I want to know the "why's".....

[MissionMama]

Why??? I just need answers. The reasons behind the answers, actually.

 

We have been at this since 10/2013...acute onset overnight every single symptom for PANS (neuro typical son, then 11 - now 13yo). Strep titers always within range. Two rounds of HD IVIG back in 2 & 3 of 2014 brought our DS back to better than ever and held for over a year. Relapse after a virus almost one full year ago in Spring of '15...we just limped along with a round or two of abx, throw in some steroids, and we ended up not being able to dig out so we went back to HD IVIG in October '15, December '15, February '16 and most recently week before last....every time we start making some real progress, he gets exposed to someone or something that makes us lose our ground and left scratching our heads. We can deal with everything except the school refusal!!! It is real...not something that can be CBT'd or ERP'd....or at least not that we have had any stitch of luck with, in fact in some ways it has done more harm than good.

 

We have a fantastic neurologist, an awesome naturopath, and have jumped from one therapist to the next. The therapy piece of this treatment has been the most disappointing piece of all....and I am constantly wondering what we are missing. Is it time for the next line of treatment? The immunosuppressants of some sort? His system seems so jumpy and aggressive....or is that just me making excuses and trying to make sense of it all? We were doing SO solid until my daughter came down with a fever and vomiting this past Thursday...by Saturday the decline was evident in DS. Negativity, irritability, agitation, depression, anxiety.....

 

Any thoughts on the theory of a jumpy immune system? Is that even a thing? Shouldn't IVIG help that? He is on low dose of Luvox, 1/4mg of guanfacine to help with attention/anxiety (started this about a month ago, just takes him down a notch?), 500mg of azithromycin just once a week, and some basic supplements from the naturopath (multi vitamin, methyl B12, folinic acid, probiotic, ashwaganda). He has the muther effer gene mutation (you know, the MTHR??), along with 40% of the population....that is about all I got from this near empty and tired feeling jacked around mother tank of mine....

 

Sorry for this scattered and unproofed post....I will take and an all ideas for those that want to chime in!!

 

Thanks,

 

Kristi

 

 

[putting together the piece]

Sounds like an underlying infection weakening the immune system and allowing everything to hit him hard.

Did you check for Lyme?

[LNN]

I also kept thinking Lyme. I always thought my DS was "classic" Pandas, with sudden onset after two confirmed strep infections/scarlet fever. When Pex didn't help him improve, I blamed exposure to others with infections (his BFF had 8 back-to-back strep/ear infections and then a T&A). IVIG didn't help. So after 2 1/2 yrs of insisting this was pure Pandas, T&A, steroids, pex, IVIG, trips across the country to see Pandas experts, I listened to all those who kept suggesting Lyme and we tested thru Igenex and found Lyme.

 

Using Igenex is important because 1) they test for two very distinct Lyme bands - 31 and 34 - that other labs don't test for and 2) they report on IND indeterminate bands (bands that react but aren't dark enough to be called positive) as well as positive bands. Because he has so many IND, any other lab would've reported he was negative for Lyme. But our dr was able to see 5 IND and said it made sense to treat for Lyme. This was key. We treated for Lyme (a hard and long journey) and I got my son back. He's been off abx for 3 yrs. For the first 2 of those yrs, he had 1-2 week-long true Pandas flares when he got sick during the winter. Abx ended the flares within a week. This year, he got sick without any Pandas symptoms.

 

You'll say "but he went a year without problems" - so maybe he contracted Lyme at the end of that year. And since you're used to seeing Pandas symptoms and Lyme can look a lot like Pandas, you would've just assumed it was Pandas returning.

 

In my experience, if your child can't go longer than a few weeks or months without flaring, you're missing something - mycoplasma, Lyme, bartonella, mold...something is keeping his immune system in overdrive.

 

Also, with MTHFR, you may want to consider methylfolate instead of folinic acid (the transformation goes from folic acid to folinic acid to methylfolate - MTHFR mutations hinder the first two transformations). But it doesn't sound like that's at the root of your problem.

