Dear 2015, You Can Kiss My...

On January 1, 2015, Kennedy woke up at Disney World with influenza, Type A. We spent that morning in a strange urgent care clinic, checking her lungs and obtaining a prescription for Tamiflu. She spent her last two days at Disney World in a wheelchair, insisting on making the most of our family vacation but too weak and tired to do it on her own. That flu turned into double pneumonia, with both lungs taking a long time to heal.

The thing is, I knew. Before she even woke up that morning, she was moaning in her sleep which is a sign of fever for her. I knew it was a bad omen...ushering in the year with a major illness. I knew it was an omen of what the rest of the year was going to be. I tried to ignore it. Afterall, we were in the most magical place on earth. But I knew. 

And sure enough, it didn't stop there. The aftermath of the flu took almost eight weeks to recover from. And right on top of that, we learned the disappointing news that her scoliosis was worsening and she would need a brace to try and stabilize things. Ah, the brace. From the moment the words were out of the doctor's mouth to the fitting appointments to the nights in that torture device. So many tears were shed. Every morning she would wake up in pain and I knew that wasn't right. The doctor kept encouraging her to wear it more, wear it longer at night. That she would get used to it and the pain would stop. But that didn't happen. She got a lot of passes on wearing it over the summer and those were the mornings she would wake up not in pain. 

If anything, I can say that it was a great summer. The beach, scuba diving in Florida, Greece. But as the summer went on, it was more than just the pain from the brace that we were seeing. It was shortness of breath. It was tiring easily. It was fatigue. It was watching her put her hand to her chest. It was seeing her sit on the pool steps while her friends were out swimming in the deep end. It was the quietness, the not wanting to talk or do anything. But I still wasn't sure what was going on. And I still wasn't putting all the pieces together. 

So...I decided to follow her doctor's advice and see a chiropractor to see if he could help with the back pain from the brace and just some range of motion things. He took x-rays to see what he could move and what he couldn't. As he was showing Kennedy the x-rays, the alarms went off in my head. I kept it quiet till I got home and then immediately emailed her doctor. We went back and forth and then I went in for a conference with him. And from there:

He recommended spinal surgery but isn't doing surgeries so sent her to Duke --> Duke ortho thought her symptoms were a little off and sent her to pulmonary before deciding on spinal fusion --> pulmonary discovered her lung function was at 36% and diagnosed her with Restrictive Lung Disease --> both ortho and pulmonary decided she needed an MRI of her brain and spine --> Chiari Malformation and Syringomyelia were diagnosed --> brain decompression surgery was performed to prevent paralysis and permanent damage --> recovery from brain surgery was hell --> and now we're ushering 2015 out with a headache that won't go away, along with some other Chiari symptoms. 

To put it mildly, 2015 SUCKED in Kennedy's world of health. It is not what we had in mind when we cheered at the stroke of midnight last New Year's with Mickey Mouse and all our family. We are not sad to see it go. Not at all.

But, I'm not all that ready or excited to usher in 2016 either. We will be ringing in the New Year in California, which we hope is a great time and the plane ride is not a trigger for any Chiari symptoms. But as soon as we come back, we head straight in for repeat lung function tests. And maybe I should be hopeful that the decompression surgery was a positive thing for Kennedy's lung function. But I'm not. Because I hear her breathing. And I hear her struggling. And I know she's still in trouble. 

After lung function tests, it will then be a matter of figuring out if 1) it could be from Chiari and that the syrinx hasn't fixed itself. And if that's the case, what do we do/how do we fix it. Or 2) if it is from the Restrictive Lung Disease and spinal fusion is necessary soon. I can assure you that none of us are ready for another MAJOR surgery, least of all Kennedy. She just wants to be a normal teenager (oxymoron?). 

So as we prepare to tell 2015 to kiss our a$$, we prepare for 2016 to kick us in ours. Hope for the best, prepare for the worst. And keep fighting daily. She is my fighter. She is my strength. 

Grief is REAL

Believe it or not, I'm a pretty private person. And Kennedy even more so. Which is kind of ironic seeing that we share so much (but trust me, not all) about this journey. In the end, writing helps me. Removing thoughts from my head and putting them elsewhere; a blog in this instance. But I also share what I do in hopes that it helps others, or inspires others, or lets others know they aren't the only ones feeling the way they do or facing what they are facing.

