Our Update

[sf_mom]

We just returned yesterday from Chicago and our second IVIG treatment with Dr. K and things are going well.

 

 

Post First IVIG:

 

Week 1: Rapid improvement with all TICs, mild OCD, mood liability and AD/HD type behavior

 

Week 2 - 6: Our son seemed to be symptomless... nothing noticeable

 

Week 7: Increased mood liability, talkative, dark circles under his eyes, constantly complaining of headache and stomach ache.

 

Week 8: Mood switching, talkative, hoarding his brother/sisters toys in his drawers, occasionally hitting his head to turn brain off '3 times', some night waking, continued to complain daily of headache and stomach ache. We had a teacher parent conference the day prior to leaving for Chicago and his teacher confirmed our concerns and change in personality over last couple of weeks.

 

 

Post Second IVIG:

 

Week 1: We saw an immediate sensation of symptoms.. HYPER - acted like he could run five miles yesterday.

 

 

We were able to confirm RF in my son's friend and the following are the results of the labs.

Adrian's markers that are consistent with Rheumatic Fever

 

Confirmed Strep Grp A diagnosis: 11/8/07

 

Abnormal Lab findings - elevated

 

RESULT RANGE

WBC 18.5 5.0 - 14.5

Neutrophilis 74 30 – 49

C-Reactive Protein 25.9 0 - 8

ESR 20 0 - 15

Anti-DNase B titer 680 <170

 

PNEUMOCOCCAL AB TITER 14 SUBTYPES

 

STREP PNEUMO 2/14/08 11/12/09

TYPE 1 <1.00 0.26

TYPE 3 10.27 3.93

TYPE 4 <1.00 0.20

TYPE 8 <1.00 0.43

TYPE 9 <1.00 0.16

TYPE 12 <1.00 0.21

TYPE 14 1.43 0.81

TYPE 19 1.40 1.00

TYPE 23 1.47 0.59

TYPE 26 9.00 5.16

TYPE 51 1.49 1.00

TYPE 56 <1.00 0.24

TYPE 57 1.43 1.85

TYPE 68 <1.00 0.36

 

 

We are planing on doing further testing on our younger son Romy to see if his IgG subclasses are deficient. With Corsa, it appears that either he was exposed to strep again or the IVIG donor antibodies were starting to wane and the underlying bacteria/cross reactive antibodies are still present and the reason for the creeping increase in symptoms over the past couple of weeks. While we were there, another boy Corsa's age was being treated and I was shocked at how similar their appearance was 'dark circles, chapped lips, etc'. We plan to measure Corsa's Step Antibody Titers next week, six weeks and 12 weeks. If we see creeping symptoms again we will retreat.

 

Dr. K mentioned the possibility of there being deposits in the Basil Ganglia.... IVIG would resolve those deposits. We discussed IV antibiotics... he has tried this and it was only successful when the onset was caught immediately. As far as PEX, his thoughts were only if the child was older and presented mostly with TICS and was followed up with IVIG. His feeling was the IVIG helps to regulate the bone marrow/immune system. Dr. K is trying to find out more about the exotoxins of the S. Pryogenes in Kawasaki's patients and called several people to discuss while we were there.

 

That is all that I can remember at the moment.

 

-Wendy

[JDuffner]

Wendy,

 

First let me say I am sorry for everything you are going through. I feel your pain and understand it well.

 

Wow, that sounds a lot like my daughter Belle (dark circles or red under eyes and chapped lips all the time).

 

How old is Corsa? Belle is 6.5. She has Kawasaki's at 3 and underwent IViG. Looking back through her medical history though, I think she had her first exposure to strep at 9 months, her first confirmed case at 2.5, she had a Hep A vaccine 20 days before KD, 10 days before KD she had cellulitis in her hand. Her kD symptoms were not typical. High fever (104.7), raspbery tongue, red eyes, rash only on her toes, knuckles and elbows, sed rate was 129 during KD, platelet was 441, can't recall creative right now I need to look it up. They had her on high dose aspirin on day 1 and IViG on day 4. No heart implications, they believe 3 years out........

