Unenviable decisions: Is it ethically justifiable to withhold parenteral nutrition from infants with ultra-short bowel syndrome?

Starting or stopping

For the purpose of this essay, I assert that withholding treatment or intervention is morally equivalent to withdrawing a treatment or intervention. This assertion is supported by a plethora of ethical research and is outlined in many medical ethical guidelines and texts. ^{6 – 11}

Short bowel syndrome

Short bowel syndrome (SBS) is defined as a small bowel length of <200 cm, ¹² and ultra-short bowel (USBS) syndrome when the small bowel is <10 cm in length. ¹³ In SBS, there is insufficient small bowel to facilitate adequate nutrition and, in order for survival, parental nutrition is provided to treat inadequate nutrition.

Gastroschisis is not the only condition that results in children developing SBS and SBS does not automatically equate to bowel transplantation. Additionally, PN formulations are improving so children with SBS are able to be managed on PN for longer periods of time with relatively reduced cumulative damage to their livers. However, due to the relatively small numbers of SBS patients per center, the reported outcomes from children with SBS are often presented as a whole in the literature, rather than by their different underlying pathologies. For articles that have undertaken subgroup analysis for the underlying pathology that led to the SBS, there appears an increased risk of adverse outcomes in children where gastroschisis was the underlying reason for the SBS. ^{14 , 15} The exact mechanism for this has not been well described. However, gastroschisis does consistently appear as an independent risk factor for nutritional failure, inability to wean from parenteral nutrition (PN) and increased need for bowel transplantation in children with SBS, with some studies reporting up to 88% of their gastroschisis-related SBS cases requiring bowel transplantation. ¹⁵ Outcomes from bowel transplantation in children are problematic as many studies report only 5- to 10-year follow-up, which whilst helpful, does not give an indication of the course of treatment over a child’s lifetime. However, as with children undergoing any transplantation, successfully transplanted organs do not function indefinitely, and the recipient is likely to need to undergo successive transplants when their original fails after a period of time. Even in cases where the bowel transplant has been successful, the graft-failure rates are 55% at 5 years and 66% at 10 years. ¹⁶ Whilst the additional time, the transplant has given the patient and their family is not to be dismissed, the prospect of long-term PN ± bowel transplantation remains an unenviable process to go through for any patient and family.

Provision of parenteral nutrition

Infant A’s four remaining centimetres of small bowel were insufficient to enable adequate nutrition to be absorbed via the bowel and nourish a growing body. Therefore, in order to provide sufficient nutrition for growth and development, PN would be required. Parenteral nutrition can either be given alongside enteral feeds, whilst the infant grades up the amount of feed that their bowel can tolerate, or, it may be the only source of nutrition for the infant if they are unable to take any feed via their digestive system (total parental nutrition or TPN).

Parenteral nutrition is a medical laboratory-created solution that contains the necessary components of nutrition in order to allow for the growth and development of the infant. ¹⁷ It is provided via central venous catheter. PN in any form (total or partial) is not a benign intervention. It is a medical creation, designed to overcome situations where an infant or adult would not be able to take sufficient nutrition via their digestive system. Similar to any other medication it was developed with a specific therapeutic goal and an acknowledgement that, as with all medications, there are undesired side effects. It was not initially developed for life-long use, although as its use has increased it is being utilised in more extreme situations in an attempt to preserve life. Overall, long-term survivability on home PN is improving significantly. ¹⁸ This means children established on home PN have better survival rates than ever before; however, this is dependent in part on their underlying pathology. ¹⁸ This survival time is important in allowing them to develop, interact with their world and form meaningful relationships. This benefit is significant and should not be dismissed lightly. However, long-term PN is a huge undertaking for both the child and the family. Long-term and home PN still carries significant mortality and morbidity, for example, PN-associated liver damage. ¹⁹ With exposure to long-term PN, the liver becomes dysfunctional and ultimately fails. Liver failure carries its own significant morbidity and mortality. In addition, it means for individuals with intestinal failure on PN awaiting a bowel transplant, the majority will develop liver failure before a suitable bowel is found; and therefore, the majority will require a bowel and liver transplantation, making the transplant even more problematic. ^{20 , 21}

