Buster

Hi Karen, I'm not an expert on this topic. Perhaps one of the members who has multiple sclerosis or knows someone with MS can comment. The topic is really well discussed in that forum and I think they'll have a really reasoned opinion there. In general, I understand anything over a month is considered long term and requires a careful taper to avoid lots of nasty withdrawl issues. Prednisone is used in MS to help close the BBB. It has all sorts of side effects not the least of which is that sustained high-dose use necessary for significant anti-inflammatory seems to weaken bones. The papers I've found on longer term pred use are all in the MS, Lupus, athsma, or rheumatoid arthritis space. Not sure what to say here, but presume your doctor is well versed in the risks/benefits. Not sure where to draw the line either. We personally were okay with a short burst (5 days) but were worried about some of the longer term effects. Regards, Buster

We too had dilated pupils. We thought it was akathesia from being on an SSRI and switched dosage and SSRI. However, we do think it was just the PANDAS now.

Hi Pixiesmommy, Under HIPAA you have rights to her medical record with three exceptions: psychotherapy notes can only be given to other licensed psychotherapists records created for use in litigation are not made available to you if access would result in harm to you or another person These are federal requirements and their license can be in jeopardy if they fail to comply. In particular, they are required to present to you a privacy notice that includes information about how you can obtain copies of your medical record. If a written request is required, the privacy notice must tell you this. If you are denied access, you can file a complaint with the U.S. Department of Health and Human Service's Office of Civil Rights. Your state's medical privacy law might also enable you to file a complaint with state regulators. For a state-by-state guide to health privacy law, try http://hpi.georgetown.edu/privacy/records.html If you want a sample letter that is quite effective, use http://www.privacyrights.org/Letters/medical2.htm Given the severity of sanctions that happens if a provider fails to comply I am really surprised you are having any difficulties. Technically, HIPAA gives providers a maximum of 30 days to provide the records. One 30-day extension is allowed for good reasons. State laws typically provide even less time (and cannot extend the time). Essentially the state law can put more pressure on providers but cannot relax the requirements to provide records. I highly recommend sending registered letter using the form above -- don't call them, just send the letter. They can charge you a modest fee for the records -- typically this fee is waived but can be 1-5cent/page. Even if they have failed to respond to your demand before, you should restart the clock by sending them a new letter. If they fail to respond to you then within 180 days of the breach you can file a complaint with DHHS ( http://www.hhs.gov/ocr/privacy/hipaa/compaints/ ) Upon receipt of a complaint, the DHHS will contact the party. A person or organization that fails to follow the Privacy Rule can face an immediate fine of up to $25,000 and in extreme cases can involve the department of justice. If the DOJ get involved then this can be 10 years in jail and a fine of $250,000. Bottom line, you should be able to get the records. Buster

I think it is almost impossible to see the sawtooth while you are in it. If you document symptoms on say a 1..10 score every week and plot this over time then you can see the sawtooth pattern. We have some 10 symptoms we track creating a maximum of 100 and a minimum of 0. We use 1-3 to indicate that the symptom happened but was irradic, 4-6 to indicate it was present pretty consistently, 7-9 that it was severe enough to be incapacitating. The easiest way to see a 7-9 is to think of the contamination fears and having an inability to wash hands because there are ghosts coming out of the tap -- take it that this is really severe and pretty out there. In our case, when you plot the symptoms this way the sawtooth pattern emerges. If you want to see a sample graph send me an IM. Is this just a recent event or has he had these for a while? It's possible, pink eye is often caused by either a virus or by a staph or strep bacteria. Defeat Autism Now -- it usually refers to a doctor who specializes in Autism.

