Adele is almost Two!

Adele will be two next month. In the last year she has grown taller and more confident. She walks with her cane in usually her left hand and holds on to someones' finger with her right hand. She loves to sing, dance and rock on her rocking toys: the caterpillar is almost too small, but the Radio Flyer rocker and a giant rocking horse from Aunt Theresa are used every day! Her favorite song is "I like the Flowers" but she sings her ABC song, Twinkle Twinkle, Eensy Weensy Spider, to name just a few. Her brothers have been big Star Wars fans since before Christmas, and she hums the Imperial March right along with the others! She is learning her baby dolls, and loves to feel their fingers, plastic hands and ears, and their toes. She knows all her body parts and is so cute when we ask her where they are; she gets the giggles easily especially when Mommy asks "Where's your bellybelly, Adele!" She knows a tickle is coming! She knows her brothers and says all their names. She explores the ever-patient Henry the dog. She LOVES the feel of the wind and laughs every time. Adele has several books with Braille labels and a collection of touchy books. She loves to sit on Mommys' lap for reading time.
This summer Adele and her parents travelled to Boston to the ICAN convention. The International Childrens' Anophthalmia Network offered all kinds of valuable information, especially networking with other familes(25 attended) with anophthalmic children. They also were priveledged to be seen by the folks at the Albert Eienstein Institute, some of the premiere researchers in this rare disorder. Mom and daughter anophthamics are rare but present worldwide; the genetic mutation can be carried by Adele's brothers as well. They learned as well that Adele's current treatment modalities need to be changed, and so are to go to Columbus to have the solid orbital implants removed and replaced by dermal fat grafts and likely the osmolar orbital expanders as well.(these are small grain-like substances that absorb fluids in the orbit and expand to many times their size). Adele will likely have her eyelids glued shut while these processes take place, so artificial eyes are down the road a bit. We don't mind at all; we are used to seeing her with closed lids as it is rare that she stretches them enough for us to glimpse the white conformers.
This year Adele's therapists noted that her gait is very unusual and that she is very flat-footed. She is to be fitted with orthotic braces to stabilize her ankles and arches. Over time, these should help her develope a more stable stance and walking gait. 
So a long road ahead yet, but Adele is developing as she should! She is bright and happy and learning her world! We are so priveledged to know this little girl!

Reflections on the First Year

August 25th marked Adele's first birthday. Her celebration actually took place a few days before, with all the family gathering at Aunt Theresa and Uncle Paul's house. Everyone passed the babies around, including a few new newer and newest ones! Mommy wrote "Happy Birthday First Birthday Adele" in Braille using mini M&M's under the writing for the sighted folks already on the cake. A special cupcake was ready for Adele, who doesn't like to get her hands messy; after all, who likes to goo up their eyes, which is how Adele uses her hands: too"see"!!  But with Mommy's help tasting the icing, she was a little more interested!  Her best fun was after everyone sang "Happy Birthday"......one of her current tricks is to clap her hands when she hears, "Yay!!"... so when everyone yelled "Yay!!", she clapped her hands.....so, everyone yelled "Yay!!" again,,so she clapped her hands....so again the crowd yelled!!!! This was a powerful moment for her, controlling us all like that, and we laughed and laughed at her obvious delight!

Adele's first surgery took place just a few weeks before her birthday and has changed her appearance dramatically. She is on her second set of conformers in the eye sockets since the orbital implants. One of the current battles is to keep the right conformer in place. We are pretty certain she is right handed, as that conformer is her favorite to remove. Usually, when she is in bed and winding down for sleep, she travels around and up and down in her crib. Then at some point, PING, she pops out that right conformer! In networking with other families of anophthalmic children, apparently this is a common thing.  After all, they are non-functioning, and a baby and young child cannot yet understand the importance of leaving them in place to allow for growth. So, often several times a day, we wet the conformer with contact solution and put it back in!! We have read that anophthalmic adults and older teens chose to removed their eyes when not out in public or when swimming etc., as they can be itchy and uncomfortable, kind of like a tight shoe or a necklace or bracelet.

Adele is coming along on her Orientation and Mobility. With the great help and instruction of Anna at CABVI, we are all learning how  to stimulate her to move, and she DOES!!! Mamma designed a sensory "room" and a friend's husband built it for her. It is wonderful, for Adele and for her brothers! She stands, moves around in it, and listens to her voice echoing. We have caught her "clicking", and wonder if she is doing some echolocation practice!!

And so on we go!!!! Every child's life is an adventure, and Adele's is certainly no exception!!

We are planning now for her next benefit, and hope to make this fun gathering an annual event!

