Recommendations

People have the right to be involved in discussions and make informed decisions about their care, as described in your care.

Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off‑label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

1.1 Service organisation

Access to services

1.1.1 Refer adults with cerebral palsy to a multidisciplinary team experienced in the management of neurological impairments if:

  • their ability to carry out their usual daily activities deteriorates or

  • a neurosurgical or orthopaedic procedure is being considered that may affect their ability to carry out their usual daily activities.

1.1.2 Recognise that reassessment by the multidisciplinary team may be needed by adults with cerebral palsy at different points in their lives to ensure that their changing needs are met (for example, pregnancy and parenting, decreased mobility due to hip arthritis, and loss of care and support from a parent).

1.1.3 Commissioners and service providers should develop pathways that allow adults with cerebral palsy access to a local network of care that includes:

  • advocacy support

  • learning disability services

  • mental health services

  • orthopaedic surgery (and post-surgery rehabilitation)

  • rehabilitation engineering services

  • rehabilitation medicine or specialist neurology services

  • secondary care expertise for managing comorbidities (for example, respiratory, gastrointestinal and urology services)

  • social care

  • specialist therapy services (for example, physiotherapy, occupational therapy, speech and language therapy, and dietetics)

  • wheelchair services.

1.1.4 Ensure that adults with cerebral palsy, their families and carers, and their primary care teams are provided with information about their local network of specialist services.

1.1.5 Explain to the person with cerebral palsy and their family members and carers their right to a care and support needs assessment, in line with the Care Act 2014, and discuss with them the type of support available.

1.1.6 For advice on access to services when adults with cerebral palsy transfer from hospital to home, refer to the NICE guideline on transition between inpatient hospital settings and community or care home settings for adults with social care needs. For the principles of social care provision, see the NICE guideline on people's experience in adult social care services.

1.1.7 Recognise and address any personal barriers to accessing primary and secondary care for adults with cerebral palsy. For example, this may involve:

  • ensuring healthcare professionals have the skills and training to overcome communication difficulties

  • providing treatment and support for mental health problems

  • ensuring that the person has an advocate, if needed

  • providing support to help with social and emotional factors, such as fear of stigma, lack of motivation and exhaustion.

1.1.8 Recognise and address any physical and organisational barriers to accessing primary and secondary care for adults with cerebral palsy. For example, this may involve:

  • improving physical access to buildings

  • providing advice and information on accessible transport services, for example, local community transport services

  • ensuring that appropriate equipment (for example, hoists and wheelchair weighing scales) and adequate changing and toilet facilities are available

  • extending appointment times, if needed.

1.1.9 When an adult with cerebral palsy is admitted to hospital, the staff should always offer advocacy, and health and personal care (including toileting, washing, nutrition and hydration) even if the person has a family member, carer or advocate there to support them.

1.1.10 Give information to people with cerebral palsy about national screening services (for example, breast, colon and cervical cancer screening) and encourage them to attend screening appointments. Explain that screening services will have to make arrangements to provide screening services that are accessible to people with cerebral palsy.

Moving into adults' services

1.1.11 For young adults with cerebral palsy moving from children's to adults' services, ensure that transitions to adult services are managed in line with the NICE guidelines on cerebral palsy in under 25s (see the section on transition to adults' services) and transition from children's to adults' services for young people using health or social care services.

To find out why the committee made recommendations on access to services and moving into adults' services, and how they might affect practice, see rationale and impact.

Ongoing care

1.1.12 Consider regular reviews for adults with cerebral palsy, tailored to their needs and preferences. Agree with the person the frequency of review and which services should be involved based on their needs and preferences.

1.1.13 Offer an annual review of the person's clinical and functional needs, carried out by a healthcare professional with expertise in neurodisabilities, for people with cerebral palsy who have complex needs (such as Gross Motor Function Classification System [GMFCS] levels IV and V) and any of the following:

  • communication difficulties

  • learning disabilities

  • living in long-term care settings

  • living in the community without sufficient practical and social support (for example, being cared for by elderly, frail parents)

  • multiple comorbidities.

1.1.14 Discuss with the person with cerebral palsy (and their family and carers, if agreed) what information should inform the regular or annual review, and who should receive clinical information following review (for example, their GP).

