Fixing a Chiari Malformation Part One: Processing.

Yesterday was a long, harrowing day. Husband's father says its always good to be a surgeon's first patient of the day. If you know my husband and his dad, being prompt isn't an aspiration, its a sin. Being early is always best, which yesterday we were. On the other hand, my sister says being late is better, regardless of your surgery order. I understand both of their reasoning this morning.

We were early and second in line for surgery (that's the royal we or is that the author's we? or maybe just "wee" because I am still tired and haven't had enough coffee). Husband's check in time at the hospital was supposed to be 6:30am Thursday morning, but for my husband it was 6:09am. It would have been 5:58, but there was a line at the surgery check in desk.

I don't know what time we were taken back to "processing", which is basically where they make you wait to get to the pre-operative area, get fully admitted by your pre-op nurse and get naked, dressed in one of those horrible (yet fashionable?) hospital gowns while listen to the hustle and bustle of nurses, techs patients and alarms. I can't tell you what time it was then, but I can tell you that the nurses were looking forward to lunch.

If you want to calm a nervous patient, this is not the place nor the time to do it. The communication is nill, you get checked on by someone who has no information about how long you will be waiting before something very scary happens and the standard answer is "I'm not sure". I don't think in the six hours my inlaws, husband and me waited in the pre-op area that any confidence in the unfamiliar hospital and its staff was gained. Lost, maybe, but definitely not earned.

The coffee stand did have excellent coffee, but they should have given away a free Xanax for every surgical patient family member. Or maybe a shot of something to take the edge off. Even better, they should have given my husband something to relax him and something for the pain he endured going without medication since 10pm the previous night. The nurse did finally ask if his pain level warranted a medication, but not until 30 minutes before he left for surgery.

You know what's great? I was reminded as different as my husband is from his dad, they still have many similarities. One of those is an inability to cope when situations and circumstances are out 100% out of their control. It was just a little scary when we got to hour four. Not to mention, my silly sense of humor isn't understood by my father-in-law all the time. He must think I'm nuts at this point. My using re-framing to deal with my husband's wait time wasn't understood much either, so I finally explained very nicely that having a big, clear picture shown to you in a different light is helpful and calming. And then I had a cigarette or two because I needed a break. I love my fathers, but sometimes they need a woman's perspective in a blunt way. That was not the time. And my father in law still thinks I am nice. I think...  

But finally he did leave for surgery. An orderly who educated us on how and when to steal stuff from the hospital (even though I didn't see anything worth stealing) zipped us up to the Preparation Area, or rather the husband got to go there and I was given the world's fastest instructions to the waiting area. Naturally, after all this wait, my inlaws had gone off to eat lunch meaning they missed this part.

I checked into the much nicer surgical lounge (why they call it a "lounge" I have no idea because no one looked to be lounging) then ran off to find my inlaws. I was entirely too nervous to hit the right buttons on my cell phone. Actually, I was so nervous that it took me a good 5 minutes to find an elevator to the cafeteria. And naturally that meant I couldn't direct my husband's parents to where they needed to go.

Epic Daughter-in-law Fail. I went to the car for a smoke (sorry, this has been sooo stressful!) and called everyone that I had intended to call post surgery at the time they expected to hear outcome to say "yay, he just went into to the prep area". My kid said "okay Ma, call me in 4 hours". Typical Asperger's-- memorized the length of the surgery and wanted no more info. Now if everyone were like that...

Part Two will follow later. I have 30 more minutes of solitude and coffee before we go visiting (so, you know, happy ending and all).

The Pooping Brain

We are at the two week mark. On the 19th, my husband gets to have life-saving, or perhaps quality of life-saving surgery to fix the issue of his hind-brain pooping itself into his spinal canal, mostly. Who knew that 7 months ago, when I started this absurd blog to add more comedy into our lives that it would sorta sum up our family's biggest issue since the whole Cape School District went all Lifetime movie on us?

The greatest thing about my husband's rare, congenital medical condition is that its a rare congenital medical condition. Most doctors only read about it in text books. Google has only 381,000 results when you search the term Chiari Malformation. To give an comparison, Cancer garners 345,000,000 hits. Specific cancers results are still in the millions. If you google my husband's specific for of Chiari there are a meager 7,690 hits.

