Billboards: an old – but also new – way of projecting the Huntington’s disease cause

The San Diego Chapter of the Huntington’s Disease Society of America (HDSA-San Diego) has taken its advocacy to a new level with billboards announcing a chapter fundraiser, the Fifth Annual Team Hope Walk on April 10.

At five high-traffic locations in the San Diego area, the billboards went up on March 7 and will remain up for 30 days, according to Misty Daniel, the HDSA-San Diego vice president. Misty originated the billboard idea, the first time it’s been used in the San Diego chapter, one of the most active in the country.

The billboard calls on the public to “register today” for the Hope Walk at the website www.TeamHopeWalk.com.

The HDSA-San Diego 2016 Team Hope Walk billboard on Mission Gorge Road south of Twain Avenue in San Diego (photo by Gene Veritas, aka Kenneth P. Serbin)

“As a group, we’re not very good advocates for ourselves,” Misty said of the HD community in a phone interview. “This disease affects as many people as cystic fibrosis, but you’ve heard of cystic fibrosis.”

Huntington’s disease still lacks such instant public recognition, Misty observed.

“It’s not just that it’s a rare disease, but it’s rarely known,” she explained. “I’m tired of educating physicians and telling people that I have this in my family and them not having a clue about what it is.”

Misty said she wants the billboards to bring awareness of HD to the “point where I say, ‘I lost my mom to Huntington’s disease,’ and they’ll say, ‘I know what that is. I’m sorry.’ It’s getting harder and harder to use Woody Guthrie as my person to reference. More and more people are saying: who is Woody Guthrie?”

Guthrie – one of the greatest American folk singers of the 20th century and the composer of “This Land is Your Land” – died of HD in 1967, at 55. That year his wife founded a network of HD support groups and advocates that would become HDSA.

Billboards are a traditional, “old media” way of generating publicity. However, in this case they project a 21st-century imperative: the need to cure neurological diseases. They reflect the ongoing high level of creativity in a community facing one of the most devastating of those disorders (click here to read more).

A passionate, resilient advocate

At 38, Misty is a passionate, resilient veteran of the HD cause.

She became an advocate for HDSA-San Diego when she was just 19. She lost her mother Rosie Shaw to the disease in 2007. Misty tested negative for HD two years later. A brother with HD died in a motorcycle accident, losing control of the vehicle perhaps because of the disease. Her sister Brooke, 46, has HD symptoms. Three other siblings remain untested.

Misty speaks at HD events such as the chapter’s second annual convention, held March 5. (I’ll have more on this event in a future article.) She advocates for the passage of the Huntington’s Disease Parity Act, a bill in Congress to update the Social Security Administration’s long outdated definition of HD as a disease causing only involuntary movements. That situation prevents people affected by psychiatric and cognitive symptoms from qualifying for Social Security disability payments. In addition, she assists drug makers involved in HD clinical trials and other HD research.

Misty also helps organize the local Hope Walks. About a year ago, she started thinking about the possibility of billboards.

“HDSA does not spend any money on advertising – not nationally or locally – because we want as much of the dollars raised to go to HD care, advocacy and research as possible,” HDSA CEO Louise Vetter said in an e-mail. “This is an important policy and principle in how we steward the funds raised for our fight.”

So Misty and other advocates raised the $2,000 needed to pay for the billboards. One of Misty’s untested siblings donated $1,800. The Clear Channel media company, the owner of the billboards, charged a nonprofit rate.

She pointed to key participants in the effort: HDSA-San Diego board secretary Jamie Jirik; HDSA staffer Stephanie Alband; former Clear Channel Radio employee Sherry Toennies; and board member Paul June, whose branding firm designed the billboard.

“At the end of the day, this is what HDSA-San Diego is all about: grass roots efforts,” Misty said.

Misty Daniel at the 2014 San Diego Hope Walk (photo by Gene Veritas)

Catching families’ attention

“Huntington’s disease has carried a stigma for so long,” Misty said of the need for the billboards. “We continue to perpetuate the stigma generation after generation.”

The fear in HD families is “overwhelming,” she continued. That fear “is not only palpable but it is a real reason to control how we talk about” HD and share about it.

However, Misty has sensed a change in the community.

“With the launch of the Internet and social media, more and more people are confident about sharing their stories and talking publicly about Huntington’s disease,” she said. “This is what’s going to whittle away that stigma, more and more people talking about it.”

