Your mind stays sharp while your body fails.
At the end, you’re fully present mentally and trapped inside a body that no longer works. You cannot talk, eat or drink, and eventually, you cannot breathe.
It is a terrible way to die.
That was the extent of my knowledge about Amyotrophic Lateral Sclerosis on Monday, December 10, 2018.
That afternoon, I sat in a basement exam room of the university hospital, Dad at my side. I’d been summoned a few hours earlier from across town, where I was in the middle of getting a haircut when my phone rang.
“The doctor wants to talk to you,” said Sam, my 12-year-old nephew. He’d accompanied Poppa to the doctor so they could spend some quality time together. They didn’t get much of that since Sam had moved to Miami a year earlier.
“It’s not urgent,” he reported, “but get here soon, okay?”
I rushed to the hospital anyway, my mind racing with explanations for why I had been summoned.
Here we sat, three hours after that call. Sam had been whisked away gently to the lounge for some cookies so we—Dad, the doctor, and I—could talk.
We faced an aging neurologist. Or, as Dad would say, a neurologist who was “eighty years old if he was a day.” He leaned back in an uncomfortable chair, his legs crossed, faintly shaking his head side to side. It was actually palsy that caused Dr. Gutmann to appear that he was constantly refuting an idea or suggestion. I found it unnerving.
This could not be good.
The neurologist took a deep breath and began. “I’m going to tell you what I think it is. I’m not yet certain, but I’m sure enough that I think you should know.”
I realized I was holding my breath. And then he said it.
“I think this is Lou Gehrig’s disease.”
This doctor’s visit was the conclusion to a saga that began nine months earlier, in March 2018. My father had retired at age 68 from his full-time job as an inspector for underground petroleum storage tanks. He would’ve kept working, except his joints had thrown in the towel. After a lifetime of highly physical jobs, he’d had both knees replaced, several foot surgeries, and a back surgery to fuse several vertebrae together. It was just too difficult to do the everyday work, and he was due for another foot surgery soon.
So they bought the cake and balloons and celebrated his last day. Within a week he had his right foot operated on and drove to Florida to spend a few weeks with my oldest sister, Jessica, and her son Sam.
He was in a boot from the surgery for a few weeks, and as soon as he was cleared to remove it, Jes noticed that my dad’s right calf was visibly smaller than his left.
No one was alarmed; they chalked it up to surgery on the foot. But as his foot got stronger and he recovered from surgery, the calf muscle didn’t.
When he returned to Iowa in May, he saw a doctor. They suspected a pinched nerve from the spine surgery, and MRIs were done. Nothing showed up. They looked at his pelvis. No pinched nerve there, either.
One doctor sent him to a specialist in multiple sclerosis, but that doc was emphatic: “You do not have MS. This is a pinched nerve. Back to your spine surgeon you go.”
The spine surgeon, however, was finished with this puzzle, and refused to see him. By this time, it was July and the tiny muscle was causing a pronounced limp. He was struggling to lift his right foot and could no longer flex his foot very well. It was getting worse, not better.
We decided to try the university hospital, 2 hours from where my dad lived, but only 10 minutes from my house. We started with an orthopedic surgeon who specialized in the spine. He, too, couldn’t find a pinched nerve. We went through the MRIs with him, frame by frame.
He referred us to a neurosurgeon, who was one of the biggest jerks I’d ever encountered. “I can’t fix this with surgery,” he muttered. He suggested we see a neurologist instead.
We were frustrated and sick of bouncing between doctors. I started inquiring with Mayo Clinic, to see if we could get him an appointment there. Maybe they could find the pinched nerve that no one else could.
While we waited for a callback, Dad went to one last appointment at the university: the neurologist we’d been referred to by the neurosurgeon.
We were sure it would be another dead end. I didn’t even bother accompanying him to that appointment—we were that certain that they’d find nothing.
Instead, I got my hair cut, and was happily chatting away with my stylist when Sam called, summoning me to the university neurology clinic.
And now it’s time for a breakdown
Thirty minutes after Dr. Gutmann first said the words “Lou Gehrig’s Disease,” Dad and I stepped out of the exam room and into a shadowy hallway. We’d been in the hallway just an hour before, yet somehow everything looked different now. The universe had turned upside-down while we sat in that windowless room, discussing life expectancies and FRS scores, medications to prolong life and clinic visits.
Sam reappeared by my side.
“Lydia,” he asked quietly. “Is Poppa okay?”
I didn’t know what to say.
