Mayonnaise

I love mayonnaise.   I don’t know how to spell the word well, but I love it mayonnaise nonetheless.   My husband hates mayonnaise.  He has been known to throw a perfectly delicious hamburger out the window, just because it had mayonnaise on it.   Or wait, was that the time he got cheese on his hamburger?  

Maybe we should circle back to me, and how I love mayonnaise so much that I’ve been known to eat mayonnaise sandwiches.   I’ve debated if it was really necessary to have tuna fish in tuna salad, forgoing the latter for extra mayonnaise.   There is no such thing as too much mayonnaise as far as I’ve been able to tell thus far in my life.

I was on vacation this past weekend with my in-laws, husband and children.   We were in a time share they were spending the week in after we stayed for the first weekend.   I was making a sandwich just before we departed and assumed there would likely be no mayonnaise in the refrigerator because maybe like son like parents and mayonnaise was low on their priority list of condiments to stock for a week-long stay.

I was pleasantly surprised to find a jar of mayonnaise in the refrigerator door and said as much to the room in general as I prepared to spread some onto my bread.    My mother-in-law immediately said, “oh yes, I don’t travel anywhere without mayonnaise.”

I knew I liked her for a reason.

The Big Boy Update:  My son was interested in a catalog today of children’s items.   My husband got him a pen and told him to circle all the things he wanted for his birthday and Christmas (because he kept asking about Star Wars items.)   About ten minutes later I heard my husband say from the next room, “ah, I see you circled everything on this page.  Oh, and you circled everything on this page too.   And I see you also circled everything on this page.  Okay, okay, you can keep working, you still have time before bed.”   We had to promise to keep the catalog and pen out so he could continue his work for tomorrow.

The Tiny Girl Chronicles:   My daughter is remembering all sorts of things of late.   I don’t know if it’s the temperature starting to change or the feeling that fall is coming, but she’s asking questions I about things I’m surprised she remembers.   Today she asked me, “when is it time for bring your head housing?”  It took me about three minutes to figure out with follow-on questions to figure out she was asking when it would be time to do gingerbread houses.

Heavy Wiring

Did I mention we were having solar panels added to our house?   I honestly can’t remember if I have. We’ve been looking at and considering for two years now and it’s finally happening after lots of evaluating, considering, applications and approvals.

The panels aren’t up yet, but we’re prepared for them.   We had two crews in our house today getting their exercise going from the attic to the basement to the attic to the basement, etc. trying to get all the wiring in the places they needed to be for when the panels are up on the roof and ready to send their sun-powered energy direct to our house.

A thank you goes out to our builder again, for his foresight in recommending we put chases in the house.   The addition of the PVC chase from our attic to the basement mechanical room has made this installation much more straightforward than it otherwise would have been.

One of the crews today was scheduled to be on the roof, but due to weather otherwise known as rain, they worked inside alongside the first crew.  Tomorrow if the weather holds, we should have panels on the roof.

The Big Boy Update:  My son has been such a help lately when his sister has been upset.   She’s taking prednisone and it has thrown her off kilter, off balance and out of whack.   When she’s been her most upset, screaming at not wanting breakfast because she hates all food and all drinks and is also complaining she’s starving at the same time, he calmly eats his meal and tries to be helpful if he can.

The Tiny Girl Chronicles:  My daughter’s vision is so hard to quantify.   My husband messaged me last night while I was out, “on one hand she lost interest in the TV, saying she had trouble seeing it. On the other hand she is now making a Mario level and I asked her, ‘can you see better today?’ and she said, ‘yes.'”

Paitence, Persistence and Positive Attitude

One of my parent’s good friends is a retired ophthalmologist.   My  mother was talking to him today and catching up.   He asked how my daughter was doing and my mother gave him an update from when she saw my daughter just a few days ago.   He told her he had some advice, and would she pass it on to me.  

My mother send me an email with his advice in it and I think the timing could not have been more appropriate.   Here’s what he said:

Patience — recovery will take time.
Persistence — Stay the course with the treatments the doctors are using.
Positive Attitude — this will be transferred to your daughter. She needs to sense a positive environment, not a negative environment.

I have been trying to have a positive attitude personally, and with anyone who asks how my daughter is doing, but it’s been growing more difficult.   It’s been weeks and her vision hasn’t gotten much better from what we can tell.   I understand things are healing and through that process her vision won’t necessarily show improved vision quickly, but it doesn’t help when I see her unable to do discern the most basic of things.

Several times recently I’ve delivered a less-than-positive update to someone who’s asked about my daughter.   Tomorrow, I’ll remember my parent’s friend, Gerry’s advice.

The Big Boy Update:  We were at the beach this past weekend eating at a Mexican restaurant on their outdoor patio.  Small birds were swarming at the periphery of the area, looking intently at our table.   My children discovered they could throw food to the birds and they’d come up to eat it.   My son loved doing this and nearly refused to eat his meal, he wanted to feed the birds so badly.   He said, “I named all of them ‘Cutie’.”

The Tiny Girl Chronicles: My mother’s friend, Gerry, also suggested we don’t ask my daughter questions about her vision, and that’s something we try very hard to do.   My daughter did, however tonight, ask for a vision game.   She asked for the game I played with her that threw me into a tailspin of despair when I thought she couldn’t see anything out of her right eye.   She asked if we could play the game where she tells me the color of the jelly bean with one eye.    We did the game and she could tell colors of beans in my hand about six inches from her face in both eyes, which was a relief to me.

Credit Card Jacked

Saturday morning on vacation this weekend I was woken up by repeated text message ding sounds from my phone.   I’m not unaccustomed to text messages incoming in the early, dark hours of the morning, what with all the running coordination my neighbor and I do, but this was unexpected.   It was multiple messages all within the same minute.    I grabbed the phone and looked at a collection of messages that looked like this:

Given that I was a) asleep and b) not outside the United States, I think we can all guess what was happening.    I wasn’t upset, because with the great text message at point of sale feature a lot of credit cards have now, I knew about the fraudulent charges as they were happening.    I grabbed my phone, went into the bathroom so my talking wouldn’t wake up my husband and called the number.

The fraud person verified it was me while I logged onto my Bank of America account on my iPad.   I noticed the charges—more had come in by now—weren’t showing up on my card.   The gentleman helping me, Andrew, said, “the transactions are being blocked before they ever get to you.   They were flagged suspect when they came in.”

As he and I talked through the card being cancelled and a new card being sent to me, he said, “boy, they are really trying to use that card.”   About that time I got several more charges, this time at over $400 each.   Shortly after that he had disabled the card and the messages stopped.

I’ve never had anything like this happen to me.   It was fairly exciting for a few minutes.   But it didn’t keep me from going back to sleep afterwards, though.

The Big Boy Update:  At lunch today we were talking about friends and toys when my son piped up and said, “don’t forget, I have a hologram of everything I like.”

The Tiny Girl Chronicles:  I wish I could say my daughter’s vision was better today than it was the day before or even the week before, but it’s so hard to tell.   There are easy, close, simple things she can’t see at all and then other things in the same range of vision she seems to be able to see.   She can’t see much at all at a far distance from what I can tell, and that doesn’t seem to change.   We went on a big carousel today and at the top she could see a large, blue roof, saying she saw the blue umbrella.    Again, sounds depressing, sorry.  I was hoping to have a better frame of mind today, but it’s so hard to watch her stare blankly into space and not even try to look at things around her.   Tomorrow, I’ll put my positive hat back on and report more.

Back Seat Bad Idea

We have a different kind of car seats now for the children that can be quickly and easily moved from one car or seat to another.   On a whim, last week I moved one of the seats to the third row.   We had a nice time having an adult sit beside each child.

For school, the seat was returned to its usual spot but this weekend we moved the seat back again to the third row, because it’s a lot easier for an adult to get into the second row and eliminates crawling over a seat from one side.     This, however, turned out to be a bad idea.

There were arguments.   There was crying.   Jockeying for position to get first in the car was observed and parents yelled (that would be me.)  

Unequal is never as good as equal when it comes to children.

The Big Boy Update:   My son wanted to catch fish with fishing nets my mother-in-law had gotten for them today.  My son didn’t catch any, but my husband caught several small fish at the edge of the shore at an inlet.   My son wanted to feel the fish and we let him reach down into the bucket to touch the fish.   He touched it, snatched his hand back and said it tickled.   About that time the fish jumped out of the bucket.   My son screamed and ran after it as it swam away.   He was so excited, running in circles in the splash, he confused the fish and my husband re-caught him.

The Tiny Girl Chronicles:   My daughter asked us to close the doors to the laundry room closet on her this afternoon.   When she was shut inside in the dark she yelled out, “I am the queen of night!”

The Countless Coins

When I was young we would go to Wilmington, NC to visit my father’s mother.   My grandma lived in an old, creaky house that managed to be warm and inviting in some areas while being dark and scary in others.    There were rooms that were never opened, food in the refrigerator that looked like it had been wedged into the back for years and old, unknown things in corners of rooms that were exciting to an inquisitive young child.

