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.

Make-A-Wish concert

Several weeks ago, we had the privilege of attending the second annual Make-A-Wish benefit concert hosted by Elon University. The concert featured a number of a cappella groups from North Carolina and was organized by Nick Cook, a student at Elon, as well as a member of one of its a cappella groups, Vital Signs.

Nick and Bella

Nick with his sister Bella at the concert.

Nick has a 6-year-old sister, Bella, who has Down Syndrome and was diagnosed with acute lymphoblastic leukemia when she was 3 years old. (Bella’s now in remission!) Not long after her diagnosis, their family took a trip to Disney World to celebrate Bella’s life. Their family had the time of their lives at Disney, and it was Make-A-Wish who made that possible.

When we arrived at the concert hall, I expected to find about 100 attendees; instead there were about 500. I spoke very briefly about Callie’s diagnosis and what it means for our lives—how our goal is to give her the best life possible while we have her here with us. Back in 2013 Make-A-Wish sent us on an adventure to Vermont. We’ll always be grateful for the “snowy day” (as Callie’s always called it) experience. Nathan made a video of our trip that played at the Elon concert. No words can take the place of pictures.

The Elon concert raised over $4,300 for Make-A-Wish! Granting a wish costs, on average, about $6,000. We’re looking forward to meeting that goal at next year’s concert.

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.

 

Wedding fun (and other news)

Alicia and Callie

Alicia, the bride, and Callie before the ceremony.

The event of the spring for our family was my sister’s wedding. Alicia got engaged last August and asked Callie if she would be her flower girl. So from August until April 19, when she started down the aisle in Nathan’s arms, flower basket in hand, she talked constantly about her upcoming job.

And boy did she relish her role! With a happy, yet serious, smile on her face, she dropped the petals one by one—on her way in and out of the ceremony.

Callie and Nathan

Nathan helps Callie, the flower girl.

The wedding couldn’t have been at a better time because Callie has had several illnesses this spring—some before and some after the wedding. She would’ve been heartbroken if she had missed the wedding.

As always, those illnesses took a toll on our family. We’ve been concerned because she’s never had so many problems in such quick succession. With each illness she becomes weaker and as a result, more vulnerable to complications. She finished her most recent course of antibiotics a week ago, and we’re anxious to see her return to her typical strength.

For now, we’re looking forward to our extra time together this summer before our big girl heads off to kindergarten in the fall.

5 on the 5th

Today Callie is officially a 5-year-old! And she’s certainly proud of it. She talks constantly about all the big-girl things in her life — soon she’ll get a special big-girl bed; we take her to the potty like a big girl; and she’ll start kindergarten this fall.

Callie's 5th birthday

Callie opening her birthday presents.

Last year on Callie’s birthday, she was starting to get significantly ill. We’re so grateful that we’ve had another year with her. But in all honesty, there is always a sadness we feel, particularly during celebrations.

Callie asked for a bicycle this year, thinking she could ride it. Then she asked for crutches, thinking they would help her walk. Our oldest son Isaac, who’s 12, asked us to look on the Internet to see when a cure is coming for SMA.

But oddly enough, this disease has also brought us joy. The joy of listening to Callie interact with her nurses who come to care for her at night. The joy of seeing her 3 brothers naturally adapt whatever game they’re playing to accommodate her. These are things we never would’ve known without SMA.

In Callie’s 5 years, we’ve learned that sadness and joy can both exist at the same time. And once again, we’re grateful for this time.

Happy Birthday Callie-girl!

Chipper again

Good news — Callie is back to her chipper self. She pulled through what we hope has been the worst of this illness.

Callie has always been a happy girl, and she’s never lost her spunk even when she’s sick. But each time she’s ill it’s a reminder of how incredibly fragile she is. We called the Hospice nurse out to our house twice on Sunday night because she was doing so poorly and we weren’t sure what to do. At some point, there really wasn’t anything else we could do except wait things out.

Fortunately this morning, after having slept the vast majority of Sunday and Monday, Callie really turned a corner. It’ll take some time for her to recover — we’ll still have aspiration issues to deal with — but it’s amazing to see once again all the life she has in her.

A difficult weekend

Callie has had a rough weekend. What started as extreme fatigue late last week seems to have turned into aspiration pneumonia.

We’re thankful to have good guidance from Callie’s doctors and nurses, access to medicine and medical equipment and the support of friends and family. As usual, our goal is to help Callie rest and recover at home.

We’ll post more updates as things change.

Summer news

Next week Callie will start her last year at Child Enrichment Preschool. Summer has been enjoyable and fleeting for our family. We live near a YWCA where our older boys have been swimming almost every day. And Peter and Callie enjoyed their 3-day-a-week summer preschool sessions.

In early July, Callie had an appointment with Dr. Kravitz, her pulmonologist at Duke. Her lung tests looked good in that they’ve pretty much stayed the same with no decrease in capacity that we can tell.

Callie on boat rideSince Dr. Kravitz is Callie’s primary specialist, we also discussed our continued commitment to a palliative approach with Callie’s care. With SMA, there is no treatment, but there are many interventions that can be done. It’s easy for us to cling to the idea that these interventions can somehow save her. They can buy her time, but at what cost?

Callie’s spine is now curved forward and we know that eventually it will impact her lung function, if it hasn’t already. We could put rods in her back, but we’ve chosen not to. Even if we found a surgeon willing to operate on her, it would take Callie away from everything she loves: her school, her home and her family. She would be in tremendous pain. And to what end? What would be next? As horrible as it is, our little girl was not made to survive.

I have a friend who was watching her husband die from ALS. She said to me recently, “I’m trying not to make selfish decisions.” That’s how we feel as well. And truth is, the longer we prolong Callie’s life, the more we put her at risk for more complications and more pain.

Callie on beachThere’s a different kind of pain associated with SMA that Callie is now experiencing: the pain of knowing she’s different. She talks a lot about walking. We’ve been honest with her and have told her that she will walk when she gets to heaven. But just a few days ago, she asked Nathan if he would teach her to walk.

This summer, with the help of our children’s social worker with Hospice, we took our older boys, ages 10 and 12, out for an evening and told them that Callie is likely to die from SMA. Back in February, two days after her birthday, we almost lost Callie. She’d already been sick, but suddenly her muscles were too exhausted to coordinate her breathing. Her oxygen levels dropped, her heart rate spiked and she turned gray. We had to force oxygen into her lungs and it took a total of 8 mg of morphine to relax her muscles enough to where she could coordinate her breaths on her own again.

Our oldest son was at a friend’s house that night. We realized he could’ve come home and his sister could have been gone. We don’t want our children to feel the responsibility of adult problems, but because of SMA they do have adult problems and we need to all be able to talk about it honestly.

Callie and NathanLast week we went to the beach with my family and had a great time. Callie absolutely loved it! We took her bath seat and put it in the sand and let the waves wash up to her.

We also had an unexpected surprise: My little sister got engaged while we were there! The wedding is set for the spring, and Callie’s been asked to be the flower girl. She’s very excited about getting a pretty dress and the importance of her job. She could drive her wheelchair down the aisle, but I’ve been thinking that maybe her daddy should carry her down the aisle.