Ericka Shepard, a 50-year-old pharmacist and patient advocate in New Hampshire, has a rare eye condition called iridocorneal endothelial syndrome (ICE syndrome) glaucoma. The disease can be difficult to diagnose and typically affects middle-aged women. In The Glaucoma Foundation’s July 2023 newsletter, Shepard describes her disease journey and offers advice to others living with the disease.*

About ICE syndrome glaucoma

Glaucoma is an umbrella term for multiple diseases of the optic nerve. Glaucoma can be classified as either primary (independent of other medical conditions) or secondary (resulting from another condition). ICE syndrome glaucoma is a rare secondary form.

In ICE syndrome glaucoma, dysfunctional endothelial cells on the inside surface of the cornea migrate over to the iris and the structure where fluid exits the eye. This can close off the drainage system, causing secondary angle-closure glaucoma and a rise in eye pressure, damaging the optic nerve which serves as your eye-brain connection.

We don’t yet know why ICE syndrome occurs. Some experts think it’s set in motion by a virus like herpes simplex. We do know that it:

• Typically affects one eye
• Most often occurs in middle-aged women
• Likely isn’t hereditary

Difficult to diagnose

Diagnosing ICE syndrome isn’t easy. The diagnostic process for ICE syndrome often involves spectral microscopy, a high-resolution imaging technique for helping identify changes in corneal endothelial cells. This type of imaging is also called specular microscopy.

She visited her local eye doctor when she began losing vision and experienced a ‘gray veil’ descending over her left eye, lasting minutes to hours. Shepard was diagnosed with glaucoma and referred to a specialist at Tufts University. 

The treatment journey

Shepard was initially prescribed various eye drops that all failed at lowering her eye pressure. When she was eventually diagnosed with ICE syndrome (the delay being partially due to the fact that she didn’t have visible signs of iris and pupil distortion), an Ahmed shunt was surgically implanted in her eye to improve fluid drainage.

The device managed her eye pressure for several years, but began failing in 2022. Since her pressure was in the 20s at the doctor’s office, both she and her doctor assumed that she had time to consider her options before taking further action. 

Shepard sought multiple opinions, one of which was to rent a home tonometer for monitoring her own eye pressure. She soon discovered that, when she was home, her pressure was spiking into the 40s. A quick decision was made to implant a Baerveldt shunt, which has made a significant impact.

Using her experience for good

Shepard now counsels new home tonometer users as a Glaucoma Patient Ambassador for MyEYES. “Doctors’ offices are busy,” she says. “It is critical to know your disease, be compliant with your current plan, and follow diagnostic measures like renting a tonometer to improve outcomes.” Ambassadors like Shepard guide new users throughout their home tonometry journeys, a role that Shepard describes as “gratifying.”

“It’s been amazing talking to hundreds of patients,” she shares, “all with different stories.” 

Her advice

Shepard also shares her story in order to raise awareness of ICE syndrome glaucoma and help other women avoid similar struggles and losses. 

“My advice to all,” she says, “[i]s if you are a middle-aged woman experiencing vision problems in just one eye, have unilateral glaucoma, and no family history of glaucoma, you should ask your doctor about testing for ICE using specular microscopy.”

*The Glaucoma Foundation. (2023, July). Living With Glaucoma: Patient Advocate and MyEyes Glaucoma Patient Ambassador Ericka Shepard. Eye to Eye News – The Glaucoma Foundation Newsletter. https://www.flipsnack.com/clempgraph/july-eye-to-eye-2023-web/full-view.html