Sometimes things come clearly to me, an epiphany of sorts. And today's epiphany - as I'm sitting here literally trying to figure out what is wrong with me - is that the stages of grief that they talk about are indeed real. And that it is okay to grieve in situations other than death, such as a medical diagnosis. A couple of weeks ago, someone casually said in a conversation that thank goodness it wasn't brain cancer (and she wasn't the first one). Yes, I'm thankful for that. Truly thankful. We lost an angel friend just a few months ago due to brain cancer and in the couple of years that we had the honor to love him here on earth, we saw what that fight is like. No child deserves such a diagnosis. And I hope we never hear those words.

But what should be understood is that it is okay to grieve over any diagnosis that means that life will change from what you have known, or what you anticipated for the future. Grieving isn't just reserved for certain diagnoses. Is my child dying? I certainly hope not (although it could happen at anytime whether it is Chiari-related (it does happen) or not...we're never promised tomorrow). Does my child have to learn a new norm, one that comes with lifelong pain and issues? Absolutely. And it is okay to grieve this. I know this because I've been here before. This isn't our first devastating diagnosis. But sometimes a reminder is needed.

Denial: I was in shock with the diagnosis. Scoliosis is what we thought we were dealing with, not her brain. I was numb. I did what I had to do to get through each day, and more importantly to get Kennedy through each day. Leading up to surgery. Surgery day. Recovery. There is a grace in this stage...nature's way of letting in only as much as our soul can handle. And as we start to let in more and more, we start to begin the healing process.

Anger: Once I knew that Kennedy was going to be okay, I was just pissed off. Once she was able to eat, able to communicate effectively again, able to go through a day without tears, I felt it was okay to just be mad. At everything and everyone. I mean, for the love of the universe, didn't she already have enough she was dealing with? Did she really need something else? I think I'm completely out of this stage now, but it took a lot out of me to be so angry. It was exhausting and I hated it. I hated to be hating. It is not me. Luckily, I was able to leave my anger in my happy place, to roll out with the tide, to be broken apart by the crashing waves. I hope my anger stays there, is broken apart enough that it doesn't come back to haunt me.

Bargaining: I am still in and out of this stage; the latest being due to the scare of possible meningitis due to the hole that appeared in her surgery site. To be honest, I've been bargaining since the pregnancy test said positive and I felt in my heart that something wasn't right. And I will bargain as many times as I need to in order for my girl to be okay, to have a good life. Whether it works or not - and obviously it isn't working great lately - it is in our nature to bargain with someone, or with God, or with the Universe, or with Pocahontas if the reason fits.

Depression: And this is where my epiphany started today. As yet another morning dawned when it was hard to wake up, hard to get out of bed. Another day when I decided I could not fathom showering, dressing, and going into the office where I had to see people. I've been so damn tired lately. And my body just hurts. And I have no energy. And I don't want to move. I was sincerely starting to think something was wrong with me. Then it hit me...I've reached the depression stage. I'm sad. I'm sad that Kennedy had to receive another diagnosis. I'm sad that Kennedy had to have brain surgery and the tough recovery this has been. I'm sad that she will have to suffer from this disease and its effects for the rest of her life. I'm sad that this disease is still so unknown. I am sad that this may or may not fix her breathing issues and we may need to jump right into another surgery. I am just sad. And living in a fog. I know that this kind of depression is not mental illness. I know that this kind of depression can't be rushed. I know that this kind of depression is not something that you can just snap out of. It is a necessary step to get where I need to be.

Acceptance: Obviously, this is what I'm working toward. Accepting is not saying that I'm okay with it. I never will be okay with it. It is knowing that this is what it is and we must go on the best we know how. It is learning to live with our new norm. It is learning to live with the new diagnosis without letting it control lives, without worrying about the what-ifs unless they happen. Our goal for the acceptance stage is to live again, not just to survive.

Although I've chosen to share my grieving process, Kennedy's grieving process remains private. A lot of it remains private from even myself, and that's okay. We all deal with grief differently. But the bottom line is that once we're ready, we will come to acceptance and move on. We will live again, not just survive. It's what we do, afterall.

Ground Zero, Again

We are struggling. Who knew that brain surgery, even with the 36% lung function complication, was going to be the easy part.

