The Courteau family kicked off the Summer of 2019 with a trip to California for the Cure SMA conference. It happened to be at Disneyland which was a treat for all of us!  Emma had a blast wheeling around with other kiddos just like her, splashing in the pool with Dad and going on rides.

They returned and bounced into a whirlwind week.  Over Memorial Day weekend they had made an offer on a new home in Covington Washington.  While this house is a little bit further away from Gramma and Poppa, and away from their favorite neighbors in North Bend, it does focus on main story living.  EVERYTHING they need is downstairs, and upstairs has a bonus room and guest room (reservations being accepted now).

Emma stayed with Gramma and Poppa for a few days as her parents got the new house ready.

Emma is very happy, and her mother is too.  No more carrying her up and down the stairs all day!

Emma has continued with her busy schedule of physical therapy, hippotherapy with Lola, water therapy and occupational therapy.  The weeks are flying by.

She had dose #10 of Spinraza at the end of July and is continuing to amaze us with her strength gains.  Her favorite thing to do is to work on standing.  She is one determined girl.

Erin spoke last week for the first time as a Biogen Patient Ambassador and has a few more events lined up for the rest of the year.  And she is the chair for the annual Pacific Northwest Walk and Roll.  Moving away from the grief of their own diagnosis and helping other families has been very healing for her.

If you like to contribute to the EmmaStrong Walk and Roll team goal – you can donate here:  Walk and Roll

Stay tuned…the summer isn’t over yet!

The following is a guest post by Emma’s Gramma Kathy.

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Sometimes your heart speeds up a bit and you feel a surge of hope.

That happened  2 days ago when the FDA approved Zolgensma, the first gene therapy to treat SMA, for type 1 children.  Zolgensma delivers a healthy copy of the SMN1 gene that is missing. A video was published of a little boy that was part of the trial. He was running! AveXis  essentially saved his life.

This first approval was for the dosage given to babies up to age 2. Emma is 2 years 3 months and is not be eligible for this round.  There are more trials in progress for children over 2, that would include Emma. Assuming the trials have the same results, we could be looking at a whole new world.

So my heart speeds up… I feel so much hope for Emma to be free from sleeping with a bipap at night, from enduring the cough assist and suctioning machines, free of the pulse ox on her toe, free from the feeding tube in her little tummy. Do I even dare to hope that she could get into a sitting position by herself, or stand on her own, or walk?

Just to be not scared of losing her to a random cold would be enough.

Here is the link.  You may need a tissue.

Zolgensma Approval Press Release

NBC News Video – Zolgensma Results

Gramma

For a better reading experience, check out this post online.

Our little gem celebrated her second birthday recently and her life’s been full of new experiences, songs, tabby apps and lots of little surprises and accomplishments.

Emma’s started hippotherapy once a week at the Redmond Little Bit Therapeutic Riding Center. As their website describes…
“In (HPOT, from the Greek “hippo” meaning “horse”) the therapist uses the movement of the horse to address the patient’s challenges, such as deficits in balance, strength, endurance, coordination, communication, and attention. This therapy does not teach riding skills; rather the goals are geared towards independence with activities of daily living and increased participation in family, school and community life.”

 

 

And Emma loves it! Note that she’s doing some backwards, trick riding too.

The idea is that as the horse moves, Emma has to counter the movements to maintain her balance. So for every step the horse takes, Emma has to take a “virtual step” so it’s as if she’s walking the distance too. The therapy team is unbelievable and obviously love their work.

Emma also continues her aqua therapy once a week. In the water gravity backs off a bit and lets Emma move much more freely. She’s at the point now where she’s able to stand in the water, almost unaided, with no foot weights.

Emma, the bathing beauty, checking out the surf.

All the therapy is obviously moving everyone in the right direction.

Here’s Emma at home, standing with her AFOs on, showing Mark some of her Tabby apps.

Even Joy, the cat, is impressed!

So between trips to the Seattle Woodland Park Zoo, rides on the merry-go-round or just strolling around the neighborhood with Mom, Emma’s life is pretty full.

 

 

Oh, by the way…. Emma just finally got her gait trainer and so…

…EVERYONE LOOK OUT!

Spinraza dose Number 7 is coming up on April 9th.
Each shot is another step up.

Thanks again for your continued support, help, prayers and
all those Morning Glory muffins for Poppa.

 

Emma’s Mom, Erin, contributed the following post on the eve of Emma’s 7th Spinraza dose.

Enjoy.

Bath time… a daily favorite!

