10 Steps Forward

Welcome.

You have come to the home for patients and families facing NMOSD or MOGAD. Hearing the diagnosis of a rare and serious condition such as NMOSD or MOGAD can be difficult. At first, such a surprising diagnosis can feel confusing, frightening, maddening and even isolating. No promises or platitudes can take away the real concerns of having such a diagnosis. Yet, four simple words and the power of rare they represent can offer strength and solace to help you find your way:

You Are Not Alone.

For patients, families and caregivers facing a new diagnosis of NMOSD or MOGAD, experience from those who have been down this road can provide hopeful and helpful information. Building upon experience and wisdom of amazing patients and caregivers, three immediate themes stand out above all:

✅ Gaining a Bigger Perspective

The area of neurology focusing on NMOSD & MOGAD has undergone a revolution in just the last decade. From zero approved therapies and little insight into the causes of these diseases, to three approved therapies, dramatic improvements in the speed and accuracy of diagnosis and essential new understanding of how these autoimmune diseases occur—it may be said that here has never been a better time to be diagnosed with NMOSD or MOGAD. There are incredible efforts in play to prevent relapses, stop them immediately if they do occur, better manage symptoms and cure these diseases once and for all. A bigger perspective focuses on the good to come.

✅ Stronger Through Expression

There can be a natural tendency to feel like such a diagnosis is somehow a judgement about you or your path in life. Being diagnosed may feel unfair, deepen worries of what tomorrow will bring or even give rise to feelings of guilt or reduced self-worth. We understand. But please know this: a diagnosis of NMOSD or MOGAD is not your fault—it does not make you any less meaningful or valuable or beautiful as a person—and it is not your identity. Voicing how you feel and wanting to find answers are not signs of weakness. Expressing yourself can make you stronger.

✅ Knowledge is a Superpower

A Journey Together.

Each individual who hears the diagnosis of NMOSD or MOGAD is a special person. While such a diagnosis can be difficult to accept and may lead to important life changes, it can also be the beginning of a journey to demonstrate the power of rare. In this sense, each person affected by NMOSD or MOGAD has a unique process and experience. Respecting and appreciating each personal journey is essential. It is also helpful to know that you do not walk the path alone—we are on a journey together to save and improve lives.

If you are like most everyone, the words neuromyelitis optica or aquaporin-4 or oligodendrocyte or the like came out of the blue when you first heard them from your doctor. The fact that the central nervous system and immune system are perhaps the most complex in the entire human body—and they intersect in NMOSD or MOGAD—adds to the mystery. Yet, due to recent efforts focused on advances in science and medicine, we know a more about how these autoimmune illnesses occur and how to minimize their effects. Learning can be truly empowering.

A diagnosis of NMOSD or MOGAD
is not your fault—
it does not make you any less meaningful
or valuable or beautiful as a person—
and it is not your identity.

10 Steps Forward.

Cureageous

Anyone who has ever used a map, GPS or digital navigation system can appreciate this fact: someone had to go there before you to help find the way. Likewise, we are grateful to the patients who have chosen to share experiences along their NMOSD or MOGAD journey with an intention to help others who follow. Based on the collective wisdom of patients, family members and caregivers who have been on this path, below are 10 Steps Forward that may be useful for those newly diagnosed with NMOSD or MOGAD:

1. Process the News in Healthy Ways:

Unexpected news about your health can be life-changing and create deeper uncertainties. Accurate diagnosis and appropriate treatments can help reduce the current and future effects of these diseases. It may be helpful to realize that such illnesses may not progress, and initial symptoms such as vision loss or paralysis may resolve completely. A focus on healing can aid recovery, and taking time to come to terms with the experience can promote a beneficial long-term plan. Sometimes patients feel a sense of urgency to react upon hearing the diagnosis of NMOSD or MOGAD that can do more harm than good. Many patients report that sharing emotions with those they love and who love them is comforting and can help address the anxiety that comes with the unfamiliar and uncertain. It is also important to tell your doctors how you are feeling emotionally as well as physically. One of the most helpful things a newly diagnosed patient or family can do is to talk with other patients, families or advocates who have walked this road before them. Our Promoting Advocacy for All resources can help you connect with others.

Looking to the Future. It is only natural to be concerned about how NMOSD or MOGAD may affect your health and your future. We understand that such a diagnosis comes with a lot of new information and may give rise to uncertainties or fear. Here are a few considerations based on experience and wisdom from patients and caregivers as well as healthcare providers:

Can I believe everything I read on the internet ?

Much of the information about NMOSD or MOGAD available on the internet is outdated or focused on worst-case scenarios. Some websites state the average survival following diagnosis is 4-5 years, after which paralysis or death will occur. The fact is there have been many amazing advances in rapid and accurate diagnosis and effective treatment of these illnesses that tell a very different story for most patients. In the last 15 years, improved speed and accuracy of diagnosis has allowed more rapid and effective treatments that can help to enhance recovery & prevent long-term disability. Laboratory and imaging test results are constantly improving, and new treatments to permanently cure these diseases are advancing. Today, there is more research than ever focused on understanding the causes of these illnesses and finding better ways to stop them—once and for all. From these perspectives, there is greater reason for optimism than ever for patients & loved ones diagnosed with NMOSD or MOGAD.

