Comprehensive care of the ambulatory child with cerebral palsy (GMFCS I and II): A Canadian perspective

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Background

The term ‘cerebral palsy’ (CP) refers to a heterogeneous group of non-progressive neurodevelopmental conditions characterized by impairments in motor function that limit activity and are caused by a disturbance to the developing fetal or infant brain ^[1]. Many individuals with CP have co-occurring conditions, such as seizures, vision or hearing impairments, cognitive, communication, or behaviour challenges, or secondary musculoskeletal problems ^[1]. There are numerous ways to classify and describe CP, such as by limb distribution (topography) ^[2], hypertonia or movement disorder type ^[3], or function ^[4].

The Gross Motor Function Classification System (GMFCS) ^[4], which was developed in Canada but is used internationally, is a 5-level classification system for CP that describes motor performance in everyday life. Distinctions between levels are based on an individual's gross motor functional abilities and needs for assistive equipment. The GMFCS has strong inter-rater reliability, is valid in discriminating levels of functioning, and is easy to use. It is validated as a predictive tool for future gross motor function, which is helpful for prognostic discussions with families, goal-setting, and intervention planning ^[5]. Children classified as GMFCS level I can walk without limitations and only experience difficulties with speed, balance, and coordinating more advanced motor skills. Children classified as level II walk well on flat and familiar surfaces but require support when navigating uneven surfaces, crowded areas, and long distances. In a Canadian population registry, over 60% of children with CP were found to fall into GMFCS levels I and II ^[6].

Within GMFCS levels I and II, the two most common CP subtypes are spastic unilateral or hemiplegic CP, typically caused by a stroke or cerebral malformation, and spastic bilateral CP, which is often seen in children born preterm and is typically caused by periventricular white matter injury ^[7]. Severe intraventricular hemorrhage is another common etiology of CP in children born preterm, but this injury can lead to unilateral or bilateral sequelae. Common early warning signs that should trigger further evaluation for CP include: 1) Hand preference before 12 months; 2) Stiffness or tightness in the legs before 12 months; 3) Inability to sit by 9 months; 4) Persistent fisting of hands beyond 4 months; and 5) Delays or asymmetry in movement or posture ^[8]. While CP is a clinical diagnosis, practice guidelines recommend (when feasible) confirmation of an injury or brain malformation using magnetic resonance imaging (MRI) ^[9].

Health and medical care

Many children with CP functioning at GMFCS levels I and II are appropriately cared for within the ‘medical home’ established by their community paediatrician. When questions arise around diagnosis and treatment, multidisciplinary CP clinics may be available locally for consultation. As a general rule, risk for medical comorbidity increases with GMFCS level, and also relates to the underlying timing and extent of brain injury. A recommended list of medical comorbidities to monitor for with children who have CP is provided in the “Health” section of Table 1: the CP Health and Wellness Record. ‘Clinical pearls’ for care of children functioning at GMFCS levels I and II follow here.

Pain

One-third of ambulatory children with CP are reported to be limited by pain ^[10]. Common causes of pain include increased muscle tone, muscle fatigue or weakness, ill-fitting orthotic treatments (e.g., braces or casting), as well as gastrointestinal causes that are common in the general population (e.g., gastroesophageal reflux disease (GERD), or constipation). Paediatricians should ask children and parents directly about pain during clinical visits, and screen for potential sources when needed ^[11][12].

Seizures

Epilepsy occurs in 1 of every 4 children with CP, and may occur at any GMFCS level ^[13]. Children with spastic unilateral CP due to stroke or cerebral malformation are particularly vulnerable to seizures, whereas in children with spastic bilateral CP from periventricular leukomalacia (PVL), the cortex is often relatively spared, such that seizures develop less frequently. The side effects anti-epileptic medications, such as sedation affecting learning and behaviour during the day, may further compound the developmental effects of seizures that are not well controlled.

Nutrition and feeding

Children functioning at GMFCS levels I and II have fewer oral motor feeding challenges than children with more extensive motor impairment, but may be at risk for overweight due to decreased physical activity. They are also at increased risk for developing cardiovascular and metabolic diseases in adulthood ^[14].

Vision

Children with CP commonly present with refractive errors, strabismus, and visual field loss reflecting the patterns of cortical or white matter damage. For example, inferior field defects in PVL, and homonymous hemianopsia in hemiplegia, may lead to tripping and falls that can be mistakenly over-attributed to motor impairment.

Hip surveillance

When children experience delayed weight-bearing, which alters muscle balance around the hip, the hip joint can develop signs of subluxation or dislocation. Hip surveillance should include clinical examination (e.g., asking about and examining for hip pain, and measuring range of motion) and a radiographic anteroposterior view of the hips, because displacement can occur without signs or symptoms. Children functioning at a GMFCS level I have the lowest rate of hip displacement and therefore do not require hip x-rays. Children functioning at GMFCS level II, or those with hemiplegia who walk with one hip adducted and internally rotated (called a Winters, Gage, and Hicks Type IV gait pattern) require monitoring for hip displacement. Evidence-informed hip surveillance care pathways for children with CP are available for reference ^[15].

