Neuroprognostication for Patients with Amyotrophic Lateral Sclerosis: An Updated, Evidence-Based Review.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder that presents and progresses in various ways, making prognostication difficult. Several paradigms exist for providers to elucidate prognosis in a way that addresses not only the amount of time a patient has to live, but also a patient's quality of their life moving forward. Prognostication, with regard to both survivability and quality of life, is impacted by several features that include, but are not limited to, patient demographics, clinical features on presentation, and over time, access to therapy, and access to multidisciplinary clinics. An understanding of the impact that these features have on the life of a patient with ALS can help providers to develop a better and more personalized approach for patients related to their clinical prognosis after a diagnosis is made. The ultimate goal of prognostication is to empower patients with ALS to take control and make decisions with their care teams to ensure that their goals are addressed and met.

Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease.

BACKGROUND: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is the most common neurodegenerative disorder of the motor system in adults. Pain in ALS is a frequent symptom especially in the later stages of disease and can have a pronounced influence on quality of life and suffering. Treatment of pain therefore should be recognised as an important aspect of palliative care in ALS. This is an update of a review first published in 2008. OBJECTIVES: To systematically review the evidence for the efficacy of drug therapy in relieving pain in ALS. We also aimed to evaluate possible adverse effects associated with the different drugs and their influence on survival and quality of life. SEARCH METHODS: On 2 July 2012, we searched the following databases: the Cochrane Neuromuscular Disease Group Specialized Register (2 July 2012), CENTRAL (2012, Issue 6 in The Cochrane Library), MEDLINE (January 1966 to June 2012), EMBASE (January 1980 to June 2012), CINAHL (January 1982 to June 2012), AMED (January 1985 to June 2012) and LILACS (January 1982 to June 2012). We checked the bibliographies of trials identified and contacted other disease experts to identify further published and unpublished trials. SELECTION CRITERIA: We searched for randomised or quasi-randomised controlled trials on drug therapy for pain in amyotrophic lateral sclerosis. DATA COLLECTION AND ANALYSIS: We collected data using a specially designed form and analysed them using the Cochrane Review Manager software. MAIN RESULTS: We found no randomised or quasi-randomised controlled trials on drug therapy for pain in ALS or MND. AUTHORS' CONCLUSIONS: There is no evidence from randomised controlled trials about the management of pain in ALS. Further research on this important aspect of palliative care in ALS is needed. Randomised controlled trials should be initiated to determine the effectiveness of different analgesics for treatment of pain in ALS.

Behavioral activation and therapeutic exposure for bereavement in older adults.

The development and clinical trial of a 5-session behavioral intervention for complicated bereavement (CB) is presented. We conceptualized CB in terms of Major Depression (MDD) and Post-traumatic Stress Disorder (PTSD) and consequently applied treatment components of Behavioral Activation and Therapeutic Exposure (BA-TE). In order to assure standardization of treatment, control costs, and engage patients, a multi-media, multi-context format was adopted to address avoidance and withdrawal behaviors conceptualized as central pathogenic responses in CB. Participants (N = 26) were assessed before and after BA-TE treatment via structured clinical interview and standardized questionnaires in terms of PTSD, MDD, CB, and health concerns. The number of days since the death of the loved one was widely variable and served as a covariate for all outcome analyses. ANCOVAS revealed statistically significant improvement, irrespective of how many days since death had elapsed prior to initiation of intervention, on structured interviews and self-report measures for most outcome variables.

Treatment for familial amyotrophic lateral sclerosis/motor neuron disease.

