Immune deficiency... again & Questions

[browneyesmom]

My daughter was diagnosed with an immune deficiency in October 2010. At that time, her labs were:

 

Total IgG: 588 (normal range 698-1560)

IgA: 44 (normal range 53-204)

 

Some of you might remember that she was initially given a low dose of Immune globulin... and got a LOT worse. We had 5 calls to 911 in a span of 2 weeks, then she was finally admitted to the hospital and had her first high dose infusion Christmas week 2010. She woke up Christmas morning (part two infused during the night, Christmas Eve) and said she felt better than she had in a long time - more like herself. When I asked her how long since she's felt like that, she responded that it had been so long, she did not remember. She still had a long way to go, but episodes that had been lasting up to 4 hours (3 hours aggressive rage - attacking me, up to 30 min thrashing and up to 30 min limp - seemed unconscious... then sometimes unable to speak for a while), dropped immediately to 20-30 minutes and eventually, vanished.

 

She had six more high dose infusions, last one was early June 2011. We stopped them over the summer to see if her numbers were improving. Two months later (August), we checked them and they were in the normal range for IgG!!! Yippeeee!!! So, no IVIG since June. After being on homebound ed most of last year, she returned to school with a late start and modified schedule and a confidence I had not seen in her in years.

 

BUT... sigh... there's always one, isn't there? Early fall, she had a haemophilus parainfluenzae infection in her throat. By mid-October, she had another strep infection. She improved slightly after abx, but not a lot - she was also hospitalized twice in the fall for migraines, which didn't help - she's back on homebound, trying to catch up on schoolwork, but it's not going well right now. She has been getting worse in recent weeks. Dr. L ran labs on her again - no infectious triggers, although she is treating aggressively for strep, just to be sure we're not missing it - dd never has elevated titers and won't let anyone get a decent swab of her throat right now b/c it hurts too much... but her hx of strep is very strong. The following are her Immunology lab results:

 

Her IgA is 33 (normal range is 58-358), but we're always told it's not so low that she can't have IVIG.

 

Her total IgG is 551 (normal range is 759-1549)

 

Subclass 1 is 335 (normal range is 456-952)

Subclass 2 is 151 (normal range is 147-493)

Subclass 3 is 31 (normal range is 12-179)

Subclass 4 is 40 (normal range is 1-168)

 

Obviously, she needs IVIG again - her doctors all agree on this and we'll be setting that up this coming week.

 

Her total IgG is lower than it was in October 2010, when they first found the deficiency. I'm concerned she may be showing signs of a long-term deficiency and what this might mean for her future as far as infusions, insurance, etc.

 

Does anyone else have this problem with their child not being able to maintain IgG in the normal range? Are you doing lifetime Ig infusions and if so, does it have to remain high dose once the PANDAS is under control or can it be safely dropped to just maintain levels at some point?

[thenmama]

Hi Denise,

 

My ds was recently dx'd with an immune deficiency. He also has low IgG in subclass 1, too. Wonder if there are any trends with the specific subclass that our PANS kids are lowest in? Since the majority of IgG is made up of the IgG1 subclass, frequently those with low subclass 1 are low overall (or may eek up to low normal if another subclass is high-- this has happened to my ds with a very high subclass 4) .

 

IgG 1 usually reaches its full, or adult, level in early childhood-- our immuno said that by 7 (ds's age) it should be there so deficiencies would not be developmental (I'm pretty sure it was subclass 4 that she said comes later). My ds is also deficient in IgM-- his levels fluctuate between entirely absent and barely there but detectable and that also should be at adult levels by now. He also has specific antibody deficiencies and she said at 7 he should hit protective levels from vax.

 

Now, we know there's a high likelihood that ds's deficiencies will persist b/c when my dh was having blood drawn at his physical we had the doc run all of the usual PANS tests and b/c he gets slammed by illnesses and we knew about ds's deficiencies we had them run his panel and he's IgM deficient, too. They didn't run labs for subclasses or SAD so I don't know if those are low, too. Anyway, the immuno said that ds is past the age he should have adult levels AND his dad has some of the same deficiency so his would be considered likely to persist and he may need ongoing IVIG treatments b/c we need to give him passive immunity.

 

The reason I bring up the likelihood of persistence w/ ds is not to worry you. I found out through all of this that insurance companies often factor the likelihood that the child's deficiency will persist over time into the coverage decisions. In other words, it makes a stronger case for coverage if it seems like the deficiency is not developmental or due to some acute causal factor. So our immuno said the fact that dh also has an immune deficiency would make a strong case when requesting coverage for ongoing treatments.

 

Our docs said it is possible that down the road, once his PANS is treated and he's doing well, he may be able to try going down from the HDIVIG to the usual Immune Deficiency protocol as long as it continued to work for him. Two of the options were the regular dose IVIG and depending on his circumstances there's also the subcutaneous.

 

 

So sorry your dd is having such a rough year, especially after she'd just gotten back in her groove and was doing so well! Please let her know my dd says hello and sends her best wishes.

 

We're thinking of you all!

[browneyesmom]

 

Our docs said it is possible that down the road, once his PANS is treated and he's doing well, he may be able to try going down from the HDIVIG to the usual Immune Deficiency protocol as long as it continued to work for him. Two of the options were the regular dose IVIG and depending on his circumstances there's also the subcutaneous.

 

So sorry your dd is having such a rough year, especially after she'd just gotten back in her groove and was doing so well! Please let her know my dd says hello and sends her best wishes.

 

We're thinking of you all!

 

This is exactly what I plan to ask our Immunologist on Monday morning when I meet with him. Since this is the second time she has dropped like this, I suspect it is going to persist also and although I'm dead tired, it's what I was trying to ask in my initial post. You answered it here - I want to know if I can stabilize her with the HD IVIG, then drop her to the weekly subQ Hizentra or whatever without compromising the PANDAS issues.

 

The only thing is that when we did that last year, she got soooo sick and I think the LD IVIG was the problem. If she is stable first though, I theorize, maybe it would be ok. My understanding from last year is that the weekly subQ Hizentra is the same as getting a monthly 0.4 mg/kg dosage. Of course, I'll be verifying that also.

 

Thanks for the well wishes - we will get her back on track ... just working out how to get there. She returns the hello and best wishes... maybe we can connect again once things calm down a bit. Thanks so much for your response.