End of life care in CF: patients, families and staff experiences and unmet needs.

UNLABELLED: Palliative care is not well understood in CF. Unmet needs of patients with CF, their families and staff were explored. METHOD: Focus groups and interviews with forty-two participants (12 patients, 10 family members and 20 staff) were conducted at a university teaching hospital. RESULTS: Thematic analysis identified six themes. Knowledge: Patients and families felt their knowledge of palliative care was limited. Psychological frame: Hope and a positive psychological frame was essential to coping, however, this was a hindrance to the acquisition of information. Denial as a coping strategy resulted in a lack of preparation for declining health. Treating team: High expectations were placed on the treating team. Psychosocial support was valued. Communication: Timing, honest and clear discussions were important. Engagement with palliative care service: Increased palliative care. Unmet needs: The emotional burden of caring for dying patients/families and balancing hope against death was a challenge. CONCLUSIONS: Opportunities exist to improve care.

Teaching family carers about home-based palliative care: final results from a group education program.

Without the considerable support provided by family carers, many patients receiving palliative care would be unable to remain at home. However, family carers typically lack the required information and skills to prepare them for such a role. Pilot work has demonstrated that group education programs for family carers can be readily developed; they are feasible, accessible, and useful. This project sought to build on our pilot research to further examine the effectiveness of a group education program by evaluating the outcomes with a larger number of participants. The program aimed to prepare primary family carers for the role of supporting a relative with advanced, noncurative cancer at home. The psycho-educational program consisted of three consecutive weekly sessions presented in a group format, conducted at six home-based palliative care services across metropolitan and regional Victoria, Australia. The following dependent variables were measured at three time points: carer competence, preparedness, rewards, and information needs. The three time points were: commencement of the program (Time 1), upon completion (Time 2), and two weeks later (Time 3). A total of 156 participants (including the pilot phase) completed Time 1 questionnaires and 96 completed all three time periods (62%). Between Time 1 and Time 2, the intervention had a statistically significant positive effect on preparedness, competence, rewards, and having informational needs met. Outcomes were maintained at Time 3. There was no difference in the effectiveness of the intervention for participants in regional areas compared to participants in metropolitan areas. This study demonstrated that a group education program to prepare family carers for the role of supporting a dying relative at home was effective. Implications for further research and practice are outlined.

End-of-life care in adults with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a life-limiting congenital disease, with most patients dying at a young age of progressive lung disease. Lung transplantation offers hope for many but may not occur. There is little to guide the provision of palliative care to this young population who maintain hope in the possibility of lung transplantation. METHODS: To inform the development of an appropriate model of palliative care, a medical record review of CF patients dying within a 5-year period without lung transplantation was undertaken. The aspects of care that were preventative, therapeutic, or palliative were quantified. RESULTS: Of the 20 records studied, all died in hospital. Only 15% of patients had a do-not-resuscitate order agreed to more than 1 week prior to death, increasing to 90% at the time of death (median of 2 days before death). Opioids were prescribed for 1 patient (5%) at 1 week prior to death, increasing to 85% of patients in the last 24 hours of life (median of 36 hours before death). During the last 24 hours of life, intravenous antibiotics continued in 85%, and assisted ventilation in 90% of subjects. CONCLUSION: We conclude that the circumstances surrounding the death of patients with CF holds challenges for their effective palliative care. CF patients continue life prolonging and preventative treatments until the last hours of life. There is an urgent need to examine palliative care approaches that may usefully coexist with maintaining transplantation options in the end-of-life care of this population.

Work disability in adults with cystic fibrosis and its relationship to quality of life.

UNLABELLED: With increasing numbers of cystic fibrosis (CF) patients surviving to adulthood, issues related to vocation inevitably arise and warrant specific attention. We examined the percentage of participants with CF currently working and explored risk factors for work disability among adults with CF. METHOD: We recruited 50 consecutive patients from an adult cystic fibrosis service. Demographic, employment history, illness severity indicators and CF-attributed work disability factors were evaluated. Demographic risk factors for work disability using the illness severity measures of FEV(1), S-K score, CRDQ, and recent hospitalisation as independent variables were determined. RESULTS: Factorial analysis of a disability index (DI) indicated no dependency on FEV(1) or S-K score, but dependency on quality of life indices (p<0.05), age (p<0.05) and hospital admission rate (p<0.05). Hours worked per week were dependent on quality of life (p<0.01) (mastery of disease domain), fewer hospital admissions (p<0.01) and age (p<0.05). Sixty-eight percent of the sample reported that CF resulted in significant impediments to employment. However, few had sought vocational guidance (6%). CONCLUSION: Determinants of workforce participation shows that hours worked and perceived disability are more dependent on mastery of disease, age, and time in hospital, than on clinical severity scores. Health professionals may assist productivity through career counselling or tailored programs.