Attitude of Gratitude - The Cure

Written as a Facebook status on May 25, 2019.

There has been some incredible news in the SMA Community that came out this week. I think, my Aunt Maureen has said it best, "Science rocks!!"
Yesterday, after hearing about the "cure" for 31 years, it is finally coming true.  Of course, it is 30 years too late for Emma and for me too. I have been blessed to be able to witness the effects of gene therapy first hand and I must say that it is like seeing a miracle every single time, Unfortunately, the old adage, miracles happen every day, came true too late for so many families. In most situations, I aim to be the optimist and for the most part, that is the case with gene therapy and its approval. However, my heart hurts, aches, for so many families, the parents, and siblings who never got to witness their children grow up. My Emma, my sister, passed away 27 years ago this year, for others, it has been even longer, and for still others, it has (only) been a week. In this case, despite what I grew up knowing, life is not fair (sorry to my grandpas). I don't say that lightly as fairness is not something I have ever chosen to focus upon, but in this case, I will make an exception. It is not fair, that Logan will  never dance at his wedding; it is not fair that Andy will never protect his little sister from a heavy down thunderstorm; it is not fair that Jerika will never get to hold her firstborn child; and it is not fair that Emily will never have a ballet recital. there are so many that I could go on for pages, but please know that I think of them always. I don't want to be the downer on this incredible news but I promised, long ago, to never forget, that I don't break my promises. I guess my message is;  be grateful for what you have, to those who came before you and to those who didn't make it. We are the survivors, we are the lucky ones. We have battled every day to survive and we made it. We aren't sure how much it will help us, yet, but we are here, we made it without a magic "pill". Don't tell us that we don't understand, we have seen everything and have witnessed everything. Just be grateful and have an attitude of gratitude! 

Remembering Jordan

At this very moment, two of my friends are preparing to lay their forever 4-year-old daughter to rest. This should never be the case. 2017 has been a particularly difficult year for many of my closest friends. Too many of them have had to say goodbye to their children in spite of the fact that for the first time in history there is a viable treatment for SMA. It is not right and it will never be okay for a parent to have to bury a child regardless of the cause. Today my heart is heavy for Jerry and Brieen as they say their final goodbye, here on earth, to their sweet daughter Jordan. Jordan will be remembered as one tough cookie, brimming over with sass, a smile that could melt the hardest of hearts, and eyes that shone brighter than the brightest star in the sky. Her time was short but her mission was clear; change what is known about SMA. She brought hope to this world and even though she may be gone her mission of hope will live on in the hearts of hundreds, if not thousands, of people who knew of this little spitfire. Jordan, you have touched my heart and will live on as I promise that you will never be forgotten...not by me, not by Brenda, not by the nurses, RTs, PCAs, OTs, PTs, doctors, and on and on at Nationwide Children's, and really anyone who was ever blessed enough to really know you and your incredible parents... You will live on in the hearts and minds of so many people Jordan. Keep sending us ❄️snowflakes❄️, okay 👑sweet princess👑?

Never Lose Courage

Today is the last day of August and for people who are not directly impacted by Spinal Muscular Atrophy (SMA), it is the end of SMA Awareness Month. Today is the last day of newsfeeds filled with facts, photos, fundraisers, and post with pleas for "outsiders" to take notice of the world of we live in 365 days a year, 7 days a week, 24 hours a day. SMA Families live with the knowledge that every day is an important day. There are no guarantees in life and even fewer when you have a life impacted, daily, by the restrictions of a label of a "terminal illness". It's important to recognize that life, no matter who you are or what you do, is terminal. We are all destined to live a life that will, ultimately, end in death. It is a fact and it may seem incredibly callous to be so blunt on such a sensitive subject. That is not, however, what we want people to focus on. We must remember that the important thing to focus on is the time between the day we are born to the moment we draw our last breath. This is never truer than when you, possibly, have an accelerated time table of the "in between" which is what many individuals with diagnosed with SMA are told they face. Whether or not that is true is, often times, the subject of heated debates that never truly cease with a definitive agreement on what "the truth" is. This is largely due to the fact that it is all up to the individual. It comes down to whether one chooses to focus on the quantity of time or the quality of time. That choice, of what to focus on, quality or quantity, can and, often times, does change depending on the situation, the day, the hour or the moment.

So as the month of August draws to a close, we ask that as you go about your day and the days ahead you make a conscious choice to never lose courage in the face of the fact that someday it will come to end. Make the most of your time in between. It will take an insane amount of courage to take the day as it comes. It will take courage to focus on whatever you choose, whether it be quantity or quality, and do so unwaveringly. Life takes a level courage that is unmatched. Having the courage to live in the life you have been handled is the most courageous thing that anyone will ever have to do. Courage is a choice and it is a choice that must be made repeatedly throughout a lifetime and, sometimes, one that must be made repeatedly in the span of a day. It is a solitary choice that individuals must make on their own. The choice to have courage or not is one that will impact others. It is not an easy choice but rather the opposite, maybe the most difficult. Whatever you choose in other aspects of your life, the choice of courage will undoubtedly make all the difference. Choose to Have Courage and Never Ever Lose It!

Choices

Over the years writing has almost always been my go-to stress relief method and a great way for me to express my opinion or feelings about a wide range of topics. I have recently taken a huge step back from the SMA Community because I feel that I can better serve the community by keeping my opinions to myself. That being said there is something that I feel a need to write. This may never go further than my fingertip or it may go out with a bang of a blog post. I won't know until I am finished and even then I may hold on to it for a few weeks or months and decide to release it when the level of tension isn't as high. Whatever this ends up being it is most important for me to get this out of my system before it festers and becomes something that I cannot control.

