HILO, Hawaii Island — You’ve been infected with rat lungworm disease. Your doctor has confirmed it with a test of your spinal fluid — currently the only accepted method of diagnosis. What should happen next?

Unfortunately, even doctors disagree about the answer, though most agree that there’s currently no “cure” and that the worms eventually die of their own accord in humans.

Hilo Medical Center’s resident rat lungworm expert, Dr. Jon Martell, is charting his own aggressive course of treatment, which flies in the face of conventional wisdom at the federal and state levels:

• The Centers for Disease Control’s web page on the disease maintains that “even if infected, most people recover fully without treatment.” But it adds, “Sometimes the symptoms of the infection last for several weeks or months, while the body’s immune system responds to the dying parasites.”  It observes that “The most common types of treatment are for the symptoms of the infection,” including pain medication, rather than treatments to cure the disease itself.

• “No anti-parasitic drugs have been shown to be effective in treating angiostrongyliasis, and there is concern that they could actually make the symptoms worse because of the body’s response to potentially more rapidly dying worms,” claims the Hawaii Department of Health’s rat lungworm page.

Jack Russell Brauherr after regaining consciousness at Hilo Medical Center. He considers his treatment there to have saved his life.

Courtesy of Jack Russell Brauherr

For many people, rat lungworm disease is just a set of mild flu-like symptoms. But for some, it’s a nightmare that can involve bone-racking pain, paralysis, brain damage and even death. For the sufferers of those heavy-duty cases, the idea that there is no effective treatment can be hard to take.

One such case was Jack Russell Brauherr. The Puna resident gardened, raised fruit trees and served as a volunteer firefighter until last January, when he developed flu-like symptoms.  He seemed to to fight them off at first, but by mid-February, he was suffering from delirium and mostly bedridden.  When his brother finally brought him to the Hilo Medical Center, he was unconscious — and stayed comatose for over a week.

Martell immediately suspected the disease and confirmed it with a spinal tap.

Then he started Bauherr on a treatment regimen that violated the conventional knowledge listed on the CDC and DOH sites. Brauherr credits Martell with saving his life.

Hilo Medical Center, which has handled at least 80 identified cases of the disease, has been treating some severe cases such as Brauherr’s with a combination of a steroid, Prednisone, and a second drug called Albendazole, which is designed to kill the worms.

The DOH and CDC rat lungworm sites are “essentially using outdated information,” says Martell: “I just don’t think that they represent the state of the art on this or even the authorities. This (rat lungworm disease) is treated in China and Southeast Asia all the time. Australia has been very aggressive with the use of Albendazole.”

Dr. Jon Martell of Hilo Medical Center thinks the federal and state approaches to rat lungworm don’t “represent the state of the art.”

Courtesy of Hilo Medical Center

Even if the worms eventually die on their own, Martell argues, the longer you leave them in the body, the more damage they can do. The developing worms burrow through organs and often end up in the brain, where they develop, he says, into “sub-adult adolescent worms.”

“When they’re sub-adults they want to get out of your brain and back into your bloodstream where they can party and make baby worms,” he says. “They can do a considerable amount of damage trying to break out. In my mind that makes a pretty convincing case to do something with an anti-parasitic if you can.”

“We know certain anti-parasitics are safe to use in humans,” Martell says. “Albendazole is proven to be safe in humans, and it is proven to be effective for other worm diseases in humans, including some in the brain.  In addition, we know that Albendazole, given to mice that are infected with rat lungworm, does decrease the number of worms … there’s no reason to believe that it would not have the same effect in humans.”

Martell maintains that the studies that don’t show effectiveness of antiparasite drugs in treating rat lungworm disease are flawed because they don’t have enough test subjects and they don’t distinguish mild cases from moderate and severe ones.

“Our experience in Hilo Medical Center is that we have a significant number of patients with moderate and severe disease and we have been able to use Albendazole safely and effectively in this population,” he says. “When we approach our patients who are in this condition, we have a protocol to determine if they have mild disease, moderate disease or severe disease, and we treat them based on the severity of the disease and our experience in treating patients who are afflicted with rat lungworm.”

Dr. Vernon Ansdell of the University of Hawaii Manoa medical school, is curious about the Hilo Medical Center approach, but also skeptical.

University of Hawaii Manoa

Dr. Vernon Ansdell is watching Hilo Medical Center’s approach with interest, but also with a healthy dose of skepticism.

Ansdell,  an associate clinical professor of tropical medicine at the University of Hawaii Manoa’s John A Burns School of Medicine, heads the Subcommittee on Clinical Management of Angiostrongyliasis in the Governor’s Task Force on Rat Lungworm Disease, which is working on developing new clinical best practice guidelines for the disease.

He says the big question is, “By killing the parasite, are you actually making things worse or making them better?”

One of the big causes of the disease’s symptoms, he says, is that the body’s immune system can inflame its own tissues in reacting to the alien presence of a dead worm. If all the worms die at once, it could actually aggravate those symptoms.

But, Ansdell says,“There’s no real evidence that it (an anti-worm drug) does harm, so long that it’s used with steroids to suppress the inflammation.  The other side of the coin is, is it doing any good? There really isn’t any evidence that it’s beneficial, but we don’t have all the evidence at this time.”

What’s needed, he says, are “good, controlled trials, where you have hundreds of patients that are treated with steroids alone, and then hundreds of people that are on steroids with the antiparasites.  The only place you could get that would be in countries like China, Vietnam or Thailand.”

A patient like Brauherr, who was obviously losing his fight with the disease when he checked into the hospital, had little to lose by trying the anti-worm drug. Even with the drug’s help, if it helped, he’s still left with the damage done by the disease.

When he awoke from his coma, he had to relearn basic skills like walking and even swallowing, because of the damage the worms had done to his nervous system. He still has trouble with everyday tasks such as driving.

“I probably won’t be able to do physical stuff like running or biking again,” he says. And he’s fought his last fire.

Even if the worms eventually die on their own, Martell argues, the longer you leave them in the body, the more damage they can do.

Both Martell and Andell worry that Hawaii will see more cases like Brauherr’s, thanks to a new arrival to the islands: the semi-slug, an invasive species that resembles a slug but carries the vestigial remnant of a shell on its back — and a massive load of worms.

“One of those big African land snails may have 50-100 copies of the disease, whereas a little semi-slug may have thousands,” says Martell.

The rash of recent cases in Puna and on Maui corresponds with the expanding territory of the semi-slugs on the islands.

“We’re seeing more severe cases here in Hawaii than in Thailand or China, and that may be because of the semi-slug,” says Ansdell.

This adds urgency to the search for the best possible treatments.

“I would think we’ll have preliminary guidelines out the early part of next year,” says Ansdell.

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