. L and Dr. T first saw the numbers, both said, it's CVID and she is a candidate for montly lower dose IVIG. At the time, she was a couple of weeks into her abx protocol and was showing improvement (and not sick with illness) so we decided to wait and retest the immunoglobulin numbers in a couple of months. She ended up getting probably to 80% of her old self on the abx for about three weeks then got sick and her PANDAS symptoms flared at which point Dr. L changed her recommendation to a single time high dose approach -- focusing on the PANDAS, not the CVID.

 

Meanwhile, we went to an immunologist who said based on her numbers, he would NOT treat her with montly IVIG at this point, but would wait and see, watch the numbers and her health and if the numbers don't go up, maybe he'd do it down the road (I think at a lower dose every 3-4 weeks). He felt she might outgrow it and it did not make sense to jump into that protocol at thsi point. Dr. L (our treating PANDAS doc who ordered the IVIG) isn't 100% sure that she really has PID and thinks that (and I hope I'm not misquoting here, but this is how I understand it) that it's possible her numbers are skewed as a result of the PANDAS. Dr. T agreed that he would do the IVIG once at this point and see how things progress.

 

All of this said, when we first got the bloodwork that identified the low immunoglobulin numbers, in searching for an immunologist, I came into contact with the doctors from NIH's Primary Immune Deficiency Clinic -- not a trial, but a clinic where they are looking at PID patients; they don't treat, just review the case, run labs and work with your doctors around a plan of action for addressing the PID and any other related stuff. DD will be seen by the NIH folks later this month. They are NOT affiliated with the NIH PANDAS study and I'm not exactly clear what experience any or all of the doctors have with PANDAS there (or if they'll bring in Swedo's folks to consult), but I'm hoping that having DD seen there might help shed some light on the PID-PANDAS link. I'll be curious to see if they will encourage more frequent IVIG. I will report back here with any info I think might be helpful to the group.

 

Good luck, MDMom! I hope the IVIG is helpful for your son!

Edited January 1, 2013 by Hayley

[Jtsmama]

Sorry it took so long! I don't Ching on acn much. My son does scig weekly did his immunodeficiency because he gets aseptic meningitis from ivig. We do the scig weekly to keep his base levels higher, fewer reactions. He doesn't have the dips & spikes. When we started high dose Ivig for pandas, we were already doing it weekly felt that was the best option. He gets 1g m/kg every 4 weeks of Privigen

[Jtsmama]

Sorry it took so long! I don't Ching on acn much. My son does scig weekly did his immunodeficiency because he gets aseptic meningitis from ivig. We do the scig weekly to keep his base levels higher, fewer reactions. He doesn't have the dips & spikes. When we started high dose Ivig for pandas, we were already doing it weekly felt that was the best option. He gets 1g m/kg every 4 weeks of Privigen

[Jtsmama]

@Hayley cvid is a type of primary immunodeficiency. There are over 150. Cvid is combined variable, means there is more than one category involved.

[Hayley]

Thanks, Jtsmama, since my last post I have figured that out! For what it's worth, there is a Primary Immuno Deficiency clinic at NIH -- it's a clinic, not a study. They are looking at the big picture for people with all types of PID and of all ages and they work with your doctors to advise on treatment plans. My daughter is currently being seen in the clinic and we have someone from the PANDAS study who consults on the case. So far, they haven't touched the treatment plan recommended by Dr. L because it seems to be working. They are, however, tracking my daughter closely and may advise to repeat the IVIG if she doesn't hold steady over time. The doctors have been wonderful and it will be interesting to see how things pan out. I understand that despite a high incidence of autoimmune disorders among PID clinic patients, my daughter's case is the first PANDAS case referred to the clinic (and the first contact between the PID clinic and the PANDAS study -- the PANDAS study folks say they haven't had immunodeficient kids screened for the study which I thought was surprising). HOWEVER, since my daughter entered the study, I believe another case popped up that the two departments are collaborating on. You have to apply and be accepted in the study and I'm not sure if they are interested or willing to take any other PANDAS kids right now, but if anyone thinks they might be a candidate, there is info on the clinic here: http://www.niaid.nih.gov/topics/immunedeficiency/pidclinic/Pages/Default.aspx. Feel free to pm me if you are interested in more info.

Edited March 19, 2013 by Hayley

[PowPow]

What kind of igg levels are considered significantly low?

My child total was 691 (14years old) and igg subclass 4 was 5.. I think these are not a big deal, but could they be?

[Hayley]

What kind of igg levels are considered significantly low?

My child total was 691 (14years old) and igg subclass 4 was 5.. I think these are not a big deal, but could they be?

 

Prior to IVIG, my DD7's IGG level was 394. The paperwork I have from the lab has the range at 572-1474. I don't know if that is age specific or not. I don't think her subclasses were ever tested.

[twoplusone]

We do HD IVIG every 21 days....every case is different. You can definitely tell when his infusion is wearing off starting at about 2 1/2 weeks.