Fighting for the option to care for Huntington’s disease patients at home

With the woefully inadequate care that some nursing homes have provided to Huntington’s disease patients, a number of HD families have fought hard to create alternatives.

Few embody the fight for quality care better than Raima and Mike Fernald of Saco, Maine. They have twice advocated for bills in their  state legislature to improve the predicament of Raima’s two HD-stricken sons, John Irving III and Chris Irving, fathered by her first, HD-affected husband, John Irving, Jr.

Both sons died in Raima’s arms – Chris in March 2012 at 37 and John last month at  39 – after a long and painful struggle by the Fernalds to overcome bureaucratic inertia and bring them to the couple’s home in Saco, a coastal town south of Portland.

“That was my whole goal, having them come home,” said Raima, 55, whom I interviewed at length twice last week. “I did not want them to die in a strange place or different place. Their father died in a hospital. Nobody really knew where he was. I found out only a few days after he died because a newspaper reporter was doing an article and was compelled to find me.He died alone and I could not let that happen to my boys.”

Though HD dramatically shortened their lives, John and Chris helped establish an important legislative legacy in Maine.

In 2007, together with other advocates, the Fernalds successfully pushed for passage of An Act To Prevent Inappropriate Transfers of Residents of Nursing Facilities.

Even as they mourn their double loss, the Fernalds are now advocating for a pending bill, LD 488, An Act to Improve Access to Home-based and Community-based Care in the MaineCare Program. (MaineCare is the state’s Medicaid program.)

“We’re very supportive of the bill,” said Louise Vetter, the CEO of the Huntington’s Disease Society of America, noting that the society reviewed the legislation “to make sure it was strong” and “doesn’t have loopholes that could negatively affect families down the line.”

According to Vetter, LD 488 is the “best current example” of advocacy to facilitate home-based care for HD patients. The bill is “unique in terms of the issues and the involvement of the Maine Affiliate” of HDSA.

“There is evidence that home-based care is more efficient in overall cost savings and effective quality of life,” Vetter noted.

However, she added that the country is currently in a “wait and see” mode about the future of home-based care because the Patient Protection and Affordable Care Act (Obamacare) has yet to take full effect. The act supports home-based care, but lacks clarity on implementation, which will take place at the state level, she said.

Raima and Mike Fernald (family photo)

Exorbitant but inadequate care

From about 2003 to 2007, Chris spent time in four different institutions in another state, Raima said.

“They had no place in Maine to care for him, because Chris was considered to be a high-risk liability because of trying to kill himself,” Raima explained. “Because of his behaviors, no one wanted to take him.”

Raima said the care was inadequate in all of these institutions. She recalled her very negative experience at the last one, where Chris spent the longest period, about 16 months. She said she observed blood smears on the bathroom wall, which she attributed to the apparent lack of dental care; bursitis on Chris’s knee; ripped clothing on Chris; and food on the floor.

“I had to go in and shave him,” she said. “I had to go in and cut his hair. I had to do all of that. He looked horrible. I even have pictures. The hygiene on him was not good.

“We heard that the cost of his care was more than $300,000 a year, because he was shipped out of state. That was told to us by the Department of Human Services. That’s why they finally stood up and listened to us. They knew they were spending too much money. The care was not being overseen for that kind of money!”

According to Raima, the Maine Department of Health and Human Services claimed that, despite the large sum spent for Chris, it could not afford to send anybody to evaluate his care.

“That’s sheer stupidity,” she said, adding that she kept detailed notes on her sons’ care, including the information about the out-of-state costs.

Attorney Jack Comart, the litigation director for the non-profit legal aid provider Maine Equal Justice Partners, confirmed the high cost of the out-of-state care. In his recollection, the annual cost for Chris exceeded $350,000. He said that the cost per individual in the Maine group care home later occupied by Chris and John was $140,000 a year. Once they came home, the costs dropped even more – to just $52,000 per person, according to Comart, who has assisted the Fernalds and advocated for the legislative bills

With the passage of the Inappropriate Transfers bill, some 25-30 outsourced Maine patients with various conditions returned to the state, where they could be closer to their families, Raima said.

