Sam and Debbie Feldman were in the prime of their lives — successful careers, living in a Connecticut suburb, and raising a couple of boys — when Sam was diagnosed with progressive supranuclear palsy (PSP), a rare, degenerative brain disease that has similar symptoms to Parkinson’s disease and amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease.
PSP is a frontotemporal disease (FTD) that affects brain cells which control balance, walking, coordination, eye movement, speech, swallowing, and thinking. For Sam, it affected his gait, often triggering falls, caused his vision to declined, and made swallowing difficult. While his body was affected by the crippling symptoms of the disease, his mind remained sharp, even up until his final days.
Symptoms of PSP begin, on average, when a person is in their early 60s, but they can start when someone is in their 40s. Sam was diagnosed in 2004 and passed away in 2007 at the age of 52.
Debbie recently shared her family’s story in an ABC News article where she also explained that she wants to memorialize her husband’s legacy as well as that of her father, who died after a battle with multiple sclerosis (MS). She developed a keepsake jewelry collection, called Linked, to bring comfort to customers who could empathize with her husband’s story.
Each charm is coined with a phrase — such as “Be Brave,” “Can't Measure Love” and “Let the Light Shine Through” — that reflects on a memory Debbie shared with her husband.
“I created this line of jewelry to give words to the person who has trouble expressing themselves,” Feldman told ABC News. “Each piece of jewelry is linked to a personal sentiment. I want the jewelry to connect people with heartfelt emotions.”
To help raise funding for research, 10 percent of the proceeds from Linked's pendants and bracelets can be earmarked either for the fight against PSP or MS. Funds are given to CurePSP or the National Multiple Sclerosis Society.
What is Progressive Supranuclear Palsy?
PSP is a neurodegenerative disorder that has no known cause or cure. Also called Steele-Richardson-Olszewski syndrome, it results from deterioration of cells in areas of the brain that control body movement and thinking. The disease, which worsens over time, affects the majority of one’s senses and overall well-being.
Statistics show about three to six in every 100,000 people worldwide have the disease, according to the National Institute of Neurological Disorders and Stroke (NINDS). It is slightly more common in men than women, but has no known geographical, occupational, or racial preference.
Researchers are investigating why brain cells degenerate in those who suffer from this disease, said Jeff Bronstein, M.D., Ph.D., a neurologist based in Los Angeles, California.
“I think it's really encouraging that we are getting more and more of an understanding of PSP,” Dr. Bronstein told ABC News. “It really makes the studies a lot easier to do. We are getting closer.”
What are the symptoms of PSP?
According to CurePSP, symptoms of this frontotemporal disease include:
- Loss of balance
- Changes in personality
- Weakness of eye movements, especially in the downward direction
- Weakened movements of the mouth, tongue, and throat
- Slurred speech
- Difficulty swallowing
However, symptoms can vary between patients.
A person can suffer with balance issues, blurred vision, uncontrolled eye movements, unexplained falls, stiffness, slurred speech, and trouble swallowing solid foods and liquids, Dr. Bronstein explained. Some people are affected by changes in mood and behavior — such as irritability, forgetfulness and outbursts. Additional symptoms include depression, urinary problems and constipation.
PSP can be misdiagnosed as Parkinson’s disease because it shares many of the same symptoms. Problems with eye movement, behavior changes and memory loss are key identifiers that help a physician or neurologist diagnose this disease, Dr. Bronstein noted.
“It can be very hard to make the diagnosis very early on for that reason,” he told ABC News. “There is no blood test. We treat the symptoms specifically.”
Medication may mask symptoms, but it does not change the disease progression. Patients are predisposed to head injuries, fractures, choking and pneumonia.
Non-pharmacologic methods can also help remedy discomfort. Walking aids and devices such as chairlifts can reduce the chances of patients falling backwards. Bifocals or prescribed glasses, called prisms, can fix problems with looking down. Physical therapy may help with stiffness by keeping joints limber, and occupational therapists have utensils that assist people in feeding themselves as swallowing becomes more difficult.
How long can you live with PSP?
PSP is a progressive, degenerative disease. Prognosis varies. It is possible for a person diagnosed with PSP to live six to eight years after the first symptoms appear.
The onset of PSP is usually seen around the age of 60, but could occur earlier, according to NINDS. The disease typically affects men more than women.
The most common cause of death is pneumonia. There currently is no known effective treatment for PSP.