[dasu]

Sorry to hear this.

 

I have nothing constructive to add except to state that my kids are in that very same boat. I know what its like, seeing how each exposure (and no signs of infection) progressively sets them back in a cumulative sense. The only unturned stone so far is mold but we are moving a week.

 

Have you given your kids time to heal before sending them back into the real world? I understand that repeated HD IVIG will depress Iggs, and we have observed that for both kids. Have you tested for that?

 

One more point - have you done strep carrier testing for your family and pets?

Edited March 14, 2016 by dasu

[MissionMama]

Thanks for the responses so far. Of course Lyme and co is in the back of my mind....the very back, I will admit. We have almost zero exposure to tick bites where we live (urban-ish Washington State), and he has never had any *classic* symptoms. That said, the naturopath wants to see how the next 3 months go with some consistent IVIG under our belts and if we are still playing the up and down seesaw then she will order a whole slew of tests that may determine underlying infections, including lyme, (yes igenex), various infections, even mold is on the list (we built our house 10 years ago...I can't think of where mold could be....though it is very wet here in the PNW).

 

The theory of his immune system being in overdrive makes sense to me....just need to find out why I suppose. For any of these tests we need to wait at least 6 weeks post IVIG so that we don't get any of those antibodies in the mix. We have not tested anyone in the house as carriers for strep....and I suppose we haven't done anything out of the ordinary to try to minimize his exposure to the real world post IVIG's either. We have never been instructed to do that - is there a protocol that we are missing here?

 

I would have so much more patience with it all if he would just get to school!! And honestly if he was able to do any amount of work here at home we could at least bridge the gap with some sort of academics aside from the newspaper, mad libs (these can get hard! who knew?) and random documentaries here and there. But for him, it isn't school as a place, or about the people....it is great and the people are great, and my kiddo is very well liked....but there is something cognitive going on that triggers anxiety around doing any "work"..... He is such an articulate and communicative kid and it is confusing for me (and him) to not be able to put a finger on why it is the work that causes him the angst. Can he not concentrate? Can he hear in the classroom okay? Can he see? All of the questions that may lead to some good information always lead back to the same place: which is a very sad and frustrated "I don't know"....it is just heart wrenching. None of our kids want to live this way.... Okay, now I am just venting!

 

Just so sick of it all...and I admit to being in a why me mode at the moment. All the *normal* chatter at drop off for my daughter, parents b*itching about the most inconsequential things (trust me, I wish it was me!). I really don't want any attention, but it does sting a little that most of the questions about DS have stopped - even from some of our very good friends - but I suppose I don't blame them. How many times for the same questions with the same sigh's and I don't knows can anyone take? That being said, how about a flippin' casserole or something??? I'd love a random greeting card to accompany all the medical mail that comes...??? It is all so isolating.

 

Thanks to all my fellow warriors....keep any advice or commiseration coming. You know what they say about misery....

 

Kristi

[Sirena]

Hi Kristi,

 

It is so crazy and difficult--and so hard for the whole family...we are 10 months in with a severe case and while it's better than a month ago, it's still so debilitating. Anyway, just a couple other thoughts...we've started antioxidants and I feel like it's helping--buts that's ancedotal. Also--how about ANA? Maybe there's a clue there.

 

sirena

[dasu]

We are all there. Isolated, frustrated, abandoned, whatever.

 

A lot of the misery is from kids who are just sick but attack us parents verbally and physically, refuse to cooperate with about anything and instead try to order us parents around. Abuse from kids, abuse from doctors, abuse from insurance companies, etc. About the only two places I feel welcome are Dr L's and their psych doc.

