Clinical · Healthcare

Reflections from the North

Time at the APF and Symposium was like enjoying a sumptuous meal – course after course of rich food and wine – and it has taken a few weeks to digest it all! It is a wonderful opportunity to hear the wealth of work being undertaken both in research but also in the management of those currently living with MND. Often thought provoking presentations that trigger ideas of how it could impact on your own practice and that cascade of information and experience, coupled with the numerous opportunities for networking and forging new partnerships, make this event an inspiring few days. Here are a few of my thoughts on the APF. There were 18 presentations on the day covering a broad range of topics, with presentations on management models, cognitive and nutritional issues, technology and innovative research. Highlights included:

David Oliver presented a paper on the effects of withdrawal of NIV on the professionals involved. He stressed the need to discuss this widely, once poor respiratory function has been assessed, and NIV proposed, and he talked about a study of doctors in the Association for Palliative Medicine for GB and Ireland. A questionnaire asked about the scale of the challenge for them in terms of withdrawal of NIV, covering emotional, practical and ethical issues. Only 60% of respondents had been involved in withdrawal of NIV. Professionals were often unclear about the legality and use of Advance Care Planning. Some professionals felt that withdrawal of NIV was euthanasia or assisted suicide, despite ethical advice that this was not the case. In terms of communication, some patients did not understand that deterioration will continue with NIV, many had unrealistic expectations of the intervention and there was often limited discussion with patients, families and staff. The recommendation to the field was that the implications of NIV must be discussed at initiation, and then continue with ongoing discussion and advance care planning. MDTs should consider their views before being faced with a patient asking for withdrawal. Catherine Knights talked about model developed in SE England to support professionals and to improve quality of care. It developed from the SE Regional MND Forum, set up in 1999, and now offers a way of engaging professionals despite the current climate of cuts and statutory reorganisations. Flexible networking, information sharing and educational opportunities allow professionals to enhance their practice and continue to offer quality of care to people affected by MND.

Patsy Pynn described how her team in Sydney have now incorporated a Social Worker into the MND Team. This has significantly increased awareness of the specific needs of this small but unique client group, strengthening the role as an advocate and enabling continuity of SW care from referral until death, whether in the community, respite or residential or hospice care. Patsy mentioned communication as a particular area where professional continuity is particularly important, and gave quotes from one family member and one person with MND:

The loss of my mother’s ability to communicate was by far the most heartbreaking loss of the ALS experience. It is difficult enough to grieve, to express fears and pain and to share one’s heartfelt sentiments when one can communicate easily. How much more traumatic it is when communication is impaired.” Linda Centres “Beyond Hope and Despair” 2001

“I guess I had no idea how difficult life was going to be to have to write everything down or use lite writer. They seem to think handling a lite writer will solve everything but they don’t realise how many times you have to repeat yourself as staff changes and new staff want to know about your care.” Joan O’Hare MND client 2004

Interestingly there are still no other designated allied health professionals specifically assigned to MND patients in the team, creating additional stress on MND patients and family members because the professionals change as the patients enter the final stages of the disease.

Susan Woolley presented her work on the ALS Cognitive Behavioural validated measure (ALS-CBS) which is a 5 minute screening tool for use in the clinic setting. It can be administered by any clinic staff, and responses can be verbal or non-verbal. The caregiver completes a behavioural questionnaire. Research suggest that patients with cognitive deficits are less compliant with interventions and have significantly shorter survival so a tool to help triage patients, prior to neuropsychological testing, is important to identify patients most at risk. Domains covered in the screen included attention, concentration, eye-movements, mental control and verbal fluency tasks. The behavioural questionnaire looked at apathy, emotional responsiveness, disinhibition, frustration, irritability and confusion. In trials of the ALS-CBS the cognitive section differentiated ALS-FTD from other ALS patients with 100% accuracy. Cognitively normal ALS patients could be distinguished from those with any cognitive deficit with 71% specificity and 85% sensitivity. In the clinic necessary strategies for the team and the family and future management issues can be identified and for those unaffected, testing can then continue on average once a year. Identification of impairment can help the family to accept it and plan accordingly. An example given was where the patient still managed all the family finances but the identified impairment allowed other family members to gradually take on this role. The tool could also help distinguish other causes of apathy. Ref: Woolley et al, Amyotrophic Lateral Sclerosis. 2010 May 3;11(3):303-11.

Meenakshi Wadhwa discussed her work on nutritional intervention and use of BMI as an indicator of survival. Individualised nutritional management and education of the person and family, including a high calorie diet from disease onset, may improve ALS signs and symptoms and quality of life.

