Pediatric Glaucoma

July 2008:

After consultations with 2 doctors, Dr. Lama in Hackensack and Dr. Kane at the Columbia University Eye Institute in NYC, we decided that Dr. Kane was the right doctor for Emily. Although Dr. Lama has worked with children, he was not a pediatric Glaucoma specialist and therefore wasn't able to accommodate Emily's needs. He basically closed her file, referred us to a specialist in Boston and pushed us out the door. I was very hesitant to burden our family with traveling all the way to Boston unless I absolutely had to, so after we got home, I contacted Emily's cornea doctor. Dr. Zaidman had already called and spoken to Dr. Kane about Emily's condition so I felt more comfortable going to him. He only works with babies and children, and accepts our insurance which is a blessing. Also, he is able to take her eye pressure readings in the office without sedation or putting her under anesthesia, which is HUGE for us.

On July 8th we went into NYC to see Dr. Kane. Our appointment was for 2:30 but we were not see until 6:00 - yes we sat there for 3 1/2 hours before Emily's name was called, but everyone told us he was worth the wait. While we were waiting, we had been talking with another parent whose daughter also had corneal transplants and sees Dr. Zaidman. Apparently she was also referred to the doctor in Boston and had already been there to see him. She lives on Long Island and the doctor from Boston said she should see Dr. Kane instead since he was so much closer. Through her we found out that Dr. Kane studied under the doctor from Boston, so that made us feel good. If he was telling her to come back to NYC and see Dr. Kane, that saved us a trip and we knew we were in the right place.

During the exam, Dr. Kane went over her medical history and then attempted to take her pressure. I was amazed...he kept her upright on Jason's lap, got her attention with some blinking toys and was able to get her pressure readings without a problem. He has a different tool that the other doctors don't have and that allowed him to work his magic...no sedation, no anesthesia...no crying. I couldn't believe it. Her eye pressure was 34 in the Right eye and 44 in the Left eye. That was higher than it has ever been. He told us she would need surgery since the medications don't seem to be working and if the pressure remains elevated it will cause damage to her optic nerve and she will lose her vision. This surgery is very complicated because of her abnormal eye structure, and there is a possibility that it could interfere with the cornea transplants. Because her eyes aren't normal, the typical Glaucoma surgery of creating a new passage way for the fluid to drain from wouldn't work. So she would need a drainage tube implanted. He adjusted her oral medication from once a day to 4 times a day and said he wanted to see her in the office once more before surgery. He mentioned that he would ask his secretary to look for some time in the OR for her and we would discuss it at the next visit. We made her next appointment for July 30th before we left that night (which was 8:00 pm).

On the way back down Rt. 80 heading to pick up the kids, a tractor trailer drove next to us. It read "Kane is Able..." I think Kane is a trucking company obviously, but the irony in that is astounding.

Meanwhile we saw Dr. Zaidman again and he wants us to try and lower the frequency of the steroid drops to see if that helps with the pressure. She still needs the steroid to help prevent rejection of the transplants, so we have to monitor the clarity of the corneas. If it looks like it's getting worse, we have to increase the dose and then try again in a few days to lower it. She's also been on liquid Prednisone for a month now, which was dropped from twice a day to once a day since the swelling in her left eye has come down a little. The oral steroid basically makes you blow up like a balloon, so she has puffed out quite a bit. We affectionately call her our little marshmallow! It's cute, but hopefully she will lose some of that baby fat when she becomes more active. Dr. Zaidman doesn't want to keep her on the Prednisone long term, so we hoped she would come off of it soon.

We had recently enjoyed a much needed vacation at the beach, albeit not a completely stress free one as we had anticipated. But we all had a good time and hoped to come home refreshed and with a renewed sense of strength to face Emily's impending surgeries and new course of treatments. We came home on a Sunday and were expecting to see Dr. Kane on July 30th for her next office visit, but instead came home to a couple of messages from his secretary "reminding" us that her surgery was scheduled for Tuesday July 29th! WHAT??? Apparently the secretary neglected to call and let us know that she scheduled Emily's surgery...nice right? Thankfully we had come back from vacation in time.

