Alistair Gascoigne

An integrated model of provision of palliative care to patients with cystic fibrosis

Palliative care of patients with cystic fibrosis (CF) is often undertaken by CF teams rather than palliative care teams because of the specialist nature of the disease and the potential role of lung transplantation. We developed an integrated model of provision of palliative care whereby most care is delivered by the CF team using palliative guidelines and pathways, with additional support available from the specialist palliative care team when needed. We report our experience of the terminal care of 40 patients with CF with regard to the circumstances of death, lung transplantation status, specific symptoms and provision of palliative treatments. The transition from disease modifying treatments to palliative care was particularly complex. Patients had a high level of symptoms requiring palliation and most died in hospital. Palliative care is a crucial component of a CF service and requires the specialist skills of both the CF and palliative care teams.

Sleep-related breathing disorders following discharge from intensive care

Objectives: To determine the incidence of sleep-related breathing disorders and nocturnal hypoxaemia in patients discharged from ICU following prolonged mechanical ventilation.¶Design: Prospective, consecutive patient observational study.¶Setting: The medical and surgical wards of a University Hospital.¶Patients and participants: Fifteen consecutive, adult patients discharged from the ICU who had received more than 48 h of mechanical ventilation were studied. Ten healthy volunteers acted as controls.¶Measurements and results: Overnight, multi-channel pneumographic studies were performed on all patients and controls. Chest and abdominal wall movement, air flow, oxygen saturation and snoring were continuously recorded. Data was analysed by both visual inspection of the traces and by computer-based algorithms. An apnoea/hypopnoea index was calculated for each patient and volunteer. Volunteers had an apnoea/hypopnoea index of less than 5 and had no episodes of nocturnal oxygen desaturation (SaO2 < 90 %). Despite oxygen therapy 13/15 patients had episodes of desaturation and 9/15 spent more than 2 h with an SaO2 < 90 %. Eleven patients had an abnormal apnoea/hypopnoea index (range 5–34 events/h). Four patients had predominantly obstructive events while 7 primarily had hypopnoeas.¶Conclusions: Significant overnight oxygen desaturation is common in patients discharged from ICU who have received prolonged mechanical ventilation. This group also has a significant incidence of sleep-related breathing disorders and this mechanism is likely to be important in the pathogenesis of the hypoxaemia.

Catastrophic circulatory collapse following re-expansion pulmonary oedema

Re-expansion pulmonary oedema is a recognised but rare complication following the rapid drainage of a large pleural effusion or pneumothorax [1,2], usually occurring on the side of re-inflation. The pathogenesis of the pulmonary oedema is poorly understood but is thought to be due to micro-vascular shearing resulting in neutrophil activation and adhesion to the vascular endothelium resulting in increased micro-vascular permeability [3-7]. Few reports appear in the literature of invasive haemodynamic monitoring following this catastrophe. We describe a patient who sustained fatal pulmonary oedema arising in the contralateral lung, with pulmonary flow catheter data documenting the initial circulatory collapse following the aspiration of a massive pulmonary effusion.

A service evaluation of an integrated model of palliative care of cystic fibrosis.

Background: Patients with advanced cystic fibrosis have severe symptoms with a complex trajectory of exacerbations and recovery. They are often awaiting lung transplantation, and many die without receiving specialist palliative care. Aim: We introduced an integrated model whereby palliative specialists joined the cystic fibrosis team to provide palliative care in parallel with standard care. Design: A service evaluation of this model of care was undertaken in a prospective case series documenting symptoms and outcomes, the views of the cystic fibrosis team and the experience of the palliative specialists. Setting/participants: Over 3 years, 28 (10%) of 282 patients attending the cystic fibrosis centre had specialist palliative care. Results: They had advanced lung disease (mean forced expiratory volume in 1 s (FEV1) = 0.86 L (25% predicted)), and 17 died: 6 were on a transplant waiting list at death; 10 were unsuitable and 1 died post transplantation. All who died over these 3 years had specialist palliative care. Four patients had successful transplants. Assessment showed a high prevalence of breathlessness, cough, pain, vomiting and fatigue, with a significant impact on daily life. The cystic fibrosis team rated this model of care highly, felt that palliative care should be members of the team, and thought that patients had found it helpful. The palliative specialists gained knowledge of cystic fibrosis, found it beneficial to meet patients earlier in the disease, and identified unmet needs in managing bereavement and the effects of deaths on other patients with cystic fibrosis. Conclusion: This model has been successful in overcoming the difficulties in access to specialist palliative care for patients with cystic fibrosis.

Palliative Care in the Intensive Care Unit

The intensive care unit (ICU) is a setting in which critically ill patients receive high-intensity treatments such as mechanical ventilation to sustain life. These patients are at high risk of dying such that ICU clinicians manage dying patients and their families on a daily basis. ICU care is associated with a high level of symptoms and palliation of suffering is important throughout the course of treatment. Treatment is inherently intrusive and some patients with advanced disease may not wish to undergo ICU care, particularly if their deterioration indicates progression of their disease rather than a reversible complication. Failed ICU care may impose additional suffering and detract from the end of life experience. It is important to recognize when treatment is merely prolonging the dying phase. The focus then switches to making the dying process comfortable and dignified. Intrusive treatments are withdrawn and opioids and benzodiazepines are used to relieve distress. Because of the severity of the illness, the dying phase is often short, over a few hours.

P231 A prospective cohort study of integrated palliative care of cystic fibrosis (CF)

There are 140 deaths in the UK each year from CF, often on a transplant waiting list and often without specialist palliative care. A palliative physician and nurse joined our team in 2011, providing palliative care in parallel with standard CF care. We undertook a prospective study documenting symptoms and outcomes, the views of the CF team and the experience of the palliative specialists. Over 3 years, 28 (10%) of 282 patients at our Centre had palliative input; their mean age was 31 (range 18–47) years and mean FEV1 was 0.86 L (24%); 17 (61%) died - 6 were on a transplant waiting list, 10 were unsuitable, and one died post transplantation; 4 have had transplantation and no longer need palliative input, 7 are in on-going care; 15 (88%) of deaths were on the CF ward and 2 at home. All patients who died had had palliative care. The main symptoms were breathlessness, cough, pain, vomiting, fatigue and low mood. The mean palliative assessment score was high at 2.9, indicating that life was dominated by symptoms. Palliative interventions included opioid, benzodiazepine, anti-emetic and anti-depressant medications and non-pharmacological interventions included relaxation techniques, massage, acupuncture and cognitive therapy. A survey was completed by 16 members of the CF team: all felt that palliative specialists should be part of the team and rated the model of care highly with a mean score of 4.1 (scale 1–5); 11 thought that patients had found input very helpful and 5 helpful; one patient declined a palliative consultation. The palliative specialists had increased their knowledge of CF, found it useful to meet patients earlier and had no difficulty in providing palliation in parallel with standard CF care. Their workload was high and they identified additional needs of bereavement counselling and managing the effects of deaths on other CF patients. This integrated model was successful in overcoming barriers to specialist palliative care. Palliative specialists have improved their knowledge of CF and the CF team have learnt palliative skills.