Let’s Talk About Neuromyelitis Optica Spectrum Disorder (NMOSD)
Occasionally mistaken for multiple sclerosis, NMOSD can cause serious vision and mobility problems. Help is here.
Maybe you woke up one morning with pain in both eyes or numbness in your leg and had no idea what was happening to you. Or, perhaps your GP referred you to a neurologist after suspecting NMOSD. Whatever the scenario, you’re probably nervous, confused, and maybe even a little scared. That’s normal! Here, we share the realities and challenges of this rare condition, but also the best treatments, helpful lifestyle changes, where to find your NMOSD community, and all the crucial information you need right this minute. We’ve got you, so keep reading.
Our Pro Panel
We went to some of the nation’s top experts in NMOSD to bring you the most up-to-date information possible.
Benjamin M. Greenberg, M.D.
Director and Professor of Neurology
Transverse Myelitis and Neuromyelitis Optica Program; University of Texas Southwestern Medical Center
Michael Levy, M.D.
Director and Associate Professor of Neurology
NMO Clinic and Research Laboratory and Massachusetts General Hospital
Maureen Mealy, Ph.D., R.N.
Adjunct Associate Professor of Neurology
Johns Hopkins University School of Medicine
It can be, because the symptoms—blurry vision, impaired mobility—can be similar and even MRI scans can be misleading. Also, the two conditions affect mostly women. But there are differences. NMOSD attacks take place during a shorter period of time, are worse, and leave people more disabled. People tend to be older—on average, in their late 30s and 40s, instead of in their 20s. And 80% of people with NMOSD have specific antibodies not found in MS.
It’s another inflammatory attack of the central nervous system (CNS) that can leave you more disabled, especially if it occurs in the same place (like your spinal cord). About half of all NMOSD patients have relapses in the first year, so preventing these attacks is key. That’s why all NMOSD patients are put on immunosuppressant medications.
The signs of another attack aren’t subtle, and the symptoms usually get worse over the course of a few days. If your first attack left you with recurring blurry vision, the second one might make you go blind in one eye or it may affect another part of your nervous system, like your legs or arms. But these relapses vary from person to person, and there’s some data that suggests preventative meds are associated with milder relapses.
Primary care doctors can treat NMOSD, but it also helps to have a neurologist who specializes in neuroimmune disorders—like multiple sclerosis (MS) or transverse myelitis (TM)—on your team, too. Even if you only see the neurologist once or twice a year, this specialist can work with your primary care provider to deal with changes in your health and keep your doctor informed about the latest research in the disease.
What Is Neuromyelitis Optica Spectrum Disorder, Anyway?
You’d be forgiven for thinking that neuromyelitis optica spectrum disorder—NMOSD for short—was a neurological disorder. This group of inflammatory diseases is actually an autoimmune condition that affects your central nervous system (CNS), which is comprised of the spine, optic nerves, and brain.
NMOSD is like multiple sclerosis (MS) in that way. In fact, for many years, doctors thought NMOSD was a variation of MS—and some still misdiagnose people who have NMOSD with the better-known condition. But NMOSD (also sometimes called neuromyelitis optica, or just plain old NMO, its official name before 2007) is a different thing altogether.
Similar to MS, your immune system suddenly goes haywire and attacks healthy tissues in your body. But unlike that condition, NMOSD targets a specific water-channel protein known as aquaporin-4 (AQP4), which is responsible for transporting water in and out of the brain and spinal cord. Even though AQP4 is also in other parts of your body, including your lungs, the immune system focuses its attack on the protein in the CNS, carpet-bombing it with all its disease-fighting might as if it were a foreign pathogen, like invading bacteria or a virus.
There is a variation of NMOSD that involves a different antibody. In a small subset of people—just 10% to 11%—with this condition, the immune system goes rogue on an altogether different protein called the myelin oligodendrocyte glycoprotein, or MOG. MOG is found on the protective coating that surrounds nerve cells, called myelin. Those who have this form of the disease tend to have less severe attacks with better recoveries.
Regardless of which protein is the target, the attack produces swelling (a.k.a. inflammation), which, 90% of the time in NMOSD, damages tissue in one of two places:
The optic nerve: This is the nerve that connects the eye to the brain. Inflammation here can lead to blurry or obstructed vision, or even blindness—sometimes temporary, sometimes permanent.
The spinal cord: Inflammation here can lead to numbness and an inability to pee, with paralysis—again, sometimes temporary, sometimes permanent—a real possibility.
Damage to the brain, in particular the brainstem, occurs in just 10% of cases, and it may cause uncontrollable hiccupping and horrible nausea, which usually (and thankfully) subside after a few weeks.