It feels like we're starting over. Starting from scratch. When Kennedy was born, we lived in a fog. We scheduled many appointments, met many different kind of doctors, researched and learned everything we could about Goldenhar. We watched and worried about everything. Is she going to live? Is her heart affected? Does her one kidney work? Can she hear? Will she speak? Will she meet milestones and if so, will it be in the normal range? Will she have learning problems? We learned what Goldenhar meant in general terms. We learned what best case scenario meant, as well as worst case scenario. And I had never felt so alone in my entire life.

But we learned. And whether we wanted to or not, we became experts. We knew what we were dealing with on a daily basis, but we also knew that anything could pop up at anytime. And it did. All the damn time. But we knew how to handle it. We knew where to get the help we needed. We knew how to get through it, or adapt to it. We knew. It is our life.

And now here we are back at Ground Zero. But it's Ground Zero plus Goldenhar. Which is even more complicated. And even harder to navigate.

Chiari Malformation I. Those were the words that changed our life yet again. Those are the words that took us all the way back to Start. One thing that was so hard about this is that is happened so fast. We went from chiropractor to orthopedic surgeon to pulmonologist to MRI to neurosurgeon to surgery. We had ten days between the neurosurgeon's official diagnosis and surgery, and that was only because he was out of town the week in between. With her chiari came syringomyelia, pockets of spinal fluid (CSF) in her spinal column. She has one large syrinx and several smaller syringes throughout her spinal column. Because of these, and because of her gait and other neuro tests, surgery was considered urgent in order to prevent permanent damage and paralysis.

There wasn't a lot of time to digest all of this before we were thrown into brain surgery. There wasn't a lot of time to research the best neurosurgeon. There wasn't a lot of time to get a second opinion. There just wasn't time to absorb all of this before action had to be taken. And that has thrown me for a loop.

So here we are again. Back at Ground Zero. And similar to Goldenhar, this Ground Zero sucks. There is not a lot of information to be found. It means something different for every single patient. There are a multitude of doctors that have never heard of it. And if they have heard of it, most can't pronounce it correctly (KEY - R - EE) and have no idea what it entails or how to treat it. So once again, we're starting over. We're learning what we can. We're seeing how it affects Kennedy. We're learning how to get through it. We're learning what doctors will deal with what issue. And again, I'm feeling pretty damn alone.

Like Goldenhar, this is a lifelong battle that Kennedy will have to wage. There is no magic cure, there is very little research being done so no cure is in the near future. Surgery prevented damage that would have been detrimental, but so far has caused more issues with Kennedy than she had or could handle. That's hard to swallow but not unheard of. Recovery from decompression surgery can take a year or longer to get to her new normal. And we have no idea what her new normal will entail. Chiari is not considered to be fatal, but it can be. She now has a part of her brain with no bone to protect it. Luckily as of right now, she does now have Chiari-related sleep apnea. But that can always change. And at any point in time, another decompression surgery could be necessary. In addition, there are other conditions that often go along with Chiari. So far, those don't seem to be in play but again, that can always change. Like Goldenhar, we have to always be ready for the other shoe to drop.

The thing is, I could take all of this much easier if it were me. Or even if it were Ryan. I'd trade with her in a second. I would take cancer, MS, or any other detrimental diagnosis if it meant that Kennedy didn't have to deal with this for the rest of her life. I've watched her the past 13 years. I've watched her go through 20 surgeries now, too many major illnesses to count. I've watched her die and be brought back to life and I watched her almost die a second time. I've watched her fight her way back and learn how to eat, walk, and talk again. I've watched her suffer chronic back pain. I've watched her learn to adapt to so many new problems. I've watched her miss out on team sports. I've watched her be stared at and whispered about. Every single moment of watching has been nothing but heartbreaking, although more awe-inspiring than one could ever imagine. But now to have to watch her on this new journey, this new diagnosis, this new Ground Zero. This new Ground Zero plus her already established Goldenhar. I fucking hate them both.

I'm so filled with anger at all of this. No one promised that life was fair but for the love of mac and cheese...this too? It isn't an easy illness. Many chiarians qualify for disability as they age. Some will lose their battle much too young. Others will suffer through it the best they can. The lucky ones will have few to no symptoms. Only time will tell Kennedy's new normal.

And Kennedy? She shared with me this week in a note her feelings about this. It was heartbreaking, gut wrenching. It made me cry. It made me sick. I won't share...her feelings are private. Sometimes too private but that's how she copes. But this little girl, because she's still little, who never complains, she is struggling too.

