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?

I want you to know...

To all the SMA kids growing up now, here are some things that I want you to know. I want to tell you some things that have helped me to live a great life despite SMA. I’m going to give you some bullet points that I hope help you live the extraordinary life I know is waiting for you.

• Don’t let your SMA define you. It will impact your daily life, yes, but don’t let it be all you are. You are smart, beautiful, talented, and sassy already at age two. I know that you have so much more inside of you that is just yearning to get out. Yes, you HAVE Spinal Muscular Atrophy but you don’t have to BE Spinal Muscular Atrophy.
• Your abilities are far greater than your disabilities. You can do so much more than you cannot do. Sometimes it may seem like your limitations are greater but I know that the challenges you have and will face will make you a stronger person. You will get frustrated by your uniqueness from time to time but remember that we are all talented in different areas. You may not be able to walk but I know that you are so smart. You can teach the world so much. With a bit of imagination and some help from your family and friends, you can master just about any activity. Embrace your differences, for they are what will keep you going!
• Never give up! Your mother and father have given you a fantastic starting point by loving you unconditionally and giving you the best chance to succeed in life, both physically and emotionally. But you know that a lot of this is up to you and your tenacity. You have so many choices to make now and in the future. You can decide, every day, whether or not to be limited by what you can’t do. If you can, be excited by the potential of each and every day. You have so much to show people, don’t give up!

Thank you for letting me be a small part of your life. I can’t wait to see all the things you have to teach us.

Newly Diagnosed Families

I was recently asked how we deal with talking to newly diagnosed families. This was my response

We come into contact with a lot of newly diagnosed families because of B4SMA. It can be a lot of work, emotionally and physically, to help families get the information they need to make the right decisions for their families. First of all, for your own mental health, remember that you can only help people who want to be helped. Offer up your story, what you know to have been helpful for your family. Give them links to websites that you got your helpful information from (FSMA, SCF, GSF, FightSMA, MFMF, etc). Be sure to share support/supply organizations as well (FSMA Care packages, Cole's Quilts, B4SMA, etc.). Mostly, from personal experience, be prepared for the family not to agree with the path you chose for your family. Should they decide, for example, to use palliative care instead of NIV offer to give them contact information to families who have chosen similar paths. Do not push your beliefs but share openly your experiences, both good and bad. It is not always easy to help but if you are prepared for various situations ,you will be fine.

We are SMA

Spinal Muscular Atrophy (SMA) is a neuromuscular condition that many people have never heard of until it is too late. Diagnosis Day – SMA comes into your life like a bomb, forever altering the hopes and dreams that lay before you moments earlier. You have just been told that your child has Spinal Muscular Atrophy and will, likely, not make it to their second birthday. Will your child be a dancer? A painter? A musician? An athlete? An actor? A scholar? According the statistics, the doctors, even internet resources, the answer is a wholehearted, unquestioning, NO. Your child will fight to breathe, swallow, move, eat, and possibly even lose their precious smile. You will learn more medical terminology than the entire cast of Grey’s Anatomy and worse yet, you will retain the information and be able to argue your position to the most educated physician without hesitation. You will learn what it means to have a g-tube vs. a gj-tube, a Trilogy vs. an LTV, and a tracheotomy vs. NIV. You will fight, fight for the care you want your child to receive, fight with others who do not understand your decisions, fight for your child’s life. And despite all of this, you will be reminded on a daily basis that no matter what you do, your child will not survive beyond the age of two. You will say, to yourself and others, “no, my child will beat the odds…we will be the lucky ones”. Perhaps you’re right and, yet maybe you’re not. There is no way to know.

But let’s back track…Why can’t you live life to the fullest? Why can’t your child, despite physical limitations, be whatever he or she aspires to be? Yes, it’s true that there is no treatment, no cure for SMA. But that doesn’t mean life must come to a halt. You can choose to focus on the statistics, the hopelessness touted everywhere you look. Or you can choose to live your life, with the knowledge that your child has SMA, and do whatever you can to ensure that your child enjoys life to the fullest extent. Go to the beach, the library, Disney World, a movie, the zoo, the mall. The options are limited only to what you’re willing to try. SMA may limit your child from washing their own face but it does not limit their ability to learn. Your child will absorb every bit of information you throw at them and will understand more than a “normal” child their age ever could. Teach them everything you can. With education, your child can be the scholar, the athlete, the musician, whatever he or she wants to be. They can be anything because you have the courage to believe that anything is possible.

