Every 6 months or so Seattle Children’s Hospital has what they call “SMA Clinic”. That day all the families with SMA kids come in for an afternoon of clinic appointments. Yesterday, January 10, 2018 was Emma’s first one. She was accompanied by her parents, Mark and Erin and her local grandparents, Jerry and Kathy.
The day started off with an informal luncheon “meet & greet” where the families get to spend time with each other and get caught up on each family’s triumphs, setbacks, accomplishments and news. It’s really neat to meet face-to-face with families that we’ve been corresponding with on social media.
We caught up with Audrey and her Mom, Stephanie. Audrey was sporting an incredible tutu for the day.
Isaiah brought his Mom, Anastassia who helped Isiah show off some of his upside-down gymnastics.
Emma wasn’t sure she wanted to participate in that!
Ummm…. Dad, DO NOT do that to me!
After lunch, each of the family teams checked into their own examination room for the afternoon. Over the next 4.5 hours ten or so specialist teams came in to meet with Team Emma. They spanned the areas of Physical Therapy, Occupational Therapy, Nutrition, Rehab, Pulmonary, Social Work, Genetics Counseling, and an MDA rep.
First Emma had all her vitals checked. She’s put on almost a pound since she started on tube feeding and has moved up to 55th percentile on the weight charts.
The PT folks ran her through the Children’s Hospital of Philadelphia’s Infant Test of Neuromuscular Disorders (CHOP INTEND Whew!) This was the same test that Emma took before her Spinraza treatments began so the results will be a great measure of her progress. We’ll get the results in a couple of weeks and will let you know. There was lots of very positive feedback on how far Emma’s progressed. She did great, although she did need a some encouragement from Dad and Grandma to “strut her stuff” for Dr. Sarah.
Oh… OK… You want me to reach for Kitty!
The OT specialist talked with us about the various devices and technology available through the hospital and donating organizations that will help Emma as she grows and deals with real life. It’s a pretty incredible list: everything from neck braces for Emma to support her head while Mom’s driving around town; to play trays and “tomato” chairs; to support vests to help Emma maintain her posture.
Then Dr. Alexis from the Rehab team spent some time reviewing Emma’s Spinraza treatments history, discussing next steps and, of course, playing with Emma a bit to see for herself how far Emma’s come along.
It had been a long afternoon already and Grandma Kathy had to make a run to the hospital cafeteria to buy more food pouches for Emma and snacks for the rest of us.
Hmmm… Good!
Next was Dr. Cam who reviewed Emma’s nutrition specs, answered a bunch of questions about Emma’s tube feeding process and schedule. She made a number of suggestions as to how Mark and Erin can move Emma up to the next feeding plateau.
Then it was a visit from the SCH Social Work team. They offered all kinds of help and support for various logistical and social challenges… everything from getting Erin a Handicapped hanger for her car to making arrangements for a service dog for Emma when she grows up a bit. There are almost too many options and the choices are sometimes overwhelming.
A quick visit and update from Kiko, the scheduling nurse and then we saw the local hospital rep from the Muscular Dystrophy Association. We talked about all the different volunteer opportunities we’ll have once we get some spare time back.
Next up was the Hospital Genetic Councillor, Dr. Katie. She reviewed the genetic process leading to SMA and explained how genetic engineering is making huge strides in gene manipulation to cure diseases like SMA. She also answered many questions about how genetics might work for future offspring and then captured all of Mark and Erin’s sibling data and hereditary information for future reference.
The last team was Dr Chen and the Pulmonary team. Emma had been having some issues dealing with her BI-PAP and Dr Chen was able to answer all their questions and had a bunch of great suggestions. She was very complimentary of how well Mark and Erin have been adapting to Emma’s nighttime challenges. We thought we were home free but Dr Chen wanted to show us how Emma’s Cough Assist machine would work. It’s essentially a stronger BI-PAP machine that will PUSH air into Emma’s lungs, then strongly PULL air along with phlegm, mucus and other nasties OUT! They set the inhale/exhale pressures, then pressed the mask to Emma’s nose to see if she could activate it. Boy, did she ever! What we didn’t realize is that if there is no phlegm, mucus and other nasties present, then the sucking out part… really sucks! Emma let us all know how well her lungs worked right after that test.
Then it was time to pack everything up and fight the traffic home.
Emma was up till about nine o’clock playing with Dad, then slept all night.
The whole day was like drinking from a fire hydrant,
but it was a great experience.
EmmaStrong2017 