Vision loss and/or major mobility issues are super scary symptoms, to be sure, and most of the time you have no idea what hit you. That’s understandable. NMOSD is rare—you’ve likely never heard of it before. Here are some more quick facts:
Roughly 15,000 people in the U.S. have NMOSD, with between one and four cases per 100,000 people being diagnosed every year.
Women are nine times more likely to have the most common form of NMOSD then men.
On average, people have their first attack at age 39.
Children as young as 3 can have the disease (though this is incredibly rare). Children account for about 4% of NMOSD cases.
Seniors can get it, too, with late onset occurring any time after 55.
Is the Damage From NMOSD Temporary or Permanent?
There’s no cure for NMOSD, but unlike the different types of MS, this disease doesn’t follow a progression path to more serious stages of disability.
Instead, the degree of your injuries from NMOSD depends on two things:
The damage that’s done after the initial attack
Whether or not you have additional attacks after the first one
With each relapse, typically more damage occurs—either to the same part of the CNS or to a different one. For instance, your first attack can leave you unable to see clearly out of one eye, or reliant on a walker to get around. But subsequent attacks can leave you mostly blind in that same eye, or forever confined to a wheelchair. Or, alternatively, a subsequent attack can target your spinal cord rather than your optic nerve (or vice versa).
That means reducing the likelihood of another attack is key, especially since you can usually recover much of your sight and mobility between relapses—if you get treatment in time. But recovery varies from person to person, ranging from none to full, and most likely depends on the severity of the attack, how quickly you received treatment, what type of treatment you got, and even your age. This is true of repeat attacks, too, though when they happen again in the same place (say, the spinal cord) your ability to bounce back is usually pretty limited.
Luckily, there are more treatments for NMOSD than there were even two short years ago—and doctors are finding out new things about this disease every day. While you may have to live with NMOSD from now on, we’ve got the answers you need to help you live your best life.
What Are the Types of NMOSD?
There are two forms of NMOSD, and the difference between them comes down to whether you suffer a single attack, or whether you go to have two or more. At the outset doctors cannot tell which kind you have (so treatment is essentially the same). They are:
This is the most common type, affecting 90% of patients, according to the Guthy-Jackson Foundation, a nonprofit in Beverly Hills, CA, dedicated to funding basic and clinical research on the disease.
If you're diagnosed with relapsing NMOSD, you have a roughly 60% chance of having another attack in the first year, and a 90% chance of having one within three years. The good news is that preventative treatments can cut your chances of having relapses, and probably lessen the severity of an attack if you do have one.
This is the least common form, affecting between 5% and 10% of patients, men and women equally. This is essentially a one-and-done form of the disease—you have one bad attack that progresses quickly (in hours or days), and then you do not typically have a relapse. But doctors won’t be able to tell if you’ve had a singular event until years have passed, so they treat you as if you have the relapsing kind.
This initial attack is usually more severe, leaving 20% of patients mostly blind in one or both eyes, and in 30% of cases, permanently paralyzed in one or both legs, too. That’s scary to read, we know. But it’s the rarer form of a very rare disease, and you’re much less likely to experience an attack again.
What Are the Risk Factors for NMOSD?
So what causes NMOSD, you ask? The short answer: It’s a mystery, as it often is for inflammatory autoimmune disorders. Scientists suspect people with NMOSD may have a genetic predisposition for the condition that gets triggered by something—a virus, say, that mimics the composition of the aquaporin-4 channel protein. After your immune system eradicates the original virus, it confuses AQP4 with the original virus and attacks the spinal cord or the optic nerve, thinking the virus is still lurking there.
That’s the theory, anyway. Having said that, there may be other risk factors that may trigger your immune system to go into overdrive and turn on AQP4 (or the alternative protein, MOG).
Such risk factors include:
NMOSD isn’t a genetic condition—so far, no one has isolated the genes that could predispose you to this, and it’s extremely rare to find multiple family members who have the same condition. But many NMOSD patients have a family history of autoimmune disorders such as lupus, psoriasis, and/or Sjogren’s Syndrome. In fact, there’s some confirmed overlap with lupus—you could have NMOSD and test positive for lupus, even though you have no signs of the latter condition.
If you’re African American, you have a greater risk of developing NMOSD. When researchers from Johns Hopkins looked at the demographics of 187 patients from three NMOSD clinics across the U.S., they found that while African Americans comprise just 13.4% of the U.S. population, they made up 37% of NMOSD cases, compared to patients who were white, who made up 48%. The remaining 15% of NMOSD cases were a combination of Latinos, Asian Americans, and Native Americans, more in keeping with general population trends, according to the U.S. Census Bureau.
As previously noted, relapsing NMOSD, which the vast majority of people with this condition get, is like many autoimmune disorders: It strikes women far more often than it does men—in this case, nine times more, according to some estimates.
