Living with ALS

One of the top stories in 2014 was the Ice Bucket Challenge for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease. But this was more than just a social media phenomenon for 43-year-old John Wilde of Carthage, Ill.

The concept was simple: Post a video on social media of yourself being doused with a bucket of ice water and challenge others to either do the same within 24 hours or “forfeit” by making a monetary donation. Not only did this raise money for ALS but also raised awareness about a devastating neurological condition for which there is no cure.

For several weeks in late July and August, the Ice Bucket Challenge generated more than $200 million in donations for the ALS Association and its affiliated chapters across the country and around the world. An online viral sensation, it drew worldwide attention to ALS while offering encouragement and hope to families of patients with the disease.

John and Holly Wilde

John and Holly Wilde

John first noticed something was wrong in February 2013, when he stumbled and fell while attending his son’s basketball game. He dismissed it, but over the next several weeks other signs emerged—unexplained fatigue, weakness in his hand, and numbness in his arm. He also fell on several other occasions, as if his legs had suddenly failed him.

Over time, “I had to really concentrate on being able to walk, putting one leg in front of the other,” he says. “Unexpected. No reason.”

John went to his local doctor and then to Mayo Clinic, where he was diagnosed with ALS in April 2013. The disease attacks nerve cells that control voluntary muscle movements. Over time, the muscles weaken, causing a wide range of disabilities and loss of movement. When muscles in the diaphragm and chest wall fail, patients lose their ability to breathe without support from a mechanical ventilator. Most ALS patients die from respiratory failure, usually within a few years after symptoms appear.

For John, his wife, Holly, their three children, and their extended family and friends, it was a stunning new reality.

John was encouraged to begin regular visits at an ALS clinic, where he could get follow-up care and he and his family could get the information and emotional support they’d need.

They chose UI Hospitals and Clinics, two hours from their home in Carthage.

“It’s closer for us than Mayo (in Rochester, Minn.), so that was a factor,” Holly explains, “but also, our families have gone to the UI for medical procedures, so we’re very familiar—and very comfortable—with Iowa City. We felt like it was a good place for us.”

Three weeks after John’s diagnosis, the Wildes had their first of every-three-months appointments at the ALS clinic at UI Hospitals and Clinics.

“They talked us through what would happen and what to expect,” John says. “They helped us put a game plan together—bucket lists, things we wanted to do together. They helped us approach it from the perspective where it’s not just affecting me; it’s affecting everyone who’s in my life.”

“We’ve fallen in love with the people at Iowa. Dr. (Andrea) Swenson and (nurse clinical specialist) Erin Springer have treated us like family,” Holly says. “Even though you know things are bad and you can see things deteriorating, you never get the impression that it’s hopeless.”

Physically, John didn’t appear affected by ALS for the first months following his diagnosis—he could still move and walk on his own. But by February 2014, he needed braces for his legs; eventually, he switched to a walker, then a motorized wheelchair. By summer, John’s breathing ability had declined considerably. He began using a BiPAP (bi-level positive airway pressure) machine to assist his breathing while sleeping as well as a cough-assist device to help him clear his lungs of mucus and reduce the risk of respiratory infection.

By late August 2014, tests showed that John’s breathing was getting worse. His weakened diaphragm muscles prevented him from exhaling carbon dioxide, which made inhaling oxygen even more difficult.

Andrea Swenson, MD

Andrea Swenson, MD

John’s medical team, led by Andrea Swenson, MD, clinical assistant professor of neurology, suggested that John participate in a clinical research trial of a diaphragmatic pacing system (DPS) that could potentially slow down the progression of diaphragmatic weakness. The NeuRx Diaphragm Pacing System™, approved by the Food and Drug Administration for use in ALS patients, has been used for years to assist patients with spinal cord injuries. The goal of the multi-center trial, Swenson notes, is to determine if it could help ALS patients, as well.

“We’re interested in how the DPS will affect a patients’ respiratory function over time—whether it reduces their need to use a BiPAP, whether it improves patients’ quality of life (by making breathing easier), and whether the system ultimately impacts patients’ length of survival,” Swenson says.

Placing the DPS can be done with traditional “open” surgery or, preferably, using a minimally invasive laparoscopic surgery, says Hui Sen Chong, MD, clinical assistant professor of surgery at the UI and the research team member who performs the placement procedure.

Hui Sen Chong, MD

Hui Sen Chong, MD

“With the laparoscopic approach, we make four small incisions to implant four electrodes in the diaphragm,” Chong says. “The electrode leads are connected to an external pulse generator (EPG), a small device that sends electrical signals to the diaphragm.”

These replace the disease-weakened signals normally sent by the brain along the nerves. The new signals cause the diaphragm to contract, which exercises the muscles.

Swenson notes that ALS patients must be at a specific stage with their disease to qualify for the study.

“We do tests to determine if the patient’s diaphragm has reached a certain level of weakness to see if they qualify for the study,” she says. “But the diaphragm can’t be too weak, either, or we won’t be able to stimulate it and see if the device has the potential to help.”

Tests showed that John was indeed a good candidate for the study. In October 2014, he underwent the DPS procedure. Since he’s had the system in place, John says the pacing has gone well.

The DPS study is just one example of clinical trials available to patients at UI Hospitals and Clinics, Swenson says.

“When people talk about the ‘academic difference,’ this is what separates us from other hospitals,” she says. “We’re trying to get answers to questions about ALS and other diseases and offer these types of treatment options to patients.”

For the Wildes, participating in the DPS trial offers the possibility of extra time.

“Obviously, there’s a clinical research aspect to this—to see what kind of impact this system will have for patients in the future,” John says. “But I’d be lying if I said I was only doing this for experimental purposes and the greater good. It’s a dual purpose. I hope to be able to extend my life but also give back.”

“The most important thing is to gain time, so that John can be here as long as possible to see our kids (Hunter, 14, Forest, 11, and Sage, 9) continue to grow,” Holly says. “And also the hope that this will make things easier for him so he doesn’t have to struggle to breathe. Also, you never know who this (disease) is going to affect next. So if any of our friends or family were to face something like this, perhaps this study could help give them hope.”

Hope, like what the Wildes felt this past summer while attending a girls’ softball game in Unity, Ill., near Carthage. They watched as the players did a team version of the Ice Bucket Challenge. John asked Holly to push his wheelchair so he could thank their coach for the team’s support of ALS awareness. Moments later, the team was thanking him for his effort and asking the Wildes to pose for a group photo.

“They put it up on their Facebook page. That had me smiling for several days,” John says.


This story is also appearing in Health at Iowa.