Forging an easier path through graduation: Improving the patient transition from paediatric to adult critical care

Abstract

Adolescence and the journey to adulthood involves exciting opportunities as well as psychosocial stress for young people growing up. These normal experiences are potentially magnified for teenagers living with chronic illness or disability and their families. Advances in care have improved survival for children with a variety of serious chronic medical conditions such that many who may once have died in childhood now survive well into adulthood with ongoing morbidity. For those with highly complex needs, care is often provided at major paediatric hospitals with expertise, specially trained personnel, and resources to support young people and their families for the first decades of life. At the end of adolescence, however, it is generally appropriate and necessary for young adults and their caregivers to transition to the care of clinicians trained in the care of adults at general hospitals. While there are some well-managed models to support this journey of transition, these are often specific to certain conditions and usually do not involve intensive care. Many patients may encounter considerable challenges during this period. Difficulties may include the loss of established therapeutic relationships, a perception of austerity and reduced amenity in facilities oriented to caring for adult patients, and care by clinicians with less experience with more common paediatric conditions. In addition, there is a risk of potential conflict between clinicians and families regarding goals of care in the event of a critical illness when it occurs in a young adult with major disability and long-term health issues. These challenges present genuine opportunities to better understand the transition from paediatric to adult-based care and to improve processes that assist clinicians who support patients and families as they shift between healthcare settings.

Key Words: Paediatric intensive care; Disability; Adult intensive care; Transition; Critical care

Core Tip: Paediatric patients with serious chronic diseases are now often surviving to adulthood and most will need to transition to adult oriented care facilities. This can be a challenging experience for patients, carers and clinicians. For patients who may require admission to an intensive care unit, better engagement with critical care specialists may improve the process of transition, leading to a better patient experience and improved clinical outcomes.

INTRODUCTION

Improvements in care have enhanced the quality and duration of life for many children with complex chronic conditions[1,2]. Children with conditions that were once considered incompatible with long-term survival are now often living well into adulthood, with an associated need to transition to an acute care system that may be unfamiliar with their background conditions, family circumstances and care needs. For children with straightforward requirements, or where there is a well-established program for transition, few difficulties may be encountered[3]. However, given improved survivorship, many more children with complex care needs now graduate to adult care each year, with potential challenges frequently encountered by them and their families. Some patients will need critical care support in an adult oriented intensive care unit (ICU). Common underlying conditions, patient impact and associated critical care requirements are outlined (Table 1). In many situations, families become highly knowledgeable and develop extensive expertise in the coordinated provision of care. Many such children receive sophisticated levels of support in their home environment that even acute hospital wards with trained staff may find challenging to replicate. Therapies once confined to the hospital setting can now be provided in the home setting, including mechanical ventilation, tracheostomy care and artificial feeding delivered by teams of family members and paid carers supported by hospital-based ambulatory care professionals[4].

Table 1 Disease phenotypes, specific conditions and potential clinical manifestations relevant to intensive care.

Chronic disease phenotypes	Specific diseases	Key manifestations relevant to intensive care
Primary single organ chronic disease	Cystic fibrosis	Respiratory failure, pneumonia, pancreatitis (acute/chronic), liver failure, and distal intestinal obstruction syndrome
	Haemophilia	Life or limb threatening haemorrhage
	Congenital heart disease	Heart failure, post operative care (e.g., shunt upgrade), endocarditis, and single ventricle physiology
	Insulin dependent diabetes mellitus	Diabetic ketoacidosis and cardiovascular complications
	Organ transplantation	Organ failure and opportunistic infection
Complex disability	Cerebral palsy with neurocognitive disability: Perinatal hypoxia, traumatic brain injury, severe infections of the CNS, and neuronal migration disorders (e.g., lissencephaly)	Seizures, pneumonia, post operative monitoring respiratory support, chest wall deformity (scoliosis), abnormal bulbar tone, nutritional deficiency, intellectual disability, and polypharmacy
Rare conditions	Metabolic or mitochondrial conditions (e.g., urea cycle disorders and amino acidopathies)	Hyperammonaemia, neurological problems, metabolic acidosis, organ dysfunction, seizures, intellectual disability, and need for blood purification therapies (e.g., renal replacement therapy)
	Genetic abnormalities (e.g., Duchenne syndrome and Marfan syndrome)	Neuromuscular weakness, progressive heart or respiratory failure, and airway difficulty
	Chromosomal disorders (e.g., Down syndrome)	Pneumonia, immunodeficiency, congenital heart disease, cervical spine instability, and intellectual disability
Technology dependence	Tracheostomy with long term ventilation (e.g., cervical spine injury)	Pneumonia, respiratory failure, and home ventilation
	Long term gastric feeding tube, TPN dependence (e.g., short gut syndrome, bulbar palsy, and neurocognitive disability)	Nutritional deficiency, line related sepsis, and device malfunction

