A Guide to Living with NMOSD

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune ailment with inflammatory characteristics, and which affects the central nervous system (CNS). It manifests as optic neuritis, myelitis, and brain lesions. Relapses or attacks occur in patients with this fatal condition at intervals of weeks, months, or years, followed by remissions.

Managing Fatigue and Loss of Vision in NMOSD

Relaxation (or rest) is a crucial component of the overall therapy strategy for individuals with NMOSD, as well as exercise to maintain a sufficient fitness level. NMOSD sufferers often experience fatigue, which has been linked to poor sleep, pain, and sadness. It may be helpful to manage energy utilization while carrying out everyday tasks to lessen weariness. Maintaining energy levels may be achieved by setting priorities and completing the most important activities first thing in the morning. Exhaustion can be minimized by striking a balance between sleeping and doing your daily business.

NMOSD’s visual impairment may greatly impact the quality of life. Patients may be able to continue their daily routine using technology like voice-activated software and tactile devices.

Managing Pain and Diet in NMOSD

The problem of chronic pain in patients with NMOSD may last a lifetime. Although it might start in particular locations on the body, pain typically spreads over the whole body. The mental anguish of the patient might be made worse by persistent pain. Patients try to handle the impact of pain on their everyday tasks with the assistance of a medical team that consists of physiotherapists, pain doctors, nurses, and psychologists.

Managing Weakness

The arms and legs of the patients with NMOSD may become weakened, numb, or paralyzed during an NMO flare-up. Moving, working, or everyday tasks may be more challenging. To assist you in improving your arm and leg function, your doctor can recommend physical or occupational therapy.

Therapists may show you how to utilize aids like canes and walkers. Additionally, they may design specialized physical treatment regimens to strengthen and stabilize problem regions.

If an NMOSD patient wants to feel better, he can do the following things.

Yoga: It reduces pain, keeps you flexible, enhances mood, promotes better sleep, and relaxes the body.

Exercise: It can help overcome bowel and bladder problems, boost energy, helps with depression and fatigue, and promotes better sleep. Before starting any exercise routine, you must consult with your doctor.

Living With Vision Problems

Your spinal cord and optic nerves are most impacted by NMO, also known as Devic’s disease or NMOSD. Your eyes communicate with your brain via optic nerves. NMO damage may result in issues like:

• Loss of vision
• Eye pain
• Blurred vision
• Difficulty in seeing colors in the dark

Muscle Spasticity

NMOSD may sometimes result in spasticity, a painful disease marked by stiffness and muscular spasms. To maintain your flexibility and enhance your mobility, a physical therapist may give you exercises to do, and you need to use splints, a sort of brace.

The treatment of NMOSD-related spasticity can be by:

• Botulinum toxin injections: often called Botox; it relaxes your muscles. The results persist for many months. If just a few of your muscles are spastic, it will work well for you.
• Serial casting:  This treatment holds your ankle, wrist, or elbow joints in positions that gradually increase joint mobility using a succession of casts.

Bowel and Bladder Control

Living with NMOSD is very difficult. One problem is bladder and bowel control. Your bowel and bladder function may suffer if NMO damages your spinal cord. Constipation is common. You may have incontinence (problems regulating your urine and intestines), or urgently need to use the restroom. Your signs may improve if you:

• Do regular exercise
• Consume a fiber-rich diet
• Drink lots of water
• Do not eat citrus fruits, including tomatoes, and acidic foods; also avoid caffeine.

Causes of NMOSD

The clinical condition of NMOSD has been linked to two distinct antibodies. An assault on the aquaporin-4 (AQP4) water channel, found in the optic neurons and spinal cord, is the most frequent cause in the adult population. Proteins called aquaporins (AQPs) move water across cell membranes. An antibody biomarker termed the NMO-IgG is detected in the blood of almost 70% of people with NMOSD (anti-AQP4 antibody). More than half of all NMOSD with pediatric-onset is caused by autoantibodies against myelin oligodendrocyte glycoprotein (MOG), which is present in up to one-third of NMOSD patients who test negative for antiAQP4 antibodies.

Treatment of NMOSD with SOLIRIS

The FDA-approved drug for individuals with neuromyelitis optica spectrum disorder (NMOSD) is SOLIRIS (eculizumab). The patients of NMOSD are anti-aquaporin-4 (AQP4) antibody-sensitive. Data about the safety of SOLIRIS is not available for children with NMOSD.

Mechanism of action of SOLIRIS

In contrast to conventional multiple sclerosis lesions, NMOSD lesions exhibit substantial complement activation, eosinophilic infiltration, and vascular fibrosis. The primary humoral immune system-mediated attack on AQP4 is the pathophysiology of NMOSD. A humanized monoclonal antibody called eculizumab blocks the terminal complement protein C5 from cleaving into C5a and C5b. Although the precise mechanism for its effectiveness in NMOSD is unknown, it is thought to be connected to the prevention of the membrane assault complex’s development, which is mediated by C5b.

The Clinical Indication for SOLIRIS

Eculizumab, or Soliris, was the first medication approved by the FDA for treating adults with anti-aquaporin-4 (AQP4) antibody-positive NMOSD.

Previously, the FDA had approved Soliris for the treatment of the following conditions.

• Paroxysmal nocturnal hemoglobinuria (PNH)
• Atypical hemolytic syndrome (aHUS)
• Anti-acetylcholine receptor antibody-positive generalized myasthenia gravis (gMG)

Administration and Dosage for SOLIRIS

It is advised to administer SOLIRIS intravenously once per week for the first four weeks, followed by the fifth dose one week later and then every two weeks.

Common Side Effects of SOLIRIS

Severe allergic reaction from SOLIRIS is the most common side effect.