Volume 9, Number 2

Summer 2000

• Steinmann Named Research Director 
• What Is Exfoliative Glaucoma?
• Shaffer International Fellow from Mongolia Joins Research Team 
• Pharmacia & Upjohn Scholarship Brings Researcher from Greece 
• New Clinical Fellows Assume Responsibilities 
• Scholler Foundation Renews Support for Searchlight on Glaucoma 
• Dr. George Spaeth Named Louis J. Esposito Glaucoma Research Professor 

Steinmann Named Research Director

Beginning a new era in research on the Glaucoma Service, William Steinmann, MD, MSc, was named Research Director of the Glaucoma Service of the Wills Eye Hospital, at a meeting of the Foundation Board May 16, 2000. Dr. Steinmann, while retaining his position as Director Jack R. Aron Chair in Primary Care Medicine at the Tulane Center for Clinical Effectiveness and Professor of Medicine at Tulane has begun overseeing a major reorganization of the research activities on the Glaucoma Service through frequent videoconferencing and on-site visits.

An internist with a strong background in epidemiology and ophthalmology research, about ten years ago, Dr. Steinmann, while on the faculty of the Clinical Epidemiology Unit at the University of Pennsylvania, supervised the research activities of the Glaucoma Service for a number of years.

Himself diagnosed with glaucoma three years ago, Dr. Steinmann has a personal as well as academic interest in ensuring the validity and usefulness of research conducted on the Glaucoma Service. He explained, “Many of the important studies that shape our beliefs reveal poor biostatistsics or study design. Inadequate patient and control group numbers result in studies without the power to show anything meaningful. Poorly selected control subjects invalidate conclusions, and confounding variables bias results.”

Commenting on Dr. Steinmann’s appointment, Glaucoma Service Director and Foundation President Dr. George Spaeth affirmed: “While research has always been a priority for the Service, Dr. Steinmann will bring our efforts to a new level of productivity and sophistication, renewing hope to glaucoma patients around the world.”

 

Dr. William Steinmann (center) with Wills Ophthalmologist-in-Chief Dr. William Tasman (left) and Glaucoma Service Director and Foundation President Dr. George Spaeth.   Photo by Jamie Nicholl.

What Is Exfoliative Glaucoma?

Richard Wilson, MD
Jeffrey Henderer, MD
Ken Parker, PhD

Glaucoma comes in many forms. So much so that it's really best to speak of "the glaucomas" rather than simply "glaucoma." A major distinction is between the "open angle" glaucomas and "closed angle glaucoma." Open-angle is by far the most common. In this form intraocular pressure becomes too high for the optic nerve cells in the retina to tolerate and they become damaged and die. If enough become damaged and die, areas of the visual field are lost.

If a narrow angle becomes closed, the reason for the pressure rise is easy to understand. The aqueous fluid which fills the eye is made behind the iris. This fluid flows throughout the back of the eye and then out the pupil into the anterior chamber of the eye. The purpose of this fluid is to carry nutrients and oxygen from the blood circulation to the tissues lining the inside of the eye and to wash away waste products. The aqueous fluid leaves the eye through the trabecular meshwork or drain of the eye.

If the iris is situated too close to the drain in an eye with a narrow angle, the iris can be sucked into the drain and completely block it, causing the pressure to build to intolerable levels. On the other hand, even when the "angle" is fully open and there is nothing visible blocking the drain, intraocular pressure may become too high for an eye to tolerate. The reason for this is unclear.

Exfoliative Glaucoma
In one very interesting type of open-angle glaucoma, however, the cause of the glaucoma is obvious -- "exfoliative glaucoma.” "To exfoliate" means "to come off in flakes, scales, or layers, as skin, bark, rock, etc." The exfoliation syndrome is a condition that affects the entire body, but presently the only known adverse effects are seen in the eye. What flakes off in the eye in this condition is an amyloid-like substance (a protein of some sort), readily visible through the examining microscope on the lens surface. Through the microscope, the protein looks like small flakes, not unlike dandruff.

Presently, we have no idea from where this protein comes. Although there are leads, no definite gene has been identified. It seems the main reason people get glaucoma from this condition is that the protein material lodges in the trabecular meshwork (the drain of the eye) and prevents fluid from leaving the eye as easily as before.

It is unclear how long most individuals have exfoliation before the build-up of exfoliative material in the drain of the eye causes the pressure to rise and glaucoma to develop. When it does, however, this elevated pressure can occur as intermittent "spikes" throughout the day. Because the increased pressure is not constant, it may or may not be noted by an examining doctor. In any case these sudden increases may be very difficult to lower. Medications to decrease the eye pressure are often effective in this situation and are used in much the same way as medications for primary open-angle glaucoma. If medicines do not seem to be lowering the pressure enough, then either laser or cutting surgery (trabeculectomy) can help greatly.

Exfoliation material on the front surface of the lens.

Exfoliation material on the tiny fibers (zonules) that hold the lens in place, causing them to detach from the lens at the 1 o'clock position.

