In the world of the Internet, “going viral” can be a good thing, referring to something that rises above obscurity in the vast universe of uploaded content on wings of sudden popularity. One man in Kingston, Richard Engnath, is hoping to get that kind of attention, in the universe of public awareness, for a pair of real human viruses called HTLVs. That abbreviation stands for human T-lymphotropic virus, meaning a virus that has affinity for T-lymphocytes (T-cells), which are some of our essential immune cells.
HTLVs have been languishing in relative obscurity, at least in this country, since their discovery in the late 1970s and early 1980s. Two forms had been found: HTLV-1 and HTLV-2, and the first form was associated with leukemia. Then, in 1984, a related virus was discovered: Originally dubbed LAV (lymphadenopathy-associated virus) and also HTLV-3, that virus is now known as human immunodeficiency virus (HIV)—the pathogen responsible for AIDS.
As HIV jumped into the limelight, its cousins were left in the dark. The HTLVs are, like HIV, retroviruses that carry viral RNA into cells and anchor it there permanently. But unlike HIV, which demolishes T-cells from within and sends immune cell numbers crashing, HTLVs don’t kill their host cells. Instead, they produce viral proteins with mixed influences on immune cell machinery, and people who test positive for HTLV-1 or HTLV-2 often are symptom free. But in an estimated 5 percent of cases, serious illness arises.
From Frying Pan to Fire
Engnath would like to have never met the HTLVs. But a suite of tenacious symptoms led him down the path to discovering them, and he has since become a one-man HTLV encyclopedia, and quite possibly the nation’s foremost expert on the subject.
His story begins decades ago, when he emerged relatively unscathed from a risky period of experimenting with injectable drugs—a newly emerging activity in the 1960s. Though he contracted hepatitis C, he’s amazed and grateful he didn’t get anything worse. But about five years ago, his luck ran out.
“I was drug free and smoke free by then,” he explains, “and I hadn’t gotten HIV. I had a friend I had known a long time, and I was thinking it might be nice to have a child with her. I knew she had been in jail, related to a drug habit, but she said she didn’t have HIV. So I took a chance and had unprotected sex with her.”
Within a few weeks, Engnath began having symptoms. “The first thing I got was swollen glands in my throat and neck, like a mild case of mumps, but it spread to other lymph nodes in my underarms, shoulders, and groin, and into other areas, and I got mild otitis, earache.” With those and other problems, Engnath wondered if he’d gotten HIV after all. But his HIV tests kept coming back negative while symptoms kept progressing. An infectious disease specialist told him it was just his imagination.
But Engnath kept looking for answers. “Four months into this, I was on the phone with the Albany Medical Center, and someone at the HIV center said it could be HTLV-1 or HTLV-2. I had never heard of them. I got the medical librarians to download scores of papers about these viruses for me. [Engnath doesn’t have a computer.] There is a lot of research information out there, and some of it said that seroconversion time, when the viruses can be detected in blood, can take up to two years.”
The HTLV Info Void
Over the next several months Engnath visited doctors to get tested periodically, and found that each time he needed to explain what HTLVs were and that tests did actually exist. “I got a local doctor to test for HTLVs four months into my illness, but it came back negative. At seven months I went to an infectious disease doctor in Poughkeepsie, but he wasn’t willing to order the HTLV test again since it had already been negative. At eleven months I went back to the office of the doctor where I first was tested, but he had left the practice and the remaining staff knew little about it and wouldn’t test me. They told me they didn’t want me to come back anymore.”
Finally he convinced an infectious disease doctor in Monticello to test him again for HTLVs, and in January 2006 it came back positive. A more advanced test, through a doctor in Albany, came back positive, for HTLV-2.
Little is known about what HTLV-2 does in the body, but Engnath knows what it’s done to him in just a few years. He describes it as a core syndrome that’s very nasty, with infections and intermittent painful arthritis, slowly progressing neurological problems, and chronic fatigue. He has tinnitus, a constant ringing in both ears that worsens with posture and blood pressure changes, and he has vertigo. About two years ago he developed pain and pressure around the eye, with blurred vision—a condition called uveitis. His hepatitis C has started to become a problem.
“None of these symptoms are completely incapacitating, but they are very disturbing,” he says. And when they are at their worst, he gets angry that public information about these is nonexistent, even though for decades their capacity to cause illness has been known. “If I’d known about HTLVs, I would have gotten tested before I had unprotected sex, and I could have asked her to get tested too.”
The Nature of the Beast
HTLVs are transmitted between people when cells from an infected person come into contact with T-cells of another person. Such cell-to-cell transfer can happen through blood-to-blood contact, such as by sharing drug injection needles or receiving unscreened blood or organs (donors in the US have been screened for HTLV since 1988), and also by sexual intercourse. Infected mothers can pass the virus to offspring in breast milk.
Once infected, most people appear to have little indication of it. But over time, in some people, HTLV-1 can cause adult T-cell leukemia/lymphoma (ATLL), also called adult lymphotropic leukemia (ATL). In this aggressive disease, poorly functioning immune cells collect in skin, lymph nodes, and vital organs, causing swollen lymph nodes, an enlarged spleen and liver, skin rashes, increased infections, and chronic fatigue. With chemotherapy, remission rates are as high as 80 percent, but recurrence is common within a few years, and secondary malignancies may show up years later.
