Jeannie’s Brain Welcome to the inside of my head

This is not a tech post. Tech friends please feel free to move on. My next post will be tech-related.

I was born with two eye diseases in both eyes. Fuchs Dystrophy, and an optic nerve abnormality that developed into Optic Nerve Head Drusen. Everyone is born with a single layer of cells beneath their cornea. These cells act as pumps to keep the cornea clear. In people with Fuchs Dystrophy, the cells are puny and die off, causing the cornea to fog up. It becomes increasingly opaque until it’s like trying to see through waxed paper. Eventually, the layers of the eye break down causing blisters that then burst. In some people these cells die very slowly as they age and the effect is barely noticeable. Such people may never even know they have the disease. In others the cells die quickly, suddenly, or simply at a much younger age. I am one of second type of person.

The cure for Fuchs Dystrophy is a cornea transplant, first in one eye, then the other. Cornea replacement surgery has been done for about a hundred years. Until the past few years, the entire cornea was removed and replaced with a donor cornea. The new cornea was held in place by stitches. The recovery time was about two years, the risk of rejection was fairly high and severe astigmatism often resulted. Very recently, due to advances in microsurgery, it is now possible to remove only the defective layer of cells through a tiny incision. The donor layer of cells is then inserted and held in place by an air bubble. It is necessary for the patient to lie flat until the cornea adheres. Steroid eye drops are then used indefinitely to avoid rejection.

In December 2007 I had this type of cornea replacement surgery, called a DSAEK, in my right eye, which had the worst vision. Because cataracts form with age and the replacement cell layer would be destroyed during future cataract surgery, my lens was also replaced. All eye surgery is done while you’re awake. The anesthetist apparently thought I was only having cataract surgery – a fifteen minute procedure, rather than a DSAEK plus cataract surgery – which takes at least an hour. The anesthetic wore off fifteen minutes in. The surgery was…about as nightmarish as you can imagine. I spent the other 45 minutes clutching the operating table to stop myself from jumping up and running out of the room. The end was the best, three hypodermic needles into the eye, one at a time.

The procedure(s) went perfectly, but I reacted badly. I looked and felt as if I had been assaulted. The eye looked like something from a monster movie: all of the white was deep red and the iris totally black. It took months rather than weeks for me and the eye to recover. The stitches, which were not supposed to hurt, felt like chicken wire in my eye. And I still did not see clearly.

There is often a film that develops on the replacement lens after cataract surgery, so surgery with a YAG laser was done to remove that. This procedure was supposed to be painless. In me it felt as if my eye was being shot repeatedly with a machine gun. And that’s what the eye looked like afterward. It finally healed. I still did not see clearly.

The surgery had also left me farsighted. I’ve been nearsighted since birth and wish to remain so, it’s natural to me and everything I do is close up. Lasik surgery cannot be done on someone who has had a cornea transplant, so I had a PRK. This is a refractive procedure that was done before Lasik became common. In a PRK, the top layer of the cornea is scraped off with a scalpel, then the cornea is shaped by a laser. The pain was unbearable. It felt like I had a large blister on my foot, the blister skin was removed, then the shoe put back on. Only it was in my eye. After the recovery I could see close but still not well.

If you Google Fuchs Dystrophy and Optic Nerve Head Drusen you will find a lot of sites with short, inaccurate information about how they only effect people in the sixth or seventh decade of life. You will also read that these diseases rarely result in serious vision loss. Neither is true. I know teens who have needed cornea replacement surgery for Fuchs and children who have completely lost sight from Optic Nerve Head Drusen. I suppose it can be more prevalent in older people because everything deteriorates with age. The other patients at my cornea surgeon’s office are older than my parents, and the employees at the eye surgery center knows me as, “the young patient”.

The true reason my vision was so poor only became apparent after the cornea was clear. The Optic Nerve Head Drusen had thinned and damaged the optic nerve fibers. This damage is worse in my right eye. In both eyes, my vision is dimmed and there are parts of it missing – sort of like missing pixels on a monitor – especially around the edges. The damage is progressive. I have put off having the DSAEK in my left eye for as long as possible. In spite of the Fuchs fog, the left is still my better eye and the procedure does not always go so perfectly. Complications could develop. I may be able to avoid surgery for a year or two.

There’s nothing wrong with my macula and with Optic Nerve Head Drusen, center vision lasts the longest. I’m hoping I won’t be blind until I’m too senile to notice. I see better on a monitor than I can on paper or in the ‘real world’. I’m legally blind and cannot drive. I’m also unable to cross traffic alone as I won’t necessarily see a car heading toward me. I’m blind if light is facing me. I also have chronic stress fractures of my metatarsals and cannot walk much without causing fractures, so staying put is not much of a problem.

This is not an incredibly contrived excuse for spending so much time online. I’d probably be here anyway.

Eye Bank