Upper Limb Pathway

Greater Glasgow and Clyde

Upper Limb Pathway

Population

What type of child/young person would it be appropriate to see within this pathway?

Children and young people aged from 0 to 18 years who have a Neurodisability that is affecting the muscle tone of the upper limb will be seen in this pathway. Neurodisability is an umbrella term for conditions associated with impairment involving the nervous system and includes conditions such as cerebral palsy. Children who have a history of an acquired brain injury or brain cancer that has caused a change to the muscle tone of the upper limb will also be seen in this pathway. However not all children with cerebral palsy will need to be seen through this pathway, for example children with diplegia may have unaffected hand function.

What Does A Typical Referral Look Like?

Current referrals for this group include newly diagnosed young children where the Occupational Therapy service is requested to provide advice and therapy to encourage bimanual hand use, for postural management and seating of more complex children.  Older children are referred because of difficulties with self-care tasks such as putting hair up, managing toileting independently, dressing, using cutlery, writing and recording work in school.  Older children may also be referred due to poor hygiene in the hand and difficulty with cleaning and dressing by carers.

 

Evidence Base

This pathway was developed following benchmarking of local, national and international services and review of the literature -

 

The National Institute for Clinical Excellence (NICE) published guidelines (updated Nov 2016) for the Management of Spasticity in Children and Young People. The guidelines recommend that a management programme is developed and implemented in partnership with the child or young person and their parents which is individualised and goal focused.

Ongoing monitoring of the child or young person’s condition should be made for review of the response to treatment, worsening of spasticity, the development of secondary consequences of spasticity e.g. pain or contractures, and for the need to change individualised goals.

Physical therapy (in this context, physical therapy encompasses occupational and physiotherapy) is recommended by NICE and this includes strength training and the use of task-focused active-use therapy such as constraint-induced movement therapy and bimanual therapy to enhance manual skills. It is recommended that this intervention is provided as an intensive programme over a short period (e.g. 4-8 weeks).

Further evidence for this pathway has been obtained from the Cerebral Palsy Upper Limb Pathway (CPUPS), the Swedish care pathway for children with cerebral palsy. CPUPS encompasses the hip surveillance protocol which has been adopted in Scotland as CPIPS (Cerebral Palsy Integrated Pathway Scotland). CPUPS also includes a care pathway for management of the upper limb of children with cerebral palsy. This pathway includes routine measurement of upper limb range of motion and monitoring of occupational performance and is carried out by Occupational Therapists across the country.

The Scottish OT Cerebral Palsy and Neurodisability Network has spent the past 3 years working on an upper limb management pathway. This work has included reviewing the current literature and collating information about available assessment and treatment options. The work of the Network is ongoing and will be published on the NES Clinical Knowledge Publisher and NES Managed Knowledge Network. This work has helped to inform the development of this pathway.

The literature also provides evidence and guidance on the role of Occupational Therapists in the management of children who have Cerebral Palsy with upper limb involvement.

Interventions include:

  • Bi-manual therapy.
  • Constraint-induced movement therapy.
  • Functional, goal directed training.
  • Splinting (including thermoplastic, neoprene, Lycra and Kinesiotaping).
  • Strength training.
  • Botulinum Toxin A in tandem with one or a combination of the above therapies.

Staged Journey of Care

Children with Neurodisability affecting the upper limb will routinely be managed at the specialist level, although some interventions and programmes will be carried out by the team around the child or young person for example carers applying splints or carrying out home programmes.

Overview of the Upper Limb Pathway

Aims

To reduce the impact of spasticity and abnormal tone on the long term function and wellbeing of young people with Neurodisability by:

  • Monitoring spasticity and range of motion of the upper limb.
  • Monitoring occupational performance and providing intervention in a timely fashion.
  • Monitoring splints.
  • Ensuring effective multi-disciplinary working: Being the referral source for interventions such as Botox will ensure that it is done when there are functional goals and that therapists are ready to provide the appropriate follow up intervention.

Clinical Knowledge Publisher (CKP) of the Pathway

The Upper Limb Pathway in Depth

First Contact

Children who are referred to the community paediatric Occupational Therapy service will be offered a First Contact appointment within six weeks of the referral.  The purpose of this appointment is to determine the concerns that the family and child have and to direct them to the best pathway to meet their needs.

Children who are identified as having abnormal tone in the upper limb and are over the age of 5 or who have previously been known to the service will be directed to the Upper Limb Clinic.  Where possible the date of the next clinic will be provided at the First Contact Appointment.  Some children will be referred to the Occupational Therapy service as a Priority 1 and will not attend a First Contact appointment.  If the child is not already known to the Upper Limb clinic, they will first be allocated to an Occupational Therapist and inclusion in the clinic will form part of their care plan and ongoing management.

