Double digits!

Callie’s had a busy life since we last posted in the fall.

She’s continued to enjoy having a baby-girl cousin. Callie has regularly managed to claim first dibs on any holding and feeding of the baby.

Callie and Peter in the snow. We got about 15 inches in December.

We had a great Thanksgiving with our families in South Carolina, and Callie got her requested Lalaloopsy oven, a modern version of an Easy Bake Oven, from Santa.

We also had a really big snow in mid-December. Callie has always loved snow. She insisted on being outside as much as possible. She even went sledding. Things like that make me incredibly nervous, so I’m grateful that Callie has Nathan, who’s much calmer than I am. Otherwise, Callie would have fewer experiences that fall into the thrill-seeking category of life.

Callie and Peter spend a lot of time together.

Callie had a couple of health scares in December and January. We were never able to determine an exact cause. She didn’t have a cold or pneumonia. She was just extremely tired, had trouble with aspiration and began experiencing apnea (a pause in breathing) while she slept. Fortunately, adjusting her Bi-pap settings directly addressed this complication, and we haven’t had any trouble since then.

And on February 5, Callie turned 10 years old. This is a milestone many people, including us, weren’t sure she’d reach. At the same time, it’s difficult to look back and see how strong she used to be. She’s worn her Bi-pap all night for several years, but as recently as October she didn’t need it for the majority of the day. Now she wears it anywhere from 20 to 22 hours per day, and it seems she wouldn’t survive more than a day or two without it.

Callie's picture of our cat Gracie — Callie painted a portrait our cat Gracie. Gracie wasn’t terribly impressed.

Callie’s not one to be slowed down or underestimated. She loves painting, homemade Rice Krispy treats, baseball, our new local ice cream shop, Wonderful Wednesday at our church, and Dragons: Race to the Edge on Netflix.

Occasionally she lets some of her heartache come through: Like a few weeks ago when she said, “If I could ride a bike, I would get one with a basket on the front. I’ve always thought it would be fun to carry things in it.” But she always comes around to embrace the brighter side of her life, and everyone around her can’t help but to follow her lead.

An autumn update

Early last week, we took Callie to see her pediatric palliative care doctor so we could discuss some problems Callie has been having the past couple of weeks. Callie had become extremely fatigued, needing naps and asking to wear her bi-pap during the day. Callie’s stomach was also hurting when she ate, but she didn’t have any other symptoms, such as a fever or cough.

Visiting Hogwarts at Harry Potter World in Orlando.

Callie naturally has some days when she’s tired, especially after having a lot of activity. We’d been to Harry Potter World with my parents as a special trip, but that was back in October. And despite all the wand waving, playing in the pool, and traveling, Callie had seemed okay during the trip.

As we were telling the doctor how tired Callie had been, she and Peter were up to their usual antics — pretending, laughing, putting on plays with the owls they’d just made out of construction paper with a volunteer in the waiting room.

On previous visits to this doctor, Callie hadn’t had any of her brothers with her. She cried and was apprehensive the entire time. The doctor enjoyed seeing this side of Callie — the side we see so frequently and the reason we were there for that appointment: We don’t want to see that joy diminish.

Callie with her new cousin, Olivia, the daughter of my younger sister and her husband.

Peter and Callie told the doctor all about their new baby cousin, Olivia. Callie was thrilled the new baby was a girl, and she immediately inducted Olivia into our “Girl’s Club,” which until then consisted of Callie, me, and our girl kitty. Peter wanted me to show the doctor the picture of when Olivia, at 6 days old, reached out to touch Callie’s face. The doctor was appropriately impressed, which only widened Callie’s smile.

Eventually we started discussing the reason for our visit. The doctor thinks Callie is probably not maintaining sufficient oxygen levels because of the compression on her lungs from her worsening scoliosis. As a result, she’s building up carbon dioxide (CO2), which causes sleepiness. The stomach pain is still mysterious, but we suspect it ties into the respiratory problems as well.

We developed a plan: more cough assist and bi-pap to help Callie clear more CO2 and an adjustment to her medication to help with the pain. So far, between keeping Callie on her bi-pap essentially all day and night, and adjusting her medication, we’re seeing an improvement in her energy level, appetite, and pain. In the meantime, the doctor said she would be reaching out to other specialists to see if they have any other theories or ideas.

Callie’s continuing to live the life she enjoys and is looking forward to visiting our families for Thanksgiving. Being the youngest of four children, she’s used to sibling arguments and having to present her case for what movie to watch, where to eat or whether to play with Lalaloopsy dolls or army men.

So she’s very pleased that she managed to “call” holding Olivia first, knowing her brothers will simply have to wait their turn.

Wrapping up another year

Callie with one of her best friends, Ella Rose.

Callie just finished third grade. It was a good year, with the exception of shedding tears over math.

The kindness of children who are in Callie’s life always amazes us. Everyday, a group of girls has recess inside with Callie instead of going outside to run around on the playground with other classmates.

The girls play with mini Lalaloopsies, draw, or play teacher together. Friendship is one of the biggest blessings in Callie’s life.

