I thought I would start by addressing a few of the most common questions we’ve been asked since finding out Dirk’s diagnosis of ALS. The first one being, how did we figure it out?

Around Mother’s day 2022, Dirk pointed out to me for the first time that he felt like he was slurring a few words here and there which he had noticed beginning in March. I hadn’t noticed it until he made me aware of it and then I started hearing it more frequently, mostly when he was tired. We wrote it off in a few different ways assuming it was related to how much he was speaking for work on video calls, maybe a shift in his teeth or just in regards to how tired he was. 

A few weeks later other people close to him started to notice the slurring of words, specifically his mom on a long drive when she couldn’t turn to look at him. Shortly after that he had a virtual doctors appointment to rule out anything serious like a stroke. The virtual doctor didn’t think it was anything serious but told him to make an appointment with his primary care physician if it was bothering him.

At the time he didn’t have a primary care physician so he made an annual checkup appointment with a local doctor but it was a months wait to get in at the time. Shortly after on Father’s Day weekend we went away as a family to Suncadia. During that trip I became seriously concerned about his speech and how much more he was slurring his words and asked him to call the doctors office and see if he could get in sooner. They moved his appointment up to later that week on June 27th.

His primary care doctor ruled out anything serious according to her and scheduled some bloodwork, a swallow test, an MRI and a referral for a neurologist and planned to follow up a month later. The swallow test and MRI came back negative in early July. He also had a dentist appointment on July 12th which ruled out anything teeth related. 

His neurology appointment at Valley finally happened on July 25th. The doctor did a thorough examination and came to the conclusion he believed it was Progressive Bulbar Palsy and scheduled an EMG for 4 days later. It was probably one of the worst weeks of our lives as we waited for the confirmation of the worst. 

The EMG showed nerve damage in his tongue, right arm and right leg (which were the only places he tested). He believed it confirmed the diagnosis of Progressive Bulbar Palsy which was progressing into ALS and recommended we get a second opinion at The University of Washington. Our appointment at UW was scheduled for October 7th. After many prayers and making his tentative diagnosis public, a friend was able to pull some strings and got his appointment moved up to September 7th.

We then proceeded to meet with his new doctor at the UW who did a thorough physical exam similar to the first neurologist and came to the conclusion he believed it is early symptoms of bulbar onset ALS. He ordered dozens of blood tests, a lumbar puncture, a lung test, another swallow evaluation and speech therapy to try to rule out his hypothesis. A month later at a follow up appointment the results were in for majority of his tests and everything was negative. 

If you don’t know how ALS works, there is no test to show you have ALS. Instead, they test for every other possible disease or deficiency possible and if all of those are negative, they land on the conclusion it is ALS.  At his October 5th follow up appointment his doctor ordered more blood tests and an additional MRI with contrast but we decided to move forward with the assumption it is ALS so we can get started on a treatment plan and therapies. 

Since then he has also had a lung test which came back as his lungs are in a normal range for his age and height which is great. 

What’s next?

He has an MRI with contrast as well as swallow and speech therapies scheduled for November. He has begun taking Riluzole. We also applied for Radicava (Edaravone) and Relyvrio (AMX0035). They needed the lung test and a liver panel to submit to insurance to see if he qualifies for those two drugs. Because the disease is still early for him though its highly likely he will get denied until his symptoms progress.  He is currently a 44 out of 48 on the ALS Functional Rating Scale. Note, the drugs above are not cures but only treatments that can potentially help slow the disease a little. Each drug has show in clinical trials to potentially slow the disease by 4-6 months. 

The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) is an instrument for evaluating the functional status of patients with Amyotrophic Lateral Sclerosis. It can be used to monitor functional change in a patient over time. It measures speech, salivation, swallowing, handwriting, cutting food and handling utensils, dressing and hygiene, turning in bed and adjusting bed clothes, walking, climbing stairs and breathing. A score of 48 means you are perfectly healthy with no issues. 

See my “Next Steps” blog for an even more detailed explanation of his upcoming tests, drugs and supplements.