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Introduction
This BTS Clinical Statement addresses the risk assessment, prevention and management of community-acquired pneumonia (CAP) in people with a learning disability of all ages. Each section is summarised with key clinical practice points. The recommendations made are based on a comprehensive review of the published evidence, where available and pertinent, but are predominantly based on expert opinion aimed at providing useful pragmatic guidance.
Learning disability is the preferred term used in the UK to refer to individuals who have ‘significantly reduced ability to understand new or complex information, to learn new skills’ and a ‘reduced ability to cope independently which starts before adulthood with lasting effects on development’.1 Individuals with learning disability represent a widely heterogeneous group of people and can be associated with a broad range of primary diagnoses and comorbidities.2
There is no definitive record of the number of people with learning disability in England. However, Public Health England (PHE) estimates that in 2015 the population was 1 087 100, including 930 400 adults (approximating 2% of the adult population). The estimated prevalence of learning disability in children and young people (CYP) is 2.5%.3 Far fewer individuals with learning disability were recorded in health and welfare systems. For example, only 324 291 children and adults were identified as having learning disability on general practitioner (GP) practice-based registers in 2021.4 It is likely that those registered have a more severe learning disability or more commonly associated conditions, for example, Down syndrome. This is important, as it means that many individuals with learning disability do not have their diagnosis recognised within health and welfare systems potentially increasing their vulnerability through lack of access to regular health reviews and other relevant public health programmes, for example, vaccination programmes. A series of measures have been introduced by the National Health Service (NHS) to help improve the identification of people with learning disability.4
The life expectancy of people with learning disability is reduced compared with the general population; only 37% of adults with learning disability live beyond 65 years of age compared with 85% of the general population.2
Pneumonia is the most common cause of death among adults with learning disability and is also a common cause of death in CYP with learning disability, with bacterial pneumonia accounting for a significant proportion of these (adults 24%, children 21%).5 A further 16% of adult and 3% of paediatric deaths in the learning disability population are caused by aspiration pneumonia (AP).5
CAP is a major contributor to the increased hospitalisation risk that has been described for people with learning disability and results in longer hospital stays than the general population.6 People with learning disability also experience increased rates of repeated admission secondary to CAP.7
As CAP is a major cause of death in people with learning disability, prevention, early detection and proactive management are key to reducing mortality from avoidable causes.8 It is important that public sector organisations make reasonable adjustments in their approach or provision to ensure that people with learning disability have equitable access to good quality healthcare.9 10
Scope
Learning disability is variously defined. For the purposes of this statement, learning disability is defined in accordance with the UK Department of Health and Social Care definition.1 Learning disability is a term that is widely recognised by health and social care professionals in the UK although it is acknowledged that ‘intellectual disability’ is becoming the more widely accepted term internationally.11
The most common classification of pneumonia is based on where the patient was when the pneumonia began. CAP is the term used to describe an acute infection of the lungs that develops outside the hospital setting in a patient who has not been recently hospitalised. CAP is a heterogeneous disease with multiple potential pathogens including many bacteria and viruses. Microorganisms causing CAP reach the lungs either by inhalation of droplets created by sneezing or coughing from an infected contact or by microaspiration after colonisation of the nasopharynx with a potential pathogen. CAP can also occur as a result of large-volume aspiration (macroaspiration) of oropharyngeal or upper gastrointestinal contents. CAP occurs when there is a defect in normal host defence, a virulent pathogen overwhelms the immune response or there is exposure to a large infectious microbial inoculum. This statement considers all potential CAP aetiologies but does not address CAP secondary to COVID-19 which is covered in detail elsewhere.12
Although learning disability may be an isolated finding, it is commonly associated with other medical conditions or comorbidities.13 Learning disability is commonly identified in individuals with neurodisability.14 Neurodisability describes ‘a group of congenital or acquired long-term conditions that are attributed to impairment of the brain and/or neuromuscular system and create functional limitations. Conditions may vary over time, occur alone or in combination and include a broad range of severity and complexity. The impact may include difficulties with movement, cognition, hearing and vision, communication, emotion, and behaviour’.15 Comprehensive reviews and guidelines for the respiratory management of many specific neurodisability subgroups have already been published.16–19 While this statement does not attempt to replicate their detail, it includes information relevant to this patient group where deemed relevant to CAP.
Annual health checks (AHCs) were introduced for all people 14 years and older with learning disability in an effort to minimise the health inequalities faced by this group.20 The statement group felt that AHCs provide an important opportunity to integrate a focused assessment on risk factors for CAP and respiratory morbidity. Consequently, the statement is structured with an emphasis on risk factor identification and instigation of preventive measures.
This statement applies to people with learning disability across all age ranges. CYP refers to any person under the age of 18.
METHODOLOGY
The clinical statement group (CSG) was chaired by Dr Julian Legg, with membership drawn from experts in respiratory medicine (adult and paediatric), neurology, palliative care, primary care, nursing, speech and language therapy, physiotherapy and geriatrics. Lay/patient input was provided by representatives from NHS England’s LeDeR programme and additional clinical advice was also sought on matters relating to gastroenterology and oral health. The CSG identified key areas requiring Clinical Practice Points and the overall content was developed to reflect the scope approved by the BTS Standards of Care Committee (SOCC). Following discussions of broad statement content, individual sections were drafted by group members. A final edited draft was reviewed by the BTS SOCC before posting for public consultation and peer review on the BTS website June-July 2022. The revised document was re-approved by the BTS SOCC in September 2022 before final publication.
Summary of clinical practice points
General
Healthcare professionals should be able to recognise risk factors for CAP in people with learning disability.
The full assessment of risk factors and the introduction of appropriate preventive measures require a multidisciplinary approach involving physicians, nurses, physiologists, physiotherapists, speech and language therapists, oral hygienists, dentists, dietitians, radiologists and community learning disability teams.
Engagement and assessment
Engaging effectively with the person with learning disability and their parents/carers is fundamental to the early identification of CAP risk factors and the judicious implementation of preventive care and treatment.
Every encounter is an opportunity for healthcare professionals to build a strong foundation to get it right for all future healthcare.
Clinical encounters should be well planned to enable reasonable adjustments to be implemented including adequate clinic space, collaboration between healthcare professionals to minimise visits, additional appointment time and appropriate communication aids.
A detailed history and clinical assessment of respiratory health should be part of every consultation for people with learning disability (notably at each AHC) and should be directed at identifying risk factors for CAP and the need for further investigation.
Identifying risk factors and preventive measures
Lung function testing
Vital capacity (VC) and peak cough flow (PCF) are measures of respiratory muscle strength and should be measured as part of the respiratory assessment in all people with learning disability who have a neuromuscular disorder (NMD) or where there are clinical concerns regarding weak cough or poor secretion clearance.
Patients with NMD who have a VC <60% predicted should have an overnight assessment of breathing.
People with learning disability over 12 years of age who have a PCF <270 L/min should be referred to a specialist respiratory physiotherapist for cough assessment.
Long-term assisted ventilation
As part of the decision to commence long-term assisted ventilation, people with learning disability and their parents/carers should be fully informed regarding the rationale for assisted ventilation, what it is likely to achieve, its limits and associated potential harms and burdens.
Continuous positive airway pressure (CPAP) or non-invasive ventilation (NIV) may reduce the rate of respiratory exacerbations in patients with poor secretion management and frequent episodes of CAP.
Airway clearance
People with learning disability who have repeated episodes of CAP, a reduced PCF or where there are concerns regarding secretion clearance should be referred to a specialist respiratory physiotherapist for cough assessment and, where indicated, introduction of tailored airway clearance techniques (ACTs).
Mechanical insufflation–exsufflation (MI-E) is the treatment of choice in patients with NMD to enhance cough efficacy.
People with learning disability who cannot clear secretions with conventional therapies (breathing exercises, manual techniques and positioning) should be considered for positive expiratory pressure (PEP) devices, MI-E, high-frequency chest wall oscillation or intrapulmonary percussive ventilation.
Physical activity
People with learning disability should be encouraged to be as physically active as possible. Exercise programmes should be individualised and agreed on by a known healthcare professional.
Consideration of prophylactic antibiotics
In patients with frequent episodes of CAP, enteral prophylactic antibiotics may be considered following the optimisation of modifiable risk factors for CAP and appraisal of individual circumstances including frequency of infection, degree of neurological impairment and the patient’s/carers’ informed views.
Azithromycin is the preferred first-line enteral prophylactic agent in the absence of contraindications.
In patients with frequent episodes of CAP and ongoing respiratory tract colonisation with bacteria (notably Pseudomonas aeruginosa), a trial of long-term nebulised antibiotics may be considered. A challenge test should be performed prior to initiation.
Aspiration
Eating, drinking and swallowing difficulties
Eating, drinking and swallowing (EDS) difficulties may be a contributing factor to CAP.
Early involvement of speech and language specialists is essential for all those with a potential history of EDS difficulties.
Gastro-oesophageal reflux disease (GORD)
GORD can be difficult to recognise in people with learning disability and may present with atypical symptoms such as rumination, haematemesis and extensor posturing.
Where GORD is deemed a potential cause of recurrent AP, referral to a specialist service should be considered for further investigation and management.
Fundoplication or jejunal feeding may be considered in carefully selected patients with severe GORD and recurrent episodes of AP where other risk factors have been excluded or optimally treated.
Excessive oral secretions
The identification of posterior drooling as a risk factor for CAP can be challenging, necessitating careful history and examination. Frequent coughing, gagging, choking, ‘wet’/gurgling sounds or daytime anterior drooling that ceases at night may be indicative.
Optimisation of comorbidities and other factors contributing to sialorrhoea may help improve saliva control. These include inadequately controlled GORD, nasal obstruction leading to open-mouthed posture, inadequate postural management, poor dental hygiene and medication side effects.
First-line pharmacological management comprises antimuscarinic drugs (hyoscine hydrobromide, glycopyrronium). Dose titration is necessary to avoid drying secretions excessively since lower airway secretions may become thicker and secretion clearance may become difficult.
If first-line pharmacological management is unsuccessful, salivary gland botulinum A toxin injection and surgical interventions should be considered in problematic sialorrhoea.
Poorly controlled seizures
The diagnosis and management of epilepsy in people with learning disability should be optimised according to national guidance.
Oral health
Oral health should be regularly assessed in people with learning disability and an oral healthcare plan developed, ideally in consultation with the person’s dentist.