Will I recover my vision or regain mobility ?

Every first episode of NMOSD or MOGAD can be frightening and happen when it is least expected. Many patients experience impaired vision, weakness or paralysis of limbs, bladder & bowel dysfunction or other effects as first symptoms. Such effects are concerning and underscore the need for rapid and accurate diagnosis and treatment. It is important to avoid sugar-coating the seriousness of these diseases, and patients may experience long-term disability. The good news is that many patients go on to recover in-full or in-part following a first episode of NMOSD or MOGAD. It is also important to know that there are experimental methods already in clinical trials to help restore functions to optic nerves, retina and spinal cord—and to find cures.

Will I be able to have children ?

NMOSD in particular predominantly affects females, and often at a time in life when they look forward to raising a family or establishing their career. Most women diagnosed with NMOSD or MOGAD can plan for healthy pregnancies and deliver healthy babies. There is no evidence to date that would suggest NMOSD or MOGAD are heritable or can be specifically transmitted to a child. It should be noted that some women diagnosed with NMOSD have an increased risk for future miscarriage, but this risk is relatively low. Also, it is important to communicate clearly with your doctor and healthcare team if you are pregnant or planning to become pregnant. The reason is certain medications used to treat NMOSD or MOGAD may not be safe for use during pregnancy. Some patients choose to address family planning issues before beginning medications that may be potentially harmful to fertility. On a brighter note, many of the medications used as maintenance treatment are being carefully studied for safety in mother and baby during pregnancy—and for their effectiveness in preventing relapse following pregnancy. Long-term safety studies continue even after medicines receive regulatory approval.

Answers to other frequently asked questions can be found here.

We are here with you and for you.

2. Know the NMOSD & MOGAD Basics:

Protecting the body against internal threats such as cancer and external threats such as infection are key roles of the immune system. Your immune system can usually tell the difference between “self” tissue friends—and cancer or infection foes. But in NMOSD and MOGAD, the immune system mistakes normal proteins in the body as threats and reacts to them as it would if cancer or infection. This immune system mistaken identity that leads to symptoms is called autoimmune disease. In NMOSD and MOGAD, proteins the immune system mistakes as threats are enriched in the central nervous system—specifically the spinal cord, optic nerves and components of the brain. In NMOSD, this protein is aquaporin-4 (AQP4); in MOGAD the protein is myelin oligodendrocyte glycoprotein (MOG). When the immune system reacts to these self-proteins, it causes inflammation that leads to immune molecules and cells flooding into the central nervous system. In turn, this response can injure cells and the insulating material that normally covers nerves (myelin), known as demyelination. Further, fluid accumulation can cause swelling in the confined compartments of central nervous system. The combination of loss of myelin and swelling in the spinal cord, optic nerves and brain can lead to classic symptoms of NMOSD or MOGAD—namely weakness, numbness or paralysis in one or more limbs, reduced vision in one or both eyes, loss of bowel or bladder control, and impairment of other functions that are controlled by the central nervous system. This knowledge may help to explain why NMOSD and MOGAD are called inflammatory demyelinating autoimmune diseases. The latest GJCF NMO Patient Guide and NMO Resources App are rich in information that may be helpful to learn more about NMOSD and MOGAD and empower your journey.

Today we know more than ever about how
NMOSD & MOGAD occur and how to minimize their effects

3. Understand Your Specific Diagnosis:

Through recent studies it has become clearer that there are several forms of NMOSD or MOGAD. Here is a quick guide to help decode your specific diagnosis:

*Serology*:	The study of auto-antibodies that help differentiate forms of disease:
	Antibody:	anti-AQP4 (NMOSD) or anti-MOG (MOGAD)
	Serostatus:	positive, negative or unknown
*Imaging*:	Diagnostic methods that are performed to visualize affected tissues:
	MRI:	magnetic resonance imaging
	OCT:	optical coherence tomography
*Phenotype*:	The location & type of signs or symptoms of disease in an individual:
	Location:	optic nerve; spinal cord; area postrema; brain; brainstem
	Clinical Signs:	optic neuritis; transverse myelitis; brain lesions; etc.
	Symptoms:	vision loss; paralysis / weakness; incontinence; etc.
*Prognosis*:	The difference between recurring and non-recurring disease activity:
	Relapsing:	recurring and/or worsening symptoms
	Monophasic:	one disease episode; stable thereafter

