Saturday, June 27, 2009

No Glasses Needed!

Emily had an appointment with her regular Ophthalmologist, Dr. Morgan, on Thursday and he was very impressed with how well she is doing. Her corneas looked good. No scratches or ulcers. And he couldn't believe at how well she was walking around and engaging in everything she saw. "It's obvious she is seeing, If I didn't see it, I wouldn't believe it" he said.

She is slightly nearsighted but he feels she is doing well enough right now that she doesn't need glasses! I was so relieved at that because it's just one more thing for me to keep track of. We fought with her last summer with the glasses and now that she is a toddler with an opinion, I know she would probably hate wearing them. And if she's seeing well enough that she doesn't need them...then thats just FANTASTIC!

We don't need to go back to him for 4 months....she'll be 2 by then!

Finally, some good news...

Thursday, June 25, 2009

Meeting With a Social Worker

A few months ago I got a letter from my insurance company that made my heart jump out of my chest. It's purpose was to tell us that the hospital that Emily's goes to for her cornea surgery and care, was no longer a participating hospital. I went into panic mode...her surgery was coming up soon, so now what? Out of network benefits? What was our deductible? Fighting with the insurance company about which procedure code was covered and which ones weren't? I didn't need this, not now....

That letter lead to a conversation during one of Emily's therapy sessions. It was actually an annual review meeting with Linda her therapist, Andrea the early intervention district coordinator and Terri from the Commission for the Blind. I had mentioned the letter and questioned what services might be out there for us to utilize in a situation like this. There was a suggestion that maybe a social worker might be helpful to us, maybe she could help us tap into local or state resources.

A month or so later, I met with Julie who was assigned to Emily's case. At our first meeting I was encouraged by what might be out there for us. I had already applied back in January of 2008 for SSI (Supplemental Security Income) but we didn't qualify based on income. Everyone I talk to can't understand how we wouldn't have qualified because of the severity of Emily's condition, but apparently you have to basically be living in a cardboard box in order for the state to even look at your case. I can't remember what the income level was for a family of 5 but we were above it...that doesn't mean we can afford hospital bills, prescriptions and various other expenses that we incur because of Emily's condition. Who gets to decide that if you make X amount of dollars a year, you can afford to have an ill or disabled child? ? ? I think that is a bunch of crap, if the government can bail out all these major corporations because they screwed up their business and handled their money poorly, why can't they help a small family pay for their 18 month old's Glaucoma medication, or transplant surgery to enable her to see? I guess we made too much money and should budget better...

Julie had given me hope that she could come up with something we would be able to utilize, but at our next meeting she had nothing. She kept coming up with possible parent support groups, local clubs that might offer medical equipment if needed down the road and random other stuff. The support groups and local club information was great, but that wasn't what we needed. We needed financial help and she wasn't finding any.

At our next meeting, she once again really didn't have any information for me about financial help. She called Terri and spoke to her about a few things related to the commission, but Terri said that alot of the financial help and equipment related expenses that they were able to help provide families with in the past was not available anymore, mostly because of cutbacks in the program and the economy. The only 2 questions that we got answers to were ones that I asked...1 - how does the commission follow Emily through into school and 2 - what if Emily needed glasses, would they still be able to help provide them? I learned that once she turns 2 (which is 4 months from today) we should contact the school district and start the testing and evaluation process to qualify her for preschool....PRESCHOOL? Holy crap...Emily is going to be in preschool next year. WHAT? No, this can't be...this is my baby, my little girl who just started walking 2 months ago, I am sooooo not ready to even think about her going to school. But I guess the process takes a while, so she suggested getting her into the system as early as possible. As for the glasses, apparently the commission now works with Lens Crafters and can provide a voucher for one free pair of glasses per year. Good to know now that the place where we got her glasses last year is gone. Hopefully we won't have to worry about that anytime soon, but at least we know it's there in the event that we do.

Ironically the two bits of information that I found helpful were answers to questions I asked as she was walking out the door. Re: would that work? So many people have asked me about setting up a fundraiser for Emily, but I have no idea how that would work, or even if I would feel comfortable setting it up for her myself. She told me she would get information for me and let me know what she comes up with. The other thing I mentioned was about the ridiculous cost of all of her medications....then all of a sudden she says...oh give me a list of what she's on and we can work with the pharmaceutical companies on subsidizing or eliminating your out out of pocket expenses.

I hope she can come up with something, we are barely keeping our heads above water with these medical expenses.

2nd Cornea Transplant and Cataract Removal Left Eye

June 4th 2009:

Emily had her second cornea transplant in her left eye today. She also had a cataract removed and a lens implant put in.

This could very well be her last chance for a clear cornea and vision in her left eye. Dr. Zaidman had previously told me that if this one rejects, he's not sure he would feel comfortable doing the transplant a third time. With each surgery, the success rate drops. But hopefully we wouldn't have to go down that road.

We arrived at the hospital very early, went through the standard pre-op waiting...waiting...waiting...a few eye drops for dilation and antibiotics to avoid infection. Finally the nurses said it was time and I put on my white bunny suit and blue surgical cap that has become so familiar to us. I held Emily as we walked down to the OR doors and Jason said a teary goodbye and I love you to her, then I continued on with the nurses as the doors shut behind me. This time there was no 45 minute wait, we went straight into the OR where Dr. Zaidman was already waiting for us. The anesthesiologists quickly instructed me to lay her down so they could get started right away. She cried and put up more of a fuss than usual, but once the mask was on she calmed down. Within seconds she was asleep. I held her hand, kissed her cheek, told her I loved her and that I would be there when she woke up. "Be strong baby Girl" were my last words before I let go of her hand and walked away.

I walked back out into the hallway in tears, trying to get the bunny suit off before I completely fell apart. Once I saw Jason and we hugged, I just let it all out. I was so scared for her, this was her 12th much more of this can she handle? We slowly walked down the hallway and made our way out of the surgical area. I was starving and food seemed to be the only distraction I could think of at the moment. We went to the cafe, milled around for about 10 minutes before I finally decided on just a muffin and soda. We went and sat down in the back corner by a window. We didn't talk much, just ate our food and kind of sat in silence for a while. Jason put his ipod earbuds in and fell asleep, I emptied my purse and organized my wallet...anything to keep my mind off of what was happening. I sent a few text messages, talked to a friend for a few minutes and then resorted to just sitting and thinking.

I hate sitting in the waiting room, it always feels like time goes by so much slower. We sat in the cafe for about another half an hour before I woke Jason up and we walked down to the gift shop. I got a candy bar and picked out a cute stuffed animal for Emmy. It was our tradition, she always gets something from the gift shop. I wanted to get another puppy for her since she seemed so in love with the one we gave her a few weeks ago from her last surgery. Jason said "it doesn't matter what kind of animal you get her, she's still going to call it a puppy" was the first time I had smiled or laughed all day. I settled on a cute golden retriever.

We walked back down to the waiting room and sat down. I had just gotten comfortable, put my sunglasses on to make it darker in the room and closed my eyes, hoping to catch a small nap. Within minutes Dr. Zaidman popped his head in and said she was done. He talked with us as we walked back to the recovery room. He said she did very well, and he was very pleased with how the surgeries went. She did receive the lens implant to replace the cataract that was removed, and with the new cornea, we held high hopes that her vision would be intact and much better than it was before. But we'd have to wait until the next day to find out.

We went back into the recovery room where she was still waking up from the anesthesia. She was hungry, thirsty and just needed to be loved. I held her for the entire time we were in recovery and she drank as much as she needed to. We were released fairly quickly and instructed to come back first thing the next morning to see Dr. Zaidman. She slept all the way home and when we got there she was walking around fairly well even with her eye patched. She had no problems sleeping overnight, neither did we...we were all exhausted.

