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Doctoring, Eyes, Raccoons and Health Insurance

I GET WEIRD THINGS. Last year my right eyeball blew a pupil and lost sensation. The MRI was normal and in two weeks it all went away. No diagnosis.

Earlier, when I had tried allergy shots to make life more livable, I suddenly lost circulation to a finger and random joints swelled and hurt. It might have all been related to my immune system, but we never really knew. Six months later, the neuro-ophthalmologist attributed it to ciliary ganglionitis, but even that name tells what, not why.

During the summer I seemed to need more light to read small print, but on August 1 I had a normal routine eye exam, with the same glasses prescription I’d had for years. On August 17, my life-partner, Jim, quit his job and with it went our health insurance.

Six days later, I noticed light-bulb-like flashes, followed by light gray ”lace” that obscured parts of my vision when I lifted my coffee cup to drink. It took me two days to figure out that the coffee cup in my right hand was obscuring the vision of the right eye, allowing me to see lace-like scotoma in my left field of vision.

By August 25, my left eye, forehead and bridge of my nose ached. Before calling my doctor, I polled my hospital friends, with a consensus of a not too worrisome vitreous detachment.

My eye doctor, Dr. ClassicGoodDoctor, knew better, and demanded that I get in to see him. His even-tempered, non-committal demeanor turned to consternation as each test and possible diagnosis didn’t pan out. As I was doing special retinal pictures, he called Dr. RetinalAboveandBeyond, who was driving home and detoured to Dr. ClasicGoodDoctor’s office to see me. Very intense and thorough, he concluded I probably had a “white spot disease” destroying the pigment layer of the retina. He gave it a long name that I memorized.

His partner, Dr. RetinalB, saw me three days later for a fluorescein eye angiogram. It showed diffuse white spots, indicating inflammation, on the left eye retina. Some critical areas were spared, but the rest looked like the Milky Way. Dr. RetinalB and his partners decided it was a different long name, which I also memorized.

No one knows what causes it. The “lace” and lights were characteristic of retinal diseases, but he couldn’t explain needing more light to read, the eye ache or the disconnect between my visual defects and the angiogram’s pattern of inflammation. He reassured me that it would resolve in 2 weeks to 5 months and he would fight the DMV for me if any permanent visual loss threatened my driver’s license.

Since I was curious about potential inflammatory causes, given the weird happenings earlier in the year, he suggested I contact the expert at Northwestern University in Chicago, Dr. RetinalMaximoChicago, for the latest theories.

All this time I worried about health insurance. I had been denied when I was 45 years old because of an asthma attack at age 16. Who was going to insure me now with a weird disease and bizarre inflammatory events at age 57? Making a decent amount of money is irrelevant, even as a doctor, if the insurance industry thinks I’m a bad risk. I could go as broke as the next guy from catastrophic healthcare expenses.

In spite of a trial of Prednisone and Naprosyn, over the next week tiny random diamond-like lights (quite delightful, actually) and moving ”lava” (a bit 1960s hallucinatory) appeared and the gray lace turned much darker. It now obscured my vision, whether the right eye was covered or not, and the left eye ache and pain in the bridge of my nose were worse.

By September 10, the lace was black and it seemed that I was looking through smudges on glasses. Two days later, I saw another partner, Dr. RetinalC, who noticed optic nerve swelling and pallor. The eye angiogram looked very different, with inflammation next to the optic nerve and much worse at the top and gone from the bottom of the retina. He decided I had multiple sclerosis and strongly suggested another MRI.

My neurologist, Dr. Neuro, said I didn’t have MS, didn’t need another MRI and should go to see the expert in Chicago. I emailed Dr. RetinalMaximoChicago and he responded right away, even though he’s Chair of Ophthalmology at Northwestern University.

He suggested I make an appointment or send him my films. I made a tentative appointment for September 24, pending the results of my next angiogram on September 18. Since there is no cure for white spot disease other than time, I didn’t want to dump my patients, cancel other planned events and rush off to Chicago if all he would do was ooh and aah and say, “Yup, that’s what you’ve got.”

I stopped the prednisone since it wasn’t working and was just making me manic and sleep deprived. By now I was seeing wavy blue/magenta distortions of my computer screen. The ”lace” fluctuated, but the most my left eye could see of anyone’s face was a right eye and ear, part of a cheek and some hair. My right eye, which used to be my bad one, was doing all the seeing, so I wasn’t seeing much at all. For the first time since childhood, I had to wear glasses.