Good proactive oral care is essential and is most conveniently achieved by brushing teeth and gums with a soft toothbrush at least twice a day with a fluoride toothpaste using non-foaming toothpaste in those with swallowing difficulties to reduce aspiration risk.
People with learning disability may be reliant on others to provide oral care. This must not be jeopardised if care settings change, for example, on admission to hospital.
Nutritional considerations
An appraisal of nutritional status, including measurement of height and weight, should be a fundamental part of the assessment of people with learning disability.
The diagnosis, investigation and management of nutritional disorders in people with learning disability should be optimised according to national guidance.
Vaccination
Vaccine history should be reviewed at every opportunity.
Annual influenza vaccination is recommended for all people with learning disability.
Pneumococcal vaccination should be considered as specified by the Joint Committee on Vaccination and Immunisation (JCVI) recommendations.
People with learning disability should be considered as high priority in all vaccination programmes for seasonal respiratory infections.
Smoking
Every opportunity should be taken to review smoking and environmental tobacco smoke (ETS) exposure status.
Smoking cessation advice and referral to smoking cessation services should be offered to all people with learning disability who are current smokers and, where significant ETS exposure is identified, to their parents/carers.
Aetiology, diagnosis, investigation and management of community-acquired pneumonia
Early recognition
Early recognition of CAP can be challenging in people with learning disability. Soft signs, such as changes to baseline alertness and mobility, should be viewed with a high index of suspicion.
Diagnosis
A comprehensive history should be sought from the patient, using communication aids when required, with additional information gathering from parents and carers.
The severity of CAP should be assessed to guide treatment and decision-making around requirement for hospital assessment and admission. In adults with learning disability, the CRB-65 or CURB-65 should be used in conjunction with clinical judgement. In CYP, clinical features such as respiratory rate, difficulty in breathing and hydration status can be used to assess severity.
Investigation
A chest radiograph should be performed in all adults requiring hospital assessment with suspected CAP and should be considered in CYP with features of severe or complicated pneumonia.
In CYP with severe CAP and adults with moderate to severe CAP (eg, CURB-65 >2), blood testing and microbiological investigations including sputum culture and sensitivity, blood cultures and pneumococcal urine antigen should be undertaken to help guide treatment.
In all CYP and adults being assessed for suspected CAP, pulse oximetry should be undertaken as it can be a useful adjunct to clinical decisions regarding the site of care and the need for further investigation.
For hospitalised adults with CAP, NEWS2 (or an equivalent early warning score) should be calculated and tracked throughout hospital assessment and admission to monitor for signs of deterioration and to facilitate timely clinical response.
Management
Antibiotic therapy should be guided by clinical severity, known pathogens and allergies.
In CYP with CAP, supplementary oxygen should be administered to maintain oxygen saturations between 93% and 98%.
In adults, target oxygen saturations between 94% and 98% should be used in people where there is no risk of hypercapnic respiratory failure although more conservative oxygen targets can also be considered, in keeping with local guidance. For those with risk factors for hypercapnic respiratory failure (such as scoliosis, neuromuscular disease, chronic obstructive pulmonary disease (COPD) or obesity), oxygen saturations should be maintained between 88% and 92%.
In patients with retention of secretions, early involvement of chest physiotherapy should be considered.
Consideration of risk factors and follow-up
An episode of CAP should trigger a full respiratory assessment including detailed clinical history and examination, review of risk factors and consideration for pertinent investigations.
Follow-up should be arranged for all patients who present with CAP and have been identified as having risk factors so that a holistic assessment of the patient’s needs can be made, and risk factors addressed.
Palliative care considerations
All health professionals should be able to provide high-quality palliative care with support from specialist palliative care teams where necessary.
Palliative care can be delivered alongside active treatment in a parallel planning approach. The two are not mutually exclusive.
Palliative care focuses on quality of life. Developing a personalised plan in advance can document a patient’s usual quality of life, priorities and goal as well as the appropriate escalation and resuscitation plan in the event of deterioration. The patient should be supported to contribute to this as much as they are able along with family or friends who can advocate for them.
Risk factors
Important risk factors have been identified that increase the likelihood of an individual with learning disability developing CAP. These are summarised in table 1. It is important to consider these risk factors whenever a person with learning disability is assessed.
Engagement and assessment
Engagement
Engaging effectively with people with learning disability and their families is key to the early identification of CAP risk factors, implementation of proactive management to mitigate against CAP and delivery of high-quality care and treatment that is tailored to their needs. A recent model for the delivery of equitable care to CYP with learning disability provides a good framework for considering how best to engage with patients with learning disability.21 Central to this is for staff and teams to work in partnership with patients, parents, family members, carers and community partners from the outset to ensure that appropriate reasonable adjustments are made in the context of learning disability to enable comprehensive accurate clinical assessment and appropriate care and treatment.22 This may include accounting for learning disability in family/carers. The principle of making every contact count23 is vital.
Effective communication is key to good engagement. This includes understanding how the person usually communicates and providing information in accessible formats, for example, using easy-read materials. Mencap provide helpful guidance on communicating with people with learning disability.24 Used effectively, hospital passports or ‘All About Me’ documents are useful to enable people with learning disability and families/carers to share information with health staff about their needs and help them make the necessary reasonable adjustments to the care and treatment they provide.25 Where a person with learning disabilities lacks the ability or capacity to engage, involvement remains important and should be carried out using the best-interests principle that ‘any act done for, or any decision made on behalf of a person who lacks capacity must be done, or made, in that person’s best interests’.26
It is important to recognise the legal duty of healthcare services to consider the needs of all people with disabilities in the way they organise their buildings, policies and services as set out in the Equality Act 2010.27 Reasonable steps need to be made to avoid disadvantage arising from any provision, criterion, practice, physical feature or lack of auxiliary aids.
Assessment
Every opportunity should be taken to assess individuals with learning disability for risk factors for CAP (table 1). A thorough history and clinical examination are the cornerstones of assessment. Figure 1 outlines important elements of the assessment and highlights relevant findings which may suggest the need for further investigation.28
Risk factors for CAP in people with learning disability
Clinical assessment. BMI, body mass index.
When undertaking an assessment, it is important to be mindful of the risk of incorrectly attributing symptoms of physical health, such as excessive salivating, to the person’s learning disability diagnosis.5 Known as diagnostic overshadowing, this can result in symptoms being overlooked and prevent access to appropriate and timely healthcare. Maintaining awareness that those with learning disabilities can present differently to the general population is key.5
Assessment in primary care
The primary care team have an important role to play to identify those at increased risk of CAP and offer appropriate interventions and referrals as needed.
Figure 2 highlights key considerations for primary care clinicians when assessing people with learning disability.
Key considerations for primary care clinicians.
Annual health checks
An annual review by the general practitioner, the AHC, was introduced across the UK for all people with learning disability aged over 14 years to address health inequalities faced by people with learning disability. AHCs are associated with a reduction in emergency admissions for conditions such as pneumonia.29 LeDeR data indicate that there is 1.5 times greater likelihood of a person with a learning disability dying if they have not had an AHC in the year prior to their death.30
AHCs are systematic reviews during which new health problems can be identified, management of long-term conditions optimised and preventative interventions offered.31 32 The value of the AHC is greatly enhanced by appropriate preparation for the appointment32 and to improve uptake it is important to consider reasonable adjustments such as longer appointments.22 31 33 34 An easy-read precheck questionnaire sent to the patient or the family, or carer if relevant, can enable the collection of information such as vaccination history and recent dental review findings and can identify priority areas for review such as EDS difficulties.35 36 Electronic templates can be used to support the collection of information.31 33
A full assessment should be completed (see figure 1). AHCs also provide an opportunity for health promotion such as advice on healthy eating or exercise.
At the end of the AHC, a health action plan should be generated35 and held by the person with learning disability to summarise what has been discussed including recommendations (eg, adjustments to medications) and details regarding any referrals. This also provides an opportunity to share health promotion information. It is essential that this information is shared in a format accessible to each individual, for example, easy-read formats.33
Clinical practice points
Engaging effectively with the person with learning disability and their parents/carers is fundamental to the early identification of CAP risk factors and the judicious implementation of preventive care and treatment.
Every encounter is an opportunity for healthcare professionals to build a strong foundation to get it right for all future healthcare.
Clinical encounters should be well planned to enable reasonable adjustments to be implemented including adequate clinic space, collaboration between healthcare professionals to minimise visits, additional appointment time and appropriate communication aids.
A detailed history and clinical assessment of respiratory health should be part of every consultation for people with learning disability (notably at each AHC) and should be directed at identifying risk factors for CAP and the need for further investigation.
Identifying risk factors and preventive measures
While the risk factors for CAP are well described (table 1), there is often little or only weak evidence for the impact of intervention on subsequent CAP. There is, however, broad consensus regarding the contribution of each risk factor to the pathophysiology of CAP and mitigating strategies that can be employed.
This section provides strategies to (1) enable early recognition of CAP risk factors and (2) implement pragmatic proactive management to address each risk factor.
It is essential that an individualised approach is taken when planning assessments and investigations. This includes, for example, knowing how best to explain about different management options, knowing what things are likely to trigger anxiety and what strategies might help, knowing the person’s usual routine and any implications this has for the timing of interventions, as well as knowing what has worked previously and what has not. Part of any assessment should include understanding whether certain personal and environmental factors may lead to behaviours of concern, sometimes described as behaviours that challenge, such as aggression, self-injury or stereotypic behaviours. These may be displayed to generate sensory stimulation, to indicate the need for help or to avoid particular demands being placed on a person with learning disabilities.37 Involving a learning disability nurse, who is skilled, for example, in adapted and augmentative communication, supporting mental capacity assessments, maximising patient involvement and using least restrictive approaches, should be considered.
Figure 3 is a flow chart for the assessment and subsequent management of risk factors for CAP and provides links to the relevant areas within the statement.
Flow chart for the assessment and subsequent management of risk factors for CAP. AP, aspiration pneumonia; BMI, body mass index; CAP, community-acquired pneumonia; FVC, forced vital capacity; NMD, neuromuscular disorder; OSA, obstructive sleep apnoea.