Chris entered a group home in 2007, and in 2008 John joined him.

Trying a group home

Located in Saco, the group home represented a significant improvement for Chris. John also moved there. Caregivers trained for this specific assignment watched over them 24/7.

“They were the only two clients in that group home,” Raima explained. “This particular group home was put together from the bill that was passed.”

Above and below, Chris Irving (left) and John Irving III (family photos)

Even so, problems developed.

“Some of the care was okay, but some of the things were ridiculous,” said Raima. “Things did happen there, too, that told me that they were not paying attention.”

At 5 one morning, even with two caregivers present, Chris escaped from the facility. Because he was “falling all over” and his “shoes were half on,” a woman thought he was drunk and called the police, who took him to the hospital, where he spent several days before returning to the home, Raima recalled. In late 2010, John became severely dehydrated and nearly died.

A bureaucratic bias against the family home

After several years in the group facility, and with the worsening of the men’s symptoms, the Fernalds became convinced that bringing them home was the only way to provide proper care and a life of dignity.

However, in reviewing patient needs, the Maine Department of Health and Human Services determined that the state – despite having spent much more money for the group home and for the out-of-state facilities – would pay for only a limited number of caregiver hours at the family home.

In 2007, for instance, Chris would have qualified for only 15-20 hours per week, making it virtually impossible for him stay at home, because Raima had a job, usually working as a secretary or bookkeeper. With so few home care hours, the family had opted for the group home.

According to Comart, the systemic bias against the family home stems in part from the state’s goal of protecting patients from abuse at home and assuring that they receive professional care.

“They assess them differently if they come home or if they’re in a facility,” said Raima. “That’s wrong. Their needs don’t change.”

In late 2010, with a reevaluation of their more serious medical condition, the men qualified for 59 caregiver hours each.

“That was at least something to work with,” Raima said. “But I’d still be doing a lot of it myself. I quit my job to take care of them. I didn’t have to. But I did because at this point, with them being in facilities and group homes, I wanted to be the one to oversee the care.”

On February 27, 2011, John and Chris came to live in the Fernalds’ basement, which Mike had turned into a bedroom using several thousands of dollars in assistance from a charitable organization, Motivating Miles.

Raima, Mike, and/or caregivers attended to the boys 24/7.

Raima shaving Chris at the Fernald home (family photo)

“I actually lived there for two years,” Raima said. “Mike lived upstairs alone. I slept on the couch. John and Chris had their own beds. A caregiver would come every day. Each one had a caregiver all day long.”

Earning $10 an hour, Raima received money for 40 of the 118 total hours allotted for Chris and John. Professional caregivers covered the other 78 hours, paid at the same rate.

To qualify as a paid caregiver, Raima paid $250 to take a 60-hour class that certified her as a personal service specialist and became an employee of the same home healthcare company that provided the other caregivers, she said.

That still left 50 hours – two full days – of uncovered care, which, of course, fell back on the family.

“I still had to be here 24/7,” said Raima. “There always had to be two while they were here. After Chris passed away, there was only 59 hours left. I could do 40, but got only 19 hours of outside help.”

Advocating for home-based care

With Mike also helping care for the two brothers, the Fernalds conceived and advocated for LD 488, the Home-based and Community-based Care act.

“We couldn’t deal with the fact that John and Chris would get so many hours in a group home, but at home fewer hours,” Raima said. “We were very unhappy with that the whole time they were home. We didn’t have enough help.”

Agitated and irregular sleep is a symptom of HD, so care became especially difficult when both men walked around their room at night.

“We fought to get more hours,” Raima said.

As amended, and if passed and signed by the governor, LD 488 would initiate a program to benefit 25 Maine patients, who would receive for home care up to 25 percent more than the maximum allowed for home care, which, as noted above, is currently $52,000. With the supplement, the total for home care could be as much as $63,000.

In 2011, the local newspaper featured the family's fight against HD (family photo).

