Chiari Malformation

It's been quite some time since I've posted. Life got hectic with Zach in school and the twins growing up. I've been through a couple of jobs and finally landed where I think I was always supposed to - in a classroom of my own. The last semester was a difficult trial because I stepped into a classroom halfway through the year, but even on my toughest day, I was still happier at work than I had been for as long as I can remember. I've been offered a position back at the school and can't wait to have my class for the whole year.

Nicholas has been overall stable in his health and has been improving so much and so quickly. He went from a walker to walking to working on going up and down stairs. His vocabulary has increased significantly and he's holding full, really hilarious. conversations. We went through a fairly painless, though time consuming, process to get Nicholas transitioned from the Early Steps program to Orange County Schools when he aged out of Early Steps. He was evaluated and an IEP was drafted. He now goes to a pre-school program in a school near us for a full day program, and even rides the bus from daycare to school and back daily! He loves the bus, the driver, and the bus aide. He is completely enamored with his teacher.

Emily and Zachary both continue to grow into really great humans. Zachary is finished with first grade and chomping at the bit to start second! Emily had her first dance recital and absolutely loved being on stage. Zero stage fright from her at all.

In May, we were in Boston for my sister's graduation from her graduate school program. While there, Nicholas scared us with what we thought may be a shunt malfunction, so we got to visit Boston Children's Hospital. We had heard nothing but amazing things about this facility and can back that up with our own experience. During Nicholas's brief stay (which ended up being a stomach bug that he shared with all of us, not a shunt malfunction), we had to get a copy of a recent MRI for BCH to compare to. Luckily, he had had an MRI in March for a totally unrelated issue. While in BCH, Mike got a copy of the MRI and we were able to read it. We don't typically get copies of his images because 100% of his care is through the same hospital system and all his providers can access his records at any time, so this was the first time we had read over the radiology report from the MRI. On that report, we saw the words Chiari Malformation Type 2. We had only recently been made aware of Chiari Malformation because Nicholas's neurosurgeon's contract was not renewed (much to the angst and chagrin of his entire patient population), and the neurosurgeon is one of the top physicians with knowledge of Chiari Malformation in the Southeast. We had joined forced with the Chiari population to protest the release of this amazing neurosurgeon and thus had learned of this condition.

Chiari Malformation Type 2 most frequently occurs with a type of Spina Bifida. The cerebellum and brain stem extend through the base of the skull and cause severe issues like debilitating headaches, problems in gross and fine motor skills, problems with speech, and feeding issues. We had never heard of Nicholas being diagnosed with Chiari and certainly hadn't seen the majority of the issues that occur with Chiari Malformation, but the feeding issues piqued our interest. We obtained a copy of the radiology report from BCH and Chiari Malformation was listed on that as well.

Nicholas will be having a fairly standard surgery later this month - getting tubes, getting his tonsils and adenoids removed, and getting a cyst in his nose removed. After the ENT appointment where it was decided to go forward with the surgery, we were contacted by the surgeon who, because of Chiari being listed on the MRI, needs neurosurgery's approval to go forward with surgery. The neck has to be hyperextended to remove the adenoids and this could be devestating for a patient with Chiari, so we scheduled an appointment with the partner of our former neurosurgeon to get approval for the surgery. During that appointment, the neurosurgeon confirmed the Chiari diagnosis, though he doesn't seem to have the more severe Type 2. We were told that lots of people have Chiari, it usually doesn't matter until the symptoms start causing problems with the quality of life. Neurosurgery didn't think his Chiari would be problematic during a tonsil/adenoid removal and granted approval for the surgery.

Part of the reason (among others) that Nicholas's tonsils are being removed is to see if it will help with his feeding. Apparently Nicholas has enormous tonsils and the speech therapist thinks that Nicholas may be instinctively protecting himself by not eating due to the lack of space in his throat. The neurosurgeon warned me that with a Chiari diagnosis, the reality may be that he has a symptom of his Chiari that may need to be addressed. We've decided to go ahead with the ENT surgery (since there are other issues that removing the tonsils will resolve) and if his feeding doesn't improve then we need to regroup with neurosurgery to determine how much of a factor the Chiari might be in his failure to feed by mouth.

It was scary and frustrating at first to receive yet another diagnosis, but as family, medical staff, and Nicholas himself have reminded us, a diagnosis is just something written on a piece of paper and doesn't tell any part of the story of this little boy. He's strong, and joyful, and funny, and amazing and if we need to figure out how to address the Chiari then we know that he will face this challenge with the same upbeat, cheerful, and determined attitude that he's faced all other challenges thrown in his path thus far.