A vibrant life

The disease that will eventually end Jon Ferraiolo’s life showed up first in his throat and his tongue. Recovering from a lingering cold at the time, he attributed his difficulties with swallowing and his slurred speech to that ordinary ailment.

But 15 months later, in July 2014, a doctor gave Ferraiolo a devastating diagnosis: amyotrophic lateral sclerosis (ALS), commonly called Lou Gehrig’s disease. The progressive neurodegenerative illness affects nerve cells in the brain and the spinal cord, which lead muscles to stop working and to atrophy. After his speech became slurred, he lost use of his fingers, hands, arms and legs.

Eventually, he will stop breathing.

But the disease does not affect the software engineer’s mind. He is fully aware of everything around him.

More than 20,000 Americans have ALS at any given time, according to the ALS Association. Famed theoretical physicist Stephen Hawking died of the disease on March 14. Other notable persons who were afflicted include baseball great Gehrig, pitcher Jim “Catfish” Hunter, U.S. Senator Jacob Javits, actor David Niven, singer Dennis Day and jazz musician Charles Mingus, to name a few.

“He has a pretty terrible disease. For the most part, he faces it with courage and dignity,” his wife, Karen Kang, said recently in their Palo Alto home.

In fact, ALS isn’t stopping Ferraiolo, 64, from leading a full life. Not knowing whether he has months or years left, he and Kang decided to transcend ALS as best they could. They have found acceptance, peace and happiness, they said. Life is marked not by drudgery but by milestones: the birth of a grandson; the weddings of two daughters; the publication of a book that he hopes will change the world; visits and celebrations with friends. With the help of technology, caregivers, family and friends, Ferraiolo is still doing what he wants to do and said he enjoys each day.

Ferraiolo forms his words slowly now, using his breath to help coax out the sound.

“My joke about ALS is: Surrender your pride and dignity. After that it’s pretty easy,” he said.

Hear Jon Ferraiolo and Karen Kang tell their story in their own words in a documentary film by Weekly Photographer/Videographer Veronica Weber.

When symptoms started to manifest in 2013, the couple went from specialist to specialist seeking an answer — an ear-nose-and-throat physician, a few neurologists. One doctor thought Ferraiolo had gastric reflux.

They even went to a singing teacher when someone suggested that the mechanics of singing and breath control would help him to project his voice.

“We were on a wild goose chase,” Ferraiolo said.

A neurologist specializing in neuromuscular diseases at Stanford University Medical Center’s Neurosciences Center delivered the diagnosis.

“She did further testing and sat us down and said, ‘Has anyone talked to you about ALS?’” Kang recalled.

“We just looked at each other. And I said to her, ‘How sure are you of this?’ And she said, ‘About 70 percent.’

“At that point, I’m looking at Jon and I’m trying to say (to myself), ‘Don’t fall to pieces. Don’t start crying and sobbing because Jon will think that there is no hope, and there’s a 30 percent chance that this diagnosis isn’t right,’” she said.

When they got home, they let the diagnosis sink in.

“We just sat together holding hands and crying. It was really a very devastating diagnosis,” Kang said. “They didn’t tell us anything about what to expect. We had seen the movie about Lou Gehrig, so we sort of knew what happened, and I read something online about it.

“It was just really scary. How are we going to manage this? What’s going to happen? What is life going to be like?” she said.

Ferraiolo recalled considering how the disease would affect Kang and their children.

“For me, it was much easier than for you. I know things happen — when you get older things happen. My ticket was ALS. Other people get cancer or Alzheimer’s. I thought, ‘Well, I’ve had 60 years of great luck.’ I worried more about the women in my family than me. And they take it much harder.”

Kang had already planned a 40th wedding anniversary trip to France for that September. They wanted to go to the place where they met during the Stanford-in-France overseas-study program and to visit the cow pasture where they had camped out during their first trip together. So they did. Ferraiolo could still walk 10 miles a day then and carry his luggage, she said.

A year later, bringing three pints of coconut ice cream home on his bicycle, Ferraiolo lost his balance and did a faceplant on the pavement. The disease was progressing.

“The voice went first. The second thing to go was my thumbs. I only had four fingers working on each hand. Then the arms went and now the legs,” said Ferraiolo, who’s in a wheelchair.

He’s able to rotate and move his chair using specialized headgear activated by the sides of his head.

