December 22, 2006
Research and Clinical Update — from ALSA’s National Office
Yokohama Symposium Reports --Clinical Sessions
Summary by Jennifer Brand, MPH, Director, Patient Services
Allied Professional’s Forum
Jette Moeller, OT, has worked with the Danish Association for Neuromuscular Disorders, has been an ALS counselor, and she has recently been concentrating on the study of different ways of coping with ALS/MND. Her study objectives were to acquire more knowledge about which personal factors are important to ALS patients and their spouses in order to cope with the physical, psychological, economical and practical problems that arise from having ALS; and which coping strategies can help people to have a good quality of life despite having ALS. Preliminary results of a questionnaire developed to assess these items are as follows: finding life meaningful and manageable for ALS patients seems to depend on what the individual patient considers important in order to preserve quality of life; and information provided by professionals, degree of disability and dependence on physical help are important aspects, but not crucial, for coping with the disease.
Twenty-five percent of ALS/MND patients begin their disease process with bulbar symptoms. Up to 80% of all ALS/MND patients will, at some point in the disease process, develop bulbar symptoms. The resulting dysphasia and swallowing difficulties were studied by Julia Johnson, a speech language pathologist. Ms. Johnson works at King’s College Hospital Neurosciences Centre in London, U.K., as Head of the Neurology SLT service. The objectives of her study were to assist timely management of patients’ nutrition and hydration to avoid complications of aspiration pneumonia, malnutrition and dehydration and to determine whether a patient’s pleasure from eating orally influences their decision to have PEG placement. It was found that patients that did still find pleasure in oral intake were less likely to opt for PEG placement.
Rachael Marsden, a qualified nurse, works with the Oxford MND Care Center as part of the MND team in the U.K., and Rachel Botell, a special registrar in neurological rehabilitation who works at the Oxford Center for Enablement in the U.K., discussed sexual well being in the MND clinic. They used the Ex-PLISSIT (Permission, Limited Information, Specific Suggestions, Intensive Therapy) model to discuss sexual relationships of the patients. They found that despite their own awareness and comfort in talking about sexuality, it was rarely discussed in their practice. For a six month period, they focused on asking all ALS/MND patients directly about sexual well being. Challenges included when and how to raise the subject within the consultation and questioning assumptions on age, gender and disability. It was more difficult to initiate such conversations with patients they had known longer as opposed to patients new to the center. Bed mobility and sleeping issues were found to be good topics to prompt discussion about sexual well being and relationships. Using the Ex-PLISSIT model allowed for discussion among team members, exchange of knowledge, and promoted review with the patient at subsequent visits.
Dorothee Lule, Ulrike Singer and Albert-C Ludolph from the Department of Medical Psychology and Behavioral Neurobiology at the University of Tubingen, Germany, explored the caregivers situation and how it influences his/her relationship to the patient with ALS. It was found that caregivers had considerable constraints on their life that influenced their mood and quality of life. All did feel, however, that they had sufficient time off from their caretaking duties. The caregiving did not interfere significantly with interpersonal relationships, such as those with children or parents. The relationship between patients and caregivers was perceived as better than before diagnosis and in more than half of the cases, conflicts between caregivers and patients actually decreased. Patients were also perceived as positive people. All caregivers in the study perceived their duties as important and rewarding, and the more severe the patients’ disability became, the more reward the caregivers felt.
Family caregivers’ experiences of bodywork in ALS/MND (managing the body of a loved one with ALS) were discussed by Robin Ray, a lecturer in nursing at La Trobe University in Melbourne, Australia. It was found that caregivers are not well prepared for the realities of managing the physical and emotional demands necessary to care for their loved one. Caregivers expressed difficulty in coming to grips with the changing bodily function and the personal tasks they have to do for their loved one. These issues cross recognized boundaries of intimacy and rules that govern their relationship. Family caregivers need assistance with physical care and psychological support, but the form that support takes needs to be negotiated within the social structure of the family.
Developing a care model for ALS patients with expert cooperation in Japan was presented by Fumie Tsubai, a medical social worker to National Hospital Organization National Miyagi Hospital ALS Care Center. In Japan, an initial diagnosis is usually presented to the family rather than the patient. The family then decides whether or not to notify the patient of the disease. There is also great variation in the care of patients among different hospitals as well as physicians. In 2002, the Japanese Society of Neurology developed the Guidelines for the Treatment of ALS in an effort to standardize care of those with ALS. The guidelines do define “notification of the disease” as notification to both the patient and family; however, the implementation of these guidelines is unsatisfactory. It is suggested that working with a multi-expert team beginning early in the care of someone with ALS may be effective in facilitating the understanding of the disease among the patient and his/her family.
