Now that we have decided to move forward with the assumption that my husband, Dirk, does in fact have Bulbar onset ALS, what happens next?

First, let me answer what Bulbar onset ALS is so you are familiar.  ALS is Amyotrophic Lateral Sclerosis (also known as Lou Gehrig’s disease). ALS is a nervous system disease that weakens muscles and impacts physical function. In this disease, nerve cells break down, which reduces functionality in the muscles they supply. The cause is unknown.

When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS, and when individuals first notice speech or swallowing problems, it is termed “bulbar onset” ALS. As the disease progresses, muscle weakness and atrophy spread to other parts of the body.

For my husband it started with essential weakening his tongue causing slurred speech.

So what are we trying?

We are starting with pursuing the current FDA approved drugs. He has started Riluzole this week. And we have applied for the oral version of Radicava (Edaravone) and also AMX0035 or now known as Relyviro. He may or may not get approved by insurance for those drugs since his symptoms haven’t progressed very far yet but we are praying otherwise.

What do the FDA approved drugs do?

Relyvrio was designed to slow the disease by protecting nerve cells in the brain and spinal cord destroyed by ALS. In a 24 week trial of 137 patients, they experienced a 25 percent slower rate of decline in performing activities such as walking, talking and cutting food compared with those receiving a placebo. The data and subsequent analyses also indicated those patients lived several months longer.

Radicava works by relieving the effects of oxidative stress, which may be related to the death of motor neurons (nerve cells) in people with amyotrophic lateral sclerosis (ALS). Keeping motor neurons healthy may help to preserve muscle functionhas been shown to slow the loss of physical function by 33% (approximately one-third) vs placebo.

Riluzole works by blocking the release of glutamate. Too much glutamate is believed to injure nerve cells. In clinical trials it was associated with a 35% reduction in mortality in a clinical trial.

You may take all three drugs together if approved by insurance.

What else are we trying?

We have also started a few supplements after some research that we hope may help including a probiotic, Cucumin and L-Serine. And while we wait to hear about Relyviro, he is taking Tudca, which is actually one half of the drug as it’s easily accessible without a prescription. We are continuing to do research on things that might help slow his progression. At the moment we haven’t researched or pursued clinical trials. 

He will be starting speech therapy soon and we’ve started learning a little sign language to try to help extend his language capabilities. The speech therapist can also offer technology recommendations and solutions to help extend communication.