1.1.15 Record details of the person's review and share the information with relevant people (for example, healthcare professionals and social care practitioners), with the person's permission.

1.1.16 Identify who will be the main point of contact for the person with cerebral palsy (and their family and carers, if agreed) between reviews, and provide information on how to contact them.

1.1.17 If an adult with cerebral palsy chooses not to have regular reviews, offer the person (and their family and carers, if agreed) information on when to contact a healthcare professional and how to access the specialist services that they may need. Ensure that the person's GP and multidisciplinary team are aware that they do not want to be reviewed regularly (with the person's permission).

1.1.18 For adults with cerebral palsy and learning disabilities, follow the recommendations in NICE's guideline on care and support of people growing older with learning disabilities.

To find out why the committee made recommendations on ongoing care and how they might affect practice, see rationale and impact.

1.2 Function and participation

Communication

1.2.1 Be aware that speech and communication needs in adults with cerebral palsy may change with time and social circumstances.

1.2.2 At every review, ask adults with cerebral palsy (and their families and carers, if agreed) about any changes in their hearing, speech and communication.

1.2.3 Explore with the person with cerebral palsy who has communication difficulties whether they have a potential need for alternative and augmentative communication systems.

1.2.4 Ensure that training is provided for people with cerebral palsy using alternative and augmentative communication systems and their families, carers and other key communication partners in home, care, social or work environments.

1.2.5 Be aware that adults with cerebral palsy and poor intelligibility of speech may still prefer to use speech as their main means of communication.

1.2.6 Refer adults with cerebral palsy who have communication difficulties to speech and language therapy services to assess their need for intervention, which may include:

To find out why the committee made the recommendations on communication and how they might affect practice, see rationale and impact.

Vocational skills and independent living

1.2.7 Identify and address any factors that prevent people with cerebral palsy from participating in activities, including:

  • physical barriers, for example, access to buildings

  • personal barriers, for example, carers with unmet training needs

  • organisational barriers, for example, policies and situations that put people with cerebral palsy at a disadvantage.

1.2.8 Ask adults with cerebral palsy what they enjoy doing and if they find it difficult to participate in a chosen activity, assess their physical and mental health, and address any factors identified that may be affecting participation, if possible.

1.2.9 If adults with cerebral palsy have complex physical, cognitive, language or sensory needs, consider referral to occupational therapy services to assess the person's functional needs and provide individualised support.

1.2.10 Give adults with cerebral palsy information about assessments of vocational and independent living skills that is tailored to the person's functional abilities and goals (see NICE's guideline on patient experience in adult NHS services for advice on information giving and NICE's guideline on people's experience in adult social care services for details of the information that local authorities should provide).

1.2.11 Refer adults with cerebral palsy who would like to live independently to a professional with expertise in independent living (for example, an occupational therapist). Give information and advice, which could include:

  • adaptations to their home

  • housing

  • leisure activities

  • statutory welfare benefits.

1.2.12 Refer adults with cerebral palsy who would like to work, or who are already working, to a professional with expertise in vocational skills and independent living (for example, an occupational therapist). Give information and advice, which could include:

  • continued education

  • job seeking or access to work schemes

  • employment support to include workplace training and job retention

  • occupational health assessment or workplace assessment

  • statutory welfare benefits

  • supporting a planned exit from the workforce if it becomes too difficult to continue working

  • vocational rehabilitation

  • voluntary work.

    See also NICE's guideline on workplace health: management practices for advice on improving the health and wellbeing of employees.

To find out why the committee made the recommendations on vocational skills and independent living and how they might affect practice, see rationale and impact.

Electronic assistive technology

1.2.13 Discuss with adults with cerebral palsy the potential role of electronic assistive technology if they have problems with participation and independence.

1.2.14 If adults with cerebral palsy have complex physical, cognitive, language or sensory needs, consider referring them to services providing information, assessment and provision of electronic assistive technology.

1.2.15 Be aware that using electronic assistive technology may mean that the person with cerebral palsy needs less contact with their carers, which may reduce their social interaction.

