immunodeficiency

[peglem]

What are your experiences w/ IVIG when dealing w/ both PANDAS and immune deficiency? I know (according to Dr.K) high dose IVIG is supposed to reboot the immune system, so that the patient's own immune system stops making the antibody errors that cause PANDAS symptoms. And yes, I do know that hasn't exactly played out in all cases.

 

But, does immune deficiency make a difference in IVIG recovery and need for more than one?

 

Here's my experience so far: March '10, 1st IVIG, but very low dose- 0.47g/kg, then another of the same dosage 3 weeks later. These were prescribed on the basis of immune deficiency. They made PANDAS worse.

Mid May '10, we did the 1st high dose IVIG-1.5g/kg, saw some improvement w/in 2 weeks, then did another HD in June. She is doing very well now with her worst PANDAS symptoms (rage and aggression) and did not have any difficulties w/ recovery from the 2nd. Right now, she is the healthiest that she has ever been- I can't remember her ever being this healthy.

 

Her pediatrician said he thinks her immune system is "crap" and thinks we should continue monthly IVIG, for at least a year. I guess I'm willing to do that, but really would like to hear about the experiences of others in the same position. Money isn't an issue here because it is covered by insurance.(before you get jealous, consider the fact that she qualifies for state secondary insurance based on the fact that she is not expected to ever be able to live independently) And I wonder at what point do we back off of IVIG and see what her own immune system can do? Also, she is IgA and IgE deficient, which is not addressed by IVIG.

[melanie]

Hi Peg

 

Well you kindof know our deal.1g every 21 days immune deficient dx.We are looking at 2 more years and aside from some anger issues dannys doing ok,Although the past week hes been OFF...Angry ,he started a new school today so maybe hes got that on his mind??

[laurenjohnsonsmom]

I'm going to do my best at adding to this thread as Lauren has had 2 ivig treatments and will be having her third in early August. Most of these things are way over my head as I am not the "braniac" like Buster (Buster-that's a compliment)!

 

Lauren's IGg levels are borderline "low normal/high abnormal" and the way that Dr. B explained it to me (and the reason why we together, decided to do continuing IVIG) is that Immunodeficiency (or immune deficiency) is a state in which the immune system's ability to fight infectious disease is compromised or entirely absent. Most cases of immunodeficiency are acquired ("secondary") but some people are born with defects in the immune system, or primary immunodeficiency. A person who has an immunodeficiency of any kind is said to be immunocompromised. An immunocompromised person may be particularly vulnerable to opportunistic infections, in addition to normal infections that could affect everyone.

 

with the above taking into consideration, some people have low total IGg levels but for some reason are NOT immunocompromised (have chronic repeated infections) BUT some people who have low normal/abnormal IGg levels DO suffer from chronic infections (more than 1 a quater/4x a year) as in Laurens case. Lauren was sick constantly the entire year leading up to her sneezing manifestation last fall (she missed 26 school days in the 5th grade and manifested in the beginning of 6th grade). She succumbed to strep infections and other viruses/bacteria throughout her childhood (more than you care to count) as early as 21 months of age when she had scarlet fever. Lauren continues to have repeated infections including strep three weeks after both IVIG’s 1&2. As Dr. B puts it, “having the good antibodies in her does not keep infection away but limits it severity”. She might “slip a foot off the wagon” but without the IVIG/antibodies she probably would have “fell completely off the wagon”. Are we doing this the right way? Who knows? Is every child and PANDAS case different? Absolutely!

 

Peglem and Melanie, I’m sure there are specific differences in how your children are immunocompromised. Are there IGg levels low? Do they have chronic infection?

 

Hoping this came out right.

 

This is one f the questions Dr. B is going to address when we add the Q&A to the PRN website (he was supposedto have that back to me by end of June, he's a busy man these days). He says it will be done soon so hopefully it will be more detailed and "doctorly" (is that a word) then my two cents!