In recent months there has been what seems to be an ongoing argument and to call it a discussion doesn't give it the level of anger, disrespect and quite honestly disregard for others feelings that has come to a head. It comes in different packages and finery, from the headline news to Hollywood scripts to quiet corners of family homes but ultimately it boils down to a certain topic that has been heartbreaking for generations; the right to die or live. It can be looked at through a thousand different lenses and a kaleidoscope of colors from rosy to the bleakest of blacks. Most recently it has been called into question by the story of Julianna Snow and it's counterpoint in the "Dear Julianna" campaign, the controversy surrounding the best selling novel, by Jojo Moyes, turned movie "Me Before You", to most recently the headline "Appleton teen makes heartbreaking decision to die".
 As I sit in my new apartment listening to "Hold On" by Wilson Phillips, I have come to the conclusion that I can remain silent no more. I have stood on the sidelines of all of these controversial topics, silence at my side, I have read and reread the variety of opinions from people who have inserted themselves and their opinions into one or all of the aforementioned topics. People who feel that they must speak out for those who can't or those who they feel have an incorrect perception of the tales flooding Facebook newsfeeds. This is in my own opinion one of the biggest issues; assuming that you know how someone is feeling or should be reacting to a certain situation. Sure, you may have the same diagnosis, you may have experienced something similar at the same age in the town, or you have professional experience with a similar set of circumstances and made a completely different choice for yourself or your child or chihuahua so you feel that need to share that. Power to you, your parents, your cousin three times removed and your 2lb chihuahua who lapping up cappuccinos from a ceramic teacup while you type out your scathing rebukes of people who did it "wrong" in your opinion. Note: I have nothing against chihuahuas, they were the only dog that I could think of that started with a 'C'. That really is your prerogative to put the world on blast in whatever form suits you best. Difference of opinion is what makes the world go round but what makes it truly impactful is when you take a few extra moments to understand that your experience is just your experience and it does not necessarily reflect the experiences of any other person on the planet.
In my younger years I mistakenly thought that my experiences were universal and over the years as I have grown as a person I have come to realize that while my life may include aspects that others can relate to, no one else has lived my life and on the flip side I have not lived anyone elses. When it comes to the three examples I listed above I have not lived with Charcot-Marie-Tooth (CMT) the disorder that Julianna had, I have never been a physically active (eh-hem, not to mention, fictional) male who ends up paralyzed like Will Traynor in "Me Before You", and while I do have the most in common with the "Appleton, WI girl", I am not in her body. Let's back track a bit and make a case for each individual story and how similar and dissimilar my own life experiences are. Let me begin by saying that when it comes to Julianna Snow's story that has been raked over the coals so many times that I have sometimes felt physically ill from the opinions I have read. In part I must say that I followed the journey of Julianna's family before it became a controversial story in the eyes of all of America from coast to coast. Her family shared their journey to Julianna's choice on a blog and while I was not a faithful follower, I did read what I could. When their story became the stuff of every family's nightmare by ending up as fodder for hundreds of adults with disabilities, I had already been following for several months. At the time I did not feel the need to add my voice to the public outcry because I was one of the few who didn't see Julianna's choice as anything more than what I watched my own sister decide. My sister, Emma, had Spinal Muscular Atrophy (SMA) type 1 and when I read THIS conversation that Michelle, Julianna's mom, shared with the world it was like being transported back to March of 1992 when Emma and I had a eerily similar conversation. That conversation with my sister made a huge impact on my life even though I was only 4 at the time. I would never claim to know what it is like to lose a child or to use palliative care to ensure that your child is comfortable but having that experience with my sister has made me far more empathetic to individuals and families who have chosen that care path. I did not then and would not now join the "Dear Julianna" campaign because while I am not now on a palliative care path I can understand how someone, yes, even a 5 year old, might very well choose to never go to the hospital again and to choose to be at home when the time to fly comes. In truth, who would choose to go out in the hospital attached to more machines and invasive mechanisms than ever or at home surrounded by the people and things that you are most familiar and comfortable with (and this can totally include machines, if so chosen)? This, quite seamlessly, brings me to the backlash surrounding the movie "Me Before You". I must first say that I have not yet seen the film so my analysis is based solely on the novel of the same title. I began reading "Me Before You" written by Jojo Moyes a few weeks after watching THIS trailer that had been posted by a friend on Facebook. There are, in my opinion, not enough books let alone movies that explore the reality of someone with a disability falling in love and that was my impression of what the book would be about. Once I purchased the book on Kindle I did read the description on GoodReads and braced myself for a tear jerker upon reading some of the reviews that alluded to the right to die theme of the novel. As the release date of the movie grew closer I began to see more and more people post scathing reviews of the movie they hadn't seen based on a  book they hadn't read. I'll leave my feelings about that particular aspect at the door. I did not agree or disagree with the multitude of opinions because I knew something that everyone else seemed to have totally overlooked. "Me Before You" is  fiction, aka pretend, aka Will Traynor did not then and does not now exist. Sure, the reason that it is a best seller and was turned into a movie is because, like thousands of other works of fiction, it is believable and could easily be a biography. But, and this is huge, it is really hard for me to be up in arms against a work of fiction. That part put aside, I cried my eyes out at the end of the book. I have always been a bit of a weeble when it comes to any part of the right to die movement. I feel that life is so precious and far too often people in the medical profession don't provide individuals and families the option to choose life. That is an issue for me beyond this book and the stories at the forefront of the right to die movement. If all of the options are presented equally to the individual or family in each case and they still decide to pursue any of the options then I cannot personally disagree with any choice. Again this makes it an easy transition to the "Appleton teen makes heartbreaking decision to die" headline that is now at the forefront of the ongoing argument of people who fall on opposite sides of the aisle. This is by far the most difficult situation for me to share my feelings about. The "Appleton teen" is not just another face in the crowd to me. In addition to the fact that I have the same diagnosis I have also personally known this now teenager since she was a little over a year old. She has a name, a family, and numerous friends who all love her immensely and I am blessed to be a small part of that circle. Knowing her means that I know that her decision is not a flippant one made because she is having a bad week or because she is suicidal due to depression. Knowing her means that I have watched her undergo surgery after surgery in an effort to relieve the unending pain she has been experiencing for more years than any person should. Knowing her means that as I read the backlash from people who do not it breaks my heart with each word of "I would NEVER allow this" or "I would NEVER choose this" or "If it were me/my child". First take a minute to think about and reread what you just typed, take the focus off your own situation for a minute and realize one extremely important thing...This. Has. NOTHING. To. Do. With. Y-O-U! This is not your story and you should be thankful that it isn't so take one giant step back and realize that. You, like me, may have the same diagnosis as her but it is not your decision.

