Bent, not Broken

“She hides her curve very well.. We are now at the point in her Scoliosis where it is impacting her lungs and heart. She needs surgery now.”, her Orthopedic Surgeon. So many things in this little girls’ life have felt emergent. Your spine is a crucial part of your anatomy holding your weight against the forces of gravity, allowing you to stand and walk upright. It gives you the ability to bend over and pick things up, touch your toes  and reach high for something in the cupboards. The spinal cord is the nerve highway connecting your brain to the rest of your body. The spine is composed of vertebrae, a series of bones. These are a series of rubbery round discs that provide cushioning and shock absorption. Muscles and ligaments connect the vertebrae keeping them aligned one on top of the next. Each vertebra has a donut shaped body and their stacked holes create a channel for the spinal cord.

Several years ago I took her to Urgent care for a bad cold, The x-ray tech at the time said to me, “Wow I see she has Scoliosis.” I had not once in Zephie’s life heard that she did. Since he was just a tech, and not officially supposed to tell me a diagnosis I followed up with her pediatrician at the time. He said she seems fine and we could wait a few years to start down that path. It is not uncommon for scoliosis curves go undetected until they become quite severe. Curves don’t always cause much deformity that is visible or cause adverse health affects until drastic. What I know now leads me to believe dipping our toes in the water then would have prepared us a lot more for where we are today. However, surgery is pretty much the only answer that was in the books for her whether we started the journey then or now. 

I know we are meant to be where we are, and how we got here. She has idiopathic syndromic scoliosis. Meaning she has it because she has a chromosome deletion. Really though, that’s a broad assumption we hear for many things in her life. The truth is, they just don’t know why. This type of Scoliosis acts differently than other types of scoliosis and often progresses in a different manner.  We don’t know how quickly it progressed. However, towards the end it has definitely progressed faster than we wanted it to. The research with scoliosis is ongoing. Evidence does show that while not one specific gene plays a role in it. A combination of genes do play a role in it happening. We don’t know if that is part of her missing chromosome pieces or not. There are also environmental and dietary factors that play a role in its progression that are being explored. Her Pediatric Orthopedic Surgeon mentioned that these types of curves do what they are going to do, pretty much regardless of what you try to do to stop them, other than fusion. 

There are 3 sections to your spine, cervical. thoracic, and lumbar. Cervical are at the top connecting your spine to your skull. Basically your neck area. Thoracic are in your chest and and attach to your ribs. Last in the lowest part called your lumbar attaching to your pelvis. The lumbar section is the largest and most responsible for flexibility. This is predominately where Zephie’s curve is. Her curvature in her spine was last measured at 100 degrees in her lumbar region and growing. Her fusion will be done from L1 to her pelvis. Interesting to note she is extremely flexible everywhere in her body. I have a suspicion this hinders and contributes to her ability to function so highly with such dramatic curve. Her muscle elasticity is helping her compensate. While at the same time having low muscle tone when she was a baby and toddler hindered the growth of a strong spine for her. 

Scoliosis can also contribute to your body being unbalanced. As is the case for Zephie. As she has grown her body has compensated for the curve. One hip is severely higher than the other. In which the doctor suspects has resulted in one of her legs also being longer than the other. The thoracic part of her spine is also curved. We believe this is a result of her body compensating for the intense pressure the lumbar curve has placed on her body. The body has a miraculous way of surviving! 

November was our very first appointment with the Pediatric Orthopedic Surgeons for her Scoliosis. This was after I told her pediatrician I wanted a specialist to tell me that things could wait. The stature in her back looked stunted to me, and my gut said it was time to walk this road. 

How did we get here, it seems like just months ago we were praising the phase of normalcy we had been in. The truth is Scoliosis has been there for years without much attention. One thing I have graciously learned about Scoliosis over the past 5 months is the diagnosis has captured so many amazing souls. Those on the journey young and old in every stage are such optimistic people despite the daily hurdles. This may come as no surprise why Zephie too is on this journey. Scoliosis affects 2-3% of the population. It is the most common muscular deformity in children compromising 20% of all cases. While scoliosis does affect both male and females equally. For unknown reasons curves in girls are 10 times more likely to get worse, and five times more to need medical intervention. Her Chromosome Deletion is about as rare as diagnosis’s come, Scoliosis is a bit more normal. The positives of this being one of the adventures on her journey is that there is lots of information out there.  There are experienced Doctors that have seen this before and can adequately help her.  While most are not as severe, she isn’t the 1 out of 15 in the world with this.

I recall a dear family friend telling me about a week after her heart surgery as a baby, “ Zephie is incredibly lucky to have you as a mother. I know you will do everything in your power to make sure she lives a life just like all other kids.” Normalcy, what does that even mean as a parent, right? We all have unique situations that make it feel like we have more on our plates than we can handle. It may take 10 extra steps for every one step it takes her brother and sister, but Zephie will and does persevere. I give every ounce of energy, every single day to make sure she is given the opportunity to feel like they do. It isn’t at all about what misfortunes Zephie has been handed. Perhaps to her they are fortunes. She has life, the ultimate gift. She spends it loving on people and doing what those around her are doing, laughing, loving and giving every single chance she gets. I don’t know many people as genuinely kind as she is straight from her soul. She believes in everyone. When there is a limitation she needs help with she does her best to improvise. 

Surgery is coming soon. It is very necessary she has the surgery soon as it is impacting her breathing in daily life. Zephie didn’t come to us and tell us she feels pressure in her lungs and it is hard to take deep breaths. She improvised continuously, until the signs were so obvious that her little body is fighting toobhard. 

That is what we as her parents will do right along side her. Fight; with love, wisdom and advocacy for her. So she can continue to shed light in this world. Love is powerful, and so is her determination. Thank you to everyone for the love and support. It means the world to us. For every act of kindness and all the love we are shown, we can pour right back into this sweet gem of ours. 

You’ve got this Miss Z! Lets go climb that mountain baby girl!

Loves,

Momma

If you are so inclined, we have a fundraiser set up to help with the medical costs of this procedure that insurance won’t be covering. Thank you!

https://www.gofundme.com/bentnotbroken-for-zephie