Estimated read time: 13-14 minutes
SALT LAKE CITY — When Sean Cunningham's new hospital bed was moved to a space just feet from the front door of his Sugar House home, his young boys were worried about where the Christmas tree would go.
Colin, 9, and Ian, 7, know their dad is sick, but at this age — first and third grade — they think it's like a cold or flu. And Sean and his wife Whitney are trying to shield them as long as they can.
Sean Cunningham suspected carpal tunnel syndrome when it became hard to hold a pen or type a year ago. On his 46th birthday in March, after many tests, he was diagnosed with sporadic amyotrophic lateral sclerosis — ALS or Lou Gehrig's disease, as it's more broadly known.
That's a diagnosis that brings dread. ALS is a nervous system disease that weakens muscles and physical function. Its progress is individual but the final result is distressingly the same: ALS is terminal. Sporadic means that the reason Sean has ALS is unknown, which is true in the majority of cases. Just 5% to 10% of ALS cases are genetic. The rest are a mystery, though veterans seem to have higher risk.
Many people know about ALS from the Ice Bucket Challenge a few years ago, where people let someone dunk them with ice water to raise money for research. That provided significant research funding. But Whitney and Sean Cunningham now know a lot more than that about a disease for which he had no known risk factors, no genetic markers and no reason to worry.
When he was diagnosed, he was an associate professor and child psychologist trained in neuropsychology at the University of Utah School of Medicine, working with children who have neurodevelopmental disorders at Primary Children's Hospital. The disease has put him in a consulting role. Whitney, who teaches children with visual impairment at the Utah School for the Deaf and the Blind, is reluctantly acing a crash course in the disease but finds it hard to keep up with the speed at which ALS is attacking her husband. She's grateful for the "privilege of flexible work," as her job entails home visits to children with visual impairments.
Sean said the alarm bells were sounding when the carpal tunnel neurological exam became unusually detailed in February. He was sure he saw fear in the neurologist's eyes. By the time he got the diagnosis, he'd done more research and had a sick hunch it was ALS. At that point, "everything was scary," but he was still in denial. The Cunninghams even went to Cedars-Sinai in Los Angeles in hopes of a different diagnosis. ALS was confirmed on their 10th wedding anniversary.
Of all the lessons the couple has absorbed, by far the hardest is that ALS is always fatal. That knowledge jump-started their focus on future-crucial tasks like getting a will done and making sure Whitney knows all the financial and do-it-yourself things that have typically been Sean's job. While he can no longer wield a hammer, he knows how important it will be financially for her to handle that kind of chore. She is well on her way to being a "handywoman," because Sean has always been a handyman.
They have a lot of living to do right now, though, and they're giving that their full attention, building memories with the boys and embracing the joy that pops up quietly in small moments.
A terrible disease
People who have ALS lose the ability to walk, to use their hands, to eat and eventually to breathe, said Brian Frederick, executive vice president of the national ALS Association.
"We think of ALS as the worst disease," Frederick told Deseret News, "because it has a devastating impact on families. Often somebody diagnosed with ALS has to go through a very long journey to get that diagnosis and it's fraught with the anxiety of not knowing. Once they are diagnosed, they are often told there aren't a lot of options. Because of ALS's symptoms, a person diagnosed with it frequently loses the ability to perform in the workplace, so they leave jobs. Often the spouse is the primary caregiver and is eventually forced to quit or lose their job to help care for their loved one."
Frederick calls ALS "physically, emotionally and financially devastating."
But the past five years have brought some hope in the form of three new treatments that slow it down somewhat. Relyvrio, for instance, can provide as much as an 18-month survival benefit, he said. And access to multidisciplinary care can lengthen lives, he added.
Gaining months or even days can make a difference in being able to see a loved one's wedding or graduation, said Frederick. "We try to think about how valuable days or months are and make sure they have as many days, months and years as possible."
The Mayo Clinic says that "treatments can't reverse the damage of ALS, but they can slow the progression of symptoms. They also can help prevent complications and make you more comfortable and independent."
ALS impacts more men than women, the reasons again unknown.
