You wouldn’t judge a book by its cover, would you?
When thinking about the knotty matter of decision-making about resuscitation of sick children, we need to remember:
Every child’s life matters.
To always listen to the child, their parents and those who know the child best and include them in best-interests decision-making.
To always make decisions within an ethical framework and record the process of decision-making and who was involved.
To ensure pathways are in place to record and make visible best-interests’ decisions about levels of intervention.
So, what can help us with an ethical framework? Our friends at the General Medical Council have laid this out for us, in ‘Treatment and care towards the end of life: good practice in decision-making’.
This helpful document reminds us that:
- Equalities, capacity and human rights laws reinforce our ethical duty to treat all children, neonates and young people fairly.
- We must always listen to child, parents and others close to them. We must take account of their views. Where there are different views, our primary duty of care is to the child.
- Decisions must always be in a child’s best interests.
- We should weigh benefits, burdens and risks of treatment.
- We should consider all relevant factors in the decision-making process.
- Decisions must start from a presumption in favour of prolonging life.
When making decisions, even in a dire emergency, we are reminded to:
- Take account of up-to-date, authoritative clinical guidance.
- In case of uncertainty, seek further expert opinions early.
- Explain, justify and document all factors considered in decision-making.
- Not rely on personal values when making best interests’ decisions.
We must be careful not to make judgements based on poorly informed or unfounded assumptions about the impact of disability on the child or young person’s quality of life.
So, you wouldn’t judge a book by its cover, would you?
Disabled children and young people come in all shapes and sizes. We can’t all be experts in each child, which is why it is so important for us to listen to parents and those who know the child best.
Please do beware of falling down the rabbit hole of preconceived ideas about what a child’s quality of life is like – always ask parents and familiar carers what the child is like when well. They may have photos and videos they can show you of their child having fun and taking part in everyday activities.
Disabled children may appear, sound and behave differently when unwell. This may be because they cannot tell you where it hurts or how they feel. They may present with behaviours that others may see as challenging. They may not tick the expected boxes on the usual screening tools, for example, for sepsis. Their temperatures may be low when you might expect them to be high, they might have high or low heart rates and may not be able to mount the expected increased work of breathing, because their respiratory muscles may be too weak for them to do so. Their control centres may not work as expected.
Hasty decisions based on preconceived ideas can lead to poor outcomes, including premature death. None of us want that.
So, what’s all this about premature death in people with learning disabilities?
If you are ready for a shocking read, then take a look at Death by Indifference, written by Mencap in 2006, but just as relevant today.
If you prefer some e-learning on the subject, take time to work through the Disability Matters session: Equal Access to the Best Health Outcomes Matters. This was co-produced with disabled children, young people, parent carers and other experts.
Then there’s the Confidential Inquiry into Premature Deaths of People with Learning Disabilities report. This report showed that women and men with learning disabilities die 29 and 23 years, respectively, sooner than women and men without learning disabilities.
So what about children? The series of reports on Why Children Die show that more than half of all children who die in England have a pre-existing, life-limiting condition. So, we all need to pay great attention and ensure we achieve the very best outcomes for these children and all children.
The team at Bristol University have built on the work they did for the CIPOLD report and now lead on the Learning Disabilities Mortality Review (LeDeR) programme. This links to the Child Death Review programme and considers the circumstances of every death of anyone with a learning disability at any age. The latest annual report can be found here.
Between 01/07/2016 and 31/12/2019, 516 children aged 4-17 years were notified to the LeDeR programme. Of these children and young people who died, 43% were from Black and Minority Ethnic groups. 46% had profound and multiple disabilities.
Whilst 7% of the deaths reported to the LeDeR programme were of children and young people aged 4-17 years, the death rate overall in the 5-19 years age group in England was 0.3%.
The LeDeR programme 2019 report highlights good and problematic practices that the multidisciplinary team, including people with learning disabilities, identified.
Good practices included:
- Good care coordination across agencies and specialities
- Excellent end-of-life care
- Person-centred care, adjusted as the child or young person’s needs changed
Problematic areas of practice to reflect and learn from included:
- Delays in responding to signs of illness or investigating illness.
- This is known as ‘diagnostic overshadowing’, where clinicians may see the disabilities and think all presenting signs and symptoms can be explained by those, rather than undertaking a careful and structured clinical assessment to identify the underlying cause, such as pain from appendicitis, constipation, sepsis etc.
- Poor quality multidisciplinary team working.
