Life with a chronic health condition can feel overwhelming and lonely, especially when it’s a rare disease that often goes misdiagnosed. This is the reality for the estimated 10,000-15,000 people in the U.S. who live with neuromyelitis optica spectrum disorder (NMOSD), sometimes referred to as Devic’s disease. NMOSD is an unpredictable autoimmune disease driven by severe attacks to the spinal cord and optic nerve, which can cause lasting pain, vision loss, paralysis and sensory loss.1
Christine, who lives with NMOSD, says this about experiencing an attack:
“You’ll get an ‘attack’ out of the blue, and you don’t know how it’s going to affect you or how long it’s going to take to recover. I tell people it’s sort of like multiple sclerosis (MS), which NMOSD is often misdiagnosed as, but all the damage is done during that one attack. NMOSD doesn’t build up over time: instead, you just get the attack, then wait and see if you get better with whatever treatment you’re using.”
Early intervention and effective management are key to preventing permanent damage caused by NMOSD attacks. We talked with Dr. Ahmed Obeidat, a neurology specialist, about his patient-centered approach to treating NMOSD.
What have you learned from helping people living with NMOSD?
I’ve been working with adults who have MS and other autoimmune disorders of the central nervous system, including NMOSD, since 2008. Over those 14 years, I’ve come to understand that each patient is different, from their symptoms and treatment expectations to how they handle the emotional burden of living with a chronic rare disease. I’m continuously inspired by the strength and determination of the NMOSD community and have learned how powerful the patient voice is. As physicians, it’s our responsibility to truly listen to our patients as we make treatment decisions.
What role should NMOSD patients play in their care?
In my practice, the patient is the captain of the care team. We encourage them to talk openly with us. It’s through this openness that a long-lasting relationship is formed that leads to the best outcomes. Every patient, no matter who their doctor is, should feel empowered to speak up and ask for the care they deserve.
How can patients better advocate for themselves?
I tell my patients to keep a journal of their symptoms and flag any changes or irregularities. For instance, if they are more tired than usual or they can no longer do a certain activity—no matter how unimportant it may seem—I want to know about it. It’s also important for me to be aware of any side effects they are experiencing from their treatments so we can determine together whether there may be a better option for them. Remember, you know your body best and if something doesn’t seem right, speak up. If a doctor isn’t taking your concerns seriously, or it seems as though they aren’t listening to you, then it may be beneficial to get a second opinion from a different specialist.
How does the science of NMSOD influence treatment decisions?
NMOSD occurs when the body’s immune system, comprised of B cells, antibodies and other key elements, mistakenly attacks healthy cells in the central nervous system, leading to inflammation. B cells have multiple functions in the body to protect us from disease, such as producing antibodies that are helpful in recognizing and fighting infection.2 In the case of NMOSD, these antibodies recognize the body’s cells as “invaders.” Understanding the role of B-cells in NMOSD is important because it allows us to decide with our patients whether a B-cell depleting medicine may be right for them.
How have options for treating NMOSD changed over the past few years?
The NMOSD treatment landscape has changed immensely over the past few years with the U.S. Food and Drug Administration (FDA) approval of three medicines. For instance, there is now an FDA-approved medicine called UPLIZNA® (inebilizumab-cdon) that works by reducing the number of B cells in the body.3 It is an intravenous (IV) infusion, which means it is delivered through a needle that is placed in the patient’s arm. It is given twice a year after two initial start-up doses.3
What should patients consider when choosing a treatment with their doctor?
It is important to understand the safety and efficacy (how well it works) of different medicines. It can be helpful to visit the official website for each medicine to learn about the results of the clinical studies on which FDA approval was based, the side effects that others have experienced and how the medicine is given.
I also discuss lifestyle preferences with my patients. For instance, would they prefer to have a healthcare professional administer their treatment or do it on their own from home? Another common discussion is around dosing and whether they prefer to have to take a medicine less frequently.
Overall, I want patients to be an advocates for their health and approach treatment decisions as a shared decision-making process with their healthcare team—remembering there are options available.
How do you know when it may be time for a patient to try a different medicine?
In general, the most common reason patients are transitioned to a different medicine is if they have a new attack or serious side effects. But there can also be more subtle reasons to switch, like nagging symptoms, side effects that are no longer manageable or a dosing schedule that is too frequent to maintain.
Overall, every patient experience is unique and so too are the reasons for switching treatments. I encourage my patients to keep track of their symptoms and how they are feeling and have an open dialogue with their doctor about their options.
Dr. Obeidat participated in this interview in partnership with Horizon Therapeutics.
IMPORTANT SAFETY INFORMATION
What is UPLIZNA?
UPLIZNA is a prescription medicine used to treat adults with neuromyelitis optica spectrum disorder (NMOSD) who are anti-aquaporin-4 (AQP4) antibody positive.
It is not known if UPLIZNA is safe or effective in children.
Who should not receive UPLIZNA?
