What a difference a year makes.

Exactly one year ago, on this day, I was sitting at my very first appointment at the pediatric opthamologist.  I knew something was terribly wrong with Julia's eye.  I could tell in the way the pediatrician had talked to us at her well-baby visit the evening before.

This is the day of the appointment.  I immediately noticed the cataract in the picture.

That day is pretty blurry, yet some things are crystal clear.  I remember what I was wearing (black pants/purple sweater) and what Julia was wearing (turquoise flower outfit).  Julia was amazing all day.  We were there for several hours doing several tests.  I was terrified that she would scream, because she had been screaming most of her short life.  But she didn't.  I think she just knew, in her baby wisdom, that this was a rough day for mommy and she was quiet the whole day.

I remember hearing the words "cataract, surgery, contact lens."  I know I asked what would happen if we didn't have surgery.  Getting the answer, "She will be blind in that eye," was not reassuring.  I remember the fellow explaining that the lens is shaped like an M&M and they were going to "suck" the chocolate out (that is the cataract) and I remember being told about a stalk.

Waiting for first surgery.

I actually didn't freak out until the next day.  Once I got on google and started doing some research, I realized that my daughter probably had phpv.  I realized we would be patching.  And I was terrified that this wouldn't work.  That my baby wouldn't see.  That her eye wouldn't grow and wouldn't develop.

And then we waited for surgery.

Which is ironic, because here we are again.  One year later, and we are again waiting for surgery.

We have been through 3 surgeries, countless patches, at least 10 contact lenses, 3 sets of glasses and probably 15 eye doctor appointments.  I've endured the stares, the comments, the snickers and laughs from strangers.  I've also experienced the support of wonderful imoms, friends and family. 

After second surgery.

Other imom's told me, "One day your life won't be consumed by the EYE."  I don't think I believed them.  But it has happened.  Her day is more about being an active toddler and less about the EYE.  Yes, she wears glasses.  And yes, she still patches.  But she also walks, climbs, plays with toys, and throws food off her highchair.  Don't get me wrong - the EYE is always there, in the back of my mind.  But I am learning to trust my mommy instinct and trust the doctors.

We lost a lot of these.

I can't believe it has been an entire year since we first learned about the EYE. 

This year was long, painful, wonderful and momentous.  It inched along like a turtle, yet flew by in an instant.

I never imagined this day would come.  A day where I finally feel like we are normal.  Just a little family, living life, enjoying one another, and sticking patches on an eye.

My little man.

My beautiful doll.

LOVE

 

 

No, no, no...

 NO GLAUCOMA!!!!!


No time to upload pics or anything, but I want to shout from the rooftops that Julia does NOT have glaucoma!  Pressures are normal, optic nerve is not cupping, and measurements are great.  Doc said that they are not sure why her eye grew so much in such a short time - but I know why.  Prayer.  We have been praying for her eye to look normal and be as healthy as can be.  And her eye grew and is healthy!  Her EUA went great!

Doc said that for having PHPV her eye looks as good as it can.  We caught the cataract early, we are patching, staying on top of everything - she looks great!

We discussed the problems with contacts and the surgeon feels we should see how this next contact fits and then possibly discuss doing the IOL sooner than later - possibly after she turns a year old.  It is something we will need to discuss more, but I am hopefull that he thinks she is a candidate for an IOL!

Ear tubes went in great - she had pus in her ears when they drained them so it is a good thing we did the tubes.  She was crabby when she came out of anesthesia but very happy to get her bottle!  She was a trouper all day - even when she was hungry.

I am so thankful for all my friends and family who pray for Julia and send us good thoughts.  We so appreciate it!  We know we still have a long road with lots of patching and contacts, but we are thankfull for the good news today.  We are going to celebrate this victory!

Lost...

I am feeling lost.  Afloat.   Adrift.  The highs and lows are taking their toll and I am feeling defeated. 

Yesterday we saw the eye doc and got a new contact.  This custom made piece of silcone was carefully placed into Julia's eye.  The doc said it looked great.  I said it looked great.  Even the fellow working with the doc said it looked great.  This is the 4th try at getting a contact to fit her eye.  Last time the doc ordered a contact that is flatter.  It didn't stay in (in fact, we lost it at church last Sunday.  Imagine me, my mom, and my dad all crawling around on the floor of the sanctuary with flashlights trying to find a contact. Yep - that was us.)  So this time he ordered one that is bigger.  12.5 mm to be exact.  An adult usually wears a contact that is 14 mm.  Julia started at 11.3.  12.5 is usually the size contact an 18 month old would wear and Julia is only 7 months. 

