Helping the Keratoconus Patient Cope

By Bezalel Schendowich, O.D., F.I.A.C.L.E.
OCTOBER 1999

This O.D. offers practical suggestions about keratoconus that all eyecare practitioners should know.

In the vast majority of cases, keratoconus is a disease that affects healthy people. True, there exists a multitude of conditions and syndromes that occur more commonly in keratoconus sufferers than in the general population, but by and large, the typical keratoconus patient is physically healthy, mentally intact and an active member of society.

Keratoconus patients, like anyone suffering from a change in their normal levels of function, have a tendency to worry. This feeling is magnified because it involves a change in vision, one of our most important sensory functions. Reduced vision is perceived as a critical and non-replenishable loss.

Keratoconus patients are worried. They worry about their family life, their work and their social life. In particular, younger patients worry that studying or staying out late will no longer be possible because of the discomfort they experience from overwearing their contact lenses.

Many keratoconus patients worry about going blind. Many also fear that they will need corneal transplant surgery, even though we reassure most of them that they may not be part of the 10 percent of patients who, eventually, do need the surgery.

Parents who don't have the disease themselves worry about their child's eye condition and whether or not a corneal transplant will be necessary. They also suffer from guilt feelings when they consider the possibility of having passed on a genetic disease to their offspring. Alternately, parents who have keratoconus wonder if their children will develop it as well.

Many patients fight valiantly to retain their quality of life, even while their corneal condition worsens and best corrected vision disintegrates. As the face of the world around them changes, they try many coping strategies and are quite inventive while attempting to remain active housewives, teachers, engineers, actors, lawyers and doctors.

Many times, work that depends on independent transportation must be abandoned, free participation in sports might have to be curtailed and the choice of activities altered. The difficulties incurred when wearing contact lenses -- lenses popping out, dust catching under lenses, multiple vision with or without the lenses -- make it difficult to participate in activities such as basketball, tennis and cycling.

For many patients, "keeping your eye on the ball" can turn into "hunting for errant contact lenses in the grass."

Why Improved Training in Recognizing Keratoconus is Needed

The number of scientific articles describing the pathology, theory, biochemistry and clinical diagnosis of the disease is rapidly increasing. Major articles have appeared in the last 2 years in both optometry and ophthalmology journals reviewing the condition. Hopefully, this increased growth in published information will have a positive educational effect on eyecare practitioners.

The truth of the matter is that in many cases, a presumptive diagnosis of keratoconus can be ascertained easily during the history stage of the examination if the examiner has a properly sensitized index of suspicion. The preliminary identification of the cause of a patient's distress can be finalized in less than 5 minutes through a series of simple tests, even without corneal topography.

While all of this is true, we are still left to wonder, "Why are so many patients improperly diagnosed?" Perhaps, an answer to this question can be found in a review of some basic practice statistics.

Although every eyecare practitioner (ECP) has learned the physical characteristics of keratoconus at one time or another, not every ECP will have the opportunity to use this information in his practice. An average practice cares for a patient population of roughly ten thousand, and keratoconus occurs in approximately two patients in five thousand. This indicates that many ECPs are likely to see only four such patients during their practice lifetime - at most.

Considering that eyecare practices specializing in keratoconus and other corneal conditions exist and treat hundreds of keratoconus patients, it's easy to understand how the random keratoconus patient might be misdiagnosed in an otherwise nonkeratoconic practice.

It's sad when a patient who is recommended to attend the clinic of a local, well-known ECP leaves the session seeing slightly better with a new pair of spectacles, but no wiser about his condition.

Even sadder is the busy contact lens fitter who somehow or other misses casual signs and "red flags" in the patient's ocular history.

Saddest of all is the practitioner who insists that his patient will benefit from amblyopia therapy when, in fact, a standard tri-curve lens will immediately improve acuity from a vague 20/200 to a quite acceptable 20/30+.

Fitting the Needs of the Keratoconus Patient and the Keratoconus Patient's Corneas

Regardless of our individual expertise as practitioners, we are seen by the patient population as experts in the eyecare field. All keratoconus patients expect to be fit easily, accurately and with the least expenditure of time and money.

As those of us with some keratoconus experience know, every keratoconic cornea has its own individual personality, making many patients schizophrenic -- ocularly speaking. Many patients are fit with radically different right and left lens systems in order to satisfy their visual needs. The time, expense, fitting skills and patience that this work requires may seem to be less than adequately compensated for and occasionally abused by the frustrated patient. Nevertheless, we must vault the impossible by putting ourselves into the shoes of the patient; and we must attempt to understand the patient's confusion and distress.