Upper Limb Clinic

Clinic Attendees

Initially the Upper Limb Clinic will be conducted by the child’s named therapist and the Advanced Practitioner in Neurodisability.  Band 3 and 4 staff will assist in the setup of the room and preparation of assessment forms, and elements of the assessment etc.  The intention is that in the future the Upper Limb Clinic will be organised and run locally by a band 6 Occupational Therapist who has an interest in Neurodisability.  This therapist will be supported by the child’s named therapist and/or Band 3/4 staff.

Clinic Activities

There are 4 parts to the clinic:

 

Assign Manual Ability Classification System (MACS)

The MACS was developed to describe how children with cerebral palsy use their hands when handling objects in daily activities. It is based on the child’s self-initiated ability to handle objects & need for assistance or adaptation and classifies both hands together rather than each hand separately. Determination of the level is by asking people who know the child best and not by assessment.

There are five levels:

 

The benefits of classifying children’s function are:

  • Improved communication within the multi-disciplinary team and with parents and carers.
  • It sharpens focus on function rather than severity and type of Cerebral Palsy.
  • It also provides a basic format for better understanding the range of variation in CP for example for students and newly qualified therapists.
  • It can help guide goal setting to be realistic and achievable.

Please note that the MACS is a classification system, not an outcome measure and is therefore not sensitive to changes with intervention.  Evidence suggests that MACS classifications are stable over time i.e. children who have a MACS of 4 at the age of three will continue to have a MACS of 4 throughout their life.

 

Determine Functional Concerns

Discussion with the parent and child to determine concerns and the impact of upper limb disability on the child’s participation in everyday activities, play, leisure and school. For children with some level of independence or hand function the Abilhands-Kids Questionnaire will be conducted. This questionnaire is available for free from Rehabscales.  It is a questionnaire designed for children with Cerebral Palsy to determine if they are independent, have difficulty, or cannot do a range of day to day tasks, such as removing a coin from a pocket, opening a bag of crisps.

For children with more complex needs several questionnaires are being considered: CP-QOL, and the Paediatric Evaluation of Disability Inventory (PEDI).

Range of Motion Measures

Range of motion measures will be taken, following the protocol of the CPUPS. Two therapists, a plinth and appropriately sized goniometers are required. One therapist positions the joint while the other places and reads the goniometer. Shoulder and elbow measurements are taken in supine; with forearm, wrist and finger measurements taken in sitting.


Measurements are then compared to the CPUPS traffic light system to identify if measurements fall into green, amber or red values.
The modified Tardieu score is also taken at the elbow, wrist and long finger flexors to determine if spasticity is present. This assists in identifying children who may benefit from Botulinum Toxin-A injections.

Review Splints and Orthoses

Children who are known to the service, may already have been supplied with splints such as thermoplastic night splints from Orthotics, neoprene functional splints, Lycra garments. Children are requested to bring any splints to the Upper Limb Clinic which provides an opportunity to review the splint, its effectiveness, compliance with wearing regime and fit. Children who have Lycra garments will not be re-measured for their garments during the Upper Limb Clinic but will be asked to bring the garment to check that it continues to meet their needs.

Clinic Outcomes

If the child is all or mostly within green values of the CPUPS traffic light system and there are no functional concerns then no intervention will be provided and the child will be reviewed according to the protocol outlined in Follow up (see below).

If there has been a change in ROM or functional concerns have been identified, the next step will be dependent on the child’s MACS level; children with a MACS 1-3 follow one pathway and children with MACS 4 and 5 follow another, as detailed below.

Intervention - Children with MACS 1-3:

Assessment

Additional assessment may be required to obtain more specific information about the underlying causes of the child’s functional difficulties. A range of assessment tools can be used including:  Assisting Hand Assessment for children with Hemiplegia; Melbourne Assessment; and others.

In order to help the child set realistic and accurate goals, a task analysis of the child attempting or performing the task needs to be made.  For example if the child wishes to be able to tie their shoelaces, observation of the task will allow the therapist to determine if the child has difficulty reaching their laces because of elbow spasticity, if they have poor motor planning, poor hand strength for grasping the laces etc.

Goal Setting

Setting of functional goals that are Specific, Measurable, Agreed, Realistic and
Time based (SMART); the Goal Attainment Scale or other goal setting tool can support writing of goals and the use of goals as an outcome measure.

Referral to Motor Disorders Clinic

If the measurement of ROM and observation of functional performance suggests the presence of spasticity, the child or young person should be referred for upper limb botox.

Evidence in the literature is clear that Botox for the upper limb is most effective when coupled with goal directed therapy. Intervention following Botox should commence 2-4 weeks after the injections are made and should be intense with therapy sessions ideally occurring twice per week for 4 to 6 weeks and with home programme activities carried out by parents or carers.