1.2.16 If adults with cerebral palsy are already using electronic assistive technology, discuss at every review any:

  • problems or concerns they have with their equipment

  • potential changes in their needs.

1.2.17 Ensure that training is provided for adults with cerebral palsy using electronic assistive technology, and for their families or carers, if appropriate.

To find out why the committee made the recommendations on electronic assistive technology and how they might affect practice, see rationale and impact.

Physical activity

1.2.18 Discuss with adults with cerebral palsy (and their families or carers, if agreed) the importance of physical activity in maintaining general fitness and physical and mental health.

1.2.19 Provide information on accessible local services that support people with cerebral palsy to take part in physical activity.

1.2.20 Consider referring people with cerebral palsy to services with experience and expertise in neurological impairments that can provide support with physical activities (including sport) and tasks of daily living. Depending on local service provision and the person's needs, this may be to any of the following services:

  • physiotherapy

  • occupational therapy

  • orthotic and functional electronic stimulation services

  • rehabilitation engineering services

  • wheelchair services.

Orthopaedic surgery

1.2.21 If people with cerebral palsy have problems participating in physical activities because of pain or joint problems that do not respond to any other treatments, consider referring them to a musculoskeletal specialist or an orthopaedic surgeon with experience and expertise in managing musculoskeletal problems in adults with cerebral palsy.

To find out why the committee made the recommendations on physical activity and orthopaedic surgery, and how they might affect practice, see rational and impact.

1.3 Managing abnormal muscle tone

Agreeing goals for treatment

1.3.1 Discuss with the adult with cerebral palsy (and their family and carers, if agreed) treatments for spasticity or dystonia, including:

1.3.2 Discuss with the person with cerebral palsy (and their family and carers, if agreed) the balance between the benefits and harms of treating spasticity and dystonia. In particular, explain that some people use their spasticity or dystonia to help their posture and ability to stand, walk or transfer, and that treatment may affect this.

Initial management of spasticity and dystonia

1.3.3 Be aware that adults with cerebral palsy may have both spasticity and dystonia. The severity of symptoms for both conditions may fluctuate in response to health, social and emotional wellbeing, and environmental factors.

1.3.4 At every review, discuss with the person with cerebral palsy (and their family and carers, if agreed) factors that may exacerbate their spasticity or dystonia, such as:

  • bladder problems (for example, urinary tract infection or bladder stones)

  • constipation

  • emotional distress

  • pain

  • posture

  • pressure sores

  • changes in home or work environments, including seating

  • medication changes and side effects.

1.3.5 Before discussing further management options for spasticity or dystonia with a person with cerebral palsy:

  • address any modifiable factors identified that may be exacerbating the spasticity or dystonia and

  • review their physical management programme.

To find out why the committee made the recommendations on agreeing goals for treatment and initial management of spasticity and dystonia, and how they might affect practice, see rationale and impact.

Spasticity

Enteral muscle relaxant drug treatments

1.3.6 Consider enteral baclofen[1] as the first-line drug treatment for adults with cerebral palsy and generalised spasticity causing:

  • functional impairment or

  • pain or

  • spasms.

1.3.7 Start enteral baclofen[1] treatment with a low dose and increase the dose gradually over about 4 weeks to achieve the optimum therapeutic effect.

1.3.8 If enteral baclofen[1] is ineffective or not tolerated by adults with cerebral palsy and generalised spasticity:

  • refer the person to a tone or spasticity management service or

  • discuss other drug treatment options (including other enteral muscle relaxants) with a tone management specialist.

1.3.9 Do not offer diazepam for spasticity in adults with cerebral palsy, except in an acute situation when spasticity is causing severe pain or anxiety.

1.3.10 Do not rapidly withdraw muscle relaxant drugs, particularly if adults with cerebral palsy have taken them for more than 2 months or at a high dosage. Reduce the dosage gradually to avoid withdrawal symptoms.

Botulinum toxin type A injections

1.3.11 Consider referring adults with cerebral palsy for botulinum toxin type A[2] treatment if:

  • they have spasticity in a limited number of muscle groups that is:

    • affecting their care (such as hygiene or dressing) or

    • causing pain or

    • impairing activity and participation, or

  • a tone management specialist agrees that treatment targeted to focal muscle groups is likely to improve their function and symptoms.