Scoliosis

Children with unilateral CP are at risk for scoliosis due to muscular imbalance during rapid growth periods, such as puberty. This condition is usually mild but requires monitoring. Should adolescents with CP develop scoliosis, surveillance for hip subluxation may need to be reinstated.

Hypertonia

Hypertonia can be treated using a variety of management options and requires consideration of child and family goals, the anatomical location of the hypertonia, as well as functional classification. Indications to refer for further management with oral medications or botulinum toxin A injections include hypertonia that limits function or participation (e.g., causes tripping and falling, affects gait, or limits use of hands), causes pain, or impacts caregiving or self-care (e.g., dressing or hygiene).

Developmental surveillance

Children functioning at GMFCS levels I and II often have challenges with balance and motor coordination. Therefore, safety in the home and school environments should be screened. Gathering information on the child’s stability on stairs and uneven surfaces (e.g., on grass or in the playground) and ability to manoeuvre in busy environments (e.g., hallways and crowded spaces), is valuable for alerting the child’s care setting or school to potential falls. Use of an ankle-foot orthosis (AFO) is recommended, not only for increasing stability but also to stretch hypertonic calf muscles while the child walks and plays ^[16]. Often, however, children of school age do not wear their AFO, either because they require help with donning them at school or because they are self-conscious about how they look. Support from teachers and staff at school can help overcome these barriers.

Upper extremity involvement due to hypertonia, weakness, and impaired sensation of the hand and arm affect activities of daily living such as dressing (e.g., navigating buttons and zippers), eating (using a fork and knife), and handwriting. Assessing bimanual hand function (e.g., the ability to open a water bottle, uncap a marker, or start a zipper) can help determine whether modified constraint therapy ^[17] or hand-arm bimanual therapy ^[18] could improve function.

There is a common misconception that all children with CP require active therapy throughout their lives. Goal-directed, activity-based therapy is much more appropriate and effective, particularly when children reach school age, because it allows individuals to participate in their own way and promotes healthy, active living ^[16]. Focusing on participation is more important than engaging in therapy to promote ‘normal’ function.

Children with CP have an increased prevalence (of an estimated 8.7%) for autism spectrum disorder (ASD), compared with the general population ^[19]. Diagnosis of ASD is often delayed, perhaps due in part to typical early warning signs (e.g., reduced eye contact, lack of pointing, and impaired joint attention) being affected by motor impairments in CP, or to social communication concerns being overlooked compared with motor concerns ^[20]. A strong index of suspicion for ASD in this population is required for timely diagnosis, and early referral to a developmental specialist, particularly when a child’s presentation is complex, may be needed.

School age children with CP are at increased risk for difficulties with attention and impulsivity, language development and articulation, and learning, which can affect academic performance and school life. Psychoeducational assessments can clarify a child’s learning profile and assist with accessing appropriate school supports and targeted placements, as well as with planning for school transitions and accessing provincial/territorial disability benefits later on.

Wellness and participation

When caring for children with life-altering conditions such as CP, focus on the six ‘F-words’ in childhood disability is key: function, family, fitness, fun, friends, and future ^[21]. Promoting family and child involvement in recreational and community activities, and developing connections with inclusive local programs can build confidence and individual ability while enhancing well-being, both socially and emotionally. Providing such opportunities is important for mitigating the social obstacles, behavioural challenges, and mood disorders that affect children and youth with CP more often than their typically developing peers ^[22].

Extending the concept of wellness to include the child’s whole family can improve outcomes ^[23]. Health care advocacy, and having access to community-based services and supports, can assist with family-centred goal-setting and achievement while building capacity for self-advocacy. The caregivers of children with CP experience some unique stressors and they may also contend with physical and mental health challenges, particularly when children have difficult behaviours or low functional abilities ^[24][25]. Health care providers who can identify such challenges early and refer those experiencing them for family-centred interventions can help reduce parental stress significantly ^[26].

Summary

In Canada, children with CP who function at GMFCS levels I and II are commonly followed by paediatricians. This practice point and the accompanying CP Health and Wellness Record provide a focus for clinical care by describing the specific needs of children and families, offering a framework for monitoring development, and suggesting strategies for optimal health care, well-being, daily function, and participation in everyday life.

Acknowledgements

This practice point has been reviewed by the Community Paediatrics and Fetus and Newborn Committees of the Canadian Paediatric Society.

CANADIAN PAEDIATRIC SOCIETY DEVELOPMENTAL PAEDIATRICS SECTION
Executive members: Melanie Penner MD (President), Sabrina Eliason MD (Vice President), Anne Kawamura MD (past President), Angela Orsino MD (Secretary-Treasurer), Angie Ip MD (Member at large), Elizabeth Mickelson MD (Member at large)
Liaisons: Allison Lamond MD (CPS Residents Section)
Principal authors: Anne Kawamura MD, Amber Makino MD, Scott McLeod MD

References

Disclaimer: The recommendations in this position statement do not indicate an exclusive course of treatment or procedure to be followed. Variations, taking into account individual circumstances, may be appropriate. Internet addresses are current at time of publication.