CNS: Central nervous system; TPN: Total parenteral nutrition.

The jarring experience of transitioning to adult hospital care is familiar to many patients and their families. While individual clinicians often work hard to safely bridge potential gaps between the paediatric and adult clinical services, processes are often not sufficient to ensure seamless care. Potential barriers to a patient and family centred graduation process often arise from the somewhat fragmented structure of healthcare as well as resource limitations. However, a lack of familiarity, differences in expectations and knowledge deficits are also important (Table 2).

Table 2 Barriers and enablers to effective hospital and intensive care transition.

Barriers to effective transition	Potential enabler
Lack of knowledge relating to specific conditions and natural history	Medical letter/document summarising the condition, the key long term care components, formal transition meeting, consultation and process
Unknown medical, paediatric history/unavailable medical records	Patient “passport” - summary of care/paediatric history, current primary community clinicians, prior subspecialists, allied health clinicians. Handover process between paediatrician/intensivist to primary care clinician and emergency, ward and intensive care clinicians
No primary carer in hospital	Identify hospital and medical unit for ongoing inpatient care, transition letter with primary outpatient/video appointment introductory consultation
Uncertain acute care management	Emergency care document outlining medical condition, personalised care requirements (e.g., acute management of respiratory infection/failure), important medical record, current medications, care that works/care that doesn’t work
Inadequate facilities and hospital resources	Hospital visits to identify important facilities regarding access, locations, parking, medical supplies, establishing key contacts at hospital

DEVELOPMENTAL PAEDIATRICIAN PERSPECTIVE

Paediatric and adult medicine models of care have many similarities but also some distinct differences. Childhood is the time when young people are growing and gaining new skills. The primacy of growth and development, coupled with the changing role of parents from guardians, advocates and communicators, to observers and guides, are key differences between paediatric and adult medicine. While childhood development often emphasises skills attained in early life, ongoing development occurs on a continuum from infancy to childhood, adolescence and subsequently on to adulthood. Part of the paediatrician’s role is to adapt to these changes and to prepare the young person and family for transition to adult care.

For young people with complex healthcare needs and their families, the transition to adulthood will usually involve leaving a familiar care setting, making this period potentially challenging and stressful. In addition to different roles for parents and carers, expectations regarding case management inevitably change, with adult services usually relying on general practitioners to coordinate most aspects of care outside of hospital[5]. For the majority of adolescents, their emerging adulthood is closely linked to self-advocacy and autonomous decision making. While there is considerable variability in this process amongst healthy adolescents, for young people with chronic illness and disability, this journey can involve additional challenges. Emotional maturity, cognitive capacity, social skills and communication differences may all require additional support for a young person living with disability as they assume responsibility for medical decisions. Additionally, for some young people with reduced cognitive capacity, a parent or other appointed person may need to assist with decision making or maintain a guardianship role well into adulthood.

An important role for doctors is to ensure that patients understand their condition and, as much as is possible, share information relating to the likely long-term prognosis in a way that meets the needs of the patient and family. This is particularly important for conditions in which the underlying pathology is progressive in nature, such as neurodegenerative conditions. Also, while some conditions, such as cerebral palsy, may be considered to be somewhat static with regard to the underlying pathology, important associated comorbidities may be progressive and ultimately life limiting (e.g., progressive restrictive lung disease from scoliosis, or lung injury from recurrent aspiration events). However, the complex interplay of individually specific factors mean that illness trajectories may vary considerably between patients making accurate predictions regarding long term prognosis and quality of life difficult[6]. When a young person with complex needs is deteriorating, decisions about management priorities, quality of life, burden of treatment, and the patient’s experience of treatment are essential considerations that guide discussions regarding what constitutes the most appropriate approach to care[7].

