Palliative Care in Cystic Fibrosis: an integrative model of care Jackie Robinson Nurse Practitioner, Auckland City Hospital Professional Teaching Fellow, School of Nursing, University of Auckland May 2016

Hospice

Hospice Care Developed in recognition of unmet need for patients dying in hospital Initially set up ‘in opposition’ of mainstream health care Providers of ‘terminal care’ for those with cancer Inpatient and community based

Hospice Palliative Care A philosophy and approach to care Grew out of a Western model of death and dying Based on principles of a ‘good death’ Death at home with family and friends An aware death A time for personal growth and resolution of persona Death free from pain and suffering Embraces a holistic model of care Physical, psychosocial, emotional and spiritual care

Definition of Palliative Care New WHO definition (2002): Palliative care is an approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain and other problems, physical, psychosocial and spiritual.

‘New’ Palliative Care Range of diagnostic groups (life limiting or life threatening illnesses) Mainstreaming and up streaming Less about prognosis and more about need Increasing use of technology (medicalisation) Spotlight on the ageing population

Who provides palliative care? “Palliative Care is provided for those affected by life limiting illness as an integral part of standard clinical practice by any health professional….” Palliative Care Subcommittee, NZ Cancer Treatment Working Party 2007

Specialist Palliative Care …is provided by those who have undergone specific training and/or accreditation in palliative care, working in the context of an expert interdisciplinary team of palliative care health professionals.

Patients Move Between Providers Depending on Their Need Provision Specialist Generalist & Generalist Needs Complex Intermediate Basic

Evolution brings new challenges…

Cancer Predictable disease trajectory Extensive support mechanisms in place for most Increasing use of palliative anti-cancer therapies Living longer with advanced disease Increasing complexity and acuity

Chronic Non-malignant Disease Increasing survival with chronic disease Poor quality of life Focus of care on acute exacerbations More likely to die in hospital Less likely to have discussed end of life issues with a health professional Death appears sudden Skilbeck et al (1998) Palliative Medicine,12(4): 245-254 McCarthy et al (1997), Jnl of Royal Society of Medicine, March;90(3):128-131

Difficulties ahead…

Uncertain and unpredictable illness trajectories

“Prognostic paralysis”

Cystic Fibrosis Prognosis has improved but can be unpredictable Paradoxically entwined with lung transplantation Difficulties addressing EOL issues Sudden change from intensive treatment to palliative care

Background Most palliative care is provided by the CF team using a palliative care pathway Support from specialist palliative care services Retrospective review of all CF deaths over 10 years Integrated care pathway and palliative care guidelines Structured approach in providing symptom management and psychological needs Provides a plan of care

Findings 212 patients (16-59 years) attended CF Centre; 40 died (mean age 28.6 years) Post transplant (n=5) Progressive disease (n=35) 4 died at home 31 died in hospital 1 died in ICU 16 received ‘long term planned palliative care’ 6 received full active therapy until death 13 had an abrupt change from active therapy to palliative care

Symptoms and treatments

End of Life Issues Keeping well for as long as possible Relieving symptoms Fears of choking to death ACP discussions 7 received a specialist palliative care consultation

Integration?

Challenges Lack of understanding of Symptom management and illness trajectory Disease progression Palliative care Unrealistic expectations (patient and clinician) Complex integration of multiple services Lack of communication between health care providers Reciprocity in skill and knowledge exchange

Uncertainty Discussing uncertainty leaves room for hope, increases trust in the clinician and allows time to make difficult decisions Failure to disclose uncertainty prevents patients and families from discussing their hopes and fears

Hope "a multidimensional dynamic life force characterized by a confident yet uncertain expectation of achieving a future good which, to the hoping person, is realistically possible and personally significant" Dufault K, Martocchio BC. Hope: its spheres and dimensions. Nurs Clin North Am 1985; 20(2): 379-391

Hope Hope is important in both living and dying Supporting hope does not become a promise Hope and uncertainty are not mutually exclusive Tendency is to focus on hope alone

A new way of integrating care… Introducing a palliative care approach has been historically based on the presence of certainty Hope for cure while planning for the ‘what if’s’ is never a wasted conversation Polarizing palliative care with active treatment is not useful Traditional model of palliative care is not effective for everyone

Take Away Points Palliative care is provided by everyone Palliative care and ‘active treatment’ can be provided at the same time Aligning ourselves with hope while preparing for the ‘what ifs’ is essential Integration provides opportunities to do it differently Recognise when referral to specialist palliative care might be helpful