 

lll bite. Here is my stream of consciousness rant. I have really struggled with the isolation and sense that life is passing us and our kids by. Other people are moving along with financial plans - their stock portfolios, their 401ks, 529s while all of our money goes to medical treatment. We see people taking vacations, we can hardly leave the house without anxiety. The parents are involved in activities outside the home, moms exercise or get to meet up and talk, dads get to go out and hunt and fish; we, if fortunate enough to get the kids asleep, sit in silence and browse around on the ipad. We watch their kids excelling in school, sports, arts, music; we call a few minutes of surreptitious home school a success. Well they do play piano well enough. Families at church talk so much about instilling morals and values in the kids; ours have no respect for parents, talk about cutting heads off and weird proto-sexual notions that bounce around in their head. If I get on facebook everyone has these super cute pictures of their beautiful kids; ours are posing obscenely; my daughters hair is a frizzy mess because she cant bear to comb it; both kids teeth are yellow because they refuse to brush their teeth;yet I am just happy they are wearing any clothes at all;.

 

I was reading to my kids a while back from the Little House series. When I was a kid I took away the adventure they lived and the awe of moving to wild and fresh places before civilization came. Nowadays I appreciate those books because I see the adversity they endured and overcame. This was old school, they were supposed to grin and bear even starvation. I have taken in a lot of lessons. IOne of my favorite quotes is from Ma "The sooner you accept that life is hard the more you can appreciate the good things in life". Appreciation of the simple things shines through those books, whether it was joy for a single piece of Christmas candy or over the joy her family brought her.

 

PANDAS is transforming me as a person. I no longer get spend time fawning over little things, like houses, clothes, new cars. Some of that is not by choice, some of it is. I fought it at first but now have moved into acceptance, and with it realization that my values were being changed in this awful mess. I saw this very same change occur with my sister who has two PANDAS kids. I did not get it at the time, but I do now. So many things she valued and sought to instill in her kids were stripped from her - love, education, achievement, culture, art, world travel. She went from plotting out educational experiences and inspiring vacations to plotting out doctors visits and churning through endless rounds of psych meds and alternative treatments. She fought so hard for her kids and had to focus on friends and care. She is recovering some of her life now that her two sons are older (and dysfunctionally stuck at home sadly). But she is also able to enjoy seeing her youngest son thrive in college and a engage in a rich social life.

[libera]

Our son is also experiencing extreme school anxiety and refusal. We put him in a partial hospitalization program but still ended up pulling him from his school because he just couldn't overcome the negative thoughts about it. I really do think it is based more in OCD than anxiety/performance issues. I want to have him tested for Lyme (and we lived in a high Lyme area for awhile) but getting out of the house proves to be very challenging at the moment.

 

 

DASU, appreciated your rant . . . I could relate on so many levels. Yes, the ever elusive "normal" . . . I pretty much avoid Facebook and social media because it just reinforces how far from "normal" we are.

 

 

 

Our dog tested positive for Lyme a few years ago. Has anyone heard of this causing a reaction in the PANDAS kids? I should probably have the dog and cat tested for strep too. Is there a specific test I should ask the vet for?

 

 

[ibcdbwc]

I would like to add just 1 cent to this conversation. My son is 14. An interesting development occurred in that he is now able to describe what a flare feels like for him. This never used to be. When he was not flaring, he barely remembered the flare itself. And in a flare, communication was next to impossible.

 

But a light bulb occurred. He can now describe it. He can now remember it. Unfortunately he also lives in fear of "it happening again." When he is flaring, his brain is swollen. To him, he feels like his head is wrapped tightly in a dark cloth. He can still hear noise and see light but he cannot process quickly enough. It's like everything is completely muffled and filtered. He's present but things are moving along without him. He simply cannot follow along in class. And that makes him feel so sad and so anxious. Because normally, he is sharp and follows right along. In fact normally he's used to being very efficient and at the top.

 

When reading text in a flare, he must read words over and over and over to make any sense of them. His short term memory in a flare is completely gone. His working memory is also gone. With complex math for instance which is normally easy for him - he still knows how to do the problem but gets lost in the steps. He cant remember which steps he's already completed. And simple math concepts like adding positive and negative numbers are all but forgotten. There is some sort of plug in his brain. Some sort of block. I have caught him a number of times, literally smacking himself trying to figure it out.