When we arrived at the hospital the next morning, I was nervous. I couldn't wait to see what her new eye looked like and how it changed her perspective on the world. We took the patch off and I could immediately see how much better her eye looked. It was not cloudy anymore, it was clear! Her window to the world suddenly got much clearer and I'm sure she knew it. Her doctor was very pleased with how it looked and explained the new eye drop schedule. The swelling wasn't bad either...and no bruising! It felt amazing, she had been given another chance to see and you couldn't get me to stop smiling.

When we got home, she was back to her happy self...playing and asking for 'cracker' and 'ba ba'. You could see that her vision was better, she wasn't hesitating as much while walking around and she just had this new found confidence.

It was awe inspiring...she could see again.

Perferation in Her Cornea

April 2009:

Yet another surgery to treat the Glaucoma...

Emily would be having the second cornea transplant and cataract surgery soon, but the pressure in her left eye needed to be brought under control first. At the last appointment with Dr. Kane, her eye pressure in the left eye was 28, normal is between 10 and 20. During an ultrasound of her eye, Dr. Kane was able to see that the valve and drainage tube was still working. Her eye was producing more fluid than the one tube could handle, so the plan was to put a second tube in to help drain the fluid more efficiently. However, a complication came up at the hospital even before she was in the operating room. Dr. Kane came to talk to us and check Emily out and was alarmed at what he saw. He believed that the weakened area of her cornea actually had a small perforation in it and if that was the case it would have to be taken care of quickly. He immediately called and spoke with Dr. Zaidman, her cornea doctor, and he agreed that if there was a perforation that it would need to be repaired.

The surgery took just over 3 hours and when Emily was in recovery Dr. Kane explained to us what he found. The lens of her eye was pressed up against her cornea and no fluid was in between, which meant that fluid was leaking out, basically her eye was deflating. A leak meant that there was a perforation. Because there was no fluid in between the cornea and lens, there was no space to implant the second tube. So the scar tissue surrounding the valve and first tube was removed to try and aid in the flow of the fluid. The perforation was fixed by using a procedure called Amniotic Grafting. Basically a small piece of preserved amniotic tissue, which is similar in make up to the layers of the eye, is used as a patch to help cover the area where the perforation is. I was once again amazed at how advanced our medical technology has become, but after doing some research on it I discovered that amniotic tissue has been used in surgical grafting since 1910 and in eye surgery since 1940. Just amazing!

Once the graft heals it will integrate itself into her eye and become transparent. But this is only a temporary fix. This new complication may change how quickly she will have the cornea transplant. She was scheduled for that surgery on June 4th, but I would be taking her to see Dr. Zaidman and would have more information then. The biggest risk right now is infection, if her eye were to get infected and pass through the tiny perforation, it would be disastrous for her. But she is on 2 types of antibiotic eye drops to make sure that doesn't happen. Once she has the transplant, that entire area should be replaced with the new cornea so we are trying to remain positive about the outcome and the vision she will have in that eye.

Just as it was back in February, Emmy's eye was bruised, swollen and red. But as usual she was just as happy as can be! It's heartbreaking as well as heartwarming to see such a happy little girl go through so much and always come out on the other side with a smile. She is definitely one special little girl!

Next Glaucoma Surgery

February 2009:

Next Glaucoma Surgery:

The surgery went well, although it was more complicated than expected. She had the valve in her left eye replaced because the first one was not working anymore. Apparently the entire valve was encased in scar tissue, which was not allowing any fluid to drain from the eye properly, making it impossible for her eye pressure to be controlled. We had noticed within the previous couple of weeks that the "bubble" (for lack of a better word) on her left eye was bigger than it was before so we knew the pressure was high. Dr. Kane said it was alot of hard work, but he got all of the scar tissue out and removed the old valve successfully. The new valve is apparently designed by Dr. Kane as he told us he had the company modify their design in order for it to work better. He placed this valve in a different area and at a better angle, so hopefully it will work much better for her.

After the surgery she had a patch over her eye for protection. The next day we took her back to Manhattan so Dr. Kane could examine her and when he took the patch off she had a pretty bad black eye. Her eye itself was really red and combined with the bruising underneath, it looked bad.

The next steps for her were to heal from this surgery and maintain lower eye pressure. Then in April she would have another cornea transplant and the cataract surgery done at the same time. We were unsure whether she would have a lens implant or not, since her doctor himself had not decided. If she does not get the lens implant she will have to wear glasses or a contact lens to maintain any vision in that eye.

We are taking one step at a time, and right now we are concentrating on just helping her heal.

Cataract, Scar Tissue...Another Surgery

November/December 2008:

At the beginning of November, Emily went in for an exam under anesthesia with Dr. Zaidman (her cornea specialist). Because of the ulcer and infection she had in her right eye, he thought it was important to find out exactly what was happening. Sadly we did not hear the kind of news we were hoping for. Due to the ulcer/infection she now has scarring on her right cornea and unfortunately it's right in the middle so we believe it is obstructing what vision she has. He prescribed a new eye drop to try and help reduce the scar tissue and we're hoping that over time it will dissipate. The Dr. also discovered that the pressure in both eyes was elevated again and increased the dosage of her Glaucoma medication. In addition to that, we also found out that she had a cataract in the left eye, which had decreased what little vision she had in that eye even further. Dr. Zaidman wanted to revisit the possibility of re-doing the corneal transplant in her left eye, but said he would like to get her eye pressure under control before doing it. To say the least we left the hospital with dashed hopes for her, but were trying to remain positive.

On December 17th she went under anesthesia again, this time in Manhattan with Dr. Kane, her Glaucoma doctor. He wanted to look further into why her eye pressure was not stabilizing at a lower level since the valves were implanted over the summer. During the exam the pressure was 14 in the Right and 24 in the Left, but that might have been a little lower than what it actually was due to the anesthesia. He confirmed that she did have a cataract in the left eye and said that he could not see through it to examine her retina. He found that the drainage tube for the left valve is possibly too close to her cornea and that could be the reason why it wasn't working as well as it should have been. Ultimately, she would need surgery to shorten the tube in the left eye and have it moved to a better location once the cataract has been removed. He said he could do both of those procedures at the same time, but it would require the assistance of another doctor. He also said that this should be done before Dr. Zaidman does the corneal transplant again in that eye, and he will call and speak to him about Emily's case to come up with a surgical timeline. At that point we were looking at possibly sometime towards the end of January, but that wasn't definite yet.

Last year was a very difficult and trying year for us and our family. In 2008 Emily had been through 6 surgeries and countless exams under anesthesia. She has come so far from where she was before, but at the same time still has a long road ahead of her. Everyday we thank god for giving her the strength to overcome the obstacles she has encountered. Anyone who has met Emily knows that she is probably the most happy and content baby you will ever meet. We hoped that 2009 will bring good news, good health and a more positive outlook for Emily's condition.

Scratched Cornea, Corneal Ulcer and Infection

October 2008:

We had encountered a new problem, and a possible set back in Emily's right eye. She started to develop what looked to me like pink eye in her right eye. I started giving her antibiotic drops, just in case, until I could talk to a doctor. Later that night we noticed a small grey spot on the same eye. The next morning the grey spot had become what looked to me like an indentation. It literally looked like a crater in the middle of her eye. Her cornea doctor was not in the office but we were told to take her as soon as possible to her Ophthalmologist. We got in to see him first thing the next morning. We discovered that she has a scratch on her cornea and sometimes that can cause the indentation that I was seeing. She was supposed to just continue with the antibiotic drops to help with the healing process, but we followed up with her cornea specialist yesterday and he changed her course of treatment. The scratch is still there and the "crater" (for lack of a better word) is actually considered a corneal ulcer. This is not good news.

There are two possibilities of how things can go for her. The first is that it can heal quickly and not have any lasting complications, which is obviously what we are hoping for. However, it could also heal and cause scarring, which will cause the cornea to become cloudy which leads to decreased vision and possible rejection of the cornea transplant. Her doctor said it's a 50/50 chance that it could go either way. If there is an infection in the ulcer, which he swabbed her eye to test for, would also be very bad. I wouldn't know the results until the next day. Unfortunately, it came back positive.