Peglem, absolutely. I've got a growing stack of papers indicating asymptommatic carriage is not benign. I'll dig for the others and perhaps post a few. There was a very nice little study done in 1988 by Gerber known as "The group A streptococcal carrier state. A reexamination" ( http://www.ncbi.nlm.nih.gov/pubmed/3128949 ). He split the groups into three groups: those with negative throat cultures, those with positive throat culture and postive rise in antibody titers, and those with positive throat culture and no significant rise. He showed that treating those patients in group 3 with antibiotics had comparable and dramatic clinical response to treating those in group 2 (i.e., the antibody titer rise was really not useful clinically). There was also a very nice paper in 1997 by Rogers, "Strain prevalence, rather than innate virulence potential, is the major factor responsible for an increase in serious group A streptococcus infection" ( http://www.journals.uchicago.edu/doi/pdf/1...875?cookieSet=1 ) While this paper was about superantigens, there is some very interesting items towards the end of the paper where the author writes "Colonization may occur in individuals without M serotype-specific antibody and manifest as a benign infection or carriage. The course of infection and progress to severe infection may primarily depend on an individual's state of immunity and genetic constitution as well as on antibody levels to the numerous extracellular products secreated by GAS." Buster

The term "psychogenic" refers to movement disorders that seem to respond to psychotherapy, placebo, are not consistent (implying some form of voluntary participation/choice), or seem related to a psychiatric illness. The term "organic" refers to, well, tics, tremors or other sterotypies (implying non-choice). Essentially the authors are trying to classify those movement disorders that respond to psychotherapy (or placebo) and those that do not. I can't tell you if these terms are used pervasively. One of my old professors used to say that most PhDs are granted on the achievement of a student to overcome their own terminology -- a kind way of saying that sometimes the terms sound good on paper but have limited use :-) I referred to the paper because they did use a combination of antibiotics and steroids and it was an interesting recent case study. Buster

We agree. 82 weeks ago when this nightmare started we went from calm to insane (this ramp took a mere 4 weeks). It took us 8 weeks to get a handle on what might be happening from an accidental comment from a psychiatrist. Then forcing the doctors at the hospital to run a strep culture (you'd be amazed how much they argued), then dealing with their surprise when it was positive, then "forcing" them to treat with augmentin, then her symptoms getting so much better that they were ready to release her... wow. Of course then we got home, symptoms started ramping again, then discovering that her sister also had strep, then discovering the whole cycle started again because we didn't have a strong enough prophylactic dose. Wow! In retrospect, we can see this, at the time, we really just were clueless what was happening and incredibly scared. I still can't believe the set of events that aligned so we met the parents on this forum, that we met a psychiatrist who had a clue, that we met Diana who was struggling herself, ... wow! I think daily of how grateful I am to everyone on this board and particularly to Diana who, well, nudged us (not sure of the right word), to try a different prophylactic dose of azith.... We essentially strong armed our pediatrician. I laugh now when I think of how insistent we were. In the first week, nothing, then on 10th day symptoms dropped. She said "I want to get better"... all symptoms disappearing over next month. Wow. There really truly is that moment when you doubt everything and need some hope. Thanks to Diana and the rest here who helped us. For the next 20 weeks, a blessing. Then another exposure/exacerbation (4 weeks to resolve), then 2 weeks quiet. Then another exposure/exacerbation (6 weeks to resolve), then another exposure exacerbation (6 weeks to resolve), ... this kept happening with her sister bringing strep into the house. We did get her sister treated and cleared of strep in between, but kindergarten seems to just be a petri dish for strep. We tried to convince our pediatrician to put the sister dd6 on prophylactic antibiotics -- that was a non-starter. In retrospect, would have been a great thing to do... Anyway, long story shortened, we value each day that got better (the see-saw from terrible to just bad) and were frightened of each exacerbation that seemed to make the baseline worse until after 60 weeks we were awfully close to where we had started -- all the symptoms were back -- contamination fears, severe OCD, ... It was this lack of reset to a baseline that took us to consider IVIG. I was trying to explain in another post that it was like trying to stay ahead of snow storms while shovelling the driveway. Eventually, we called in a snow plow to help. We did get a reset and are now back at a calm spot. We think we could have gotten here with just the antibiotic prophylaxis if we didn't get so many exposures so close together. Regards, Buster

