Clinical · Healthcare

Final thoughts

A month after the symposium the buzz of the sessions is still with me.  So much exciting research into the many and varied aspects of the disease, and so many opportunities to network with colleagues across the world.  Here is a resume of the Symposium presentations that resonated most with me.

Holistic Care and Quality of Life  

Dr Frank Brennan opened the first session with a presentation of the role of Palliative care. He argued for early involvement and meticulous planning of interventions, with close liaison with the patient and family. He referenced a poem called White Water Rafting and Palliative Care written by Bruce Dawe in 2007 for his late wife Gloria, which describes how alone families may feel in coping without the support of professionals.

  • Addressing issues of sexuality in Terminal Disease –

The meaning of sexuality and intimacy for patients and partners with life-limiting disease. A workshop was held later in the conference.

Ref: Marsden, R., Botell, R. (2010) Discussing sexuality in an MND Clinic. Nursing Standard 25 (15-17) 40-46

A focus group of professionals were asked about the barriers and facilitators to good decision making. Professionals experienced in MND care were able to identify ways of enabling patients in decision making, and to break down barriers, which included funding and staff resources, crisis management and poor inter-team communication. Cognitive impairment could be a challenge to decision making.  Pro-active partnerships between the patient, carer and MDT provided an environment for optimal and timely decision making.

Translating evidence into practice

Dr J Rosenfeld opened the session with a thought provoking talk on how to bring together the desire for evidence based practice with often more efficacious and numerous non-evidence based interventions that clinicians commonly use in clinics based on the collective experience of treating MND. Finding the point at which evidence and experience meet is a challenge. Barriers to evidence may be the cost of trials; not enough patients to study every intervention; disease heterogeneity; and the ethics of withholding treatment.

Surveys of patients were completed to ascertain the satisfaction, feature use, comfort and function of the power wheelchairs issued.  Most felt their quality of life had improved after provision and the features that were essential were tilt, recline and power elevating foot platform, contoured backrest, pressure relieving and positioning cushion and headrest.

  • Systematic review of ALS service users’ perceptions of services and decision making in care  –

An examination of the different perspectives of providers and service users of palliative services.

Cognitive Change

Dr John Hodges gave the opening presentation, reviewing the evidence on MND and FTD.  The high rate of FTD features (such as changes in behaviour and social cognition, apathy, planning, organisation, motivation and language abilities) have a significant impact on caregiver burden.  The greatest predictor of caregiver stress is changes in behaviour rather than motor deficits.

  • MND and frontotemporal dementia: the role of language dysfunction –

Language impairment is prominent in both MND-FTD as well as non-demented patients. It may be an independent clinical feature of MND and affect all levels of language processing.

  • Brief Cognitive assessment in ALS and its correlation with detailed neuropsychological testing –

The objective of the study was to determine the prevalence of cognitive dysfunction in a cohort of patients in a MDT clinic and to compare the diagnostic accuracy of a brief cognitive assessment based  on the Philadelphia Brief Assessment of Cognition with the Frontal Behavioural Inventory. The conclusion was that the ALS-BCA (a simple 5 minute questionnaire) could be used in the clinic setting effectively to detect subtle cognitive impairments.  It was highly specific for dementia and moderately sensitive for mild and moderate impairment.

International Perspectives on Care Practice

Dr Orla Hardiman opened the session with a comprehensive discussion of the appropriate time to engage palliative care colleagues.  Models have included the clear division between neurological management and then Palliative care at the end of life, the gradual increase of palliative care, which works alongside neurology from the beginning but gradually becomes the greater partner, and a model where both neurology and palliative care co-exist in management with trigger points along the disease trajectory dictating the specialist needs.  There are however currently no standards or guidelines to support this clinical management, and their development would assist in the production of a framework for active palliative care engagement in ALS.

  • Opinions and behaviours of Japanese and American neurologists regarding Tracheostomy Invasive Ventilation (TIV) –

Neurologists from 6 centres in Japan and 5 in USA were surveyed to look at the differences in use of TIV. While no American neurologists said their role was to make treatment decision for their patients, 21% of Japanese neurologists do so. When asked what proportion of their own patients got TIV, 5% of American and 30% of Japanese said more than one-quarter of their patients did so. When asked whether they generally suggest and encourage use of TIV, 79% of Americans and 38% of Japanese said ‘never’ or ‘almost never’.

In terms of withdrawal, comparisons are difficult as in Japan, withdrawal of permanent ventilation is subject to prosecution.

Interestingly when asked whether, if they themselves got ALS, they would accept TIV only 7% of Americans and 14% of Japanese said ‘yes’ or ‘probably  yes’, compared to 76% of Americans and 71% of Japanese who said ‘no’ or ‘probably no’.

The question was left – who is making best use of TIV – underuse in USA or overuse in Japan?   

The aim of the study was to determine the ability of health individuals (caregivers and age matched controls) to anticipate a patient’s emotional well-being and his decisions on hastening death.  Patients reported a good well being and a low wish to hasten death. Caregivers and the matched controls rated the patient’s well being significantly lower. They also thought the patient would rate the wish to hasten death higher. Healthy individuals influencing decisions concerning the patient’s life like caregivers, physicians and politicians should take these findings into consideration.

Respiratory Management

Dr Chio presented an overview of NIV and in a population study over the last 10 years, on the use of NIV in Italy, there has been an increase in use from 15% to 22%.  Several factors influence the outcome of NIV.  These include severity of bulbar impairment, lower ALS-FRS-R score, poor nutritional status, respiratory function, as well as age, marital status and if male. Neurobehavioural dysfunction in ALS has a negative effect on use of NIV.

Compliance to NIV can be improved if patients are adequately trained, have MDT support, and if there is careful choice of appropriate interface. In addition aggressive management of secretions and maintenance of an effective cough, as well as training and support of patients, caregivers and families will influence successful use.

This study looked at the possibility of identifying predictors of those who would refuse NIV and if this correlated with caregiver variables. 17 patients accepted NIV and 11 declined. Levels of carer distress were high across both groups and caregiver health was negatively affected, particularly emotionally and psychologically, in both groups. Carers in the second group (no NIV) were rated as more neurotic, less resilient and commitment was low.  Caregivers should be seen as the ‘hidden patient’ and their needs should be included in clinical treatment packages, ie with regard to information and decisions. 

These findings also translate into the provision of a PEG.

Details of the final sessions, including the presentation on ‘C9orf72 – a major new causative factor in familial and sporadic ALS’ given by Dr Traynor, and Professor Talbot’s final summary can be found on the website at