For example your doctor may have stated that you have a positive anti-AQP4 antibody serology, meaning that you have anti-AQP4 auto-antibody in your bloodstream. They may have explained that you have reduced vision in one eye due to its inflamed optic nerve (optic neuritis) that was seen on MRI imaging. Or they may have mentioned you have a long area of inflammation in your spinal cord (called longitudinally extensive transverse myelitis, or LETM) in the thoracic region of your spine that accounts for your limb weakness / paralysis and loss of bowel / bladder function. This case would be considered NMOSD, with anti-AQP4 positive serology and optic nerve / spinal cord lesions that are consistent with symptoms and confirmed by MRI. In another example, the doctor may have detected anti-MOG autoantibody in the blood and brain lesions observed by MRI which are would be consistent with the diagnosis of MOGAD. These are examples, and there are several possible locations in the central nervous system that can be affected by NMOSD or MOGAD. While such specific information can be concerning, it can also be empowering to know exactly what kind of NMOSD or MOGAD you have and how best to treat it going forward. Your healthcare team can help explain further.

map of words relating to medicine, scient, immunology

4. Treating Your First Disease Episode

Because NMOSD and MOGAD feature inflammation within the central nervous system, an important first step in treatment is to put out the inflammation fire to minimize or prevent injury to optic nerves, spinal cord or brain. Treatments designed to quickly reduce inflammation in an NMOSD or MOGAD onset episode or relapse event are known as rescue therapy. There are three rescue therapy regimens most often used for this goal, either alone or in combination: 1) intravenous corticosteroids; 2) intravenous plasma exchange; and 3) intravenous immunoglobulins. It can be reassuring to understand how these treatments are done and how they work to help resolve NMOSD or MOGAD disease episodes.

Intravenous Corticosteroids.

One of the first rescue therapies commonly used in NMOSD or MOGAD onset episode or relapse is an intravenous corticosteroid. Such medicines are delivered directly into the bloodstream through a small intravenous catheter. The catheter may remain in place for several days to allow for daily dosing without the need for repeated needle sticks. The two most commonly used corticosteroids to treat NMOSD or MOGAD are intravenous methyl-prednisolone (IVMP) or intravenous dexamethasone (IVDX). These drugs work by inhibiting many molecular and cellular functions involved in inflammation. The medicines bind to receptors on cell membranes, which in turn bind to DNA to stop the production of proteins that drive inflammation, such as white blood cell activities. Numerous clinical studies have demonstrated that rapid IVMP or IVDX treatment can reduce risks of central nervous system injury due to inflammation, and promote better long-term outcomes. Key functions of IVMP or IVDX include: a) they can work relatively quickly to suppress inflammation; b) the bloodstream delivers these medicines across the blood-brain barrier so they access sites of inflammation in the central nervous system; and c) they are usually well tolerated by most patients. There are potentially serious side effects of IVMP or IVDX if used long-term, including increased risks of infection, osteoporosis / brittle bones, kidney stones, cataracts and weight gain. However, these issues are not typically seen with short-term use of IVMP or IVDX as rescue therapy for NMOSD or MOGAD onset episodes or relapses.

Intravenous Plasma Exchange.

Another treatment that may be used in addition to other rescue therapies for NMOSD or MOGAD onset or relapse episodes is called plasma exchange (also known as PLEX or plasmapheresis). This procedure introduces two catheters into separate blood vessels in the body, typically one in each arm or just below the collar bone. The goal of PLEX is to remove the harmful molecules from the bloodstream (e.g. anti-AQP4 or anti-MOG antibodies), without depleting the body of healthy blood cells. To do so, blood is slowly removed through one of the catheters and passed through a machine that sorts cells from fluid portion of blood (plasma). Here, the antibodies are removed but the cells are returned to the body (along with buffer fluid and albumin) through the opposite catheter. PLEX treatment for NMOSD or MOGAD usually takes 3-4 hours, and may be repeated every other day totaling up to 5 treatments. After PLEX treatment, more than 95% of all antibodies are removed from the blood, including anti-AQP4 or anti-MOG antibodies. PLEX can be used in addition to IVMP or IVDX therapy, as these drugs are too small to be removed from the bloodstream by the PLEX process. Helpful functions of PLEX include: a) rapid removal of potentially harmful autoantibodies from the bloodstream; b) little or no impact on other immune functions such as white blood cells, T cells or B cells; and c) the procedure is often well tolerated by most patients. There are potential side effects of PLEX, including increased risk of infection (as antibodies protective against infection are also removed), a need for larger catheters that remain in place for about a week, and use of medicines to prevent blood coagulation (which may cause bruising at the catheter insert sites or elsewhere).

Intravenous Immunoglobulins.

A third strategy may also be used in treating NMOSD or MOGAD onset or relapse episodes. After completing PLEX (if used), your doctor may administer intravenous immunoglobulins (IVIg) into your bloodstream. Immunoglobulin is the scientific name for “antibody”. You may be asking — “why would I want antibodies put into my body if PLEX was just used to remove them ? ” Here is the answer: IVIg is a pool of many different kinds of antibodies from many healthy people. Some of these antibodies are believed to block the actions of harmful autoantibodies, such as anti-AQP4 or anti-MOG. This concept could be thought of as fighting fire with fire: using good antibodies to protect against bad antibodies. Key functions of IVIg include: a) rapid inhibition of potentially harmful autoantibodies that may have not been removed by PLEX, or if PLEX is not used; b) return of antibodies that may help protect against infection; c) no known impact on other immune functions such as white blood cells, T cells or B cells; and d) the use of IVIg is almost always well tolerated by patients. The potential side effects of IVIg are usually limited to allergic or infusion-related reactions, which are typically preventable by antihistamines or other pre-medications. It is important to note that IVIg is not given prior to PLEX, as PLEX will remove these potentially beneficial antibodies, just as it removes potentially harmful antibodies.

