Finally Some Answers

12/1/07:
Emily had her eye exam under anesthesia at Westchester Children's Hospital. We arrived at 1:00 as she was scheduled to go in at 2:00. Originally we were told that we would stay in the recovery area for an hour after she came out of the anesthesia, but when we got there they told us that because of her age they needed to keep her for 6 hours. If she did well we could go home, otherwise if she had any problems (ie...apnea) they would have to keep her overnight. We were completely unprepared to stay that long and were pretty upset about it, but there was nothing we could do. They didn't take her in until about 3:15 (we didn't leave the hospital until almost 10:00). I went into the operating room with her and watched her fall asleep, tears rolling down my face. The entire time she was in there, my heart was filled with such anxiety. Would we get some answers? Will he be able to help her see?

After the exam was finished, Dr. Zaidman came out to talk with us and said she did very well and was in recovery. During his exam he determined that she has Peters' Anomaly, not CHED as previously suspected. The pressure in her eyes was normal, so Glaucoma has been ruled out for now, but there is always a chance that she will develop it. Almost 70% of children with this condition do. The difference between CHED and Peters Anomaly is that CHED only affects the 5th layer of the cornea (there are 5 layers) and Peters Anomaly affects multiple layers. I believe he said it was the 2nd, 3rd and 5th layers. The only treatment for this condition is cornea transplant surgery. One good thing that was found during the exam was that other than the corneas, her eyes are completely normal so hopefully this means that she will have the best vision possible after the surgery.

From what we understand, her condition is caused by a genetic abnormality and was formed during the first trimester of pregnancy. There is currently no prenatal testing that could have detected this problem before she was born.

The doctor told us that he had already cleared a place in his schedule for her surgery at the beginning of January. Within the following week we would be hearing from his office as to a tentative date. Because the surgery relies on a donor cornea, the exact date of the surgery would not be known until the day before. The tentative date would give us an idea of about when it would take place. The Eye Bank in NY was going to be notified that a cornea for her would be needed around that time. When one becomes available, we would be notified and the surgery would be scheduled for the next day. So basically we would be on stand-by for the week surrounding the date we are given. It could be a few days before or a few days after. The surgery itself will take about an hour and a half, but if her body accepts the donor cornea, it should last her entire lifetime. Whether or not she will need to stay overnight at the hospital wasn't known yet, but we would prepare for it just in case. The day after the surgery she would need to be seen in Dr. Zaidman's office for a follow up and from there on she would be seen about 3 times a week for the first month and then twice a week for the second month. The frequency would eventually decrease down to once a month, but because the second eye was going to be done about 8 weeks or so after the first one, the frequency of doctors visits would once again go back to 3 times a week and so on. She would also need to be followed by a Pediatric Ophthalmologist. We were hoping to find one who is in our area, or at least closer to home than Westchester.

As for her prognosis, that was still up in the air and would greatly depend on the quality of the donor tissue, her body's ability to accept it, and the further development of her eyes and vision as she gets older. She would need visual therapy, so I started the process of contacting a state organization to enroll her in Early Intervention. Through this program a therapist would come to our house and work with her. She would need as much visual stimulation as possible to give her the best chance to see well. She also would most likely need glasses, probably as soon as her eyes are healed from the surgery. The specifics for after the surgery is complete were still not known, but we were taking one step at a time. Looking at the big picture and what lies ahead for her and us was and still is completely overwhelming, so we were trying to just focus on what the next step was and go from there.

This was the beginning of a very long road for her and for us as a family. We would need all the love and support we can get, as we tried to get through this very difficult and trying time. We tried to remain positive and optimistic about her future, but as you can imagine this is a very emotionally difficult process. Christmas was just a few weeks away and I just wanted to put this aside and try to enjoy the holiday season. Our little girls first Christmas...and "the calm before the storm".