The Future of Fuchs' Dystrophy

What are the first symptoms of Fuchs’ dystrophy? Are patients tested for it at routine eye exams?

Early symptoms include blurry vision or glare that is often worse in the mornings and improves as the day goes on. Ophthalmologists and optometrists can look for Fuchs’ dystrophy during routine eye exams using the slit lamp microscope. We can perform more specialized tests for patients diagnosed with Fuchs’ to monitor progression, including specular microscopy, which uses specialized microscope photos of the endothelium cell layer of the cornea, and pachymetry, which is a test of corneal thickness.

What is the typical course of the disease?

It can be quite variable. With mild forms of Fuchs’ dystrophy, patients have only minimal visual symptoms and do not need treatment. However, some patients develop worsening vision, glare, or halos that can affect quality of life and require therapy such as medication or surgery. Fuchs’ dystrophy can also become progressively more severe, resulting in corneal blindness or eye pain; however, modern treatments prevent these problems in the vast majority of cases.

Who is most at risk for developing Fuchs’ dystrophy?

Fuchs’ is a progressive disease, and the age of onset typically occurs between age 40 and 50. It is a genetic eye condition, affecting about 4% of Americans over age 40. Women are three times more likely than men to develop Fuchs’ dystrophy.

How has Fuchs’ dystrophy been treated in the past and what new treatments are being offered at the Sue Anschutz-Rodgers Eye Center?

Historically, early in the disease we prescribed sodium chloride eye drops until vision was heavily impaired, at which point a full-thickness corneal transplant, or penetrating keroplasty (PKP), was performed. We still use eye drops early in the disease, but we now utilize better surgical options that have a lower risk of rejection and provide better visual outcomes than corneal transplant surgery.

Descemet’s stripping automated endothelial keratoplasty (DSAEK) and Descemet’s membrane endothelial keratoplasty (DMEK) are partial-thickness corneal transplantation procedures that have been successful in leading to positive visual outcomes. Another new procedure, Descemet’s stripping only (DSO), is the stripping of an inner membrane called Descemet’s membrane and allows for improved visual outcomes in some patients without requiring corneal transplantation.

What does effective treatment mean for patients with Fuchs’ dystrophy?

Effective treatment can improve visual acuity, contrast sensitivity, glare symptoms, and eye pain,and restore vision-related quality of life for patients with Fuchs’ dystrophy. We can do this more successfully now than ever before.

What does the future of Fuchs’ treatment look like?

I think the future of Fuchs’ treatment is bright! Globally, treatment of Fuchs’ dystrophy and other disorders of corneal endothelial cell function is an area of great interest for researchers. There are several clinical trials across the U.S. involving newer eye drops, such as Rho-kinase inhibitors to help with endothelial cell propagation, used in conjunction with surgery to provide more rapid and complete healing. Additionally, there is a lot of work going on to evaluate the safety and effectiveness of injection of donor cells into the eye, avoiding the need to transplant tissue.

With time, I suspect that we will move away from corneal transplantation for this disease and shift toward newer medications that can facilitate healthy endothelial cell regeneration as well as injection of cultured endothelial cells into the eye. These advances, while still in development in the research community globally, will allow patients access to treatment at an earlier stage of disease and in an even safer manner.