Shades of Blue

Yesterday was your average day. We had two appointments and then spent the afternoon playing in the yard. Aria's seizure activity has been increasing and changing. Her phenobarbital levels are in a therapeutic range (31 - we shoot for between 30 and 40). Her vomiting has become quite frequent so we've had to back off her feedings but this is just part of life we've come to accept. Even with all that going on, we had a good day. Daisy got her first skinned knee of the season and Aria basked in the breeze of a beautiful Spring day.

Around 11pm I hooked Aria to her feeding pump in her bedroom. I then went downstairs and finished up on some laundry and, after putting the laundry away, I snuck into Aria's room and added her nighttime meds to the med port of her g-tube. Then I went to sleep.

About an hour later I got up to use the restroom and, of course, there was no toilet paper in the master bath. So I cursed and walked down the stairs to the laundry room to get some toilet paper. As I walked up the stairs I heard a faint squeak coming from Aria's room. We've been trying to ignore her middle of the night antics (she thinks she gets to just hang out in the middle of the night) but something about this sounds made me want to do more than just peek my head in the door.

I walked to her door and turned on the light and there was my girl. Flat on her back. Blue. Maybe grey. Judging by the color of what was coming out of her mouth she was choking on her medication that had been administered via g-tube an hour before. Had she been struggling the whole time?

I grabbed her by the head and flipped her over and started beating on her back.

Those who are "in the club" and get to go through this kind of shit understand the million and one thoughts that all occur at once.

This is it.

At least she won't die alone.

If I were in the hospital they wouldn't be allowed to resuscitate her. Should I be doing this?

Should I call an ambulance? There's no time!

I need to get air in her little lungs.

Why can't I yell for my husband right now?

Why can't I make a sound?

Should I be doing infant CPR?

She's the size of an infant.

She's not an infant.

Air is air. I need to give her air.

And as I went to give her air she coughed a big cough and inhaled. I continued to beat her on the back to get as much out as possible and the color started to return to her body.

I disconnected her feeding tube and sat on the floor with her in my arms just rocking back and forth as I sobbed. I squeezed her fingers and toes, counting the seconds it took for the color to return. Once I was sure her oxygen saturation was back up I laid her back in her crib.

I listened to her lungs. They sounded rattly but I know from past experience that this is pretty typical. I decided to wait a couple hours to check again. I curled up on the floor next to her and felt consumed by guilt as I heard her cough...my little fighter was clearing her own lungs. Did I give her meds too fast? Was the rate wrong on her feeding pump? How was my child allowed to struggle alone while I was in the next room? She must have been terrified.

I stayed with her and, once her lungs sounded clear, I got some sleep myself.

I woke up in good spirits. I laid in bed with Daisy while I checked the news and facebook before going to get Aria from her room. Kevin had already left for work. But then as I approached her door I felt dread. It brought me back to the days early in her life where I was afraid to enter her room every morning. Those days I would enter the room with my eyes fixed to the floor. I'd take a deep breath and hope that when I looked up she would be breathing.

As I approached her bed I saw my sweet Aria just chilling in her crib as if nothing had happened. She just gave me her crooked little Aria grin as she realized a form was hovering above her.

I sobbed for awhile as I tried to hide from Daisy. My friend Tracy called at the perfect time during my breakdown. Tracy is a pastor at a local Lutheran church who I met at spin class and something told her that I needed her to reach out to me this morning.

Then my friend Hannah came over. She tried to convince me to go sleep while she watched the kids but I refused.

You see, I need to use every day we have. The line between life and death is a thin one. Sometimes the pressure to make every day count can be a bit too much but I had to make today count.

The girls and I went for a jog at the park and played on the swings. My friend Hannah had helped me assemble my new double jogging stroller earlier in the day. I was thrilled to learn that Aria loved the sensation of flying through space as her big sister yelled "faster!"

Today I tried to act like it was just another day knowing the guilt I would feel if Aria passed one night and I didn't choose to seize every moment during her last day on earth.

I love you, Aria. Keep fighting.