Misty hopes that the billboards will catch the attention of other HD-affected families in the San Diego area who until now have had no contact with the chapter, events, and support group.

According to Vetter, advocates in the Upper Midwest used billboards in the early 2000s. In addition, some national ad campaigns have run in magazines like TIME.

“Billboards are a great way to raise visibility of the Team Hope program and HD to the broad San Diego community,” she said. “They can spark curiosity to learn more about HD, as well as fuel engagement to take part in the Walk.

“Additionally, these billboards can validate the participation of those who are already part of our fight to help HD families. When you see an ad or a billboard about something you are involved in, it breeds pride and a deeper connection to the cause. For the HDSA San Diego Chapter, this can mean new volunteer leaders and the ability to have a greater impact.”

More than a logo

To stir broad interest, the billboards portray a family at a previous Hope Walk in another city.

Unidentified, the family has a sign with the words “I’m walking for: Ray Jacks – our brother.” (On the billboard it’s not clear whether the surname is “Jacks” or “Jackson.”)

Another angle of the billboard (photo by Gene Veritas)

As of this writing, I was unable to identity neither the family nor the person for whom they were walking. Regardless, Misty stressed that including the photo was highly important. (All HDSA walkers sign a waiver permitting use of their photos, she noted.)

“We wanted to make sure that the billboard had actual people on it, because this is a disease that has been faceless for too long,” she said. “We felt that if it had only a logo, we would be doing the Huntington’s disease community a disservice.

“No, this is not a clipart. It’s a real family.”

Later Misty hopes to reach out to the family.

“I want them to know they’re on this billboard,” she continued. “This generation is walking on behalf of those who can no longer walk for us.”

It strives for a cure, she said, to prevent another generation from suffering from HD.

Please support my family’s team, the "Serbin Family Team," in the 2016 HDSA-San Diego Hope Walk by clicking here to join us and/or make an online donation.

Featuring music, prizes, food, family fun, and more, the walk will take place at Coronado Tidelands Park, 2000 Mullinex Drive, Coronado, CA, 92118. Walk registration starts at 8 a.m. and the event at 9 a.m. Proceeds benefit HDSA.

A selfie of Gene Veritas, aka Kenneth P. Serbin, at the billboard

After a Social Security setback, HDSA steps up advocacy on Huntington’s disability criteria

The Huntington’s Disease Society of America (HDSA) is calling on the HD community and its supporters to urge the Social Security Administration (SSA) to update and expand its listing (description) of the disorder so that patients suffering from all types of symptoms can qualify for disability benefits.

Listings are medical criteria for evaluating disability claims involving diseases and disorders as determined in the Social Security Act. Written decades ago, the current SSA listing for HD mentions only the motor symptoms, the involuntary movements caused by the deterioration of the brain. However, HD also produces cognitive decline and psychiatric and behavioral issues, which usually leave patients disabled. Both of these symptom types often occur before the motor symptoms.

In the past decade, HDSA worked for the introduction of a bill in Congress, the Huntington’s Disease Parity Act, to change the HD listing and eliminate the two-year waiting period for benefits. HDSA has also directly lobbied the SSA.

Initially, the SSA responded favorably. It took a big step in April 2012 by listing juvenile onset HD as eligible for a Compassionate Allowance, thus quickening the approval of applications for benefits, which can take many months and even years and sometimes require applicants to resort to an arduous appeals process. Later that year, adult onset HD also became eligible for the allowance. (Click here to read more about Compassionate Allowance, HD, and Social Security benefits.)

However, when the SSA on February 25 released its draft of updated neurological listings – the first such revision in more than 30 years – the description for HD remained woefully inadequate.

A ‘glaring omission’

“We were certainly disappointed when the listings … made no practical advancements in the ability of their (SSA) examiners to have a true picture of the clinical manifestation of Huntington’s,” said HDSA CEO Louise Vetter in an interview today. “They continue to depend on the motor symptoms. There is a large body of knowledge obviously that gives the cognitive and behavioral symptoms equal importance in the progression of the disease.”

HDSA CEO Louise Vetter (photo by Gene Veritas)

On March 27, HDSA issued an analysis of the SSA HD listing that it posted on its website (click here to read more).