What we don't need is negativity. Or unsolicited advice. Or to hear the words that it will be okay, or that she is strong and will get through this. Or even pity. We just need love. And understanding. Understanding that just because she has surgery and will hopefully recover from that, this battle has only just begun. Our lives have been changed forever, once again, by a diagnosis. If you've never had this happen with your child, please know how lucky you are. I hope you never do. If you have, then you know how hard it is, how different it is than a diagnosis of a spouse, a parent, or yourself. The heartache it brings as a parent, not being able to do anything about it. Not being able to take away the pain.

The support we have received has been amazing. We've had dinners, desserts, gifts delivered. Sincere offers to help in any way people can. Not to mention all the check-ins, prayers, and positive vibes being sent our way. We truly appreciate everything that is being done. Kennedy and I are both mentally and physically exhausted. A card in the mail or dinner at our door has helped tremendously...more than you can imagine. A text or message to either of us to say hey, how are you. Don't believe either of us if we say okay. Because it isn't true. We are struggling.

UPDATE on KENNEDY: She is on a strong steroid taper again, in hopes of helping her headache. She has had her headache for three weeks now with no relief. With the steroids, it is down to a 2/3 on a 10 point scale. Her 2/3 is like my 5/6, due to her high pain tolerance. Headaches are worse when she looks down or tries to focus so catching up on homework has been tough. She can only take Tylenol, no ibuprofen, and Tylenol is just not touching it. She is fighting insomnia, which is frustrating for anyone let alone a 13 year old child who usually sleeps like a rock. Even with valium, sleep eludes her. She still has back/hip/leg pain and her gait is off for which we're seeing her orthopedic surgeon on Thursday. She still isn't in school but we're going to try a couple of classes this week if we can keep the headache down to a lower number. Thankfully, the nausea and vomiting are gone for now. What we don't know is what this has done learning-wise. We hope she can focus, learn, and remember things like she did prior to surgery. If not, that will be another huge blow. We also don't know if this has helped with lung function or not and won't till January. Her syrinx is no better, which we knew it wouldn't be for months post surgery, but it is no worse either so that's a relief. It is still day by day as she struggles with the physical and we both struggle with the emotional and mental toll it is all taking.

Only the Beginning...

I've started this blog several times this week. And several times I delete the little that I've actually been able to write. And this has confused me because I need to write. But it dawned on me this evening. Brain surgery may be behind us. Surgery recovery may be finally going our way. But this new journey, it is just beginning. And I haven't yet wrapped my brain around all of this.

It has been a medical whirlwind since I saw Kennedy's latest spine x-rays in the chiropractor's office on August 27th. It kicked off a meeting with her orthopedic surgeon, to an appointment with the head of pediatric orthopedic surgery at Duke, to lung function tests with a pulmonologist, to an MRI of the spine and brain, to an appointment with a neurosurgeon, to brain surgery scheduled ASAP. A lot of new medical terms were used, a lot of information was thrown our way, a lot of new diagnoses were discussed. A lot of tears were cried. A lot of fears were founded. A lot of emotions were expressed. A lot of stress was lived. It was indeed a whirlwind.

But she survived it. She survived brain surgery. She survived anesthesia with 36% lung function. She reinflated her lung on her own. She walked herself out of the hospital. And she is kicking ass at recovery (okay, maybe with the help of some steroids, but still).

But this is only the beginning. Chiari Malformation is a lifelong disease, a battle that Kennedy will fight for the rest of her life. Surgery was performed to give the brain more room, to try and dissolve the syringes that were causing the spinal fluid not to flow correctly, and to prevent any permanent nerve damage and paralysis. What we don't know is whether the surgery will help with any of her symptoms. We don't know if her headaches will be fewer and less intense. We don't know if her breathing will improve. We don't know if this will help her fatigue. We don't know if her internal thermostat problems will go away now.

And what I fear the most, we don't know if this surgery will be the cause of even more symptoms and issues. I need her back in school proving to me that she is still my math whiz, that she's still my smart cookie who learns easily and quickly. Her cognitive abilities have been a silver lining on this journey, something that was a positive in her life of so many negatives and uncertainties. Now that has become an uncertainty. And we don't know if there was permanent damage before the surgery; the surgery will not reverse any damage. We hope that her lung function and gait were not permanently damaged before (although the gait is definitely a little worrisome right now). But she had to have it. We know that - there was no choice. With the amount of pressure that was released with her surgery, and the size of her largest syrinx, the doctor was surprised she wasn't exhibiting more symptoms at this point. I think it was only a matter of time. And only a matter of time before permanent damage set in. She may have had Chiari for years, but the symptoms came on suddenly and quickly.

