Logan is a 6yo who is presented to ED by his mother as “just not himself”.
Logan is well known to your local paediatric team for managing his GMFCS 5 spastic quadriplegic cerebral palsy. He has many comorbidities, frequent hospital attendance and multiple unplanned admissions for aspiration pneumonia, seizures or irritability. cause.
Tonight is no different.
Logan’s mother describes that today was no different to any other afternoon in their household and patiently recounts that, between his evening meal and bedtime, Logan became “not his usual self” and was moaning more and crying. There have been no other infectious symptoms, specifically no fever, although Logan’s 4-year-old sister has a URTI.
You search through the medical notes for a list of medical issues and diagnoses, and it looks something like this:
Cerebral Palsy secondary to neonatal Hypoxic Ischaemic Encephalopathy
GMFCS 5
Cortical blindness
Developmental impairment
PEG fed
Fundoplication
Scoliosis
Aspiration pneumonia
Seizures on multiple agents
Intrathecal baclofen pump
Multiple tenotomies
Failure to thrive
Dependent for all cares
Hip subluxation
Family breakdown
Polypharmacy
Iron deficiency
Constipation
Multiple urinary tract infections
Eczema
Previous pressure wounds
Patients like Logan have high care needs. As the acute care paediatric doctor, how can we best care for Logan?
There are several types of cerebral palsy. A thorough description of these is beyond the scope of this article. Indeed, the goals for the management of the child with CP include the following: to promote optimal function; to maintain general health; to foster the acquisition of new skills; to assist and educate parents and caregivers; and to anticipate, prevent, and treat the complications of this disorder. GMFCS is a severity classification system, with some further details below. Rather, this article will propose a framework by which the acute care paediatrician may approach the profoundly neurodevelopmentally impaired child. Frameworks aside, we strongly encourage you to understand these young people in terms of what they CAN do and their roles within their family and society.
So, a framework by which to approach the child with GMFCS5 cerebral palsy,
A) presenting with irritability? and
B) the complications of cerebral palsy?
Once adjusted for acuity, there is a reasonable argument to utilise the same framework for both questions. Before we get to the framework, some prognostic indicators for young people like Logan;
Most patients who can sit independently at 2 will walk.
Young people who cannot sit at 4 are unlikely to walk.
A young person who cannot lift their head to prone will likely require nasogastric or PEG feeds.
Median survival for this cohort of kids is 17 years.
For many young people with this constellation of pathology, their clinical course is marked by stepwise deterioration often centred around a severe respiratory illness. As this is a common mode of death for these patients, there may be a profound parental concern that this illness is “the one”.
An approach to the child whom has cerebral palsy and is irritable?
An approach to complications of cerebral palsy?
Musculoskeletal
Hip dislocation – altered stability of hip &pelvis, commonly occurs in UMN patients.
Pathologic fracture – risks include osteopenia of chronic disease, effectively a sedentary lifestyle & anticonvulsant.
Spasticity – May be increased due to pain or the cause of pain outright.
Baclofen Pump dysfunction – may present as irritability.
Scoliosis – due to truncal spasticity, poor muscle control & weakness.
Neurodevelopmental
Seizures – common in Cerebral palsy, most frequently relapsing, remitting clusters of seizures.
Baseline cerebral irritability – CP can be conceptualised as an encephalopathic process.
Ventriculoperitoneal shunt dysfunction is also Occult hydrocephalus in children with cerebral palsy.
Meningitis – usual baseline paediatric risk, plus increased if shunt, other risks
NAI risk – is under-reported in this patient population
Environmental change – exquisite environmental sensitivity, potentially due to reduced cognitive capacity, other UMN symptoms may preclude robust assessment.
Foregut and Lung (and other Gastrointestinal)
Oral cavity – Dental pain & cavities. It is exacerbated by bruxism, spasticity, and reduced solid diet.
URTI(!) – common things are common, ala Hickam’s Dictum!
Aspiration pneumonia –
Intra-abdominal sepsis – As noted Paediatric Watch
Constipation – spastic bowel leads to increased transit time.
Special senses
An interesting overview of irritability and pain in the severely neurologically impaired child, listing several of the below pathologies;
Ocular trauma
Otitis Media – may affect hearing long term.
Gynae & Genital
Skin & pressure areas
Urinary retention & infections – voiding dysfunction is identified in most children with CP
Infections
Of course, this is not an exhaustive list of the potential complications for a child with GMFCS 5 Cerebral palsy. We hope this framework is useful for the practitioner caring for these young people in acute and chronic settings.
What is the GMFCS?
Also of interest may be the Communication Functioning Classification System (CFCS);
Other resources
https://file.lacounty.gov/SDSInter/dhs/218115_RLOCFOriginalFamilyGuide-English.pdf
https://onlinelibrary.wiley.com/doi/full/10.1111/dmcn.13328
https://www.rch.org.au/clinicalguide/guideline_index/Cerebral_Palsy_Pain_and_Irritability/
https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1469-8749.1997.tb07414.x
Burns, Fiona & Stewart, Robbie & Reddihough, Dinah & Scheinberg, Adam & Ooi, Kathleen & Graham, Kerr. (2014). The cerebral palsy transition clinic: administrative chore, clinical responsibility, or opportunity for audit and clinical research?. Journal of Children’s Orthopaedics. 8. 10.1007/s11832-014-0569-0.