I’m sure the house had been painted at some point, but the amount of paint flaking off seemed to be more than what was holding on.   I spent a lot of time looking at the paint because there was a front porch swing and I loved swinging.   There was a massive, old tree in her side yard.   As an adult I can appreciate the majesty of that tree (which lives only in my memory) because there were huge branches going all across the whole of the side and back yard.   The trunk itself must have easily had a diameter of seven feet.    And those branches, oh the branches.   It was a tree climber’s dream.  I spent many fun summers days at my grandmother’s home where she raised my father and aunt.

My grandmother had several siblings.   One of them lived across the side street from her house.   I didn’t go over there much as it was more on the scary side of houses than the interesting kind.   I do have one particular memory of their house though, and that was of the coins.

I was too young to have any interest in money myself, but I did know what money was, especially coins.   We went over to my great uncle and aunt’s house one time for a dinner or something because I was there for a while and I got to wandering around.    I had noticed there were some containers with coins in them in the main room and didn’t think too much about it.   But when I went to one of the rooms off from the living room, I didn’t expect to see buckets and bins full of coins, arranged in somewhat haphazard manner, taking up a lot of the floor space in the room.   There weren’t as many containers in the other areas of the main floor, but when I got to looking I notices more vats of money in other places I hadn’t seen initially.

As an adult I would have been intrigued.  I would have wanted to know just how much money a bucket full of nickels or dimes or quarters was worth (they were organized by kind.)   I would also have been interested enough to have to go and ask what was up with all the money, in clearly non-trivial quantities, left unattended around the house.   As a child, I was too shy to ask.

Later, when I had come home after my week’s visit, I asked my father about the coin mystery.  He said it was because they owned a laundromat and the coins were from their machines.   So the mystery of the money’s source was solved.  But why were they all over the house and not deposited in a bank, getting back into general circulation and taking dramatically less floorspace up?   Was my uncle distrustful of banks?   Did he hate rolling coins to deposit them?   Or did he enjoy being surrounded by the success of his business?   I may never know.    Maybe my father does.

The Big Boy Update:  At dinner tonight my mother-in-law saw a brindle-colored greyhound dog and said, “look at that beautiful dog.”  My son looked over and said, “no, he’s dirty.”

The Tiny Girl Chronicles:  We were on the highway after lunch today, heading out of town for a weekend’s vacation.   My daughter asked from the back seat, “are we going lemon speed?”   My husband told her that yes, we were going the speed limit.

Fitness Update:  Okay, four one-tenth mile runs with seven pound weights in each hand doesn’t sound like much of a killer workout but this morning I asked my husband to bring me the Advil to the bed, because I didn’t want to get up to get it.

Oh Say Can She See

Are you singing the US National Anthem now?  Sometimes titling a blog post is obvious and sometimes it’s challenging.   Sometimes, as I sit down to my computer to write something here, it just appears out of no where.   Tonight, the first line of the national anthem appeared.   The question is, is it relevant?

With everything going on with my daughter’s eyes from a technical perspective, I haven’t spent a lot of time talking about the observational side of her vision.   What can she see?  If she were older she would be able to describe what was happening.   She may not be completely aware of how her vision is changing over time.   Is she able to compare what she can see now with what she remembers seeing before?  What we can tell is she doesn’t want to talk about it, so she is aware things have changed and she can’t do or see things she could before.  

Here are some examples of things we’ve observed.   And when I tell you these things, keep in mind some of the time, small children just don’t want to answer questions, so we’re guessing only a portion of these observations are accurate.

- In the dark we talked about car lights.   I asked her if she could see red lights and white lights and if she could tell me where she saw them (red lights being brake lights in front of us and white lights being the oncoming lanes of the highway.)   She could see the red lights, but couldn’t discern the yellow flashing turn signal.

- Road signs (huge, big and small) she can sometimes see them, just before they pass us.   She can get the color correct some of the time, but if the sign has words or other things on in large contrast, the sign usually is “white” to her as the colors blur together.

- She doesn’t recognize people from far away or even middle-range.  If you get close to her (don’t speak or she’ll know it’s you from your voice) and she’ll recognize you and not be even remotely surprised that you’re there.

- She knows where to look for an elevator button and is happy to press the up button or the button for floor number three, but she can’t read the numbers on the buttons some of the time.   She got confused the other day because the button labels were round and black and to the side of the steel button on a steel panel.   She was only able to see the contrast of the label and not the row of buttons.

- She couldn’t see the peas on the counter today at her place.   She was looking around the counter with her hands to try and find the remaining ones to eat.

- She will use an iPad for a time, but pulls it very close to her face, usually within three inches.  

- She has been willing to watch some television, but we’ve found a way to pull a bar stool up closer to the television over the fireplace.   We’ve heard her middle-range vision is better than her near vision.

- She was on the play set in the back yard and I was on the porch.   I asked her, “how many arms do I have up?”   She responded, “two!”  then, “one!”  then, “three!”   I counted that one as a big, “I don’t know.”

When I read over this list it sounds fairly depressing, but she is getting enough information to function in her daily life on most levels.   People don’t know she has a visual issue going on most of the time unless they’re around her for a while.   I fully plan on putting up a version of this post at some point in the near future, listing all the things above that she can do with ease now that her eyes have healed.

The Big Boy Update:  My son explained to me you call the bad guys “mega monsters” when they’re hugenormous.  

The Tiny Girl Chronicles:  I got to sit in a car beside my daughter the other day.   Typically, my children are in the back seat and the adults are in the front or third row of the minivan.   This past weekend we put one of the car seats in the third row and I got to sit beside her.   She thought it was loads of fun to sit beside an adult.

Fitness Update:  Measly two miles, but I did run some of it carrying fourteen pounds of extra weights.   That’s not fun.

In Ocular News Today…

This morning my mother and I took my daughter to the Duke Eye Center for another evaluation under anesthesia for her eyes. I was very grateful my daughter was able to get an earlier time slot in the day because small children on prednisone don’t like to be told they can’t eat when they’re ravenously hungry and easily outraged due to their medication.

Here’s the update from the evaluation:

They did a fluorescein angiogram of her eyes again to view the blood flow.   She had low blood flow before and as a result, aberrant vessels grew to try and bring more blood to the eyes.    Today they saw the best blood flow they’ve seen since we first noticed symptoms.   The major blood vessels to the eyes look to be getting normal blood flow.  It’s not clear if the periphery vessels are getting the amount of blood they need, mostly because it’s not known what blood flow they were getting prior and/or if some of the vessels even existed.    But from a vasculation perspective, this is very good news.

The Avastin injection to reduce the aberrant blood vessels in her eyes looks to have made some headway.  Vessels are looking smaller and no more appear to be growing.   Great news.

They injected her eyes with kenalog, a corticosteroid, which is another way to try and reduce inflammation in her eyes so they can heal.   There was discussion about reducing the steroid drops or the oral prednisone, but since progress is being made, the decision was to keep everything as it is now and re-evaluate in two to three weeks.

Her retinal detachments look better in both eyes.   We knew the right was worse than the left but I asked about the locations of the detachments today.  Both eyes are detached in the lower half of the retinas, which is where fluid would when upright.   The left eye detachment is in the bottom back and the right eye is most of the back, although some of the top was spared.

We have a new item thought:  The edges of the retinas at the bottoms are coming up a little.  The question is why is this happening?   Is it a function of the drainage from the serous retinal detachments from settling fluids?  Is it the beginnings of a tractional retinal detachment?  Note: this is another type of retinal detachment so I’m thinking we should total bonus points for having yet another thing happening to my daughter’s eyes.   The question is, is it the jelly in the eye pulling on the retinas in some way?   If this is the case and it’s related to pulling, something can be done about it later.  For now, the situation is mild and at the very edge of the retinas.   This is not good news, but hopefully the door prize for, “Most things going wrong with your eyes at the same time,” will be pretty spectacular.

The Remicade my daughter had via infusion last week takes six to eight weeks to get to full strength, so we will hopefully see improvements continuing going forward.   The recovery time for retinal detachments of this type are slow, so we’re looking at another few months before there is great vision happening.   GREAT VISION.   That’s right, you heard me.  (It’s coming, I promise.  It’s just taking its time to get here.)

My daughter’s main doctor, the one who has orchestrated everyone and everything to get us here, including  all the experts in their fields being called in to help try and save my little girl’s vision, told me today that everyone would still very much like to find a diagnosis for what she has.   She’s got something, “similar to” multiple things, but none are a real match.    She went to a conference last week and discussed my daughter’s case with other doctors who’s specialties would give her additional opinions.    She told me, “no one has come up with anything we haven’t tried or looked into, but they’re all thinking about it and will let us know if they think of anything.”   She said everyone was equally puzzled.

One last thing: one of my daughter’s “eye conditions” got renamed or re-categorized as a result of talking to others at the conference.    One doctor said, “I don’t think she has Anterior Segment Dysgenesis, I think it’s more appropriately categorized as Persistent Fetal Vasculature.”   I’ll let you look that one up on your own, but what was interesting was I found out that little curtain of vessels she has over her field of vision is not remnants that haven’t shriveled away, they’re blood vessels that are still functioning.  

How’s that for a technical eye update?  So to sum up, very good news today but we have a long way to go to get back to where we were.

The Big Boy Update:  My son came home from school and said, “Mom, can you call me ’Spike’ today?”   I don’t know where it came from, but I told him I’d be glad to.   He followed up, saying, “but only for today, not tomorrow.”