Until this was resolved, she was not getting any drops in that eye and was now on 2 types of ointment. The antibiotic/steroid ointment seemed to be bothering her because she was rubbing her eyes alot and had not willingly opened them yet. She woke up and about an hour later went back to sleep, probably because it's just easier to keep her eyes closed right now. She just seemed so miserable and it broke my heart. We don't know how she scratched her eye, and we will probably never know. But things like this happen and we just have to take it as it comes.

After 2 days of Emily being miserable, I called the doctor and he told me to take her off of the one ointment. Apparently she was having an allergic reaction to it. Great, just great. Why can't this poor little girl catch a break? Why of all things did she have to scratch her EYE???

Please God, I'm not sure I can handle much more....

Tuesday, June 23, 2009


What happens to a man when he spills his heart over a page
And he watches words float away then
His feelings lie on a page alone

There waiting for someone who cares to read them
To open their eyes to see them
To see if they can make his thoughts their own

To find out that maybe your life's not perfect
Maybe it's not worth what he gives away

You can see that this broken soul is bleeding
So you can see your feelings inside yourself
And wonder through my heart

Letting you see through me now only consumes me
Forget your pain and watch me fall apart

What happens to a soul when he's trapped inside his emotions
And all of these words he's spoken
They bind him to the life he's left behind

And every new step he takes
He knows that he might not make it
To all of these dreams that he has yet to find

Maybe your life's not perfect
Or maybe it's not worth what he gives away

You can see that this broken soul is bleeding
So you can see your feelings inside yourself
And wonder through my heart

Letting you see through me now only consumes me
Forget your pain and watch me fall apart
As I fall apart

You can see that this broken soul is bleeding
So you can see your feelings inside yourself
And wonder through my heart

Letting you see through me now only consumes me
Forget your pain and watch me fall apart
As I fall apart

~3Doors Down

It's The Only One You've Got

How do you know where you're going
when you don't know where you've been?
You hide the shame that you're not showing.
And you won't let anyone in.
A crowded street can be a quiet place
when you're walking alone.
And now you think that you're the only one
who doesn't have to try.
Then you won't have to fail.
If you're afraid to fly,
then I guess you never will.

You hide behind your walls of maybe nevers.
Forgetting that there's something more
than just knowing better.
Your mistakes do not define you now,
they tell you who you're not.
You've got to live this life you're given
like it's the only one you've got.

Memories have left you broken
and the scars have never healed.
The emptiness in you is growing,
there's so little left to fill.
You're scared to look back on the days before,
And too tired to move on.
Now you think that you're the only one
who doesn't have to try.
Then you won't have to fail.
If you're afraid to fly,
then I guess you never will.

You hide behind your walls of maybe nevers.
Forgetting that there's something more
than just knowing better.
Your mistakes do not define you now,
they tell you who you're not.
You've got to live this life you're given
like it's the only one you've got.

What would it take
to get you to say that I'll try?
And what would you say
if this was the last day of your life?

"It's the only one you've got"
~3 Doors Down

I found this song powerful and moving, it just struck a nerve with me for some reason.

Monday, June 22, 2009

Falling Apart

August 2008-

When Emily had her first cornea surgery back in January of 2008, the only thing that kept me going was the idea that by the time summer came and we went on vacation that all of her surgeries would be behind her. I envisioned enjoying the summer at the beach, doing fun things with friends and making up for some lost time with the boys. So in June when we were told that we'd have to take Emily to a Glaucoma specialist and she'd probably need more surgeries, I was devastated. We had already been through so much and just when we thought things were looking up, we get sucked right back down into this hell. It was like someone had punched me in the chest and knocked the wind right out of me.
After returning from vacation and being thrown face first into surgeries that we weren't prepared for, I began to unravel emotionally. My husband would go about his daily routine, which at some times I was jealous of. He had a life outside of our home, complete with friends and distractions from what was going on in our life. He had an escape from the daily ins and outs of Emily's condition and raising 3 children, I didn't. It was starting to wear on me that I was essentially in this alone. When he came home, he would do his best to help out with the kids, the house etc...but no one knew Emily's eye drop schedule better than I did, so I was usually the only one who gave them to her. Jason would sometimes ask me what she needed, but it was still on me to remember. At one point we used a chart to cross off each medicine as we gave it to her, but that started to get confusing and we eventually stopped using it. Jason has always been a great source of comfort and support for me and through the tough times he has always been there for me. But I was bearing the brunt of dealing with Emily's condition and treatment, it was just a fact of life.

It was extremely stressful for me to take on that kind of responsibility by myself. I alone was and still am responsible for making sure she gets the medication that prevents her from going blind. Her steroid drops prevent the corneas from rejecting, the antibiotic drops prevent infection and the glaucoma drops keep (or are supposed to keep) her eye pressure low so her optic nerves aren't damaged. The responsibility of this was mine. The doctors can only do as much as they can in the operating room or office, but it was ultimately up to me to make sure their hard efforts were not for nothing. It just seems like alot for me to be responsible for. I didn't sign up for this, this wasn't in the plan...

I've heard friends comment on how strong I must be. "You are the strongest woman I know, i wish I had half the strength and courage that you do", "If this was happening to me I wouldn't be able to handle it, I don't know how you do it", "You are such an amazing mom, how do you get through your day without falling apart?"....Well I hope I am nominated for an award someday because if everyone thinks I am such a strong woman and am handling this so well, I am fooling them all. Inside I am falling apart, crushed by the pressure of this weight I have on my shoulders. Sometimes the pressure is so much that I feel like I can't breathe, I am suffocating on the inside. My heart is shattered with each medication or surgery that fails, each time not being put back together quite the same. I am barely holding it together and there is going to come a time when I finally just break.

That breaking point came towards the end of the summer when I was struggling to get the boys to swimming lessons on time. I had driven up to the lake and was already late. My parents were staying there and my mom came out to 'help' me when I pulled up. I was so frazzled about who had towels, sunblock on, kickboard, floaties, cooler with essential snacks and drinks, beach chairs bottle for Emily etc...that by the time I thought I had everything together we were already late for the lesson and still needed to take the 5 min walk to the beach. I got Emily in the stroller, loaded up the beach cart and started down the road. About 15 seconds later I realized that I had forgotten to give Emily her eye drops so I had to go back to the car and get them. My mom has never been really good at offering help, or seeing when someone needs help so she just was oblivious to how mentally stressed I was at that moment. I opened the door to the van and something just came over me. I couldn't function, it was like I was frozen and the only thing that I could feel were the tears rolling down my face. I don't know if it was a panic or anxiety attack, but I have never felt something like that before. I broke down in front of my mother and it was like she was surprised that I wasn't keeping it all together. She asked me what was wrong as if I had stubbed my toe or something. I don't remember what I said, but I know I was rambling on and on about alot of stuff. I had hit the wall, I couldn't go any further.

At that moment, I came to the realization that I was in way over my head. I had most of the physical support that I needed (family/friends watching the boys while I took Emily to the doctor twice a week) but I had little or no emotional and mental support. I talked with friends about things sometimes but what I needed most was someone to look me straight in the eye and say...let it out..cry, throw things, take a deep breath and scream as loud as you can. I had no emotional escape. I was so overwhelmed by my responsibility to take care of my family and be 'supermom' that I had no idea how to take care of myself. I needed time alone, time to collect my thoughts and recharge my mommy batteries, time to allow myself to re-focus on what was really important in life instead of all the stupid little stuff. Five or ten minutes once in a while wasn't enough, I needed a real break. There wasn't anything I could do about it other than check myself into a hotel for a weekend and shut out the world. No TV, no phone, no internet. Just me, a few bottles of wine and some good music. But that wasn't going to happen.

