Author Archives: christygolden

Spinraza update

Callie has had three of her four loading doses of Spinraza. The second and third doses went well, although Callie had to stay overnight the third time around because her vital signs weren’t stable enough to go home.

We thought she would panic when she heard she’d be staying overnight, and her panic would have made her respiratory status even worse. But she actually said she needed to stay. For us, that’s evidence that God provides peace in all our hearts when it’s needed most. She steadily got better during the night and we went home the next day.

She’ll receive her next dose at the end of this month. But before then, she and I are taking a girls’ trip to the mountains (with Nathan coming along as well). Callie and I have a couple books to read together, and we plan to do what we always try to do — enjoy whatever each day brings.

Spinraza dose #1

Callie received her first dose of Spinraza this past Wednesday. Although there were no complications from the procedure, things didn’t go as planned and it was a traumatic experience.

After Spinraza was approved by the FDA a year ago, Nathan and I didn’t immediately pursue the treatment. We thought a lumbar puncture (the required route for the administration of the medicine) would be too stressful for Callie. Her body was so damaged by SMA already, there was not much reason to believe the benefits would outweigh the risks.

Eventually, we decided we should involve Callie in the decision. Yes, she’s only 8 years old, but it’s her body and her life. Anyone who knows Callie, or has kept up with our family, knows she’s terrified of all medical things. But she still wanted to try this procedure, knowing it would be uncomfortable and that there was a chance it might not even work. We were sure to point out that even if it did work for her, she still wouldn’t be able to stand or walk. We explained it might help her swallow better, hold her head up better and maybe breathe more easily.

We knew she would be anxious and upset — that’s simply how it is with most medically fragile children. But after hearing her cry for hours leading up to the procedure, then listening to her scream, “Why am I doing this? I just want to go home!” when the radiologist punctured her back, it was a crushing reminder of why we’ve chosen minimal medical interventions.

Callie will need 3 more doses of the Spinraza before we’ll know for sure whether it will help her. Despite her protests, we plan to have her complete this first cycle, with a sedation plan firmly in place before her next dose to minimize her discomfort.

We’re grateful for everyone’s support and will post more updates during this treatment cycle.

Moving forward

Callie is now a third grader.

Callie with her tea set at the beach

Callie with her tea set at the beach.

Over the summer, she spent her mornings eating waffles with syrup and toast drizzled with honey. She played Minecraft on the PlayStation with her brothers, went to Vacation Bible School, and played on the beach. I’ve also been reading aloud the Harry Potter series to Callie and her brother Peter. With a homemade wand in her hand, she goes back and forth pretending to be Hermione one minute, then Harry the next.

Over the summer we also learned that Callie has received approval for Spinraza, the new and only treatment available for SMA. The drug is administered as an injection into the spine and Callie would receive the injection several times a year for the rest of her life. We’re hoping to start her injections within the next couple of months. There’s no guarantee the medicine will help, so it’s been a difficult decision to make.

Nathan and I are always weighing the pros (potentially seeing a mild increase in Callie’s arm strength) against the cons (an invasive procedure that will require sedation) when we make medical decisions for Callie. We plan to move forward with the treatment because we think there’s a chance it can improve Callie’s comfort and quality of life.

Snack time at Vacation Bible School

Snack time at Vacation Bible School.

One thing that has had a significant impact on Callie’s level of comfort is the rapid progression of her spinal curvature, known as scoliosis. Although we know Spinraza cannot reverse the damage SMA has already done to her body, we recently got X-rays of Callie’s spine in order to examine the extent of the damage, as well as determine if there’s any kind of intervention that might allow Callie to benefit more from her Spinraza treatment.

We learned that a spinal curvature of greater than 40 degrees is considered severe. Callie’s curvature is 120 degrees. After discussing a number of scenarios with a pediatric orthopedist, who regularly cares for SMA patients, we had basically one course of action: Admit Callie to the hospital and infuse nutrition straight into her veins to try to improve her nutritional status. We’d then have to use halo traction, a procedure that would involve putting 12-15 screws into her skull, attaching them to a circular metal device above her head — the “halo” —with weights behind it to slowly stretch her muscles and spine in preparation for surgery.

