Once upon a time, I had to make a life or death decision for my baby girl. Between her scoliosis and Thoracic Insufficiency Syndrome (TIS), she was going to die sooner rather than later if we didn't take action. With her syndrome, skeletal issues had plagued her since birth. At age three, we were told that the time was coming to make a decision. By age four and a half, we were told we were almost out of time. Her rib cage on the left side was not growing, her heart and her lung were being squeezed, and before long they wouldn't be able to work any longer. She had already started fighting life-threatening pneumonia infections due to decreased lung capacity, even ending up in the hospital on her first trip to Disney World. She was having trouble keeping up with her peers. Yes, it was time.
Kennedy's orthopedic surgeon was the first doctor in the state of North Carolina to be approved to implant the VEPTR, a titanium rib. She was the perfect candidate so a date was set. A month after turning five, I kissed my baby as she drifted off in the operating room. Her doctor implanted 2 VEPTR rods, one against her spine that would help with scoliosis and one further out, that would be used to stretch her rib cage.
The plan was to keep her on life support in a medicated coma for three days, for pain relief. She was young, this was a painful surgery. So we waited. We kept vigil. And we were more than ready when it was time to pull her out of the coma. Ryan and I were in the room as they pulled her off sedation and removed the ventilator. But our joy soon turned to despair. As the tube was pulled out, her throat immediately swelled shut behind it. She coded and we watched our baby die right in front of us. This sweet little girl with her blonde braids convulsing on the bed, with blood filling the mask as they tried to bag her. Her lungs were pulling blood from everywhere they could to try and breathe again. Numbers reaching zero, machines screeching. Every sight, every sound - burned into my memory forever. After what seemed like an eternity, the PICU doctor was able to intubate her again, despite the swelling. Her stats slowly crept up. And we waited another six long days to try it again. Six days of setbacks, six days of heavy sedation meds, six days of infection, six days of not knowing if brain damage was suffered. Nine days after surgery, she was extubated successfully. She suffered drug withdrawal like a heroin addict. She had to learn how to walk, talk, and eat again. She spent two more weeks in the hospital. She went through two more surgeries - one to fix a broken spinal rod and another to remove the spinal rod. The rod to stretch her rib cage worked great and several other surgeries followed over the next few years to stretch her rib cage.
And it worked. Her rib cage was stretched and stretched and her heart and her lung were given room to grow and to work. it also helped stabilize her spine/scoliosis. Several years later, her rib cage was growing on its own and her spinal curve was holding steady and the decision was made to remove the VEPTR. For five years the VEPTR did its job, but Kennedy's rib cage was finally doing what it should have been doing all along -- growing. And removal of the VEPTR meant fewer surgeries (to expand it to match the growing rib cage) and the ceasing of trigger migraines, an issue that plagued her with any sudden movement like a sneeze.
And here we are three years later. For reasons unknown, Kennedy's rib cage has slowed (or stopped?) growing on its own. The rib cage has multiple important jobs and one is to support the spine. Since growth is not happening, her spine is twisting. Since her spine is twisting, her good shoulder blade is shifting. And she would kill me if she read this, but it is also affecting her development in the front. Her skeletal system is slowly failing her.
I think she's most upset about having to wear a brace. It will be big, it will be uncomfortable, it will be a pain in the ass. She is focused on the here and now. But I'm most upset about not knowing. Not knowing whether the brace will help. Not knowing what her spine will do in the years to come. Not knowing if her rib cage has stopped growing completely. Not knowing if we're going to be facing life and death TIS decisions again. Not knowing what surgeries and other treatments she may be facing in the future. Not knowing how her heart and lung are going to fare. Not knowing why once again we're back here.
I always say that with her syndrome, I'm forever waiting for the other shoe to drop. I'm afraid that shoe is about to fall. And I can't stop it. No matter how tight I tie those strings. And as a mom, this is truly the worst feeling in the world.