Lung function testing
Spirometry
Spirometry is an important part of the respiratory assessment providing valuable information regarding pulmonary function and helping identify patients who warrant further investigation. A reliable forced expiratory manoeuvre may not be possible due to weakness, poor coordination or insufficient comprehension. In these cases, a slow VC may be obtained by asking the patient to breathe in as deeply as possible and then expire slowly for as long as possible into the spirometer.38 Where the patient cannot achieve a seal around a conventional mouthpiece, a flanged mouthpiece or an appropriately sized facemask held tightly over the nose and mouth can be employed.39 VC can be an effective means of monitoring disease progression and has been shown to strongly correlate with the number of chest infections and days of antibiotic treatment in the preceding year in children with a variety of muscle diseases.40 VC is also predictive of sleep-disordered breathing and hypoventilation in patients with NMD.41 Overnight monitoring of breathing is recommended for patients with NMD that have a VC of <60% predicted.18 VC obtained in the supine position may help to assess diaphragmatic function in patients with NMD.42
Predictive values rely on an accurate standing height which may be unreliable or unobtainable particularly in non-ambulant patients or those with a significant scoliosis. Arm span or ulna length can be used as surrogate markers of height in this context.18
Peak cough flow
Individuals with learning disability may have an impaired cough due to respiratory and bulbar muscle weakness particularly in those with neurodisability. An assisted PCF of <270 L/min is associated with an increased risk of complications from common respiratory infections in adults with NMD.43 This cut-off value is probably also relevant to children >12 years of age although, for younger children, similar risk thresholds have not been established.18 PCF is recommended as part of the assessment of secretion clearance in children over the age of 12 years with neuromuscular weakness18 or where there are clinical concerns regarding cough efficacy. Where PCF cannot be performed but the patient can perform a FVC, formal cough evaluation should be considered for patients with a FVC of <75% predicted as this can potentially indicate the need for interventions to improve cough strength.44
Tests of respiratory muscle strength
Assessment of respiratory muscle strength, including sniff nasal inspiratory pressures (SNIP) and maximum inspiratory pressures (MIP), can provide further valuable data to guide management.45 46 Both SNIP and MIP correlate strongly with VC47–49 and may be helpful in certain patients. For example, patients with bulbar dysfunction may have difficulty creating an adequate seal with a mouthpiece for spirometry but may be able to perform a SNIP manoeuvre.50
Clinical practice points
VC and PCF are measures of respiratory muscle strength and should be measured as part of the respiratory assessment in all people with learning disability who have NMD or where there are clinical concerns regarding weak cough or poor secretion clearance.
Patients with NMD who have a VC <60% predicted should have an overnight assessment of breathing.
People with learning disability over 12 years of age who have a PCF <270 L/min should be referred to a specialist respiratory physiotherapist for cough assessment.
Overnight monitoring of breathing
The overall prevalence of sleep disorders is elevated in people with learning disability51 particularly in those with an associated neurodisability.52–54 Overnight sleep monitoring enables early recognition of hypoventilation and upper airway obstruction.
The relationship between OSA and CAP has not been specifically studied in people with learning disability, but an association is increasingly recognised in other groups.55 56 Similarly, the relationship between hypoventilation and the reduced incidence of CAP following treatment has only been studied in patients with NMD.43 57 The statement group felt that the early recognition of both OSA and hypoventilation was important despite a lack of strong evidence in relation to CAP prevention particularly given the other potential benefits of appropriate treatment.
Indications for overnight sleep monitoring are listed in table 2.
Indications for overnight monitoring of breathing in people with learning disability
Several techniques are available to assess sleep-disordered breathing with varying complexity, degree of patient inconvenience and cost. These include pulse oximetry, oxycapnography, respiratory polygraphy and polysomnography. The choice of which technique is the most appropriate depends on multiple factors including patient compliance and is discussed in detail elsewhere.18 58 59
Long-term assisted ventilation
Assisted ventilation has a useful role in several clinical scenarios. Its principal uses are for the correction of OSA and the treatment of hypoventilation and may also be used as a long-term intervention to prevent atelectasis and segmental collapse where secretion management is poor and respiratory exacerbations are frequent.
NIV refers to non-invasive positive pressure ventilation with two levels of pressure applied during the respiratory cycle, and CPAP refers to the non-invasive application of positive airway pressure at a continuous level.60
The benefits of long-term assisted ventilation are well established in several conditions associated with learning disability, most notably those with NMD. NIV and CPAP can be delivered by several different interfaces including face mask, nasal mask, nasal pillows or mouthpiece. Patients with learning disability may initially struggle to tolerate NIV and CPAP particularly as they may not be able to understand the rationale for treatment despite careful explanation. They may develop a conditioned anxiety associating the sight, sound and sensation with mask discomfort and physiological arousal.61 Non-invasive interfaces delivering both CPAP and NIV can insufflate the stomach and may aggravate an existing vulnerability to reflux aspiration. Improved interface design has made it possible for NIV and CPAP to be well tolerated by most patients. Motivational and behavioural interventions may help improve adherence.61 62
It should be explained what the intervention is likely to achieve, as well as its limits, and any further escalations that may be indicated. It is also important to outline the potential risks and burdens of the intervention including any increased limitations on life at home or reduction in choice about community settings that can support certain technologies. The clinical team already supporting the person with learning disability should be involved in the discussions regarding long-term ventilation and the development of a person-held emergency escalation plan. This is an opportunity to record the patient’s priorities and preferences about this and future interventions and to explore any circumstances under which they may wish to have the intervention withdrawn.
Continuous positive airway pressure
Where OSA is identified, there should be an assessment of any potential causative factors (eg, adenotonsillar hypertrophy, upper airway hypotonia, craniofacial morphology, obesity) and careful appraisal of all treatment options. In CYP, a referral to the ear, nose and throat specialist team should be considered for upper airway evaluation and consideration for surgical management (eg, adenotonsillectomy). A nasopharyngeal airway may be indicated particularly in those unable to tolerate CPAP or with craniofacial abnormalities.
A trial of CPAP is indicated where surgical management is deemed inappropriate (due to patient age, anatomical findings, surgical risk or patient preference) or where OSA persists despite surgery. CPAP maintains patency of the upper airway by overcoming the critical closing pressure of the pharynx.63 Pressure titration is important to optimise CPAP efficacy and minimise unnecessary pressure effects. Regular follow-up is important once CPAP has been established to monitor adherence and efficacy and to optimise equipment/pressure level as necessary.
Non-invasive ventilation
NIV is indicated where there is evidence of hypoventilation. In patients with NMD, night-time NIV has been demonstrated to correct nocturnal hypoventilation and reverse daytime hypercapnia64–66 with some evidence for a reduction in frequency of hospital admission for CAP65 67 68 and an increased life expectancy in Duchenne muscular dystrophy and spinal muscular atrophy.69 70
A wide array of ventilators are available for home ventilation providing multiple different modes of ventilation. The principal aim is to match the ventilator/ventilation mode with each patient to optimise improvements in respiratory pathophysiology while ensuring maximal patient comfort and patient–ventilator synchrony. Clinically stable patients can be effectively and safely commenced on NIV in the home setting.71 Once NIV has been established, overnight sleep monitoring should be performed to ensure hypoventilation has been effectively alleviated. The frequency of subsequent review and sleep monitoring will depend on individual circumstances including age, clinical stability and compliance with NIV. Review should include assessment of NIV efficacy and adherence, evaluation of need for equipment/interface modifications and monitoring for potential adverse effects such as skin breakdown and midface hypoplasia.
Some patients (particularly those with severe weakness) may require daytime NIV due to hypercapnia during wakefulness that does not respond to nocturnal NIV alone. This can be delivered via the same interfaces that are used at night-time or via a mouthpiece. Mouthpiece ventilation enables the patient to define their own ventilatory pattern.72
Tracheostomy/invasive ventilation
Insertion of a tracheostomy may be considered for ongoing care in certain circumstances:
Requirement for prolonged daytime ventilation.
Failure to extubate following a period of invasive ventilation due to an acute deterioration.
Severe bulbar dysfunction resulting in episodes of recurrent aspiration (to facilitate management of secretions).
NIV fails to correct hypoventilation and/or hypoxia.
The decision to proceed to a tracheostomy must be fully informed with clear communication regarding the benefits, but also the burdens. For some having a tracheostomy can be transformative making eating easier, improving social interaction (without the requirement for obstructive facial interfaces) and providing stability in respiratory health. However, for others, the burden of constant intervention (with suction, etc), loss of ability to communicate spontaneously verbally and dependence on trained carers (often with a prolonged stay after insertion while a care package is established) means it can be repressive. Potential tracheostomy complications should also be considered including granuloma and fistula formation, increased secretions, swallowing difficulties and increased respiratory infections.73 Advance care planning can play a key role as part of this decision-making process in encouraging patients and their families to consider and articulate their wishes.
Clinical practice points
As part of the decision to commence long-term assisted ventilation, people with learning disability and their parents/carers should be fully informed regarding the rationale for assisted ventilation, what it is likely to achieve, its limits and associated potential harms and burdens.
CPAP or NIV may reduce the rate of respiratory exacerbations in patients with poor secretion management and frequent episodes of CAP.
Airway clearance
Secretions and cough
Ineffective clearance can result in retained secretions that may cause airways obstruction, atelectasis and segmental collapse and may become infected.
Mucus is transported under normal circumstances from the peripheral airways into the pharynx by cephalad-bias airflow and the mucociliary escalator mechanism. Secretion movement can be enhanced using the following three strategies: increasing lung volumes, increasing expiratory flows and utilisation of collateral ventilation. These are the main principles of ACTs. Once secretions reach the proximal airways they can be cleared with an effective cough.74 An effective cough comprises three components: deep inspiration, rapid closure of the glottis with contraction of abdominal and intercostal (expiratory) muscles75 and explosive decompression on glottic opening.76 If one or more of these components are impaired, the cough will be less effective.77
Assessment and airway clearance techniques
Where there is suspicion of an ineffective cough, a full assessment should be performed.17 This involves assessment of whether: (1) there is a deep breath in without paradoxical movement and (2) there is a good expiratory muscle contraction resulting in an audible rapid expulsion of air.78 Bulbar function can be assessed by asking the patient to repeat e, e, e, e, to determine whether the vocal cords are opening and closing effectively. Cough strength can be measured using PCF (see above).44
People with learning disability, particularly those with associated neurodisability, may have impaired airway clearance for several reasons. All three components of cough can be affected. The inspiratory phase can be impaired due to diffuse muscle weakness and alterations of the mechanical properties of the lungs and chest wall.79 Adequate intrapleural pressures for the rapid expulsion phase may not be possible due to weak accessory muscles of expiration. Glottic closure can be impaired due to bulbar impairment and prevented in patients with a tracheostomy.
