day 4

Things seem a bit better today. I can see shapes of things. What I see is very fuzzy, but I can make out outlines of close objects. I can see the edges of my glasses, and the showerhead when in the shower. Today the pain was pretty good until the afternoon, when I took my first meds in about 16 hours. I have worked all day, and am pretty fatigued, but that irritation the bottom right is better. I’m going to dose up on pain meds soon and sleep.

day 3

I didn’t sleep well last night. I probably napped too long. I went to the doctor and I was able to see movement, and count 2 fingers, about 6″ from my eye, but not 3. Three fingers were outside my visual field. The infection looks better, but we are keeping meds the same for now. The doctor said that the irritation is probably from the edge of the bandage contact lens. I go back on Thursday. For now, I am going to nap again.

day 2

I slept 8.5 hours last night without getting up at all. That is a first in weeks. I also napped for about 3 hours today. I also think I might be able to see some movement. I can’t tell for sure whether I am seeing movement, or if I am just seeing the light be blocked. Pain is definitely better today, but not gone. I have a lot of irritation in the bottom right side of my eye, but it’s not overtly painful. Tomorrow I see the doc again.

day 1 evening

This evening I discovered that I can not only tell the direction of the light, but I can make out distinct blobs of light. As I am sitting here, I can see one blob that is my door, one that is my TV, and one that is my computer screen.

post transplant day 1

By the following morning, I was feeling much better, and had not had any pain meds since the night before. My first post-op appointment was at 9:30 am. My sister had spent the night to help out with the kids, and she helped my aide get them up and out the door so that I could go to the doc.

I was not seeing my regular doctor, but the doctor who had assisted with my surgery. He pulled the bandage off, and did some work cleaning my eye a bit. They evidently pulled some eyelashes out during surgery, and whatever he was using to clean and hold my eyelid stung. I lived, and he survived my whining. He said that the graft looked great. The new cornea was very clear. I did still have some infection around the stitches. He could not see much else in my eye because there was a lot of blood behind my cornea.

We then tested my vision. While I still only had light perception, I could tell the direction of the light. That was an improvement. For now I am taking pred-forte (a steroid) six times a day, and zithromax, chloramphenicol, and gentimicin (all antibiotics) four times a day. I created a table on my word processor to keep track of what I needed to take when.

transplant day

I had to be at the surgery center at 6:30 am. Fortunately my sister was going with me to help. I got up and dressed, and my aide got my kids out the door to school.

I was the only person having surgery that day, so I had the entire surgical area to myself, with the exception of the staff. All the staff were incredibly nice. I got transferred to the gurney which also was the OR table, and got my vent set up so that I could breathe. The nurses then started the drops. I lost track of the number of eye drops, but there were quire a few. I had an ongoing discussion with a couple of the nurses, the anesthesiologist, and even the surgeon about whether I was allergic to the antibiotic they were trying to use. I am allergic to ciprofloxain, and I was positive that cipro is a quinolone. The nurse kept telling me that while the drug she was trying to give me was a quinolone, cipro is not, so the drug was safe. Fortunately someone set the record straight.

The anesthesiologist had a difficult time getting the IV in, but after only 3 sticks, got the IV started. They then started a diuretic to help soften my eyeball. They also put on this air pressure pump thing to compress my eye. I’m sure I was quite a sight between the vent mask, the IV, eye thingamajig, and all the leads, and wires running every which way.

One of the nurses came in to confirm which eye was going to be transplanted, then she wrote “this eye” or something like that on my forehead.

The surgeons came in, and I tried to ask some of my questions about rejection, and outcomes for people with SJS, but my doc blew off my questions by telling me “not to read so much.” That pissed me off royally, but the versed had just started, and I didn’t do anything about it.

I was then taken back to the OR, and after getting all their drapes in place, they started the numbing injections. A couple were painful, but most were not too bad. The problem was that they didn’t really numb my eye. While the surface was numb, the inside was was not, and the whole thing was very VERY painful. I suspected this might end up being the case, in part because of my experiences with the gluing, but also due to the kind of pain I had been experiencing since the gluing. This was exactly why I wanted enough versed and other sedatives to simply sleep, or at least not remember what happened. That did not happen. I was very aware and I remembered it all. I spent much of the procedure telling them it hurt, and alternately crying because it hurt.