“It’s been glacial, very slow. Three and a half years after the diagnosis, it’s been a pretty linear decline,” he added.

‘For the most part, he faces it with courage and dignity.’                                -Karen Kang, wife of Jon Ferraiolo 

“He had a slow decline and then stability, and then it felt like he fell off a cliff” in May 2016, Kang said. Though he’d been compensating for the loss of motor control in some muscles by using others, eventually those too gave out.

Suddenly, he couldn’t get on the Pilates equipment at his private sessions and he couldn’t handle their regular walk to Starbucks. He had to quit driving.

“I lost a whole bunch of functioning seemingly quickly,” Ferraiolo said.

When they met at the Stanford-in-France program in September 1974, Kang was a 19-year-old Mills College student majoring in English and Ferraiolo was a Stanford University computer-science undergraduate. She grew up on a farm in Gresham, Oregon; he was raised in relative affluence in suburban Chicago, the son of a doctor.

Their worldviews were initially as different as their backgrounds.

“Jon and I would argue about life philosophy and attitude. I’ve always been a sunny person with a positive attitude, and when I met Jon he had kind of like this darker side to him. He said he was kind of prone to depression, and he said it wasn’t good to be happy all of the time or to try to be happy all the time.”

Ferraiolo thought that being mildly depressed helped temper stronger emotions. It meant one didn’t get too excited about things.

“You wouldn’t get too depressed. You could kind of control where you were,” Kang recalled.

“I thought that was the most ridiculous life philosophy,” she continued. “I said, ‘It’s much better to be happy, so why don’t you try for happiness? And there’s so much to be happy for, and there’s so much to be grateful for. Depression should not be the rule but the exception.’”

They could not have known how much her way of thinking would make them resilient when he developed ALS.

Although they were falling in love in France, the relationship didn’t last. She returned to a boyfriend in the States; he eventually reunited with an old girlfriend.

“But she broke up with me. I couldn’t stop talking about Karen,” he said.

In fall 1975, they met again in Woodside at an overseas-program reunion. Much to her surprise, Ferraiolo came with a new attitude.

“He had found a way to be happy and to like himself or love himself, and I felt like ‘We’re two equals.’ Before, when we were in France I felt like he was dependent on me for his happiness, and I told him I wasn’t willing to take that burden on. It would mean that I could never be sad,” she said.

It was a conscious decision, he said: “I listened to her argument and concluded that she was right. It made no sense to be unhappy.”

They married and had three daughters, Nicole, Natalie and Allison, filling their lives with soccer games and breaking up sibling rivalries.

“Jon was a marvelous father and a marvelous husband and partner. He changed diapers and helped cook and clean,” Kang said.

They also had demanding careers: she as a branding strategist for high-powered Silicon Valley people; he as a software architect, engineering manager and product manager on Adobe products such as Illustrator, Acrobat and Premiere. He became known for developing the Precision Graphics Markup Language, the original Scalable Vector Graphics language specification (a standard for non-blurry text and graphics), and as a major contributor to the EPUB standard for digital books.

By the time he was diagnosed with ALS, his speech difficulties were making long phone conversations at his job at IBM impossible. He went on disability as soon as he received the diagnosis and started taking care of his affairs.

Kang pulled leg warmers over Ferraiolo’s feet and up his calves as he lay immobile in bed on a sunny March morning.

“These are the leg warmers that dancers use. Like in FlashDance. We need to get one of your sweatshirts and I’ll cut it” to have that sexy off-shoulder look, she said.

“I’m a maniac,” he replied.

“You’re a maniac? You’re going to do that dance?” she asked.

She had added an electronic door chime to an ankle cuff that Ferraiolo wears, so he can ring the bell using his other heel whenever he needs help.

Kang turned him on his side and put a cloth sling underneath her husband to lift him from the bed. Pepe, their chocolate-colored miniature poodle, barked loudly.

“I’m just at everyone’s beck and call,” she said, smiling. “It’s alright. It’s alright, I’m used to it.”

The mechanical lift, shaped like an upside-down U with handles, straps and a sling seat, whirred over the bed. The device lifted Ferraiolo, who is 5-feet-8-inches tall and weighs 180 pounds. The metal arm swung him to his wheelchair and the lowered him into the seat. Kang unhooked the sling and gently slipped it out from under him.

“Do you want your kilt or shorts?” she asked. His sister, Bobbe, made the kilt, which allows him to move more freely than if he is wearing pants.