Parenteral nutrition is not food

Parenteral nutrition shares many more characteristics with medication than it does with food. It is a laboratory-created substance that has little bearing to any naturally derived food stuff. Additionally, its route of administration is clinical and avoids the digestive system entirely, which is both its purpose and also the key feature that separates it from food. Food is an integral part of human culture. Survival instincts have programmed us to place great value on food. Humans have an evolutionary imperative to prioritise food for their own and their infant’s survival. There is enormous bonding that occurs through feeding an infant and significant parental anxiety when their child is not eating, such as when they are ill. It is a basic parental instinct to want to feed their child. As Stevens research into parental experiences of feeding their preterm infants illustrates, ‘“Feeding” encompassed more than providing nourishment needed for growth. It was an emotional experience that included feeding and being able to touch and hold their infant, look closely at the infant, and interact and “bond” with the infant.’ ²²

Whilst it has been important for us as a species to prioritise feeding our infants, it is crucial that we do not allow this instinctual desire to emotionally direct our approach to artificial nutrition for infants and children. It is a misplaced logic to equate breastmilk, formula milk or food being given orally to an infant or child, with providing artificial nutrition, such as in the form of PN via a central venous catheter. As Cranford summarises ‘anyone who believes that eating and drinking in [neurodevelopmentally] normal children are remotely similar to providing a feeding tube for severely brain damaged children has never been present at the bedside of these patients and has no good sense of the medical reality.’ ²³ Whilst the goal of artificial nutrition is ultimately the same (to nourish the child), the processes involved and the lived experience of enteral versus parenteral are contrasts in the extreme. It would be obtuse to follow a purely consequentialist view in this regard by ignoring how distinctly different the two processes of obtaining nutrition are and instead focus purely on their shared end goal of providing nutrition. The process and the lived experience do matter.

The importance of semantics

Instead of equating PN with food, a more accurate parallel for PN is that of a ventilator provided to infants with respiratory failure. Many infants may be started on a ventilator for a variety of pathophysiological reasons. Undoubtedly, when the ventilator is started, there is the hope of recovery and ultimately extubation and survival. However, this is not always possible, and a cohort of infants will become entirely ventilator dependent with no hope of survival independent from the ventilator. In such circumstances, emotionally difficult conversations take place between the clinicians and the parents and, in some situations, it will be deemed that extubation is in the child’s best interests. The ventilator is withdrawn, and the infant is allowed to pass away in their parents’ arms. This is an accepted practice and termed compassionate extubation. The infant would be on medications to ensure they were comfortable (such as morphine given with the intention of relieving any unpleasant sensation from breathlessness). The medical team would be unlikely to regard this process as suffocating the infant to death by removing the endotracheal tube (ETT) – yet, when the withdrawal of PN is discussed, the rebuttal from some clinicians is that this would be starving the child to death ^{24 , 25} – this was the case with several clinicians within our unit in the case of Infant A.

The discordant language used here is important as the inaccuracy and variability in the language used to describe the two scenarios can lead to the masking of the underlying ethical issue. To term, removal of an ETT as compassionate extubation has connotations of a morally good action. In extubating the infant, we are showing compassion and kindness towards the infant. Both qualities are generally deemed morally good traits as they avoid cruelty to another being. However, the use of starvation in the context of withdrawal of PN has immediate emotive connotations of pain, distress and marked cruelty. To starve implies not only the removal of nutrition but also insinuates a significant degree of suffering. Therefore, to semantically dress one situation as morally good and the other as morally bad creates a paradoxical situation where one infant in organ failure whose life has been deemed too burdensome to continue treatment is allowed to have that treatment withdrawn. However, another child with a different but still potentially terminal form of organ failure is to be condemned to a life of treatment that those involved may consider to not be in their best interests. Language should distil, not dictate our ethical code.

