Uriel's Journey with Superficial Siderosis: A Life of Strength Amidst Struggles by Margaret
Mary.Uriel’s journey with superficial siderosis is a story of resilience and profound challenges. Our life took a sharp and unexpected turn in 2018 when my husband, Uriel, began experiencing tonic-clonic seizures. The sudden onset of these violent and disorienting episodes led to his hospitalization. It was a terrifying time, as we didn’t yet know the cause of these seizures or the full extent of what was happening to his body.
The road to his eventual diagnosis was not straightforward; delays in scheduling essential MRIs and PET scans stretched out the uncertainty. When the diagnosis of extensive superficial siderosis finally came, it brought clarity but also a heavy reality: our lives would never be the same.
This journey began years earlier, when we sought a solution for his profound hearing loss through a cochlear implant in November 2012. At first, the implant seemed promising. Uriel wore the processor diligently for five to six years. Though it never provided perfect speech reception, it did grant him a newfound awareness of his environment—he could hear sirens, a sound he hadn’t perceived in years. This small victory gave us hope, even though communication remained a struggle.
However, when we transitioned to a new outer processor, things changed. Instead of improving his experience, the device seemed to worsen the loud internal music that had become a persistent and unwelcome part of his life. This music was not soothing; it was intrusive, relentless, and overwhelming. The processor amplified this noise, making it unbearable. Frustrated and disheartened, Uriel made the decision to stop wearing it altogether. The realization that the cochlear implant had become more of a burden than a benefit was a difficult pill to swallow, especially for me. I couldn’t help but feel guilty for encouraging him to get the implant, even though my intentions had always been to help him regain a piece of his life.
Despite his physical limitations, Uriel’s mind retains flickers of the man he once was. Although dementia has set in, currently at the lower end of moderate severity, he has managed to hold on to his lifelong love of chess. Chess has been a constant in Uriel’s life since childhood, and it continues to provide him with joy and a sense of accomplishment. In fact, his sharpness on the chessboard remains astonishing. He often defeats the computer in matches and holds his own against most opponents. One of his closest and most loyal friends visits him nearly every month, and their chess games are a highlight of Uriel’s routine.
These moments of connection and mental agility are a reminder of the resilience of his spirit, even in the face of so much loss.
The diagnosis of extensive superficial siderosis two years later brought clarity to the many puzzling symptoms he had been experiencing but also deepened the challenges. Superficial siderosis is an unforgiving condition, and its impact on Uriel has been devastating. He has completely lost his senses of hearing, taste, and smell. His cerebellum—critical for coordination and balance—has been severely affected, leaving him without the ability to steady himself. No matter what he does, balance is an impossible goal.His condition has also taken a toll on his cognitive functions. Dementia clouds his once sharp mind, making even simple interactions complicated and sometimes painful. His body betrays him further with constant tremors—his arms, legs, and head shake relentlessly throughout the day. To manage this, he takes carbidopa-levodopa four times daily, which provides some relief but not enough to ease the daily struggles entirely.Exercise, once a potential avenue for regaining strength and maintaining his health, now feels futile to Uriel. His lack of balance and physical challenges make movement difficult, and his diminishing motivation means he often sees little point in trying. Watching him withdraw from these small efforts is heart-wrenching, but I understand the frustration and fatigue he must feel.Despite these immense challenges, Uriel faces each day with a quiet determination that I admire deeply. While his world has become one of limitations and loss, he remains present in ways that remind me of his inner strength. Our lives have been irrevocably altered by this condition, and while the path forward is uncertain, we continue to navigate it together, finding moments of connection and solace where we can. His journey, though marked by hardship, is a testament to his enduring spirit and our shared commitment to facing the unknown as a team.