Doyel: ALS patients need help — and hope — now

Originally published in the Indianapolis Star by Gregg Doyel

At the end, Georgia wouldn’t look at her children. For weeks they’d been rolling her wheelchair to the living room window in French Lick, where she could look into the yard and see what they were doing out there, but at the end they left her in her bed, in her room, where she just wanted to die.

And her children wanted it for her.

Georgia was suffering from ALS, which had presented itself six months earlier as weakness in her arms, then legs, then everywhere. ALS doesn’t always move this fast – average life expectancy is 2 to 5 years after diagnosis – but it burned through Georgia like an electrical fire. Pretty soon she couldn’t eat, talk, breathe. The ALS was shutting off her muscles one at a time, leaving her brain alone, leaving her to understand exactly what was happening to her.

At the end, her children would come into her room and she’d close her eyes. They knew she was awake because of the tears running down her face.

The fourth of her six children, he visited every day. He’s the one who took her to the doctor, received her death sentence, watched as the doctor shared that fate with his mother. At the end he saw her cry. And he watched Georgia Bird die.

And Larry Bird, he can’t forget any of it.

“It’s been almost 20 years,” he says. “ALS is a brutal, brutal disease. It’s horrific, and nobody beats it.”

Doctors and scientists are working on new drugs, new technologies to combat ALS, but they are racing time, and every day another 15 or 20 Americans die of ALS. This battle, it’s impossibly difficult.

“ALS is a brutal, brutal disease. It’s horrific, and nobody beats it.”

-Larry Bird

And too often, the government and the insurance companies are standing in the way.

Her name is Maureen Burakiewicz, she’s one of my best friends, and she’s fighting ALS in Greenwood. She’s fighting it so hard.

Unlike some ALS patients who eventually decide to quit fighting, Maureen – I call her Mighty Mo – would do anything to extend her life. To hold off atrophy she walks the Greenwood Mall with her husband, Jim, who wraps a rubber band under her shoulders and catches her when she falls. She eats as much as she can, even though her daily pills fill her up like pebbles in a small jar. She was chewing medicinal marijuana gum – there was a rumor it might help – until she could no longer chew.

Maureen, 59, has two grandbabies to love. She has a daughter, Ashleigh, to watch marry Indy Eleven executive Brad Newbold in October. She has a husband, a Widia tool salesman approaching retirement age. They had dreams.

“It’s a nightmare,” Jim says.

They’re not fighting just ALS, which presented itself in December 2013 as weakness in her right hand and slurred speech, and now has Jim carrying Mighty Mo up the stairs and feeding her through a tube near her sternum. They’re fighting Medicare, their insurance company and the Food and Drug Administration.

“It’s so hard,” says Jim Burakiewicz, 60. “And (the bureaucracy) just makes it harder.”

There is hope – and for an ALS patient, hope is a miracle. But the FDA is taking too long to move on this hope, this chance, this only chance for Maureen to hold off ALS. It’s a drug called GM604, it was made by small California company called Genervon Biopharmaceuticals, and it has shown encouraging results in limited testing. To date Genervon has tested GM604 on a few dozen patients and has reported a significant slowing of ALS’ awful progression.

There are ALS patients who want to take GM604, and they want to take it right now, but a decision on its approval could be a decade away. Despite calls from ALS advocates and a massive email campaign to U.S. senators and an opinion piece in the New York Times, the FDA hasn’t put GM604 on an accelerated track that could lead to a far quicker decision. And until the FDA approves it, Maureen can’t take it.

“What does she have to lose?” Jim Burakiewicz says.

Across town I tell Larry Bird about Mighty Mo, and about GM604, and the president of the Indiana Pacers purses his lips and nods. He understands their desperation.

“If there was a drug out there, I guarantee my mom would have done it,” Bird says. “Even if it didn’t help her, it could have helped somebody else down the line. It’s a brutal, brutal … knowing you’ve got to fight that for six months, it got to the point where she goes, ‘I’m done.’ The best thing to do is just die. There was no hope.”

There is a glimmer now, but the FDA stands in the way. Gov. Pence signed the right-to-try bill last week, making Indiana the ninth to pass the legislation. The law gives terminally ill patients the opportunity to try drugs that have cleared the first phase of the FDA process.

Without FDA approval, though, there are insurance companies that wouldn’t cover it, making GM604 too expensive for most people.

Maureen is looking for more than a medicinal Hail Mary. She’s also looking to communicate. ALS attacks everyone differently, and it came after her throat. She went from slurring her speech – ALS is so horrible, Maureen’s family was hoping it was a brain-killing stroke, not ALS – to being unable to make noises beyond infantile, guttural moans.

One week she was texting me regularly, but the next her phone went mostly silent – because ALS, which already had rendered her right hand useless, had ravaged her left hand. Now she’ll spend several minutes putting together this text:

“Kool!”

Maureen’s connection to the outside world is the Internet. She sets her iPhone or tablet on her lap and uses the most responsive finger she has left to slowly scroll Facebook and respond to well-wishers. Soon that finger will stop working. Then what?