PAEDIATRIC INTENSIVE CARE PERSPECTIVE

Paediatric ICU (PICU) outcomes have improved over time, contributing to an increase in survivors living with pre-existing and acquired disability[8-10]. Long term physical as well as psychological sequelae, may diminish the quality of life for some, and lower educational outcomes can impact the lives of those requiring intensive care as children[11-13]. Changing societal expectations coupled with technological advances now mean therapies that were once confined to the acute care settings, such as ventilation or artificial feeding, may be utilised by patients in their home[11-13].

Children with complex conditions may require intensive care due to lower physiological reserve in the context of acute illness or a need for post-operative critical care support. Paediatric intensive care clinicians aim to achieve recovery to premorbid function but may also identify opportunities for improvement to long term care once the patient is discharged from hospital. In additional to critical care expertise, they understand childhood growth and development, as well as the impact of critical illness, chronic disease and long-term disability. Contemporary PICU management is highly personalised and incorporates comprehensive understanding of the child, their underlying condition and the cause of the current deterioration. For instance, a child with severe cerebral palsy and pneumonia may have a range of abnormalities of tone that requires positional airway management, management of sputum clearance or modification of sedating medication, which are equally as important as supplemental oxygen and non-invasive respiratory support. In addition to intensivists, the clinical team supporting children and families in paediatric hospitals often includes a developmental or general paediatrician, other medical subspecialists, physiotherapists, speech therapists, music therapists, educators, social workers, and a hospital in the home nurse, all of whom may have established relationships with such children and their families. Mutual familiarity, detailed knowledge of past events, familiar surroundings, shared clinical language, and dedicated facilities for carers all contribute to optimising the experience of families during heightened periods of stress. Over time, paediatric intensive care has adapted to care for children with complex needs and their families with specialised inpatient co-ordination and extension of therapies into the community and home environment[14].

When progressive decline in health becomes impactful and therapies are recognised as increasingly burdensome, it becomes important to consider appropriate limitations of treatment. Discussions relating to avoidance of non-beneficial care and end of life themes are often extremely challenging for families and trusting relationships with familiar clinicians are paramount[15]. The paediatric intensivist, paediatrician and family must contemplate the likelihood of recovery, the role of invasive therapies and acknowledgment that death may be approaching. Adult ICUs admit children on the cusp of adolescence with increasing frequency[16]. Critical illness during adolescence may raise additional complex issues such as the approach to graduated autonomy in decision making, consent, and the psychosocial elements of puberty and emerging adulthood. Survey data from health care professionals in the United Kingdom, where adolescents are managed in both adult and PICUs, suggest that staff may lack familiarity with adolescent care, psychological care, consent issues, consideration relating to patient dignity and parental presence[17].

ADULT COMPLEX CARE PHYSICIAN PERSPECTIVE

A small fraction of young adults transitioning from paediatric to adult care are particularly at risk of requiring critical care support. This population often has a mixture of complex medical needs that require input from a range of specialties, physical disability and sometimes cognitive impairment. Patients move from a trusted long term multi-disciplinary paediatric team with careful care co-ordination, to a new team and location. The adult system may be less well-resourced and may not have dedicated care co-ordination[18]. General practitioner care co-ordination can be very helpful, but may not have played a major role during the patient’s childhood if care was managed by a specialist hospital-based clinical team. Transition includes moving from parents as key decision makers to the young adult taking on this role independently or together with parents, when they have capacity. Parents may understandably find it hard to step back from this role and have often provided complex care for their child throughout their life[19].