At the September 18 appointment with Dr. RetinalB, the retinal angiogram was again very different from the others, with much more active inflammation around the optic nerve and some clearing elsewhere. This appointment led me to two realizations. The problem of needing more light to read actually made the bright light of the exams and angiograms very tolerable. The slit lamp didn’t even make me blink. But somehow each eye exam and angiogram transiently intensified the left eye ache to the point of misery.

Dr. RetinalB remained committed to his diagnosis, in spite of its rarity and his lack of experience with it. When he heard that I was thinking of seeing Dr. RetinalMaximoChicago he said, “He’ll tell you the same diagnosis that we have. Say hi to him for us.” I felt dismissed.

Coming face-to-face with an unknown force destroying my vision, I considered all I would lose if it spread to my other eye. My work, seeing my grandchildren, whitewater canoeing, the play of light on the Grand Canyon’s walls … I didn’t want to be dismissed, especially when the diagnosis didn’t fit.

I had been reading about white spot diseases, of which there are various types. None of what I read said anything about eye pain and none seemed to describe progressive vision loss and changing angiograms like I had.

The next day, with much worse, now black, ”lace” and vision, I Fed Ex’d the angiogram pictures and eye history to Dr. RetinalMaximo-Chicago. His immediate reply said, “You have very unusual eyes. I need to see you. It’s not an emergency yet, but I wouldn’t wait a month.” So I rearranged my schedule, canceling patients and events and flew to Chicago.

By this time, I’d become my own employee so we could get health insurance through the staff leasing company I use for my office. It would kick in quickly, but so would the new $5,000 deductible. This was going to be expensive, but at least not financially devastating.

At the Northwestern clinic, a Fellow took one look at my abnormal pupil (called Marcus Gunn, seen in syphilis and other optic nerve diseases), pale optic nerve and severe visual loss and asked why I hadn’t had an infectious disease evaluation. Dr. RetinalMaximoChicago concurred. More pictures and another angiogram, which was again different from all the others, led to a referral to a neuro-ophthalmologist, Dr. GruffButNice. (Parenthetically, a retinal appointment with angiogram in Sacramento was billed to insurance around $1,000, while Northwestern University only billed about $350. Hmmm.)

After much discussion with the folks back home, I was admitted to the hospital in Chicago. Dr. GruffButNice never examined me, but the Resident-in-training was exceptional, took a good history, did a reasonable exam and genuinely acted interested in my plight. They did a lumbar puncture, complicated a bit by my arthritic, scoliotic spine.

The post-angiogram eye pain, the worst it had ever been, improved by the next morning, but the post-lumbar puncture headache sucked. While in bed for the mandatory hour after the lumbar puncture, my nurse never responded to my multiple calls for relief. After the hour, I took my limp dish-rag body to the doorway, and begged another nurse to get me some relief. That’s a headache you don’t want to get.

Though they felt I had Lyme disease, they sent blood and spinal fluid for umpteen other tests also. The spinal fluid was not inflammatory, so I was discharged. Dr. GruffButNice called with the results in a week. The vision was worse again the next morning.

On October 1, I got an unexpected call from Dr. RetinalMaximoChicago saying that he had just thought of the diagnosis — Diffuse Unilateral Subacute Neuroretinitis (DUSN), caused by a roundworm quite common in raccoons.

We discussed this year’s possible exposures, including camping at the base of Mount Shasta in August, visiting my sister in northern Arkansas (and swimming in the lake) where raccoons are prevalent, and backpacking the northwest Washington State coast. Later that day Dr. GruffButNice called and left a message that all the tests were normal.

That night I couldn’t sleep. Visions of the worm reproducing in my eye and its babies setting up shop in my other eye sent anxiety bouncing off the tent walls. I emailed Dr. RetinalAboveAndBeyond. He was unaware of all that had happened since the first angiogram, but was totally willing to believe the diagnosis, saying, “One has to think ‘worm’ to make the diagnosis of worm.”

He took it all very seriously and emailed a bunch of references about DUSN to me and I searched many more online. We took advantage of Starbucks’ Wi-Fi for hours, instead of hiking. Many of my symptoms matched other DUSN cases, but it seems that everyone is different, with variable degrees of optic nerve involvement, inflammation, retinal pigment damage, visual loss and eye ache. Apparently the worm travels around, releasing toxin that damages the retina and optic nerve. The motile little bastard had spewed toxin wherever, leading to changing angiograms and intermittent eye ache.

Back and forth emails led to an appointment late October 5. Dr. RetinalMaximoChicago met Dr. RetinalB at a New York meeting both were attending and explained his diagnosis. Dr. RetinalB’s email to me seemed skeptical, reminding me that no one had seen a worm on any of the pictures, but saying he would see me Monday.