To find out why the committee made the recommendations on drug treatments for spasticity, and how they might affect practice, see rationale and impact.

Neurosurgical treatments to reduce spasticity

Intrathecal baclofen

1.3.12 Consider referring adults with cerebral palsy to a tone or spasticity management service offering continuous pump-administered intrathecal baclofen therapy if they still have difficulties with spasticity, despite enteral muscle relaxant drug treatment or botulinum toxin type A treatment.

1.3.13 When considering continuous pump-administered intrathecal baclofen, give the person (and their family and carers, if agreed) information and discuss the procedure with them. This should include:

  • the need for an intrathecal baclofen test to ensure treatment is suitable

  • the surgical procedure for implanting the pump

  • the need for regular hospital follow‑up visits to ensure optimal dosage and pump refill

  • the risks of implanting a pump and pump-related complications (for example, battery failure or catheter leakage), which can result in baclofen withdrawal or overdose

  • a review of 24-hour postural needs.

1.3.14 If continuous pump-administered intrathecal baclofen is being considered for an adult with cerebral palsy, perform an intrathecal baclofen test to assess if it is suitable before implanting a pump. This should involve a test dose or doses of intrathecal baclofen given to the person by lumbar puncture or through a spinal catheter.

1.3.15 Assess the effect of the test dose or doses of intrathecal baclofen on:

  • reducing increased muscle tone

  • reducing pain

  • reducing the frequency of muscle spasms

  • motor function, such as sitting, standing and walking.

1.3.16 Discuss with the adult with cerebral palsy (and their family and carers, if agreed) their views on the response to the intrathecal baclofen test and whether treatment is likely to achieve their treatment goals.

Selective dorsal rhizotomy

1.3.17 Only consider selective dorsal rhizotomy for adults with cerebral palsy and spasticity if other treatments have been unsuccessful or are contraindicated, and after they have been assessed by a multidisciplinary team with:

1.3.18 Discuss the impact of selective dorsal rhizotomy with the person (and their family and carers, if agreed) when it is a proposed treatment option and provide information. This should include:

  • that the procedure cannot be reversed

  • the possible complications

  • the need for prolonged physiotherapy and aftercare

  • the possible impact on function

  • that the long-term benefits are uncertain.

To find out why the committee made the recommendations on neurosurgical treatments to reduce spasticity, and how they might affect practice, see rationale and impact.

Dystonia

1.3.19 Refer adults with cerebral palsy and problematic dystonia (for example, causing problems with function, pain or participation) to a tone or spasticity management service to consider treatment options.

Enteral anti-dystonic drug treatments

1.3.20 Do not prescribe levodopa[3] to manage dystonia in adults with cerebral palsy, except in the rare situation when it is used as a therapeutic trial to identify dopa-responsive dystonia.

1.3.21 Do not rapidly withdraw enteral drugs[4] for treating dystonia, particularly if adults with cerebral palsy have taken them for more than 2 months or at a high dosage. Reduce the dosage gradually to avoid withdrawal symptoms.

Botulinum toxin type A injections

1.3.22 Only consider botulinum toxin type A[5] treatment for focal dystonia in adults with cerebral palsy when:

  • the person is under the supervision of a tone or spasticity management service, and it is part of a wider programme of therapy and

  • focal dystonia is:

    • affecting their care (such as hygiene or dressing) or

    • causing pain or

    • impairing activity and participation.

1.3.23 If botulinum toxin type A[5] is a proposed treatment option, take into account and explain to the adult with cerebral palsy (and their family and carers, if agreed):

  • that the severity and pattern of dystonia may change after treatment and

  • the potential impact of treatment on function.

To find out why the committee made the recommendations on drug treatments for dystonia, and how they might affect practice, see rationale and impact.

Neurosurgical treatment to reduce dystonia

Intrathecal baclofen

1.3.24 Only consider continuous pump-administered intrathecal baclofen[6] if people with cerebral palsy still have difficulties with dystonia, despite having enteral anti-dystonic drug treatment or botulinum toxin type A[5] treatment. Provide information and discuss the procedure, including intrathecal baclofen testing, with the person (and their family or carer, if agreed) as described in recommendations 1.3.13 to 1.3.16.