Appropriate medical management of secretions should occur prior to instigation of respiratory physiotherapy techniques. Adequate hydration is important to assist mucociliary clearance. If secretions remain tenacious despite hydration, nebulised saline can be effective.80 Excessive secretions may impact the efficacy of ACTs and should also be addressed (see Excessive oral secretions section below).
Respiratory physiotherapy should be targeted to loosening secretions and moving them to the central airways (peripheral ACTs) or clearing from the central airways and therefore enhancing cough (proximal ACTs).44 81 See tables 3 and 4 for a description of techniques that may be beneficial to individuals with learning disability.
Proximal airway clearance techniques
Peripheral airway clearance techniques
People with learning disability may have difficulty understanding the specific manoeuvres required for a proposed technique. The physiotherapist needs to gauge the most effective method for introducing the technique. This may be via demonstration, explanation at their cognitive level or by playing games. In those individuals where distress occurs despite attempts to acclimatise to the technique, choosing a technique that requires no cooperation may be the most effective.
As no one treatment technique fits all, figure 4 highlights treatment options that may be appropriate depending on the severity of learning disability and/or respiratory muscle weakness. A simple technique may often be more appropriate and effective than a complex piece of equipment. Often the physiotherapist will cycle between peripheral ACT and proximal ACT so that secretions are repeatedly moved up into the central airways, coughed and cleared.
Respiratory physiotherapy options for patients with learning disability and retained secretions. Techniques in blue are proximal airway clearance techniques and techniques in red are peripheral airway clearance techniques. Note: *intrapulmonary percussive ventilation (if available) and the *MetaNeb are used in the acute settings.
Provision of a suction machine should be considered where ACT strategies fail or for those who are unable to cooperate with techniques or have a severely weak cough and/or bulbar insufficiency. Depending on the degree of impairment, oral and or nasopharyngeal suction should be taught to parents/carers.17
Provision of respiratory equipment for individuals with learning disability often occurs from hospital-based services. It is therefore essential that there is a close communication between hospital and community services. Where community services offer rapid respiratory assessment to people with learning disability, the provision for a loan device during the time of illness may be useful.82
In certain circumstances, people with learning disability may become overwhelmed with secretions when unwell. This may be due to increased fatigue or increased volume of secretions. Therefore, people with learning disability should have an agreed (with all parties), personalised, airway clearance treatment escalation plan for them to implement when their normal day-to-day regime fails.
Clinical practice points
People with learning disability who have repeated episodes of CAP, a reduced PCF or where there are concerns regarding secretion clearance should be referred to a specialist respiratory physiotherapist for cough assessment and, where indicated, introduction of tailored ACTs.
MI-E is the treatment of choice in patients with NMD to enhance cough efficacy.
People with learning disability who cannot clear secretions with conventional therapies (breathing exercises, manual techniques and positioning) should be considered for PEP devices, MI-E, high-frequency chest wall oscillation or intrapulmonary percussive ventilation.
Physical activity
The health benefits of exercise and its impact on respiratory function are well recognised.83 Exercise increases respiratory flow and has been shown to promote the clearance of respiratory secretions from the peripheral airways.84
People with learning disability have significantly lower physical activity levels than the general population85 86 due to multiple logistic, social and personal barriers.87 Despite a lack of direct evidence of the impact of exercise on the rates of CAP, the statement group agreed that physical activity was an important contributor to respiratory health and should be optimised in people with learning disability.
Community physiotherapists are best placed to provide exercise programmes for individuals with learning disability and can tailor physical activity to the individual taking into account their physical ability. In those who are more mobile, a typical target of >30 min of moderate physical activity five times a week might be advised.
Clinical practice points
People with learning disability should be encouraged to be as physically active as possible. Exercise programmes should be individualised and agreed by a known healthcare professional.
Consideration of prophylactic antibiotics
Currently, there is no direct evidence that prophylactic antibiotics reduce the frequency or severity of CAP in people with learning disability88 although a large randomised trial in children with neurological impairment is currently ongoing.89 However, the statement group agreed that enteral prophylactic antibiotics should be considered in patients with frequent pneumonia, following assessment/management of modifiable risk factors for CAP and appraisal of individual circumstances including frequency of infection (particularly three or more episodes of CAP or two or more hospitalisations per year), degree of neurological impairment and the patient’s/carers’ informed views.16 Some support for this approach is derived from cystic fibrosis (CF) and non-CF bronchiectasis where the use of long-term macrolides appears to be safe and effective.90 91 Azithromycin is the preferred first-line prophylactic agent benefiting from good tissue penetration, broad antibacterial activity, significant anti-inflammatory properties and a long half-life (thus enabling reduced treatment burden through three times a week dosing).92 Appropriate safety precautions should be employed prior to the initiation of treatment including ensuring non-tuberculous Mycobacteria are not present prior to the initiation of treatment and obtaining baseline ECG (to assess QTc interval) and liver function tests in adults.92 Baseline lower respiratory tract infection (LRTI) rate should be established before commencing prophylaxis with efficacy reviewed 6 monthly and consideration of stopping or switching prophylaxis agent if no benefit is demonstrated. Monitoring of cultures and sensitivity from available respiratory specimens may help direct the choice of antibiotic for ongoing prophylaxis.
Chronic pulmonary colonisation including with Gram-negative organisms such as P. aeruginosa should be considered in patients with learning disability who have recurrent LRTIs. P. aeruginosa in chronic airways disease is associated with poorer outcomes.93 While the true incidence of bacterial colonisation in certain learning disability patient groups is debated,16 it is apparent that short-lived oropharyngeal colonisation with bacteria (including Gram-negatives) regularly occurs at the time of viral upper respiratory tract infection.94 However, eradication should be pragmatically considered if there is recurrent isolation of a certain bacteria, particularly P. aeruginosa. In patients with frequent pneumonias and ongoing colonisation, a trial of long-term nebulised antibiotics may be considered.95–97 A lower threshold for this treatment strategy should be employed for those with a tracheostomy who are particularly at the risk of colonisation (P. aeruginosa most commonly).98 Consideration of potential side effects should be made, including that of bronchospasm and a challenge test is advised before initiation.90
For patients with recurrent chest infections, or daily sputum production, investigation for underlying bronchiectasis is recommended.90
Clinical practice points
In patients with frequent episodes of CAP, enteral prophylactic antibiotics may be considered following the optimisation of modifiable risk factors for CAP and appraisal of individual circumstances including frequency of infection, degree of neurological impairment and the patient’s/carers’ informed views.
Azithromycin is the preferred first-line enteral prophylactic agent in the absence of contraindications.
In patients with frequent episodes of CAP and ongoing respiratory tract colonisation with bacteria (notably P. aeruginosa), a trial of long-term nebulised antibiotics may be considered. A challenge test should be performed prior to initiation.
Aspiration pneumonia
Aspiration of upper airway secretions, refluxate, saliva, food or drink can cause CAP. Aspiration pneumonia is covered in detail in the accompanying BTS Aspiration Pneumonia statement.99
The four principal risk factors for aspiration in learning disability are eating, drinking and swallowing difficulties, gastro-oesophageal reflux, excessive oral secretions and poorly controlled seizures.
Eating, drinking and swallowing difficulties
Eating, drinking and swallowing (EDS) difficulties are common among populations who have neurological, muscular, physiological or structural impairment. Swallowing is a complex integrated series of neurological and physiological events. Problems may occur in the mouth, in the pharynx, larynx or in the oesophagus.100
People with learning disability are at increased risk of EDS difficulties although its true prevalence has not been established. Population studies have found 8.1% to 11.5% of adults known to formal learning disability services present with EDS difficulties101 102 although this is likely an underestimation due to diagnostic issues and selection bias.103
The highest rates of EDS difficulties have been found in those with motor impairment,104 increased severity of learning disability105 106 and CP. Approximately 60% of people with CP have EDS difficulties.107 108
EDS difficulties are associated with significant health risks for people with learning disability including pneumonia, chronic lung disease, asphyxia, OSA and hypoxaemia during oral feeding.107 109–112 EDS difficulties also have a significant psychosocial impact with patients feeling ashamed, humiliated and isolated.113
In 2004, the NHS National Patient Safety Agency identified EDS difficulties as a prominent safety issue in people with learning disability.114 The LeDeR annual report highlighted EDS difficulties as one of the most common long-term conditions experienced by people with learning disability with 16% of deaths reported to LeDeR attributable to AP.5
Early involvement of speech and language therapy services is paramount whenever there are concerns regarding swallowing difficulties in people with learning disability.
In these situations, it is important that there is a thorough structured review of medications associated with EDS difficulties, for example, antipsychotics.115 116 The continued need for such medication should be reviewed and reduction of dose or cessation considered. Notably, antipsychotics are prescribed more frequently for people with learning disability than the general population and often inappropriately.117
The parallel BTS Aspiration Pneumonia statement covers the pathogenesis, investigation and management of EDS difficulties in detail.99
Table 5 summarises the assessment, investigation and management of EDS difficulties.
Assessment, investigation and management of eating, drinking and swallowing (EDS) difficulties in people with learning disability
Clinical practice points
EDS difficulties may be a contributing factor to CAP.
Early involvement of speech and language specialists is essential for all those with a potential history of EDS difficulties.
Gastro-oesophageal reflux disease
Gastro-oesophageal reflux is the reflux of gastric contents other than air into or through the oesophagus. Gastro-oesophageal reflux disease (GORD) is defined as a condition in which reflux leads to ‘troublesome symptoms and/or complications’.118 GORD can result in several respiratory conditions, including recurrent AP.119 Respiratory complications of GORD can result from acid, weakly acidic or non-acid reflux.120
GORD has a higher incidence in people with learning disability than the general population. The prevalence of GORD has been reported in between 31% and 75% in children with CP.121–123
It can be difficult to recognise GORD in people with learning disability due to non-specific symptomatology and communication difficulties. Therefore, a high index of suspicion should be maintained when assessing for potential GORD. Typical symptoms such as heartburn, waterbrash and epigastric pain may not be determinable and in those with severe learning disability, clinical features such as rumination, vomiting and haematemesis may predominate.124
Objective measures, including 24-hour multichannel intraluminal impedance with single or dual channel pH measurement and endoscopy with oesophageal biopsy, are recommended for the diagnosis of GORD particularly in patients with neurodisability or where accurate evaluation of a significant response to proton pump inhibitor (PPI) therapy is not possible.125 High-resolution oesophageal manometry can be used for further elucidation of GORD pathophysiology particularly if there is clinical concern regarding possible oesophageal dysmotility.126 Extraoesophageal biomarkers such as lipid-laden macrophages and bronchoalveolar lavage pepsin are not diagnostically helpful.127 In certain groups of patients with high prevalence of GORD (eg, CP), a trial of PPIs with close monitoring of discernible clinical features may be the preferred diagnostic route.
