Cornea Graft Failure

October 10, 2014

I knew this day was coming, I felt it in the deepest part of my heart months ago, but that didn't soften the blow at all. Today was just verbal confirmation from the doctor.  Emily's cornea had failed...she would need another transplant, the third one in this eye.  Her doctor seemed hopeful, almost enthusiastic about her chances of better vision.  But the risks for both failure of the graft and complications with her eye pressure and cataract were higher than the previous surgeries she had been through.  Still, the risks did not outweigh the benefit.  With her cornea badly damaged, beginning to cloud up and scar tissue blocking a portion of her vision, the reality of NOT doing another transplant meant her vision would continue to deteriorate. Surgery was necessary.

Dr. Zaidman ultimately left the time frame of the transplant up to us, we could do it as soon as January, or wait for a better time over the next 6-8 months.  I hated the thought of putting her through this surgery again, but putting it off for a more convenient time in our lives seemed pointless.  So we scheduled the surgery for January 22, 2015.  It was after the holidays and would give her enough time to heal before spring and summer. She would need to be out of school for at least a week and no gym or sports for a month.

The other issue we needed to address was the cataract. Originally Dr. Zaidman wanted to remove it and replace it with a lens implant at the same time as the transplant surgery.  But for some reason now he felt that it would be better to do the transplant first, wait for her to heal and then go back in to the the cataract surgery.  I was concerned that this would possibly interfere with the health of the new cornea, but at the same time was leery about doing both surgeries at once.  The complications that could be triggered were serious...I hated that she had a cataract in the first place.

I believe that all the surgery that her left eye endured was the reason her retina detached...too much for her eye to handle:

• 2 cornea transplants, 2 Ahmed Valve implants, multiple surgeries to remove scar tissue from the valves drainage tube, removal of the cataract, lens implant and Vitrecotomy (removal of the jelly-like substance within the eye) 

She has had slightly less complications with her right eye, but more surgery meant more chance of compromising the Trabecalectomy (new drainage path created for eye fluid to drain from), which meant we could be struggling to control her eye pressure again. And if cataract surgery was needed, disrupting the back of her eye could spell disaster and total blindness.  

I had to believe that this next transplant would be a new, fresh start for her. It would be the best donor tissue yet. There were no other outcomes I would even let my mind consider. This might be Emily's last chance...

"Now my eyes look the same"

October 1, 2014

Today we went back to the occularist to get Emily's new prosthetic lens, one that would be painted to match her good eye.  I was nervous, mostly because I wanted her to simply allow me to put the lens in, and keep it in.  But matching it as closely as possible to her right eye was extremely important. This lens would be the first of many that would help her gain self confidence, determine how others would accept and embrace her disability and most importantly, impact how she viewed herself.  We all know she is beautiful, but children and adults alike often don't see their own beauty, they see their flaws instead.  I grew up with having a very low self esteem. I was made fun of for the way I looked, my haircut, the clothes I wore...and various parts of my body that were either "too big" or "too small".  I can pin point the exact moment in first grade when my nickname was given to me by some very mean girls on the playground. I know what I was wearing, where I was and who it was that said it. My life has not been the same ever since, and from that day forward I have had a body image problem.  I didn't have a physical disability, I was a normal 6 or 7 year old kid.  I'll admit, the name calling and teasing is what I fear most for Emily socially, so her new lens needs to look as natural as possible.  It needs to blend seamlessly with her other eye in order for it to not make her good eye, look like the bad one.

We arrived at the office and I thought I heard a familiar voice, but wasn't sure.  Then I saw through the open door to an exam room my friend Michelle and her son!  We met through both Dr. Zaidman and Dr. Kane when their second son was born. Both of their boys have pediatric glaucoma, and the older boy had recently lost his vision in one eye.  We have been talking on and off for years and although I knew they were going to bring their son to be fitted for a lens, I had no idea they would be there today!  It was a wonderful surprise, not just for us as parents, but for the kids as well.  Emily didn't remember him, but that didn't stop them from talking and playing like they had known each other for a long time.

While we waited for our appointment, and for the last minute details of Emily's lens to be finished, Michelle and I laughed and cried for at least an hour. We were there for support for each other, and for each others children, and it was nothing short of therapeutic for everyone.  Our kids and respective families have been through so much, and no one can understand exactly how you feel like someone else who has been in your shoes. It was wonderful to be there to talk her through some of her fears, and have her help settle some of mine.  They weren't supposed to be there, life through itself in the way of their original appointment, just like it had for us.  It was no coincidence that they were there...it was the world working its magic for all of us who needed it.  Right place, right time, right people.

I was no longer nervous about how the lens would look, I knew it would be amazing.  But seeing it and helping to shape the outcome of either blurred lines around the iris, or a slightly bloodshot look to the whites of her eye, helped me realize it would be perfect.  When it came time to put it in, Emily was hesitant...but she let me do it.  She cried for a moment, and then opened her eyes and realized it was ok.  She was ok.  I cried...she was my Emily again...the most beautiful little girl in the world.  I hugged her probably for more of my own comfort than hers, and after wiping away some tears, I asked her if she wanted to look in the mirror and see how she looked.  And as she held the mirror up to her face she said "look mommy, now my eyes look the same again!"

In that bittersweet moment, there were no words to say other than, "yes Emily, they are!".