Deep brain stimulation

1.3.25 If adults with cerebral palsy continue to have severe and painful dystonia, despite having enteral anti-dystonic drug treatment or botulinum toxin type A treatment, consider referring them to a specialised centre with experience in providing deep brain stimulation. See also NICE interventional procedures guidance on deep brain stimulation for tremor and dystonia (excluding Parkinson's diseas).

To find out why the committee made the recommendations on neurosurgical treatments to reduce dystonia, and how they might affect practice, see rationale and impact.

1.4 Assessment and monitoring of clinical complications and comorbidities

Bone and joint disorders

1.4.1 Discuss with adults with cerebral palsy (and their families or carers, if agreed) that:

  • their musculoskeletal function may deteriorate gradually, and any changes should be investigated to identify treatable causes

  • early recognition of bone and joint disorders enables early treatment, which may improve outcomes.

Osteoporosis and fracture risk

1.4.2 Be aware that low bone mineral density is common in adults with cerebral palsy, particularly in people:

  • with reduced mobility or reduced weight bearing

  • taking anticonvulsants or proton pump inhibitors

  • who have had a previous low-impact fracture.

1.4.3 Consider assessing for risk of fractures secondary to osteoporosis in adults with cerebral palsy. Risk factors to assess include:

  • needing help with moving or having to be moved, for example, hoisting

  • history of falls

  • low BMI

  • history of low-impact fractures

  • other medical factors, for example steroid use, that may adversely affect bone health.

    For more information about assessment of fracture risk, see NICE's guideline on osteoporosis: assessing the risk of fragility fracture.

1.4.4 Consider a dual-energy X‑ray absorptiometry (DXA) assessment in adults with cerebral palsy who have 2 or more risk factors (see recommendation 1.4.3), particularly if they have had a previous low-impact fracture.

1.4.5 Consider referring adults with cerebral palsy for specialist assessment and management, for example, to a rheumatology, endocrinology or bone health service, if they have:

  • a high fracture risk or

  • a positive DXA result.

Disorders caused by abnormal musculoskeletal development

1.4.6 Be aware that, because of abnormal musculoskeletal development, adults with cerebral palsy are more likely to have bone and joint disorders.

1.4.7 Refer adults with cerebral palsy to a specialist orthopaedic or musculoskeletal service if a bone or joint disorder is suspected and causing pain or affecting posture or function. These may include:

  • osteoarthritis

  • cervical instability or spondylosis (including athetosis)

  • spinal deformity (including scoliosis, kyphosis and lordosis)

  • subluxation of the hips, wrist and shoulders

  • biomechanical knee problems

  • abnormalities of the foot structure.

1.4.8 Do not offer an X‑ray to assess for hip subluxation or curvature of the spine in adults with cerebral palsy, unless the person is in pain or their posture or function is affected.

To find out why the committee made the recommendations on bone and joint disorders, and how they might affect practice, see rationale and impact.

Mental health problems

1.4.9 Identify and address mental health problems alongside physical health problems. Recognise that the impact of mental health problems and emotional difficulties can be as important as physical health problems for adults with cerebral palsy.

1.4.10 Follow NICE guidelines on identifying and managing specific mental health problems, and psychological and neurodevelopmental disorders, in adults who have cerebral palsy, for example:

1.4.11 Tailor the identification and assessment of mental health problems and emotional difficulties to the needs and abilities of the person with cerebral palsy, in particular take into account communication difficulties or learning disabilities.

1.4.12 At every review, explore with the adult with cerebral palsy (and their family and carers, if agreed) if they have any concerns about, for example, their:

  • mood

  • irritability

  • behaviour

  • social interaction

  • sleep

  • general level of function.

1.4.13 Involve families and carers, when agreed, in identifying and assessing mental health problems and emotional difficulties in adults with cerebral palsy.

1.4.14 Take into account the specific factors that might affect identifying, assessing and managing mental health problems and emotional difficulties in people with cerebral palsy. These may include:

  • adverse effects of medicines (including the effects of medicines used for managing mental health problems on motor function or those used for managing motor function on mental health)

  • communication difficulties

  • learning disabilities

  • impaired neuropsychological and executive functions

  • comorbidities, particularly epilepsy and pain

  • side effects and drug interactions of multiple medicines (polypharmacy).

