My second daughter, Isla, was born June 15th 2009. My husband and I were delighted to have another daughter. Isla was born in less than two hours, and I made it to the hospital only 15 minuets before she arrived. When Isla was two days old, the pediatrician informed us that she might have something wrong with her eyes. We were sent to an optimoligist in town who confirmed that Isla was born with congenital cataracts in both eyes. Needless to say, we were devastated, lost, and confused as we had no idea what lied ahead.
A few days later, we traveled the soon to be regular three hour round trip, to the specialist in Syracuse, NY. At seven weeks old, on July 3rd Isla had her first surgery on her right eye. Just 10 days later she had her second surgery, on the left eye. When we brought her home, she was acting out of the ordinary and started refusing to nurse. I thought she was having a reaction from the anesthesia. Isla was miserable constantly and would no longer eat anything. She had started loosing weight and her pediatrician felt there was nothing wrong. After many Dr. visits and research at home, I asked the Dr. about reflux. Isla was diagnosed with a severe case of acid reflux. I ended up having to bottle feed because Isla refused to lie down to eat. It took an average one hour to get her to eat one once only to have her throw it up again. We were referred to a specialist here in Ithaca, Isla started to get a little bit better. She was still not eating well and acted very sick all the time. I finally referred her myself to CHOP (Children’s Hospital Of Philadelphia). After going to CHOP we learned that she was on 2x the amount of medicine for her weight and it was making her sick. Isla was also allergic to milk and was given a prescription for a special hypoallergenic formula. That night, at four months old, Isla slept through the night for the first time!
Isla wore contacts from three months until eleven months when I literally begged the dr. to let her try them. The contacts never fit right, and we made SO many trips to Syracuse because of lost (either behind her eye, or on the floor) contacts and constantly trying new ones to see if they work better. Isla was also patching at 3 months until this past December.
Because of the severe reflux Isla fell behind developmentally. She saw a Physical Therapist, Speech Pathologist, Occupational Therapist and vision therapist all once a week. Isla graduated from these therapies by her second birthday except for the vision therapy and started seeing a nutritionist when she turned three.
That February, Isla developed aphific glaucoma in her left eye, and was treated with several eye drops and was well under control. That summer, her eye started swelling and turning red, her pressure was unstable for quite some time. We made a few trips to Wills Eye Hospital in Philadelphia where the Dr. there felt Isla’s pressure was not at the point to intervene with surgery. This went on for almost two years, back and forth, pressure up and down. Mind you, as most of you know, pressure checks are all under anesthesia.
Finally April of 2012, when Isla was 2.5, Isla’s pressure was maintaining a high pressure and both Dr.’s decided it was time to put a tube in. My husband, Isla, my 7-week-old son and myself traveled the 4.5 hours to Philly for our two-week surgery stay. We had to stay in a hotel for the first week, and were fortunate enough to get into the Ronald McDonald House in Philly for the second week (because it was Easter weekend, and many people went home). My husband had to go back to work after the first week, so my mother met me down there to help out. Unfortunately it was much more stressful than helpful at that point, between her intolerance for ANYTHING, my sons constant nursing, and Isla who was completely miserable. She refused to open both of her eyes for the whole two weeks, and had to be either carried or pushed in a stroller the whole time. I had to sit on her, while my mom held her head, to put drops in her eyes every hour!!! Ok, you get the point it completely sucked!
May of 2012 Isla had toe surgery on both feet. She was born with one claw toe on both of her feet. One toe surgery was successful, the other one was not and they will have to redo it when she is ten and her growth plates are gone.
After that surgery, Isla developed glaucoma in the other eye…so the drops started all over again there. Meanwhile, she was continuing to develop well despite all the therapy, eating issues…yes, they continue to this day.
This past December, I was on my way to NC to ride up with my sister and her two children to NY for Christmas. My sisters husband is a Marine and was deployed for a year in Afghanistan, she wanted to be home with family. My flight got delayed in Philly (go figure, I HATE Philly for so many reasons), it ended up getting canceled so I got a hotel. Around five pm, my husband called that Isla was not acting normal all day and when she woke up from a nap her eye was swollen shut. He immediately drove to Syracuse to have her looked at. Isla’s eye was infected (the one with the tube in it). She was taken to the ER and they did emergency surgery on her later that night. I was barely able to talk to my husband at this point, because he was learning what was going on, dr.’s were in and out, all I got out of the first phone call was that she was in emergency surgery and that she might loose her eye!!
Here I was stuck in Philly, 100% hysterical and not knowing how to get home. My nephew offered to drive down to get me. I took him up on it, and was in Syracuse by 6am just in time for the team of Dr.’s to perform another emergency surgery since the first one did not flush the infection out.
Isla had one of the biggest teams of dr.’s I have ever seen. This was such a rare infection, the dr. was learning as they were treating. Isla’s dr. in Philly, who see’s patients from all over the world and is considered one of the best pediatric glaucoma dr.’s, has only seen this once in his career. Isla’s tube in her eye is what got the infection, no one knows why or how. The infection spread throughout Isla’s face and into her ears and sinuses. If it got to her brain, it would have been fatal. After eight nights at the hospital, Isla was released right before Christmas and was able to go home with a picc line.
That January, we went back to Philadelphia to try to have the scar tissue removed from that eye to see if any vision could be saved. During surgery, the dr. discovered that there was far too much damage from the infection. The dr. had said that it was a very vigorous infection that basically ate away at her eye and her eye was dieing. The scar tissue tore her retina, and completely covered the inside of her eye. We were told we could chose to have one last surgery done, but no vision would be restored, it would only be to try to keep the eye healthy. It had a 3% success rate and was very invasive with a painful recovery. My husband and I decided that Isla had been through enough and that we would not put her through more for only a 3% success rate.
The dr. was doing a research study on genetics while we were there so we opted to have blood work drawn during surgery on Isla. Results came back weeks later that Isla is missing part of an X chromosome. We just saw a geneticist in Syracuse last week, which was able to tell us very little, since he did not yet have the full test results. It looks like many of Isla’s random problems, may all be related to this syndrome. We have more testing to do, and should find out later this week the full results of the first test which will tell us what else can happen to Isla and when.
Isla’s eye that she lost has shrunken considerably, she no longer has a cornea, and the whites of her eye are permanently bloodshot. Luckily, it isn’t that noticeable do to her thick glasses. Isla may still need her eye removed and a prosthetic put in, but we were told it might take up to ten years for her eye to “make up its mind”. Another option is to have a shell put over her eye without removing it.
Isla still has a long road ahead of her, but she is the strongest child I have ever met!! She is doing well at nursery school and finally socializing and making friends with other kids. (Isla spent the first three years of her life afraid of most people and would not talk or play with anyone but her immediate family). Isla has been under anesthesia well over 50 times, 7 surgeries, and several tests and procedures done under Anastasia. Isla’s only seeing eye is doing well. Her pressure is holding at 21 and she see’s 20/25 corrected. My fear, is that she will end up needing surgery on that eye for the glaucoma but as of now it is holding strong. She gets me through some rough times and I have her strength to thank for that.