5. Returning Home from the Hospital:

Knowing what to expect after your first NMOSD or MOGAD episode can help calm concerns. While the symptoms of an NMOSD or MOGAD onset or relapse episode can come on quickly and without warning, resolution of these symptoms can take time. The reality is that vision, limb strength or function, bowel / bladder control and other symptoms can take weeks or longer to recover. It is possible that some functions may not return to normal. As in many aspects of life, adapting to new ways can be healthy and enhance meaning. It may be helpful to create a staged set of expectations and milestones as a recovery plan along with guidance from your doctor, healthcare team and caregivers. Setting daily, weekly, monthly and even annual routines may be useful to feel a sense of normalcy and make steady progress. There may be medicines to finish—such as tapering down or off steroids used to help treat your initial disease episode. You may also need to make visits to new doctors or clinics along the road to recovery. For example, most patients had never been seen by a neurology specialist before their NMOSD or MOGAD diagnosis. There may also be visits to physical therapy, eye doctors, infusion centers and more. It may feel like your life is now focused only on interacting with the healthcare world. Patients and caregivers can find healthy ways back to normalcy. Balancing hope and realistic expectations can be helpful to regain a sense of control, keep track of important clinical visits and milestones, and setting & measuring progress toward recovery goals with your healthcare team.

Another key step toward your recovery is to create a safe and healing environment in your home. Patients can have reduced vision, weakness, imbalance and incontinence as a result of their initial NMOSD or MOGAD episodes or relapses. Caregivers are invaluable in helping patients during such special stages of their recovery. Preparing meals, aiding with mobility, managing bathroom needs, arranging transportation to and from the clinic and assisting with grooming & hygiene practices are key considerations. In time, most NMOSD and MOGAD patients return to have quality of life.

6. Starting Your Maintenance Therapy:

Relatively soon after your first NMOSD or MOGAD episode, or a relapse event, your doctor will help you decide on a maintenance therapy. There are certain differences between treatments used to resolve a first episode or relapse—and those intended to prevent future relapses. Treatment regimens designed to prevent future relapses and assist in controlling day-to-day symptoms are called maintenance therapy. Today, there are several regulatory-approved maintenance therapies to consider with your doctor. Each of these medicines achieved 80% or greater success in preventing future relapses over approximately 2 years time in formal clinical trials. Some basic information about approved therapies for adults diagnosed with anti-AQP4+ NMOSD is listed alphabetically below:

*Drug Name*	*Brand Name*	*Dose Route*	*Dose Frequency*	*Target*
Eculizumab (ec-u-liz-u-mab)	Soliris®	Infusion	Every 2 Weeks	C5 Protein
Inebilizumab (in-eb-il-iz-u-mab)	Uplizna®	Infusion	Approximately Every 6 Months	CD19+ B Cells
Satralizumab (sat-ra-liz-u-mab)	Enspryng®	Injection	Every 4 Weeks	IL-6 Receptor

These medicines are not yet approved for pediatric NMOSD, or patients with MOGAD. However, important clinical trials are ongoing to test these and other medicines for safety and effectiveness in special populations in NMOSD and MOGAD, including pediatric and pregnant patients and those who have autoimmune or other illnesses in addition to NMOSD or MOGAD.

You and your healthcare team will work together to choose the treatment regimen that gives you a best chance of avoiding relapses, managing day-to-day symptoms and promoting long-term health and quality of life. In some cases, medicines that are not formally approved may be used as maintenance therapy for NMOSD or MOGAD. Such therapies are often considered “off-label”, meaning they have not been regulatory-approved for use in treating NMOSD or MOGAD. Examples are listed below in alphabetic order:

*Drug Name*	*Brand Name*	*Dose Route*	*Dose Frequency*	*Target*
Azathioprine (a-za-thi-o-prine)	Imuran®	Oral	Daily	Immune Cells
Mycophenolate (my-co-phen-o-late)	CellCept®	Oral	Daily	Immune Cells
Ravulizumab (rav-u-liz-u-mab)	Ultomiris®	Infusion	Every 8 Weeks	C5 Protein
Rituximab (ec-u-liz-u-mab)	Rituxan® Ruxience® Truxima® Rixathon®	Infusion	Approximately Every 6 Months	CD20+ B Cells
Tocilizumab (to-cil-iz-u-mab)	Actemra®	Infusion or Injection	Every 4 Weeks	IL-6 Receptor

Transitioning from rescue therapy to maintenance therapy can be confusing. You may ask – “Why not just continue the rescue treatments that helped resolve your first episode or relapse ?” While rescue treatments for can be very effective, they can also come with important side effects. For example, using high-doses of corticosteroids for a prolonged time can have negative effects on health. Likewise, PLEX is not usually practiced as a maintenance treatment, as frequent or constant access to blood vessels can create or increase risks of infection or other health concerns.