Samar Aoun presented work on a targeted education programme for improving understanding of end of life care for people with MND, and integrating this approach into MND care. The model consisted of 6 one day workshops held in Western Australia and Southern Australia where participants were measured pre and post delivery for their knowledge of palliative care for MND, then followed again 1 month and then 6 months later by questionnaires and interviews. Improvement in knowledge and attitudes were demonstrated and participants indicated that the gained knowledge positively influenced their clinical practice. Ref: McConigley et al, Palliat Med November 7, 2011

Robin Ray gave an interesting paper on the process of engaging patients and caregivers in discussions about Advance Care Decisions. A staged cyclic process was developed that provides a graduated approach to planning, that is grounded in the patient’s/family’s understanding of their health status and perceived quality of life. Initial conversations establish these and improve the emotional readiness by providing a lead in to advance care planning. Outcomes from these discussions guide the need for conversations with relevant others about care needs and from here the person or family members may choose to make decisions about care, informally through a Statement of Choices, or a legal Advance Directive. The circle is completed by regularly returning to Quality of Life and health condition discussions. The professional’s own readiness to discuss these issues is a factor as they can act as a ‘gatekeeper’. Comments from professionals, such as ‘it was very confronting, more confronting than doing my own will’ and ‘she (the patient) is not ready for the discussion yet’, indicated their own feelings rather than those of the patient and family. Encouragement was given to make such conversations early and non-threatening. It should not be seen as a paper, tick-box task and it is a vehicle for making sense of the future. Wishes can be recorded as short term goals, – not just how they want to die but how they want to live now. It enables the whole family to plan and relieves the decision making on caregivers. The whole MDT can be aware of the discussions by using a framework for tracking the ACP discussions.

Maryann McPhee started the section of the day on state of the art e-technology and equipment, by describing how a project was initiated called ‘Dare to Dream’ and how finance was found to equip rooms for people with MND at Calvary Health Care at the Bethlehem Hospital in Melbourne. This not only gave those in the hospital greater independence and control but the technology could be assessed for its use with people with MND, as well as facilitating realistic expectations of the e-solutions by utilising them before buying.

Justin Ware gave a presentation on the possibilities of using iPad and iPhones with the clinical outcome of maintaining access to communication, work, socialisation, education and leisure, but cautioned that too often iProducts are purchased by patients with MND without a functional goal in mind and without consideration of future access options.

John Paulin Hansen focussed on work on a flexible gaze tracking system for use in bed for late stage MND. The challenge had been to develop a low cost system using existing readily available equipment, with little clutter around the bed, and which does not occupy the physical space in front of the user. The software can be downloaded free-of-charge at and works with a web camera, Windows computer and video projector. The best tracking conditions were achieved when people were sitting rather than lying.

Gina Svolos presented the MND Aware programme, which is a web-based programme providing HSCPs with information about the management of MND. The objective is to increase awareness and to show effective ways of case management. The programme contains text, visual, audio and short video content. In NSW about 75 face to face education sessions are held a year but there are barriers for professionals in time to travel to events, and MND NSW were approached by the State Government to develop a resource for front line staff. Following consultations on stakeholder needs, learning objectives and a module structure were created. Evidence based content for each module was sourced and review was completed by people with MND, carers and HSCPs. Completing all the 26 modules would take about 6 hours. There are 9 introductory sessions, 10 on symptom management and 7 on well-being and support needs. Content is Australia specific in terms of health and social care models of delivery but the general content and case management recommendations are applicable worldwide. Ref: Nicola Yarah completed this section of the programme with a description of work being done by the Emory ALS Centre in Atlanta, Georgia on utilising technology to keep in touch with patients and families and manage symptoms. Telehealth is seen as an extension of telemedicine, using e-mail, smartphones and texting as well as a Facebook page and website. It enables patients to communicate easily, and from their own home. It often meant less time from symptom or problem onset to treatment or a solution. However it was not always an ideal alternative for face to face consultation. The website and Facebook site allowed for reliable information about ALS, research, clinical care, community resources and special events to be communicated to the person and their family. Providing options and finding ways to reduce stress and travel tothe ALS centre positively affect quality of life.

MaryJo Elmo presented additional data from the trials of Diaphragm Pacing, which indicates that it prolongs life, improves survival during PEG placement, which can be done simultaneously, and delays the need for tracheostomy ventilation. Carlayne Jackson reported on an evaluation of High Frequency Chest Wall Oscillation (HFCWO) (SmartVest) used in combination with Cough Assist. The evidence showed that airway clearance devices appear to be effective in reducing the incidence of serious respiratory tract infections in ALS patients, but that further studies needed to be undertaken to compare the efficacy of Cough Assist, either alone or in combination with the HFCWO. Jan Clarke described the results of research project to look at the incidence and recording of pain in people with MND. The study indicated that the recording of pain in case notes of those involved in the study, was very low (14.5%), despite the presence of pain being identified in just under half of the sample population. Recommendations to the field were that pain should always be enquired about at clinic appointments and clearly documented in both the medical notes and letters to other health professionals. Appropriate onward referral to specialist teams should be sought for those with pain that does not respond to regular analgesics an standard practice. Pain should be considered as an area for future research.

Rachael Marsden concluded the day with an inspiring talk about a four year collaboration with colleagues in Turkey, supported by the International Alliance of ALS/MND, which has resulted in the delivery by Rachael and Jan Clarke of a teaching programme for nurses. The objective was to produce sustainable outcomes that would continue without further input. Senior nurses who were trained on the last visit have felt sufficiently empowered to use their positions to establish their own Care Centre in Istanbul. The trained nurses have visited centres in Oxford and London and following talks with the Minister of Health for Turkey, a care centre programme is now to be established throughout Turkey.

My final thought would be to encourage any professional to consider submitting an abstract for the day. There is so much excellent practice that needs to be shared and everyone has something to add!