You should not receive UPLIZNA if you have:
- – had a life-threatening infusion reaction to UPLIZNA.
- – an active hepatitis B virus infection.
- – active or untreated inactive (latent) tuberculosis.
Before receiving UPLIZNA, tell your healthcare provider about all of your medical conditions, including if you:
- – have or think you have an infection.
- – have ever taken, currently take, or plan to take medicines that affect your immune system, or other treatments for NMOSD. These medicines may increase your risk of getting an infection.
- – have or have ever had hepatitis B or are a carrier of the hepatitis B virus.
- – have or have ever had tuberculosis.
- – have had a recent vaccination or are scheduled to receive any vaccinations. You should receive any required vaccines at least 4 weeks before you start treatment with UPLIZNA.
- – are pregnant or plan to become pregnant. It is not known if UPLIZNA will harm your unborn baby. Females should use birth control (contraception) during treatment with UPLIZNA and for 6 months after your last infusion of UPLIZNA.
- – are breastfeeding or plan to breastfeed. It is not known if UPLIZNA passes into your breast milk. Talk to your healthcare provider about the best way to feed your baby if you receive UPLIZNA.
Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.
What is the most important information I should know about UPLIZNA?
UPLIZNA may cause serious side effects, including:
Infusion reactions. UPLIZNA can cause infusion reactions that can be serious or may cause you to be hospitalized. You will be monitored during your infusion and for at least 1 hour after each infusion of UPLIZNA for signs and symptoms of an infusion reaction. Tell your healthcare provider right away if you get any of these symptoms:
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If you develop an infusion reaction, your healthcare provider may need to stop or slow down the rate of your infusion and treat your symptoms.
Infections. Infections can happen during treatment with UPLIZNA. Tell your healthcare provider right away if you have an infection or get any of these symptoms:
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- UPLIZNA taken before or after other medicines that weaken the immune system may increase your risk of getting infections.
- Hepatitis B virus (HBV) reactivation. Before starting treatment with UPLIZNA, your healthcare provider will do blood tests to check for hepatitis B viral infection. If you have ever had hepatitis B virus infection, the hepatitis B virus may become active again during or after treatment with UPLIZNA. Hepatitis B virus becoming active again (called reactivation) may cause serious liver problems, including liver failure or death. Your healthcare provider will monitor you if you are at risk for hepatitis B virus reactivation during treatment and after you stop receiving UPLIZNA.
- Progressive Multifocal Leukoencephalopathy (PML). PML may happen with UPLIZNA. PML is a rare brain infection that leads to death or severe disability. Symptoms of PML may get worse over days to weeks. Call your healthcare provider right away if you get any of these symptoms:
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- Tuberculosis (TB). TB is caused by an infection in the lungs. Before starting treatment with UPLIZNA, your healthcare provider will check to see if you are at risk for getting TB or have ever had TB.
- Vaccinations. Certain vaccines, called “live” or “live attenuated” vaccines, are not recommended in people receiving UPLIZNA. Talk to your healthcare provider before receiving any vaccinations. If you have a baby and you were receiving UPLIZNA during pregnancy, it is important to tell your baby’s healthcare provider about your UPLIZNA use so they can decide when your baby should receive any vaccine.
See “What are the possible side effects of UPLIZNA?” for more information about side effects.
How will I receive UPLIZNA?
- UPLIZNA is given through a needle placed in a vein (IV or intravenous infusion) in your arm.
- Before treatment with UPLIZNA, your healthcare provider will give you a corticosteroid medicine, an antihistamine, and a fever prevention medicine to help infusion reactions become less frequent and less severe. See “What is the most important information I should know about UPLIZNA?”
- Your first dose of UPLIZNA will be given as 2 separate infusions, 2 weeks apart.
- Your next doses of UPLIZNA will be given as one infusion every 6 months.
- Each infusion will last about 1 hour and 30 minutes. After each infusion, you will be monitored by a healthcare provider for at least 1 hour.
What are the possible side effects of UPLIZNA?
UPLIZNA may cause serious side effects, including:
- See “What is the most important information I should know about UPLIZNA?”
- low blood cell counts. UPLIZNA may cause a decrease in some types of blood cells. Your healthcare provider will do blood tests to check your blood cell counts.
The most common side effects include urinary tract infection and joint pain.
These are not all the possible side effects of UPLIZNA.
Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
1 Ajmera MR, Boscoe A, Mauskopf J, Candrilli SD, Levy M. Evaluation of comorbidities and health care resource use among patients with highly active neuromyelitis optica. J Neurol Sci. 2018;384:96-103.
2 Forsthuber TG, Cimbora DM, Ratchford JN, Katz E, Stüve O. B cell-based therapies in CNS autoimmunity: differentiating CD19 and CD20 as therapeutic targets. Ther Adv Neurol Disord. 2018;11:1756286418761697.
3 UPLIZNA (inebilizumab-cdon) [prescribing information] Horizon.
07/22 P-UPZ-US-00340