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Emma and I have had a fun week so far. We stopped by my old work at the Thompson Hotel in Downtown Seattle to say hello to the team. Emma amazed them all wheeling around the lobby in her wheelchair, all while snagging a sequined reindeer off their lobby Christmas tree. With Big Boss Amanda’s blessing, we were on our way to see the gingerbread houses at the Sheraton.

Auntie Isabel met us where we saw 6 foot gingerbread villages created by local chefs and architecture firms. Emma made a few attempts to break through the velvet rope to sneak a bite, but was pacified with her own cookie. Emma and I then got to surprise Daddy at work with some cheeseburgers and milkshakes. Everyone’s favorites!

Tuesday was another busy day with a stop at Chihuly Garden and Glass to have lunch with my former Director, Kathy Gerke. A few gifts from Kathy, including Wheedle on the Needle EARMUFFS kept Emma entertained, along with a tater tot every few minutes. She really loved seeing the Space Needle and wishing everyone she met a Merry Christmas.

Wednesday we had our weekly in home PT session with Nicole and worked on a secret holiday art project. Then Auntie JJ and baby Georgie, Emma’s most favorite baby, came for a visit.

Thursday night, with doctor’s approval, we are off to Enchant – a magical light and Christmas display at Safeco field (Editor’s note: Now known as T-Mobile Park).

Friday is the big day. Spinraza #7! As we get further away from her last injection we inevitably see an increase in muscle weakness. These last few weeks Emma has been struggling with her core and torso strength, really preferring to lay down and play instead of sitting, even with assistance. Her little body gets so tired quickly. This shot of muscle juice is just what she needs and she will continue to get them every 4 months indefinitely.

We are asked often if Spinraza is working and the answer is clear on our end: this little love would not be here without it. It is saving her life. It is working. The next question is usually delicately asking about her prognosis. The answer is always: we don’t really know, no one knows. Children who are diagnosed at birth and receiving Spinraza within the first week of life are thriving, they are like any typical child. Emma was diagnosed at 8 months, and that is honestly a little late in the game for a Type 1 child. But, Emma is STRONG and determined and so are we.

Every few weeks we hear of babies and children with SMA that have passed away – no matter how often it happens, it is always a staggering loss for our SMA community. It makes it harder that it seems to come out of nowhere – an aspiration, a quick virus, a respiratory failure. We grieve for these families. And the message they push forward is to not fear the end, to embrace the life you have now. And that is what we are doing. We are not letting fear steal our joy.

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Emma and her family are vacationing in Hawaii this week at the Marriott Ko Olina Beach Club on the island of Oahu.

Photo from Marriott’s Ko Olina Beach Club website

Emma had a surprise welcome to her room!

Somehow Marriott manager Randy Domingo and the Ko Olina Activities crew found out that Emma was coming to Ko Olina. Prior to her arrival they decked out her room in all kinds of Moana stickers, tattoos, and party decorations. There were candy leis and a couple of presents for Emma.

When Emma arrived Moana and Maui were there to give her a huge Ko Olina welcome!

Maui presented Emma with a necklace with the heart of Te Fiti (just a replica, folks!)

Emma was most impressed with the welcome while the Moana theme song played in the background. Mom and Dad were welcomed to the island with some beautiful orchid leis.

Emma got a great Moana swimsuit that was just her size…

Maui watched as Emma got into more presents…

Then it was time to thank everyone as all the attention began to sink it.

Time for one more quick shot with Moana, Emma, Mom and Maui…

And then it was time for everyone to go, as Emma puts it … BYE, BYE, BYE!

Then it was all over, but for one final welcome to Hawaii for Emma…

Many, many thanks again to Randy and his Ko Olina crew.

They’re just one more great example of what makes Marriott special!

This past Sunday, 8/19, was the 11th annual CureSMA Pacific Northwest Walk-n-Roll Day. It was a day of fun, events, games, raffles and a short walk/trot/roll/saunter around the Auburn Game Farm Park.

Here’s a shot of the whole Pacific Northwest Walk-n-Roll team.
See if you can pick out your favorite people.

Team Emma Strong signed up and committed to raise $2,500. They blew through the goal and ended up raising $5,210 for the CureSMA cause!

You can check all the fantastic people who donated here.

Emma’s day started with a bit of on-the-floor-with-Dad time, which is always a great way to start the day.

Then it was off to the park to meet up with her team.

She was a bit worried about all the attention, but carried on like a real trooper.

There were balloon magicians, ….

The dog, Charlie Tegtmeyer, to pet (maybe)…

Grandma Caralee and Aunt Shelly to hang with…

Finally, it was time to start rollin’… bowling, that is! This is Emma’s unique, but effective approach to knocking down the pins.

