Syringomyelia - MRI images to explain Auggie's condition

Syringomyelia (SIR-RIN-GO-MY-EE- LIA) is a disease that causes pockets of CSF to form in the spinal canal. These pockets can get bigger and cause obstruction to the flow of CSF fluid and eventually permanent damage to the spine and eventually paralysis if left untreated. Auggie's syringomyelia is caused by an obstruction in his brain. His cerebellum droops down in his brain stem and there is very little CSF flow around the bottom of his brain.  Decompression surgery can help restore this flow by makning more room in the back of his brain. In his case, the two top vertebrae were removed, part of the back of his skull was shaved off and his cerebellum was shrunk using electrocauterization,  The third image shows a rounded cerebellum after it was shrunk and a much smaller syrinx.

 Before surgery, the syrinx (the white fluid-filled pocket in the spinal cord) is about 110 mm long.

 3 months post-op the syrinx is 55 mm long and 12 mm wide

9 months post-op the syrinx is only 45 mm long and 8 mm wide.

Auggie has started Pre-K!

Auggie continues to amaze us!  He started Pre-K a few weeks ago and has adjusted so well.  He was never able to stay at preschool without me by his side, so we were dubious about Pre-K. But, to our amazement, on the first day of school I brought him into the classroom and showed him his desk. As he started playing with playdough and after I said goodbye to him he gave me a kiss and said, "okay, bye mama". Not only that, but he now also takes the bus to school!  It was his idea!  A year ago I never would have thought it possible.

He has an IEP with 2 qualifying conditions: Other Health Impairment is primary and Speech and Language is secondary. His Pre-K class is geared towards children who need help with speech and language. There are nine children and four staff! This gentle introduction to school has been the prefect fit for our little Auggie!  I don't know what will happen after this year, but we have to take it one year at a time and do what's right for him at the moment even if it means having kids at two different schools and at opposite ends of the city!

Chiari malformation, the silent disease that no one has ever heard of

It's Chiari (Kee-r-ee) Malformation and Syringomyelia awareness month and time for a post about Auggie's particular symptoms of this disease.  Even though he was decompressed a year ago he will always have chiari malformation.  The space created in his brain to relieve pressure and allow CSF to flow better is small.  He will never have the same pressure free flow in his brain as the typical person.  He is going to live with this for the rest of his life and some days will be bad, very bad.

Yesterday he complained of a really bad headache at 4 PM, he was in agony. He does not take medicine by mouth and he would have nothing to do with a suppository. At 5 PM he was vomiting from the pain and nausea that the migraine headache was causing. He vomited copious amounts, twice.  He was too nauseous to eat dinner and only managed to drink some of his "milk" before bed.

On most days he complains of foot and leg pain. He says that his feet feel like they have splinters in them. This is pain is probably only going to get worse as he gets older and there is very little at this point that can be done to help him feel better.  Much more research is needed in this area.  Please help spread awareness about this debilitating disease!

Blood coagulation results

Auggie's blood was checked again for various coagulation issues - all of them have improved!!  His platelets are stable at 138,000 - it's on the low end of normal, but stable.  His platelet function test came back normal as well as his CBC numbers. His Factor VII deficiency has improved from 60% to 85%. His PAI-1 improved from 3 to 4, which is still low but not in a dangerous way.  He does not even need to wear a medical ID bracelet and might not need medication before having dental surgery in October.

Hearing loss

Auggie is getting hearing aids! Our new audiologist, contrary to his old one, believes that he could benefit from wearing a hearing aid.  He has bilateral sensorineural hearing loss at 800-2,000 mhz.  We hope our late discovery hasn't affected his speech - we'll be seeing a speech pathologist affiliated with the clinic in a few months - Yes, we have to wait that long!!  He has had speech therapy for 3 years but no one ever thought his hearing was compromised.  It's always good news, bad news on this roller coaster ride.

9 month Post-op MRI Results

Auggie's 9 month post-op MRI looks good - his CSF flow has improved and the syrinx has decreased by about 60% since pre-surgery. He will need another MRI in 12 months.  I'll post images shortly.

3 month Post-Op MRI shows good news!

Auggie recovered after his constipation and is now back on track with eating and drinking.  We never did the endoscopy because he started eating again.  He is still on miralax - about 1 tablespoon daily to prevent constipation. It seems to be working.  The MRI in December showed that his syrinx has shrunk by about 30%.  This is great news. The next MRI will be in July and we hope to see further improvement then.