Since the Lorber era there have been tremendous successes in the treatment of patients with MMC [48, 50]. Over the last 30 years, continual progress has not only been made in the neurosurgical (closure of the back defect, shunting, or endoscopic techniques for the complicating hydrocephalus, treatment of tethered spinal cord and Chiari II malformation), orthopedic (treatment of scoliosis, correction of club feet), urological (preserving normal renal function, securing social continence), and psychosocial treatment of patients with MMC, but also in support for independence by modern rehabilitation programs.

Nevertheless, early decision making (for restriction) and selective treatment of newborns with MMC is still propagated [27, 69], resulting in approvals but also in critiques [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.

Lorber (1971) stated that untreated children will die quickly [44]. However, he delivered the children anticonvulsants to prevent seizures, opioids and chloral hydrate to prevent pain [25]. One pediatrician has said that these untreated children received the same care and attention as others, being fed and loved, but they also received 60 mg/kg body weight of chloral hydrate, four times a day. This was being administered for pain, but the children who were operated on did not receive this regimen. Another physician just said: we don’t feed them [79]. Consequently, the duration of life seems to depend on the attitude of the responsible doctor.

In the United States, untreated children were not given morphine or anticonvulsant drugs and were fed every 4 h, resulting in many ‘survivors’ with severe handicaps. On the contrary, in the United Kingdom the children died quickly. However, the question remains if these children were really in pain or suffered from seizures; perhaps not.

Not all physicians did follow the ‘Lorber criteria’, but treated all children unselectively, regardless of the level of the lesion. This approach resulted in multidisciplinary MMC clinics and MMC teams. The nonselective treatment of newborns with MMC started in the early 1970s and gives us a picture of long-term outcome of newborns with MMC [4, 9, 32, 48, 52] and also of the quality of life and health status of adults with spina bifida [58, 63]. Some physicians became bitter after treating children with MMC for years [2].

In the early 1980s, the ‘Baby Jane Doe (‘Stony Brook baby’) case resulted in the United States in an ethical debate on the prospect physicians make about the future life of handicapped children born with MMC. This girl was born with MMC, complicated with hydrocephalus (although diagnosed by the attending physician as microcephalic). The team of physicians told the parents that the child would spend her life “lying in bed, bottle fed....She would experience no joy, sadness or any such emotion except response to pain and would develop no cognitive skills. She might survive for 20 years” [51].

Based on the given information, the parents agreed not to treat their child. However, the physicians turned out to be wrong. Although the child developed meningitis, sepsis, and hydrocephalus (which were only treated to some extent), she survived all these complications, was taken home, became ambulating and communicating on an acceptable level of intelligence, all this despite the partial treatment [24, 25].

After the introduction of alpha feto-protein (AFP) screening in the late 1980s (followed by amniocentesis) and ultrasound diagnosis, many fetus with MMC can now be detected early in pregnancy. Nowadays, after being counseled, most pregnant couples decide to abort a fetus with MMC (in many Western countries resulting in an abortion rate of 95%, resulting in fewer children with MMC born [13, 14, 53]), seen by many as ‘prevention’ of MMC. But what advice should neonatologists, neurologists, or neurosurgeons give the parents when a full-term child with MMC is born? Is the advice not to treat, or to terminate the life of the newborn deliberately (involuntary euthanasia) an ethically defendable one?

In the 1980s, the Dutch ethicist Hertogh (1988) published his paper “Ethical considerations on the dilemmas caused by the selective treatment of newborns with MMC” in the leading Dutch medical Journal [30]. He elaborated on the philosophical and ethical aspects of the Lorber selection criteria: “The objective medical fact of a physical shortcoming is something completely different than the subjective reality of ‘being-handicapped’ itself. Studies have shown that living as ‘being-handicapped’ does not correlate directly to the severity of physical shortcomings. The question as to what can be considered as an acceptable handicap transgresses, in other words, the scope of what can be ascertained in a medical-scientific way and this, therefore, refers inevitably to the subjective experience. It has to be questioned whether the quantification of qualitative aspects of medical treatment perhaps offers nothing more than pretence-rationality, caused by the used decisive terminology. In conclusion: up to date the criterion ‘quality of life’ appears to be more deceptive than clarifying in the medical decision about the possible continued existence of a newborn with spina bifida.” [30] He also referred to the ethics of the philosopher Levinas: “Ethos cannot be founded in scientific facts; it is founded in the human relationship itself. Mutual dependency, which is characteristic for ‘human being’, makes us responsible just by itself and therefore defines ‘the disposition of ethos’ as a rational fact. This relationship always precedes facts themselves.”[30] In other words: human beings, just because of the fact that they are human beings, have an intrinsic and mutual responsibility for the well-being of others, this responsibility itself preceding physical qualities. This thoughtful paper, however, was not given much attention in the Dutch medical press.

