Episode 66: A Familiar Interest, NOT Obsession - Mary Dell MD (Medical Detective) and the Neurolobitch

Okay, okay, okay.

I know my loyal viewers (all zero of you) will know that I do tend to wax diagnostic when it comes to my health problems. So much so that, for quite a while, I was convinced my neurodegenerative symptoms (with numerous periventricular white matter lesions on MRI, hyperreflexia, mild myotonia on EMG, and other neurological signs) might well be caused by myotonic dystrophy.

Given my family's physiologic resemblance to the facies of persons with myotonic dystrophy, the logic of a DNA test seemed, pardon the pun, a no-brainer, but apparently not to the neurologist aka neurolobitch I saw at Johns Hopkins Medical Center's Neuromuscular Clinic about a year ago. (For more on that miserable experience, see Episode 44. I still haven't named her "for real," but I'm sorely tempted these days.)

As anyone with a "mystery disease" knows, it's frustrating when a definitive diagnosis is not found for the profound, disabling changes to one's body and, at times, spirit--though I do my utmost to ensure that I don't also succumb to depression, no matter how hard some days can be.

Add to all that the fact that I worked (when I used to be able to work) as a medical librarian, among other careers, and in those years gained expertise in researching medical topics for physicians, other health professionals, and the public.

So, excuse me. I think an occasional foray into the possible causes of my day-to-day miseries is hardly obsessive. Of course, tell that to said Dr. Neurolobitch at Johns Hopkins.

The fact is, my once-subtle neurological symptoms may have started very early in life, beginning at 18 months with severe dehydration to the point that I was in quarantined intensive care for three full days. The dehydration had been caused by a severe virus during which I'd apparently puked and shat myself clean through.

Viruses are possible causes of multiple sclerosis and other neuro diseases, so perhaps that was the culprit.

Popping up again at 5 years old when diagnosed with amblyopia due to strabismus, a common eye condition in children that is caused by neuromuscular weakness. My cross-eye, or squint, is not overly noticeable, thank goodness, but I am legally blind in my left eye (God preserve the right!).

You can see my strabismus (cross-eye or "squint") here--
it's getting worse as I get older!

Hinting at itself here and there throughout my childhood, characterized by frequent sick days from school and undiagnosed ADD, another neurological condition. My early report cards, as well as many of the issues I dealt with as a kid, are textbook for ADD, and I've been diagnosed as an adult. I have a fairly full-blown case, actually, and it's been exacerbated in recent years, probably from the white matter lesions in the ol' noggin.

Rearing its ugly head again when happily pregnant with my first child at 19 years old and unexpected complications that could be attributed to neurological problems (and are commonly found in pregnant women in myotonic dystrophy, which initially led me down that path) nearly cost me my pregnancy and my firstborn son, who was born prematurely, given a 50/50 chance to survive his first 24 hours, and himself developed within a few months a severe neurological condition, craniosynostosis, along with a cerebral hemorrhage that left him partially paralyzed from the time he was an infant. (J. is a miracle story in himself; that is a story I need to write--but I'm hoping he'll write it himself, now that he's an amazing 35-year-old man.)

My second pregnancy three years later was also difficult, though thankfully full-term, with odd problems such as an almost total inability to eat, very little weight gain, and the most debilitating headaches I've had since (though I had migraines in my 40s as well).  Second-son A. was born with a couple of issues and developed a few more in childhood, including ADD.

Remaining mostly silent through the rest of my twenties--but who would know when in that one decade I experienced the birth of said beautiful son J. at the age of 20, dealt with his myriad physical problems, including two neurosurgeries at 7 and 9 months of age, had my second beautiful son A. at the age of 23, dealt with his health issues, went through another neurosurgery for J. when he was 5 and I 24 years old, lost my dad to a sudden heart attack when I was 25, separated from (and eventually divorced) my husband also when 25, went through my mom's breast cancer and mastectomy beginning a year or so later, and then lost my mom to liver and pancreatic cancer when I was 29.