It becomes our role as ECPs to explain the cause of the disability to our patients by way of pictures, drawings and models. We must elaborate and educate them using prepared materials such as the National Keratoconus Foundation (NKCF) Guide. To request a copy of the NKCF Guide, visit their Web site (www.csmc.edu/nkcf/) and click on "contact us." You will then be provided with an e-mail address and a standard mailing address that you can use to make your request.

Here are some valuable tips when working with these patients:

• To build a greater rapport and to prevent misunderstandings, discuss charges and fees with the patient before entering extended periods of fitting and potential lens exchange.
• Listen attentively to patients' complaints and problem descriptions.
• Evaluate corneal topography as accurately as possible.
• Show an interest. Educate, demonstrate and explain the condition to the patient. It's important to show the patient that you take an interest in his problem, have taken the time to read and learn about the problem and can explain it in words he can understand.
• Be aware of advantages and disadvantages of different contact lens designs suitable for the keratoconic cornea.
• Know when to refit.
• Do not fear changing lens designs.
• Keep trying.
• Insist on follow-up exams.
• Know when to refer the patient for a surgical consultation.

Perhaps, the most difficult task in the practice of a keratoconus practitioner is attempting to explain to the patient why they are seeing multiple images, ghosts and starbursts, why driving at night is perilous and why these visual symptoms are most likely going to be permanent. They claim they will go crazy, and you claim that gradually, they will learn to disregard the secondary images.

Before a practitioner can enter a contact lens fitting relationship with a keratoconus patient, both parties need to understand the limits of this mode of treatment. Contact lenses are fit to improve vision, not to retard the development of keratoconus. The ECP who feels or promises otherwise has not seen a cone tip scarred from a too flatly fitting lens.

The best case scenario may be described as achieving 6/9 (20/30) or better visual acuity with lenses that do not significantly compromise epithelial health and allow for continuous wear sufficient enough for the patient to satisfy his individual needs as well as to remain an active member of society.

Additional Support

I recommend that a package of information be provided to each newly diagnosed keratoconus patient. It should contain the following tools:

1. The National Keratoconus Foundation Guide

2. A useful list of internet addresses, including:

• NKCF (www.csmc.edu/nkcf)
• Keratoconus-link (e-mail listproc@ucdavis.edu with the message: subscribe kc-link <subscriber's name>)
• Keratoconus and Cornea Transplants (www.febo.com/eyes/)

3. A letter of request for reimbursement from the patient's insurance company after the provision of services or devices (see example letter)

4. An invitation to join the local keratoconus support group in your area

One of the most difficult tasks for the ECP who undertakes the care and support of the keratoconus patient is to define how the condition changes the cornea. The NKCF Guide is a fine beginning, but I have found that a simple, three-dimensional (3-D) model helps bring across dramatically and easily, the meaning of the phrase, "a curved corneal surface becoming pointy like the end of a football." Instructions for the construction of such a model follow.

Demonstrating Keratoconus Cone Development

Explaining keratoconic corneal changes to the patient can be challenging. I consider that since a "picture is worth a thousand words," a model ought to be worth a million. I still haven't found the perfect 3-D modeling material with which to demonstrate the plasticity of the cornea and the developing cone, but with Silly Putty you can show shape changes that closely resemble those of the cornea.

Take a hollow ball (an old tennis ball for example) and cut from it parallel sides -- one smaller than the other (the front one about 2.5cm in diameter; the back one large enough to insert the Silly Putty and a finger or other probe).

Through the larger aperture, insert a flattened surface of Silly Putty into the sphere and against the inside of the wall with the smaller hole, thus, filling the smaller hole. By gradually pressing your finger, or better, a 1cm wide rounded-end probe, through the large hole against the back of the Silly Putty, you can produce a 3-D model of "time lapse" cone progression.

The two following modifications can aid dramatically in representing not only gross change in corneal shape but also the effects of corneal warpage that occur due to a too flatly fitting contact lens:

• Assure that the Silly Putty cornea is relatively thin. On the front surface, draw a cross or even a 3x3 grid. Now, when you push from behind creating the ectasia at an angle other than tangent to the surface, the grid becomes distorted, as would vision through a keratoconic cornea.
• Once your cone is protruding, you can use a relatively flat plastic surface or even your thumb to demonstrate the rubbing and scarring effects of a too flatly fitted contact lens on the cone's apex.

Support Groups: Real and Virtual

After your patient is diagnosed as keratoconic, after you have discussed the disease with him and taught him many of its aspects, and after you have fit and furnished the appropriate vision remediation appliances, what happens next?

The patient who has realized a decrease in his quality of vision and secondarily, a reduction in his quality of life, will need help and support. Many receive this support from family and friends, but many more do not. It falls within the province of the ECP to provide avenues of support to their patients.

