About Those Folds

Yesterday my daughter’s main eye doctor came to meet us prior to taking her back to the operating room.   He asked suddenly, “you don’t happen to have a USB drive on you, do you?”   My husband pulled out his key chain and handed him one of the two USB drives he keeps there.  One of the two drives I had asked him not a month ago, “why do you keep those on your keychain?  Have you ever used them?”  He said he hadn’t used them but he might need them someday.   I was so glad for his foresight.

The doctor said he was going to download all the images they’d taken on my daughter for us to take to Detroit with us.   They had already sent all her medical records and would follow-up with the operative report when they finished for the day.  

We’ve seen the retinal detachment pictures before, but it never gets less scary.   Here is one from yesterday.   Remember how I had mentioned her retina was like a crumpled sheet or had folds in it like a Shar Pei dog?   Here’s what I mean…

This is zoomed in and there is nice blood flow to the retina itself, so even though it looks pale and grey it’s still doing okay.    It’s no wonder she can’t make anything out trying to discern images being sent to that surface.

The Big Boy Update:  Tae Kwon Do high fives.   My son told me he wanted to high five today like they do in his Tae Kwon Do class.   He explained that you had to try and hit the other person’s hand while they moved their palm away just in time.   We did a lot of intentional missed high fives for the rest of the day.

The Tiny Girl Chronicles:  Tape Monster.   My daughter has this thing about tape.   She likes making drawings or sculptures or, well “things” with small pieces of paper, markers and a LOT of tape.  She has made seesaws with tape and rolled up pieces of paper attached to a base sheet.   It’s pretty impressive from a tape consumption standpoint.   Today she helped wrap a present for her friend, Rayan’s birthday.   Just when I thought she’d finished and was walking out of the room to take the present to him she stopped, turned around and said, “I think it needs one more piece of tape.”

Autoimmune or Mechanical?

Today my daughter had another long day at the hospital.   It’s tough selling these visits to a four-year-old as beneficial, helpful, or anything other than something she hates and wants to avoid, but she does her best and we’re grateful most of the time when she’s wailing it’s when she’s partially sedated and doesn’t remember it.

Overall, I would call today a win.   I would love to tell you we left the hospital elated to hear great news that her eyes are vastly improved and she should be catching flies with chopsticks in no time, but I can’t.  What we did get was some good news and a possible direction towards a diagnosis.    But let me start at the beginning of the day and get to that.

First, I want to say scheduling is no fun.   Over the past several days I’ve spent time talking to four schedulers, sending emails to three doctors and being both frustrated and grateful at the same time for what we were hopeful would happen today.   My daughter needed a brain MRI to confirm there have been no changes in since her September hospitalization scan.  Some white spots had been present, which could be normal, but to rule out anything eye-related, they wanted to do a re-scan.   We wanted that scan before we went to Detroit and we only had one time radiology could do it—this morning first thing.

Then there were our eye doctors.   We have two main doctors who have to coordinate their OR schedules to begin with but this time they wanted to add a third specialist.   The only way they could coordinate this (since different procedures take different times) was to have my daughter as their first cases.

Both of these things needed to happen before we went to Detroit on Sunday and it looked like it wasn’t going to happen.   I can’t tell you what went on behind the scenes but I’m happy to say it did all happen today.   When we got to the OR after my daughter’s MRI we found out that an operating room had been reserved for her since the beginning of the morning.   That OR had sat empty for three hours while they waited for her arrival from radiology.   I can not tell you how grateful we are for everyone at Duke who made today work.

After the procedure we spoke with two of her doctors.   Here’s what we learned:

Remember that Anterior Segment Dysgenesis or Persistent Fetal Vasculation?   It’s those vessels in the front of her eye that brought us to the hospital in March and gave us a “normal” baseline for everything else.   They were causing her to see 20/60 distant vision.   After all this, those little vessels have shrunk to mostly nothing.   If nothing else were happening in her eyes, she’s see fine at distance now.

Her eyes are still significantly detached.   The right eye is detached more than the left and the left eye is about 50% detached.   There is improvement in the detachment percentage overall though, mostly in the peripheral areas.   The macula (main focal point) is detached in both eyes and in quite wrinkly which makes seeing anything of detail very, very hard.  (Which we observe daily.)  The reduction in detachment is largely due to the improvement in the amount of fluid behind her retinas.  In addition, the tractional detachment at the far edges of the retinas appears to be almost resolved.

We have pictures of her eyes in which we can see the “folds” of the detachment.   It is quite dramatic (scary) looking.   These aren’t little wrinkles like crows feet around your eyes.   They’re more like a  a Shar Pei dog has folds in his skin.   How she’s seeing anything at all is amazing.   Serous detached retinas aren’t flat, but the amount of folding she has more than would normally be expected.   It could be in part due to the low ocular pressure she’s had.