Going from our experience, the answer would be yes. Our dd9 is like someone who is hyper sensitive to GABHS -- if it's around her with a sibling she seems to react. We don't understand exactly how it happens. We don't know if full colonization is occuring or some sort of short circuit -- like what happens for folks who have IgE/Eosinophil reactions. In terms of the immediacy, this seems to be a result of B-cells -- they remember the pattern. It seems to take them one exposure to "learn a pattern" and then subsequent exposures cause a pretty immediate reaction. Someone once referred to this as an allergic reaction. There might be something in that -- in our own daughter's case she had elevated eosinophils during an exacerbation -- but we still don't quite know what that really means. Regards, Buster

Hi Lismom, for 65 weeks, we did only antibiotics and this was very effective. Her symptoms declined dramatically after the first 10 days on azithromcyin and by 1 month we were 90% better. We had 7 subsequent exacerbations and 5 of of them were correlated with her sister (or mom) having culture positive GABHS. The 6th exacerbation was tied to H1N1 and the 7th to getting Fifth's disease (symptom showed at the fever and the slapped cheeks showed 3 weeks later). We would probably not have gone for IVIG if we had had fewer exacerbations -- it just seemed with the frequency we couldn't get her back to the baseline.... It was sort of like trying to stay ahead of snow storms -- we kept having a new storm before the results of the previous storm dissipated. Finally we called in for the snowplow :-) Terrible analogy but best I could think of before my coffee this morning. Looking at the frequency, it looked to us that it takes our dd9 about 8 weeks to get back to "normal". If we get another cumulative exposure within that time, the baseline just goes up and the exacerbation is worse. Our daughter may be an extreme case, her CaM Kinase II was really high (253) and the severity of her symptoms was super scary (not eating, body morphology issues, severe contamination fears, ...). In our case we're glad for the reset but are now back to just keeping her on antibiotic. Treat it as we got help to get back to a baseline but EAMom and I think if it weren't for the severity of the exacerbations and the frequency we probably would have just stayed with antibiotics. Regards, Buster

Technically, the antibiotics will still work (i.e., slowing the protein copying activity in the case of azithromycin) but the bodies own immune system is suppressed on prednisone. So, if there is a present strep infection, it'll be held in check, but the immune system will be slow in doing anything about it. I'd be hesitant to be on long term prednisone -- given all the side effects there (including the one you highlight). A short term burst helps break cycles of inflammation and over response by the immune system. There is a very, very recent paper out http://www.ncbi.nlm.nih.gov/pubmed/1989668...mp;ordinalpos=1 That has been looking at this treatment. You might want to look at their findings (albeit on just a single case). Regards, Buster

Trying a short answer here. Cunningham's lab is conducting a research trial where she is measuring anti-neuronal antibodies in blood serum and the interaction of those antibodies with neuroblastoma cells. It appears that those with PANDAS and Sydenham Chorea have elevated levels of CaM Kinase II activation ( http://www.pandasnetwork.org/Cunningham.NMpaper%5b1%5d.pdf ) relative to those with Tourette Syndrome. She also isolated several antibodies that seem to interfere with neuronal signalling ( http://intramural.nimh.nih.gov/pdn/pubs/pub-15.pdf ) . While still a research test (and not yet a diagnostic test), a number of the parents here have participated in the study including having measurements before and after attempted treatments (such as use of antibiotics, prednisone, IVIG, PEX, ...). At this point, PANDAS is thought to be the combination of three things: an immune response to GABHS causing B-cells to produce antibodies targeting the GlcNAC carbohydrate on GABHS a failure of the T-regulator and B-regulator immune suppressing cells that would normally stop this antibody a breach of the blood-brain barrier that allows these antibodies to reach and bond with neuronal tissue So the Cunningham tests could help you know whether #1 and #2 are occurring. Our dd9 also exhibited a ritualistic arm movement with a verbal tic during the exacerbation. We also believe she has had an underlying social anxiety that predated the sudden onset OCD. Regards, Buster

I think cough is typically exclusionary to strep. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2231494/ Typical clinical signs are: In otherwise healthy people, a sore throat is usually self-limited and rarely produces serious aftereffects. Typical GABHS patients are children aged 5 to 15 who present with fairly acute onset of fever and sore throat. Headache, nausea and vomiting, malaise, dysphagia, and abdominal pain might be present. Cough and rhinorrhea are usually absent. Edema and erythema of the tonsils and pharynx are usually present. Anterior neck glands might be enlarged and tender. A non-adherent pharyngeal exudate might be present Buster

Actually, they do sell a home rapid strep test. You can buy it off Amazon.