1.4.15 Discuss with the adult with cerebral palsy (and their family and carers, if agreed) if physical problems, such as pain, or frustration from communication difficulties or lack of stimulation are contributing to emotional distress or challenging behaviour.

To find out why the committee made the recommendations on mental health problems, and how they might affect practice, see rationale and impact.

Difficulties with eating and nutritional problems

1.4.16 Offer adults with cerebral palsy a regular weight check, and BMI or another anthropometric measurement, and identify people who may be at risk of undernutrition or obesity (see also the NICE guideline on obesity: identification, assessment and management).

1.4.17 At every review, discuss with adults with cerebral palsy (and their family and carers, if agreed) whether they have difficulties with eating or if there have been any changes in their eating habits or bowel function. Ask about issues including:

  • changes in appetite

  • changes in the person's ability to feed themselves

  • constipation

  • coughing or choking when eating

  • food refusal

  • increased frequency of chest infections (which may be related to swallowing difficulties or gastro-oesophageal reflux)

  • an increase in the length of meal times.

1.4.18 Discuss if any of the following factors might be affecting the person's appetite, eating habits or weight:

  • changes in carer support

  • depression

  • pain

  • reduced physical activity

  • side effects of medications.

1.4.19 Be aware that adults with cerebral palsy with severe spasticity and dyskinesia may have an increased metabolic rate and a higher risk of malnutrition.

1.4.20 Follow the recommendations on screening for malnutrition, indications for nutrition support, and education and training of staff and carers related to nutrition, in NICE's guideline on nutrition support for adults.

1.4.21 Refer adults with cerebral palsy to a relevant clinical specialist, such as a dietitian or speech and language therapist, if assessment suggests they have difficulties with eating or malnutrition.

To find out why the committee made the recommendations on difficulties with eating and nutritional problems, and how they might affect practice, see rationale and impact.

Respiratory disorders

Identifying and monitoring respiratory disorders

1.4.22 Be aware that adults with cerebral palsy are at increased risk of respiratory failure. Symptoms may include:

  • breathlessness

  • changes in behaviour (such as irritability or inability to concentrate)

  • daytime drowsiness

  • worsening epilepsy

  • headaches on waking

  • increasing frequency of chest infections

  • poor sleep pattern

  • sleep apnoea.

1.4.23 Recognise that some risk factors for respiratory impairment are more common in adults with severe cerebral palsy (GMFCS level IV or V), such as:

  • aspiration pneumonia

  • chronic cardiorespiratory disorders (for example, cor pulmonale or pulmonary circulation hypertension)

  • chronic suppurative lung disease

  • kyphoscoliosis

  • poor saliva control

  • recurrent chest infections.

1.4.24 Refer adults with cerebral palsy and persistent or multiple signs and symptoms of respiratory impairment, or risk factors for respiratory impairment (see recommendations 1.4.22 and 1.4.23) to specialist services.

1.4.25 Consider assessment with spirometry for adults with cerebral palsy who are suspected to be at high risk of respiratory impairment.

To find out why the committee made the recommendations on identifying and monitoring respiratory disorders, and how they might affect practice, see rationale and impact.

Prophylactic treatments for respiratory infections

1.4.26 Offer vaccinations to adults with cerebral palsy and their carers, in line with the national immunisation programme (see the Green Book for further information).

1.4.27 Do not offer prophylactic antibiotics for lower respiratory tract infections in adults with cerebral palsy, unless:

  • the person is at high risk of respiratory impairment (see recommendation 1.4.23) and

  • they are offered on the advice of a respiratory specialist with expertise in neurodisability management.

1.4.28 If an adult with cerebral palsy is at high risk of lower respiratory tract infection, consider a prophylactic physiotherapy chest care review. This may include:

  • postural management

  • advice on exercise

  • advice on training and care for family members and carers.

1.4.29 Refer adults with cerebral palsy and recurrent chest infections, if dysphagia is suspected, to a speech and language therapist with training in dysphagia to assess swallowing.