Yumiko Kawaguchi is the president of the company Care Support MOMO and is the director of Japan ALS Association and Non-Profit SAKURA. Her mother was diagnosed with ALS in 1995, and Yumiko became her mother’s caregiver. Yumiko found caregiving difficult and established MOMO to provide caregivers for families living with ALS. Her presentation focused on the SAKURA Model for advancing the personal assistant system for PALS in Tokyo. Through this model, PALS in Japan are provided with caregivers from three public home care systems. The SUKARA Model sets out to train personal assistants. The program consists of a monthly course that offers a 20 hour lesson for 10 to 40 personal assistants at a time. The course provides 9 hours of lecture on diet, communication, respiratory management, respirator mechanism, sanitary control, suction technique, legal system, professional ethics, empowerment and methods of transfer. There is also an 11 hour practical training on tracheotomy suctioning. The program has succeeded in providing more caregivers and increasing the knowledge/skills of those providing the care.
Developing an automatic suction system for patients on TPPV was presented by Dr. Makoto Yamamoto, Sub Director of Oita Kyowa Hospital and research member of the auto suction system at the Ministry of Health, Labour and Welfare in Japan. Many patients in Japan undergoing tracheotomy choose to be treated at home, and this creates a great burden on the primary caregiver. The use of an automated suction device at home could relieve the caregiver of some of the burden they carry. The system developed by Dr. Yamamoto could be used long term for patients at home because it proved to have a high level of safety.
Josee Lemoignan, BSc OT, MSc, Bioethics Candidate, Montreal Neurological Hospital, explored how individuals with ALS make decisions about assisted ventilation. The purpose of his research was to understand the experience of individuals with ALS in making choices about treatment options to relieve respiratory distress and subsequent respiratory failure. It was found that the timing of information provided to those making the choice was of major importance in assisting people with ALS to make informed decisions. Health care professionals need to provide patients more than just information on the medical/technical aspects of ventilation if the tenets of informed consent are to be respected in clinical practice.
Dilemmas associated in withdrawing tracheotomy ventilation at home were explored by Dr. Richard Sloan, Medical Director, Joseph Weld Hospice, Dorchester, UK. The use of ventilation in MND patients is becoming more common. Patients in the UK have a choice to stop ventilation whereas in Japan, this is not an option. Before beginning tracheotomy ventilation it is important that the ethics and legalities of a patient’s request to stop ventilation are understood and discussed. The emotional impact of withdraw on families and well as professionals also needs to be anticipated and discussed.
International Symposium on ALS/MND
Caregiver Support
R.A. Ray of La Trobe University, Melbourne, Australia, studied the impact of ambiguous loss and emotional labor on family caregivers. Family caregiver’s loss and the impact it had on their ability to provide effective care in ALS/MND were studied. The ambiguous nature of loss arising from the uncertainty associated with disease progression was found to compound loss. Caregivers’ feelings of emotional labor and ambiguous loss often go unnoticed but do have a great impact on the ability of the caregiver to provide continued care. Strategies such as identifying a possible confidant within the social support network and providing connections to other caregivers may enable family caregivers to manage loss more effectively.
Informal caregivers can experience poor mental and physical health while caring for a loved one with ALS/MND. A caregivers reporting of poor health appears to be related to the number of hours spent caring, particularly with tracheotomy ventilation. C. Mockford of University of Oxford, Oxford, UK, examined caregivers’ experiences of looking after someone with ALS/MND and how they experienced the support offered to them by others. It was found that not all caregivers are proactive or confident enough to actively seek support or advice. Some also find difficulty in prioritizing their own needs. Diversity also exists in the form of support needed between caregivers. It appears that standard available emotional support would be welcome from the professionals in the field, and more practical information and involvement in service provision would help both the caregiver and patient. This may also improve caregivers’ self-reported health.
Nutritional Assessment and Support
E.J. Kasarskis, MD, University of Kentucky, Lexington KY, USA, reported that maintaining optimal nutrition for an ALS patient requires knowledge of food intake and energy expenditure at various stages of the illness. Weight loss and determination of BMI (body mass index) are indirect indices of malnutrition since loss of body mass may be due to muscle atrophy and not under nutrition. His study set out to prospectively develop empiric equations to accurately predict energy expenditure in ALS patients in various functional states as their disease progresses. Developing new predictive equations based on study data will help standardize nutritional care in clinical practice and in drug trials.
