On Feb. 14, William Strange, 10, gave the nurses at Carson-Tahoe Cancer Resource Center something sweet — their own Valentine’s Day cards. In return, they surprised him with a LEGO City play set, and it made his entire morning.
The nurses have come to love William from months of visits as he sits for hours receiving infusion treatments, patiently bearing the pokes and prods from needles fighting to return to a normal, healthy life.
William Strange is fighting a wide range of symptoms from autoimmune encephalitis and complications from a central auditory processing disorder, all of which have impacted him on a cognitive, behavioral, social and emotional level.
To help spread the word about how unusual the disease is, his mother, Lora Strange, and some local friends are helping to coordinate a community blood drive from Sunday through March 2 to educate the public and call for plasma and platelet donations. The drive, appropriately scheduled after World Encephalitis Day, which was Friday, is held by nonprofit Vitalant, coming to the Strange family’s aid to help William and other children and adults in similar life-threatening situations.
Hope for William
William, a Seeliger Elementary School student, officially was diagnosed at 6 in February 2015 as a first-grader. Lora described him as a normal, healthy child, experiencing minor illness only annually until he began having a stomach flu for four weeks accompanied by a strep throat and a fever within a month. Other symptoms followed until he had a seizure and had to be taken to the emergency room.
The seizures would continue and doctors began testing for viral and bacterial causes and while most returned negative, Lora said, an electroencephalography (EEG) eventually came back indicating an encephalopathy, a type of damage or malfunction to the brain.
“When he first got sick, he was having hundreds of seizures,” Lora said. “He was in the hospital for a couple of weeks, and then they sent us home thinking that might help, and it didn’t help… When they finally got it to calm down, they gave him a high dose of steroids and then aminotherapy treatment.”
After a number of treatments, which include IVIG, or intravenous immunoglobulin, a long-term infusion immunotherapy he’s had to do every three weeks for two years, the frequency of his seizures radically was reduced but would continue to fluctuate. Lora and her husband Dennis would have to pursue different treatments at different hospitals between Carson-Tahoe Regional Medical Center and its Cancer Resource Center and Renown Regional Medical Center. Eventually they would seek care with Salt Lake City’s Primary Children’s Hospital and the University of Utah’s Pediatric Neurology department, where they’ve been under Dr. Michael Lloyd’s care.
Lloyd said despite a number of different trials, attempts to get William’s seizures under control have been problematic because research in encephalitis remains in its infancy.
“There’s different kinds of blood tests on the spinal fluid to look for different antibodies that we can tap the brain and mediate these seizures,” Lloyd said Thursday. “And we did that test on William but we could not find those specific antibodies. But (the field) is still evolving.”
Last summer, Lloyd said, they added a secondary immunotherapy, Rituximab, after the IVIG wasn’t working as well as they’d hoped. He now has about eight to 10 seizures a month.
Through the early 1990s, there were only four or five anti-seizure medications. Today, there are about 50, and some children won’t respond to them, though some might respond to certain immunotherapies.
For now, though, particularly in William’s case, a concern is a decline in his cognitive skills, Lloyd said, which could be caused by the seizures.
At home, for instance, he often struggles to make certain connections or can’t always form his vocabulary properly, and Lora said it’s difficult to know whether it’s truly in the brain or if he’s behaving as a normal child might.
“He’s amazing, though,” she said. “He’s a trouper. He’s so strong. He’s so kind and loving. … It’s been a journey for him. … And it’s not that we’re really worried about him dying from it so much … but it’s a serious condition. That’s what’s hard to explain to people. This could be a chronic thing. It could be forever.”
The medical care in its entirety has come at a cost for the Strange family, financially and emotionally. The copay for the IVIG is $5,500, and Lora said they’ve had the support of their doctors when necessary to ask for extra assistance to make a case as to why certain treatments are necessary. Lora stays at home to take care of William and his brothers, and there are the travel expenses to go to Salt Lake City, but she says they’re fortunate to stay with family in the area.
Lora said they’ve also felt blessed to receive the kindness of others who have sought to ease William’s situation in other ways and help him to enjoy life away from the confines of treatment centers and hospital rooms. He was nominated to have a night at the Reno Rodeo last year, and he was chosen to receive a check from the Reno Rodeo Foundation to go to Legoland for Christmas, among other activities.
Lora reflected on how much William’s case changed life for her and her four boys, now ranging from 13 to 5 in age, praising them for how much they’ve helped out when William’s suffered from his seizures or at any point of his illness.
“You have to come to grips with this life-changing thing,” Lora said. “Then I have to go to a breathing period because he went from a totally, perfectly healthy, normal kid one minute to someone who has something no one has heard of and you have to see how much it impacts him and how much it takes a toll. I almost call it post-traumatic stress disorder.”
Meeting Grace
While William was receiving treatment at Renown last summer, as fate would have it, the Stranges came to meet the Flynn family from Reno, whose oldest of five daughters also was undergoing treatment. The two families immediately connected when Emily had overheard Lora speaking with nurses about William and realized their children were battling the same condition.
Grace Elizabeth Flynn, 14 now, first began experiencing symptoms six years ago. When her condition didn’t improve after a year and doctors weren’t convinced she was able to improve after another year, the Flynns took her to University of California San Francisco’s Benioff Children’s Hospital. There, tests showed she had developed autoimmune Raynaud’s disease, which creates sores, narrows the arteries and limits blood circulation.
“She started developing these fevers and she would fever for three to five days at a time with high temperatures like 103 and go into a comatose state and sleep for days at a time, and if we attempted to wake her up, she either couldn’t wake up or cognitively she just wasn’t there,” Emily said.
Emily described Grace as being in an altered mental state for many days and she had lost 10 pounds in a month. She’d developed alopecia areata that affected her hair follicles, so she lost her hair, eyebrows and eyelashes.
Eventually, Grace came to a point where she would sleep three to five days at a time every other week, Emily said, and she’d developed a movement disorder and short-term memory loss.
“She’s always known who we are but can’t always understand what we say,” Emily said, later adding, “It’s been very hard. You’ve been sick, your brain’s been sick. You have to now allow yourself to do those things over again. There are a lot of rehab cognitive programs you do, and a lot of it can be behavioral and emotional.”
Emily’s husband, Kevin, is the director of pharmacies at Northern Nevada Medical Center in Sparks, which has been helpful to their family in navigating insurance, but as a family, the Flynns say to truly help Grace, the oldest of five daughters, in the long run, they want the community to know what it takes to help someone with encephalitis and autoimmune diseases.
“Both (Lora) and I have had sick children, and as you’re seeing your child, you feel hopeless and alone, and these blood drives or news articles are not just to benefit our kids,” Emily said. “Our goal is to help anyone else in our position. And we feel lucky we still have our children. There are plenty of children that will probably be in wheelchairs or not have the ability to speak or regain a lot of their mental capacity, but we are very, very blessed.”