 

The anxiety created from all of this is incredible. He literally loses his mind -- and he now knows it -- and that makes him understandably incredibly anxious in a flare. It's horrific. Add in the OCD and food refusal - ugh. My son isolates in a flare. He knows its not his normal him.

 

If your son is like mine its no wonder they don't want to go to school when they are flaring. It's painful to not be able to do the things they know they should be able to do.

Edited March 16, 2016 by ibcdbwc

[Wombat140]

Hmm, I recognise everything in that description, in fact I've had that feeling for the last few days (alongside a nasty bout of worse-than-usual OCD) and it's only now let up a bit. I've been making vague attempts to try and describe it in words to pass on to doctors but couldn't manage it, but you (and your son) have described it exactly. I'm going to bookmark this for future reference. And it DID feel exactly as if my head was jammed full of something. Which, if theory is correct, it was.

(Did he keep letting his mouth hang open and opening his eyes wide or was that just me? I kept catching myself doing that, apparently in some foolish attempt to relieve the pressure.)

[nicklemama]

As the mother of a son who has no history of tick bite and also had completely normal titers every single time it was tested and never positive for strep throat culture, I highly advise you to find someone has qualifications to diagnose lyme disease and have him tested by a specialty lab for lyme and also for the coinfections of lyme......ehrlichia, anaplasma, bartonella, babesia. My son was treated for PANDAS for two years, even though he never had strep titers and had high dose IVIG twice but it was not lasting. When I finally took him to see an LLMD, he came back positive for lyme through Igenex and positive for ehrlichia and anaplasma through Quest. He has bartonella but he has never tested positive for it. Treatment for lyme and coinfections has made all the difference. He still has the PANS portion of it, though and has small flares in behavior when he is sick or loses a tooth. He is now 12 and just lost the last baby tooth. I celebrated.

[Albymom]

A highly reactive immune system can have many causes. I have given up searching the internet trying to piece all the clues together. Our PANS expert doctor explained that once triggered, auto-immune and/or auto-inflammatory diseases can take up to five years to fully heal. Our daughter sounds very similar to your child. She has always tested negative for everything but she is classic PANS with onset 1 year ago. They eventually deduced a valid hypothesis for a cause (a rare autoimmune disorder), but her specific situation is still fuzzy and anyway it's not really important in terms of the point I want to make.

 

Our daughter responded very well to abx at first (even though she had no active infections), then she slipped. They moved her to HD IVIG (she's had 5 since Halloween), and to steroids (both oral Prednisone and IV-infused Solumedrol). Each treatment would return her to normal for a few beautiful weeks - sometimes as many as five in a row -- before a cold sore or a loose tooth or a stupid common virus would sink her again.

 

Because her relapse and remission pattern was so clear, and because it was clear she is triggered by almost any insult to her immune system, our doctors moved us to the next level treatment in January. We are now doing Rituximab infusions and Cellcept to tamp her super-reactive immune system.

 

My understanding is that these two treatments have, in the past, typically been reserved for "severe" auto immune / auto-inflammatory PANS cases, but our daughter is quite functional and she is now more behaviorally "normal" than not. When she is in remission, she can reach between 90 - 100% baseline "normal". She attends school daily (unless she gets a virus, a loose tooth, or another type of immune system insult), and when she's well she does quite well both socially and academically. It's amazing to us that she's having such a great year school-wise, despite having missed 30 days due to PANS. If you met her or taught her in your classroom, you might not even notice she was different. Yesterday, one of her teachers emailed us to say it is "truly amazing to see the difference" in our daughter between days she's not feeling well, and days when she is 100%.

