Abstract
Introduction
This is the 12th in a series of cases provided and discussed by UK clinical ethics committees. All clinical ethics committees registered with the UK Clinical Ethics Network may submit a case, which is then discussed by another committee. To safeguard confidentiality, if a case is based on an actual clinical scenario, consent from all parties is obtained. To the same end, the committee referring a case is not identified (as this would provide a geographical indicator of identity), but we do name the committee discussing the case. A member of the editorial committee (or their representative) attends the discussion of the case and writes the summary to be published in Clinical Ethics, once the discussing committee and the journal editors have approved it. In this particular case, a member of the Great Ormond Street Hospital Clinical Ethics Committee wrote the summary.
Q1: Our adolescent patient has a progressive life-limiting condition and impaired communication skills: How should decisions about her care be made?
Great Ormond Street Children's Hospital NHS Trust Clinical Ethics Committee
Great Ormond Street Children's Hospital (GOSH) NHS Trust provides a wide range of tertiary services in paediatric subspecialities across a large geographical area. The GOSH Clinical Ethics Forum was established in 1996, becoming the GOSH Clinical Ethics Committee (CEC) in 2000. In 2005, the Trust funded the UK's first consultant post in paediatric clinical ethics. The GOSH CEC is a multidisciplinary committee which meets monthly to provide a forum for confidential discussion and analysis of matters arising from clinical practice at GOSH. Where appropriate and requested, the committee provides informed, reasoned and justifiable opinions on issues raised by individual cases, records of which are kept in the patient's notes.
The committee includes representatives from surgical, medical, nursing and other professions allied to medicine. It also has representation from lay members. A number of committee members have specific training in ethics and law.
The committee provides ethical input into Trust policies and procedures, e.g. resucitation and end-of-life care. Cases and issues discussed with clinical teams in the last year include withdrawal or withholding of life-sustaining treatments, the compassionate use of innovative or unlicensed treatments and the extent to which palliative surgery should be offered to children with non-survivable conditions.
Request from an intensive care team
Sabina is a 15-year-old girl who moved to the UK from Eastern Europe when she was eight. When she is well she lives at home with her parents, two older sisters and a younger brother. Her family is close-knit and her parents always seek to promote Sabina's best interests. However, her parents also have demanding careers and their large family places significant demands on their time.
Sabina has a condition characterized by accumulation of toxins in the brain. This is partly treatable in that the rapid build-up of these toxins can be prevented, but nevertheless slow deterioration is inevitable. Sabina's lifespan will be reduced and she is not expected to live beyond her late 20s. Sabina's condition also gives rise to increased muscle tone, uncontrolled movements and a tendency to self-harm.
The condition also mildly affects cognitive function. Sabina has a reduced reading age but is nevertheless capable of articulating her thoughts. It is possible that her understanding is at a higher level than may initially appear. She seems to understand the difference between life and death and has always readily agreed to active treatment and intervention. She is also keen to engage in typical teenage pursuits, such as playing games on her Nintendo Wii when she is at home.
Sabina developed severe spasms four years ago, when aged 11. This was a distressing time for her and her family, as the spasms caused her pain and distress and affected her breathing – which can become life-threatening if left unchecked. The spasms can be controlled by very high doses of a sedative drug; however, the required dose can render recipients virtually comatose and can often necessitate artificial ventilation. An alternative is the use of a pump that delivers a minute dose of an antispasmodic drug directly into the space around the spinal column. Such a pump was subsequently inserted, following discussion with Sabina and her family. This was initially very successful in controlling Sabina's spasms, rapidly improving her quality of life.
Unfortunately, the use of such a pump brings with it concerns over infection, as an infection in this area can have serious implications. Several problems have arisen with Sabina's pump over the past year. It has needed to be re-fitted several times due to problems with blockage and infection. The pump cannot be re-inserted until any infection has cleared. While the pump is out, Sabina has to be managed on the high-dose sedative she was previously taking.
After the most recent infection, Sabina was admitted and has been cared for in the paediatric intensive care unit (PICU). She has required ventilation to curb the respiratory side effects of the sedative drug, which has made communication difficult. She uses aids such as a chalk-board but this limits any higher-level discussion.
Usually a pump such as Sabina's should last for at least another 3–5 years. However, given that Sabina has experienced recurrent problems, the chances of her experiencing another complication are significant. Each of these complications is likely to lead to a stay in the PICU and on ventilation support.
In addition to the problems with drug delivery, the team and Sabina's family agree that her general condition has deteriorated slightly over the past year. It is however difficult to obtain Sabina's view on this. Before her most recent ventilation she would answer questions with monosyllabic replies and express basic preferences.
