My name is Debbie Richardson, and I was diagnosed in 1994 with Stiff Person Syndrome (SPS), following four years of progressive symptoms. SPS is a rare autoimmune neurological disorder with an incidence of about 1 in 1,000,000, world-wide.
1993 is the year that the symptoms of SPS — a disorder I’d never heard of — became impossible to ignore.
I remember a particular early morning at dawn, still able to run, a light drizzle dampening my skin. My heart beat in rhythm to my feet hitting the ground, my body in perfect sync with the swing of my arms. With agile ease, I could jump a crack in the sidewalk, avoiding a puddle. Flying on my feet, I exhilarated in being alive.
That slight drawing of my left side was still easy for me to dismiss.
But SPS ultimately developed a life of its own that year. The “slight drawing” is now an audible “clump.”
Stiff Person Syndrome (SPS) is characterized by debilitating muscle spasms in response to external stimulus: touch, sound, volitional movement, open space, emotion, stress… life. The severity of spasms and rigidity predisposes an afflicted individual to ruptured muscles, broken bones or injury from unprotected falls.
Medication, immunomodulating therapy (IVIG), and a readjustment of my life allow me to live an unpredictable “stability” with dysfunctional ability.
My Brave Year of Firsts, Tries, Signs, and High Fives by Jaime Lee Curtis became my 2013 resolution. One “try” was to attempt my first 5K since my SPS diagnosis.
With my 5K goal in mind, a Google search listed a local breast cancer run in only six weeks. The 5K (Fun Run?) could be a platform for Rare Disease Day!
Needing a gait aid, I snagged a killer deal on Craig’s List — a jogging stroller for $60.00.
I mentally listed my syndrome challenges: unpredictable spasms, medication timing, falls, crowds, open space, balance …. The physical and mental energy required each day to just cope with SPS is consuming. How could I train for a 5K in only six weeks, minus two days for infusions and four days of medical appointments?
The only failure would be not to try.
Weighing down my stroller with a case of water for stability, I attempted a few first running steps. My syndrome causes lordosis (severe “sway-back” as it is called in everyday language). Lordosis had my hips out-of-alignment for running, and I’d have to keep my torso rigidly constrained for deep breathing. And pushing a stroller with a case of water required extra energy and the loss of free arm momentum.
And so I began my first day of training for the 5K. Controlled breathing was more like a frenzied sucking for air. Choosing the cemetery for my training track seemed appropriate — I felt like I was dying!
A gentle rain on the second day lifted my spirits. I always loved running in the rain. Frantically gasping for air again, I wondered if it was possible to drown upright in a rain. With a sloshing “clump-step … clump-step”, I hit every puddle. Struggling and soaked, I noticed some ducks from a neighboring pond honking at me with each lap. Hecklers!
A gym regular, I got on the “dreadmill” to practice snail-pacing for my third try. This is always a white-knuckled death-grip on the handles for me. Choosing a #four incline for lordosis alignment, I managed an impressive geriatric trot/walk with the song “Cherokee People” playing in my mind, homage to my native ancestry. I left emboldened.
The day after my gym practice, I crashed. A resurgence of symptoms, rigidity, foyer fear, suppressed spasms, wall walking and burning pain, summoned “Gert”, my walker, out of the closet. With SPS-imposed restraint, I rested in bed on an electric blanket for two days, questioning my sanity.
Determined, I decided on another outdoor training session after my break. But I could barely do a turtle shuffle. SPS became an archer, pulling my torso forward in a painful arc without release. Deep in thought, I waddled past the heckling honkers.
In passing a barren tree, I noticed a few brittle leaves defiantly clinging to the limbs, waiting for spring. In that moment, I chose to hang on…a defiant leaf.
During one of my morning readings, I read this scripture: “… The race is not to the swift, nor the battle to the strong, nor favor to men of skill; but time and chance happen to them all.” Ecclesiastes 9:11
I was competing with the wrong mindset. I was competing against the woman I was over two decades ago, the younger me, “pre” stiff person syndrome. I am a middle-aged, 50-something “baby bolomer”, and a neurological impairment from a rare disorder is my present reality.
With a few days of training remaining, I have a new perspective….
I’ll let you know how the 5K goes.
Editor’s note: Deb’s 5K takes place on Feb. 16. Watch for follow-up on her website at www.livingwithsps.com.
RareDisease Dialog is the official blog for the National Organization for Rare Disorders (NORD). NORD’s staff and friends will share information of interest to the entire rare disease community.
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