Support comes in a variety of shapes and sizes. One of the most popular forms of support for patients is the educational support group. No matter how well written your handout is or how clear your oral explanations are, patients like to hear about their problems and discuss them repeatedly both with their ECPs and with other keratoconus patients. Support groups can be organized with the assistance of the NKCF. The foundation also provides suggestions, newsletters and an extremely well-produced reference guide for the keratoconic patient and his family. Many practitioners have found the experience of helping their patients begin and join support groups extremely valuable.

Free virtual support from the group of 700-800 participants of the KC-link, an Internet keratoconus support group, is always warm and forthcoming. The members of this e-mail bulletin board -- both keratoconus patients and ECPs alike -- are available to answer questions and to sympathize with other patients suffering with the condition in an informal, friendly atmosphere. All a patient or practitioner has to do to join the group is send an e-mail message to listproc@ucdavis.edu with the message: subscribe kc-link <subscriber's name>. Then, the patient or practitioner will be informed about the group and its services.

Ten Common Questions Asked by Keratoconus Patients

Over the past several years, while working intensively with keratoconus patients, I have noticed that many ask the same types of questions. I've listed some of the most common ones below, along with some potential answers. However, many of these answers are subject to change due to innovation in theory, diagnostic thinking, availability of new medications and the introduction of new technology. Don't be afraid to use your own experiences and knowledge to find your own answers.

Q Why do I have keratoconus and what causes the condition?

A  Keratoconus is a genetically determined condition. Unfortunately, the exact inheritance pattern has not yet been elucidated.

Q You have said that keratoconus is inherited. How can I help my children avoid it?

A You can help your children best by insuring that they have good eye care and annual eye examinations by a competent ECP who knows your eye history and understands the condition.

Q What can I do to keep the condition from progressing?

A Currently, no treatment -- medical, surgical or contact lens related -- can retard the progress of the condition. It may be damaging to corneal tissue to delay replacing your contact lenses when corneal changes indicate that such changes are necessary.

Q Why are contact lenses for keratoconus so difficult to fit, so uncomfortable and so expensive?

A Since the keratoconic cornea is irregular, additional time and trials may be necessary to achieve the best fit. Materials, time and remakes to achieve these goals cost more money than a normal fitting. If the practitioner and the laboratory do their job correctly, the lens need not be far more uncomfortable than any other RGP lens on a normal eye.

Q Which is the best kind of contact lens to wear?

A The best lens for any keratoconic eye is one that permits the best corneal health, while allowing for the best visual acuity, without sacrificing comfort. A lens material that allows for the least corneal insult while remaining stable during production and wearing is best.

Q Why must we wear rigid contact lenses? Why not soft contact lenses?

A While the majority of keratoconus patients achieve their best visual acuity with RGPs, some early keratoconus patients do well with toric soft lenses and some later keratoconus patients need either hybrid soft RGPs or even "piggy-back" (RGPs worn over a soft lens carrier) lens combinations.

Q Why is my vision not sharp, even with an RGP contact lens? Why do I have multiple vision?

A The typical keratoconic cornea is distorted and thinned in various loci. Each distortion in shape creates areas of different depth in the sub-lens tear layer. Each of these pools will give an unclear refraction. As the RGP moves with each blink, vision is dynamically unstable as the pools empty and refill throughout the various stages of the blink. Similarly, multiple vision is an effect of areas of different refraction in the cornea caused by localized thinning and variation in curvature.

Q Why is my vision so much less sharp when I remove my contact lenses?

A Because the cornea is thinner, it's structurally less stable. This instability allows the cornea to be more easily molded. The effect of this molding is a surface of topography and, therefore, refraction different from that of the same cornea in a fresh state. The same sort of molding can occur if you sleep with your eyes resting on your hands.

Q How will I know if I need a corneal transplant?

A Conventional wisdom dictates the need for a corneal transplant when you can no longer be fitted with a contact lens that is tolerable to you and provides you with adequate vision for your needs.

Q Need I be worried about becoming pregnant, giving birth or nursing around the time of my corneal transplantation surgery?

A In general, large doses of steroid medications are used to stabilize corneal grafts and, in some cases, glaucoma medications are required to reduce intraocular pressure. It is best to plan pregnancy, birth and lactation with these considerations in mind. Arranging joint consultations with your corneal surgeon and your obstetrician/gynocologist can also prove helpful.

I know that one article, one journal edition or one continuing education course cannot change the world. My message to all ECPs, (while I may be justly accused of preaching to the choir), is that we need to be sensitive to the tenor of patient history in order to accurately diagnose and reduce the aggravation and frustration that will inevitably confront many keratoconus patients.

Dr. Schendowich is an optometrist and a contact lens specialist at Sha'are Zedek Medical Center, Jerusalem, Israel. He is the organizer and moderator of the center's Keratoconus support group. He is also a consultant to the KC-link, an internet support group for sufferers of the condition.