 

Lynn

 

www.pandasresourcenetwork.org

Educate, Communicate, Cure

 

 

What are your experiences w/ IVIG when dealing w/ both PANDAS and immune deficiency? I know (according to Dr.K) high dose IVIG is supposed to reboot the immune system, so that the patient's own immune system stops making the antibody errors that cause PANDAS symptoms. And yes, I do know that hasn't exactly played out in all cases.

 

But, does immune deficiency make a difference in IVIG recovery and need for more than one?

 

Here's my experience so far: March '10, 1st IVIG, but very low dose- 0.47g/kg, then another of the same dosage 3 weeks later. These were prescribed on the basis of immune deficiency. They made PANDAS worse.

Mid May '10, we did the 1st high dose IVIG-1.5g/kg, saw some improvement w/in 2 weeks, then did another HD in June. She is doing very well now with her worst PANDAS symptoms (rage and aggression) and did not have any difficulties w/ recovery from the 2nd. Right now, she is the healthiest that she has ever been- I can't remember her ever being this healthy.

 

Her pediatrician said he thinks her immune system is "crap" and thinks we should continue monthly IVIG, for at least a year. I guess I'm willing to do that, but really would like to hear about the experiences of others in the same position. Money isn't an issue here because it is covered by insurance.(before you get jealous, consider the fact that she qualifies for state secondary insurance based on the fact that she is not expected to ever be able to live independently) And I wonder at what point do we back off of IVIG and see what her own immune system can do? Also, she is IgA and IgE deficient, which is not addressed by IVIG.

[peglem]

Allie had chronic infections (symptomatic ones) from infancy to say, 10yo or so...but, w/ the autism dx doctors dismissed it as allergies or part of her autism. They occasionally gave her a course of abx. At age 10 we got a physician who was willing to swab for strep and investigate medically. Once he found the strep, she was pretty much on abx all the time- but only when she tested + for strep (which was all the time). By then, she was really really bad off- lost what speech she had managed to develop between severe exacerbations....oh, so many things she used to be able to do but couldn't by that time. She was a chronic strep carrier, even after T&A.

 

Now- She does not hold protective immunity to pneumovax (and it took 2 vaxes to see a rise in those titers), She falls far below normal range for both IgA and IgE, total IgG is well below normal range, w/ IgG4 being particularly low. I'm not clear on the difference between actual infection and carriage, other than carriage is asymptomatic, but is that because the symptoms are a sign of immune response and the immune response is poor in carriage?

[momto2pandas]

We are in this boat as well. My younger child has very low total IgG, abnormally low IgG3, and poor response to prevnar (1/7), so he has CVID. My older has low-normal total IgG, abnormally low IgG3, and poor response to prevnar (2/7). The younger has basically been sick with strep and other sinus infections since he was a baby. My older was reasonably healthy until last year, at which time he became continuously sick. Both are now on monthly IVIG, so we are very interested in this question.

 

Their first dose was 1.5 g/kg. We had some turning back of the pages for a couple of weeks, but then bingo, sinus infections apparently totally gone, and PANDAS pretty much 100% gone. We were supposed to get 0.75 g/kg 4 weeks later, but for administrative reasons, ended up getting it 8 weeks later, by which time we were beginning to see more sinus symptoms and PANDAS symptoms again - but really just a little. After the 0.75 g/kg dose the older child sailed along 100% great again; the younger was 98% great - but just shy of where he had been after the higher dose (bit of speech dysfluencies still). They get their next 0.75 dose tomorrow, and I am anxious. Just in the last 3 days or so we have begun to see some PANDAS moodiness again (no tics or OCD, just "touchiness") - noticeable but quite minor - and today ds4 was writing in mirror image again suddenly. I am hoping that it goes away again after the 0.75. We'll see. I have a suspicion based on the last couple of rounds that they are going to do great for the first 3 weeks between treatments, but then maybe start to slide just as we get ready for the next dose - since their levels will be very low again at that point. Their immunologist wants to move them to weekly subcutaneous IG to help keep things steadier. Not sure what to think about this, as it would essentially mean more lower doses. Seems like a good idea from an immune standpoint but I'm not sure about PANDAS.