I can't write anymore today without this going down a very dark road that I can't come back from. What I want to end with is a line from "Me Before You", slightly altered to apply to all situations, “I want you to live if you want to live. If you don't, then by forcing you to carry on..... we become just another shitty bunch of people taking away choices.” ― Jojo Moyes, Me Before You

The Bridge

If you know me at all, you know that I draw inspiration from everything. I read a lot, listen to different types of music (contrary to popular belief it is not just country), I love dialogue from movies and television, I have more than 2 thousand quotes pinned on Pinterest; anything can be inspiration for me to create. Oftentimes I get an idea that won't let me be until I do something with it and this latest video is a good example of that.

I heard the song "Help You Stand" by Anthem Lights a few weeks ago and instantly knew that I needed to do a video of SMA kiddos using the song. It was a quick turnaround time to making the video although it has been sitting in Youtube purgatory for a few weeks now waiting for the perfect time to be released into the world. Most of the videos I make as part of B4SMA Productions have simple descriptions. This video does too; dedicated to newly diagnosed families. Know that you are never alone and that there is always someone who will "Help You Stand". Simple right? Except that there is really more than that to this video and while I usually keep this kind of reasoning to myself, this time my brain won't let me.

I've been an active member of the SMA community since I was twelve. That is a lot years, a lot friendships made, and a lot of lives lost. Over the years I have changed my position on a lot of the "controversial" topics of SMA care. I have gained perspective and understanding that the twelve year old version of myself could not begin to comprehend. Being a part of the community for so long has been a blessing and a curse. You would think it would get easier because the questions and choices haven't changed much in the fourteen years. Parents still question their decisions, still think that they are alone in the fight, ask me what I would do. I pretty much still have the same answers to the questions but I think over the years I have been able to find a way to be supportive of every choice because I am not a parent. Whatever decision someone else makes is okay because I know that they are the only ones who have to live with that choice (thank you Shelle for reminding me of that regularly because I tend to forget). That fact doesn't make deciding what is "right or wrong" for the individual and family any easier. I have sincerely thought about this a lot in the last few weeks because I have been entrusted as a good person to talk to for a bunch of newly diagnosed families. This is a difficult position to be in because I know that every situation is different and as much as I may want to I can't make the process of navigating SMA any easier. I have experience with a lot of aspects of SMA and if I don't I usually know someone who does but no one can make decisions for another family so the best I can do is say "this is what worked for me" and leave it at that. Lately I have had this analogy floating around in my head and I feel like I need to get it down in writing so bear with me here as it makes sense in my head but no guarantees on paper.

When you receive the diagnosis of SMA, it is kind of like coming to a bridge that spans a huge canyon. You can't even see more than a few of the slats on the bridge because it is so foggy and dark. From what you can see the bridge doesn't look very sturdy or safe and it seems deserted. You have three choices for getting across and none come with a map or GPS. Your first choice is to take a few very tentative steps forward. You look forward and the path is dark and scary so you decide to sit down and hope that the sun will burn through some of the fog. You look down and it is all darkness. You're pretty sure thousands have gone before you, never to be heard from again, and rather than risk disappearing into the void, you pitch a tent and curse the fact you didn't pick the Disney World vacation instead of this "choose your own adventure" kind of thing. You know giving up is a perfectly acceptable option because you're only going to give yourself a few days to digest what is going on and then you will continue. You're afraid to look up when you do start walking again but you sense that you are not alone. There are others taking the bridge the same way you are and you're so thankful that you're not alone. You can do this, with the others. You have two other ways to get across this bridge too. The second one is even scarier because after you have taken a few steps you feel someone behind you. They weren't there a few minutes ago and now they are pushing you across. "Wait!" you shout because the slat in front of you is broken and you're pretty sure that you are going fall through but they keep pushing and somehow neither of you fall. You know that people behind you have crossed the bridge before and some of the ones in front of you are pulling because they know it's better if you run across the bridge. They are experts and some of them have done this journey for more years than you have been alive; surely they will get you across safely. You continually look behind you unsure if you would have been able to cross that last part if it weren't for the people tugging and shoving you across. You are getting across anyway but unlike the first way of crossing you have little time to understand or enjoy the scenery because of the sense of urgency. You get across but at the end you realize that you don't really know how you got across and there is another bridge up ahead. You realize that you got across with help and this second bridge doesn't have guides so you are going to have to figure out the way across all over again and you have to do it by yourself. The guides from the previous trek are in the background and you know if you scream loud enough someone will come but you don't know how long help will take to come. You begin the journey across the second bridge and remember to pay attention on how to get across with minimal assistance because you have no other options. You also remember to enjoy the scenery because you will never cross the exact same bridge again. The third and final option is just as scary as the other two. Again the bridge seems deserted and unstable. You begin walking and, looking over the edge, get terrified. It's so dark and you can't imagine how anyone got across. You walk slowly and stop sometimes looking behind you to see if you are still alone. Maybe you should turn around and go back? This doesn't feel right. Then you feel something brush your hand. Turning you see a smiling face. They are holding out a hand and offer to walk with you. You're thinking that it isn't always a good idea to talk to strangers but you're scared and the person says that they have walked this way once before, it's easier with a friend. So you walk together. Sometimes you feel the need to sit down and rest. Sometimes your friend sits with you and points out the beauty surrounding you, noticing things you would have missed walking by yourself because you kept looking down. Other times when you sit, your friend keeps walking and you occasionally lose sight of them for awhile. There are parts of the bridge that you have to walk alone and it is scary because you think you're never going to find your way out. But somehow you find the way and when you trip, your friend appears in front of you, offering a hand or an encouraging word. You make it across in your own time and own way.
Yeah it's convoluted but it's how I feel. When I was younger I was solidly in the  second option, pushing people across the bridge because it was the only way I knew to get there. Now I am hoping that I am more of a number three. I am honored to walk with others and point out the beauty but I also have to walk away sometimes and let you stand up by yourself. I have my own bridges to cross so I can't be your everything because I have to let you find the right path for you. Maybe our paths will cross again and maybe not but please know that I am reaching out a hand to Help You Stand whenever you need it.
So if that still doesn't make any sense try reading the lyrics below and definitely watch the video.

