Table 4.

Recommendations for Screening, Assessment, and Management of Pain in Children With a Leukodystrophy.

	Presymptomatic	Early Symptomatic	Intermediate Symptomatic	Advanced Symptomatic
Screening47-50	Birth to 3 y: FLACC (Faces, Legs, Activity, Cry, and Consolability) 4-12 y: FPS-R (Faces Pain Scale–Revised) and WBFPRS (Wong-Baker Faces Pain Rating Scale) 8 y and older: NRS (numeric rating scale)		r-FLACC (Revised Faces, Legs, Activity, Cry, and Consolability)51
Assessment Common etiologies to consider	Acute injury Infection Migraine Teething/oral discomfort/dental pain12,52	Disease-specific etiologies: Neuropathy in metachromatic leukodystrophy, Krabbe53,54 Gallbladder pathologies in metachromatic leukodystrophy55 Reflux in infantile Krabbe56	Reflux56 Spasticity, dystonia5,54,57 Contractures6 Scoliosis6 Joint dislocation6 Pressure injuries6 Compression fractures58 Constipation, feeding intolerance,60 Urinary retention6 Urinary tract infections6 Corneal abrasions5
Tools	History and physical exam consistent with applicable common etiologies, diagnostic workup as appropriate
Management  Nonpharmacologic  Pharmacologic	Targeted treatment of underlying pain causes Play/music therapy61 Guided imagery61 Cognitive behavioral therapy		Targeted treatment of underlying pain causes Therapeutic massage, application of heat or cold, Reiki61 Physical/occupational therapies61 Rocking, repositioning, music, lighting, warm water, vibration
	NSAIDs and acetaminophen46		NSAIDs and acetaminophen46 Gabapentin62 Consideration of opioids46 Spasticity management (eg, benzodiazepines, antispasmodics)6