RICE LAKE, Minn. - Paisley Forsell used to enjoy hiking, cliff jumping and bodybuilding competitions. She loves riding roller coasters.
"I like adrenaline, I guess you'd say," said the 21-year-old, who lives in the city of Rice Lake.
But although she's looking forward to a trip to Valley Fair later this summer and now is going to the gym on good days, Forsell's activities have been sharply curtailed over the past year and a half.
She can manage a quick trip into a store to pick up an item, the Hermantown High School graduate said. A longer excursion requires her to use her wheelchair, which she ordered in a smaller size to fit her 5-foot frame.
Forsell was diagnosed in February 2017 with neuromyelitis optica spectrum disorder (NMO), a rare autoimmune disease that attacks the optic nerves and spinal cord. Since then, her life has been on something of a medical roller coaster, with her treatments for NMO leading to bone deterioration that required double knee surgery twice within 10 months.
Not multiple sclerosis
For decades, NMO was known as Devic's disease, named for French physician Eugene Devic, who announced its existence in a paper in 1894. Jacinta Behne, executive vice president of the Guthy-Jackson Charitable Foundation, which funds NMO research, said Devic discovered there was a disease with some similarity to multiple sclerosis but different from MS.
Even today, Behne said, NMO is sometimes misdiagnosed as MS. That's particularly unfortunate because some common treatments for MS can exacerbate NMO. In particular, a drug prescribed for MS can hasten blindness in people with NMO.
Until Guthy-Jackson was formed 10 years ago last week, little research had been done on NMO, Behne said. The foundation, launched in California by a couple whose daughter was diagnosed with the disorder, has poured more than $55 million into research. There's still no approved treatment specifically for NMO, and no cure.
It's still a rare disease. Dr. Gary Beaver, medical director of the Multiple Sclerosis Program at Essentia Health East, is treating Forsell. He said Essentia Health-Duluth Clinic is seeing between 10 and 20 NMO patients, compared with 1,500 MS patients.
So perhaps it's not surprising that when Forsell suffered what she now knows was her first NMO attack, in May 2015, it wasn't diagnosed as NMO. Then a senior at Hermantown High, Forsell first experienced dizziness. That soon advanced to the point that she couldn't eat or drink without vomiting, she said. Over two weeks, she lost about 20 pounds. Then she started hiccuping - for three straight days.
Both intractable vomiting and intractable hiccups can be symptoms of NMO, said Dr. Michel Toledano, a Mayo Clinic neurologist who would lead Forsell's treatment less than two years later. But the connection can be missed, he said.
Forsell was admitted to St. Luke's hospital and diagnosed with benign vertigo, she said. The treatments that were prescribed didn't seem to help. But after a month, the symptoms went away.
Forsell quickly forgot about the episode. Her passion for bodybuilding had led to two fitness competitions in the bikini category, one in Duluth and one in St. Paul. In both, she placed first in her class. She got part-time jobs at the Edgewater Hotel and the Cold Fusion shake shop in West Duluth.
But in early 2017, an itch developed into a tingling sensation in her right thumb and index finger, and that spread to numbness throughout her body, particularly on the right side. She lost strength, and she'd drag her right leg when she walked. She couldn't hold a pen to sign the medical forms she'd be given during many visits to the emergency room.
Eventually, an MRI at St. Luke's suggested NMO. It was confirmed via a blood test sent to the Mayo Clinic. It was researchers at Mayo who discovered that an antibody called APQ4 is a marker for NMO, Toledano said, although it's possible to have the symptoms of NMO without having the biomarker.
Forsell was referred to the Mayo Clinic, where Toledano said he ordered the standard treatment of steroids to fight the disease. When the response to that was slow, he also ordered a plasma exchange - removing Forsell's blood, taking out the offending antibodies, and then returning the blood.
In time, patients can be placed on other immunosuppressant drugs, Toledano said, but in the meantime the steroids rarely lead to other complications. That happened to Forsell, who developed avascular necrosis (AVN), a bone disease that in her case attacked her knees.
That led to the two double surgeries - in July of last year and again in March of this year. Last month, she learned she still has AVN in both knees, Forsell said, and she eventually might face double knee replacement.
'Chances are good'
Although the NMO itself is more or less dormant, symptoms persist. Her right hand always feels numb, Forsell said, and the "tinglishness" increases in hot or cold conditions. It hurts to text, she said.
Because of the damage she sustained before being treated, those symptoms are likely to come and go indefinitely, said Beaver, whom the Mayo Clinic referred Forsell to when she returned to Duluth.
But the "chances are good" she can go on to live a reasonably normal life, he said.
"It's just going to be the fact that she's on medication to prevent her from having any further attack with her NMO," Beaver said. "She's on appropriate treatment now, so hopefully she will not get (more attacks)."
Not long ago, the average life expectancy for someone diagnosed with NMO was five years. Now it's considerably longer, Toledano said, although no one is putting a number on it.
The major concern now is that the medication affects the immune system, he said. That means patients will have a slightly increased risk of infections and malignancy over time.
Forsell and her mother, Tina Stromquist, have chosen to be optimistic and laugh at the challenges. Forsell displays a comedian's sense of timing when speaking of an ex-boyfriend who kept their puppy when they broke up.
"That was sad," she said, and then paused. "I was more sad about the dog than him, actually."
The physically fit Forsell was aghast over the weight gain that came from taking the steroid Prednisone, and with one effect of that in particular.
"I was like, 'Mom, I think I'm getting these random cuts,'" Forsell related. "She's like, 'Those are stretch marks.'"
"I was like, 'Mom, I think I'm getting these cuts.'"
Although she can't yet return to all of her former activities, Forsell is getting her life back. She's planning to go to school full time in the fall, she said, and she's getting back in shape, hoping to fit into a custom-designed bikini that she purchased before the second NMO attack. But she knows that when she gets tired, she has to put the weights down.
Forsell chooses to focus on the positive.
"Throughout this whole, entire thing, I think I've heard Paisley complain twice," Stromquist said.
"What 20-year-old could go through this crap and still laugh about it? That's the one thing we really try to do. ... We can't change it, and we have fun with it."
How to help
A benefit is planned to help Paisley Forsell's family meet significant medical expenses. Featuring a pasta dinner, bean bag tournaments, raffle prizes, silent auctions and live music, the event will begin at 6 p.m. Sept. 15 at The Other Place, 3930 E. Calvary Road, Duluth. For those wishing to make a monetary donation, an account has been set up at Republic Bank, 1619 Woodland Ave., under "Paisley Forsell Benefit" and a Go Fund Me page has been set up via Facebook under "Show Your Love for Paisley."
To learn more, call Amy Vittorio at (218) 348-0988, Kim Newman at (218) 391-1031, Tina Stromquist at (218) 340-2777 or Linda Crider at (218) 940-4049.
More on NMO
Learn more abou neuromyelitis optica from the Guthy-Jackson Charitable Foundation at guthyjacksonfoundation.org.