Spasticity in under 19s: management

Children and young people with spasticity should have access to a network of care that uses agreed care pathways supported by effective communication and integrated team working.

The network of care should provide access to a team of healthcare professionals experienced in the care of children and young people with spasticity. The network team should provide local expertise in paediatrics, nursing, physiotherapy and occupational therapy. Access to other expertise, including orthotics, orthopaedic surgery and/or neurosurgery and paediatric neurology, may be provided locally or regionally.

If a child or young person receives treatment for spasticity from healthcare professionals outside the network team, this should be planned and undertaken in discussion with the network team to ensure integrated care and effective subsequent management.

Following diagnosis, ensure that all children and young people with spasticity are referred without delay to an appropriate member of the network team.

Offer a management programme that is:

• developed and implemented in partnership with the child or young person and their parents or carers

• individualised

• goal focused.

When formulating a management programme take into account its possible impact on the individual child or young person and their family.

Carefully assess the impact of spasticity in children and young people with cognitive impairments:

• be aware that the possible benefit of treatments may be more difficult to assess in a child or young person with limited communication

• ensure that the child or young person has access to all appropriate services.

Identify and agree with children and young people and their parents or carers assessments and goals that:

• are age and developmentally appropriate

• focus on the following domains of the World Health Organization's International Classification of Functioning, Disability and Health:

  • body functions

  • body structures

  • activities and participation

  • environmental factors.

Record the child or young person's individualised goals and share these goals with healthcare professionals in the network team and, where appropriate, other people involved in their care.

Help children and young people and their parents or carers to be partners in developing and implementing the management programme by offering:

• relevant, and age and developmentally appropriate, information and educational materials

• regular opportunities for discussion and

• advice on their developmental potential and how different treatment options may affect this.

Offer contact details of patient organisations that can provide support, befriending, counselling, information and advocacy.

Ensure that children and young people have timely access to equipment necessary for their management programme (for example, postural management equipment such as sleeping, sitting or standing systems).

The network team should have a central role in transition to prepare young people and their parents or carers for the young person's transfer to adult services.

Monitor the child or young person's condition for:

• the response to treatments

• worsening of spasticity

• developing secondary consequences of spasticity, for example pain or contractures

• the need to change their individualised goals.

The network of care should have a pathway for monitoring children and young people at increased risk of hip displacement.

Recognise the following clinical findings as possible indicators of hip displacement (hip migration greater than 30%):

• pain arising from the hip

• clinically important leg length difference

• deterioration in hip abduction or range of hip movement

• increasing hip muscle tone

• deterioration in sitting or standing

• increasing difficulty with perineal care or hygiene.

Offer a hip X-ray to assess for hip displacement:

• if there are clinical concerns about possible hip displacement

• at 24 months in children with bilateral cerebral palsy.

Consider repeating the hip X-ray annually in children or young people who are at Gross Motor Function Classification System (GMFCS) level III, IV or V.

Consider repeating the hip X-ray after 6 months in children and young people where the initial hip migration is greater than 30%, and then consider repeating the hip X-ray every 6 months after this if the hip migration is increasing by more than 10 percentage points per year.

All children and young people with spasticity referred to the network team should be promptly assessed by a physiotherapist and, where necessary, an occupational therapist.

Offer a physical therapy (physiotherapy and/or occupational therapy) programme tailored to the child or young person's individual needs and aimed at specific goals, such as:

• enhancing skill development, function and ability to participate in everyday activities

• preventing consequences such as pain or contractures.

Give children and young people and their parents or carers verbal and written (or appropriate formats) information about the physical therapy interventions needed to achieve the intended goals. This information should emphasise the balance between possible benefits and difficulties (for example, time commitment or discomfort), to enable them to participate in choosing a suitable physical therapy programme.

When formulating a physical therapy programme for children and young people take into account:

• the views of the child or young person and their parents or carers

• the likelihood of achieving the treatment goals

• possible difficulties in implementing the programme

• implications for the individual child or young person and their parents or carers, including the time and effort involved and potential individual barriers.