You need something to trigger your immune system, and people with NMOSD seem to have more of the bacteria C. perfringens in their GI system than people normally do—which may mean that the immune system is constantly trying to eradicate it (and mistakenly targeting AQP4, too). The bacteria also spurs production of pro-inflammatory cells that are linked to other autoimmune disorders, as well.
It’s really tough to establish cause and effect with diet and exercise—people always overestimate the servings of fish they eat or underestimate their junk food consumption. Still, Iranian researchers looked at 100 NMOSD patients and discovered that they were more sedentary than average and consumed less fish and dairy, along with fewer fruits and vegetables. This is also true for many autoimmune disorders—eating high-fat, high-salt foods, coupled with less physical activity, can up your risk for developing such diseases.
Vitamin D Deficiency
There’s some evidence to show that NMOSD patients have low levels of vitamin D—in fact, even lower than those levels found in people with MS. The vitamin is known for regulating the immune system by keeping inflammation at bay, which is why some doctors recommend Vitamin D supplements for NMOSD patients.
How, Exactly, Does an NMOSD Attack Do Damage?
Most of the injury is done to the AQP4 water-channel protein and astrocytes—the special cells where most AQP4 is found. Astrocytes perform a variety of useful functions—they feed and water other brain cells and clean out brain debris (like excess proteins). So when the astrocytes are damaged, nearby neurons no longer can work properly.
A secondary casualty of the AQP4 attack is the loss of myelin. When this protective sheath gets damaged or destroyed—as it can when your body attacks MOG proteins—your nerves can’t function as well. If your optic nerve is attacked, for example, myelin damage can slow down or even block the brain from sending signals to your eyes. If your spinal cord is the target, signals to your limbs may be affected. Similar to MS, you can also develop scars, known as lesions, on your spinal cord, optic nerve, and brain where healthy tissue used to be, and such scarring is visible on a MRI scan—another reason NMOSD was so often confused for multiple sclerosis.
Often, the damage is so bad you usually develop symptoms in a matter of days, and sometimes even hours (which is faster than what can happen if you have MS, say). You can go to bed with a tingling in your leg and wake up the next morning to discover that it’s so numb you can’t even move it. Or you open your eyes to blurred vision. Or you’re horribly nauseous and can’t stop hiccupping, if the haywire immune response damaged to your brainstem.
What Are the Symptoms of NMOSD?
The signs of NMOSD aren’t at all subtle. They usually happen quickly—and there’s no soft-pedaling it, the symptoms can be alarming. Still, they range in degree of seriousness and are different for everyone. Milder symptoms tend to be the exception, but they also can occur.
Most of the time you absolutely know that something serious has happened. That’s because the optic nerve and spinal cord aren’t that thick, so any injury done to the tissue can wreak havoc on the pathways that communicate with the brain.
If your optic nerve was targeted...
The inflammation can happen anywhere along the optic nerve (which goes from the back of your eye through the skull to the brain), and you typically have the following symptoms in one eye (though sometimes both can be involved):
Your eye feels sore, as if someone has punched you, or it hurts when you move it up and down or side to side.
Your vision gets obscured—it could look like there's a giant gray spot in the middle of your field of vision, or like a black curtain has come down halfway.
Your vision is blurry or cloudy.
You lose your vision completely in one or both eyes or are legally blind (20/200, meaning you see at 20 feet what others can spot at 200 feet). Such vision loss may be temporary or permanent, depending on the severity of the attack and how soon you receive the right treatment.
Inflammation of the optic nerve (also known as optic neuritis, or ON) tends to be more common in people younger than 35, and when children and teens develop NMOSD, it usually involves the optic nerve.
If your spinal cord is targeted...
The inflammation can happen along any part of it, and the location influences which part of your body experiences symptoms. Symptoms are primarily motor (affecting your ability to move) or sensory (what you feel):
Numbness, or a lack of sensation, in one or both of your limbs
Weakness in your arms or legs, ranging from mild to an inability to move them
Tingling sensations in your arms or legs, or itchiness, or burning sensations
Inability to pee or move your bowels for hours, even when you have to go
Feelings of tightness, either around your stomach, legs, or arms, or around your neck and into your scalp
Pain in the back or neck
Paralysis in one or both limbs
Spinal cord attacks (also known as transverse myelitis, or TM) are more common among those over 35, although no one knows why.
If your brain is targeted...
An attack here is super rare, but when it does occur, it usually affects a small area called the postrema that controls vomiting. Those symptoms last two or three weeks and can be intense, but they go away (even on their own) and usually don’t come back. They include:
When Should I See a Doctor for NMOSD?
It won’t even be a question in your mind—if you cannot see out of one eye or are unable to pee for a day you’ll want to get yourself checked out STAT. If you have ON, an ophthalmologist will perform an exam called an ophthalmoscopy to check for swelling at the back of the eye and may give you steroids before referring you to a neurologist.
