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End of Life Care in Cystic Fibrosis

This site does not accept referrals or provide clinical advice in response to questions. If you are a New Zealand health professional seeking clinical advice, please use your local clinical pathway. If you are a New Zealand child patient, parent or caregiver seeking clinical advice, please contact your usual doctor. You can read the full site disclaimer here.


End of life care is a vast topic in itself, and it is neither practical nor desirable to review all aspects of this subject for this guideline. The Paediatric Palliative Care Clinical Network has produced a number of guidelines relating to end of life care, including:

"Palliative care for children is the total active care of the child's body, mind and spirit, and also involves giving support to the family. 
  • It begins when the illness is diagnosed and continues regardless of whether or not a child receives treatment directed at the disease. 
  • Health providers must evaluate and alleviate a child's physical, psychological and social distress. 
  • Effective palliative care requires a broad multidisciplinary approach that includes the family and makes use of available community resources; it can be successfully implemented even if resources are limited. 
  • It can be provided in tertiary care facilities, in community health centres and even in children's homes. (WHO 2002)2"

When Cystic Fibrosis is diagnosed in New Zealand, there is discussion about the condition being life-limiting, however palliative care is not specifically discussed at this time.

This guideline will highlight a few Cystic Fibrosis specific aspects of palliative care and death. Palliative care may be seen as a means to allow death at home amongst whanau/family. However the majority of children with Cystic Fibrosis who die, choose to do so in hospital. The comfort and wishes of the child are paramount when this stage is reached. It is also important that other whanau/family members do not feel sidelined by focusing exclusively on the child. Invariably, this situation will be unique to the whanau/family, and so there may be great uncertainty about what might happen - in part due to the whanau/family's spiritual beliefs, and also due to acquired misinformation from media or from others' experiences in different settings.

Recognising a palliative stage of Cystic Fibrosis care

There are no clearly defined clinical parameters for when this threshold is crossed. Initiation of palliative therapies often overlaps with continuation of routine Cystic Fibrosis therapies and active management of exacerbations.

A very general guide to defining a palliative stage is when a child with Cystic Fibrosis (CF) has:

Acute respiratory illness requiring intermittent positive-pressure ventilation (IPPV) is also associated with a high risk of death in the following 12-18 months, and may be considered a sign of having reached a palliative stage of life.

When a patient is on an active waiting list for organ transplant, there may be added tension for the primary clinical team to accommodate, between a desire to sustain wellbeing until a donor organ is found, and the onerousness (and possible futility) of continuing treatment which seems not to be effective. Discussion between team members, the organ transplant service, and possibly the regional PICU may help agree a consensus plan, which can avoid such decisions being made acutely, and (possibly) by others who are less informed of the overall clinical situation.

Acknowledging palliative stage with patient and with whanau/ family

Initial discussion of this should be unhurried, and take place in a setting where disturbance is unlikely. The SPIKES protocol3 may help colleagues to manage this conversation in a stepwise manner.

S Set up interview Mentally rehearse conversation. Privacy. Involve significant others. Sit down (no barriers), Connect (eye contact, touching, hand holding), manage time constraints.
P Patient perception  "before you tell, ask", assess patient and whanau/family understanding of situation ("what have you observed of X's progress recently?"),
I Invitation gauge patient/parent desire for more information, " shall I go over how we see your current situation?".
K Knowledge  ?start with "We are worried about you at the moment." Use appropriate vocabulary for patient/ parents.
E Emotions Observe response (sadness, silence, shock etc), Ask patient "what are you thinking at the moment?", identify reason for the emotion (the news just given). Let patient express feelings. Important to connect emotion with reason "I know this isn't what you want to hear. I wish the news was better."
S Summarise Recap on information. discuss next step: possibly refer to palliative care, timing of a further conversation with you. If family asking - ? alter vs continue current treatment, stop treatments, introduce new ones.

Table taken from Components of the SPIKES protocol for breaking bad news. Baile W et al. The Oncologist. 2000;5:302

Where services are available, it may be appropriate to introduce the child and whanau/family to the local Palliative Care team after this discussion.

End of Life planning

Following recognition that the child has reached a point where death is a distinct possibility, the next stage is to discuss end of life planning. This will include therapeutic responses to specific symptoms or clinical events. Discussions should include exploration of the child's and parents' expectations and wishes in regard to ICU intervention, and response to cardiac and respiratory arrests. Discussion at this stage may also need to include a frank acknowledgment of the child's likely mode of death.

Treatment plans should also discuss options for palliation. These are discussed in detail on the Palliative Care Clinical Network site. Additional respiratory specific options may include provision of home oxygen or NIV to aid dyspnoea. At this time consideration of stopping treatments may be appropriate, although for some this may seem like the Cystic Fibrosis team is withdrawing from the child and whanau/family. The whanau/family may wish to discuss these options further, including after considering logistic constraints to remaining in hospital, returning to their local hospital, or going home.

Death of a child

We recognise different services across New Zealand will have different models for shared provision of out of hours cover. In addition, the availability of different SMO's to attend if a child dies will vary throughout the year, for this reason there can be no prescriptive recommendations about who should attend if a Child with Cystic Fibrosis (CF) dies outside of normal working hours. In general, if a child dies in hospital we suggest the on call SMO should attend at the time. Members of the local CF team should attend as soon as they can.  If the death was expected, it may be helpful to set out in the clinical notes whether specific CF team members wish to be notified at the time of death, or not.

After the child has died, the whanau/family should be supported as per the hospital policy on bereavement.

After the death, there may be cultural or personal reasons for a whanau/family to need to complete the funereal process within a certain timeframe, or in a different geographical location. Wherever possible, the CF team should support these wishes. In the case of it not being possible to issue a death certificate, the coroner must be informed of any cultural needs to be met.

Post bereavement follow up

The whanau/family of the child who dies should be offered an appointment to see the Cystic Fibrosis team approximately 6-8 weeks after the death. However, the family must be made aware that they can defer this appointment to any time in the future, should they wish to

Cultural differences on the loss of a child

The patients we encounter will be from differing ethnic, cultural and religious/spiritual backgrounds. It is important that Cystic Fibrosis (CF) teams do not assume that a specific cultural practice applies to a particular whanau/family, based on their religion, ethnicity etc. During the conversations prior to the child's death, we should ensure we fully understand what other wishes the whanau/family might have to take their child to another location, or complete the funeral within a certain timeframe.

For information on particular religious and/or cultural group beliefs and practices the reader is advised to refer to alternative sources.


  1. /for-health-professionals/national-paediatric-palliative-care-clinical-guidelines/#All
  2. WHO (World Health organisation). 2002. WHO definition of Palliative Care.
  3. Baile W et al. The Oncologist. 2000;5:302

Page last reviewed: March 2019

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