BACKGROUND: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is a rare neurodegenerative disease. Approximately 5% to 7% of ALS/MND patients report a family history of a similarly affected relative. Superoxide dismutase-1 gene mutations are the cause in about 20% of familial cases. In those with non-familial (sporadic) ALS/MND the cause is unknown. Also unknown is whether patients with familial and sporadic ALS/MND respond differently to treatment. OBJECTIVES: To systematically review the literature and to answer the specific question: 'Is there a difference in the response to treatment between patients with sporadic and familial forms of ALS?' SEARCH STRATEGY: In May 2006 we searched the Cochrane Neuromuscular Disease Group Trials Register, MEDLINE (January 1966 to May 2006) and EMBASE (January 1980 to May 2006) for randomized controlled trials (RCTs). Two review authors read the titles and abstracts of all articles and reviewed the full text of all possibly relevant articles. We scanned references of all included trials to identify additional relevant articles. For all trials eligible for inclusion we contacted the authors to request the necessary raw data. SELECTION CRITERIA: Studies had to meet two criteria: (a) randomized controlled study design, and (b) inclusion of patients with both familial and sporadic ALS/MND. DATA COLLECTION AND ANALYSIS: We attempted to contact authors of all trials that met inclusion criteria. We obtained data regarding ALS/MND type (sporadic versus familial), treatment assignment (active versus placebo), survival and ALS Functional Rating Scale scores for four large RCTs that included 822 sporadic and 41 familial ALS patients. We could not obtain data from 25 potentially eligible studies (17 trial authors could not be contacted and eight were unwilling to provide data). MAIN RESULTS: There was no statistical evidence for a different response to treatment in patients with familial ALS/MND compared to those with sporadic ALS/MND. The pooled estimate of the hazard ratio for the interaction term (treatment x familial ALS) suggested a more beneficial response with respect to survival among patients with familial ALS/MND, but the result was not statistically significant. Estimates of the rate of decline on the ALS Functional Rating Scale also suggested a slightly better response to treatment among those with familial ALS/MND, but the result was not statistically significant. AUTHORS' CONCLUSIONS: Future RCTs should document whether patients with familial ALS/MND are included and the presence or absence of a mutation in the superoxide dismutase-1 gene amongst those with familial ALS/MND.

Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease.

BACKGROUND: Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), is the most common neurodegenerative disorder of the motor system in adults. Pain in ALS is a frequent symptom especially in the later stages of disease and can have a pronounced influence on quality of life and suffering. Treatment of pain therefore should be recognised as an important aspect of palliative care in ALS. OBJECTIVES: To systematically review the evidence for the efficacy of drug therapy in relieving pain in ALS. We also aimed to evaluate possible adverse effects associated with the different drugs and their influence on survival and quality of life. SEARCH STRATEGY: The authors searched the following databases: the Cochrane Neuromuscular Disease Group Trials Register (October 2007), the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2007, Issue 1), MEDLINE (January 1966 to October 2007), EMBASE (January 1980 to October 2007), CINAHL (January 1982 to October 2007), AMED (January 1985 to October 2007) and LILACS (January 1982 to October 2007). We checked the bibliographies of trials identified and contacted other disease experts to identify further published and unpublished trials. SELECTION CRITERIA: We searched for randomised or quasi-randomised controlled trials on drug therapy for pain in amyotrophic lateral sclerosis. DATA COLLECTION AND ANALYSIS: Data were collected using a specially designed form and analysed using the Cochrane Review Manager software. MAIN RESULTS: No randomised or quasi-randomised controlled trials on drug therapy for pain in ALS or MND were found. AUTHORS' CONCLUSIONS: There is no evidence from randomised controlled trials about the management of pain in ALS. Further research on this important aspect of palliative care in ALS is needed. Randomised controlled trials should be initiated to determine the effectiveness of different analgesics for treatment of pain in ALS.

Lack of concordance between physician and patient: reports on end-of-life care discussions.