Treatment

Treatment will follow goal setting, with or without botox.  Treatment modalities can include: Bimanual therapy ; goal directed therapy (i.e. practising the skills that the child and parent have identified as being the goal); modified constraint induced therapy ; strength training (see below for definitions of the treatment modalities).

Therapy may be provided intensely or through the provision of home programmes. Therapy sessions may be carried out by Band 3 and 4 staff with the named therapist overseeing the activities and progress.

Splinting should also be considered when spasticity is present and there is a risk of loss of range of motion.  Splinting should include a referral to orthotics for a night splint that provides stretch to the appropriate joints. A ‘night resting splint’ is not recommended as it may not maintain the long finger flexors and thumb sufficiently stretched to reduce loss of range.

Functional splints can provide stabilisation to the joints, particularly the thumb and wrist to allow a more optimal position for function. Functional splints include off the shelf neoprene splints e.g. thumb spica; custom made Lycra gloves and gauntlets; or kinesiotaping.

Intervention - Children with MACS 4-5

Goal Setting

Goal setting with the child and parent/carer. Goals may be around ease of dressing, washing the palms of the hands.

Referral to Motor Disorders Clinic

Where the ROM values are in the amber or red zone or where the presence of spasticity impacts on functional tasks the child should be referred to his or her community paediatrician for review of tone medication. If this has happened recently, referral should be made to the Complex Motor Disorders clinic for tone management. Intervention at this level may include Botox. Therapy post Botox for this group does not require the same level of therapy intervention, however goals are still essential and a treatment plan in place to ensure the goals can be met and the benefit of the Botox maximised.

Treatment

As for children who have a MACS 1-3, children with MACS 4 & 5 should be referred to Orthotics for night splints that provide stretch to fingers, thumb and wrist. Where children have no active hand function, it may be appropriate for them to wear thermoplastic splints during the day.

Parents and carers should be provided with strategies and advice to support them in the achievement of functional goals. This may include advice for positioning the child to support dressing or bathing, or how to move the child’s arm to minimise the impact of spasticity or increased tone.

Children may require a stretching programme to increase and maintain range of motion.

Follow up

The NICE Guidelines recommend ongoing monitoring of spasticity (or abnormal tone). The CPUPS guidelines ensure that children are seen on at least an annual basis throughout childhood so that changes in joint range of motion or function can be picked up quickly and intervention provided in a timely fashion.

As per the CPUPS guidelines, children on the Upper Limb Pathway will be monitored as follows:

  • Children aged 0-5: Every six months (although children with a MACS of 1 and GMFCS of 1, may be seen annually at the discretion of the therapist).
  • Children aged 6-18: Annually.

Once an episode goal has been completed, and if no further concerns or goals are presented, the child will be moved from the named therapist’s active caseload to be held at Team Level on EMIS Web awaiting review.

Interventions

Bimanual Therapy

An intensive intervention for children with hemiplegic cerebral palsy. It uses carefully planned, repeated practice of two-handed, or bimanual, games and activities to improve a child’s ability to use their hands together. Appropriate for all ages who have some ability to use their affected arm and hand.

Children with less ability may be encouraged to use their arm/hand to stabilise objects using bimanual activities. More able children may be encouraged to develop grasp, release and manipulation.

Constraint Induced Movement Therapy (CIMT)

Constraint of the non-affected upper limb to encourage use of the affected arm/hand. It must be combined with intensive skills training.

A component of upper limb motor impairment is learnt and can therefore be reversed. CIMT creates the opportunity for the child to learn how to use their hemiplegic limb.  Constraint is achieved through a splint placed over the non-affected hand. Intensive skills training is provided using activities that are achievable using the affected hand.

Multiple models available and choice of model may be dependent on age:

  • <4 years - distributed practice (8-10 wks, 1-2 hours/day), home/pre-school based, individual.
  • >4 years - intensive (2-3 weeks), camps, group based.

Highly variable individual response to treatment. Children who make the least improvement tend to have significant cognitive impairment or very dense hemiplegia.

Hybrid CIMT - combines CIMT with bimanual therapy.

Strength Training

Muscle weakness of the shoulder, arm and hand is a known contributing factor in difficulties with functional tasks. Strength training can increase muscle strength and improve functional performance. Strength training does not increase spasticity.

The resistance, number of repetitions and how resistance and repetitions are progressed will be tailored to each individual according to established strength training principles.

It has been found that strength gains achieved in a program of at least eight weeks will be lost approximately six weeks after training is discontinued. Therefore, for strength gains to be maintained beyond the program, the client will need to continue their strength training through a maintenance program.

Appropriate for children over 7 with MACS I-V.