In the past Sabina actively agreed to have the pump fitted, although she refused other higher-risk surgical interventions. During her current stay in the PICU, Sabina is regularly getting upset at her ongoing treatment. If her care team tries to provide her with information this only appears to distress her more, likely because Sabina likes the pump but dislikes going to the PICU when it has to be removed. Therefore, it appears that she is refusing some types of treatment, namely treatment involving a stay in the PICU. This latest development has led her parents to request that anything more than basic information be withheld from her, and they have expressed the view that they would prefer that plans for her future care are only discussed with them from now on.
We are approaching the Clinical Ethics Committee with the following questions in mind:
What should be done if Sabina's pump fails again? Should further revisions be performed or is the suffering involved beyond that which Sabina should be expected to experience and palliative therapy considered instead? Palliative therapy would again require large doses of the sedative drug to suppress spasms but this would inevitably suppress Sabina's level of consciousness, suppress respiration and hasten her death. To what extent should Sabina be involved in decisions concerning her care at the present moment and in the future (bearing in mind her family's concern that discussing such information may in itself cause Sabina distress)?
Response from the Great Ormond Street Children's Hospital NHS Trust Clinical Ethics Committee
Introduction
Thank you for your referral, which the committee discussed at its meeting on 15 April 2010. This case raises a number of important matters; in particular, the extent to which the views of parents should be allowed to override those of a child (whatever the child's level of competence), the circumstances in which a young person's refusal of medical treatment should or should not be accepted and the point at which it may or may not be appropriate to begin discussions about end-of-life care.
However, before the committee began its detailed discussion on the questions you asked, it highlighted a number of issues which it felt had not been explained sufficiently clearly in the referral note but which it considered important in helping to understand Sabina's clinical history and likely prognosis.
For example, it is not clear whether Sabina remains in the PICU, sedated and ventilated, or whether her pump has been re-inserted and she is being looked after at home. The committee felt strongly that this information was critical to any attempt on its part to understand Sabina's current state of mind.
Secondly, the referral does not make a clear distinction between the infections which Sabina has been suffering and the likely clinical prognosis based on her underlying (but unnamed) clinical condition. Are recurrent infections typical or untypical of a patient suffering from Sabina's condition or does the fact that she is suffering recurrent infections mean that her prognosis is likely to be different from the expected clinical path, particularly with respect to her life expectancy?
Finally, the committee felt it needed more detailed information about Sabina's cognitive abilities and the extent to which her ability to express her opinions about her medical treatment may or may not be affected by changes in her clinical circumstances; in particular, whether she is in the PICU being sedated and ventilated or being cared for at home with the pump in place.
With these caveats in mind, the committee then turned to the first question you asked it to consider:
What should be done if Sabina's pump fails again?
This question raises a number of important issues about Sabina's current and projected clinical prognosis. Based on the information in your referral letter, Sabina is unlikely to live beyond her late 20s.
The committee felt it was important to make a clear distinction between the potential consequences of infection on the one hand and on the other, the impact which Sabina's underlying condition might have on her life-expectancy. If the infections are primarily due to the pump being in place and are unlikely to affect her life-expectancy, the committee felt that the key issue which clinicians need to address is the cause of the infections rather than initiating a discussion about whether Sabina is approaching the end of her life.
If Sabina's clinical prognosis is not affected by her recurrent infections, she may well have another 10–15 years to live. It is also possible that advances in medical technologies could, in future, reduce the risk of infection for patients like her, thus improving her quality of life. It is therefore important to know whether any such advances are in the pipeline and if so, when they are likely to become available.
The issue of infection is, however, important, particularly if, as appears to be the case, Sabina is suffering from infections more frequently than might be expected. As you point out, every infection has serious implications. The committee felt it was important to try and quantify the risks and potential consequences associated with infection in the context of Sabina's overall prognosis.
The key issue is then the contrast between Sabina's overall quality of life with the pump in comparison with when it has been removed and she is in the PICU sedated and ventilated.
The decision about what should happen if the pump fails again turns on the following issues: how soon Sabina is likely to deteriorate as a result of her underlying condition, how long it is reasonable to expect that she could survive with a replacement pump in place, to what extent the risks of infection can be reduced or controlled in future and the relative burdens and benefits of replacing the pump.
If there is a realistic chance of being able to reduce the chance of infection with the pump in future and of giving Sabina the chance of a further 10–15 years of good-quality life, the committee felt that fitting a replacement pump could be justified even if this involved spending time in the PICU sedated and ventilated in order to insert it. If the pump worked well it would, in future, avoid the circumstances that cause Sabina distress – namely sedation and ventilation. However, the committee felt that it needed more information on the burdens and benefits associated with re-inserting the pump before it was able to reach a considered view about what might be in Sabina's best interest.
