Chiari Warrior

What?  How do you pronounce that?  Kee-AR-ee

My sweet and very brave Chiari Warrior  eleven year old son is approaching his year Zipperversary(6.9.15) of having brain surgery.  He was diagnosed with Chiari Malformation and Syringomyelia more than a year ago. Tomorrow we will journey four hours north of here  once again to meet with his neurosurgeon.

While recent MRI images and reports were not what we had hoped they would be, we will remain optimistic and determined to do whatever is necessary to keep him living a healthy and happy life.

“I’m just a normal kid who had brain surgery.”

Rather than write a ‘dumping’ story, I’d like to try and educate readers on this rare brain disorder to the best of my ability as a non-medical professional.

Alternate Names:

-Arnold Chiari Malformation(ACM)

-CM

-Tonsillar Ectopia

-Hindbrain Herniation

Subcategories:

There are 5 subcategories of Chiari ranging from Chiari 0-IV with CM 1 being the most common.  It remains on the list by NORD(National Organization Rare Disorders) as a rare brain disorder and  affects approximately 1 out of 1,000 people.

CM is a structural brain abnormality at the back  of the skull and spinal cord. It is believed that the area at the base of the skull is too small or narrow which results  in the hindbrain(cerebellum) and brainstem being pushed downward into the spinal canal.  Currently, doctors believe this to be a congenital defect, it can also in rare cases be acquired via trauma or injury.  Through my own personal research and meeting countless numbers of people impacted by Chiari, it also appears there may be a genetic link.

Chiari

LT-normal brain  RT-Chiari & Syringo(Syrinx)

Once the cerebellum and/or the brainstem begin to protrude down into the spinal canal, this can potentially create a spinal fluid blockage.   Some CM patients may or may not be symptomatic.

My son’s herniation was measured at over 20mm;  with  significant csf blockage resulting in Syrinx in his spine.

IMG_1024

His actual MRI image showing Chiari

 When there is csf blockage, this can also cause a secondary problem called Syringomyelia(Syrinx) which are fluid like cysts that develop in the spinal cord due to built up pressure. Overtime these cysts can get larger causing nerve damage potentially leading to paralysis if left untreated.

Symptoms:

While every CM/SM patients symptoms vary, the following are some of the more common ones.

Headache|neck pain/stiffness| blurred vision|numbness/tingling in arms or hands|syncope|ringing in ears|sleep apnea|difficulty swallowing|vertigo

My son’s symptoms- seizures|numbness/tingling in arms hands| neck stiffness|coordination & balance issues|

Treatment:

There are a number of variables that factor into the best course of treatment for CM patients. They include but are not limited to:

-drug regimen

-physical therapy

-monitoring of symptoms

-surgery

It is usually a combined team effort with your General Practitioner, Neurologist, Ophthalmologist, Radiologist, Physcial Therapist and Neurosurgeon to determine the best course of action.

It was determined that surgery was necessary for my son based on a number of factors; cerebellar herniation, csf blockage, syrinx  in spine, symptomatic and his age.

 There are a number of ways to perform this surgery and I certainly am not  by any means an expert or one to elaborate in this area.  chiari surgery

The particular surgery that my son had was three-fold:

1.) decompressive suboccipital craniectomy

2.)duraplasty

 3.)cervical laminectomy.

Phew, that’s a mouthful!  In laymen’s terms, his neurosurgeon cauterized the cerebellar tonsils, cut his C1 vertebrae, trimmed part of the base of his skull and opened the dura to alleviate pressure.

too much brain

Prognosis:

There is currently no cure for Chiari however with prompt diagnosis and proper treatment, most CM patients go on to live relatively normal lives.  While the medical professionals continue to further research the origins and best treatments for CM/SM patients,  so much more research and awareness is needed.

My one personal piece of advice from this experience is NEVER ignore symptoms.  Many people kept telling me- “there is nothing wrong with him” and  “you’re crazy.” Listen to your gut instinct!

My son has to live life a little bit differently now(he’s not so happy about not being able to ride rollercoasters or go on trampolines anymore)- it is our hope- it is my hope as a Mother of one brave boy -that through our experience that we continue to provide awareness, assistance and grow to appreciate that their scars  are worn with tremendous pride knowing a Chiari Warrior has won!

IMG_4142

#42  “No Excuses, No Quit”

Additional Resources:

Mayfield Center :

http://www.mayfieldchiaricenter.com

NORD: http://rarediseases.org/rare-diseases/chiari-malformations/

Conquer Chiari:

http://www.conquerchiari.org/index.html

*Numerous support groups on Facebook

IMG_2666

 

Be well.  

 

 

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