I was on top of the world yesterday.  I was convinced this contact would stay in.  It was SO big and was difficult to get in.  So I reasoned that it would be difficult to fall out.  I was excited to start patching regularly again (even though patch time has been increased by one hour).  It didn't phase me that the doc wants to check her pressures in 2 weeks - I was just so happy to have a contact that would stay in!

So today we patched.  And boy was she crabby!  She is teething and drooling everywhere.  We actually went through two patches because of her drool!

I wanted to enjoy the day so we went to the park.  Julia sat on a blanket in the shade and then I carried her around the playground a bit.  She seemed to enjoy it.

Then we went to McDonald's so Jed could have a happy meal.  It was chaotic, but fun.

Last stop of the day was Costco (Jed needed diapers).  As I am pushing the cart into Costco, I figured I would check her contact.  I swore I checked at the park and it was there.  But when I looked down - it was gone.  I double checked - yep, gone.  And I lost it.  I cried.  I called my mom and sobbed.  People were walking past me by the doors to Costco and looking at the crazy lady losing it on the phone.

I felt like a failure.  Why didn't I check more often?  Why had I been so sure of myself?  I was such an idiot - just assuming this one would stay in. 

And it isn't even the money.  Don't get me wrong, it is nerve wracking to think that at some point insurance will probably not pay for these lost lenses and we will be paying 150 dollars apiece.  But I don't care about the money.  I care about my daughter's vision.  And right now she is not wearing a contact on a regular basis and she is not patching on a regular basis.  Which means her brain is not connecting with her eye on a regular basis.

Adrift...not sure what to do or where to go.  I've thought about a second opinion from another eye doc - but I love our PO.  He is wonderful.  And I don't know that it is him that is the problem.  I know that because of the PHPV, Julia's eye is not shaped normally.

I've thought of glasses - but she won't keep them on and I really don't think she can see out of them.  It is like looking through a little magnifying glass.  And honestly, they really are not best practice for her situation.  We are going to attempt to keep them on her to at least have patch time for the time being.

So I guess we try again.  So another contact has to be ordered, made and shipped.  Which means it is another 10 days or so before we get it.  And hope/pray that it stays in.

Her eye is watering and she is fussy.  I think she is teething, but I worry that it is more than that.  Doc is concerned about the eye watering and wants to check her pressures at her next appointment in 2 weeks.  (For those of you that are not familiar with cataracts - because of the surgery and condition that Julia has she is at high risk for glaucoma.  Checking her eye pressures will tell us if she is developing this as well).  I am thinking if the fussiness and eye watering continues I may call and try to get in earlier just to have peace of mind.

So I am feeling down, low, lost, afraid, nervous and scared.  I am scared.  I am scared for my little girl.  It is hard feeling so helpless.  I wish I had answers and could just fix everything.  I wish I could see the future and see that everything turns out okay. 

New Friends

I had the most amazing evening last night!  I was able to meet - in person - another mom of a kiddo with phpv!  I met her through this blog and found out she lives in my state (well, our state - since we both live here).  She is on the other side but her family lives near me.  She was going to be in town to visit family so we met for dinner.  The awesome thing is that her son is only 5 days younger than Julia - they are literally the exact same age.  So we are both in the same stage of this journey.

He was so very cute!  We talked about contacts and surgery, our fears of glaucoma and how odd it is that we have to pack contact cases and solution in our diaper bags with bottles and wipes!  I felt like I was talking to someone who really GOT me - who understood the fears, worries, concerns.  She had literally walked in my shoes and it was a connection I will always cherish.

I have met other great moms through blogs.  And while I don't get to see them in person, I can feel their support and know that I have great women to turn to for advice and a shoulder to lean on.  And I know that they GET it.

Eye moms are the best.  We are strong and resiliant.  We fight for our kids vision.  This war may be long and the battles rough - but we are going to see it through to the end.  I have to believe that when I put a contact in Julia's eye and she is screaming - I have to believe that I am doing the right thing.  When I patch her eye and she is grumpy and clingy - I have to believe that we will win this war in the end.  When I take her to the opthamologist and they put in drops and use a metal contraption to hold open her eye - I have to know that this is worth the fight.  That when she is 16 and has both eyes to see out of - that she will thank me.  I am going to do what is the very best for my daughter - even if it is hard, painful, heart-wrenching and difficult.  Cause that's what mom's do.

And Kimberly, the wonderful mom I met last night, she is doing the same thing for her beautiful baby boy. 

