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We Pursue Eye Research

Corneal Conditions

Fuchs’ Dystrophy

 Cornea X Section
Figure 1

Fuchs’ dystrophy is a hereditary abnormality of the inner cell layer of the cornea known as the endothelium (Figure 1). The purpose of this layer is to pump fluid out of the cornea, keeping it thin and crystal clear. Figure 2 shows the regular arrangement of this thin cell layer in healthy eyes, while Figures 3 and 4 show how this cell layer becomes distorted as Fuchs’ dystrophy progresses with age.

Normal ECD
Figure 2
Moderate Fuchs ECD
Figure 3
Advanced Fuchs ECD
Figure 4

Fuchs’ dystrophy exhibits dominant inheritance, which means that roughly half of an affected person’s offspring may inherit it.  However, the degree to which the condition is expressed is variable, so some family members may be more severely affected than others.  The Cornea Research Foundation is collaborating with researchers at Case Western Reserve University to determine the genetic basis of Fuchs’ dystrophy.

Fuchs’ dystrophy tends to show up later in life and symptoms are uncommon before the adult years. The initial findings in Fuchs’ dystrophy are corneal guttata, which appear as dark spots on the corneal endothelium. As the condition progresses the critical endothelial cells start to die off. The normal function of these cells is to pump water out of the cornea. As they decrease in number and fail to function properly, fluid builds up in the cornea causing it to swell and become cloudy.

Symptoms and Progression

If you have Fuchs’ dystrophy, one of the first signs may be that you begin to notice glare with headlights at night or in bright sunlight. As the Fuchs’ dystrophy progresses, your vision may become foggy or blurry in the morning and clear as the day progresses. This occurs because evaporation of fluid from the cornea is decreased while your eyes are closed during sleep, and accumulation of fluid in the cornea during the night results in corneal swelling. This early stage can be treated with a special salt solution or ointment to help pull the water out of your cornea. However, as the Fuchs’ dystrophy progresses, your vision will stay blurry longer during the day, and eventually your vision may not clear at all.

Corneal transplants for Fuchs’ dystrophy

A corneal transplant is necessary when vision is diminished sufficiently to hurt your quality of life or if blisters develop on the surface of the cornea and cause pain.  The success rate of cornea transplants is very high for Fuchs’ dystrophy. Results from the Cornea Research Foundation transplant database have shown that 98% of full thickness grafts (penetrating keratoplasty) performed at Price Vision Group remained clear 5 years after surgery. These are among the best results reported in the medical literature.
Dr. Price has helped pioneer a new small incision transplant procedure known as endothelial keratoplasty (or EK for short, with specific variations named DSEK, DSAEK, DMEK and DMAEK). EK is now the preferred surgical treatment for Fuchs’ Dystrophy.  In contrast to a traditional cornea transplant (Figure 5), which replaces the entire corneal thickness, EK spares the healthy portion of the patient’s cornea (Figure 6).  Only the diseased inner cell layer is removed and replaced with healthy donor tissue. This is done through a small incision that may require a few sutures or no sutures at all.

Standard PK
Figure 5
Figure 6

Advantages of EK compared with a traditional full-thickness transplant are: 

  • the eye heals faster and stronger
  • useful vision is recovered in a few weeks or months rather than years
  • minimal activity restrictions or change glasses prescription is required

Dr. Price, the founder and president of the Cornea Research Foundation, was one of the early pioneers of the EK cornea-sparing transplant procedure and has performed over 1600 EK surgeries, more than any other doctor in the world. 

Fuchs’ Dystrophy From A Patient’s Perspective

(excerpted from Celebration of Light, 2006)

Pat Cowan Black and White Portrait

Pat Cowan was wearing orange-feathered Halloween earrings and a brightly patterned Halloween sweater when we met for her interview in October.  A spirited woman, she clearly likes to have fun, and to enjoy life to the fullest. For many years a legal secretary, Pat has transferred her passion for details into her work as a volunteer helping senior citizens make their way through a maze of healthcare and Medicare questions.  Warm and witty, she has a perfect mate in her equally warm and witty husband, Lou, to whom she has been married for 50 years at the time of this writing.

I was six years old when I got my first pair of glasses and they were thick ones.  I don’t remember much about grade school except that I did not want to be assigned a place in the “sight-saving” class.  That seemed too drastic and I didn’t want to go there!  So I told my mother that I just wasn’t going to go to school at all if that happened, and in the end, I got my way and entered a regular class instead.