My phone was at 1% battery and I needed to talk to someone. My sisters. I felt like my chest held a bomb, and I was seconds from detonation.
“Sam,” I said, turning to him. “Can I borrow your phone to call your mom?” I hoped he couldn’t hear the wobble in my voice.
He unlocked his phone and handed it to me, his eyes full of concern.
“Thanks, buddy. I’ll be right back,” I said, locating my sister’s number in his Contacts and dialing as I walked away. I was fighting a losing battle to keep my composure.
She answered a second before I arrived at the large, metal swinging doors at the hallway’s end. I could feel the knot in my throat rising up, and I couldn’t fight it any longer.
The tidal wave of panic and grief and fear that had been building since the words “Lou Gehrig’s Disease” were spoken came crashing down.
As the latch released and the heavy door swung open, I lost it.
Sobbing uncontrollably, I stumbled forward across the hallway and fell against the doorway of a janitor’s closet. It was that crushing kind of sob that chokes off your words and your breath all at once. Sobbing that consumes your body and mind so completely that you fear you’ll never stop.
Jessica, on the other end of the line, was freaking out. “Lydia, what? What is it?!” she shouted.
I gulped for air between sobs. I couldn’t say it. But I had to.
“It’s… it’s… Lou Gehrig’s disease.”
There was silence on the other end of the phone. It was just a couple of seconds, but it felt like a lifetime.
She spoke briskly: “I have to go.”
I held the phone away from my ear and stared at the screen, confused. I hung up.
I needed to get back to Sam and Dad, who were waiting for me inside the neurology department corridor. I had to get it together. I dug in my purse for Kleenex, wiped my eyes on my sleeve, and tried to breathe normally.
The phone in my hand rang; it was Jessica.
“Why did you hang up on me?!” she asked incredulously.
My brain felt scrambled. “You said you had to go!”
She sighed. “I was talking to my boss, Lydia. I’m leaving work. Tell me what’s happening.”
The shake in her voice betrayed her normal take-charge attitude. I could hear she was crying.
Dad and I had planned for him and Sam to come to my house after the doctor’s visit. It was just 10 minutes away, and Dad was going to help me install a new shelf organizer. Then the two of them would drive back to Des Moines.
“You can NOT let him drive tonight,” Jessica insisted.
We had no idea where Dad’s head was at, but we were gravely concerned about his mental state. We knew he’d be safe with Sam in the car, but still… we were taking no chances.
She was on her way to Kayla’s, she explained, and I should call her when I was leaving the hospital. We’d get Monica on the line and talk, all four of us.
“Jes,” I said. “What should I tell Sam?”
She didn’t hesitate. “The truth. I always tell Sam the truth. You can do the same.”
I wiped my eyes with the sleeve of my sweatshirt, trying to get my shit together to step back inside the clinic. I took a few deep breaths and hoped my face wasn’t too terrible. Just like my daughter, I get all splotchy when I cry. I opened the heavy metal door and started down the hallway, where Sam stood alone, my dad talking with the doctor a few doors down.
His hazel eyes looked up at me. He was doing ballet positions with his feet, lightly lifting his arms, but he didn’t take his eyes off mine. Ballet is Sam’s equivalent to fidgeting.
“Is Poppa okay?” he asked again, his voice thick with worry.
I gulped. It was important to choose my words carefully. Poppa was the only father Sam had ever known. His mother—my sister—was a single mom who’d lived with my dad all of Sam’s life. Jes and Sam only struck out on their own 12 months ago, when the pair of them moved to Miami so he could pursue dancing full-time.
“They think Poppa has a disease called ALS,” I explained. “It affects your motor neurons and that’s why his leg is shrinking. There’s no cure for it, and most people live two to five years with it, but you can live a lot longer!” I blurted it all out in a single breath.
I knew I was doing a shitty job. I could barely process anything. This was not the time to guide a youngster through the worst news of his young life.
Fate bailed me out when a moment later, Dad came back up the hallway, a prescription in his hand for a custom orthotic. His lower right leg muscles had wasted away to the point he could no longer flex his toes upward, which meant his leg dragged when he walked. The brace, said Dr. Gutmann, would help him walk almost normally.
“Let’s go,” Dad said.
I didn’t know what to say. I tried to act normally. But suddenly, I didn’t know what normal was anymore.
The three of us walked to the parking garage, then split up toward our respective cars. I plugged in my phone and put on my headset, quickly calling my husband to tell him the news, and that we were all three on our way to the house. I hung up and started dialing Jessica as I maneuvered my way out of the parking structure.
