What Happened To Her Eyes?

I've lost track of how many times people have asked me this question, "What Happened to Her Eyes?". Many times it's children who haven't learned to censor what they're thinking before it comes out their mouth. Adults inquire but in a less direct way, usually saying "Ahh, what a sleepy girl."  Either way it stings to know that the first thing people see is the difference of her eyes, even before they notice the trach. Because she isn't verbal I really don't know how much these comments affect her.  Sometimes I simply say "That's the way she was born." Although that's not the entire story.

In the past I've blogged a bit about Ainsley's eyes but I thought today I'd actually show you. As time has gone on I've become much more open about the things we've gone through, both the good and the bad because I hope that Ainsley's blog helps others: either because they are going through something similar or because people are inspired by all she's been through, her strength and spirit.   Perhaps sharing these photos will make it more clear why her eyes are the way they are. I'll have to warn you they are graphic.

Ainsley at 9 months of age, immediately before her 2nd cranial reconstruction, on 7/24/07. You can see a slight downward slant and her eyelids are a little droopy but not neither is too bad. At the time I actually thought perhaps her ptosis might get better after the reshaping of her forehead, which was bulging due to the craniosynostosis. I knew that her head shape would be different but I didn't expect her to look like a different person, mostly because of the change to her eyes. Sometimes I regret having the surgery but she needed it. The surgeons said that her brain "popped" when they opened her skull because it was under so much pressure due to the fusion of her sagital suture. Although sagital craniosynostosis is a surprisingly common condition her case was severe.

The scan of her skull before.You can see that her soft spot was larger than normal as the skull shape changed, trying to accommodate her growing brain as best it could. In addition you can see she was missing the larger wing of the sphenoid bone behind her eyes (which is practically unheard of ever) so the surgeon reconstructed that during the surgery as well otherwise her eyes would be resting against the dura.  He was able to split some pieces of bone in 2 so he was able to do the entire surgery using her own bone.

Her head shape post-surgery. The tube is a temporary drain to keep fluid and blood from building up on the brain post-surgery.

The height of swelling which may have caused stretching of the muscle that lifts the eyelid. 

The post surgery scan of her skull, after it was removed, split into pieces and reshaped by patching bone together using special absorable plates. Notice the brow above the eyes is reconstructed. It could also be that the repositioning of her brow area around the eyes that caused the change.  You can see that this was no simple surgery.  You have to give Ainsley's surgeon credit, what he did was amazing! The effect to her eyes was an unfortunate complication.

As you can see in this photo, taken 15 days later her eyes appear quite different. Of course she's still a cutie. At this time we thought perhaps there was some swelling (it can last up to 6 months) causing the change. It turned out not to be the case. We waited and hoped for improvement but it never came. She can use her brow to lift her upper lids at times, but it requires some effort and isn't a great long term solution. Although we know she can see, I do believe her vision is somewhat obscured by the droopy lids.

Similar to that surgery in 2007 the surgery being done tomorrow isn't a simple ptosis repair. The occuloplastic surgeon will make an incision at the eyelid crease, removal of some tissue from her upper lids which will then be transplanted into her lower lids to help bring them up. They will shorten the levator muscles that lift the lids and they will bring up the corners of the lower lids (canthopexy). There will be bruising and swelling so I'm not sure how long it will take for us to "see the final results". 

We appreciate your continued prayers and positive thoughts for a safe and effective surgery. We arrive tomorrow at 6:15, surgery will start around 7:30 and will take a couple hours since they are doing multiple things. Remember we are in the Pacific time zone. I will update as much as I can over the next few days.

Pray If You Will

If pressed I would have to describe myself as agnostic although that's a rather simplified statement of my beliefs. Though I would say that, I am oddly drawn to the Catholic faith and its cathedrals from time to time, particularly the older and grander ones like the St. James Cathedral in Seattle.  Oddly enough our home phone number is one digit off from theirs and from over the past 20 years we've received phone calls from confused people in crisis looking for a priest or sister. It's just a funny coincidence I thought I'd mention. There is something about stepping into one of these cathedrals that feels very special and sacred. If you've never done it I suggest you do, regardless of your religious persuasion. It is an amazing experience that leaves me feeling connected to the ancient and all beings and their suffering.  It's particularly moving at night when the desperate tend to come for solace. The warmth of candlelight in the majesty of that architecture is something unique and palpable. Their doors are open to people of all faiths or non-faiths, as the case may be. My children have never been to church so imagine their surprise when I took them to the cathedral in the evening after school to light candles for their sister. I find the room of the Mary Shrine to be particularly moving with the imagery of mother and child and all the candle holders shaped as nails and a symbol of suffering.  Something my baby girl has had far too much of.

You see, we are coming up on Ainsley's eyelid surgery (as well as the leg plate removal and brain MRI), now that it's Sunday night there are only 3 days until we'll be waking up and taking her in at dawn. I am so incredibly unsure about this decision that I will take guidance and/or reassurance from anywhere I can. So please if you feel inclined to, please pray for Ainsley: that she stays well between now and Thursday morning; that the occuloplastic surgeon has clarity of mind and execution of skill; that the results will be beneficial and the result to the appearance of her eyes will only be positive.

