Neurotrophic Keratitis

Overview

What is neurotrophic keratitis?

Neurotrophic keratitis is a rare eye disorder that affects the cornea. The cornea is the clear (transparent) outer layer of the eye. It functions like a window that focuses the entry of light into the eye. The cornea bends light, allowing it to pass through the pupil, eventually reaching the retina where specialized cells called cones and rods convert light to nerve impulses. These impulses travel along the optic nerve to the brain to become images. Neurotrophic keratitis is caused when the nerves that serve (innervate) the cornea cannot function properly. Neurotrophic keratitis causes reduced sensitivity of the cornea. When the cornea senses stimulation or pressure, the eyelid will close and tears will be produced to protect the cornea and the eye. When these nerves do not function properly, the outer layer of the cornea, called the epithelium, can break down, resulting in an epithelial defect. In more advanced neurotrophic keratitis, an interior layer called the cornea stroma can break down as well, resulting in thinning of the cornea. This is called stromal ‘melting’. In advanced stromal melting, the cornea can thin to a severe degree, which can result in a hole or opening to the inside of the eye. This can lead to infection and potentially loss of the eye. Neurotrophic keratitis can lead to a variety of complications, including poor wound healing of the cornea, scarring of the cornea, and loss of vision.

How common is neurotrophic keratitis?

Neurotrophic keratitis is a rare disorder with prevalence at approximately less than 5 people per every 10,000 in the general population.


Questions regarding your neurotrophic keratitis therapy?

You can reach the Accredo neurotrophic keratitis care team, anytime, day or night, seven days a week by phone or online.

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Call 1-877-831-8112

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Symptoms

What are the symptoms of neurotrophic keratitis?

Early on, affected individuals may not have any symptoms of the disorder (asymptomatic). Because the sensitivity of the cornea is reduced, affected individuals often do not complain of pain or discomfort in the eye. Blurred vision, red eyes, dry eyes, and decreased clarity (acuity) of vision can develop. Affected individuals may become extremely sensitive to light (photophobia). The affected cornea can be easily injured and heals slower than an unaffected cornea does (poor wound healing). This leaves the cornea susceptible to damage. Affected individuals may experience frequent or recurrent corneal erosions, which is when an opening in the epithelial layer develops (epithelial defect). Since the epithelial layer is the protective layer of the cornea, an epithelial defect can leave the eye more susceptible to infection.

More serious complications can develop, including irregular astigmatism, a condition causing blurred vision that results when the cornea becomes irregularly shaped or abnormally curved. Affected individuals may develop scarring of the cornea. As the inner layer, or stroma, breaks down and becomes thinner, the cornea can become even more irregularly shaped, and more severe scarring can develop. In the most severe instances, the cornea can perforate, resulting in loss of the inner fluid of the eye. Neurotrophic keratitis can lead to permanent loss of vision, if not properly treated.

How is it diagnosed?

A diagnosis of neurotrophic keratitis is based upon identification of characteristic symptoms, a detailed patient and family history, a thorough clinical evaluation, and a variety of specialized tests. A diagnosis should be suspected in individuals with unexplained signs of reduced corneal sensitivity and damage to the cornea.

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Medications

The following specialty medications are available at Accredo, a specialty pharmacy for neurotrophic keratitis

Medication Manufacturer
Oxervate™ (cenegermin-bkbj) Dompe

Financing Your Care

Financial assistance may be available to help with your medication costs, including manufacturer and community programs. Accredo specialists are available to help find a program that may work for you.

Financial Guidance

Life-saving specialty medication can be expensive. Learn how the Accredo teams help individuals find ways to afford the medication they need to survive in this video.

Find Support

Neurotrophic Keratitis Organizations

There are many organizations that support research and advocacy for <neurotrophic keratitis>. See below for a few of those organizations.

Support Organizations

Meet The Team

Accredo’s neurotrophic keratitis care team is dedicated to serving you and we understand the complexity of your condition. Our expert clinicians are available 24 hours a day, seven days a week to answer any questions.

Why We Do It video transcript

24-hour Customer Service Center

Call 1-877-831-8112