The Tiny Girl Chronicles:  If I were to tell you my daughter does not recover well from anesthesia, I would be telling you a huge understatement.   She is livid and unconsolable and furious and expresses everything very vocally, usually with ear-piercing screams.   She had calmed down somewhat when my mother and I got her in the car to go home, but she wasn’t done being angry.   She informed us, “everybody is poopy.”  She then proceeded to name person after person from her family to neighborhood friends to school mates to teachers, saying them each in turn, starting with me, “mom is poopy.”

Two Cents Worth

This is a literal post.   It’s about two cents and how I discovered it.   It is not about my daughter’s eyes, which will probably be featured in tomorrow’s blog post as we’re going EUA (evaluation under anesthesia) tomorrow at 7AM.   But for tonight, sit back and read all about my two cents.

We’ve done our best to reduce our energy consumption from a light bulb perspective by replacing almost all of the bulbs in our house.   Where applicable, we further added dimmer switches so we could potentially reduce kWh usage even further.   We looked at our energy usage bills and compared from prior years.   We pondered, but ultimately, we didn’t know how much energy each and everything in our house costs.    Then, we had an idea come from an unexpected direction.

Our outdoor lights were on a timer.   Originally, when we built the house, the timer was one of the manual ones with the little tick notches for portions of an hour.   We decided we wanted to go to a digital timer so we could control the low-voltage lights on a day-by-day basis.   My husband found a product called WeMo, which was wifi-aware with an accompanying app that let us set a schedule, manually turn the lights on and off and monitor energy consumption.

My husband got an idea: he would purchase a second WeMo that we’d be able to move around the house to find out how energy-costly various items were.   Yesterday, he got the second controller configured and today I put it into use.

I’d been wondering how much my 1500 watt hair dryer and unknown watt flat iron cost us each day in energy usage.    Today, I found out.   I was really surprised.   Even with the energy rates for our location, to dry my hair and flat iron it, it only used two cents of energy.   The estimated monthly consumption from those appliances for that amount of use per day, we were only at $.79 per month.   I was impressed.  

I feel better.   I think I can manage two cents per day to have my hair look less-than-crazy.    Thanks, WeMo.

The Big Boy Tiny Girl Uncle Update:  My son and daughter have been very excited that Uncle Bob arrived via plane to visit today.   They have been equally disappointed that Uncle Brian didn’t come as well.   We have been explaining that Uncle Brian had to work and he’ll be here in three weeks to see us and spend some time visiting, but they are still disappointed as of today.    Know that you are missed, Uncle Brian.

Fitness Update:  Seven miles running this morning.   I didn’t want to run at the start, and then I didn’t want to stop running at the end.

The Stay Puft Marshmallow Girl

My daughter is starting to look a little puffy.   Her cheeks are a bit plump, and her belly was sticking all the way out from under her t-shirt today.    I’m starting to wonder if we’re going to have to keep her from eating seven meals each day while she’s taking the Prednisone?

Two of the above statements are true and the third one is technically true, but it’s only due to an unexpected situation.   My daughter’s cheeks and face are indeed starting to look a little pudgy.   She is looking a little like she’s retaining water or putting on weight.   And while her belly was sticking completely out from under her shirt today and she kept patting appreciably on her stomach, that part was mostly because we sent her to school in a size 2T shirt, even though she’ll be four in seven weeks.

We found out late last night we’d misplaced a shirt.   It was International Peace Day today and the students are asked to wear their purple school shirts for a picture in which the entire student body stands in the shape of a peace sign.   A very brave teacher climbs onto the roof of one of the buildings and gets a full shot of the children, teachers and staff and then some singing is done and peace is maintained, or at least until play ground time it is.

But last night we could only find one of the purple shirts.   The two shirts move from dresser to dresser because we have two 4T shirts which get worn at different times by different children and get folded and put away with a rather carefree attitude towards who’s shirt really belongs to whom.  It was because of this that we didn’t notice one of the shirts had managed a daring escape to whereabouts unknown.    My husband and I did some bag and bin diving last night and found a very tiny looking 2T shirt and were surprised my daughter’s armpits weren’t strangled this morning when she put it on.  

But back to the pudgy conversation.    There is a lot of hunger going on all day in my daughter’s tummy—she tells us how her tummy tells her she’s hungry.    There is also a lot of anger when we don’t feed her, and apparently grapes and carrots are not what her tummy keeps asking for in the way of food.  

Hopefully the prednisone regime won’t continue for much longer or we’re going to have to get 5T clothing for her before we do her older brother.

The Big Boy Update:  Aunt Rebecca came over today.   My son wanted to tell her about all the friends in his class by pointing to the pictures of them on the refrigerator.   He wanted to point out all the ones he knew in his sister’s class too.   He even told Aunt Rebecca which ones of the girls he liked (all of them.)

The Tiny Girl Chronicles:  My daughter wasn’t pleased with what I served her from the salad buffet tonight so she decided to take over my salad and eat it.   After a while, she swapped back plates, asking if she could have some mac-n-cheese.   While she was eating her newly delivered bowl of food she noticed several leaves of salad she’d spilled on the table.   She picked up a leaf and said, “hey, here’s a piece of your salad, mom” and handed it back to me.   And while this might not be remarkable in general, it wasn’t a large piece of salad she saw.    She didn’t fumble to find it and she easily put it into my hand.

Rental is Not The Same Thing is Retinal

I have been in the land of eyes for what feels like months now but has been, in fact, less than a month.  How can it be that thirty days ago everything seemed to be fine with my daughter’s eyes and now we’re dealing with a possibly year-long treatment for a not-definitively diagnosed condition that I can explain in detail including lots of medical terms I would have been thrilled to never have known about?   I was at our Back to School Social last night and I saw a piece of paper on the table during set up and did a double take because the sheet was about retinas.   What was this?   Oh, never mind it was the “Rental” order.

We have dived deep as a family into a some cold, cold medical water and we’re doing what we can to swim back to the top, climb up to the surface and get back on our beach towels so we can return to basking in the sun.    I have folders of pictures.   I have documents of notes.   I created a Google Drive folder into which I’ve put medical test results since her medical records are confidential.   I’ve emailed, texted—good grief, have I texted—I’ve been on the phone and I’ve had conversations with so many people I lost count.

I had a list for a while with everyone who had contacted me about my daughter.   They wanted to know how she was doing or what the results of the tests were or if she was better.   I told people I’d let them know and I knew I was going to leave people out if I didn’t keep track of them.    Eventually, I got overwhelmed and the list is now incomplete by a good measure.   The good news is that by two weeks or so into things, people were catching other people up on the situation.

Then there were the conversations.   Everyone wanted to know what was happening, and if there were other people present, such as any time I was at school when other parents would overhear, they would be alarmed (as would I had I heard about a friend’s child) and I’d start the story all over again. I am pretty good at this point at giving a succinct start-to-now summary of the situation I think it’s fair to say.

Soon, I hope to be more focused on “rental” things, having put all this “retinal” stuff behind us.

The Big Boy Update:  We went to see Nana and Papa today.   As we called to tell them we were on the road this morning, my son yelled out from the back seat, “Nana, put out the tea set!”   I asked later and Nana told me during Labor Day, when my daughter and I couldn’t make it up to see them because she was in the hospital, they didn’t put out the tea set to play with because she wasn’t there.   He knew she liked playing with it and wanted to make sure it was out for her when we arrived.

The Tiny Girl Chronicles:  We were walking in a park this afternoon on a path in the woods.   I said I could smell the pine trees.   My daughter said, “I can smell the bricks…and I can smell the sun!”

The One-Four Punch

My daughter’s eye problems are being addressed from multiple directions.   I was thinking last night that instead of a one-two punch, we’re hitting it in for directions instead of one.   There are the steroid drops she gets four times each day.   She has the steroid oral medication she takes once per day, yesterday she received a steroid IV treatment and she also had an immunosuppressant dose of medication that should last a month.  

That’s four medications specifically to address inflammation in her eyes.   I really think she is on the mend.   It’s hard to tell exactly how her vision is changing because she doesn’t like to let you know.   We played the, “Road Signs” game today in which we let each other know when you see a road sign. I am TERRIBLE at this game (by design) so I can figure out what she’s seeing.    I think she can see a bit more.

She can also see some things mid-range and near-range she couldn’t see well before.   She cut up paper into small bits and was able to clean it all up and throw it away.   She recognized a letter on a die.   She also was able to discern some animal silhouettes she wasn’t able to two days ago.   But those measurements are a bit relative.   For instance, if I told you she could see the letter, “R” on a die, you’d likely guess she should have no trouble discerning the black animal silhouette shape on a legal-sized piece of paper from four feet away.  And yet she’s not that good from that visual angle.

She seems like she’s able to use her vision more now to gain information from her environment.   I hope so.   I hope tomorrow brings more evidence of better vision.

The Big Boy Update:   We went to a birthday party today for one of my children’s best friends, Keira, who lives two houses away.   The party was a lot of fun, and the cake was delicious.  I noticed as I watched my son that he makes friends quickly.   There were multiple children he just started talking to and shortly later, I noticed they were including him in their conversations, even finding him across the room to tell him to come over and see what the hermit crabs were doing.