After discussing it with Jason, I decided that I needed to go and talk to my doctor. I was not myself and I had been down the depression road once before after I had Tyler, so it was familiar territory. I guess I was just trying to tough it out and be as strong as I could before I resorted to any kind of medical help. I made an appointment with my general physician shortly after my break down. I tried to walk into the office with my head held high, knowing I did everything I possibly could to avoid the reason why I was there. I promised myself that I wouldn't cry, but the moment the doctor walked into the office and said to me 'Wow you don't look so good', i couldn't control it, I just let it all out. I explained what I'd been going through and she told me I should have come to her sooner, "don't be a hero" she said. I laughed, but I guess that's what I was trying to the mom who can do everything and handle anything. I guess there is no such person. There is no June Cleaver...even the Brady's had a maid. What made me think that I was any different?

I left the office feeling relieved but apprehensive at the same time. I was now going to be on anti-depressants, a short term fix and a long term solution. Two medications that were hopefully going to help me get my head screwed back on straight, bring back the happy go lucky person I used to be and enable me to help my family deal with our new 'normal' life. I didn't want to be in this position, but I was and there was no escaping it. I still needed the extra emotional support from family and friends, but that was a whole other issue. Something that to this day has really not been figured out. But we're getting there...

Hospital Bills

Ok, I get go to the hospital, you get treatment, they send you a bill. What I don't understand is why in this age of such great technological advances in computer systems and programs do major hospitals have to send you a separate bill for each time you go there? Who was it that coined the term "super bill"?...someone who probably figured out that billing for all hospital visits by the same person on one bill would make things a hell of a lot easier. The patient can clearly see what charges were for what date of service, how much they owe on that charge and what their current balance is. The hospital can clearly see the same information. Benefits to both patient and hospital include less paper, less chance for bills to get lost in a pile of mail, less cost for postage and less confusion on which payment was sent on which date and which date of service it was for.

This is 2009, why I am getting 5 bills from the same hospital each with a different account number, date of service, amount due and balance owed. And not only do I get these statements in the mail, but about 5 days after the bill is generated, a payment reminder is also generated. So I am getting something from this hospital just about every day. This is my answer to this issue...I collect all the bills from that hospital and wait until all of them have been received. I write a check for each one, noting the account number on each check. I put them all in one envelope and mail them so the hospital gets all 5 on the same day at the same time. GENIUS! I just saved about 3 trees doing that. The downfall is that by the time I pay everything, I am already getting bills that are due for the next month. I've asked over and over again for them to send me one statement but they can't. Why the hell not? Get up to date people. You are a MAJOR hospital in can't afford a better billing system??? Take some of the money I am paying you and spring for it, your patients will thank you. Plus you might actually get paid on time.

Stop sending me a payment reminder before I even get the bill in the mail! Are you trying to get me to slip up and send you two payments that month??? Do you take me for a sucker? Sorry, not me...I'm on to you!

Sunday, June 21, 2009

2nd Glaucoma Surgery

August 12, 2008

After waiting 4 1/2 hours in the pre-op waiting room...Emily finally went into surgery at about 3:30. We anticipated that it was going to be another long surgery since both the Left and Right eyes were being worked on, and we were right. She came out at about 7:15. The valve was implanted in the Right eye and the drainage tube was opened up in the Left. The surgery went well and we hoped to have a short stay in the recovery room. After the first surgery 2 weeks ago, she was very hungry and drank 2 bottles of glucose water and 2 bottles of formula with no problem. This time we think that her throat was much more irritated from the breathing tube because she was refusing to drink and cried every time she tried to swallow. She should have been starving since she hadn't had anything to eat or drink for over 21 hours, but without drinking very much, she would have to be admitted and stay overnight. Eventually we got her to drink some and finally left the hospital at about 9:15. Dr. Kane said the pressure in the left eye was now low, almost too low, so he was hoping it would recover to a safe level by the next day. We brought her in first thing the next morning and the level was ok. Sometimes children with this condition cannot produce fluid fast enough to build the pressure back up, so if it had been too low, he would have had to go back in and inject some synthetic fluid to help. Thankfully that was not the case. We were no longer giving her the oral medication, but would continue to use the eye drops in just the right eye until the drainage tube can be opened.

Post Op Check Up 8/15/08:

The pressure in her left eye was at 20 or just below 20 and the pressure in the right eye was at 24. The day of the surgery, both eyes were at 43, which is very high. The left eye was about where her doctor expected it to be since the tube was opened, but he was surprised at how much lower the right eye was, even without the tube completely opened. He actually said that if the pressure stays at this level or is even slightly lower, he might hold off on opening the tube. We would continue using the eye drops as we had been, and maybe that would be enough for her. In the event that she would need the tube opened, she would have that done on September 9th.

So for that moment, we were relieved and happy that things were going well for her. This is going be a lifetime battle, but hopefully since we are taking steps to correct it now - her vision won't be affected. We are taking one day at a time and we can't do much more than that.

On a side note...while Emily was in surgery a storm was passing by the hospital. No rain fell, but there was a beautiful rainbow just outside the waiting room window. Both of our families had lost loved ones last year and I have to believe that this was their way of letting us know they were watching over Emily and keeping her safe. It made me cry...

Thursday, June 18, 2009

I'll Stand Back Up

Go ahead and take your best shot,
Let 'er rip, give it all you've got,
I'm laid out on the floor,
but I've been here before,
I may stumble, yeah I might fall,
I'm Only human but aren't we all?
I might lose my way,
but hear me when I say,

I will stand back up,
You'll know just the moment when I've had enough,
Sometimes I'm afraid, and I don't feel that tough,
But I'll stand back up,

I've been beaten up and bruised,
I've been kicked right off my shoes,
Been down on my knees more times than you'd believe,
When the darkness tries to get me,
There's a light that just won't let me,
It might take my pride, and tears may fill my eyes,

But I'll stand back up,
I've weathered all these storms,
But I just turn them into wind, so I can fly,
What don't kill you makes you stronger,
When I take my last breath,
That's when I'll just give up,

So, go ahead and take your best shot,
Let 'er rip, give it all you've got,
You might win this round but you can't keep me down,

'Cause I'll stand back up,
And you'll know just the moment when I've had enough,
Sometimes I'm afraid and I don't feel that tough,
But I'll stand back up,

You'll know just the moment when I've had enough,
Sometimes I'm afraid and I don't feel that tough,
But I'll stand back up.

- Sugarland

Wednesday, June 17, 2009

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 sedation, 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:

Information about Pediatric Glaucoma:

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

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.

In Office Sedation

I brought Emily to see Dr. Zaidman to be sedated in the so he could test her eye pressure. It took her about 45 minutes to finally fall asleep, but once she was out...she was out. I did about 20 laps around the hospital with her in the stroller trying to get her to sleep, the doctors and people sitting in the waiting rooms must have thought I was either very lost or completely crazy. After she was asleep he took her pressure readings and unfortunately the pressure is elevated again. It also appears as though there is a little bit of swelling in the right cornea now too. I asked him if he thought her left eye was rejecting and surprisingly he said no. Increased pressure can often cause swelling and the cloudiness that we see, so if we can get the pressure under control her eyes should clear up. So it's like a double edged sword, good that he doesn't think it's rejecting, but not good since she's got the high ocular pressure. We are currently trying to find a Glaucoma specialist who works with young children. Both Dr. Zaidman and Dr. Morgan are looking for names of people in Manhattan, but we would much rather find someone more local if we can. My mom got the name of a doctor in Hackensack from one of her eye specialists. We would meet with him as a consultation and then he would do an exam under anesthesia at the hospital to test her pressure. Hopefully we can avoid that for a little while since her pressure was just checked yesterday. They don't do in office sedation so she would have to go under anesthesia again and I just can't bear to put her through that again right now. (and I'm still recovering from the first 7 times). I wanted to find out if her test results could be faxed to the other doctor and hopefully that would be enough for right now. But for the moment Dr. Zaidman had decreased the steroid drops in both eyes to 5 times a day and prescribed another eye drop for the pressure and an oral medication called Prednisone to reduce the swelling in her eyes. She will be going back to him next Tuesday for him to check on the swelling.

