Transverse Myelitis: A Unique Journey

One of the first things you discover upon trying to learn about transverse myelitis is how little is currently known about it.

Go searching for transverse myelitis information online, and you’ll find a few facts and observations regularly repeated:

• Transverse myelitis (TM) is inflammation of the spinal cord, often resulting in damage to myelin, the protective insulation around nerve cell fibers.
• Damage to the spinal cord can result in symptoms such as paralysis, pain, loss of bowel and bladder control, sensory changes such as numbness or tingling in the limbs, and muscle weakness.
• It can affect people of any age, but there is a statistical spike in TM onset for two age groups: children aged 10 to 19, and adults aged 30-39.
• Recovery can vary, with some patients regaining significant function and others remaining seriously and permanently affected.

TM patients who remain paralyzed or continue to experience significant muscle weakness may require seating & wheeled mobility equipment to live as independently as possible. That means they could be coming to you for evaluation, support and assistive technology.

A Personal Journey

Sanford J. Siegel is president of the Transverse Myelitis Association (myelitis.org), which also advocates for consumers with acute disseminated encephalomyelitis (inflammation of the brain and spinal cord) and neuromyelitis optica (inflammation of the optic nerve and spinal cord).

In speaking with Mobility Management, Siegel notes that he has “no medical training at all, but I’ve been totally immersed in this for over 20 years.” That’s when his wife, Pauline, a kindergarten teacher, was diagnosed with TM. Following that, the Siegels worked with a family in Tacoma, Wash., whose young daughter had been diagnosed with TM, to create an organization to support and educate affected individuals and families.

That was in 1994, and at that time, Siegel says there were no medical centers of excellence for TM, and no physicians specializing in it. Today, Siegel says, “There is a transverse myelitis center at Johns Hopkins — Dr. Carlos Pardo-Villamizar is the director of that center. There is a transverse myelitis center at University of Texas Southwestern in Dallas; the head of that center is Dr. Benjamin Greenberg. There is a specialist at University of Cincinnati’s medical center, Dr. Allen DeSena.”

The establishment of TM centers, Siegel explains, is important not just so newly diagnosed patients can get the best care and rehabilitation, but also so doctors can systematically collect information on those patients. “They understand that that’s how we’re going to learn about the disorder,” Siegel says. “And because they have established these centers, they attract more patients than anywhere else because they hung the shingle up.”

While that progress is laudable, so much about TM, from cause to prognosis, remains unknown.

How Does Transverse Myelitis Present?

What the medical community does know about TM includes the fact that, despite having some symptoms in common with spinal cord injury, TM is an autoimmune disorder rather than a neurological one.

As Siegel says, “The spinal cord was totally fine until the immune system went at it. So a spinal cord that’s damaged from trauma — the analog is that the immune system is doing damage to the spinal cord in the same way that trauma is doing damage to the spinal cord.”

Siegel says physicians don’t entirely know what the immune system is doing during the onset of TM. But they do know that inflammation is the result. “A lot of the damage that is caused in a trauma to the spinal cord is caused by disruption of blood supply to the cord,” he says. “Same thing happens in an inflammatory attack. Because it is such a small chamber that the spinal cord is housed in, the inflammation is also going to constrict blood supply. So it’s not just the inflammation, and it’s not just the immune system that is going to cause damage. It’s also the same sort of issue as [with] a trauma: the constriction of blood supply to the nerves.”

Pain, muscle weakness and unusual sensations, such as numbness or tingling, are reported by many TM patients. Siegel’s wife, Pauline, had noticed lower-back pain for days prior to the acute onset of TM.

“She thought she pulled a muscle in her back,” Siegel recalls. “She didn’t feel right for about a week; she was complaining about this lowerback pain. And then on a Sunday night, she was taking a shower, got out of the shower, had this horrible stabbing pain in her lower back and went over on the floor.”

Pauline was instantly paralyzed.

“It happened immediately to her, but the fact of the matter is that it was probably over a period of a week that this inflammation had started up in her spinal cord,” Siegel now says. “Whatever happened on that Sunday night, when what was a minor thing became a major catastrophe, I can’t tell you. And I’m not sure that modern medicine can describe it, either, because I don’t think they really understand what happens at that critical mass.”