Help You Stand | SMA Awareness

Lyrics to
"Help You Stand"by Anthem Lights

Picked up the phone again
Put it right back down
That was such a lame attempt
To try to help you out
If I said to myself I'm not scared I'd be lying
Don't know if I know what to say
But I'm trying
So I picking up the phone again

I hear you fell down
I'm here to let you know
Not giving up
Not gunna let you go
I'm not judging
I'm just loving
Not pointing my finger
But I'm reaching out my hand
I just want to help you stand
Oh oh oh
Just want to help you stand
Oh oh oh oh

The tone that's in your voice
I can recognize
The shaking from your choice
I can empathize
I'd said I had never been there I'd be lying
Jesus picked me up when I was crying
Broken down on the ground
That's why I'm calling you now

I hear you fell down
I'm here to let you know
Not giving up
Not gunna let you go
I'm not judging
I'm just loving
Not pointing my finger
But I'm reaching out my hand
I just want to help you stand
Oh oh oh oh
Just want to help you stand
Oh oh oh oh

He'll meet you where you are
He loves you where you are
He's not pointing a finger
He's just reaching out his hand
Oh oh oh oh

He'll meet you where you are
He loves you where you are
He's not pointing a finger
He's just reaching out his hand
He just wants to help you stand

I hear you fell down
I'm here to let you know
Not giving up
Not gunna let you go
I'm not judging
I'm just loving
Not pointing my finger

I hear you fell down
I'm here to let you know
Not giving up
Not gunna let you go
I'm not judging
I'm just loving
Not pointing my finger

 

I hear you fell down
I'm here to let you know
Not giving up
Not gunna let you go
I'm not judging
I'm just loving
Not pointing my finger

 But I'm reaching out my hand
I just want to help you stand
I just want to help you stand

 

 

Offering Advice

[An excerpt from a conversation on a Facebook SMA support group. For privacy purposes names have been altered.]

I hate conversations like this. I hate that someone even needs to ask questions like this. The side effects of a diagnosis of type 1 SMA are potentially devastating no matter how you approach the use of equipment and g-tubes; there are no guarantees and that, truthfully, sucks. That being said, equipment and g-tubes are a valid option and one that should be examined on an individual family basis. I would never suggest that the equipment is right for everyone but I believe it is extremely dangerous to suggest that a family continue orally feeding if the child is obviously aspirating. Whether or not it is what you would choose for yourself matters very little when a life is at risk.

In case you're wondering what makes me qualified to be discussing this, I will give a brief history on my life experience because I do have some. I am 26 years old and have SMA. What type? Probably debatable on a number of levels but my neurologist at OSU says that I am a super strong type 1. Maybe he is right and  maybe not but I am one thing for sure; me. I, some how, (I choose to say by the grace of God) survived to the age of 8 without breathing support and until 16 years old without a g-tube. I am living proof that you don't have to have those things to survive but I am also proof of how horribly things can go if you don't use them. I don't suggest that anyone, no matter the type, follow my path because it is a miracle that remains unexplained that I survived. It was difficult and I would not want anyone else to have to go through everything that I did to get to this point. I now have a g-tube, j-tube, and a trach. No one told me to make these decisions and the doctors even tried to  talk me out of them. My options at the time of trach placement were 1) live in the hospital 2) get a trach or 3) die. Perhaps my life experience should have told me to give up and die since no one agreed with my decision. But I am stubborn and refused to live a life isolated in the hospital or die.

"Carl", it is admirable that you have been able to survive without any support, it really is. However, I think that you need to be careful when doling out advice to families with small children. My old physician regularly told other families not to worry about equipment because their child was similar in strength to me so they would not require assistance for many years either. More than 5 of these children ultimately passed away because they didn't have what they needed because they were predicted to be like me. That is a lot of guilt to live with day to day even though I was not directly involved. The likelihood that there is another person in the world who is exactly like you or I is very unlikely. This is why I am always happy to share my story with anyone but I am not going to say that they will have a much better chance at life if they follow me. My journey is not typical for a type one and I am well aware of how blessed this makes me. It is reckless and dangerous for individuals who are not typical of the disease process to run around selling snake oil to people who are sincerely desperate for any kind of hope. I am so honored to be able to share my story but I have a responsibility, as do you, if you remain a part of the community, to point newly diagnosed families to people who have experience with the average path of a type 1. We can be a source of hope to families only if we are realistic and the reality is we are not the norm. I don't believe in false hope but I do believe that people can easily be misled into believing that if they ignore their child's weaknesses they will improve and be like you. The reality is that everyday children are dying in spite of their parents doing everything they can to keep them alive.  Life is tenuous for everyone but even more so for individuals with SMA. Share your story of hope but do it with respect for people who have lived differently. They have valid and important information to share as do you but no one is going to respect your opinions and experience if you blatantly disregard and disrespect everything that others do because it isn't your experience. You can be an example but not if you don't show a bit of compassion towards others. Respect in this community is earned not freely given away just because you're an adult with SMA. You need to show respect for parents by answering questions when they are posed. You can't be an example if you get offended by people asking for clarification. You may have a wealth of knowledge but it's of no use to anyone if you keep it for people you deem worthy. You're not a true type 1 and I am not either but I accept that I don't know everything. I don't claim to know what to do for the weak type 1s. I know my journey and that is all.

Dear Me

Dear Me (The grammar in this letter is completely screwy since I don't make a habit of talking to myself, especially in the third person but I will give it a go.),

Me at 13

I'm writing to you from thirteen years in the future. Since I know that math has never been your best subject, this is your twenty six year old self writing to your thirteen year old self. I regret to inform you that math doesn't become your best subject until your second quarter of college when you abruptly change majors and end up in "practical math", where you learn to balance a checkbook and make a budget. You get the only A in the class of twenty five students. No, I will not tell you to switch degree paths earlier in your college career because your original choice gives you a lifetime worth of entertaining stories to share with your friends and family. I will say, however, not to stress yourself out so much when you do decide to change majors three weeks into college; no one will be disappointed in you and you will find yourself in your second degree choice.