Warning: Predinone causes weight gain...

I had to stay at the hospital until she woke up from the sedation, so while I was on my way to the coffee shop, I passed by the Chapel. I wheeled the stroller inside and sat down to say a prayer for her. I was just so overcome with emotion about how little she is and how her life has just started, but yet has been through so much already. As she lay there in the stroller sleeping so peacefully, I just held her hand and cried. I no longer find myself asking "why" this happened to her, but instead asking God to heal her and not take away the sight that he just gave her. She's finally starting to see the world and unless we get the pressure in her eyes lowered, damage can be done and her vision will deteriorate. We just have to take a deep breath and do the best that we can to get her what she needs. We're trying to remain optimistic and be strong for her. But that's so hard right now.


June 2007:

Why was Emily's left eye bulging out and starting to get cloudy? My gut was telling me the cornea was starting to show signs of rejection. I couldn't help it, nothing else made sense.

I took her to be seen by her Ophthalmologist, Dr. Morgan. He patched each eye to test her vision and found while she is focusing quite well with her right eye, she is struggling a little with her left. That kind of seems expected because the surgery in her left eye was done 2 months after her right, so it should still be trying to play catch up. But since she is having problems with swelling and some cloudiness in her left cornea, we aren't sure whether the vision issue is related to that or not. He said that it might be a possibility that the left eye was starting to reject but he could not say for sure and left that up to Dr. Zaidman to determine. Originally he wasn't going prescribe glasses for her and wanted to see her back in 3 months, but he changed his plan when I told him that Dr. Zaidman wanted her to be seen early in June to get glasses. He hesitated, but then dilated her eyes and gave me her prescription. He said he was going to get in touch with Dr. Zaidman and find out what he thinks is happening with her left eye. As you can imagine, I left the office feeling very overwhelmed and sad. I got in the car and cried.

Why is this happening? Why can't this poor little girl just be done with all this drama and get on with the business of being a baby? Like learning how to crawl, walk, talk and drink from a sippy cup. Why are we sitting here worrying about donor tissue rejection, glaucoma and glasses for an 8 month old? This is not fair, non of this is fair. She should not have to go through this. Why is this happening? Did we not say enough prayers? Is god not listening? Why her, why this tiny child? No one has an answer for me.

I wanted to wait until her two doctors talked to each other before getting her glasses. It took 2 weeks for me to finally hear back from Dr. Morgan and they both agreed that she should start wearing them. So that afternoon we went and picked out a cute pair of pink glasses for her. Her frames are on backorder for 2-3 weeks so the store loaned us the ones they had as a sample until hers come in. We picked them up put them on her for the first time, she looks so cute! It's going to be a huge adjustment for her and us, but she can see much better now (i guess) and that's what is most important. She pulls them off just about every 2 seconds, but once she starts to realize that she can see better with them on, she'll learn to leave them alone. I just can't imagine how differently her world looks now. I've ordered sun clips for her so her eyes will be protected from the sun - just in time for the summer.

Follow Ups And Other Happenings

During the exam when the broken stitches were fixed, the doctor checked the pressure in her eyes again and found that there wasn't a drastic change with the new medications, but also noted that there is no evidence of any damage to her eye (thankfully). He explained that children are much more able to handle the increased occular pressure than adults or the elderly because their blood vessles and are more plyable. Which basically means that an elderly person with the same pressure readings would have experienced damage to their eye and thier vision would be comprimised because of it. Thankfully this was not the case for Emily right now. He decided to up the frequency on the one eye drop to see if that would make a difference.

Emily's Christening was on April 13th. It was a very special day for her and she was such a good girl in church. It was very nice of our Pastor to mention her in the prayer and concern part of the service. He explained briefly about her condition and a special prayer was said for her continued healing and improvement in her vision. God was smiling down on her that day, we just know it.

On April 14th she had another exam under anesthesia to remove her stitches. Her doctor could not remove all of them because the ones that had just been replaced were not ready to come out yet. The pressure was tested again and found to be lower, but not quite low enough. He decided to add an oral medication called Diamox. If that didn't work he said there was one or two other things that we could try, but we were hopeful that we wouldn't need to. Since the medication is a pill for adults with Glaucoma, it is not commercially available in a liquid form for children. So we had to have it specially made at a compound pharmacy. Emily didn't seem to mind taking it since it's flavored like tutti fruiti. She smiles everytime we gave it to her!

On April 17th she had an appointment with Dr. Morgan, her Ophthalmologist. He said that he was pleased with how the cornea's looked and confirmed that she is also far-sighted in the left eye as well. Her retina appears to be normal (which is great news) and overall her eyes look good. Although she is gaining a lot more control of her eye movement, often one or both eyes will turn in too far. I wouldn't say that she is cross eyed, but there are times when it is obvious that her eyes are not looking in the same direction. This can sometimes be corrected with glasses, which she will probably need. At some point in the future (a year from now or so) we will discuss the possibility of having her eye muscles surgically corrected, but that is not a huge concern right now.

On May 1st she had the remaining stitches removed. Her doctor said her eye looked like it was healing nicely. Because of the extra surgery and stitching during the initial transplant and the problem with the broken stitches, her left eye may take longer to heal than the right one did. The cornea itself looked good, but it was not quite as clear as the right one. The Dr. said that with time and additional healing it should eventually look the same as the right eye. The good news was that her pressure readings were much lower and within an acceptable range! We finally found a combination of medications that worked! That was one of my biggest concerns, so when he told us to continue with everything we were using, I was thrilled. When we left the hospital that day, it was like a huge weight was lifted off of our shoulders.

Finally...her surgeries were over, her stitches were out and her exams in the hospital were done. Now we could move on to the next phase of her treatment and begin to show her the beautiful world around her. She has been through so much in the first 6 months of her life, but she has been so strong and we are so proud of her!

Linda, a visual therapist, had been coming to our house twice a month to work with her and track her visual growth. At our last session Linda told me that she is doing very well and is showing improvement at each session. She is tracking a wide variety of colored objects up to about a distance of 5-6 feet. Although not all objects hold her attention to that distance, there have been a few so we know she can see at least that far. She is showing visual preference, which means that when given the choice of looking at two objects, she sees both of them but prefers to look at one over the other. She is also showing visual memory, meaning that when two objects are placed in her visual field and then one is taken away or hidden, she will look at one and remember about the other one and look for it. Both of these things are a great sign that her vision is improving and she is doing well.

We also had Beth from the Commission for the Blind come out and see Emily. They run their own early intervention program and can offer assistance, support and guidance to families of visually impaired children. She did her own evaluation of her vision and was really very pleased with her progress. I asked her if she had any experience with Emily's condition and she said that she only know's of one other case in the area, a child in Bergen county, but she personally has not worked with that child and has little information about the condition and how it will affect Emily as she grows up. Because it's so rare and there aren't many babies who have had the cornea transplant surgery, her future is really unknown. While she is doing well right now, the possibility of the donor cornea being rejected is always there. In reality she could be 4 years old and show signs of rejection, but we have to stay positive and believe that we won't have to ever deal with that. Beth told me that by far Emily is doing great and there are many parents of blind and visually impaired children who would jump at the chance to be in our shoes. So we really have to count our blessings and be thankful for the vision that she has. If she needs glasses to see, thats totally fine. At least she will be able to see. And we can't ask for more than that. Beth mentioned that she will be trying to get a few toys for her, ones that we can use to help her tracking ability and distance. And when we are ready to order glasses for her, she will be able to help us with that as well. She won't be able to provide help with every pair of glasses she will need, but any help we can get is wonderful. If anyone has information on any programs that would provide glasses for visually impaired children, please let us know. We know of the Lions Club, but have yet to really get information on how their program works or how to contact them.