As Kennedy's brain learns its new normal, as it adjusts to less pressure and more spinal fluid flowing, we also know that the diagnosis of Restrictive Lung Disease (RLD) is still there. Whether it was the Chiari or the RLD causing the low lung function, we won't know until repeat lung function tests in January. We're hoping that the fact that she was able to stop her lung from deflating on her own, and that she was able to keep her respiratory stats up after surgery, that Chiari was the cause and that the surgery was performed before permanent damage was able to take hold. If Chiari was not the cause, then we'll be scheduling a spinal fusion for this winter/spring. I don't think any of us can even fathom that right now. The hope is improved lung function tests and we postpone spinal fusion to allow her to grow some more (and recover from major brain surgery). But we will do what we have to do...the girl has to be able to breathe. Right now, any major respiratory illness could be enough to hospitalize her, if not worse.

No matter what, the RLD is an official diagnosis and one that we never wanted. It is a result of her Goldenhar, the result of her rib cage not forming correctly, not growing correctly. Her left lung does not have room to expand. It may be the cause of the current lung function. If not, it may cause issues in 5 years, 10 years, 20 years, or 40 years. Or it may never cause serious issues. It is something we will monitor on a regular basis and cross the bridge when we get there, most likely with a BIPAP or an external ventilator, trying to avoid a trach and ventilator for as long as humanly possible.

Our hope of all hopes is that she will live a long, somewhat healthy life. We know that many with Chiari and many with RLD qualify for government disability. Kennedy has both. We hope that it doesn't come to this for her. That she can pursue her marine biologist dream. That she can keep scuba diving for as long as she can. We're thankful for those in the dive community who are keeping vigil for Kennedy, assuring her that she'll be back in the water soon. She has to have hope. We all have to have hope.

This journey has never been easy. 20 surgeries, many hospitalization, many many more illnesses. Bad news and rare things happening around every corner. As her mom, my heart breaks. We only want the world for our children. Not knowing what her future, what her world, will be like makes it hard to breathe at times. Watching her suffer over and over. Watching how unfair life can be for her. The day I learned I was pregnant, I knew something wasn't right. Little did I know that that feeling would come back to haunt me over and over again, all at her expense. I would take it all myself if I could. I know it is hard for her to live this life. I cannot discount that at all. But I hope with all of my heart that she never has to know what it feels like to be in my shoes, to watch her child suffer like she has.

She is strong and she keeps me going. She is sarcastic and keeps me laughing. She is sweet and keeps me loving. She is my hero and keeps me humble.

A glimpse into the heart of a special needs momma during a storm

This blog is my catharsis. I write to get all these thoughts and feelings out of my heart and into words. I share because maybe it will help someone. Or will give someone a glimpse into someone else's sometimes complex and difficult journey and they won't feel alone. And if no one reads it? No worries. Because it is for me and hopefully by the end of writing a new post, I'm feeling more at peace.

Needless to say, my thoughts and feelings have been all over the place over the last few weeks. At times, I feel like we're living in a blur. At other times, the days drag on while we wait for answers, a plan. I'm a planner. I don't do well with surprises and unexpected bumps in the road, so this has all been a little overwhelming. I'm also usually a calm and happy person who tries to always love life, so overwhelming does not fit me well. I'm feeling a little out of sorts, to say the least.

Moms are made to worry about our babies. We try not to let it consume us. But when we're thrown into a storm, there is no avoiding the thunder and lightning. Things weigh heavy on our hearts and our shoulders. We've all been here at one time or another. We learn to dance in the rain. We cannot control the waves, but we learn to surf.

And surfing is exactly what is happening inside my heart right now. A glimpse into the heart of a momma whose child is facing some major health issues. A glimpse into the heart of a momma during a storm...

<3 The support that has been given to us, shown to us, blows me away. From texts to cards to personal messages to flowers to food to treats to gifts...every single gesture and word means more than you can know. I don't ask for anything but prayers and good thoughts. But I've also learned that every little thing helps and I won't turn any gesture away. Life is too much at times not to accept help when it is being offered. So I accept, and I pay forward.

