Category Archives: Daily life

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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Happy 8th birthday Callie!

Callie enjoying our big snowstorm last month.

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-cakeOr 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 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.

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.

callie-gracieToday 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.

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!”

 

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.)

New problems

We’d like to thank everyone who has prayed for Callie and our family as we’ve dealt with her back pain. We were referred to a palliative care clinic at Brenner Children’s Hospital that deals with medically fragile children. Managing pain is one of their specialties, so we’ve been trying several combinations of medicines to try to find a magic solution.

A new, much bigger concern we have is that Callie needs dental surgery. For many people, this would be a basic, outpatient procedure. But with Callie’s high aspiration risk and her inability to protect her airway, this procedure will require general anesthesia, and she’ll have to be admitted to Duke Hospital.

Callie at the beach earlier this month.

Callie at the beach earlier this month. (Photo by Debbie Bagwell.)

This presents a few challenges for us. First of all, Callie has major anxiety related to anything medical. She loves her pulmonologist, Dr. Kravitz, yet still cries and begs to go home constantly when she has appointments. Another challenge is the fact that when she’s taken off the breathing machine after surgery, she’s likely to need her Bi-pap machine – which she hates. So we’re working with her now to try to get her to tolerate it.

We saw Dr. Kravitz last Friday, and as always, he’s already planning. As soon as he heard from the dentist that Callie needed dental work, he contacted one of the pediatric anesthesiologists and a doctor who leads the pediatric intensive care unit (PICU). Dr. Kravitz likes to plan for every worst-case scenario, then hope for the very best. Having him around makes us feel a lot more secure. He says that hopefully, she’ll wake up in recovery, need her Bi-pap minimally, or not at all, and be out of the hospital in 24 to 48 hours.

The procedure is scheduled near the end of August. For the past few weeks, and likely for a number more, we’ve felt overwhelmed, stressed and worried. We’ve seen some things worsen quickly, such as not being able to ride in a car seat more than 15 to 30 minutes without having to be repositioned due to pain and discomfort. Some things we can’t really pinpoint.

On a positive note, we had a wonderful trip to the beach a couple weeks ago. Callie loved sitting in the water, looking for sea shells and playing in the pool. It was nice to have such a good respite before the chaos of school begins.

Questioning our decisions…again

Callie has been doing well recently. She, of course, loves school and thoroughly enjoys her classmates, teachers and nurses. But one issue we’ve been dealing with the past couple of weeks is back pain. Callie has scoliosis (an “S”-shaped curve in her spine) and kyphosis (another curve in her spine that results in a “hunchback” look). This is nothing new, but it might be becoming more of a problem. Our back muscles hold our spine in place, and if the muscles weaken, the spine can’t remain straight.

As Callie continues to grow, the curves in her back will get worse. This affects her ability to sit, breathe and hold up her head. Callie’s back pain was so constant and severe that we took her to get X-rays last week. We were concerned that she may have fractured part of her spine, simply due to bone weakness all children with SMA have. Fortunately, the X-rays came back negative and we’ve been able to control her pain with medication.

callie-in-wsAlthough we have chosen a palliative approach to Callie’s care, which focuses on comfort and quality of life over procedures that will prolong life, Callie’s pain was severe enough for me to start researching surgical options, particularly rod placement, for correcting the curve in Callie’s spine on the chance that it might add to her quality of life.

Although I had some hope, in the end I found what I expected: Surgery isn’t recommended for children Callie’s age. And although rods have been placed in children Callie’s age and even younger, the long-term effectiveness is still unproven. In addition, there are always risks for infections, respiratory, nutritional and orthopedic complications with this surgery. I also discovered that the non-invasive approach to scoliosis, a back brace, doesn’t prevent scoliosis – it doesn’t even delay the progression.

Surgery would put Callie through a major procedure that has no scientifically proven benefit, cause her tremendous pain and put her at risk for developing serious complications.

This disease is relentless. I wish we could save her from it, but we can’t. The past few weeks have provided yet another reminder that Callie will be healed someday, but it won’t be here.

Six!

callie-6Yesterday, we celebrated Callie’s sixth birthday. She took mini-cupcakes to school and for dinner, Zaxby’s was her restaurant of choice. Her favorite presents were mini Lalaloopsy dolls – a perfect gift for children with muscle weakness – and a tiny, shelf-sized guitar.

callie-guitarDespite a couple illnesses, Callie’s health has been good overall. She’s determined and continues to excel in school, despite needing frequent breaks. Her teacher and nurse are wonderful and regularly adapt activities and play time so Callie’s always included.

Birthdays tend to be bittersweet for our family. It was around Callie’s first birthday that she wacallie-dollss diagnosed with SMA. With each year, we’ve watched her personality and intellect explode with curiosity as her body has grown more and more fragile.

We’re grateful that Callie has reached this birthday, and we are looking forward to the next.

Our school girl

Callie started kindergarten at the beginning of August. It was a very exciting, stressful and emotional transition. It was hard to leave Child Enrichment at First United Methodist Church. And it was especially difficult to leave Ms. Emily, who’s been with Callie, essentially one-on-one, for the past three years.

We always knew Callie was safe and happy with Ms. Emily around. We could never thank her enough for all the love and protection she gave Callie.

Callie at the fair

Callie enjoys a magic show as her kindergarten class visited the Dixie Classic Fair in Winston-Salem earlier this month.

Everyone who knows Callie was apprehensive about her going to kindergarten. It’s not easy for people to reconcile that a child who has normal cognition and an outgoing personality can be so medically fragile. We had to work for several months to get Callie a one-on-one nurse at school. Callie requires frequent vital sign checks and constant attention to her fatigue and respiratory status, as she can decompensate quickly.

Callie doesn’t trust people easily, so it was a relief when we realized that Callie’s kindergarten teacher, Ms. Madison, and her nurse, Ms. Tee, were going to work out wonderfully. The administration and staff at Callie’s new school have also worked hard to help make her transition to kindergarten a success. Callie instantly made friends in her class and by the third week of school, she could attend the entire day without Nathan or me there.

Earlier this month Callie’s class had its first field trip, and Callie was very excited to ride the school bus for the first time as her class traveled to the fair. It’s great to see Callie fully participate in activities with her classmates.

Callie has a full life, and kindergarten is a big part of it. Making that possible requires the time and expertise of a variety of people: teachers, therapists, nurses, custodians, friends, administrators, lunchroom staff. There are people we have never met who take care of details that make school possible for Callie. We are grateful for everyone.