The HTLVs can also trigger autoimmunity, where the body’s immune system attacks and damages normal tissues. HTLV-1 has been blamed for a syndrome of progressive nerve cell damage, causing muscle spasms and stiffness or weakness especially in the legs—a condition first dubbed tropical spastic paraparesis because it was first encountered in equatorial areas of the world; it now also goes by HAM (HTLV-1 associated myelopathy). This condition is rarely fatal but diminishes quality of life; it effects an estimated 1 percent of HTLV-1 carriers. HTLV-2 appears to be associated with autoimmune problems and some cases of leukemia, but is poorly understood.
Who’s Carrying?
In the US, the National Institutes of Health estimated in 2007 that about 300,000 people were carriers of HTLVs. But with no routine testing or reporting, there is no way to know. It is likely that infection rates are higher in intravenous drug users: a sampling in 1991 of a few hundred people in drug rehab programs found infection rates to be 20 percent in Los Angeles; about 10 percent in New York City, Chicago, and San Antonio; and 5 percent in New Jersey. Some people are coinfected with HIV and one HTLV or both; the long-term impact of coinfection is unclear. Without screening and education programs in place, infection rates are likely to go up.
HTLVs infect an estimated 35 million people worldwide and are found over much of the globe, from remote tribes such as Australian Aboriginals to modern urban populations. In some areas, such as Japan, the Caribbean, parts of South and Central America, and West Africa, the viruses are considered endemic, always present in up to about 10 percent of the population. It appears these viruses have been piggybacking on human cells for as long as 50,000 years, and likely came from monkeys, who harbor closely related simian T-cell leukemia viruses. (By contrast, HIV is suspected to have crossed to humans from chimpanzees in recent decades.) In the last few years, two new HTLV types were found in people around Cameroon; those viruses are nearly identical to monkey viruses in parts of Africa and Asia.
And though education campaigns are rare (but Japan and Brazil have them), research on the viruses is vibrant: in 2007, 350 researchers gathered at the 13th International Conference on Human Retrovirology in Tokyo to share their research findings on HTLV-1. Even so, huge gaps in information remain, and little translation to everyday public health efforts is visible.
A Mission Emerges
The Lymphoma Research Foundation appears to be the only group in the US that has prepared an educational page (about HTLV-1 and leukemia) for the public. No governmental health agency has citizen awareness materials, websites, or campaigns.
Stunned by this, Engnath writes to medical personnel and public officials and federal agencies (including Obama’s Health Reform office), collects the latest research papers, compiles and memorizes data, files Freedom of Information Act requests to scour national records, visits local newspaper and magazine publishers bringing stacks of documents, and more. He’s started the National HTLV Registry in Kingston, which some people have now heard of. Using his own funds, Engnath has created a poster about the viruses—though it is overly packed with lingo that most people won’t understand. But some people have contacted him, and he knows of seven people in the Kingston area who have tested positive for HTLV.
Engnath is hoping to convince a politician to sponsor three items of legislation in New York state, though no bills are currently in development. The first proposal would authorize and fund NY State Department of Health to develop and disseminate HTLV literature, at an estimated cost of $3.5 million. Engnath laments, “I realize that we’re in recession and the state is close to bankruptcy, so there might be difficulty passing such a bill because of the cost, even though it’s clearly necessary.”
A second tactic Engnath suggests, at a cost of about half a million, is to authorize the state’s Department of Corrections to screen inmates for HTLV and its symptoms, as is currently done with HIV. “About 80 percent of inmates nationwide are arrested for drug use or sex, so they are in the high-risk population,” reasons Engnath. “STD studies from the 1990s show three times the national rate in inmates, and 8 to 20 times higher rate of hepatitis than in the general population, so HTLV could be more prevalent among this population.” His third idea would, in theory, cost nothing: adding HTLVs to the state’s list of reportable infectious diseases, which would spur doctors to report confirmed cases (and also make them aware of the pathogens).
Another goal would be to get the viruses on the Nationally Notifiable Infectious Diseases List, so that any cases nationwide would be reported as they are for HIV and many other pathogens. To accomplish that, a member of CSTE, the Council of State and Territorial Epidemiologists, would need to first create a standard case definition for the viruses, to then be reviewed and voted upon for inclusion on the list at the CSTE annual meeting. If added to the list, HTLV cases would be reported by doctors or testing labs to their state health departments, which would notify the Center for Disease Control.
Getting the Word Out
In New York City, Rafael Ortega of the National AIDS Treatment Advocacy Project has helped Engnath translate educational materials into Spanish, and Ortega has been adding HTLV information to his presentations at conferences. “It would be good if HTLV testing were incorporated along with tests for HIV and hepatitis C and the other STDs,” Ortega says. “I’m from the Dominican Republic, and we are seeing many patients with HTLV-1 infections and symptoms there. So I believe we should bring this information to everybody and keep them aware that it’s real. We support the actions that Richard is doing. He’s trying to get physicians and politicians and people with power to support this kind of test. Testing wouldn’t be a problem if people from the health department are interested in it.”
One of the people interviewed for this article admitted that Engnath has a long battle ahead of him, and that what he needs is somebody famous, like an athlete, to get behind this. Or maybe a snappy YouTube video would get this virus to go viral, Internet style. Until then, Engnath seems the best bet that any of us has for getting the word out. He says that some folks he’s contacted have been interested in his mission and some agencies are responding, though others are not. A member of the CDC’s AIDS prevention group gave him a “pep talk,“ he says, and the CDC may develop an HTLV webpage on the Internet because of his persistence.
“I have hope,” Engnath says. “I think that sometime this year we’ll have major advances.”
HTLV National Registry 199 Wall Street, Kingston, NY 12401; (845) 339-2192
This article appears in May 2010.
