“An infant with spina bifida cannot ‘suffer unbearably’. Infants might be able to experience unbearable pain, but spina bifida does not cause it.” (Chervenak 2006) Introduction and historical overview of the treatment of newborns with MMC 22 66 44 48 50 27 69 7 23 41 42 49 79 What is considered ethical at one time, or in one country, can be considered unethical or unacceptable at/in another. The ethical debate on treating children with MMC is unique in the sense that these children will/can have a life of substantial and sometimes severe physical disability but often with normal mentation, this in contrast to severely psycho-motor handicapped children. Children and adults with MMC can be aware of their deficits. die quickly  44 to prevent seizures to prevent pain  25 we don’t feed them 79 not  died quickly. 4 9 32 48 52 58 63 2 Baby Jane Doe (‘Stony Brook baby’) microcephalic 51 24 25 13 14 53 35 38 40 71 76 78 73 Deliberate termination of life in newborns with MMC in The Netherlands 41 42 41 30 30 30 37 37 37 37 26 59 68 59 72 73 73 73 73 ‘suffering’ (acute and chronic pain, physical pain) (100% of the 22 cases); the ‘lack on ability to live or do things independently’ as the result of ‘severely disturbed sensomotoric development’ (100% of the 22 cases); the ‘lack of possibility to verbal and non-verbal communication’ (82% of the 22 cases); ‘the prospect to dependency to the medical circuit as the result of frequent hospital admissions and operations’ (77% of the 22 cases) the ‘life expectation’ (‘the burden of severe suffering increases by a longer life span’) (in 59% of the 22 cases). None of the cases led to prosecution; all cases were found to be in accordance with good medical practice. 38 39 40 35 New England Journal of Medicine 75 Pediatrics 76 Lancet 21 Acta Paediatrica 61 Journal of Medical Ethics 36 Pediatrics 8 Journal of Medical Ethics 45 45 46 46 Finally, in 2006, in The Netherlands a national multidisciplinary expert review committee has been appointed. In the case of active termination of the life of a newborn, the responsible physician is supposed to inform this committee, which then has to determine whether the criteria for life termination have been met. Subsequently this committee has to advise the Public Persecutor’s Office (OM) whether the physician should be prosecuted or not. Although such a committee seems to guarantee more certainty, it can be doubted, however, if this ‘checking on afterhand’ is of much value for the newborn in question. It seems to be more sensible to change this procedure to a ‘checking on beforehand’. Legally, this might perhaps be difficult to establish, but it seems not justifiable that legal procedures themselves do dominate the question of appropriateness of life termination; errors in judgment being unacceptable (cf. the level of certainty required for a death penalty in crime in, e.g., the USA). Analysis of the mentioned criteria for non-treatment and deliberate termination of life Suffering 73 75 76 Acute pain 79 17 18 17 18 Chronic pain 12 12 20 The ‘lack of ability to live or do things independently’ as the result of ‘severely disturbed senso-motoric development’ 73 48 32 52 62 62 Besides these results, criteria such as ‘living independently’ or ‘doing things independently’ can be confusing when deciding about the future life of vital newborns. Most people who do live a meaningful life are dependent on others or interdependent on each other. In fact, interdependency and especially the willingness to care for other (perhaps dependent) people can be considered as a criterion of a truly civilized society, certainly so when this means to offer ‘something for nothing’ (this being real mercy). The ‘lack of possibility of verbal and non-verbal communication’ 73 3 4 9 32 33 47 67 68 70 9 68 32 23 15 ‘The prospect of dependency to the medical circuit as the result of frequent hospital admissions and operations’ 71 ‘Life expectation’ (‘the burden of severe suffering increases by a longer life span’) It is clear that the longer a patient lives, the more medical care will be needed in a quantitative way. Whether this means that this will affect the experienced quality of life in a negative way remains totally unclear and cannot be predicted. Furthermore, patients with MMC do require relatively much of their medical care in their first two decades, this steadily decreasing when they grow older and when disabilities and coping with these disabilities are gradually stabilized. Can ‘suffering’ be applied as a reasonable criterion in newborns? Suffering, especially the ‘unbearable and hopeless’ aspects of it, is a complex psychosocial phenomenon and by definition strictly individualized: only the individual itself can experience the existence, the intensity, and hopelessness of it. Others (parents, physicians, nurses) can only to some degree make an estimation of it. It is therefore tentative whether ‘suffering’ can be applied to newborns at all. What is possible, however, is to establish quite precisely the level of ‘(dis)comfort’ of a newborn by making use of standardized score charts such as the Comfort score and the Visual Analogue Scale (VAS) (see below). 6 29 29 38 11 39 Can physicians predict quality of life? Counseling the parents 14 25 25 10 71 78 Does the level of the lesion matter? 66 77 65 65 31 54 55 62 64 64 In conclusion, the level of the lesion does not correlate with the actual experience of the handicaps themselves. Is the diagnosis ‘extensive brain damage’ easy to make? irreversible and severe brain damage 3 1 5 ‘Quality of life’, to conclude 11 11 45 46 Non-discrimination principle 19 19 34 People with spina bifida and hydrocephalus can live a full life with equal value to that of any other citizen and they should not be seen as a medical condition. Their views should be sought and heard by governments and health professionals, who should acknowledge the right of people with spina bifida and hydrocephalus to speak for themselves. People with disabilities have the right to aim for lives as rewarding as those of their peers who have what is regarded as a normal life. Supportive systems must be in place when they are needed. Experience over the past 30 years has improved considerably the medical outcome and the quality of life of people with spina bifida and hydrocephalus. 