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The Cornea Research Foundation is actively involved in evaluating a new corneal strengthening treatment that may help patients with keratoconus.
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What is Keratoconus?
Keratoconus (KCN) is a disease characterized by thinning and protrusion of the cornea, resulting in an irregular, conical shape (Figure 1 and 2). Irregular astigmatism occurs as the keratoconus progresses, and results in blurred vision, which can be impossible to correct with spectacles. Usually keratoconus occurs in both eyes, and involves the central cornea with the apex of the cone just below the visual axis.
Approximately 100 to 200 of every 100,000 people are afflicted with keratoconus and it occurs in all races and usually affects both eyes. Sensitive techniques, such as corneal topography, often detect keratoconus in both eyes in cases thought to be only in one eye based on physical examination and refraction. Keratoconus often starts in puberty and slowly progresses over decades and then stabilizes. In progressive cases, severe irregular astigmatism and scarring may require a corneal transplant in order to restore useful vision to the eye.
Heredity of keratoconus has not been clearly established. While there are families in which multiple persons are affected, factors such as allergic conditions and contact lens use make analysis difficult. As a general rule, chances of a blood relative developing clinical keratoconus are less than 10%.
Conditions associated with keratoconus include atopic disease (atopic or allergic dermatitis, allergic rhinitis, asthma), Down’s syndrome (5-8 % of Down’s patients), and connective tissue disorders (Ehlers-Danlos syndrome and osteogenesis imperfecta). Chronic eye rubbing is associated with keratoconus and may accelerate progression. Eye rubbing may be the common link between keratoconus and allergies, atopic disease and Down’s syndrome.
Findings in keratoconus include protrusion of the cornea, striae or wrinkles of the posterior cornea (Vogt’s striae), superficial scarring of the anterior cornea, and staining of the corneal surface epithelium with iron (Fleischer ring). Corneal hydrops, or marked swelling of the cornea in keratoconus, may occur when severe bulging of the cornea results in a tear in the deepest layer of the cornea (Descemet’s membrane), allowing fluid from the inside of the eye to permeate the cornea. Severe haziness, often accompanied by blister like lesions of the superficial cornea, results in impairment of vision and discomfort.
Treatment of keratoconus can be divided into three tiers. For patients whose vision can be adequately corrected with spectacles, no other treatment is necessary. However, these patients should be very careful to never rub their eyes, because that could make the keratoconus worse. Control of systemic allergies in keratoconus patients with antihistamines may help achieve this goal by decreasing itching of the eye and eyelids. However, systemic antihistamines cause the eyes to become drier with less tear production, which can actually cause an increase in eye itching with allergies. Therefore we recommend using eye drops to control itching, instead of systemic antihistamines, in keratoconus patients.
Corneal Cross-linking for Keratoconus
The Cornea Research Foundation of America is conducting studies of corneal collagen cross-linking (CXL) in subjects with progressive keratoconus or with corneal ectasia after prior refractive surgery. These studies are designed to assess the safety and effectiveness of CXL for halting or slowing the progression of these conditions.
Corneal collagen cross-linking involves administering riboflavin (vitamin B2) eye drops and UVA light in carefully selected parameters that strengthen the front layers of the cornea (clear covering of the eye) and avoid damage to the back part of the eye. The riboflavin and UVA light source that are used for CXL are investigational in the United States. If you qualify and decide to participate in the study, you will receive the riboflavin and UVA light at no cost. However, you will need to pay a fee for the examinations. Most insurance companies will not cover the cost, because the procedure is investigational in the USA.
Eligible For The Study?
You may be eligible if you have the following in one or both of your eyes or are:
10 years of age or older
Have been diagnosed with progressive keratoconus or have had previous vision correction surgery and now have corneal ectasia
Corneal thickness greater than 300 microns at the thinnest point
If you are female, you cannot be pregnant
Can leave your contact lens out for at least 6 weeks in the eye to be treated
If you have keratoconus, it must have gotten worse in the last three years based on your previous eye exams. You will need to contact your eye doctor or allow us to contact your eye doctor to get your eye exam records for your vision measurements, corneal maps (topographies) and contact lens or glasses prescriptions
Contact Lenses for Keratoconus
When irregular astigmatism makes clear vision with spectacles impossible, rigid gas permeable (RGP) contact lenses are the next stage of therapy. The rigid lens masks the underlying irregular cornea and functions as the new refractive surface of the eye, with the tear film filling in the space between the back of the contact lens and the front of the eye. However, in keratoconus the steep cone and irregular shape of the cornea makes fitting these lenses challenging, and the experience and expertise of the contact lens fitter is very important in determining the success of this intervention. Central corneal scars can also develop in keratoconus eyes. Usually this is caused by the contact lens rubbing on resting on the apex of the keratoconus cone. Proper fitting of the lens is very important to prevent or minimize scarring. When corneal scars in keratoconus limit vision or the ability to wear the contact lens, the scars can often be removed surgically without the need for a transplant.
Intacs for Keratoconus
If keratoconus progresses to the point where a contact lens cannot be fit or does not adequately correct vision, surgery may be indicated. Small plastic ring segments placed in the cornea can produce a more regular corneal surface in about 2 out of 3 keratoconus patients.