Ainsley is such a sweet girl. She's been through so much, she really deserves to have a good outcome.

I Am Thankful For My Family

Sorry for my late post. It's not that I wasn't thinking about you all over the Thanksgiving Holiday. The week prior to Thanksgiving I'd been thinking of writing a clever top 10 things I am thankful for, I'd even started browsing through my photos for content but it just wasn't coming together. And then the day before Thanksgiving I got the terrible news that a friend's adorable little boy from the tracheostomy forum (which is really like family) unexpectedly passed away. Angus was so well loved and will be sorely missed. Please say a prayer for Angus' family and hug your loved ones extra tight.  With this horrible news I started a post about the comparatively high mortality rate for children with tracheostomies and how thankful I am for Ainsley's continuing good health despite all she's gone through but even with a thankful spin I thought it was just too depressing for Thanksgiving Day.  And so my Thanksgiving Post has turned into a post-Thanksgiving-Post. So this year I'm going to share a picture that says it all.

In 2008 we started a new tradition (I guess by now you know I really like traditions). Evie made this beautifully colored paper mache' "thankful bowl" in class. Everyone writes what they are thankful for (anonymously) on strips of paper and puts it in the bowl and then we read the thanks out loud. I thought Adrian's said it all. Friends near and far are part of our family too.  What's most important in life is our relationships with the people we love and who love us. There are many other things I am thankful for but that one is the most important to me.

But on a lighter note I do have one to add......

I am especially thankful that I did not have to cook a turkey dinner this year!

I hope everyone had a wonderful Thanksgiving Holiday.  

On CPAP Hiatus

After getting Ainsley to sleep for an hour with the CPAP last week thought it through and started to question whether we really need to have her sleeping with CPAP on from now until the spring. And I didn't like that she was satting at 95% (while a decent number it is 5% lower for her) so I left a message for the Pulmonologist and got the message yesterday that we can take a break until February and they'll let us know if they want to increase the pressure or not then. I may put it on her from time to time to keep her familiar with it but I am relieved not to have to start the battle of wearing it every night....just yet.

CPAP Success!

Friday CPAP 30 second x 3 = 1 min 30 seconds with airflow on and cap.

Saturday 5 minutes on CPAP with cap while watching Nick Jr.

Sunday 10 minutes on CPAP with cap while watching Nick Jr.

Monday 15 minutes on CPAP with cap while watching Nick Jr.

Tuesday 25 minutes on CPAP with cap while watching Nick Jr.

Wednesday.....

CPAP with cap for 1 hour while SLEEPING!

Obstructive Sleep Apnea?......NONE

WOW! She did it. Did she like it? No. But it's one step closer to life without a trach. Her sats were a little low, so tomorrow I'll be checking with the doctor about bumping the airflow setting up to see if that brings her oxygen saturation level from 95 up to her normal of 98-100%. Also we need to decide if we are going to try to have her sleep with the CPAP on regularly for naps and/or bedtime. I feel bad about asking her to do this considering she still has a trach cannula in her airway and she only needs to use the CPAP to prepare her for using CPAP if/when she is able to have her trach removed, hopefully this spring.  I knew it would work but I also can't believe it. WOW!

Exciting CPAP Progress

Maybe you've been wondering what's been going on with our CPAP trials. The answer is not much. Kids don't like CPAP, that's no surprise. Heck, adults don't like CPAP either. But I quickly discovered that Ainsley really didn't like the feeling of the air coming into her nose and out her mouth. I tried it myself. It is very uncomfortable to try to breathe through your mouth with the nasal mask on.  When I closed Ainsley's mouth she tolerated it much better but since I can't hold her mouth shut while she sleeps I lobbied to get a full face mask or a chin strap instead. (In the mean time we continued to use the nasal mask for 15 seconds at a time before her naps.)

Getting the full face mask proved more difficult than expected. I thought our number one goal was for Ainsley to be successful and comfortable. However the respiratory therapist was concerned about safety and relayed those concerns to Ainsley's pulmonologist. Even saying to me "That maybe she just wasn't ready for decannulation."  Because she can't tolerate the nasal mask?! Logically none of it made any sense and I'll spare you all the details because it is complicated. Let's just say I spent a fair amount of time on the phone with the pulmonologist, his nurses and the respiratory therapist. In the end the pulmonologist could see the logic of my argument and I got what I wanted and the doctor wrote the scipt for the full face mask. FF another week and a half the respiratory therapist finally delivers the mask at our home, we try two for fit and voila' it makes a huge difference. Do I know my kid or what?!