A well-planned transition process can help address many of these challenges. Developing relationships between paediatric and adult services that have the skills to provide all aspects of care is ideal to minimise the need to attend multiple health services. Offering options to the patient and family, with a face-to-face meeting with the young adult, family, paediatric and adult teams can help to engender trust in the new team. Providing realistic expectations for the young adult and family regards care in the adult environment is important. This usually requires greater self-sufficiency in organising appointments and care, while providing key contacts including assistance for care co-ordination makes for a smoother transition. Future care planning should include supporting life goals such as study and work. Discussions regarding acute goals of care and treatment limitations in adult practice may be more direct and experienced as quite confronting by patients and families. Identifying those at risk of acute deterioration that could result in hospital and/or critical care admission in advance of transition enables the established and trusted paediatric team to have discussions and document goals of care in the event of serious deterioration in future. Preferably discussion and decisions to date should be raised well in advance of transition so that all parties have a shared understanding, and discussions can be continued by the new team. Understanding prognosis and likely outcomes for patients with complex medical issues and physical disability is often difficult and not immediately obvious when someone needs to attend for acute care in emergency situations. Patients with respiratory failure on long term ventilation for neuromuscular conditions can frequently be supported through acute illness and return to live in the community for years to decades[20,21]. Multidisciplinary expert input to decisions regards offering critical care support for this population is important to ensure the treatment offered is beneficial.

ADULT INTENSIVE CARE PERSPECTIVE

While many clinical presentations will be familiar to intensivists working in adult critical care, the younger age, patterns of disability and comprehensive engagement with families may be quite different to patients typically admitted to most adult ICUs[22]. Where programs exist to support the transition of children with complex conditions to adult care facilities, these generally focus on the ambulatory setting, and the potential need for adult intensive care services may be overlooked. As well as losing long-term therapeutic relationships and familiar facilities, newcomers to adult acute care services may find the environment somewhat austere, with fewer support services and less amenity for families.

The impact on individual patients, families and treating clinical teams can be considerable. Few acute care facilities routinely allow family members to stay overnight and most provide limited scope for families to participate in care[23]. Patients and families may lack confidence in clinicians honest enough to profess limited experience in the management of young adults with complex care needs. Efforts to establish trust may be further undermined by well-intentioned but poorly timed attempts to initiate goals of care discussions during the initial presentation to an adult hospital. The routine practice of discussing limitations of treatment and end of life care for highly comorbid older adults with life-threatening presentations is an established part of critical care practice[24]. Clinicians familiar with assessing critically ill older adults with life-limiting disease states may rarely encounter a young adult with long-term complex care needs. During such initial engagements, there is a risk that the clinician may misperceive the situation and seek to engage in premature discussions relating to quality of life, limitations of treatment, and end of life care with a patient and family who have a very different experience of life and expectations relating to healthcare. Unless approached with adroit sensitivity, expertise and experience, these important discussions risk generating distress and distrust for carers of young adults with complex needs[25]. Families may experience such interactions as an ill-informed judgement on the quality and value of the life of a precious family member, who has often successfully returned to live in the community after critical illness in the past[26,27]. Adding further to the complex nature of these dynamic situations, families may sometimes downplay or not perceive disease progression and might avoid acknowledging confronting realities such as impending end of life[28]. Clinicians then encounter challenges when trying to find the best way to provide care to a vulnerable patient with progressive long-term deterioration who has become critically ill, especially where invasive treatments become necessary. Healthcare workers often feel moral distress when required to provide burdensome interventions to a patient who is unable to consent to therapy and exhibits behaviours suggestive of suffering[29].

Critical care clinical areas in major adult hospitals are busy places that may present a confronting environment for young adults and their families when first encountered. While there have more recently been efforts to make emergency departments and ICUs feel more comfortable and “patient friendly”, the layout for most of these clinical spaces reflect past practices where there was less emphasis on the patient’s perspective and experience. Few ICUs have single rooms as the default patient space and privacy is often difficult to preserve. Clinical staff trained in the care of critically ill adults may be unfamiliar with practices such as families remaining constantly at the bedside and contributing to care. Space for families and other caregivers is often limited and overnight facilities or other amenities are frequently meagre or entirely absent.