A minute later, four of us were on the line together. I remember thinking, Wow, I’m 38 years old and this is the first time in my adult life that I can recall being on a call with all three of my sisters. Weird.
We talked. I explained what Dr. Gutmann had said. We cried. We took turns bursting into tears. Monica explained how she had watched the Steve Gleason ESPN360 just a few days prior, recognizing some of Steve’s early symptoms in what was happening with Dad. She’d hoped it was just a coincidence.
We discussed Dad’s new house; in just three days, he was scheduled for closing, and movers were coming on the weekend. We decided that without a definitive diagnosis, we couldn’t do anything. We were fairly certain Dad wouldn’t allow it anyway.
I hung up as the garage door opened in front of me.
I felt immense relief that I wasn’t going through this alone.
The first night
Dad and Sam stayed the night at my house, but not because I ordered them to. Dr. Gutmann called as they left the clinic and wanted to do a bit more bloodwork, just to solidify his hunch about the diagnosis.
That evening, Dad supervised while I installed the shelf organizer all by myself. He jumped in at one point to try and drive a screw into the wood frame of the cabinet; I just couldn’t get enough leverage on it. As he climbed down onto the floor and slid his upper half into the cabinet, I wanted to stop him. Shouldn’t I be stopping him?
But I didn’t want to be the person who acknowledged the fatal elephant in the room. I said nothing, and let Dad do what he wanted.
It was difficult keeping my composure. No matter how hard I tried, I couldn’t stop thinking about it. I couldn’t sleep that night, and I was relieved when 4:30am finally came and I could be distracted by going to the gym.
That morning, I’d originally scheduled an eye exam, but because I was accompanying Dad back to the hospital, I had cancelled it. My friend Denise from the gym had pulled some strings and gotten me the appointment, and I didn’t want her to see that I’d cancelled and think I was ungrateful for her favor.
Denise was an early bird, even earlier than me, so there weren’t many people around when I tried to explain why I’d cancelled. I got about 10 words out before I completely lost it.
Denise wrapped me in a hug, squeezing me tight while I cried.
As our trainers put us through the paces of the workout, I remember wanting to scream while I lay on the gym floor. Focus on the damn skull crushers, I demanded. But my mind was playing a tape on loop: ALS, ALS, ALS, ALS. It wouldn’t stop.
The looped tape would continue for days.
Later that week, Dr. Gutmann’s nurse called Dad. The “one last test” revealed no better news. He was being referred to Mayo Clinic for a second opinion and to confirm a diagnosis of ALS.
Rochester in February
We got in at Mayo quickly, by Mayo standards—his appointment was just seven weeks away.
“Allow five to seven business days for your visit,” Mayo Clinic told us, and we made plans. I found an AirBNB for the week. I would accompany him, and my sister Monica would fly in to come along.
On a frigid day in February, the three of us arrived in Rochester for Dad’s first appointment. It was a Monday afternoon. We stayed that evening until nearly 6pm as a soft-spoken Luke Wilson doppelgänger ran Dad through a full exam. It was old hat for me; this was the third such exam I’d been witness to.
Monica, on the other hand, was seeing this for the first time. I watched her surprise at how weak some of his muscles were, and how little he could do with his right leg.
Later on in the exam, both of us watched in awe as the doctor shined a flashlight down his arm in the darkened room. Tiny spots on his muscles twitched and jumped like miniature lightning strikes. I’d never seen his fasciculations before.
At 2pm the next afternoon—less than 24 hours from our arrival—we sat down with Dr. Ryan to hear his verdict. Their opinion was that Dr. Gutmann was spot on. My father had ALS.
Monica and I asked questions. And of course, we asked the one question we knew he couldn’t answer, but we asked anyway: How long can he expect to live?
That’s what sucks the most about ALS—it’s different for every patient. Some lose the ability to speak first. Others talk until their final days. Some lose movement in their arms but retain their ability to walk for years. Others must give up walking almost immediately.
There are no answers. Only averages. Two to five years after diagnosis.
We asked about breathing. The doctor explained that Dad would need to make some decisions about what level of breathing assistance he wanted. The non-invasive option was a Bi-PAP, which applies pressure on both inhalation and exhalation. The invasive option was a tracheostomy and ventilator.
The doctor explained, to our surprise, that only about 10% of ALS patients elect for invasive breathing assistance.
Monica cleared her throat. “So, if he doesn’t want to do the ventilator, what are his options?”