First Described in Sweden
The exfoliation syndrome was first described by Dr. Lindberg in 1917 in Sweden. He coined the term "capsular" glaucoma, still widely used in Scandinavia to describe the condition. Dr. Sunde further refined the clinical definition of the exfoliation syndrome into its present-day form. Of note, the exfoliation syndrome is frequently referred to as "pseudo” (false) exfoliation. This is to distinguish it from "true" exfoliation which is a splitting of the anterior membrane of the lens due to continued exposure to infra-red light. This type of "exfoliation" is frequently associated with cataract formation and is commonly called "glass-blower’s cataract" as persons in this profession are exposed to great amounts of infra-red light and can get this problem. On the Glaucoma Service, we prefer to use the term "the exfoliation syndrome" to describe the condition that is associated with glaucoma.

Who Gets Exfoliative Glaucoma?
While men and women develop exfoliation and exfoliative glaucoma with equal frequency, the prevalence (frequency) of the condition varies around the world from ethnic group to ethnic group. Scandinavia has one of the highest rates, Navajo Indians have about a 40% prevalence, and in Greenland, up to 50% of all open angle glaucomas are associated with the exfoliation syndrome. Interestingly, however, the condition has never been found among Eskimos. In the United States it is found in about 5% of the population.

Importantly, the number of persons with the condition increases with age, and the risk of the condition leading to glaucoma also increases with age. It is estimated that 5% of people who have had exfoliation for 5 years have developed glaucoma and 15% of those who have had it for 10 years.

One of the main characteristics of exfoliation is that it typically involves only one eye initially. However, after 5 to 7 years, up to 40% of persons demonstrate findings of the condition in the previously unaffected eye. Just as both eyes can get exfoliation, both eyes can get glaucoma.

Q & A with Dr. Wilson
The following exchanges between Dr. Wilson and participants (P) during a recent chat session on the Foundation’s web site deal with glaucoma caused by the exfoliation syndrome.

P:  What impact might exercise have on glaucoma caused by the exfoliation syndrome (XFS) and what types of exercise are potentially harmful?

Dr. Wilson:  Intraocular pressure spikes from XFS are not nearly as exercise-related as pigmentary glaucoma (a condition in which glaucoma is caused by pigment granules being rubbed off the back of the iris and blocking the drain in the eye). I impose no exercise restrictions, and even encourage 20 minutes of aerobic exercise four times a week.

P:  Can arthritis medication affect XFS?

Dr. Wilson:  Arthritis medications might help raise the blood aqueous barrier, i.e., make the inflammatory effects of the iris rubbing over the rough surface of the lens less prominent. I would not shy away from using arthritis medication.

P:  Are there any nutritional therapies that may potentially benefit XFS?

Dr. Wilson:  Theoretically, Vitamin E, 400 I.U. per day, and low-dose aspirin may be neuroprotective, i.e. act to lessen the effects of potential harmful influences like elevated eye pressure on the optic nerve.

P:  Does a cataract affect lens exfoliaton?

Dr. Wilson:  Having a cataract does not affect exfoliation that I know of. Having XFS does make taking the cataract out harder, as the XFS weakens the support of the lens in the eye.

P:  Is the prognosis for XFS better or worse than for patients with the more common form of primary open-angle glaucoma?

Dr. Wilson:  The prognosis is slightly worse overall, but XFS responds better to laser trabeculoplasty than any other type of glaucoma.

P:  I have the exfoliation syndrome and just had an argon laser trabeculoplasty (ALT - using laser energy to increase the outflow of aqueous fluid from the eye). My pressure dropped from 40 to 18. Is such a large pressure drop unusual? How long can I expect it to last?

Dr. Wilson:  That is the kind of pressure drop you can expect with an ALT for XFS, especially if you are over 70 years old. Unfortunately, if you are in your 50s, the eye pressure lowering effect may not last for more than a year or two.

P:  Is any (leading) research on FXS being done?

Dr. Wilson:  There is research on XFS being done, but I see no quick answers.

P:  Could anti-aging strategies like nutrient rich calorie restriction have an effect? Do you think we are dealing with a connective tissue type disease?

Dr. Wilson:  That is an interesting idea, but unfortunately not enough is known about XFS or nutrient rich calorie restriction in humans to hazard a guess.

Shaffer International Fellow from Mongolia Joins Research Team

Dr. Undraa Altangerel, from Ulaanbaatar Mongolia, has joined the Glaucoma Service as a Research Fellow for the coming year. Recipient of a prestigious Shaffer International Fellowship from the Glaucoma Research Foundation in San Francisco, Dr. Altangerel brings with her an impressive background in glaucoma. Most recently she worked in the Glaucoma Unit and Laser Laboratory of the Department of Ophthalmology at the National Medical University in Ulaanbaatar. In addition she played an active part in the Clinic’s research projects and teaching. She is continuing to work on a glaucoma resesarch project being carried out in collaboration with the Institute of Ophthalmology, University College of London, United Kingdom.