Six to Eight

A couple of weeks ago we decided to pull Daisy out of SLP group at the public school one day per week (still leaving her with 1 day of SLP per week) so that she could start 3 new therapies.

Frankly, since aging out of the birth to three program, I don't think the public schools have done a good job at meeting Daisy's needs.

So, I decided to have her evaluated at a different center who suggested OT, PT, and feeding therapy (all therapies which she was receiving in birth to three but were not being provided by the school). Daisy was only receiving 30 minutes of OT per month in the school and even though she could not safely sit in a chair or walk in the hallways the school chose to give her a special seat instead of provide PT which would help her gain the necessary skills to sit in a normal chair.

So, leaving the school so that Daisy could receive the therapies she needs in order to one day safely function in a class room was an easy decision.

Daisy has only been receiving her new therapies for two weeks but I can already tell I made the right decision...even if this decision means I have eight therapy appointments to juggle per week instead of six.

It's Killing You.

The Suspense.  It's killing you, isn't it?

I wasn't even trying to be a dramatic douche when I wrote this post about Aria's procedure in Pittsburgh on Monday.  I meant to just break it in to two posts so that my post didn't get too long.  Then life got in the way and I never wrote the second half of the post. 

I've actually been busy dealing with some horrible dental pain and an infection.  I've also been doing something for myself...or trying to do something for myself.  I'm not sure if I succeeded yet.  Basically I'm trying to get back into an old hobby because I think it would be really good for me and it is the perfect time for me to do it.  But I don't get to choose whether or not I participate so, for now, we will pretend I said nothing.

Now back to little Miss Aria...

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I have a riddle for you guys!

Q: Who has no thumbs and is getting a corneal transplant?

A: Aria!

She who has no thumbs.

Aria's bioptogen OCT exam showed that she has completely normal lenses.  She is scheduled to get a corneal transplant at UPMC by the great Dr. N on August 5 (he's at hospitals in London and India until then).  We are so excited that she just might have the opportunity to see in the near future. 

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I have another riddle for your guys!

Q: Who has no radius and is getting an eye lift?

A: Aria!

She who lacks radii.

Aria's firsts transplant will be on her right eye because it is her worst eye.  That way if the transplant fails and she loses her eye she will still have her "good" blind eye.  Judging by all the pre-transplant testing that has been performed, the doctors think that the vision in her left eye might be as good as 15%.  Of course the left side of her face is the side that is paralyzed so she can't really open that eye.  So, Aria is getting an eye lift on her left eye on April 30 so that she can start benefiting from that vision.  This will be a super simple outpatient surgery where Aria will go in for surgery in the morning and come home early afternoon the same day.  I'm not going to lie, I'm going to miss that droopy little eye.  She'll still have her crooked smile but that droopy eye is part of what makes her the perfect little Aria.

I am NOT going to miss random people exclaiming "oh, look!  She's sleeping!" when I'm out in public.  I feel like no one ever felt the need to inform me that my typical looking kid was sleeping when we were out in public but, for some reason, people are always telling me Aria is sleeping when she is not.  I've started just saying "No. She's wide awake.  She's just blind and a little paralyzed."  Then everyone gets really quiet and uncomfortable.  Yup.  Not gonna miss that one bit.    

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Good Lord I love me some limb deformity humor.

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While we were in Pittsburgh this time I had my best friend Cami stay at my house with Daisy.  I can't thank her enough for always being there for me.  I even got the guest bedroom and bath all set up for her arrival.  

I used the antimicrobial Febreze.

I must admit I'm a little excited I don't have to go back to Pittsburgh until August (the eye lift will be done at the big fancy hospital where we've spent most of our time).  After the transplant we will stay there for a week to watch closely for rejection and then Aria and I will return weekly for quite some time.  I am going to relish these next few months and really try to do some things for me and some special things with my girls.  Getting a transplant is hard work...getting a transplant from a Doctor who works in a hospital across the country is really hard work. 