OBJECTIVE: To study the effectiveness of patient physician communications regarding health care choices at the end of life. We studied communications occurring between physicians and their patients who had either terminal cancer or congestive heart failure, with less than 6 months to live. METHODS: This pilot study used in-person interviews with 22 physicians and 71 of their (matched) patients. Subjects provided paired responses to questions regarding their conversations related to end-of-life care, including resources, attitudes, and preferences. We calculated the concordance of patient and physician reports about these discussions. We examined the physicians' and the patients' agreement on the patient's diagnosis, and on whether a variety of care options were discussed. We then measured whether physicians' were aware of their patients' preferences for pain management and for place of death. Finally, we measured physicians' knowledge of whether religious/spiritual concerns or financial concerns had affected their patients' decisions regarding end-of-life care. Both bivariate and multivariate models were used. RESULTS: As a whole, the concordance scores were poor; however, concordance varied across domains of issues discussed. Patients with less education had significantly lower concordance scores. DISCUSSION: We have identified domains in which the physicians and patients may be least effective in discussing end-of-life care options. Findings may help in designing interventions to improve communication, especially for patients with less education.

Care at the end of life: focus on communication and race.

OBJECTIVE: To profile communication and recommendations reported by adults with terminal illness and explore differences by patient and physician characteristics. METHOD: This pilot was a cross-sectional study sample of 90 patients (39 Caucasian, 51 African American) with advanced heart failure or cancer. Participants completed an in-person, race-matched interview. RESULTS: Participation was high (94%). DISCUSSION: For example, only 30% reported discussion of advance directives, and 22% reported their physician inquired about spiritual support. Participants with cancer were significantly more likely to be receiving pain and/or symptom management at home, aware of prognosis, and participating in hospice. African American participants who were under the care of African American physicians were less likely to report pain and/or symptom management than other racial matches.Discussion: Although additional research on factors related to communication is important, initiation of patient-centered counseling by all physicians with seriously ill patients is essential.

Physician perspectives on end-of-life care: factors of race, specialty, and geography.

OBJECTIVES: To describe physicians' end-of-life practices, perceptions regarding end-of-life care and characterize differences based upon physician specialty and demographic characteristics. To illuminate physicians' perceptions about differences among their African-American and Caucasian patients' preferences for end-of-life care. DESIGN AND METHODS: Twenty-four African-American and 16 Caucasian physicians (N=40) participated in an in-person interview including 23 primary care physicians, 7 cardiologists, and 10 oncologists. Twenty-four practices were in urban areas and 16 were in rural counties. RESULTS: Physicians perceived racial differences in preferences for end-of-life care between their Caucasian and African-American patients. Whereas oncologists and primary care physicians overwhelmingly reported having working relationships with hospice, only 57% of cardiologists reported having those contacts. African-American physicians were more likely than Caucasian physicians to perceive racial differences in their patients preferences for pain medication. SIGNIFICANCE OF RESULTS: Demographic factors such as race of physician and patient may impact the provider's perspective on end-of-life care including processes of care and communication with patients.

End-of-life care and African Americans: voices from the community.

BACKGROUND: In 1997, the Institute of Medicine called for reform, improved quality and expanded research in end-of-life care. Yet little empirical information about preferences of African Americans has been documented. A community-campus partnership was formed to guide a needs assessment related to end-of-life care in a Southern, urban, African American community. This paper presents focus group findings related to end-of-life and palliative care. METHODS: A qualitative design of multiple-meeting focus groups was used to explore experiences, preferences, needs, and feelings expressed by family members with at least one relative who had died in a hospital (group 1) or at home (group 2). Sessions were taped and transcribed; themes were identified using systematic analytic procedures. RESULTS: Thematic analysis revealed key concerns related to health care provider communications about end-of-life care and dying. Positive communications empowered and showed respect for patients and family members and recognized the importance of their spiritual beliefs; informed them about resources available to assist dying at home; and, for patients dying in the hospital, treated them with nurturing, compassion, and diligent monitoring of the patient's medical status and needs. Other themes related to end-of-life care issues include preparation, planning, and access. A table of quotes from participants is available from the corresponding author. CONCLUSIONS: Findings suggest important clinical implications for clinicians and other health professionals. These voices from the community remind us of the heterogeneity in needs and preferences and challenge us to listen and tailor communication to each patient and their families.