Members considered that the question of suffering and distress had to be balanced by considerations of long-term benefit and overall quality of life. The committee felt strongly that this would depend on Sabina's underlying condition and whether active palliation could continue to offer her good-quality life. Some members thought that you were expressing an unnecessarily bleak view of palliative care, particularly as the options facing Sabina are, essentially, all palliative rather than curative, given that her condition is incurable. The insertion of a pump could therefore be considered as part of the palliative care package, assuming that palliative care does not begin at the end of life but is an integral part of the management of a child like Sabina, who has a life-limiting illness.
If, however, the pump fails and Sabina's underlying condition has deteriorated to the point at which there is little prospect of being able to reduce infection and Sabina is faced with the prospect of multiple stays in intensive care, sedated and ventilated, what are the remaining options available to her and to her clinical team?
If replacing the pump is no longer an option, clinicians would have to decide how to control Sabina's spasms which, unchecked, would result in her dying a distressing death which would be considered unacceptable not only for Sabina but for her family and for the clinicians involved. Controlling the spasms through sedation would lead to suppressed respiration which, without ventilation, would likely result in a relatively quick and pain-free death. In ethical terms this course of action would be in accordance with the doctrine of double effect in which the intention is to relieve pain and suffering but where it is accepted that increased levels of sedation are likely to lead to suppressed respiration and therefore may hasten an already inevitable death.
The committee felt that if Sabina's underlying condition meant that further good-quality life could not be achieved through palliative care, then it would be appropriate to discuss with Sabina's parents and with Sabina herself, the possibility of allowing her to die. The committee felt that in cases such as Sabina's where cure is not an option, there is benefit to be gained from having an agreed plan in place as early as possible so that painful and difficult discussions are not left until a medical crisis occurs.
Finally, we discussed the question of whether children under 16 should be allowed to register some form of advance directive. Although this is not legally possible at present, it was felt that if the views of a patient such as Sabina were clearly documented, clinicians would be obliged to take them into account.
Q2: To what extent should Sabina be involved in decisions concerning her care at the present moment and in the future?
There appears to be a view among the intensive care team that this latest episode is likely to determine the course of Sabina's future medical care. If this is correct, the question is whether she possesses the capacity to consent to medical treatment and the extent to which the wishes of her parents should or should not be allowed to override those of their daughter.
The referral note states that Sabina is 15. It would therefore be unusual not to involve her in decisions concerning her present and future medical care. You say that in the past she ‘actively’ agreed to have the pump fitted. This implies that she had been consulted and was able to express a considered point of view. As far as the law is concerned, Sabina is still a child until she is 16. However, the extent to which she can consent to or refuse medical treatment turns on whether she is or is not deemed to be ‘Gillick competent’. 1
In order to answer your question, the committee wanted to know much more about Sabina's cognitive development. This would help distinguish between the following factors which may or may not be affecting her willingness to express her views on her medical treatment:
her underlying cognitive abilities, regardless of her current circumstances; the extent to which, if at all, Sabina's ability and willingness to communicate are dependent on her particular circumstances; in particular, whether she is in the PICU or whether she is at home with the pump in place; the discussions, if any, which have been held with Sabina regarding her present and future medical care and the hopes and fears she may have? Has she been asked what she would like to happen if her pump fails again?
We are told that Sabina's condition ‘mildly affects’ her cognitive function, that she has a reduced reading age but is nevertheless capable of ‘articulating her thoughts and that it is possible that her understanding is at a higher level than may initially appear’. We are also told that Sabina appears to understand the difference between life and death, has always ‘readily agreed’ to active treatment and is also keen to engage in typical teenage pursuits such as playing games on her Nintendo Wii.
However, we are also told that before her most recent ventilation Sabina would answer questions with monosyllabic replies and express basic preferences, statements which seem to be at odds with the earlier description of ‘mild’ cognitive impairment but which may also be due to a number of factors, for example, her medication. It might also be possible that she is suffering from depression because of the prospect of her impending stay in intensive care and the impact of ventilation on her quality of life. This in turn could also affect cognitive function and so it is important to establish whether this is the case.
It is unclear what steps have been taken to facilitate Sabina's ability to communicate. You say that when her care team tries to provide her with information while she is in the PICU this seems to result in more distress. Is this due to the nature of the treatment itself, the fact that she dislikes being in intensive care, or that while she is in intensive care, communication is more difficult? If it is clear that certain situations appear to cause Sabina distress, then it may be appropriate to defer the discussion on her medical care until such time as she is willing and able to engage in it rather than not involving her at all.