- The needs of disabled children and young people are usually multifaceted and require a range of expertise to adequately assess and address them all.
- Poor advanced care planning.
- Clinicians can be reluctant to have those difficult conversations with families about the risk of both sudden and unexpected death and also the risk of deterioration and death. We all need to do better on this.
- Problems with the direct provision of care.
Recommendations from the LeDeR report included:
- Identification of a key worker to coordinate care and communication for disabled children and young people.
- Timely advanced care planning embedded in care pathways and clinical practice, responsive to changing needs.
- Better discharge planning and better community support.
- Consistent support and communication throughout each child’s life.
So where is the evidence about what parents think about end-of-life decision making? Dr Sarah Mitchell is a GP who is really interested in this and has written a useful paper in BMJ Open on the subject.
Sarah and her team interviewed parents and reported that:
- Parents have significant knowledge and experiences that influence decision-making process
- Trusted relationships with healthcare professionals are key to supporting parents making end of life decisions
- Verbal and non-verbal communication with healthcare professionals impacts on the family experience.
- Engaging with end of life care decision-making can be emotionally overwhelming, but becomes possible if parents reach a
‘place of acceptance’
- Families perceive benefits to receiving end of life care for their child in a PICU
With regard to the last statement, preferred place of death is, in my experience, different for each family. Some choose home, others need to know that no stone has been left unturned right to the end, so choose intensive care. Whilst this may not always sit comfortably with paediatric intensive care teams, for some families it is what is needed to bring them peace in their journeys of grief. I have been witness to what I would consider to be ‘good deaths’ in a wide range of settings, including homes, hospices, children’s wards and intensive care units over many years. What matters most and what families remember, are how the child’s needs were identified and addressed at every step and how they are families were kept in the loop about what was happening, including being fully involved in all decision-making.
It’s all well and good listening to my views as an experienced disability paediatrician on the subject, but much better to hear directly from a mother who has been on the advance care planning journey. Here are Kay’s words, describing her and her family’s journey with her son Matthew. If you prefer to hear her speaking directly, please check out the Disability Matters e-learning session Advance Care Planning Matters.
“Matthew had quite severe learning and physical disabilities. He was a very complex child and we used the Emergency Health Care Plan to help plan for the future and to enable us to communicate fully with other health professionals in the healthcare setting.
Matthew didn’t have the capacity to actually make decisions for himself although he was a very wilful little boy who had very clear likes and dislikes, so he could make decisions for himself that were relevant to his day to day needs like what he wanted to eat, where he wanted to go. He had no formal communication, but as parents and the people who were involved with him we learned to read what he was trying to tell us, and as I say, he was very clear on what he liked and disliked doing but wouldn’t have been able to make the kind of big decisions that as parents we were responsible for making for him.
We did have a very large team involved in Matthew’s care and we discussed the Plan with his consultant paediatrician but she also took into account the views of people like the occupational therapists, physiotherapists, the surgeons who were dealing with him as well as the parents, myself and Matthew’s dad. We were all involved in the discussions around the development of the Plan and what was in his best interests.
Matthew’s Health Care Plan went everywhere with him and it lived in his communication bag on the back of his wheelchair. So it went with him to school, to his respite and to his dad’s house when he was there and also it was with him whenever he needed to go into hospital.
The Emergency Health Care Plan actually protected Matthew’s rights should he ever become seriously unwell. It clearly stated that he needed full resuscitation and any treatment that was available to him and clinicians then could use the plan to make judgments on what treatment would be necessary but he was able then to access a full range of treatment that would be available to any other child in a similar circumstance.
Matthew was very well for the early part of his life but when he turned 11 he had an accident, a quite serious accident and we were able to use the Emergency Healthcare Plan to access full range of medical interventions for him, as a result of that he did end up in intensive care for the first time. After that we did go through a period when he was 12 he had a twisted bowel, so he was literally in and out of intensive care and needed to be resuscitated on quite a few occasions, unfortunately, but his plan enabled us to access all of this medical intervention for him and saved his life in that instance.
Over a period of about a year, Matthew became more and more unwell. He needed regular trips to Intensive Care and regular trips to the hospital. The Plan actually went with him to hospital in Newcastle. It gave us the assurance that during these periods of Matthew being very, very poorly that the doctors in the hospital that weren’t used to dealing with Matthew, the Plan gave them all the information so we didn’t have to go through it every time that he was admitted. They trusted what we were saying because it was backed up by the Plan”.