I am deaf, and with the drape over my other eye, I had no way for them to communicate with me. Someone, I am assuming the aneshesiologist, kept grabing my hand. I’m guessing to let me squeeze it when things hurt so bad. The problem is that I have a myopathy, so I can’t really squeeze that hand hard. It was not effective at all.

The only good thing was at one point, either when my cornea was off and they were putting the other one on, or when they first put the other cornea on, I was able to see a little. I could see some of their instruments as they came down into my eye. But for the pain I was experiencing, that was pretty cool. Until that point, we had no idea whether or not my retina was intact. I knew if I was seeing that, something was still intact.

Finally they were down to stitching. I couldn’t feel the actual needle going in and out of my eye, but I had that deep pain when they tightened each stitch.

After what seemed like forever, they were finished, and I was taken back to the pre-op area. I was pretty out of it, not from the drugs but because I couldn’t get a handle on the pain. After a few minutes it started to improve and my sister came back. I needed to use the restroom, thanks to that diuretic, but they made me use a bedpan. The nurses were good, and we had no mess. After they got a couple of blood pressures, and they were sure my oxygen levels were staying up, they let me get dress and go home.

I had started out with a metal shield taped over my eye, but they took it off so that I could wear my glasses. They told me to put it back on whenever I had my glasses off. I went home and slept for most of the rest of the day.

I slept in 2 hour spurts for the first 8 hours home, because I kept needing to use the restroom. Between naps I drank ginger ale and ate jello. I had a couple of slices of cheese for dinner. I was still hitting the vicodin pretty hard. The pain in many ways was a lot better than before surgery, but the surgery experience left me with no tolerance for any pain after.

the naysayers

Despite the pain, and wanting to do anything to help that, I had a good deal of anxiety about the transplant. SJS experts were telling me not to do it, and I was, and am, well aware of the risks.

Emergency cornea transplants have a high failure rate. This is due to several reasons, poorer donor tissue quality and the poor condition of the eye which is necessitating the emergency surgery. In people with SJS, emergency transplant success is even less likely. The ocular surface is so poor for SJS survivors that transplants are rarely successful to begin with, and when you add on the other issues related to emergency transplantation, you generally have a recipe for disaster.

I didn’t have a chance to discuss my concerns fully with my doctor before surgery, but I don’t think I had a lot of options. I don’t have the money to travel to Miami or Boston to see the SJS experts, let alone pay for treatment. In addition, the infection was not controlled. The Wednesday before surgery, I not only had only light perception for vision, but I could not even tell where the light was coming from. My doctor could not visualize any part of my eye because it was so filled with pus, and we didn’t know the condition of my retina, iris, lens, etc.

I made a decision that the risk of a failed transplant, and the concomitant risk of losing my eye was worth the chance to have the pain stop, find out the health of the rest of my eye, and even a small chance of the transplant being successful and regaining some vision.

Will the naysayers be right? I don’t know, but at this point I still think I made the right choice. I got lucky and received great donor tissue which increases my chance for success. I also know that my eyes are not as dry as most SJS survivors which also increase my chances for success. As for ultimate success, all I can do is wait.

transplant coming

I went to the doctor on May 2, prepared to make a speech about how I could not continue in this condition indefinitely. I actually never got there. The doctor took one look at my eye and said we need to do a transplant, and soon. Things began moving quickly. I talked to the transplant coordinator who initally began to set things up for a week later, but after talking to the doctor said that we would go as soon as we could get tissue.

That afternoon I got busy calling my pulmonologist (I use a ventilator, so respiratory complications are a big issue for me) , and making other arrangements for the surgery, like dealing with my kids, attendant care, and the like. Fortunately my sister was able to take the day off work to be with me and stay with me after surgery. We got no word on tissue status that afternoon.

Thursday morning was another flurry of calls. There were concerns from the anesthesiologist about using anesthesia and my respiratory status. There were also concerns about my last history and physical. Because I have multiple disabilities, and I see several specialists, I rarely have an official physical done. Between the pulmonologist checking out my heart, lungs, nose, ears and throat, the GI doc checking out my stomach, gtube, gut. etc., the nutritionist monitoring blood levels and metabolism, the neurologist checking out my muscles, and the eye doc managing my eyes, there isn’t much left for my PCP to check. They weren’t exactly looking for my most recent gyn exam for surgery. After a number of phone calls, a flurry of faxing of releases, and more phone calls, it was decided that the transplant could be done at the surgery center attached to my cornea specialist’s office.