The First Time Emily Read To Me In Braille

September 30, 2014

Today, Emily came home with Braille homework. The night before I had watched her struggle to read some fairly simple words that had been enlarged for her.  She was having such a hard time just seeing the words, reading them was even more difficult.  She didn't like to read out loud in Braille at home.  I'm not sure why, maybe she just didn't want to be bothered with it.  So I suggested to her therapist that giving her homework might help.  So when she came home with Braille homework a week or so later, she knew she had to do it.  I can't quite articulate the emotions I experienced at the moment she began to read, but it was the first time she really read to us in Braille and I was in tears from the first word.  The video really just speaks for itself, but if I had to choose three words to describe it they would be strength, pride and hope...

Braille may one day, possibly sooner than we think, become her main way of reading and writing.,,it's a reality we all now have to learn to accept.  I am so proud of her! 

Viseobook - Emily's Coolest New "Tech Toy"

I visited Emily's school and got to see the new Viseobook that the NJ Commission for Visually Impaired was able to provide for her.  Its Awesome!  Its a portable desktop version of the CCTV that she had been using.  I love it and wish she could have one for home!


Check it out here:

http://www.aph.org/visiobook/

Why School Picture Day Is So Stressful

For the normal average parent, school picture day probably isn't very stressful.  The hardest part is deciding what outfit your child would wear, how their hair would be done and what backgroud to pick.  But for me, taking any kind of photo of Emily has always been a source of heartbreak and anxiety.  And school photos are the worst kind.

The stress of it all for me has deeply seeded roots that go all the way back to Emily's first Christmas in 2007. She was only about a month and a half old when we went for our traditional family Christmas photos. That year, the JCPenney studio we usually go to had hired new people to accommodate for the holiday busy season.  Normally it wouldn't have been a big deal, but when you combine an inexperienced photographer with an emotional postpartum mom and a newborn who can't see, the results were bound to be disastrous.  The raw emotion of our new baby's uncertain future always sat just beneath the surface of the "happy new mom" exterior that I tried to portray in public. Inside I was anything but happy.  A myriad of emotions ran through my veins at any given moment, not knowing when they would rear their ugly head and boil to the surface, spewing out for all to see. That day, in that studio...it happened.

I don't remember what her name was, but she was "older"...not a fresh young girl who would welcome the idea of working with a new baby and 2 boys under the age of 5. She was in a hurry, we were a number to her and she was definitly NOT a "kid friendly" person.  She had no idea how to get the boys to smile and I could feel my blood pressure rising as we came to doing shots of just Emily or the boys and Emily together. This precious little girl, all dressed up in her pretty dress, tiny shoes and matching beret hat could not see anything. She couldn't see the camera, she couldn't see the photographer and she couldn't see me.  Her eyes wandered all over the place, most of the time they were not even both open at the same time.  And even after explaining to the woman that she couldn't see, we just wanted a good photo of her looking "in the right direction"...they turned out terrible.  The girl in the photo was not my daughter, she wasn't the little princess I dressed up earlier than day. She didn't look....normal.  I stood there at the computer crying my eyes out while this insensitive woman kept trying to tell me they looked great, that she looked perfect. I remember being so heartbroken that I just kept crying and saying that I hated the pictures, all of them and that I didn't want to buy any of them.  Thats when another photographer came over and started trying to calm me down. I explained how unhappy I was with not only the photos but with the photographer herself, that she was so insensitive to our familys needs.  I took a deep breath and they reassured me that they would try again to see if someone else could get better photos.

After talking with Alyce, a sweet young girl named Rebecca came in and was very helpful in working with us in getting some better shots of Emily alone and with the boys.  I didn't expect a miracle, but for a professional photography studio I did expect some level of understanding that not every family can just walk in, smile, pose and be done.  Rebecca and Alyce went beyond their studio time limits for us, and in the end the photos came out much better than the first ones.  I was so grateful for the time and effort they took to help make our family photos memorable, in a good way. To this day, I am very close with Alyce and keep in touch with Rebecca through facebook.  Rebecca eventually moved on from the studio and now Alyce is the ONLY person I trust when it comes to taking Emily's professional photos,  She knows how to talk to her, help her look in the right direction and is patient, knowing that she is working with a child who literally cannot control where she is looking.  She has truly become part of our family!

Some people might think that a picture is just a picture, but to me its a moment in time that I cannot ever get back.  Its a memory of what was happening in my life at that time, and years down the road it would become the only memory I will have of those moments.  I refused to look back at Emily's first Christmas and feel even more heartbreak than necessary.  It was a difficult time in our lives and I didn't want to look back and not recognize my own daughter, nor did I want her to look back at those photos, if she would be able to see them at all, and feel bad about herself.

How does all of this relate to school pictures? Well fast forward about 5 years to when Emily was in preschool.  It was the year she lost vision in her left eye, and her eye began to shrink, cloud over and her eyelid no longer opened very wide.  The school photographer obviously had no sense of how to capture her in a aesthetically pleasing way because she wasn't smiling, looking at the camera and her good eye was turned very far inward.  Keep in mind that this was the BEST of the multiple shots they took of her that day.  When I got the photos back, I actually broke down in tears, it was so hard to look at because the girl in that photo was NOT my daughter.  It was the most visually disturbing image of her that I have ever seen.  A few days later I drove to the studio office and asked for my money back. They said I could look at the other images and see if one was better, but none of them were acceptable at all.  So they refunded me my money.

The next year, Kindergarten, I wrote her teacher an email asking if she could please accompany Emily to the photo area and help her know where to look and guide her in how and when to smile. I cried that morning, knowing that I wasn't there to help her. I had no control over how and when the camera shutter would click, how many shots would be taken and if she was smiling, eyes opened or closed or even looking in the right direction. Its something normal sighted parents take for grated. They send their kids to school and don't think twice about how their smile will be captured.  For me its a stressful and depressing unknown.  Its something I struggle with every time I try to take her photo myself...I want her to look her best, and I want her to look as "normal" as possible.  I have to take dozens and dozens of photos to just be able to pick out the best one or two that look "ok".  When we are with friends or in a group setting, my heart breaks because all those other moms are able to just say "smile" and they click once or twice and get a great photo of their child's beautiful eyes looking straight at them, often with a perfect smile to match.  Emily doesn't fit into that mold, and I often lose the photo opportunity that everyone else gets because it takes so long to get her to understand where to look and smile.