Carter et al. support this position, acknowledging that whilst there is the potential for suffering when nutrition is withdrawn, and that ‘such a consideration does have moral weight, … the moral weight is no different than that of the infant for whom the ventilator is withdrawn.’ ²⁶ To continue to use emotive language, such as starving, in the context of withdrawal causes potential confusion as to the true moral issue: if treatment has been deemed not in the patient’s best interest, then that treatment should not be continued. To continue treatment in this context shows disregard for the patient as a living, feeling being in their own right. This does not mean, however, that there is no consideration of the potential suffering that may occur when a specific treatment is withheld or withdrawn. For brevity, it seems acceptable that one can countenance that alleviating suffering where possible is morally good. Therefore, in the same way, a patient having their ETT removed should receive good quality palliative care and medication to keep them comfortable in their final stages, so to, should patients having PN withheld/withdrawn. Carter et al. argue that it is accepted practice to ‘give morphine to take away the suffering attributed to air hunger. Is this any different than providing morphine to take away food hunger?’ ²⁶

There is a practical difference between the withdrawal of a ventilator in respiratory failure and PN withdrawal for gastrointestinal failure and that is predicted time to demise. It would be reasonable to assume that death of the infant will likely occur more rapidly after removal of the endotracheal tube (ETT) then would death after removal of PN provision. This has the potential to cause concern amongst parents and clinicians who may be apprehensive about managing an infant who may have a prolonged deterioration after removal of PN in gastrointestinal failure. However, this is an emotional argument. The morally important element is the alleviation of suffering during the dying process. This is important and good quality palliative care should be provided to all infants who are dying, regardless of the underlying pathology and length of time the dying process may take. After withdrawal of the ETT in infants with respiratory failure, death is likely to occur within a short period of time. However, this is not always the case, and some infants in this situation may live for days after extubation. The length of time the infant remains alive after removal of the ETT does not alter whether or not it was ethically justifiable to remove the ETT in the first place. If ventilation was no longer providing overall benefit to the infant, after accounting for the burden of ventilation and intensive care, then it is ethically justifiable to remove the ETT. Good quality palliative care should be provided throughout the process of the ETT removal and death to ensure the comfort of the infant, for however long the process of dying may take. Similarly, for infants where the benefits of continuation of PN are outweighed by the burden PN provision brings, PN could be ethically withdrawn and for however long the infant subsequently lives, good quality symptom control and palliative care are implemented to ensure the infant is comfortable and not distressed. For example, one of the concerns expressed is often around the infant feeling hunger. However, even when PN is continued, infants on PN often continue to show signs of distress as they wish to suck and enterally feed. ²⁷ Therefore, it cannot be argued that provision of PN alleviates this drive to suck and feed in infants. Rather, the treating team should endeavour to optimise symptom management whether the infant is for active treatment with PN or for palliation and PN removal. In these cases, the team may consider alternatives, such as the use of sham feeds (where the feed is provided but then subsequently removed from the infant’s stomach via nasogastric tube), so the infant is able to feel satiated from the act of feeding, whilst reducing risks of complications from not being able to digest the ingested milk.

Apprehension about the ability to adequately manage discomfort following reorientation of care should not be the singular foundation for justifying continuation of intensive care. In every case, there needs to be a clear benefits and burdens analysis of management options. In order to do this accurately, it is important that those involved are able to distil their cultural values from their moral viewpoint. To insist on providing nutrition via PN with the justification that it is morally wrong to not feed the child seems overly simplistic. The analysis needs to be more specific. Parenteral nutrition solution is a licensed, prescribed medical treatment. As such, there should be a benefits and burdens analysis regarding the provision of PN (as outlined in Table 1), and not an assumption that PN should be provided simply because it fulfils the social expectation that it is a positive thing to feed our infants.

OPEN IN VIEWER

Table 1.

Benefits and burdens analysis as relates to Infant A.

	Benefits	Burdens
Parenteral Nutrition	- Source of nutrition. - Allow growth and development. - Continuation of life allowing growth, interaction and meaningful engagement with family.	- PN is not curative. - Physically attached to PN infusion equipment for long periods on a daily basis. - Central line infections. - Thrombus formations. - Problematic line access. - Poor growth, nutritional deficiencies and the complications these pose. - Secondary liver failure. - PN will not satiate hunger and Infant A would continue to want to suck and feed orally.
Bowel Transplantation	- Continuation of life, allowing growth, interaction and meaningful engagement with family.	- Poor survival rates (approximately 50–55% at five years 5 ). - Life-long immunosuppression with risk of sepsis, rejection and significant impact on daily life.
Withholding/Withdrawing PN	- Avoidance of the negative aspects of PN provision (as outlined above in this table). - Freeing of the family to engage in a shortened but valuable period of family life with their infant, with less hospital based, invasive procedures and monitoring.	- Shortening of life secondary to inadequate nutritional intake. - This would lead to death. This burden is stark, irreversible and consequently given heavy ethical weighting.

