Rose is nine years old and has the most severe form of cerebral palsy.

She requires a wheelchair for mobility, a gastrostomy for feeding and is dependent on others for all her care. She is not able to communicate verbally but can express joy and pain. Rose suffers severe seizures from time to time despite anticonvulsant medication. She attends a special development school. She has an evolving scoliosis.

Rose’s paediatrician has the following discussion with the parents.

• What does Rose enjoy?
• What does she find difficult?
• As they think of the future:
  • What is most important?
  • What are their hopes?
  • What are their fears?
  • What are their goals?

She records this discussion in the document provided.

Rose has required a couple of admissions recently for pneumonia. She was quite unwell during the last admission and needed non-invasive ventilation. She found this distressing and it took her a few weeks to recover. She now requires suction at home to manage oral secretions.

Rose’s paediatrician reflects on recent events with the family. She tells them she is worried that Rose is becoming increasingly vulnerable to respiratory tract infections and that children at this stage of their illness are at risk of becoming seriously unwell. She says that she noticed how difficult the last admission was and tells them it is important they think together about how to best care for Rose through a similar illness in the future.

She says,

‘I know these are hard things to think about but if Rose was to become very unwell again …

• What would be most important to you and Rose?
• Have you had any thoughts about where you would want to be (home, hospital, hospice) if it looked like time were short?
• Is there anything in particular you wish to avoid?’

Rose’s parents say that they are aware of her deterioration and when it is clear she is dying, they don’t want to ‘prolong the inevitable’. They hope to care for her at home at the end of her life. However, they feel that overall, her quality of life is currently good and, for now, they want to provide her with a reasonable opportunity to recover from this acute illness.

There is a discussion about levels of ventilatory support and the parents say they don’t want Rose to be intubated but are unsure about BiPAP (a form of non-invasive ventilation) and will need guidance about that at the time.

This discussion is documented and the paediatrician explains that she will also complete a ‘Goals of patient care form’.

She tells them this is simply to let colleagues know where she and the family are up to in their thinking, and that it is not a legally binding document.

She also makes a referral to palliative care, explaining that this team can provide important supports and that they often help with care for patients where the future is uncertain.

The goal of care decided on is indicated as ‘B: Life-sustaining interventions but with some limitations’. Rose is not to be intubated and she should not receive cardiac compression or inotropes.

Rose struggles at home for two months with increasing suction requirements. She is tired and shows little interest in things that normally bring her pleasure. She has not been back to school. She presents to the emergency department with probable aspiration pneumonia and is in significant respiratory distress.

The emergency department physician talks to the family about Rose’s condition and tells them he understands from the documents he has read that they are eager to give Rose an opportunity to recover, but they don’t want to burden her too much with interventions that might cause her distress. They confirm this and tell him the last two months have been very difficult for Rose.

The emergency department physician, the admitting unit and the family agree they will trial BiPAP and intravenous antibiotics for 48 hours. If Rose finds BiPAP distressing or is not showing signs of recovery after this period, the parents want to discuss the possibility of taking her home.

Two days after admission it is clear Rose is deteriorating and that she is really struggling with BiPAP.

The parents say they don’t know what to do. Rose’s paediatrician reviews with the parents the conversations they have had over time: how Rose loved school but can no longer attend, how they wanted to avoid unnecessary suffering at the end of her life, and how they hoped to be at home.

She says that based on this, it is her opinion that BiPAP should be stopped and the focus should now be exclusively on comfort. The parents agree and the goals of care document is changed to reflect the new goal. Arrangements are made for Rose to be cared for at home.

Charlie is four months old and has spinal muscular atrophy type 1. He is already very weak and struggling to feed. It is thought that he only has a few months to live. The neuromuscular treating team talk to the parents and explain Charlie’s illness and prognosis and the different approaches to managing his condition.

At an outpatient appointment a couple of weeks after the diagnosis, the nurse coordinator asks the family if they have had a chance to reflect on their hopes and fears, and their overall goals for Charlie’s care. The parents say they hope he will be comfortable as his condition unfolds. They are fearful of seeing him gasping for breath as he deteriorates.

The nurse asks if it would be OK to discuss how deteriorating respiratory function and distress would be managed. At their request, she briefs the neurologist and asks him to join them.

There is a discussion about what is most important to the family, where they would want to care for Charlie and anything they especially want to avoid. The parents say that comfort is the most important thing and they want to avoid hospital as much as possible. They do not want Charlie to struggle for breath.

The neurologist explains that every effort will be made to ensure Charlie’s comfort and that a goals of care document will be completed so that any health professional involved in care will know what the goal is and what interventions are appropriate and what interventions are not. Together they decide on care as indicated by ‘C: Primarily symptom management with some non-burdensome interventions’, The paediatrician explains that he will communicate that cardiopulmonary resuscitation, ventilatory support and intravenous antibiotics are not consistent with Charlie’s goals of care.

Given Charlie is alert and hungry, naso-gastric tube feeds are currently appropriate, although the rate may need adjusting according to the volume Charlie can tolerate.

Oxygen may or may not be helpful. If trialled, the goal will not be to achieve a particular oxygen saturation but to see if it eases Charlie’s respiratory difficulty.

A referral is made to the palliative care team who visit at home later that week and work through step 4 with the family, just in case Charlie deteriorates very quickly.