ALS Patient Care - Past, Present and Future: A Conversation with The ALS Association's Vice President of Patient Services

The Continued Conversation with Mary Lyon, The ALS Association's Vice President of Patient Services

HOPE: How did you decide to enter the ALS patient services field?

LYON: When I was at UCLA, I saw an advertisement for my current position at The ALS Association in a nursing magazine. I began to think about the people with ALS for whom I had given professional care. And, I recalled two personal experiences with neighbors who had ALS. It all came together for me at that time, and I embarked upon one of the most rewarding and challenging jobs that I've ever had.

HOPE: Do you enjoy the challenge?

LYON: Being in contact with patients frequently is one of the tremendously inspiring and fulfilling aspects of my job. People with ALS inspire me; they motivate me to do the hard things necessary to develop innovative programs and services that make a difference for large numbers of people. It's also about improving the approaches and knowledge for healthcare professionals who are on the front lines in caring for people with ALS. I couldn't ask for more in my work.

HOPE: How has medical management of ALS changed?

LYON: For more than a decade, there have been clinicians in the ALS community leading the way toward positive, meaningful and comprehensive care addressing the wide range of symptoms ALS presents. The ALS Association Centers build on this philosophy by offering a multidisciplinary team approach integrated with chapter staff who coordinate home and community services. In years past, people with ALS didn't get as much information or support as they do today. Often, they didn't see their physician on a regular basis. The literature indicated that people with ALS didn't have pain. Today, we understand there can be certain kinds of pain, and it can be treated. We can help people make choices to increase their survival and live a fuller life with this disease. Helping people be comfortable, reduce anxiety, minimize difficulties with breathing and managing secretions, and psychological symptoms; all this is relatively recent in the 160 year history since ALS was first described. In addition, there is a focus on providing care and support for caregivers, especially as the time of death draws near. Today, it is not uncommon to find physicians and other healthcare professionals in the home - whether through hospice or through the The ALS Association services - to relieve suffering, provide comfort and encourage people to resolve issues with family members, to get closure and to find peace in areas of their lives before they die.

HOPE: Please describe the innovative areas of clinical management research that The ALS Association is funding to improve living with ALS.

LYON: The ALS Association is the leader in ALS clinical management research, and this is one of it's most exciting programs. We began the program in 1998 and have funded 14 studies. This research ranges from looking at how exercise programs might help people with ALS, to the benefit of early non-invasive ventilation, to nutritional research, to looking at how to measure quality of life, cognitive changes and how they impact people with ALS.

HOPE: How has end-of-life care evolved?

LYON: We believe that a palliative care approach should be used throughout the whole course of the disease, and that there really is not a particular turning point where care changes. The precepts of palliative care - relief of suffering, dignity, prevention of pain, support, and quality of life - are applied from the time a person receives the diagnosis through the entire course of the disease. However, the intensity of those services may change over the course of the disease. Early on, when a person is still employed and physically active, palliative care is likely to be less intense than near the end of someone's life. It's a change in philosophy that looks at living to the fullest, maximizing function and fighting this disease continually throughout its course. The palliative approach augments this perspective by supporting the person in their choices, minimizing suffering, minimizing pain, and helping with the psycho-social pain. Within the last few years, The ALS Association has taken a leading role in developing a "peer" work-group report (http://www.alsa.org/resources/continuum.cfm) on how we can improve end-of-life care for people with ALS, recognizing that many people in the healthcare community are ill-equipped to provide good palliative care. This report evaluated the current status of dying with ALS in America, and how to improve care - then offered a series of recommendations to provide better palliative care, throughout the disease progression. A key recommendation is improved medical management, which includes education for healthcare providers, education and information for patients and families, as well as changing healthcare policy to provide access to and reimbursement for hospice by changing the Medicare guidelines for eligibility and improving reimbursement for palliative care interventions.

HOPE: What forces are responsible for changes in patient care that you see coming in the future?

LYON: There are many things that we can look at today, think about where we were several years ago, and predict what the next five or ten years might hold. We have an aging population in the United States. We know that ALS is a disease of aging with the average onset, statistically, at about the fifth decade. So as more and more people move into the later decades of life, we expect to see more people developing ALS, and the number of people living with ALS at any one time in the United States will certainly rise. There are also observations and thoughts that perhaps the incidence of ALS is increasing. We don't have statistical evidence from scientific studies to validate this observation conclusively, and more research in this area is needed. The other trend that is important for us to know about is that there are more options for living longer with ALS, with a good quality of life. I'm referring to noninvasive and invasive ventilation, the feeding tube and a more aggressive approach to the symptoms of ALS. As technology improves, more people are likely to choose interventions that will extend their lives.

HOPE: Given these changes and the expectation that more people may have ALS in the future, what preparations need to be made?

LYON: We need to be planning now for an increase in the number of people in the U.S. getting ALS, based on an increasing number of people and an aging of our population. Also, we need to prepare for people with the disease to live longer. That means The ALS Association will expand services. We're planning to build resources - financial and human resources - to be able to have the infrastructure through our chapters, support groups, clinics and certified ALS Association Centers to provide more services to more people in more areas of the United States. The medical community is considering how to expand medical training programs for physicians and other healthcare providers. Another big consideration is how will the medical insurance benefits keep up with an increasing population of patients and more patients choosing interventions that will allow them to live longer and therefore increase costs? Other considerations are out-of-pocket expenses and costs for long-term care. In addition, the impact on the family caregivers and other family members can be significant, and we need to begin thinking about how to support not only the patient's needs, but also those of the family.

HOPE: How has our thinking changed about the affect of ALS on cognitive function?