“Based upon HDSA’s analysis, this listing leaves many of the same gaps unaddressed and is worse than the listing we have now,” the society states. The proposed listing puts even more emphasis on the motor symptoms. (To obtain benefits, patients must be examined by at least one doctors and provide any medical documentation requested by SSA.)

The HDSA analysis found an “inconsistency between the listings (that do not mention any non-motor symptoms) and the preamble, which states that: ‘When these disorders result in solely cognitive and other mental function effects, we evaluate the disorder under 12.02/112.02 (for juvenile).’” The number twelve refers to the mental health listings, where general issues with neurological disorders such as HD are also described.

HDSA argues that “unless these other sections are spelled out in the listing itself, the examiner (and even a medical professional who is unfamiliar with HD) won’t know that they should look at the preamble because the person with HD appears fine in person and often lacks the insight to describe symptoms.”

The “glaring omission” of non-motor symptoms in the SSA listing “will cause individuals disabled by non-physical symptoms to be denied,” the HDSA analysis concludes.

One advocate told me privately that the SSA and HD specialists “don’t always speak the same language.” For instance, what HD specialists describe clinically as “apathy,” a hallmark symptom of HD, must be found in the SSA criteria under “anhedonia.”

Educating the bureaucracy

As of this writing, the SSA point person had not responded to my phone message requesting comment on the HD listing. Cheryl Williams of the SSA Office of Medical Listings Improvement is indicated as a contact person in the Federal Register, where the SSA published the proposed neurological listings.

Vetter offered “two plausible explanations” for the inconsistency in the neurological listing.

First, the SSA is “struggling with a change in leadership” after Commissioner Michael Astrue finished a six-year term, retiring in February 2013. Astrue had approved the Compassionate Allowances and even appeared in a video made specially for the 2012 HDSA national convention. Since Astrue’s departure, SSA has operated under an acting commissioner, Carolyn Colvin. Thus, “new staff” received the task of revising the neurological listings, Vetter observed.

Second, Vetter noted the complexity and length of a document covering numerous neurological conditions.

“I recognize that it’s a tall order to conveniently define HD,” she said. “This is a bureaucracy looking for simple definitions, and HD is not easily characterized in convenient language. From that perspective, they’re trying to simplify a complex disease. That is inevitably going to lead to some gaps, and we’re calling them out on that.”

Pressing for a meeting

Vetter added that, at some level, the SSA doesn’t sufficiently understand HD. HDSA is seeking “direct dialogue” with the SSA, but so far the SSA has refused to meet.

Vetter speculated that, if the SSA meets with HD community representatives, it fears “the slippery slope” of having to meet with all disease groups.

HDSA, Vetter said, must convince the SSA of the uniqueness and complexity of HD and therefore the need for more “guidance” for the medical examiners who determine whether HD patients receive disability benefits.

“We should be granted an exception,” she declared. “We won’t take no for an answer on this one.”

How to send a message to Social Security

HDSA CEO Louise Vetter has formally submitted the HDSA analysis as a comment for SSA consideration, as have several dozen other HD advocates and family members.

Vetter urged HD advocates, family members, and supporters to also submit comments to the SSA. You can view others’ comments and add your own by clicking here. Your can simply state that you support the HDSA's input on the Huntington's listing, or you can leave a personalized comment of greater length.

The deadline for comments is April 28.

Removing barriers to understanding HD

Advocacy on this issue forms part of a larger effort by HDSA and the HD community in favor of the Huntington’s Disease Parity Act of 2013, a bill that would direct the SSA to update and expand its HD listing and waive the two-year waiting period for HD patients to receive Medicare benefits.

The current setback is “not a reflection on the HD community’s momentum at all,” Vetter said. “I know that we still have many strong advocates in the SSA. They are trying to find a way to accommodate HD in a complex process. Hopefully they’ll be able to accomplish that quickly. I don’t think we should be disheartened.”

Little by little, HD advocates are “removing barriers” to understanding of the disease, she added.

To learn more about how you can assist with HD advocacy, contact the nearest chapter of HDSA or write advocacy Jane Kogan, HDSA’s manager of education and advocacy, at jkogan@hdsa.org.

A Huntington’s Thanksgiving message: gratitude for health and the ability to work

Thanksgiving, my favorite holiday, leads me to take stock of the many good things in my life.