The surgery was at the Columbia University Children's Hospital. We got there at 10 am and she was taken into the operating room around 12:20. We knew it was going to be a long surgery so we were prepared. Overall the surgery took 3 1/2 hours. The valve was implanted on the bottom of her Left eye, just underneath her lower eyelid. Normally it is on top of the eye, but this was the only place he could put it because of the previous surgery and scar tissue. You can't see the valve or the drainage tube since it is under the first layer of tissue. This is the first step of a two step process. First the valve is implanted and the drainage tube is tied off to prevent rapid draining of the fluid. Her eye needs to heal before the drainage tube can be opened. She would have the tube opened and the surgery on the Right eye done at the same time, which I guess is a good thing. One less trip to the hospital and one less time she's under anesthesia. Then she would have to go back 2 weeks later for the other drainage tube to be opened. We were told that all of the drainage tissue in her Left eye was non-functional so this surgery was the only option for her. This is most likely a result of the cornea surgery, which is why the pressure skyrocketed and her eye became so swollen shortly after that surgery.

The corner and bottom of her eye was red, and I hadn't seen under her bottom eye lid yet since I didn't want to pull it down and disrupt anything or cause her pain. Other than some hesitation to open her eyes on the second day, she seemed to be relatively unaffected by it. Initially she had alot of swelling around her eye and eyelids, but that had gone down quite a bit. She was now on an antibiotic/steroid ointment 4 times a day. Since the 3 types of pressure reducing eyedrops she was on was not really effectively working, Dr. Kane no longer saw the need for them to be used in the Left eye. He feels that the oral medication should be all she needed. She was still using the eye drops in the right eye since he didn't want to change too many things all at once. I assumed he would discontinue those drops once she had the surgery on the right eye. He also didn't want her to use the steroid eye drops 5 times a day, so he reduced that to only twice a day. She still needs that to help prevent the rejection of the cornea transplants, so that could cause a problem. Hopefully it won't. Right now her cornea's are not as clear as they were when the transplants were first done. However, it was very possible that the increased eye pressure was the reason. In a normal case of Glaucoma, one of the signs is cloudiness of the cornea. But since she already has a condition where that happens, we can't be sure whether or not the cloudiness is from the Glaucoma or from the transplants being rejected. We also don't know if this will be a complete fix for the Glaucoma or whether she will need further surgeries and additional medications down the road, but we were hopeful that it would. We were also trying to remain optimistic that this surgery wouldn't compromise the cornea transplants, as that is a risk as well. We can only take one day at a time and pray that once all this is over, we can learn how to manage her condition instead of constantly trying to treat it. The ultimate outcome would be for the Glaucoma to be controlled by these valves and the oral medication, and for her cornea's to clear up once the pressure is controlled.

We are still trying to understand her condition and how this surgery will help her. There are a few different types of valves, but Emily has what is called the Ahmed Glaucoma Valve. I have found some information online about her condition and the surgery she had that seems to clarify things a little.

Information about the valve and treatment:
http://www.ahmedvalve.com/resources/PB_english.pdf

http://www.glaucoma.org/treating/glaucoma_valve.php

http://willsglaucoma.org/treatments.htm

Information about Pediatric Glaucoma:
http://willsglaucoma.org/whatisglaucoma.htm

http://www.glaucoma.org/learn/rare_forms_of_g.php

http://www.pgcfa.org/glaucoma.htm

http://www.pgcfa.org/kb/entry/61/

The Question and Answer portion of this webpage has some pretty good information as well:

http://www.pgcfa.org/kb/11/

She hasn't been wearing her glasses because the ointment is very messy and gets all over her lenses. She's absolutely not allowed to rub her eye and at that point I'd rather not bring more attention to the area by fighting with her all day to keep the glasses on. She wears an eye shield at bedtime to help prevent her from accidentally rubbing/poking or bumping that eye. Any of that could cause the stitches to break and that will be very bad.

I feel like this is a losing battle.