Typically dd6 (dd9's sister) does not have any normal symptoms of strep. She does have enlarged tonsils and occasionally she'll say her throat hurts but that is very seldom. On the sensitivity to strep / allergic reaction -- technically an allergy seems to imply an IgE and Eosinophil reaction -- but what I meant was that it just seems like dd9 is about the most sensitive measurement device -- when she would act up, sister would be culture positive for strep. Over the last 75 weeks, there were 10 distinct exacerbations. Each exaerbation lasted 4-6 weeks. In 7 of the exacerbations the onset was correlated with someone in the house having strep. In three exacerbations her sister wasn't positive for strep. On one, we think it was a bad throat swab because the doctor we saw was pretty clueless how to do one (can you believe it). We went in a week later her sister was culture positive (i.e., we didn't believe the original test, and sure enough were right). On the other two, they seemed to be more a response to a viral infection. One was fifth's disease (both kids got the classic slapped cheeks 3 weeks later). On the other, we were at disneyland (great petri dish) and we think we got a form of H1N1 -- whole family out for 5 days. I know I'm repeating myself by I really think the model is: GABHS trigger of an immune response causing B-cells to release faulty antibody failure by T-regulatory and B-regulatory cells to stop the B-cell inflammation causing a breech of the blood-brain barrier such that the antibodies reach the neuronal cells Given that the half-life of the antibodies seems to be around 4-6 weeks, this means a break down in the BBB could lead to an exacerbation up to 8-12 weeks from exposure. If the GABHS is really not gone (i.e., minor colonization or limited invasion) then there'd be enough to keep triggering the B-cells but maybe not enough to cause ASO to rise. It all would be fascinating if it weren't our own children in the mix. Best regards, Buster

This is a complicated question to answer. It is possible to culture positive for GABHS -- but I'd recommend if there is a sibling in the house, to culture the sibling. In our house, when our PANDAS child acted up invariably (90% of time) her sister was positive for GABHS. We think it is like a peanut allergy -- our dd9 is just really sensitive to it. I don't know how much strep invasion you need for a positive ASO and AntiDNAseB -- but even when both tests are used correctly at exactly the optimum time after a positive throat culture -- they still have a 31% false negative rate. ASO alone has a 46% false negative rate. What also has been interesting is when dd9's sister has a positive culture, dd9 is in an exacerbation (that's how we knew to bring in sister) but dd9 does not show positive ASO or positive culture. It just makes me think we're measuring sort of the wrong thing and that we need to measure sensitivity to GABHS rather than sensitivity to an exotoxin of GABHS. It's also possible it's a viral infection happening and it's just affecting the BBB allowing antibodies to cross. Really hard to say. Regards, Buster

DD9 -- 183 (baseline)-- separation anxiety, verbal tic, defiance 253 (exacerbation) -- significant exacerbation, contamination fears, motion abnormalities, verbal tic, lashing out, skipping meals 119 (post-IVIG) -- some residual separation/social anxiety, no other symptoms.

IVIG is about your child being sick sick -- meaning this isn't just traditional OCD or Tics but rather that the pathogenesis (i.e., cause) is auto-immune. An auto-immune disease is about being sick sick. Presuming you meant IVIG rather than just IVs, IVIG does have documented benefit for those with immune disorders. The exact mechanism is not known, but it is thought to help close the blood-brain barrier (anti-inflammatory) and suppress certain auto-antibodies. That is a pretty short time. In our case we saw a decline for the first week post IVIG and then improvement after that. Don't know. Risperadal acts to regulate the amount of dopamine and seratonin. Are you still on Risperdol now or did you stop it? How long were you on it? I'm arguing that while you are waiting to schedule IVIG and considering the expense of it, protecting against another strep exposure may help n the effectiveness. In the literature the studies of IVIG on sydenham chorea, kawasaki's disease, ARF, and PANDAS have all had the patients use prophylactic antibiotics before/after treatment.