<3 Silence is golden. There are no right words but any words help. I'm not one that believes that everything happens for a reason. Or that if He brings you to it, He will bring you through it. But those words are better than none, if that's what someone truly feels. This doesn't mean I'm looking for comments on every status I post, because I'm not! Just know that if you don't know what to say, simple words go a long way. And this doesn't just apply to us -- this is sound advice for any special needs parent.

<3 The MRI scares me. I should rephrase that the brain MRI scares me. It was added to the spine because of Kennedy's recent headaches. Could they be from the spinal curve and/or lack of oxygen? Yes. But scoliosis can cause issues with the brain. We also know Kennedy has larger than usual pockets of fluid in her brain and it has always been a wing and a prayer that this hasn't caused any issues. And who knows what else could show up in the MRI. I want it over with and the results back. Not knowing is beyond stressful.

<3 We've been here before. But then, her rib cage was still stretchable and we saved her organs for six years. We're beyond that now. There is no good way to fix it this time. They will straighten her spine which will hopefully relieve some pressure on her lungs and heart.

<3 But she will always have the diagnosis of restrictive lung disease. This can mean different things throughout her life. It could mean she will never scuba again. It could mean that a bipap, oxygen cannula, portable vent, or even a trach/vent are in her future. Or it could mean that she just struggles with breathing when active. What it definitely means that every cold, sinus infection, flu, and pneumonia become dangerous to her. For those with restrictive lung disease, these are the things that are life-threatening.

<3 I've been posting a lot -- this has all been moving fast. And they have not been positive posts which actually bothers me, because I try so hard to be a positive person and promote positivity. I'm a silver lining kind of gal. But right now, it is difficult. I'm not looking for likes or comments. I'm just trying to keep people updated and to cope in my own way. When we faced this before - 3 surgeries, one month in the hospital, 9 days on life support, watching her die and be brought back to life, watching her go through drug withdrawal, having to eat and talk and walk again - there was no such thing as social media in my life. It was hard to keep those who loved Kennedy updated, and hard to find the support that we all need when going through such a storm.

<3 I worry about Kennedy. She is pissed. She is scared. She is upset. And she feels crappy. But yet she gathers her strength and gets through all of these stupid tests and appointments as best as she can. I hate that her life sucks so much at times. But as my cousin (by marriage) reminded me today, her life is so good at times too and those are the memories that we have to focus on through all of this, and focus on the good things to come. We had one of the best summers ever (especially Kennedy) and for that I am thankful. And we hope that she can return to "normal" life before next summer. I hope her soul is only bruised during all of this, and not broken. Thirteen is a rough age for a normal girl. let alone one who has to walk this path.

<3 Surgeries suck. This one will especially suck. It scares me that she might stay intubated in PICU for a couple of days. Intubation is always difficult. Extubation is always difficult (especially if not immediate after surgery). Surgeries on lung patients are dangerous, especially when her lung and heart will physically be shifted during surgery. Spinal surgeries can mean a lot of blood loss. And moving the spine is scary, even with nerve mapping in place. I know that it is a surgery that happens every day. And I wish that made me feel better. But I have that child that never quite follows the rules.

<3 I worry about Katie. I'm thankful she is the older sister for my own selfish reasons. I'm thankful that this journey has helped shape who she is and who she is becoming. I'm thankful she is there for her sister. But I know waiting for word from all of these tests and appointments is stressing her out. I know the uncertainty of her sister's future is upsetting her. And I know leaving when Kennedy is still in the hospital post-surgery is going to be hard for her.

<3 I worry about Chelsea. My one prayer has been that she remains stable till after we get through all of this. I don't think any of us could handle having to say goodbye in the midst of this. So far, she's staying pretty stable. But she doesn't do well if her people aren't here and a week in the hospital may be enough to send her over the edge. Kennedy is her baby and she tends to worry about her, even with her dementia.

<3 I feel bad for my friends. I know it isn't easy being my friend at times. It can probably be exhausting in times like this. I don't fall apart often but when I do, they are always there to pick me up and put me back together.

<3 I also have the irrational fears that serve no purpose except to drive me insane. What happens if surgery paralyzes her? What if she doesn't make it? What if I die in a car wreck tomorrow and she has to face all of this without me? And the list goes on. And you can tell me that these are ridiculous as many times as you want...you know they just come with the territory.

<3 No child of any age should be facing their 20th surgery. That's it. No. Child. Ever.

(forgive typos -- the problem is I can spell, I just can't type. and i was too tired to reread through it)

I can't even think of a title...

