The ALS Journey – Part 1

How it began

In the fall of 2019 my husband Jon, was driving his new dream truck to a full-time desk job and was an avid runner, hiker, and fisherman.  He took care of the yard work, and pool cleaning, and helped with many things inside the house.  I was working part-time at our daughter’s homeschool co-op, keeping our daughter on track with her classes, helping our son manage his Crohn’s disease, and spending time with my parents.  

In 2020, Jon started noticing that his ankles were stiff in the mornings.  Of course, we attributed it to his age and the activity he was doing.  During the next year, it became difficult to walk after standing and then it started getting difficult to stand from a seated position.  In addition, he started slurring words occasionally.  I would look at him like “What in the world”?  He would shrug and we would laugh it off.  After a few months, the slurring happened more frequently and was more severe at the end of the day when he was tired. He was also experiencing more fatigue than usual.  Since it was time for his yearly exam, he made an appointment with the Primary Care Physician.  The conversation went something like, “I must be getting old because my ankles and knees are giving me problems and I’m tired a lot but I don’t know what is going on with the the speech.”  Her reply was, “You aren’t old enough for that and I am concerned. Let’s refer you to a neurologist.”  GULP!

The Neurologist

On April 12, 2023, we saw a general neurologist whose assessment said that there was a concern for anterior horn cell disease and motor neuron disease.  He ordered an excessive series of labs, an MRI of the brain and cervical spine, and an EMG of the upper and lower extremities.  After doing all of the testing, we had the follow-up appointment on May 16th. The tests had ruled out many Neuromuscular diseases, but not ALS and we were referred to a Neuromuscular Specialist.  DOUBLE GULP!

In July we took a road trip to Idaho to see Jon’s family. On that trip, we started to notice that Jon’s reaction time was slow which caused some concern in congested areas.  Needless to say, he only drove on the open highway on the way home. He decided to try driving to work after we got home, but on the Friday of that week, he called me to come pick him up because his legs felt odd and he was very fatigued.  That was the last day he drove himself to work and I became his driver. The benefit of that was that we spent time together in the mornings and had more conversations.  The downfall was that the gas bill doubled.

The Neuromuscular Specialist

The physician we were referred to is a leading Neurologist in Phoenix, AZ.  He has his own practice that includes a research center.  We saw him in August of 2023, and he assessed that he did not think it was ALS.  He said there was a possibility of Primary Lateral Sclerosis (PLS) or Myasthenia Gravis. However, he asked if Jon had long-term disability through his work, and talked to us about getting long-term care insurance. Then ordered a few more labs, gave a prescription for Baclofen (a muscle relaxant used for Motor Neuron Diseases), and scheduled a follow-up for 3 months later to check progression.  During the 3 months, we did research on the difference between long-term disability and long-term care insurance.  We spoke to two insurance agents and were presented with two different options for the insurance part and ended up purchasing the short-term care insurance option.  It is better suited for what we are looking at.  (For more information: https://www.elderlifefinancial.com/long-term-care-insurance/short-term-vs-long-term-care-insurance/). Then we had to see how that fit into the budget. Unfortunately, we decided to trade Jon’s truck in for a new base model Escape to make up for the difference.  We also decided to sell our small fishing boat since we didn’t have the truck and it was apparent that getting in and out of the boat was going to be difficult for Jon to do.

At the appointment in November, the doctor said Jon had “subtle speech slurring, limb muscle strength is entirely preserved, can stand without assistance, and the gait is unusual with some stiffening, but not consistant”. A muscle biopsy was ordered to rule out mitochondrial myopathy. We left feeling like we were imagining the symptoms that we knew Jon dealt with every day.

At the biopsy follow-up in December, we were told that the biopsy was normal and Jon was told to increase the Baclofen dosage and start exercising by walking fast on a treadmill or outside. We had been trying that already, but walking fast was not possible without feeling like he would fall. By this point, we were getting frustrated. We felt like what Jon was feeling daily was being disregarded and we were not getting help.  More praying and discussions led us to make some changes.

Part 2 coming soon!

Lessons from this part of the Journey

• Prayer, praise, and tears are good medicine for the soul.
• Do research, but not too much Dr Google.  It’s scary!  I prefer looking at Mayo Clinic for general diseases and Barrow Neurological Institute for Neurological diseases.
• Talk to friends and family about what is going on.  Keeping it to yourself and “handling it” isn’t being strong.  It will break you.
• It’s ok to be mad about the circumstances, just don’t allow bitterness to settle in.
• Grief isn’t just for physical death.  It applies to the loss of dreams for the future, things you enjoy doing, and even selling a Ford Ranger.