OMSLife Foundation Conference in Cinncinati, OH 2015

I was lucky this weekend to be able to visit Cinncinati, OH and attend a conference for Opsoclonus Myoclonus Ataxia Syndrome put together by the OMSLife Foundation, run by Mike Michaelis.  There were several speakers including Dr. Gilbert who spoke about OMS/OMA (Opsoclonus Myoclonus Ataxia), Dr. DeSena who spoke about immunology and treatment options, Dr. Brian Weiss about Oncology, specifically neuroblastoma, and Dr. Wendy Lopez for child psychology.  This was held at the Cinncinati Children’s Hospital, lunch and beverages provided, reading materials (parent handbook) as well.  It was so nice to see some of the other parents in person and the founder and his daughter were just so sweet and very professional non profit organizers (I am always so appreciative to find a good foundation who really make a difference for us families.  There are bad charities out there and this is not one of those.  VERY happy with this foundation)

The first speaker was Dr. Gilbert who spoke about OMS/OMA and although a lot of the information I already knew, there was a lot of things I didn’t know either.  One thing I learned was that all children with OMS/OMA don’t necessarily have opsoclonus and myoclonus.  They may have one or the other or both.  This fact alone made me feel confident Zane has OMS/OMA.  When recalling the symptoms of OMS/OMA during his speech I was remembering a lot of things Zane used to do that he no longer does.  One thing being tremors, and fevers.  He tremored so bad we had to swaddle his arms down and he had to be snuggled in a blanket tight for him to be comfortable.  He did this the second he was born.  What our doctors always thought was interesting is the every EEG test showed that he was not having seizures and when you hold his arms the tremors would stop.  If they were seizures his arms would continue to tremor even after being held, we were told.  He stopped doing this slowly after his neuroblastoma was removed and I dismissed this as a normal reaction for a kid born with cancer to do.  Or maybe it was the fact he also has a positive Costello Syndrome diagnosis? We here so often that doctors are still learning about Costello Syndrome and anything he does that can’t be explained we just assume is because he has a major genetic disorder.  However not all kids with Costello Syndrome have had cancer and that I know of only a couple (if any) have reported to also have Opsoclonus Myoclonus Syndrome.  So we just have to sorta treat things one at a time according to trial and error and by symptom.  If the symptom is unexplainable its sometimes forgotten about.  Like this one.  Fevers… this doctor said most OMS/OMA parents said their child had a big fever before their onset of symptoms.  Zane did at one point spike a 104.9 fever with no sign of a virus causing it.  He went on to have seizure like activity for almost 24 hours, three rounds of sedation before we could get him to stop.  his heart rate was over 220bpm and between neuro and cardiology everyone was pretty concerned about him for that day.  Neuro said the EEG shown no seizures during this time.  What was that? I don’t know.  No one has the answer still.  Could it have been OMS/OMA? I think so. Some more symptoms are fussy, irritability that is not consoleable.  Definately!  He was so fussy he’d sleep no more than 15minutes at a time, he would scream a blood curdling scream that is not really describeable.  It was a sound you’d only hear in a horror movie.  I am not exaggerating.  Coming from a baby, it was weird.  We were dropped by the occupational therapist from Curative twice because she did not know how to work with him.  (This was before I found the best therapy company for Zane: Vision Forward.  Who have worked tirelessly to helping Zane through these issues)  we started giving him anxiety medication when he was 3months old because of this and he still takes it.  If he’d get his dose even 30minutes late the screaming would return.  Nowadays he has worked his way down from 3 doses a day to 2 and if he gets one late, no big deal.  You don’t even notice.   They have bouncy eyes.  This was the one most significant symptom he has always had but did not have when he was born.  He started doing this after his neuroblastoma was removed.  At the conference they described this as… if you saw say someone bring in a big plate of food and you fix your gaze to look at that food then your brain shifts your eyes away from the food, but because you want to see it you switch them back to the food… causing a left and right nystagmus movement in the eyes.  With OMS/OMA the brain shifts the eyes up, down, left, right, diagonal, all over making it a very signature eye movement only seen in kids with OMS/OMA and the movement is called Opsoclonus.  The muscle jerks/tremors are called myoclonus and ataxia is the off balance stager.   Movements controlled by the cerebellum of the brain seem to be the ones targeted which include smiling, swallowing, impulse control, emotional regulation and social understanding.  They are doing some research about the cerebellum (Slow progress because the data can only be found in an autopsy and OMS/OMA is not necessarily a life threatening disorder, very few have died), but there are cells in the cerebellum called “Purkinje” that they’re theorizing may be affected.  This could be that OMS/OMA completely destroys these cells OR intereferes with these cells changing the cellular make up of the cerebellum which is why you cannot see OMS/OMA on an MRI, EEG, or any medical test.  They believe “Time is Tissue” since they do not know the answer to this, it’s possible the longer you wait to treat OMS/OMA the more cells could be destroyed if that is the case.  Otherwise if they’re only being interfered it’s possible to unblock them with treatment such as IVIG, Acth/steroids and chemotherapy.