First, I am thankful that I have not yet experienced the classic symptoms of Huntington’s disease. At year’s end, I will turn 54 – at least several years beyond the age of onset for my HD-afflicted mother. At my annual HD checkup last month, my neurologist said I was symptom-free.

After testing positive for HD at the age of 39 in 1999, I had expected to be incapacitated by now. Doctors and scientists still don’t know enough about HD to explain why someone like me has remained asymptomatic beyond the parent’s age of onset. As I have written in the past, I keep striving toward a well-informed strategy for avoiding onset.

I am thankful for many of the other reasons people celebrate Thanksgiving, especially family. My HD-free daughter is a thriving eighth grader, and next month my wife and I will commemorate 21 years of marriage.

This year, I am particularly thankful that, without the symptoms that typically disable HD people, I can still perform my job as a university professor.

The financial burdens of HD

This past Monday night, attending our local HD support group’s annual holiday potluck, I saw once again how Huntington’s causes the involuntary movements known as chorea and inexorably robs people of their cognitive abilities, severely hampering their capacity for work.

As a result, HD devastates families financially. When an HD-affected breadwinner loses his or her job, family income falls dramatically.

Other family members often must reduce their own work hours to help care for the sick individual. In some instances, they change occupations to adjust to care needs. They might even quit salaried work altogether.

As the story of caregivers Mike and Raima Fernald illustrates, local and state government assistance in such situations is sorely lacking. The HD community also has fought to improve access to Social Security benefits, denied to many because outdated government guidelines don’t account for HD’s cognitive and behavioral symptoms.

In the case of my parents, HD wiped out their “golden years.” Instead of enjoying retirement, my “HD warrior” dad became my mother’s full-time caregiver from the mid- 1990s until her death in 2006 at the age of 68.

Instead of trips and time with their grandchildren and friends, they struggled together to address my mom’s decreasing ability to walk, talk, and eat. In the last six months of her life, they spent several thousand dollars per month on nursing home care.

“HD is more than a disease,” I wrote as my heartbroken father precipitously lost his own cognitive abilities in the wake of my mother’s death. “It is a destroyer of families.”

One thing I fear most about the inevitable onset of my own HD is that it could put my family into a similar financial bind. Both my wife and I work full-time, and my salary currently accounts for more than half of our income.

“What if I become disabled before my daughter finishes college?” I frequently ask myself. “What if my wife must work many extra years to compensate for my lost income? What if I become a burden to my family?”

Happy to be busy

News reports about the needy at Thanksgiving remind me of the troubling backdrop to the holiday: the anemic state of our economy.

As a local radio report noted yesterday, “food stamp cuts that took effect this month will make Thanksgiving even harder for low-income San Diegans. A typical Thanksgiving meal will cost the average family nearly $50 this year, which is far beyond reach for the nearly half-million people in San Diego County who struggle every day to put food on the table — especially the 270,000 people who depend on food stamps for meals.”

So this Thanksgiving I am grateful that I can work, help support the household (including our daughter’s private school tuition), and save for the future. I’ve also enjoyed the present with family vacations and home improvements such as my just remodeled home office, which replaced a rickety and ugly setup persisting from my days as a frugal graduate student and starting assistant professor.

This year, I’m thankful for the health that has allowed me to have a highly productive year at the University of San Diego, where I am wrapping up my fifth calendar year as department chair. It’s been especially busy as my colleagues and I search to fill an exciting new position in the history of science, technology, and medicine.

With the gift of being asymptomatic, I’ve also maintained the pace of my HD advocacy, with major trips to Iowa in August and to the World Congress on Huntington’s Disease in Rio de Janeiro in September. Until today I’ve written 26 blog articles this year, my second-highest annual output since starting At Risk for Huntington’s Disease in 2005.

This is article No. 27, written with a deep sense of gratitude for the gift of life, family, and health – and the hope of effective treatments and a better future for the entire Huntington’s community.

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).

Playing in the fourth quarter of life as Huntington’s disease looms

Seeing my mother succumb to Huntington’s disease at the age of 68 and living in fear of the onset of my own symptoms, I have come to appreciate the preciousness of time.

One of my closest HD confidantes and I frequently measure time in terms of the four quarters of a football game. We see most people like us, in our early fifties, as playing somewhere in the third quarter, the prime of life.