The bill states that additional funds will become available as long as Maine’s Department of Health and Human Services “determines that such additional services are medically necessary, are likely to delay or prevent the institutionalization of the person and are not likely to result in the cost of services under the waiver for that person exceeding the estimated cost of comparable services in a nursing facility for that person.”

For the program in fiscal year 2013-2014, the bill projects $93,265 in spending from the state’s general fund and $150,916 from its federal expenditures fund.

Because part of the funding is from the federal Medicaid program, the state would have to obtain approval from the federal government, said attorney Comart.

Under federal Social Security legislation passed in 1983, any state can apply to expand home-based care.

Testimony from the HD community

On March 7, 2013, Raima, Comart, and ten other individuals offered personal or written testimony in support of the bill before the Committee on Health and Human Services.

“Not all people will want to, or be able to care for their loved ones at home, just as not all families should be allowed to, but the ones that can should receive more help to do so,” Raima stated. “Right now the State spends an enormous amount of money to send people out of State and in State nursing homes at a considerably higher cost that they would spend if they provided more fairness and flexibility in the current MaineCare program to allow willing family members to care for their loved ones. Many people will not do so because they cannot financially do so. The current program is unfair to consumers and their families, and it creates an unfair incentive to put loved ones in facilities, group homes, nursing homes, and at a greater cost to the State.”

“No one can care for a person with HD at home without a huge amount of financial, professional, and physical support,” stated Nancy Patterson, 55, the chairperson of the HDSA Maine Affiliate. LD 488, she added, would provide an affordable option for families to keep their loved ones at home and “alleviate the financial, social, and emotional strain” caused by HD.

Assessing the bill and its chances

The bill unanimously passed both the Maine House and Senate. However, because the bill would require increased state spending, it must pass muster at a joint House-Senate appropriations committee before going to Republican Governor Paul LePage.

Comart, however, is skeptical about the chances for the bill’s passage.

“I’m not confident in the end they’ll adopt it,” he said. “It’s sort of a shame. It’s not a lot of money to fund it…. We have a big budget deficit. We have a Tea Party governor. He cut taxes mostly for the wealthy last year. That created a big budget deficit. That’s being funded by lots of cuts in programs.”

LePage has vetoed many bills, including some that don’t include increased spending, Comart added. LePage has not yet stated a position regarding LD 488.

Like Vetter, Comart noted that LD 488 resonates with a national trend towards home- and community-based care stimulated by the passage of Obamacare. This trend goes against the “bias” in the Medicaid program in favor of putting people in institutions, he added.

“It’s been an issue for a while,” Comart said. “People generally don’t want to go in nursing homes. They want to stay in their home.”

However, even though LD 488 and home-based care in general represent savings in the long run, the fiscal stress of both the federal and state governments makes it “really challenging” to move in that direction, Vetter said.

In Maine, which has the nation’s tightest nursing home eligibility standards, there are waiting lists for some nursing homes, Comart said. To the extent people move into community or home care, the state would not save money it beds are filled off a waiting list, he explained.

Engaging in the public process

Comart called for Maine families to support the bill.

“Families need to get as involved as they can in this public process,” he said. “ I know it’s hard to go to legislative hearings, but it does make a difference for legislators to see and hear from families facing these problems.”

Comart pointed to the example of the Fernalds, who have raised HD awareness in other ways, including the publication of a children’s story about the disease.

Above, Mike (left) and actor Chuck Norris during a Mike Huckabee presidential campaign event in 2008. Below, Mike in a clown costume. Mike advocates for HD whenever he can. (family photos)

“They’ve been great advocates,” Comart said. “They’ve been out their publicly. They come to the public hearings. They send out e-mails about things. They met with the governor. They’ve done everything on top of caring for their two sons. It’s pretty amazing.”

“People need to not take no for an answer,” Raima said. “If you’re talking to one person and not getting anywhere, go see someone else.”

She added: “We all have to step up to the plate and fight for our HD loved ones.”