While her Kindergarten picture came out ok, it wasn't great.  But it wasn't terrible either.  So this year, first grade, I once again recruited her teacher to try and help her out.  This is how she looked before school that day. I posted it on facebook with the caption "school picture day for Emily, aka the most stressful day of September". A few people asked why it was so stressful, and I once again had to explain...

Hospital Follow Ups

Aug. 25-26, 2014

Emily did really well taking the oral antibiotics, steroids and eye drops.  By the time we headed to see Dr. Lalin, the retina specialist that Dr. Morgan had suggested, most of the redness and swelling had gone away. Dr. Lalin's office was in Morristown near the hospital and we had never been there before.  We waited a loooooong time. And as a new patient, they knew nothing about Emily.  Not a surprise that when we finally got called into the room for the nurse to talk to us and do the preliminary exam, that we left just a minute later...she was blind in the eye  we were there to examine. After what we had just been through the last thing I wanted to deal with were people who had no idea who Emily was or what her medical history entailed.  The girl in the first exam room was short with me, so I was short with her in return. No she doesn't see any light, shadows or objects. She's blind in that eye and we are just here as a follow up to make sure there is no infection inside. We left the room and I went back out to the waiting area where we once again...waited. Over all before we were actually brought back into a real exam room we waited a hour and a half.  Then we waited another 45 minutes before Dr. Lalin came in.  We quickly reviewed her latest issue and then were escorted to yet another room, where we, yep you guessed it, waited. He came into the room, did a quick ultrasound of her eye and confirmed that there was no infection inside the eye.  He also chimed in with a very condescending comment regarding her seeing eye saying "her cornea graft is failing, it should probably be replaced".  Thanks, I didn't notice that before.  I don't feel we needed to go see him at all...such a waste of my time and money.

The next day we went to see Dr. Kane, and he did a much more in depth exam of both eyes, as well as his own ultrasound.  He agreed, no infection inside or behind the eye which was good news. Also good news was that her pressure was still good in the right eye, maintaining at around 12.  However, he did feel that the cornea was starting to interfere with her vision, he couldn't really get a good look at the cataract or the retina because the cornea was so rough and hazy.  He agreed...she needed another transplant.

The infection was gone, but cornea surgery was once again on the horizon.  It wasn't a surprise, I knew it was coming...I just didn't know how soon it would be.

We were barely recovering from this crisis...I don't think I could tackle another one so soon.

Peri-Orbital & Orbital Cellulitis

Aug. 18 - 21, 2014

As hospital stays usually go, you don't get much rest...which seems counter intuitive because in order to heal or get well, rest is usually the first thing your doctor tells you you need. I get it, nurses have orders to take vitals and dispense meds at all hours of the day and night, its their job.  Christine always made sure she was super quiet and quick...letting us go back to sleep right away.  No bright lights switched on or loud sudden entrances into the room.  Most nurses just walk in like its 2:00 in the afternoon and everyone is wide awake! I truly appreciated how much she tried to avoid disturbing Emily.

When we woke up Monday morning, they brought us breakfast and we actually had to switch rooms. After we ate breakfast, Dr. Morgan came in to examine her and told us that he personally looked at the CT and in his opinion the pocket of fluid inside her eye was really the retina that had detached, not a sign of infection.  So it appeared, at least for the moment, that no surgery would be needed, THANK GOD.  However, once she was discharged we would need to see a retina specialist just to be sure.  He diagnosed her with Periorbital and Orbital Cellulitis, which is an infection of the interior and exterior skin surrounding her eye and inside her eye socket. I didn't get very much detailed information about what any of that means or what causes it, so I of course googled it.

Basically everything I read says that it can be caused by one of a few different types of bacteria, most commonly Haemophilus influenzae, Staphylococcus and Streptococcus. The first of these three , Haemophilus influenzae, is the common cause for sinus infections, but Emily wasn't sick prior to showing signs of the eye infection.  She also didn't have a Staph infection or Strep, although I was suspicious of Strep on the day this began because she had thrown up a few times.  So how else could she have gotten this? Other ways bacteria could infect the eyelid or skin around her eye include scratches, insect bites and other skin injuries...none of which she had.  I started thinking outside the box and only came up with one other possible reason...water. It was summer after all and she had been swimming in my in-laws pool 2 days before she showed any symptoms, and in the lake at my moms house a day or so before that.  I asked Dr. Morgan if bacteria from the water could have gotten into her eye and caused this infection, he said it was "possible"...but without a scratch or place for the bacteria to enter the skin, it wasn't likely.  We will never know how or why this happened, which is frustrating because we want to make sure it never happens again! 

One of the biggest risks involved with orbital cellulitis is that if it isn't treated quickly enough, it could cause permanent damage to her eye and cause blindness.  While the issue of vision loss wasn't a concern because she already had no vision in the left eye, I was scared out of my mind that it could spread to her good eye. I asked probably a half a dozen times and  I was always reassured that there was little chance of it spreading.  There is a "wall" so to speak (called the septum) that acts as a barrier between the eye sockets. I was of course relieved to hear that but still scared...you never know what can happen.  

