Editor's Note

The Black Swallow

Offering hope and an open mind is vital to the patient with a bleak prognosis.

Not long ago, Rick Bedlack, an associate professor of neurology at Duke University (where I also work) and the director of Duke’s amyotrophic lateral sclerosis (ALS) clinic, gave an interview to the journal The Lancet Neurology. He was asked, “If you had not entered your current profession, what would you have liked to do?” His answer: “Be a rock star.”

When he arrives to address the monthly science café I host, Bedlack looks the part: tight pants, red velvet blazer, dark hipster glasses with thick frames, pointy shoes, and spiky hair. And while he confesses that he possesses no musical talent, he embodies the spirit of the best musicians: iconoclastic, rebellious, and creative. He is a rock star — with an enormous heart to boot.

As Ice Bucket Challenge patrons know, ALS, sometimes called Lou Gehrig’s disease, is a devastating condition in which the motor neurons that control voluntary muscle movement are gradually lost. It most often occurs in late middle age. There are no effective treatments and most patients survive no more than three to five years after their diagnosis — they die wheelchair-bound, unable to move and, finally, unable to breathe (physicist Stephen Hawking’s decades-long plateau is one exception).

As former NFL defensive back Steve Gleason told Sports Illustrated, “When doctors first diagnosed me, their basic sentiment was, ‘We’ve got nothing. Hang in there.’ Thanks a lot, dude.” If you have no personal experience with ALS and want a better sense of its enormity, I recommend the recent documentary about Gleason (Gleason) as well as an older film (So Much So Fast) about ALS patient Stephen Heywood and his brothers Jamie and Ben, founders of PatientsLikeMe.

Bedlack and his colleagues have refused to accept this sense of futility. Bedlack likes to refer to the iconic 1990s science-fiction TV series The X-Files. Like the show’s tortured FBI agent Fox Mulder, Bedlack is regularly confronted by perplexing phenomena (albeit not of extraterrestrial origin). In his case, the phenomena are ALS patients who try alternative and off-label treatments (“AOTs”) and then claim that their symptoms have improved or, in a few cases, have disappeared altogether, despite little to no scientific evidence. These treatments range from the mundane (vitamins) to the exotic (hyperbaric oxygen, chelation therapy) to the dangerous and expensive (stem cells). Bedlack’s team has found infections, heart attacks, and blood clots among ALS patients who’ve tried AOTs. Worse still, patients who experiment with these treatments often either decline to participate in or make themselves ineligible for legitimate clinical trials of ALS treatments.

“If a doctor tells an ALS patient, “We’ve got nothing,” then the patient is going to look elsewhere. Like Fox Mulder, they want to believe. And the Wild West of the internet is only too happy to help them.”

Nevertheless, ALS patients will often try at least one AOT and the reason is obvious: If a doctor tells an ALS patient, “We’ve got nothing,” then the patient is going to look elsewhere. Like Fox Mulder, they want to believe. And the Wild West of the internet is only too happy to help them.

Many neurologists dismiss AOTs out of hand. After all, such treatments have little to recommend them and enormous potential to make an ALS patient’s life — and bank account — even worse. Bedlack again quotes The X-Files: “Trust no one.” And he doesn’t. But occasionally he sees something amid the snake oil and carnival barkers that gives him pause.

On a website promoting “energy healing,” Bedlack came across the story of a patient named Nelda Buss, who was diagnosed with ALS at a reputable center by a well-known neurologist, and had progressed to the point where she was quadriplegic and preparing to die. But, the story went, after undergoing energy healing, she experienced a near-
complete recovery. Bedlack found Buss, studied her medical records, and was convinced that her diagnosis and progression were real. And indeed, two years later Buss’s motor function was almost normal.

This led Bedlack to dig up rare, published cases of ALS patients who had experienced reversals — progression followed by recovery. Of course, there could be many explanations for such occurrences, but given the dearth of effective treatments for ALS and having watched so many patients suffer and die, Bedlack thought it best to keep an open mind. He reached out to the energy healer, who responded tersely: “For those who believe, no proof is necessary. And for those who do not, no proof will ever be enough.” That was the last Bedlack heard from him (he has since died).

When Bedlack’s patients come to him with AOTs, Bedlack does not send them away or treat them with contempt; he is not a paternalist. Rather, he believes in shared decision-making, which, by the way, is associated with better outcomes (at least in some diseases). Of course, shared decision-making is both labor- and time-intensive. So, in 2009 Bedlack launched ALSUntangled, a team effort by some 100 like-minded ALS investigators to systematically assess AOTs and report back on them to their patients. The ALSUntangled site (alsuntangled.org) now lists more than 200 uninvestigated AOTs, which are ranked based on how many votes users cast to determine which ones should be investigated next. The reviewers ask hard questions and make every effort to investigate the clinic and/or treatment. Capsule reviews are published on the ALSUntangled website, and the complete reviews are published for free and available for anyone to download.

Bedlack has found no shortage of real con artists and bogus concoctions peddled out of strip malls to desperate and unwitting ALS patients and their families. And even the benign treatments are usually no better than placebos. “The truth is out there,” he says in another nod to The X-Files. “But it’s not always easy to find.”

“I think hope is one of the most powerful things a doctor can offer.”

He has found some potential therapies that merit further investigation with rigorous scientific tools (he has started two other studies that are looking specifically at presumptive ALS reversals). And he has learned that many AOT providers are actually well meaning — they just don’t always understand how science works. But they give their patients things that traditional providers sometimes do not: optimism, respect, and responsiveness. Bedlack describes traveling to an alternative ALS clinic and hearing patient after patient tell stories about how doctors at academic medical centers — “the biggest and best” — would extend them nothing but “gloom and doom,” not return their calls, and often not even look them in the eye.

“That’s not me,” he says. “I think hope is one of the most powerful things a doctor can offer.”

Bedlack has a single tattoo on his wrist — a black swallow. It was inspired by a patient named Tim, he says, a young man in his twenties who came to see him after his diagnosis. Tim arrived with an entourage of other young people, many of whom had mohawks, hair dyed various bright colors, and many piercings. “I thought I’d walked into a punk rock concert,” remembers Bedlack. All of them — the self-styled “Often Awesome Army” — had a black swallow tattoo; Tim had a whole sleeve of them. ALS ran in his family, and his mother had died when he was just a toddler. She left him a book, and on every page she had drawn a black swallow. When Tim turned 16, he got every one of those swallows tattooed on his arm in memory of his mother. When he started having symptoms, his friends showed their solidarity by each getting a tattoo of one of the swallows. The Army would come to the clinic as a group, ask questions about ALS research, help Tim enroll in studies, and hold fundraisers for him. Bedlack was moved.

“Toward the end of his disease, when I saw that he kept maintaining this incredible spirit, I said, ‘Do you mind if I get one of those tattoos?’ Now, whenever it pops out of my sleeve when I go to sign a patient’s chart, I remember him. And I remember the black swallow as the tattoo that sailors used to get because it was the first bird you’d see when land was approaching. It reminds me that if I haven’t already said something hopeful to that patient — about a clinical trial, something I read in a journal — then I make sure to tell them that. To tell them that they’re not lost at sea.”