On May 30th Emily saw her cornea specialist for the first time since all the stitches were taken out. The good news is that she does not need to be on the antibiotic eye drops anymore, so we can stop using that. But another medication is being added to the list instead. We've noticed that her left eye seems to appear swollen, but it's hard to tell from day to day whether it's the same or worse than 3 weeks ago. Originally the doctor said that it could be from the extra surgery on that eye, or just part of her condition. But because the left cornea still doesn't look as clear as the right one, he suggested using an over the counter saline drop to try and clear it up. He said that he's not sure why it is like that, but it's possible that when the stitches broke it caused something to happen and it's just not resolving itself. The new medication is called Muro 128 and is used for treating corneal edema...which would explain the swelling we see. It works by drawing out the fluid in the cornea. She will get that 3 times a day and hopefully it will clear everything up. She's only been on it for 2 days now and we can already see an improvement. Her eye doesn't look as swollen as it did before, so hopefully it's working. She won't need to go back to see her cornea doctor until the end of we are about 1 month in between visits now...a far cry from when only a month ago we were going twice a week. When she goes next time, she will be sedated in the office so that he can test her pressure again. She will have to take a medication that will make her fall into a deep sleep. So it's like she'll be under anesthesia, but not really. (kind of scary for me) I'll be there with her the whole time and just have to stay for a little while afterwards until she wakes up. The doctor joked around saying that it will be like she's having the best sleep you could possibly imagine and that many parents ask if he can prescribe some of it for them! Hopefully her eye pressure will still be ok and we can just continue with what we are doing now.

Here is her eye drop schedule as it stands now:

Pred Forte (steroid) 5 x day Right eye - 6 x day Left eye
Cosopt (pressure) - 2 x day both eyes
Xalatan (pressure) - 1 x day both eyes
Diamox liquid (pressure) - 1 x day
Muro 128 (corneal swelling) - 3 x day Left eye
Dilation drop - 2 x week Right eye only

On June 5th she was going to go back to Dr. Morgan for another eye exam. I was anticipating that he would prescribe glasses for her, so pretty soon she would be able to see even better than she could now. I couldn't imagine how much her world was going to change. She was now 7 months old and her personality had really started to bloom. She was getting extra tummy time to strengthen her core muscles since she isn't sitting on her own yet, but hopefully she will soon. She had spent so much time in the car seat going back and forth to the doctors that she was really robbed of tummy time as a newborn, so she's got some catching up to do. She loves to blow raspberries, especially while she's eating her fruits and veggies! And we think she said "da da" the other day but we haven't been able to get her to say it again. She sometimes just says one "da" so I guess that's a start.

Broken Stitches

1 week after having the Left cornea transplant, we had unplanned visit to the hospital operating room. I brought Emily to her doctor for her usual check up on her eyes. When the doctor looked at her left eye, he thought he saw a scratch on her cornea or possibly a broken stitch. He couldn't be 100% sure what it was or if there was any damage unless he put her under anesthesia to find out. So, what was supposed to be a quick appointment ended up being an all day event at the hospital. Back into the OR...

There were four broken stitches....not one, but four. The doctor doesn't know how or why they broke, but it had to have happened between Monday and Friday of that week because when she was checked on Monday it was fine. He replaced the stitches and she is doing well. We will have to go back the next day to his office so he can look at it again. He said that the bottom part of the cornea is healing nicely and hopefully with new stitches the top part will too. Thankfully it was something that was able to be fixed and not something wrong with her eye or with the new cornea, but it was pretty scary for us.

Please God, no more surprises...

Cornea Transplant #2

Second cornea transplant. Everything was just about the same as the first time around...except this time Jason went into the OR with her. She went in at about 11:00 and came out around 1:30. Dr. Zaidman came back into the recovery area to talk to us and he said the surgery went well. He first had to do an exam on the right eye to see how that eye was doing, and then did the surgery in the left eye. Because of some additional scar tissue, the surgery was slightly more complicated than the previous one, but she got through it was doing well. He had to repair the area where some scar tissue was in addition to the cornea, so she has a few extra stitches this time, which is causing some increased swelling and irritation. He explained to me that the extra stitches are actually on the surface of her eye, making it feel bumpy to her. So each time she blinks it's causing some irritation. That area should heal quickly and will look just fine. The pressure in her eyes was still elevated, so she is now going to be using two new pressure drops instead of the one she was previously using. We're not sure why the first one was not working, but hopefully these new drops will do the trick. Some of the increased pressure is due to the use of the steroid drops, but she will need to be on those for a year so these other drops will be necessary to counteract it.

After some confusion about the hospitals age policy for extended observation in the post-op recovery room, we established that she didn't need to stay for the full 6 hours this time. We left after only 4, which still felt like it was 12, but we were just thankful to be able to leave while it was still daylight out.

We had to be back at 8:15 on Friday for him to take the patch off. We had the same anxiety that we did the first time, wondering what her eye looked like. Just after we took the patch off, she opened her eye for the first time and had an irreplaceable expression on her face. It was like she said to her self "wow, that seems different, I can see better now". It was amazing to see her react like that. Although it was swollen, he was able to get a peek at her eye and said it looked good. Then he went down the list of eye drops that she is on and explained what to do with the Left eye.

Her eye drop schedule:

Vigamox (Antibiotic) - 4 times a day in each eye
Pred Forte (Steroid use to prevent rejection of the donor cornea) - Reduce to 6 times a day in the right eye and gradually build to 8 times a day in the Left. By this Friday she will be at 8 per day.
Cosopt (To reduce eye pressure) - 1 time in each eye before bed
Xalatan (To reduce eye pressure) - 1 time before bed in Right eye only. This will change to both eyes in about 3-4 weeks.
Cyclopentol (Dilation drops) - Twice a week in Right eye only.

That's 24 or 25 eye drops per day. It is very stressful to say the least. But we will do what ever it takes so that in the end she will have the best possible vision.

She had a pretty rough weekend, crying the entire time she was awake. Jason and I got only a few hours of sleep that Friday and Saturday night. We believe it was a combination of a few things that made her so upset, but she got back to her regular happy self pretty quickly. Since the surgery she has been opening her left eye much more and we had already noticed some improvement in her focusing on some objects. She was scheduled to have the stitches taken out on April 14th and then she would go see Dr. Morgan, her pediatric ophthalmologist on the 17th. One thing we still needed to look into was finding a pediatric glaucoma specialist that is in our area (and insurance). Because her eye pressure may become a long term issue, Dr. Zaidman said it might be something we'll have to look into.

Emily also had her first meeting with a visual therapist from the early intervention program. We were very pleased with her ability to track shiny items, and of course anything that had a light in it. She used a fiber optic color changing light and was able to get Emily to track it on all directions, even from a distance of 5 feet, for over 20 minutes! She will be coming every 2 weeks to work with her and give us some additional ideas on how to work with her on our own. I will also soon be meeting with the therapist from the commission for the blind. They have their own early intervention program for visually disabled children, so we will get some extra help from them as well. From what I understand, they'll be able to provide us with some toys and objects that we'll be able to use here at home. And if and when she needs glasses, they should also be able to help us with that too.

Emily had just turned 5 months old. Time has gone by too fast. She had already started on solid foods, first eating oatmeal cereal and then moved onto rice. She seems to be taking to them well and soon we'd be adding fruits and veggies! She loved lying on the floor and would frequently squeal with excitement when she was extra happy. She loved to sit and watch baby einstein video's...which are great for her since they are chock full of visual stimulation. She started doing this super cute nose scrunch thing recently, like she's reacting to something that doesn't smell good, it's the cutest thing! So far we hadn't noticed that her vision problems have caused any kind of developmental delay in other areas, which is a good sign...

