Part 2

“We are well on our way to a unified theory of biology that will merge body and environment, brain and mind, genome and microbiomes.”

 — Deepak Chopra

The Microbiome: “over a trillion organisms in our gut”

How can the microbiome manipulate ALS disease progression? How can this information be used when developing ALS treatments? Dr. Bedlack: “I used to be on Twitter and got a lot of ideas for ALSUntangled.com. Now, we just use phone and email.” After reading a tweet on his phone asking about fecal transplants, he assumed the asker was talking about fetal stem cell transplants. But no! They were talking about fecal transplants—transplanting the stool of a healthy person into the colon of a person with ALS. “As I read about this, I was blown away. I had no idea that the microbiome family of organisms in our gut was so large: there are over a trillion organisms in our gut. I had a vague recollection back when I was an intern of the fact that alterations in this family could cause diseases of the gut. I was not aware, however, that in recent years, there is mounting evidence that there is something called a gut-brain axis, where alterations in this family of organisms can affect diseases of the brain.”

Certain organisms may be able to produce neuroprotective substances. For example, butyrate can leave the gut and get into the brain and certain organisms may secrete pro-inflammatory substances, which can possibly accelerate the progression of degenerative diseases including ALS. There are multiple ALS animal models that are based on inserting an abnormal gene into a mouse, fly, fish, or worm. It recapitulates many of the features of human ALS. It has been shown that, simply by changing the composition of the microbiome in these animals, the progression can drastically speed up or slow down.

“Now a bunch of us are racing to be the first to try to really understand if there a difference in the microbiome of people with fast progression or slow progression for these ALS reversals. If there is a difference, is it something that we could manipulate? For example, if there is an identifiable organism, maybe we could make a probiotic and put it into an ALS clinical trial in the future.”

Treatment: Tips & Suggestions

Dr. Bedlack recommends that clinicians who treat ALS regularly go to meetings such as the Northeast ALS Consortium, Motor Neuron Disease Association, and American Academy of Neurology to keep up to date on treatment options and new findings. “Beyond attending meetings, I’ve seen 3,500 people with ALS in the last 21 years. People with these diseases, they’re terrified. These are terrifying diseases. I’ve got a family member with a neurodegenerative disease. I’m terrified.”

What can help? Something not found in a textbook. The importance of the following:

• Optimism:
  • Providers can convey a sense of being proactive and hopeful and not cause the patient any additional gloom and doom.
• Responsiveness:
  • People have questions. They have problems. They don’t expect or want to wait too long to receive a reply from a doctor. If a patient sends an email saying, “I’m having trouble. I’ve got too much saliva. I’m choking on it, what can we do?” and it’s a week before they get an answer, that’s unacceptable. People with these diseases need a response within 24 hours.
• Respect:
  • Practitioners need to be respectful of a patient’s ideas. Nobody cares more about their disease than they and their families do, and they may have different ideas about treatment. Rather than being paternalistic, be flexible. Be armed with information and ready to engage in shared decision making. The website ALSuntangled.com covers topics/treatments about which it states, “This doesn’t look very promising at all, and we don’t recommend that people do it.” However, that doesn’t mean everyone agrees. There may be somebody who wants to try the treatment and wants help in doing so. “If I feel like supporting a treatment is not particularly risky compared to what else is going to happen to the patient, then I’ll support it.”