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By Larry Beresford

Clare Langley has seen a lot of liv-ing—and dying—in the past sev-

en years. A month after the Rockville, MD, resident became engaged to Ray Langley, a microbiologist at the Na-tional Institutes of Health, her father died under the care of a Maryland hos-pice. Shortly before she married Ray, he began to show symptoms of muscle weakness. He received a definitive di-agnosis of ALS—amyotrophic lateral sclerosis, a fatal, incurable neurologi-cal condition—soon after the birth of their first child, Mary.

Their second child, Cathleen, was stillborn at full term, and their third child, Kevin, was born just three months before Ray died in December 2003. With a lot of advance planning, Ray was able to be in the delivery

room for his son’s birth, although by that point he was wheelchair-bound and could no longer speak. “We had developed a tremendous way of con-versing without words. I could tell what he was telling me by his looks and his emotions,” Clare recalled in a recent interview at her home.

For the last 18 months of his life, Ray and his family also benefited from the interdisciplinary support of Montgomery Hospice, which has established a reputation for its open-ness to ALS patients and families and its skill in managing the physical and psycho-social symptoms of this in-credibly trying and tragic diagno-sis. Ray, who is remembered for his spirit, his patience and his resourcefulness in confront-ing his illness, was able to die at home, comfortably

Hospice Stretches in Response to the Enormous Needs of ALS Patients

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and with his family sleeping peace-fully around him.

Approximately 5,600 new cases of ALS are reported in this country each year. Half survive more than three years after diagnosis and an es-timated 30,000 Americans today are living with ALS. The disease relent-lessly attacks motor neurons. As they atrophy and die, the patient gradu-

Improving Quality: Time to Act A Conversation with Don Schumacher

by Merilyn Francis

Quality is the hot topic across the spectrum of healthcare systems

since the Institute of Medicine (IOM) released its two sentinel reports: To Err is Human: Building a Safer Health System (1999) and Crossing the Quality Chasm: A New Health System for the 21st Century (2001).

According to these reports, “[t]he U.S. healthcare delivery system does

not provide consistent, high-quality medical care to all people. Americans should be able to count on receiving care based on their needs and...the best scientific knowledge....” THis means hospices and palliative care organizations need to become full partners in the efforts to improve the quality of care.

Since their release, the Center for Medicare and Medicaid Services

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For more information on ALS, visit www.ALSA.org

Lead Story: ALS

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ally loses the ability to control all vol-untary muscles, eventually including those used to speak, eat and breathe. Often, however, mental faculties are unaffected, leaving an active mind in a paralyzed body.

Because there is no cure for ALS, ex-perts say the primary focus of treat-ment is on symptom management, quality of life and adaptation to the many losses experienced by patients and families—in short, palliation. As a result, hospice care, with its skills in psycho-social-spiritual and family support and maximizing quality of life, is well suited for ALS patients. It does, however, require learning some new skills and gaining comfort with treatments and equipment that are not customarily used in hospice care. An example is non-invasive positive pressure ventilation (NIPPV), also known as BiPAP, which provides re-lief for symptoms of dyspnea and sleep-disordered breathing.

Although existing national hospice eligibility criteria for ALS patients have been questioned by research-ers and providers for not identifying appropriate patients soon enough, Montgomery Hospice is one of a growing number of providers that have embraced the challenges of this disease by encouraging appropriate and timely admissions, document-ing their rationale for admissions and collaborating closely with the local chapter of the ALS Association and local ALS clinics that care for these patients.

Montgomery Hospice nurse case manager Michele Orr, who has be-come a champion for ALS patients and their needs within her agency,

sees ALS patients as “heroes” for their courage and endurance. “Each pa-tient is a challenge. You have to figure out how to communicate with each one,” she says. “It’s also a disease of symptom management. Each time I get a new ALS patient, there’s a new symptom.”

Clare Langley says the hospice care her husband received was not just help-ful, it was essential. “Hospice stepped in and really got to know our needs. Between Hospice and the ALS As-sociation, we had all the information we needed,” she explains. “I can’t say enough about what Hospice brought to our family. We really needed it for the sanity of our family to keep go-ing and for the expertise in knowing what was likely to happen next.”

Since Ray’s death, Clare and her five-year-old daughter have attended be-reavement support meetings through Montgomery Hospice’s Connections program. “What Mary wants is to know that other children are in a similar situation to hers. What I like about it is being able to tell your story again to somebody who is going to listen. It helps you cry a little more. My life is so busy sometimes, I feel like I’m not crying enough.”