SO here goes. We spent the majority of the day at Duke Children's today, meeting with a pulmonologist, getting lung function tests, meeting with the pulmonologist again, having a chest CT scan.

I wish I could say that we bypassed the new diagnosis we were dreading, but no such luck. Kennedy was officially diagnosed with severe restrictive lung disease. Symptoms have been exacerbated due to the worsening scoliosis, but her rib cage is the main factor causing this. A normal lung function is 80 or above. Kennedy's overall lung function was in the 30s today. The one caveat to that is that we don't know what Kennedy's normal baseline was, before she started to show symptoms. It could be that even though her lungs are normally clear (minus illnesses) and that her pulse ox is usually great (minus illnesses), that her normal function has been in the 50s for years. We just don't know. But no matter what, 30s is not good. How she is functioning in normal everyday life as well as she is is beyond anyone's guess.

So, will spinal fusion fix her? Bottom line is no one knows. It might help lung function. It might not help lung function. But what it will do is put her rib cage back in a better place which will help stabilize her lungs and keep it from getting any worse. Right now, her lung on the left is being squeezed and twisted due to the shifting of the spine which is pulling the rib cage with it. So does she need spinal fusion? Absolutely. And soon. This has all happened so fast and we do not want it to progress any further and put her in any more danger.

Kennedy will also be going for an exercise study and a sleep study within the next few weeks. The immediate needs that she might have based on these tests will be restrictions in the realm of physical exercise, and the possibility of needing a BIPAP for sleeping. We're praying we're not at this point yet. These tests will also give him a better perspective of how to handle her before, during, and after surgery as he will now be involved and trying to prevent any issues before they even arise. Although he has cleared her for the surgery, surgery on someone with lung function that low isn't ideal. The possible need for keeping her intubated after surgery is strong at this point.

So some questions we're left with:

- Will her CT show anything else that we're missing? It could, but the pulmonologist doesn't think so. It was done after our other appointments but he will call if there is anything else we need to know as a result of it.

- Will her MRI of her spine and brain scheduled for Friday show anything else we need to know about? We don't know and we won't know till next week. It's main function is to give the orthopedic surgeon a better look at her spine and how it is currently functioning. He wants to make sure we're not missing anything related to the spine. We've added the brain to rule out anything unusual there that could be causing her headaches, which we hope are just from the scoliosis and the lack of lung function.

- What does this mean for the future? There is no good way to fix the rib cage so this is a disease she will live with from here on. We hope that the spinal fusion will help stabilize and keep it from getting worse. If the sleep study shows sleeping issues because of this, then a BIPAP will be introduced. This is the first step in breathing assistance for those with restrictive lung disease. There will always be a chance for much more intense breathing intervention as she gets older, if the disease continues to progress. Will it progress? Great question with no certain answer. It will always be there. It might get worse, it might not. Only time will tell. As of this point, every cold, sinus infection, flu, and especially pneumonia becomes more dangerous than it ever has been. Think about how hard it is for you to breathe at times with just a cold and most of you have normal lung function.

- What does this mean for her new-found passion of scuba diving? The answer is still out on this one as well. We're on hold now until after the spinal fusion. The doctor will then perform more lung function tests to determine if she's improving, is stable, or is getting worse. The good news is he didn't immediately say no way, nuh uh. He is open to allowing her to continue depending on future lung function tests, especially given that she has been diving with no lung issues (only sinus) for the last year. And given that our awesome dive instructors are okay with his assessment. When we talked about this over lunch, she teared up but being the mature person she has always had to be...said that as much as she wants to do it, it isn't worth dying from. Of course, she's hoping for the best possible outcome on this question and to swim with the sharks soon.

This is one storm that will never pass, so it's a good thing we have learned to dance in the rain.

No Strangers to Disappointment, But Still...

For those who have been waiting...

We are no strangers to disappointment on this journey. My Timehop app has proved that over the last couple of weeks, as I've relived the nightmare of Kennedy's second ear reconstruction and the MRSA that destroyed all hope of her having a permanent ear four years ago. And that's only one example. We're never ready for disappointment, and we're never equipped to handle it gracefully. But we do what we have to do to get through it and get her healthy and do the best we can for her. 

Once again, disappointment has reared its ugly head. And once again we weren't ready and I'm struggling with how to deal with it. Goldenhar is complex to say the least. It involves many body parts and systems and there is never really much rhyme of reason as to when the next major hurtle presents itself. 