Emily had been on very strong IV antibiotics, fluids to keep her hydrated and a round the clock series of eye drops.  Thankfully the nurses and doctors trusted me to give her the drops, it made me feel like I had some control over helping her get better.  The nurses had to scan her bracelet and then scan the drops so there was record of them being given, but they often scanned them all and left them with me to give to her 5 min apart.  Monday was rough, I could barely open her eyelid to get the drops in, and the swelling seemed to be continuing to get worse.  The infection wasn't responding to the antibiotics as quickly as we had hoped.  Jason and I had both stayed with her Sunday night, but there was only room for one of us to stay the next night, so Jason took the boys home and planned to come back the next morning.  Needless to say I didn't get much sleep. Nurses for both Emily and the girl she was sharing a room with were in and out all night and the awful chair that converts to a bed was ridiculously uncomfortable to say the least.  I tossed and (tried) to turn, but all I could do was worry about her.  I should have asked if they had any Xanex available for parents...it should come as an add on to your meal choices for each day.  Seriously.

When Dr. Morgan came in on Tuesday with the on call doctor who had seen her the day before, they both agreed she had begun to turn the corner. Yes, the swelling was bad, but it wasn't getting any worse and looked slightly less red.  She would remain on the IV antibiotics and eye drops, and stay overnight again that night, with hopes she would be able to go home the next day.  I wasn't prepared for more than 1 night in the hospital.  But to be completely honest, I would keep her in the hospital for weeks if its what was necessary to get rid of this infection.  I hated seeing her like this, but once again she proved to me that nothing will keep her down.  The child life specialists came to visit after the doctors left and brought her some paints and playdough to play with.  And the second she could get up out of her bed, IV attached of course, she wanted to walk down with her IV pole to the playroom and get back to being a kid.  It was so good for her, and us too.

Wednesday morning, things looked better, not drastically, but there were improvements. The on call doctor and all the residents made rounds in the morning, just as they had done everyday. I kind of felt like I was on Grey's Anatomy with the head resident presenting Emily's case and everyone else answering questions about her condition and meds.  I was able to be part of it and ask questions myself if I wanted to, but most of the time I just stood in the background and listened.  A decision had been made to transition Emily off of the IV antibiotics and onto oral medication to prepare her to be released.  But they agreed that she should be kept overnight once again so that they can monitor how well the oral meds are working. I agreed that it made sense to keep her one more night to observe how things went once off the IV.  She would be on 2 oral meds, an antibiotic and a steroid, plus all but one of the eye drops she had been on so far.  They stopped the Timolol since we knew it wasn't an issue of eye pressure.  

We made it though one more day, spending a lot of time outside the room, now that she was free of her IV it was easier to move around the floor.  Emily loved the playroom and even spent some quality time playing with a younger girl with cancer.  The simplicity of the two of them playing seemed so innocent, just two girls being kids, despite the difficult pain and suffering they both shared in their own way. It heartwarming and heartbreaking at the same time, very bittersweet. That night, just as she had previously, Em spent some time in the media room playing video games with Matthew and Tyler.  We even went back down to the playroom and had a family game of Sorry.  I could tell Emily was feeling a little better because she got bored and kept leaving the game to go play with other toys. So I won for both of us :)

 We ordered  just about the same things as we had been from the hospital cafeteria for every meal. Emily would only eat either the chicken fingers or mashed potatoes...she even asked for them at breakfast and I had to say no, even though I'm sure we could have gotten them if we really wanted to.  Thank god for the small snack room where we could replenish simple things like juice, soda, Lorna Doone cookies, crackers...and coffee.  Its basically what we lived on since the meals were, well...hospital meals. I think that speaks for itself.  Sadly our days at the hospital were becoming more routine, and it almost felt like we were "settling in".  I hated feeling like that, it wasn't home, this place wasn't comfy or cozy.  It was designed to help kids feel more comfortable, but it shouldn't ever feel like home.  I had a feeling that the beautiful bald headed little girl in the playroom felt more settled in than we did and that makes me sad. This was hopefully our last night here and I was getting antsy to go home.  We lucked out and had our less than desirable roomate leave during the day and were able to move over to the larger side of the room, and hoped that we would be able to get a better nights sleep without noise from the other side of the curtain. Jason brought home alot of the bags of "stuff" we collected over the last few days including most of the stuffed animals and gifts friends and family brought her when they visited.  I couldn't fall asleep...too many things running through my mind, so I stayed awake watching TV until it automatically shut off around 11:00.  I eventually fell asleep but was woken up around 1am with the noise of another patient coming into our room...a baby, who was coughing and crying on and off all night.  The second I fell asleep...he'd cry and wake me up. Yes...I was ready to go home.

Thursday morning I was up and ready before I normally would have been.  Jason arrived and we were ready to go home...except we had to wait...and wait...and wait.  The doctors were later than normal for rounds, and we were helpless without discharge info and scripts for medication.  I was crawling out of my skin.  Emily's eye looked alot less swollen, still red, but not nearly as much.  If they didn't discharge her today I might have seriously lost my mind!

Eventually we were cleared to go home and no one was happier than Emily, she missed our two dogs, one of which we had just adopted days before she ended up in the hospital.  She was scared Cassie wouldn't remember who she was.  I assured her that no one, even puppies, could ever forget her!  We left with instructions to see a retina specialist, Dr. Lalin, in a few days and numerous scripts for meds. The nurses were kind enough to send us home with the remaining pre-dispensed dosages that she would have been given that day, which was a blessing since it meant we didn't have to rush to the pharmacy. We could just go home and rest.  

We hadn't planned on a 5 day hospital stay, but we got through it.  It wasn't easy on any of us, but Emily was probably the most tolerant of the situation.  She was the one going through this and incidentally was the one who complained the least.  God Bless Her. I wish I was as strong as she is.