So the question remains, what is acting on her eyes?   Is it immunological in nature?   There is a new idea the doctors are considering and will be working with Dr. Trese (pronounced “Tracy”) in Detroit. Could the issue be mechanical in nature?

We had been told before that my daughter has small eyes.  The name for this condition is Micropthalmia.   There are all sorts of other things that will scare the pants off you if you go reading about Micropthalmia on the Internet, so don’t.   Just read on here because we’re not going to have all that nonsense with my daughter.  

Today for the first time they measured my daughter’s eyes to get a good understanding of how her eyes are not normal.   She meets the criteria of abnormal in three ways: 1) her corneas are small, 2) her lenses are round instead of elliptical and 3) her eyes themselves are small.   To what degree or percentage below normal ranges I don’t know but the amounts are measurable.

Then there’s the issue of ocular pressure.   It’s been low—too low.   The eye needs structure and there needs to be a certain amount of pressure in the eye to hold it up, just as you need enough air in a balloon to keep it firm.   If the fluid producing cells aren’t producing enough fluid, the normal draining process in the eye can remove the fluid faster than it’s created.    Today our doctors looked at those fluid producing cells again.   The good news is they look normal and intact.

So what does this all possibly add up to mean?  One theory is that there is a leak.    Fluid that should be staying in the eye is leaking through the choroid layer which could have possibly caused the detachments and is continuing, preventing her eyes from healing.  One of our doctors told us today that human eyes start from a single cell that divides initially divides and that her onset happening at almost the same time in both eyes could make sense even.    It’s a theory, but it’s one they’re going to investigate more, including treatment paths to help get her eyes back to where they can manage themselves.

Overall, a good day I think.   I am excited as I write this because I believe we’re getting close to an answer and a solution.

The Big Boy Update:   My son informed everyone at the table, “when I go potty by myself I just don’t wash my hands.”

The Tiny Girl Chronicles:  Hospital day.  My daughter had three different sedation medications and she didn’t wake up better at the end of it as a result.   Let me tell you something: an incoherent, screaming, floppy child who demands to be put down is quite the challenge.    Today my daughter  moved on to other, more aggressive ways to tell me she’s angry other than “you’re poopy!”   When I didn’t comply with her irrational demands today she slurred at me, “put me down or I’ll punch you in the face!”

Walking by the Paper

I was at Michael’s today looking for a wreath door hanger when I came across the origami paper section.   I glanced downward at it and kept going without much thought.   In a flash I absorbed the types of papers, sizes of papers and selection of choices for the origami naive or enthusiast that might stop there to make some selections.    I know a lot about origami paper because I’m one of those origami enthusiasts myself.    I’ve had likely some of each of those kinds of paper and even more likely still have some of them in an origami paper drawer upstairs.  

Today though, I just walked by without a second thought.   And then I had one…one of those second thoughts.   I thought how years ago I would have danced in the aisle to find that much origami paper so readily available, so close, so easy to find.    When I was young and in my origami youth I wanted to try it all.   I wanted to find beautiful kami, washi, duo and fiber papers and make models that were a delight fold simply because the paper was the right kind of paper for the model.    But paper was hard to find.    This was pre-internet shopping.  

I would go to cities on business trips and search used book stores for origami books.   I’d troll craft and stationery stores for papers and I’d visit asian markets in the hopes there would be a few packs of paper off in a corner.    Even internationally I was always on the lookout for papers that might be just what I was looking for to make one of my favorite modular balls.   I still have today some wrapping paper I brought home on a plane in a tube from Germany I had found on one trip.   (Not being able to use the beautiful paper you found because it’s too beautiful is a whole other issue.)

Today there are great sources for origami paper online and even the local stores have a reasonable selection.   I had forgotten what an adventure finding good paper to fold used to be until today when I casually walked by a collection of origami papers I would have been in awe of twenty years ago.

The Big Boy Update:  We were at a restaurant for lunch yesterday and the family behind our table had a baby in a high chair.   My son loves babies, just loves them.   He couldn’t stay in his chair he was so excited.   He kept getting closer to the baby and talking to us about him.   Then he started to sing “Twinkle Twinkle Little Star” to the baby (my son’s favorite baby song.)   The mother was very gracious about the whole, very cute, thing.

The Tiny Girl Chronicles:  My daughter was mad at her brother in the back seat yesterday.   I didn’t see what was going on but she said very angrily to my son, “just stop pulling me or I’ll hit you in the face!”

Fitness Update:  I know, right?  Where the heck have I been?  Sitting on my butt is what.  Not doing a dang exercise thing since the Las Vegas marathon.   I ran five miles today in some crazy early morning fog in the dark.   It was like Jack the Ripper spooky weather out.  