The only significant neurological issue I can think of during the joy of my twenties (SARCASM, Sheldon!) was the onset of a very weird sleep problem I only recently identified as hypnogogic hallucinations. These are true hallucinations that happen as one is falling asleep due a criss-cross in the brain states of wakefulness and REM sleep. They are associated with narcolepsy, which I actually qualify for based on a Multiple Sleep Latency Test (MSLT) administered a couple of years ago; however, my diagnosis is idiopathic hypersomnolence (IH) based on that same test. The test has you sleep for a while during the day, then wake for a bit, then sleep, then wake, etc., and the amount of time it takes for you to fall asleep during those naps is calculated. Anything less than 7 minutes is diagnostic of either narcolepsy or IH, and my average was 4 minutes.

Fortunately, the hypnogogic hallucinations, which were absolutely terrifying (men breaking into my room to do me harm, and they were just as real as anything I've seen in true wakefulness), stopped after a couple of years. They began the night Mom died, though that first one was actually very comforting and arguably NOT a hallucination but a visitation from my mom, who came to the side of my bed and told me not to worry, she was fine. The ones that followed, though, were beyond frightening.

In my thirties and early forties, I began experiencing a profound lack of stamina. I honestly thought it was because I was getting "old" (funny in retrospect, now being fifty-something!). My co-workers told me they worried they'd walk by my office and see me dead at my desk--that's how bad I looked (and felt). I attributed my fatigue to my rather full load--I was a single mom of two beautiful, challenging boys, a summa cum laude college student cum part-time secretary cum program associate/coordinator cum graduate student cum grant writer cum graduate student in a second discipline cum academic reference librarian cum medical librarian cum English professor cum world traveler (Europe, Mexico, India).

After sending a Christmas letter out during my thirties, I received a reply from a friend who told me my letter sounded like a "cry for help." It probably was. But I would not be denied. I wanted it all. No doubt, stress has played a huge role in my failing health.

One day in 1996 a nurse in my office took my blood pressure and told me to go to the Emergency Room immediately, where I clocked in at 183/113. Oh, it got much worse over the years, but that was a startling number for someone who'd always had normal pressure.

My uncontrolled hypertension would take a big toll on my life from that point forward, resulting in numerous hospitalizations, heart problems, and a series of medicine cocktails that would keep my pressure fairly normal for a while until they didn't, and then it was out of control. My last semester teaching, it was running 200+/120+ much of the time.

It took thirteen years for the reason for this uncontrolled hypertension to be discovered. Is it any wonder I don't always trust what even the best doctors are telling me?

After all, son J. had been UNdiagnosed by his pediatrician even when I'd raised concerns about his head size and his refusal to use his left hand for anything, including reaching for toys. Trying to reassure me, I guess, the good (ahem) doctor told me, "Oh, he's just a right-handed baby with a funny-looking head."

Um. WRONG. A second opinion revealed J.'s craniosynostosis and left hemiparesis and was followed by those neurosurgeries and years of physical therapy.

I repeat:  Is it any wonder I don't always trust what even the best doctors are telling me?

So, I was almost fifty when I finally learned the cause of my hypertension--an adrenal problem known as hyperaldosteronism that actually begins with a problem in the brain. A problem, in fact, in the hypothalamus, which for some time I've theorized is the culprit in many of my problems. (See Episode 46 for more on that pesky hypothalamus.)

In my hypothalamus, according to my docs at NIH, an abnormal secretion of the hormone CRH begins a cascade of disasters that results in my adrenals becoming hyperplastic (too big, with too many cells) and secreting too much of the hormone aldosterone, which leads to intractable hypertension, heart problems, and god knows what else. I'd never been happy with the "essential hypertension" B.S., so finding the cause was good if a little late for my heart and kidneys.

