Wednesday, March 20, 2013

Tough Choices

Last Monday we had an appointment at the Neurodegenerative Rare Disease clinic at Children's Hospital of Pittsburgh.  It was Naomi's second appointment there.  The clinic follows kids with all sorts of degenerative diseases longitudinally.  Dr. Escolar is the doctor in charge and I guess for some diseases she and her husband have done bone marrow transplant with some success in halting the disease.  Not so for Batten's, but it is a good resource for us to ask questions and have OT/PT/Speech/Hearing eval with folks who are used to seeing kids like Naomi.

 Naomi has been doing worse.  Over the last two months she seems to have regressed some.  Her sleep has been very difficult.  She is getting up early and not napping well despite us trying all the tricks we have tried in the past (early bedtime, late bedtime, restricting napping etc.)  As a result she has been very prone to tantrums and has had poor ability to tolerate anything for any amount of time. She is shreiking again when her sister makes any noise.  She is impossible after napping.  It is very upsetting to see her like that for us - we want her short life to be a  happy one.  She has not been eating well either.  We are down to oatmeal and strawberry soy yogurt (yuk) and it is 30-45 minutes of struggling and we are noticing things like lots of spitting out and hard swallowing.

We explained all of these things to the folks at the clinic.  They felt like all were signs of the beginning of regression.  Especially the difficulty eating and swallowing.  They recommended a feeding tube.

Living day to day it is easy to put Naomi's future out of your mind.  Sure she is different, but to us her different is normal.  We are not around normal 4 year olds all the time and in a sense don't fully realize how limited Naomi is.  She has always been kinda difficult to feed and very picky, and sure she has gotten more difficult and more picky, but we always felt like that was just her.  It's hard for someone to tell you that it isn't just her, but her degenerative disease.  Before this we could try to forget, or pretend that somehow Naomi was going to be okay (well, not okay but not getting any worse).  This feeding tube suggestion has shaken us out of our delusions.

We are going to meet with the surgeon next week.  We are going to get the feeding tube.  We know that it not going to prevent any further regression or change Naomi's course, but it will truly make her life better.  We won't have to torture her to eat or drink.  She will still  be able to do all the things she likes to do like swimming.  It still sucks.  Our reality sucks.

Naomi decided she needed a nap just as we got to the 9am.

Naomi had a good time showing off all of her moves to the Physical Therapist, especially her Big Kicks.

Lydia got bored and cranky had to be mostly stripped down for some play time.   She loves to be unclothed.  Just wait until summer, Lydia!

Naomi, Lydia and Clementine having a morning snuggle.

Naomi and Clementine watching a show.

Naomi and Lydia right before Naomi got on the van to go to school.  Naomi has been picking her outfits and her hairstyles.  She prefers two 'Dakota' (the pony she rides on Saturday mornings) ponies.

Bear on a belly!

All dressed up for school.


  1. The girls look so cute together!

  2. Kerri (Aiello) JulianMarch 26, 2013 at 12:33 PM

    What beautiful girls!!! You guys are in our prayers!