I have ALS?? What the $%&# is ALS?

As some of you know, I spent this past week at Mayo Clinic to obtain further testing, consultation and treatment for my ALS diagnosis.  This morning, I met with one of Mayo Clinic’s most esteemed ALS physicians for a lengthy evaluation and discussion (Dr. Eric Sorenson – also a U of I graduate – who knew there were smart ones) .  The guy was phenomenal on all levels.  My EMG test came back very good; with the exception being abnormal nerve readings from my tongue, which was expected given my symptoms.  My limb strength and limb nerve readings remain strong, although he did note some potential precursors on my extremities.  But, right now the symptoms are completely confined to “Bulbar Palsy” (speaking, chewing, swallowing difficulties….although I have yet to experience the swallowing challenges).  He said more than likely it will eventually spread throughout my body, but he’s seen cases where it stays in the bulbar form for 10, 20, 30 years or even bulbar cases where it never spreads, and you die from other causes.  The more normal trajectory is far less years, but we are not normal.  There is no real rhyme or reason to progression; it’s amazing how little they know about this disease.  He said the one year post-first symptom mark is an important milestone as the rate of progression won’t change much (good or bad) after that.
He did note a statistically proven correlation between age and rate of progression (the younger you are, the slower it generally progresses).  The cases that don’t spread for many years/decades are often in young people, with bulbar onset.  Bulbar onset is the ALS version you seemingly want to have as there are cases where the disease will stay as only bulbar onset and not spread to the limbs for decades (I feel so ‘lucky!’).  Bulbar onset is sometimes referred to as "top down onset" starting with the dysarthria (slurred speech), and limb onset is referred to as "bottom up onset" beginning with limb weakness in the feet or arms.  Many patients do not receive an ALS diagnosis for years, but I have caught it early and started a couple drugs, which have varying results but can’t hurt.
Dr. Sorenson did say that with my symptoms so confined and comparatively mild, coupled with the good EMG results, I would likely be excluded from interventional clinical trials at this point.  He said that could very well change in just a couple months, so it is kind of a double edged sword.  I’ve never been happier to be excluded from the club.  He said the biggest symptom accelerator of this disease is fatigue, so I will be getting plenty of rest. I could get used to this.
All in all, it was a very mentally and emotionally uplifting visit to Mayo.  They really know how to treat patients.  As far as symptoms, the speech and chewing remain difficult but manageable after a good night of sleep.  The cloudy headaches after a few minutes of force-speaking remain a challenge.  I am still going to Northwestern next week for a multi- disciplinary consultation and then Dr. Sorenson would like to see me in 3 months.  Given our great experience, we are considering doing ongoing treatment at Mayo as it's typically only a few days every 3 months, depending on the progression.   We will continue to keep you updated.
Lastly, I cannot thank you all enough for the outpouring of love, support and inspiration I have received.  Seriously, you guys are freaking unreal. UNREAL. I am truly blessed to have the greatest parents, brother, sister, family, friends and most amazing girlfriend ever (she is adorable!).  It is remarkable what a diagnosis like this will do to your outlook on life.  I am no longer preoccupied with the petty stress elevators like being late, arguing with my brother or complaining when I’m dragged out for shopping.  Just go out and enjoy life…. It is a beautiful thing.