by Karen Dandurant
news@seacoastonline.com
Published September 13, 2018

PORTSMOUTH – Susan and William “Towny” Manfull of Portsmouth want to raise awareness about the unusual disease that claimed the life of their beloved 26-year-old daughter, Alexandra “Alex” Manfull on Aug. 7.

Alex passed away of complications of PANDAS (pediatric autoimmune neuropsychiatric disorder), an often-misunderstood condition associated with streptococcal infections. According to the PANDAS Network, it occurs when “strep triggers a misdirected immune response (resulting) in inflammation on a child’s brain. In turn, the child quickly begins to exhibit life changing symptoms such as OCD, anxiety, tics, personality changes, decline in math and handwriting abilities, sensory sensitivities, restrictive eating, and more.”

Albanian doctor Dritan Agalliu was one of the first to identify PANDAS. A neurologist, his area of study was the blood brain barrier and the mechanism that could breach it.

PANDAS Network estimates that PANDAS/PANS affect as many as 1 in 200 children. While the condition is associated with children, adults can develop it.

“Alex never had a strep throat as a child,” said Susan Manfull. “It wasn’t until she was older, in 2009 that she got one, then another. She was finally diagnosed this past summer. She did all the research and said to me – mom, this is what I have.”

Alex was 17 when she got her first strep throat, and 19, a sophomore at Princeton University when she got the second in 2011.

Alex had been treated with antibiotics and was about to enter a round of intensive treatment but died days before it was scheduled.

Dr. Beth Latimer, of the Latimer Neurology Center, who practices in Washington, DC. is considered an expert in PANDAS/Pans disorders. She said the condition is close to other diseases such as epilepsy and rheumatic fever but it is often misunderstood and misdiagnosed. She is the doctor who was about to treat Alex.

“It is not a new, or a rare disorder,” said Latimer. “It has been recognized since the early 1900s. Before that people died for lack of treatment. Turkey is big epicenter for this condition. After 1940, when we had penicillin and other antibiotics, people sometimes got better.”

Latimer said there is a genetic component to PANDAS, but one other reason it seems to be emerging is the discontinuation of routine tonsillectomies.

“Anyone who grew up in the 1950s, almost routinely had their tonsils out at the first sign of infection,” said Latimer. “That doesn’t happen now. If you were born in the 1980s, chances are you have your tonsils.”

Most people deal with strep without complications. Others, those who develop the condition are seeing complications to their brain. Latimer said in the 1900s, the condition was referred to “as a separation of thought.”

“They morph and change,” said Latimer. “There are nuances of OCD, anxiety, dilated pupils and it can totally change their handwriting. They have abnormal REM and are not getting rest when they sleep, so they are tired and want to sleep a lot. They have dangerous, bad dreams and episodes of rage. They dream about death and dying.”

The difference between most psychotic conditions and PANDAS is that the people suffering from it know their feelings and actions are irrational; they just can’t control it, said Latimer.

Diana Pohlman founded the Pandas Network, in part because her two children both developed PANDAS as children, and because she had such a hard time finding the resources to cope. Her children, now 18 and 14 are doing well, but she said it was a nightmare road for her son, less so for her daughter.

“I recognized it right away in my daughter Katelyn because Garrett had it first,” said Pohlman. “He went through horrendous times and I had to fight to find out what was going on and what I could do because no one was talking about PANDAS at the time.”

Garrett’s case included violent rages and suicide attempts that would occur in fugue states. Pohlman said he got strep four times in a short period and changed overnight.

“He had severe OCD and feared radiation from the television and lights,” said Pohlman. “He couldn’t stay in class because he thought he would die, or I would die. He became psychotic and tried to jump out of cars, or in front of cars.”

Katelyn was treated successfully, but not before she developed OCD symptoms, and hallucinated things like giant spiders.

Susan said Alex did some things in college that, while unsettling, the entire family chalked up to the “pressure cooker” of Princeton and her heavy class load. She said they never saw a violent episode.

“She told me she took the shortest route to her classes, and I later learned she was counting the steps,” said Susan. “She would get upset if anything disturbed that count and she would have to start over. She was not anorexic, but she started counting calories. At her graduation, she had clearly lost weight, but we never thought it was due to a syndrome.”

Alex was a top student but turned her thesis in late. Susan said the reason was that she kept going over it, making changes. She continued to work on it after it had been turned in.

“She developed a compulsive picking, of her skin, and psoriasis, something she never had before,” said Susan. “She developed social anxiety, something she never had before. She declined to attend a holiday party at our neighbors, saying she felt uncomfortable around people. She slept a lot, but she had been working a lot and we didn’t see it.”

Alex found work in finance after graduation and that led to her discovery of PANDAS.

“She was doing research for a hedge fund client on hemophilia,” said Susan. “She ran across an article on PANDAS, and she knew. She went to a clinician and discussed her symptoms without ever mentioning PANDAS. He listened then asked her if she had ever heard of it.”

Alex sought and received a tonsillectomy. Susan said her psoriasis improved by 70 percent and she felt better. The family felt better because she seemed OK.

When Alex went to the west coast, it was for work, and because Dr. Latimer was going to start infusion treatments.

“She was so excited,” said Susan. “She felt validated and that she was going to be cured. She was in an ER for an appointment and the doctors think she got infected there and had a sudden flare of the disease. It took her life.

“It must have been a pretty heavy infection, as Alex was already on antibiotics,” said Towny. “We know she was planning her future.”

Pohlman said it wasn’t until she started Googling the right word that she found PANDAS.

“The word was basal ganglia,” said Pohlman. “The nerves in my son’s brain were being attacked. Strep was found in his blood. How did doctors not know? I learned the condition was closely related to Sydenham Chorea and rheumatic fevers. It is like a rheumatic fever of the brain.”

Latimer said treatment involves high doses of antibiotics, for extended periods of time, followed by steroids if unsuccessful. Plasma treatments can be tried to remove the excess “bad” antibodies that are being produced.

In a Facebook message, Towny and Susan said, “Alex was a force of life. She was prolific writer, voracious reader, artist, photographer, New York Times crossword whiz, television/film buff, fashionista, runner, financial analyst, dear friend to many, and daughter extraordinaire. As a graduate of Phillips Exeter Academy and Princeton University, she had a successful start on Wall Street, had just started an exciting job in finance in Washington, D.C., and was described as a rising star. Alex had her whole life in front of her. Instead, she succumbed to PANDAS. “Alex’s life, especially in her last years when she battled the debilitating symptoms of PANDAS, is a testament to her strength of spirit and character. The harsh reality is that there are only a handful of physicians and researchers who are even aware of PANDAS and most health insurance companies do not pay for treatment. Alex was fortunate and persevering enough to connect with one of the best physicians in the country — not everyone even knows how to begin such a search. It is urgent that this situation changes.”

Pohlman started a blog, at the suggestion of a friend who was a neurologist. From there PANDAS Network grew. She said her blog got a hit in five minutes, and by the end of the month, there were a 100, with 60-100 coming in every month.

“I thought of leaving it when my kids were older and well,” said Pohlman. “When the right treatment was found for my son; he was better almost overnight. But I realized there was no one who was going to keep this going. I have to, because people need to know.”

The Alex Manfull Memorial Fund supporting Research, Education, and Treatment of PANDAS in Adolescents and Young Adults has been established in the hope that other young adults, as well as children and adolescents, shall have greater treatment options and that never again will another life be cut short due to PANDAS. http://www.pandasnetwork.org/alex-manfull-memorial-fund/.