The Tiny Girl Chronicles:  I have told my daughter for over a year now that I would help her learn how to “pump” but that I would not push her in the swing.   She has recently had a lot of pushing from relatives visiting and was angry at me today when I told her, again, that I would help teach her how to pump.   She said angrily, “I know how!” and then proceeded to show me exactly how she knew how.   I didn’t say anything until she was swinging quite high, on her own power, and then I said, “now you can swing as high as you want, any time.”    I went inside and had my husband look out the window to see her still pumping away

Fitness Update:  Eight miles of running this morning.   I think the last days of warm mornings may be coming soon, but for now, they’re welcomed.

I Swear, It’s Better

I can’t promise you my daughter’s vision is better, because she won’t tell us.   I’m not sure at three-and-a-half she can even evaluate what the difference is in her vision from day to day or hour to hour, but from observations, I think she can see better.  

We had her first Remicade (Infliximab) infusion today, which is an immunosuppressant.   She had a bonus dose of Solu Medrol steroids.   Tonight she’ll have prednisone orally and then she’s getting those prednisoLONE drops in her eyes four times per day.    We are not missing steroids on any angle.   This has just got to work.

So how is her vision getting better?   On the one hand, she’s dilated twice a day, which prevents her from focusing well.  But she does seem to be seeing blobs more clearly now.   She noticed several things from a distance I don’t think she was able to do two days ago.   Tonight she knew what letters were on small dice and could see small pieces of paper she had cut up on the floor when she went to clean up.

She even was interested in television.   She stood in the kitchen and looked over tat the TV and I thought she had distance vision for a minute but then I realized she was eating yogurt and was following the rule of food stays in the kitchen.   She was standing right at the edge of the room and looking towards the TV.  

So I think we have progress.   I’m also holding my breath.   But I’m getting excited.

The Big Boy Update:  Have I mentioned my son loves clothes?  He likes to pick out what he wears and he has very particular opinions of what he does and does not want to wear.  What he picks out, he looks good in.  He knows he looks great in camoflaudgye, orange and blue.   He is also good at matching outfits.    He is my little Style Captain.

The Tiny Girl Chronicles:  My daughter really, really hates getting an IV placed.   (Who doesn’t?)   Today was quite traumatic to her until the IV was in and the medication flow was started.   Because we’re going to be doing this every month for some time, they gave her a “medical kit” including a plain cloth doll that she can color and mark veins on the arms.   The kit includes everything they use to place, flush, clean and remove the IV excepting the needle.    She loves the kit, practicing while we were there for four hours today and then showing Uncle Jonathan, dad and me how it worked when we got home.

Rheumatology

I hope I spelled that right. Rheumatology is quite the assortment of letters my mind hasn’t figured out how to type in the proper order yet. So let me do an update of where we are now from where I was two nights ago when I was fearing the worst.

Yesterday we did two things, first we went to the pediatric eye center at Duke and were seen by one of my daughter’s eye doctors.   We then ran across campus, and yes, we ran, and it was fun.   Okay, stop, I have to tell you about the running because it’s a quaint little story.

We’re at the Duke Hospital campus a lot.   There is the Duke Eye Center there, the main hospital and the Duke Children’s Hospital all in close proximity to each other.   There is also a huge parking garage with an underground tunnel that connects into the main hub of all this exciting hospital stuff.   My daughter has decided that there is nothing better than running through that long tunnel.   She loves looking back at the blob that I am in her vision and seeing if I’m farther away or getting closer.   She laughs out loud in that little tinkly girl laugh as she runs and looks back, runs and looks back.    And here’s the thing about that laugh and her run: it makes people smile.

My daughter makes people smile in a way I’ve never seen before.   She is in a place where people have pain and sadness.   They’re tired or scared or overburdened with medical news or they’re just plain done for the day after working a thirty-six hour shift.   The last thing on people’s minds as they go to and from the parking lot is running and laughing gleefully.

When I figured this out, I started running after her.   I would look exhausted and laugh back.   Adults would make comments to me and smile and I would make comments back about doing research on how to bottle youthful mirth and energy.   I didn’t stop her running, because I was also seeing if she could navigate around people and obstacles (which she can.)   And now it’s our thing.   We can get from one place to another place in the hospital in half the time, with five times the smiles, all because of a little girl’s running.

So where was I, ah yes, Rheumatology.   We arrived and I spent a very pleasant thirty minutes talking to one of the two leading doctors working on my daughter’s case from the rheumatology side.   Here is what they have decided she may (or may not) have and what the course of treatment will be:

She fits in part, but not well, some of the conditions of the following:

• Behcet’s Disease:  fits some of the eye symptoms, no incidence of oral or vaginal sores
• Sarcoidosis: fits some of the eye symptoms, does have light hair and skin but dermatology did not think there were any connecting dermatological findings to match
• Vogt-Koyanagi-Harada Syndrome: fit more of the eye symptoms and onset but doesn’t meet all the criteria
• Retinal Vasculitis:  idiopathic diagnosis

The good news is it doesn’t matter which or what she has, there is one treatment that’s been in use for  twenty years that has high efficacy for all of them:  Remicade (Infliximab).   Starting tomorrow (pending the healthcare approval has gone through, which I think it has) she will have a monthly IV infusion of this medication.  She’s going to be on the prednisone for a bit longer while we taper it off and some steroid drops for her eyes as well, but hopefully this will help get the inflammation gone and give the immune system time to reset.   There is a good track record of patients with similar situations gaining most or all of their prior vision back, although it’s going to take several months to see where we will ultimately be. 

The above diagnosis options don’t necessarily mean she has a condition for the remainder of her life, this may be a single event and after her eyes have healed and her immune system has had a chance to “reset” she’ll be tapered off the medications.   We’re not sure how long that will be, but it will likely be at least six months.  

So we have a plan.   Tomorrow we put it in action.   I’m already planning my blog posts with titles about all the amazingly small things she can see from a distance.

The Big Boy Update:   My son came home with a collection of little papers on which he’d done drawings.  My husband asked him what they were.   His description of them, in order was as follows: 1) “Greyson”  2) “Sidney”  3)  “Greyson and Sidney: Sidney is the one with the hair.”  4) “Greyson and Sidney behind each other” 5) “X’s” (there are a collection of X’s on the page.)  6) “I don’t know.”  (There is a suspiciously heart-shaped drawing in the center of the page.)   Any guesses who my son is in love with at school right now?

The Tiny Girl Chronicles:  My daughter is getting better and better at the eye drops.   She’s wanted an ice pack for here eyes (a psychological comfort item) since the beginning.   But I’ve noticed she’s used it for shorter and shorter period lately.   Tonight she said to me, “mom, I don’t need the ice pack anymore.”

The Work-In

First, can my daughter see better today than she could last night?  Well, that’s an interesting question. This morning I would have told you an unqualified no, but this afternoon I was surprised at what I saw.    My retina surgeon neighbor had suggested having her tested at distance with the eye doctor charts (pictures for children) and see what she would test at from six to eight feet away.    This morning I grabbed some animal silhouettes off the internet and printed them out full sheet.    Before going to school, I asked my daughter if she could tell me what they were.   I got through three of them.

She couldn’t tell me from eight feet away all the way down to one foot away that it was a huge giraffe on the page.   She didn’t know if the big butterfly was a bird or an airplane, and the only reason she guessed bird was because I asked her, “do you know what animal this is?”   When I got to the third sheet she flatly refused to answer and was irascible all the way to the car.   And yeah, I was depressed.

I did what any lady would do at that point: I went to get my hair done.   Well, I had the appointment and since I now know I have grey hairs, there’s no reason I should tell people about it.  You all won’t tell, right?   On the way to the appointment I got a text message from my daughter’s doctor.   Could I bring her over to Duke early, before the Rheumatology appointment to have her ocular pressure checked by her associate?    Why yes, yes I could; I could totally do that.   “THANK YOU!” I texted her back.

So with freshly-colored hair, I pick my daughter up and we head to the eye center to meet Dr. Kim.   We weren’t supposed to have an appointment.   She was just going to sneak my daughter in, do a quick pressure check and we would presumably sneak out the back door or something.    I got there though and there was some confusion at the front because they have a system and the system was confusing them what with this whole ad-hoc non-appointment thing.   Dr. Kim came around from the back and was talking to them.   I came up and said, “is this about a co-pay?  If it makes it easier for me to pay a co-pay and this becomes an official appointment, then that’s fine with me.”   The co-pay was reasonable and, as I explained it to them, they were helping us so much be working us in that I felt a co-pay was the least I could do.

Then, as the lady beside me complained about extended wait times while I simultaneously read a sign about first visits taking two- to four-hours to complete, Dr. Kim came back and asked for us to come back.

My daughter does this thing now at doctors offices where she acts like a frog and hides under chairs, folding herself into the fetal position.    But Dr. Kim was friendly and she got what she needed out of her without any forcing necessary.   The first thing to note is that her ocular pressure isn’t high.   It’s right there in the happy normal range, even a bit low, which is fine.   The second thing is that Dr. Kim thought she saw a little bit of droopiness in the retina of the right eye.   That sounds dreadful, but it could be an indication the fluid behind the retina has drained some, which is exactly what we want to know.