The photo below shows a good side by side comparison of what her eye looked like on Monday, at its worst...and then on Thursday after we were discharged. On the left, she couldn't even open her eyelid it was swollen so much.  On the right, there was still some interior redness and swelling, but it was much, much better.

Needless to say, the puppies were super happy to see her when we got home :)

An Unexpected Hospital Visit

Aug. 15, 2014

Today started out as a pretty normal day.  The kids and I took our new puppy Cassie to the vet for the first time and then hoped to spend the rest of the day doing some fun summer stuff.  But after we got home, Emily started complaining that her left eye hurt, specifically her lower eye lid.  I checked to see if maybe an eyelash or something else was in her eye and that's what was causing the irritation, but I didn't see anything.  I flushed out her eye well with some saline drops and she went back to playing.  A few minutes later she came back complaining of the same thing. I didn't see anything wrong but repeated the drops again.  By the third time she came back to me, now crying in pain, I knew something else was going on.  I gave her some antibiotic drops and Tylenol, and told her that it might help if she went to lie down in her bed and rest her eyes.  A little while later I heard her crying for me and running to the bathroom, to throw up.  Not a good sign.

My immediate thought was that she either had a stomach bug or strep throat.  Strangely enough Emily doesn't get a sore throat with strep, she throws up and gets a fever.  So if she developed a fever I knew we would be headed to the doctor.  She threw up one or two more times, and then eventually fell asleep in bed.  I kept checking her for a fever, but she never felt warmer than normal.  A part of me wondered if the upset stomach and eye pain were related, but it didn't make much sense at the time.  I thought about when we were at the Vet's office earlier, she was touching the exam table and had pet another dog that was in the waiting room.  Maybe she had touched something and then rubbed her eye, causing the irritation?  But how did the upset stomach come into play? Could it be a complete coincidence that these two things happened at the same time, but weren't caused by the same thing?  I wasn't convinced.  I kept checking Emily's eye throughout the night, it did begin to get irritated and bloodshot and she continued to say it hurt.  I had hoped that overnight it would resolve itself.  Although it was her left (bad) eye, I was still concerned about it being an infection that could spread to her right eye.

Ironically we were supposed to go to the Ocularist to get her painted prosthetic lens the next day...obviously we needed to cancel that appointment which was disappointing since we were making such good progress with the lens.

Aug. 16, 2014

I woke up before Emily and went in to check on her.  She didn't feel like she had a fever and hadn't thrown up all night.  I was really hoping she was better, sadly that wasn't the case.  When she woke up, her eye was even more red and swollen...and it was a lot more painful.  I gave her more eye drops, Tylenol and let her put a warm wet washcloth over it to see if that would help at all.  It didn't seem like pink eye, there was no discharge and it wasn't itchy.  Because it wasn't a cornea related issue, my first instinct was to call Dr. Kane...but it was Saturday. Why does it seem like all emergency issues happen on a weekend when you can't get in touch with your normal doctor?  Am I the only one this ever happens to?  Because it happens almost every. single. time.  Anyway, I had Dr. Kane's cell phone number, because in the past we've had to reach him for surgical follow up issues when his office was closed, but I didn't think that was his weekend emergency contact number.  I called his office line and got a message that said the office was closed for the next week...wait, what?! Ugh, frustration set in and my blood pressure began to rise.  The message said that he could be reached at his emergency number, which apparently is his cell.  But in my head, I knew if he was on "vacation" from his office, that he was at his upstate NY home, which was over 2 hours away.  I know this, because Emily and I were invited to visit there once after she had surgery just before Thanksgiving one year.  IF she needed to be seen...either he would suggest going to NY Presbyterian emergency room, or up to his NY home.  I didn't want to interrupt his vacation if I didn't have to, so I called Dr. Morgan, who is her more local Ophthalmologist.  If anything, he and the hospital he works out of were a lot closer than going to NY.

Around 9:30 am I called Dr. Morgan and of course got his emergency line.  I had his service page him.  Over an hour later he hadn't called back, so I had him paged again.  Another hour or two passed.  Within that time, Emily had thrown up again, which was strange since she was fine all night and had eaten something earlier.  I called a third time...this time I received a phone call within a few minutes.  He apologized for not returning my calls earlier, apparently he was at the gym and someone went into cardiac arrest and he had to do CPR to save the guys life.  OK, valid reason for not answering a page.  I explained what was going on with Emily and he suggested that he call in some oral antibiotics, Augmenten specifically, to try and get some more meds in her system since it seemed that there was some kind of infection going on.  When I mentioned that she had recently thrown up again, he said that if she can't keep the medicine down that we would have to bring her to Morristown Hospital where she would need to get IV medication.  Wonderful.  If she was able to keep it down, he would call us first thing the next morning (Sunday) to see how she was doing.

He called in the medicine to the pharmacy right away, except when I got there to pick it up, it was Amoxicillin instead of Augmenten.  I figured that maybe he changed his mind on what to give her and didn't really question it.  Thankfully she was able to keep the meds down and spent the rest of the day sleeping on and off.  She would wake up asking for another warm washcloth for her eye and then go back to sleep.  It was obvious that whatever was going on, really was taking a toll on her body and not just her eye.