Can We Play Someone Bring the Rock Inside?

This is a game my daughter is mad about.   She is crazy happy to play it any time.   It’s not even a game, but she and my son think it is.   Mostly she thinks it a game that never has an end.   She has never tired of playing the game that I’ve ever witnessed.   It goes like this…

Put two children in the bathtub and wash vigorously.   Present towels to wet, giggling children ready to exit the tub.    Cover children up on the bath mat after they drop into the fetal position on the floor because, “I’m cold!” is shouted two seconds after exiting the tub.    Tuck the towel all around and under them to keep the cold out and then…wait a minute…where did the children go?  “hey mom, do you see the children?  They’re gone.”  <pause>  “Wait a minute, did someone bring a rock inside the house?   What’s this big rock doing in the bathroom?”   This is followed by sitting on the child and waiting for them to giggle enough so that the towel is pulled back and we—surprised—discover it’s our missing children.

There are variations on the patter, but the game is always the same.   My daughter wants to play it “one more time” again and again because my husband is so good at coming up with so many different things to do with large, unexpected rocks in the house.

Recently my daughter was making peeping sounds, including doing so under the towel so my husband modified the game and cracked the “egg” only to discover a little baby chick or duck or dinosaur (in the case of my son) instead of our children.  

My daughter has now changed the name of the game.   At the end of bath time she now says, “can we play someone bring the egg inside?’”

The Big Boy Update:  The children were talking about growing up in the car this morning as we discussed my son’s upcoming birthday.   He said, “I’m going to be bigger just like Mario after he eats a mushroom.”   Since I grew up myself playing Super Mario Brothers, I had to tell him how much I liked his analogy.   Maybe we’ll get mushroom pizza for dinner on his birthday.

The Tiny Girl Chronicles:  Yesterday she started talking about Uncle Jonathan.   She told my husband his nickname was “T-Stack”.   I’m not sure where she came up with this but when I messaged Uncle Jonathan about it he said, “I’m cool with that.”

Putting Up the Glitz

Today was holiday decorations day here at the house.   We don’t have a specific day we decorate as a family, but we don’t do it before Thanksgiving or before the start of December.   After we get into the month of Christmas we get it done whenever it seems to be a good time to do so.  

This morning I decided it was about time to get to it.   My son has a birthday party here at the house next Saturday so ideally we’d have some things out before then and doing the decorations on a weekend could involve the children in the process.  My daughter was interested in helping and my son was interested in playing outside with his friends.   No surprise there.

I brought down all the bins from last year and had no idea where things were because I’d completely rearranged into new, larger bins at the end of last year when we took everything down.    It turned out to be easier with fewer, larger bins.    My husband is still recovering from hernia surgery but we talked him into a tree shopping adventure with me promising to do all the lifting.    

Into the car with my children not listening when we told them how cold it was outside and one very unhappy, tutu-wearing child suffered through the majority of the outdoors part of the tree purchasing. My husband got us a wreath, a tree and he got a little tiny tree to put in their room.   They were very happy about this mini-tree for their room.

The rest of the day was mostly playing outside with children and neighbors while my husband and I did some work getting things up in and out of the house.   He had to move slowly and couldn’t pick things up, but he got a few things completed like the little candle lights and the plug-in trees by the front door.  I got everything done but the main tree in the basement which we had planned to do another day.

Did I mention before how much I dislike glitter?   I found several “glittery” things in my Christmas decorations that I purchased at some point.   They were of the sturdy glitter type in which the glitter is well-affixed, but that doesn’t prevent a glitter episode when the item is handled by an exuberant small child.  

Holiday glitz is up, glitter and all.

The Big Boy Update:  “Mom, there’s someone we don’t know at the front door.”   I was so proud of my son today.   We have told them to not open the door if it’s someone they don’t know and to come get one of us.   When I walked out to the foyer I saw it was our mailman, Pat.   I introduced my son to Pat and they shook hands.   I told him Pat was our friend and he could open the door for Pat any time.   We are very fond of our mailman, Pat.

The Tiny Girl Chronicles:  Diaper free.   My daughter has been asking to go to bed in underpants for a while now but we haven’t been wanting to take the step with her eye situation.   Tonight when she asked again I decided why not.    She does come downstairs some mornings with a completely dry diaper, which she likes to show me.    We have an alarm set for 11PM to go up, wake her up and have her go to the bathroom.   That worked well for my son when he was learning to hold it all night.   I’ll let you know how it goes.

Luminries

My best running buddy friend and neighbor had this idea to do a luminary event for our neighborhood.   She wanted it to be a service project for her children to help raise money for something.   Last year she mentioned this to me at about December NearlyChristmas-ish and I said something like, “that sounds like a great idea” and didn’t say anything else.    This year we talked about it in November and there was time to plan it and do it right so I said something like, “hey, I’d really like to do that luminary thing with you this year, let’s get to planning it.”