About her vision though, while we were in the room and I was explaining how she couldn’t see much at all, we observed her doing things that showed she could see some things.   She dropped a piece of granola bar and could find that, but she didn’t see the smaller pieces.   Then, I asked her if she could find the whales in the room.   And this is where I heard the happiest sound of the day.  My daughter looked around in a not-so-well lit room and from about six feet away from one of the walls, gasped.   She gasped because she saw the whales.   She ran over and looked (with her nose against the wall) to find crabs, fish and even a grouping of three, “Memo’s” (Nemo from Finding Nemo,)

We went to another room that had a magnetic Tic Tac Toe game on the wall.   She was able to work the game again and again with the magnet, even organizing the X’s and O’s in a nice pattern along the edge.   I don’t know if she could have done that last night.

So are things getting better?   I hope they are.   It is possible the fluid behind her eyes is also moving around and this morning because she’d been asleep all night, it had moved in such a way that her vision was worsened.

We left the appointment (which was FAR more than a quick pressure check) and headed off to our first Rheumatology appointment, which is the topic of tomorrow’s blog post.

The Big Boy Update:  My son is in love.   Well, he’s in love again.   This time it’s with a friend at school my children have insisted is named, “Cin-ney.”   I have tried to explain her name is “Sidney” but they will hear nothing of it.

The Tiny Girl Chronicles:  I picked up my daughter today before she was done eating her lunch at school.   She was very happy to have found baby grapes packed for her.   The petit Black Corinth grapes are so much fun to eat.   She insisted on holding them in her hands like a baby bird until she got into her car seat and could eat them one by one.   We packed more for lunch tomorrow.

And So I’m Scared

I’m going to cut this short, mostly because I’m tired and would really, really like to have a drink right now only it’s late and I think I’m more tired than thirsty.  Anyway, I had a vision scare with my daughter tonight.   I had been in email contact with her doctor and wanted to get back to her with some more specific information on what she can see and what she just can’t.    The result of my little test—which involved jelly beans—was that my daughter can see almost nothing in the right eye.   So yeah, I panicked.  

I lost it.  I couldn’t keep it together.   I called my neighbor and asked for advice.   She told me it was time to call the doctor directly.   I complained, saying I didn’t want to use her personal phone number in the evening and I should go through normal channels.   My neighbor said this was one of those times to not give a fuck and make the call.   If you haven’t read The Subtle Art of Not Giving a Fuck, now is the time to take sidetrack and do so.  

She was right.  I made the call.   I left my daughter’s doctor a message and I also called my neighbor who is a retina surgeon and specialist and ask her for some advice too.   They both called me back and spent lots of time talking through the current state of my daughter’s eyes.  Or, more to the point, what we think the state of her eyes are; it’s hard to tell when she doesn’t want to tell us anything, even with jelly beans.

Dr. Grace is going to look into things tomorrow.   My neighbor suggested we change one of the medications to a different variety.    Several other people are looking into connections they have in the field of vision, children, retinas, inflammation and/or anything else that might be germane to what’s happening now.

The one thing that’s calmed me somewhat is my neighbor’s advice about how the atropine is affecting my daughter’s eyes.  She said don’t test her vision up close, because the drops aren’t allowing her to focus within that range.   Ask questions at six to eight feet away and see what we find out that way.

Hopefully the behavior we’re seeing now, which is my daughter not using the right eye at all (which I found out was more detached) may be okay.    It’s just not the improvement we were all hoping to see.

The Big Boy Update:  We eat at a sushi restaurant fairly often.   The tables there are glass topped, and underneath are carved scenes in wood.   My son may have noticed them before, but the last time we were there he suddenly became interested in them.   He told us an entire story about what was happening to the people under the glass.

The Tiny Girl Chronicles:  More test results continue to come in.   I think we’re at over twenty-five tests or battery of tests coming back now, all of which show normal or negative results.   And yet we have inflammation and vision problems.   So frustrating.

Fitness Update:  Five miles running.   I almost slept in.   I’m glad I didn’t.

Making The Lunches

My children are eating lunch at school this year, which is something new.   Last year we picked them up at school and had to make a decision if we were going home for lunch or going out.   This year I think without a doubt, I prefer the children having lunches at school.   We have an opportunity to pack a healthy lunch and snack for them each day and they have an enjoyable meal with their friends and teachers.  

Getting the whole lunch making process down has taken a little time to get up to speed on though.   First, what should they take to school?   I’m asking them to be a part of the lunch making process, but that doesn’t mean they get to pack their lunch boxes with candy and ice cream.   Based on some observation and advice from other parents, I purchased zippered lunch boxes for each of the children and then got some small plastic containers of varying sizes to pack the components of their lunch into.

The children are getting good at helping pack the fruit and vegetable section of their lunch boxes.   They get choices (from me) and then decide if they want more grapes or celery or carrots, for instance.   They have a “crunchy” container as I’ve named it that can be packed with things like pretzels, veggie straws, etc.    Then there is their favorite container, the smallest, that fits just about four animal crackers or other dessert item.  

The main container is for a sandwich commonly.   They’ve recently been liking ham and cheese rollups but we’ve sent in sandwich halves, pasta or even left over pizza from meals out.   If the items they pick our are small enough, we can add in a string cheese or a yogurt.    Overall, the goal is to pack the amount of lunch they’ll be hungry for, and no more.   I’ve started getting closer to the right amount of food recently as their lunch boxes are coming home empty.

I enjoy working with them to get their lunches prepared the night before, having them ready in the refrigerator in the morning.   I’ve heard other families put the lunches together in the mornings.   I wouldn’t want the added rush of making a meal in addition to our regular morning meal.   Making lunches at night has been fun for all of us.

The Big Boy Update:  My son got his lip busted open today.   It was a complete accident by his friend who was running up to give him a big hug when his head got in the way of my son’s lip.   It was bleeding a good bit and my son was saying some rather unkind things about Malcolm and how he was never going to play with him again.   His lip was puffed up tonight, but is looking fine.   My son told dad this evening he was not nice in the words he said to Malcolm.   I’m glad he was able to reflect on what happened.

The Tiny Girl Chronicles:  The steroids are having an effect on my daughter.   She has amped up her hunger level and if you don’t bring food in time, boy, you might be in for some words from her.   Her temper is much shorter and her anger level is much more intense.   Other than that, her vision appears to be about the same.   It’s going to be hard to tell until she’s off the dilation drops.

How Often Do You Butch?

Today’s topic isn’t about my daughter’s eyes.   This is largely in part because she seems to be stable or possibly improving in what she can see.   We were at a birthday party today at a farm and she was able to move around people, obstacles and even pumpkins in a pumpkin patch with ease.   It’s almost as if she can see normally.   But then something happens that makes you question that observation.   She was in a group of people and couldn’t find me because she couldn’t hear me talking (I was eating an apple.)   Someone had to walk her over to where I was.   It’s a big mystery what she can and can’t see because she doesn’t want to talk about it and is getting more aware of subtle, vision-based questions we’re asking her.

Now, back to my normally scheduled, never predictable, random blog post topic for the day…

I was in the kitchen the other day with some clementine oranges that had gone past their prime.   They weren’t peeling well and I didn’t want to send them to school with the children.   They did though have plenty of juice in them if squeezed, I thought.    I got out a cutting mat and looked at the big block of knives, trying to decide which one to use to cut the oranges with.

I pulled out several and then decided on the butcher’s knife.   I decided on that knife for no good reason other than I didn’t think the knife ever got used and it was high time it was put to use for something.    My husband walked in as I was trying to balance a little orange against a huge knife.   He heard me say, “this knife really isn’t good for this job.”  He tried to be helpful, explaining which knife I should have used (he does know his knives) to which I called out, “I know which knife to use, I just wanted to get some sort of use out of this butcher knife.”   He said something about using it and I said, “how often do you butch?”  to which he replied “oh, only about once a year.”

The moral of the story is that butcher’s knives are terrible at cutting up small oranges.   The other moral to the story is we didn’t need to get a butchers knife, seeing as we rarely butch in our house.

The Big Boy Update:  My son was playing in the hay barn at a farm birthday party today.   He was having a tremendous amount of fun with some of his classmates from school who were also at the party.   He cried out, “I am the master of hay!”   Grandpa tried to get him to leave to try some of the other activities at the farm but he was having none of it, saying, “I don’t want to leave; I’m burning a lot of calories.”

The Tiny Girl Chronicles:  My daughter was talking about her birthday party coming up in November.   We asked her where she’d like to have her party.   She told us, “somewhere up North.”

Fitness Update:  In preparation for the marathon in November we ran twenty miles today.   We talked about it at the end and think we could have done the 26.2 miles if we had more hydration and some Gu packets.   I’m a bit tired now, after spending a lot of the day at an outdoor birthday party at a farm.   I’ll sleep well tonight.

Back to Blue?

I don’t have much new to report about my daughter’s eyes today other than to say if you were around her and didn’t know she was visually impaired right now, you wouldn’t know.   She got her helmet on by herself, pulled her balance bike out of the driveway and then biked up and down the street, stopping in time, just in front of us as she biked past.   She did this again and again, and even I wondered if she had her full visual capabilities she was so aware of her surroundings.    

But one question about what you’re holding in your hand or can she tell you where the dog is in the yard and you realize very quickly she has no ability to discern details eighteen inches away.   Her ability to navigate in her environment and accommodate to her vision changes has been impressive.   She knows she can’t see well, but she’s working through it. 

