5. Nonarteritic Anterior Ischemic Optic Neuropathy
… is a terrifyingly intricate way to say “ocular stroke.”
An ocular stroke occurs when the optic nerve experiences a marked, sudden reduction in blood flow and, therefore, oxygen. It can be part of a larger stroke that affects other areas of the body, or a standalone event limited to visual sensitivity.
Like traditional strokes, ocular strokes vary greatly in severity. The condition affects approximately one in 10,000, including a prominent New York Times opinion writer named Frank Bruni. In February 2018, Bruni published a poignant piece strikingly titled “Am I Going Blind?”
The entry’s introduction eloquently showcases the mortifying, unheralded alarm the disorder evokes: “They say that death comes like a thief in the night,” he writes. “Lesser vandals have the same M.O. The affliction that stole my vision, or at least a big chunk of it, did so as I slept. I went to bed seeing the world one way. I woke up seeing it another.”
Bruni describes the vision loss as “a thick, dappled fog” affecting the right half of his visual field, which caused not only extreme blurriness but a sort of off-kilter feeling that left him “felling drunk without being drunk.”
Ocular stroke victims live not only with vision loss but also a lifelong sense of foreboding, because having one significantly raises the likelihood of suffering another. Bruni reported that, over the next five years, he faced a 20% chance of a similar event in his remaining good eye. In other words, he has a one in five chance of becoming functionally blind.
The pattern of my vision loss, however, did not suggest a stroke. While it remained a “maybe” on the diagnosis docket, doctors kept searching for a likelier suspect.
4. Devic’s Disease
One of the disorders that most closely mirrored my visual symptoms was, unsettlingly, among the most frightening prospects.
Also known as neuromyelitis optica, Devic’s Disease can be fairly described as multiple sclerosis on steroids. Visually, while MS tends to attack only one eye with optic neuritis (episodic flare-ups attacking the optic nerve), Devic’s Disease typically presents with substantial scotomas (blind spots) in both eyes.
My vision loss was bilateral (both eyes), and a neuro-ophthalmologist confirmed my optic nerves were thinner than normal. In layman’s terms, the cameras (my eyes) were fine but the cables were frayed, significantly blurring the feed.
Devic’s is, simply, brutal. Like its cousin, MS, it is characterized by immune attacks on the optic nerves and the spinal cord. Unlike MS, Devic’s tends to rob large chunks of both vision and mobility without warning – a relapse/remit pattern that mimics MS but with more debilitating episodes. Depending on what sections of the optic nerve and spinal cord are affected, symptoms can include blindness in one or both eyes, weakness or paralysis in the legs or arms, painful spasms, loss of sensation, uncontrollable vomiting and bladder or bowel dysfunction.
An MRI of my spine came back… inconclusive. Unfortunately, the next step was infinitely more painful: a spinal tap.
The fluid was normal. No MS or Devic’s… not yet anyway. The search continued.
3. Coloboma of the Eye
Derived from a Greek word meaning “curtailed,” colobomas comprise a set of conditions in which normal tissue in or around the eye is missing from birth. This can include incomplete formation of the macula, lens, uvea, optic nerve or, oddly, the eyelids. Visual impairment can run the gamut from virtually asymptomatic to virtually blind.
If confirmed, mine would a moderate case of the optic nerve variety, which involves an abnormal optic passageway that is somewhat “excavated” or hollowed out. Though it is present from birth, it’s not uncommon for mild or moderate cases to go unnoticed until adolescence or early adulthood because, as a doctor explained to me, “you can only see the world through your eyes.” My poor eyesight was normal to me; it took flunking some eye exams to show me it was demonstrably worse than average.
On its face, the notion of a birth defect made some sense, as my mother suffered from preeclampsia – a condition that can cause dangerously high blood pressure – while carrying me. (In fact, three years later, during her second pregnancy, she died from it.) Preeclampsia can choke off oxygen to fetuses, resulting in birth defects.
However, more commonly colobomas are genetic, and there wasn’t evidence of them running in my family. With no formal diagnostic test, another disease was added to the “maybe” column. It was a good maybe, because colobomas typically don’t worsen.