If you can’t go to the bathroom or are paralyzed and go to the ER, you may get an MRI that will show signs of inflammation in the spinal cord. Again, you’ll probably be referred to a neurologist for treatment and further testing (though you may be given a course of steroids at the hospital to get the inflammation down).
Some people don’t get lucky, though—NMOSD is a rare disease, many doctors are unaware of it, and some may not even think you need an MRI. That’s a problem, since the faster you get treatment, the greater your chances of recovering most of your mobility or vision and preventing another attack.
Your best bet is to find an NMOSD-savvy neurologist by going to the Guthy-Jackson Foundation’s Connect the Dots tool. After you’ve been diagnosed, that person can work with your primary provider, since NMOSD patients need a healthcare team to manage their disease.
How Do Doctors Diagnose NMOSD?
The most reliable test to diagnose NMOSD if you have the symptoms is a simple blood test. There are NMOSD-specific antibodies in nearly 90% of people with NMOSD, so that’s the easiest way to confirm a diagnosis, even though you have to wait a couple of weeks to get the official results.
In the meantime, your symptoms from an NMOSD attack will be treated aggressively, often with medications used to relieve MS symptoms, like steroids to bring down inflammation. (Remember, it’s preventative drugs for MS that don’t work well for NMOSD, but these two conditions do sometimes treat symptoms from a haywire immune response with the same approach.) If you don’t have antibodies, doctors look for other clues to make sure you don’t have MS or some other neurological disease.
The diagnostic tests for NMOSD include:
Your doctor will measure your vital signs—heart rate, body temperature, and blood pressure—and check on how well the major systems of your body are functioning. You’ll also be asked questions about your medical history, as well as the health history of your family.
Your blood will be drawn to check for specific NMOSD-related antibodies—the test is called an NMO IgG antibody test. Around 75% will test positive for AQP4. About half who test negative for AQP4 go on to test positive for MOG, the protein on the myelin sheath. There’s ongoing debate whether having MOG antibodies means you have a different disease altogether (called MOG Antibody Disease). While you may have the same symptoms as NMOSD, if you have MOG antibodies you tend to heal better and make a fuller recovery. For now, treatment of MOG and AQP4 NMOSD is similar, and both disease courses are classified as variations of NMOSD. Then, there is a small group of patients that don’t test positive for either antibody, which then makes the condition tougher to diagnose.
Magnetic Resonance Imaging (MRI)
This is an imaging test that uses powerful magnets and computer waves to make a detailed picture of your brain, optic nerve, and spinal cord. The lesions of NMOSD tend to be much longer and more inflamed than those in MS (but not always). The lesions can be found on the optic nerve, the spinal cord, and the brain stem, though sometimes there are lesions in other parts of the brain.
This is performed to examine cerebrospinal fluid that runs through the spinal column. In NMOSD, this may reveal AQP4 antibodies or high levels of inflammatory proteins called cytokines. If you have high levels of a protein called immunoglobulin instead, you probably have MS.
Optical Coherence Tomography (OCT)
This is a photograph of the back of the eye, including the retina. In many NMOSD patients, the retinal nerve layer has thinned out because of optic nerve damage.
What Are the Best Treatments for NMOSD?
There are two stages of treatment. The first comes as soon as possible after an attack, and the goal is to put out the fire—to tame inflammation and stop further damage from occurring. Then, you’re put on a regimen of medications designed to prevent (or at least cut down) the number of relapses. Every person with NMOSD—monophasic and MOG-antibodies included—gets preventative meds, which you’ll take for the rest of your life.
There are also treatments for specific symptoms. While you can go into remission, there is always some residual damage that can cause old symptoms to flare up. The triggers can be anything from an infection, like a urinary tract infection, or hot, humid weather, or stress. And for nearly every NMOSD patients, there’s nearly constant pain from nerves misfiringe—though there are ways to lessen that pain, so keep reading.
While you’re waiting for your antibody testing to come back, you’ll receive acute treatment—meaning urgent interventions—to relieve your symptoms. These include:
Corticosteroids: A high dose of these anti-inflammatory medications, via an I.V., will suppress your immune system so it’ll stop attacking itself. If your attack was relatively mild, then this will probably be the only treatment you need. What happens next depends on the response: If your symptoms don’t get better, or an MRI still shows inflammation, then doctors will move onto the next method.
Plasmapheresis or plasma exchange: Think of this as a type of dialysis for your blood. Your blood goes through an I.V. and into a machine, which is designed to take out the proteins causing all that damage. Those include:
the complement proteins, which normally clear up cell damage but attack healthy cells during inflammation
the cytokines, those inflammatory proteins
This process takes two weeks and is usually done for two hours every other day (sometimes all in the hospital, sometimes on an outpatient basis). Not everyone gets this treatment, but if you’ve had a bad attack with serious symptoms—you’ve lost most of your vision in one eye or can’t move your leg—doctors will do both treatments without waiting for results from the steroids.