Dr. Altangerel is accompanied by her husband, Enkhbat, their seven-year-old son Achit and their four-year-old daughter, Enerel.

Dr. Undraa Altangerel at the Heidelberg Retinal Tomograph in the Glaucoma Service Diagnostic Laboratory.

Photo by Jamie Nicholl

Pharmacia & Upjohn Scholarship Brings Researcher from Greece

Dr. Chrysssa Terzidou, from Thessaloniki Greece, will serve as Research Fellow on the Glaucoma Service for 6 months. A generous scholarship from Pharmacia & Upjohn has made it possible for Dr. Terzidou to participate in the Service’s research activities. She has published and taught extensively in Greece as well as internationally.

Photo by Jamie Nicholl.

New Clinical Fellows Assume Responsibilities

Three new clinical fellows begin their year-long training in July:

Dr. Lorenzo Pecora completed his ophthalmology residency at the Nassau County Medical Center in East Meadow New York after receiving his medical degree from SUNY Health Science Center in Brooklyn and serving an internship at Staten Island University Hospital. In addition to his clinical training, Dr. Pecora has published research in the areas of genetics and the pigmentary disperson syndrome in glaucoma and a new glaucoma surgical procedure, non-penetrating trabeculectomy.

Dr. Leslie Stiff Jones received her medical degree from Howard University and completed her ophthalmology residency at Howard University Hospital. In addition to her clinical background, Dr. Jones spent a year on the Public Health Service of the Department of Health and Human Services as a Howard Hughes Scholar, on leave from from Howard University to spend a year doing basic research on cellular mechanisms of inflammatory eye disease. Dr. Jones was also involved in glaucoma screening projects in the Washington, DC area.

Dr. Paul Harasymowycz received his medical degree from McGill University and completed his ophthalmology residency at the University of Montreal, where he served as Chief Resident. Dr. Harasymowycz has research interests in “normal tension glaucoma,” neuroprotection, and “new” surgical alternatives, such as “deep sclerectomy.”

New Clinical Fellows (from the left) Drs. Lorenzo Pecora, Leslie Stiff Jones, and Paul Harasymowycz in the middle waiting room of the Spaeth/Katz/Myers Practice.

Photo by Jamie Nicholl.

Scholler Foundation Renews Support for Searchlight on Glaucoma

The Scholler Foundation announced in June they will continue to support publication and distribution of Searchlight on Glaucoma. Speaking of their generous commitment, Dr. George Spaeth wrote E. Brooks Keffer, Jr. of the Foundation:

“We are extremely grateful for the Scholler Foundation’s sustained commitment to us over the course of many years, and for your belief in the work we do. Your support for the Searchlight has been a critical factor in our ability to reach tens of thousands of patients each year, and on their behalf, I offer thanks as well.  As you know, we have linked the Searchlight with our Foundation’s enhanced website, and the site was recently named one of the top five glaucoma websites in the world. We are very encouraged by this expansion of our ability to reach even more individuals concerned about glaucoma.

“The Searchlight is absolutely central to our mission. It allows us to change the fundamental way patients think about glaucoma and, more pertinently, about themselves. The Searchlight not only educates but also helps patients and the public become active participants in learning, teaching, and caring. As our research activities have grown, so also, obviously, have the costs of research. The Searchlight has become a gentle but effective instrument helping patients make choices, and one of those choices is to support research with a commitment of time or finances.”

 

Foundation administrative assistant Nancy Petrongolo (left) and bookkeeper Lois Belavsky in the new Foundation office in one of the converted row houses on 9th Street, across from the Hospital.	Dr. Steinmann (right) with Glaucoma Service Staff member Dr. Jonathan Myers (left) and Clinical Coordinator Diana Meashey.
Dr. Steinmann (left) with Pharmacia & Upjohn Medical Sciences Liaison for Ophthalmology Richard Tyburczy.	Dr. Spaeth (left) and Glaucoma Service Staff member Dr. Jeffrey Henderer speak with Dr. Steinmann during a videoconferencing session.

Dr. George Spaeth Named Louis J. Esposito Glaucoma Research Professor

Foundation President Dr. George Spaeth was named the first Louis J. Esposito Glaucoma Research Professor at a July 14th meeting of the Board of City Trusts, the governing body of Wills Eye Hospital. The move came at an especially propitious time as the Foundation and Hospital are augmenting their research capabilities. The professorship is expected to enable Dr. Spaeth to devote a greater portion of his time to ensure that the Glaucoma Service is performing the best clinical research into the causes and treatment of glaucoma anywhere.

One of four endowed research professorships seeded at $1 million each (the three others are in cornea, oncology, and pathology) two years ago, Dr. Spaeth’s professorship was named in honor of Louis J. Esposito, Chairman of the Board of City Trusts and long-time supporter of Wills Eye Hospital. A series of private donor contributions increased the endowment funds of Dr. Spaeth’s professorship to a total of $2.2 million