Then, if she tolerated the halo traction and managed to gain weight, she could have back surgery with a tracheostomy placed. When we told the orthopedic surgeon we’d already decided against Callie having a tracheostomy, he said if we didn’t consent to a tracheostomy, he wouldn’t do the surgery. Based on his experience and research, she wouldn’t be able to come off the ventilator after surgery, so without a tracheostomy for breathing, he would be performing a surgery he knew would be fatal.

Nathan and I have done plenty of research on this topic, so none of this surprised us. But it was still very hard for me to hear. In the few months leading up to this orthopedic appointment, I had allowed my mind to drift towards things that might magically come true, thanks to Spinraza. I’d allowed myself to hope that Callie might even walk one day. Sitting there in the doctor’s office sealed what I already knew to be true — this dream of Callie’s, of our entire family, was impossible.

I’ve had to do my best to let go of that and move forward. After all, as Albus Dumbledore told Harry Potter, “It does not do to dwell on dreams and forget to live.”

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Second grade and summer recap

Callie at school

Callie on the first day of second grade

Callie is in second grade this year. Her nurse during the school day knows her well, and Callie has the same teacher her 8-year-old brother Peter had last year, so the transition has been smooth.

Callie’s spring and summer were filled with activities. The highlights for her were a trip to Carolina Beach in May and Vacation Bible School at our church in July. Callie loves to sing, even though it can make her really short of breath. We love to listen to her sing because she has such a classic, out-of-tune, child’s voice (video below).

This fall, just like last fall, Callie is a cheerleader. At first, we weren’t sure if it was worth the risk because she’s weaker and has more difficulty maintaining a comfortable position than she did last year. But not long before the season began, Callie said, “Why haven’t you signed me up for cheerleading yet?”

There’s always an undertone of anxiety that feels as if it’s settled in our hearts permanently. It doesn’t take much – Callie needing oxygen at night; seeing a tired look in her eyes – for it to rise to the surface. We constantly remind ourselves that this is her life, and no matter how tired she is, she insists on living it to the fullest.

We’re going back to Carolina Beach again soon. Callie and Peter made a list of things to do while there. Some of the highlights include eating dinner on the balcony, watching the sun set, sleeping in the bunk beds, getting up at dawn, eating bacon and eggs, and drawing in the sand. With the exception of getting up at dawn, we’re looking forward to it.

Our 7-year-old

callie-7

Callie turned 7 years old today. Zaxby’s was her choice for dinner, followed by a strawberry cake with a snowman on top.

We’re glad to have reached this milestone. With each birthday comes an awareness that it could be her last. We feel that very acutely this year. We’ve seen some recent changes in Callie’s overall strength and fatigue levels, and her last illness was the most severe we’ve seen in years.

We’re so grateful for the time we’ve been given, and we’re proud of her determination, spunk and kindness.

Happy Birthday to our one and only girl-baby.

Getting back home

As Callie’s overall health has improved over the past couple days, her anxiety has grown worse. Ever since we arrived at the hospital on Friday, she has asked constantly when she would be able to go back home. This morning she started begging for us to take her home. We explained to her that she’s at the hospital so she can get better, and she answered, “But I have to go home. It’s the only way I’ll get better.”

We realize that 6-year-old children can be impatient and demanding, especially when they’re in uncomfortable situations. But over the years we’ve also learned to listen to what Callie’s actions, as well as her words, are saying. It became clear that her anxiety over this hospitalization had gotten to the point that it was affecting her ability to recover.

So today, we brought Callie home. She’s certainly not well yet, but she’s in high spirits and is being her usual little self. We’ll still be dealing with challenges in getting her fully well, but we’re very hopeful.

We are very grateful for the care she received at the hospital but home is simply where she belongs.

Our brave cheerleader

“In my dreams, I can walk.” Those were Callie’s words to Nathan recently as he was getting her out of bed to start the day.

When kids have asked Callie why she can’t walk, she’s always said, “Because it’s the way I’m made.” That’s now been replaced with, “Because it’s the way I’m made……and I don’t like it.”

