The human eye has inspired a seemingly endless supply of expressions. If someone gives us the evil eye, we may keep our eyes peeled for trouble, or if we find ourselves up to our eyeballs in work, we may cry our eyes out. Whether filled with stars or serving as the window to the soul, the eyes can express thoughts, indicate emotions, and even signal honesty or trustworthiness — all in addition to gathering and processing information that helps us more effectively navigate the world.
But what happens when those nonverbal communications are disrupted? For patients with thyroid eye disease (TED), challenges like impaired vision and disfigurement not only complicate their sight, but they also can be a barrier to making meaningful connections with others. Scheie Eye Institute aims to address these issues to give individuals with TED a second chance at unencumbered sight.
Launched in November, Scheie’s Thyroid Eye Disease Program offers advanced care for patients with TED, also known as Graves’ eye disease or Graves’ orbitopathy. The program — one of only a few in the country and the only of its kind on the East Coast — provides both medical and surgical interventions and treats patients at any stage of their disease.
“Patients with TED often struggle not only with physical discomfort, but also with the psychological burden of their disease. This newly established program aims to provide an underserved patient population with individualized, comprehensive, and compassionate care, and with the support of a multidisciplinary team whose combined expertise and commitment to excellence can profoundly change their lives,” said Joan M. O’Brien, MD, chair of Ophthalmology and director of the Scheie Eye Institute, which is based at Penn Presbyterian Medical Center.
Coordinating Care
César A. Briceño, MD
Madhura Tamhankar, MD
TED is an autoimmune disease that typically develops in individuals with hyperthyroidism caused by Graves’ disease. The most common orbital disorder in adults, it is approximately three times more likely to occur in women. Smokers in particular are at significant risk for severe, prolonged symptoms.
The condition occurs when the body’s immune system attacks the muscle and fatty tissues behind the eyes, resulting in inflammation that can cause the eyes to protrude and the eyelids to swell and redden. These changes can create a bulging, staring appearance that strains the eyes, and patients often report excessively dry or watery eyes, tightness and discomfort, and pain when attempting to look in different directions. In certain cases, the effects on the muscles can lead to eye misalignment and double vision, and severe inflammation can put pressure on the optic nerve, causing visual impairment and even blindness, albeit rarely.
“TED is so challenging to treat because it is multidisciplinary by nature,” said ophthalmologist César A. Briceño, MD, an oculoplastics and orbital specialist. “Oftentimes, a patient needs a care team with multiple specialists within ophthalmology in addition to their endocrinologist, rheumatologist, and primary care provider.”
The TED program aims to streamline what could be an overwhelming care coordination process, starting with a complete understanding of a patient’s vision disability and health and cosmetic goals. Program leaders Briceño and Madhura Tamhankar, MD, a neuro-ophthalmic and strabismus specialist, jointly evaluate patients and work together to determine the best treatment. Depending on severity, medical management includes treating underlying thyroid dysfunction, stabilizing hormone levels, eliminating smoking habits, and reducing eye pain. If a patient requires surgical rehabilitation, though, the process can take upwards of 18 months.
Treating with Complex Surgeries
The first step is decompression surgery, which addresses the protrusion problem by removing bone and soft tissue from behind the eye to create more space. If patients present with double vision or experience it following decompression, realignment surgery on the eye muscles can correct their vision. Finally, surgery on abnormal and disfigured eyelids can improve the appearance and function so they can close properly. While the process can be long and challenging, patients generally share the same sense of relief at the end.
Because these types of complex surgeries are difficult to perform, “a lot of practitioners only feel comfortable reserving these procedures for the most severe cases, such as for patients whose bulging and tightness is causing a loss of vision,” Briceño explained. “No one would argue their need for surgical intervention, but there’s another subset of patients with severe facial deformities who may not be in pain and may still see well, but are suffering nonetheless. That’s where we can add value and provide a service that other institutions haven’t been able to offer.”
Anxiety, depression, and low self-esteem can be common among TED patients. Indeed, watching their own faces become deformed (or “monstrous” as a patient heartbreakingly said to Briceño) with no clear path to recovery can lead to a lost sense of identity and a reluctance to interact with others for fear of being mocked, judged, or stared at. Briceño and Tamhankar are attuned to these issues and invite patients to talk about them openly. They also can refer them for further counseling to learn how to manage the emotional toll of the disease.
As the program continues to grow, Briceño and Tamhankar hope to launch research protocols exploring vision-related quality of life and how to ensure the best possible physical and psychological outcomes for TED patients. As a high-throughput clinic that cares for dozens of patients at different stages in one place, the program is also uniquely positioned to become a testing site for future pharmaceuticals that could potentially eliminate the need for surgical interventions in favor of medical management. “By aiming to create a center of excellence in managing TED, our goal is to offer our patients novel therapies when they become available in the future,” Tamhankar said.
“We strongly believe that a program that combines two subspecialties under one roof will enable our patients to gain a better understanding of their condition and of what it will take to restore their eyes,” she continued. “Given the many layers involved in managing this disease, patients often don’t have high expectations when they first come in, but after they see the outcomes, they’re so grateful — they are themselves again.”
Looking Ahead
The Thyroid Eye Disease Program is only one example of the incredible work being done at the Scheie Eye Institute.
The Primary Open-Angle African American Glaucoma Genetics study has entered its fifth and final year studying the genetic risk factors that contribute to a high incidence of glaucoma among African Americans. And, since the FDA’s approval of a gene therapy for a rare, inherited form of retinal blindness just over a year ago, the Penn and Children’s Hospital of Philadelphia (CHOP) researchers involved — including Jean Bennett, MD, PhD, and Albert M. Maguire, MD, of Penn Ophthalmology, and CHOP ophthalmologist Samuel G. Jacobson, MD, PhD, and Katherine A. High, MD, president and head of research and development for Spark Therapeutics — have also received national and international acclaim for their contributions.
As faculty and staff continue to collaborate across specialties, develop enhanced, innovative services for new patient populations, and conduct pioneering vision research, one thing is clear: all eyes are — and will continue to be — on Scheie.