written on June 26
Friends and family,
We hope your summer is off to a great start! Today is a GIFT…embrace it, live it, love it! Find someone who could use the gift of YOU this week and give it. Never underestimate your ability to make someone else’s life better! Even something as simple as your words or your voice can have a positive, profound impact on others.
Love to you all!
Jeff and Tara
This post is mostly educational in nature. Tomorrow (Tuesday, June 27th) is our quarterly ALS Clinic day. We meet every 3 months with a team of specialists to evaluate and monitor Jeff’s progression and manage his symptoms. Clinic days last approximately 4-5 hours and Jeff will see:
* A Neurologist to assess his condition
* A nurse practitioner who coordinates his care to make sure he’s getting everything he needs
* A nutritionist to talk about keeping his body nourished
* A respiratory therapist to help monitor/evaluate his breathing. (Breathing issues is what ultimately causes death for ALS patients)
* A team of physical therapists to keep him strong. Jeff’s body can not build muscle (and therefore working out is ineffective), but we want to maintain the muscle he currently has for as long as possible
*An occupational therapist to show him how to do small but important things like getting dressed (not needed quiet yet)
* A social worker to make sure we are getting the services we need
* A research coordinator to make sure we are aware of (and enrolled in) ALS studies and clinical trials
* A speech pathologist to help with communication (not needed right now)
* An equipment team – braces, walkers, wheelchair seating specialist, etc who helps us understand mobility options that can improve quality of life (not needed right now)
JEFF’S CURRENT PHYSICAL STATUS:
* Muscle atrophy in his back, shoulders, arms and core
* Difficulty raising his arms (challenge washing hair, putting away the milk/dishes)
* Unable to lift heavy objects (push a grocery cart, push a lawnmower, load/unload car, etc.)
* Difficulty tucking in a shirt and buttoning a collar
* Unable to reach behind neck to flip a collar or put on a tie
* Difficulty shaving
* Difficulty extending arms (for example, changing the radio station in a car, shaking hands, taking a plate from a server at a restaurant)
* Difficulty opening a water bottle
* Core strength is weakening, which makes getting up from a kneeling or seated position from the floor challenging
* Legs are stable and he can walk (not run). He did well on our challenging hike a few weeks ago! Praise and prayers that his legs will remain strong.
* Jeff is still working full time and does his job exceptionally well!
* Jeff’s attitude is nothing short of inspiring – he is positive, happy, and fighting with all his might. Proud of him.
WHAT IS ALS?
ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that is 100% terminal. “A” means no. “Myo” refers to muscle, and “Trophic” means nourishment – “No muscle nourishment.” When a muscle has no nourishment, it “atrophies” or wastes away. “Lateral” identifies the areas in a person’s spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates, it leads to scarring or hardening (“sclerosis”) in the region.
There is no cure. So far, medical advances are aimed at slowing progression. Radicava is a new drug that is estimated to be released in August 2017. We will inquire about Jeff’s eligibility at our appointment tomorrow. Radicava is shown to slow progression by up to 30%. Still no cure, just a slowing of progression. The price tag? $145,524 per year (hopefully insurance will cover this entirely, or at least a significant portion of it). The drug is given intravenously (IV) on a DAILY basis for 14 consecutive days, followed by 14 days off the drug…forever. Intense.
Average life span: 3-5 years
As ALS progresses, it eventually takes away the ability to walk, dress, write, eat, speak, move, swallow, and breathe. Complete paralysis, with the mind intact. How fast and in what order this occurs is very different from person to person.
The onset of ALS often involves muscle weakness or stiffness as early symptoms. Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows.