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.

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.

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:

• use of alternative or augmentative communication systems
• intensive speech therapy to improve the intelligibility of their speech.

  See also NICE’s guideline on patient experience in adult NHS services for general advice on how to provide information and communicate with adults receiving care.

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.

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.

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.

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:

• personal treatment goals (which should be documented) and
• the benefits and risks of treatments (for example, the risk of deterioration in function) as part of their multidisciplinary treatment strategy.

  For further information on supporting people to actively participate in their care and shared decision making, see NICE’s guideline on patient experience in adult NHS services.

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.

Spasticity

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.

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.

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:

• attention deficit hyperactivity disorder
• depression in adults and depression in adults with a chronic physical health problem
• generalised anxiety disorder and panic disorder in adults
• autism spectrum disorder in adults
• challenging behaviour and learning disabilities
• mental health problems in people with learning disabilities.

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.

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.

Respiratory disorders

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.

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. Method of botulinum toxin type A injection in treating focal spasticity

Is guided botulinum toxin type A injection using electrical localisation (electrostimulation or electromyography) of muscles more clinically and cost effective than ultrasound-guided injections or clinical positioning for localisation of injections in treating focal spasticity in adults with cerebral palsy?

To find out why the committee made the research recommendation on the method of botulinum toxin type A injection in treating focal spasticity, see rationale and impact.

2. Selective dorsal rhizotomy treatment to reduce spasticity

What is the clinical and cost effectiveness of selective dorsal rhizotomy compared with intrathecal baclofen to reduce spasticity in adults with cerebral palsy?

To find out why the committee made the research recommendation on selective dorsal rhizotomy treatment to reduce spasticity, see rationale and impact.

3. Detection and management of respiratory disorders in primary and community care

Can detection and management of respiratory disorders in adults with cerebral palsy be improved in primary and community care?

To find out why the committee made the research recommendation on the detection and management of respiratory disorders in primary and community care, see rationale and impact.

4. Prophylactic antibiotics for respiratory disorders

Are prophylactic antibiotics clinically and cost effective in the management of respiratory symptoms in adults with cerebral palsy with significant respiratory comorbidity?

To find out why the committee made the research recommendation on prophylactic antibiotics for respiratory disorders, see rationale and impact.

5. Splinting to improve or maintain posture or function

What is the optimum regimen for splints applied to the upper limb in adults with cerebral palsy to improve or maintain posture or function?

To find out why the committee made the research recommendation on splinting to improve or maintain posture or function, see rationale and impact.

6. Augmentative and alternative communication systems

Are augmentative and alternative communication systems clinically and cost effective in promoting communication for adults with cerebral palsy who have communication difficulties?

To find out why the committee made the research recommendation on augmentative and alternative communication systems, see rationale and impact.

Why the committee made the recommendations

How the recommendations might affect practice

The recommendations on access to services reinforce legislation and best current practice.

Service organisation for networks of care is variable, so changes to practice will depend on the availability of services within a particular local area. Providing information about local networks of care may incur an initial cost to set up.

There may be an increase in the number of referrals. Training will also be needed to ensure healthcare professionals can manage referrals rapidly because there is a limited number of healthcare professionals with expertise in neurological disorders. However, recommendations with criteria for referral will help to reduce inappropriate referrals and variation in referrals.

Full details of the evidence and the committee’s discussion are in evidence review F1: configuration of services for adults with cerebral palsy and evidence review F2: barriers to access to primary and secondary care.

Return to recommendations

Why the committee made the recommendations

There was limited evidence for the effectiveness of different service configurations. One study suggested that fewer visits to emergency departments and hospital visits were associated with having consistent outpatient care provided by a single doctor. Although the committee recognised that this may reflect the value of continuity of care, they agreed that the quality of the evidence was not good enough to make a recommendation.

Further evidence showed that changing the configuration of services to include the time and staff for an annual review could be cost effective if there is a reduction of at least 1 emergency department visit per year per person. It was not clear from the evidence that this could be achieved when offered to all people with cerebral palsy. The committee discussed the variation in the needs of people with cerebral palsy and, based on their experience and knowledge, identified groups with more severe or complex health and social care needs who would most benefit from being offered an annual review. The committee agreed that annual review is likely to prevent an emergency department visit for this group and so would be cost effective. The committee decided that the annual review should be carried out by a healthcare professional with expertise in neurodisabilities who would have the skills and experience needed to identify potential issues that could lead to an emergency department visit. It was noted that adults with learning disabilities should already be offered an annual health check in primary care.

As an example of complex needs, the Gross Motor Function Classification System (GMFCS) levels IV and V were used. The committee acknowledged that the GMFCS is not validated for use in adults. However, it has been shown that at age 12, a GMFCS level I strongly predicts that the person with cerebral palsy will retain good mobility into adult years and a GMFCS level V strongly predicts that the person will be a wheelchair user in adult life. Therefore, at the mild and severe ends of the spectrum of mobility in cerebral palsy, there is stability of GMFCS levels into adulthood. In the absence of an alternative descriptive system validated for use in adults, the GMFCS is used here 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.

Although the evidence did not support annual reviews for all people with cerebral palsy, the committee agreed that regular reviews are important to check for any new problems and ensure that people’s needs continue to be met. They agreed that regular reviews should be considered, tailored to the person’s needs. The frequency of review was not specified because it will depend on person’s needs and wishes.

It is important to discuss who should be involved in the review with the adult with cerebral palsy. Information from different healthcare professionals and social care staff may be essential to fully understand the person’s clinical needs. Good record keeping and sharing of information ensures that the outcome of the review is known to all relevant people and that the appropriate actions are taken. The committee also agreed that information about the review and any changes to the person’s needs should be shared to ensure integrated care.

To help maintain continuity of care between reviews, the committee agreed that there should be a single point of contact, which could be a department or service in primary or secondary care.

The committee recognised that some people may not need or wish to have regular reviews. To ensure that they still have access to specialist services when needed, the committee agreed that they should be given information on how and when to do this.

How the recommendations might affect practice

Adults with severe impairment are already likely to have more frequent contact with services. Therefore, providing an annual review for adults with severe impairment is likely to result in a minor change in practice and reduce variation. There may be bigger changes in practice for people with less severe impairment if they are reviewed more often. However, the costs of this will be offset by the benefits of more frequent checks, such as early identification and management of new impairments or deterioration of function.

Full details of the evidence and the committee’s discussion are in evidence review F1: configuration of services for adults with cerebral palsy.

Return to recommendations

Why the committee made the recommendations

There was limited evidence to support interventions to improve communication between adults with cerebral palsy and their communication partners. However, the committee acknowledged that communication is a basic human right and that adults with cerebral palsy should be supported to communicate, express themselves and live as independently as possible.

The committee was concerned that communication difficulties and changes to communication needs are sometimes missed. Based on the members’ experience, the committee agreed that increased awareness of this and a check for any changes to speech, hearing and communication at every review would help ensure that communication needs are recognised.

Based on their experience and knowledge, the committee agreed that alternative and augmentative communication systems may help some adults with cerebral palsy to meet their communication needs, support independence and improve quality of life and social relationships. Therefore the need for these systems should be discussed with adults who have communication difficulties. The committee noted that there is variation in the availability of training in these techniques, which is vital for their effective use. They also highlighted that personal preference is important, and that some people may not wish to use alternative or augmentative communication systems in place of speech as their main means of communication.

There was some evidence that intensive speech therapy or supplemented speech (using topic and alphabet cues) improved speech intelligibility, but the committee agreed that it was not sufficient to recommend these specific interventions. However, using this evidence and their knowledge and experience, the committee agreed that referral to speech therapy services would enable adults with communication difficulties to be assessed and offered suitable interventions.

The committee also noted that the recommendations support the fundamental rights of people with disabilities to freedom of expression and opinion, and access to information as set out in Article 21 of the UN Convention on the rights of persons with disabilities.