Aug. 17, 2014

Dr. Morgan called just after 9:00am on Sunday morning.  Unfortunately the antibiotics had not helped because Emily's eye was much worse than it was the night before.  He suspected that it could be more than just an infection, that maybe it was a pressure related issue and he was going to contact a retina specialist to consult with him on it.  He would call us back in an hour and let us know what we should do, suggesting it might be necessary to bring her to the hospital.  After hearing that, I prepared for a hospital visit just in case and then waited...and waited...and waited.  I knew in my heart that this wasn't a pressure related issue.  Her eye wasn't functioning at all since she lost all vision a couple years ago, so there was no reason for a build up of pressure.  It made no sense to me.  I tried calling him back on his cell phone a couple of times and left multiple messages.  It had been over 3 hours since we had spoke and with the amount of pain Emily was in at this point, I made the decision to just bring her to the Emergency Room.  Her eye was getting worse and I couldn't wait any longer to get her help.

On our way to the hospital, I called and left another message telling Dr. Morgan that we were on our way down.  We stopped and dropped off the boys at my parents house and then made our way to the ER.  When we got there I told the nurse that I had been talking to Dr. Morgan and explained the whole ordeal over the last 2 days.  I also called again and left him another message letting him know we were now AT the hospital.  When we were finally seen by the ER doctor, I was told Dr. Morgan had called and spoken to them and told them he felt it could be an issue of high eye pressure. They insisted they try to take her pressure, give her 3 different glaucoma drops and start an IV for fluids and antibiotics.  If it helped to reduce the eye pressure, redness and swelling then they would have some explanation as to what was going on.  But I knew the eye pressure concern was a waste of time.  Dr. Morgan did call me and tell me that he spoke to Dr. Lalin, a retina specialist, and this is what they felt would be the best course of action to start with.  I told the nurse and the ER doctor that she had dealt with high eye pressure in the past, we know exactly what it looks like when the eye is bulging or a bubble forms in a weak spot of the cornea graft.  This was not that AT ALL.  At that point I bit the bullet and called Dr. Kane.  I hated to do it, but I knew the doctors were going in the wrong direction with this.

Dr. Kane immediately agreed with me that this was not a pressure issue, but by that point they had already given her the glaucoma drops and were waiting to take her pressure again.  He felt very strongly that there was an infection of some kind going on and that either an ultrasound or a CT of her eye was needed to make sure the infection wasn't inside or behind her eye.  What he said next made me feel like I got punched in the stomach and stabbed in the heart at the same time.  If the infection was either inside or behind the eye, it would be necessary for them to do one of two things; either do surgery to go in and clear out the infection, or remove her eye completely.  I felt sick.  I wanted to throw up.  I wanted to scream at the top of my lungs that this wasn't fair.  I was not mentally prepared for that at all and emotionally it would wreck me.  It would damage Emily's spirit and I couldn't imagine how she would handle losing her eye completely.  This just couldn't be happening, not now, not to her.

When I got off the phone with Dr. Kane, I explained everything to Jason and inadvertently Emily overheard the part about removing her eye.  She started to get upset and crying about not wanting them to take her eye out.  I tried to reassure her that I would do my best to make sure that didn't need to happen.  I quickly found the ER doctor and explained that Dr. Kane insisted they do an ultrasound or CT.  There wasn't an ultrasound technician on duty that would be qualified to do it on her eye, so we were forced to do the CT, which was fine because it would show in more detail what was going on.  Within 10 minutes she was brought to radiology.  They ordered the CT with contrast and I waited outside in the hallway.  I remember standing there saying a prayer quietly, but out loud. "Please let her be ok, please let her be ok, please let her be ok..." I probably said it 50 times or more before she came out of the CT room. Images of her with no eye kept flashing through my mind, I had to force myself to push the thoughts of  her wearing a full prosthetic eye for the rest of her life out of my mind.  I just couldn't go there. Now, we had to wait for the results.

Back in the ER room, we sat...and waited, and waited, and waited.  Emily's eye was getting more swollen and her pain level was increasing.  While we were waiting for the CT results, they gave her a dose of morphine to try and help alleviate the pain she was in.  A short while later the doctor came in and said that the CT showed a fluid pocket inside her eye which could be a sign of infection, but nothing was seen behind the eye, which was at least some good news. I couldn't help thinking that since her retina had detached a couple years ago, is it possible that the retina was what the radiologist was seeing as a "pocket of fluid"?  Maybe, just maybe, it wasn't what they thought it was.  Because of the location of the "fluid", a retina specialist would need to determine the next course of action.  They decided to admit her overnight in hopes the IV fluids and antibiotics would help and that by morning the swelling would be less.  Ironically they never bothered to re-check her eye pressure so I guess Mom's do know what they are talking about sometimes. I just wish they would have listened to me from the beginning, it would have saved some time and aggravation.

The silver lining of todays ordeal...A good friend of mine is a pediatric nurse at Goryeb Children's Hospital in Morristown, where we were, and I had texted her earlier to let her know Emily would probably be admitted overnight. She just happened to be on duty that night and we were lucky enough to have her as Emily's nurse!  What a blessing because it put me at ease knowing that Emily would be in excellent hands! We were able to get situated in a room and chat for a little bit about what was going on.  It was nice to see a familiar face, one who already knows so much of Emily's medical history.  Jason's brother and his family happened to be passing by the area and decided to stop by to see Emily and say hello.  At first when they arrived, Emily seemed to be awake enough to know they were there, but she sat up in bed and the morphine must have kicked in...wow was she loopy!  I haven't ever seen her like that before, it was kind of scary, but we knew it was the meds and that it would wear off as she slept.  Then I noticed something really weird. As Emily was taking a sip of juice, her face started to get all blotchy, not all in one place, but in random spots. Christine, our nurse, also noticed it, but then it was gone!  Weird.  She at first thought it could be a reaction to the morphine, so that's what we were attributing it to.  But as it kept happening over and over again, we got to thinking...what else could it be from? There was no rhyme or reason to it, it showed up in different places each time and was gone as quickly as it showed up.  Then I remembered that she had the CT with contrast...could it have been a reaction to the dye? We might never know, but within a few hours it stopped happening.  We settled in for a long night, and tried to get some sleep, we knew the next day would be a difficult one.