Melanie -- please know I feel your pain here and the difficult decisions you have to make. I can't advise you on the IVIG -- I can say that we had an enormous improvement in symptoms 2 weeks into starting prophylactic azithromycin. If it is PANDAS the very, very first thing is to ensure you've gotten rid of the GABHS and protect against having another attack. IVIG has a *lot* of risks and is more a last resort rather than a first path. In our case we were pretty desparate and our child was at an all time peak of symptoms when we finally convinced a doctor to try azithromycin -- I basically said that I didn't care if it was just placebo effect or anything else, but that good medical science on the balance of risks/benefits indicated that this was a reasonable trial based on best medical evidence. On the 10th day of daily azith -- we had substantial improvement in symptoms -- I'm not kidding. In June it was as if a fog cleared and she said "I want to get better" -- oh, my goodness. Over the next month almost all symptoms abated. So please, please see if you can try the low-cost, low-risk azithrmycin route for an extended 30 days before jumping at IVIG. I personally think that IVIG without prophylatic antibiotics is just throwing the treatment away. Regards, Buster

I can echo dcmom. We had a cliff of symptoms in June of last year when we put our dd on azithromycin. On the 10th day (after commencement) we had a break-through where she finally could vocalize that she wanted to get better and then symptoms dissipated over the next month until by mid August she was 90% back to normal. In our own case, we kept a daily log and then a weekly log because outside of that time window it is impossible for us to "remember" what happened. In addition my dw would mark major events on a calendar. When we plotted the last 75 weeks, there is a noticable ramp and decline in symptoms. We plotted each symptom on a 1..10 scale (emotional lability (raging), lashing out (physical striking), measurement rituals, skipping meals, contamination fears, hallucinations, suicidal vocalization, obsessive questions, handwriting anomolies, ...) where 2-3 meant it happened but was irradic. 4-6 meant it was consistently present, 7-10 it was disabling. The contamination fears and rituals are the easiest to see as disabling -- they basically intrude to such a degree that they prevent doing anything else for the typically 1-3 hours that the symptom is present. The sawtooth pattern and episodic nature was really clear when plotted against this 75 weeks. Symptoms +/- 30 points over 10 weeks -- looking in retrospect very sawtooth. Regards, Buster

Hi laurenjohnsonsmom: I'd like to add my welcome here too and thank you and your daughter for your collective courage in facing the media. Whether you knew it or not, you were helping all of us on this forum (and maybe even more so, those who haven't yet found this forum) who struggle with an illness that the medical community at large is either ignorant of or skeptical and dismissive about. Whether your child has PANDAS or something else should come out over time. The symptoms certainly are consistent with PANDAS. In the show it was raised that you were already connected with Dr. Leckman and thus are probably pretty well versed at this point about PANDAS. You might find the brief post on the history of PANDAS helpful : http://www.latitudes.org/forums/index.php?...amp;#entry36300 The current working assumptions are that PANDAS is similar to Sydenham Chorea and has three main components: an immune reaction to a streptococcal infection that produces a faulty B-cell antibody response (trigger) -- a focus of Kirvan/Swedo a failure of the T-reg and B-reg cells to suppress the faulty antibodies -- a focus of Leckman a breach of the blood-brain barrier that allows the antibodies to interact with neuronal tissue (visibility) -- a focus of Yaddanapudi The antibodies tend to remain in the blood system for 4-6 weeks (even after the removal of the original antigen (i.e., strep)). Many on this forum think the effectiveness of high-dose antibiotics (augmentin or azithrmycin) is that these help against strains of strep that go intracellular (acting more like a virus) and preventing the immune system from "calming down." In addition, many on the forum have found difficiencies in Igg immune response indicating that the traditional dosage of antibiotics (for those with healthy immune systems) may be insufficient for these children. Hopefully your association with Dr. Leckman will help NIHM and others focus more research on this very important topic. Regards, Buster