However, I’m well into the fourth quarter. I’ve already reached my mother’s age of HD onset, and I will be extremely lucky to reach 60 without a serious reduction in my brain power and the start of chorea, the shaking and trembling experienced by most HD patients. Indeed, I cannot imagine life beyond 60, a time when my only child will be in college. I’m deeply saddened that, in an era when more people than ever are working into their late seventies and even eighties, I may have to stop in just a few years.

Because HD is inevitable, I know the symptoms will start, even as I hold out hope for some scientific breakthroughs. Maybe I’ll get a “mild” case – or maybe I’ll suffer just as badly as my mom. Like hers, most cases of HD I know devastate people physically, behaviorally, and cognitively, leaving them mere shadows of themselves.

So, these days I’m throwing long passes, aiming for touchdowns.

I’m also starting to focus on putting my affairs in order to facilitate matters for myself, as well as my wife and daughter, if HD becomes so bad that I can no longer work or take care of myself.

It’s time to prioritize. That includes stepping back a bit from this blog. Regular readers will notice that recently I’ve written much less. After a period of intense writing, I need to replenish my emotional energy.

And, in perhaps the most important process of all, I’m learning to accept my defeats, the disease, and, ultimately, my mortality.

Throwing long in the publishing world

As an activist for the Huntington’s Disease Society of America (HDSA), I’ve strived to help build awareness, although my need to remain anonymous and avoid genetic discrimination has, until recently, stymied that goal in terms of reaching out personally to people.

In mid-2010 I started to exit the terrible and lonely “HD closet” by making speeches about my family’s struggles with HD. Since then, I’ve made some ten presentations, most recently at the HDSA annual convention.

As a writer, I decided to attempt some long passes in the hope of generating greater media exposure about HD and the need to research and treat neurological disorders.

This is my moment.

Towards my goal, I’m working to publish a book about my family’s experiences with Huntington’s and scientists’ and drug firms’ quest for effective treatments. I hope to add to the excellent writings of other HD authors, including Jim Calhoun, Trish Dainton, Susan Lawrence, Carmen Leal-Pock, Sandy Sulaiman, and Alice Wexler.

How to ‘sell disease’

In today’s world, publishing a book on HD is an especially daunting challenge. With the rapid decline of traditional bookstores and the rise of the e-book, publishing is undergoing a revolution. It’s also become a virtual monopoly of an elite of blockbuster authors.

One clear message is that “disease doesn’t sell.”

Furthermore, so-called orphan diseases such as HD – with an estimated 30,000 affected people and some 250,000 at-risk – are orphans not only for the drug industry, but for the media.

Despite the terrible drama of conditions such as HD, in this information-saturated age it’s hard for people to grasp a disease that doesn’t directly affect them or loved ones.

However, in June came the encouraging news that former Palm Beach Post reporter SusanSpencer-Wendel signed a book contract for $2.3 million to chronicle how she will fulfill her “bucket list” of desires as she struggles against Lou Gehrig’s disease – a condition with approximately the same number as affected individuals as HD. She also received a seven-figure movie deal.

Indeed, disease can sell – if one has good media connections like Spencer-Wendel and discovers a way to link a story to trendy themes.

As a gene-positive HD person and HD activist, I believe disease should sell. The imminent tsunami of people affected by neurological disorders will add enormous stress on caregiving communities and the healthcare system.

Not in my wildest imagination have I thought a publisher would pay millions for a book about HD, but I do hope that, by earning at least a modest fraction of that, I could help insulate my family from financial crisis in the event of my illness and make a substantial donation to HDSA.

Focusing on the basics

As I’ve reflected on my goals, I’ve also come to recognize the danger of my ego taking my focus away from what matters most.

“Vanity of vanities!” the biblical Book of Ecclesiastes tells us. “All is vanity.”

I will continue to write about HD and strive to publish a book. However, as I head deep into the fourth quarter, other goals take on increased importance.

“Forget about the glamor,” I told myself. “Get to the basics.”

Later I quipped to myself: “God doesn’t read resumes!”

For 10 days in June, I got away from the worries of writing, career, and Huntington’s disease by traveling with my family to restful spots in northern California.

After visiting the La Brea Tar Pits in Los Angeles, we spent several days hiking in Yosemite National Park. We traversed the expansive and hot Central Valley, drove down the Avenue of the Giants in one of the state’s virgin redwood forests, strolled along the idyllic shoreline of Crescent City, took in the wild coast of Mendocino County, and celebrated our HD-free daughter’s twelfth birthday in San Francisco.