When deciding who should deliver physical therapy, take into account:

• whether the child or young person and their parents or carers are able to deliver the specific therapy

• what training the child or young person or their parents or carers might need

• the wishes of the child or young person and their parents or carers.

Ensure that any equipment or techniques used in the physical therapy programme are safe and appropriate, in particular for children or young people with any of the following:

• poorly controlled epilepsy

• respiratory compromise

• increased risk of pulmonary aspiration

• increased risk of bone fracture due to osteoporosis (for example, those who are unable to walk, malnourished or taking anti-epileptic therapy).

Encourage children and young people and their parents or carers to incorporate physical therapy into daily activities (for example, standing at the sink while brushing teeth in order to stretch leg muscles).

Consider including in the physical therapy programme 24-hour postural management strategies to:

• prevent or delay the development of contractures or skeletal deformities in children and young people at risk of developing these

• enable the child or young person to take part in activities appropriate to their stage of development.

When using 24-hour postural management strategies consider on an individual basis low-load active stretching or low-load passive stretching.

Offer training to parents and carers involved in delivering postural management strategies.

Consider task-focused active-use therapy such as constraint-induced movement therapy (temporary restraint of an unaffected arm to encourage use of the other arm) followed by bimanual therapy (unrestrained use of both arms) to enhance manual skills.

When undertaking task-focused active-use therapy consider an intensive programme over a short time period (for example, 4 to 8 weeks).

Consider muscle-strengthening therapy where the assessment indicates that muscle weakness is contributing to loss of function or postural difficulties.

Direct muscle-strengthening therapy towards specific goals using progressive repetitive exercises performed against resistance.

Following treatment with botulinum toxin type A, continuous pump-administered intrathecal baclofen, orthopaedic surgery or selective dorsal rhizotomy, provide an adapted physical therapy programme as an essential component of management.

Ensure that children and young people and their parents or carers understand that an adapted physical therapy programme will be an essential component of management following treatment with botulinum toxin type A, continuous pump-administered intrathecal baclofen, orthopaedic surgery or selective dorsal rhizotomy.

Reassess the physical therapy programme at regular intervals to ensure that:

• the goals are being achieved

• the programme remains appropriate to the child or young person's needs.

Consider orthoses for children and young people with spasticity based on their individual needs and aimed at specific goals, such as:

• improving posture

• improving upper limb function

• improving walking efficiency

• preventing or slowing development of contractures

• preventing or slowing hip migration

• relieving discomfort or pain

• preventing or treating tissue injury, for example by relieving pressure points.

When considering an orthosis, discuss with the child or young person and their parents or carers the balance of possible benefits against risks. For example, discuss its cosmetic appearance, the possibility of discomfort or pressure sores or of muscle wasting through lack of muscle use.

Assess whether an orthosis might:

• cause difficulties with self-care or care by others

• cause difficulties in relation to hygiene

• be unacceptable to the child or young person because of its appearance.

Ensure that orthoses are appropriately designed for the individual child or young person and are sized and fitted correctly. If necessary seek expert advice from an orthotist within the network team.

Be aware when considering a rigid orthosis that it may cause discomfort or pressure injuries in a child or young person with marked dyskinesia. They should be monitored closely to ensure that the orthosis is not causing such difficulties.

The network of care should have a pathway that aims to minimise delay in:

• supplying an orthosis once measurements for fit have been performed and

• repairing a damaged orthosis.

Inform children and young people who are about to start using an orthosis, and their parents or carers:

• how to apply and wear it

• when to wear it and for how long:

  • an orthosis designed to maintain stretch to prevent contractures is more likely to be effective if worn for longer periods of time, for example at least 6 hours a day

  • an orthosis designed to support a specific function should be worn only when needed

• when and where to seek advice.

Advise children and young people and their parents or carers that they may remove an orthosis if it is causing pain that is not relieved despite their repositioning the limb in the orthosis or adjusting the strapping.

