(BPT) - Sally is a second grade teacher who loves spending time with her family and being outdoors — she has always been healthy and active, enjoying hikes and practicing yoga. Four years ago, she started experiencing a strange pain in her right arm and hand.
“It felt like a combination of numbness and shooting pain,” describes Sally. “I thought I had a pinched nerve, so I made an appointment with my physician to figure out what was wrong.”
Difficulties with diagnosis
Her first doctor’s visit resulted in an unexpected diagnosis — Sally was told that she had suffered a stroke, which did not make sense to her. Questioning the diagnosis and wanting more clarity about her health, she met with a neurologist, requesting an MRI to evaluate her cervical spine. The MRI showed lesions on her spine, which led to another diagnosis: multiple sclerosis (MS). As her arm numbness worsened, Sally’s care team worked to determine her specific type of MS and discuss potential treatment options.
First treated with oral prednisone, Sally’s symptoms did not improve, and she experienced non-stop vomiting for 24 hours. She later learned this can be a symptom of a rare disease — neuromyelitis optica spectrum disorder (NMOSD), but at the time, she was still being treated for MS. When the oral prednisone did not alleviate any symptoms, her care team tried IV steroids next, which helped improve the numbness, but the pain continued. Sally’s neurologist pursued further testing — a spinal tap and blood test — to understand why no treatments were successful. These tests showed the presence of aquaporin-4 antibodies, which are associated with NMOSD but not present in patients with MS. About three months after her initial symptoms, she was accurately diagnosed with NMOSD.
“It was scary receiving a diagnosis for a disease I had never heard of,” recalls Sally. “Learning more about the condition and the progressive nature of the disease left me feeling uncertain and worried about how this diagnosis would impact my future.”
NMOSD can place a heavy burden on patients and their families as attacks and disease severity are unpredictable and could lead to permanent disability.
What is NMOSD?
Neuromyelitis Optica Spectrum Disorder (NMOSD) is a rare, debilitating disease of the central nervous system characterized by immune-mediated damage to the optic nerve, brain stem and spinal cord. NMOSD may cause a wide range of symptoms including permanent blindness, muscle weakness, paralysis, pain and fatigue. The variety of ways that NMOSD manifests may cause delays in diagnosing the disease.
NMOSD affects approximately 10,000-15,000 people in the U.S., and the median age of onset is 40 years (onset ranges from ages 3 to 80).[1],[2] The incidence rate may be up to 9 times as high for women as for men, and the prevalence of the disease is around 2-3 times higher in Black and Asian populations.[3],[4]
The journey to wellness
“The numbness and shooting pain I was dealing with was due to inflammation of the spinal cord,” said Sally. “My neurologist explained to me that NMOSD can also cause inflammation of the optic nerve, causing vision problems and eye pain, including sudden blindness. Fortunately, I have never experienced any symptoms in my eyes.”
After her diagnosis, Sally was referred to an NMOSD Center of Excellence and learned about the different treatments available and although there were no FDA-approved treatments at the time, Sally was enrolled in the N-MOmentum clinical trial for Uplizna® (inebilizumab-cdon). Uplizna, which was approved by the FDA in June 2020, is the first and only B-cell depleter approved for the treatment of NMOSD in adult patients who are AQP4 antibody positive.
“My physician explained that Uplizna is designed for patients with NMOSD and works by depleting the specific B cells that are believed to cause the disease,” Sally said. “After learning more about the drug and talking with my physician, it felt like the right course of treatment for me and that it would fit into my lifestyle.”
Since beginning her treatment with Uplizna, Sally hasn’t experienced any side effects, and most importantly, she hasn't had any additional NMOSD relapses. “While I still take certain precautions knowing that I have a suppressed immune system, I have been able to continue doing the things I enjoy,” says Sally.
This story is Sally’s personal experience with Uplizna. The most common side effects of Uplizna include urinary tract infections, joint pain, headaches and back pain, and Uplizna may cause serious side effects, including low blood-cell count. Always discuss with your doctor the right treatment option for you.
How NMOSD differs from MS
As in Sally’s case, NMOSD may be difficult to diagnose, because its wide range of symptoms overlap with other illnesses, especially MS. In fact, 41% of NMOSD patients reported receiving an initial misdiagnosis of MS.[5],[6]
Differences between NMOSD and MS:
NMSOD
MS
NMOSD and MS are treated in different ways, and early detection and treatment help ensure the best outcomes. Differential diagnosis of NMOSD and MS is important, because MS treatments may be ineffective or may exacerbate NMOSD.[7],[8]
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:
Before receiving Uplizna, tell your healthcare provider about all of your medical conditions, including if you:
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:
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:
See “What are the possible side effects of Uplizna?” for more information about side effects.
How will I receive Uplizna?
What are the possible side effects of Uplizna?
Uplizna may cause serious side effects, including:
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 the FDA at 1-800-FDA-1088.
For more information and the full Prescribing Information, visit www.Uplizna.com.
[1] Hamid SHM, et al. What proportion of AQP4-IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients. J Neurol. 2017;64(10):2088–2094.
[2] Mealy MA, Wingerchuk DM, Greenberg BM, Levy M. Epidemiology of Neuromyelitis Optica in the United States. Arch Neurol. 2012;69(9):1176-1180.
[3] Wingerchuk DM. J Neurol Sci. 2009;286(1-2):18-23).
[4] Flanagan EP, et al. Epidemiology of aquaporin-4 autoimmunity and neuromyelitis optica spectrum. Ann Neurol. 2016;79(5):775-783.
[5] Beekman, J, et al. Neuromyelitis optica spectrum disorder: patient experience and quality of life. Neurol Neuroimmunol Neuroinflamm, 2019;6(4).
[6] Neuromyelitis optica spectrum disorder. Retrieved from: https://wearesrna.org/living-with-myelitis/disease-information/neuromyelitis-optica-spectrum-disorder/.
[7] Eaneff S, et al. Patient perspectives on neuromyelitis optica spectrum disorders: Data from the Patients Like Me online community. Mult Scler Relat Disord, 2017;17:116-122.
[8] Wingerchuk DM, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology, 2015;85(2):177-1.
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