And we have.   The planning is done, the ordering of estimated supplies was made, the calculation of percentage of funds to go to the hospital was announced and a Signup Genius page invitation was created.   We have pre-sold over one-thousand luminaries for our neighborhood.   On December 19th, we’re meeting at our clubhouse, neighbors will pick up bags of sand, luminary bags and candles and pay $10 for each package of ten luminaries.    Over $7.50 per ten-pack will go to towards the hospital.

Today we put bags and candles into larger paper bags, ready for pickup.   We scooped sand into produce plastic bags.  You know those thin bags you put apples or tomatoes or other produce in?    Those suckers are sturdy.   You can put ten pounds of sand into one of them and tie them shut and they hold up if you’re not too rough with them.   We tried to come up with some sort of “reuse” plan and those bags were great because they were just getting sent back to the grocery store for recycling.

My garage is packed with sand, bags and candles in preparation for the 19th.   We’re ready for 1200 luminaries to be sold.   We’re getting close to that number already.   The neighborhood should look lovely on the 19th.   We hope the message of giving of your time to help others will be one my children and my neighbor’s children understand as well.

The Big Boy Update:  My son loves my neighbor’s four girls.  I think he tried to impress them the entire day while they were here.  He didn’t run out of energy even through two movies and pizza.   It only went downhill at the very end of the night when he started pulling his penis out in the living room.   As it turns out, it was itching badly from some eczema and it wasn’t him trying to take it “to the next level” with the ladies.  

The Tiny Girl Chronicles:   My daughter has been focused on drawing and coloring a lot lately.   She colored in over the last three days one of those “Velvet Elvis” sheets (as I like to call them).  It was a 18”x24” poster with a geometric design in white, surrounded by black fuzzy material.   You can color anywhere that’s white, but not on the black.   This works very well for my daughter given her limited vision.  She was so proud of it when she finished that I put it on our refrigerator so we can appreciate it over the next week or so.

Another Opinion (Referral)

We’ve been working on this for some time, and as of yesterday we’re taking my daughter for a second opinion on her eyes.   I dislike that phrase, “second opinion” because the number of eye specialists that have been involved in my daughter’s case as well as other, cross-discipline doctors, has grown into quite the list.  I had to create a spreadsheet just to keep track of them.

The care we are receiving at Duke University Hospital and the Duke Eye Center is excellent and yet we are still struggling because we have no diagnosis for my daughter’s condition and the treatment she is receiving is not seeing the results we had hoped for.   She is still well beyond legally blind and the school system’s eye specialist evaluator suggested a school for the blind and the introduction of braille as soon as possible, if, and she agreed we were on uncertain ground, her vision were to remain at the current state.

Throughout the last several months, starting at the point my daughter was initially admitted to the hospital on September 2nd for an expedited workup, we have been collecting information on where to go, if needed, for an additional opinion/evaluation.   I have second spreadsheet on all the doctors/locations people have suggested.    We have heard the phrase, “one of the best in the nation” many times and we are so grateful for all the suggestions and advice from our friends and friends of friends and even friends of friends who had no idea who we were but wanted to help out a family who had a little girl who suddenly went blind.

Of the suggestions we collected, I made a list of those most mentioned and specifically, those recommended by experts in the field of ophthalmology and retinal disorders.   I emailed our two main doctors and asked for their advice and recommendation.   They agreed with the one name we had heard more than any other, Dr. Michael Trese in Denver with Associated Retinal Consultants, specialists in “diseases and surgery of the retina and vitreous.”

We knew there would be no way we could get an appointment with Dr. Trese quickly ourselves.   Our doctors have been working with Dr. Trese’s office to facilitate a visit for us and as of two days ago, we are scheduled to have my daughter evaluated under anesthesia by Dr. Trese and his team on December 14th.   We have made travel arrangements and we are anxiously awaiting the appointment.

More when we return.   Hopefully some additional insight.

The Big Boy Update:  My son was in a hurry to get to Tae Kwon Do the other day as we waited at the red light just before the parking lot.   He said, “we should cheat” when I told him we couldn’t go yet because it wasn’t our turn yet.

The Tiny Girl Chronicles:  In addition to my daughter’s upcoming evaluation by Dr. Trese in Detroit, we have another evaluation under anesthesia (EUA) next Wednesday.   She will have a brain MRI just before which will show if any changes have happened in her brain since the prior MRI in early September.   There will be another specialist looking at her during her evaluation as well.    I’ve also talked with her rheumatologist yesterday and we are going to reassess the immunosuppressant medication after the EUA and MRI to determine if any changes should be made in her course of treatment.