A Mystery Disease: One Woman’s Struggle to Get Diagnosed with NPH

“I always say, I have hydrocephalus but it doesn’t have me.”

Merlin Bott remembers the day she first started feeling sick. She woke up one morning in October 2013 with a severe case of vertigo. It quickly got worse and over the next few days she couldn’t keep anything down, kept falling over and started having problems with her short-term memory.

She consulted her ear, nose and throat doctor, who ended up performing a balloon sinuplasty procedure, thinking she had a terrible ear and sinus infection. Unfortunately, her symptoms only continued to worsen. Soon, she became so dehydrated that she had to be hospitalized.

“At the hospital, they thought I had h pylori because I kept throwing up and they also thought I might have lung cancer so they ran several tests. They even thought I might have some type of dementia since my memory was so bad at that point. Then, since I was a flight attendant they thought I caught a rare disease overseas like SARS or TB. So I was quarantined for 48 hours. They called in the CDC to come in and do independent tests on me. The tests came back negative for everything,” Merlin explained.

Finally, doctors performed a lumbar puncture and discovered that she had excessive fluid in the brain. She was told she likely had hydrocephalus but not to worry about it and was told she could go home. Wanting to seek a second opinion, Merlin and her husband Chris sought out a neurosurgeon at the Cleveland Clinic. The neurosurgeon performed an MRI and gave them bittersweet news.

“He said she needs brain surgery and she needs it now. I was hesitant and thought, ‘isn’t there a pill that can help her get better?’ The doctor said there wasn’t and the next day she had surgery to have a shunt placed,” said Chris, who has been with Merlin every step of the way throughout her journey. “It was a horrible feeling. When you’re told your loved one need brain surgery you just think, ‘oh my god will our lives ever be the same?’”

It turns out that Merlin had Normal Pressure Hydrocephalus (NPH), a type of hydrocephalus most commonly seen in older adults. People with NPH typically have difficulty walking, cognitive impairment, and impaired bladder control. Merlin had all of those symptoms.

Since NPH symptoms often mirror those of other diseases that affect people over 60, like Alzheimer’s, Parkinson’s or other neurodegenerative conditions, many people with NPH struggle to be diagnosed correctly. In fact, it’s estimated that 1 in 10 dementia patients actually have NPH but may never be diagnosed.

In total, Merlin spent 29 harrowing days in and out of the hospital.

Since her shunt surgery, life for Merlin changed for the better. While her recovery wasn’t easy, she slowly regained her ability to walk. The vertigo disappeared and her memory improved. Today, Merlin is 66 years old and is enjoying her recent retirement from over 35 years as a flight attendant.

She considers herself one of the lucky ones.

“I came close to dying and was down to 85 pounds. But I’m a strong person and fought hard to come out of this, with Chris’ help. I always say, I have hydrocephalus but it doesn’t have me,” Merlin said.

A big part of her recovery was finding the Hydrocephalus Association, which helped her connect with other individuals who are living with NPH.

“I started participating in the Hydrocephalus Association WALK to End Hydrocephalus and met others who have NPH. Connecting with others who have what I have was so important because I didn’t know what hydrocephalus or NPH was and had never met anyone with this condition,” Merlin said.

She hopes HA’s research efforts will help identify more ways to treat hydrocephalus and lead to a better understanding of why NPH develops.

“I don’t know how I got NPH. We need to know more about hydrocephalus and we need more treatments other than brain surgery. If we can keep doing research and find other treatments and a cure, I would love to see that in my lifetime,” she said.