We’re doing something a little different this time around on WTKM. I’d be willing to bet that most of you have never heard the word nystagmus before, so let me break things down a little.
Nystagmus is an involuntary, rapid eye movement. It can be caused by several things, including drug overdoses and head injuries, but it can also be congenital. In its congenital form, it’s largely idiopathic, meaning doctors don’t really know what causes it, though it appears to be genetic.
Nystagmus itself isn’t lethal. In fact many patients have no other significant health problems. But I’m presenting it to you today for two reasons:
In May, I’ll be publishing my 7th full length novel, MAGIC IN THE HEADLINES. AJ, the main character, has congenital nystagmus. Because it’s a fairly unusual condition, I wanted to provide a basic primer on it here.
Secondly, I didn’t just pull this condition out of a hat. My mom was born with congenital bilateral conjugate nystagmus, which means both eyes move in unison. In her case, horizontally.
When my mom was born in the 1960s, doctors told my grandmother that if she’d had blue eyes, she’d be completely blind, and if she were a boy she’d be dead. They also told her that the rapid eye movement was the result of being premature. I don’t know at how many weeks my mother was born, but she was, at the time, classified as premature. In the interest of privacy, I don’t want to go into detail about her health, but she did have other minor developmental issues as a child, most of which she either grew out of or were corrected with time. The one thing the doctors could not fix, however, was her eyes.
Patients with nystagmus don’t see the way “normal” people do. The rapid eye movement means they only catch snatches of vision, like watching a poorly timed stop-motion film. In the case of my mother, she doesn’t know any other way of seeing, so it’s hard for her to describe what it’s like. She has nothing to compare it to.
The stop-motion effect also means she has a distinct lack of depth perception. I was really into baseball as a kid, and it was always a challenge when she would play with me, for both of us. Catching the ball was out of the question, and she always preferred pitching to batting because she couldn’t tell when the ball was in range. When I was little, everyone still thought she could learn to drive, and she would practice going up and down our long driveway in a hideous green sedan. My dad insisted she back down the driveway to the house, which had an unusual effect: because her head was turned so far, it “trapped” her eyes, reducing the movement. Additionally, because she was turned to the right, it meant relying more on her right eye, which is both her dominant eye and the one with better vision. My mom will never get her license or be able to drive on the freeway, but if you need someone to drive in reverse for long distances, she’s your girl!
From the time I was about four or five, I was my mom’s “seeing eye daughter.” When we had to cross the street, I would tell her when the cars were far enough away for us to cross (we don’t have many sidewalks or cross walks where I grew up), or when a driver waved to us to cross. I would read subtitles in movies for her, or small print directions in cookbooks or craft books. I threaded her sewing needles. While her nystagmus hasn’t put huge limits on her life, she does need help now and again. She can’t drive and computers can be challenging (some color combinations make the eye movement worse, and she needs some pretty intense screen magnification), but for the most part she did all the normal mom things you could hope for (well, except driving me to 4-H meetings. But she was still there for every single one).
The thing about nystagmus is that because it’s pretty rare and not life threatening, not a lot of research has been done into it. What research there is generally focuses on the brain damage that accompanies head trauma or overdose victims, or those with much more severe forms. Congenital nystagmus often appears with partial or total deafness, learning disabilities, or other issues, none of which my mom has. Researching just the eye movement, the history and causes, was a challenge. In the end, I turned to social media asking for current patients or their relatives to tell me their stories. What did their doctors say was the cause? What other issues did they have? Were they premature? Did they go on to lead normal lives? If it was caused by premature birth, at how many weeks did it take effect? How early did the baby have to be to have developmental issues, but still survive in the days before incubators were commonplace?
In the end, I walked away with a few answers, but not as many as I’d hoped. I found stories dating back to the late 1800s of children, male and female, born with various forms of nystagmus. One of them was premature, and kept warmed in a box by the wood stove for several weeks. Others were full term. Some were the children of addicts, others had completely healthy parents who didn’t even take medication.
As with many congenital illnesses, it appears less frequently now than it did pre-1950. Maybe because healthcare is better, including prenatal health and diet, prenatal testing, etc; maybe it’s because our medications have improved, or perhaps there are environmental factors at play. It’s hard to say with the information I found available.
I can say that my grandma’s doctor was probably wrong when he attributed the nystagmus to premature birth; we now know it’s probably genetic, though what gene it’s carried on or what defect causes it we don’t know. He was probably right though when he said my mom would be blind if she had blue eyes, and wouldn’t have survived if she was a boy. Blue eyes are often linked to blindness because they are a recessive gene, and baby girls have a higher survival rate because we carry 2 X chromosomes, so if one is faulty in some way, we have another to fall back on. Boys lack this advantage.
So why does all this matter? With so little information, why am I sharing it?
My mom is the only person I’ve ever met with rapid eye movement like this. She was bullied as a child and even now adults are thrown by it. I remember as a kid when my mom was trying to teach me manners, I wasn’t sure if I should try to follow her eye movement to “look her in the eye”, or if I should just stare at her eyeballs. It’s impacted by stress, emotion, and exhaustion, so it would get faster and more erratic when she was angry or frustrated. More than once I accidentally ended up laughing while she was trying to discipline me (sorry, Mom).
I want to shed light on this, so readers know this is not something to be afraid or to make fun of. It can be surprising, yes, but often the people who have this condition aren’t bothered by it (mostly). It’s just a fact of life for them, like having brown hair or being a little on the short side. Yes, it requires a work around. Yes, it is a disability. But those work arounds can happen, and patients are completely capable of living full, independent lives.
I also wanted to explain a teensy bit about some of the struggles AJ has in the book, and why they exist. She does drive, yes (very few places required driver’s tests or even licenses in 1925…that would change quickly), but as any of her passengers will tell you, it’s not well advised. She does wear glasses, but like my mom’s prescription, they are magnification only, to help her better grasp those snatches of vision that can be so fleeting. She often needs to get closer to something to see it, and doesn’t always know when danger is coming because she lacks the visual cues. People are confused by her eye movement, and don’t always know how to handle it. She has a medic alert bracelet so that if something happens, she doesn’t get treated for an injury or drug overdose when the problem is something different (ex. if she were to have a heart attack or stroke (heaven forbid) and lose consciousness, trying to treat her for an OD instead could be lethal).
AJ isn’t just hampered by her vision; she also suffers from migraines and fairly severe asthma. The migraines are brought on by a combination of her vision, and her ability to see and sense ghosts (you were waiting for that, weren’t you? There’s always a ghost in my books).
AJ and my mom share few traits outside of their disability, but my mom was still a huge influence on her, and I want my mother to be proud of her “granddaughter”, even if she is fictional.
Like what you see? Check out Would this Kill Me in the 1800s: Strep Throat.