browneyesmom

Thanks for all the input!!

Our Ped suggested today that we do some alternative testing for heavy metals, food sensitivities, etc. I mentioned wanting to check for MTHFR also. DD12 has Strep related PANDAS and an Immune Deficiency. She has been tested multiple times for Myco P, Lyme & related (Igenex & Western Blot)... all of these are always negative. I'd appreciate input on the list of what others have checked, please? TIA!

My daughter is in her third severe exacerbation. She has an immune deficiency also, which was initially found in October 2010. That led us to IVIG; she's had 7 HD IVIG; after a break since June 2011, the most recent one was last week. Her levels dropped lower than they were in October 2010 when tested again recently, so we're back to HD IVIG as we work to stabilize her and also clear the endless strep infections... again. Our local Immuno is suggesting and I anticipate we may have to go to long term low dose once she is stable, but I'm not sure it is advisable to do the low dose (even after stable on high dose)... I need more input on that one and will be discussing with other members of her medical team. Does anyone else have an immune deficiency in the mix and if so, how are you handling it? Is it working? Has anyone successfully done LD IVIG once their child is stable on HD IVIG? TIA!

Just wanted to add that the immune deficiency is what we discussed recently as my daughter has one and that is when the Neuro-Immuno at CHKD offered to order it for her and monitor her as we determine how many she needs. I anticipate we may have to go to long term low dose once she is stable, but I'm not sure it is advisable to do the low dose (even after stable on high dose)... I need more input on that one.

I can't speak for Richmond as from what I understand, people in that area are having trouble finding local help, but try CHKD in Norfolk. There is a new Neuro-Immunologist there with an interest in PANDAS. They definitely run Gammunex IVIG there; it's where my daughter had her first high dose inpatient & that's what she got. The Neuro-Immuno offered to order it for her again recently. I think I lost your email when my computer crashed, but it might be in my cell. Feel free to email or call me if you need a name and phone number. I'd be interested to know more about your experience with Critical Care as that is who we have now. I'm also considering switching to CHKD or Georgetown... I'll know more after our next appt with Dr. L.

You know though... I wonder if we might be better off having it deleted, if Sandy Georgia is going to continue to control it. There are far better links out there now, where people can gather information and Wikipedia is NOT known for being a reliable source... as illustrated by this issue.

I too, tried briefly to take on the Wikipedia page and soon tangled with Sandy Georgia. Thank goodness for Buster, for working on this so diligently!! I had to pull back from it as dd12 is still struggling with the immune deficiency. I'm going to look to see if I can figure out how to rate the page!

What a great story - thank you so much for sharing it!

It updated last night!

Labs I often see run include: Strep throat swab - rapid and/or culture ASO & Anti-DNase B titers (these are strep titers), but keep in mind that not all kids elevate titers - for example, mine never has elevated titers, even with a raging strep infection. Streptozyme Mycoplasma Pneumoniae titers IgG and IgM Tick-borne infection titers, including Lyme, Babesia, Ehrlichiosis, Bartonella... Quantitative Immunoglobulins (including IgA, IgE, IgG & IgM) with subclasses on IgG Pneumococcal antibodies - 14 serotypes Vitamin D - many of our kids are deficient Ferritin Ceruloplasmin CBC with differential and platelets BMP is sometimes run as well as urine porphyrins Research literature that might help: From Research Subgroup to Clinical Syndrome: Modifying the PANDAS criteria to Describe PANS (Pediatric Acute-Onset Neuropsychiatric Syndrome). Swedo, SE, et al, 2012. Currently available online here: http://pandasnetwork.org/wp-content/uploads/2012/02/2161-0665-2-113.pdf Neurocognitive functioning in youth with pediatric autoimmune neuropsychiatric disorders associated with streptococcus. Lewin AB, et al. (September, 2011) Clinical Factors Associated with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections. Murphy, TK, et al (2011) The Immunobiology of Tourette's Disorder, Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus and Related Infections: A Way Forward. Murphy, et al (2010) Maternal history of autoimmune disease in children presenting with tics and/or obsessive–compulsive disorder. Murphy, et al (2010) Passive transfer of streptococcus-induced antibodies reproduces behavioral disturbances in a mouse model of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection. Yaddanapudi, K et al (2009) Antibody-mediated neuronal cell signaling in behavior and movement disorders. Kirvan CA, et al J Neuroimmunol. 179(1-2):173-9. 2006; Epub 2006. Streptococcal mimicry and antibody-mediated cell signaling in the pathogenesis of Sydenham's Chorea. Kirvan, CA, et al (2006) Therapeutic Plasma Exchange and Intravenous Immunoglobulin for Obsessive Compulsive Disorder and Tic Disorders in Childhood. Perlmutter, SJ, et al (1999) Note: This IVIG study is likely the best we have for supporting it's use until NIMH publishes the results of their current study; it's available online here: http://intramural.nimh.nih.gov/pdn/pubs/pub-5.pdf

Thanks bigmighty - this was her 7th HD IVIG and I completely agree with you. We start hydrating heavily 3 days prior and continue through 3 days after. I don't yet know what caused her symptoms this particular time, but it wasn't lack of hydration. We'll be establishing a protocol for future infusions.

