700K people are likely living with this condition
Many people were unfamiliar with normal pressure hydrocephalus when superstar musician Billy Joel recently announced he was diagnosed with the condition. But the illness may be more common than we think, and is going undiagnosed.
“Around 700,000 people in the U.S. are likely living with this condition,” said Dr. Abhay Moghekar, associate professor of neurology at Johns Hopkins University. “We actually don’t have accurate prevalence measures, these are just estimates, but it’s likely a highly prevalent condition and just not being diagnosed.”
Moghekar said one reason NPH is not diagnosed is because the symptoms, which include trouble walking, urgency to urinate and cognitive problems, are common with normal aging or with other disorders like Alzheimer’s disease or Parkinson’s disease.
Amanda Garzon, chief operations officer of the Hydrocephalus Association based in North Bethesda, said, “We know that it has one of the highest misdiagnosis or under diagnosis rates, only potentially about 20-30% of people are actually being diagnosed properly because there is such a lack of awareness, even among medical professionals.”
A clinical diagnosis of the condition where people have too much fluid in their brain is only possible with a CT scan or an MRI. Another reason NPH may not be being diagnosed properly is the misconception that you need to have all three elements of the condition.
“You can have only gait problems and often those are the first to start,” Moghekar told WTOP.
While NPH can strike at any age, it’s more seen and diagnosed in children, characterized by an oversized head relative to their body.
And while the cause is not known, it can develop after brain trauma. Some adults who are not diagnosed until later in life also have oversized heads.
Garzon said the bad news is for kids who suffer from NPH, they sometimes have more brain surgeries than they’ve had birthdays.
“In children, there’s a failure rate of 50% of all shunts placed within the first two years,” she said. “Children will have more brain surgeries for a certain time in their life than birthdays.”
As far as treatment goes, Moghekar said there is no medication to treat the condition.
“The only treatment is placement of a shunt in the brain,” he said. “The shunt needs to be active for the rest of your life.”
The shunt allows the excess brain fluid to be directed to another part of the body, where it will not have detrimental effects.
Despite the fail rate, shunts are the only treatment right now.
Moghekar said the National Institutes of Health recently wrapped up a study on the shunts, with results expected later this year, which could help bring awareness and more diagnoses.
“I think that’s going to be a game changer because that’s going to provide us and medical community with a gold-standard evidence to show that, in fact, shunts really help,” he said.
For people suffering with the condition and for their families, support is very important.
“We want to make sure that people can find us because we have developed so many free resources and we have support groups around the country,” Garzon said.
Garzon said Billy Joel being willing to reveal his diagnosis is “a gift to the general population” and will lead to many more people knowing about NPH and getting help.
As for Joel continuing to make and perform the music so many people love, Moghekar said if he gets the right treatment and therapy, “I think he has a very high likelihood of being back on the tour.”