Making these decisions concerning withholding and withdrawal of treatment is incredibly difficult and can be divisive. Due to the necessity of having multiple people involved in these discussions, each with their own internal moral value system, decision-making models have been developed to aid parents and medical staff in coming to an appropriate decision on behalf of the infant or child. These will be discussed now.

Just because we can, does not mean we should

Simply because a medical treatment exists, this does not equate to an absolute necessity to provide the treatment to an individual. Medical management should be appropriate and proportionate to the condition and to the individual circumstances. ²⁸ Putting individuals through extensive invasive treatment feels justifiable when there is a reasonable hope of survival with an acceptable quality of life at the end. However, it becomes less justifiable as the odds of survival diminish. Beyond a certain point, intensive care may more closely resemble torture than treatment, and this can create significant moral distress for staff and parents alike. ^{29 , 30} However, exactly where the line between benefit and burden lies will differ from person to person. One of the hardest situations in which to make these types of decisions is surely the field of neonatal and paediatric palliative care, where there is a stark contrast between our hopeful expectations for our children’s long and happy lives, and the reality in these cases that their mortality may be imminent.

Decisions relating to a person’s health and well-being have the potential to have the largest impact on their life. It is therefore increasingly acknowledged that the patient themselves should, where possible, be the final decision maker. The medical team should provide information and outline various management options, but, ultimately, the patient should be able to opt for whichever options most closely aligns with their own priorities, even if this is not the option preferred by the medical team. This goes to the heart of the commonly accepted philosophy of what constitutes a person. Some argue that a ‘person’ is a being capable of consciousness to the extent that it can ‘consider itself as itself in different times and places’ ⁷ (p. 18), and that persons are, ‘capable of valuing their own existence’ ⁷ (p. 19). To be able to do so is to then be able to consider one’s own future and place value on having some say in creating the specifics of that future. Infants evidently lack the cognitive properties to afford them self-awareness; therefore, infants would not be considered moral persons; rather, they may be morally considered to be non-persons. Harris states that ‘of course non-persons can be harmed …. by being subjected to pain for example…’ ⁷ (p. 19). It seems reasonable to accept that infants are morally different from adults due to their more limited cognitive abilities. They do not possess the ability to consider, invest in or deliberate about their own future, but, as non-persons they are still deserving of not being subjected to harm by full moral persons. However, this cleaving into two distinct groups, that of ‘persons’ and ‘non-persons’, seems to overly simplify the nuance of the ethical space in which infants reside.

Infants occupy a unique space due to their potential; they may not yet be considered full moral agents; however, they do usually have the capacity to develop into one. In addition, they are entirely the product of full moral persons, who have their own views and values and would likely be intending to raise and make decisions for their infant within that value system. The current legal distinction between an infant’s rights in-utero (no legal protection) and ex-utero (full legal protection) seems overly simplistic and a compromise to legal precision, not representative of the larger moral situation. With the line between the two beings (infant and parent) so intertwined, potentially a more reasonable ethical position to adopt in relation to medical decision-making is to consider the value system and the moral position of the parent as being representative for the infant. To try and view a parent and an infant as two discrete entities, one possessing of personhood and the other not ignores the physical and almost spiritual connection between the two. It is uneven, difficult ground to attempt to uncouple an infant’s interests from their parents. There is a visceral link between parent and child which starts in the womb, where the infant is created, nurtured and protected by the mother’s body and during this time the interaction from mother to child is established through movements, kicks, voice, touch and a uniquely shared future. This connection is rearranged but continued during and after birth, as the infant remains in close proximity to their parent, in-tune with and entirely reliant upon them.