In 1993, baby Rianne was born in a small town in the Netherlands with a MMC at level of the first and second lumbar vertebrae complicated by hydrocephalus. The lower limbs were deformed and paralyzed. Computed tomography (CT) scan of the head confirmed the diagnosis of severe hydrocephalus. On the first day of her life, the decision was made by the pediatrician and the gynecologist in consultation with a neurologist, neurosurgeon, and pastor not to operate the back lesion and the hydrocephalus. On the fourth day of her life, at the request of the parents, the gynecologist Prins terminated the life of the baby by a lethal injection. In an interview, Prins stated [37]: “Closing the defect and fighting infections with maximal treatment could enable the child to survive for years. No one knows how long”. On the question of what an operation could have accomplished, Prins answered: “The defect would have been closed. The possibility of secondary infection, which was an immediate threat to her life, would have been diminished. Her brain pressure would rise, however, and it would have been necessary to implant a shunt”. On the question of how he would calculate the benefit of treatment, he answered: “It (the operation) would have prolonged her life without treating the symptoms and consequences of the defect because the operation would not have alleviated them. The operation would only have minimized the possibility of infection, but it would have created the necessity for repeated operations involving the shunt and the urological system because there were obstructions in the passage between the kidney and the bladder. Her brain damage was so extensive and the possibility of movement so limited. She would never have been able to walk or sit unsupported; she would have been incontinent in her bladder and bowel. There was sensory damage as well; she could not feel sensation in her lower body. She would have started life with repeated operations, need for persistent medical attention, no possibility for self-support, and very low communicative ability” [37]. As a definition of a severe handicap he stated: “This can relate to the estimated possibility of becoming happy” [37], and he further referred to the Dutch Association of Paediatrics who developed criteria, including the lack of capacity to communicate, lack of capacity for self-maintenance, persistent need for medical assistance, and the existence of pain. All these criteria grouped together gave a picture of the baby’s quality of life. Because most of the criteria were satisfied, the baby was considered to be severely handicapped, resulting in a very poor expected quality of life; the child was not operated on because of the severity of the expected handicaps.

On the necessity of life-termination, Prins (1997) explained: “The baby was in a great deal of pain and the normal analgesics did not work well. So it would have been necessary to use narcotic analgesics—morphine-like drugs. And that would have caused depression of consciousness. It is clear to me that the use of such drugs would have depressed normal functioning—behavior, expressions, and sensibility. Using morphine-like drugs would have caused a situation of floating between heaven and earth. It would have been dishonest and should be considered as unethical and unjust. There is no moral difference between killing and withholding useless medical treatment. There is an emotional difference. And there should not be a legal difference” [37].

Although elements of the offense of murder were proven, the physician was acquitted based on the unavoidable conflict of duties between his duty to prolong life and his duty to alleviate unbearable suffering [26].

In 1996, three Dutch child neurologists and one neurosurgeon questioned in the leading Dutch medical journal the active termination of life of newborns with MMC [59]. They defended the opinion that (severe) handicapped life could also be meaningful. Deliberate termination of life was not considered appropriate; sedation and the delivery of opioids were mandatory in the case of suffering. Not treating a child with MMC should not result in a more severe handicapped life; the decision to withhold treatment was only made in the case of severe pulmonary or cardiac complications with the prospect of early death. The possible suffering of the parents should not be an indication for deliberate termination of the life of a newborn with MMC. They referred to the study of Steinbok et al. (1992) [68] for their argument that the life of children and adults with (severe) MMC can be meaningful [59].

In 2003, the Dutch physician/medical ethicist Van de Vathorst stated that whenever it is decided not to treat a defective newborn, deliberate termination of the life of this newborn should be regarded as a moral demand, based on the principle of ‘mercy’ [72]. She illustrated her arguments with a case of a newborn with a thoracic MMC. Interestingly enough, the life of this child was not terminated. It died by itself, a few days old, because of pulmonary insufficiency. Although she received a national award for this paper, it resulted in several disapproving letters to the editor.

In January 2005, two pediatricians, one physician, and one pediatric neurologist reported on the deliberate termination of life of 22 newborns in the Netherlands between 1997 and 2004 [73]. Explaining the fact that all 22 cases were newborns with MMC and hydrocephalus, Verhagen et.al. referred to the ‘baby Rianne case’, stating that “her life was dominated by unbearable suffering” and “since this case it is established that newborns with a severe form of spina bifida do suffer severely” [73].