The committee then discussed the views of Sabina's parents and why they have asked that, from now on, she is not involved in discussions about plans for her future care. Some committee members wondered whether their reluctance to involve her may be due to Sabina's apparent distress at being in the PICU and the understandable wish her parents may have to protect her from further distress. It is, however, not clear whether their wish to exclude her from discussions extends beyond Sabina's stay in the PICU.
This would be important in determining the extent to which their rights could be allowed to override those of their daughter in terms of what she should be told. It was felt that in general, there should be a presumption in favour of taking parental views into account; a view explicitly endorsed in the Department of Health guidance. 2 However, in Sabina's case, a decision about whether the views of her parents should be permitted to trump hers would depend on a detailed assessment of her overall cognitive abilities.
If there is clarity about Sabina's cognitive function and if she is deemed to have capacity, she should be involved in discussions on her future medical care. However, it may be preferable for these discussions to take place at an appropriate time and preferably not when she is in the PICU. At this point, a decision on her part to refuse medical treatment should be respected. The committee feels that it would not be right, either morally or ethically, to enforce medical treatment on a 15-year-old but that they should always have the right to change their mind. 3
If Sabina is not deemed to be competent and that this is a state unlikely to be affected by her immediate circumstances, then the question of what is in her best interest will be left to her parents and her doctors to decide. Every attempt should be made, however, to involve Sabina in these discussions, provided this does not cause her unnecessary distress.
The committee made one final point. It felt that although it was right to refer this case to the clinical ethics committee in order to ensure that decisions involving Sabina are properly made, it would be advisable to involve colleagues who are not part of the intensive care team in future decisions about her care. As intensivists, you are the gatekeepers to a limited resource, namely intensive care beds, and it is therefore extremely important to ensure that issues of resource, however unintentionally, are not allowed to influence judgements about what is best for Sabina.
In summary:
(1) It is important to establish the cause of Sabina's repeated infections, whether they can be controlled or prevented in future and what their role in her overall prognosis might be. If they are likely to be controllable and Sabina's clinical prognosis suggests that she has a further 10–15 years left to live, it may be justifiable to re-fit the pump even if this means that she may have to endure periods of time in intensive care. If, however, it is unlikely that her chances of infection can be reduced and she faces prolonged episodes of ventilation and sedation, the impact on Sabina's quality of life may be such that it would be reasonable to start discussing the possibility that if the pump fails again, Sabina should be allowed to die. (2) The extent to which the wishes of Sabina's parents should be respected depends on a thorough assessment of Sabina's cognitive abilities. Legally, in terms of her ability to consent to treatment, Sabina remains a child until she is 16, and so those with parental responsibility retain the right to consent to or refuse treatment on her behalf. However, the issue which must be determined is the extent to which Sabina is competent to understand and make informed choices about her present and future medical care. Only when this is determined can a decision be made about whether her parents' wishes can be respected and in any case, every attempt must be made to ensure that Sabina's views, however they are expressed, are acknowledged and respected.
Membership of Trust Clinical Ethics Committee
Ayesha Ahmad, Lay Member; Sarah Barclay, Vice-Chair and Lay Member; Joe Brierley, Consultant Intensivist, GOSH; Dr Kate Bull, Medical Advisor, Family Policy, GOSH; Harriet Coniff, Clinical Psychologist, GOSH; Jonathan Elwood, Legal Advisor, GOSH; Madeline Ismach, Head of Psychosocial Services, GOSH; Carol Jennings, Clinical Nurse Specialist and Renal Transplant Co-ordinator, GOSH; Vic LarcherReverend James Linthicum, Anglican Chaplain, GOSH; Tracy Lunnon-Wood, Principal Clinical Psychologist, GOSH; Rosie Midson, End of Life Care Manager, GOSH; Grainne Morby, Manager, Patient Advice and Liaison Service, GOSH; Kiera Parkes, Clinical Nurse Specialist; Andy Petros, Consultant Intensivist, GOSH; Janet Radcliffe-Richards, Professor of Practical Philosophy, University of Oxford; Isabelle Russell-Eggitt, Consultant Ophthalmologist, GOSH; Ellen Schroder, Non-Executive Director, St Mary's NHS Trust, Paddington; Bea Teuten, solicitor and accredited mediator; Dr Mark Thomas, Consultant Anaethetist, GOSH; Eleanor Updale, Lay Member, Dr Neil Vass, General Practitioner.
Footnotes
*
The author attended this meeting as a representative of Clinical Ethics and did not take part in the discussion of this case.