Strangling of patient in nursing home a shuddering reminder of subpar care for Huntington’s disease

The strangling and serious injury of a 49-year-old, late-stage Huntington’s disease patient at an Oregon nursing home has shocked the HD community and provided a shuddering reminder of the subpar care, fueled by ignorance and approaching neglect, that some affected by the disease face.

Anne Haskins was allegedly strangled by another patient who used  a call cord ripped from the wall after Haskins, wheelchair-bound and cognitively disabled, wandered into the other woman’s room shortly before 9 p.m. PDT on May 28, said Rebecca Ambrose, 29, Anne’s daughter.

There’s no evidence Anne deliberately provoked the other patient, whom police described as suffering from “severe dementia,” but her HD chorea – the involuntary movements typical in HD – means she can inadvertently hit people with her arms.

Anne was taken to Bay Area Hospital, located in Coos Bay, OR. After the attack, her heart rate dropped to the dangerously low rate of around 30 beats per minute, said Rebecca in a phone interview on May 30.

Anne is currently in the hospital’s cardiac unit. Doctors offered the option of a pacemaker, but the family decided against one because they believe it would simply help to prolong suffering, Rebecca said.

“She may have lost too much oxygen to the brain to recover from this,” she added.

The incident took place at Avamere Rehabilitation of Coos Bay, a private facility where Anne has resided since August of 2009.

Anne Haskins, grandson Andrew, and dog Scarlet, about ten years ago, before HD left her unable to speak and care for herself (family photo)

‘Where was the staff?’

According to the Coos Bay police, the alleged perpetrator is under observation in the psychiatric ward at Bay Area Hospital. Avamere has prohibited the alleged perpetrator from returning to its facility, Rebecca added.

“Where was the staff???” Rebecca exclaimed in several private Facebook HD discussion groups. Rebecca agreed to allow inclusion of her Facebook comments in this article.

On the night of the attack, the certified nurses assistants (CNAs), the main caregivers at the facility, should have put Anne to bed by 7:30. However, she was still moving around in her wheelchair around 9. No CNA noticed that she entered the other woman’s room. A CNA came upon the injured Anne sometime later, said Rebecca.

The police received a call for help at 8:49 p.m. According to Officer Randy Sparks, the lead detective on the case, a nurse, responding to the call alarm from the room, intervened to assist Anne.

“It just makes me angry,” Rebecca said. “I felt that it could have been foreseen. It makes me angry to think that my mom could be killed, and neither the person who did it nor the nursing home could be liable for it.

“How could one bedridden patient strangle another bedridden patient and no CNA have a clue? There were five CNAs on the floor, according to the director of the home.”

According to Deborah Nedelcove, Avamere’s vice president of risk management and its chief compliance and privacy officer, 42 residents currently occupy the 90-bed Coos Bay facility.

Above, the strangulation mark on Anne's neck. Below, daughter Liz with Anne in Bay Area Hospital (family photos)

Detective Sparks has concluded his investigation and forwarded his report to the district attorney’s office. However, those authorities have already have informed Rebecca that criminal charges will not likely be filed because of the mental state of the alleged perpetrator, Rebecca explained.

The alleged perpetrator is not currently under arrest.

“The police can investigate if there is a criminal action by a patient,” she added. “They cannot investigate neglect by nursing home staff.”

Avamere’s response

Debbie Lane, the Avamere director of nursing, refused comment on the case, as did Britta Milius, the nurse in charge when I called the facility the evening of May 30.

VP Nedelcove, who works at the Wilsonville, OR, corporate headquarters of the 50-facility private company primarily doing business in Oregon and Washington, declined to comment on specifics of the case but offered some observations about the facility and Avamere’s policies, procedures, and philosophy.

“I have never heard of an incident like this,” Nedelcove, who has some thirty years’ experience in health care, said of the strangulation and Rebecca’s allegation of inadequate monitoring of patients. “This is an isolated incident. It was not expected. You can’t account for people who decide at a moment’s notice to do something.”

Nedelcove insisted that Avamere CNAs “definitely keep an eye on all of our residents all the time…. There are many residents in our facilities, and many of them have behavior issues.”