The committee discussed that there was a need for more research on alternative and augmentative communication systems. Current practice is to offer these systems in preference to intensive speech and language therapy for people with cerebral palsy and communication difficulties. However, there is only limited evidence to support this in children with cerebral palsy and no evidence was identified for adults. The committee developed a research recommendation to determine the effectiveness of augmentative and alternative communication systems in promoting communication for adults with cerebral palsy and communication difficulties.

How the recommendations might affect practice

There is currently variation in practice and the recommendations will help to address this and reinforce best practice.

Initially, an increase in referrals to speech therapy services and subsequent management is likely. However, this will decline as variations in practice are reduced.

There may be an increase in the use of augmentative and alternative communication equipment, and related training, which will involve additional costs. However, the benefits of being able to communicate would outweigh the additional costs of increased use of augmentative and alternative communication systems.

Full details of the evidence and the committee’s discussion are in evidence review D4: interventions to promote participation: communication.

Return to recommendations

Why the committee made the recommendations

There was very little evidence on specific interventions for vocational or independent living skills training. However, the committee acknowledged the benefits of increased independence, social and occupational integration, participation in the community and access to work for adults with cerebral palsy. Based on their experience and knowledge, the committee highlighted that people with cerebral palsy should be asked what they enjoy doing. If they have problems in participating in their chosen activity, they should have access to support. This should include assessing for and addressing any barriers to participation and support, and may involve referral to occupational therapy services, particularly for people with complex needs.

For adults who wish to work or live independently, the committee agreed that referral for specialist support to access training, work placements and leisure activities would be beneficial. Some evidence showed that people with higher educational attainment and fewer physical complications were more likely to gain paid employment, but the committee agreed that support should be an option regardless of ability, to enable all adults to reach their full potential. For adults with cerebral palsy who are in work, referral could support access to workplace and equipment assessment, and workplace and job retention training. The NICE guideline on workplace health: management practices was highlighted by the committee as an important resource to refer to.

The recommendations support compliance with the Equality Act 2010 to protect people from discrimination in employment, training and education. They also reflect the fundamental rights of people with disabilities to independence, social and occupational integration, participation in the community, access to training and to engage in work, as set out in the UN Convention on the rights of persons with disabilities.

How the recommendations might affect practice

These recommendations are intended to reinforce good current practice. There will be a cost associated with addressing external factors (such as access to buildings). However, this must be done to comply with government policy and legislation. Where they are not currently being implemented, some services may need additional investment in resources.

Full details of the evidence and the committee’s discussion are in evidence review D1: interventions to promote participation - vocational and independent living skills.

Return to recommendations

Why the committee made the recommendations

There was no evidence identified on electronic assistive technology for adults with cerebral palsy. Based on their experience, the committee agreed that adults with cerebral palsy and complex disabilities would benefit from access to electronic assisted technology, which may enhance their independence, quality of life and improve their opportunities for employment. The recommendations support legislation such as the Human Rights Act 1998 and the Equality Act 2010. The recommendation on referral to services providing electronic assistive technology is also in line with the NHS England service specifications on environmental control equipment for patients with complex disability (all ages).

The committee was unable to recommend any specific electronic assistive technology devices because of the lack of evidence. They agreed that services providing electronic assistive technology should provide devices tailored to the person’s needs.

Variation in training and ongoing reviews of electronic assistive technology equipment were highlighted by the committee. Training is important for both the adult with cerebral palsy and their family and carers to ensure that the devices are used to their full benefit. By discussing the use of their equipment at each review, the healthcare professional can ensure that any problems with equipment or changes to the person’s needs are identified.

The committee noted that people using electronic assistive technology may need less support from their carers and healthcare workers. This may reduce the person’s social contact, which can have a negative impact. Based on their experience, the committee agreed that it is important that healthcare professionals take this into account when discussing the risks and benefits of electronic assistive technology with the adult with cerebral palsy, their family and carers.

How the recommendations might affect practice

These recommendations are intended to reinforce good current practice, and support government policy and legislation. Where they are not currently being implemented, some services may need additional investment in resources.

Full details of the evidence and the committee’s discussion are in evidence review D3: electronic assistive technology.