I’ve talked about the future for her vision and how there is no indication that as of today we can’t get her vision back.   However, I have been warned about one thing, and that is the appearance of her eyes.   When we noticed a change in her pupils two-and-a-half weeks ago, it was the start of some significant changes in the appearance of her eyes. 

Today, her pupils are not round.   The left eye pupil is not completely dilated, even with the dilation drops.   Her right eye is almost completely dilated, but what we can see isn’t round.    The other change is the color.   Her eyes were a light blue before, now they’re a drab grey with a hint of blue.  

Two eye doctors have told us the least of their concerns is the visual appearance of her eyes and that her visual ability is what they’re most concerned about.   We agree completely.   But, they’ve warned us, what has happened has caused scaring in her eyes and we may need to be prepared for her eyes to never be blue again and her pupils may not return to circular.   They may not fully dilate either. 

It’s so hard to tell right now because of the dilation drops and the irritation in her eyes due to the drops and injections they’ve given her.   Her eyes have also been irritated in part from the retractors used to evaluate her eyes.    She’s not been bothered by any of it, which has been a good thing. 

The Big Boy Update:  My son told us the other day (out of the blue) that he wanted for his birthday a chocolate warrior cake.   His birthday is in December.   It would seem he’s planning ahead.

The Tiny Girl Chronicles:  My daughter has decided when you’re in a large, open lobby in a building you need to be quiet.   She will shush you. telling you to be quiet.   I wonder if it’s because she’s more aware of sound and she can’t see the open space, but she can hear it?

Fitness Update:  Five miles running this morning.  That’s it.  Nothing else exciting happening.

Anterior Segment Dysgenesis

Yesterday I wrote about the current situation with my daughter’s eyes and the state of her vision.   I realized when I went to bed that I’d forgotten one.   To explain, let me go back in time.   When my daughter had her three-year-old wellness checkup she failed her eye exam.   We weren’t particularly concerned because she wasn’t that interested in looking at the pictures on the wall or answering the questions from the nurse.   Later in the year, our school was visited by the Prevent Blindness group from our state.   She failed their test as well, so we decided to have her evaluated by an eye doctor.

It took a few months to get in to see the highly recommended doctor we selected, but we weren’t overly concerned.   Yes, my daughter seemed to like to sit close to things, but she had no issues seeing.

When we had our appointment, Dr. Grace had a challenging time seeing what was happening in my daughter’s eyes.  She suggested an evaluation under anesthesia to learn more about her eyes.   On March 18th of this year we did just that.   I’m very glad we did, because it showed her eyes were normal with two exceptions and definitely didn’t show any indication of the issues she’s having today.   The first is that she has somewhat smaller eyes.    You know how these things go though, there’s a spectrum of sizes and my daughter is on the smaller side although her eyes look normal visually.   The name for this is Micropthalmia.   There are increased risks, but it’s not a big factor in anything she has today from what we’ve been told.

The second thing they discovered she has is Anterior Segment Dysgenesis.   Fancy term but it can be broken down to mean something that happened during her development in the front of her eye.   That term is broad though, and can mean lots of things didn’t go just right in the front of her eyes.   In our daughter’s case, some vessels are present across her field of vision.   Here’s a picture of one of her eyes from the March 18th evaluation.  The eye looks a little strange, but that’s just the angle, it’s all normal.   The thing you want to look for is the curtain of vessels inside the iris on the lower half of the eye.   Both eyes have this.

What does this mean?   It means we don’t quite know how she sees the world.   She was seeing 20/20 up close and 20/50 on March 18th, so it wasn’t impeding on her vision too much.

Our hope is we can get her vision back to what it was on March 18th.   So far there are no indications we can’t, but there is a lot of healing and reduction in inflammation that needs to happen in order to get there.

The Big Boy Update:  We have never explained to my son that someone is “Indian” or “black.”   We have a lot of diversity in our neighborhood and school.   Yesterday my son was telling me how he had lunch with Keira.  I told him Keira didn’t go to his school, but he persisted.  Finally he said, “the DARK Keira mom!”   Then I realized, we have a new African American family this year that has a daughter named Keira.   His description was just what I needed to know.

The Tiny Girl Chronicles:  We went to another doctor’s appointment today and had to use multiple elevators to get there.   My daughter suddenly exclaimed, “that’s the elevator’s mouth!” as one of them closed.   She was excited about the rest of the elevators during the day, telling me as their “mouths” opened and closed.  

Fitness Update:  I ran to school to pick up the children today.   It was a nice six mile run through the park, on the greenway, through the art museum property and then over the highway on the pedestrian bridge to get to school.  I met Nana in the car in pickup line to get the children just in time.

The Cascading Eye Effect

Tonight’s post is an attempt to explain the series of events that have led my daughter to her current state of ocular affairs—otherwise known as “she can’t see crap.”   And just so we’re all on the same page here, the only way I’m dealing with this without becoming a completely depressive, blubbering mess is to treat it with humor, positive attitude and an immense amount of thankfulness and gratitude to those around me who are helping my daughter, and by extension my husband and me in our understanding of what’s going on.

So now, based on a commitment in last night’s blog post when I was too tired to finish writing up my thoughts, I committed to explaining what’s going on in my daughter’s eyes.   I thought I knew last week, but I was wrong (well, many of us were at that point.)   My understanding has evolved now, but I have the most menial sense of confidence that I’m getting it all right.   So if I spell medical words wrong or put components of the eye in an entirely different area of the body, it’s me getting up to speed on the whole medical eye realm.

The prevailing thought in what’s happening to my daughter’s eyes is that everything started with inflammation.   The cause for that inflammation is the problem no one’s been able to solve.   Rheumatology is driving her case at this point, but as test after test come back normal or negative for inflammatory and autoimmune causes, we’re still at a big zero on the inciting event/cause front.

Inflammation can be a bitch.   Here’s what the doctors speculate happened (but not necessarily in this order):
1) Inflammation —> low blood flow —> aberrant vessels growth anterior to the eye

• Some inciting event or agent caused the vessels in the eye to be inflamed. 
• The inflammation constricted or restricted blood flow to the eyes.
• The nerves in the eyes sent messages to have new vessel growth to combat the low perfusion
• New blood vessels are grown in the anterior portion of the eyes, near and around the drainage region

2) Inflammation —> low blood flow —> serous retinal detachment

• Inflammation and lack of blood flow cause fluid to build up behind the retina, causing serous retinal detachment

3) Retinal Detachment —> aberrant vessel growth anterior to the eye

• Low blood flow causes retinal detachment which in turn sends messages to grow additional vessels in the eye

I said in last night’s post I was going to put up a picture of an eye and point to things with my virtual finger, letting you know where all these things were happening.   Here’s a diagram of an eye:  

The fluid buildup is behind the very large “retina” which covers the right two-thirds of this picture.   Is the fluid in only one place?  Is it moving around as my daughter changes positions?   We’re not altogether sure.   The fluid buildup from a serous retinal detachment drains to the Choriod layer, but for some reason isn’t being pumped out like it normally is.   The current suspect: inflammation.  

The aberrant vessels have grown not in the back of the eye, but in the anterior portion of the eye, close to the drainage region which is a three-dimensional area close to the Schlemm’s canal.   This isn’t one or two or three vessels, but a large network of itty bitty, teeny tiny blood vessels she doesn’t need.   Hopefully the Avastin they injected into her eyes yesterday to combat the growth of the vessels will be helpful.

So for now, inflammation is the enemy.   We will know more in two weeks.   Hopefully prior to that the fluid will reduce some behind her retinas, but to be fair, healing from this type of retinal detachment commonly takes weeks or longer to recover from.

The Big Boy Update:  My son wore his rain boots to school today.  He insisted on doing so because our weather station (which is notoriously inaccurate) said rain.    For dinner in the eighty-four degree weather, he wore his rain boots again and this time wore his warm rain jacket.   He has utmost faith in our weather station it would appear.  Funny enough, a brief bit of rain happened while we were at dinner, although it was so quick, we only knew because cars were wet when we left the restaurant.

The Tiny Girl Chronicles:  My daughter decided to wear her rain boots to school.   Her old ones were too small so I had her pick some new ones out online.   She wore them today and told me when she got home about her friends who told her they liked her new boots.   My son told us that, “everyone likes her new rain boots.”

Fitness Update:  I went to the fitness room today and tried out just doing some free weights, being mindful of form and careful of injury.   I have no aspirations for big muscles, only toned ones.  Therefore, I don’t mind one bit if I use the lowest weights on the rack, as long as they work for me.

No Bad News Is Good News

Today was a long day at the hospital for a procedure with my daughter in which she was sedated so a team of doctors could look at her eyes.    At the end of the day my daughter finally got to eat, which was the best part of the whole experience for her because she was so hungry and for me because she had been talking about food for over five hours.   She literally crammed as much bread as she could get into her mouth and only after doing so did she realize she couldn’t chew or swallow without removing some.    Anesthesia will do that to you though.  

As an aside here, I can’t begin to thank those of you who have contacted us with ideas and references.   Of particular note, through a neighbor, I found out we have a retina surgeon and specialist just a few streets over.   She heard about my daughter’s case and called me on Labor Day evening to talk to me, ask questions, give advice and most importantly, give us some hope.   The outpouring of support has been tremendous from so many people.  Thanks to everyone.

