Friday, September 17, 2010


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 " 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.


CureSMA4Stella said...

So excited about your new blog, MJ! You are awesome and always manage to make me laugh or think about things in a different way. Hugs and love!!!

Shelle Harman said...

I am loving the new blog.. One more way to keep tabs on my girl! I love you!! Sending my angle to have a dream date with you tonight! Hope its a great one.. Maybe Atlantis or something awesome!! XOXO