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Why the committee made the recommendations

How the recommendations might affect practice

The recommendations on interventions to maintain physical function reinforce current best practice and should not lead to additional resource use. Any initial costs of specialist advice would be outweighed by the potential savings from avoiding complications, injuries and maintaining a wider range of abilities.

Full details of the evidence and the committee’s discussion are in evidence review D2: interventions that improve or maintain physical function and participation.

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Why the committee made the recommendations

The committee noted that there is a lack of understanding about the relationship between spasticity and dystonia. Based on their experience, they agreed that a better understanding of these conditions and the factors that affect them is likely to lead to more effective decisions about management. The committee discussed factors that commonly trigger or worsen symptoms of both spasticity and dystonia, and their concerns that these may sometimes go unrecognised.

The committee also discussed the balance of benefits and risks of treatment to reduce spasticity and dystonia. In particular, some people with cerebral palsy make functional use of their increased muscle tone from spasticity and dystonia, for example, to help them walk or transfer independently. For these people, a reduction in spasticity or dystonia could have a negative impact on function. To ensure informed decision making, the risks and benefits of treatment should be discussed with each person and specific treatment goals should be agreed.

There was limited evidence on treatments for spasticity and dystonia in adults with cerebral palsy, but based on their experience and expertise, the committee agreed on a stepwise approach to treatment dependant on tolerability and effectiveness. This is from the least invasive to the most invasive treatment option, which is reflected in the order of recommendations in the individual sections:

• identifying and managing any factors that might be exacerbating a person’s symptoms
• reviewing their physical management programme
• considering enteral (oral or via a feeding tube) drug treatment and referral if spasticity and dystonia are still inadequately managed
• considering more invasive treatment options.

How the recommendations might affect practice

The recommendations reflect current good practice and will help to eliminate variation.

Full details of the evidence and the committee’s discussion are in evidence review A1: pharmacological treatments for spasticity and evidence review A3: interventions for dystonia.

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Why the committee made the recommendations

How the recommendations might affect practice

Overall, the recommendations reflect current good practice and will help to eliminate variation, particularly in referrals to tone or spasticity management services.

The recommendation to consider enteral baclofen as a first-line drug treatment to manage spasticity should not lead to a large increase in costs because enteral baclofen is relatively cheap and already widely used. Despite this, the committee were unable to make a stronger recommendation because there was no comparative clinical evidence that baclofen was the most effective option.

There was clinical evidence to suggest the cost of botulinum toxin could be outweighed by its benefits when treating focal spasticity. The focus on referral for focal spasticity that is causing pain, impacting care or impairing activity is likely to reduce the number of inappropriate referrals.

Any additional costs of specialist input is expected to be balanced by a reduction in potentially inappropriate treatment and related adverse effects. There may be a change to practice because enteral diazepam will no longer be prescribed routinely, and this may result in a small cost saving.

Full details of the evidence and the committee’s discussion are in evidence review A1: pharmacological treatments for spasticity.

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Why the committee made the recommendations

There was some limited evidence with high uncertainty suggesting that both intrathecal baclofen and selective dorsal rhizotomy are effective in reducing muscle tone in adults with spasticity. However, there are risks involved, both in having surgery and of long-term complications. The committee highlighted the importance of discussing the procedure with the person and their family or carers, so that they fully understand what the treatment involves and the potential risks and benefits.

How the recommendations might affect practice

The recommendations reinforce current best practice and should not lead to additional resource use. Specialist services already exist and neurosurgical procedures are currently available for the treatment of spasticity. Including specific criteria for referral should reduce the number of inappropriate referrals to these services.

Full details of the evidence and the committee’s discussion are in evidence review A2: neurosurgical treatments for spasticity.

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Why the committee made the recommendations

There was limited evidence on treating dystonia in adults with cerebral palsy. The committee discussed that it is a specialist clinical area and that the benefits and harms of treatments would need to be assessed by a person with expertise in tone management. Therefore, the committee agreed that adults with cerebral palsy should be referred for specialist management if they have problematic dystonia.

How the recommendations might affect practice

Overall, the recommendations reflect current good practice and will help to eliminate variation, particularly in referral. There may be a change to practice because levodopa will no longer be prescribed routinely, and this may result in a small cost saving.

