An Unsolved Medical Mystery

Last May, 64-year-old Army veteran Jeff Paulus received a dreaded diagnosis from his doctor: Lou Gehrig’s disease.

The rare neuro-muscular illness progressively paralyzes and usually kills those afflicted within five years. There is no known cure. Studies indicate veterans may be at greater risk of contracting it than the general population. Still in the early stages of the disease, Paulus is able to continue working, with help from a walking cane. He realizes he faces an increasingly challenging future.

Patients with amyotrophic lateral sclerosis (ALS) – known as Lou Gehrig’s disease because of the famous baseball player whose life it claimed – suffer from degeneration of nerve cells in the brain and spinal cord. The cells lose their ability to  control muscle movement when the degeneration prevents communication between the nervous system and voluntary muscles.

As the disease progresses, patients lose the ability to move their arms and legs, speak and swallow. Eventually, that loss of control can extend to the muscles responsible for breathing. Patients must get mechanical ventilation to continue breathing, or else die from respiratory failure. About 25,000 patients in the United States have ALS, and about 60 percent of them are men.

Faced with his diagnosis and planning how to live with the disease, Paulus explored what military benefits might be available. But at that time, only veterans who had served in the Southwest Asia theater of operations between Aug. 2, 1990 and July 31, 1991, or veterans who developed the disease while on active duty or within a year of leaving the service, were eligible for disability benefits – without proving that something related to military service had caused their ALS. That was a difficult burden of proof, considering that researchers exploring the question have been unable to determine the actual cause of the disease. Fortunately for Paulus and other veterans diagnosed with ALS, VA guidelines recently changed. The disease is now presumed to be service-connected for U.S. veterans from all eras, as long as they have had at least 90 days of continuous active service.

VA’s decision to change eligibility requirements relied heavily upon a 2004 study of men in the service; it found that, from 1910 to 1982, servicemembers had a nearly 60 percent greater risk of the disease than men who had never served. The increase “appeared to be largely independent of the branch of the service and the time period served,” Harvard University researchers concluded. Earlier, a couple of Gulf War veteran studies found that they were also at increased risk.

Despite such studies, VA initially opposed making the change in guidelines. Jeff Faull, a veteran with ALS who is vice chairman of the ALS Association’s Veterans Affairs Issue Team, credits The American Legion with helping to overcome the resistance. “There’s strength in numbers, so, thankfully, the Legion lent us its numbers,” he says.

Toxicologist Mark Brown, director of VA’s Environmental Agents Service, says evidence of the link was thin, causing VA’s hesitation. A review committee of the Institute of Medicine, which VA had asked to evaluate the research studies, found in 2006 that there was “limited and suggestive evidence of an association between military service and later development of ALS,” and they questioned whether it was fair to ask sick veterans to wait for more evidence as their lives slowly ended. The small number of ALS cases also makes it difficult to effectively conduct studies, Brown says. Only about 2,000 veterans were on the National Registry of Veterans with Amyotrophic Lateral Sclerosis, maintained by VA from 2003 to 2007.

“It’s an awful disease, but, fortunately, it’s a rare disease,” he says. VA’s new rule establishing ALS as a service-connected disease became effective last Sept. 23. That recognition provides a minimum 30-percent disability rating for veterans with ALS, which can be raised as the disease progresses. Those who were denied disability assistance before the rule change can now re-apply to get benefits. No retroactive payments are available.

Disability benefits may include housing and vehicle modification, as well as monthly disability compensation; medical benefits may provide equipment such as power wheelchairs. Many factors determine the benefits to which each applicant is entitled, and veterans are encouraged to get help from American Legion service officers  when filing their applications.

American Legion Deputy Director for Veterans Affairs & Rehabilitation Steve Smithson, a Gulf War veteran who has fought to provide VA benefits for ALS sufferers, is pleased by the decision that reduces veterans’ burden of proof. “They’re not going to have to worry about jumping through hoops to establish service connection,” he says. “Before this new presumption regulation, in most cases they were denied and had to appeal.” Getting service-connected benefits was extremely difficult unless veterans were diagnosed or developed symptoms while still on active duty or within a year after service, he says.