You are going to be sorely disappointed to learn that your life at twenty six doesn't look anything like you anticipated but please take heart in knowing that you are alive. In a few short years your ability to survive will be questioned regularly by the experts in your disease. You have already outlived your supposed expiration date by quadruple at thirteen but, brace yourself because, from a health standpoint, your life gets a thousand times harder than it is right now. Sorry, I know that statement will terrify you because you hate hospitals, surgeries and, most of all, needles, but I won't lie to you. I want you to cherish what you have now without giving away the story. You're in for a lot of battles, physical and emotional, in the coming years. I'll give a small spoiler and tell you that you will get what you want most, as far as medical care is concerned, when you are twenty. You know, the thing you begged Dad and Dr. Bach for this past summer? Yeah that; you get it and although it is a battle that you have to fight, don't worry because you, ultimately, win that one, with your wit and ability to talk a million miles an hour fully intact. Now, don't get too excited just yet. You have a lot of battles before and after that one and convincing Dad turns out to be one of the easier ones. You will be shocked to learn that you speak up for yourself with passion and are pretty convincing now that you are supposedly a grownup...you still don't feel like one at twenty six but treasure the fact that you don't always have to make decisions for yourself at thirteen. I know that right now you want to be solely responsible for your life but it's not quite as much fun as you anticipate. Slow down and enjoy letting Dad and Brenda be in charge. I know you're not going to listen to that because you are stubborn (and you still are) but I had to say it to at least to pretend to be a responsible adult.

Now you're probably thinking "well at least I will have the plan that I made with my best friend, surely that must work out". Sorry to burst that bubble too, sweetheart.  Your plan to be best friends forever, get married to brothers (preferably from the Backstreet Boys or The Moffatts), adopt a girl and boy, live in a beautiful ranch-style home next door to your current best friend is a big fat no go. No spoilers for this part of your life because I want you to make the same decisions even though they are seriously painful at the time. You will get through it, a little beaten and a lot bruised. It will hurt terribly the first time one of your so called friends says "oh! you have not died yet?". But you will learn to respond with witty lines like "I feel alive but it is quite possible I am haunting you from beyond the grave." and brush it off. You will learn that you relate better to people ten to twenty years your senior and you will find true friends, I promise. You will find acceptance in the strangest places and you will find love. It won't last long, it won't be expected or in the usual way but it will be real.

You're probably getting the impression that at thirteen you're living life at the best it is going to be and I don't want to leave you with that impression. You have a lot of difficulty ahead of you, I can't change that and quite honestly wouldn't want to. You're going to scare yourself plenty in the next few years but you will also find unbelievable joy. You have so much greatness coming your way. I know that one of your biggest concerns right now is having a purpose in life. Trust me when I say it will find you. You'll never believe how much influence you will have in the community. Be careful though because you have a lot of responsibility too and that could overwhelm you when it happens the first few times. You will get to live in your favorite state for more than six years (so far) so cherish your proximity to the ocean and authentic Italian food while you have it. Don't live life inside your game of Sims (yes, I know it's fun but it is really isolating in the long run so play with your own life in the real world sometimes please. Your vitamin D levels will thank you later on). You will be told again and again that you can't do something because of your physical limitations but you will find a way. You will go to college and be just as miserable as your 

Me at 26

classmates. Please talk to them and don't hesitate to form friendships with the ones who are willing to see past the equipment and wheelchair, they are worth it. Do me a favor and try to get and remember the name of the guy you sit behind in Bioethics because you will see him often after college and he will know your name. When you start going to a new hospital in Ohio trust Brenda's sixth sense when she says that you are really sick. Trust will remain a huge issue for you but don't stress about it too much because you will adjust and learn who is worthy of your trust...even if you end up burned a few times before you figure it out. It'll build character, supposedly. Most of all trust yourself, you're usually right and even when you're not you are learning from the experience.  You're going to be different than you're currently picturing yourself both inside and out. It's a bumpy, curving, twisting, road filled with hills and valleys but it is so worth it. Really, it is!

 

Love Yourself

PS You're going to love Miss S. even more in a few days and she will be a part of some major days in your history. It will be a scary day in American history but when she offers you a hug, accept it because she needs it as much as you do.

Passion

Passion. I've been reading some extremely passionate posts as of late that are getting to me. Since I have been unable to think about much else than the hidden agenda behind the posts I am going say something.
Passion is hugely important in any life, that is a fact. Having passion for something gives life purpose and direction. Passion is something that allows us to explain why something is important to us with conviction. That being said, forcing your passion down other peoples' throats doesn't endear you to that person any faster. I am passionate about many things but I don't believe that anyone else has to be passionate about what I am. I am totally comfortable being alone in passion and don't need validation from anyone else.
I was going to leave it at that, cryptic as it is, but I can't allow myself to let it go. Perhaps I am in the minority but I feel the need to speak for myself whether or not anyone agrees with me. I know that the words "inspiration", "hero", and recently "warrior" are irksome to many adults with disabilities. I don't resent any of these words, when used in context, of people who genuinely find my life story to be encouraging. I don't relish the responsibility associated with being an inspiration to someone but, simultaneously, I am not phobic of the title either. It is a personal choice to accept or condemn the use of these words and I have chosen to accept the term, assign it to others, and live life to the fullest no matter what. However, more than anything, I find it hurtful to say that families who have lost their children to SMA should not refer to their lost loved ones as "angels", "heroes", or "inspirations". I will not say that every family must subscribe to these titles as it is a personal and familial choice. I will say that families who choose to use any or all of these titles should not be condemned for doing so. I do consider many of the battles being fought by tiny children worthy of being referred to as heroic and inspirational. I look at the struggles faced by many of the children and find myself in awe of their strength to push through the toughest of times. Mothers and fathers who have lost their children and remain dedicated to the community and awareness are the most heroic in my eyes; my own father being one of them. SMA, whether you choose to recognize this or not, steals the lives of individuals every year and it is a reality that parents around the world have to live with. It is disheartening, to say the least, that certain individuals can't see how their statements could be extremely hurtful to those who have lost someone to SMA. I am in agreement that SMA isn't a wicked aspect of my life, I have things about myself which I have no control over and certainly can not change. It does not make me or the disease wicked...It just is... I must completely disagree that assigning titles such as "hero", "angel", or "inspiration" make someone lesser or to be ridiculed. We do not know that family's journey to using those words to describe their loved one and should not judge what we do not know.
In conclusion, I know August is a month of passion for the SMA Community geared towards awareness. Don't worry about the wording of your awareness posts if you choose to include warrior and angel in your posts. In my opinion warriors are individuals who fight battles; whether those battles are fought with the mind or with the body matters little in the long run. If you are fighting for something, anything, you are a warrior to me. Fight on

