MILFORD — Karen Mey lies motionless beneath a tan electric blanket pulled up to her chin. Next to her recliner, a machine the size of a toaster oven huffs rhythmically like a person in deep sleep, pumping air through a corrugated tube into a plastic valve in her neck.
Even though her body is immobile, Mey’s eyes are animated, filling with mirth or tears as she participates in the living room conversation among her husband, daughter, and a personal care attendant. Mey smiles often, and she voices affirmation (“Uh-huh! Uh-huh!”), disagreement (“Uh-uh”), dismay (“Awwww”), and words that only those who know her well can understand.
At age 54, seven years after amyotrophic lateral sclerosis started destroying the connections between brain and muscle, Mey remains a vibrant presence in her family’s life. But as her husband and children piece together the resources to keep her home, they are confronting a new worry.
Mey, who needs constant care, can stay home in large part because her family was lucky enough to find Jenny Gauvin, the 33-year-old personal care attendant who tends to her 40 hours a week. Personal care attendants — known as PCAs — typically help with such tasks as dressing, toileting, walking, and light housework. Few are willing or able to do what Gauvin does: take on the complex life-or-death job of managing a patient who relies on a ventilator to live.
But Gauvin, who has been studying nursing, expects to start her clinical training in a few months, and then she will no longer be able to care for Mey every day. When that happens, Mey’s husband, Steven, is not sure how he will manage. “When Jenny’s gone, it’s all going to come down on me,” he said.
The Meys face a quandary shared by hundreds of other Massachusetts families whose loved ones suffer from ALS, a fatal disease that gradually destroys the nerve cells that control muscles: It’s hard to find workers able and willing to care for ALS patients at home, and it’s even harder to find a facility willing to take them, especially if they need devices to help them breathe.
A proposal that might have helped was on the table in recent negotiations with the PCAs’ union, 1199SEIU United Healthcare Workers East. The plan would have created a new category of PCAs, who would undergo training and receive higher pay to care for complex patients, such as those who use ventilators.
PCAs won a raise to $15.40 an hour, but the PCAs’ contract with MassHealth, the state’s Medicaid program, which took effect July 1, did not include the plan for training and enhanced pay. PCAs skilled at managing breathing equipment often demand $30 an hour or more from their clients, who pay the difference out of pocket.
Although ALS is a relatively rare disease, the struggles of ALS families reveal systemic failures that affect the elderly and anyone with a disabling illness, says E. Spencer Ghazey-Bates, a Northampton elder-care lawyer who has represented ALS patients fighting for adequate home care.
“We’ve been too focused on institutionalizing people. Community-based resources are not there,” Ghazey-Bates said. “The system is just broken.”
About 5,000 people in the United States are diagnosed with ALS each year, and an estimated 16,000 are living with it at any given time, according to the ALS Association.
The illness starts slowly, perhaps with occasional tripping or difficulty grasping a cup. Gradually all the muscles weaken, until the person is completely paralyzed and, eventually, can no longer breathe. People typically die three to five years after an ALS diagnosis, but there is great variety in the illness’s trajectory.
Through it all, the mind usually stays sharp. Because the disease tends to strike at middle age, patients often have children whom they want to see grow up. So the desire to remain home is strong.
Racing to plug gaps in care
Private health insurance does not cover long-term care in the home. By law, Medicare covers up to 35 hours a week of home health services for an indefinite period of time, but advocates say Medicare’s payment policies discourage home health agencies from providing aides to chronically ill people.
As a result, in practice, people pay out of pocket for home care or rely on the state-run Medicaid programs. In Massachusetts, the MassHealth program offers several avenues to qualify for coverage and several types of in-home services.
But gaps remain, forcing some patients to deplete their savings with out-of-pocket payments. And the system can be clunky: ALS often progresses rapidly, but it can take weeks or months to win approval for equipment or caregiving that is needed right away.
“When I talk to families that are dealing with this, they are running back-to-back triathlons trying to support their family member,” said Jean Z. Batty, government affairs liaison with Compassionate Care ALS, a charity that supplies ALS patients with equipment not covered by insurance and advocates for their care.
These triathletes of love and care can be found around the state.
In Northborough, Mounia Mrissy waited two months to get MassHealth approval for in-home help for her husband. She was forced to leave 50-year-old Noureddine Haddad, at home alone, even though ALS has left him unable to use his hands and barely able to walk, because she needed her job as a pharmacy technician to support the couple and their 9-year-old twins. He fell several times, waiting for help for hours alone. Mrissy said she still doesn’t have as many hours of help as she needs.
In Northbridge, 60-year-old Scott Murphy was ultimately forced to leave his home. He’d lived there for 15 years after his ALS diagnosis in 2004, dependent on a ventilator since 2016. Although he qualified for in-home nurses through MassHealth, too few nurses were available to fill the necessary shifts and earlier this year his wife, Kathleen, had to give up on home care.
But the only place in the area that could take him was a hospital, New England Sinai, an hour’s drive away in Stoughton. The hospital determined it wasn’t safe for Scott to go home for Christmas, even though Kathleen is an ICU nurse. So Kathleen, their three children, and four grandchildren spent this past Christmas Day in the hospital. Scott, Kathleen said, was very quiet. “He seemed sad.”