The hypothalamus, by the way, is known to cause many of the problems in multiple sclerosis (MS), which my neurologist says he'd diagnose me with if my MRI had shown lesions in my spinal cord and not just the confetti he'd found in my brain. However, I've since learned you CAN have MS without spinal lesions, so maybe, in fact, that IS my mystery disease.

Also during my forties I'd learn I have Hepatitis C, obviously not a neurological problem, but one I'd acquired quite possibly due to these underlying neurological problems when the placenta after J.'s birth did not fully detach from the uterus and led to severe bleeding--just one of the weird problems during that pregnancy that are, in fact, listed as common in pregnant and birthing moms with myotonic dystrophy, as well as other neurological conditions. I was given two D and C's (uterus-scraping procedures) during which I needed extra pints of blood (transfusions) and acquired the Hep C virus in those early 1980's days before the blood supply was screened. Thank God it wasn't HIV.

No doubt the Hep C doesn't help matters, neurologically or otherwise--but I hope I'll be a candidate for all these new treatments coming out.

Finally (I hope), in my forties and early fifties I would be diagnosed with my arguably most severe health problems, which I've described on The Mary Dell Show ad nauseum, so I'll just quickly list them: cardiomyopathy, kidney disease, and this mystery neurodegenerative disease (among other, somewhat less serious conditions).

The heart and kidney issues aren't surprising, given the years of astronomical hypertension I've endured. And, by the time my neuro problems were diagnosed by the eminent neurologist Dr. A (the antithesis of Dr. Neurolobitch, by the way), I wasn't surprised. I'd done hours and hours of research on my signs and symptoms by then and was certain many of my problems originated in my brain. 

All in my head, yes. But not made up, as Dr. Neurolobitch implied during my consult with her.

My point in recounting all this is that I now believe my neurological issues began much earlier than anyone, including me, had thought--from the time I was a baby in quarantine with a nasty virus, perhaps.

Another point that I cannot help but bring up is the way Dr. Neurolobitch treated me during the 40-some (if that) minutes she consulted with me. Given the numerous correlations of my health problems (and some in my family) with those of myotonic dystrophy, I don't think a DNA test for that condition was out of order. In fact, I'd found medical guidelines--the Bible for docs these days--that pointed to exactly that.

In addition to that good (ahem) doctor's skepticism about every single thing I told her, she informed me that my neurological exam was completely normal--this despite highly reactive reflexes the neuromuscular fellow in the room commented on several times.

My local neurologist had already found hyperreflexia, so Dr. Neurolobitch's ignoring this calls into question every single other thing she "didn't" find during her exam.

Dr. Neurolobitch held firm to her NO DNA TEST decree, so that I am now paying, or would be paying if I could find the name of the credit bureau to which my bill has been referred, for having the DNA test done on my dime, despite having no income at the time due to my disability and now drawing a meager but life-saving amount each month from Social Security.

It was no skin off the doc's perfect white teeth for her to order that test, and I've seen forums in which that test was, in fact, ordered for patients at Hopkins with only hints at myotonic dystrophy, but she was far more interested in being RIGHT and putting me down, given my medical librarian theories, than she was in following proper guidelines.

Finally, to appease me that day, Dr. Red Queen (as she is known in my previous episode on that visit, in reference to my series on today's medical system--Down the Rabbit Hole) said she'd send me for an EMG (a painful test with needles stuck into your muscles) that she was certain would be normal.

Wasn't she surprised when that test DID indicate mild myopathy? Even with that, she still wouldn't order a DNA test for me, which is why I still owe money for one.

Although Dr. Neurolobitch Red Queen did end up being right about the myotonic dystrophy--and thank God, since the main reason I wanted that test was that I was concerned about my sons and nieces, since the disease is hereditary, her supercilious attitude was way out of line.

I dunno, I think curiosity is an important attribute in a diagnostician. Instead, this woman had mocked me from the moment she walked in the, an experience unlike any I've ever had in my many, many doctors' visits over the years. The most overt was when I told her about the cranial neuropathy I'd been experiencing--a weird, electrical impulse shooting down the side of my nose and across the top of my teeth--and she cut me off, saying "Yeah, yeah, yeah."