Seize the Moment

For days now I have been trying to think of a way to say what I want and not have it sound cliché or, worse yet, stupid. It is ridiculous how many people find a phrase and say it so much that it starts to lose the intended meaning. That is exactly what I don't want and, while it is flattering to be quoted, I didn't write this so don't quote me.  There, now that that is out of the way, let's get to the point...

For most of my life, at least since I was 9, I have said that quality of life is more important to me than quantity of days. There is quote that might better explain it; "I'd rather have thirty minutes of wonderful than a lifetime of nothing special." ~ Steel Magnolias. It is the truth for me. At 9 years old I probably could not have expressed it in such a heartwarming manner but I certainly have lived my life in this manner for a very long time.

Unfortunately, the last year or so has kind of flipped that around for me. Certainly there have been moments where the quality far outweighed the quantity of time (see this collage of photos from June 2012) but more often than not I have found myself simply existing. That was never part of my plan but we don't always get to control how the moments play out in our lives.

 I learned very early on that I can control very little in my life and that is, a lot of times, a great thing. I would not have had some of the amazing experiences I have had if I insisted on being in control of the situation. Control is important in some situations (medical treatment, for example) but I am far more accustomed to not being in control...but I digress.

Existing seems like the easiest path when you're not feeling well. It is easier to sit back and relax than to continually push for a quality moment. That being said, I have come to the conclusion that I don't want to live that way. I have been given the opportunity, denied to so many, to live. I should not waste that opportunity by simply existing in it. Will every day be fun filled, memory making, never forget kind of day? No, probably not but that doesn't mean that I don't owe it to myself and life to live it.

I recently finished a book, Forever is Over by Calvin Wade, that basically summarized what I want out of life in the first chapter. Obviously I am well aware that not everyone can or will choose to live their life this way but, if you do, do it well and with passion. For yourself, your children, everyone you know because life is worth it.

Enjoy it, learn from it and more than anything, never take your health for granted. Don’t just seize the day. Seize the moment. Every single one.

PS I Love You

(This post is a few days late or a few months early, depending on how you want to look at it. Either way it is important to me so please, read on...)

In the traditional sense of the word love, I may be the most naive 25 year old ever but that is okay with me. You see, I don't have an epic love story, pop music love song (containing far too many "baby, oh" lines), not even a love limerick to be shared with the world. Nope, nothing. But my love story is better than any Hollywood blockbuster or chart topping ballad; it is real and heartbreaking.

Logan with his B4SMA Blanket

It isn't a fairytale but it does begin long ago and far away in a land called Arizona. At the time, I was a junior in high school. I spent a lot of time on the internet because, other than precalc, I finished school work pretty quickly and was weeks ahead due to being homeschooled. On this particular day I came across a post on an SMA website pleading for help. The poster had a good friend who had a son who was critically ill and in the hospital. At 6 months old, this baby had already been resuscitated several times by his mother and father. Jennifer, the friend and poster to the SMA website, and I became fast friends. We would email frequently about ways that the hospital could help Logan do better. We also sent Logan a blanket from B4SMA. The blanket was covered in teddy bears with wings. I had chosen the fabric months earlier because it reminded me so much of my sister, Emma, who lost her battle with SMA in 1992. When Jennifer said that Logan's nickname was Logibear, I just knew that he had to have the teddy bear angel blanket.

After Logan was discharged from the hospital, Jennifer got me into contact with Shelle, Logan's mom. Over the next few months we communicated almost daily. I fell in love with Logan's spirit and his family. During the fall of 2005, Logan's dad, Chris, asked me which baseball team I liked better, Mets or Yankees. Being raised in NY one would think that I would have a preference but I really don't like baseball so I didn't have answer for him. I told Chris that I didn't like baseball but I liked the Steelers, knowing that Chris was a huge Pittsburgh Steelers fan. He was so excited by that response that he said someday Logan and I would be engaged and married.

Now, I know what you must be thinking. An approximately 17 year age difference is not okay. But it was the real thing. I don't mean that I was robbing the cradle. I loved Logan and he me. Just like a real boyfriend and girlfriend we sent each other gifts and "emailed" and Skyped often.

In February 2008, I was in the hospital recovering from the flu and trach surgery. I was discouraged and just generally unhappy, having been in the hospital for more than 50 days at that point. I received an email with several photos attached from Shelle. (I'll include the pictures below so you get the full effect)

Needless to say I smiled for the first time in days and quickly responded with my own picture (which I can't seem to find, regretfully). In any case, I said yes and throughout the SMA Community we were considered engaged. Logan sealed it with a promise of a ring as soon as he saved up enough allowance.

After over a year of being engaged to Logan, the unthinkable happened. For all intents and purposes it was a typical Monday for me. I slept in, not having class at all that day. Around 11, Brenda woke me up and I instantly knew by the look on her face that something was wrong.

"What?" I asked, not trusting my voice to say anything more. Brenda looked right into my eyes and said the worst two words in the world.
"He's gone."