Clinical teams in adult practice may also be unfamiliar with the underlying condition for some patients, or the nature of associated disabilities and care needs. This may result in the inappropriate conflation of disability with disease states as well as exaggerated perceptions of suffering and inappropriate judgements regarding quality of life[30]. Training and education for clinicians working in adult hospitals may help them differentiate between disability and disease states and to also develop effective communication with families regarding proposed treatment plans and recommendations for situations where progressive deterioration is likely.

FAMILY AND CARER PERSPECTIVE

The period of transition, or graduation for parents and families of adolescents with disabilities and chronic conditions can be stressful; in contrast to other families, parents can see their responsibilities increase, rather than decrease. This is in contrast to other parents, who often gain more freedom during this time[31]. Additionally, many parents during this transition time are the primary carers with responsibilities that include supporting personal care, providing medical treatment, supporting meaningful activities and advocacy roles. There are additional pressures of parents feeling a sense of responsibility to provide a reasonable quality of life for their adult child, with mothers frequently maintaining the role of main carer[32]. These combined experiences may result in the transition or graduation period being associated with a sense of loss of control which cannot be avoided or postponed. Families can feel a loss of support, reduced access to therapy and departure from a familiar environment. Additional to the change in hospital setting, families may be leaving a familiar educational setting, further compounding anxiety and stress[33,34]. These “multiple graduations” occurring simultaneously can cause extreme stress for families, with some suggesting that the transition of adolescence to adulthood having a similar impact to when they received their child’s initial diagnosis[33].

An additional impact is the sense of a loss of a relationship with the paediatrician and nurses. The relationship with the paediatrician has usually been established early in the patient’s life and has often moved beyond just a clinical relationship. Families lose the security of a paediatric ward that can come to feel like home and a team that has become like family. In contrast, families and patients may experience relationships in adult hospitals as lacking patient centred care and feeling more disease focussed[35]. In turn, fears can be exacerbated for families, just through the physical difference in environment and aesthetics. Paediatric hospitals are colourful environments, with toys, games and play therapists. In contrast, adult hospitals have disease focused information on posters, drab colour schemes and limited amenity[36].

A successful transition requires a positive narrative involving both patient and family exploring expectations, empowering autonomy and a sense of collaboration with a key coordinator who manages the process[37]. It is also important to note that the construct of adulthood can look very different for those with intellectual disabilities, and ideas about autonomy and full adult rights may also differ. For those unable to make decisions for themselves, substitute decision makers must assume this role in the patient’s best interests[38]. For many patients, the focus in the new clinical setting is on the young adult making their own health decisions, however for patients with severe intellectual disabilities, parents (and particularly mothers) are making these decisions regarding all stages of transition. In turn, the effectiveness of transition or graduation for young adults with severe intellectual disability is determined by the strength of the parent’s advocacy[33].

CONCLUSION

The changing outcomes of paediatric intensive care, medical progress and the improved societal approach to disability means that more children with complex conditions will grow into adult life. Critical illness and first admission to an adult ICU can be a turbulent experience for young adults, families and clinicians alike because of the lack of familiarity with underlying conditions and response to therapies, loss of long-term clinical relationships, and the different environment of adult health services. Anticipating the need for intensive care and establishing a transition process can ensure better preparation for the unpredictable need for intensive care. Many excellent programs have been established for patients with specific underlying conditions such as cystic fibrosis and congenital heart disorders, however, major gaps exist for patients with other diagnoses, and few generally anticipate potential need for intensive care engagement. Improving the process of transition requires that senior clinical leaders prioritise these issues and implement effective proactive strategies in collaboration with young adult patients and their families. Work has already commenced in places such as the United Kingdom, where the Paediatric Critical Care Society and the Intensive Care Society jointly developed guidance for paediatric to adult critical care transition (available at https://ics.ac.uk/resource/paediatric-to-adult-c-c.html). Many jurisdictions and organisations have overarching recommendations for these processes, however, implementation and impact at an individual patient level is often lacking and a dedicated effort is required to achieve durable change[39].