I shit you not. I was as shocked as you are. But it happened.

After being the object of her obvious, shrivelling skepticism about that and everything else I said to her, and then, adding insult to injury, telling me my neuro exam had been normal, I asked her,

"Well, why then did my neurologist say he'd diagnose me now with MS except that I have no spinal lesions?"

The look on her face was priceless, but she had a ready answer: "I've been consulted about myotonic dystrophy. I have nothing to say about that."

Really?

No matter what my referrals might have been for when I've seen other doctors at Hopkins, they've all at least attempted to find out what might be the cause even if it wasn't exactly what was thought at first.

Even the best (and most gorgeous) diagnosticians go down a few closed alleys now and then before arriving at a correct answer. It's called the scientific method.

Now, HERE's a diagnostician!
And don't point that thing if you're not going to use it, Dr. House!

Any patient is due basic respect from a physician. I'd be fine with deleting every snarky reference to Dr. Neurolobitch if she'd admit she'd fucked up when she saw me.

In fact, I'd say the Red Queen had more to learn from my visit to her than I did.

I doubt, though, that she's the type of person who could admit she SUCKS at taking histories and treating patients as human beings.

So I'm a medical librarian that came up with the theory of myotonic dystrophy and was wrong.

Well, guess what? Dr. Neurolobitch's perfect arc of a life that led her to an important position at Johns Hopkins didn't prevent her from also being WRONG--about my hyper-reflexes, my myotonia, and my diagnosis of degenerative neuromuscular disease.

And wrong about me. I am not the attention-seeker, time-waster, and malingerer she obviously believed me to be.

Maybe I'll never have a definitive diagnosis. I've seen enough posts about patients seeking diagnoses, particularly in neuro conditions, to know that mysteries are not always solved.

However, after reading that MS, in fact, does not HAVE to include lesions in the spinal cord, I think I'm just going to say I have MS from now on.  I certainly have many, many of the signs and symptoms, and it's easier than saying to someone, "Well, I have some kind of neurodegenerative disease that no one can find the name for."

Simplifies things, doesn't it?

And, while labels can be damaging, anyone with a mystery disease will tell you that a diagnosis is hugely helpful.

I, at least, remain curious, but I'm also resigned. Whatever is happening isn't going to be cured, whether it's MS or myotonic dystrophy or any other neurodegenerative disease. It just would be nice if someone cared enough to help me find out what it is. Is that too much to ask of the so-called best physicians in the nation? Is it too much to ask that they, too, be human?

From now on, I'm having my docs refer me to the University of Maryland Medical System rather than Hopkins. As physicians in a teaching institution, maybe they will demonstrate enough curiosity to help me find out what I can call this thing that is taking away my life, day after day.

___________________________

P.S. Curse you, Blogger!!  I had a much pithier version of this episode published that I lost, and there's NO WAY to get previous versions. Talk about frustrating! Time to start composing in a word processor. What a pain.

Episode 65: Healing Nature, Ferns, and Fairies

Healing happens when we ingest certain foods and medicines, but many other factors come into play when a person is fighting for his or her health, as have I for some time.

Healing, however, does not necessarily come from what we put inside our bodies; healing can come from without. My wonderful massage therapist Hania is a case in point--after an hour under her ministrations, I feel like a new woman.

In my case, though, the greatest sense of healing comes to me while in the forest. Yes, many of the plants I've identified in my woods and yard do have medicinal properties, but in this case I'm talking about healing on an aesthetic and spiritual level. And this is where ferns and their allies come in.

Ferns are not edible except as fiddleheads, the newly emerging sprouts in the spring, and only two or three species of ferns produce palatable fiddleheads:  the ostrich fern, lady fern, and, arguably, bracken. Eating fiddleheads of other ferns may make you sick.