Kang wheeled Ferraiolo to the door, now dressed and with Pepe on his lap.

“You know, between Jon and Pepe, they keep me going,” she said, attaching a leash to the dog as they prepared for a walk. Pepe whined anxiously and bolted for the door, taking the lead. Kang guided the wheelchair down the street in their Duveneck/St. Francis neighborhood.

Construction workers renovating a nearby house banged loudly as the couple strolled past verdant landscaping in the dappled morning light.

Kang struggled to keep the wheelchair level around a sloping driveway. Curves are the hardest.

“There’s one thing I’d like people to understand — that they park too far up on the sidewalk. Especially with the motorized wheelchair, you can’t get through,” she said.

She stopped momentarily. The tulip magnolia petals had fallen, littering the ground around the wheelchair with pink blossoms.

Ferraiolo took in the beauty of the plants and flowers as the couple rounded a curve. He recited a William Wordsworth poem, “I Wandered Lonely as a Cloud,” with Kang joining him, and they smiled.

“I wandered lonely as a cloud

That floats on high o’er vales and hills,

When all at once I saw a crowd,

A host of garden daffodils;

Beside the lake, beneath the trees,

Fluttering and dancing in the breeze.”

As ALS has made it difficult for him to travel, he has had to rely more and more on his “inward eye” and his memories to give him joy, Kang said later, recalling the final verse:

“For oft, when on my couch I lie

In vacant or in pensive mood,

They flash upon that inward eye

Which is the bliss of solitude;

And then my heart with pleasure fills,

And dances with the daffodils.”

The couple used to go to Edgewood Plaza Shopping Center for coffee, but Kang doesn’t like to drink it in front of Ferraiolo anymore. He can no longer take liquids by mouth, and he misses the taste of coffee, he said.

One joy they’ve added to their life since the diagnosis is Pepe, who is part of Ferraiolo’s therapy, lifting spirits and bestowing affection. Kang calls Pepe their designer dog: She asked a breeder for a pet with characteristics that would complement Ferraiolo’s condition, and as a result, Pepe is strong enough to jump on his lap, is gentle enough to be a good lap dog and possesses a lively personality.

“Pepe has been such a godsend. He wakes Jon up in the morning with kisses,” she said.

Back at the house, Kang donned medical gloves. She brought out two plastic bags containing solutions of Radicava, a drug that can help some ALS patients by slowing their symptoms. Ferraiolo started the treatments in December, late after the onset of his disease, so it isn’t known what benefits he will derive.

He said he feels less tired, his breathing isn’t as difficult and he isn’t twitching as much all over since he has started the Radicava.

“I used to have twitching in my hands all of the time. You could feel your hands moving, but it was a disconnect. You couldn’t stop the tremors,” he said.

He lost 25 pounds in a year because of them, he added.

Kang straightened tubing and inserted it into one of the bags. She hung it from a stand, adjusting the drip flow as Ferraiolo watched television. She attached the line to a port embedded in his upper chest. It will take about 20 minutes for the treatment to finish, and then they’ll start the second bag.

Meanwhile, she attended to another procedure to clear his lungs of mucus. Setting up a nebulizer that can push air deep into his lungs, she filled a plastic reservoir in a face mask with drugs and placed the mask over his nose and mouth. The nebulizer machine whirred, spreading the medicine in a fine mist as he breathed, opening and relaxing Ferraiolo’s lungs.

It was also time to eat. Ferraiolo cannot drink liquids, so Kang feeds him medicines and additional nutritional supplements through a port embedded in his stomach. She crushed the medications and added liquid to another container, stirring to dissolve. She raised Ferraiolo’s shirt to access the port.

“It’s just a little button like this. You just open it,” she explained.

She attached a tube and used a large, cylindrical syringe to flush his line with water. She then poured the medications and nutritional supplement, a thick, pallid, milkshake-like substance, into the syringe. The liquid flowed directly into his stomach.

“Chocolate cake? Baked salmon? What are you eating?” she said, as the liquid disappeared.

“I don’t taste or even feel it,” he said.

They keep CNN on with closed captions because the nebulizer drowns out the sound. When the device finished dispensing the medication, she hooked Ferraiolo up to a cough-assist machine. It exercises his lungs to make them stronger by pushing in air and helps dispel mucus. He exhaled loudly into the mask, and Kang measured the amount of exertion on a meter. It tells her if he is meeting targets for keeping his lungs strong.