 

Yet for the year after KD she had many healkth issues, joint pain, gut pain, hypothermic temperartures of 94.4, pupil dilation, explosive behavior, etc. A year after KD she had at least 3 episodes of Strep. Since the age of 2 I think she's been ill every 15 days. She always had episodic blips and explosive behaviors issues, but everyone kept attributing it to sensory. A year ago she had another Hep A shot and with 5 days started with severe motor tic, vocal tics, body jerks, OCD, ADHD type behavior, mood lability, talkative, hyper, night sweats, explosive behavior and a slow regression in fine motor skills and memory loss. After a year of being sent to specialist after spoecialist and everyone saying it was nothing....we just got the PANDAS diagnosis two weeks ago. Her Cam Kinase was 172. She's been on Zithro 200mg twice weekly for five wekes now with no real improvement. Last week she went on prednisolone twice a day five days and there was a huge improvement. First time she was able to write a story in class, first time she was able to verbally explain a math problem sequentially in class. Two days ago she read a 46 page 4 chapter book. Her self esteem is low, her anxiety is high. As luck would have it she started with a fever two days ago. Today she is having more behavior issues. I met with Dr. Keller yesterday and we are going to push forwrad with IviG and IV Abx.

 

Did Dr. K do IViG with IV Abx on Corsa? How often does he feel Corsa will need IViG? Did Corsa have any lasting ramifications, heart wise, from KD? Why does Dr. K think Corsa was so hyper after the second round of IViG? Belle is like this and has a super sensitive consitution when it comes to anything other then homeopathy medications. She's on supplements, a GF/CF diet, no food colors, etc. Does Corsa have a IEP at school? What IViG did Dr. K use? Did you just call to do the first consult? Belle's godparents live in Hinsdale and if the route with Dr. Keller does not work we may want to consult with Dr. K.

 

Thanks for all your help!!!

[mama2alex]

Wendy,

 

Thanks for posting the update. Glad you are back and it went well! I didn't know Corsa's symptoms were getting worse the last few weeks before the 2nd IVIG. I'm so glad to hear that you saw such great results again.

 

Jennifer

[sf_mom]

I'd like to respond in more detail but the following is our brief history. It is actually Romy, our other son who had Kawasaki's...... Our older son Corsa got sick but recovered on his own. Six months later he got his first TIC also after annual vaccinations. Adrian was just diagnosed with RF this past week. I've got to run but will add more tomorrow morning.

 

Brief History of Exposure in 2007

 

November 2007 (IMPORTANT HISTORY) Sunday, November 4, 2007 we had a play date with a boy named Adrian who had a swollen gland on the left side of his neck the size of a golf ball. Exactly 10 days after play date Corsa had what looked like pink eye in both eyes and a high fever for 4 or 5 days. Exactly 10 days after the start of Corsa‘s illness our 5 ½ month old baby boy Romy started showing signs of a similar illness. However, Romy symptoms seemed to be much worse with extreme restlessness and vomiting even after IV fluids. Within 24 hours of Romy getting sick he was admitted to the hospital and eventually diagnosed with Kawasaki’s. Romy was treated with IVIG on day 5 of high fever. I asked if Kawasaki’s was contagious and explained my older sons symptoms. I was told that they didn’t think Kawasaki’s was contagious and it was highly unlikely our other son had Kawasaki’s the week prior. I have recently discovered that our son‘s friend had peeling palms and feet (skin coming off in sheets) with a prior fever and rash at the time of play date as well as the swollen gland. The peeling palms and feet was not communicated to Adrian‘s Dr. when his mother sought treatment for swollen neck gland. Adrian was diagnosed with strep throat Monday, November 5th, 2007 and treated with several courses of antibiotics. It was eventually determined he had weak immune system, sinusitis and irritated adenoids that were eventually pulled June of 2008. THE SWOLLEN GLAND DID NOT RESOLVE ITSELF UNTIL ADRIAN’S ADENOIDS WERE PULLED. Our sons friend, still suffers from headaches, daily joint pain, weak immune system, extreme shyness, separation anxiety, OCD behavior, TICS, chapped lips with cold sores and is repeating Kindergarten.

