The Backstory

“Out of suffering have emerged the strongest souls; the most massive characters are seared with scars.”  (Khalil Gibran)

I first heard this quote on the tv show, Criminal Minds, several years ago and it has stuck with me ever since. I'm not sure I could come up with a better quote to describe the life Kennedy has led thus far: With all of her suffering, she is truly the strongest soul that I know; And her character, which many perceive as massive, is seared with physical, emotional, and mental scars. 

It wouldn't be fair to share our journey for an ear, if we didn't include all the road bumps we endured to get there (and I will apologize now as this might be a long blog post). See, having a craniofacial syndrome is similar to always waiting for the other shoe to drop. There are, of course, the obvious physical differences that one can see from the beginning. Then there are the things that are found through the numerous tests that are required. But even then, you don't know what you're dealing with. Because at anytime, you can get a piece of news that will floor you. Just when you thought you knew the battle you were fighting, the battle changes.

Within an hour of birth, we had a diagnosis. And we were given a flash training on what the diagnosis definitely meant for Kennedy and what it might mean in the near or distant future. But nothing can prepare you for all that you will go through.

Before leaving the hospital, we learned that she was deaf in the affected ear, that she only had one kidney, that she had trouble swallowing due to facial paralysis, that torticollis affected her neck muscles, and that her sinuses were extremely small for a full-term baby. At six weeks old, we were in the cardiologist's office having a full heart check-up. With hemifacial microsomia, heart problems are not rare. Luckily, for us, we received the good news that her heart seemed to escape the syndrome. At six months old, we had a physical therapist coming over to perform neck exercises twice a week. With a screaming baby. Yeah, that lasted long. For the first two years, we made bi-monthly trips to the development center to assess whether she was developing on track. For all the stress that caused, they should see her now!

At age 2.5, Kennedy underwent her first surgery at UNC. She was born with no enamel on her baby teeth and she had a host of dental problems. The dentist decided to do everything in one surgery and I was too new to all of this to disagree. She had numerous crowns placed, teeth pulled, root canals. I did put my foot down in the middle of surgery when the dentist wanted to pull all of her front teeth out as a precaution. Being her first surgery, we had no idea that it would turn into such an ordeal. After surgery, she was having trouble breathing. Only one of us was allowed with her in recovery and I took a break to call grandparents. I just remember breaking down in the hallway, the first time I realized how fragile her life could be. We eventually went home that night only to rush back to the ER the next afternoon with a partially collapsed lung and doctors who were in disbelief that 1) someone did that much dental work on a 2 year old in one surgery and 2) that UNC let her go home the night before. Had I been wise enough then, that would have been the last surgery at UNC. I will say that in order not to make a scene, I've never taken Kennedy to another dental appointment. That dentist, although she doesn't realize it, is lucky that Ryan is so laid-back.

Soon after came the three year old appointment which found a major vision deficit in her right eye and scoliosis/kyphosis in her back. Two major blows in one visit. Around this same time, we knew for sure that she wasn't hearing us well, so at age 3 Kennedy received her first hearing aid in her "good" ear. Make that three major blows. That was a huge adjustment for her, but also a huge help. At the eye doctor, we learned that the eye on her syndrome side has never learned to focus. Her other eye was overcompensating though so we never had a clue. We tried patching and a contact and to this day, it still isn't much better. Hopefully there is a fix for this one day in the future of medicine. In the meantime, we pray that nothing happens to her "good" eye. So I am the parent always yelling about things where a child could get their eye poked. Because if it is my child, then she has to lead a blind life and I cannot imagine that path.

We then visited with the orthopedic surgeon from her torticollis days and the bad news just kept coming. Not only did she have progressed scoliosis and kyphosis, but one side of her chest wall was not growing properly. And as we debated and discussed our options, her heart and lung were being squeezed. She fought pneumonia after pneumonia battle. She had no energy and was losing her spirit. We finally decided that she would be the first person in the state to receive the fairly new VEPTR, a titanium rib, to help with both the scoliosis and the rib cage. One would run the length of her spine, the other attach from top to bottom ribs and be expanded on a regular basis to stretch her rib cage. I won't go into all the details but the highlights were: major surgery to put the equipment in, 3 days on life support for pain relief, taking the ventilator out only for her to crash, watching her die (see my note on my page or Team Kennedy page for more on this), watching her be brought back to life, on life support for another 6 days while we wait to see if there was any brain damage, finally waking up only to learn to walk, talk, and eat again, a second surgery to fix one of the bars after it broke part of her spine off, constant infections, a third surgery to take the second bar out, PT, OT, 23 days in the hospital, and it goes on and on.

The VEPTR saved her life, has allowed her heart and lung to recover and grow normally, and she has never had pneumonia again. But we've faced many other battles since then. Eight months after the VEPTR month from hell, we were once again making the decision to have surgery to save her life. At this point, her sleep apnea was so bad that she was stopping breathing multiple times a night. What was supposed to be a simple T&A turned into: trouble controlling pain (ie, them not listening to us that she has high pain med tolerance due to life support the previous summer), a lung collapse, life support for 5 days, horrendous nursing care, a strep-pneumonia infection in her lungs from the life support equipment, a crash of her antibodies to fight off infections and 2 years of constant sinus infections following this. And a promise from me that my child will never undergo surgery at UNC hospitals again. Which in turn, led to me turning elsewhere for ear reconstruction options.

The years following brought multiple surgeries to lengthen the remaining VEPTR and two surgeries to give her a bone-anchored hearing aid (her lifeline and by far, the best decision we've ever made). But the doctors and hospitals really knew her by this time. These other surgeries were very uneventful and after a quick fix with a shot, she was finally healthy again. And thinking that things were finally going our way for once, we started thinking it might be time to begin the ear reconstruction process.

Kennedy's backstory plays a lot into our decisions on our journey for an ear. Kennedy's backstory have created many scars. We've been on this journey for an ear since the moment she was born but who knew all this journey would entail. And as we constantly wait for the other shoe to drop, we make the decision to jump ship...

(NEXT BLOG: Jumping Ship)