And then it was time to really get ROLLIN’!

Everyone had a GREAT time, for a GREAT cause.

Then it was time to hurry home and update Emma’s blog on her “Tabbie”….

And then enjoy some quiet time to contemplate the day…

Thanks again to everyone who donated to CureSMA on behalf of the Emma Strong Team!

Last week Emma and her parents and Poppa Jerry and Grandma (Mema?) Kathy attended the 2018 CureSMA conference in Dallas, Texas. Most of you know that CureSMA is a foundation dedicated to funding SMA research, SMA education, and supporting caregivers impacted by SMA. It was a hectic, but rewarding 4-day conference.

It was quite an ordeal getting all  of Emma’s travel gear to the airport, through security, onto the plane, off the plane, onto the Hertz bus, into the mini-van and to the hotel. Whew!

Not too happy with TSA, on the plane with Poppa and Grandma, with her “headset” on and reading to Grandma.

And No! Emma didn’t sit between them… just while everyone was boarding.

The conference was incredible! Over 1,700 attendees; kids scooting around, between and sometimes over people; the Jadewaters water park… even just the hotel was very, very  impressive.

During conference breaks, Emma floated around the water park’s lazy river float trail about 100 times over the four days of the conference. She freaked out the lifeguards as they had never seen a little kid floating by with a neck collar flotation device. I think Mark enjoyed himself as well.

Hmmm… good water, Dad!

Back at the conference there were updates from the 18 or so research and clinical trials that are underway pertaining to SMA. They ranged from gene therapy, to gene editing, to muscle restoration, motor neuron rejuvenation to genetic testing and forecasting. The overall approach that they’re all taking is to develop drug and other therapies that will

FIRST SLOW….   THEN STOP….   THEN CURE SMA

There were breakout workshops on topics about nutrition, PT & OT, caregiver support, early education through public schools, legal and estate planning for families with SMA kids and many other topics.

There were demonstrations of the latest technologies in motorized wheelchairs, robotic arms and genetic testing packages.

The conference is very unique in that it’s really two conferences running parallel to each other. It’s an SMA scientific update conference where researchers from around the world meet and discuss breakthroughs and new ideas. While a second conference for us “common folk” has less scientific, but more life-specific sessions. It really gives the medical and scientific researchers a first-hand understanding of the impact this terrible disease has on SMA patients and their loved ones.

While Emma’s parents attended the sessions, she thoroughly enjoyed herself in the SMA childcare lounge. It was one-to-one babysitting. By the last day, the caregivers were competing to see who got to take care of Emma.

Wheeeee!

The highlight of the conference was Thursday night when there were numerous wheelchair races around an obstacle course between SMA kids and the researchers and medical staff. Of course, the kids won every race, frequently lapping the poor researchers who struggled to complete the course in their loaner wheelchairs.

After the races there was lots of “all-inclusive” games for everyone, snacks and photo ops.

Emma’s not too sure about this particular cowboy/horse combination.

And you should have seen these kids zooming around freaking out their poor parents and many of us innocent bystanders.

Friday we had some time off so we piled into the van and toured Dallas. Turns out the city has changed a bit over the 30 years since we lived there. Eventually we found our old house on Whitehall Drive and then stopped by the Mustangs at Las Colinas for a photo with the ponies.

Friday night was DANCE NIGHT! You haven’t lived till you’ve found yourself in the middle of a dance floor surrounded by twirling, swirling motorized wheelchairs… some with robotic arms that they used to pirouette around the floor.  Others had a slightly tamer approach to the dance as you can see below.

Gettin’ down with Dad!

All in all it was an exceptionally valuable conference to attend. Mark and Erin got to meet and spend time with lots of other SMA parents; Kathy had ample opportunity to spend time with Emma and Jerry got his fill of good, technical background information.

There’s more about the conference here with lots more detail and pictures.

And don’t forget to support Team Emma Strong in the 2018 CureSMA Pacific Northwest Walk-n-Roll event.  As of today, Team Emma Strong is in second place having already raised  over $800 for CureSMA. You can donate to her team here.

Yesterday, May 1st, Emma had a Gastrostomy feeding Tube (G-tube) “installed”. The procedure was done under anesthesia after the Seattle Children’s team did a little snooping around to check out a small shadow on a recent x-ray of her tummy area. The endoscopy showed that it was nothing more than a narrowing of her duodenum… nothing to worry about.

Emma already relaxing after her surgery.

Emma (and Mom) will spend a couple of days in the pediatric intensive care unit (PICU) but already, as of the morning of May 2, our little heroine has a new look.

