LCH (Why I’m Like I Am)

There is nothing on this earth as unreasonably reasonable sounding as being told to start at the beginning. Sure, there are some things with clear beginnings: open the mummy’s tomb, and all of a sudden people you never ever met are suing you for the next fifty years for unleashing some pox on their households. Often, though, the beginning isn’t really a single point in time. And if it is, how can you know? It would imply that you knew something more about the longer outcome — or that there will be a longer outcome. Usually, there isn’t. Correlation does not imply causation, and vice versa.

When it comes to poxes, illness, and just general odd duckery, it’s difficult to picture a single point in time. Every little sniffle or cough or fever or loose tooth might be relevant. It might not. And I can guarantee you, the people who will say start at the beginning will tell you that either all of it or none of it is.

That’s bullshit.

Here’s what I can say for sure, now. I have been ill for a long time — at least ten years, possibly more. I took a real, solid, measurable downturn three years ago, though I didn’t realize it at the time. I suppose it might have been going on longer than this, but truthfully, this is life, and you don’t think about all that stupid extraneous stuff in a longer view if you can work through it other ways. Who has time for that?

I am admittedly a bad patient, because I will gladly ignore anything I can get away with, including shortness of breath, hallucinating, peeing ice cubes,  you name it. I don’t want to be bothered with going to the doctor — I have other things to do. But eventually, it got bad enough it got in the way of the other things I had to do, so I had to go.

And it was all very, very general. Flu like symptoms. Exceptionally normal looking. And that, folks, is sometimes the way it is. No one is negligent (not even me, alas, although it does get you a reputation with physicians and others you really don’t want or deserve). There is nothing to see. It’s hard to fix a problem — or even a symptom — if you can’t identify much about it.  It’s also why it’s extremely hard to buy, after a certain amount of time, that “a diagnosis isn’t important.”

That is also bullshit. The only thing it changes about my situation is everything. It doesn’t change what it was, what it has been, or who I am. It changes everything about how I will then be able to live my life. I had a physician, well meaning, tell me that I had to know no diagnosis was certainly better than a bad one.

No. No, it isn’t.

As far as diagnoses go, mine could be worse. I could also have never gotten sick, but you don’t get to choose that any more than you get to determine what the hell it is that might be going on in the first place. I knew that something, especially in the last three years, had changed radically. Things were breaking that were… not usual. I developed central diabetes insipidus (partial) due to a posterior pituitary tumor (that radiologists often miss on my MRIs). My thymus gland grew from the normal pea size or so to about the size of a lime or hefty golf ball. Because thymomas are associated with other issues that I experienced (muscle weaknesses, shortness of breath, difficulty swallowing, speaking) it had to come out. The removal was uneventful, but for the reaction thereafter… my blood sodium levels bottomed out. This set my electrolytes in a weird spin where I can be peeing up the wazoo (and dehydrating) but my thirst response won’t kick in and I can still have low blood sodium.

So I lost eighty pounds in less than a year (did I intend to? Of course, I had for years! It just didn’t work that way no matter how hard I tried.) I began to sweat at night so badly that I had to change clothes, or sheets, two, three times nightly. I couldn’t pull on socks without turning purple and struggling to breathe. I would get motion sickness, vertigo, walk into walls. And save for the frighteningly low electrolytes (you should never have your doctor offer to send the ambulance to your house when your labs come back) and some slightly off (ever so slightly off) blood counts… nothing too interesting.

But for the tumors. They took awhile. And the trick is, people get lumpy. Usually, though, they don’t find what was spotted last time around. The really funny thing now is that we know what I have — and oh my god, so much random stuff that made no damn sense at all does make some sense in retrospect. It might not be all related, of course (that’s always the kicker.  Just because I have a diagnosis doesn’t mean everything that ever was or will happen down the pike must by default be part of the deal.)

The irony is that while it is apparent I have an adult onset Langerhans cell histiocytosis (not the pulmonary type, which seems to be the one sort of presentation that isn’t so varied), there are lingering questions about what LCH really is. It’s an orphan disease in all senses — not enough kids have this disorder for research funding to be considered feasible — and it is three or four more times likely to happen in children than adults. Just like “cancer” describes a process and the different types of malignancy have their subtypes, there’s a decent chance that certain commonalities tie the histiocytic disorders together in all ages, but truthfully…

There’s a lot of ambiguity, anyway. It doesn’t seem like LCH is cancer in the traditional sense of the word, and there is most definitely an immune system reaction that runs amok somewhere in the course of things (and from what I gather, “runs amok” is probably a better description than venturing a guess on whether it is an over- or under- immune reaction). Better yet, before the doctors saw that Langerhans cells were a common factor, the various presentations were grouped under the histiocytosis X heading. Now, it seems, that while your run of the mill skin/immune type Langerhans cells stain up and look much like ones that gather in these lesions, the guilty parties might be cells that take on similar properties without actually being Langerhans cells to start. Were the Langerhans cells framed?

What it means is uncertainty all round. What it means is learning to roll with what you need to roll as much as is possible, because you have no choice. No one wants it this way. There’s not a whole lot you can do.

I’m at the beginning of the “knowing what I am rolling with” phase. It beats not knowing. Not knowing doesn’t help. Knowing that there’s stuff we don’t know is a little different.

Come ride with me.

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