But ferns are healing to me without their ever touching my body, much less going inside it. A woodlands filled with ferns and their allies, moss and Solomon's seal and Jack-in-the-Pulpit, to name a few, is not just a forest; it is a sanctuary.

Although there's no lake in my yard, my woods remind me of one of my favorite lines by one of my favorite authors in one of my favorite essays--one I can't decide whether or not to teach this fall given its "old-fashionedness" (however, this is also a point in its favor).

In "Once More to the Lake," E. B. White describes an early morning on the lake he'd visited as a child. Of moving through the waters in his canoe, E. B. writes that he is "keeping close along the shore in the long shadows of the pines . . . being very careful never to rub [my] paddle against the gunwale for fear of disturbing the stillness of the cathedral."

That's how I feel about my woods. A cathedral with a ceiling painted not by Michelangelo but by the filigreed limbs of maple, basswood, and black oak.

I have been unaccountably blessed in life to have purchased my modest home, my "cottage," as I refer to it, which sits on the edge of a forest. With its northeastern exposure, my wooded mountain slope produces countless members of woodlands species.

It is here, while I sit under the green hardwood canopy on one of the huge sandstone boulders scattered across my property, that the stresses of hypertension, cardiomyopathy, and neuromuscular/degenerative disease fall away from me. No longer am I Mary Dell, individual human with compromised vigor and health; I am a part of the forest, this vital ecosystem, this magical place where fairies still roam.

I breathe in the ancient air, knowing my landscape of ferns and moss is one of the oldest to exist on this planet. I breathe in the rich scent of humus--that moist, nearly black soil resulting from the slow decay of yearly leaf litter, fallen from trees that in autumn turn my little mountainside gold and red. I breathe in the eons of this planet in this sacred place. I breathe in the healing that comes from nature's undiluted elements.

"My" woods--highlighting hostas; I will post a new
photo filled with ferns and moss soon! You can see
a few fern fronds on the right here, though.

I'm thinking that "nature therapy" needs to become more emphasized for the chronically ill--for anyone who is in need of healing, in fact, whether mental or physical. The idea formed (though I realize now it's not an original one on my part) after watching a Facebook video in which several firefighters grant the request of a man dying in a hospice. They roll his gurney with him, gaunt and pale and looking hundreds of years old, lying there, from the pavement to a wooded path, and then they take him through the woods. The man's dying wish was to be outside among the trees and flowers and underneath the blue sky, not a bright white ceiling with blinding hospital lights.

Where would you rather die?

So that got me thinking. It's a crime, really, how we close up oldsters in ever-smaller spaces that have little to no access to the wild, wonderful outdoors--or even a nice, tame park. I'm struck when watching older television shows, and some more current ones from the UK, in which persons trying to heal spend time in sanatoriums or other spaces that include expanses of grass and gardens and woods--and the sick persons can be found outside on a bench, or even sitting in a wheelchair, not stuck in bed in a tiny airless room.

Nowadays, sick equals small, supposedly sterile places. And that's just wrong.

In fact, I found a Web site, naturetherapy.org, that describes the very approach I envisioned. It's somewhat annoying that they have trademarked the term "Nature Therapy," which seems so simple it shouldn't get a trademark, but so be it. At least the healing arts are on to this wonderful approach.

The healing aspect of the forest doesn't end when I leave it. My healing also comes from learning all I can about the plants in my woods and anywhere else. While it's certainly possible to find healing in simply sitting outside and soaking up the sunshine and perfumes and microscopic things-we-know-not that check the degenerative processes (I have to believe), another way of connecting with and becoming part of "nature" is learning as much as we can about it. (I use "nature" in quotation marks because we are ourselves a part of nature, of course; we cannot truly be separated from it--but we humans do a very good job of removing ourselves from our Mother Earth with structures and pavement and ideas favoring urban life and man's "primacy" over everything else on this blue marble.)