Callie and her teacher, Miss Packer.

There were bumps along the way this school year. In December and January, Callie completed the four loading doses of Spinraza and we ultimately decided not to continue with the treatments.

Nathan and I didn’t see any improvement in Callie’s strength or stamina, and we were putting Callie at risk with every treatment, which was administered by spinal injection.

We have always believed Callie should have a say in decisions that affect her life. After asking her on several occasions if she was sure she wanted to stop the treatments (something she’d been begging to do after the first injection), she finally said, “I can’t do that [Spinraza] for the rest of my life.”

It’s her life to live, and we’re grateful for the peace God has given her heart.

In February, after her ninth birthday, Callie got what we think was the flu with recurrent pneumonia. She stopped eating and, for a couple days, was withdrawn and not very interactive.

After an uncertain couple of weeks, Callie began improving, and we’re grateful for the additional time we’ve been given with her.

As always, Callie is making the most of her life. She’s looking forward to a busy summer:

We recently got a trailer that hitches on to the back of Nathan’s bicycle for Callie to ride in. Not being able to ride a bike has always been one of Callie’s greatest heartbreaks, but she was determined to do the next best thing. She loves it.
We had Vacation Bible School this past week, something that’s always one of the highlights of her summer.
The minute she heard about a cheerleading camp, she wanted to be signed up, even though it’s a new place where she doesn’t know anyone.
We’ll be making a trek to the beach where she’ll no doubt want to stay outside all day.

So many people help give Callie the high quality of life she has.

Callie with her nurse, Alisa.

Her school nurses, Alisa and Marsha, enable Callie to attend school regularly. Her teacher, Miss Packer, encouraged Callie through her tears and all.

Callie’s older brothers play such a significant role in her life. The grief of her diagnosis has gotten only more intense for them as they’ve gotten older.

Callie with her nurse, Marsha.

But they’ve never distanced themselves from her — on the contrary, they play on the beach, pull her on a snow sled, have tea parties, visit Callie’s made-up universe called Twintopia, and cheer her on in everything she does.

Like any other child, Callie will get her feelings hurt or feel left out. She can get so tired that she can’t play like she wants to. But it never stops her from embracing life.

We take each day as it comes, and as long as we’re seeing her smile, everything feels okay.

Callie and her brothers at Carolina Beach.

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.

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.

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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Happy 8th birthday Callie!

Callie enjoying our big snowstorm last month.

Callie is 8 today! She’s very excited about the big day, just like any 8-year-old would be.

Days like this remind us how normal our lives are, despite the time and effort Callie’s care requires. We have support from so many people — nurses, people who have given money, Hospice of the Piedmont, co-workers who generously prepare a meal for us every week.

We are grateful for this help because it helps Callie live a life that is not defined by her diagnosis. Life seems so normal that, sometimes, when someone asks how Callie is doing, it takes us a second to recall the details of her daily challenges.

This sense of normalcy has been disrupted over the past six weeks, ironically, by a piece of very good news. The FDA on Dec. 23 approved a treatment for spinal muscular atrophy. The drug, Spinraza, could be available for Callie this spring or early summer. Had this drug been available when Callie was younger, it might have rescued her from the devastating effects of SMA.

Now, though, SMA has twisted her spine. She has contractures, subluxations, almost no muscle mass. She is weaker and more vulnerable than ever. She can sit up for shorter time periods. She depends on her bi-pap machine for breathing support every day. This medicine has arrived too late to prevent these effects for Callie.

But her spirit is so strong, and we’ve learned not to underestimate Callie’s determination. So over the past few weeks, we have been considering whether this new treatment could help Callie even in small ways. We know it won’t reverse the damage SMA has done to her body. But could it help her stay in her power wheelchair longer, giving her more independence? Could it give her enough strength to sit up longer, which could enhance her quality of life by allowing her to interact more with friends and family?

callies-cake Or would the treatment only slow the progression of her condition, requiring her to endure more pain with little or no benefit to her quality of life? There are no simple answers.

Next month we have an appointment with a neurologist, and we’re hoping he will provide some more information to help us determine whether this new treatment would be good for Callie.

Until then, she’ll celebrate her birthday with food from Barberito’s, one of her favorite restaurants, and a cake with a snowman family on it that Callie helped design. This coming week she’ll go to school hoping the cafeteria will serve her favorite lunch, beefy nachos. She’ll watch her favorite shows, Sofia the First and Phineas and Ferb. She’ll draw pictures, tell jokes, and live her life.

Second grade and summer recap

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 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.

A progress report

Callie is thrilled to be home, and although she still has a long way to go, we began to feel optimistic today that she would be able to recover from this bout with pneumonia.

She is still too weak to breathe on her own for very long. She depends on her Bipap machine for breathing support. She also could not survive without the machine that helps her cough.

Today while resting in bed her cat, Gracie, jumped into her bed to curl up beside her. Callie was glad to have the company.

We expect it will take a few weeks for Callie to return to her full strength, but things seem to be going in the right direction.

Once again, we are grateful for the love, support and prayers that have been shared with Callie and our family during this illness.