And a pic of us will follow - I was a goof and forgot my camera so as soon as Kimberly emails the pic of us and our babies - I'll post it!

The search for a contact

So you may think that Julia has lost a contact and we are searching for it.  (Knock on some wood) That is not what has happened.  Rather, we had to really search to find a contact that fit.  In hindsight - we actually were pretty blessed that it only took about 3 weeks.  Other moms try contact and contact with no luck.

Two weeks after surgery we went and saw the great Dr. H who is the pediatric optomotrist at St. Louis Children's Eye Center.  He is wonderful!  He had gotten Julia's chart the week before and ordered some contacts to try even before he had seen her for the first time.  That first appointment was good and I was impressed by Dr. H's honesty.  He answered my questions with truthfullness and compassion. 
"Will my daughter be able to see?" -  We just don't know.  We are going to treat her as if she is going to gain 20/20 vision.  Only time will tell.

"Is her eye going to grow?  Is it always going to be small?"  -  Again, we just don't know.  Some kiddos the eye grows and you can barely tell it is smaller.  In others, it doesn't.  Only time will tell.

He had contacts to try, but unfortunately Julia's insurance had not come through yet and in order to try them we would have to pay $400 for the contact and the fitting.  He suggested we give the eye another week to heal and another week for the insurance.  It was stressful and I was worried about her not having a contact, but we waited.

The next week, I called before the appointment to see if the insurance had come through.  She had been denied.  I was crushed.  I didn't know how we would keep paying for contact after contact.  I immediately called our benefits person at our employer and the doctor promised he would call the insurance as well.

So at that appointment we paid for the contact and tried to get one to fit.  Dr. H had ordered 3 different silsoft by Bausch and Lomb.  These are the common contacts used in aphakic infants.  We tried the first one and knew there was no way it would work.  You could see the edges lifted all around her eye.  Because of the phpv, Julia's eye is very, very steep so standard contacts won't sit securely on her eye.

He had also ordered a few custom made ones and we found one that seemed to fit, even though it wasn't quite the right prescription.  So we left with that contact but instructions to not patch since the prescription wasn't quite right.  That afternoon was a huge mixture of emotions.  We found out the insurance had approved the contact so we would be reimbursed, but the contact fell out multiple times.  The last time it fell out we found it on the floor next to the end table.  I burst into tears.  I didn't know how I was going to get through the next few years always worrying about the contact falling out.  How would she go to daycare if the contact won't stay on for more than an hour!  I lost it and sobbed in James' arms.

I emailed Dr. H and told him the contact wouldn't stay in and could he order a different one.  He did and in the meantime we kept her contact out.

The new one arrived a week later and it was the steepest curve the company could make.  A normal eye has a base curve of about 40 - Julia's was measured at 60.  Her contact is literally custom made to specifics just for her.  This one was also a little bigger, so it stayed in better.  We went home and started the patching journey.

 Amazingly, the contact has never fallen out (knock on some more wood and say a few prayers).  Other than some sickness that lead to an eye infection (more on that later), Julia has been wearing her contact for about a month.  We patch for an hour a day and see the fabulous Dr. H in a few more weeks.  Patching isn't easy - she gets pretty grumpy and clingy when patched.  But we keep on going knowing it is our only chance!

Surgery

Julia's surgery was scheduled for December 22nd.  At first, she was an emergency add-in to the surgeon's full schedule.  So for several days we did not have a time for the surgery because she was going to be "squeezed" in.  The day before surgery, we got a call that the doctor had a cancellation and they were re-doing the schedule.  She was the youngest patient, so she would be the first surgery of the day.  I trusted the surgeon as he was the best in the area (people fly in all the way from California to see him) and he does this type of surgery all the time.  Julia could not have any formula after 2 am, no liquids at all after 4 am and we had to be at the hospital at 6am.