To find out why the committee made the recommendations on prophylactic treatments for respiratory disorders, and how they might affect practice, see rationale and impact.

Discussing the management of respiratory failure

1.4.30 If a person with cerebral palsy has symptoms of respiratory failure, or is at high risk of developing respiratory failure, discuss their management plan with them (and their family or carers, if agreed), including:

  • assessing the effectiveness and tolerability of treatment

  • treatment goals and escalation plan of treatment

  • managing complications

  • options for managing progressive respiratory failure.

Assisted ventilation for respiratory failure

1.4.31 Consider home-based non-invasive ventilation for adults with cerebral palsy and respiratory failure.

1.4.32 If a person with cerebral palsy is having home-based non-invasive ventilation, carry out a review with a multidisciplinary team every 3 to 6 months. Assess and discuss with the person (and their family and carers, if agreed) the effectiveness, tolerability and whether agreed goals are being met.

1.4.33 If a person with cerebral palsy is having non-invasive ventilation, discuss with them (and their family or carers, if agreed) their preferences for future treatment. Agree a plan for what should happen if an intercurrent infection (occurring during the progress of respiratory failure) causes an acute deterioration in respiratory function and record this in the person's advance care plan.

1.4.34 If the agreed treatment goals are not met by non-invasive ventilation, consider alternative treatment options, such as tracheostomy or supportive care, and discuss them with the person with cerebral palsy, taking into account their preferences for future treatment (see recommendation 1.4.33).

Assisted ventilation for sleep apnoea

1.4.35 If an adult with cerebral palsy has sleep apnoea, follow the recommendations in the NICE technology appraisal guidance on continuous positive airway pressure for the treatment of obstructive sleep apnoea/hypopnoea syndrome.

To find out why the committee made the recommendations on discussing the management of respiratory failure, and assisted ventilation for respiratory failure and sleep apnoea, and how they might affect practice, see rationale and impact.

Pain

1.4.36 Be aware that some adults with cerebral palsy have difficulty communicating or are unable to communicate that they are in pain.

1.4.37 Assess for the presence, severity and location of pain in adults with cerebral palsy using pain assessment tools such as:

  • numerical rating scales

  • visual analogue scales

  • faces pain scales

  • body maps.

1.4.38 If an adult with cerebral palsy has difficulty communicating:

  • discuss with their family or carers how best to identify pain and include this information in their care plan

  • use observational or descriptive pain scales to assess the presence, severity and location of pain.

    See also NICE's guideline on patient experience in adult NHS services for advice on communication.

1.4.39 Ensure that health and social care staff (and families and carers, if appropriate) caring for adults with cerebral palsy have access to a range of pain assessment tools and that they have been trained in their use.

To find out why the committee made the recommendations on pain, and how they might affect practice, see rationale and impact.

Terms used in this guideline

Advance care plan

Defined by international consensus as: 'A process that supports adults at any age or stage of health in understanding and sharing their personal values, life goals, and preferences about future medical care. The goal of an advance care plan is to help ensure that people receive medical care that is consistent with their values, goals and preferences during serious and chronic illness.' Sudore et al. (2017) Defining advance care planning for adults.

Alternative and augmentative communication systems

Alternative and augmentative communication systems are a variety of methods (for example, signing, use of visual symbols and eye gaze technology) that can be used to help people with disabilities communicate with others. These systems or methods of communication can be used as an alternative to speech or to supplement it.

Anthropometric measurements

Body measurements that include weight, height, knee height, mid-upper arm circumference, waist circumference, head circumference and skinfold thickness measurements.

Comorbidities

In the context of cerebral palsy, comorbidities are health problems caused by the brain disorder that also caused the motor impairment that is the core problem of cerebral palsy, but are not a direct complication of the motor disorder. For example, visual impairment and epilepsy are described as comorbidities because they are caused by the brain disorder. Hip dislocation and scoliosis are complications of the motor disorder and would not be described as comorbidities.

Electronic assistive technology

Any piece of equipment or system that is used to increase, maintain or improve function in people with disabilities, and is electronically powered (mains electricity and/or battery). These may include communication aids, environmental controls and access to computers.

