I Took the Road Less Traveled By: Histiosaywha?

So after the sweet GP saw me, she sent me off to get myself magnetized in the giant picture taking tube of magnets. I went for a double load of fun with an MRI and MRAs of my head and neck (arteries, nothing scarier than seeing a disembodied carotid or two hanging there on a screen, folks).

Surprise, I also was scheduled for an appointment today (booked just before the MRI went on) to see the neurologist. First, sorry, I love you internets, but I called my mom first before I said anything. Cause, like, she’s been waiting too.

The images had been read, and the neurologist — who I know I gave a bad deal in November because my stand in GP didn’t send records, and that ain’t fair — said he really wanted to see the images before he read the report.

His idea was this: I am a freak of nature. I think it’s funny because he winced when I said it. He thinks (now, who the hell would have mentioned this before?) that the diabetes insipidus is the real key here. Why the hell did that happen? And better yet, why did it happen with all this other weird random crap?

Because (stop me if you heard someone who is friggin’ brilliant say this before) these are an odd combination of symptoms that do sort of… point in a direction. It’s just one of those directions that no one goes. No one goes there for a damn good reason.

What popped in his mind? Ahem. Well, histiocytosis X — which is I guess technically not the term used anymore, because they’ve started to piece together the different sorts of disorders that fall under histiocytosis like disorders. Some are malignant, but then they end up being called sarcomas I guess… Mostly they gang up on little kids… under the age of 10. It isn’t really something that comes up a whole lot with adults. It can kill you (and be warned: sites do have patient pages on this stuff, and most people make them for their kids. It is sad. It is absolutely devastating, but it is a sort of thing that bodes worse for the under two crowd). It doesn’t have to kill you. It can make life miserable. I am gathering it doesn’t have to.

There are lots of different types… and we don’t know for sure if that’s what that stupid little intensity change on my pituitary means (okay, it wasn’t little. I mean… he actually didn’t need to point it out). He isn’t sure, but he’s pretty sure it isn’t normal given the circumstances… Keep in mind there’s some silliness that 25% of the population has a pituitary tumor at any point in their lives with no real sign. Most people who do have symptoms  from pituitary changes don’t count diabetes insipidus among them. One almost immutable fact about histiocytosis kinds of infiltration: there is a huge incidence of central diabetes insipidus.

But it is a weird sort of thing. It was categorized as a cancer, but it isn’t always. I mean, it behaves like one, and it grows stuff on organs and such… Many adults with Langerhans Cell (who weren’t kids with LCH previously) have a single lung nodule (I’m not saying, I’m just friggin’ saying). They are also smokers, the story goes, but I tend to wonder if it’s just because they are more likely to biopsy a smoker’s lung than a non-smoker.

But it isn’t a cancer, and they’ve learned a lot in the past few years, so oncologists aren’t so much “in” on it — especially with adults. It tends to be handled by hematologists, who all tend to be in the oncologist business — this is also pretty understandable.

The belief is that it originates (like some cancers) in the bone marrow (bone lesions are pretty par for the course. My skull looked okay. but adults and kids are often different in the bones affected). At heart, it is an immune disorder, or at the very least, a nice indicator for an immune system gone stupid.

But oncologists, hematologists, and immunologists don’t necessarily deal with it.

The chances of Langerhans’ Cell happening in an adult (past 10 years old or so) I guess is a one or two in a million (so buy a lottery ticket today!) The non-Langerhans’ histiocytosis shebangs are even less common. Erdheim-Chester has a registry of some couple hundred people worldwide. My money is on it being more common — but sometimes things don’t ever get figured out, never mind in time, so who knows?

If you’re one of those people inclined to look (and I know some of you are), it is recommended no stock be taken in prognosis for adults (hell, don’t pay attention for the kids, either… just enjoy your kid and do your best, because doctors can’t predict much of anything sometimes and timeline never make anyone feel less pressure) . The sample pool is small, and someone probably peed in it anyway. Treatment — and reaction to it — is different, and has varying levels of success. The idea is that there is a history of success, and it is real, honest to god success. Well… I mean, no one gets off this ball alive anyway. I also have a skewed view of success — but I was over the idea that this was something curable ages ago, folks.

There are other disorders (such as neurosarcoidosis) that cause these things, and again, we’re getting to the point that they’re all weird and treating them is a variation of seeing what works (often steroids, low doses of chemo, MS medications).

The neurologist is… well, dammit, he was sweet. There. I said it. I mean, he was nice enough when I saw him in November, but I’m not a good patient to dump on someone unannounced.  The doofus rounds doctor who called him in with my low blood sodium (she thought I had erratic blood pressure… no, I have low blood sodium, dumbass) tipped him off that I was more interesting than he’d thought. He was downright warm and friendly in the elevator. Today, he was very… god, dare I say it, tender (he still can’t pick up the phone and didn’t apparently have laryngitis the other day when they tried scheduling me though, just  sayin’).  He inquired about Frank the Bunny’s take on my diabetes insipidus… He saw her note in the system, and he suggested that histiocytosis X was a likely suspect at this point.

Except this time, I wanted to hug him when I left his office. I mean…. I know. Freaky.

He’s asking a couple of radiologists to take a third and fourth look at my MRI — the report didn’t mention any oddities, but he thought it looked odd and (ahem) he said while the person reading made a difference only in theory, some readers are more theoretical than others. It’s easier to miss an abnormality than pick one out (bet it’s harder to detect something that isn’t there ought to be, but hey).

And because I am weird, and he has the contacts, I am going to MGH. He is running my case by his colleague there, and I’ll be sent through for work up. But he knows this person, and this is what this person does — well, weird brain stuff — and the idea is that I won’t necessarily be treated by neurologists, but the neurological aspect is now showing up on imaging studies. They won’t necessarily need to go digging in my brain (hands off, folks) because the beauty is that… well, I have other lumps (sigh) and usually skin and lymphatic systems are involved if diabetes insipidus is present.  And removing the growth doesn’t do much.

So we have someone (well, two someones, as the Dorktor had thought this was possible back in the day before this all hit the fan) who figures that it has to be one of these disorders. The evidence to support it comes from what’s been seen and what hasn’t, and a plan is starting to take shape. It’ll take time.

We told him that at this point, time is not an issue so long as the plan moves forward with it. Determining what we’re dealing with to the best of everyone’s ability will aid in treating and managing. Treatment is usually… well, it takes a while to have an effect. I can be patient if the other parties are putting in their two cents.

I wanted to hug the neurologist. I feel dirty.

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