Chronic Deterioration Of The Cornea

Aug. 1, 2014

After 3-4 days of not seeing the bubble on Emily's cornea, we thought it might have been a fluke thing.  But it finally came back...and only for a brief moment or two.  Jason's parents were watching the kids while I worked a couple of days during this week, and of course the bubble has to resurface when I'm not around right?

It went away as quickly as it showed up.  In fact, my mother in law wasn't sure she even saw it.  But I'm sure it was there.  So I called and made an appointment for Dr. Zaidman to see her.

I hadn't seen the bubble personally in a while, and it wasn't there the day we went to Zaidman's office.  But based on what I described and the current condition of the cornea, Dr. Zaidman said that what we saw was ultimately a corneal blister.  Its a fluid fillled bubble that may come and go, which was the case for Emily.  He went on to describe everything that we had seen and experienced thus far. 

The reason for the appearance of the bubble is fairly simple, it's a sign of chronic deterioration of the cornea.  As the cornea starts to fail, the 5 layers stop absorbing fluid or letting fluid pass thru them properly. The bubble is essentially an indication that at least one or more layers are no longer working as they should. When the bubble appears, it's filled with fluid. If it disappears the fluid has eventually been reabsorbed. The danger is when the bubble appears but doesn't not go away. If that were to happen, it means the cornea has stopped functioning and Emily would need another cornea transplant as soon as possible. 

So as long as the bubble continued to come and go, we could hold off on the transplant and hope that things would improve. 

For the moment, we were cautiously optimistic since the bubble hadn't been showing up very often. I tried to not constantly look for it, because up until this point I was obsessing, looking at her eye every 5 min. 

My heart was breaking because I knew her eye was failing. But at the same time I felt somewhat relieved that whatever was happening wasn't of an immediate concern. The panic I felt that day at the shore when I first saw it had subsided, but honestly it was always just under the surface anyway. Each time something doesn't look quite right with her eye, my heart jumps out of my chest and runs around bleeding all over the place...It's a vicious cycle I can't seem to escape from. 

A Bubble...

July 24, 2014

In a previous post, I referenced the fact that we never seem to be able to go on summer vacation without there being some kind of crisis with Emily.  Why can't this poor girl just enjoy summer without having some major problem ruin it?  Maybe its a coincidence, but I honestly doubt it.  Last year it was a trip to the local ER for a virus that manifested itself  in the form of a fever and rash. This year, our vacation was turned upside down by a bubble.

On the first day of the year, we sat down as a family and wrote a list of things we wanted to do during 2014...a bucket list of sorts.  Our goal was to cross off as many of those things as we could, realistically speaking of course.  One my contributions to the list was to go crabbing while we were down in Ocean City.  I used to do it all the time with my dad as a kid, and we hadn't really done that yet.  The kids like to go surf fishing with my dad while we spend the day on the beach, so crabbing on a chillier, overcast day seemed to make sense.  Why waste a good beach day right?  On this particular Thursday, only having 2 full days of vacation left, we decided to try a few different crabbing spots.  Jason took the kids for a bike ride on the boardwalk in the morning.  I didn't go on the bike ride because I wasn't feeling very well that day, so I went with my parents, sister and sister in law and found a small dock near the marina to set the crab traps.

Jason and the kids took a while to get there, and by that time we had already caught a few crabs.  The kids were so excited to see what we caught, but Emily was afraid to look at them too close.  She was standing next to me while I was sitting down on the dock, and as I looked up at her face, I saw something that immediately stopped me in my tracks and made my stomach turn.  A bubble...on the center of Emily's right cornea.  I wanted to include a photo of the bubble so everyone could understand what we saw, but I lost all the photos from my phone.

It was like someone punched me in the stomach and stabbed me in the heart at the same time.  Its that feeling you get when you know something really bad just happened and you have absolutely no control over it.  You have no way of changing it or making it go away.  You can't fix it, un-do it, go back and time and prevent it or go into the future to see if everything will be ok.  It's the kind of moment I have had over and over and over again since Emily was born.  I felt my stomach turn, my heart begin to bleed internally and tears prick the corners of my eyes.  But I had to hold it together.  I didn't want to alarm Emily, and scare her. I also didn't want to draw attention to the problem before I could process this in my own mind.  I asked Emily if she could see ok, if her eye hurt, or if anything felt wrong to her.  She said no, it didn't hurt and yes she could still see fine.  I took a few pictures of what I saw, and my sister saw me wiping away my silent tears. She asked me if I was ok so I quietly told her and Jason what I saw and we all knew it was bad. We finished crabbing and headed back to the house.

When we got back to the house, I gave Emily extra antibiotic eye drops just in case there was some kind of bacterial infection going on.  Speculation began to grow about how and why this was happening.  Did a piece of sand get in her eye?  Was it the salt water?  Did she get poked by a beach toy or sea shell? Was it caused by severe dry eye from the wind?  Was her eye pressure elevated?  All kinds of theories were being considered.  I put a call into Dr. Zaidman, her cornea doctor, and of COURSE its after hours and no one is in the office.  I called the emergency number and had them page the Ophthalmologist on call. Because I was trying to keep the problem from becoming an all out vacation ruining crisis, I didn't alert my mother to what was going on.  I needed to take care of this without the added anxiety and second guessing.  I was doing what I needed to do, call and talk to her doctors.  Now I just needed to wait to hear back from them.

