E1: A terrorist without a belt

Part 2: The problem with the branches

Wherein Florida. Upon a roof. A Cephalopod on a hot tin roof? Not a cephalopod, a breakdancing frog. And then the cemetery up the street and the real problem: the problem with the branches. Also, a bit of herpetology and a spinal tap. On ice, lots of it.

 
 
 
  • Chapter eight. The flip.

     As we walked a few hundred meters, I felt the flip. We were on the other side. I'm used to being the healthy one. The doctor in control. Moving fast from patient to patient, putting out fires, making diagnoses. Prescribing treatments, comforting treating curing.

    Now, we were on the other side. I was the helpless family member watching a disaster. Personal disaster in slow motion. And Danielle was the patient. The sick. The one who waits to see the doctor.

    The shit had hit the fan. I called the daycare. And told them to expect the grandparents and that they would be late.

    Fortunately, we didn't have to wait long. Dr. Wong and my other colleagues came to our aid.

    We had a CT angiogram, a CT venogram.

    An echocardiogram. EKG. Blood work. And then it was over. It was time to go home. Danielle was given aspirin and Plavix. And those two drugs, and her little strokes, and baby Micah, and me, we went home.  Discharge diagnosis. Stroke in a young person. Everything just came crashing down very suddenly. And it was just this whirlwind of, uh, medical professionals and people didn't know what was going on. And that was very disconcerting.

    When we got home, it was late. Danielle and Micah went to sleep in the guest room. The other two kids were sleeping at my in-laws and I started a frantic search for what the hell was going on. Not in a textbook and not an up-to-date and not on pub med, but in the dark anxious recesses of my mind. 

    Chapter nine. Snakes got to eat. 

    I heard a baby crying. But it didn't sound like Micah in the other room. It was a disturbed, deranged animal baby.

    It's years earlier, when I was a kid. We were around the dinner table with my family on vacation and Florida.  It's one of those evenings thick with cicadas and there is this terrible crying coming from outside. A baby? All of us, get up from the dinner table to go and see.

    It's a frog. And it's not even a whole frog. It's half a frog. And it's not on the ground. It's literally suspended in mid air, half on and half off the edge of the roof. You know those two back jumping legs that make a frog, a frog. They are inside the giant gape of a snakes unhinged jaw. It looked so impossible that a tiny snake could open its mouth so wide.

    It looks like the frog has been chased across the roof and just as it was about to jump. The snake caught it and so now the half of frog, just its face and its front legs, they are break dancing. And they're breakdancing in mid air.

    It was almost funny. But it wasn't funny. The frog was screaming.  It knew it was going to die.

    I stood quietly watching the frog for its long engulfing until it went quiet, its face still free.

    It was an awful thing to see. My dad just said, the snakes got to eat.

    And I had forgotten that snake engulfing you from behind feeling until that night after the MRI.

    Danielle was sleeping with Micah and the baby's room. I was in the big kid's room on the rocking chair, staring out into the snowy dark. The house was all quiet. And the snake struck. I suddenly realized what was going on. Stroke in a young person. New migraines with aura. Vascular cognitive dysfunction.

    Cadasil.

    C A D A S I L. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

    I remembered Danielle's mother getting an MRI for headaches a couple of years before. And I remembered the non-specific abnormality in the white matter. And suddenly, it all became so clear. That moment every doctor knows when the long-sought diagnosis crystallizes. "Of course!" you think. How did I not see it before?

    Danielle comes out of the baby's room. She's groggy. “What's wrong?” she asks me. “I figured it out,” I say. “What?” “Remember, your mom had an MRI for her headaches.” She shakes her head. “She did like last year, maybe, it was full of stuff that I didn't think it was a big deal at the time. But not normal. But that, and the headaches and the strokes. Now it makes sense. It's called CADASIL.”

    She's quiet, standing in the middle of the room. She says, “I need to finish feeding Micah.” She does not see the black ocean around her. She does not see she is drowned. The kids are drowned. Genetic autosomal dominant.

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy or CADASIL is a way of your brain slowly losing its blood supply. Inch by inch. Year by year. Function by function. White scar by white scar on your MRI. And when it has engulfed your wife, then it waits. Let you return to dinner. Waits until your children have reproduced. And then it slithers up inside one of them. From its coiled up hiding. And starts its slow, swallow again. And when it's finished with your son and the screeching has stopped and you are sitting at dinner one day, your daughter says she has a headache. Or her hand is tingling. And you watch again. Nothing you can do. The snakes gotta eat.