In Billerica, Karen Hayes, 60, worries about growing old without money. Her 62-year-old husband, Franny, struggling with ALS since 2016, uses a BiPAP, which forces air through his nose to make up for the weakness in his diaphragm. Franny occasionally chokes on his phlegm and needs suctioning, and relies on a personal care attendant with experience providing respiratory care.
For his extra skills, the attendant expects — and the Hayeses believe he richly deserves — more pay than the MassHealth rate. So Karen, who works as an executive assistant for the pharmaceutical company Takeda, kicks in an additional $15 an hour of her own money.
“We have to use our life savings to supplement our caretaker’s pay,” she said. “I’m afraid I’ll go bankrupt.”
And in Milford, Steven Mey, 57, lost his job as a Xerox repairman; he says it was because he needed so much time off to care for Karen.
Kristina, their 23-year-old daughter, postponed her education for two years to help her parents. Stevie, their 21-year-old son, attends Worcester Polytechnic Institute but comes home almost every week.
But the family still needs help. They hired a PCA, but she was comfortable only doing laundry and housekeeping, and feared going near the ventilator.
Karen needs much more than clean laundry, however. At any moment her heart rate can spike and oxygen levels plummet if she has trouble breathing, usually because mucus is clogging her throat.
Through Compassionate Care ALS, the Meys connected with Jenny Gauvin, who had learned how to manage ventilator care while assisting a previous patient. Gauvin got involved with ALS care after a close friend died of the illness in 2013. Because she loves the work, she’s not demanding $30 an hour; Steven said he tries to supplement her income somewhat but declined to provide details.
When she looks at Karen, Gauvin said in an interview, she doesn’t even see the equipment or the disability. “She’s always smiling. That’s what makes it worth it.”
Gauvin said she “falls in love” with her clients. “They’re like family to you,” she said. But the work can be also emotionally draining; eight of her patients have died. Seeking a change, Gauvin decided to pursue a career in nursing.
“This line of work has changed my life forever,” she wrote in a recent text message to the Globe, explaining that taking care of ALS patients has taught her “to live every day to the fullest, to only live positively. . . . I have all of my patients to thank for that.”
One day at a time
It takes about an hour, and two or three people, to get Karen up for the day.
On a recent morning, Steven handles the initial tasks. He disconnects the ventilator — Karen can breathe on her own for a few moments — and attaches a tube that infuses steam and medication into her lungs for about 15 minutes. Then he attaches the Cough Assist, which expands and contracts her diaphragm to induce a cough. Karen grimaces as Steve inserts a long thin tube all the way into the lungs to pull up secretions.
Then Gauvin and the Meys’ daughter, Kristina, employ a mechanical lift to bring Karen into the living room, where she settles in the recliner. Gauvin goes into the kitchen to prepare breakfast: the coffee-colored brew that she will infuse into Karen’s feeding tube.
How will the family cope when Gauvin curtails her hours for nursing training? Gauvin is trying to convince Steve that he’ll need to find someone else.
“He’s going to reach a crashing point; I’ve seen it,” she said.
Steve said they’ll keep managing one day at a time, as they’ve done since Karen was diagnosed in 2016.
The first signs came when Karen found her hands no longer had the strength to tie up Kristina’s hair for her cheerleading practice. She thought she had carpal tunnel syndrome, but as her other limbs weakened, the diagnosis of ALS finally emerged.
From the beginning, Karen insisted that she wanted to keep living, no matter what, and in late 2018, her resolve was put to the test when she ended up in the hospital with trouble breathing.
Karen faced a stark choice: have a hole surgically cut in her neck and a plastic valve inserted, and remain tethered to a machine the rest of her life; or accept that death would come within about a month.
She chose the ventilator. It’s an unusual decision in the United States, where only about 5 percent to 10 percent of ALS patients opt for a ventilator, according to Karen’s doctor, Sabrina Paganoni of Massachusetts General Hospital. But among those who do, Paganoni said, studies have found that “their quality of life is maintained and they are happy,” provided they have good support.
The number of people who choose a ventilator may be increasing, as new technologies enable continuing communication with family and friends.
Karen uses a Tobii, a device that responds to eye motion, enabling her to call for help, type letters, browse Facebook, answer e-mails — and to speak. The computer reads each word she types and then each sentence out loud.
Asked how she’s feeling, Karen says, in the computer’s voice: “Most days are good and I’m happy, but there are days when I get sad. I don’t like the winter because I don’t get out very much.”
She expresses no regrets about going on the ventilator.
“I always wanted to do whatever it would take to keep going. The only time I doubt my decision is when I see how it affects my family.”
Kristina blurts out: “We’d rather do this than not have you!”
Tears form in Karen’s eyes.
Steve, now also crying, says: “As difficult as it is, I love her to death.”
Steve and Karen met as students at Watertown High School, where Steve, a football player, broke the unwritten rule that players date cheerleaders and instead chose a girl he found more beautiful.
These days, Steve says he misses the romance of their previous life, but otherwise, “We cry and talk and we do everything we used to. We watch TV shows. She wants to see my daughter and my son both graduate, and she wants to live.”
Correction: An earlier version of this story gave incorrect information about Medicare coverage of home health care services.
Felice J. Freyer can be reached at firstname.lastname@example.org. Follow her on Twitter @felicejfreyer