Cornea Transplants for Keratoconus
Historically, in about 1 out of 5 patients, keratoconus progresses to the point where a cornea transplant is needed to restore a more normal shape to the cornea. Data from the CRFA database of corneal transplant shows that transplants performed for keratoconus are in the highest category for successful outcomes. In most cases, Dr. Price recommends the newer targeted transplant technique known as deep anterior lamellar keratoplasty (DALK) to help minimize the risk of graft rejection.
Keratoconus Support Group
For more information about keratoconus or to participate in a keratoconus chat group, you may wish to visit the National Keratoconus Foundation website at: http://www.nkcf.org.
Keratoconus from a Patient’s Perspective
(Excerpted from Celebration of Light, 2006)
Pastor Clarence Moore has a warm and ready smile that immediately put me at ease when we met in his comfortable office at the Northside New Era Baptist Church. His lifework is an inner city ministry that he relishes as his life’s calling. A humorous man, Pastor Moore enjoys speaking in a playful manner to elicit a grin or a giggle. Equally eloquent at other times, he inspires listeners to strive to find their life purpose in order to serve the world and to serve God.
My vision challenges began when I was in my early twenties and working at General Motors in the purchasing department. One day a co-worker came to my cubicle and said, “Clarence, do you realize how close you are holding that paper to your face?” It dawned on me then that I had gradually adjusted to a significant loss of sight and it shocked me to learn how much accommodation I had made just to carry out my work. Not long after, an incident happened in a softball game that was equally sobering. A normal ground ball came towards me and hit me right in the forehead. My wife asked me later, “How did you let that happen? Did you not see that ball?” I had to admit to her that my eyesight was diminishing. I had not fully realized it myself until then. I should also mention that my Mother has been legally blind all her life. She had two detached retinas and, when I admitted my failing sight, I wasn’t sure but that I might be headed down that same road.
At the time I was supervising a good Christian lady who wore thick Coke-bottle glasses. Peggy and I became good friends and one day she came to work without her glasses and I was startled. I asked her where they were and how was she able to see without them. She said, “Oh, I have to tell you about the miracle. I had laser surgery with a young doctor in Indianapolis named Dr. Francis Price.” I had never heard of laser surgery and she was so enthusiastic that it made a strong impression on me. Being free of those thick glasses made a positive difference in her self-confidence and her appearance.
Time to Get Help
I couldn’t see well enough to drive at night although it was hard to admit it. One frightful night, I picked up my two small children from the babysitter and I simply could not get home. I could not turn left or right or read the road signs. I still remember vividly how frightening that night was. I had to stop and call my wife to tell her what was happening. I made it home, finally, but only because God was looking out for us. On another occasion, I drove some friends home and hit two or three mailboxes on the way and scared us half to death.
A few years later I accepted my calling into the ministry. By then, I was seeing so badly I could not drive myself to seminary classes and so my wife had to provide transportation. My eyesight was very blurry, and I had to enlarge things in print in order to be able to read them so I studied by listening to my wife read chapters to me. She would read and then tell me what she thought were the key points and we would discuss them. You know, even to this day, when I see colleagues I went to school with they give credit to my wife, Hope, for my A’s in seminary. They would overhear her talking to me in the halls between classes and they thought she was my “secret weapon” for academic success. In keeping with her character, she always defended me by pointing out that I had a great memory and that’s how I got such good grades.
When I graduated seminary and became a minister, I could not see my Bible or my sermon notes. I couldn’t go back and forth between the two to find the right phrases as I preached. As a result, I was very limited and ineffective; good, passionate preaching requires the ability to stay in the flow as you inspire people. I felt my limitations were not what God wanted. There had to be a better way and I decided that it was time to do something about my sight.
My optometrist had treated me for keratoconus and astigmatism and referred me to an ophthalmologist in Kokomo, Indiana so I started there. After the exam, I told him I wanted a second opinion and I asked him to recommend someone. He responded that he’d like to handle my condition himself and told me that I needed a corneal transplant. After he explained that he had just started doing these procedures, I thought it over and got a referral from him. It was Dr. Francis W. Price, Jr. in Indianapolis.
Probabilities in my Favor
I came to Indianapolis to meet Dr. Price. He was a young man around my own age but he seemed very knowledgeable and he had experience with all kinds of things that impacted the cornea. I wanted to put the probabilities in my favor; I thought my chances of improvement were better with Dr. Price even though having his care and surgery would mean traveling to Indianapolis.
Dr. Price was an impressive doctor. He first tried hard contact lenses on me for about three months but I couldn’t wear them. He explained how the football shape of my eye differed from the baseball shape of a normal eye. The lenses irritated my eyes and he said, “Well, we have no other choice but this transplant procedure.” I was scared to death, of course, but I knew that he was skillful. His communication of my condition to my wife and me was outstanding. I was encouraged by his prognosis. The other thing that impressed me was sitting in the lobby and realizing how many people came from all over the Midwest to see him. I had this thought, “All these people can’t be wrong.” Especially my friend Peggy who was so high on Dr. Price; I had great trust in her judgement.