Jean-Jacques Rousseau, the French philosopher whose ideas helped spawn the French Revolution (and the U.S. one, actually, through English writers who took up his ideas), called botany "the salutary science," or the "remede dans la mal" (remedy for illness). That was in the 1700s, for heaven's sake! Why have science and medicine taken us so far away from our valuable relationships with the plants that surround us and through which our planet, and we humans, breathe?

Well, so much for philosophy. No doubt you'll hear more of these ideas on upcoming episodes.

In the meantime, here I will mention the ferns I've found on my property, which may or may not be of interest to you, Dear Viewer--but call this my virtual plant collection. Rousseau also recommended this nearly lost diversion; gathering and pressing flowers and plants was a pastime of his while in exile from France for his revolutionary ideas. This is a gentle activity that can be done even by the likes of me, as weak as I've become in the last few years.

And so I've started my pressed plant collection by attempting to preserve fern species in my yard. I'll post photos if they turn out.

Ferns are notoriously difficult to identify. Since I haven't positively identified all the ferns in my yard, I'm listing some that may well grow there, based on descriptions and photos from a wonderful Web site, Discover Life.

I'll put a question mark next to those I'm unsure of and, if I make a positive ID, will then take the question mark off. Obviously, all of this is far more useful to me than you, so feel free to go on to another episode of The Mary Dell Show!

Ferns Found on My Property

Eastern Hayscented Fern - Dennstaedtia punctilobula

Glade Fern? - Diplazium pyncocarpon

Goldies Woodfern ? - Dryopertis goldiana

Intermediate Woodfern? - Dryopteris intermedia

Interrupted Fern ? - Osmunda claytoniana

Lady Fern?

Marginal Woodfern ? Dryopteris marginalis 

Mountain Woodfern ? Dryopteris campyloptera

New York Fern ? - Thelypteris noveboracensis 

Northern Maidenhair Fern - Adiantum pedatum

Rock Cap Fern - Polypodium virginianum - I discovered this darling little fern on a boulder behind my house and transplanted a small amount to a rock in my woods-edge garden. It's done extremely well and has even spread a little.

Spinulose Wood Fern ? -  Dryopertis carthusiana

Virginia Chain Fern ? - Woodwardia virginica

Ferns I've Found In the Area (Not in my Yard)

I am more certain of these IDs because they were impressive finds, and ones that don't look much like others.

Lycopodiella appressa - It took me a while to identify this strange-looking plant. Little did I guess it's a type of fern (well, fern-ally, actually)! I found it in the Savage River area near Honey's summer place on the River.

Hanging Clubmoss, Creeping Cedar,  - Lycopodium digitatum - (Actually, a fern ally, not a fern per se) - This is a creeping plant with needles or leaves (I'm not sure which) that look like cedar. This grows very close to Honey's summer place on the Savage River. He tells me of a man his mother had seen for many years, a sort of mountain man, who knew "everything" about the woods. He would collect this pretty plant and make wreaths of it for Christmas.

Ostrich Fern - Matteuccia struthiopterus - This is a real beauty, and a Very Big Fern. Ostrich fern fiddleheads (early sprouts) are one of the two species that are palatable when sauteed.  I've seen this fern growing in many places in Garrett County, Maryland, one of the state's three Appalachian counties and the county next to mine.

One day, on my way to teaching English at the community college, I stopped on the roadside and tried to dig up one of these oversized ferns, but I couldn't dig deeply enough to actually uproot it! They like very moist areas, even growing in streams, and I got my feet wet that day to no avail!

Honey's sister has a huge swath of very large ferns in her yard, and she bequeathed us some when she and her husband thinned them out. I don't think they're ostrich ferns, however, because they don't have that huge, meaty root. A few of these to-be-identified large ferns ended up over at the cottage, and I can't wait to see them take off. If they're happy there, I should have a nice swath of them as well, since I planted them in a moist, shady place similar to their home in Honey's sister's yard.

The woods behind my home are filled with ferns, and I've been transplanting a number of them into the yard proper. Every moment I spend in those magical, fairy-filled woods makes me stronger inside and out.