While many details of transition planning will be unique to a patient’s specific circumstances, key principles are useful to provide a framework in which the individualised planning is organised (Table 3). Important foundations for these guiding principles are the requirement for them to be patient centred, promote timely effective communication and coordinated by appropriately trained clinicians with clearly defined roles. Educational support for carers[40], paediatricians[41] and adult intensive care teams[42] would also likely ensure that these key groups are best prepared to best manage the complexities of transition. Allied health specialists such as psychologists, social workers, and physiotherapists are also crucial in the preparation and hand-over phase of graduation, especially for the most highly complex patients[43]. Families experiencing social deprivation and financial hardship are especially at risk of encountering serious challenges when transitioning to adult services. Specialised services able to coordinate relevant support services play a crucial role in assisting carers negotiate processes that are often complex and disjointed.

Table 3 Optimising the transition from paediatric to adult oriented acute care.

Key principles	Timing	Key considerations
Patient and carer centred approach	From outset	Ensure shared understanding of health-related issues and promote concept of shared responsibilities for complex decision making: Identify possible future need for ICU; where appropriate, include patients and carers in meetings where planning will occur; coordinate transition with other relevant services, e.g., educational programs; and clear outline of expectations and possible arrangements relating to carer presence at bedside during inpatient admissions
Clear outline of responsibilities	Important throughout, but especially during transition process	Best initiated by paediatric service, and appointment of key clinical lead at adult service responsible for ongoing coordination of care
Multidisciplinary	In anticipation of transition and during process of hand-over	Well-coordinated approach by all clinical team members: Specialist medical, intensivist, nursing, allied health, and family doctor
Staged	During years of mid to late adolescence in anticipation of adulthood, consider initiating engagement with adult clinicians at 14-16 years of age	Recognise transition as a process rather than an event: Possible overlapping “shared care” approach during period of transition; orientation visits to new facilities prior to first admission; and avoid admission to adult facilities until transition is complete
Documented: Relevant past history; outline of discussions regarding treatment preferences; and suggested approach to likely clinical problems	Initially developed early during patient’s life and maintained as a useful guide for unplanned emergency presentations as well as during hand-over process at transition to adult system	Must be accessible to relevant clinicians: Secure online “living” document; up to date portable, e.g., hard copy or USB with patient/carers; can include a “patient profile” of care preferences, including tailored approach to managing issues such as communication, anxiety, respiratory support, etc.; outline of consent processes; and advanced care planning
Funding and resources	Well in anticipation of transition process	Any changes in funding or availability of resources are identified and arranged prior to commencing transition process

ICU: Intensive care unit.

For a small but important group of patients, there may be a role for early engagement with palliative care teams within paediatric and adult systems[44]. Palliative care teams can offer much more than intervention during the terminal phase of life and they are likely to provide benefits such as improved psychological health, better symptom control and a lower risk of non-beneficial care in some circumstances[45]. While this approach may help some patients, referral and engagement should ideally occur considerably prior to the initiation of a transition process to avoid potential conflation of end-of-life care with graduation to the adult system.

An important potential barrier to improving transition processes is the need to ensure adequate funding for this important work. Particularly during periods of austerity and budgetary constraints, it can be difficult to obtain financial resources required to pay for such initiatives. However, it is likely that the costs of a well-designed system would be substantially offset by savings achieved through avoidance of hospital admissions, reduced length of stay, and the prevention of complications. In addition, some elements of an improved system can be implemented and minimal or no cost by utilising existing systems in a way that is better coordinated and promotes more effective communication between paediatric and adult clinicians. While additional resources are obviously beneficial to such endeavours, much can likely be achieved through low-cost initiatives that seek better utilisation of existing capabilities. Many organisations already employ disability support experts (or their equivalent) who may be available assist with coordinating communication and documentation of plans.

Ultimately, while it is likely that better coordination of the transition from paediatric to adult care could improve efficiency and resource utilisation, the major underlying imperative is the need to provide improved care for patients and families. There is a strong ethical case for better supporting patients with complex health needs during this process and ensuring that families are provided with appropriate supports during what is often a difficult journey. In addition, working to create better systems and a critical care environment that is more sensitive to patient needs would improve the experience of all who are admitted to the ICU regardless of background circumstances[46].