Just as important as deciding which maintenance therapy may be best for you, carefully planning how to shift from rescue to maintenance therapy is also important. The ABCs of transitioning from rescue treatment for a first episode or relapse to a long-term maintenance therapy include:

A) Decision Making:

This step toward your long-term maintenance therapy considers all of the available information about your particular type of NMOSD or MOGAD, safety and efficacy data of medicines available to you, as well as practical considerations such as frequency and mode of administration, cost and convenience of therapy.

B) Induction Phase:

The induction phase is a next step in the process of transitioning from rescue therapy to maintenance therapy. Once you and your healthcare team have decided on your long-term treatment plan, a series of preparatory actions will take place. A first part of this plan is determining a good timeline for tapering down or off rescue steroids. Stopping steroid treatment too quickly can cause serious side effects, so this tapering is usually achieved over a series of several days to a few weeks. During this time, laboratory screening is done to ensure you have lowest risk factors related to your planned maintenance therapy. For example, a routine blood draw will allow your doctor to review your white blood cell count and antibody status, be aware of any silent infections you may have, assess the functions of your liver and so on. Your doctor may suggest or require that you update your vaccinations before you may receive any maintenance therapy. Certain vaccinations are required to reduce risks of particular infections before beginning some maintenance therapies. Once all these boxes are checked, your doctor or healthcare provider will prescribe your maintenance therapy to begin. The initial dosing of maintenance therapy is more frequent than it will be thereafter. This stage in your maintenance therapy is called the induction dosing, and is designed to quickly bring your medicine up to optimal levels for safety and effectiveness.

C) Maintenance Phase:

Depending on your specific treatment regimen, you will be on your way to long-term interval dosing of maintenance therapy within a few weeks of induction phase. Interval dosing of maintenance therapy is determined by the type of medication you will use (see table, above). At regular time points, your doctor will request that you have your blood drawn for routine laboratory monitoring to be sure the maintenance therapy continues to be safe for use. In some cases, slight variances in dosing interval may be directed by your doctor based on laboratory findings such as counts of certain types of white blood cells, antibody levels, liver function or other measures. Often, once a patient has received several maintenance phase doses any initial laboratory abnormalities can return to normal as the body adapts to the new medicine. Thereafter, it is very important to adhere to a schedule of regular check-ups with your NMOSD or MOGAD specialty neurologist and other healthcare team members.

7. Helping to Prevent Future Relapses:

Perhaps the most essential goal of healthcare in developing a treatment plan for NMOSD or MOGAD is minimizing the chances of having a future relapse. Your maintenance therapy is certainly one important part of this plan. Yet, there are other things you and your healthcare team can do to help achieve this goal. These actions include adhering to your maintenance treatment dosing, laboratory monitoring and regular check–up schedule. Likewise, keeping your physical activity to a reasonable level—which may include a healthful exercise regimen if instructed by your doctor—and minimizing unnecessary stress wherever possible may lower relapse risks. Use caution and be sure to let your doctor know before beginning other medications or non-prescription supplements. Avoid smoking if at all possible—and if you drink alcohol, do so in moderation and with responsibility. Certain recreational drugs may interfere with maintenance therapy and alter neurological functions; please consult your healthcare team on this topic. Maintaining a healthy diet rich in fruits and vegetables, low to moderate amounts of salt and caffeine, and which contain high-quality proteins such as fish or poultry can also lower inflammatory set points. Enjoying adequate amounts of healthful sleep sessions containing 3-4 hours of REM sleep may also be helpful to enhance immune health and reduce risks of relapse. This type of sleep occurs late in the course of a sleep cycle, so it is important to get plenty of healthful sleep. In these ways, a holistic approach to care may enhance health and aid maintenance therapy in reducing risks of relapses.

icons including a stethoscope, clock, bowl of fruit, no smoking, wine, sleep

8. Reducing Your Risks of Infection:

Infections are the most common side effects from maintenance therapy for NMOSD or MOGAD. Often, these may include urinary tract infections (UTI) or upper respiratory infections (URI), and most are treatable with appropriate anti-infective therapy. Some maintenance therapies increase risks of special kinds of infection, including reactivation of latent viruses that cause shingles, cold sores, genital lesions or hepatitis, latent bacterial infections such as tuberculosis or fungal infections caused by normally harmless yeasts or molds. And just to be on the safe side, your doctor may test to see if you harbor a latent infection caused by a virus called JCV. This virus is found a many people, but rarely causes disease, and when it does usually only in individuals who have certain types of suppression of their immune system.

An important part of any plan to reduce risk of preventable infections is appropriate vaccination. Receiving your vaccinations in advance of beginning maintenance therapy, receiving boosters on schedule and keeping immunizations up to date may all help to reduce risk of infection in persons living with NMOSD or MOGAD. Your doctor is required to have you vaccinated against a specific type of brain infection called meningitis if you use certain types of maintenance therapy. Immunization schedules that are generally recommended by the Centers for Disease Control (CDC) for children or adolescents and adults are available here. Please note: vaccinations are not one-size-fits all, and your healthcare team will help you navigate which immunizations are best for you based on your NMOSD or MOGAD treatment regimen and other factors.