According to the authors, the deliberate termination of life was necessary because of the presence of “unbearable and hopeless suffering, acutely and long-term, with no other proper medical means of alleviating the suffering” [73]. All parents consented to the proposal of termination of life; in four cases, they explicitly requested for it. For the public prosecutor, the termination of life was acceptable if four requirements were properly fulfilled: (1) the presence of hopeless and unbearable suffering, (2) consent of the parents to termination of life, (3) consultation of another physician or of a multidisciplinary MMC team, and (4) a proper and careful execution of the termination. These four criteria eventually became the cornerstone of the so-called ‘Groningen Protocol’, a protocol meant to insure that the decision for life termination, the executing itself, together with reporting to the legal authorities, are all conducted in a proper way. This protocol was approved and adopted by the Dutch Association of Paediatrics in 2005.

The motivation for the judgement of ‘unbearable and hopeless suffering’ in the 22 cases of spina bifida was [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.

This article of Verhagen et al. (2005a) gave rise to only a few reactions in the Netherlands. Kompanje et al. (2005) [38] questioned the validity of ‘unbearable suffering’ as a useful criterion. They also stated that with the use of modern palliative care, ‘suffering’ always can be dealt with in an adequate way, as did Laane (2005) [39, 40]. De Jong (2006) [35] made a plea for ‘letting them die’ instead of ‘making them die’ in the case of an untreated newborn with MMC.

On request of the editor of the New England Journal of Medicine, Verhagen and Sauer (2005) published another article concerning the Groningen protocol and the 22 cases [75] and a similar article appeared in Pediatrics [76]. The reactions in the international medical press were mostly negative. In his article “Control of suffering on the slippery slope of care” in the Lancet, Feudtner (2005) also addressed the poor quality and the insufficiency of the offered palliative care and questioned whether legalization of active termination of life might not lead to the abuse of it [21]. Saugstad (2005) stated in the Acta Paediatrica: “Neonatologists must be extremely careful not to start on the slippery slope ending in the Dutch practice of euthanasia of newborns. I would strongly warn against this attitude, which I find non-compatible with Western humanistic traditions” [61]. Jotkowitz and Glick (2006), in the Journal of Medical Ethics, were worried: “We feel an important line has been crossed if the international medical community consents to the active euthanasia of severely ill infants and are concerned about the extension of the policy to other at risk groups” [36]. Bondi et. al. (2006), in Pediatrics, made a plea for an open discussion: “Rather than performing an in-depth examination of the philosophical, ethical, and moral implications of this protocol, the authors focus on its technical details and acceptability within the European medical community. The Groningen Protocol centers not on patient self-determination, but instead on the perception of the patient’s current and future suffering. Although the authors note that an open discussion of these issues is ‘extremely important,” they fail to initiate it. We hope that thoughtful ethicists and legislators will initiate the open discussion that the authors advocate” [8]. In their article ‘Why the Groningen Protocol should be rejected’, Chervenak et al. (2006) concluded that: “The justification they offer is woefully inadequate. It relies on appeals to the concepts of “hopeless and unbearable suffering,” the “best interests of the patient,” and “medical-ethical values,” but it never explains these concepts, and it gives no ethical argument for their clinical application. In fact, not only is no argument for the Protocol given, none can hope to succeed. The Groningen Protocol should therefore be rejected as the basis for obstetric and neonatal practice in The Netherlands and throughout the world.” (11)

Manninen (2006a), finally, made an important contribution with her article “A case for justified non-voluntary active euthanasia: exploring the ethics of the Groningen Protocol” in the Journal of Medical Ethics [45]. She made a thoughtful plea for the ethics of the Groningen Protocol: “What the Groningen Protocol proposes to do is both ethical and also the most human alternative for these suffering and dying infants.” Newborns eligible for this protocol “do face no future at all—that is, the infants must be terminally ill”, “will die soon no matter what actions doctors undertake, modern medicine can do nothing to save them”, “have no prospects, have crossed the threshold into a comparatively valueless state of existence”, “will spend the remainder of their lives in a chronic state of nothing but pain and suffering” [45]. Interestingly enough, on November 23 an additional letter of herself, “Regarding the Groningen Protocol” [46], was published in the same journal. Some key passages of this letter: “It has recently been brought to my attention that the physicians of the Groningen Hospital who originally proposed the Groningen Protocol have proposed to violate the first two conditions of the original Groningen Protocol: (1) The suffering must be so severe that the infant has no prospects for a future; (2) There is no possibility that the infant can be cured or alleviated of her affliction with medication or surgery. For example, Dr. Verhagen has maintained that the Protocol could apply to infants that are not necessarily terminally ill, but nevertheless may face a compromising future. In essence, the Groningen Protocol has begun to make quality of life judgments. If the Groningen physicians have commenced making quality of life judgments, they have strayed away from the original purpose of the Protocol, and this, I believe, may have concerning ethical implications. As I write in my paper: “...the Groningen Protocol does not make quality of life judgments, and it is because of this very important requirement that the Protocol strikes me as humane and morally permissible, for it seems utterly vicious to extend the life of a suffering infant with no prospects for a future. If the infant did have a possible future ahead of her, I would be more hesitant to condone a legal practice that begins to make quality of life judgments, for such judgments can be, and have been, subject to error.” [46] In her opinion, in these end-of-life decisions ‘quality of life judgments’ should not be made and, in a way, with this letter she also highlights the inconsistencies in the reports by Verhagen et al. Being of crucial importance, the subject of ‘quality of life judgments’ will be discussed more extensively in paragraphs 4 and 5.

