Fuchs' Dystrophy - A Rare, Progressive Cornea Disease
If you haven't heard of Fuchs' Dystrophy before you are not alone. Since this eye disease is rare, many people have never heard of it. It is a rare, progressive disease that can lead to blindness. Less than 1% of the United State's population suffers from this disease.
What is Fuchs' Dystrophy?
Fuchs' Dystrophy or Fuchs' Corneal Dystrophy is a rare disease that slowly destroys the cornea over time. This disease affects the cells in your cornea, specifically your endothelial cells. These cells compromise a single layer of cells on the inner surface of the cornea. The corneal endothelium is responsible for managing fluid transport across the eye and helps to maintain the fluid balance in the cornea and in the eye necessary for proper vision.
Fuchs' Dystrophy destroys these endothelial cells gradually and without any apparent reason. As more and more cells are destroyed over time the endothelium becomes less able to transport fluids out of the eye which in turn causes the cornea to swell. This swelling starts to cause distortions in vision. As the disease continues to progress the eye starts to take on more and more water. This can be very painful and can result in extensive vision loss. As the cornea swells tiny blisters can develop which can be extremely painful.
This disease is a slowly progressing disease. At first suffers notice that their vision is impaired in the morning and that it gradually improves throughout the day. This is due to the fact that the eye retains more fluids during sleep that slowly dissipate throughout the day. As the disease progresses this vision loss and fluid retention will become constant.
In many cases this disease is genetic although some people with the disease do not have a family history of the condition. It is more common in women than in men and is generally not diagnosed until people are in their 50s or 60s. If a parent has the disease their children all have a 50% chance of developing the condition as well.
What makes the disease worse?
Fuchs' Dystrophy will progress with time and with age. There is nothing that can be done to stop the progression of this disease. Treatments administered in the early stages control the symptoms of the disease only and do nothing to slow the progression of this disease. However, many patients with Fuchs' dystrophy notice that the disease can dramatically worsen after cataract removal surgery especially if the dystrophy was previously mild. If you have Fuchs' Dystrophy it is important to weigh the pros and cons of cataract removal with your eye doctor to minimize your risk.
What are the treatment options?
The treatment plan for this disease depends on the stage of the disease. In the early stages treatment is typically focused on controlling the symptoms. Often eye drops and ointments are used to reduce the swelling in the cornea by removing excess fluids from the eye. If sores or blisters develop on the cornea soft contacts may be used or surgery may be performed to reduce the pain.
As the disease progresses a cornea transplant is often the only way to retain vision. In fact Fuchs' Dystrophy is one of the leading causes of U.S. cornea transplants. A corneal transplant is the only way to cure this disease. Cornea transplants have been a very effective cure for Fuchs' Dystrophy, especially in the short term, but some studies do suggest that the new cornea may have problems over the course of many years.
Another treatment option for those suffering from Fuchs' Dystrophy is known as Deep Lamellar Keratoplasty or DLK. This is an alternative treatment that may be used in place of a cornea transplant. During a DLK procedure only the deep layers of the cornea are replaced with donor tissue rather than the full cornea. DLK requires no stiches and has a much shorter recovery time than a traditional transplant. There are also fewer complications like rejection for patients that undergo DLK over a traditional cornea transplant.
Fuchs' Dystrophy is a rare disease that destroys cornea cells and can eventually lead to severe vision loss. While this disease is not preventable, it can be cured with a cornea transplant and many of the symptoms can be controlled in the early stages.
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