34 As a recognition of the importance of non-discrimination on the basis of disability (that is to say, present as well as expected disability) or (expected) quality of life in deciding whether or not a newborn child with, e.g., spina bifida should be treated or whether or not it is justified to deliberately terminate such a child’s life, Dorscheidt (2006) recommends to involve the legal concept of ‘objective justification’ in the medical-decision making process. By using a particular questionnaire, the physician in charge may take advantage of this concept when considering (and deliberating on) how to act properly and professionally. Through this, the physician can ensure the disabled newborn child’s rightful claim to equal protection of its right to life and its right to health(care). Doing so would offer possibilities to replace the usual doctor-centered approach of decision making by a more patient-centered approach as well. 11 Palliative care for untreated newborns with MMC 43 57 28 Oxford Textbook of Palliative Care for Children 16 16 Intensive Care Medicine 56 72 European Journal of Pediatrics 60 60 To date, it is not clear why these recommendations should not hold for cases of untreated spina bifida and hydrocephalus in the Netherlands and why the Dutch Association of Paediatrics does not adopt these recommendations. Illustrative case 1 2 Fig. 1 Photograph of the newborn, showing the extensive MMC, extending from +/− the 4th thoracic vertebra till the midlumbar region  Fig. 2 Photograph at the end of the surgical procedure. The MMC was closed by making use of extensive rotational flaps together with free skin grafting  3 Fig. 3 a Green bars Green/black bars b a  When untreated (the first 3 days), the child was not discomfortable. Closing of the back lesion, in this case certainly a major procedure, did not contribute significantly (and only temporarily) to more discomfort. In other words, closing of the open spine can be qualified as a proportional treatment modality. The same holds true for placing a ventricular shunt. Treating an active hydrocephalus adequately contributes to the well-being of the patient. In 1 month’s time, this active treatment resulted in a stable situation of overall well-being in this patient. Only paracetamol and low-dose morphine were necessary in the treatment of actual discomfort. 73 4 5 Fig. 4 The same child at 7 months old; fixating and following with the eyes, vocalizing, normal movements of the arms, moving both legs vigorously (perhaps nonvoluntary), still being partially fed by a nasogastric tube  Fig. 5 a b n  78 73 76 Synthesis and conclusion There is no evidence that newborns with MMC and hydrocephalus do either ‘suffer’ unbearably or hopelessly and certainly not without the prospect to relieve this suffering by standard care. ‘Suffering’ itself is a nonconclusive, and in newborns, inapplicable denominator that should not be used anymore in this debate. Although they will in their future life be confronted with handicaps, sometimes very severe, their future prospects and their actual experienced quality of life cannot be predicted with such certainty at birth that their lives can be regarded as hopeless or meaningless (‘quality of life judgments’ as such being unacceptable in this decision making). Possible discomfort in these newborns can easily be treated in a straightforward way by active treatment (closure of the defect and shunting the hydrocephalus) and, when necessary, by the use of a professional pain/symptom protocol. The decision not to treat such a newborn, when based on expected handicaps, possibly violates the ‘non-discrimination’ principle (Dorscheidt, 2006). When not being treated, they are not terminally ill because of the MMC and/or hydrocephalus per se; they are ‘terminally ill’ because of this nontreatment decision. Not being terminally ill, it is not ‘humane’ or ‘merciful’ to terminate their life, this also being not in accordance with international legislation and international medical recommendations. When untreated and when it is the intention to alleviate actual discomfort (this question of intent being crucial to a moral and legal analysis of end-of-life decisions including active termination of life), this can always be achieved in an effective way by using one of the widely accepted palliative protocols. Such a child can and should be cared for in a respectful and dignified way, providing all its actual needs (which apparently is not death itself). This being the case, there is no indication whatsoever for the deliberate termination of the life of children born with MMC. While Verhagen et al. (2005, 2006) must be credited for bringing the discussion about deliberate termination of newborns in the open, to date, there does not seem to be much in their qualifications that can be quantified properly, especially not in cases of MMC, which is prone to quality of life judgments (and thereby neglecting the needs and rights of the newborn). To date, the Groningen protocol, therefore, cannot be regarded as very useful. Being a tool, it seems to have been changed in a means in itself. To gain more usefulness, at least it has to be extended in a way as proposed by Dorscheidt (2006), by adding charts such as the Comfort Score and the VAS and by adding palliative medication charts. Thus, it should be changed from a doctor-centered protocol to a patient-centered protocol. 71 Finally, to date it remains unclear on what grounds the Dutch Association of Paediatrics has adopted the Groningen protocol and why international legislation, international human rights instruments, and international medical recommendations appear not to hold for the Netherlands, especially not in newborns with MMC.