Where GORD is deemed a potential cause of recurrent AP, referral to a specialist service should be considered for further investigation and management.
Table 6 outlines management options for GORD.
Management options for GORD
Clinical practice points
GORD can be difficult to recognise in people with learning disability and may present with atypical symptoms such as rumination, haematemesis and extensor posturing.
Where GORD is deemed a potential cause of recurrent AP, referral to a specialist service should be considered for further investigation and management.
Fundoplication or jejunal feeding may be considered in carefully selected patients with severe GORD and recurrent episodes of AP where other risk factors have been excluded or optimally treated.
Excessive oral secretions
Sialorrhoea is an increase of saliva in the mouth. Sialorrhoea is common in individuals with neurodisability and most often occurs as the result of poor oral motor control and swallowing difficulties in individuals with neuromuscular conditions, rather than because of excess saliva production.
Sialorrhoea can be anterior or posterior. Drooling occurs when sialorrhoea is anterior and saliva spills out of the oral cavity. Posterior drooling is not visible and is more challenging to diagnose; it may accompany anterior drooling but can occur in isolation. In individuals with swallowing difficulties, posterior drooling causes saliva pooling in the oropharynx and hypopharynx. This creates a risk of aspiration and subsequent respiratory disease.
A history of frequent coughing, gagging, choking, chest congestion or ‘wet’ or gurgling sounds may indicate the presence of posterior drooling. Diagnosis of posterior drooling can be challenging; fibre-optic nasopharyngoscopy and laryngoscopy may allow direct visualisation of aspiration of secretions. In most cases, careful history and examination is adequate to guide the need for intervention.
Drooling can be assessed quantitatively with a variety of tools for severity and frequency (eg, drooling quotient, number of bib changes) which can help evaluate response to interventions.128
Management of sialorrhoea
Treatable causes of sialorrhoea include inadequately controlled GORD, nasal obstruction leading to open-mouthed posture, inadequate postural management, poor dental hygiene and medication side effects. If sialorrhoea continues despite optimisation of contributing factors, then specific sialorrhoea interventions should be considered. There is a paucity of evidence for the management of sialorrhoea in adults with learning disability; most of the evidence relates to those with motor neuron disease and Parkinson’s disease. In CYP, sialorrhoea management has been studied most extensively in CP.
Table 7 outlines the most common interventions for sialorrhoea.
Sialorrhoea interventions
Clinical practice points
The identification of posterior drooling as a risk factor for CAP can be challenging, necessitating careful history and examination. Frequent coughing, gagging, choking, ‘wet’/gurgling sounds or daytime anterior drooling that ceases at night may be indicative. Optimisation of comorbidities and other factors contributing to sialorrhoea may help improve saliva control. These include inadequately controlled GORD, nasal obstruction leading to open-mouthed posture, inadequate postural management, poor dental hygiene and medication side effects.
First-line pharmacological management comprises antimuscarinic drugs (hyoscine hydrobromide, glycopyrronium). Dose titration is necessary to avoid drying secretions excessively since lower airway secretions may become thicker and secretion clearance may become difficult.
If first-line pharmacological management is unsuccessful, salivary gland botulinum A toxin injection and surgical interventions should be considered in problematic sialorrhoea.
Poorly controlled seizures
The prevalence of epilepsy in people with learning disability is significantly greater than in the general population (22.2% in a recent meta-analysis).129 Seizures are associated with an increased risk of aspiration, including vomit or saliva.130 The risk of aspiration is further increased by the reduced muscle tone or drowsiness induced by medications used to control or terminate seizures.17 National guidance should be employed for the optimal diagnosis, investigation and management of epilepsy.131
Clinical practice points
The diagnosis, investigation and management of epilepsy in people with learning disability should be optimised according to national guidance.
Oral health
Poor oral care and decaying teeth are risk factors for pneumonia in older people with some evidence in younger age groups.132 133 People with learning disability experience more problems with their oral health than the general population.134
The oral cavity is a complex microenvironment consisting of multiple bacterial and fungal species, their associated biofilms and a cytokine milieu influenced by constant inflammatory stimulation.135 Poor oral health promotes colonisation of the oral cavity with organisms such as Haemophilus influenzae and Klebsiella pneumoniae. It is postulated that microaspiration of these organisms can initiate AP. The risk of AP appears to be greatest when these factors are compounded by chewing and swallowing difficulties.136
In studies of older people and intensive care patients, oral healthcare consisting of tooth brushing at least twice daily and professional oral healthcare once a week reduces the incidence of AP.137 138 Dental care was associated with decreased recurrence of severe pneumonia in a recent cohort study in children with neurological impairment.139
Without further research in this area, and given the relative simplicity and absence of identified risk, the best advice would appear to be to maintain good oral hygiene in people with learning disability to reduce the overall bacterial load. Routine oral care includes brushing the teeth, tongue, palate and gums with a soft brush at least twice a day with a fluoride toothpaste cleaning all tooth surfaces to remove plaque and food. At the end of 2 min brushing, toothpaste should be spat out without rinsing. In patients with EDS difficulties, non-foaming toothpaste should be used to reduce the risk of aspiration of the product.
People with learning disability often rely on others to perform their mouth care, so it is important that carers have the knowledge and skill to manage the person’s oral care. An oral health assessment should be undertaken for each individual and an oral healthcare plan developed, ideally in consultation with the person’s dentist. All children are now encouraged to have their first dental check by 1 year of age and being seen at this early stage helps parents to put preventative measures in place to maintain oral health. Many people with a learning disability can be seen in general dental practice, but community dental services or special care dental services are available to help those who are unable to use general dental services. Accessible information may help the person with learning disability and their carers to maintain good oral health (Ref. 140 provides an excellent source of information with links to additional resources).
Clinical practice points
Oral health should be regularly assessed in people with learning disability and an oral healthcare plan developed, ideally in consultation with the person’s dentist.
Good proactive oral care is essential and is most conveniently achieved by brushing teeth and gums with a soft toothbrush at least twice a day with a fluoride toothpaste using non-foaming toothpaste in those with swallowing difficulties to reduce aspiration risk.
People with learning disability may be reliant on others to provide oral care. This must not be jeopardised if care settings change for example, on admission to hospital.
Nutritional considerations
Data from systematic review demonstrate that being underweight is associated with an increased risk of CAP.141 Contributing factors include alteration to respiratory and diaphragmatic muscle structure and function leading to weakened cough and impaired airway clearance. While systematic review data are inconsistent regarding the relative risk of pneumonia in overweight or obesity,141 142 comorbidities associated with obesity, for example, reduced mobility, OSA and GORD, may negatively impact chest health.143
Underweight and obesity are more common in people with learning disability than in the general population.144–146 Specific learning disability diagnoses are associated with an increased risk of obesity, for example, Down syndrome and Prader-Willi syndrome, whereas diagnoses such as autism can be associated with sensory feeding aspects leading to restricted diet and undernutrition.
Identification of underweight and overweight/obesity should be further assessed and managed by an appropriately skilled multidisciplinary team (MDT), in accordance with current national guidance.147–150
The appropriate frequency of weight and height monitoring should be determined according to individual circumstance and should be included in the AHC. Body mass index (BMI) should be calculated. BMI poorly reflects body composition in individuals with neurological impairment151 152 due to alterations in body composition in some neurodisability conditions, and alternative measures (eg, skinfold thickness) are recommended.125
There is some evidence of efficacy for intensive individualised weight management interventions for overweight adults with learning disability, but these are costly and not widely available.153 More readily accessible interventions (eg, group weight loss programmes) may require the provision of social support, encouragement and role modelling by well-informed carers.153
Management of underweight depends on aetiology and may include optimisation of the mealtime environment and routines, postural management and positioning, optimising parent/caregiver mealtime techniques, use of adaptive equipment, calorie supplementation and enteral feeding intervention.
Clinical practice points
An appraisal of nutritional status, including measurement of height and weight, should be a fundamental part of the assessment of people with learning disability.
The diagnosis, investigation and management of nutritional disorders in people with learning disability should be optimised according to national guidance.
Vaccination
Both influenza and pneumococcal vaccines have been demonstrated to significantly reduce hospitalisation rates for CAP across a wide range of populations.154–156 Evidence suggests that vaccine coverage rates are lower157 and potentially avoidable hospitalisations due to vaccine-preventable pneumonia increased in those with learning disability.158 A patients’ vaccine history should be reviewed at every opportunity.
In the UK, annual influenza vaccination has been available for all people with learning disability since 2014, but this has not led to an appreciable increase in uptake. PHE has provided guidance to support GP surgeries in making necessary arrangements to maximise influenza vaccination of people with learning disability. This guidance also contains a useful resource repository.159 For those individuals with a learning disability with a needle phobia, influenza immunisation using the live-attenuated nasal spray preparation while less effective in adults may be considered as a reasonable adjustment.160
Learning disability is not currently a specific indication for pneumococcal vaccination although this is regularly reviewed by the JCVI.161 Pneumococcal vaccination forms part of the routine childhood immunisation programme (pneumococcal conjugate vaccine-13). Individuals with an incomplete or unreliable vaccine history should be considered unimmunised, and specific guidance is available for appropriate immunisation in this situation.162 The JCVI has defined clinical risk groups where further pneumococcal vaccination (pneumococcal polysaccharide vaccine (PPV)-23) is recommended.163 These include people with chronic respiratory disease including children at the risk of aspiration due to neurological disease. PPV-23 is considered to provide long-term protection with a single dose unless the patient has asplenia, reduced splenic function or chronic renal disease when 5 yearly boosters are recommended.
Consent must be obtained before the administration of any vaccines164 and for people with learning disability there must be an assessment of mental capacity and if necessary, a best interest decision made.26 165 166
Clinical practice points
Vaccine history should be reviewed at every opportunity.
Annual influenza vaccination is recommended for all people with learning disability.