    At some point, I fell asleep next to Danielle and Micah. And when I awoke, I had forgotten it for a moment. It was morning, you know? Those few seconds. And then it's back. I ran downstairs and looked up her mom's MRI.

    I was wrong. It wasn't an MRI of the head she had had. It was of her inner ears. And it wasn't last year. It was a few years ago and it wasn't for headaches. It was for ringing in the ears. I put my head down on the desk, relieved, but feeling how untethered I had become.

    Yeah. Forget it. 

    Danielle came down with the baby. And I told her to forget about what I had said last night. But she looked at me. And I could see, she didn't know if I was now trying to backtrack and cover up something terrible I'd let slip, or if she could believe me. She just nodded very passively, probably not knowing which she preferred.

    Chapter 10. The problem with the branches 

    I went outside to clear my head, and the outside, by which I mean everything not inside, was completely covered in ice. I mean, everything. The front porch. The steps, the driveway, the cars, the sidewalk, the street. The light poles. Yes, even the verticals. It was like someone had literally come and poured water over the entire world and then frozen it.

    I shuffled slowly down the driveway. A few meters up the street from where we live is a large Mulberry tree that looms over the cemetery wall. Well, its one large branch thick as an elephant's leg was coming off at a weird downward angle. And underneath it, pinned and squished like a bug, totally covered in ice, the neighbors Toyota Rav four. Then the tree limb kept going into the street and block the road. The tree trunk reached up over the cemetery wall. And then two thick as street pole branches, angled up like a Y, and that's where the problem started. From there, the ice had made the twigs easy to break.

    A gust of wind came up and I literally saw the tree shatter in front of my eyes. A pile of twigs and small branches, totally encased in ice came clank ring down like a pile of coins hitting the ground. 

    What I had not taken into account and the terror of the past 12 hours, the MRI, and then the emergency room. And then my late night nightmare was the other part of the MRI.

    Not the strokes, but the problem with the branches.

    The arterial tree.

    Whoever had taken their dirty little fingers and dabbed five strokes onto Danielle's brain had done something worse. They had gone right up to the main arteries going into the brain, the tops of the internal carotids, where they branch into the anterior and middle cerebral arteries, and they had grabbed a hold of those blood vessels and they had squeezed like that Rav four. Pinned and squished down to nothing. Her brain was running on slivers of blood dental floss sized. 

    In 10 years of neurology practice and training, I had never seen anything quite like it

    I remember that MRI at the Western and looking at the scan after and realizing how inflamed that vessel was or how small that vessel was and how big of a difference the inflammation makes.   

    Chapter 11. Ninety-nine red balloons floating in the summer sky, panic bells, it's red alert. There's something here from somewhere else. 

    The next day, Danielle went to get a lumbar puncture. At this point, I knew our lucky balloon had been popped. Our bubble burst. I knew we were in trouble. Our years of good luck had sprung a leak. But things were about to get worse.

    Now, the reason it's called a lumbar puncture is because the lollipop shaped sack of fluid that protects your brain and your spinal cord gets popped and the cerebral spinal fluid that bathes your brain and keeps it nice and floaty and buoyant and protected, it starts to drip, drip out like SAP from a maple tree.

    And that's spinal fluid that bathes your brain, it should be what we call bland. Boring.

    Like water. Uh, still not sparkling. Clear. Calm.

    Danielle spinal fluid was not calm.  

    The white blood cells. The lymphocytes were elevated and the protein too, which meant something in or around the brain was not happy. She had an itis.

    I've always thought the Latinate suffix itis as in meningitis or encephalitis or appendicitis, it doesn't quite have the same searing heat as the more apt, English translation. Inflamed.

    The elevated white blood cell count, that was the smoke. We were seeing the smoke and the spinal fluid.

     The headaches, the cognitive changes, the strokes, her squished down blood vessels. They were the house on fire. She was being officially diagnosed by neurologists who were not her husband.

    Danielle had central nervous system vasculitis and that, my friends, is not a good diagnosis to have. Our balloon had been popped.

    But who had started the fire?

    Where did it come from?

    Who had attacked her nervous system?

    We didn't know.