We all went about the task of preparing dinner; the crab we caught, a nice steak and other seafood we bought at the market.  Our traditional seafood dinner while at the shore was something we always looked forward, I didn't want to ruin it for anyone.  So I put a smile on my face, poured a glass of summer wine and tried to hold it together.  When the phone rang with a NY area code, I went out to my car and took the call.  I didn't want anyone hearing this conversation.

I gave the resident ophthalmologist a brief run down of Emily's history and then explained the bubble that I saw.  He gave me his cell phone number to text him the picture I had taken, so he had a better idea of what I was talking about.  We hung up so I could send the photo and he said he would call me back.  Later on, he called again and explained that it was hard to determine what was going on without actually seeing her...(no shit, I hate this answer...for gods sake just tell me what you think it could be and if its an emergency situation or not).  He went on to say that there could be a number of reasons that this occurred, and that the best way to help her was to take her somewhere to be seen. Because we are 3 1/2 to 4 hours away from the doctors and hospital I trust, we had a decision to make.  Either we could take her the CHOP (Children's Hospital Of Philadelphia) which was about an hour and a half away, or wait until the next day and Dr. Zaidman's office would call me with the names of doctors in the area that he would recommend going to.  Dr. Z would call and speak to whoever we decided to see.  The resident left the decision up to us and told us to call him back if we decided to go to CHOP so he could get in touch with someone there.  Otherwise, we would hear from the office in the morning.  Obviously he didn't seem to think it was a true emergency situation, or he wouldn't have given us a choice.  

I hung up and immediately fell apart.  A wave of various emotions washed over me in a matter of seconds.  I was scared for Emily and what this could mean for her vision.  But there was a part of me that felt defeated, disgusted and surprisingly angry.  Our family vacation was over. Once again, we couldn't just enjoy our trip.  We couldn't escape the reality of Emily's medical problems. There was no escape, there was no vacation from her eye disease, we would never be free from it.  It consumed our lives and that made me more angry than words can express.  It made the decision I had to make, both for Emily and our family, even more difficult.  Was it selfish of me to just want to finish our vacation and deal with this when we got home?  It made me sick to think we would be spending the last full day of our trip in a hospital or doctors office instead of on the beach.  I mean physically sick.  I wanted to throw up, and I almost did right there in my car.  I was sobbing uncontrollably, banging my hands on the dashboard out of anger and frustration, desperate to wake up from this nightmare.  A large part of me was sad that either I would have to take Emily by myself and leave Jason to spend the day with the boys, or that Jason and I would both go, therefore depriving them of spending the last day on the beach having fun as a family.  Or, a third option was to just pack up, leave a day early and drive Emily straight to Dr. Zaidman's office the next day.  Why would I waste time and money to go see doctors who don't know her, needing to explain her entire medical history to someone
WE don't know...someone we don't trust.  Ending our vacation early was a real option for me, I didn't want to do it, but I would if it meant doing what was best for Emily.  I must have been crying so loudly that someone, I honestly don't even remember who, came out to the car and opened the door to see if I was ok.  It might have been my sister or sister in law, it could have even been Jason. I really don't remember.  All I remember is how I felt...helpless.

I eventually calmed myself down enough to be able to walk back inside.  I found Jason and we discussed what our options were.  We were leaning towards waiting until the morning to either find a doctor or just go home early, going to Philadelphia seemed like the last thing we wanted to do. But then...I looked at Emily's eye again, and the bubble was gone.  Just Gone.  She wasn't in any pain or discomfort and she was acting fine.  WHAT JUST HAPPENED??????  Did the extra eye drops help to alleviate the problem?  That's the only thing we did differently, its the only thing that made sense.  My heart suddenly didn't feel so heavy...and maybe...just maybe,,.it meant that we didn't have to end our vacation early.

We had a very nice dinner...and I tried to enjoy the rest of the night to the best of my ability.  A bottle of wine helped.  A lot.  None of us (who were aware) could believe that the bubble we all saw, was no longer there.  We all assumed that if it had popped, that she would have been in pain, or we would have noticed real damage on her eye.  But it looked fine, or at least as good as it did prior to the bubble appearing and she never complained about it hurting.  It was one of the most bizarre things any of us had witnessed.  I just prayed it wouldn't come back again...

The next morning, her eye looked ok, no bubble.  Dr. Zaidman's office called me with the names of somewhat local doctors that we could go see.  But when I told Daria that the bubble disappeared within an hour of giving her the extra eye drops, she was shocked.  She relayed the information to Dr. Zaidman who agreed that if the bubble didn't come back that it would be ok to just wait until we came home to have him examine her. If he felt she still needed to be seen, we would have just come home instead of involving another doctor.  But he was ok with waiting until the following week as long as the bubble didnt come back.  While I was relieved to think we could try and have a nice end to our trip, I knew it would be jaded by the prior nights events.  I insisted she wear sunglasses at all times while she was outside, and if we went to the beach, she would wear goggles in the water.  We tried, and I use the word loosely, to have a normal day.  I fought off tears most of the day and was checking her eye constantly.  I struggled with just "letting her be a kid" playing on the beach.  She wasn't a normal kid, she doesn't have the luxury of throwing caution to the wind.  At any given moment her life can be turned upside down.  Its a difficult balance between the need to shield and protect her from the dangers that could jeopardize her vision and the thought of if she loses her vision tomorrow, she will never be able to experience life the same way again...

The bubble didn't return before we left to come home, so I had hopes that everything would be ok and that it was a one time freak thing.  Little did I know that as we were trying to enjoy the last day of bright sunshine on the warm sandy beach, the black cloud of 2014 was just beginning to form over our heads.