Trying to explain the whole kit and caboodle to people is difficult. My husband doesn't look sick. He doesn't sound sick. Unless you live with him, you won't notice that he has trouble finding the right words to express his thoughts and slurs them ever so slightly. You don't realize that if he lays his head back too far, he sorta just passes out. You don't see him in moments of dizziness or mental fogs. And he won't tell you about the pain that makes the simplest things difficult-- like getting out of bed or getting out of the car. The symptoms of Chiari Malformations are subtle and most cases can be managed non-surgically, except with the type that is progressive-- his type. That's the kind normally discovered during autopsy.

I'm not sure if the rareness is because its uncommon or that it's subtly means doctors miss it. It's only diagnosed by MRI, ya know, where they see the brain kinda in the wrong place. The way its diagnosed means that before MRI, you were kinda outta luck and therefore, studies on live patients was near impossible. On top of this, there is no recognized or accepted course of treatment...or rather, there is no particular protocol for treatment. Thus, treatment recommendations can vary widely from doctor to doctor.

It gets better: Of patients that have surgery, only 53% are successful and don't need additional surgeries. The rest need surgeries to fine tune or add to the original surgery. This is neither astounding or comforting, except that the surgery will keep my husband's brain from killing him at worst and destroying his neurological function at best.

The one thing that I can say is that if you have a rare condition, every doctor, nurse and tech that treats you gets a medical boner. My husband is officially a rockstar patient that has essentially livened up medical careers of a dozen or so people in the medical field. This is not a bad side-effect of his condition. It's actually reassuring that he is on the mind of everyone on his treatment team-- from his internist to his neurosurgeon, everyone is on the same page. Normally coordination of care is something a patient has to advocate for himself, but not in my husband's case. The medical world of slower, lower Delaware is falling all over him.

Even the kid's neurologist went the extra mile with us at my boy's last med check. Dr. Genius-Awesome-Pants took time out to discuss my husband's surgery and reassure us everything would be a-okay. I know that if I have a question and can't get in touch with any other doctor, Dr. Genius-Awesome-Pants will be there to help.

His words helped the other day:

"You've got a great doctor who knows what he's doing. The surgery is more common than you'd think. With this, they aren't poking around in your actual brain. They do the decompression and fix the covering over the brain, and that's that. It's not like grabbing a tumor where one wrong move damages you. It's structural and the only big risk is infection, barring that, you'll do fine. The youngest patient I've had with Chiair surgery was three and she's 100% recovered now". 

An example of the incision aka "zipper"
Don't know what happened to this photo-- sorry
to whomever I borrowed it from if I did so without credit!! Opps.
Feel free to contact me for my apologies.

Not only was it important for the kid to hear, it meant a lot for us parents. Of course we are worried. It's not like getting your tonsils out. I fell in love with my husband's brain, so I don't want it to change. I just want him to live, have a quality life without pain, and live happily ever after.  I want to laugh every time he uses brain damage as an excuse for doing something stupid, without the "what if" factor. I don't want his brain to poop out of his skull anymore.

We have two weeks until the surgery. We have a world of support and are busy making the preparations for all scenarios. My sister is coming Monday so I can breath a little easier. I can run away when I need to, I can have support when I've calmed everyone's fears.

Another example

Two more weeks until my husband becomes a "zipperhead".

I am updating this to add my husband's actual zipperhead scar. Please feel free to share this image with others to spread Chiari Awareness. This was my purpose in writing this post, along with processing this surprise that turned our family inside out.

His scar is smaller and not zipper-like at all. I don't know if it was done endoscopicly or if my husband's skin was more pliable due to his dramatic weight loss. I do know that he has sutures underneath his skin that extend about eight inches. These have not dissolved yet, so he is still healing and recovering. Life with Chiari is certainly different, but at least it is life. Here is Dr. Sugarman's handy-work:

Regular

With a diagram!!

If you'd like to use you either photo in your own blog, share it with friends and family or show to anyone else you think is fabulous or anyone needing an awareness education, feel free to distribute, but please, no alterations.