 

Corsa: CaM Kinase 124 (Blood Draw taken 11 days post 5 day steroid burst), Deficiencies in 10 of 14 Strep Pneumococcal Antibody Titers, ASO 244, Anti-DNAse-B Negative

 

Romy: CaM Kinase 148, Deficiencies in 13 of 14 Strep Pneumococcal Antibody Titers, ASO Negative, Anti-DNAse-B Negative.

 

Adrian: CaM Kinase 147, Deficiencies in 12 of 14 Strep Pneumococcal Antibody Titers (Blood Draw in Feb. 2007, Retested Pneumococcal Antibodies 11/12/09), ESR and CRP Elevated in 2007, Anti-DNAse-B 680

 

 

 

 

Wendy,

 

First let me say I am sorry for everything you are going through. I feel your pain and understand it well.

 

Wow, that sounds a lot like my daughter Belle (dark circles or red under eyes and chapped lips all the time).

 

How old is Corsa? Belle is 6.5. She has Kawasaki's at 3 and underwent IViG. Looking back through her medical history though, I think she had her first exposure to strep at 9 months, her first confirmed case at 2.5, she had a Hep A vaccine 20 days before KD, 10 days before KD she had cellulitis in her hand. Her kD symptoms were not typical. High fever (104.7), raspbery tongue, red eyes, rash only on her toes, knuckles and elbows, sed rate was 129 during KD, platelet was 441, can't recall creative right now I need to look it up. They had her on high dose aspirin on day 1 and IViG on day 4. No heart implications, they believe 3 years out........

 

Yet for the year after KD she had many healkth issues, joint pain, gut pain, hypothermic temperartures of 94.4, pupil dilation, explosive behavior, etc. A year after KD she had at least 3 episodes of Strep. Since the age of 2 I think she's been ill every 15 days. She always had episodic blips and explosive behaviors issues, but everyone kept attributing it to sensory. A year ago she had another Hep A shot and with 5 days started with severe motor tic, vocal tics, body jerks, OCD, ADHD type behavior, mood lability, talkative, hyper, night sweats, explosive behavior and a slow regression in fine motor skills and memory loss. After a year of being sent to specialist after spoecialist and everyone saying it was nothing....we just got the PANDAS diagnosis two weeks ago. Her Cam Kinase was 172. She's been on Zithro 200mg twice weekly for five wekes now with no real improvement. Last week she went on prednisolone twice a day five days and there was a huge improvement. First time she was able to write a story in class, first time she was able to verbally explain a math problem sequentially in class. Two days ago she read a 46 page 4 chapter book. Her self esteem is low, her anxiety is high. As luck would have it she started with a fever two days ago. Today she is having more behavior issues. I met with Dr. Keller yesterday and we are going to push forwrad with IviG and IV Abx.

 

Did Dr. K do IViG with IV Abx on Corsa? How often does he feel Corsa will need IViG? Did Corsa have any lasting ramifications, heart wise, from KD? Why does Dr. K think Corsa was so hyper after the second round of IViG? Belle is like this and has a super sensitive consitution when it comes to anything other then homeopathy medications. She's on supplements, a GF/CF diet, no food colors, etc. Does Corsa have a IEP at school? What IViG did Dr. K use? Did you just call to do the first consult? Belle's godparents live in Hinsdale and if the route with Dr. Keller does not work we may want to consult with Dr. K.

 

Thanks for all your help!!!

[sf_mom]

Jennifer,

 

You know we really didn't realize he had creeping symptoms until the day before we left for Chicago. Originally, we thought the mood liability, headaches, stomach aches were from medications. I NOW know that headaches and stomach aches are the main symptom of STREP!!!!!! AND, once we had our teacher parent conference we were very grateful to already have had the 2nd IVIG schedule. Hitting his head to turn his brain off started while in Chicago... Honestly, it couldn't have better timing. He needed the second round and I think Dr. K might have suspected this in advance because his main presentation originally was mostly TICs. Its odd how the symptoms creep back up in reverse AD/HD type symptoms, mild OCD.... no real TICs over the past two weeks.

 

We saw IMMEDIATE improvement over night. Still a little HYPER today but doing much better than yesterday. I am hopeful that this will be it for IVIG but are not ruling out others.

 

We should talk sometime soon....

 

How is Alex doing?