I should have paid more attention to the anesthesiologist talking about using little anesthesia, but more about that later. I wanted the surgery at the surgery center because I would not have to be admitted, there was a lower risk of developing a secondary infection, and the transplant (and my agony) would not be delayed. If it were done at the hospital, there would have been more consultations with my specialists, likely pretreatment with dantrolene to prevent malignant hyperthermia, and as a result, a longer hospital stay, and increased infection risk due to the longer stay, not to mention what to do with the kids and dogs during a longer hospitalization. I would have figured it out, but was glad I didn’t have to.

Mid-morning on Thursday I got a call from the transplant coordinator telling me not to eat or drink anything because there was a chance the surgery might happen early that evening, but she called a couple of hours later to say that we were positively on for 7:30 am on Friday, May 4.

infection

That Monday, the doctor scraped my cornea to both get a culture and debreid the ulcer to promote healing. Yes, that is as painful as it sounds (think scalpels and other nasty instruments in your eye), and the doc prescribed more antibiotics.

That Wednesday the infection was much worse, and it was clear we were dealing with a resistant bug. The doc gave me a shot of vancomycin in my eye — not in the eyeball, but in the inside of my lower eye lid. I am allergic to vanco, but we decided the risks from a local reaction from the relatively small amount of antibiotic were less than not treating the infection in that manner. I did have a reaction, but it was controlled with benedryl, but the infection continued to get worse. At this time I had no vision left aside from light perception. I was experiencing excruciating pain, and was struggling to get through the day.

That Friday, April 27, I was hopeful that the infection was somewhat better. The amount of pus and discharge was less, but pain was worse. When I saw the doctor that afternoon, I learned why. My cornea perforated from the infection. The doctor treated the perforation with superglue. Literally. The doc took me into the OR, and with local anesthetic used medical grade cyanoacrylate glue to seal the hole in my eyeball. The doctor then topped the glue with a cellulite disk and a bandage contact lens to hold things in place. I also got another shot of vanco, along with a shot of gentimicin. Through all the eye problems, I had not yet experienced pain like that. I got through the weekend through the use of lots of vicodin (I can’t use anything stronger due to my other disabilities).

That Monday I again went to the doctor, and the glue was in place, it wasn’t better but it wasn’t worse. I didn’t see my regular doctor, so we just continued what we were doing with the antibiotics.

I went home and began doing more research about corneal perforations, and consulting with folks on an ocular surface listserv I belong to. At that point I did not care if we saved my eye or not, I knew that I could not continue to live that way. The pain was still intense, and I knew that I could not function on vicodin indefinitely.

Stevens Johnson Syndrome

Stevens Johnson Syndrome (SJS) is a drug reaction that causes the skin and mucous membranes to blister, ulcerate and slough off. I’ve had this reaction three times, the first in 1994 from Bactrim, a sulfa drugs. The second bout was in 1999 from hydrochlorothiazide, also a sulfa drug, that was given to me in a prescription misfill. The third time I had SJS was in 2002 from macrobid, an antibiotic.

While SJS has many long lasting effects, the most problematic is the ocular damage that results. Because the ocular surface is made of mucous membranes, they can blister and scar during the SJS. This leads to constant corneal irritation, dry eyes, and reduced vision.

In my case, I had few ocular effects from the first two bouts of SJS, but had more the 3rd time. Within a few months after the 3rd SJS, I was unable to see anything more than lights and shadows due to corneal ulcers and infection. Over the next couple of years my eyes recovered some, and my vision pretty much stabilized in the 20/400 and 20/650 range. My vision remained low because of scaring on my corneas, as well as blood vessels that had grown into the normally clear cornea. It wasn’t great, but was functional and livable. I was managing the SJS effects by wearing bandage contact lenses around the clock, and using a combination of eye drops to prevent infection, reduce inflammation, and keep my eyes moist.

For four years that routine worked well, and my eyes were relatively stable. While I had flare-ups, I was easily able to manage them by increasing the antibiotics and steroids, and letting my eyes rest.

In early April, 2007 I developed eye infections in both eyes, and increased antibiotics and steroids, and seemed to have kicked it.

Two weeks later symptoms of light sensitivity, increased pain, redness and inflammation returned to my right eye. I took the weekend off as much as possible to rest and hit the meds hard. My eye did not improve, so I made an appointment with my cornea specialist.

I saw the doctor on Wednesday where he found a corneal defect and prescribed a stronger steroid to be used every hour, along with my usual antibiotics. Despite my efforts, by Monday I had a raging infection.