Frederick said solid numbers on exactly how many have ALS are lacking. While it can affect young people, ALS more commonly strikes in middle or older age.
The association worries about misdiagnosis — and missed diagnosis, Frederick said. It's possible that earlier diagnosis would mean more options. And there's great interest in learning risk factors in hopes of prevention. "The key to unlocking it is finding a biomarker that ALS doesn't yet have. We will get there, hopefully. Doctors don't over-diagnose, but rather under-diagnose the disease."
Frederick believes there's a reluctance to deliver the diagnosis. "Because there's not a specific biomarker, they may hope it might be something else."
Military service-related cases
One of the biggest mysteries is why people who serve in the military are diagnosed with ALS at a higher rate than the general population.
Congress designated ALS a service-related disease in 2008. That means veterans get benefits others might not get in terms of help with care or needed medical equipment.
Teresa Thurtle, of Las Vegas, knows all about the genetic risk — and has learned about the increased risk associated with military service, too. Her father and her grandmother both died of ALS at age 49. She's acutely aware that's just 10 years older than she is now. Her grandmother lived with ALS for three years after diagnosis, her father just nine months. She carries the gene. And she served in the Air Force, so she's taking it seriously but she's not letting it keep her up at night.
She thinks people are so focused on finding a cure that they are ignoring preventive medicine.
If you have the gene, she notes, it doesn't mean you will get the disease or pass it on. She's heard there is a 50/50 chance of inheriting the gene, but the chance of developing ALS could be higher or lower. The military connection puts her in a higher-risk class, said Thurtle, who teaches political science and government online at Gulf Coast State College in Florida.
Ashley Lee, 38, of Winter Garden, Florida, lost her dad Darrell Lee in October to ALS. He had been in and out of the hospital many times. He was also an Air Force veteran who served in Vietnam.
Lee, a single mother, has raised her stepdaughter Kaylei, who she legally adopted. Her husband died in a motorcycle accident in 2013. She and her daughter had been dedicated in caring for Lee's dad.
She is working on a doctorate in psychology and intends to work at a Veterans Administration hospital with ALS patients and families, since she knows how difficult it can be. In the meantime, she serves on a "boatload" of patient advisory boards, is an Elizabeth Dole Foundation fellow and is the Northeastern ALS consortium ambassador.
When her dad, who raised her alone, was diagnosed, she said she put all her fear and grief — and there was a lot of it — into finding out what would help him. She wants to help others with that, too.
Sean Cunningham understands Lee's yearning to help those struggling with medical challenges. He said were he able, he would take what he's learned as a patient and put it into practice with the work he did, focusing on a "smooth handoff" to connect patients with services. Not just telling families how things in health care might work, but helping make it happen. That's often missing in health care, he said. He's seen people fall through the cracks and his illness has made even clearer how important it is to help families find resources. He'd work on explaining things in ways that the families could understand clearly.
He'd also help schools better understand what families need, he said.
Systems to get care were set up for able-bodied people, according to Sean. Recently he was asked to fill out a medical form. He explained that he can't use his hands and that is why he was asking for help. The woman told him she couldn't help him until he filled out the form. That was a maddening conversation.
Whitney can fill it out. But what about people who don't have a Whitney to act as an informal aide? he wondered.
While he's personally delighted that vets get lots of assistance with ALS treatment, equipment, etc. — "Vets deserve it in spades!" — he wishes more help was available to others with ALS, as well.
Looking ahead
While they lose the ability to talk and eat and breathe — eventually needing a ventilator — those who have ALS don't lose their ability to think. In the last days of his life, Lee's dad communicated by closing his eyes for yes, and his daughter always nearby to act as his interpreter. She finished her master's degree thesis sitting by his bed.
Someone with ALS eventually "may not be able to move eyes, talk or communicate at all. Still, they are 100% in there and still have mind clarity. People assume they can't feel and don't understand. They do," Lee said.
While the young Cunningham boys don't understand what the future holds, Colin and Ian like to help their dad, giving him a glass of water or opening jars. "They take pride in it," said Sean, a tall, strongly built man with ginger hair and a very direct gaze. He speaks slowly but clearly, the fact that there's effort involved because of ALS only slightly noticeable.