Preventative, NMOSD-Specific Drugs
Before 2019, there were no NMOSD-specific drugs. Instead, doctors treated the condition with off-label drugs that had been approved for other autoimmune disorders like lupus. But in 2019, the results of three NMOSD-specific drugs in clinical trials were published. Those studied included:
All three drugs are known as immunosuppressants—they suppress your immune system so you’re less likely to get another attack. And they all have the same basic side effect: Because your immune system is tapped down, you are more vulnerable to infection, which can be frightening during, say, a novel coronavirus pandemic. Some keep your immune system suppressed longer than others, so you may want to take that into consideration when discussing your options with a doctor.
In addition, the cost of these drugs can be sky-high (think in the hundreds of thousands of dollars per year, though insurance may cover most of these costs). Convenience, too, is a deciding factor, as Soliris is a bimonthly infusion, so you may prefer another approach, like taking an oral pill daily at home.
Now, onto the specifics:
Soliris (eculizumab): This is a 35-minute infusion that you get every two weeks via an I.V. at the doctor’s office. It’s specifically designed for those patients who have AQP4 antibodies. It works by blocking two specific complement proteins, but it can also up your risk for meningitis, so you need to get a meningococcal vaccine before you start treatment. Still, Soliris has shown efficacy in preventing relapses. In clinical trials of 143 patients, it reduced the number of relapses by 94% over the 48-week trial. The negatives include some side effects, including respiratory infections, diarrhea, and headaches.
Uplizna (inebilizumab): This is a 90-minute I.V. infusion that you get every six months (after getting the first two infusions over two weeks) at your doctor’s office. Like Solaris, it’s also designed for patients with AQP4 antibodies and in trials, lowered the risk of another relapse by 77%. Side effects include UTIs and hepatitis B, so you must be screened for hepatitis B before you start the drug.
Enspryng (satralizumab): Like the other two drugs, this medication is only for people who have AQP4 antibodies. But unlike the other two, Enspryng is not an I.V. infusion. Instead, it’s an injection you can give yourself every four weeks— your doctor will show you how—after the initial dose, which is three shots in the first month. In one of the clinical trials, about two-thirds of the 63 people taking just Enspryng were relapse-free after two years. In another, 41 people took Enspryng along with another treatment (like Immuran or CellCept). Of these folks, only 11% had relapses. Side effects include a sore throat, runny nose, fatigue, and joint pain, but affected roughly 15% of those in the trial. You must be screened for both hepatitis B and tuberculosis before you start the drug.
If you do have a relapse, you’ll be given the steroids and the plasma exchange again, and your doctor may discuss switching you to another preventative medication.
There are three immunosuppressant medications that have had the best results for NMOSD, even though the evidence for them is based on studies that look at small number of patients. Please note that none of these drugs is 100% effective—at best, each lowers the odds you’ll have another attack.
Rituxan (rituximab): This, too, is an I.V. infusion. You get two courses in the first two weeks, and then one every six months. In one study, researchers took a 10-year retrospective look at 90 NMOSD patients and found that those taking rituximab cut their relapse rate by 88%, with two out every three patients achieving full remission.
CellCept (mycophenolate) and Immuran (azathioprine): These are tablets you take every day. Some doctors will also suggest you take the steroid prednisone for some time along with these meds, although this powerful steroid can cause serious side effects, so it’s not a long-term solution. Of the two, CellCept seems to cut the relapse rate more effectively than Immuran, according to one retrospective study.
What Happens During NMOSD Remission?
After an attack—and once treatment has brought down inflammation—your body begins to repair the damaged tissue. Some patients heal better than others, and make full recoveries, and others don’t (though doctors think having the MOG antibodies is a sign that you may do better long-term), and they don’t exactly know how this process works.
What they do know is that the healing process often feels worse than the attack itself, because neurons, once blocked by damage, start communicating with the brain again. So if you first felt numbness in your leg, as nerves heal you might feel tingling all the time, or have nearly constant (and painful) muscle spasms.
Or, if you couldn’t empty your bladder at all, you now have a hyperactive one that forces you to up two or three times a night to go to the bathroom. Believe it or not, these are good signs—the reason why they’re called positive symptoms.
Healing usually takes six months or more, and it’s never linear—you’ll have good days and bad ones. Something as benign as a bitter cold or hot, humid day can trigger your old symptoms, and you might have blurrier vision for a short period of time or need a walker to get around for a few hours or days.
Physical therapy definitely helps you regain your mobility, though it may never go back to what it once was. Depending on the severity of your first attack, your vision problems from optic neuritis may improve, but you may have long-term effects like halos, trouble seeing in the dark, or even some permanent vision loss.