Full details of the evidence and the committee’s discussion are in evidence review A3: treatments to reduce dystonia.

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Why the committee made the recommendations

How the recommendations might affect practice

Overall, the recommendations reflect current good practice and will help to eliminate variation.

Full details of the evidence and the committee’s discussion are in evidence review A3: treatments to reduce dystonia.

Return to recommendations

Why the committee made the recommendations

Based on their experience, the committee noted that there is a lack of awareness, both among adults with cerebral palsy and healthcare professionals, that people with cerebral palsy are at increased risk of bone and joint complications, and that musculoskeletal function may worsen over time. Common complications include osteoporosis and conditions caused by abnormal musculoskeletal development, such as scoliosis and subluxation of joints, or abnormal movements leading to degenerative change, such as cervical myelopathy. Increasing awareness and discussing this with adults with cerebral palsy will enable early identification and management of these conditions.

How the recommendations might affect practice

The recommendations for risk assessment and DXA scanning are unlikely to change current practice. DXA scans should already be considered under NICE’s guideline on osteoporosis.

The recommendations could increase referrals to specialist services. However, the impact of this is likely to be balanced by better treatment and prevention of hospital stays.

Full details of the evidence and the committee’s discussion are in evidence review B1: disorders of bones and joints.

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Why the committee made the recommendations

No evidence was found on assessing and monitoring mental health in adults with cerebral palsy. However, from their experience, the committee acknowledged that healthcare services for adults with cerebral palsy tend to focus on physical rather than mental health. Greater awareness of mental health problems and the specific challenges of identifying and managing them in adults with cerebral palsy would help to ensure that such problems are recognised and managed. Alongside this, the committee highlighted that discussing the person’s mental wellbeing at each review would help to identify any concerns and ensure that support for mental health problems is included in the person’s care plan. Important insights about a person’s mental health can often be gained from people close to them, so the committee agreed that (with consent from the person) family members or carers should also be asked if they have any concerns.

Physical problems and common frustrations that can affect emotional wellbeing in adults with cerebral palsy were highlighted by the committee because they are often overlooked, but can negatively affect mental health and behaviour.

The committee noted that there are many relevant NICE guidelines related to mental health conditions that would apply to adults with cerebral palsy, and other NICE guidelines relevant to those with communication difficulties or learning disabilities.

How the recommendations might affect practice

The recommendations will reinforce current best practice and help to eliminate variation.

Full details of the evidence and the committee’s discussion are in evidence review B2: monitoring and assessing mental health.

Return to recommendations

Why the committee made the recommendations

There was some evidence on tools for assessing nutritional status in adults with cerebral palsy, but the committee concluded that the evidence was not good enough to recommend a specific tool. In addition, many factors can affect feeding and nutrition, so they agreed that a single tool is unlikely to be suitable for everyone.

Based on their experience, the committee agreed that assessment should be individualised to reflect each adult’s needs and circumstances. Current good practice includes regular weight checks and BMI or anthropometric measurement, and talking to the person and their families and carers about eating problems and other factors affecting nutrition and weight. People identified as at risk of undernutrition or with eating difficulties can then be referred to a specialist to assess for and treat specific problems. Because the recommendations focus on individualised assessment, the committee agreed that specific tools for assessing nutrition are not a priority for further research.

The committee discussed the role of people caring for adults with cerebral palsy and agreed that training should be provided in line with the NICE guideline on nutrition support for adults, to help pick up any problems between reviews.

The committee noted that adults with dyskinetic cerebral palsy or severe spasticity may have an increased metabolic rate and need to increase their calorie intake to prevent malnutrition. The committee recognised that reduction in dyskinesia or spasticity by treatment such as intrathecal baclofen may result in weight gain. They agreed that, from their experience, this can go unrecognised and that greater awareness could help people get the support they need.

How the recommendations might affect practice

The recommendations will reinforce current best practice and help to eliminate variation.

Full details of the evidence and the committee’s discussion are in evidence review B3: monitoring feeding and nutritional problems.