Avamere is conducting an internal investigation of the incident and, based on its conclusions, may alter procedures at the facility, Nedelcove added.

Seeking assistance

However, Rebecca has already contacted state oversight agencies and local media outlets.

She has also obtained assistance from the Northwest Chapter of the Huntington’s Disease Society of America (HDSA). However, HDSA cannot assist with placing Anne in a different facility because Anne, before symptoms worsened, had refused to give power of attorney to any of her relatives, preventing the sharing of medical information with an outside agency, Rebecca explained.

Rebecca posted pictures of her mother’s injury on the Avamere Facebook page, but the company removed them and then blocked her from posting additional images. She also placed a sign on the front door of the facility denouncing the strangling but doesn’t know if it remains.

Rebecca and other family members fought a hospital’s staffer's recommendation that Anne return to Avamere and will place her in a different facility, Myrtle Point Care Center.

Rebecca is also consulting private attorneys about potential legal action.

Denouncing neglect

“I’m really disgusted with this,” said Rebecca, a family advocate for a non-profit who tested negative for HD in 2006 and has identified some 50 descendants of an HD-stricken great grandmother who are at risk of inheriting the mutation. “I’ve told them I’m not going to be quiet about this.

Rebecca Ambrose (personal photo)

“I feel like I already have to be robbed of my mother. I feel a lot of times like I have to be the mother to my siblings and my child, and I have to take on a lot of what a grandparent would do, because my mom isn’t able to.

“I can still visit my mother. That’s being taken from me slowly. I didn’t expect my mom to be in a nursing home and have an incident that could cause her death. I always thought her disease process would cause her death. I understand that there are going to be falls out of the shower or the bed. But there’s no excuse for somebody to strangle my mother and for her not to be protected in facility that gets $80,000 a year to care for her.

“I’m livid and I’m horrified. My mom used to watch that movie One Flew Over the Cuckoo’s Nest. I hate that movie. It’s one of the saddest movies I’ve watched in my life. That’s the state of nursing homes in America today.

“I just want to talk to whoever is going to listen to me. This can’t happen to people –when you entrust someone’s life! My mom is in a facility for her own protection, not to be neglected. I can assure you that nobody in my home would strangle my mother. I wish there were options that were not for profit. These people do it for profit.”

Care providers: a mixed bag

According to Rebecca, in early 2012 a man visiting Avamere to see his wife became angry at Anne and tried to punch her because an employee had accidentally taken his chair to Anne’s room.

Anne also suffers from bed sores, and she sometimes does not get her spoon-fed evening meal until late at night, Rebecca said. The CNAs bathe Anne, left incontinent by HD, just once a week, which understandably leaves a patient uncomfortable.

“It’s really a mixed bag with the care providers,” Rebecca said, referring to the CNAs, the main caregivers but also the lowest rung in the nursing home hierarchy. “Some love and care for her and take the time to feed her and meet her needs. Others fear her and skip over her as a patient or try and put it off on somebody else.”

Rebecca said that she has witnessed CNAs taking as long as two hours to respond to a call for assistance from patients. Nedelcove said CNAs usually respond within minutes.

Because the law prohibits a patient from being restrained, the facility cannot legally set the brakes of Anne’s wheelchair, to which she is bound by two straps.

As a result, Anne bumps into other residents in the dining room, knocks over food, and inadvertently hits people with her arms because of her chorea. To avoid these difficulties, Anne takes her meals in her room, Rebecca said.

Many of the CNAs have few or no qualifications, Rebecca continued.

Nursing homes hire “anybody off the street,” she said, adding, however, that several good facilities exist for HD people in various parts of the country.

“It’s an entry-level job,” Nedelcove admitted, noting that it’s “not a glamorous field.”

“It’s a calling rather than a profession,” she said.

However, she emphasized that all Avamere CNAs receive academic and clinical training and are state-certified.

“Most of them come to us with a great deal of experience,” she said.

A criticism of public agencies

In 2009, Anne was sent to the nursing home to recover from an operation needed after her HD symptoms had caused her to fall and injure her brain.