 

-Wendy

 

 

 

Wendy,

 

Thanks for posting the update. Glad you are back and it went well! I didn't know Corsa's symptoms were getting worse the last few weeks before the 2nd IVIG. I'm so glad to hear that you saw such great results again.

 

Jennifer

[faith]

Wendy,

glad things went well.

What did you mean that Dr. K 'suspected' that he'd need a second IVIG because his presentation was mostly tics? and re the strep, was he exposed, or he had it?

 

Faith

[melanie]

Jennifer,

 

You know we really didn't realize he had creeping symptoms until the day before we left for Chicago. Originally, we thought the mood liability, headaches, stomach aches were from medications. I NOW know that headaches and stomach aches are the main symptom of STREP!!!!!! AND, once we had our teacher parent conference we were very grateful to already have had the 2nd IVIG schedule. Hitting his head to turn his brain off started while in Chicago... Honestly, it couldn't have better timing. He needed the second round and I think Dr. K might have suspected this in advance because his main presentation originally was mostly TICs. Its odd how the symptoms creep back up in reverse AD/HD type symptoms, mild OCD.... no real TICs over the past two weeks.

 

We saw IMMEDIATE improvement over night. Still a little HYPER today but doing much better than yesterday. I am hopeful that this will be it for IVIG but are not ruling out others.

 

We should talk sometime soon....

 

How is Alex doing?

 

-Wendy

 

 

 

Wendy,

 

Thanks for posting the update. Glad you are back and it went well! I didn't know Corsa's symptoms were getting worse the last few weeks before the 2nd IVIG. I'm so glad to hear that you saw such great results again.

 

Jennifer

 

Was Dr K able to get the insurance to cover any of the second IV?

[sf_mom]

TICs are harder to get eradicate via IVIG. Corsa was exposed to Strep at his school about the time we saw a slight increase in behavior although I suspect the first IVIG didn't fully do the job.

 

Dr. K recommends with older children that have primarily TICS to do PEX with follow-up IVIG.

 

-Wendy

 

Wendy,

glad things went well.

What did you mean that Dr. K 'suspected' that he'd need a second IVIG because his presentation was mostly tics? and re the strep, was he exposed, or he had it?

 

Faith

[sf_mom]

We think so...... Corsa was coded for Encephalitis and Rheumatic Fever Chorea. They did cover the first time and it was applied towards our deductable with the same coding. BUT, I understand not every child is coded similarly by Dr. K.

 

-Wendy

 

 

 

Was Dr K able to get the insurance to cover any of the second IV?

[smartyjones]

Dr. K mentioned the possibility of there being deposits in the Basil Ganglia.... IVIG would resolve those deposits.

 

Wendy - glad all went well. what do you mean by the deposits in the basil ganglia?

[JDuffner]

Wendy,

 

I can't thank you enough for all the information, especially just coming off a chicago trip and IVIG! When Belle was three and they were talking titers, sed rates, etc....I thought it was all over...then moving to a different state they kept pushing the vaccine issue (she will NEVER have another vaccine, her curernt doctor agrees).

 

So Romy and Corsa are both considered PANDAS? I guess what I am trying to figure out is did Adrian indeed have KD undiagnosed? I thought they "say" KD is not contagious and rare for siblings to get? I'm trying to figure out the KD/PANDAS connection.

 

The last question for you. You mentioned "TICs are harder to get eradicate via IVIG. Corsa was exposed to Strep at his school about the time we saw a slight increase in behavior although I suspect the first IVIG didn't fully do the job.

 

Dr. K recommends with older children that have primarily TICS to do PEX with follow-up IVIG." What constitutes "older children?" Belle will be 7 in March and she is still having tics (nose sniffing, some vocal, some finger flicking at times). She has anxiety, chews on shirts, etc. I'm trying to figure out what additional tests I need for Belle before moving forward and would she be considered a candidate for PEX first according to Dr. K or IVIG first.

 

Jacque

[coco]

So glad to hear you son is doing so much so quickly!! What was the ivig dosage Dr. K used? Was it the same 2-day dose as the first time?

