Last week, I shared a post from Meg Apperson. With her permission, I am sharing more blogs from their journey. Not only does it remind me of why we do what we do with Therapy Clinics International and the challenges the people we work with face, it also reminds me of how to live, honestly and with faith, in the face of all the challenges we are experiencing during this time. I hope you enjoy these blogs over the next few weeks and they encourage you as they’ve encouraged me. For more about Meg and their journey, check out her blog at http://www.fourfinelives.com/blog-1/2020/9/3/mbvry3t58c88qyhn71pcc54c2ocvvb.

I’ve avoided sharing Avery’s update in written form because I’m not sure I can do the complexity justice, but many people who have been awaiting our news are not on Instagram and blogging is still the most efficient way to communicate with everyone. 

There will be typos in this post. It will not be a work of art or literary masterpiece. It is what it is. 

In the next few weeks, Avery will be undergoing a 2-part surgery. 

Part 1: a second chiari decompression 

Part 2: posterior vault expansion with distractors

To understand why Avery needs these surgeries, let’s go back to the beginning… Avery was born with bicoronal craniosynostosis. After birth, we discovered that she also had some degree of bi-lambdoid craniosynostosis. Lambdoid sutures make the shape of an upside down V and are located in the occipital portion of the skull—the lower back part, right above the neck. We have done several surgeries to simulate suture-like space in the front of Avery’s skull, but we have never been able to address the lambdoid sutures because Avery has two very large emissary veins that occupy a lot of real estate there. Normally, the surgeon would simply cut those veins and the body would redirect that blood flow, but Avery’s veins were so large, the surgeon wasn’t able to predict how much she relied on them. 

We are going to have to cut those veins now. 

Because of how many sutures were fused at birth, limiting the space that her brain could occupy, Avery was born with a condition called “chiari malformation.” Traditionally, a chiari malformation is a slight herniation of the cerebellar tonsils into the foremen magnum—the bony opening on which the skull sits. In Avery’s case, her herniation is severe. Avery’s cerebellar tonsils, half of her cerebellum and a portion of her fourth ventricle have herniated into her spinal column and are—to put it mildly—crushing her brain stem. 

The brain stem is the control center for many of the body’s involuntary and some voluntary functions. In other words, it’s the whole enchilada and we have to ease the stress on it or Avery will never have a chance for a pain-free life with maximum functionality. 

Some of you may remember that we did a chiari decompression back in 2015, right before Avery’s first birthday. Because her dura (the membranous covering that encases the brain and CSF) was so vascular, the surgeon wasn’t able to do anything other than to remove bits of bone from some of her cervical spine and surrounding areas. This time, we have to go big or go home, because we can’t afford to do much more to those bones without risking neck deformity and cervical spine instability. We will risk that even with this next surgery, but it’s still warranted to preserve Avery’s brain stem. 

The real challenge, and the most risky part of this entire scenario, is that Avery needs to have her cerebellar tonsils removed. This would be a concerning surgery on anyone, but in Avery’s case, removing those tonsils that are in direct contact with her brain stem, means that her brain stem could very easily sustain an injury. And if that were to happen, we would need to make the call to let her go.

Quick recap: Surgery Part 1 is a chiari decompression, which will involve removing bone, cutting the dura and adding a patch (called a duraplasty) and most importantly, removing her cerebellar tonsils which are crushed against her brainstem. If Avery’s brain stem is injured in this process it could kill her or take away her involuntary and voluntary abilities—speaking, swallowing, breathing, blinking, all gross and fine motor abilities, etc. 

Part 2. of the surgical plan is to do a distractor surgery which would involve cutting the entire back portion of Avery’s skull off so that it’s free-floating. Then external hardware would be attached to either side of her head—hardware that we would turn twice a day for a month. She would have to keep the distractors in for several months. 

We have friends who have had this exact surgery with no issues, but the surgeon’s concern for Avery is her sagittal sinus, which runs down the center of the skull, because it is unusual. Because we’ve surgery in the area before, he isn’t sure what type of scarring or adhesions he may find. His main concern is that an air bubble could get into the channel and give her a stroke. 

To complicate this part of the surgery is the fact that Avery has a VP shunt. Expanding the part of her skull that carries the shunt may pull the catheter out of her ventricle and allow her CSF to build up again. This is something that would be a risk for the entire three months that she has the distractor hardware in. 

Additionally, putting external hardware into the area of her skull that houses her shunt could allow bacteria into her shunt and ventricles, another issues that would have to be closely monitored for the duration of the distraction period and six months afterwards. 

Quick recap: Part 2 of the surgery is expansion with distractors. A stroke, shunt malfunction or infection are the main concerns. A stroke is the immediate concern, while shunt issues will be long term.

The entire surgery will take all day. Avery is listed as urgent, so her procedure will take priority, but scheduling anything in this time is craziness so I don’t have a date yet. 

When I was explaining to Avery, in five year old terms, what we were planning to do, her eyes light up and she said me, “Will this take away some of my headaches?!” Seeing the excitement in her face, the hope that a major, agonizing surgery would take away some of her chronic pain, broke me. Once our conversation was done, I went away and wept over the kind of pain that a five year old must feel to see this type of surgery as relief. 

After we had left the doctor’s appointment, Avery’s surgeon called me. “I hope you know me well enough to know that I would never suggest something with these risks unless I thought it was absolutely necessary—unless I thought it was the best chance we could give her for a better life,” he said. And he’s right. I do know him and trust that he wouldn’t gamble like this unless he felt sure this was our only option, our only chance. 

So, I signed the consent and now we wait. It takes my breath away to write this post. I don’t fear losing her as much as I fear caging her inside an increasingly broken body. I can’t relegate that heart and soul to prison, trapped inside a dying body. 

I haven’t quite made the leap from agonizing over the risks to celebrating all the good that may come as the result if the surgery is successful, but I’ll get there when I’m ready. Let grief be grief and life be life and always be prepared to let it go.