Children's Hospital is wonderful!  Everyone was so friendly and helpful and supportive of the parents.  My mom went with me and James for the surgery.  We got there, checked in and were taken back to a room in the day surgery area.  Julia was put in a little gown and she had to have 2 different drops in her eyes three times - 5 minutes apart.  She was amazingly happy.  Here she is on the hospital "crib":

When they came to get her for surgery, I was so scared.  I won't lie and say I was strong - I wasn't.  I cried as the nurse carried her away.  They told us it would be a couple hours depending on what they found.  They were doing a EUA (exam under anesthesia), a lensectomy and vitrectomy.  They did the iv after giving her some gas so I didn't have to see that.  Thankfully, they were back to see us in about 1 1/2 hours.  The doctor reported that everything had gone really well.  They had removed the cataract and when they went to take out the "stalk" it had just fallen away.  It was still attached at the back of the eye, but the retina was good and he was very optimistic about her regaining vision in that eye.  I was so happy!  The surgeon came and told us basically the same thing as the other doctor.  Then the nurses came and told me the best news - we could go back to recovery and see Julia.  We went into recovery and she was all swaddled and cozy.  We carried her back to our room and she started to wake up.  We fed her some pedialyte and some formula.  Since she kept that down, we were able to go home!  She had an eye shield that had to stay on until her appointment the next day, but I could tell that her pupil now looked black and normal!



She actually was fine the rest of the day!  We planned to go back to Children's the next morning to have the shield taken off and have her eye looked at.  We weren't sure when she would be getting her contact but we were hopeful that it would be soon.  I was just glad to have the surgery behind us so we could move forward and start saving her sight!

Google is not always your friend

After the appointment with the Eye Clinic, I started googling everything I could about PHPV and cataracts.  It was awful.  There is very little information out there about PHPV and most of it is pretty grim.  I read about micro-eyes, prosethic shells, blindness.  It made me crazy!  However, I did find some wonderful blogs from other moms that helped me put things into perspective and realize that I would adjust to this new "normal."  I found cute patches and tips for putting contacts in infants.  But deep down I was petrified.  I was afraid of what they would find in surgery - would the retina be okay, would she be able to see?  These questions wouldn't leave me.  I basically ran on auto-pilot for the week in between her doctor appointment and the surgery.  So my advice to others - limit your google!  Not all the information applies to you.  Take what does and forget the rest!

Appointments

Julia went for her 2 month well-baby check up on Dec. 13th.  I was still worried about reflux and her screaming, but I did mention to her doctor that her eye looked "funny."  She looked at it with a light and agreed that something was wrong.  Suddenly the appointment was all about "the eye."  No more was said about reflux!  She explained that she would be calling a pediatric opthmalogist in the morning to try and get Julia in quickly.  She mentioned cataract and other problems, but I honestly was in such a daze that I don't remember.  The doctor even mentioned that she never sees anything like this and asked if the new nurse practictioner could come and take a look.  I knew then that whatever was wrong with Julia was serious and I started to get scared.  She had her 2 month vaccinations and I took her home.  On the ride home, I remember calling my parents and crying that something was wrong with her eye.  I knew it had to be something serious because they don't send you to a specialist the very next day for routine problems.  My mom and dad were reassuring, but I was nervous.

The next day I got a call at work from St. Louis Children's Hospital Eye Clinic asking me if I could have Julia there at 10:30.  I didn't want to go alone, so my dad went with me.  I am so thankful he was there so that I had another set of listening ears.

Julia was examined by a fellow and he immediately told us that she had a cataract in her right eye.  He explained that your lens in your eye is shaped like an M&M and the inside of hers was cloudy.  They would have to remove the front of the lens and "suck out" the cloudiness.  She also appeared to have a "stalk" in her eye that they would try to remove as well.  Her eye was also smaller than the left eye.  I asked if surgery was the only option and he said that we could not do surgery, but that she would never see out of that eye.  At the moment, she was completely blind in that eye.

They did several tests that day - lots of lights shining in her eye, drops and even an ultrasound.  They determined that the blood vessels that form in utero to develop the eye had not dissipated.  Instead a "stalk" was left and it had caused the cataract.  She had PHPV (or PFV).  They wanted to schedule surgery right away as she already had not had any brain connections with that eye for 2 months.  The doc explained that they wouldn't know the extent of the involvement of PHPV until they were in surgery because right now they couldn't see to her retina because the cataract blocked the way.  After surgery she would have to wear a contact lens (CONTACT - did you say contact in an infant?) and patch to force the brain to connect with that eye.

I know there were a lot of questions I should have asked during that appointment but I was stunned and shocked and scared.  We scheduled surgery for the following Thursday - Dec. 22.  Right before Christmas.

First Post!

So, I have decided to start a blog to share my journey. I have gotten so much support and encouragement from reading other blogs, that I figured I would put my story out there too. Well, not my story, it is more the story of my beautiful daughter, Julia. She is my little diva! She was born with PHPV (or PFV) which is Persistant Hyperplastic Primary Vitreous. This caused a cataract and blindness. Our goal is to now help her save her sight! This involves patching and a contact. I'll share more later - but this is at least a start!