Chances are, you’ll slowly get better—you may start out in a wheelchair but after a few months just need a cane for longer walks. But if you wake up not being able to see out of one eye again or find you cannot move a leg (if your previous attack only involved your eyes), then call your doctor immediately. Your physician will determine if these are signs of a new attack.
Because NMOSD is a lifelong condition that needs to be managed well, you’ll probably have a treatment team that includes:
An NMOSD specialist, usually a neurologist that has experience treating related diseases (like MS or TM), who you may see a couple of times a year and who can keep your primary care provider in the loop about new treatments. This may be someone at an MS center or hospital affiliated with a medical school or someone from one of the two NMO centers (one at Johns Hopkins and one at the University of Texas) who will collaborate with your primary care doctor or neurologist.
A primary care doctor or local neurologist, who can manage symptoms and medications
A physical therapist, who will work with you to recover your strength
An occupational therapist, to help with daily living
A social worker or therapist, who can give you coping strategies to help with mood
Does NMOSD Have Complications?
Most of the time, yes—including emotional ones. Even if you make a nearly full recovery, you don’t really know when you might have another attack. And that uncertainty can take its toll. Many NMOSD patients report one or more of the following complications:
At least two-thirds of NMOSD patients—and possibly as many as 91% of them—have constant, often debilitating pain, making it the number-one complaint. This can include:
Constant, disruptive tingling
Feeling like you’re on fire when something touches you
Again, it seems all wrong but such symptoms are a good sign—it means the nerves in your CNS are communicating again, even if they have an increased response, perhaps to make up for the nerve cells that were destroyed.
Doctors can treat this pain with meds, including anti-epileptics like gabapentin, antidepressants, and opioids. But because the NMOSD lesions in the spinal cord are broad and long, they disrupt more connections between the pain pathways going to and from the brain, leading to more severe pain. One result: Medications don’t work as well for people with NMOSD as well as they do for, say, MS patients. Plus, they can lead to medication overload, which can add other side effects.
Pain is complex, and involves the actual physical discomforts as well as the way it affects your thoughts and emotions. Reducing pain has to involve both medications and non-pharmaceutical treatments, including:
Mindfulness meditation: Focusing on your breath may not always lessen the intensity of the discomfort (studies on this for other chronic pain patients are mixed), but it can improve your quality of life by decreasing stress and boosting a sense of calm and acceptance.
Cognitive-behaviorial therapy (CBT): This short-term therapy aims to give you a way to reframe thoughts as well as strategies to help you cope with your pain. And while it may not lessen the severity (again, studies are mixed), like mindfulness, it can make pain more bearable.
Yoga and tai chi: These mind-body exercises can improve mindfulness and build up your muscles, preventing the wear and tear on your joints that can lead to more pain.
Electrical stimulation: This can include older devices like TENS, which uses electrodes placed on your skin to deliver low-voltage currents that block nerves from transmitting pain signals to the brain. Another device is the newer Scrambler therapy, a specially designed machine that reorganizes the inappropriate signaling of the nerves by directly stimulating sensory pain receptors called C delta fibers via pads put on the areas where you feel the most pain. Scrambler therapy has to be done by a specially trained therapist, but preliminary evidence for NMOSD looks promising—in one small study, patients were able to cut their pain in half after 10 days of therapy.
Muscle Spasms or Stiffness
Muscle spasms or tightening are another source of pain. This can range from feeling very stiff in the morning to really bad Charlie horses that happen 10 times an hour. Or, muscles that are so tight you can’t move your arm because it’s flexed or frozen in place. Physical therapy is key with these symptoms, as well as daily stretching to help you regain flexibility.
If your life is upended and you suddenly can’t walk or see well, then it makes sense that you feel down much of the time—and one study found that 40% of NMOSD patients were either moderately or severely depressed. But scientists also suspect the inflammation in your brain from NMOSD may also affect your mood (as it does in MS), though more studies need to be done on this aspect. What is true is that depression can make your pain and exhaustion worse, so it needs to be treated. Medications can help, but so can things like exercise (even chair yoga) and CBT.
Spinal cord lesions can affect your genitals too, and that can lead to problems that interfere with pleasure. So if you’re a guy, you may not feel any sensations in your penis, or you could have problems ejaculating, or even becoming erect. If you’re a woman, extra-sensitive nerve endings and/or a lack of lubrication can make sex painful—or, you may be too numb to feel any kind of stimulation (vaginal or clitoral).
Plus, other symptoms like pain or fatigue can put the kibosh on your libido, as can medications. This lack of intimacy is a huge issue—and it may be affecting nearly all NMO patients, though no one really knows—but you may be too embarrassed to bring it up with your doctor. Don’t be—speak up so you can get help. Vaginal lubricants or estrogen-based creams can help with dryness and pain, as can meds for erectile dysfunction.