Consider the following orthoses for children and young people with upper limb spasticity:

• elbow gaiters to maintain extension and improve function

• rigid wrist orthoses to prevent contractures and limit wrist and hand flexion deformity

• dynamic orthoses to improve hand function (for example, a non-rigid thumb abduction splint allowing some movement for a child or young person with a 'thumb in palm' deformity).

Consider ankle–foot orthoses for children and young people with serious functional limitations (GMFCS level IV or V) to improve foot position for sitting, transfers between sitting and standing, and assisted standing.

Be aware that in children and young people with secondary complications of spasticity, for example contractures and abnormal torsion, ankle–foot orthoses may not be beneficial.

For children and young people with equinus deformities that impair their gait consider:

• a solid ankle–foot orthosis if they have poor control of knee or hip extension

• a hinged ankle–foot orthosis if they have good control of knee or hip extension.

Consider ground reaction force ankle–foot orthoses to assist with walking if the child or young person has a crouch gait and good passive range of movement at the hip and knee.

Consider body trunk orthoses for children and young people with co-existing scoliosis or kyphosis if this will help with sitting.

Consider the overnight use of orthoses to:

• improve posture

• prevent or delay hip migration

• prevent or delay contractures.

Consider the overnight use of orthoses for muscles that control two joints. Immobilising the two adjacent joints provides better stretch and night-time use avoids causing functional difficulties.

If an orthosis is used overnight, check that it:

• is acceptable to the child or young person and does not cause injury

• does not disturb sleep.

The network team should review the use of orthoses at every contact with the child or young person. Ensure that the orthosis:

• is still acceptable to the child or young person and their parents or carers

• remains appropriate to treatment goals

• is being used as advised

• remains well fitting and in good repair

• is not causing adverse effects such as discomfort, pain, sleep disturbance, injury or excessive muscle wasting.

Consider botulinum toxin type A treatment in children and young people in whom focal spasticity of the upper limb is:

• impeding fine motor function

• compromising care and hygiene

• causing pain

• impeding tolerance of other treatments, such as orthoses

causing cosmetic concerns to the child or young person. Consider botulinum toxin type A treatment where focal spasticity of the lower limb is:

• impeding gross motor function

• compromising care and hygiene

• causing pain

• disturbing sleep

• impeding tolerance of other treatments, such as orthoses and use of equipment to support posture

• causing cosmetic concerns to the child or young person.

Consider botulinum toxin type A treatment after an acquired non-progressive brain injury if rapid-onset spasticity is causing postural or functional difficulties.

Consider a trial of botulinum toxin type A treatment in children and young people with spasticity in whom focal dystonia is causing serious problems, such as postural or functional difficulties or pain.

Do not offer botulinum toxin type A treatment if the child or young person:

• has severe muscle weakness

• had a previous adverse reaction or allergy to botulinum toxin type A

• is receiving aminoglycoside treatment.

Be cautious when considering botulinum toxin type A treatment if:

• the child or young person has any of the following:

  • a bleeding disorder, for example due to anti-coagulant therapy

  • generalised spasticity

  • fixed muscle contractures

  • marked bony deformity or

• there are concerns about the child or young person's likelihood of engaging with the post-treatment adapted physical therapy programme (see recommendation 1.2.15).

When considering botulinum toxin type A treatment, perform a careful assessment of muscle tone, range of movement and motor function to:

• inform the decision as to whether the treatment is appropriate

• provide a baseline against which the response to treatment can be measured.
  
  A physiotherapist or an occupational therapist should be involved in the assessment.

When considering botulinum toxin type A treatment, give the child or young person and their parents or carers information about:

• the possible benefits and the likelihood of achieving the treatment goals

• what the treatment entails, including:

  • the need for assessments before and after the treatment

  • the need to inject the drug into the affected muscles

  • the possible need for repeat injections

  • the benefits, where necessary, of analgesia, sedation or general anaesthesia

  • the need to use serial casting or an orthosis after the treatment in some cases

• possible important adverse effects (see also recommendation 1.5.10).

Botulinum toxin type A treatment (including assessment and administration) should be provided by healthcare professionals within the network team who have expertise in child neurology and musculoskeletal anatomy.