On a daily basis, there are also things you can do to help reduce risks of infection. Such routines include: 1) maintain best hygiene practices—keeping hands clean, avoiding touching your face or rubbing your eyes, frequent bathing & daily grooming including good dental health all add up to help guard against infection. 2) optimize urinary tract health—urinary tract infections or UTIs are among the most common infections in patients being treated for NMOSD or MOGAD. Such infections are usually caused by germs that normally colonize the human urinary tract. Drinking plenty of water can help maintain hydration and promote healthy urination to help clear microbes from the urinary tract. Cranberry juice or pineapple juice may help prevent UTIs because these fruits contain molecules that impair microbial growth. These strategies may need to be balanced in patients experiencing urinary incontinence. For patients having bowel or bladder dysfunction, frequent changing of incontinence underwear can avoid UTIs; and 3) optimize respiratory tract health—respiratory tract infections are commonly reported in patients receiving maintenance therapy for NMOSD or MOGAD. Such infections include viral colds or flu, COVID-19, as well as bacterial sinusitis, bronchitis or related conditions. Left untreated, these infections can lead to pneumonia or other more serious situations. Along with best hygiene practices as above, patients who have experienced recurrent URIs may find that routine masking reduces risk of infection, particularly in setting of increased chance of exposure. Maintaining healthy levels of vitamin D may enhance your immunity against infection. However, there is a “goldilocks effect” when it comes to vitamin D: too little and too much are not healthful. Your doctor can help to ensure your vitamin D levels are just right. In the same way, using zinc tablets or high-dose vitamin C to prevent or treat colds or flu may or may not reduce risks of infection, but they could interfere with maintenance therapy medications. It is best to consult your doctor before using any supplements; and 4) get out in front of infection—even the most careful among us will eventually come down with some common infections from time to time. The cardinal signs of infection are redness, swelling, pain and fever. The cardinal symptoms of infection include fatigue, weakness, aching bones or joints, pain or itching before or during urination, as well as nausea, vomiting or diarrhea. If you begin to experience any such signs or symptoms—contact your NMOSD or MOGAD neurologist and other healthcare team members right away. To quote a useful adage: an ounce of prevention is worth a pound of cure.

graphic of a shield surrounded by microbes

9. Managing Day-to-Day Symptoms:

Preventing relapse and minimizing chances of side effects from medication such as infection are priority goals of maintenance therapy. Yet, patients diagnosed with NMOSD or MOGAD may experience day-to-day symptoms that also need to be addressed. These symptoms may include pain, fatigue, bowel or bladder incontinence or retention, cognitive dysfunction and insomnia. It is important to note that mental wellness can be negatively affected as well, and may present as anxiety, depression or grief. Below are a few themes for consideration that come from the wisdom of patients, caregivers and experts in their NMOSD and MOGAD journeys.

Relapse or No Relapse ?:

One of the most important questions patients face in their NMOSD or MOGAD experience is whether symptoms are day-to-day or represent the early signals of a relapse. A key to minimizing chances of long-term disability is to recognize and treat a relapse at its earliest possible stage. This fact supports a prudent way to decide when to call your doctor to discuss new, changing or worsening symptoms:
if in doubt—talk it out with your NMOSD or MOGAD neurologist & healthcare team.

Without a clinical examination, there is no certain way for you to tell if new, changing or worsening symptoms are warning signs of a relapse. However, here are a few themes that can be helpful for you and your doctor to consider together—perhaps quickly over the telephone or a telehealth visit:

Are you having signs of infection, such as fever, chills, cough or urgency in urination ?
Have you been under new, prolonged or intense periods of stress in recent weeks ?
Are you having prolonged episodes of nausea or vomiting, pain or extreme fatigue ?
Have you experienced a lack of sleep or a change in nutrition in your daily routine ?
Are your symptoms in a new location in your body and have they lasted > 24 hours ?
Are your symptoms recurring in a prior location in your body and lasting < 24 hours ?

Symptoms that are consistent with a relapse (e.g. eye pain or vision loss, numbness or weakness in a limb) but occur in a new location in the body and persist for 24 hours are important to let your doctor know right away. Symptoms in prior sites that resolve in < 24 hours are often not relapses.

Should I call my doctor about a possible relapse ?
If in doubt — talk it out…

Chronic Symptoms.