Bladder and Bowel Issues
If NMOSD affected your ability to go to the bathroom during an initial attack, your bladder can come back during the healing process with a vengeance. That means more trips to the bathroom, and (if you’re a woman) more UTIs and trouble emptying out the bladder. Sometimes Botox shots can help with a hyperactive bladder, as can other medications. So this is another issue you must bring up with your provider, no matter how embarrassing.
It’s unclear whether exhaustion is a primary symptom of NMOSD or a secondary one, a result of pain, too many medications, and just the physical energy it takes to get around when you have to learn new ways to walk. Finding strategies to deal with pain can help you feel less tired, as can gentle forms of exercise, like yoga, tai chi, and stretches.
What Is Life Like for People With NMOSD?
When you’re left with residual weakness, fatigue, pain, sexual and bladder dysfunction, and problems seeing it can change the way you interact in the world—physically, emotionally, and socially. Going back to a job can be harder or nearly impossible. It can really become a vicious cycle—if you have to get up to go to the bathroom several times at night, then you’re not sleeping well. A lack of zzz’s can make your pain worse, which in turn, can affect your mobility and the desire to do any kind of physical activity. It soon turns into a cycle that can wreak havoc with your quality of life.
There are things that can stop the cycle, and the first step is to be proactive in your care. If you feel your doctors are throwing up their hands on your recovery, try to find new ones. There are also other things you can do to speed the healing process and set yourself up to thrive in your new life, no matter the challenges.
Physical and Occupational Therapy
Even if you must use a walker or cane, a physical therapist can show you how to align your body to avoid further wear and tear on your joints, which can cause more problems down the line. Or they can teach you exercises that help you regain your balance and strength, so you walk better and more safely. An occupational therapist can give you tips on how to rearrange your home to make it easier for you to get around if you can’t see as well, or if you need help navigating with a cane or other device.
Even if you only get one session ever with a physical therapist who will point you in the right direction, there’s much you can do on your own. What helps is to keep moving in some way, whether you swim, lift light weights, or stretch every morning. (Or all three on different days of the week!) Regular physical activity, especially the kind that gets your heart rate up a bit, can reduce chronic inflammation, help with depression, and even alleviate pain.
While there’s no concrete evidence that eating more fruits and vegetables can prevent relapses, there is anecdotal evidence that NMOSD patients feel much worse when they eat foods that promote inflammation, like red meat, sugary treats, and simple carbs. And vice versa—patients feel better when they eat fish high in omega-3s, more produce and legumes, and heart-healthy fats. So experiment—add meatless dishes to your rotation, add veggies and salads to every meal, and cook with olive oil. It may make a difference.
Most NMOSD practitioners will recommend you take a vitamin D supplement to help lower inflammation. If yours doesn’t, ask for a blood test to check your levels and then have your provider recommend one based on what the labs show.
Having a rare disease that no one knows much about can be really isolating. Besides friends and families, finding others who are living with this disease can be a godsend, whether they’re on Facebook or live nearby. Luckily, there are organizations that can offer information as well as connect you to other NMOSD patients as well as specialists.
Where Can I Find My NMOSD Community?
You can feel really cut off from your old self when you first learn you have NMOSD—you have to learn how adapt to your new normal and restructure your life while explaining it to the many folks who’ve never heard of the condition. So engaging with others who are going through what you are is critical—whether you’re getting help or lending support to someone who’s a few steps behind you on this journey. For inspiration, we’ve put together this list to help you feel less alone.
Top NMOSD Instagrammers:
Follow because: Before she was diagnosed in 2019, she was starting up her own business as a life coach. And while NMOSD has thrown a bit of a wrench in her plans, she didn’t let it stop her—she let it fuel her. Now, she uses her influence and knowledge as a certified life coach to bring you the Brain Diet Podcast, which in turn, helps her NMOSD.
Follow because: Her story doesn’t just include her being able to walk after NMOSD knocked her down, but it also follows her weight-loss journey—one she took so her body is better able to fight her autoimmune disease. Like so many, she was misdiagnosed with MS for years (a decade!), and now she’s working hard to find her new normal in NMOSD.
Follow because: While she doesn’t sugarcoat the hardships and WTF’s of NMOSD, she also has such an appreciation for the love and support around her that it helps you find the silver linings. More silver linings—or copper, really—is her foundation, Coach Pennies, that’s dedicated to helping those with rare diseases.
Follow because: Mother, wife, entrepreneur, and, oh yeah, mega-NMOSD advocate. She shares a constant behind-the-scenes look at life with NMOSD, including infusions and parenting with a chronic illness. And, she’s an open book with NMOSD Q&As. Bonus: She posts content in English and Spanish, and has tons of videos.