We had planned a hiking vacation to the less-traveled areas of Zion National Park in Utah. That week-end we backpacked into absolutely gorgeous Kolob Creek canyon. The next morning, on a side hike without packs, Jim lost his balance on a boulder, fell backward into me, and we both fell backwards over rocks and into the stream. He landed on soft me.

Every one of my limbs got beat up by the rocks. I could barely stand on my right ankle. (A later x-ray showed a small fracture.) I “hiked” 6 and a quarter miles out on the sprained ankle, while Jim carried my pack.

Using hiking poles to hop out of the canyon took five and a half hours, during which I noticed that I could see through only a fourth of the left visual field.

I cried. For five and a half hours I worried that they wouldn’t find the worm, that my symptoms would again be minimized by Dr. RetinalB, and that I would eventually go blind in that eye. I planned to change doctors and go anywhere in the world to get my eye fixed. The doctor who originally described DUSN and its cause had died, so Latin America, where DUSN is relatively common, seemed the next best bet.

At the Monday appointment, Dr. RetinalAboveAndBeyond took control. Like a kid at an Easter egg hunt, he announced that a plain color picture showed the worm. He laser photocoagulated the area, but could see the worm on the retinal surface waving at him, then diving deep. The follow-up picture showed the worm moving away from the lasered area. He re-lasered a larger area. This time it worked because the worm was closer to the deep pigment, where the laser works better.

Jim says the whole thing was like Star Wars, with audible and neon green laser zaps fast and frequent, each causing an odd kind of deep eye throb. I kept limping back and forth between the picture room and the laser room. The follow-up picture showed no worm.

I received a steroid injection into the side of my eye to reduce inflammation, but it didn’t feel like it helped. The prolonged procedure and possibly the worm spilling its toxic guts led to intolerable burning pain, like someone holding a match to my eye for hours. Profuse watering soaked the eye patch. I finally slept after taking pain medication left over from my last surgery, which had only required two pills. Compared to this eye pain, the surgery was a mere scratch.

The next four days felt like I had just come out alive after an Indiana Jones adventure. I either slept or couldn’t do anything. I spent the time in x-ray, CT scan, the sinus doctor’s office to evaluate the bridge of the nose pain and the pathologist’s office, looking for a worm in the nasal scrapings.

Dr. RetinalMaximoChicago went back to Chicago, looked for a worm and found it in a totally different place than we had. He sent the picture to Dr. Worm, an expert at Vanderbilt in Tennessee, who ID’d it as Baylisascaris procyonis, the raccoon worm, as opposed to Ancylostoma, a dog hookworm, which is the other cause of DUSN. Mine was the larger one, 1–2 mm in length and maybe a micron in diameter. Tiny.

I discussed the need for anti-worm medication with infectious disease specialists, Dr. Friend and Dr. UCDavis, who each gave me completely different advice, neither having ever seen the disease. I started the medication at the dose described in DUSN studies. The eye ache resolved over the next three days, so I was sure the worm was dead and didn’t have any friends, at least in my eye. Follow-up pictures confirmed no worm, but I knew the Wicked Worm Was Dead by the lack of ache and the slightly improving, rather than worsening, vision.

Turns out there are raccoons and Baylisascaris in all the areas I’ve visited this year and in Sacramento where I live, but none of the eye doctors here have seen this disease. I don’t eat dirt or play with raccoons. More people must be ingesting the worm and not getting the disease, or there is something special about the people who do get DUSN that allows the worm to do its damage… or people have the disease but are not getting the correct diagnosis.

Moral #1: If the doctor is ignoring symptoms because they don’t fit his or her diagnosis of choice, see someone else, preferably an expert willing to listen and find a diagnosis that fits.

Moral #2: All doctors should experience being a patient, especially the fear of an unknown diagnosis and the problems of communication.

Sequel #1: Dr. Worm in Tennessee said that any visual improvement should occur within a few months, but there is no guarantee the optic nerve and retinal layer will regenerate. My ”lace” turned back to gray, became less dense and now obscures only the nasal half of the visual field. It has been stuck there for two months now.

Sequel #2: The worm pill I took is the only medicine in my life that I’ve taken faithfully as prescribed, not missing a single dose. Baylisascaris likes many tissues in the body, including brain. Much of the brain fog that I was attributing to familial ditziness lifted after the course of medicine. I choose not to imagine other worms flitting around in my brain.

Sequel #3: Few people with DUSN have such a well-documented course, with serial angiograms, so my eye has become a good teaching case. It’s sad to think that I’m finally fascinating because of a worm and a miserable experience.

algerhardt@sbcglobal.net