Thanks Buster!

In this review, they state: "‎"Our extensive review of the literature has elucidated the mechanism with which a tick bite or sore throat may develop into neuropsychiatric disorders. TLRs (Toll-like receptors) in haplotype-variable individuals may play a role in multisystemic disease formation," states Dr. Rhee." Has anyone seen this: http://www.prnewswire.com/news-releases/researchers-publish-long-awaited-lyme-disease-and-pandas-review-despite-controversies-140052803.html

Thanks T!! This is part of why I brought her to the ER - I didn't like that it was so much worse on rising. I'm exhausted and going to sleep, but the short version is they gave her a 72-hour steroid in the ER and within about 10 min, her headache (which had been improving a little along with fever reducing and nausea improving) dropped from about a 6-7 on 1-10 pain scale to a 3-4. She was also likely exposed to the GI virus through my ex over the weekend, so it was a hard call this time, but really glad I took her in to ER tonight. We're all going to sleep!

Thanks... still waiting for a call back... she got a little better for a while.

Not a doc, but have seen a good bit of labs over the last few years that we and others have run. Immunology labs that are often run are Quantitative Immunoglobulins with subclasses and Pneumococcal Serotypes. Have you also ruled out known potential infectious triggers? Labs I've seen run include: Strep throat swab - rapid and/or culture ASO & Anti-DNase B titers (these are strep titers), but keep in mind that not all kids elevate titers - for example, mine never has elevated titers, even with a raging strep infection. Streptozyme Mycoplasma Pneumoniae titers IgG and IgM Tick-borne infection titers, including Lyme, Babesia, Ehrlichiosis, Bartonella... Quantitative Immunoglobulins (including IgA, IgE, IgG & IgM) with subclasses on IgG Pneumococcal antibodies - 14 serotypes Vitamin D - many of our kids are deficient Ferritin Ceruloplasmin CBC with differential and platelets BMP is sometimes run as well as urine porphyrins Often, we see docs running these as well - to rule out other conditions: EEG MRI Testing for metals

My dd 12 had her 7th IVIG Mon & Tues.... was the best she has ever done. Premedicated with 500 mg Acetaminophen and 25 mg Diphenhydramine. They started her rate at 25 mL/hour, increasing by 25 every 15 min until they got to 125, where she remained for the rest of the infusion. She was awake, playing a game online, reading or chatting the entire time. I made notes for next time.

My dd12 has had several HD IVIG infusions. She is immunodeficient on top of having PANDAS. She was off them for a while (last one was early June) to see if her immune system would stabilize, but labs recently showed numbers lower than October 2010, when it was found - for IgA & IgG. Interestingly, her IgM is not dropping - not sure what the deal is there. In any case, back to HD IVIG (Gammunex 2g/kg - she's had several times before), which she had Monday & Tuesday. She ran better than she ever has both days - did great! However, by last night (day 2 of infusion), had a severe headache (she's also prone to migraines) and nausea... called on call doc assuming it was side effect of the IVIG infusion and gave meds indicated for pain/migraine. By 2 am, she was awake with fever around 100.5, vomiting and severe headache that is worse when she rises - rating 6-7 lying down, 8-9 on rising. I know there is a GI bug going around our area right now with these symptoms as every local doc office I am calling is telling me they are inundated with it (and having the immune deficiency, she tends to get everything going around); yet, they are also symptoms of IVIG. She has never had side effects with IVIG before... wondering if that headache on rising is seen with kids who do have these side effects. TIA for input.

Thanks for the input so far... bumping for anyone else who wants to take part.

I agree with WD... many of have doubters all around us. You could direct them to this forum, where there are plenty of 'stories' about families struggling through this. Still, I'd focus your energy on your child's needs and finding a doctor who can help. You're overwhelmed enough in this - don't let them distract you from getting the help your child needs.

Ever since dd12 was hospitalized twice this past fall with severe, relentless migraines, I've wondered if there is a link. They started about the same time she had another strep infection and started her third severe decline into another exacerbation. We know that migraines are an inflammation of the blood vessels in the brain and PANDAS is an inflammation of the basal ganglia... not exactly the same thing, of course, but that's a lot of inflammation going on in one kids brain. I'm starting to see others posting about their kids having migraines, so now I'm wondering if there is a link... please see poll.

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.

I'm thinking of you guys, Peggy and praying for helpful answers and healing! Hugs

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?

Thanks for all the feedback... my heart sank as I read each story of new infections and exacerbations. I hope all are improved very soon! My daughter is having an exacerbation right now. She's had two severe - the one last winter was horrific as some of you may recall. By end of summer/start of school year, I would estimate she was about 85% improved; however, she's been declining in recent weeks and I'd say she's maybe 65% now. We see Dr. L tomorrow, thankfully and prophylactic abx seem a likely topic of discussion... if not another IVIG infusion - she's had 6 and was improving - even her IgG labs came up to the normal range - just not so sure they are holding there.