Loss of bowel and bladder control — e.g., bowel incontinence, loss of the ability to urinate — is also common. “I’m thinking that at acute onset, around 80 percent of people who have TM have bowel and bladder problems, and that many of them are not urinating,” Siegel says. “They’re losing the ability to urinate at acute onset.”

Siegel listed the major symptoms of TM:

• Paralysis or motor weakness. TM can strike anywhere along the spinal cord, so some TM patients require ventilators to breathe.
• Spasticity.
• Paresthesias (i.e., abnormal sensations such as “pins and needles” prickling and numbness) and nerve pain.
• Bowel and bladder dysfunction.
• Sexual dysfunction.
• Fatigue.
• Depression.

“And then there are all these secondary problems, which you find in traumatic SCI, like autonomic dysreflexia, pressure wounds and similar sorts of problems,” he adds.

What Medicine Does & Doesn’t Know

While the cause of traumatic spinal cord injury is obvious, much less is known about what causes TM. In fact, the medical community is still seeking answers to some very basic questions about TM.

Starting with how common it is.

“We do not have great data,” Siegel acknowledges. He says TM centers probably have the best collections of data, but that’s basically confined to what they gather from their own patients. The medical community does know that transverse myelitis is rare — “I believe the published incidence is 1 to 5 in a million, and that incidence study was actually done in Israel in 1980,” Siegel says. “It was probably a pretty good study, because they have a centralized medical system so they were able to go to their hospital systems to count up numbers, which we do not have here in the United States.”

The industry’s best estimate is between 30,000 and 35,000 people in the United States are currently living with TM. While there are those statistical spikes for people aged 10-19 and 30-39, people of any age can experience a TM event. Siegel says he’s aware of seniors in their 70s or 80s having TM, and he has heard too many stories of parents putting a seemingly healthy baby to bed at night only to find the baby paralyzed in the morning.

Accurately diagnosing TM is also still evolving.

“Formal diagnostic criteria for transverse myelitis were not done until 2002, and it was done by Dr. Douglas A. Kerr, who was the first transverse myelitis specialist in the world,” Siegel says. “He started the TM center at [Johns] Hopkins.”

Siegel says the medical community has been aware of TM for a century, but he adds, “There’s really not very much understood in the grand scheme of things about all autoimmune disorders. We really don’t know what causes them. So TM is inflammatory, and it is believed to be autoimmune. And I say believed to be autoimmune because there’s no biomarker, and they’ve not found an auto-antibody.

“One characterization of TM is post infectious. About a third of cases are associated with some kind of viral, bacterial or fungal infection. What seems to be happening is a person is coming out of that infection they might have had. My wife had the flu, and she was coming out of it. Her symptoms were diminishing, she was feeling better. And she started noticing lower-back pain.”

Siegel lists what else the healthcare industry knows about TM: “It is for most people mono-phasic,” i.e., most patients experience a single attack rather than multiple ones. “Essentially the way TM gets diagnosed is [physicians] identify inflammation in the spinal cord through MRI and a spinal tap. It can happen at any level in the spinal cord, and they don’t understand why at one level versus another level.”

The fact that magnetic resonance imaging (MRI) can diagnose TM shows how the medical community is still at the beginning of understanding the disease.

“MRIs were not discovered until the 1980s,” Siegel says. “They were discovered and they were put into sort of general population use in the 1990s. I just told you the diagnosis is done with MRI and spinal tap. So people have been diagnosed with TM over a long period of time, and it’s a diagnosis of exclusion.

“It is not a new diagnosis. However, we are in a totally different landscape today than we were I would say 10 or 15 years ago, from the time that Dr. Kerr started this specialization. We understand so much more about this disorder than we did previous to his specialization. Having said that, we don’t know the cause. There is no biomarker, and it remains a diagnosis of exclusion. And it is likely that what doctors call TM is more than one thing. So we are at the baby steps of understanding what this is, there’s no doubt about it.”

Difficulty in Diagnosing & Responding

One of the reasons TM is so difficult to diagnose, Siegel says, is its symptoms can be seen in a range of other conditions as well.