Enjoying these natural and human treasures together gave us a deeper appreciation of our home state. It also strengthened our family bonds and deepened my commitment to my daughter as she prepares to embark on a new adventure at a private school just as she enters adolescence.

At Glacier Point in Yosemite National Park

On the dock at Crescent City

Confronting the hard reality

In the HD movement we all need to strive for the big successes – such as big fundraisers, media attention, advocacy for stem-cellresearch, improved Social Security legislation, and other pressing needs.

But, as our community knows so tragically, both individuals and families need to prepare for the hard, scary reality of HD.

Instead of writing, this summer I’ve focused on dealing with the inevitable onset of symptoms – and my eventual death.

Already in January, as I prepared for the potential fallout of going more public through my writing, I had participated in an HDSA webinar on genetic discrimination. On July 11, I took part in another webinar titled “preparing for the unknown,” which discussed the importance of establishing end-of-life directives for caregivers and loved ones. On August 8, after my annual appointment for cognitive testing at the HDSA Center of Excellence for Family Services and Research, I picked up a copy of a sample advanced directive.

This summer I also reviewed the slides from a March webinar on “workplace accommodations for HD” – an especially crucial topic for me because I plan to continue as long as possible in my position as a university professor.

Receiving this information has helped me start to prepare mentally, emotionally, and logistically for the onset of HD

Putting things in order

In recent months I’ve fantasized a lot about retirement – from both my career and the HD movement.

“Our culture thinks it’s cool to be exhausted,” I wrote recently in my notes about this fantasy. “We wear it as some kind of badge of honor. I myself have been like this. But it’s absolutely nuts! I need to pace myself, keep getting down time. It’s so true what I’ve heard in Brazil: Americans live to work, Brazilians work to live.”

In particular, this summer I’ve also felt a powerful urge to put my life in order, especially those areas I’ve long neglected because of time spent on HD activism. In the fourth quarter, it’s time to take stock of my life – and to enjoy doing so.

I began by transferring the songs from several hundred music CDs onto iTunes. Listening to many of these songs for the first time in decades brought a flow of good memories from my twenties and thirties.

Next, I reorganized my home office for the first time since we moved to this home in September 1999. I threw away garbage bags laden with hundreds of old 3.5-inch diskettes, checks and check registers going back to the early 1990s, and numerous other unneeded items.

I like the idea of traveling lighter on my journey with HD and through life.

I finally caught up on our home movie collection, started scanning old family photos that are beginning to fade, and filed work and HD-related CDs and DVDs in a storage case I had bought about four years ago.

I like caring for plants. I potted three new ones and placed them by the window. It felt great to get my hands dirty and to smell the soil. Sunday evening is watering time.

What causes this desire for order? The natural rhythm of life? A side effect of HD’s subtle psychiatric symptoms, which can include obsessive-compulsive behavior? Just plain fear of onset?

Whatever the cause, the greater sense of order has brought me a sense of comfort, of preparedness for HD and whatever else life might bring, of living the moment.

Shifting passions, accepting fate

I’m in a fight for my life against HD. Ironically, that means that perhaps it’s time to stop fighting so hard. Fighting too hard can worsen stress. A positive family life, exercise, tranquility – these are the real keys to personal survival.

I have a stable job, a loving family – and the tremendous gift of so far having avoided HD’s classic symptoms.

Tranquility and stability will help me negotiate the dramatic shift in my professional career from an emphasis on Latin American history to the history of science and the chronicling of the HD movement.

In one of my recent dreams, I plunged down a Rio de Janeiro hillside on the back of a wheelchair driven by a disabled man – undoubtedly an HD man – who, like my mother, could not speak.

I used to value traveling to Brazil. Savoring those experiences brings a warm glow to my heart. As a professor and father, I pass on those experiences to the next generation.

Now I’m becoming excited about new kinds of travel: through the biotechnological revolution, through my own mind in search of its meaning.

Yet, despite the vast progress in brain research of recent decades, the drug industry still has not produced a single remedy for neurological disorders. Although I never abandon hope, I also understand that a treatment may not arrive in time to save me.

Ultimately, tranquility and stability will help me prepare spiritually for the onset of HD: the realization that, in the end, I must accept my fate.