Looking back, I can recall my eyes tiring easily and having many reading problems in school.  I had a lot of difficulties with depth perception, too; I could hardly see my hands in front of my face.  I also had continuing trouble seeing a ring of “circles” around lights, like red and green traffic lights, the sun, and the moon.  I just thought everyone saw these objects the way I did.  It also seemed that every time I got a new prescription for glasses, I would have to watch where I was going very carefully so I wouldn’t stumble and fall.

My mother had developed macular degeneration as she aged, and because of that I thought I should see an ophthalmologist for eye exams, instead of an optometrist.  The doctor I chose assured me that I did not have the same condition as my mom; but he told me that I had extremely bad astigmatism and cataracts that were starting to form.  From 1989 to 2002, my story is typical of what happens to many people with Fuchs’ Dystrophy.  I went to a different ophthalmologist who did a test for dry eyes but never mentioned Fuchs’ to me.  He advised the use of Preservation drops during the daytime and Preservation night ointment that helped. He never mentioned Muro.

I had several bouts with blisters but never understood what caused them. My doctor never addressed the condition with me, and he just kept insisting that I use the eye drops and the ointment.  Whenever I called his office, it seemed as if I always got the same answer: there were no appointments open and I would have to wait until the next day.  Then the next day would come and my eye would feel a bit better and, since it was always difficult to get time off work, I would decide not to keep the appointment. 

Learning about My Condition
My eyes usually felt like I had sand in them and they often itched.  I later found out that the itching was caused by allergies, something else that was never diagnosed or discussed with me.  Finally, in sheer frustration with what felt like arrogance in my latest doctor combined with the lack of help I was getting, I decided to change doctors again.  I found an optometrist I liked who was recommended by several friends; once again during the exam I was told that I did not have macular degeneration, but I did have cataracts and a lot of astigmatism.

When I had the next attack of blisters, I stopped in my optometrist’s office and he told me to wait because he wanted to examine my eyes.  He gave me some numbing drops and an antibiotic.  He told me that if I had any more trouble with my eyes, he was going to refer me to an ophthalmologist he recommended.  Blisters, by the way, are miserable.  It feels like you have a paper cut in your eye and you just can’t get comfortable no matter how often you blink or what you do.

I was growing tired of living with cataracts and I wanted to do something about them in order to enjoy life more.  I decided to see the ophthalmologist who had been recommended; during the exam I was diagnosed with “Fuchs’ Dystrophy.”  It was the first time I had ever heard the term.  The doctor told me that he would not operate on my cataracts because he was afraid it would cause the Fuchs’ to accelerate.  He advised me to use Muro ointment and that helped.  He also told me that he did not think I was quite ready yet for a transplant. It was November of 2004. 

I went home and got immediately on the Internet to find out all I could about Fuchs’.  I read everything I could find on the topic, and I also joined Fuchs Friends, an online chat group and resource site (http://www.fuchsfriends.com) for people with the condition.

A Disappointment Leads To Hope
I was sure the time had come for me to visit a corneal specialist and, with great hopes, I had my first appointment in January 2005. I explained about the glare, the circles around the lights, the feeling of sand in my eyes, not being able to drive at night, not being able to focus in the morning and needing increasingly stronger magnifying glasses to read. The doctor advised against me having a corneal transplant because he thought I was not “ready” for it. 

I was disappointed but I had done my homework and so I asked him about DLEK. He said “no” to that, too, because he felt it was “too new a procedure.” He mentioned that a physician friend of his was working on a three-year study at the Mayo Clinic and when it was completed, he thought by then he would begin performing DLEK on patients.  He had also just opened a new laser clinic and it seemed to me that he was more interested in that than in helping me.  Neither Lou nor I were positively impressed with him. 

Looking back, this disappointment was actually a good thing because it helped me become really clear about my feelings: I knew I did not want to wait three more years before doing something to improve my vision.  I was ready for a change!  The people in Fuchs Friends had already told me that Dr. Price was the doctor I should be seeing.  I decided to call him for a consultation and it has been one of the best decisions I have ever made.  My appointment was in March 2005.

First Impressions
My first impression of Dr. Price and his team was “They are fantastic!”  Everybody was just so friendly and made me and Lou feel really comfortable.  They were very understanding and I just loved all of them.  I knew I was in the right place and I felt great confidence in Dr. Price.  I had never been given tests for glare or corneal thickness but they were part of the work-up. His staff also did several other tests that were new to me.  The whole atmosphere in the clinic was so supportive, up-to-date and professional. I learned a lot as I went through the process; it opened new doors of information for me and increased my sense of positive anticipation and eagerness to have the surgery.  Finally, after all the doctors I had seen, I had found the right one!