Pneumococcal vaccination should be considered as specified by JCVI recommendations.
People with learning disability should be considered as high priority in all vaccination programmes for seasonal respiratory infections.
Smoking
In adults, tobacco smoking is a recognised risk factor for CAP167 and ETS exposure in childhood is associated with an increased risk of hospitalisation for CAP and increased severity of disease once hospitalised.168 Smoking status should be included in an AHC35 and a smoking/ETS exposure history taken when people with learning disability present with pneumonia.169 Simple screening questions can be employed to identify those children at risk of ETS exposure.170
Smoking cessation advice and referral onto smoking cessation services should be offered to all people with learning disability who are current smokers169 171 and, where significant ETS exposure is identified, to their parents/carers. Reasonable adjustments within smoking cessation services should be considered, including access to longer appointments and providing easy-read information.22
CYP should be encouraged to avoid developing nicotine dependency through the use of vaping, although the authors acknowledge that there is a role for vaping as part of smoking cessation in those who are already established smokers.172
Clinical practice points
Every opportunity should be taken to review smoking and ETS exposure status.
Smoking cessation advice and referral onto smoking cessation services should be offered to all people with learning disability who are current smokers and, where significant ETS exposure is identified, to their parents/carers.
Aetiology, diagnosis, investigation and management of community-acquired pneumonia
CAP is an extremely heterogeneous disease with a wide range of presenting features particularly in people with learning disability. Detailed information gathering from all potential sources (patient, families, carers) is essential to enable accurate diagnosis and optimal management.
This management of CAP in people with learning disability is directed by national guidelines.169 173 174 This section provides a precis of these guidelines highlighting specific considerations for people with learning disability.
Aetiology
In both adults and children with CAP, the most common causative bacteria are Streptococcus pneumoniae and H. influenzae.169 175
In people with learning disability presenting with CAP, further consideration should be made as to whether this pneumonia was triggered by an aspiration event and whether the oral cavity microbiome or colonisation may be contributing.176 The emerging consensus is that community-acquired AP is commonly polymicrobial, and studies have identified a significant role of other causative bacteria such as Gram-negative bacteria (eg, Klebsiella spp, Enterobacter spp and Escherichia coli) and Staphylococcus aureus.177
Viruses may also be the causative agent including influenza A, influenza B and, particularly in children, respiratory syncytial virus.178–180 Bacterial coinfection may occur in the context of a viral pneumonia178 181 with the most common bacterial coinfections including S. aureus and S. pneumoniae.
Early recognition
Early recognition of CAP can be challenging in people with learning disability. A high index of suspicion should be maintained to significant changes from normal that are recognised by parents/carers. Typical symptoms and signs, such as cough and fever, may be absent and more subtle features may predominate including reduction in oral fluid intake, reduced appetite, reduced mobility, altered behaviour or increased somnolence. Tools such as RESTORE2 may be used by carers to help identify soft signs of deterioration in health.182
Clinical practice points
Early recognition of CAP can be challenging in people with learning disability. Soft signs, such as changes to baseline alertness and mobility, should be viewed with a high index of suspicion.
Diagnosis
Symptoms and signs
Symptoms that are typically associated with CAP include cough, history of fever, rapid breathing and breathlessness.169 173 Symptoms vary considerably with age—young children may present with intermittent apnoea and grunting respiration while chest pain may be the predominant symptom in teenagers and adults.169 173 183 A detailed clinical history should include enquiry about swallowing efficiency, recent choking on medication/food/liquids, neurological and gastrointestinal symptoms, the frequency of previous pneumonia and risk factors for CAP.
Typical signs of CAP include increased work of breathing, tachypnoea, fever, dullness to percussion and crackles or bronchial breathing on auscultation.169 173 The incidence and nature of individual signs are age dependent. For example, head bobbing is a sign of increased work of breathing observed specifically in infancy.173
Clinical features of CAP in individuals with learning disability should be appraised with appropriate consideration of individual circumstances and any associated comorbidities. A clinical history should be sought from the patient using communication aids as necessary (see Engagement and assessment section). Parents and carers should be consulted for further detail and may serve as the primary source of information where direct communication with the patient is not possible.
Clinical examination should be adapted to account for associated comorbidities which may impact potential signs of CAP and, if available, details of the patient’s baseline clinical status for comparison can be helpful. For example, scoliosis per se may result in features of CAP including tachypnoea, increased work of breathing and asymmetrical chest findings such as reduced breath sounds on the concave side.184
Assessment should include consideration of the clinical features of risk factors for CAP in people with learning disability (see Engagement and assessment section, table 1).
Assessing severity
CAP severity in CYP with learning disability is determined by clinical assessment of symptoms and signs.173 There are currently no validated severity assessment tools for CAP in children. Table 8 details clinical features that help define the severity of CAP. In addition to assessing severity, decisions regarding admission to hospital and management should take account of any underlying risk factors together with the ability of the parents/carers to manage the illness in the community.
Severity assessment in CYP (adapted with permission from BTS Guidelines for the Management of Community Acquired Pneumonia in Children)173
In adults with learning disability and CAP, the CURB-65 score should be used to assess severity in conjunction with clinical judgement.169 In patients being assessed in the community setting, the CRB-65 score should be used. Although these scoring systems have not been validated specifically for adults with learning disability or neurodisability, they have been validated within the general adult population and demonstrated to be good predictors of mortality in CAP.185 Patients with a low risk score (0–1) have a low risk of mortality and can be managed safely in the community, unless there are alternative reasons for admission to hospital, such as social concerns. Patients with a moderate (2) or high risk (3–5) score should be admitted to hospital for the management of CAP. Further investigation and treatment in adults with CAP will also be guided by their CURB-65/CRB-65 score (figure 5).
CRB-65 score and CURB-65 score. DBP, diastolic blood pressure; SBP, systolic blood pressure.
If the patient is identified at high risk of mortality, or fails to respond to initial treatment, the clinical team must initiate honest conversations with the patient and their family/carers, explaining the possibility of death and exploring their wishes and priorities. Even if full active intervention is being pursued, it is still important to warn loved ones of a possible poor outcome.
Clinical practice points
A comprehensive history should be sought from the patient, using communication aids when required, with additional information gathering from parents and carers.
The severity of CAP should be assessed to guide treatment and decision-making around requirement for hospital assessment and admission. In adults with learning disability, the CRB-65 or CURB-65 should be used in conjunction with clinical judgement. In CYP, clinical features such as respiratory rate, difficulty in breathing and hydration status can be used to assess severity.
Investigation
Radiological investigations
A chest radiograph can potentially provide valuable information in individuals with suspected CAP including the detection of consolidation and effusions. Interpretation may be difficult due to comorbidities such as reduced diaphragm excursion in NMDs, underexpansion of the lungs in thoracic deformity and distortion of anatomical relationships in scoliosis.
In the community setting, it is not necessary to perform a chest radiograph unless the diagnosis is in doubt, there is a lack of clinical progress on treatment for suspected CAP or if the patient is likely to have an underlying pathology.169 173
In adult patients with learning disability, a chest radiograph should be obtained in all patients admitted to hospital.169 A chest radiograph is not considered routine in CYP with learning disability admitted to hospital with CAP173 but should be considered in those with features of severe pneumonia (particularly if hypoxic or with evidence of significant respiratory distress) or where there is clinical suspicion of a complicated pneumonia (absent breath sounds, dull to percussion, decreased chest expansion).
Chest radiography is part of the diagnostic work-up in cases of suspected AP.177
Microbiological investigations
The routine collection of respiratory specimens or blood for microbiological analysis is not indicated in the majority of individuals with CAP.169 173
In children, the collection of specimens for analysis should be considered in those with severe CAP and in those patients with a history of recurrent/frequent CAP or previous infection with methicillin-resistant S. aureus or P. aeruginosa. Specimens should include nasopharyngeal and/or nasal swabs for viral PCR, sputum for culture and blood for culture and serology for respiratory viruses, Mycoplasma and Chlamydia.173 Tracheal suction aspirates should be sent for culture in patients with a tracheostomy. Urinary antigen detection may help as a negative predictor of pneumococcal infection in older children, but positive tests are non-specific and may represent carriage.173
In adults with learning disability, investigation should be guided by their CURB-65 score. Further microbiological investigation is indicated in all patients presenting with CAP and a moderate or high risk CURB score (score of ≥2).169 Investigation should include collection of blood and sputum specimens for culture, prior to initiation of antibiotic therapy where possible, and pneumococcal urine antigen testing. Legionella pneumonia should be looked for in the context of high severity pneumonia, during an outbreak or if the patient has other specific risk factors. In patients with high severity CAP who are not responsive to ß-lactam antibiotics or with a strong clinical, epidemiological or radiological suspicion of ‘atypical’ pneumonia, further investigation should be considered for Mycoplasma pneumoniae, Chlamydia spp and Pneumocystis jirovecii (if risk factors are present). Molecular testing of upper respiratory tract samples should also be considered as part of microbiological investigation.186
In adults presenting with CAP, HIV testing should be considered,187 with CAP a recognised ‘indicator illness’.
General investigations
For patients who are managed in the community, the assessment of oxygenation by pulse oximetry can be a useful adjunct to clinical decisions regarding the site of care and need for further investigation.169 173 It is important to appreciate the potential limitations and technical issues associated with oximetry in certain groups of patients (eg, those with movement disorders).188 Blood investigations are not generally indicated in the community setting.169 173
In hospitalised children with learning disability, white cell count, C reactive protein (CRP) and procalcitonin are not routinely indicated as they are not clinically useful in predicting disease severity or outcome and cannot reliably differentiate viral from bacterial infection.189Pulse oximetry is an essential component of the diagnostic work-up and subsequent management.173
Adults with learning disability being assessed in hospital for CAP should have pulse, blood pressure, temperature, respiratory rate, pulse oximetry and level of consciousness measured and an appropriate early warning score calculated (eg, NEWS2 or an equivalent scoring system) as part of the assessment.190 This is particularly relevant in adult patients with learning disability, where a delay in recognition of clinical deterioration has been identified as a potentially avoidable contributory factor to deaths.5 Full blood count, CRP, urea and electrolytes and liver function tests should also be performed.169 An arterial blood gas or ear lobe blood gas should be performed if the patient is at risk of hypercapnic respiratory failure, including from obesity, kyphoscoliosis, neuromuscular weakness or underlying COPD, and requires supplementary oxygen, in keeping with current national oxygen guidelines.191 If an arterial blood gas is required, then local anaesthetic should be used.191 Patients being admitted should also undergo a venothromboembolism risk assessment, using a validated tool and low-molecular-weight heparin should be prescribed in patients unless the risk is felt to outweigh the benefit.169 192
Clinical practice points
A chest radiograph should be performed in all adults requiring hospital assessment with suspected CAP and should be considered in CYP with features of severe or complicated pneumonia.