    She was started on prednisone to quell the fire in her blood vessels.

    Usually, we doctors are not satisfied with patients having natural God-given problems. And so along the course of the patient's illness, we're prone to give them doctor-created problems. Iatrogenic complications like little party favors. Thanks for coming with your autoimmune CNS vasculitis.

    Here's a little more suffering for you. Compliments of Dr. So-and-so. For Danielle, that Iatrogenic party favor was something in the form of a new balloon. In her words, here this time in her head.

    And then what happened from the lumbar puncture?

    Me feeling really sick that night in the shower and throwing up. I couldn't stand it, sick or being in hospital before, um, you know, at least you can sit up and read or watch TV or call someone on the phone or something. But this was like, every time I lifted my head, I would feel, I would get a headache or just feel nauseous, so I had to basically lie down. Otherwise, I would get a headache. It was very discombobulating. I would get not dizzy, but just like, as like, as if I had a really bad sinus cold or my head was full of pressure like a balloon,  a balloon.

    Chapter 12. Central nervous system terrorism. Home grown.

    We moved in with my in-laws and I set up shop at their living room table with my computer. And paper after paper, review articles on CNS vasculitis, I began to read everything I could get my hands on

    Primary CNS vasculitis is not good. It's an auto-immune disease of unknown cause it doesn't kill that many people or disable that many people, but that's not because it's not awful, but because it's rare. In five years of residency, and at the time, six years of neurology practice I had maybe seen it a handful of times before. And now, my wife had it?

    Sometimes very rare infections can mimic CNS vasculitis and Danielle's excellent doctors had checked for these. Gram stain and culture for bacteria. Fungal cultures. Viral pcr viral cultures. But everything came back negative. There was no sign of infection and she had no other systemic illness, no rash, no joint pain. There was no sign of inflammation anywhere else in the body.  It's hard to explain how severely narrowed the arteries were. Going to Danielle's brain. The confluence of the circle of Willis, where the arteries meet each other at the top. Was interrupted by areas. Where there was no blood flow at all. And so her dominant hemisphere was being fed by a left MCA artery that kept getting narrower and narrower on each success of scan.  One night, out of her earshot, I sat on the edge of Danielle's parents' bed. And I tried to explain to them. That Danielle. Might not make it through this. Or worse might have a catastrophic stroke. Leaving her paralyzed. And unable to speak. It's hard to explain the terror that we were living with. Waiting for a bomb to go off, but you can't diffuse it because you don't know where it's planted. And you don't know why it's there and you don't know who put it there. CNS vasculitis home grown terrorism. I hated this diagnosis. 

  • Calabrese, L., Mallek, J. (1987). Primary Angiitis of the Central Nervous System: Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine, 67(1), 20-39.

    Chabriat, H., Joutel, A., Dichgans, M., Tournier-Lasserve, E., Bousser, M. (2009). CADASIL. The Lancet Neurology, 8, 643-653.

    Gonzalez, R., Schaefer, P., Buonanno, F., Schwamm, L., Budzik, R. Rordorf, G., Wang, B., Sorensen, A., Koroshetz, W. (1999). Diffusion-weighted MR imaging: Diagnostic accuracy in patients imaged within 6 hours of stroke symptom onset. Radiology, 210(1), 155-162.

    Kley, N. (2001). Prey Transport Mechanisms in Blindsnakes and the Evolution of Unilateral Feeding Systems in Snakes. American Zoologist. 41: 1321-1337.

    Paul, S., Roy, D., Mondal, P., Bhattacharyya, R., Ghosh, C., Das, S., Krishna, H., Patra, C., Kiran, J., Benito-León, J. (2022). Primary angiitis of the central nervous system – A challenging diagnosis. Journal of Neuroimmunology, 366, 577844.

    Raskin, N. (1990). Lumbar Puncture Headache: A Review. Headache: The Journal of Head and Face Pain, 30(4), 197-200.

    Warach, S., Dashe, J., Edelman, R. (1996). Clinical outcome in ischemic stroke predicted by early diffusion-weighted and perfusion magnetic resonance imaging: A preliminary analysis. Journal of Cerebral Blood Flow and Metabolism, 16(1), 53-59.

    Yuh, W., Crain, M., Loes D., Green, G., Ryals, T., Sato, Y. (1991). American Journal of Neuroradiology, 12, 621-629.

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