In February, we went to visit Kennedy's orthopedic surgeon and I knew it wasn't going to be great news. Last August had been the best x-rays of Kennedy's recent years, down to a scoliosis curve of 28* and her rib cage still growing on its own. Going into that February appointment, I knew her "good" shoulder blade was sifting which most likely meant her spine was shifting. The February x-rays showed that the curve had worsened to 38*, that the spine was starting to twist, and that her shoulder blade was being shifted. It was time to brace. Kennedy has been wearing her torture device almost every night since, trying to keep things stable and pull her shoulder blade back into place. 

I knew it wasn't working...you could tell by just looking at her. But in the meantime, we had taken her surgeon's suggestion to visit a chiropractor, to see if he could help loosen things up and try to gain more mobility and flexibility. He took x-rays so he knew what he could and couldn't move, what had a chance of benefiting from chriopractic techniques. A couple of days later, we went in to discuss the x-rays and hear his plan for moving forward. As he brought the x-rays up, Kennedy listened to him explain what we were looking at and how he was going to help her. I couldn't even listen to his word...I was looking at the x-rays thinking HOLY SHIT. They were so much worse than her February x-rays. Not only was the scoliosis curve measuring at 60*, her rib cage was also shifting and her lung and heart were clearly in danger. 

Which brought us to Friday's appointment. I emailed the x-rays off to her orthopedic surgeon and he agreed that there was reason for concern. So Katie and I went in to see him Friday morning. He sat in his office with us, answering all my questions. He tried to be optimistic, but bottom line is that surgery is inevitable. Highlights, if you can call them that (and I'm probably forgetting some):

- The spinal curve itself isn't the big concern. But it is causing many other issues that are huge concerns. Because she has always dealt with spinal issues, he thinks this is a blessin as she isn't in constant pain like other girls her age would be who are just developing scoliosis. 

- Because of the curve, her rib cage on the left has shifted drastically. This is causing her heart and lung to be moved/squeezed/in danger. Kennedy has always had lower capacity than most kids her age, but we have noticed this worsening over the last few months. 

- In addition to the shift of the rib cage, he is also afraid that the left rib cage is also starting to collapse inwards. There is no easy fix for this. 

Her doctor is not performing surgeries right now so we meet with a Duke doctor on Thursday. It will be his ultimate decision on what needs to happen and when. But it sounds like:

- A spinal fusion will be done to straighten the spine as much as they can. This may get her down to the 20s, but it also depends on how flexible her spine is once they would get in there. 

- She will most likely have to do respiratory function testing and nerve mapping before undergoing surgery.

- Spinal fusion means at least a week in the hospital, some of this in PICU. There is a chance that the surgeon will decide to go a different route, which means she could be stuck in the hospital for months in traction, between two different surgeries.

- Her current doctor is worried about the huge shift this will cause on her rib cage/lung/heart all at once and she may not be able to be extubated until at least 24 hours post-surgery. With her history of extubation, this scares the hell out of me. 

- Spinal fusion would mean missing 4-6 weeks of school, and 3-6 months to return to regular activities. She will have to learn how to move in a whole new way. 

- Her spine will quit growing where it is fused. However, he thinks she will gain at least one inch upon straightening. 

Timing is a huge question right now. Her current doctor isn't sure she can wait till summer, which is when they try to schedule these due to the school aspect. And we're not sure that would be the best choice anyway...she starts high school next August and stressing over healing in time for that may be too much for all of us. With her increasing shortness of breath and fatigue, he is thinking it will probably need to be sooner rather than later. This will cause some extra heartache due to some things that are in the works, including a possible trip to Disney and a trip to the Bahamas so she can scuba with a renowned shark expert. But no time is a good time. 

I hate Goldenhar Syndrome with all of my being.

For now, I've had the blessing of keeping all of this from Kennedy. With the death of a friend from cancer (something else I hate with all of my being) and his celebration of life today, she has had enough heartache to handle at the moment. But between now and Thursday, I must fill her in. Those who have lived through the age of 13 with a girl can only imagine how this is going to play out. At a time when we have to work to bring her out of her teenage moods, we were warned that this will probably make that even worse. Life will be a nightmare for awhile in the Hendershott/Goodwin household. 

In the end, we will depend on the love and support of Team Kennedy. What would we do without you?