1. 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

2. Hospice

3. 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

4. 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

5. 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.

6. ‘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

8. 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

9. 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.

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

12. Evolution brings new challenges…

13. 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

14. 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): McCarthy et al (1997), Jnl of Royal Society of Medicine, March;90(3):

16. Difficulties ahead…

18. Uncertain and unpredictable
illness trajectories

19. “Prognostic paralysis”

20. 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

22. 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

23. 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

24. Symptoms and treatments

25. 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

26. Integration?

27. 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

29. 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

30. 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):

31. 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

32. 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

33. 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