I remember an early conversation between Dr. Price and I about the transplant; he said to me, “We are going to get you the best cornea in the world.” I jokingly replied, “Well if I have to have a new cornea, make sure it’s blue.” He looked at me funny and asked, “Excuse me?” I did not know then that corneas are clear without any color, and so I explained further, “Well if you have blue eyes, you get promoted. I may have the wrong color skin, but with blue eyes, I might have half a chance of getting promoted.” At that, we both laughed together but he had the last word and said, “Well I can’t help you with a blue one; you’re stuck with brown eyes!”
It was the year 2000 and I had my left cornea, my “worst” eye, replaced with a transplant. During surgery, I was put to sleep and when I woke up, I had no pain in my eye at all. One admonition I remembering receiving before being discharged was not to sneeze and I can remember feeling I wanted to sneeze so badly, but I would not let it happen. (It is hard to hold back a sneeze for hours, I can tell you!) Twenty-four hours later, Dr. Price took my bandages off. I looked at him with awe and said, “Wow, I can see you really well.” I know that God used him to work a miracle. He had changed my world, but he didn’t want any accolades. I was ready to shout it from the rooftops but he was calm and just patted me on the shoulder, saying, “You did well.”
When I got home that day, I could see my dear little children’s’ faces just as clearly as I had seen Dr. Price and it was a moment I will never forget. It makes me emotional even now just remembering it. And I could also see my lovely wife’s face clearly, too. What gifts from God!
My recuperation took 3-4 months and I was off work during that whole time. I had been assigned to work in a chemical area and I really didn’t want to be in that environment while I was healing. Neither Dr. Price nor I thought it a good idea for me to return to work under those circumstances. I also could not do any heavy lifting or even bend over to tie my shoes. Other than that, I just rested and took life a bit more slowly than usual. When it was over, I was able to drive again, even at night, and I had no more blurriness. I could enjoy reading stories to my children. I could go to soccer games and actually see my child score, rather than just hear about it from another parent seated nearby.
In 2004, I had a corneal transplant in my right eye. Dr. Price predicted that it would do better than my first transplant because, in the interim, he had gained even more experience and the technology had gotten better. My left eye had become my dominant eye and the right eye had become dependent. With correction in both eyes my overall vision would be much better. Dr. Price did two relaxing incisions after the transplant to improve my vision as much as possible and I had LASIK twice. My vision became 20/30 – another of God’s miracles.
How Life Has Changed
The miracle of sight allows this preacher to “…rightly divide the word of Truth…” as I preach the Gospel. The sight that has come through the hands of Dr. Price allows me to not just function as an average pastor, but my sightedness has allowed me to use all of my gifts with greater confidence. I owe my ministerial work to him and his staff. God used each of them to bring miracles into our lives.
I have been able to take a trip to Africa with my family, for example, and I was able to see all the sights and experience the people, the land, etc. I was able to travel to Greece this summer, too, and all of these things have come because I have been given the miracle of being in Dr. Price’s care. He has literally opened the world up to me.
My children are now grown; Curtis is the oldest and he’s at medical school, Courtney is a teacher and Cristen is a writer. I am proud of them and sharing life with them as a sighted parent is one of the many “residual blessings” God has given me with my sight.
I believe Dr. Price’s work goes far beyond him, and I hope he realizes this. God is using him to touch lives and work miracles. In essence, Dr. Price is actually giving a new life to each of his patients and there’s no way of gauging how far that life reaches and how many other lives will be impacted.
I think God used vision failure as a tool to reorder my steps. I had been active in my church but I didn’t have ministry on my mind. He used my lack of sight to really talk with me and get my attention. There’s a similar story in the Bible about Paul on the Damascus Road who was blind. God sent Annais to uncover the scales from Paul’s eyes and restore his sight. I don’t think I would have heard God call me to a higher mission if I had not been losing my sight. And He could have left me in that state but then Peggy walked into my office that day without glasses and I saw that look on her face and I witnessed a new life emerging.
Each day when I wake up I am reminded of the fact that I could lose my sight again if these corneas are rejected. That knowledge continually gives me the sense of being in the presence of God. I have empathy for drug abusers and alcoholics that I work with in my ministry. I have a sense of their struggles as I sit across from them. Not that I struggle with my sight every day but I know the potential is there to lose it, just as they can lose their sobriety. In that sense, we are sharing a journey of gratitude.
Advice to Readers
There is, indeed, help for those who struggle with sight issues. Someone like Dr. Price and his organization are worth a visit or a consultation. I have noticed one thing about him: he has incredible patience. He’s not in this for the money because he takes so much time with his patients; he does not just run into the exam room and rush you into surgery. He spends time getting to know his patients. There were days when I felt impatient and wanted him to go faster, but he would just take his time to find the best care possible for me. Readers should try and find someone like this when they are seeking a doctor to help with vision problems.
In closing, I would tell readers that the accounts you are reading are of real and normal people whose quality of life has been enhanced immensely as the result of taking a worthwhile risk. Have courage and have faith in God as you go forward to find the best care for your eyes.