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Thank you to all of you who have supported my family and me as we've ridden the roller coaster of trying to find out if our child is a transplant candidate.  All of your support and kind words have really helped to get me through.

Nostalgia

 The girls and I took our first walk of the spring on Friday. 

I'm going to miss these walks.  You see, we bought our big yellow American dream home when our dreams were different.  We close on the sale of our home on May 1 and the purchase of our new home the same day.

We've purchased a home that is more accessible for Aria and will accommodate the ataxias of those in our family affected by other conditions.  It's a beautiful home but it isn't the home we bought when I thought I would be a stay at home mom to five healthy typically developing kids.  It's hard to imagine a time when I thought having all the bedrooms on the second floor made sense.  Well played, Universe.

Our current neighborhood is this little loop that no one even knows about so it allows my children to run freely through the streets.

Our new home is in the neighborhood that everyone lives in.  Seriously.  Almost all of my girlfriends live in the new neighborhood.  In fact, one of my besties and biggest supports will share a backyard with me.  Our livers are in BIG trouble.  It's a great neighborhood but it's a big neighborhood with a lot of cars so I think our walks are about ready to change.

I'm sad to be leaving my dream home but so happy at the same time.  There is so much disappointment wrapped up in this pretty yellow house with the wrap around porch...so many dreams that will never be.  It was time to leave.

I love our new home.  It's perfect for the family we ended up with.  It's only slightly smaller than the big yellow house but the main level has only three bedrooms and is considerably smaller than what we live in now and is just right for our family.  There is also a finished bonus room above the garage that we plan on using as a sensory/therapy room for the girls.  The basement has a fairly private entrance (from the garage), two bedrooms, a bathroom, and a kitchen...perfect for a live in intervener.

Have I mentioned that I'm going to get Aria a live-in Intervener?  I don't know how I'm going to do it or how we will afford it but I am going to find a way to get Aria an Intervener and I always find a way to get my sweet Aria what she deserves.

 

The Back Story

So.  I guess I never really explained what happened last time we were in Pittsburgh that contributed to my little nervous breakdown.

We had a wonderful examination with Dr. N.  He actually had the top pediatric corneal transplant specialist from South America visiting at the time so we got to visit with both of them.  She had a lot of great ideas about different visual activities we can do to work on improving Aria's visual development now.  She even showed me some really awesome visual skills that I didn't even realize Aria has.  I loved having two of the world's best in a room focusing solely on my kid.  At that very moment I was convinced that Aria was the luckiest visually impaired child in the universe just to have all that expertise surrounding her.

Upon examination, Dr. N and his colleague both agreed that some of the findings from her previous testing were wrong (I guess the ERG can be a little unreliable on children like Aria).  Particularly, they thought the result that said her cones and rods weren't functioning well in at least one of her eyes was wrong.  They thought her cones and rods proved to function quite well during her behavioral examination.  Such great news!  They both agreed that Aria's left eye is her best eye and that we should proceed with a corneal transplant on the right eye.  They would start with the right eye just in case the transplant fails.  That way Aria would still have the use of her "good" blind eye if she loses her right eye completely.  They also said that we should proceed with an eye lift on her left eye as soon as possible so she can get better use out of it now (the left side of her face is where her paralysis is so her eye is only partially opened).

Then Dr. N went to get the ultrasound machine to take some measurements to prepare for the transplant surgery.

Neither physician could find a lens.  They were shocked...no one had anticipated this finding generally related to a FOXE3 genetic mutation in our child.  At times they thought they saw a lens but then they couldn't find it again.

Dr. N looked at us and told us that if a child doesn't have a lens a transplant would fail 100% of the time.  He said that not having a lens meant that Aria would no longer be a candidate.  He then said that there was a chance that she did have a lens but that they just weren't seeing it since Aria was totally freaking out or that the lens had grown attached to the cornea and that's why they thought they were seeing something sometimes when looking with the ultrasound.  He said, if this were the case, that Aria might still be a candidate but he would have to bring a specialist into Aria's surgery who would be able to separate the tissues of the cornea and lens while the transplant is taking place.