In CYP with severe CAP and adults with moderate to severe CAP (eg, CURB-65>2), blood testing and microbiological investigations including sputum culture and sensitivity, blood cultures and pneumococcal urine antigen should be undertaken to help guide treatment.
In all CYP and adults being assessed for suspected CAP, pulse oximetry should be undertaken as it can be a useful adjunct to clinical decisions regarding site of care and need for further investigation.
For hospitalised adults with CAP, NEWS2 (or an equivalent early warning score) should be calculated and tracked throughout hospital assessment and admission to monitor for signs of deterioration and to facilitate timely clinical response.
Management
Antibiotic management
All CYP with learning disability who have a clear clinical diagnosis of pneumonia should receive antibiotics as bacterial and viral pneumonia cannot be reliably distinguished from each other.173 Antibiotics administered orally/enterally are a safe and effective treatment for CYP presenting with even severe CAP.193 Intravenous antibiotics should be used when the patient is unable to tolerate oral/enteral medicines (eg, due to vomiting) or presents with features of septicaemia or complicated pneumonia. Antibiotics should be prescribed according to local and national guidelines.174 Microbiological results may guide alternative antibiotic choice after consultation with local microbiology services.
In adults with learning disability, presenting with CAP to hospital, risk stratification using the CURB-65 in hospital settings or the CRB-65 in community settings should be undertaken194 195 in conjunction with clinical judgement (table 9).
Risk stratification using CURB-65 in hospital settings or CRB-65 in community settings. Should be employed in conjunction with clinical judgement
Antibiotic choice should be guided by clinical severity, known pathogens and allergies. Antibiotics should be initiated as soon as possible, within 4 hours174 of confirming the diagnosis of CAP or within 1 hour if clinical signs of sepsis . If treated with intravenous antibiotics, this should be reviewed within 48 hours and consideration made to switch to oral antibiotics.174 Antibiotics should be stopped after 5 days, unless the patient is clinically unstable or microbiological investigations suggest that a longer course is required.174
The recommendations for treatment of AP in patients with learning disability is that described in the parallel clinical statement ‘Aspiration Pneumonia’.196
Oxygen
All patients admitted to hospital with CAP should have their oxygenation checked.
CYP with learning disability who have been admitted to hospital should be treated with oxygen if their oxygen saturation is <92% in air.173 Oxygen should be administered via nasal cannulae or face mask to maintain oxygen saturation between 93% and 98%. An increasing body of evidence supports the use of high-flow nasal cannula (HFNC) oxygen in cases with significant respiratory distress.197
In adults with learning disability presenting with CAP, oxygen should be prescribed to a target oxygen saturation of 94%–98% unless at risk of hypercapnic respiratory failure.191 Oxygen should be administered in keeping with BTS Emergency Oxygen guidelines.191 More conservative oxygen therapy with lower target saturations is increasingly employed and a target of 94%–96% may be optimal.198 Patients with learning disability may be at high risk of hypercapnic respiratory failure due to underlying COPD, obesity, scoliosis or neuromuscular weakness. In this group of patients, target saturations should be 88%–92%.191
There is a role for HFNC oxygen in adults with learning disability presenting with CAP who have acute hypoxic respiratory failure. HFNC therapy is better tolerated than conventional face mask oxygen.199 The heated humidification is beneficial for mucociliary clearance200 as compared with conventional oxygen therapy which can be drying to the mouth and to respiratory secretions. In acute hypoxic respiratory failure, it has been demonstrated to be both safe and effective.201
Airway clearance
In most cases of pneumonia, lung tissue consolidation exists without excess secretions. In this situation, there is no evidence that respiratory physiotherapy is of benefit.202 The BTS guidelines state patients with pneumonia should not be routinely treated with ACTs.173 194 203 However, where retention of secretions is present or a patient has recurrent excess secretions, ACTs are indicated194 203 and early referral for chest physiotherapy should be considered.
Clinical practice points
Antibiotic therapy should be guided by clinical severity, known pathogens and allergies.
In CYP with CAP, supplementary oxygen should be administered to maintain oxygen saturation between 93% and 98%.
In adults, target oxygen saturations between 94% and 98% should be used in people where there is no risk of hypercapnic respiratory failure. For those with risk factors for hypercapnic respiratory failure (such as scoliosis, neuromuscular disease, COPD or obesity), oxygen saturations should be maintained between 88% and 92%.
In patients with retention of secretions, early involvement of chest physiotherapy should be considered.
Escalation of care
Patients with learning disability admitted to hospital with CAP who have signs of sepsis or acute hypoxic or hypercapnic respiratory failure should have early involvement of critical care services. The LeDeR report5 has highlighted the need for early involvement and a lower threshold for admission to critical care for CYP with learning disability. Although there is little evidence to help guide decision-making for adults with learning disability requiring critical care, patients with learning disability should have the same access to critical care services as other service users.204
Care should be taken to avoid diagnostic overshadowing, defined as ‘symptoms of physical ill health mistakenly attributed to either a mental health/behavioural problem or as being inherent in the person’s learning disabilities’.205 Such diagnostic overshadowing may prevent a clinician recognising a patient’s deterioration or delay initiation of key treatments or discussions with critical care services regarding escalation to HDU or intensive care unit.
It is important to remember that patients with profound or multiple learning disability, at presentation with a severe intercurrent illness, can neurologically deteriorate which can result in clinicians making inappropriate assumptions about the quality of life at baseline. In addition, making a judgement on a patient’s quality of life without full assessment risks providing inadequate, impersonal care. Where a patient is unable to communicate their quality of life, proxies are almost as good at detecting changes in quality of life as the individual.206 Any advance directive (or advance care plan (ACP)) by the patient should also be considered along with previous discussions around appropriateness of escalation between clinicians, the patient and their family. The clinical frailty score should not be used to make escalation decisions for people with learning disability or long-term stable disability as it has not been validated in these groups.12
There are no studies to suggest that patients with learning disability requiring acute ventilatory support for pneumonia have adverse outcomes on critical care compared with other patient groups. Importantly, studies in children with neurodisability requiring paediatric critical care did not show any association between baseline functional status and outcome from critical care admission.207 Decisions should be made on whether intubation and ventilation or other forms of ventilatory support are appropriate according to the trajectory of the patient in conjunction with relevant comorbidities. If a patient, including those with profound or multiple disabilities, has a stable trajectory, then they should not be excluded from access to critical care or from invasive ventilation.208 Other factors that should be considered in decision-making include comordities or significant neuromuscular weakness, which may impact on the appropriateness of invasive ventilation. Discussions around ventilation should involve the patient, if they have the capacity to be involved in decision-making, or proxy where possible.
It is essential that adequate time is given to discuss treatment expectations around escalation. The views of the patient and their advocates (parents/carers) should be fully explored, often as part of advance care planning. This can reduce the risk of significant disagreement which may negatively impact trust between staff and patients and their advocates. Strategies to promote an effective relationship between healthcare professionals and people with learning disability and their advocates include:
Learning disability training for all healthcare staff209 to promote reasonable adjustments and avoid potential discrimination or assumptions about quality of life.
Early identification of patients with a learning disability (eg, automatic alerts on electronic patient records) to enable early referral to learning disability liaison teams and proactive care.
Recognition of the expertise held by parents and carers, and valuing their role as a vital part of the MDT.
Regular transparent MDT discussion to review progress and goals and for care responsibilities to be negotiated and agreed.
In the rare cases where conflict does occur, steps should be taken to maintain effective dialogue between parties. Failure to recognise conflict and resolve it at an early opportunity may lead to court action or public confrontation.210 Offering a second opinion and/or requesting the opinion of a local clinical ethics committee can support the patient, their advocates and health professionals to come to the best decision for the patient. Formal medical mediation may also be valuable.211
Previous LeDeR reports have highlighted the inappropriate use of Do Not Attempt Cardiopulmonary Resuscitation (DNACPR) documents in patients with learning disability, with ‘DNACPR’ forms completed incorrectly with learning disability as the sole reason to not attempt resuscitation.5 Any deliberations around decisions about cardiopulmonary resuscitation must be clearly discussed with the patient or family/carers.212
Clinical practice points
In patients with the signs of sepsis, acute hypoxic or hypercapnic respiratory failure, early involvement of critical care services should be undertaken.
Decisions around escalation to critical care should involve patients, families and carers along with clinical teams who are involved in the delivery of routine clinical care to the patient.
Decisions around escalation of care should take into consideration the trajectory of the patient prior to admission.
The clinical frailty score should not be used as part of decision-making in patients with learning disability presenting with CAP.
Consideration of CAP risk factors and follow-up
An episode of CAP indicates an increased risk of subsequent CAP213 particularly in patients with CP.214 215 It is paramount that the opportunity is taken to make a full respiratory assessment, review the possible contribution of risk factors and arrange follow-up, further investigation and management as necessary. Where a deterioration in an underlying degenerative condition has been identified, the ongoing risk of further episodes should be acknowledged. This is an opportunity for advance care planning to take place to ensure the patient’s wishes for future treatment can be clearly communicated to clinicians responding to a future event.
Follow-up should be organised according to radiological findings, the severity of CAP and need for further evaluation of risk factors.
CYP hospitalised with severe pneumonia, empyema or lung abscess should be followed up after discharge until they have recovered completely and their chest X-ray has returned to near normal.173
For adult patients presenting with CAP, follow-up in a respiratory clinic or primary care is recommended at 6 weeks.169 A follow-up chest radiograph at 6 weeks should be undertaken for those at risk of underlying malignancy such as those with a significant smoking history or those over the age of 50 years.194
All patients who present with CAP and have been identified as having risk factors should have a follow-up arranged so that a holistic assessment of the patient’s needs can be made, and risk factors addressed (see Introduction section).
Clinical practice points
An episode of CAP should trigger a full respiratory assessment including detailed clinical history and examination, review of risk factors and consideration for pertinent investigations.
