Hospice Stretches in Response to the Enormous Needs of ALS Patients

Lead Story: ALS

room for his sons birth, although by that point he was wheelchair-bound lare Langley has seen a lot of liv- and could no longer speak. We had ingand dyingin the past sev- developed a tremendous way of conen years. A month after the Rockville, versing without words. I could tell MD, resident became engaged to Ray what he was telling me by his looks Langley, a microbiologist at the Na- and his emotions, Clare recalled in a tional Institutes of Health, her father recent interview at her home. died under the care of a Maryland hospice. Shortly before she married Ray, For the last 18 months of his life, he began to show symptoms of muscle Ray and his family also beneted weakness. He received a denitive di- from the interdisciplinary support agnosis of ALSamyotrophic lateral of Montgomery Hospice, which has sclerosis, a fatal, incurable neurologi- established a reputation for its opencal conditionsoon after the birth of ness to ALS patients and families and their rst child, Mary. its skill in managing the physical and psycho-social symptoms of this inTheir second child, Cathleen, was credibly trying and tragic diagnostillborn at full term, and their third sis. Ray, who is remembered for child, Kevin, was born just three his spirit, his patience and his months before Ray died in December resourcefulness in confront2003. With a lot of advance planning, ing his illness, was able to Ray was able to be in the delivery die at home, comfortably
By Larry Beresford

and with his family sleeping peacefully 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 estimated 30,000 Americans today are living with ALS. The disease relentlessly attacks motor neurons. As they atrophy and die, the patient graducontinued on page 5

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

Improving Quality: Time to Act

A Conversation with Don Schumacher
by Merilyn Francis

uality 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 September 2005

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 scientic knowledge.... THis means hospices and palliative care organizations need to become full partners in the eorts to improve the quality of care. Since their release, the Center for Medicare and Medicaid Services
continued on page 7

continued from page 3

Lead Story: ALS sees ALS patients as heroes for their courage and endurance. Each patient is a challenge. You have to gure out how to communicate with each one, she says. Its also a disease of symptom management. Each time I get a new ALS patient, theres a new symptom.

ally loses the ability to control all voluntary muscles, eventually including those used to speak, eat and breathe. Often, however, mental faculties are unaected, leaving an active mind in a paralyzed body. Because there is no cure for ALS, experts say the primary focus of treatment is on symptom management, quality of life and adaptation to the many losses experienced by patients and familiesin 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 relief for symptoms of dyspnea and sleep-disordered breathing.

A National Commitment to Access

Given its commitment to promoting timely and appropriate access to high-quality hospice and palliative care for all Americans in need, NHPCO is planning a series of disease-specic technical assistance monographs to explore the challenge and opportunities of selected noncancer diagnoses. These reports will explore hospice eligibility issues, the current state of the art in prognostication, specic clinical challenges, tools and resources to help providers master those challenges, and realworld stories of hospices that have mastered the disease and the patients they serve. ALS is the rst non-cancer diagnosis to receive such a treatment, with the planned publication of an ALS monograph and proposed new interim guidelines for hospice eligibility for ALS patients planned for the fall of 2005. This article is a brief overview of the topics that will be covered in greater depth in that monographwhich 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-specic reports is to encourage hospices to make an armative commitment to expanding access for the disease in questionand to provide tools and inspirational stories to help them fulll that commitment. New interim guidelines for determining prognosis in ALS have been developed by a national work group convened by NHPCO. Similar work groups eventually will revisit all of
continued on next page

... I cant say enough about what Hospice brought to our family. ...
Clare Langley

Clare Langley says the hospice care her husband received was not just helpful, it was essential. Hospice stepped in and really got to know our needs. Between Hospice and the ALS Association, we had all the information we needed, she explains. I cant say enough about what Hospice brought to our family. We really needed it for Although existing national hospice the sanity of our family to keep goeligibility criteria for ALS patients ing and for the expertise in knowing have been questioned by research- what was likely to happen next. ers and providers for not identifying appropriate patients soon enough, Since Rays death, Clare and her veMontgomery Hospice is one of a year-old daughter have attended begrowing number of providers that reavement support meetings through have embraced the challenges of this Montgomery Hospices Connections disease by encouraging appropriate program. What Mary wants is to and timely admissions, document- know that other children are in a ing their rationale for admissions and similar situation to hers. What I like collaborating closely with the local about it is being able to tell your story chapter of the ALS Association and again to somebody who is going to local ALS clinics that care for these listen. It helps you cry a little more. patients. My life is so busy sometimes, I feel like Im not crying enough. Montgomery Hospice nurse case manager Michele Orr, who has become a champion for ALS patients and their needs within her agency, September 2005

the non-cancer diagnoses contained in the National Hospice Organizations (NHO) 1996 document, Medical Guidelines for Determining Prognosis in Selected Non-Cancer Diagnoses, (1996). What we had then is not as good as what we have now in the evidence base. Wed like to get more appropriate patients and plan a continual process of improvement for the prognostic guidelines, says Dr. Stephen Connor, NHPCOs Vice President of Access for End-ofLife Care, Research and International Development. NHPCOs Vice President for Medical Aairs, Dr. Perry Fine, has shared the draft interim ALS guidelines with Medicares hospice scal intermediary medical directors. Four out of ve responded favorably in principle to the idea of improved, evidencebased prognostic criteria for hospice eligibility. It is not known whenor ifthey 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 McCluskey of the University of Pennsylvania. McCluskeys ALS clinic at Penn referred 97 consecutive patients it considered hospice-appropriate to hospices in the region. All died under hospice care within an average of 85 days. However, only ve of the 97 would have met Medicare medical criteria based on NHOs Medical Guidelines at the time of their hospice admission.

Lead Story: ALS

... (ALS patients) have helped to teach us to think outside the box.
M. Christine Kriebel

tients. Fourteen patients a year is hardly a tidal wave, but it is a signicant enough start to have taught us the value of taking the risk, says M. Christine Kriebel, Wissahickons director of patient services. I dont feel we are ALS experts. The benet we bring is that we were willing to make this commitment. We said to the experts, Teach us about your disease, she says. I also think theres a certain 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 positive way, adds Dallas Forshew, nurse practitioner for the ALS clinic at the University of California-San Francisco. 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 professional judgment about the appropriateness of referrals. We know this population. Were not often wrong. Larry Beresford is a freelance writer based in Oakland, CA and a frequent contributor to NHPCO publications. You can reach him at larryberesford@hotmail.com.

...

McCluskey is an advocate for hospice care for ALS patients. Virtually all of our patients who dont opt for mechanical ventilation get hospice. But in my view, the bar is set too high for hospice eligibility, he says. These are very dicult cases; the system should not be. Several years ago, he contacted Wissahickon Hospice, also part of the Penn system, with specic ideas about how hed like to work with hospice. We met with Dr. Leo and I sent my sta through his training, says Lisa Stanley, director of home care and hospice services at Penn. In the scal year ending June 30, 2005, Wissahickon Hospice admitted 14 patients with ALS, approximately one percent of its total admissions for the year. They averaged 163 days on service, compared with a length of stay of 45 days for all pa-

ALS is commonly referred to as Lou Gehrigs Disease.

September 2005