Rehab Clinic

Wednesday Taye and I headed to Seattle for his rehab clinic checkup. There is a new director of the clinic, Dr. Apkon. I must say I was pleasantly suprised! I really, really liked her and she is super knowledgeable about SMA.... she has even worked with Dr. Swoboda and is on the FSMA advisary board! She seems to be pretty aggressive with treating SMA and suggested that we keep our eyes open to get Taysen into another clinical trial.. Since he is doing well on VPA we would want to make sure he would be allowed to stay on it for a trial, as many of them have exclusions . I will surely take her advice and keep my eyes open. reading books while waiting.

Taysen is at the age where they start measuring lung function with PFT's (pulmonary function test) He has done it the last 2 times now, and is not very consistent so we didn't get too much info... other than he is for sure above 75%. I think that's bad.. his pulmo thinks that's "pretty good" he always says that though, so that doesn't mean too much to me. Really looking forward to our appointment with Dr. Schroth (pulmonologist) in Madison to get some really detailed info about his current lung function and what we can do about it.
Taysen had to do a lot of waiting. He got bored. He decided to color on himself. He then told everyone who came into the room that he was a tiger and to look at his tiger stripes (growl)

admiring his artwork


What a long day.. we usually have treats after appointments. Kind of a tradition I guess.

and this is my favorite picture - talking to River about his day after a long day apart :)

Also, I heard back from U of Utah already and it turns out that sweet little River is not even a carrier for SMA! I was thrilled to hear this, although I certainly hope (expect?)that by the time he has children of his own, carrier status won't matter b/c SMA will have a cure by then. wishing. hoping. praying. believing. Thanks for checking on my shining light.

Gardening

Our first... and hopefully not our last... attempt at gardening. We planted a week ago and everything is still alive. The boys had a great time helping, "shobeling" and looking at worms. Thanks Gigi and Grandpa for your help!

River and his "shobel" Rivies loves to help around the yard. He's a real workhorse too! He will shovel, rake, sweep.... "can I hewp you mommy?"

Salt Lake City #4

Taysen made his 4th trip to Salt Lake City, Utah to see his SMA specialist Dr. Swoboda. May 07 we made our first trip there to get Taye started on Valproic Acid/Carnitine and we go back every year for a check up. We arrive on Wednesday, and stay in the hospital. Thursday Taysen has an EMG and DEXA scan. Friday he has Physical Therapy where he is scored on things he can do, and also on manual muscle testing and range of motion. I love Utah, it's so beautiful there. The hospital staff is amazing and my boys actually look forward to going every year!
Thursday morning was Taysen's EMG. Of course his sidekick is right next to him through it all. here they are watching a DVD while waiting for the versed to take effect.
Taysen's MUNE (combined muscle and nerve response) was 5.3 this year. Pre VPA Taysen's MUNE was 2. One year later it was 3.4, and last year he had a huge gain and it was 5.6. My first reaction was panic.. it's a huge fear of mine that the drugs will stop working before something better comes along and we will be stuck sitting here watching this disease progress and unable to do a thing about it.
After talking with Dr. S I do feel a little better.. she said he had some collateral innervations which is good, and she thinks his number went down a bit due to the massive growth spurt Taysen has had and the amount of stress that puts on his weak little body. I know she is not the type of Dr. to blow smoke so I really hope this is the case. She said there is only so much that HDAC inhibitors can do (VPA is one of those), so even maintenance at this point would be good. I am so ready for a stem cell cure.. Trials are set to start on type 1's soon, so i just hope we can hang on so that Taysen will be able to receive some type of benefit from them. Like it or not people, stem cells cure diseases and my hero deserves a cure. like 4 years ago.

You can see the electrodes on his hand and also the tool she is holding delivers the shock to the muscles which shows the response on her screen.
She felt Taye's back looked good, no scoliosis, and his joints were all exhibiting normal range of motion. She noted that he is not really expanding his rib cage when breathing so we reiterated the importance of breathing support especially while sick, and also consistent use of the cough assist.

Tayesn's DEXA scan showed a significant improvement in lean muscle mass. This is great news, since that number has steadily declined the past few years. I have to say that this is from his swimming, since that is the only thing we changed. Taye continues to swim about 5 nights a week here at home and loves it!. He also had an improvement in bone density (from all the standing) which is great since these kids tend to have very brittle bones that break easily.

Here he is getting his DEXA scan. He has to lay completely still. At this point the versed has worn off, he is so brave!
We also tested River for SMA. Usually in families, kids will have the same type, but she said that she does have some families w/ type 2 and 3 -in which case River may not be presenting with symptoms yet so we felt it was best to get him tested to cover our bases and also to find out if he's a carrier for when he has kids some day.
I am so thankful that Taysen is one of her kids because there is such a HUGE difference in knowledge between a specialist who sees and aggressively treats SMA kids every single day and our doctors at Seattle Children's.
We will go back and see her in 6months-a year... It depends on how he does. Next on the agenda for Taysen is a pulmonologist/MDA check-up at Children's and also another blood draw. She couldn't get enough blood from him and after 4 pokes we gave up. I can't stand listening to him scream and beg them to stop hurting him.. YES it has to be done but also IT SUCKS AND IT'S NOT FAIR that my boy has to go through all of this crap. And YES, I'm allowed to feel that way from time to time. I am THANKFUL for my son and PROUD of him for all that he is.

here he is racing down the halls..... that boy loves to "run"