Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Diagnosis and Management in Young People: A Primer

Abstract

Myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) is a complex disease that affects children and adolescents as well as adults. The etiology has not been established. While many pediatricians and other health-care providers are aware of ME/CFS, they often lack essential knowledge that is necessary for diagnosis and treatment. Many young patients experience symptoms for years before receiving a diagnosis. This primer, written by the International Writing Group for Pediatric ME/CFS, provides information necessary to understand, diagnose, and manage the symptoms of ME/CFS in children and adolescents. ME/CFS is characterized by overwhelming fatigue with a substantial loss of physical and mental stamina. Cardinal features are malaise and a worsening of symptoms following minimal physical or mental exertion. These post-exertional symptoms can persist for hours, days, or weeks and are not relieved by rest or sleep. Other symptoms include cognitive problems, unrefreshing or disturbed sleep, generalized or localized pain, lightheadedness, and additional symptoms in multiple organ systems. While some young patients can attend school, on a full or part-time basis, many others are wheelchair dependent, housebound, or bedbound. Prevalence estimates for pediatric ME/CFS vary from 0.1 to 0.5%. Because there is no diagnostic test for ME/CFS, diagnosis is purely clinical, based on the history and the exclusion of other fatiguing illnesses by physical examination and medical testing. Co-existing medical conditions including orthostatic intolerance (OI) are common. Successful management is based on determining the optimum balance of rest and activity to help prevent post-exertional symptom worsening. Medications are helpful to treat pain, insomnia, OI and other symptoms. The published literature on ME/CFS and specifically that describing the diagnosis and management of pediatric ME/CFS is very limited. Where published studies are lacking, recommendations are based on the clinical observations and practices of the authors.

Keywords: adolescent; chronic fatigue syndrome; diagnosis; joint hypermobility; management; myalgic encephalomyelitis; pediatric; postural tachycardia syndrome.

Figure A1

The three main pathophysiologic influences on orthostatic intolerance (OI) are shown in this Figure. Increased pooling of blood, or defective vasoconstriction in the lower half of the body, along with lower intravascular volume are each important, and can be present in the same individual. Upon assumption of an upright posture, exaggerated sympathetic nervous system and adrenal hormone responses lead to increased release of epinephrine (Epi) and norepinephrine (NE). It is postulated that the relative balance of NE to Epi is one factor in determining whether the phenotype of OI is POTS or neurally mediated hypotension (NMH). Some patients with POTS in the early phase of orthostatic testing go on to develop a classic NMH pattern later in the test.