For a while, Anne had hospice care, as her weight had fallen to about 90 pounds. However, after her diet and weight improved, she no longer needed hospice.

Public agencies will not fund the 24-hour home care that would serve as an alternative to placing the patient in a nursing home, Rebecca explained.

“They will pay the nursing home over $6,000 a month to pay for somebody to give such little attention to my mother that she could get strangled, but they will not pay for better care at the same rate in my home,” she said.

Grossly misunderstanding HD

CNAs, Rebecca said, need better preparation in order to take “care of our elderly and the most vulnerable in society.”

Those vulnerable include thousands of HD patients, who, along with their families, face enormous difficulties in finding facilities that understand the disease and will take in someone with HD.

Rebecca recalled her family’s encounter with a past director of nursing at Avamere.

“They usually deal only in comatose patients, not the kind that can call down the hallway,” she said. The nursing director told Rebecca that “my mother needed to stop calling down the hallway, because HD is not a crutch and my mom is responsible for her own behavior.” The nursing director stated that if Anne couldn’t control her calls down the hallway, she could be evicted from the home.

“If you even say the word Huntington’s disease, nursing homes don’t want to talk with you,” Rebecca said, noting that most facilities focus on young people who are developmentally delayed or on the elderly, thus missing the middle years, the period where most HD people experience onset of symptoms.

Rebecca worked to educate the Avamere staff about HD. She arranged for the facility's previous director to participate in HDSA-sponsored caregiving webinars. He passed on information about HD to many of the staff. This “gave them some enlightenment,” Rebecca said.

However, because of recent high turnover at the home, including the removal of that previous director, few current employees have knowledge of HD, she said.

HDSA’s response and recommendations

Staffers at the HDSA national office in New York expressed deep concern about the incident and are closely monitoring the situation in Coos Bay. However, HDSA cannot comment on the specifics of the case in order not to violate patient privacy.

HDSA urges families to carefully research facilities before placing a loved one. It provides a number of publications, articles, and other materials regarding long-term care on its national website.

It also offers free in-service trainings for long-term care facilities.

As previously reported, many in the HD community have asked HDSA to provide funding for care. However, with an annual budget of only $8.5 million, the organization could not begin to provide such assistance. Families must rely on Social Security, Medicare, Medicaid, and other government programs, as well as long-term health care insurance and other private insurances.

(In a future article: how segments of the HD community have strived to provide better care for patients).

From a paralyzed genius, lessons of determination and caregiving for the Huntington’s community

In my fight to avoid the onset of Huntington’s disease, I have sought inspiration in model lifestyles, outlooks, and individuals.

One of my heroes is Stephen Hawking, the theoretical physicist who pioneered the science of black holes, Hawking radiation, the origins of the universe, and the quest for a “theory of everything,” an explanation of the ultimate forces and laws that govern the universe.

Hawking achieved all of this while surviving five decades with ALS, amyotrophic lateral sclerosis, known in the U.S. as Lou Gehrig’s disease and in Hawking’s native England as motor neuron disease. As is well known, ALS has completely paralyzed Hawking’s voluntary muscles, relegating him to existence in a motorized wheelchair with an on-board computer through which he speaks.

Hawking at the White House with President Barack Obama in 2009 (photo from www.hawking.org.uk)

When doctors diagnosed Hawking with ALS in his early 20s, they gave him two years to live. However, through sheer determination and with the support of his devoted wives and numerous friends and nurses, Hawking not only survived but achieved remarkable accomplishments. On January 8 he celebrated his 70th birthday.

Like millions around the globe, I am awed by Hawking’s brilliance, moved by his triumph over ALS, and cheered by his good humor and kindness.

I have just finished Stephen Hawking: An Unfettered Mind, a new biography by Kitty Ferguson.

It richly details Hawking’s extensive achievements in physics and his views of the origins of the universe, including the question of God. Reading about Hawking and his ideas, I feel the enormousness of the universe and, as he does with ALS, put the disease that claimed my mother, and that I face, in a healthy perspective.