Before starting treatment with botulinum toxin type A, tell children and young people and their parents or carers:

• to be aware of the following rare but serious complications of botulinum toxin type A treatment:

  • swallowing difficulties

  • breathing difficulties

• how to recognise signs suggesting these complications are present

• that these complications may occur at any time during the first week after the treatment and

• that if these complications occur the child or young person should return to hospital immediately.

To avoid distress to the child or young person undergoing treatment with botulinum toxin type A, think about the need for:

• topical or systemic analgesia or anaesthesia

• sedation (see the NICE guideline on sedation in under 19s).

Consider ultrasound or electrical muscle stimulation to guide the injection of botulinum toxin type A.

Consider injecting botulinum toxin type A into more than one muscle if this is appropriate to the treatment goal, but ensure that maximum dosages are not exceeded.

After treatment with botulinum toxin type A, consider an orthosis to:

• enhance stretching of the temporarily weakened muscle and

• enable the child or young person to practice functional skills.

If an orthosis is indicated after botulinum toxin type A, but limited passive range of movement would make this difficult, consider first using serial casting to stretch the muscle. To improve the child or young person's ability to tolerate the cast, and to improve muscle stretching, delay casting until 2 to 4 weeks after the botulinum toxin type A treatment.

Ensure that children and young people who receive treatment with botulinum toxin type A are offered timely access to orthotic services.

Perform an assessment of muscle tone, range of movement and motor function:

• 6 to 12 weeks after injections to assess the response

• 12 to 26 weeks after injections to inform decisions about further injections.
  
  These assessments should preferably be performed by the same healthcare professionals who undertook the baseline assessment.

Consider repeat injections of botulinum toxin type A if:

• the response in relation to the child or young person's treatment goal was satisfactory, and the treatment effect has worn off

• new goals amenable to this treatment are identified.

Consider treatment with continuous pump-administered intrathecal baclofen in children and young people with spasticity if, despite the use of non-invasive treatments, spasticity or dystonia are causing difficulties with any of the following:

• pain or muscle spasms

• posture or function

• self-care (or ease of care by parents or carers).
  
  At the time of publication (July 2012), intrathecal baclofen did not have UK marketing authorisation for children younger than 4 years, nor did it have UK marketing authorisation for use in the treatment of dystonia associated with spasticity. Where appropriate, informed consent should be obtained and documented.

Be aware that children and young people who benefit from continuous pump-administered intrathecal baclofen typically have:

• moderate or severe motor function problems (GMFCS level III, IV or V)

• bilateral spasticity affecting upper and lower limbs.

Be aware of the following contraindications to treatment with continuous pump-administered intrathecal baclofen:

• the child or young person is too small to accommodate an infusion pump

• local or systemic intercurrent infection.

Be aware of the following potential contraindications to treatment with continuous pump-administered intrathecal baclofen:

• co-existing medical conditions (for example, uncontrolled epilepsy or coagulation disorders)

• a previous spinal fusion procedure

• malnutrition, which increases the risk of post-surgical complications (for example, infection or delayed healing)

• respiratory disorders with a risk of respiratory failure.

If continuous pump-administered intrathecal baclofen is indicated in a child or young person with spasticity in whom a spinal fusion procedure is likely to be necessary for scoliosis, implant the infusion pump before performing the spinal fusion.

When considering continuous pump-administered intrathecal baclofen, balance the benefits of reducing spasticity against the risk of doing so because spasticity sometimes supports function (for example, by compensating for muscle weakness). Discuss these possible adverse effects with the child or young person and their parents or carers.

When considering continuous pump-administered intrathecal baclofen, inform children and young people and their parents or carers verbally and in writing (or appropriate formats) about:

• the surgical procedure used to implant the pump

• the need for regular hospital follow-up visits

• the requirements for pump maintenance

• the risks associated with pump implantation, pump-related complications and adverse effects that might be associated with intrathecal baclofen infusion.

Before making the final decision to implant the intrathecal baclofen pump, perform an intrathecal baclofen test to assess the therapeutic effect and to check for adverse effects.