Adeles Adventures in the World

Adele is now ten months old!!! And what an adventure those ten months have been, for that baby girl and all her friends and family!!  Learning the world of blindness has been a bittersweet adventure; we love to see her grow and learn, but we also know that there is now and always will be SO MUCH that she will be missing!!!  But that is our grief, and not hers.
She is a laughing, chattering, wiggling, prissy girl!!! She has her tricks: Mommy has taught her to shake her head when she hears, "No,no,no,no!!!", and wave her hands when she hears, "Hi,hi,hi,hi!" She sleeps soundly when she sleeps, which is usually ten to twelve hours at night and with a nap or two in the day.  This means she has some circadian rhythm, which not all anophthalmic people have.
So many wonderful people have intervened on her behalf: there are her ocularist, Kathy, and the doctors, along with her therapist Rhonda at Clinton County Early Intervention and especially Anna with Cincinnati Association for the Blind and Visually Impaired.  These folks are working tirelessly to assure Adele developes to her full potential!! We her family are in your debt!!! Thank you!!!
When Adele was three months old, her friends and family arranged a benefit for her, to help with the expenses as they began to mount. One lovely evening in October last fall, we all gathered at Cindy's Friendly Tavern in Loveland, for a chili supper and raffle.  What a wonderful time!!!! Adele's family and friends all pitched in!  The original Batmobile came thanks to Sharon and Chirs. Two bands played, courtesy Uncle Tim's band and Aunt Melissa's band!! They had the place rocking!! Channel 19's Meagan Mongello interviewed Adele and her mommy and put a plug in for the benefit as well.....The local vendors contributed greatly to the success of an awesome raffle, organized by Aunts Emily and Lori, assisted by cousins Jacqui, Lindsey, Jennifer, Laura, and Meredith. Cousins Ben, Mya,Tyler, Shawn, Max, Aunts Monica, Theresa, Pat, Ellie, Patti, and Uncles Tim, Larry,and Paul,all contributed their time and energy in so many ways!!  Big Al came through with an awesome white chil to feed the masses, and Grampa's texmex chili was a big hit!!! Thirty gallons of chili fed the generous patrons!!! At the end of it all the "take" for Adele's donation account was over ten thousand dollars!!  This year of frequent conformers has drained that amount to near zero. But we NEVER could have made it THIS far without all of you!!!!  People who had seen Adele's story on TV would pull up, jump out and thrust money at us, just to help out!  We have been so very humbled by all of this outpouring of support!!! Thank you, thank you, thank you!!!!
As Adele grows, she is becoming more mobile.  She has yet to crawl, but sits up strongly, and rocks back and forth on all fours. One of her favorite things is to stand and hold Daddy's hands and lean WAY forward and back, very fast!! She is learning where she is in space!! Then she will let go of hands and concentrate very hard on balancing. But for now, she quickly grabs us for support. No problem there, little girl!!!
Adele holds her own bottle,(kudos to Aunt Lori!) eats well (for Daddy!) and is taking some finger foods like graham crackers.  And she is now the proud owner of two teeth, which are VERY sharp! Her upper gums are next and we see that several more will erupt any day now.
On July 26th, Adele has her first surgery. Dr Nunnery in Indianopolis will implant some sort of orbital expanders; his plan is that, given Adele's CT scan measurements, expanders that are close in size to adolescent orbits will be inserted. He hopes that Adele need never have this particular surgery again.  Other surgeries to help with eyelid formation will likely be in her future.
At some point after this we would like to have a VEP test done: Visual evoked potential can determine if anophthalmic or microphthalmic people are able to detect light. We know through networking with other families that this is sometimes possible, and there have been a few instances in Adele's life that have made us wonder if, at times, she does have some perception of bright light. More noticeable is her natural sleep pattern, uncommon in anophthalmia.
This next Saturday, Helping Hands Golf Outing has their annual outing, with Adele chosen as this years' benficiary! We are not at all familiar with golf, but will be on hand to volunteer and  to cheer on the competitors!! What IS a golf cheer, anyway? Or is it just that quiet clapping?
Well, though this entry may be lengthy, there was a lot to say!!!
Adele is a happy, healthy baby, loved by and nurtured by all who know her!!! We are so glad to know her and have her with us!!!! And she is certainly bringing joy as she grows and learns!! She sees us with her little fingers, and is teaching us so much as well. It is a different way to see. But it is a wonderful way!!!
  

