Where do I start?

We saw the first neurosurgeon a few weeks ago. He feels strongly that Rainbow's headaches can be managed pharmacologically rather than surgically. This is very desirable because surgery is very very dangerous for Rainbow. We have to see a neurologist to get started figuring out if there is a medication that will work for him. Neurologist isn't scheduling until January. Called around, pretty much everyone who is taking new patients is scheduling roughly that far out. We see the next neurosurgeon Oct 26th. Rainbow is no longer allowed to play on trampolines or in bounce houses because his Chiari Malformation makes those activities dangerous. His trampoline is his favorite thing in our back yard. Suck.

Additionally, Rainbow has a "malformation" of his back (his musculature is very asymmetrical and his spine is slightly "bent"). We showed this to his neurosurgeon and he said "Yeah, that's weird. He needs to see an orthopedist." Is anyone keeping up with the doctor count? Appt with the orthopedist in a few weeks. He also has symptoms the neuro doesn't think are associated with his chiari and may be autoimmune (Pediatric Rheumatologist for that one, add another).

Last week Rainbow had a random (very low) fever, no other symptoms at all. We consulted with his immuno and it resolved on its own and we thought "huh, weird." Last night he woke up freaking out because his head hurt (not really all that new) but when I went to put my hands on his head he was burning up. His temp was 102. We spent a good amount of time on the phone with his immunologist's office (the on-call doc was new to us and was freaking awesome) and three hours on the ledge of "which is more dangerous taking him to the ER or keeping him home?" It took ibuprofen and acetaminophen to break his fever but then he slept from 3am - 7am. Woke up a little grumpy but ate and had no fever.

Dr D's (his immuno) nurse called at 8:15 "how soon can you get him here?" Um... crap, that's not terrifying.

His immuno did a full updated history, spent an hour with us, did a full exam, and.... nothing. Like, seriously, nothing. He doesn't even have a runny nose. That probably sounds like a good thing but to us it's scary. We don't know what we are dealing with.

She ordered bloodwork. Being the boyscouts that we are we were prepared with this numbing cream (don't leave home without it). So, we slathered it on and waited for it to do its work. We went to our usual blood draw lab (how screwed up is that, these people know us on sight) and Raz screamed and screamed and screamed. The phlebotomist hit a vein twice, I saw it, the blood wouldn't come. The phlebotomist's acted like this was the weirdest thing they had ever seen, awesome.

I slumped down to the floor and called his doctor. She didn't want him poked again. He's too worn out. He needs to hydrate like crazy and we will try again tomorrow. If it doesn't work we will have to go to the hospital where they have ultrasounds to do this...

He is sleeping now.

The Neurosurgeon

I do this thing when I'm freaking out. I research. I read and read and read and read and look up terms and read some more. In preparation for this appointment I read every paper this neurosurgeon had published in the last twenty years. I also read a bunch of related papers.

I think I do this because it makes me feel like I have some semblance of control over the situation, and because I'm a scientist. Research is what I do.

The thing is, in all of these papers, cases where Chiari Malformation was successfully managed non-surgically were never mentioned. We went into this appointment certain we were looking at an inevitable neurological surgery, on our immune deficient three year old. On the drive there we were both jumpier than a long tailed cat in a room full of rocking chairs.

So. Much. Fear.

Then, a crazy thing happened. We were given hope. We were given hope that our child may get to live a normal(ish) life without anyone ever slicing his head open...

We spend A LOT of time in doctor's offices. While we've developed strong relationships with Rainbow's regular doctors and feel heard by them anytime we see a new specialist we brace to be rushed and brushed off. It's exhausting and frustrating and infuriating. Dr. Mapstone asked tons of questions, examined Rainbow, then sat with us comfortably in absolutely no rush and talked with us about his evaluation. He took Rainbow's immune deficiency very seriously and factored that into his decision about whether or not to recommend surgery. He answered every question we had honestly. We felt heard and we felt that this doctor was going to make decisions with Rainbow's quality of life and health as his foremost concerns.

The bottom line is that there is a very strong possibility that we will be able to manage Rainbow's Chiari Malformation medically, avoiding surgery, possibly forever. If we can, working with a neurologist, find a medication that manages his pain without unacceptable side-effects then he will have an MRI once a year to evaluate his Chiari and go from there. The doctor made it very clear that if his pain cannot be managed or if he develops other symptoms Rainbow needs to come back to him as soon as possible.

We have a plan.

There are so many unknowns and this neurosurgeon was also very upfront about the controversy in his field regarding the best course of treatment for Chiari. Some of the other neurosurgeons with whom we've consulted are more aggressive in their treatment and we still have an appointment at Dallas Children's next month.

We will be adding three more specialists to his team to evaluate an apparent spinal malformation, to possible remove his "huge" tonsils, and to determine if he has Ehlers Danlos Syndrome or possibly some other condition causing the odd sensations and pain in his limbs.

But... No one is going to be cutting his head open in the foreseeable future and I'll take that.

Until next time...