Protecting the bones and muscles of children with cerebral palsy
9 September 2019
As part of ‘Steptember’, the fund-raising month for people with cerebral palsy, three researchers from the Auckland Bioengineering Institute (ABI) will talk about their potentially life-changing research into the disease.
Cerebral palsy is a movement disorder caused by damage to parts of the brain before, during and after childbirth, that affect a child’s ability to control movement, balance and posture. It is the leading cause of childhood disability in New Zealand.
It is not a progressive neural disease – the lesions that cause the disease are static – yet paradoxically the musculoskeletal systems of children worsen over time.
Using a combination of imaging techniques and computational modelling, researchers at ABI hope to better understand how, and why.
That includes a project led by Dr Geoffrey Handsfield, who was awarded a $1 million Aotearoa Foundation Fellowship. “Broadly speaking, the goal is to better understand the progression of cerebral palsy in the musculoskeletal system, particularly how the disease worsens over time and impairs muscles, bones and walking ability,” he says.
His team includes Stephanie Khuu, a physiologist now doing a PhD in Bioengineering, who is investigating why the muscles of children don’t regenerate after exercise.
“Typically, when children exercise their muscles, the muscle is injured, but then it regenerates. Our muscles do that on a day by day basis.” Damaging our muscles can even produce physiological adaptations that actually improve performance over time, similar to the improvements you see after ‘damaging’ your muscles in the gym by working out.
“But we think that with CP something is going wrong that doesn’t allow them to regenerate, and so over time, they progressively degenerate.”
Cerebral palsy is not a progressive neural disease – the lesions that cause the disease are static – yet paradoxically the musculoskeletal systems of children with the disorder worsen over time.
Understanding why things go wrong requires understanding why things go right. Which is why Ms Khuu is investigating the process of muscle regeneration in both typically developing muscles and in the muscles of individuals with cerebral palsy. Building a computational model of the environment in which muscles regenerate in normal circumstances will help clarify what hampers those regeneration pathways in children with cerebral palsy.
There are some possible culprits, says Ms Khuu. For example, the muscles of individuals with cerebral palsy have been found to have high levels of collagen, which explains why the muscles are rigid. Levels of satellite cells, the progenitor cells needed for muscle fibres to regenerate when they have been damaged, are low.
The problem is unlikely to be about one particular type of cell, but a complex interaction of myriad cells. “We want to be able to pinpoint those differences down to specific mechanisms or pathways.”
She has identified a number of different cell types which she will use as agents and, using computational modelling, programme them to behave as they would in a normal physiology. This will allow her to compare that to the muscle environment of those with cerebral palsy. A deeper understanding of the composition of the muscle environment in those with cerebral palsy, at a molecular and cellular level, could open up the potential for therapeutic interventions.
Dr Julie Choisne, also from the ABI, will talk about her project funded by the Health Research Council, looking at bone deformation in children with cerebral palsy.
Children with cerebral palsy typically walk in a way that compensates for their abnormal muscle activity, in a way that is most efficient for them but is not good for their bones,” says Dr Choisne. “The mechanical load on the foot, for example, is different from what it should be, which deforms the bones’ alignment in the ankle.”
Dr Choisne is drawing on the CT scans of 200 children aged four to 18 years old, sourced through the Victorian Institute of Forensic Medicine, to build a paediatric population-based atlas of the normal development of the tibia, femur and pelvis bones. She will use that data to build a computational model of both normal and abnormal development.
By combining that model with wearable sensors developed at the ABI, she hopes this will allow for a quick and inexpensive assessment of a child’s gait, its potential impact on their musculoskeletal system, and what interventions may help. If specialists could assess a child as early as possible, teach and help them walk better early on in their lives, “they might not even need an assistive device to walk when they get older, and most of them won’t end up in a wheelchair.”
Stephanie Khuu, Dr Geoffrey Handsfield and Dr Julie Choisne will present their research at A Brighter Future for Children with Cerebral Palsy, Monday, 16 September University of Auckland, Grafton Campus
The event is free, but people are advised to register with Eventbrite
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