update

Lets see...  Since our last posting Adele has been busy!  She was sent to Cincinnati Children's Hospital for a CT scan which we were able to get on disc and look at for ourselves.  Although we can't really tell what we're looking at, it seems to be very vague.  We see Dr. Kulwin this Wednesday, the oculoplastic doctor who requested the CT, and we won't be surprised if he asks for a more detailed MRI.  We were previously warned before Adele's CT that she may have to be sedated and admitted if she couldn't be still for the short 3-5 minutes the scan would take to run, but our little trooper did great!  With a little "sweet-ease" concentrated sugar water on her passie, Adele was able to snooze for a few minutes during the scan.  Following the scan, we met with Gina Carrol from the Cincinnati Association for the Blind and Visually Impaired, and were able to discuss with her our hopes for Adele, and how their association could be of assistance.  We were happy to hear how impressed she was with Adele's attentiveness at such a young age, and were all in awe of how she was working so hard to hold her head up for such a long time during our visit.  It was great to talk to someone who understands that blindness does not mean living a complacent life.  The following day, Neal and I took a trip with Adele to Indianapolis for a visit to the ocularist by the name of Kathy Hetzler.  An ocularist is a person who makes and fits artificial eyes and scleral cover shells.  The prosthesis is made of plastic and worn inside the empty socket or over the unusable eye tissue.  The trip took three hours one way, and took all day.  Although long, the drive gave us a nice opportunity to spend some time together, and for us to get closer to our baby girl.  Upon arrival to the ocularist, Kathy measured Adele's sockets by placing a small conformer in each orbit, and determining how much bigger hers would need to be made.  We were then asked to leave for a couple of hours while her conformers were being made, and got to have a nice lunch and shopping break.  When we returned, Kathy put Adele's conformers in - her right side went in easily, and her left had to be readjusted after struggling to get it in - although Adele didn't seem to enjoy getting her conformers put in (she showed off her set of lungs!!), she doesn't mind at all that they're there.  Because of Adele's age and rapid growth, the ocularist said that she will need a new "set" every three weeks or so.  The conformers look like a tiny clear concave shell attached to a small clear "peg" that sticks out so as to be able to move them around (stretch the tissue) and to be able to easily remove them (see our pics).  Adele does look "different" with them in, but I can already tell that her orbits appear to be much fuller than before (her lids aren't sunken in anymore!).  Our family and Adele has adjusted well to her conformers (the boys ask "What are those plastic things in her eyes?") but has posed a new challenge to taking her out in public.  As a proud parent it is instinctual to want to "show off" Adele, but with her conformers I feel as if I don't want to frighten those that are unaware of her condition - and don't want to take the time, or don't have the energy to explain her condition to the waiter, the cashier, or the nice old folks walking down the street.  Kathy did tell us however, that she anticipates Adele having actual prosthetic eyes around the time when she turns six months old.  She asked us to be thinking about an eye color (never thought we'd get to choose our child's eye color!) and we can't wait to see her with them in.  I know in actuality that she won't see us, but to see what it would be like to have her "look" at us will be both wonderful and heartbreaking.  This week holds another doctors appointment, and in two weeks we'll be visiting Indianapolis again, but things are looking up!  We've started to emotionally stabilize, but still have our moments...  looking forward to what the doctor has to say and will keep you posted when we hear! 

So it begins...

This week marks the start of Adele's journey into the world of Oculoplastic surgery, ocular prosthetics, and orbital reconstruction.  Yesterday we took Adele to meet the Oculoplastic Surgeon by the name of Dr. Dwight Kulwin.  Our family is very familiar with Dr. Kulwin, as he was my oculoplastic surgeon as a child.  Before her appointment, our family was very anxious and anticipating a visit that would result in disappointment.  We were happily mistaken.  Dr. Kulwin informed us of different courses of action that could be taken in Adele's situation.  He told us that if Adele is truly anophthalmic (no eyes or remnant eye tissue) that the treatment is mostly all surgical in order for her orbits and related structures (sinus' - brow) to grow.  However, if there is any tissue at all (even microscopic) her orbital structures will "want" to grow and may only need assistance through surgery.  If she is truly anophthalmic, there is a tissue transplant procedure (tissue is transplanted from her buttocks to her ocular orbits) that can also potentially stimulate orbital growth. - - (After hearing that, I was more than happy to offer some of my butt tissue!!)  Also, if Adele has no ocular tissue, she may be a candidate for "expandable ocular implants" rather than the "conformers" that are used otherwise.   We were glad to be given so much information, and happy to discover that different treatments may be available.
Also, Adele and I see one of the Children's Hospital Human Genetics doctors tomorrow, and will take a trip to Inianapolis to see the Ocularist next week (the people that will make Adele's conformers and if needed a scleral schell).  We'll keep you posted!!   

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Welcome to the Blog for Adele's Eyes