Fuchs' (fooks) dystrophy affects the cornea — the clear front window of your eye. This disorder causes swelling in the cornea that can lead to glare, cloudy vision and eye discomfort.

Fuchs' dystrophy usually affects both eyes and can cause your vision to gradually worsen over many years. But most people with Fuchs' dystrophy have a mild type and don't notice much change in their eyesight.

Some medications and self-care steps may help relieve your Fuchs' dystrophy signs and symptoms. But when the disorder is advanced and you've lost vision, the only way to restore vision is with cornea transplant surgery.

As the disease progresses, Fuchs' dystrophy symptoms usually affect both eyes and may include:

  • Glare, which is an early symptom and reduces contrast perception or affects vision in low light.
  • Blurred vision, which occurs in the morning after awakening and gradually improves during the day. As the disease progresses, vision can take longer to improve or may not improve.
  • Distorted vision, sensitivity to light, difficulty seeing at night and seeing halos around light
  • Painful, tiny blisters on the surface of your cornea
  • A cornea that looks cloudy or hazy

When your ophthalmologist examines your cornea, he or she looks for:

  • Irregular bumps on the back surface of the cornea (guttae)
  • Corneal swelling
  • Corneal haze
  • Thickening of the cornea, measured by a special instrument (typically an ultrasound)

When to see a doctor

If you experience some of these signs and symptoms, and especially if they get worse over time, see an eye specialist (ophthalmologist). If symptoms develop suddenly, call an ophthalmologist for an urgent appointment. Other eye conditions that cause the same symptoms as Fuchs' dystrophy also require prompt treatment.

Normally, the cells lining the inside of the cornea (endothelial cells) help maintain a healthy balance of fluids within the cornea. Healthy endothelial cells prevent the cornea from swelling and help keep the cornea clear. But with Fuchs' dystrophy, the endothelial cells slowly die off, and probably do not function correctly, resulting in fluid buildup within the cornea (swelling or edema). This causes corneal thickening and blurred vision.

Fuchs' dystrophy can be inherited. The genetic basis of the disease is complex — family members can be affected to very variable degrees, and sometimes not at all.

Factors that increase your risk of developing Fuchs' dystrophy include:

  • Being female, as Fuchs' dystrophy is slightly more common in women than in men
  • Having a family history of Fuchs' dystrophy
  • Being over 50

If you suspect you have Fuchs' dystrophy, make an appointment to see an eye specialist (ophthalmologist). In some cases, you may be referred to an ophthalmologist who specializes in corneal disease.

Here's some information to help you get ready for your appointment.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, ask if you need to do anything in advance.
  • List symptoms you're experiencing. Record when you first noticed them and what situations make them worse.
  • List key personal information, including any family history of eye conditions.
  • List all medications, vitamins and supplements you're taking.
  • Consider taking along a family member or a friend who drives. You may not want to drive yourself home if your pupils have been dilated for the exam. And your companion can help remember information provided during your appointment.
  • List questions to ask your doctor.

Your time with your doctor may be limited, so preparing questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For Fuchs' dystrophy, some basic questions to ask your doctor include:

  • Are there other possible causes?
  • What kinds of tests do I need? Do I need to prepare for these tests ahead of time?
  • Is my condition likely temporary or ongoing?
  • What treatments are available, and what do you recommend?
  • How rapidly will my condition progress?
  • How will my vision be affected?
  • I have these other health conditions. How can I best manage these conditions together?
  • Do I need to restrict my activities in any way?
  • Do you have brochures or other printed material I can take home? What websites do you recommend?

Don't hesitate to ask any additional questions that occur to you during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions, such as:

  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Do your symptoms change throughout the day?
  • Do you have a family member with Fuchs' dystrophy?
  • Have you noticed any changes in your vision?
  • If so, does your vision seem to be worse in the morning and improve during the day?

You may undergo tests such as the following so that your doctor can determine whether you have Fuchs' dystrophy. These tests will also measure the shape, clarity and thickness of the cornea.

  • Visual test. You'll be asked to read letters and numbers on a chart to measure how well you see at various distances. This is called a visual acuity test and is a routine part of eye exams.
  • Staging. Your doctor will try to determine the stage of your condition by examining your eye with an optical microscope (slit lamp). He or she will then study the cells lining the back surface of your cornea (endothelial cells). If you have irregular bumps (guttae) on the back surface of the cornea, you may have Fuchs' dystrophy.
  • Corneal pressure test. Your doctor may numb your eyes with drops and then briefly touch your eye with a special instrument that measures eye pressure.
  • Corneal thickness. Your doctor may use a special instrument to measure the thickness of the cornea.
  • Corneal cell count. Sometimes your doctor might use another special instrument to record the number, shape and size of the cells that line the back of the cornea.

Some nonsurgical treatments and self-care strategies may help relieve your Fuchs' dystrophy signs and symptoms. If you have severe disease, your doctor may suggest surgery.

Medications and other therapies

  • Eye medication. Your doctor may prescribe eyedrops or ointments to help reduce the amount of fluid in your cornea.
  • Soft contact lenses. Your doctor may suggest you wear soft contact lenses. The contacts act as a "bandage" to relieve pain by smoothing out microscopic swelling on the surface of the eye.

Surgery

Most people who have surgery for advanced Fuchs' dystrophy experience much better vision and remain symptom-free for years afterward. Surgical options include:

  • Replacing the inner layer of the cornea. This is the first-choice option for people who have decided on a surgical solution. Two techniques your doctor may consider for you are Descemet stripping endothelial keratoplasty (DSEK) and Descemet membrane endothelial keratoplasty (DMEK). Only the back of the cornea is replaced with healthy tissue from a donor. These procedures are usually done with local anesthesia in an outpatient setting.
  • Transplanting the entire cornea. This surgical procedure, known as penetrating keratoplasty, replaces the full-thickness cornea with a healthy one from a donor. This procedure is not used much anymore for Fuchs’ dystrophy. But it might still be the best choice in some cases.

Experimental treatments

Clinical trials involve studies of new ways to diagnose or treat a condition. They don't always offer a definite diagnosis or cure. But they may allow you to benefit from the latest knowledge about Fuchs' dystrophy. Ask your doctor if you're eligible for any clinical trials.

In addition to following your doctor's instructions for care, you can try these techniques to help reduce glare or make your eyes feel better.

  • Apply over-the-counter (nonprescription) salt solution (5 percent sodium chloride) eyedrops or ointment.
  • Wear wraparound sunglasses with ultraviolet protection.
  • Dry your eyes with a hair dryer. Hold it at arm's length and direct warm — not hot — air across your face two or three times a day. This helps remove excess fluid in the cornea and dry out microscopic swelling and blisters on the surface of the cornea.

Remember that no matter what your concerns or prognosis, you're not alone. It may help to reach out to professional counselors or others who are living with this condition.

Jul. 22, 2014