But I gush.   Let’s get back on topic.    The title of this blog post tells it all.   We don’t have any more bad news, and in fact, there is some good news.   First, we weren’t expecting repaired vision today.   We had been told it would take time and if they saw some improvement today, that would be a good sign.    And that is exactly what they saw: small signs of improvement.   It’s enough of a change that they believe treating with steroids to reduce the inflammation is the correct treatment unless something is uncovered in some of the more complicated tests which have yet to come back.

We’ll be treating with oral steroids, topical steroid drops and dilation drops for the next two weeks and then she’ll be evaluated again.   The lead doctor on the case today said, “we’re not out of danger yet, but this is a move in the right direction.”

I’m tired and heading off to bed tonight, but tomorrow I plan on putting a spiffy picture of an eye up here and talking about how some of the things I thought were going on were close, but not exactly right and how the four issues my daughter is having in her eyes are likely related to one cause.    Teaser, I know, right?

The Big Boy Update:  Our house phone line rang today and my son, for some reason, decided to answer it.   When my husband asked about it he said, “yeah, I answered it but I told them I was a kid. The didn’t talk back so I hung up.”

The Tiny Girl Chronicles:  My daughter was recovering from anesthesia this evening and apparently didn’t like us talking around her.   Suddenly she said in a slurred voice, “let’s play the quiet game. 1…2…3”   We got the message she wanted us to be quiet but about ten seconds later we started discussing discharge procedure again.   As soon as we did, my daughter said aloud, “you lose.”

A Normal Day

It seems like it’s been a while since we’ve had a normal day here.   To my son, most of the days have been normal, but then he’s done his normal things such as going to school, playing with friends and trying to sneak a snack from the pantry when he’s not supposed to.    My daughter’s days have been interrupted with three days of hospital stay, which she didn’t particularly want, but didn’t seem to mind that much either.  

We followed up her hospital stay with some out patient visits for IV steroids which was fairly easy in and of itself, only it took a long time to do, so it took a big chunk out of our day.    Other than that though, we’ve been trying to do our normal, boring thing around here.

Tomorrow my daughter goes back to the hospital for an evaluation under anesthesia in which an entire team of not only eye doctors, but eye specialists, each in their own particular field, will look at my daughter’s eyes in the hopes of finding a cause and a solution to what’s been happening to her.

But for today, we had a normal day.   Everyone went to school—including me, I had a committee meeting—and our afternoon was spent with the children running around, playing with their friends in and out of the house.   I baked some bread, this time venturing into something I’ve never done before with a cinnamon and raisin swirl.   I don’t know if its up to Uncle Bob’s baking standard, but I’m sort of a bread baking neophyte here, so it’s all good to me.

Tonight we went to a hibachi dinner restaurant with Uncle Jonathan and before the chef arrived we asked the children what their career plans were, mostly because they were both unhappy they weren’t “buh-dults” as my daughter calls us.   Their responses follow:

The Big Boy Update:  “When I grow up, I want to hunt down chickens.”

The Tiny Girl Chronicles:  “When I grow up, I want to hunt down coupons.”

Fitness Update:  Four miles, mostly walking though as I was on the phone about tomorrow’s procedure for my daughter.

The Intrusion of Sound

This blog usually contains several sections, the first being something or other that’s been on my mind lately.  After that I write a little section on each of my children, commonly documenting something they’ve done or said that’s of note for the day.   Over the past two weeks though, the main thing on my mind has been my daughter’s eyes, so her section has ballooned into the focus of my daily posts.    I think we can all agree, I hope her eyes will go back to their unremarkable selves soon, but for now, she’s front and center…

The Tiny Girl Audio Update:   My daughter has, fairly suddenly, been much more interested in music.   She’s always liked songs and singing, but she’s been intent on both hearing and singing songs of late.  It is not coincidental in my mind that her recent intense interest in songs has coincided with the decline in her vision.  

I’m not saying she’s doing that thing where one sense overcompensates for another, but she has put more thought into hearing things while she’s not able to do much with her vision beyond the range of six inches.  

My daughter’s been asking for specific songs and when I play them she’s been intent on singing loudly along.   When a song is finished she’ll say things like, “I know that song now,” or “I’m going to tell dad I know another song,” or  “I can do that song too.”  It’s been as if she wants to collect songs into her repertoire.  

She’s been getting better at singing songs but she needs help with the lyrics.   I’ve been trying to help her with some of the words, but when I tell her what the lines are, she complains at me because she wants to sing the words (or the sounds) she thinks the singer is singing instead.

The Always Tactful Big Boy Update:   My son was talking to Nana at our neighbor’s Labor Day bar-be-cue this afternoon.   Nana was going to go back to the house to get another drink but changed her mind when my son told her, “no, Nana, you don’t need another drink because you’re starting to get a little fat."

The Future of Vision

My daughter’s sudden vision change has been on everyone’s mind of late.   We’ve all been trying to understand what happened to cause the change, how to stop whatever it is and how to get her back to being able to see normally again.   At this point we don’t have an answer to the “what” part.  Not a single theory has been presented that would explain the reason for her sudden and acute loss of vision.

We also don’t have an answer as to how to stop whatever it is that’s causing the problem but the overwhelming suggestion across all the doctors we’ve been working with is to treat with high doses of IV steroids.  If the cause is inflammatory-based or is some sort of auto-immune response (although neither are confirmed) then steroids should make a significant difference and will help with a plan moving forward.

The last part is how to get her vision back to what it was.   This will be dictated in part by how well the steroids are doing their job.   I had hoped the solution was to get the fluid gone from behind her retinas and her vision would show significant improvement quickly, but that may not be the case.   We’re not panicking, though.   Just like we wouldn’t say, “oh no, she broke her leg, she’ll never be able to walk again!” we’re not thinking, “her retinas are detached, she’ll never have good vision again.”  The point here is that healing takes time.   And just as with a leg break, there is a chance the leg won’t be like it originally was before the break; with her eyes, it’s a similar situation—we’re not sure what her final visual outcome will be yet.

So, let’s focus on the ocular issues she has right now and look at how healing needs to proceed in order to get her vision back to where it was:  Initially, we thought the beginning of the visual symptoms my husband saw (pupils not being round) started everything off.   We know a little more now and will know even more after this week’s upcoming re-evaluation of her eyes, but some of the problem has been going on for longer than two weeks.

What the eye doctors saw—we had three specialists look at her eyes on Wednesday—was low blood flow to her eyes.   Were her vessels extra small to start?   We found out her eyes are slightly smaller than normal, but does that factor in at all?   She has vasculitis in the blood vessels feeding into the retina which has caused a thickening of the vessel walls and inflammation.  The body’s response to vasculitis is to grow more blood vessels, only the kind of vessels that grow are undesirable.   They’re commonly in the wrong place and may even leak.    My daughter has some of these extra blood vessels, which means the situation isn’t brand new as it takes time to grow vessels.

So the question is, what is the cause of the lack of blood flow to the eyes?   Is it an inflammatory issue?   Can it be corrected?     The blood flow issue is the one the doctors are looking for a cause and solution for, because without good blood flow to her eyes, her vision won’t improve and may continue to worsen.

The second issue, which is the primary cause of her sudden vision change, is the bilateral retinal detachment. Within a very short time (hours? days?) both retinas detached.   This is rare and doesn’t happen without other diseases, conditions or trauma.   The type of retinal detachment she has is Exudative Serous Retinal Detachment which means fluid from the eye flows into the space between the retinal layer and the layer beneath it.    This is uncommon to happen in adults and almost unheard of in children.

It’s also not “fixable” in the standard way you would fix a torn retina with reattachment surgery.   The first thing that needs to be done is for the fluid between the layers to go away.   At that time we can look at the process for addressing the detachment.   We’re going to see a retina specialist (or possibly two) on Wednesday who will have more information for us.   I don’t know what to expect with a child with an uncommon eye condition as far as healing and/or repair goes.

But what about her vision?   What can she see?   I can tell you this, she is not blind in the extreme sense of the word.   She sees things and can discern items quite well at very close range (from two to five inches.)   Today while we were at the hospital we played a game with flash cards.    She could tell what about two-thirds of the cards were.   Of the other third, some of them I’m not sure she would have guessed with her original sight, but some of them she definitely would have know, like the flashlight on the top of the left stack (the ones she said, ‘I don’t know’ to):

She can navigate around a room or outside with ease.   She doesn’t miss stairs and can interact with you easily.    But she is not able to see more than big blobs for a lot of her world right now.   Those flash cards at five inches she can discern, but at eighteen inches, she’s not sure where the card is.   When you put something out for her to take in your palm, she has to hunt to find it.    There are about fifteen more things I could write here  I’ve observed that have almost made me cry to see happen, but I know her vision situation isn’t permanent in its current state.  

So what is the future of her vision?   I can’t tell you.   We feel very confident that it will be dramatically better than it is today.   We’re not completely sure it will be back to one-hundred percent of what it was.   There is also a chance for a worse outcome, but there’s no evidence to suggest we need to be considering that as a possibility today, so we’re not, dammit. 

The Big Boy Update:  Nana brought her cap guns to town.   There are no projectiles, just a popping sound as the hammer strikes a chemical dot on a roll of paper.   My son *loves* playing with these.    My dog and my daughter, on the other hand, don’t like the sound.