Top NMOSD Organizations:
Guthy-Jackson Charitable Foundation: The parents of a teenager started this foundation dedicated to researching the disease. You’ll not only get good information on the site via videos, but you can find a list of NMOSD specialists in every state and country with its Connect the Dots tool as well as support groups, both on Facebook and in various cities across the country.
Siegel Rare Neuroimmune Association (SRNA): This nonprofit is focused on a variety of rare neuroimmune disorders, including NMOSD, plus has information on children and teenagers, as well as adults. SRNA hosts a yearly summer camp for kids with NMOSD and other neuroimmune diseases. (In 2020, due to COVID-19 concerns, it’ll be held online.) You can also find information on clinical trials as well as support groups and a wealth of information, including a newsletter.
The Sumaira Foundation for NMO: Sumaira Ahmed, an actress and model, started this nonprofit after she was diagnosed with NMO. The organization has sponsored a “Demystifying NMO” podcast that delves into topics like sex and pregnancy when you have NMOSD, as well as a section highlighting patients’ stories. You can also become a TSF ambassador (or find one in your state or region).
NMOSD Statistics: UpToDate. (2019). “Neuromyelitis Optica Spectrum Disorders.” uptodate.com/contents/neuromyelitis-optica-spectrum-disorders
NMOSD and Antibodies: Journal of Neurology, Neurosurgery & Psychiatry. (2020). “Prevalence and incidence of neuromyelitis optica spectrum disorder, aquaporin-4 antibody-positive NMOSD and MOG antibody-positive disease in Oxfordshire, UK.” jnnp.bmj.com/content/early/2020/06/23/jnnp-2020-323158
Children and NMOSD: Children (Basel). (2019). “Differential Diagnosis of Pediatric Multiple Sclerosis.” ncbi.nlm.nih.gov/pmc/articles/PMC6617098/#B1-children-06-00075
Race and NMOSD: Archives of Neurology. (2012). “Epidemiology of Neuromyelitis Optica in the United States: A Multicenter Analysis.” pubmed.ncbi.nlm.nih.gov/22733096/
Relapse Rates: Guthy-Jackson Charitable Foundation (n.d.) “NMO: What You Need to Know.” guthyjacksonfoundation.org/tools-for-download/
Gut Bacteria and NMOSD: Annals of Neurology. (2016). “Gut Microbiome Analysis in Neuromyelitis Optica Reveals Overabundance of Clostridium Perfringens.” pubmed.ncbi.nlm.nih.gov/27398819/
Diet and Exercise in NMOSD: Acta Neurologica Belgica (2018). “Environmental Risk Factors in Neuromyelitis Optica: A Case-Control Study.” pubmed.ncbi.nlm.nih.gov/29500680/
Vitamin D and NMOSD: Autoimmune Diseases. (2020). “Vitamin D and Demyelinating Diseases: Neuromyelitis Optica (NMO) and Multiple Sclerosis (MS).” ncbi.nlm.nih.gov/pmc/articles/PMC7187724/
Rituximab and NMOSD: JAMA Neurology. (2014). “Comparison of Relapse and Treatment Failure Rates Among Patients With Neuromyelitis Optica: Multicenter Study of Treatment Efficacy.” pubmed.ncbi.nlm.nih.gov/24445513/
Eculizumab and NMOSD: U.S. National Library of Medicine. (2019). “A Randomized Controlled Trial of Eculizumab in AQP4 Antibody-positive Participants With NMO (PREVENT Study).” clinicaltrials.gov/ct2/show/study/NCT01892345
Uplizna and NMOSD: USFDA. (n.d.). “Drug Trials Snapshots: UPLIZNA.” fda.gov/drugs/drug-approvals-and-databases/drug-trials-snapshots-uplizna
Scrambler Therapy in NMOSD Patients: Neurology. (2020). “Scrambler Therapy Improves Pain in Neuromyelitis Optica: A Randomized Controlled Trial.” pubmed.ncbi.nlm.nih.gov/32269109/
Depression in NMOSD Patients: Neurology, Neuroimmunology and Neuroinflammation. (2016). “Insufficient treatment of severe depression in neuromyelitis optica spectrum disorder.” ncbi.nlm.nih.gov/pmc/articles/PMC5079380/
Enspryng and NMOSD Relapses (1.): The Lancet (Neurology). (2020.) “Safety and efficacy of satralizumab monotherapy in neuromyelitis optica spectrum disorder: a randomised, double-blind, multicentre, placebo.” pubmed.ncbi.nlm.nih.gov/32333898/
Enspryng and NMOSD Relapses (2.)The New England Journal of Medicine. (2019.) “Trial of Satralizumab in Neuromyelitis Optica Spectrum Disorder.” nejm.org/doi/full/10.1056/NEJMoa1901747