The lead researcher of the 2004 Harvard study, epidemiologist Marc Weisskopf, also applauds VA’s decision. “I thought it was a great move,” he says. “There are many scientists out there who would want to see more data.” However, he adds, “the evidence is pretty strong.” The Institute of Medicine described the study as “high-quality.” Paulus, who was in the Army from 1967 until 1993 – including a year in Vietnam – promptly sought to take advantage of the rule change, with assistance from The American Legion. He hopes the benefits will help him purchase a modified van and create easier access to his second-floor bedroom. Problems that started with his right leg later spread to his left leg, making stair climbing difficult. He also experiences twitching throughout his body.

No one knows what caused his ALS, but Paulus is aware that the toxic defoliant Agent Orange was used extensively in the region of Vietnam where he served.

Vietnam War-era veteran Jim Flint, 65, who was also assisted by The American Legion, wonders if his ALS might be connected to his three-day exposure to an Agent Orange-treated area.

For Flint, a Purple Heart recipient, the battle with ALS began about nine years ago. He first noticed a problem when he couldn’t use his wrist effectively to open a car door. “For a long time, that was the only symptom I had,” he says. He went to a doctor, who pointed out some atrophy in his hand and sent him to a neurologist. “That started me off on a year-long series of tests before they came to a diagnosis.”

Because he was diagnosed in 2003, long before the recent change, Flint faced the daunting task of proving the disease was caused by something in his service experience.

“In 2005, my condition worsened and I ended up on a ventilator,” he says. “That caused some apprehension for me, because I had no disability rating at that time.” Hearing that the cost of 24-hour medical care can be $200,000 to $300,000 per year, Flint, who is bedridden and can’t use his arms and legs, says he would have been bankrupt.

Fortunately, his post-military experience as a lawyer came in handy. He spent months searching for all recent disability decisions involving ALS, and finally came across a Florida case that gave him the ammunition he needed. It indicated that applicants only had to provide physicians’ statements suggesting the disease was war-related. Since medical experts haven’t been able to determine the cause of ALS, and exposure to environmental hazards – including Agent Orange – are among possible causes being explored, neurologists were able to support Flint’s application for benefits.

He now lives at home and uses a tele-medicine system to take his vital signs daily and send the results to a VA hospital where they are monitored. A computer allows him – through his eye movements – to perform tasks like changing TV channels and turning on lights.

The cause of ALS, despite numerous studies, still remains a mystery. The diagnosis among younger veterans may offer a possible clue; ALS is most commonly diagnosed in the early 60s. Faull, a Navy veteran who served two tours in the Persian Gulf as a nuclear electronics technician, was diagnosed with the disease in 2007 at age 38.

“We are seeing ALS in veterans at an age when we generally do not see the disease,” Faull said during a congressional hearing. “What will we see 10, 15, 20 years in the future, as the men and women serving today leave the military?”

About 10 percent of ALS illnesses are inherited. Among wide-ranging suggestions of causes for the other 90 percent are vaccinations, pesticides, radiation, formaldehyde, lead, physical stress, tobacco, recreational or medicinal drugs, and head injury. “Nothing really is ruled out,” says Brown, the VA toxicologist.

The search continues.

And while the  veterans’ ALS registry has been discontinued, Faull urges those who’ve served and now suffer from the disease to sign up with the Roll Call of Veterans maintained by the ALS Association. This year, a national registry for all U.S. patients – administered by the Centers for Disease Control and Prevention – was also created. “The disease is 140 years old and there’s still no known treatment or cause,” Faull says. But, he adds, “More has happened on ALS research in the past decade than in the past century.”

Margaret Davidson is a writer who specializes in the coverage of medical issues.