On April 27, I had the first surgery on my right eye to remove the cataracts and have a cornea replacement.  The surgery was a breeze and I had no pain whatsoever.  Dr. Price always makes me feel calm because he is so matter of fact and calm himself.  Dr. Ali, his assistant that day, was great, too, and my husband even took pictures of both of them.  Lou later told me that it was like a coffee klatch in the waiting room while I was having surgery! Other family members were waiting for their loved ones, and everyone talked with one another and shared their feelings and their stories.  We were among kindred spirits.

The Surgery
Let me briefly explain what the surgery was like and what happened.  Many people are afraid of eye surgery but it is actually a comfortable procedure and nothing to be afraid of.  I was given several drops in my eye and then told to wait for awhile before taking me back to the operating suite.  In the prep room, I got into a surgical gown and cap and got an IV of a relaxing medication.  I went into “la la” land.  Lou was with me right up until they administered the anesthetic.  I had an injection to freeze the eye and soon after, I got pretty sleepy.  I had a softening patch put over the eye.  They woke me as they took me into the surgery room, and I was semi-awake from there on.  I could hear Dr. Price’s voice and I was aware of what was going on around me.  I did not feel a thing; no pain and no discomfort at all.  At the end of the surgery, Dr. Price put a contact lens in my eye and then I was wheeled into the recovery room. 

We stayed overnight at a local hotel nearby and I had to lie on my back all night.  I had an early appointment the next day; Dr. Price examined my eye to make sure the air bubble pressing against the cornea was still in place.  We came back the second day for another exam and I was amazed at how well I could see!  Three big E’s on the visual acuity chart and the whole first line!  He released me to go home and wanted me to have follow-up with my optometrist.

I rested quite a bit that first week after surgery, and I also had my first check-up with my doctor.  He monitored my eye drops and started me on PredForte and then removed the contact lens two weeks after surgery.  After four months, my vision was 20/25, the best I have ever seen in that eye since I can’t remember when.

More on the Horizon
I am still not done; I have another surgery scheduled for my left eye for cornea transplant and cataract removal.  I can hardly wait to see the results.  I have told everyone I know that I think Dr. Price is a miracle worker.  I would recommend his practice, Price Vision Group, to anyone and everyone that needs help.  Everyone on his staff is so caring and thorough in their examinations.  You are in good hands with them.

I am at an age to be considered a senior citizen now and I want to travel with my husband, Lou.  By the way, I have decided to say that I am only 29 years old.  After all, age is only a number by which to judge my experience! I celebrate each year as another “anniversary” of turning 29!  (Jack Benny celebrated being 39, but I think women ought to be allowed to be a bit younger.)

When Lou and I go on trips now, I can see all the colors of the trees and the sky. I can see the faces of my grandkids clearly.  I can thread a needle again and have taken up my sewing after not being able to do it for ten years. I can focus my eyes and read the daily newspaper again; it has been so hard to see for such a long time that it’s just great to be alive!

The most important lesson I have learned from regaining my sight is the appreciation of the gift of vision.  It has enabled me to volunteer again for something I love: being able to help senior citizens.  I volunteer for seniors in Illinois helping them with their medical bills and insurance questions.  Some of these people are surviving on such a small amount of money that it makes you wonder how they can live.  I am so glad I can offer help that makes a practical difference in their lives.  Without my sight, I would not be able to do this work.

Advice to Readers
My advice is if you have eye troubles, go for help now!  The longer you wait, the worse it will get.  Do it now!  It will feel like opening a door into a new world. Any discomfort you have will soon be gone and there’s no sense in waiting.  If you get ten years of improved vision from it, isn’t that worth it?

I would also say “reach out” to other people for support.  For those readers who have Fuchs’ Dystrophy, there’s a great group called “Fuchs’ Friends.” This is one of the nicest groups of people you could ever know.  For example, one woman I met there had breast cancer last year and she said later that she could not have gotten through it if it hadn’t been for the support of the people in Fuchs’ Friends.  We are a great collection of people and I would like to directly say “thank you” to everyone in the organization.  Without your website and your knowledge, love and loyal support, I would never have found Dr. Price, my miracle worker.

I’ll close by saying that my inspiration in life comes from God, Lou, my great husband of 50 years, my two dear daughters and five beautiful grandchildren, my caring family and friends and my deep love for the beauty of nature.  My philosophy of life is this: if something can be done, I want to do it.  I know that Dr. Price is the same way and that’s why he keeps finding new solutions and is able to help so many people.