Follow-up should be arranged for all patients who present with CAP and have been identified as having risk factors so that a holistic assessment of the patient’s needs can be made, and risk factors addressed.
Palliative care considerations
This section may be relevant for patients with learning disability who fit into any of the following categories:
Patients already known to be reaching the end of life who develop pneumonia either as a direct consequence of their underlying condition or due to their increasing frailty.
Patients who may have had multiple episodes of pneumonia, where there is a deteriorating trajectory and there is concern that their quality of life is getting worse.
Patients who have a significant symptom burden or who appear to be sick enough to die because of their pneumonia, even if there is uncertainty and active treatment is still being pursued.
Many of the patients who fit into these groups may subsequently recover partially or completely. In these patients, taking time to consider a palliative approach should not be considered to conflict with active treatment.
Definitions
End of life
Patients are ‘approaching the end of life’ when they are likely to die within the next 12 months. This includes patients whose death is imminent (expected within a few hours or days). In practice, the term ‘end of life’ is interpreted in different ways but usually refers to the final phase of illness.
Palliative care
Palliative care is an approach that improves the quality of life of patients (adults and children) and their families/carers who are facing problems associated with life-threatening illness. It prevents and relieves suffering through the early identification, correct assessment and treatment of pain and other problems, whether physical, psychosocial or spiritual (WHO definition).
Palliative care
Palliative care (encompassing end-of-life care and a period of possibly weeks/months/years earlier) may be useful for patients with advanced progressive illness, even if there is still hope of cure.216 There has been very little research looking at the palliative care needs of those with learning disability.217 218 Early referral to palliative care teams is recommended specifically for those with learning disability.9 217 218
A specialist palliative care team will be able to assess and manage physical, psychological and spiritual symptoms to reduce suffering and distress. They can also offer valuable input into complex clinical decision-making challenges and support the people around the patient. There is a potential role for specialist palliative care teams with expertise in learning disability or additional training in end-of-life care for learning disability nurses.219
The ‘surprise’ question is a simple test when considering whether a patient is likely to benefit from palliative care. Health professionals can ask themselves ‘Would I be surprised if this patient were to die within the next 12 months?’. If the answer is no, this provides recognition that a patient may have transitioned into a new phase of illness.220 Palliative care can be delivered alongside active treatment when appropriate (parallel planning).
Breaking bad news
Breaking bad news to patients with learning disability requires experience and an understanding of their capacity. The ARCH model217 was developed as a specific framework for breaking bad news to people with learning disability, particularly focused on helping carers. It suggests considering four parts: Ask, Repeat and clarify, Check level of understanding, Help person to express feelings.
Breaking bad news should not be avoided because someone has a learning disability. Supporting them in understanding what is happening to them can improve quality of life and reduce anxiety at the end of life.221
Legal aspects in decision-making
All people with a learning disability should be involved in making decisions about their care to the extent they are able. The presumption in treating all patients is that they have capacity to make decisions; however, if this is in doubt, their capacity should be formally assessed following country-specific guidance.222 Mental capacity is both time and decision specific. If the patient does not have capacity, to make a particular treatment decision, the best course of action may be determined by the medical team in conjunction with the patient’s family/friends on a ‘best-interests’ basis. This will consider the patient’s previously expressed values/preferences, and whether anybody else has the legal authority to make decisions on the patient’s behalf.
If the patient previously had capacity, they may have chosen to nominate a trusted family member/friend to make healthcare decisions on their behalf (Lasting Power of Attorney (LPA) for Health and Welfare in England and Wales or Welfare Power of Attorney in Scotland). In the event that the patient loses capacity to make relevant decisions, this grants decision-making power to the elected attorney.
If the patient does not have capacity and has no LPA, or has never had capacity, applications can be made for a deputy (England and Wales) or Guardian (Scotland) to be appointed. However, an appointed deputy may not make decisions to discontinue life-sustaining treatment.
Advance care plans
ACPs are a formal personalised care plan which is patient-held and which includes details about the patient’s condition, discussions and decisions made with them and their family/carers regarding symptom management, appropriate escalation/resuscitation in the event of a life-threatening deterioration, their wishes and ambitions. This plan is a core element of their palliative care. A healthcare directive or ACP may be useful in patients (with mental capacity) to support them in discussing and making decisions about their future health.9 223 224
It is recognised that patients with learning disability are less likely to have had these discussions, and as such, more likely to have a poorly coordinated death which was less likely to have specialist palliative care input.9 More comprehensive ACPs may include details about parallel planning, and this can be important in recognising the unpredictability of life-limiting conditions and making multiple plans of care for different eventualities225 including treatment of future pneumonias. ACPs may also advocate for further active interventions. Advance Decision to Refuse Treatment are legally binding documents which can be tailored by the patient to specify exactly what treatments they would not wish to have in specific situations. These are best completed with legal advice to ensure clarity in documentation.
To support decision-making and weigh up benefits and burdens of treatment, quality of life must be considered. This can only be truly assessed by the individual. It may be that a patient chooses to focus on aspects that are important to their quality of life such that they make choices that may pose further risk to their health (eg, continuing oral diet despite evolving bulbar dysfunction). This may be acceptable if the patient has capacity and has been fully counselled as to the ongoing risks.
Making a judgement on a patient’s quality of life without full assessment risks providing inadequate, impersonal care. Where a patient is unable to communicate their quality of life, proxies are almost as good at detecting changes in quality of life as the individual206 where health professionals may underestimate it.
For those lacking mental capacity, an ACP can still be useful to reflect the preferences and recommendations from the family/friends and clinical teams that know them.
Treatment of infections in end of life
Palliative care is focused on improving the quality of life and weighing up the benefits and burdens of further intervention/treatment.
It is particularly important to review both the benefits and burdens of investigations and treatments when a patient is reaching the end of their life. In the last few days of life for example, it may be that there is no overall benefit in continuing potentially life prolonging but burdensome treatment.212
Continuing intravenous medications, chest physiotherapy or other burdensome treatments when a patient is at an advanced stage may significantly reduce their quality of life, particularly if it limits their choice of preferred place of care. There are some suggestions that continuation of antibiotics (oral or intravenous) may even prolong the dying process and reduce comfort.226 Others hypothesise that development of a serious infection, left untreated, leading to sedation may provide for a peaceful death.227 Conversely, antibiotics in the end of life have also been associated with improved symptom management.228
Ultimately this comes down to communication with the patient (or proxy) to encourage shared and informed decision-making. Ideally, these discussions would have already happened as part of advance care planning.
Clinically assisted nutrition and hydration in end of life
Clinically assisted nutrition and hydration (CANH) may need reassessment at the end of life. Individualised decisions should be made weighing up benefits and burden, and based on the patient’s and family’s understanding, concerns and wishes around food and fluid. As a medical intervention, it is legal/ethical to withdraw/withhold CANH at the end of life. There is clear guidance on the management of this at end of life in those lacking capacity.212 229 In the last few days of life, it is unlikely that CANH will improve symptoms or prolong life but may cause additional discomfort.
Symptom management
Patients with learning disability may not be able to clearly communicate their symptom burden. It is essential that someone who knows the patient is involved in guiding the clinician in assessing symptoms and distressing behaviours. Using a tool like the Disability Distress Assessment Tool can be beneficial in making this assessment.230 There are also pain tools that can be useful in assessing non-verbal patients such as Wong-Baker FACES pain rating scale, Visual Analogue Scale and FLACC.
Symptom management should be tailored to the patient, their comorbidities and symptoms. This can be complex and the benefit of a local palliative care team advising on and anticipating the management of symptoms cannot be underestimated.212 231 Non-pharmacological methods should always be considered alongside pharmacological methods. Clear, honest and open communication can reduce anxiety and symptom burden in end of life. As part of symptom management, it is important to reassess the need for interventions (eg, blood tests) and to consider rationalising the medication burden.
Preferred place of care
Preferred place of care and place of death may have been discussed as part of an ACP. It is important to recognise that where possible patients should be cared for where they are likely to be most comfortable for end-of-life care. Options may include home, hospital, hospice or another residential setting. Although home is often considered to be the most desired place, it may not be right in all situations so other places should be explored. It must be noted that to support patients with learning disability who die in residential care settings may require increased input and support for carers who may not be experienced in end-of-life care.224 Carers in residential settings report that they often feel unheard in decision-making about their clients.224
Bereavement support
Bereavement support is a continuing aspect of palliative care. Hospital bereavement services, GPs and specialist palliative care teams will be able to provide guidance on where the family/friends of the deceased can access this. Consideration must also be given to how to support carers and other residents in residential settings with bereavement, particularly when the person dies in another setting.232 Bereavement distress can be mitigated with clear communication with family/carers—updating them on the patient’s condition particularly through honest discussions when death is anticipated.233 Families also value staff making contact with them after death for example, through a condolence letter/card or offering a follow-up appointment to answer any questions they may have.
Clinical practice points
All health professionals should be able to provide high-quality palliative care with support from specialist palliative care teams where necessary.
Palliative care can be delivered alongside active treatment in a parallel planning approach. The two are not mutually exclusive.
Palliative care focuses on quality of life. Developing a personalised plan in advance can document a patient’s usual quality of life, priorities and goal as well as the appropriate escalation and resuscitation plan in the event of a deterioration. The patient should be supported to contribute to this as much as they are able along with family or friends who can advocate for them.
Acknowledgments
We are grateful to Drs Akshay Batra and Emma Jones, University Hospitals Southampton, for their review of elements of the manuscript relating to gastro-oesophageal reflux. The authors would like to thank Katy Kerr, Health Education England, for her help and guidance relating to the oral health sections of the statement. Professor Simpson is a National Institute for Health Research (NIHR) Senior Investigator. The views expressed in this article are those of the author(s) and not necessarily those of the NIHR or the Department of Health and Social Care
References
Footnotes
Twitter @michellechatwin, @AlisonTavare
Correction notice This article has been corrected since it was published Online First. Supplementary material has been removed as it should not have been included and some minor changes have been made to the text and figures.
Contributors All authors contributed equally to the development of the statement. The lead author had responsibility for final submission.
Funding BTS received funding from NHS England for the development of this statement. NHS England played no part in the scoping or drafting of the document.
Competing interests MC discloses that her clinical practice is at the Royal Brompton Hospital and National Hospital for Neurology and Neurosurgery, University College Hospital. She also works part time developing the education and research programme at Breas Medical.
Provenance and peer review Commissioned; internally peer reviewed.