Common chronic paediatric conditions

Essentials

1
Children with chronic medical conditions make up 10–20% of paediatric emergency department (ED) presentations.
2
These patients have a longer ED stay and have increased rates of hospital and intensive care unit admission.
3
Liaise with the medical teams who know these patients well.
4
Trust the parents/caregivers: if they are worried, you should be worried.
5
Minimise pain and anxiety associated with procedures.

Introduction

Chronic health conditions are defined as those expected to last more than 12 months and cause functional limitation for the child or adolescent and/or require ongoing medical care. They include both chronic illness (such as asthma or diabetes mellitus) and chronic physical disability (such as cerebral palsy, hearing or visual impairments). Paediatric patients with chronic medical conditions ( Table 1.2.1 ) make up 10–20% of presentations to tertiary paediatric emergency departments (EDs). ^, Compared with children without chronic medical conditions, these patients have a longer ED stay and are more likely to be admitted to both the ward and the paediatric intensive care unit (PICU). It is therefore essential that the emergency physician is equipped to deal with this potentially complex group of patents.

Table 1.2.1

Common chronic health conditions in childhood

Neurological	Epilepsy Cerebral palsy Ventriculoperitoneal shunt Spina bifida
Cardiac	Congenital heart disease Arrhythmias
Respiratory	Asthma Cystic fibrosis Bronchopulmonary dysplasia
Renal/urological	Vesicoureteric reflux Chronic renal insufficiency Nephrotic syndrome
Haematooncological	Haemoglobinopathies Coagulation disorders Cancer Immunodeficiency
Endocrine	Diabetes mellitus Obesity
Developmental	Autism Attention deficit/hyperactivity disorder
Other	Psychiatric disorders Gastroenterological problems Ear, nose and throat problems

The emergency physician may be involved in the initial diagnosis but is mostly required to recognise and manage disease complications and acute illness unrelated to the chronic condition.

This group of patients can be very complex, with numerous medical and psychosocial issues and a baseline abnormal examination. It can be extremely difficult for the emergency physician, in a time-pressured ED scenario, to gain a thorough understanding of the patient and the level of deterioration. It is very important to listen carefully to the parent or caregiver’s concerns, as well as involve the patient’s primary care team where possible. The parents are often well placed to advocate for their child, and they usually know the limitations to home management for their child.

Chronic illness brings with it a range of stressors for the child and family, including those surrounding painful procedures such as venous access. As with any child, but especially in this population who are likely to be subjected to multiple procedures over time, it is critical to minimise the trauma surrounding potentially painful procedures. Tools used may include distraction, parental presence, play therapy, positioning on a parent, topical anaesthesia, pharmaceutical analgesia or procedural sedation. In the appropriate clinical setting, oral midazolam 0.5 mg/kg up to 20 mg can be mixed with sucrose and administered 20–30 minutes prior to attempts at intravenous access. Inadequate pain management is known to have long-term negative effects on children, including diminishing effects of adequate analgesia for subsequent procedures or needle phobia, which make future procedures more traumatic for the child and more difficult for the clinician. ^,

This chapter will provide an overview of some of the more commonly encountered chronic paediatric conditions, including cerebral palsy (CP), spina bifida, cystic fibrosis (CF), the ex-premature infant and autism spectrum disorder.

Cerebral palsy

Introduction

CP is not a single entity but rather a heterogenous collection of clinical syndromes characterised by abnormal motor patterns and postures ( Table 1.2.2 ). It is the most common chronic motor disability of children, with a prevalence of approximately 1/500 live births. CP describes children who have a nonprogressive brain injury or lesion caused by an insult to the developing brain during the antenatal, perinatal or early postnatal period. The degree of motor function may vary widely and can be communicated quickly using the gross motor function classification scale ( Table 1.2.3 ). Children may have associated impairments including epilepsy, or intellectual, speech, visual or hearing impairment. It is important to remember that many children with CP have normal cognition.

Table 1.2.2

Cerebral palsy motor syndromes

Spastic	Most common type Hyperreflexia, weakness, hypertonia May be a: - hemiplegia: unilateral arm and leg involved - diplegia: both legs involved - quadriplegia: all limbs and often trunk and facial muscles involved
Dyskinetic	Athetotic: involuntary, slow, writhing movements
Dyskinetic	Dystonic: trunk movements are more affected than limb muscles, resulting in a twisted position
Ataxic	Voluntary movements are not well coordinated
Hypotonic	Muscle tone is decreased
Mixed	A combination of the above

Table 1.2.3

Gross motor function classification scale (GMFCS)