In order to know exactly what was going on with Aria lens-wise she would need to be examined with a machine called a Bioptigen Optical Coherence Tomography (OCT) machine under general anesthesia.  The Bioptigen OCT is fairly new technology and there are only three of these machines in the country...and Pittsburgh has access to one of them.

So, after a short insurance battle, we found ourselves back in Pittsburgh today.

A little post-op/post-answers snuggle

Today we got definitive answers as to whether our child would be allowed the opportunity to see.

We're Back

In Pittsburgh, that is.

Aria is having one final test done under general anesthesia tomorrow. This isn't a test that typically has to be done before a corneal transplant but we've learned to expect the unexpected with Aria.

We are hoping to know for sure if she is a transplant candidate by Tuesday.

Wish Miss Aria luck!

ABA for Daisy

When Daisy turned three we decided to allow an autism diagnosis for her. We did this for a number of reasons but our primary reason was so she would be able to receive services for which she was no longer eligible once she aged out of our states birth to three program. We also did it because our kid is totally autistic and I was finally at a point where I wanted to be able to say "she's autistic" (read: give me an effin break I'm doing the best I can).

One therapy from which I knew Daisy would benefit was Applied Behavioral Analysis (ABA) Therapy. So, as soon as we got her autism diagnosis we went to the awesome ABA therapy center we have in our town for an eval (Our small town actually has an ABA center that kids travel to every day to receive services.) Daisy was evaluated by the center and they suggested that she receive 24 hours of therapy per week. I had her doctor write a prescription for the therapy and, of course, our insurance company denied it saying they needed more info.

The thing is that Daisy's multidisciplinary eval ending with an eval by a neurodevelopmental pediatrician who specializes in autism spectrum disorders was scheduled for five months out. Everyone preaches how important early intervention is but then it takes half a year to even get a freakin' eval. It's frustrating at best.

So, I wrote an evaluation on Daisy including a sensory profile and an in depth discussion of her social issues and self injurious behaviors and sent it to her regular pediatrician. He and I talked about it. He copied and pasted the report into his office notes and sent it in to the insurance company...and Daisy got approved for ABA therapy.

Bam. Overeducated stay-at-home-mama for the win.

So, three months after we initially requested ABA therapy we were finally approved. She was only approved for 30 hours per month (basically two mornings per week) but our foot was in the door and i was thrilled. We hit the ground running! The first thing that HAD to be done was that Daisy's private therapy room needed to be decorated. My best friend Cami and I went to Hobby Lobby and tried our best to pimp out an otherwise stagnant room.

We even left little notes on her marker boards so she would know how much we love her.

And, late the night before Daisy was to start therapy with her own private ABA therapist, I went a little crazy with family photos, paint, and mod-podge. Good Lord I love mod-podge.

Daisy had a great first day at the center and has been thriving there. There are a couple of other high functioning kids like her who she has social group with and she is learning to allow people into her space. She is also learning how to do things like say "hi everyone" when she enters a room and "goodbye friends" when she leaves. I know she doesn't truly mean it and she isn't legitimately acknowledging the presence of the people around her but it's a start to learning socially appropriate habits. I am so proud of her.

Yesterday Daisy finally had her evaluation with the multidisciplinary team and the neurodevelopmental doctor. At the end of all the evaluating the doctor looked at me and said "she is autistic".

No shit.

Now that she has some really good evaluations by some really well qualified professionals I'm hopeful she will be able to get the 24 hours of one-on-one therapy she needs each and every week. ABA works best when there is continuity and two mornings per week just isn't enough. This new doctor will be writing the insurance company to ensure that Daisy gets the amount of therapy she needs and for that I am so thankful.

At the end of our appointment yesterday the doctor discussed Daisy's unusually high cognitive functioning. He said, "I see about 40 autistic kids per day and I've gotten really good at predicting which ones are going to do well and which are not. i predict by the time Daisy is seven years old that you will walk into my office and tell me that you don't think your kid is autistic anymore."

We can only hope.