Discussion of respiratory management in caring for ALS patients is essential for their comfort, health and end-of-life decisions. This session covered a range of information from original diagnosis of respiratory failure, use of a diaphragm pacing system and experiences of termination or failure of ventilation from the perspectives of health professionals working in ALS centres in Germany, Ohio USA, and the UK.
To begin the session, Dr Rafiq from the University of Sheffield discussed the detection of respiratory failure in patients with ALS using the standard day time transcutaneous carbon dioxide monitoring and respiratory function tests (what doctors currently use) compared to an evaluation of symptom history. The most common symptoms at the time of suspected respiratory failure included shortness of breath on exertion, the patient noticing a change in their breathing, difficulty coughing and fatigue. All of the patients who had respiratory failure confirmed through overnight capnometry also confirmed symptoms of respiratory failure through the questionnaire. The most common symptom was shortness of breath, found in 74% of patients with confirmed respiratory failure, compared to only 15% of patients showing daytime hypercapnia. The respiratory function tests were not found to be a reliable indicator of respiratory failure, and Dr Rafiq argues that an improved questionnaire with a designated cut-off score could have a very strong predictive value for respiratory failure.
The next speaker was Pamela Cazzolli, an ALS nurse consultant who has worked with patients with ALS for 20 years and is currently based in Ohio, USA. Ms Cazolli described factors apart from oral secretions that may contribute to failed non-invasive ventilation use. This study looked at 157 patients in a community-based setting, with assessment through home-visits. Levels of oral secretions, tolerance of non-invasive ventilation and its duration were assessed until death or tracheostomy invasive ventilation was initiated. Of the 157 patients, 135 used non-invasive ventilation. At the point where non-invasive ventilation was no longer used, patients showed a range of severity of oral secretion indicating that this is not always the reason for discontinuation of non-invasive ventilation. Instead, 10% of cases were due to end-of-life decisions; 7% became intolerant due to morphine sulphate/O2; 4% reported a sudden inability to breath and 3% began tracheostomy invasive ventilation following CPAP intolerance. Ms Cazolli concluded that there are several other factors than excessive oral secretions that are associated with failed non-invasive ventilation, which carers need to be made aware of.
The following talk was given by Dr Katz looking into the survival of ALS patients who had undergone diaphragm pacing. In this retrospective study, three groups of patients were included: one set who had undergone diaphragm pacing, one group from a study by Lechtzin et al. and one group of control patients from the minocycline trial. Comparisons between these groups found that, after adjustment for different variables, there was roughly a six month increase of life expectancy following diaphragm pacing compared to the other two patient groups. However, there were differences between the groups, and so a larger, properly comparable study into diaphragm pacing may be needed.
Next to speak was Dr Meyer from the Charité Universitätsmedizin in Berlin, talking about his 12 years of experience working with ALS patients and discussing the palliative care following cessation of ventilation. Following elected withdrawal of ventilation, there are two palliative care options: the first is intensified symptom control with benzodiazepines and morphine sulphate as needed and the second is deep sedation. Over the 12 year period, 41 ALS patients underwent elected withdrawal from ventilation. 70% of these patients were using invasive ventilation methods while the rest were using non-invasive ventilation. Other factors looked at within this group of patients included age (an average of 60 years but a wide range from 35-84); length of time using ventilation (median of 15 months but again a wide range from 0.03-53 months) and average ALS-FRSr score, a rating scale for the severity of ALS in patients (those patients with invasive ventilation had a more severe ALS-FRSr score than those with non-invasive ventilation). There were also two patients in the group with complete locked-in syndrome and five with incomplete opthalmoplegia (paralysis of the eyes). Of this group of patients, roughly half underwent the intensified symptom control route and half were deeply sedated, depending on the patients’ likelihood of acute dyspnea. Patients who used invasive ventilation did not necessarily require deep sedation. The palliative care route could also be changed depending on the patient’s condition or their wishes. Dr Meyer concluded that ultimately the choices and procedures during ALS when close to death are not well studied, and although both intensified symptom control and deep sedation provide sufficient treatment following termination of ventilation, more work needs to be put in to improve patients’ quality of life.
The final talk from this session was given by Professor Faull from the LOROS Hospice in the UK. Her talk looked at the experiences of people surrounding patients following a patient’s request for termination of ventilation. In the UK, if a patient refuses medical treatment and still has the capacity for that decision, then that wish must be respected and complied with. In this study, 67 people were interviewed about their experience following termination of non-invasive or invasive ventilation, including relatives of the patient, doctors and other health care professionals from across the UK. Notably, these cases were extremely rare even for doctors involved, but extraordinarily memorable and emotional. This qualitative study looked at each person’s recall of the event. Doctors and other health workers spoke of feeling uneasy about following through with withdrawal of ventilation, and that their ethical and clinical decision-making was conflicted with their personal emotions, feeling that “I am causing the death”. Family members also spoke of the understandable conflict of emotions, relief on the one hand where their loved one has chosen this and has ended their journey and tragic loss on the other. They also talked more about the whole process leading to the decision to end treatment, and that this decision was often triggered by a loss of communication from the patient. The family members also spoke of sensing that the professionals were inexperienced, which highlights the need for better training so that both families and patients are reassured in this situation. Professor Faull concluded that doctors and other health professionals need more guidance in this area, with the suggestion of mentoring schemes from those who have experience to improve patient care.
Part of the excellent structure of the ALS/MND Symposium allowed research scientists to attend clinical sessions and vice versa, and as a research scientist working towards their PhD, I found this session important and fascinating to attend. The session followed respiratory management from when a patient is first diagnosed with respiratory failure on to termination of ventilation, highlighting areas for improvement at each stage so that better patient care can be achieved.