Matthew’s paediatrician says:
“Matthew developed a lot of new symptoms that were unexplained; his seizures were becoming less controlled and we needed discussions around how we were going to treat these new symptoms as and when they appeared and what needed to be done for them.
So at this point in time, having taken account of the views of both of Matthew’s parents and the whole of the multidisciplinary team, we made a decision in his best interests that, at that time, further intrusive procedures or further intensive care was not going to be helpful or appropriate for him and might cause him further distress. So, together, we changed the wording on Matthew’s Advanced Care Plan at that time to reflect the possibility of him being allowed a natural death when his time came, recognising that we would always be there to manage his symptoms and always be there to support his family”.
“Matthew had had a chest infection and was having great difficulty breathing and his dad brought him up to hospital. We called all the family, it was obvious that Matthew was dying at this point. We had all the family called from all corners of the UK to come and say goodbye to him. We sat for hours and hours with him in the hospital ward and he was almost pronounced dead when all of a sudden, he decided it wasn’t quite his time and he took a great big breath and started breathing normally and all his colour came back. His dad described it as, like, “re-booting” his system as he wasn’t on any treatment. He was having no medication or anything and he just decided “No, it’s not my time. I’m going to come back” and we had him for another five weeks after that.
Then one day, approximately five weeks after the “re-booting” incident, Matthew wasn’t very well at all. He was due to see his paediatrician in clinic that day, but I phoned and said that “I don’t think he’s up to actually travelling to hospital,” so the paediatrician agreed to visit at home, so we waited. He’d had a massive seizure. I’d had to give him medication to bring him back from the seizure. When his paediatrician arrived, it became obvious that he was deteriorating very, very quickly and we decided that we were just going to keep him at home and see what the outcome would be, whether he would “re-boot” again or how it would play out this time”.
Matthew’s paediatrician continues:
“So Matthew was really very frail on clinical assessment at this point, so I needed to put in place the right procedures to make sure that his needs were met and the family’s needs were met. He was very peaceful and he was essentially drifting off to sleep. He didn’t have any difficult symptoms at that point that needed any changes in medication or changes in his Plan. What we did at that point is I made arrangements for if Matthew was to slip away in the night, a colleague to be able to come out and support the family and to confirm his death at that point. I also contacted the Coroner, because our Coroner liked to know in advance about any child’s death and if there is a death that is likely to be expected as defined under the Child Death Review Procedures, then our Coroner liked to know in advance. So, we made all of those arrangements and let the family have their special private time together”.
“We spent quite a few hours with Matthew – we called close family this time around. We didn’t get everybody coming from the far end of the country to be with Matthew. So, the time when Matthew did die, it was very, very peaceful. We had some quality time with him. We had a wonderful five weeks planning memories, planting memories for the other children and we spent those few hours reflecting on that and talking and supporting each other through the inevitable outcome of Matthew dying in the early hours of the morning.
We found that, when dealing with the professionals around Matthew’s death, that being able to change the wording of the Emergency Health Care Plan it kind of validated for us that what we wanted … we wanted Matthew to be peaceful and at home when he died, surrounded by his toys and his family. We felt that the Plan, when we read the wording of it, was quite a shock to see that he should be allowed to ‘die with dignity’ but it validated what we were feeling, that it gave us permission to ask that he could die at home where we wanted him to be. It made us feel that we weren’t asking for anything that was out of the ordinary or not possible and felt that we were actually more in control of the situation when the time actually came for Matthew to pass away”.
Achieving a supporting a child or young person through a good death is an important part of our job, when death is inevitable. We need to ensure we always steer the best possible course through the tricky waters of decision-making, protecting the rights of all children and young people to the best possible outcomes.
So, back to the key messages
- Every child’s life matters.
- Always listen to the child, their parents and those who know the child best and include them in best-interests decision-making.
- Always make decisions within an ethical framework and record the process of decision-making and who was involved.
- Ensure that pathways are in place to record and make visible best-interests’ decisions about levels of intervention.
If you want to read more about advance care planning, look at:
Horridge KA. Advance Care Planning: practicalities, legalities, complexities and controversies. Arch Dis Child. 2015;100:380-385
If you want to see and hear examples of the conversations that underpin advance care planning, more free e-learning can be found here:
To listen to discussions between paediatricians and a parent about signs of sepsis in disabled children, tune in to Episode 4 of the RCPCH sepsis podcast series here.
Thank you for your time and all the best for your advance planning and decision-making.