Enteral drug treatment

Drugs given by mouth (oral), or via a nasogastric tube, gastrostomy tube or jejunostomy tube.

Escalation plan

A record of the interventions that a person would find acceptable, in line with their values, goals and preferences. It can be used to indicate that more intensive or invasive interventions would be unacceptable to the person. For example, an escalation plan of ward-based care only, indicates that the person would not want invasive monitoring, intubation and ventilation, which are undertaken as part of intensive care.

Executive functions

Cognitive processes that are important for the control of behaviour. These include planning, organising and monitoring behaviours leading to goal attainment, inhibitory control, working memory and cognitive flexibility. As a result of injuries to the frontal lobes of the brain, these processes can be disrupted.

Gross Motor Function Classification System

A 5‑level clinical classification system that describes the gross motor function of people with cerebral palsy based on self-initiated movement abilities. People assessed as level I are the most able and people assessed as level V are dependent on others for all their mobility needs.

The GMFCS is not validated for use in adults. However, the GMFCS level at age 12 has been shown to be a good predictor of mobility into adulthood, especially at the milder and most severe levels. It is used here in the absence of a validated system for use in adults because it is readily understood by people with cerebral palsy, their families and carers, and health professionals involved in the care of adults with cerebral palsy.

Key communication partners

People who regularly interact with the person with cerebral palsy in any environment. Communication may be by speech, using communication aids, signing, facial expression or a combination of these.

Rehabilitation engineering services

Centres that design, develop and adapt technological solutions to overcome challenges to function, activity and participation for individuals with disability. This includes assessment and provision of assistive devices to help with posture, mobility and communication (for example, electronic assistive technology).

Review

A planned clinical appointment between an adult with cerebral palsy and a healthcare professional or multidisciplinary team. They may explore common concerns, physical symptoms, mental health, pain, nutrition and communication to ensure an individualised approach to care. The healthcare professional may be a GP, specialist nurse, rehabilitation specialist or therapist. This also allows the opportunity to address general health issues that affect people as they grow older.

Review of 24‑hour postural needs

Part of a 24‑hour posture review that considers all the relevant postures that an individual has the ability to adopt over the 24 hours of any given day, including postures to allow for participation in daily activities. The 3 core postural orientations are lying, sitting and standing. An example of a postural need is support and positioning in bed.

Supportive care

Care focused on relieving symptoms caused by serious illnesses such as respiratory failure. It can be given at any point during a person's illness to help them feel more comfortable and improve their quality of life.

Transferring

Moving from one surface to another (for example, from a bed to a wheelchair) independently or with assistance.



[1] At the time of publication (January 2019), oral formulations of muscle relaxant drugs are usually not licensed to be given via an enteral feeding tube so administration via this route would be off-label. See the General Medical Council's Prescribing guidance: prescribing unlicensed medicines for further information.

[2] There are several different preparations of botulinum toxin type A and the licensed indications vary between them. Refer to the summaries of product characteristics for the individual products for licensing and prescribing information.

[3] At the time of publication (January 2019), levodopa did not have a UK marketing authorisation for this indication. The prescriber should follow relevant professional guidance, taking full responsibility for the decision. Informed consent should be obtained and documented. See the General Medical Council's Prescribing guidance: prescribing unlicensed medicines for further information.

[4] At the time of publication (January 2019), oral formulations of anti-dystonic drugs are usually not licensed to be given via an enteral feeding tube so administration via this route would be off-label. See the General Medical Council's Prescribing guidance: prescribing unlicensed medicines for further information.

[5] At the time of publication (January 2019), botulinum toxin type A did not have a UK marketing authorisation for this indication. The prescriber should follow relevant professional guidance, taking full responsibility for the decision. Informed consent should be obtained and documented. See the General Medical Council's Prescribing guidance: prescribing unlicensed medicines for further information.

[6] At the time of publication (January 2019), intrathecal baclofen did not have a UK marketing authorisation for the treatment of dystonia. The prescriber should follow relevant professional guidance, taking full responsibility for the decision. Informed consent should be obtained and documented. See the General Medical Council's Prescribing guidance: prescribing unlicensed medicines for further information.

  • National Institute for Health and Care Excellence (NICE)