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.