The room was silent for a long moment. The doctor squirmed uncomfortably.
I spoke up. “Well…there are no other options.”
Monica’s face looked pale, her eyes wide. She nodded.
The doctor was impressed at how much we already knew about the disease, which made me proud. We were informed. We were ready to fight this thing right alongside our dad.
The three of us returned dejectedly to our AirBnB. I felt dulled, like everything was half-muted.
We got on a call with all four sisters and Dad. We talked about the diagnosis, and about how we wanted to go on a trip, Dad’s choice. (“Somewhere warm with a beach,” he said. He wasn’t picky.)
A snowstorm was approaching, so I started the four-hour drive home soon after the call ended. I noted how different it felt this time versus when Dr. Gutmann delivered his diagnosis back in December.
There was no shock this time. Just resignation. And grief.
I didn’t approach the Mayo appointment with more than a tiny sliver of hope. I had read enough by then to know that Dad’s was a textbook case. He had all the symptoms, and combined with his 50-year career in the petroleum industry, it added up nicely.
(Some studies of sporadic ALS patients—that’s the term for ALS that isn’t passed down from parent to child, which is 90% of cases—have pointed to toxin exposure as a possible cause. It’s not certain yet, but they’re looking into it. Even without that certainty, I’m convinced petroleum had something to do with Dad’s diagnosis. Lead, benzene, tolulene… the list of toxins my dad handled daily is long and scary.)
But honestly, we can’t say for sure what caused it. No one can. At this point, the only known risk factors for sporadic ALS are being male, between the ages of 50 and 70, and having served in the military, particularly in the Persian Gulf. Those are pretty vague.
Three months later
It’s mid-May now. My father spent the rest of the winter in Florida, where two of my sisters live. He rides his bike often. He visited New York City last month while my nephew competed in dance, and played frisbee in Central Park. He goes to the beach and lounges by the pool. He rests. He watches westerns.
He wears his orthotic brace, but not as much as he should. He’s had a couple of near-misses with falling. He gets tired and forgets to focus on picking up that right foot, and the toes trip him up. My sisters work to keep his calorie intake up—ALS patients burn a ton of energy from muscle twitches, and nutrition helps keep the muscle breakdown from happening prematurely.
Friends ask me often how he’s doing, and my answer is consistent: “the same.” It sounds negative, but for a person with ALS, “the same” is encouraging. That means the disease is being held at bay.
For now, at least.
We’re going to Aruba in July. We’d hoped to wait until winter, but wanted Dad to fully enjoy the activities of our vacay. We’re going off-roading with ATVs and dune buggies, playing beach volleyball (he’ll skip that one, because he’s never been one for “ball sports”), and who knows what else. We’re having family photos taken in August at the Iowa State Fairgrounds—a place that’s packed with memories for our family.
ALS is a part of our lives now. There isn’t a day that goes by that I don’t think about it. My family attends ALS Association fundraisers, and are each spearheading teams in our local ALS walks (shameless plug: donate to my team here).
The ALS Association of Iowa has been amazing, and I don’t say that lightly. They have webinars on nutrition, classes on caretaking, and provide free equipment whenever we need it, like walkers, wheelchairs, and hospital beds. We haven’t needed to ask yet, but I know someday we will.
Thankfully, an ALSA representative will join our family later this year when we all sit down to have some seriously tough conversations about end-of-life care and long-term plans for caring for Dad.
I’m so grateful they exist and that they’re able to be so hands-on. The ALSA truly helps the families of people with ALS. I’m a whole-hearted supporter of their mission.
The grief process when your loved one is diagnosed with ALS is different from losing a loved one suddenly. Not only will we be devastated when he’s gone, but we’re devastated daily while he’s still here, knowing what’s to come.
My husband doesn’t know what to do with me. I’m irritable, easily stressed out, and quick to lose my temper. He can’t fathom what I’m going through, and what my entire family is going through. I can’t explain it well enough for him to get it, either. All I know is that I’m not myself, my stress level is sky-high, and I hate it.
I also know I’m lucky. Some people never get to say to their fathers what they want to say before they’re gone. One of the only upsides of this diagnosis is that there’s no excuse for words left unsaid.
This diagnosis sucks. It sucks so bad. But we’re getting through it. Day by day, we’re getting through.
The road ahead is rough and I’m dreading it. But we’ll get through that, too. We’re a close family, and this has drawn us even closer. I talk to all of my sisters more now than I ever did before.
We’re in this together, and I’m so grateful for that.