Later that day I did our usual routine: CPAP for so many seconds with a reward of of reading a book, repeated two or three times. She seemed much calmer and was able to increase the number of seconds over the following days from 15 seconds, to 20, and then to 30 seconds at a time! We even strapped the mask on rather than just holding it, which she doesn't like. All these times she was capped so she was actually breathing through her nose and mouth with the CPAP airflow on. She was doing well! Although she wasn't liking it she wasn't panicked like she was with the nasal mask.

So I thought it was time to up the ante and really do it. At bed time last night we strapped the mask on. She'd fallen asleep on me during a movie so she wasn't too happy but she eventually calmed, the airflow was on but she would start to cry if we put the cap on. So unfortunately we didn't get to see if the CPAP actually helps the obstructive sleep apnea. But we did see that she can tolerate wearing the mask and the feeling of the air on her face (it was on). We even put her down to sleep and she was fine though she stirred a little after an hour or so, so we took it off so we could get a better night's sleep.

We decided the next step is to increase the amount of time she will breathe with the mask on while capped and we think we need to do that while she's awake instead of when she's tired. And we need to make her bed more comfortable and get her to learn to sleep on her back instead of her stomach or side. I am really hopeful. What I don't know is if the CPAP will overcome the obstruction though chances are good. But I do think that she will be able to learn to sleep with this gizmo on. I can almost taste life without a trached child and it's oh so sweet.

The full face mask.

Doing it for 30 seconds.

Her favorite part. I let her turn the machine off.

Her last night, sleeping with the mask on (no cap though) and with airflow to her face.

A Day To Truly Celebrate

When you have a child with medical issues you get a LOT of mail from your insurance company. I hate to bump my Halloween post with such mundane topics as Medical Insurance but I must.

Often you get to a point where you just ignore the EOB's, which is dangerous, or anything else that comes from the insurance company. Sometimes, but not often, what they send is important and GOOD. Yeah, that's what I meant to say, GOOD.


This is what I found in the mail today. Some people will say that the happiest day of their life was the day they were married, or the day their first child was born. Not for a mom with a medically intense kiddo who is steadily climbing their way to their life-time maximum benefit of 2 million dollars.  Today is the happiest day of my life! I no longer have to worry that my child will not get the medical care or therapy she needs. While it's true Ainsley receives Medicaid for secondary coverage due to her intense medical needs (ie. the trach and g-tube) that would stop if she gets her trach out. And then what? What a relief! We were getting a little too close to that 2 million dollar mark for comfort.

Sometimes I feel conflicted about the massive cost of Ainsley's life. But what people may not realize is that the doctors don't give you an estimate when they offer a life-saving surgery like a tracheostomy or a craniectomy. I had no idea. And besides it was never really our choice. The unthinkable option of ending her life at birth was never really brought up. Bio-ethics was not consulted. I am grateful that it is not my choice whether my child's life is worth 2 or more million dollars. I am grateful for medical insurance. And I am grateful for my health. And oddly, I am grateful for Ainsley's health. Despite her birth defects (I hate that term) she is actually very healthy and happy.

In addition I received a letter from insurance stating that they needed photos of her eyes to consider approval for Ainsley's eyelid surgery (duh). But when I called the eye surgeon's office they said that was already done and they just got the approval yesterday! So I left a message with the orthopedic surgeon to call me back so we can look at our schedules and coordinate with the eye surgeon. Tomorrow I happen to have an appointment with the orthopedic surgeon so perhaps we'll know then. Then we'll cross our fingers that nobody gets sick. We just finished passing around a cold and I'm still not feeling like myself. Hopefully it won't be like her other surgeries last year that were postponed multiple times due to illness. Whenever it happens, at this surgery they will:

• remove part of the upper eyelid, place that piece in the lower lid and lift the lower corners of her lids (canthopexis)
• remove the plates from her legs from the April hip surgery
• a CT scan of her cerebellum (just to see, now that she's bigger the second looked different from her first and so it would be nice to know for sure what the cerebellum malformation truly is)

It's a bit to coordinate and if you can believe it the eye surgery is normally OUTPATIENT, but the plate removal requires an overnight stay. Who would think removing some plates would be more serious than cutting apart the eyes. Doing the surgeries at the same time will save an anesthesia. Same with the brain scan. Going in once instead of 3 times sure would be nice.

I am scared. And hopeful. It's been 3 long years since her second cranial reconstruction.  Sometimes I have to look back at these pictures a professional photographer friend took the day before that surgery in 2007. If I didn't have these pictures I don't know if I could remember how different her eyes looked before, or believe it. There are hundreds of photos so there is just no denying the change.

 

  

Did I say I'm nervous? I'm no longer naive enough to assume a surgery is going to have a positive cosmetic result. The eyes are delicate, and important. We've agonized over the decision but believe it's the right thing to do. My hope is that this surgery might restore her eyes to the level of ptosis she had prior to that surgery. And I hope that she can SEE (not that she can't, but you know it's a little more difficult for her) My fear is....well you can imagine. I'm excited too, in a sick to my stomach way.  I just wish Steve was home to celebrate. He's back to crazy work hours to meet his game deadlines. Busy times ahead.