[sf_mom]

Corsa is definitely PANDAS and we are still investigating Romy situation (labs are not looking great). We plan to test Romy's IgG subclasses if they are low... we will retest in 3 months and if they are still low we will probably treat with IVIG. Its so hard to tell behaviorally with a 2 1/2 year and Dr. K doesn't want to rush into any treatment without further testing. Adrian has the RF diagnoses because there was confirmed strep at the time. Typically, kids with Kawasaki's don't have positive cultures, elevated ASO titers and Anti-DNAse-B. THEY DON'T THINK its contagious but they don't really know. The S. Pyogenes Exotoxin (same strain in RF) that was found in 100% of Kawasaki's patients (taken from study) takes 10 days to incubate.... Our kids got sick exactly 10 days apart. S. Pyogenes is one of the many strains of STREP http://pyogenesgonewild.com/

 

When Dr. K refers to older he means 10 + approaching puberty. Dr. K doesn't perform PEX... only IVIG. He has done PEX in the past, years ago non-pandas (I believe) but there are too many 'risks' with PEX in his opinion. He rather try IVIG several times... BUT, if you are going to consider PEX, his thoughts are only do it for older children, primary presentation TICS and with follow-up IVIG.

 

Your Daughter doesn't seem far off from where Corsa was... He responded really well to the first treatment and so far with the second treatment. The most recent creeping symptoms were mild but who knows what would have happened had we not had the second IVIG scheduled.

 

You should definitely have the following labs done if haven't already.

 

White Blood Cell Count

Red Blood Cell Count

Anti-DNAse-B

Strep Pneumo Titers

IgG Subclasses

CaM Kinase 'Madeleine Cunningham'

 

-Wendy

 

 

 

Wendy,

 

I can't thank you enough for all the information, especially just coming off a chicago trip and IVIG! When Belle was three and they were talking titers, sed rates, etc....I thought it was all over...then moving to a different state they kept pushing the vaccine issue (she will NEVER have another vaccine, her curernt doctor agrees).

 

So Romy and Corsa are both considered PANDAS? I guess what I am trying to figure out is did Adrian indeed have KD undiagnosed? I thought they "say" KD is not contagious and rare for siblings to get? I'm trying to figure out the KD/PANDAS connection.

 

The last question for you. You mentioned "TICs are harder to get eradicate via IVIG. Corsa was exposed to Strep at his school about the time we saw a slight increase in behavior although I suspect the first IVIG didn't fully do the job.

 

Dr. K recommends with older children that have primarily TICS to do PEX with follow-up IVIG." What constitutes "older children?" Belle will be 7 in March and she is still having tics (nose sniffing, some vocal, some finger flicking at times). She has anxiety, chews on shirts, etc. I'm trying to figure out what additional tests I need for Belle before moving forward and would she be considered a candidate for PEX first according to Dr. K or IVIG first.

 

Jacque

[EAMom]

Her Cam Kinase was 172. She's been on Zithro 200mg twice weekly for five wekes now with no real improvement. Last week she went on prednisolone twice a day five days and there was a huge improvement.

 

Hi...I would really encourage you to up the Azith. from 2x weekly to daily. How much does your dd weigh? Our dd (currently 54 pounds) has been on 250mg/day Azith. since June 08 (we also had IVIG Aug 09). Many PANDAS patients find that lower doses of abs (Azith. or Augmentin) or the smaller guns abs (eg. amoxciilin) just don't cut it. Full strength (or maximum dose of abs for their weight) is what some of these kids need (even with IVIG).

[sf_mom]

I missed this fact in your first post and totally agree with EAMom. Our son is 45 pounds and on 250 Azithromycin daily. Twice weekly is not enough.

 

-Wendy

 

 

 

Her Cam Kinase was 172. She's been on Zithro 200mg twice weekly for five wekes now with no real improvement. Last week she went on prednisolone twice a day five days and there was a huge improvement.

 

Hi...I would really encourage you to up the Azith. from 2x weekly to daily. How much does your dd weigh? Our dd (currently 54 pounds) has been on 250mg/day Azith. since June 08 (we also had IVIG Aug 09). Many PANDAS patients find that lower doses of abs (Azith. or Augmentin) or the smaller guns abs (eg. amoxciilin) just don't cut it. Full strength (or maximum dose of abs for their weight) is what some of these kids need (even with IVIG).