Ballerina Avery

Ballerina Avery

Monday, January 23, 2012

Avery is doing AMAZING!

Avery is just doing Amazing! She is walking everywhere and talking up a storm. Everyone we see is completely impressed with her improvements and we are too! She is getting so much stronger. Her fine motor skills have improved. She dresses babies, colors, cuts with scissors, drinks out of a big girl cup(when her crazy, clean freak mother lets her)she even opened her own presents at Christmas time. She still is falling quite often..but does not hesitate to get right back up and keep going! We found out the hard way that people with Pompe bruise easily and fast. She got her first HUGE goose egg at her daycare Christmas party. It stuck out so far and was just nasty looking. Then she fell over in the sitting position and bumped her head on the wall and INSTANTLY got another huge goose egg. We decided to Google it of course...and it is not uncommon. We just ice it right away to avoid some of the swelling. She puts sentences together that sometimes I still need to translate! We are very proud of her for being so determined. In her mind and ours...there is no other option but to be DETERMINED!

New braces for A

We visited Dr. Dabrowski (Physiatrist) on 1.18.12. He prescribed new braces for Avery. They are similar to the ones she has now but will be hinged around the ankle for better movement. He was AMAZED at her improvement and is expecting her to keep changing! So we will be visiting him again in 3 months..appt set for April. Will keep you posted!

6 month check up

We had a 6 monthish check up with Dr. Stockton on Dec. 20. We just went over her progress, he examined her and we got a to do list a mile long. She needs/ed to see Pulmonology, Ophthalmology,  Psychology for a Bailey developmental assessment and 2 tests that need to be done by her physical therapist when we go back.

We had the pulmonology appt. already and he thinks her lungs sound great :) He did prescribe a cough assist machine for her. This will help her get things up(mucus and phlegm) that people with normal strength do with no problem. We will do it twice a day as a preventative and if she is sick 6-8 times a day. That will be coming as soon as we jump through the several hoops our insurance makes us jump! Ophthalmology is scheduled for Feb. I left a msg with Psychology and need to call them back because I have not heard  back. Will keep you posted on those appts. We go back in 6 months.

They also mentioned:  Avery had DNA analysis to identify the mutations in the gene. Two mutations were identified (as expected given that she has the condition). We know that both of you are carriers. However, we always recommend confirming your carrier status by DNA analysis. Parental testing would look for the mutations identified in Avery. Carrier testing will confirm your carrier status and identify the specific mutation on either side of the family so that other family members could be tested to determine their carrier risks. If you are interested in carrier testing, you should contact your insurance company to see if they cover the CPT codes for the testing. If so, testing can be performed the next time you are here.
We are not sure if we are going to do this at this time.

Taking a break!

Wow..it has been nice having a little break from Kids in Motion Therapy. We are still doing the school therapy one day a week. We plan on going back to KIM in Feb. 2 reasons: we had to wait for our insurance to renew in Jan., and their policy is you have to take a break after a steady 6 months in therapy. I guess that is for insurance reasons on their end? Who knows..who cares...we got a much needed break!

Changes with Genzyme?

http://amdapompe.ehclients.com/downloads/news/Pompe_Community_Update_January_13.pdf

Article about changes that will be coming with Myozyme? I talked to Dr. Stockton about it and he filled me in some. More to come when we find out more. He says the two drugs (Myozyme and Lumizyme)are the same. Lumizyme is not FDA approved in the US at this time.  I guess we will wait and see. He says Avery can't be changed without our consent.

Avery walking

Infusion 15,12.9.11/ Infusion 16, 12.23.11/ Infusion 17, 1.6.12/ Infusion 18 1.20.12

OK...Sorry..it has been awhile! I've been so busy. So here is a brief recap on the last few infusions.

#15 was a nightmare...3pokes. They had a good vein on the first one and she moved when I was holding her. Then Grandma Lorraine held her and the poked her and couldn't get the vein...so they poked around for a few minutes until she was screaming and crying so hard I told them to stop. Avery and Mommy took a little break and went for a walk. When we came back they got it on the 3rd try :( Poor thing! Part of the problem was we made Avery upset before we started by placing a bag to catch urine on her. She was due to have labs done. They needed urine and blood. We survived!

#16 Two days before Christmas so Wade was off work and took her by himself. He said it was a quiet day and they were the only ones there. They got it on the first poke. After infusion Wade took her to get a chest x-ray as part of a routine check up.

#17 Mom and Grandma Lorraine took her this time. It was a good day. She was great. First poke and we were out of there pretty early.

#18 Again, Mom and Grandma Lorraine took her. It went good this time too. Avery was pretty grumpy and refused to sleep like always. We managed and survived :)

Thursday, January 5, 2012

I'm behind on posts.

I know I've not updated on the last couple of infusions. I promise..I will update soon. I will also take some video of our little miracle. She is getting stronger every single day. She is walking everywhere! She even tries to run every now and then! AMAZING!