According to the practice parameters of the American Academy of Neurology, percutaneous endoscopy tubes (PEG) should be recommended for accelerated weight loss and symptomatic dysphasia soon after the onset of these difficulties. P. O’Conner, Forbes Norris ALS Research Center, San Francisco, USA, discussed compliance as it relates to specific elements in the practice parameter for PEG placement. The objective of this study was to determine how specific clinical parameters affect compliance for PEG intervention. Compliance with recommendations for PEG appears to be dependent on the degree of dysphasia. If patients feel their swallowing is intact, they will be less willing to undergo PEG placement, regardless of weight loss or respiratory difficulty. It is not clear if non-compliance is related to dysphasia or under reporting of severity.
Palliative and End of Life Care
S.J. King, School of Nursing, Deakin University, Victoria, Australia, explored what happened to people with ALS/MND before their diagnosis. Being told that one has a life threatening disease is devastating, but for some, it can be something of a relief after years of uncertainty and traumatic experiences that lead to diagnosis. The purpose of this study was to describe the extended and sometimes demoralizing process of diagnosis for people with ALS/MND. Distress in participants in this study seemed to have more in common with the stress linked to post-traumatic stress disorder (PTSD). They continued to relive the diagnosis experience in their dreams and daily lives many months after diagnosis, which had a negative impact on their well being. It seemed that by re-telling their stories, participants had the opportunity to debrief, therefore preventing the long-term reliving of the diagnostic process.
The role of palliative care in ALS/MND is changing. Due to increasing pressures on services, there is a tendency for them to be involved later in the disease progression. DJ. Oliver, Wisdom Hospice, Rochester, UK, discussed the development of a collaborative approach in the care of people with ALS/MND. At times increased collaboration may be necessary such as when decisions are made, and decreased collaboration may be needed when the patient is relatively more stable. Regardless of the interventions made, the patient and family must still be made aware of the potential for deterioration and the need to anticipate and prepare for end of life.
International Perspectives on Care and Quality of Life
M.R. O’Brien, Edge Hill, Ormskirk, Lancashire, UK, discussed what could be learned about living with ALS/MND from personal illness narratives posted on the Internet. The narratives posted on the Internet, as opposed to those appearing in other media, are more poignant and intense, are more likely to be written as they occur and rarely undergo editing. This paper explored what PALS write about their illness, why they write about it and what the benefits of writing are. It was found that illness narratives enable individuals to document how illness had impacted their lives and the lives of their loved ones. Key themes included condition updates, tips on how to manage particular symptoms/disabilities, and a focus on what can still be done versus what has been lost was common.
E.P. Simpson, Methodist Neurological Institute, Houston, TX, USA, discussed house calls and multidisciplinary outreach for homebound PALS. Advanced stages of disease often render PALS homebound and therefore unable to access medical therapy, participate in clinical trials and receive the support of a multidisciplinary medical team. A team was organized (comprised of a neurologist, physical therapist, social worker and patient services coordinator) to visit patients in their home every three to six months , and each visit had a duration of one to two hours. This experience suggests that this approach to house calls is a successful mechanism to maintain a continuum of care when patients are no longer able to leave their homes for medical care. Future benefits of such a model include reduced hospitalizations, increased completion rates for required study visits, increased compliance with recommended interventions, and improved quality of life for both PALS and caregivers.
Respiratory Management
J.A. Andrews, Eleanor and Lou Gehrig MDA/ALS Research Center, the Neurological Institute, Columbia University, New York, USA, described the frequency, demographics, clinical features, and survival patterns of patients with respiratory onset ALS and respiratory symptoms at diagnosis. This paper reviewed 502 consecutive charts of patients evaluated at their outpatient ALS Center with Institutional Review Board (IRB) approval. They found that respiratory onset ALS is rare and is more common in males. Respiratory onset and respiratory symptoms at diagnosis occurred in older individuals, and dyspnea on exertion (difficulty breathing that results in shortness of breath) was the most frequent initial symptom. Onset to diagnosis and survival was not different from non-respiratory onset ALS.
Results of diaphragm pacing in ALS were presented by R.P. Onders, Case Medical Center, Ohio, USA. Therapeutic electrical stimulation has been shown to help maintain the strength of peripheral muscles in ALS by maintaining physiological activity, contractile properties and calcium levels. The laparoscopic diaphragm pacing system (DPS) is a low-risk outpatient system that stimulates and conditions the diaphragm. This paper evaluated in a phase I trial that the DPS system can be safely implanted and used for conditioning the diaphragm in ALS patients.