I	Can run, jump and climb stairs, but speed, balance or coordination is impaired
II	Can walk, run and climb in most settings but may need mobility equipment for safety or travelling long distances
III	Can walk using assistance and can self-propel manual or powered wheelchairs
IV	Capable of walking with a walking frame but mostly relies on wheeled mobility
V	Little or no voluntary control of movement and requires extensive support in all situations

Complications seen in the emergency department

Children with CP are susceptible to illness because of their increased risk of epilepsy, respiratory complications and feeding difficulties, as well as complications associated with increased technology dependence. Increasing CP severity and complexity are associated with increased frequency of presentations, higher urgency of presentation and higher rates of hospital admission. The majority of presentations to the ED are due to respiratory or neurological causes, followed by gastrointestinal and musculoskeletal complications. ^,

Respiratory complications

Respiratory illness is the leading cause of mortality in individuals with CP, and lower respiratory tract infections are the most common reason for children with CP to require hospital admission.

History

Predisposing factors include:

Oropharyngeal motor incoordination (leading to aspiration of food and secretions)
Gastroesophageal reflux
Poor cough and airway clearance
Immobility
Kyphoscoliosis
Sleep apnoea
Malnutrition, leading to atrophy and weakness of respiratory muscles

Causative organisms of pneumonia are similar to other children, though anaerobes may also be involved in the setting of possible aspiration. Risk factors for serious illness include previous PICU admission and severe scoliosis.

Examination and investigation

Assessment is similar to other children with respiratory illness:

Respiratory distress (respiratory rate, intercostal/subcostal recession, grunting)
Oxygen requirement
Respiratory failure (conscious state, pH and CO ₂ on venous blood gas)
Wheeze and bronchodilator responsiveness

Note that chest X-rays will be difficult to interpret in those with severe scoliosis.

Management

Oxygen and respiratory support

Give oxygen if the saturations are <90% in room air
If the child has chronic respiratory failure, aim for saturations 90–95%
Consider respiratory and airway support as necessary.

Antibiotics

Follow local guidelines for community-acquired pneumonia
Have a lower threshold to start antibiotics in children with severe CP; these children are more likely to have complications of pneumonia and worse outcomes
Penicillin is thought to provide adequate anaerobic cover for aspiration pneumonia
If very unwell, consider adding Gram-negative cover.

Salbutamol

Use if there is evidence of bronchospasm.

Secretion management

Nebulised saline may help to mobilise secretions (5 mL normal saline via nebuliser)
Oropharyngeal suctioning if the child is unable to swallow secretions
Chest physiotherapy may be helpful in those with poor cough

Feeding

If moderate or severe respiratory distress give intravenous (IV) fluids at two-thirds maintenance
If respiratory distress is less severe and the child is percutaneous endoscopic gastrostomy (PEG) fed, continuous feeds at two-thirds maintenance may be appropriate.

Consider PICU referral if severely unwell, as per local guidelines. Remember to take account of the child’s prognosis, level of understanding, quality of life and advanced care directives if available when formulating a management plan.

Neurological complications

Seizures

Up to 50% of children with CP will have epilepsy, and increased seizures or status epilepticus are common ED presentations. It is important to consider possible precipitants such as intercurrent illness, CNS infection, change in medication, poor compliance or ventriculoperitoneal shunt malfunction if present. Management of afebrile seizures is discussed in Chapter 8.3 .

Cerebrospinal fluid shunt complications

Children with CP are more likely to have cerebrospinal fluid (CSF) shunts in situ. The assessment and management of shunt complications is discussed in Chapter 8.1 .

Gastrointestinal complications

Percutaneous endoscopic gastrostomy tube complications

Many children with moderate or severe CP will require PEG placement due to malnutrition or recurrent aspiration secondary to oromotor or oesophageal dysphagia. Gastroesophageal reflux disease (GORD) is also common in this patient population and if severe may require fundoplication in addition to PEG placement. Common PEG complications seen in the ED include infection, leakage and inadvertent tube removal. An approach to these complications is discussed in Chapter 29.16 .

Constipation

Chronic constipation is a common complication of CP. Contributing factors include reduced fibre and fluid intake, reduced mobility and difficulty achieving optimal toileting posture, and prescription of anticholinergic or opiate drugs which increase transit time. Management of constipation is detailed in Chapter 7.13 .

Musculoskeletal complications

Musculoskeletal pathologies tend to be progressive in children with CP. Spasticity is common, affecting approximately 85% of children with CP to varying degrees. Long-term management goals are to reduce contractures, therefore preserving function and reducing pain. Management of spasticity includes physiotherapy and splinting, as well as medical management, with surgery reserved for severe cases. Hip dislocations and pathological fractures are more common in this population and must be considered in the patient who presents with irritability with no other cause found.

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