Watching the Lion King… Hey!  Something’s missing!

Anyway, all is good.

The night before Emma’s G-tube surgery Mark and Erin arranged with Jeanna Rice of Jeanna Rice Photography to take a few photos of Emma and the family.

Enjoy!

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On Monday, April 23rd Emma was scheduled for her first Spinraza maintenance dose… and she was ready!

“Hi everyone!”

You may remember, that she received 5 loading doses beginning last Halloween.

April 23rd was special as it was her first maintenance dose. That dose is identified by many parents as a “telltale” event that appears to accelerate developmental improvements.

So early afternoon, Erin handed Emma over to the Seattle Children’s injection team while she was sound asleep (Emma, that is). Next time Erin saw her, in the recovery room, she was crying inconsolably (Emma and her Mom, almost).

We are NOT happy!

Apparently Emma woke up soon after Erin turned her over and the team had great difficulty keeping her still. The first lumbar attempt missed the mark as Emma was kicking around so much. But they nailed it on the second attempt and administered the dose.

As usual, Erin wasn’t allowed into the recovery room until the team completed all their health checks of Emma after the injection. It was a LONG wait!

It was good news and bad news from the medical team. First, the bad news… Emma is so active now (kicking and moving) that for the next injection (in August) they’ll need to do it with her under anesthesia. Of course, the good news is that she’s now so active that they have to do that!

I know… grasping at straws.

Anyway, after a bit of quiet time with Mom and a couple show-stopping stink-eyes for the recovery nurse, Emma began to return to her normal, happy self.

Getting better…. HAPPY again!

And finally, it was time to get ready to head home.

We’re outta here!

But Emma won’t be home for long. She’ll be back at Seattle Children’s next Tuesday (May 1st) to get rid of the pesky nasal-gastric tube and have a more permanent G-tube placed directly into her tummy. She’ll likely end up in the PICU again, but the family hopes she can be home by the weekend.

Stay tuned.

When is a cold, not a cold?

When it’s pneumonia.

Middle of last week  the sun was out, the temperatures were almost balmy and Emma enjoyed lots of walks around the neighborhood.

Nothin’ better than being out for a walk while enjoying lunch.

But this past weekend Emma started coughing, sneezing and became very lethargic. Her parents were already sick with bronchitis and walking pneumonia. Fortunately they had Grandma Kathy out there to take care of everyone.

Early Monday morning Seattle Children’s Hospital asked them to bring Emma into the Emergency Department (ED). Her O2 levels had been fluctuating too much and they were concerned. She had just endured her second sleep study in 2 weeks and now she was back, under observation again.

How is someone supposed to sleep with all these connections?

They hooked her up again and Emma alternated between suctionings (where they use a  tube connected to a Mucus Suction machine to draw all the mucus from her sinuses, throat and lungs) to chillin’ with Grandma.

Sporting the latest in pulse/ox toe monitors and green sock IVs.

All day Monday Emma would be suctioned, be happy and play a bit, tire quickly, fuss a bit then nap some more.

Late on Monday, she was moved up to the Pediatric Intensive Care Unit (PICU)… they stressed that it wasn’t because Emma was in any imminent danger, but that the PICU was better prepared to deal with her frequent suctionings. We think that that was a grand attempt to make the family feel better about having Emma in INTENSIVE CARE.

Emma with her Personal Penguin heading up to the PICU.

In the PICU Emma was suctioned every 4 hours for the first 48 hours and had to wear her BIPAP for 23 of every 24 hour period. She would eventually get to the point where she would sleep soundly with the BIPAP pumping away, only to be woken up after 4 hours, suctioned out and then patted back to sleep.

It was hard on everyone too, because there wasn’t much time to hold, rock or play with Emma in between sessions.

Dad doing his “Lion King Emma presentation” for the hospital staff.

Tuesday passed with suctionings going from 4 hours, to two hours… the wrong way. Emma developed a bit of digestive upset and the staff wasn’t sure if it was because of the change in her formula or the pneumonia causing a lot of fluids to “go the wrong way”.

But Emma kept sleeping well with her BIPAP on, watched a couple of in-room movies and had some good snuggles with Penguin, Mom and Dad.

 

Late Wednesday (yesterday) Emma was able to have better (near naked) snuggles with Mom.

Hmmmm…. much better!

And, despite regular suctionings, is getting closer to her old self.

Now if I can just yank this off, my pink toe will go away…

The family is hoping Emma will continue her progress, with less frequent suctionings, and maybe even get to be home to see what the Easter Bunny brings.

We’ll keep you posted.