I didn't even have to ask who, I just knew. Logan hadn't been sick and yet I knew, deep down inside my heart, that he was gone. It was not long after that my cellphone rang. It was Jana, one our SMA friends, calling to tell me what happened to Logan. As I listened I cried. He was only four years old and he was gone. After I hung up the phone, I threw myself into making a video of Logan's glorious life. Shelle and Chris used the video at the celebration of Logan's life.

I never got to meet Logan in person but everyday, he reminds me of what true love is really about. It doesn't matter that we were 17 years a part in age or thousands of miles a part in distance. Logan loved me and I him...I always will. Love isn't about numbers, it is about the signs I get on my worst days or my best or even while writing this post. Logan would have been 8 on the 5th of this month and I still miss him every day.

P.S. I love you, Logibear

The Ugly Truth

 

In the winter of 2010, I wrote that I was privileged to have SMA. Now that privilege doesn't seem to ring as true for me. Shocking, right? I am usually the girl you can count on for the upbeat and eternally positive. Don't worry she didn't go anywhere permanently she is just on brief hiatus. Today I am a grouch. I am angry. I am whatever the inverse of happy go lucky is and most of you don't know that side of me. But please don't be afraid because I think you will understand, once I get to the point. Bear with me...

At 25, I have had my fair share of bad days. I remember clearly the first day that I realized I was different. Oh the horror of being 6 years old and realizing that I was, to my udder amazement, not the stereotypical first grader. Staring at the brick wall of my elementary school, in a self imposed time out, I came to the realization that I was different. This epic awareness of my differences was terrifying at age 6. Where had I gone wrong? Why did I have to be different? As the reader, you feel for 6 year old me, don't you? Suddenly aware of the huge differences between myself and the other 6 year olds that made up my playground playmates. But as the reader you are also assuming that I cried, hot streams of anger, because I could not participate in some physical activity that segregated me because of my physical limitations. That is where you would be wrong. Those tears of inequality streamed down my tiny round face because I had suddenly realized that I was not and would never be African American. Yes, it seems ridiculous now that it had not occurred to me previously that the whiteness of my skin disqualified me as an African American but the 6 year old me was devastated . My differences in physical ability or lack thereof didn't impact my self esteem until much later in my life.

Now, what was the point of that story, right? My point is that, in general, the situations that are supposed to make me mad, don't. I am not angry that I have SMA. I am not grouchy that the things that I could do 5 years ago are a distant memory of abilities. I am privileged that I am alive. I am privileged to have been raised by individuals who didn't let me have pity parties often. Today sucked and everyone would agree with that assessment, SMA or not. Having said that, it doesn't mean that tomorrow won't be a great day. (it probably won't be because it is going snow and ice tonight but that is another story for another day) Seeing that today was not so great doesn't guarantee that tomorrow won't be at least a tiny bit better. Having SMA isn't the privilege; being alive is!

Its Not in My Chart or my Declaration of Independence

The following document is something that I got from a great friend. He gave the bare bones and I filled it in with information that I think is important. The information below is intensely personal and real. It was written over the course of 2.5 years and contains a lot of information that is not public knowledge; until now. Comments are always appreciated but please be gentle with my life. Please feel free to take components of this and make it your own. Parents reading this, consider creating something smaller and similar for your child. It can be as simple as your child's likes and dislikes. Whatever you think physicians, nurses, respiratory therapists, other therapists, etc need to know that isn't in a medical chart.
 

Hospital Experiences

As many of you know, I was released from an 11 day hospital stay on Friday. My second shortest ever. During those 11 days, I learned a lot about myself and the people caring for me. Let's begin with the fact that I have been going to this hospital for about 4 years. Despite the fact that they have recently relocated, it is the same hospital. Going to this hospital, I know what, and usually who, to expect. This time was different, very different. Since the relocation of the hospital, the floor that I used to be on has been divided. Basically, the RNs chose up sides based on their interest in intensive care or asthma and cystic fibrosis. This was a bit concerning for me at the beginning...I've grown attached, felt comfortable with, knew who would take awesome care of me. Day 2 of the hospital stay brought my first change in care providers: a new nurse, a new male nurse. Let me state for the record that I have zero issue with nurses gender and quickly found a groove with him. He teased me, I teased him. That made for a great report between the two of us. During my stay, I ended up with 6 new-to-me RNs, quite possibly my record. I didn't get along with all of them. Some days were spent wishing that those twelve hours would hurry the heck up, while others were spent wishing that the 30 minute breathing treatment could last just a little bit longer. This isn't to say that any of medical professionals were bad at caring for me; they just didn't fit. I, personally, didn't care for their reactions to certain situations, their level of attention given, or any number of little quirks. We just didn't fit. For people who are hospitalized frequently or for a long period of time, the hospital staff becomes the epicenter of the patient's social life. Good or bad, right or wrong, you are forced to spend 24 hours a day with these people. Like it or not, you have at least 4 hours with someone who may or may not know anything about you, your underlying condition, or the reason you're there. It is a job to many, nothing more, nothing less. They are being paid to make sure you survive their shift. Sometimes the individual will go above and beyond, remembering that you are a person, basically trapped, and treat you with kindness and respect. I cannot say that I experienced more than either kind of medical personnel, but having just one in your day can, unintentionally, ruin it. That being said, my carers, for the most part, brightened my days. From the RT who held my hand and wiped my tears during the 7 attempts to place a PICC line to the nurse who hasn't taken care of me since my second hospitalization and fell right back into our routine. From the PT & MT who teased me mercilessly just to make me smile to the RT that would bring students for me to teach to break up the day. From the RT student who reminded me that I am just a girl and it is okay to talk about normal 24 year old things to the physician who stayed all of a Sunday just to insure everything went well for the following day's surgery. Hospitalizations are never fun but you make it bearable.

Change

Change is something that many people do not embrace. I happen to be one of them. Whether it is something small, like bad weather canceling an event, or something big, like an unpredicted medical crisis, I don’t like change. For me, I think most of my problem with change results from the lack of control. I don’t have control over much, physically, because of my physical disability and when control of my plans is revoked, I hate it.