We cite the same article but try to provide the simple bulleted list: 46% of culture positive children do not have an ASO rise 54% of culture positive children do not have anti-DNAseB Contrary to popular opinion, the combination of ASO and Anti-DNAse-B failed to confirm GABHS in 31% of culture positive children. If they bring up carriage, I ask if they mean asymptomatic or symptomatic carriage. The AMA recommends treatment of any symptomatic carriage to prevent dangers of ARF. The estimated asymptomatic carriage rate is estimated as being ~8-10% by WHO, there is no explanation yet widely accepted for this carriage. The asymptomatic cases are treated when there is either prior RF, SC, Kawasaki or one in the household. Asymptomatic carriage is still contageous but just less so than symptomatic carriage. This is why siblings are often treated. With respect to PANDAS and GABHS carriers -- this is not studied. Buster

First and foremost, thank you for your post and in particular for being the parent we all try to be -- getting the best help for your kid. There is lots of useful information on this site. In particular, you might find the history of PANDAS research helpful at: http://www.latitudes.org/forums/index.php?...amp;#entry36300 Most of the parents on this panel arrived here by luck. In our case it was after the 20th call to find a psychiatrist that could treat a 7 year old with a sudden onset eating disorder and body morphology problem where the psychiatrist mentioned "have they done a strep test" -- What??? We had never heard of PANDAS before that. Our daughter presented with a very odd movement disorder where her right hand touched her stomach, her sternum, then the back of her hand touched her chin and this would happen over, and over again. It was somewhere between a tic (rhythmic) and chorea (arhythmic) but mostly was a compulsion. She presented with very severe OCD that was inhibiting her from eating -- this was the scariest aspect. I mention this because after months of trying different things we were able to have a breakthrough from help from other parents on this forum. The bottom line is let us (the panel) know if you have questions that you want a parent's perspective on... We have all spent an enormous amount of time helping doctors find informative articles and actually get beyond some of the provocative titles to look at the actual research and findings. You are already linked with great researchers so that part of this forum may not be useful to you -- however, the second aspect of finding a set of parents who really, truly know what you are going through and the incredible emotional drain this disease and the fight takes on our children, ourselves and our family -- well, we're all here for you. Best regards, Buster

BriCandy, Wow. No we didn't do a spinal tap before the IVIG. If you do that, you should have your doctor contact Madeleine Cunningham so that they can check for anti-neuronal antibodies in the CSF -- this way you'd show that the antibodies are crossing the blood brain barrier. We think that PANDAS is five separate things: an abnormal B-cell antibody response to GABHS (the triggering event -- but not the showing event) a failure of the B-regulatory and T-regulatory cells to inhibit these antibodies (the failure) a breach of the blood-brain barrier that allows the antibodies to interfere with neuronal tissue (the showing event) even after the original antigen (GABHS) is removed, the antibodies stay present for 4-6 weeks the B-cells are memory B-cells and thus exquisitely sensitive to GABHS in the future So it is totally possible that sinus infection 3 week prior was #1 and the H1N1 triggered the breach in #3. In our case, IVIG caused an increase in symptoms immediately following IVIG, but these abated in 14 days -- such that we are now symptom free at 15 weeks post IVIG. Please, please, please get on some form of prophylactic antibiotics -- preferably azithromycin if augmentin didn't have any effect. I'd hate for you to go through all of this and get a re-exposure and be right back where you started. The steroids will help suppress the immune system but that seems a really odd thing to do when you are just doing IVIG -- i.e., strengthening the immune system. Bottom line, please get at least some prophylactic dose so that it's not yet another complicating variable while you look for the effectiveness of IVIG. Best regards, Buster P.S. Did you have Cunningham tests pre-IVIG?

When our dd9 was at her worst we would see a noticable improvement with Advil. Curiously we didn't see it with Aleve. Strangely, the first time we tried it was when she had a fever. In 30 min, she fell asleep. So at night, she tended to fall asleep after advil. In the morning, her behavior improved. No explanation. We wondered about Cox-II inhibitors and the research about alzheimers... but no answer. Just a curious observation that it helped. Buster

Oh, I don't have them confused -- only their names. One has a place on a dart board (joke)