At night he wears a ventilator, a non-invasive device that pushes air in and out of his lungs.

“It waves my diaphragm muscles. I get short of breath at night if I don’t have it. I sleep better. I used to wake up many times in the night because my breathing would stop,” he said.

Recently, they were able to add a computer screen above his bed so he can be entertained while on his back. He used to wake up in the night with nothing to do for hours, unable to move or get up on his own. It was boring, he said.

The computer screen uses eye-gaze technology to allow him to surf the internet. The device uses infrared light to read his eye movements. He uses the same device on his computer. He can type by moving his eyes across the monitor. He wrote an entire book, the self-published “Holy War for True Democracy,” using only his eyes.

“I can type with my eyes as fast as people do with their fingers,” he said.

‘Three and a half years after the diagnosis, it’s been a pretty linear decline.’                                                     -Jon Ferraiolo

At mealtime recently, Kang sat down next to him in the kitchen. Forkful by forkful, she fed him: scallops, stir-fried tomatoes and onions, sweet potatoes.

Ferraiolo ate hungrily; often he doesn’t have the energy to eat as much as he needs. He stops eating when he is tired and not when he’s full.

“It’s really rough when the food is really good. You want to keep eating,” he said.

Kang ate her dinner in between feeding Jon his mouthfuls.

“A lot of times I’ll sit here and eat with Jon. I’ll take a bite and he’ll take a bite and I’ll take a bite.”

Kang said she feels fortunate that they can afford help. Caregivers come in the morning to wash, shave and dress Ferraiolo; a masseur gives him therapeutic massages that help keep his muscles from freezing up. She squeezes in personal time or work when they are there; in the afternoons, she takes over caregiving.

“One of the hardest things is the everydayness of it. I’m a very creative person and I like to have a lot of change in my life and do different things. And yeah, it can wear you down a bit,” she said.

Ferraiolo gives Kang space and time to herself. He’s told her to take a vacation, to go off with her sister or to hire more caregivers. She takes line-dancing, goes to an exercise class and walks with friends.

She also receives mental health support.

“I had never seen a therapist before, but a friend whose husband had passed away from ALS said that she would like for me to see a therapist — not because I might think I need it right now — but to find a therapist before you feel like you are desperate and you need to talk to someone. There’s a tendency to put a happy face on, and you don’t often show everything that’s going on in your life. And sometimes, if you just keep pushing all of this stuff down and you never let it surface, it can have bad consequences,” she said.

It’s important for not only Kang to have a life beyond ALS but also for Ferraiolo, she said.

“I know that people so often will just kind of put him in a box because he’s a person with ALS. But he has many interests beyond ALS, and he’s a very intellectual person and likes to engage, so with Jon I’d like people to see that he’s … a person with a personality and a history and a future.

“I would wish that people would see me in the same way,” Kang continued, “because sometimes people come up to me — and everyone’s well-meaning — but there’s a sad face that approaches you and, like, that your life is really terrible and (they’re) commiserating and feeling bad or sorry for you. And for the most part I don’t feel sorry for myself.

“There are days, yes, that aren’t going so well. Every once in a while, every three months, I can go in the closet and I’ll have a good cry and just kind of wallow in self-pity for an hour. But beyond that I don’t really pity myself and I don’t really look for other people to pity me or to pity Jon or our life together because there is a lot of joy in life,” she said.

Ferraiolo reflected on living with ALS.

“We both have blessings and burdens. Even people who are healthy have burdens like family issues, emotional issues, and employment issues, financial issues. I’ve got problems; you’ve got problems. Everyone has problems. I’ve got another problem with ALS, but I’m not different than anyone else. Everyone has issues. But because I have ALS, I don’t have to work anymore, so I have less stress and I can focus on experiencing love with family and friends. And I also think life is quite amazing, if you take the time to pay attention. So I have no regrets. I think I have the burden of ALS and the blessings along with it.

Ferraiolo was talking almost nonstop on a warm day this past April. Someone told him he was talking too much and that he’d use up all of his energy. But it was an exciting day: Daughter Natalie was getting married.

In his bedroom, the caregivers helped to dress him for the wedding to be held in their backyard. They put his arms through the sleeves of a freshly pressed white shirt and buttoned his cuffs. Underneath, he wore his “The Greatest Dad in the World” T-shirt, which Natalie made for him when she was young. The print is now faded from many washings.