Some examples: Article 25, UDHR: “Everyone has the right to a standard of living adequate for the health and well-being of himself, including medical care and the right to security in the event of disability. All children shall enjoy the same social protection”. CESCR, par. 26: According to standard Rules: ”States should ensure that persons with disabilities, particularly infants and children, are provided with the same level of medical care within the same system as other members of society.” UNCRC: “In its examination of States parties reports, the Committee should commit itself to highlighting the situation of disabled children and the need for concrete measures to ensure recognition of their rights, in particular the right to live, survival and development”. UN, General Counsel: “We will take all measures to ensure the full and equal enjoyment of all human rights and fundamental freedoms, including equal access to health, education and recreational services, by children with disabilities and children with special needs, to ensure the recognition of their dignity, to promote their self-reliance and to facilitate their active participation in the community”. Proposal text of the American delegacy for the UN Convention to Promote and Protect the Rights and Dignity of Persons with Disabilities: ”States Parties reaffirm the inherent right to life of all persons with disabilities, shall take all necessary measures to ensure its effective enjoyment by them, and shall ensure that disability, or perceived quality of life, shall not serve as a basis for infringement of the right to life.” [19]

These international political statements and agreements are also reflected in the resolution of the International Federation for Spina Bifida and Hydrocephalus (IF) as formulated at the 12th International Conference (“The Right to be Different”) for Hydrocephalus and Spina Bifida in Toulouse [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.
• Most adults with spina bifida underline that their quality of life is not automatically—and should not be given as—a reason for abortion [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.

When it is decided that surgical treatment is not a feasible option in a newborn with spina bifida, it is regarded by some to be an act of ‘mercy’ to terminate the life of the newborn [72]. This notion, however, is a misconception: they are not terminally ill because of the MMC and/or hydrocephalus per se; they are ‘terminally ill’ because of the decision not to treat their congenital malformations. The needs of these untreated newborns, however, are still the same as the needs of all newborns: proper care, feeding, comfort, respect and love; till death (and apparently not death itself). The ‘need’ for life termination in these patients can only be regarded as an indication of insufficient palliative care. Life termination as an act of mercy can only be applied to hopeless situations that can occur, e.g., on battlegrounds or in mountaineering, when there are no proper means indeed to alleviate real unbearable suffering. This is obviously not the case in a modern equipped hospital (at least, it should not be) with experience in palliative care and pain management.

This is all confirmed in the paper of Sauer in 2001, “Ethical dilemmas in neonatology: recommendations of the Ethics Working Group of the CESP (Confederation of European Specialists in Pediatrics)” in the European Journal of Pediatrics [60]. In this paper, some ethical principles, applicable to every newborn, are being formulated, like: “decisions to withhold or withdraw treatment should always be accompanied by optimal palliative therapy and dignified and comforting care” and “every form of intentional killing should be rejected in paediatrics” [60]. This is followed by: “However, giving medication to relieve suffering in hopeless situations which may, as a side effect, accelerate death, can be justified”.

All period long, from birth till 1 week after the shunt revision, the well-being of the baby was estimated by making use of the Comfort score and the VAS and also medication used for treatment of pain or discomfort (paracetamol and morphine) was recorded (Fig. 3a,b).

Studying these data and particularly the amount of medication needed, several conclusions can be made:

• 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.

In summary, this case not only demonstrates that there is no such thing as ‘suffering’ in these newborns, but also the incorrectness of the statement of Verhagen et al. (2005a) [73] that “the suffering of these newborns cannot be alleviated by any means in a proper medical way” (Fig. 4). The overall impression of this case is in accordance with the preliminary results of the Rotterdam Prospective Study on Discomfort in Newborns with MMC: there is no “acutely unbearable suffering” in these newborns (Figs. 5a,b)

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.

To throw more light on these end-of-life decisions, Verhagen et al. should consider to extend their retrospective study, focusing on the medical aspects of the 22 cases. Thus, it must be possible to get a basic understanding of the extent of the malformations themselves, of possible co-morbidities, whether or not quality of life judgments were made, how ‘unbearable suffering’ was ascertained, how palliative care was offered, and why this failed. Such a study would contribute significantly to the open discussion they propagate. Verhagen is undoubtedly correct indeed in his appeal: “It’s time to be honest about the unbearable suffering endured by newborns with no hope of a future”. [71]