A National Commitment to Access

Given its commitment to promot-ing timely and appropriate access to high-quality hospice and pallia-tive care for all Americans in need, NHPCO is planning a series of disease-specific technical assistance monographs to explore the challenge and opportunities of selected non-cancer diagnoses. These reports will explore hospice eligibility issues, the current state of the art in prognos-tication, specific clinical challenges, tools and resources to help providers master those challenges, and real-world stories of hospices that have mastered the disease and the patients they serve.

ALS is the first non-cancer diagno-sis to receive such a treatment, with the planned publication of an ALS monograph and proposed new inter-im guidelines for hospice eligibility for ALS patients planned for the fall of 2005. This article is a brief over-view of the topics that will be cov-ered in greater depth in that mono-graph—which will be distributed to all NHPCO member hospices and a select list of other professionals. The aim for the ALS monograph and the other disease-specific reports is to en-courage hospices to make an affirma-tive commitment to expanding ac-cess for the disease in question—and to provide tools and inspirational stories to help them fulfill that com-mitment.

New interim guidelines for deter-mining prognosis in ALS have been developed by a national work group convened by NHPCO. Similar work groups eventually will revisit all of

. . .“I can’t say enough about what Hospice brought to

our family.”—Clare Langley

. . .

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Lead Story: ALS

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the non-cancer diagnoses contained in the National Hospice Organi-zation’s (NHO) 1996 document, Medical Guidelines for Determining Prognosis in Selected Non-Cancer Di-agnoses, (1996). “What we had then is not as good as what we have now in the evidence base. We’d like to get more appropriate patients and plan a continual process of improve-ment” for the prognostic guidelines, says Dr. Stephen Connor, NHPCO’s Vice President of Access for End-of-Life Care, Research and International Development.

NHPCO’s Vice President for Medi-cal Affairs, Dr. Perry Fine, has shared the draft interim ALS guidelines with Medicare’s hospice fiscal intermedi-ary medical directors. Four out of five responded favorably in principle to the idea of improved, evidence-based prognostic criteria for hospice eligibility. It is not known when—or if—they will formally adopt new eligibility criteria based on advances in the medical literature, Fine says. However, in the meantime, NHPCO aims to give hospices new tools to document why they think a patient is appropriate for admission.

The new ALS prognostic criteria are based on research by Dr. Leo Mc-Cluskey of the University of Penn-sylvania. McCluskey’s ALS clinic at Penn referred 97 consecutive patients it considered hospice-appropriate to hospices in the region. All died un-der hospice care within an average of 85 days. However, only five of the 97 would have met Medicare medi-cal criteria based on NHO’s “Medi-cal Guidelines” at the time of their hospice admission.

McCluskey is an advocate for hos-pice care for ALS patients. “Virtu-ally all of our patients who don’t opt for mechanical ventilation get hos-pice. But in my view, the bar is set too high for hospice eligibility,” he says. “These are very difficult cases; the ‘system’ should not be.” Several years ago, he contacted Wissahickon Hospice, also part of the Penn sys-tem, with specific ideas about how he’d like to work with hospice. “We met with Dr. Leo and I sent my staff through his training,” says Lisa Stan-ley, director of home care and hos-pice services at Penn.

In the fiscal year ending June 30, 2005, Wissahickon Hospice admit-ted 14 patients with ALS, approxi-mately one percent of its total ad-missions for the year. They averaged 163 days on service, compared with a length of stay of 45 days for all pa-

tients. “Fourteen patients a year is hardly a tidal wave, but it is a signifi-cant enough start to have taught us the value of taking the risk,” says M. Christine Kriebel, Wissahickon’s di-rector of patient services. “I don’t feel we are ALS experts. The benefit we bring is that we were willing to make this commitment. We said to the ex-perts, ‘Teach us about your disease,’” she says. “I also think there’s a cer-tain strength and tenacity about so many ALS patients. They have also helped to teach us to think outside of the box.”

“We look at hospice in such a posi-tive way,” adds Dallas Forshew, nurse practitioner for the ALS clinic at the University of California-San Francis-co. “We try to get our patients into hospice as early as possible. We want patients to have a good experience, and we think hospice wants that, too.” Hospices can learn to do ALS care well with a knowing mind and an open heart, she believes. “But you have to trust ALS physicians’ profes-sional judgment about the appro-priateness of referrals. We know this population. We’re not often wrong.”

Larry Beresford is a freelance writer based in Oakland, CA and a fre-quent contributor to NHPCO pub-lications. You can reach him at larryberesford@hotmail.com.

Lead Story: ALS

. . .“(ALS patients) have

helped to teach us to think outside the box.”

—M. Christine Kriebel. . .

ALS is commonly referred to as “Lou Gehrig’s Disease.”