Statistics matter not, nor do other people’s opinions. Perhaps the cure is a moment away or maybe a millennium away, it matters not to you. Your child is a part of your life, in this moment. Tomorrow, well that is mystery as of right now, and that is okay. You will take today and make the most of it because, for now, it is the best day ever.

Listen

So yesterday was all about why I blog and Spinal Muscular Atrophy (SMA). Today, I'm killing 2 birds with one stone, so to speak.
My friend and Respiratory Therapist (RT), whom I shall refer to as 'A', is giving a presentation to new RTs and asked me to help her by explaining things from a patient's perspective. Easy! But I also think that it is important to share my thoughts on respiratory care and SMA with all of you (who may or may not give a hoot about the subject).
I'm kind of a unique case, at least that is what A says about me. I have first hand experience with NonInvasive Ventilation (NIV) and tracheotomies. I successfully used NIV for 13 years and adored the freedom that it afforded me during my formative years. I used a BiPAP machine for about 2 years with a monarch mask. I used it nearly 24/7 and enjoyed a fairly active lifestyle (although Brenda was made into a pack horse; toting my BiPAP, suction machine, Cough Assist, and 2 marine batteries everywhere we went, on her back...and no, she doesn't have any back problems to this day). At age 9, after spinal fusion surgery, I required more ventilation than the BiPAP machines at the time could provide. I was switched, with no problems, to an LP10 ventilator. I learned how to use the vent with a mouthpiece for daytime ventilation and a mask over my nose for nighttime use. I was transitioned to an LTV950 ventilator somewhere around 7th or 8th grade for daytime use only, still using my trusty dinosaur LP10 at night. Just prior to an extended vacation Brenda suggested that I try to use the LTV 950 for sleeping so that we didn't have to bring both ventilators with us on vacation. Not being a huge fan of change, I took quite a bit of convincing but eventually caved and tried it. Suffice to say the LP10 saw it's last night of use ever! I am huge LTV fan and always will be. My NIV system served me well until the beginning of 2008 when I caught the flu and required hospitalization. After recovering from the flu I discovered that any time I was in my wheelchair, my airway would collapse. After trying several hundred techniques to keep me breathing, I decided to have a tracheotomy placed. It was a remedy that allowed me to continue the life I had planned for myself. Getting a trach was definitely an adjustment for everyone in my life but it has allowed me to continue living a great life.
A asked me to write about what I think RTs need to know about SMA. First and foremost, I have found that many individuals in the medical profession see the lack of movement that accompanies SMA and assume that a mental disability accompanies it. The exact opposite is generally the case with people, children included, living with SMA. An internet search of SMA may come up with hundreds of thousands of depressing facts that leave people with little hope and even less knowledge. My friend PB recently said it perfectly "SMA.....it is a diagnosis not a prognosis". I know that RTs don't make proclamations about life expectancy but they do support families during some of the most terrifying moments of their lives. So please, know that when you say something in a room with any patient, not just SMA, we can hear you.
SMA is, overall, an extremely complex condition. It affects breathing, eating, proper digestion, movement, tolerance towards certain amounts and types of nutrition, basically everything. With that knowledge though, know that each individual with SMA is different from the ones you have seen before. Our lungs don't all "play fair" with the same settings, some will have wild heart palpitations after an albueterol treatment and others will calmly settle into a comfortable sleep afterwards, some enjoy a great chest physical therapy session while others will scream for the entire time. We're all different from each other, just like you are different from the individuals sitting around you.
I can't possibly tell you everything I know about SMA and respiratory care. But I will leave this with a bit of advice. Listen. That's all you have to do! Listen to your patients and their families, especially the chronically ill ones, they know what works and what doesn't. You have the ability to make a huge difference in the world just by listening.