Questions to ask at first Pediatric Chiari NS Consult- 27mm herniation

[MLShelly]

My 5 year old son Jacob was diagnosed with Chiari I Malformation in July 2012 and we are scheduled for his first Chiari Specialist NS consult tomorrow morning.  I hoping that someone could share any "tips" on good questions to ask to make the most of this appointment.  

Here is the background so far.  We are already required to bring all MRI reports, films and related medical records/related history to tomorrow's appointment.   Jacob has thus far been asymptomatic to pain and has a 27mm tonsillar herniation and borderline Platybasia (due to angles measure at base of skull on Brain MRI).  Spinal MRI came back normal (no syrinx, tethered cord or scoliosis).  It was not mentioned on the brain or spinal MRIs but my untrained eye (comparing him to "normal MRIs) I would say that Jacob has a retroflex odontoid, slightly compressing his brainstem but it was never noted by two (non-Chiari) Neurologist or regular Radiologist.  According to me only, the top of his cervical spine seems to curve at the C1/C2 area and pressing on the brainstem).  

Jacob has experienced developmental delays since he was 13 months old, including gross and fine motor delays (Hypotonia), Sensory Integration Disorder and ADHD-like behavior (previously had the following additional issues which are now resolved: delayed Speech, Cognitive, Self Help and Social Skills-all now considered age appropriate for a 5 yr old).  Prior to learning of the Chiari, Jacob also had four unexplained acute "breathing" episodes all at school earlier this year.  As a result he was found to have both a mold allergy and a premature heartbeat (atrial contraction) with no cardiac restrictions.  We are not certain if the breathing issue is Chiari or environmental (school mold) related yet since this occurred 4 times in a single month (before his Chiari diagnosis) and no breathing episodes since and never outside of school.  

We are meeting with two Chiari Specialist Neurosurgeons in mid September (tomorrow) and another mid October to discuss Jacob's new Chiari diagnosis for the first time.  Thanks for any advice you might be able to provide.

[selmaS]

  Appeal to the ins provider that u need a specialist and there is not one in state....I would try that b4 taking on the costs....that way u get them to a true specialist  to make sure they have the best care.

This dx is frustrating enuff but to deal with ins is the cherry on top we do not need or want....

Good luck and keep us posted on how this Dr at JFKNI is.....

[MLShelly]

Thanks Selma- These are all excellent suggestions.  

To all- The one issue that we have for now is that my kids are insured through a NJ Medicaid HMO that has no out of network benefits and there are no out of state providers.  I've heard that you can request authorization to go out of network but the HMO has to first convince the Neurosurgeon to accept their really low Medicaid reimbursement (which I can't imagine happening).  So tomorrow we're seeing NS Thomas Steineke at JFK Neuroscience Institute in NJ and then NS Neil Feldstein from NY Columbia Presbyterian (I'm paying cash for the 2nd consult since out of network).

I do have the option of transferring my kids from their Dad's court order insurance coverage (where I now pay nothing) to my employer's on January 1st. The first problem with this is that they might say that Chiari is a pre-existing condition with a waiting period.  Second problem is that I screw myself over financially for the long term- currently paying zero for kids insurance and moving them to my coverage will end up somewhere around $20K per year out of pocket until the kids are adults because both have so many different Non-Chiari medical, behavioral, dental and prescriptions.  

[selmaS]

   Hi and welcome to the Chiari forum.

First since ur DS had breathing issues please look into sleep apnea as with children this seems to be a bigger issue as they do not recognize it to tell us...but this is very important...have a sleep study done, and since he is so young u may want to consider a home sleep study...look up NovaSom and ask the Dr about it , it should be completely covered by ins.

Next u want a CINE MRI to see if he has a CSF obstruction.....as for asymptomatic a child that young has no idea what "normal" is so he may have pains or odd sensations , even blurred vision at times and since having it since birth will not know to complain about it. We adjust to what we always have and accept much of it as our "normal" and do not know other wise....so do not mistake him  not crying out in pain as not being symptomatic.

  The Platybasia  also known as  basilar impression is not uncommon for those with chiari....and to have it also retroflexed  deff look at  Ehlers-Danlos as he also may have cervio cranial instability.

Being in NJ u can travel in a few directions toward NY to TCI, or to MB to Dr Henderson, or NC to Dr Rosener.....

Please see our list of Drs compiled by the members here and research them and see a few and compare...find the right Dr for ur DS and make sure they r well experienced with chiari and all the related issues.

In the meantime have his pediatrician get the sleep study done.