 

I've heard that some clinics are moving to Ritux and Cellcept treatment sooner than they would have in the past for kids with continued, highly reactive immune system responses. I encourage you to look into the next level of treatment for your child. Is it terrifying to consider a form of chemotherapy (Ritux) along with an anti-organ rejection drug (Cellcept) for your child? Absolutely. While is was not a decision we made lightly, in some ways wasn't really a decision at all. When the choice is to continue this push-and-pull heartbreak of remission and relapse, when you live in constant fight or flight mode just waiting for PANS to kidnap your kid at any minute, stealing more days from her beautiful life, and when your child has reached steroid toxicity levels and nothing else seems to "holding" her at well for more than a few weeks at a time, then moving to the next level isn't really a choice.

 

Our daughter's risk for infection is higher because she is now taking these drugs, however, as our doctor put it: "If she gets an infection, we admit her and treat the infection and she goes home. The number one, most critical priority is protecting her healthy brain." The goal is to suppress the auto-inflammatory cycle long enough to give the brain time to heal. This can take up to five years. Many kids are on this protocol for many non-PANS-related conditions and they live very normal lives.

 

Good luck.

[MissionMama]

A highly reactive immune system can have many causes. I have given up searching the internet trying to piece all the clues together. Our PANS expert doctor explained that once triggered, auto-immune and/or auto-inflammatory diseases can take up to five years to fully heal. Our daughter sounds very similar to your child. She has always tested negative for everything but she is classic PANS with onset 1 year ago. They eventually deduced a valid hypothesis for a cause (a rare autoimmune disorder), but her specific situation is still fuzzy and anyway it's not really important in terms of the point I want to make.

 

Our daughter responded very well to abx at first (even though she had no active infections), then she slipped. They moved her to HD IVIG (she's had 5 since Halloween), and to steroids (both oral Prednisone and IV-infused Solumedrol). Each treatment would return her to normal for a few beautiful weeks - sometimes as many as five in a row -- before a cold sore or a loose tooth or a stupid common virus would sink her again.

 

Because her relapse and remission pattern was so clear, and because it was clear she is triggered by almost any insult to her immune system, our doctors moved us to the next level treatment in January. We are now doing Rituximab infusions and Cellcept to tamp her super-reactive immune system.

 

My understanding is that these two treatments have, in the past, typically been reserved for "severe" auto immune / auto-inflammatory PANS cases, but our daughter is quite functional and she is now more behaviorally "normal" than not. When she is in remission, she can reach between 90 - 100% baseline "normal". She attends school daily (unless she gets a virus, a loose tooth, or another type of immune system insult), and when she's well she does quite well both socially and academically. It's amazing to us that she's having such a great year school-wise, despite having missed 30 days due to PANS. If you met her or taught her in your classroom, you might not even notice she was different. Yesterday, one of her teachers emailed us to say it is "truly amazing to see the difference" in our daughter between days she's not feeling well, and days when she is 100%.

 

I've heard that some clinics are moving to Ritux and Cellcept treatment sooner than they would have in the past for kids with continued, highly reactive immune system responses. I encourage you to look into the next level of treatment for your child. Is it terrifying to consider a form of chemotherapy (Ritux) along with an anti-organ rejection drug (Cellcept) for your child? Absolutely. While is was not a decision we made lightly, in some ways wasn't really a decision at all. When the choice is to continue this push-and-pull heartbreak of remission and relapse, when you live in constant fight or flight mode just waiting for PANS to kidnap your kid at any minute, stealing more days from her beautiful life, and when your child has reached steroid toxicity levels and nothing else seems to "holding" her at well for more than a few weeks at a time, then moving to the next level isn't really a choice.

 

Our daughter's risk for infection is higher because she is now taking these drugs, however, as our doctor put it: "If she gets an infection, we admit her and treat the infection and she goes home. The number one, most critical priority is protecting her healthy brain." The goal is to suppress the auto-inflammatory cycle long enough to give the brain time to heal. This can take up to five years. Many kids are on this protocol for many non-PANS-related conditions and they live very normal lives.

 

Good luck.