“I’ll sometimes get phone calls from these people [who believe they might have TM], and what I have to say to them is there is absolutely no way you can diagnose TM from symptoms because there are a large number of things that can happen to the human body that can cause exactly those same symptoms. People can get neuropathies from diabetes. People can get all of these paresthesias or bowel and bladder issues or mobility motor weakness from a disc problem, from stenosis. There are vascular problems that can cause these symptoms. Lupus and Sjogren’s and other rheumatic disorders can cause similar types of symptoms.”

Knowing the best way to respond to acute TM — i.e., while the inflammation is occurring — is another critical piece of information that healthcare professionals don’t yet have.

“To me the most significant unknown is we don’t know what it is,” Siegel says. “That’s number one. Number two is there’s a window to treat this disorder, and that is while the inflammatory attack is going on. Once the inflammatory attack has resolved — I don’t want to diminish the significance of it by any means, but the only thing we can do for a patient is aggressive rehabilitation therapy. The only way to spare the spinal cord is to knock down the inflammatory attack as quickly and as effectively as possible, because the inflammation is damaging the cord.”

But Siegel points out that to date, “There has not been a single scientific study, not a single clinical trial, on any acute therapies for transverse myelitis.”

Instead, he notes, “All of the decisions being made about how we treat a person acutely is based on expert judgment. So we are at the very beginnings of understanding this disorder, but it is a significant group of people in the U.S.”

Siegel surveyed Transverse Myelitis Association members from 1997 to 2004 in a study that involved more than 500 respondents. “We looked at whether or not the distribution of our cases could be explained in any other way besides population size of the state,” he says. “It was just a very, very simple analysis. And it has its flaws because we didn’t ask people how long [they] lived in that state. We took their current address and we looked at the distribution, and we asked the question, ‘Can you explain the distribution of TM from these 500-some patients based on anything beyond population size for that particular state?’

“And the answer to that question was no. The largest numbers were in California and New York, and the smallest numbers were in Utah and Rhode Island. And they sort of distributed by population size in between. So you could conclude that it sort of looks random in its distribution.”

Treating inflammation during the acute event of TM is critical, but the apparent randomness of TM incidents means patients experiencing a TM event don’t go to one of the few TM specialty centers, but rather “They go to their community hospitals down the street,” Siegel says. “And because it looks random in its distribution, that community hospital down the street probably hasn’t seen a whole lot of it.”

That can lead to difficulties in getting the right treatment during a critical window of time.

“I listen to nightmare experiences from patients in emergency rooms all the time,” Siegel says. “Because if a person goes into an emergency room with the symptoms of traumatic spinal cord injury, and they have no history of traumatic spinal cord injury — no car accident, no diving board accident, no fall — and [physicians] do all of the imaging studies and they see no compression of the cord, and they see no evidence of any structural explanation that could explain what they’re thinking looks like traumatic spinal cord injury, it’s an unpleasant experience for the patient.”

When more neurologists learn about TM, often from the few specialists in the country, the ripple effect can be encouraging.

“Dr. Kerr, Dr. Greenberg, Dr. Pardo and the other doctors who are developing expertise in these rare disorders have done a tremendous job of educating neurologists,” Siegel notes. “I am going to say that over my 20 years of doing this work, I have noticed a very significant improvement in better diagnosis, more rapid diagnosis and more rapid treatment, which makes a difference. Getting a diagnosis and getting people on aggressive acute treatment as rapidly as possible makes a difference. It sure seems like it does. So it matters, and these doctors are passionate about getting the education out there.”

Siegel then mentions something that probably sounds familiar to ATPs and clinicians, who know that seating & wheeled mobility topics aren’t discussed much in university lecture halls.

“The doctors that specialize in this are a group of physicians who are a sub-group of neurology,” he explains. “It looks to me like you could go through all of medical school, and if you get a slide on TM as part of your medical training, that’s probably about as much as you’re going to get. And how much you’re going to be exposed to TM even as a neurology resident is probably going to depend on where you do your residency. Doing a neurology residency doesn’t mean you’re going to learn very much about TM. If you’re at Hopkins, you’re probably going to get some exposure. If you’re at UT Southwestern, you’re going to get some exposure.”