Because NMOSD and MOGAD affect the central nervous system, they can cause symptoms beyond specific inflammation and demyelination. Understanding and addressing your day-to-day symptoms can help to manage or even eliminate them. Here are some useful themes based on experience from patients, caregivers and neurology experts:

Pain:

As NMOSD and MOGAD can be demyelinating, these diseases may reduce, disrupt or exaggerate pain signals within the body. Some pain signals may come from actual tissue lesions caused by tissue inflammation or edema. This type of pain is known as nociceptive pain. This type of pain usually involves nerve injury, and is often described by patients as having sharp, shooting, stabbing or burning sensations that wax and wane from day to day. Nociceptive pain is typically associated with areas in the central nervous system that have active demyelinating lesions. Pain signals may also result from the miscommunication of nerves to brain, even if there is no specific tissue lesion or injury. This type of pain is known as neuropathic pain. Neuropathic pain is often described as pins and needles, tingling, numbness or loss of sensation. It can localize to sites in the central nervous system affected by NMOSD or MOGAD, but may also occur at sites distant from the spinal cord or optic nerves, such legs and feet. Another type of pain that may be experienced in NMOSD or MOGAD is called neurospastic pain. This type of pain occurs if muscular tone is excessive due to altered nerve pathways controlling the muscles. Stiffness or cramping of muscle can be significant, leading to temporary or sustained pain. A fourth type of pain is known as radicular pain. This form of pain means it begins in one place in the body and radiates to other places. Most commonly, radicular pain may originate in the lower back and radiate to the legs, knees or feet. Radicular pain may be due to NMOSD or MOGAD, but of course can occur in otherwise healthy individuals particularly with aging. Pain that is caused by NMOSD or MOGAD may flare up from time to time or occur chronically. Depending on the type(s) of pain, its severity and acute or chronic pattern, certain medicines may be helpful for you. Different types of pain often require different treatments. Your neurologist or a pain specialist may prescribe medications; if so their use is often customized to each person to find the lowest dose that achieves relief. Some of these medicines target nerve tissue to interfere with pain signals, others target broader ways that pain occurs. Physical therapy may also be part of healthy pain management, including special exercises, stretching routines or yoga approved by your doctor. Pain may also benefit from icing or warming methods, therapeutic massage or alternative methods such as acupuncture or related practices focusing on the affected areas of the body. Medications, exercises or other practices to manage pain should be under the direction of your NMOSD or MOGAD specialist and/or physical therapist.

Fatigue / Lethargy:

Fatigue or lethargy can be common symptoms in NMOSD or MOGAD patients, especially early after diagnosis. Patient experience teaches that fatigue can result from a number of factors, some of which are due to NMOSD or MOGAD or their treatment—and some of which are not directly due to these illnesses. For example, fatigue can result from increased stress, pain, depression and other psychological factors as well as suboptimal sleep.

Bowel / Bladder Issues:

Bowel or bladder incontinence or retention can be experienced by NMOSD and MOGAD patients when inflammation in the central nervous system is localized to nerves that control these functions. It is important to understand the difference between bowel or bladder incontinence or retention and gastrointestinal or urinary tract infection. Infection is usually characterized by burning, itching or perhaps even blood present in urine. Similarly, gastrointestinal infections tend to be associated with nausea or vomiting, loose stool or diarrhea, and blood may be present in the stool. Fever often accompanies infection, but is usually absent in basic incontinence or retention.

Cognition Issues:

Some NMOSD or MOGAD patients report a sense that they lose the ability to concentrate or maintain clarity in thinking from time to time or for longer periods of time. Autoimmune diseases that affect the central nervous system have the potential to affect functions of the brain—so it is only natural that there may be concerns about diminished cognition. However, it is also important to realize that some loss in the ability to concentrate or remember names, faces or facts is also natural in the course of aging.

Sleeping Issues:

Trouble sleeping meets us all at some point in life. Disrupted patterns of sleep or the inability to sleep healthfully is called insomnia. Insomnia can be caused directly due to NMOSD or MOGAD affecting neural sleep centers located in the brain stem. It can also be caused by other factors, including pain, depression or stress. In some cases, NMOSD or MOGAD can result in a condition known as narcolepsy—where patients experience an excessive or uncontrolled urge to sleep. Quality sleep is an important part of a complete health & wellness plan.

Psychological Issues:

Any difficult diagnosis can lead to concerns that affect optimism and outlook. A sense of loss, grief and fear are not uncommon in patients who have a diagnosis of NMOSD or MOGAD. In turn, these feelings can manifest as depression or isolation syndromes that add to physical effects of these conditions. Addressing emotional or psychological impacts caused by NMOSD or MOGAD is as important as addressing their medical impact. It is key that patients and caregivers communicate their feelings openly and honestly to healthcare providers. Doing so is not a sign of weakness or giving up—just the opposite—asking for help is a sign of refusing to give up.

Every patient affected by NMOSD or MOGAD may experience one or more of these issues, and no two patients have the exact same journey. Each of these symptoms may be occasional, episodic or they may persist. The key is to make sure your neurologist and other members of your healthcare team know about any intermittent or chronic symptoms that negatively affect your quality of life. Treatments and methods to minimize or resolve such symptoms are available, and clinical studies are ongoing to understand such issues and find ways to stop them.

Asking for help is not a sign of giving up—
asking for help is a sign of refusing to give up.