The next speaker was Dr. DeSena who talked about the treatment options and new research that is out there plus theories of what other treaments may be possible in the future based on what has worked in other similar neurological disorders such as Multiple Sclerosis.  ACTH or Steroids daily, 6months to a year of Rituximab or Cyclophosphamide (chemotherapy) and IVIG (Immunoglobulin infusions) are the current treatment options.  Some kids only need one of the above, some need all.  Everyone has trialed and errored to figure out which one works because not every child responds the same to treatment.  Some may be “super responders” to one treatment and “non responders” to another.  For Zane, I believe he is a super responder to Immunoglobulin and it has worked very well for him over the past year.  I don’t have any plans to take him off it at the moment, but someday may try to see how he does without it.  The autopsy data they have recieved said 1 case reported loss of Purkinje cells in the cerebellum.  another case shown demyelination and IGG deposits along Purkinje cells.  Very interesting.  They’ve also found B Cells in spinal fluid seem to be high in OMS/OMA patients.  They’ve found neuroblastoma patients have an up regulation of “BAFF” at the tumor location.  (This makes me want to have oncology test Zane’s old tumor for BAFF.  They do still have a peice of that in the tissue bank)  He talked a bit about sleep issues and thought it would be good to have the neurologist chime in on his sleep studies to look at what his body is doing during sleep.  Zane has pretty significant sleep apnea still even after he has everything surgically removed that would cause blockage of his airway.  At the moment no one knows how to get him to breathe better at night.  Pulmonary is in charge of his sleep studies, but I’m going to talk to our neurologist about also taking a look on those to see if she can keep record of what is going on.  If for anything , research on if he is improving with IGG Treatment.  Dr. DeSena suggested that when infection is present (when Zane gets sick from something) A short course of steroids is a good idea (Hes always responded WELL to any steroid treatment he’s gotten. or to give IGG Infusion at the onset of infection symtpoms which i just did last Wednesday when I saw he had a runny nose.  He looked A LOT better after the infusion, almost completely better.  One mom noticed something I also noticed with Zane and that is that during his week off when he does not get an IGG infusion, he seems to start relapsing or having more significant irritability, prone to illness and more opsoclonus.  When he first gets his infusions, he looks the best and happiest almost immediately after.

Dr. Brian Weiss with oncology presented some interesting facts about neuroblastoma.  The big one was the kids with OMS/OMA tend to do very with fighting cancer.  Neuroblastoma is the most common tumor for children, but uncommon for a child to have cancer.  It is the most solid tumor for kids under one years old and the second most for all ages (behind brain tumors).  The best way to find a neuroblastoma is probably an MRI.  Children who get neuroblastoma over one years old are almost always high risk cancers UNLESS they also have OMS/OMA.  Sometimes kids will have OMS/OMA symptoms and not be able to find a neuroblastoma.  They could be hard to find because OMS/OMA is so good at attacking neuroblastoma that the tumors could be too small to find.  Most children who have OMS/OMA found their cancer due to auto immune response and symptoms where kids who don’t have OMS/OMA usually find theirs through physical symptoms such as tender belly, bones hurting, etc.  So they are discovering neuroblastoma differently than the “norm” for OMS/OMA patients.  It is high unlikely a low risk neuroblastoma would recyr somewhere else in the body so a full body scan is not necessary.  If it recurred somewhere it would be at the site of the original tumor.

Dr. Went Lopez helped us by giving us some advice on how to handle their anxiety and “rage attacks”.  She thinks its a good idea to have them assessed by a child neuropsychologist (IQ, Global Behavioral, ADHD, Mood/Anxiety testing) to get a baseline asessment to then reasess now and then to check in on any progress made to see if treatments are working or need to be tweaked or if they’re having an cognitive fall back.  What is a “rage attack”  it is basically a fit that no mater what you do it’s completely ineffective at calming them down.  They suggested to know how to recognize a “rage fit” and rages can also be a sign of relapse.  She gave a lot of good reading material advice and resources that I plan to follow up with.  One thing she said that I really liked was, “Parent how you want your child to act at 25, not how you want them to be in 5 minutes”  goood advice!  Thank you! I’m already replaying that one in my head, I think it’s sticking!

Thank you Mike and the doctors who came to speak to us!  Thank you to the parents for showing up and sharing your stories!  I have a lot to work with here and a new found confidence on how to make Zane’s quality of life as optimal as possible.  I really appreciate being invited and being able to make this conference this weekend!  Thank you all so very much!

Annnnnd a picture of Zane (no post would be complete without his cute face right?)

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Zane making such big progress at school!  He puts one foot in front of the other and goes through the actions of walking, he just needs to work on his balance and this kid will be on the go!  (2 and 1/2 months old)

Here is a link to the OMSLife Foundation : www.omslifefoundation.org

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