But if you’re a physician doing your residency outside the few TM specialty centers, “how much exposure you’re going to get, it’s not going to be a lot.”

What ATPs & Seating & Mobility Clinicians Should Expect

Patients with TM may largely experience treatment during the acute event and during subsequent rehabilitation that’s similar to what traumatic SCI patients experience.

“If a person presents with paralysis — and I believe many become paralyzed or at least develop significant enough motor deficits that they’re going to end up in a rehab hospital — they’re going to show up as a TM patient in a rehab hospital that treats people with traumatic SCI and stroke,” Siegel says. “And they’re going to be treated in exactly the same ways as a person with a traumatic SCI. [Healthcare professionals] have no reason to do anything else with them. There isn’t a therapist in those centers that’s going to say, ‘This person has TM, we need to do these things as opposed to what we do for a person who has a traumatic SCI.’ They’re treated exactly the same way. Is that good? I don’t know. But I know that we probably don’t know to do anything differently for those [with TM].”

Siegel says aggressive rehab seems to bring good results for TM patients, but he adds, “We have lots of mobility issues. We are supporting the orthotics industry. We have lots of people in chairs, including my wife. There are so many complicating issues in TM because of problems like spasticity and contractures.”

How different life will be after TM depends on what part of the spinal cord was impacted, and how much recovery occurs. Recovery may be one of the ways that TM can significantly differ from traumatic spinal cord injury.

“It depends on where on the cord they’re impacted,” Siegel says. “It depends on the severity of the attack. And it depends on their recovery. My wife got back enough function that she doesn’t have to cath; she’s able to urinate on her own. Bowel [control] didn’t come back as well, but it’s a very manageable symptom for her. She doesn’t have as much spasticity as most people have, so she was very fortunate in that regard. Wasn’t fortunate on the nerve pain at all: She’s got horrible nerve pain. But she went from being totally paralyzed to having I would say decent mobility. She’s not going to walk a city block, but she can get up and transfer, and she can use a walker to go across the room. That’s a huge thing.”

At least partial, but significant, functional recovery from TM can be possible for years after the initial onset of symptoms, Siegel says.

“This is purely anecdotal,” he notes. “But I’m going to say that from my observation, I definitely see changes in people over time without any limit on that time. Definitely in the way of sensory. People change dramatically, where somebody had no temperature sensation, and then they get temperature sensation back. Or they had no sensation, and some kind of sensation returns. It may not be normal sensation, but if you felt nothing and now you’ve got something, that’s change. I definitely see that.

“And people get back motor strength. In my experience, I’ve never seen somebody who was totally paralyzed be able to gain back enough motor function that they were walking on their own. I’ve not seen that. But I’m going to tell you that I think the doctors in our community think that really aggressive physical therapy could get a person out of a chair.”

So what are best practices for working with patients with TM?

“At the end of the day, when you’re getting a person fitted for some kind of assistive device, whether they need a walker or a scooter or a chair, you’re going to look at the same things that everybody else is looking at,” Siegel says. “Are they having problems with pressure wounds? Do they need to be doing pressure releases? Do they have enough upper-body strength to wheel a wheelchair? The same issues.”

And while ATPs and clinicians always need to consider how their seating & wheeled mobility clients’ needs change over time, it may be especially important with this population, given how patients with TM can regain function years after their initial event.

“Patients need to be evaluated over time because things change,” Siegel says. “What worked for a person last year might be different than what would work best for them this year. Symptom changes can be fairly dramatic for a person with TM over time, and that [should be kept] in mind while people are being evaluated for the sorts of adaptive equipment they’re using for ambulation.”

It’s clear that consumers with TM can experience wide ranges of symptoms for reasons not yet well understood by healthcare professionals. And that their prognoses can vary more than those of consumers with more typical spinal cord injuries. But Siegel points out that this truth hardly makes TM more idiosyncratic than other mobility-related conditions.

“Everyone’s on a unique journey with TM because there are so many variables,” he acknowledges. “Where on the cord? How severe the damage? How good the recovery? How complicated the symptoms?

“But the MS population is also tremendously variable. And don’t you also have that with traumatic SCI?”