Living With Fuch's Dystrophy

Understanding treatment decisions.. Ariel Skelley

Most of us are familiar with the most common eye problems - glaucoma, cataracts, dry eye syndrome and macular degeneration. Information concerning these conditions seems to be easy to obtain. However, Fuch’s Dystrophy is a less common eye affliction and those affected by it usually leave the doctor’s office with several unanswered questions. Educating yourself about the condition can be a challenge.

If you or someone you know has been diagnosed with Fuch's Dystrophy, the following will help you become more informed.

What is Fuch’s Dystrophy?

Fuch’s Dystrophy is an inherited eye condition that causes a disorder in the cornea, the clear dome-like structure on the front part of our eye. The cornea is composed of six layers of cells with endothelial cells being the last layer on the back side of the cornea. One of the functions of the endothelial cells is to continually pump the fluid out of the cornea, keeping it compact and clear. When these cells start to fail, fluid builds up in the cornea causing stress on the cells. The cornea swells and vision becomes cloudy. In severe forms of Fuch’s Dystrophy, the cornea can decompensate.

Will a diagnosis of Fuch’s Dystrophy profoundly affect my life?

The severity of Fuch’s Dystrophy can vary. Many patients do not even know they have the condition while others can become frustrated with decreased vision.

However, most patients maintain a good level of vision to function very well with daily activities.

Will Fuch's Dystrophy cause me to go blind?

With today’s technology, blindness is almost non-existent for patients suffering from severe Fuch’s Dystrophy. It is important to understand that Fuch’s Dystrophy does not affect the retina, the light sensitive receptor layer of the cornea or the optic nerve, the nerve cable that connects the eye to the brain. The cornea gives the eye  most of its refractive power. In severe forms of the disease, a corneal transplant or a new procedure called DSEK can restore near normal function to the cornea.

What kind of symptoms might I experience with Fuch's Dystrophy?

Some patients complain of blurred vision in the morning that seems to get a little better as the day continues.

This is because fluid builds up in the cornea overnight. As you wake up and go about your day, the eye is open to the environment and the fluid actually evaporates out of the cornea and vision tends to get better. You can also experience seeing rainbows or halos around lights, glare, and your vision could appear foggy. Some patients complain of eye pain or a foreign body sensation in their eyes.

Will treatment affect my daily lifestyle?

Treatment of Fuch’s Dystrophy in the early stages is quite simple. Usually, it involves instilling a 5% sodium chloride solution or ointment into the eyes to draw out the fluid. The 5% sodium chloride is a salt based compound that is usually instilled two to four times per day. Some patients get better results by using the ointment formulation that they only put into their eye at night.

What happens if my Fuch's Dystrophy gets worse?

Some patients never develop a severe form of Fuch’s Dystrophy.  However, if it advances to a more severe stage, you may develop bullous keratopathy.

This is where fluid filled bullae or blisters form and make vision blurry and can erupt and cause significant eye pain and foreign body sensation. In this case, some doctors will place a bandage contact lens on your eye and prescribe medicated eye drops. If the condition worsens, your doctor may recommend a corneal transplant or a DSAEK procedure. DSAEK (Descemet’s Stripping Automated Endothelial Keratoplasty) is a procedure in which only the back part of the cornea is replaced with healthy endothelial cells. A DSAEK procedure has less complications than a total corneal transplant and the resulting vision is much better.

Will my children inherit Fuch's Dystrophy?

Some cases of Fuch’s Dystrophy seem to have no genetic pattern. However, most cases have what is known as an autosomal dominant inheritance pattern. This means that if you have the condition, and one of your parents had the condition, each child has a 50% chance of having Fuch’s Dystrophy.

SOURCE:  Afshari, Natalie A. MD; Aaron B. Pittard, MD; Adnan Siddiqui, BS; Gordon K. Klintworth, MD, PhD. Study of Fuchs Corneal Endothelial Dystrophy Leading to Penetrating Keratoplasty, Arch Ophthalmol. 2006. 

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