There is another way, but Medicare won’t allow it. It’s eye-gaze technology that costs upwards of $20,000, and the Burakiewiczes don’t have that kind of cash. They do have Medicare, but Medicare has made the cruel decision that, if it does help cover such technology, it will prevent Internet access for 13 months. The machine offers a computerized voice to speak a patient’s eye-chosen words, but most of what it can do remains out of reach – literally locked up, as if it were something wicked instead of something wonderful.

“For 13 months (the machine) is considered a rental, and you can’t use it to go online,” Jim says. “How many people die before those 13 months are up? The Internet is their whole world, and they can’t use it. It’s insane.”

The miracle of eye-gaze technology, reduced to one use.

“A $15,000 voice box,” says Clare Durrett.

Durrett is the associate executive director of Team Gleason, the foundation put together by former New Orleans Saints defensive back Steve Gleason, who was diagnosed in 2011 with ALS and has become one of ALS patients’ most visible advocates.

Gleason is a paralyzed force of nature, the power behind the Steve Gleason Act making its way through Congress, aiming to remove the 13-month Internet cap on eye-gaze technology.

It’s a slow process through the halls of government.

And people are dying out here.

Chad Smith was coaching girls basketball at Northview High in Brazil, just east of Terre Haute, when his arms started to twitch. He kept it to himself, stopped coaching basketball, kept riding his bicycle long distances, kept coaching the Northview track and cross country teams. He ran with his distance runners and beat them, no surprise for a 35-year-old former all-state runner at Sullivan High and later an all-conference runner at the University of Southern Indiana.

But the kids at Northview were gaining on him.

And then Chad Smith started falling off his bike.

Two years after his ALS diagnosis he can’t coach, can’t walk, can’t feed himself. He lives with his dad and stepmom, and his dad was telling me that Chad now struggles to swallow.

“He chokes a lot,” Charlie Smith says.

I tell him about my friend Maureen, how she was choking on food until she got a feeding tube, and that even now when she treats herself to an occasional sip – Mighty Mo loves O’Doul’s – she might struggle with it. She was in the emergency room earlier this week with pneumonia. Imagine that, I tell Charlie Smith. Choking on non-alcoholic beer.

“If you don’t get him a feeding tube,” I tell Chad Smith’s father, “Chad could choke to death.”

Charlie pauses. He knows that. And he knows his son a lot better than I do. Charlie Smith knows, for example, that Chad has an eye-gaze machine that is locked out of the Internet. Chad’s only way to live beyond the borders of Charlie and Robin Smith’s home near Terre Haute is to venture virtually – but thanks to Medicare, Chad is almost a year from using the Internet. Charlie Smith isn’t sure his son is willing wait.

“I don’t know how far Chad wants to go to maintain his life,” Charlie Smith says.

This is what ALS does. It leaves people – Larry Bird’s mother, Charlie Smith’s son – welcoming death. There are exceptions, Maureen Burakiewicz among them, but the resources it takes to live with this monstrous disease are enormous. Steve Gleason has such resources. So does O.J. Brigance, another ex-NFL player who now works in the Baltimore Ravens front office.

Brigance was diagnosed with ALS in 2007 and is paralyzed, but he has eye-gaze technology. We email from time to time – me writing with my fingers, O.J. with his eyeballs – and I asked him this week about his fight. And how ALS patients need so much breeze in their sails, not the spitting headwind they face from the government, Medicare and insurance companies.

This is what O.J. Brigance wrote back, using his eyes.

“I just received an email from a friend who is battling ALS and he is looking for ways to shorten his life, due to the tremendous financial strain of living with ALS,” Brigance wrote. “He no longer wants to be a burden to his family. This is heartbreaking to me because my friend is a brilliant guy with so much more to contribute to life.

“When I hear about the different measures and regulations that slow viable pharmaceutical solutions from getting to patients it is disappointing. Lives are being lost every day there is a delay. Quality of life is essential until a cure is found. If legislators were able to walk a day in our shoes they would see how critical augmentative communication devices are to staying interactive in the world around us. Unlocking devices should not even be a question. I do agree that more sense of urgency should be given to the needs of ALS patients to fast track viable drug solutions.”

While the government twiddles its thumbs, Maureen can’t use hers. The ice-bucket challenge last summer brought worldwide awareness and funding to the ALS fight, but global attention has shifted to other things. In Greenwood, Maureen’s attention is on Ashleigh’s wedding. It’s in seven months and Maureen wants to dance at her daughter’s wedding with Jim, who catches her when she falls.

Friends and family will watch, and we will cry. The U.S. government should be there. The FDA and insurance companies should see this. Instead they are locking life-affirming technology and mulling potentially game-changing medication.

People are dying out here.

For god’s sake, you coldhearted bureaucrats, they’re dying.

Find Star columnist Gregg Doyel on Twitter at @GreggDoyelStaror at www.facebook.com/gregg.doyel