Stitches Out

We brought Emily back to Westchester to have the stitches taken out and ended up leaving there with some discouraging news. Her eye pressure was elevated, which could mean she was developing Glaucoma. The cornea itself looked good, but her doctor wasn't sure if the pressure was elevated as a side effect of the steriod drops she was on, or if it was Glaucoma. So he gave us a prescription for Lumigan to try for a few weeks to see if it helped.

Bottom line...cornea ok, but possibly at the expense of her vision. Glaucoma steals your vision slowly. So even if she can see through a nice clear cornea, the increased pressure causes damage to the optic nerve and you go blind. Not what I wanted to hear.

5 Days Post Surgery

5 days since her surgery and all things considered, she was doing great. Her doctor said the cornea looked "excellent"! She was going to have the stitches taken out on March 5th. She was getting antibiotic eye drops 4 times a day and would soon be getting the steriod drops 8 times a day. She seems to be dealing with all of this really well and we are so proud of her.

Giving her the eye drops was one of the most stressful parts of her post op care. Trying to remember what she was given and when was difficult, but we quickly figured out how to give them to her without causing her to get upset. She is such a strong little girl.

Cornea Transplant #1

Emily had her first eye surgery and she did wonderful! We got to the hospital at 9:00. She was scheduled to go in at 10, but they didn't bring us back into the surgical center until 10:30. After giving her a few antibiotic eye drops, and putting a very fashionable blue hospital gown on, we were able to walk her down to the operating room. I was allowed to go into the room with her and stay while they started the anesthesia. I stood/sat just outside the operating room holding Emily for almost an hour before the doctor was ready to bring her in. Why the hell did you bring us back there then? Jason was outside probably starting to freak out and wonder what was going on, why was it taking me so long to come back to the waiting room. The entire time I was there, I was in tears. It would come and go. I started talking to her to ease my nerves, telling her how much we loved her and that when she woke up we would be there to comfort her. the nurses were great, one kept coming over and giving me tissues. She even sat and talked with me about her condition for a little while, I think she really wanted to know about her and that was comforting. But I was scared to death. They finally were ready and I brought her into the OR. Machines, monitors, lamps and probably 6-8 people filled the room. I knew she was in good hands, but a 3 month old baby shouldn't be in there to begin with. This is not how she was supposed to start her life.... but that's where we were and there was no other option for her. I laid her down and the nurse put the mask over her to allow her to breathe in the anesthesia. She was asleep in seconds. I tearfully kissed her goodbye and whispered I love you in her ear...then sobbed as I walked out of the OR doors, leaving her alone in such a scary place. I didn't know what to do with myself... I didn't want to leave her side. Jason and I hugged as soon as I walked out and took off my bunny suit (big white jumpsuit I had to wear) and we both cried holding each other in the middle of the hospital hallway. I didn't care who was around me, my emotions were so high and I felt helpless. The surgery started at about 11:30 and took just over 2 hours. The only thing that got us through those 2 hours was food and mindless stuff on the waiting room TV.

After it was over, her doctor said the surgery went very well and she did great. His exact words were "bring her back in the morning and we'll take the eye patch off, and then she can start to see". Those words hit us really hard...she was finally going to be able to start learning how to see, and her entire world was about to change. Wow, what an emotional moment that was for us. After 3 months of waiting, worrying and wondering, our little girl was about to begin seeing the world. There really are no words that can describe how wonderful that made me feel. A few minutes later we were allowed to go back into the recovery room to be with her. She had a patch over her eye, but it didn't seem to bother her at all. Because of her age, she needed to stay in the recovery area for 6 hours for observation, so we were there for what seemed like forever. She was such a good baby, only crying when she needed to be changed or was hungry. Even the nurses couldn't believe how quiet she was and said they'd never had a baby seem so content after surgery before. We finally left the hospital just after 7pm and got home at about 8:30. We went to bed almost right away since we had to get up first thing in the morning to go back to the hospital. We couldn't wait to see what her new eye was going to look like, and were so excited for her to really be able to "see" for the first time.

We returned to the hospital at about 8:00 the next morning and after a short wait in the waiting room, we were finally allowed to take the patch off. Yes, mommy cried...again. I was so emotional, just waiting to see what hew new eye looked like. How would her brain react to having the patch taken off? What would she be able to see? Would she know she was looking at Mommy and Daddy? It was amazing, and honestly trying to put into words how I felt at that moment is nearly impossible. Her doctor was very happy with how little swelling she had and was impressed that she was opening her eye so much since typically babies don't open their eyes very much on the first day. He gave us a few prescriptions for some antibiotic and steroid eye drops and ointment. She will have to get 1 drop of each one in her eye 4 times a day. As the healing process continues, the frequency of drops will increase. She will need to be seen twice a week for the first month so that the condition of the eye can be monitored and in about 5-6 weeks she will have the stitches taken out.

The swelling had continued to go down as the day went on and she had been keeping her eyes open alot more. She was doing great and we were excited for her!

One thing we noticed alot more since the surgery was that her eyes sometimes aren't moving together or looking in the same direction. (lazy eye) That would hopefully get better as her eyes learn how to focus on objects and her eye muscles get stronger. During the week following her surgery she was scheduled for an evaluation from the early intervention team. They would evaluate her and if she qualified, they will set up a program for her that is tailored to her visual and developmental needs. The road we have in front of us appears to be a long one, but we're just putting one foot in front of the other and trying not to look to far ahead. Hopefully we won't hit many bumps and at the end our little Emily will have 2 healthy eyes and the best eyesight possible.

The Evil That Is Pink Eye...

Seriously? Pink Eye? Not once, but twicewithin the 3 weeks before the transplant. Emily's surgery had to be postponed because obviously if there is any sign of infection, she can't go through with it.

We have no idea where it came from, but now I was super sensitive to everyone that was by her. Were they sick, did they wash their hands. What did she touch? Where is the Lysol?

Evil you are pink eye! Pure evil.

Emily's First Christmas

Emily's first Christmas was nice...everyone seemed to be just going about their normal life. I tried to do the same. But it broke my heart when I realized that she might not ever be able to see the beautiful twinkling lights on our Christmas tree, or know what Santa or a Reindeer looks like. She won't even know what a fat man in a red suit looks like because she has no idea what the color red is. Pretty wrapping paper wouldn't matter, except to those who are watching her open a gift or want to take a picture for their own memory. The prospect of Emily being blind hit me really hard over the holidays. She wouldn't know how beautiful snow looks as it's falling either...

I tried to go about my holidays the only way I knew how, baking cookies, shopping, enjoying the sounds of the Christmas music as I wrapped presents. But I knew that a few short weeks later my life would be anything but familiar.

Emily: 2 months old

Some good news....

I finally got sick of waiting for the other shoe to drop, so I called and spoke with the genetic counselor at Hackensack to find out the results of all those tests Emily had....

All the genetic and metabolic testing that was done has come back normal! So we can safely say that her eye condition is an isolated problem and isn't the cause of some other underlying issue.

That is a huge sigh of relief for us.

Finally Some Answers

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".

Depression Sets In

I will be the first to tell you that I had Post Partum Depression in the months after I had Tyler, my second son. I didn't see it at first, but after months of crying for little or no reason and feeling like nothing was ever going to go my way, I broke down and talked to my doctor about it. I was on medication for a few months and as I started feeling better I took myself off of it. I probably wasn't supposed to do that, but I was much better and not taking the meds anymore gave me hope that the PPD was over.

This was an entirely different type of depression, this was not PPD. This time, my tears were coming from such a deep seeded place of sadness. I would have some good moments when I would just look at her while she's sleeping and think 'wow, how lucky am I to have such a beautiful little girl'. But in all honesty, the moment she would wake up and open her eyes, my heart would sink. It was like reality came back and kicked me straight in the gut. Sometimes when she would cry, I would stand and rock her to calm her down. Having her head lying right over my heart so she could hear the sounds she was used to before she was born. I specifically remember one day about 2 weeks after she was born where I just stood in the living room rocking her and crying my eyes out. All I wanted to do was protect her from all of what she was about to go through. Why couldn't I protect her? I am her mother, mothers are supposed to protect their children. How come I can't do that for her? What kind of mother am I?