When Callie was diagnosed with SMA 5 years ago, Nathan and I were determined to do everything within our power to give her as normal a life as possible. We did for a while, but it’s gradually gotten harder to do. We knew she would one day gain the sad realization of how physically limited she is. We knew this disease would destroy her body and eventually take her life. What we didn’t know, and had come to fear in recent months, was that this disease might also destroy her spirit.

Callie cheerleadsBut this month, Callie told us otherwise. For her entire life, she’s watched all her brothers play soccer and flag football for a local Upward sports league. She’s always watched the cheerleaders intently and announced to us that this year, she wanted to be one. If anyone other than Callie had suggested this idea, we would’ve emphatically said no. We wouldn’t want her facing the sadness of not being able to do the things the other girls can do. We wouldn’t want her having to answer the constant questions that come from curious children — “How can you cheerlead if you can’t walk?”, “How do you take a shower?”, “How do you go up and down stairs?”, “Can you go to school?”

But Callie’s brave. She’s not like us. She’s gone to her practices and had her first game this past Saturday. She doesn’t care that she’s too weak to lift her arms in the air, much less her pom-poms, or that she can’t move her legs, or that she’s always a few motions behind the other girls.

When we got to the field Saturday, Callie looked at us and said, “I’ve been waiting for this my whole life!”

 

We’re back home

Callie and "Foxy," her favorite stuffed animal, before heading home.

Callie and “Foxy,” her favorite stuffed animal, before heading home.

Thankfully, Callie had a good night and we were able to come home this afternoon. It’s a big relief since she never stopped asking for her brothers.

She is, however, not very happy about having 8 missing front teeth. She says she looks like a vampire – which isn’t entirely untrue. :-)

Her pain has been minimal and her appetite is starting to come back. Overall, this procedure has gone as well as we could have hoped.

Thank you, everyone, for your caring concern.

Today’s surgery

We’re very happy to report that Callie’s surgery went well. She was breathing on her own not long after leaving the operating room.

After going under anesthesia, Callie got dental X-rays for the first time so that the dentist could determine exactly what Callie needed. As it turned out she had one cavity filled and a total of 9 teeth pulled, including all 8 front teeth. Callie’s jaw is very small, and her permanent teeth were trying to come in but couldn’t because it was so crowded.

The other extraction was in the back of her mouth. That tooth was so deformed that the dentist thinks something went wrong when it was developing.

After the dentist finished, Callie had an uneventful extubation, and she didn’t even need her Bi-pap. She has slept most of the afternoon and evening and remains in the pediatric intensive care unit so that her breathing can be closely monitored.

The funniest part of the day happened just before surgery. Nurses and other health care professionals routinely confirm a patient’s identity. So when the anesthesiologist asked Callie if she was “Callie Golden,” Callie shook her head and quickly said, “No.”

Our hope is that Callie can go home tomorrow. Thanks, as always, for the steady flow of prayers and well wishes.

Dental surgery tomorrow

Callie is scheduled to have her dental surgery tomorrow morning around 11 am. We’ve made some progress at home with getting her to wear her Bi-pap, but she still won’t sleep with it on. After she’s extubated, our hope is that if she’s awake enough to scream about not wanting to wear the Bi-pap mask, her oxygen levels will be good enough so that she doesn’t need it. But we plan to go in armed with books and movies to keep her occupied in case that doesn’t happen.

We will post an update about Callie’s surgery either Tuesday night or Wednesday morning.

As an aside, people are usually interested in how our boys interact with Callie. We always say that they, naturally, adapt everything they do to accommodate her. This video shows just that. Isaac and Ezra had new Star Wars lightsabers, so one afternoon, Isaac decided to get some of the action on video. That turned into him wanting to make a little movie. And you can guess who he decided to cast as the heroine.

(Note: Callie’s glasses are just for play. Isaac and Peter recently got reading glasses, and when Callie had her eye exam, she cried when they told her she didn’t need any. So Nathan couldn’t help but get her a pair of fake glasses to play with.)