So when forced to deal with change, I begrudgingly meet the challenge head on and try to accept it. That doesn’t always work for me and the people in my life. My most recent experience involved my very least favorite kind of change: a change in my medical team. Most people are aware that I have a great deal of complex medical needs which requires my physicians to be willing to think outside the box for the majority of treatment options. Throughout my 24 years of life, the members of my team have changed more times than I care to recount. Most of these changes were as a result of the physician moving on but some have been removed from my team because of their unwillingness or inability to think of me as more than medical record number blah blah blah. Despite the medical establishment’s general unwillingness to be open to someone defying the statistics, I must continue to utilize their services. My medical team, as of January of 2012, was finally beginning to take the shape of a winning team. With physicians from most of the –ologies, our success rate wasn’t great but they were learning to listen to me and my family. Then my world came crashing down…one of the physicians, the one that I trusted the most and had known the longest, announced that he was moving to another state for the next 2 years. What? Why? What am I going to do? He assured me that I would be in good hands and he would still be available for consultation if I needed him. Having previously been involved with almost all of his colleagues, who left me unsure of their competency at best and afraid for my life at worst, I was heartbroken and frightened. Unless he reads this, he’ll never know that I was literally counting the days until his return. While he did his best to reassure me that all would be well, I still had reservations. That was until he told me who my new doctor would be in his absence. Relief washed over me as we discussed, at length, who the new specialist would be and his understanding of my complex needs. Great! It’ll be just like old doctor was still here, right? Wrong, very, very wrong…

While the new physician is a kind person and listened to us, his lack of knowledge was concerning. Knowledge is power, right? So why after years of education, was his best answer “I’ll have to get back to you”. Better than pretending he knew exactly what to do but still. I was open minded going into the appointment but came out discouraged and confused. My new physician may not be a part of my team for very long but he will know one thing when we’re finished… Medically speaking, I’m difficult. I don’t follow the natural progression associated with any of my diagnosis and for the most part, I am infamous for throwing curve balls. That being acknowledged is a major part of being a successful team member on my medical roster. I’m difficult, I know that. But don’t count me out just because I’m beyond your level of expertise.

Ignorance is Bliss

Not much gets me as fired up and passionate as someone questioning my quality of life. Lately, in the communities I’m a part of, the topic of quality of life for someone with a severe physical disability has come up. Although the issues are separate topics (one blogged about by a family, the other two by nationally televised programs) I found myself feeling frustrated by the beliefs expressed in all three forums.

There is an old saying that goes “ignorance is bliss” but, in my opinion, that couldn’t be further from the truth. Lack of understanding, in my experience, leads to a great deal of misunderstanding. Looking at the child version of myself, you would not have thought anything was wrong with me…you would have thought that I was an average baby girl. My parents learned that I had a potentially fatal disease called Spinal Muscular Atrophy or SMA. In 1988, they weren’t offered much, other than “take her home and love her”. To this day my father says “as if we would have done anything else”. The truth is, my parents did exactly what they were told but also took the diagnosis and its accompanying timeline of a maximum of 2 years and lived. As a child, my parents insured that my life, and that of my younger sister’s who also had SMA, was full of ordinary things. We attended school, went to the beach, family vacations, spent time with friends, had tea parties, and so much more. There were medical things that had to be addressed too. We had daily breathing treatments, physical therapy, speech therapy, doctor appointments, shots and hospital stays. It wasn’t the life that my parents imagined when they found out they were having a baby but it was an awesome childhood. In 1991, my father became an unwitting single parent after my mother was killed in a car accident. He was forced to take on the role of mother, father, doctor, nurse, chauffeur, chef, and so much more. In the next year, we also lost my sister to SMA and we became a family of two. Life changed drastically as we moved and attempted to start over. Despite the hardships, my dedicated caregiver, Brenda, ensured that my childhood was nothing less than extraordinary. SMA limits my ability to do almost everything independently. I have never had the ability to sit, crawl, stand, or walk but that didn’t keep us from doing anything I could think of, from horseback riding to canoeing to flying in an airplane. My world was only limited to my imagination. Despite what passersby’s may have thought when they looked at me, I have lived an extraordinarily happy life and I’m not done yet.

Quality is defined as “The standard of something as measured against other things of a similar kind; the degree of excellence of something”. So I guess that one could say that you don’t know quality until you have something to compare it against. In this instance, no one can judge what quality is to another person. Like it or not, I feel that my life is a quality one worth living. Despite needing to rely on life giving equipment, I enjoy myself and feel that today, at the very least, is worth it. I can’t judge another person’s quality of life because I don’t live it but my inability to accurately predict that there will be quality moments in every day should not allow me to quantify their days. We’re not born with an expiration date that is visible to the world and as such no one knows how long they have. I agree with most people that however many days you have; they should be made the most of. But, that being said, into every life there must fall some unpleasantness. Not many children enjoy car seats, yet any responsible parent makes their children use them because they are to protect them. Similarly, my father chose to protect my life by providing certain equipment that may not have been my favorite. Everyone has the right to decide what is best for their family and child. I don’t have to agree with anyone else’s choices but please don’t force your choices onto me. My life, my choice. I know that life is a terminal experience and that my days are fewer than they were last week but I am determined to enjoy this “terminal illness” because that is the hand God chose to deal me. It can be hard but life would not be worth fighting for if it was easy.

From the outside looking in, you might assume that I have nothing to live for but you would be wrong, dead wrong. Yes, I require a trach and ventilator to breathe. Yes, I have a feeding tube because I can’t eat enough by mouth. Yes, I use a power wheelchair because I can’t walk. Yes, I have been in the hospital longer than I can count. But when I wake up in the morning and look out the window, I’m thrilled that I have the opportunity to live another day to its fullest. I have bad days, like everyone else, but despite what you might think, I also have some amazing days. My quality of life is probably better than some able bodied individuals. I know, at 24 years old, I’ve outlived the doom and gloom prognosis given to my parents by more than ten times. I know that tomorrow is not promised to anyone so, despite what you might see as limitations too large to overcome, I will live this day, and every one after it, to the fullest. My disabilities are made disabling by others ignorance. “Ignorance is bliss”…or is it?