I cried alot....I Cried A LOT. I knew my where my depression was coming from and honestly didn't want to waste anytime trying to fix me, I just wanted to fix her. She needed to see the doctors more than I did, so I put myself on the back burner. Trying to convince myself that I was strong enough to get through this. But where do I get the strength from? Is there a magical "be strong" fairy out there who would come in the middle of the night and sprinkle some glitter on me? Is it like coffee? Have a cup of strength in the morning and it will get your day started off right? How do I get through my day without completely falling apart? Most of the time I didn't. I would spend many days just crying, holding Emily as tight as I could, not wanting to put her down for fear something else would happen. If I put her down, I couldn't protect her anymore. So I held her, almost all day, as much as I could. Nothing was going to happen to her as long as she was in my arms.

But that is not reality, all of that was in my head and I knew it wasn't true. How could I protect her from what she was about to go through, if this problem developed while she was still inside my womb? How could I allow this to happen to her? Why didn't I have that mothers intuition feeling in my gut that there was something wrong for all of those 9 months? How did I not know this was happening to her? I felt helpless.

Wednesday, June 10, 2009

There Are No Words

After we brought Emily home and tried to adjust to our new reality of life, I became very distant from family and friends. It wasn't that I didn't want or need support from them, I just couldn't bring myself to talk about what was going on. I forced myself to take a phone call from my best friend while lying in bed with Emily sleeping next to me. I hadn't really talked to her much about the specifics of things, and I just completely broke down and sobbed to her on the phone. I was so scared for her, so sad for her and didn't know how to deal with what was going on. I had family, friends, doctors giving me names and numbers of doctors and specialists. Particularly ones in Philadelphia. After we made contacts in NYC, for some reason I completely avoided making the call to a doctor in Philly, mainly because I didn't want to rehash the entire story. It was too raw, too emotional for me to talk about. So I never picked up the phone. I didn't want to drive 3 hours to PA on a weekly basis if we could go to NY. I just couldn't handle it.

Three days after we brought Emmy home, we went into the city to see the cornea specialist at Columbia. The pediatric doctor who saw her in the hospital was also there. Initially a few disorders were ruled out, including cataracts. But as we sat in the exam chair and the two doctors flipped through medical books trying to figure out what the hell was wrong with her I could feel the anxiety building up inside me. Ultimately, we got no definitive answer as to what the problem is or how we will need to correct it. Further testing would need to be done to give a correct diagnosis. We were told that they were not sure if she could see, or how well she will be able to see in the future, but one thing was pretty clear, she would most likely need to have cornea transplant surgery. I was numb, I couldn't put two words together to form any coherent thought, I just wanted to get out of that building and as far away from that office as I possibly could. Before we left, we were referred to a pediatric cornea specialist at Westchester Medical Center who would hopefully correctly diagnose her condition and perform the surgery which would need to be done within the next few months. Jason and I left the office without looking at or speaking to each other. We got into the elevator and I just began sobbing, holding Emily as tight as I could. There were no words to say at that moment.

I did the best I could to pull myself together when the elevator door opened and I was thrust back into the busy lobby of the Eye Institute. We walked out the door and back to the van in the parking garage without saying much. I got to the van, put Emily back in her car seat and kissed her cheek. Jason put the stroller in the back of the van and got in the drivers seat. The moment I closed my door, I completely fell apart. That was probably the lowest moment of my life. I don't think I have ever cried that hard before in my life. What originally started out as tears of sadness quickly turned to anger and panic. We needed to leave, I could not stare at the parking garage wall anymore. I screamed for Jason to get us out of here as fast as he could. It was like I couldn't breathe....just get us out of here - and NOW.

We called our families to meet at Jason's parents house so we could explain what we had been told. When we got there, we sat down at the table and just put it all out there on the table. No one wanted to believe what we were saying, but we all knew it was reality.

When Emily was 12 days old we took her to see the Geneticist who ordered some blood work and urine tests, to which we have not yet received any results. It's very unlikely that there is any underlying genetic or metabolic reason for what has happened, so these tests are basically to just rule everything out.

Comic relief....While we were there, we were left alone with Emily for a little while to try and collect a urine sample. Instead Emily pooped like she had never pooped before. There was yellow baby poop everywhere, all over her, her clothes, the diaper changing pad and the floor of the office where we were changing her. We ALMOST ran out of wipes, which would have been disastrous. And the kicker is that she peed really good into the collection bag, but all the poop contaminated it. Damn poop! Now we had to start all over. Do you know how hard it is to stick a urine collection bag over a newborn baby girls' girlie parts and actually get something to stay inside? Babies that little don't sit up, they lay down. When they lay down, all the pee just goes down into the diaper, it doesn't go sideways into a bag...that would be defying gravity. Who the hell came up with that form of urine collection for a 1 week old? Boys...ok probably not a problem...but girls? Not going to work. We were sent home with a whole bunch of collection bags, we needed to collect more at home and bring it to the lab when we got enough for the testing. Well that didn't go over well either. We ran out of bags because they kept leaking pee all over the place, so I had to get more bags from LabCorp. What a freakin pain in the ass that was. We had to freeze her pee and add tiny little bits at a time into the container because we could never get a full bag. Once all the bags were gone I brought her pee-cicle to the lab and they sent it to The Mayo Clinic to be analyzed. Now the wait begins to get the results, with fingers crossed for only good news.

When we finally met with the cornea specialist at Westchester, he examined her and pretty much came to the same conclusion as the previous doctors, that further testing would need to be done to be 100% sure of what is wrong. He narrowed it down to one of a couple possibilities. Glaucoma, C.H.E.D. or Peters Anomaly. He doesn't think it's Glaucoma but he can't rule it out just yet because getting an accurate eye pressure reading on an infant is difficult. We knew from the first doctor we saw that Peters Anomaly seems to be unlikely because there are usually other indications of that disorder which don't seem to be present in Emily, ie...heart or lung problems. So Dr. Zaidman seems to think it is CHED, which stands for Congenital Hereditary Endothelial Dystrophy. The course of treatment for this would be the cornea transplant surgery. We had to pretty much come to terms with the fact that she will need to have the transplant surgery, we want her to be able to see and if this is what needs to be done, then we will get through it. We will take one step at a time and move forward with treatment as we go along. Here is what we knew would happen. In order for the doctor to fully examine her and get a true diagnosis, he would need to put her under anesthesia in the operating room. This is when he would be able to do a retina scan, take accurate readings of the pressure in her eyes to rule out Glaucoma, and run other tests. That was to be done when she was about 5-6 weeks old so that she can tolerate the anesthesia better. Once this exam is done we would know for sure what is wrong and could then further discuss a treatment plan. In the event that it is Glaucoma, he had given her an eye drop prescription that will prevent the pressure in her eyes from getting worse. She needed to stop using the drops about 4-5 days before the exam under anesthesia so an accurate reading can be taken. If she does need to have the surgery, it would be done one eye at a time. The first eye would be done when she is about 8-9 weeks old, and the second eye about 4-6 weeks later. At about 8-9 weeks is when her eye will best be able to accept the donor cornea. Any earlier than that and the risk of rejecting the transplant is higher. The second eye will be done after the first eye has had time to heal.

After the surgery has been done, she would need alot of eye drops, follow up doctors visits and would probably need to wear glasses. But if she can see we will do whatever it takes. We just want her to be able to see the world and the people that love her.

It was a tough pill to swallow, but we left his office with a feeling of hope. A feeling that maybe she will be ok in time. And that is all we can ask for, for there to always be hope for her.