Wow. Albymom. Thank you for this summary. Very insightful indeed. I am going to send you a private message as I have some additional questions, so check your folder soon.

 

Thanks so much!

[wisdom_seeker]

Bump. I wish we could pin this, for there's so much wisdom in this thread.

 

I love

• Dasu's rant (I can so relate!),
• ibcdbwc's detailed, heart-breaking (and oh so accurate) description of what a flare is like.
• Missionmamma's wish that her kid would just go to school, and confusion about why he's not. (Describes our house! DS is brilliant, can crack jokes, play certain videogames, and yet most any schoolwork fills him with terror. I think you two put your finger on why.)
• the discussion of when/how to suspect and test for Lyme,

and, last but not least,

• Albymom's thoughtful description of why she's gone to IVIG + rituximab + cellcept.

Interestingly, my son's neurologist just requested rituximab along with IVIG for his first infusion. On recommendation from the PANS clinic, I believe.

 

Of course, Insurance denied both. IVIG is "not medically necessary for encephalitis/PANS". Rituxan is investigational for this condition.

 

The clinical notes the MD sent along as documentation included mention of his PANS diagnosis, as well as her diagnosis of autoimmune encephalitis, and that's what insurance is now latched onto . Dunno if we can recover from that.

 

About the rituximab... I was surprised by the rituximab, so I started reading about it in encephalitides, and the literature I found on anti-NMDA receptor encephalitis also shows better results when rituxan is added early, with suggestions that doctors consider giving the combo as first line treatment. So even though I'm apprehensive, I'd go for it.

 

Both in autoimmune encpehalitis and cancer treatments, I believe that aggressive treatment early has the best outcomes. Which is why I keep wondering if we should just stop waiting, pay for the Tx ourselves, and hope to get reimbursement if/when he has a great response.

 

As far as this topic, I'll add as well.

*I* want to know why (or how come)

• ICD-10 still has no diagnostic code for autoimmune encephaitis?
  
• MDs treating anti-NMDA R encephalitis or other encephalitides are able to order IVIG and/or PEX and rituximab, since most insurance plans don't cover those?
  
• it appears that MDs for lots of kids on this forum have gotten HD IVIG paid by insurance, even though immune deficiencies are generally only treated with LD IVIG. How do they justify that?
  
• And, my newest frustration:
  How come a phone call to insurance is able to trigger an appeal without the patient or MD realizing it! My MD's office insisted she was working on an appeal, yet Anthem heard a verbal request and ... upheld their previous decision.
  How come insurance service reps aren't required to get a positive confirmation, such as "are you requesting an appeal?" "Do you want to add any information, or include any specific questions for us to answer?"
  
  I believe what happened is that Anthem's provider line took our MD's request for peer-to-peer review, in which our MD asked that "another MD review this" and took it as a request for a 1st line appeal. So now it looks like we've blown that -- with no additional information given to the insurance, and of course, no additional rationale given by the insurance. This wouldn't be terrible if 1st appeal only looks at the plan language anyway, and only the 2nd appeal can rule on "exceptional / extenuating circumstances (like diagnoses w/o an ICD-10 code). In that case blowing the 1st appeal may not matter, but I still would have wanted to at least pose Qs like "what is it that you claim will be more effective than rituximab for his condition?".

i'm tired, pissed. Did I say discouraged? Hopefully I'll feel better in the morning, but I've been having a ton of trouble falling asleep. Maybe my kid isn't the only one with intense anxiety and dark intrusive thoughts!

 

So this was both a rant and a real set of questions. Empathy and real answers are both welcome!

Edited May 26, 2016 by wisdom_seeker

[dasu]

I have spent hours and hours on the phone talking with UHC reps. Ive sent many emails, back and forth. And I try to work hand in hand with our PANDAS provider. Each attempt at communicating with UHC reveals something new. A new story, a new claim, a new code. They are extremely disorganized, or at least appear to be. They really have no policy at all, other than to minimize what they cover.