Much of our dialogue around how to approach decision-making for infants is predicated on a model which considers three distinct agents in the situation: the infant, the parent and the medical team. This model illustrates the construction of extreme individualism that pervades Western culture and ignores the closeness between parent and child: the two are so tightly linked physically and in terms of shared interests that I would argue they could be viewed as one agent, and not two. However, in the current individualist construct, the infant is considered discrete from its parents. It is acknowledged that the parents will have investment and attachment to the child, but they are considered to be individuals separate from the infant. The problem with the individualist approach is that it stems from the rigid idea that significant moral standing is acquired by seeking evidence of structural biological markers. For example, some would regard the fetus as acquiring moral standing when it acquires a heartbeat. This seems arbitrary as the heart is no more essential to the being’s development than other essential organs, such as the brain or lungs. Others may argue it is when the infant is born and emerges from its mother. This again seems tenuous as the timing of birth depends on many factors. Induction of labour, for example, at 38 weeks gestation enables that infant to acquire full legal rights once delivered, compared to another infant at 38 weeks gestation who remains in utero until being delivered at 41 weeks. Biologically, the two are virtually identical in terms of development. Yet bizarrely, geographical location in relation to a vaginal passage is the deciding factor in ascribing rights. Others may argue that full moral standing occurs only when the individual gains self-awareness, usually later, during adolescence. In all of these approaches, the basis for acquiring moral consideration is predicated on individual, often biological, characteristics of the being in question. To insist on a rigid individualistic framework seems to ignore the great value and impact that beings have on those around them. Instead, a relational approach to personhood could be considered more fitting. ³¹

Relational personhood is based on the construct that personhood is acquired gradually within the space of ongoing human relationships, not simple biological criteria, such as the moment of birth. The idea of an infant gradually acquiring personhood seems reasonable as sentience and self-awareness are acquired gradually over the course of childhood. Many other cultures have a relational approach to personhood where infants are regarded as indistinct from the mother in early infancy. The infant and mother are regarded as one unit and the mother takes the name of her infant to symbolise the complete unity between the two. ³² It is the infant’s interaction and sharing of food and bodily fluids, initially breastmilk, that incrementally establishes it within its social community. It is through this interaction with the social community that personhood is obtained. ³² Personhood is not a fixed rite, but a fluctuant, fluid process. This construction of personhood allows for acknowledgement of the growth and development of an independent self that occurs over childhood. It acknowledges the pivotal and unique relationship between parent and child, which is overlooked by more individualist approaches. Infants are not entirely distinct moral persons from their parents, but rather they are entwined initially within their parent’s personhood, and over the course of childhood and the infant’s increasing interactions with the wider community the ties between mother and child are loosened; gradually becoming two discrete moral persons. Until then, infants should be considered a unique moral status; the language of which the ethical community is still navigating ^a . ³³ Infants are simultaneously a constellation of both being a person, by virtue of their interaction with their parents’ personhood, and a non-person, by virtue that they inhabit their own physical body, and so are deserving of care and consideration. The tethering of the infant’s developing personhood to their parents has significant repercussions for medical decision-making. Personhood implies an ability to conceive and value one’s own future, and this concept is acknowledged through the medical ethical tenet of autonomy over decision-making. In the same way, the personhood and autonomy of a mentally competent adult should not be overruled by medical staff, even if the patient’s decision is contrary to medical advice ^b , ³⁴ the shared personhood of infant and parent should also not be overruled, unless there are serious concerns, such as in safeguarding situations.

Making best interests decision

The best interests approach is commonly cited as the paramount concern when determining the course of medical management in relation to a child. It is enshrined in the United Nations Declaration of Human Rights ³⁵ and is adhered to as a guiding principle in English common law. Best interests phrasing aims to embody the ideal that the infant is at the centre of the decision-making process, rather than the agenda of any of the other stakeholders taking the forefront. However, the phrasing is vague and instantly prompts the question of who is to determine what constitutes ‘best’ in the event of disagreement between the various involved parties. The use of the word best implies there is one universal consensus that will be the only truly conceivable management option. However, when different people are given the same philosophical or medical scenario, they may select different options or outcomes for what they would regard as best. When the patient is able to vocalise their preferences then the principle of best interests works well as these can be ascertained and matched to the individual. However, this becomes problematic in the case of infants.