LYON: As far back as about 80 years ago, notations in the medical literature observed cognitive changes linked to people with ALS, but there wasn't much investigation of these observations. Within the last several years, there has been an increasing recognition that some people with ALS also have a degree of cognitive dysfunction, abnormality or even dementia. The source of deficits or cognitive abnormalities being observed and measured in the studies have to do with verbal fluency, judgment or multiple daily function - putting the pieces of activities together in a meaningful way. Sometimes, people with ALS and cognitive impairment - when presented with a number of options for their care - either choose not to accept any of the options, or they may be willing to accept them, but don't use equipment or participate in the therapy. Ironically, sometimes being aware of the cognitive difficulties that can be associated with ALS can help the family have a better understanding of the patient's behavior. In ALS, the cognitive dysfunction can operate on a more subtle level than what we associate with Alzheimer's disease.

HOPE:Are subtle cognitive changes sometimes overlooked?

LYON: Yes, perhaps the reason cognitive changes haven't been recognized in the past is not because they weren't there, but because there are so many things to deal with in ALS, such as psychological issues and communication difficulties. Understandably, people will be angry, depressed or anxious; perhaps these emotions mask what's now being observed and described more as physiologic cognitive changes.

HOPE: What type of clinical management programs or interventions comes directly from research that The ALS Association has funded?

LYON: We have four completed studies that have been published. A promising new instrument is in the final stages of development that could more effectively measure the quality of life of ALS patients. This is important because we measure the impact of clinical trials or medical interventions on the quality of life along with the effectiveness. When evaluating a potential new medication, we want to know if it's effective. However, we also want to know the impact of any side effects on the patient, and the quality of life measure provides this. Another study The ALS Association is funding indicates a benefit in longer survival for patients who use noninvasive ventilation in the early stages of the disease. We're sharing this information throughout the ALS community.

HOPE: What are some of the suspected risk factors in ALS?

LYON: Today, very few risk factors can unequivocally be linked to ALS. One of the few risk factors is gender. Men tend to develop the disease more often than women until women reach the age of menopause, and then the incidence is about equal. Another risk factor is age. We know ALS is a neurodegenerative disease of aging. That's not to say that younger people - people in their twenties and even sometimes in their teenage years - don't get ALS. They do. But statistically more people get the disease in the later decades of life. There have been numerous studies of potential risk factors over many decades - some positive, some negative - but none that have been absolutely conclusive. The NIEHS (National Institute of Environmental Health Sciences) continues to investigate environmental health risk factors. There are studies looking at the potential risk factors of smoking and diet, as well as toxic environmental and occupational exposures. Also, a study of 1991 Gulf War veterans compared solders who were deployed with those who had not been deployed. Among the group deployed, nearly two times as many developed ALS. More and more, there is interest within the scientific community to look at genetic susceptibility along with an environmental exposure that may play a role in the genesis of ALS.

HOPE: Describe what happens when a person diagnosed with ALS and their family come to a chapter of The ALS Association.

LYON: The patient and the family are welcomed as a part of The ALS Association family. They are assured that, as difficult as ALS is to face, they are not alone. There are people in the community who will be with them every step of the way, and The ALS Association staff has information, services, answers, support, resources, a shoulder to cry on and a person to laugh with - someone who really understands.

HOPE: What is an ALS Association Center?

LYON: The centerpiece for medical management of ALS is our 19 certified ALS Association Centers. In partnership with our local chapters, the certified centers provide the highest level of care and coordination of care available and are truly centers of excellence. Patients and their families who attend ALS Association Centers for their care will have a very unique experience. There is a comprehensive team of experts that include the neurologist; social workers; nurses; and physical occupational, respiratory and speech therapists, plus a liaison from the local chapter. These professionals provide expert care and education as well as treatment interventions to aggressively manage the symptoms of ALS. This is done in a team approach, and the patient and their family are vital members of the team. In fact, the patient drives the decisions and the plan of care. Nowhere else is there a partnership between local chapter and the medical team to plan care that crosses all of the traditional medical and healthcare system boundaries. The ALS Association Center program provides individual care for patients, whether they're in the hospital, in the clinic, at home or another facility.

HOPE: How does an ALS Association Center become certified?

LYON: The requirements are quite rigorous. Essential among them is the requirement to have a full, multi-disciplinary team. In addition, we're looking for centers involved in research. Most ALS Association Centers are conducting clinical trials or clinical management studies in which patients can participate. A center may have basic science laboratory research or clinical management research going on in their clinic. And, they must adhere to the standards The ALS Association has set for excellence.

HOPE: Are there plans to certify more ALS Association Centers?

LYON: We are very interested in increasing the number of ALS Association Centers so more people with ALS across the country will be able to have reasonable geographic access to one or more of them.

HOPE: What is an ALS clinic?

LYON: The ALS Association, through its 40 chapters, has a number of relationships with ALS clinics that have not yet been certified as ALS Association Centers. The clinics have a close partnership with a local chapter, and in most cases, these clinics are working toward certification, have a multi-disciplinary approach to care and a commitment to the criteria and the mission of the ALS Association Center program.

HOPE: What clinical management research do you think holds the most promise for the near future?

LYON: We need to learn more about what will improve survival, function, muscle weakness, and quality of life, and be able to offer those treatments and interventions to patients. This will go a long way toward improving living with ALS. There is technology being developed and refined that we hope will one day enable people to communicate their thoughts and needs even if they are fully paralyzed and can't use traditional communication devices. There will be a huge impact if we are able to develop ways to support breathing beyond even what we're able to do now with non-invasive and invasive ventilation, other assistive devices, other procedures that perhaps patients and families administer at home that can help keep the pulmonary and respiratory systems stronger for a longer period of time. We're doing some early exploration into that kind of treatment. Also, perhaps there's an opportunity, early in the disease, to provide a nutritional plan that would help people with ALS maintain strength longer.