In the garden with Pepe wearing a tuxedo and dozing on his lap, Ferraiolo escorted Natalie down the aisle as Kang pushed the wheelchair.

The wedding was the latest in a series of milestones.

“Since he was diagnosed, our oldest got married. She just had our first grandchild in December. Also in December one of our twins, Allison, got married. In April, the second twin, Natalie, got married,” Kang said this summer.

After grandchild Theo was born, Ferraiolo and Kang made the arduous trip to Washington, D.C., to visit. It was their last trip, and it was tough. He developed a lung infection and spent miserable hours on the plane sitting in an awkward position. No one was strong enough to straighten him up. They had to lug a wheelchair and his breathing equipment.

But now Nicole and Theo come to visit frequently. They put the infant in Ferraiolo’s lap and fold the new grandfather’s hands over the boy’s tiny body.

Some people hide away when they get the diagnosis of a degenerative disease. But Ferraiolo and Kang want people to know there are many more good times than bad, they said.

It takes reaching out to people sometimes — to make one’s needs known and to help others feel comfortable.

The more people can talk about an illness, the less there is stigma. When people are confronted with something totally foreign, there’s a tendency to stare, Kang said.

“You read about how ALS patients are trapped and imprisoned in their bodies. People think that must be terrible. … It’s very hard for people to understand how it is possible for someone to be paralyzed and to be happy,” Kang said.

“I’ve talked to people with breast cancer and Parkinson’s disease who are just shut-ins, and no one comes over. It doesn’t have to be that way. It may be years of living with the condition. Are they just going to be hermits? You have to open up the blinds and let the sunshine in. It’s not healthy for the person, the family and society,” she said.

Every month Ferraiolo and Kang have multiple visitors. Sometimes guests stay overnight and sometimes they come for afternoon tea.

“I would just encourage people, if they do have a loved one with an illness and that loved one feels comfortable about it, let everyone in the family know that it’s fine to visit,” Kang said. “I just think that there’s no shame in having an illness. It’s not something that you did to make this happen. It’s not a poor reflection on your life. It just is. And all of us have something going on in our lives.

“Every time I look around, you know, there are friends with issues — health issues with parents or children or other loved ones or partners — and I just think that it takes a village. Not only to raise a child but also just to be and to live,” she said.

All of the little things that people worry about, that they’re concerned about, all of those things don’t really mean anything because life’s all about enjoying and living to the fullest in the moment that one is in, she said.

‘I want people to know that you can live in the moment with someone that you love who has ALS.’                           -Karen Kang

“It’s not wishing for something in the future, not just thinking about the past and how maybe the past was so great, but truly living in the moment. I want people to know that you can live in the moment with someone that you love who has ALS and find great joy in that, that just because someone has a disability or is not the same person in terms of their capabilities, they really are the same person inside. And hopefully that’s the same person that you fell in love with. And that person is still there.

“Even if their voice changes and they can’t walk anymore or they can’t hug you anymore, they’re still that person, and they still love you the same. And certainly I still love Jon the same, or maybe even more, because we have time to think about what we have, and hopefully we’ll have a lot more time in the future.

“But you never know that. You never know that, if you have ALS. You never know that if you’re a perfectly healthy person who walks outside, crosses the street and has an accident and is no longer around. So I think all of us should strive to live every day like it’s your last day,” she said.

Ferraiolo and Kang recalled a trip they took to Hawaii in 2017. The disease had progressed significantly. It was April Fool’s Day, and he was in a rented floating wheelchair, relaxing as foot-high waves lapped at his legs. A 3-foot sneaker wave suddenly tipped the chair over, knocking Kang, who was on one side supporting the chair, off her feet. She was pinned under the chair, and the weight broke her leg.

Ferraiolo was face down in the water, his face buried in sand. He couldn’t breathe and he couldn’t move.

He wondered, after all he had been through with ALS, if this would be the end — if he would die on a vacation island, smothered by sand and water. One way or the other, he accepted his fate in that moment, he recalled.

He’d had a good life with no regrets.

But it was not the end for Ferraiolo. Two burly Hawaiian men came to the rescue.

When they lifted Ferraiolo up and everyone began frantically brushing the sand out of his nose and eyes, they found him laughing.

Weekly Staff Photographer/Videographer Veronica Weber contributed to this story.