Eventually, he'll be too weak to talk and his life will largely be internal, one of thoughts instead of action. Already, his mobility is divided between using a wheelchair and a walker and he's experienced the stereotyping some people do when they see someone with a disability. Strangers often talk down to him or ignore him.
Whitney says they're trying to focus on the hidden blessings. They have been given some time to prepare for what's coming, which many people don't have. End-of-life plans are largely in place.
They're also learning that they have some really good friends who are very specific with their offers to help, rather than offering a noncommittal "let me know if I can do anything." Those folks are usually busy if you actually ask, Whitney said, adding she often doesn't know what she needs because this challenge can feel overwhelming. A co-worker sent her son and his friend over to hang their Christmas lights without being asked recently — a huge blessing that boosted everyone's spirits. Another friend called and told her to put the laundry on their porch. "I want to do your wash and will bring it back."
She gets emotional talking of such goodness.
When it became obvious that their house would need renovation so Sean can get into the bathroom by himself, they were outgunned financially. They were stunned and touched by the support they received from a GoFundMe page a friend set up. Without outside help, they couldn't do it. Right now, they're living with what sounds like an endless supply of buzz saws as workers create a roll-in shower and demolish then rebuild the backside of their home to connect the bedroom and bathroom and do other repairs that will make the home livable for him as ALS progresses. The construction chaos itself is a reminder they are loved.
They've experienced a lot of support from the kids' school, from colleagues and friends. That's been hard for Sean, who says he always did things on his own. But he's grateful.
"This whole experience has opened my eyes to the kindness of others. I feel like I owe them something," he said. "On the flip side, I realize how much conflict there is and it all seems trivial." His wife nods and adds, "If the worst part of your day is someone parking in front of your house, you have it good."
Sean plans to enjoy every single moment he can draw a breath — even moments that might earlier have frustrated or annoyed him. "This has reshaped how I view the present," he said. "I view it one moment at a time and cherish every aspect."
Like most folks, he sometimes complained about what the kids were doing or about irritations at work. Now he would trade everything to have more of all of it. "This makes me realize what I have and how fortunate everyone is," he said.
An avid outdoorsman, Sean compares ALS to backpacking, which was one of his passions. When friends asked why he loved to strap on a heavy bag and wander, he never had to think about his answer. "You come across novel situations when you're backpacking and have to invent ways to overcome obstacles."
That sounds very familiar to a man with ALS. It can be frustrating, but every success is very gratifying, he said.
As he talks, Whitney occasionally wipes her eyes as she cuddles the family's ginger-colored maltipoo, Archie. She admits that she is frightened. Raising their children will be her task. And Sean is worried he won't be able to do enough, to give enough, to teach enough, to ensure that everything will be all right when he's gone. "I am sure there will be a domino effect," he said. "I feel strongly I need to always take care of my family. With this disease, I feel I am being dismissed from that role. There are many unknowns."
So he's trying to give his family its own smooth hand-off. He likes things organized and well-planned and this disease seems to be anything but that. Still, he is doing his best.
This Christmas, the tree has moved, but they are keeping what they can the same, honoring beloved, comfortable traditions.
He's still skiing with his boys, though in an adaptive ski program that helps people with disabilities and probably not for as long as he would hope.
This past January, before his diagnosis, the family had an amazing time at Disneyland. There are memories he hopes his children will keep as they get older.
He doesn't avoid talking about the time, coming way too fast, when he won't be able to swallow or talk or move any part of his body. He's sure he's been cheated of 40, maybe 50 years. But he's lived well with the time he has had, finding meaningful work, backpacking part of the Appalachian trail, loving a good woman and his kids with all his heart, he said.
Faith has played a huge part in his past and his future. He calls the support of Capital Church, which they attend, "wonderful." In September, he went home to Michigan "essentially to say goodbye to family and friends." He got wobbly and fell and ended up in the ICU there. Back home, he fell again and was hospitalized. He is becoming decidedly more frail. But his ties to people just get stronger.
It's a journey with challenges. And like backpacking, victories, too, Sean Cunningham said.