 

Anyway, my guess just based on what we've seen so far is that we're probably a ways from being able to go off of IVIG - if only for reasons of immunocompetence. If their immune systems had truly been "rebooted" to a functional state, I don't think we would see this deterioration right before each new dose. Their immunologist wants to treat for a year and then see how they do.

 

He also told me that immune deficiency with poor prevnar response tends to have a developmental component and that kids often grow out of it. That's why he feels that we need to stop each year to see if their immune systems have developed the capacity to function on their own. He did say that by the 1 year timepoint kids are generally so much better that the parents really don't want to stop, but that it's important to give their bodies a chance to try to function on their own.

[Jtsmama]

My 10 yo son started IVIG 2 yrs ago for a PIDD, functional antibody deficiency in his case. Has helped alot with the immune problems. No hospitalizations other than belly bugs for dehydration. Has grown alot! We saw Dr Murphy today and am trying to get her to order the high dose ivig recommended by Dr K via email. He only receives 10g/mo Gamunex for the PIDD. We had the issues of getting sick when infusions were 4wks apart, then tried 3wks, better but not great, now he does them every 2 wks. He has had drip rate/headache problems from the first time, so have not been able to increase his dosage. I would love to talk to any parents that have tried the IVIG for PANDAS. The abx are not working at this point, so don't really know what to do with him. The Immuno doesn't know where to start, and the Neuro is not treating him for the PANDAS any longer.

[peglem]

My 10 yo son started IVIG 2 yrs ago for a PIDD, functional antibody deficiency in his case. Has helped alot with the immune problems. No hospitalizations other than belly bugs for dehydration. Has grown alot! We saw Dr Murphy today and am trying to get her to order the high dose ivig recommended by Dr K via email. He only receives 10g/mo Gamunex for the PIDD. We had the issues of getting sick when infusions were 4wks apart, then tried 3wks, better but not great, now he does them every 2 wks. He has had drip rate/headache problems from the first time, so have not been able to increase his dosage. I would love to talk to any parents that have tried the IVIG for PANDAS. The abx are not working at this point, so don't really know what to do with him. The Immuno doesn't know where to start, and the Neuro is not treating him for the PANDAS any longer.

You would not have to increase the drip rate for higher dose- just the amount of time it takes for the procedure. Lower dose IVIG is pro inflammatory, higher dose is anti-inflammatory. What abx is not working for him? Have you tried higher doses of either zith or augmentin XR? What helped my daughter alot when she had exacerbations while on full strength (treatment dose) zith, was to switch to a 2 week course of rifampin+augmentin to clean out intracellular bacteria, then go back to the full strength zith.

[Jtsmama]

You would not have to increase the drip rate for higher dose- just the amount of time it takes for the procedure. Lower dose IVIG is pro inflammatory, higher dose is anti-inflammatory. What abx is not working for him? Have you tried higher doses of either zith or augmentin XR? What helped my daughter alot when she had exacerbations while on full strength (treatment dose) zith, was to switch to a 2 week course of rifampin+augmentin to clean out intracellular bacteria, then go back to the full strength zith.

 

 

He has built up a tolerance to Augmentin, and Zith from using for years. Was on Augmentin 14 days for an ear infection, and didn't touch it. We have used Omnicef for the PANDAS for the last 3 years. He was on low dose Omnicef then taking off after a few months. Then put on a high dose of Omnicef, but was on Abilify at the time and that med seemed to make the PANDAS worse. I asked Dr Murphy about using Pcn, Zith or Augm but she said they are not as broad spectrum and build up a tolerance quicker. The issue with the infusion is he would have to be admitted into the hospital for the high dose IVIG if he were to get it. I did ask the Inf Dis specialist about doing 2 weeks on one abx then switch to another then back. I have read that the kids get the High Dose, then what? they go back to their normal dose of IVIG if being treated for a PID, or what is the next step?