AEVR Hosts Thyroid Eye Disease Congressional Briefing

Click Here to watch a video of the Briefing

On November 19, AEVR’s Decade of Vision 2010-2020 Initiative held a virtual Congressional Briefing entitled Thyroid Eye Disease: What Have We Learned from Research?  that recognized the first-ever TED Awareness Week. The event featured Gary Lelli, MD, Vice Chair of Ophthalmology at Weill Cornell Medicine in New York who is a board-certified ophthalmologist specializing in oculoplastic surgery. He was joined by patient advocate Christine G. a small-business owner from California who described her  experience with TED, focusing on its quality-of-life implications.  

Dr. Lelli explained that TED, sometimes called Graves’ ophthalmopathy or Graves’ Eye Disease, is an autoimmune disease that occurs in up to 50 percent of individuals with Graves’ disease. Graves’ disease causes the thyroid to overproduce thyroid hormones, resulting in an overactive thyroid, also known as hyperthyroidism. TED can occur in patients who already know they have thyroid disease–– about 80% of TED patients will present symptoms within 18 months of their Graves’ disease diagnosis––or it may be the first sign of a thyroid condition. TED can also occur in people living with Hashimoto’s hypothyroidism or normal thyroid levels. While TED most often occurs in individuals with hyperthyroidism, it is a distinct disease that requires separate treatment, and treating hyperthyroidism may not resolve visual symptoms and changes. TED can be associated with a family history of the disease, and although it usually affects middle-age adults, it can occur at any age. Although females have a greater incidence of TED than males, the disease is often more severe in males. Smoking can increase the risk of TED by 7-8 times and cause a longer “active” phase of the disease.

In the “acute” phase of TED, the main symptoms include inflammation and enlargement of the muscles and fat behind the eye (in the bony socket) causing the eyeballs to bulge out, a condition called proptosis. If the eye is pushed far enough forward, the eyelids may not close properly when blinking and sleeping, causing the cornea to become unprotected, dry, and damaged. The enlargement of the tissues and muscles of the eye may prevent it from properly working, which affects eye alignment (called strabismus) and movement, which can lead to diplopia, or double vision. In severe cases, the inflammation and enlargement of the tissues, muscles, and fat behind the eye may compress the optic nerve, which connects the eye to the brain, thereby causing vision loss.

Although medical therapy during the “acute” phase of TED has included steroids, radiation, selenium supplements, and other immunomodulatory treatments, these have generally been ineffective in stopping the progression of the physiological changes in and around the eyes and may have their own side effects. As a result, most patients have sought surgical intervention during the “chronic” stage of the disease, which includes orbital decompression, extraocular muscle surgery, eyelid repositioning, and soft tissue draping. 

Researchers funded by the National Institute of Health (NIH), especially its National Eye Institute (NEI), and by private industry have sought to identify the molecular pathways that lead to manifestation of the disease. They discovered that a protein, Insulin-like Growth Factor-1 Receptor (IGF-1R), is overexpressed in Graves’ disease and subsequently that a fully human monoclonal antibody called teprotumumab inhibited IGF-1R expression, blocking autoantibodies from attacking orbital cells, reducing inflammation, and preventing excessive cell growth/buildup behind the eye. In a series of safety and effectiveness clinical trials, teprotumumab had been shown to be highly effective in reducing eye bulging and double vision and demonstrated a positive benefit-to-risk profile in treatment of TED. As a result, a drug therapy version of teprotumumab was approved in early 2020 by the U.S. Food and Drug Administration (FDA) for the treatment of TED.  

Dr. Lelli observed that effectively managing TED requires early diagnosis and treatment, active monitoring, and co-management of the disease—the latter ideally engaging physicians who specialize in the thyroid, endocrinology, and ophthalmology. Regarding what the future holds, he urged more research into the pathophysiology of the disease, especially into metabolic pathways during the chronic stage of the disease where patients appear to be clinically stable, to save them from surgery. “TED has traditionally been a watch and wait disease, but it doesn’t have to be that way anymore,” he concluded.        

Patient advocate Christine related her experience with TED and how it became a significant challenge to her quality of life. Despite experiencing eye bulging and severe double vision, she has continued to run her business and engage in activities of daily living, which include swimming and biking. As a veteran of multiple surgeries, she discussed how she had to become her own advocate for proper treatment that has resulted in resolution of her double vision. Christine noted, “when presented with TED, most people are going to pull inward, isolate, remove themselves from their previous way of life. It is important that a person with TED reach out and not pull inward. There is help available, now more than ever. So reaching out, advocating for one’s own health, insisting on living a full life, is the most hopeful road to recovery from TED.” She concluded by stating that, “I feel as though I have come out of a tunnel and into the light. And although I have dealt with the consequences of TED well before this new drug therapy, I am happy that others will not have to experience what I have.” 

In the question session, Christine spoke about the life-isolating aspect of the disease and related it to the current COVID-19 quarantine. She also recognized that many TED patients have a hyper-sensitivity to wearing masks.