An Unfettered Mind also portrays Hawking’s struggles with ALS, his utter dependence on caregivers for survival, and his and his first wife Jane’s fight to improve life for the disabled.

For HD activists, I believe that Hawking’s life offers valuable lessons to help strengthen our resolve to fight and demand better care for HD patients.

The ravages of ALS

Whereas HD is a fully genetic disease, only about ten percent of ALS cases are inherited. Researchers do not know the cause of the other 90 percent.

Like HD, ALS is debilitating and deadly. As Ferguson points out, ALS causes disintegration of the nerve cells in the spine and brain that regulate voluntary muscle activity. The muscles controlled by these nerve cells atrophy. Eventually every voluntary muscle in the body becomes compromised, making movement of any kind impossible. After diagnosis, most ALS patients live only a few years, dying from pneumonia or suffocation.

Patients experience no pain, and, unlike with HD, remain lucid to the very end. As Ferguson explains, patients in the final stage are prescribed morphine for panic and depression.

Hawking’s symptoms began as clumsiness during his third year as an undergraduate at the University of Oxford. Upon starting graduate studies at the University of Cambridge, he had difficulty tying his shoes, and his speech started becoming slurred.

Hawking experienced frequent fits of choking. In the summer of 1966 his fingers started to curl; writing by hand became almost impossible. A few years later he started using crutches. It took him 15 minutes just to climb the stairs at home. By 1971 he needed a wheelchair.

By the time Hawking became Cambridge’s Lucasian Professor of Mathematics – a chair also held by Isaac Newton – he “could no longer walk, write, feed himself, or raise his head if it tipped forward,” Ferguson writes of his condition in 1979. “His speech was slurred and almost unintelligible except to those few who knew him best.”

Hawking had several brushes with death. One occurred during a 1985 trip to Switzerland, where he became so ill that the doctors put him into an induced coma and on a life-support system. In order to save his life, Jane decided to have him undergo a tracheotomy, which was performed in Cambridge.

Hawking could no longer speak and could only communicate by spelling out words letter by letter; a helper would point to the letters on cards held out for him to see and select with a nod. Also, Hawking now needed 24-hour care from nurses.

Ferguson recounts how a computer expert in California enabled Hawking to communicate more rapidly by giving him a program the man had invented called “Equalizer.” It allowed Hawking to select words from a computer screen, and it also had a voice synthesizer – the famous Hawking voice known around the world. A student made Hawking a mouse-like tool that allowed him to operate the computer by squeezing a switch with his hand.

Because ALS continues to destroy his muscles, today Hawking can no longer use the mouse-like device. He now operates the computer by twitching a cheek muscle and thus activating a low-power infrared beam that prompts the computer.

“It is, of course, nothing short of miraculous that Hawking has been able to achieve everything he has, even that he’s still alive,” Ferguson concludes. “However, meeting him and encountering his intelligence and humor, you find yourself taking his unusual mode of communication and his obviously catastrophic physical problems no more seriously than he seems to himself. That is the way he wants it. He chooses ‘not to think about my condition, or regret the things it prevents me from doing, which are not that many.’ He expects others to adopt the same attitude.”

Although ALS has destroyed his body, Hawking plans to make further contributions to science. In a documentary produced several years ago, he declared through his voice synthesizer: “Hello. My name is Stephen Hawking: physicist, cosmologist and something of a dreamer. Although I cannot move, and I have to speak through a computer, in my mind I am free.”

Personal and institutional supports

In Ferguson’s account, Hawking has thrived so long only with the love and support of Jane and his three children with her. (ALS patients can still have sex.) Numerous professional colleagues, friends, the nurses, and other caregivers and assistants also provided Hawking with crucial support.

In the early years of the marriage, Jane cared for Hawking and coordinated the fulfillment of his needs. An intellectual in her own right, she postponed her graduate studies in literature, and she usually kept in the background as Hawking acquired accolades and fame. Ferguson reflects on how Jane chose this life path at a time when the feminist movement influenced many women to strike out independently of their husbands.