Before intrathecal baclofen testing, inform children and young people and their parents or carers verbally and in writing (or appropriate formats) about:

• what the test will entail

• adverse effects that might occur with testing

• how the test might help to indicate the response to treatment with continuous pump-administered intrathecal baclofen, including whether:

  • the treatment goals are likely to be achieved

  • adverse effects might occur.

Before performing the intrathecal baclofen test, assess the following where relevant to the treatment goals:

• spasticity

• dystonia

• the presence of pain or muscle spasms

• postural difficulties, including head control

• functional difficulties

• difficulties with self-care (or ease of care by parents or carers).
  
  If necessary, assess passive range of movement under general anaesthesia.

The test dose or doses of intrathecal baclofen should be administered using a catheter inserted under general anaesthesia.

Assess the response to intrathecal baclofen testing within 3 to 5 hours of administration. If the child or young person is still sedated from the general anaesthetic at this point, repeat the assessment later when they have recovered.

When deciding whether the response to intrathecal baclofen is satisfactory, assess the following where relevant to the treatment goals:

• reduction in spasticity

• reduction in dystonia

• reduction in pain or muscle spasms

• improved posture, including head control

• improved function

• improved self-care (or ease of care by parents or carers).

Discuss with the child or young person and their parents or carers their views on the response to the intrathecal baclofen test. This should include their assessment of the effect on self-care (or ease of care by parents or carers). Consider using a standardised questionnaire to document their feedback.

Intrathecal baclofen testing should be:

• performed in a specialist neurosurgical centre within the network that has the expertise to carry out the necessary assessments

• undertaken in an inpatient setting to support a reliable process for assessing safety and effectiveness.

Initial and post-test assessments should be performed by the same healthcare professionals in the specialist neurosurgical centre.

Before implanting the intrathecal baclofen pump, inform children and young people and their parents or carers, verbally and in writing (or appropriate formats), about:

• safe and effective management of continuous pump-administered intrathecal baclofen

• the effects of intrathecal baclofen, possible adverse effects, and symptoms and signs suggesting the dose is too low or too high

• the potential for pump-related complications

• the danger of stopping the continuous pump-administered intrathecal baclofen infusion suddenly

• the need to attend hospital for follow-up appointments, for example to refill and reprogram the infusion pump

• the importance of seeking advice from a healthcare professional with expertise in intrathecal baclofen before stopping the treatment.

Implant the infusion pump and start treatment with continuous pump-administered intrathecal baclofen within 3 months of a satisfactory response to intrathecal baclofen testing (see recommendation 1.6.13).

Support children and young people receiving treatment with continuous pump-administered intrathecal baclofen and their parents or carers by offering regular follow-up with the network team, and a consistent point of contact with the specialist neurosurgical centre.

Monitor the response to continuous pump-administered intrathecal baclofen. This monitoring should preferably be performed by the healthcare professionals in the regional specialist neurosurgical centre who performed the pre-implantation assessments.

When deciding whether the response to continuous pump-administered intrathecal baclofen is satisfactory, assess the following where relevant to the treatment goals:

• reduction in spasticity

• reduction in dystonia

• reduction in pain or muscle spasms

• improved posture, including head control

• improved function

• improved self-care (or ease of care by parents or carers).

Titrate the dose of intrathecal baclofen after pump implantation, if necessary, to optimise effectiveness.

If treatment with continuous pump-administered intrathecal baclofen does not result in a satisfactory response (see recommendation 1.6.21), check that there are no technical faults in the delivery system and that the catheter is correctly placed to deliver the drug to the intrathecal space. If no such problems are identified, consider reducing the dose gradually to determine whether spasticity and associated symptoms increase.

If continuous pump-administered intrathecal baclofen therapy is unsatisfactory, the specialist neurosurgical centre and other members of the network team should discuss removing the pump and alternative management options with the child or young person and their parents or carers.

As the infusion pump approaches the end of its expected lifespan, consider reducing the dose gradually to enable the child or young person and their parents or carers to decide whether or not to have a new pump implanted.