The Tiny Girl Chronicles:  “Is that the flushing toilet?”   My daughter has an IV in her arm that’s been there for several days.   She has gotten familiar with some of the medical terminology being used around her.   Before they give her any medication or hook up to her IV line they do a “flush” by pushing water through the line first to make sure it’s still working.    She asked today if it was the flushing toilet.    She doesn’t like the line flushing part and after the flush was done I heard her mumbling under her breath, “bad toilet, bad toilet.”

Lets Go Ride a Bike

If you couldn’t tell, the title of this post was to be sung to the tune of, “Let’s Go Fly a Kite.”   I’ll wait  here while you go back to the beginning so you can have appropriate musical accompaniment to this post.  

Okay, now that we’re on the same page, let’s talk about where we are with my daughter and her eye condition.  She’s home and she’s very happy about that.   She and I were alone last night because my husband went back to Pinehurst to continue playing in the member/guest golf tournament with my father-in-law.     We’re trying to have our holiday weekend as close to normal as possible and there was really no reason for my husband to sit around with me in a tiny hospital room when he could take my son and our dog with him and they could enjoy being together and doing normal things.

Today, my mother-in-law, son and dog drove here in the morning.   My husband and father-in-law came to town after playing golf and we’re all enjoying the end of the day together.    This morning, my daughter got up, came to see me and then told me what she wanted for breakfast:  Poptarts, which we save for special occasions.

I told her we were out of the strawberry.   I asked her if she knew where the chocolate ones were and she said yes and then she left to go get her breakfast.  I went into the kitchen a few minutes later to find she had gotten the stool out and gotten the chocolate pop tart pouch (avoiding the other flavor.)  She had put the tarts at her seat.  When I came in, she was standing on the counter with the cabinet door open, getting the plate to put the Poptarts on.

She ate breakfast and then wanted to play with our neighbors.  I texted them while she went outside by herself and colored with chalk for a while.   Soon enough, Keira came over and they played for some time while I went to the grocery store to get groceries.   (They went to my neighbors house to do an art project and my neighbor was watching her.)

When I got home, my daughter and I went to the hospital for an outpatient IV steroid infusion.   This took a long time and she was unhappy, angry and difficult to deal with.   She is in pain from the lumbar puncture and when she gets stressed she yells that anything hurts her body.   This isn’t because it’s that bad, it’s because the steroids are at such a high dose, they’re putting her on edge, making her a little easier to agitate and making her a far more intense person to be around.    We know it’s medicine though and it’s not for long so we’ve been doing the best we can.

When we got home Nana, my son and dog were here.   My daughter went out to play with my son and neighbor again and came back in a while later.   Then, she told me she wanted to ride her bike.    I told her I’d be glad to ride with her.   We got her helmet on and then she told me about the seat issue she’d been having with on her balance bike.   When I understood the seat was rotating, we got a hex wrench and fixed the problem.    Then she and I went out on our bikes and began riding up and down on the road.

But what about her vision, you’re asking me?   The thing is we don’t know what it is now, if it’s changing constantly with the fluid build-up behind her retina and how it’s different from what the rest of us see.   She doesn’t want to talk about it so we have to infer from things she says and does.   For instance, on the way to the hospital today she said, “look mom, there’s a rain cloud up ahead.”   She was right, there was.   Then, she told me she could see road signs.    This was interesting, because road signs are generally not that interesting to a child, but she wanted to tell me she saw them and what colors they were.   I got the impression she wasn’t seeing them clearly, but that she knew they were there from how they were differentiated from the surrounding greenery.

At the hospital there were board games.   She played Candy Land with her own rules.  She couldn’t find the path on the board to move her piece without help.   She wanted very badly to move to the special spots like the candy cane or gingerbread space, but she couldn’t find them so she gave up.  Later, we did a puzzle together and she had more success, but the patterns were bigger and the lines were bolder.

Tonight, she asked to watch a television show, which she hasn’t been doing much since her vision issues started.   We pulled over the tall, bar stool close to the TV above the fireplace and started a show.   She asked my husband to move her back a little.   We didn’t know if that meant there was a specific focal length she can see more in than others.

While she was biking, my husband came out and was standing in our neighbor’s driveway.   My daughter and I were fairly far away at about three houses.   I asked her if she saw someone at Bryna’s house ahead.   She couldn’t see him.   As we got closer she saw someone and thought maybe it was Bryna.   When she got right up on my husband at about two feet away, she said, “hey daddy.”

There are lots of other examples I can give, but it’s all confusing with what she can and can’t see.   We know the healing process won’t be immediate, but we want to see results now; stat.  

The Big Boy Update:  My son understands his sister is going to the hospital and it’s with her eyes.   He can even talk about it.   He doesn’t seem worried about her and is sort of more matter of fact about it.   I suppose it’s because he’s a child and doesn’t have the weight of knowledge on his shoulders that we do.

The Tiny Girl Chronicles:  My daughter’s vision may be fluctuating (see above for more details) but it’s hard to tell.   We played a game today with Disney characters on cards.   She could immediately identify the characters she knew.   Hopefully her vision at close range will continue to improve.

Home for the Holidays

My daughter was discharged this evening after an unexpected three days stay in the hospital for her sudden and unexplained vision changes.    We were planning on going out of town to spend the Labor Day weekend with my in-laws but now it looks like we’re going to be home for the holidays because my daughter will be returning to the hospital over the remainder of the weekend for an IV steroid infusion each day.     My in-laws have decided to come here instead and knowing them, they’ll bring the party with them.

So where are we since yesterday?  Today my daughter had a lumbar puncture in which pressure was taken and five vials of spinal fluid were collected for testing.   A further seven vials of blood were drawn for other tests.   Other than that, we were waiting around for information to arrive and decisions to be made about the course of action to take for my daughter.

We got results back from all the radiology from yesterday.   The echocardiogram showed my daughter’s heart completely normal.   The angiogram was normal.   The brain and neck MRI showed normal with some findings within normal tolerances and variances.  She also had a hearing test today which was normal (but we could have told you that without a test.)

Doctors from each discipline (genetics, rheumatology, neurology, ophthalmology, cardiology and possibly other specialties they called in) have been getting together to meet, talk on the phone and exchange ideas and information about our daughter.   She has been the hot case, because no one can figure out what has caused her situation.    So what is her situation exactly?

First: she has Exudatative Serous Retinal Detachment.   This kind of detachment is uncommon in general and super uncommon in children.    It’s also not the type of detachment that’s associated with most conditions such as Marfan’s.

Second:  She has inflammation in the eye area.   This is being treated with IV steroids but the question is why does she have the inflammation?   Is the inflammation something systemic that’s only presenting in the eyes at this point?  What if it’s an autoimmune issue?  We don’t know, but without an identified cause, will it come back and will it cause troubles in other places such as her brain?  Bloodworm CRP and ERP show negative on the inflammation area which is good but doesn’t answer why only in the eyes.

Third:  Apparently there has been inadequate blood flow to the back of the eye for longer than just this incident, because new vessels have grown in the area of the retina in order to try and accommodate for the low blood flow.   Could this vasculitis over time contributed to the detachment?

Fourth:  The sudden and dramatic change in both eyes is causing everyone to suspect something that has yet to be identified.   Perhaps the vasculitis could have caused the retinas to detach, but the likelyhood of it doing so on both eyes on the same day to such a degree is highly unlikely.

For now the team is going with the leading thought that there is inflammation behind her eye and retina is causing vasculitis which in turn is causing less blood to flow to the retinas.    But that’s the leading thought—no one actually knows what she has or what caused it.   As the attending physician and lead on her case said today, “no one has what she has.”  It’s being treated with IV steroids over a course of five days and steroid topical drops.

The work isn’t over yet though.  Tests are still being run.   I wanted to get a full list of what was being considered and tested for because many people have asked me “have they though of this?” or “tell them to consider this.”   Hopefully this list below will help out, because I don’t know all the team has considered and are testing for.   Everyone on the team has said they welcome ideas and so do we; I just don’t know enough to tell you if it’s already been ruled out.  

Here are the tests that have been called so far:
- Thyroid:  Normal
- ACE:  Normal
- CSF basic findings (protein. sugar):  Normal
- Lupis panel:  Negative
- CRP & ESR:  Normal
- CBC:  Normal
- ANA (related to Lupis):  Negative
- Urinalysis:  Normal with a little bacteria
- Liver:  Normal

There are a lot of tests still in process.   I got a report of them on discharge today and I don’t feel like typing them in, so here’s a picture:

So we’re on hold for now to see how things improve over the next week.   We’ve been advised to not expect dramatic improvements in my daughter’s vision because a retinal detachment needs time to heal, not unlike any other area of your body.  

The Tiny Girl Chronicles:   My daughter was so happy to get home she didn’t even go inside when my husband drove up.   She asked if she could go play on the swing set in the back yard before it got dark.   My husband said yes and followed her around the house.   I quickly called my neighbor and their children came running out the back of their house to play with her until it was time to go in for bath and bed.

The Big Boy Update:  Me, “what did my son do today while I was in the hospital?”  My husband, “hm…well, he convinced Nana to spoil him because he now has five new toys from the trip to Wal*Mart.”