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Why the committee made the recommendations

No evidence was found on monitoring respiratory health in adults with cerebral palsy. Adults with cerebral palsy are at an increased risk of respiratory failure, which can be life threatening. However, based on their experience and knowledge, the committee agreed that the early symptoms of respiratory impairment may sometimes go unrecognised. Greater awareness and earlier recognition and treatment may result in treatment that prevents progression to respiratory failure.

Based on their experience and some limited evidence, the committee agreed that better awareness of the risk factors for respiratory impairment would help to ensure early recognition and appropriate referral. They also wanted to highlight that these are more common in adults with severe impairment, such as a high GMFCS score. They agreed that referral for specialist assessment would enable prevention or treatment of respiratory complications in people at high risk.

The committee discussed that reduced lung volume is an important factor contributing to respiratory impairment. However, there was limited evidence available on the value of spirometry in assessing respiratory function in adults with cerebral palsy. Based on their experience and expertise, the committee agreed that spirometry should be considered for people at high risk of respiratory impairment to help identify people who may need treatment.

The committee agreed that further research on identifying and managing respiratory disorders in adults with cerebral palsy would be helpful. They developed a research recommendation to determine the most effective methods of detecting and managing respiratory disorders in primary and community care.

How the recommendations might affect practice

Better survival of children with cerebral palsy into adulthood means that this is an emerging area of practice. There are relatively few respiratory specialists with a special interest in adults with cerebral palsy. There may be an increase in referrals, which might place increased pressure on limited specialist services. However, earlier recognition and treatment will lead to improved outcomes. Respiratory conditions can often lead to hospital admission and reducing the need for this would potentially lead to cost savings.

Full details of the evidence and the committee’s discussion are in evidence review C1: protocols for monitoring respiratory health.

Return to recommendations

Why the committee made the recommendations

No evidence was identified on preventing respiratory infections in adults with cerebral palsy. However, adults with cerebral palsy are at an increased risk of respiratory impairments and respiratory failure, so the committee agreed that adults with cerebral palsy and their family and carers should receive vaccinations, such as the flu vaccination, as a prophylactic measure to prevent respiratory infections.

Applying their clinical expertise and experience, the committee agreed that the role of antibiotics is limited for prophylaxis of respiratory infections in adults with cerebral palsy. Taking into account potential adverse effects and the principles of antibiotic stewardship, the committee agreed that antibiotic prophylaxis should only be used in people at high risk of infection when it is advised by the respiratory specialist with expertise in neurodisability management. For example, this might be in people with recurrent chest infections and bacterial colonisation identified on sputum culture. The aim for these people would be to reduce acute antibiotic use and limit symptom burden.

The committee agreed that the prevention of respiratory infections is an important area for research. Many people with cerebral palsy have respiratory symptoms caused by sputum retention or recurrent respiratory infection, possibly related to aspiration. A smaller number have chronic bacterial airway colonisation with increased respiratory symptom burden and recurrent infections. The committee developed a research recommendation to determine the role of prophylactic antibiotics in improving quality of life and preventing hospital stays in people with or without persistent bacterial airway colonisation.

Although there was no evidence for chest physiotherapy to prevent respiratory infections, the committee discussed the potential benefits of postural management and exercise. Based on their experience and expertise, they agreed that a physiotherapy chest care review should be considered for adults with cerebral palsy who are at high risk of respiratory infection. The committee also noted that families and carers can help with ongoing chest care, but may not always receive adequate support to enable this. They agreed that it would be beneficial for this to be included as part of the chest care review. This could also include advice on posture, position change, opportunities to move, interventions to assist ventilation and secretion control management.

Effective swallowing (and saliva control) is important to prevent respiratory infections in adults with cerebral palsy. The committee agreed that assessment by a dysphagia-trained speech and language therapist should be considered for people with recurrent chest infections that may be caused by dysphagia.

How the recommendations might affect practice

The recommendations on vaccination reinforce current best practice.

Referral to a respiratory team for adults with cerebral palsy will reinforce best practice. However, there may be an increase in referrals, which could put additional pressure on already limited specialist services.