10. Keeping a Focus on Resilience:

One lesson learned from patients, family members, caregivers and healthcare providers is as true today as when our mission began: when one person is affected by NMOSD or MOGAD—we are all affected. This same truth rings through how we face these illnesses: we are stronger together. The GJCF is the home for patients newly diagnosed with NMOSD, MOGAD or closely related illnesses. We have an international Helpline that is there when you need to talk to someone who knows the journey. And while there may be some tough days, amazing progress has been made in developing treatments that prevent relapse and improve long-term outcomes. In this respect, we are mindful of an important concept: every patient is a person first. You may have NMOSD or MOGAD, but the disease does not have you—and it does not define you.

Every patient is a person first.

Resilience is the ability to adapt to new or challenging circumstances in ways that preserve or even enhance ability, outlook, purpose and meaning in life. In a recent study of experience and quality of life in those facing NMOSD or MOGAD, we made a discovery that offers hope and proof that resilience can be as life-enhancing as any medicine. Resilience is also important for those caring for patients with NMOSD or MOGAD, as well as their families and beyond. Everyone benefits when we put people first and focus together on resilience. Let’s help the person not just treat the disease.

✅ Adapt and Advance:

A diagnosis of NMOSD or MOGAD can feel like it brings your world to a halt—that everything you knew or hoped or planned for suddenly no longer applies. It can be a life-changing moment. But it does not have to be a change for the worse. We all face unforeseen events and challenges in our lives that call us to answer a profound question: how will I respond? Adapting to treatment and recovery may take time, but it can reveal many positive aspects of living that had been unnoticed or under-appreciated. Individuals facing NMOSD or MOGAD often speak of becoming closer to family and friends and recognizing how much people care about them. Faith can grow in ways that might not have otherwise. Purpose and meaning in each day can be defined and used to gain strength physically and emotionally. One simple key is this: adapt as best you can at that moment—and keep moving forward to new moments. Advances in therapies and cures for these diseases are coming and we are doing all we can to reach these goals at the speed of life.

Adapt as best you can at that moment—
and keep moving forward to new moments.

✅ Tell Your Own Story:

Every person facing NMOSD or MOGAD has a different journey. Expressing yourself can be therapeutic, and the fact that only you can tell your personal experience makes you rare among rare. The question is how will you tell your own story ? The GJCF advocacy team offers a number of ways for you to tell your story—and in doing so help the next person who learns of their diagnosis with NMOSD or MOGAD. The NMO Stories program is a perfect venue for you to share your thoughts in a way that may help many others. You may also find new friends and hear stories of other people facing the same diagnosis that help you. Let’s make sure the world knows about NMOSD and MOGAD and how everyone can contribute to the cure.

✅ Join a Support Group:

Individuals diagnosed with NMOSD or MOGAD often report that they initially feel isolated and afraid. You are not alone—and we are here to help. Sometimes it is helpful to connect with others who have the same diagnosis and are on a similar journey. The GJCF hosts a network of Support Groups created by patients and caregivers just who know how you may be feeling. All are welcome to find a group you relate to and with no obligation or judgement. You may also learn more about the GJCF Patient Advocacy Council promoting advocacy worldwide.

✅ Be an Active Participant:

Active participation can be a great way to find your resilience. Some people find expressing their thoughts or feelings through art enhances their wellness. The GJCF Artistry iNMOtion Program offers regular interactive workshops in which attendees are guided by certified art therapists to mindful self-expression. Others may find that journaling or constructive blogging or sharing optimism through social media give them a voice and sense of contributing to the greater good that promotes resilience. Discover a skill or ability or voice you did not know you had until NMOSD or MOGAD entered your life. Whatever your medium, you are a meaningful and rare partner on the road to cures.

✅ Consider Clinical Trials:

It may also be helpful to keep in mind that persons who face a rare disease have a special opportunity to teach the world about the body in ways that hold secrets to understanding and solving NMOSD, MOGAD and other diseases. How ? Because being rare is the exception that proves the rule. More than any common disease, each person with a rare disease may make a huge impact in science & medicine that can help others. For example, only 560 patients participated in the four clinical trials that led to three approved therapies. In other words, a small number of NMOSD & MOGAD heroes opened the door to treatments that are saving and improving lives for hundreds of thousands of patients around the world. You are the cure.

We study disability.
We promote resilience.

Keep Hope & Gratitude.

These 10 Steps Forward for the Newly Diagnosed are intended to offer useful information and specific resources to help you & yours during the first days following diagnosis of NMOSD or MOGAD. They represent a practical guide based upon the wisdom of many patients, caregivers and healthcare providers who have been down this path before you. While the GJCF does not provide any recommendations for clinical care or therapy, it does offer hope and gratitude to all those who have given so much of their energy and effort to help everyone facing these diseases. Every day we work together to solve NMOSD & MOGAD—the closer we are to a cure. And the amazing progress made in understanding and treating these diseases is opening new doors to help solve many other diseases—rare and not so rare. It was once said that NMO is “the little disease the could…”. With your help and dedication, NMO has become the little disease that did save and improve lives around the world and is now seen as a model for solving other diseases.

Every day we work together
to solve NMOSD and MOGAD—
the closer we are to a cure.