Birchley highlights that ‘Medical law reaches this standpoint [of the best interests approach] through several cases in which children’s best interests were seen to extend beyond medical interests.’ ³⁶ This holistic approach alluded to by Birchley is a vital element that is usually best represented through the parental perspective. This is because parents are able to meaningfully know the family context and value system that the infant was intended to be born into. They were anticipating a new member of their family and were planning on raising that child within their own moral code. An appreciation of the infant’s wider life experience beyond the medical treatments available is important in reaching a robust conclusion regarding the best course of management. Most children will grow and develop into adults with a similar moral code to that of their parents. ³⁷ There will of course be exceptions to this rule. However, generally, the value system of a family extends to the majority of its immediate members. ³⁸ Therefore, it stands to reason that there should be an assumption that the value system of the immediate family is the value system for the infant, until there is concrete proof to the opposite, vocalised as the child ages and becomes a discrete moral person in their own right. Due to the vague nature of the phrase, any other approach to determining best interests immediately places the parent’s moral viewpoint in potential conflict with the physician’s moral viewpoint, with a proportioning that both are of equal weighting. This is a flawed approach. The infant was not intended to be raised by the physician with the physician’s values. Therefore, by what right does the physician presuppose that they are best placed to determine that individual infant’s best interests using their own moral framework. Medical staff will have insight into the medical considerations, such as prognosis, and may have an emotional detachment that the parents lack. Parents may well need time in order to regulate their initial emotional reaction to the information and then be more clearly able to assimilate it. However, emotional investment does not immediately disqualify a parent from being able to arrive at a reasoned decision. Indeed, for want of the medical issue which has caused an interaction with the medical team, the infant should have been born and entered the family home where the infant’s parents would be permitted to make all manner of decisions for their child, with no thought to the preference of the physician who delivered the child. Parental decisions occur daily regarding the quality of food given to the child, the manner the child is spoken to, the aspirations for the child (to be sporty, polite, academic, creative) and, in terms of the medical sphere, how far to impress healthy living on the child and, topically, whether or not the child will be vaccinated.

Medical staff can become convinced that they are better placed to evaluate the infant’s best interests over the conclusion of the parents. Whilst medical staff may have the benefit of detailed information regarding treatment and prognosis, and experience with previous children who have had the same diagnosis, they do not have knowledge of the context of that specific child and the specific values of that family. Medical staff are there to provide information to the family and help them assimilate this into their own context to arrive at the best decision for them. Whilst information regarding prognosis is helpful, each child’s disease course and response to treatment is individual. Statistics do not apply to individuals. In cases where parents have engaged with medical teams, understood and have had time to process the information given to them, they are then able to become the best decision makers for their child, as they have assimilated the factual medical information and applied it to their specific circumstances. This is something medical staff cannot do as they are not part of that family. This was seen in the legal case of T (a minor) ³⁹ where the court decided in favour of the parents, whose decision was at odds with the recommendation of the clinical team. This case centred around an infant with biliary atresia, who had undergone a failed Kasai procedure. The standard next-stage medical management option was a liver transplant. In the case of T, the parents had fully understood, reflected on and assimilated the medical information from the clinical team. However, the parents determined that liver transplantation would be overly burdensome for their child and they instead opted for no medical intervention and for palliative care. The court held that the parents were well informed and loving and the role of the court was not to interfere with the reasonable decision of a parent in this context.

It would be a very different position, however, for cases where parents do not engage in listening to medical facts or have disengaged from the process or have an unshakable belief in an unrealistic outcome (e.g., that their child will make a miraculous recovery). In these cases, there would then be grounds to defer to a third party to make the decision. However, for fully informed parents who understand the information, care for their child and are in a situation where no management option carries an overwhelmingly clear benefit, then the parents’ decision should be adhered to.

Zone of parental discretion

Due to the inherent flaws in the best interests model, an alternative approach has been proposed; that of the zone of parental discretion. Here, the assumption is that parents are best placed to be the ultimate decision-makers, and, unless the medical team feels there are significant concerns of harm intrinsic to the parents' requested plan, then the management plan requested by the parents should be facilitated where possible. Withholding/withdrawal decisions inevitably carry the risk of harm, implicit in their very nature; however, these decisions can still be considered under the zone of parental discretion. The harm threshold implied by the zone of parental discretion test, relates to, in the case of withholding/withdrawal, whether or not the debated management option is conceivable as a possible choice that a concerned and caring parent would countenance, rather than is this the decision that the treating physician feels is preferable. Harm in the context of the zone of parental discretion should be applied where the decision proposed by the parents seems at odds with the conceivable range of outcomes, or where parents have ignored or avoided the medical information provided by the clinicians, or where the parents are felt to be acting in a malicious manner toward their child, in which case legal and safeguarding procedures would be followed.