The recommendation on prophylactic antibiotic use is not considered to be a change in practice. However, there may be a small decrease in use of prophylactic antibiotics, which could lead to cost savings.

The recommendations on chest physiotherapy review and referral to dysphagia-trained speech and language therapist are unlikely to have a big impact on current practice. There may be a small increase in the number of referrals. This is likely to be balanced by improved prevention of respiratory infections. There may also be an increase in provision of training and support for families and carers. However, this is likely to be balanced by improved ongoing chest care, which would reduce respiratory infections and the costs associated with them.

Full details of the evidence and the committee’s discussion are in evidence review C3: prophylactic treatments for respiratory disorders.

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Why the committee made the recommendations

The management of respiratory failure varies according to individual circumstances and preferences. The committee noted that having discussions about the effectiveness and tolerability of treatments (for example, non-invasive ventilation can be uncomfortable), as well as planning for future treatment and what to do if the person’s condition worsens, helps to identify the most appropriate treatment pathway.

Although no evidence was identified on assisted ventilation for adults with cerebral palsy, the committee noted that there is evidence supporting non-invasive ventilation in people with progressive neuromuscular conditions. The committee discussed that the course and symptoms of respiratory failure may be similar across the different conditions. They agreed that non-invasive ventilation could be beneficial, based on evidence extrapolated from these populations and the committee’s experience. The committee also agreed that it is important to review management every 3 to 6 months, which is consistent with standard practice.

The committee discussed that people’s goals and preferences for management after acute deterioration in respiratory function will vary. Based on their experience and expertise, the committee highlighted the importance of agreeing a management plan with the person (and their family or carers, if agreed) for future care in this situation. This should be documented in the person’s advance care plan. A full understanding of the options available and the person’s values, preferences and goals will lead to better shared decision making and more informed choices about care.

Based on their experience and expertise, the committee discussed that when treatment goals are not met by non-invasive techniques, alternative options such as tracheostomy or supportive care could be considered. There was no evidence available in adults with cerebral palsy, but the committee agreed that tracheostomy can be effective for some patients in maintaining quality of life.

The committee recognised that sleep apnoea is common in adults with cerebral palsy. It can affect sleep quality and therefore quality of life. They agreed that treatment would be the same for adults with cerebral palsy as in the general population and cross-referred to the NICE technology appraisal guidance on continuous positive airway pressure for the treatment of obstructive sleep apnoea/hypopnoea syndrome.

How the recommendations might affect practice

The recommendations in this section reinforce current best practice and will help to standardise practice.

Full details of the evidence and the committee’s discussion are in evidence review C2: assisted ventilation for respiratory failure.

Return to recommendations

Why the committee made the recommendations

The committee acknowledged that it can be difficult to recognise pain in people with communication difficulties. They agreed that better awareness of this would help to prevent under-identification of pain.

The evidence indicated that numerical, visual analogue and faces pain scales had similarly good reliability and validity for assessing pain in adults with cerebral palsy. Although the use of body maps was not evaluated in the evidence, the committee agreed they would also be a useful way to help localise the source of any pain. The committee acknowledged that families and carers have valuable insight into identifying whether a person is in pain, and this is especially important if the person has communication difficulties. For adults with cerebral palsy who have difficulty communicating, the committee agreed that observational and descriptive pain scales would be appropriate and useful. The committee agreed that in practice, the method of pain assessment chosen would depend on the person’s individual needs and circumstances, in particular, their ability to communicate.

The committee were also aware that people caring for adults with cerebral palsy do not always have access to suitable pain assessment tools or the training that is needed for their use. Based on their experience, they agreed that these are important to enable pain to be recognised, localised pain identified and treatment targeted effectively.

How the recommendations might affect practice

The recommendations reflect the current practice of selecting an appropriate measure from a range of pain assessment methods, depending on the person’s ability to communicate. The committee acknowledged that, although learning disability nurses currently train carers in generic pain assessment techniques, individualised training and documentation of how best to identify pain in the care plan would be a change in practice in some centres and may have a cost impact.

Full details of the evidence and the committee’s discussion are in evidence review E: identifying pain, such as musculoskeletal and gastrointestinal pain.

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