Hawking’s disease and success, as well as the presence of large number of caregivers in the home, made his marriage to Jane far from conventional. Although Ferguson does not discuss the Hawkings’ sex life during the period that they conceived their children, she does explore their ever more unusual and complex emotional and familial relationship as they lost physical intimacy. Both had affairs, and Hawking later married one of his caregivers, Elaine Mason. They divorced in 2006 amidst rumors of abuse by Elaine. At the time, Hawking refused to comment on the divorce. Ferguson indicates that no abuse took place. “The bottom line was ‘He loves Elaine,’” she concludes.

HD families also develop in highly complex ways. In addition to the debilitating symptoms, stigma, denial, and anger can lead both patients and caregivers to act aggressively, sometimes resulting in divorce or the splitting of extended families in disagreement about how to confront the disease.

However, I want to emphasize Jane’s decades-long support of her husband, and also how the family, friends, and colleagues rallied around Hawking in his battle against ALS.

The nursing staff played an especially important part. As Ferguson describes, the nurses made Hawking look nice by brushing his hair, polishing his glasses, and wiping his chin of the saliva that ran out of his mouth. They also spoon-fed him. Most importantly, they regularly cleared his throat with a “mini-vacuum cleaner” so that secretions did not build up in his lungs.

Institutional support was also crucial for both Hawking’s survival and scientific success. The British National Health Service would not pay for his 24-hour care, which the family naturally could not afford. The MacArthur Foundation, which funds academic research and other projects, came to the rescue with a grant to pay for the home care.

Ferguson points out that without such care Hawking would likely have languished in a nursing home.

Hawking’s fame and success have brought him almost endless privileges. Since 2000, he has flown frequently by private jet (paid for by others). In difficult situations, people and governments have made special accommodations for his disability.

Most disabled people can only dream of such special treatment.

The Hawkings’ advocacy

Nobody begrudged Hawking these wonderful advantages.

Recognizing the need to improve the plight of all disabled people, the Hawkings successfully pushed for greater institutional access for wheelchairs – a major struggle in the 1970s that is now often forgotten. “The Hawking image encouraged universities to set up dormitories equipped for students needing round-the-clock nurses in order to attend classes,” Ferguson writes.

Hawking firmly advocated that disabled children always be grouped with normal children of the same age.

Yearning for freedom for HD people

As Hawking’s mind remains free, I am fighting to keep my own mind free, along with thousands upon thousands of HD patients and gene-positive individuals.

In contemplating Hawking’s life, I fantasized about how wonderful it would have been for my mother to have had a device that allowed her to communicate with us from behind the horrible, impenetrable mask of Huntington’s disease.

At 52, I have reached my mother’s age of onset: each day I worry that Huntington’s will cut short my career as a college professor and writer and leave me unable to love and support my family as my daughter approaches adolescence and prepares to enter an expensive private school next fall.

Sadly for most HD people, Huntington’s destroys the area of the brain responsible for speech, thought, and memory.

Redoubling efforts for better care

The first effective treatments could arrive within the next five to ten years, but until then the HD community must focus on providing for – and demanding an improvement in – care for our stricken loved ones.

This will require us to redouble our efforts to change the nation’s outdated Social Security rules for HD people (click here to read more); provide Medicaid assistance without forcing couples to divorce and impoverish the sick individual to make him or her eligible; implement better standards of care as widely as possible; demand assistance for families caring for HD people in the home; and insist on better nursing home care. The lack of competent nursing home care remains one of the most intractable problems faced by HD families.

For now, this is our best hope for prolonging the lives of our loved ones and making their final years and months as comfortable as possible.

Perhaps HD people cannot have the advantages of a 24-hour team of expert nurses. But Hawking’s privileges point to a horizon of healthiness that humanity should aspire to for all disabled and ill people.

HD people have as much right as Hawking or anybody else to fair, decent, and up-to-date care.

In this fight, we can take a cue from Hawking.

“It is no use complaining about the public’s attitude about the disabled,” he declared at a speech at the University of Southern California in 1990. “It is up to disabled people to change people’s awareness in the same way that blacks and women have changed public perceptions.”