persistent difficulties with social communication and social interaction, and
restricted and repetitive patterns of behaviours, activities or interests (this includes sensory behaviour),
present since early childhood,
to the extent that these limit and impair everyday functioning
Sensory behaviour may be meltdown or withdrawal or other challenging behaviour when too much information or sensation is experienced. There can be hyper (or hypo) sensitivity to lighting, problems with depth perception, noises or crowds, smells (or licking), pain, taste/textures.
SIGN guidance is that (145):
children under 3 with regression in language or social skills should be referred
not to screen population
that screening instruments are not 100% reliable but have their uses
that ASD should be considered in any child with developmental, emotional, psychiatric or behaviour issues, or a genetic syndrome
in preschool children typical features may be absent
gender differences are important in terms of symptoms and level of impairment
Assessment
Lack of shared attention (or late development) eg pointing
repetitive behaviour/play
resistance to change
violent or self injurious behaviour, pica
Types
Aspergers – social difficulties in absence of learning or communication problems
Pathological demand avoidance – where underlying problem is high level of anxiety about conforming to social demands or not being in control
Therapy based on physical exercise should NOT be offered “as a cure”, nor should graded exercise programmes (which by definition use fixed increments in exercise) be used!
Instead, self management, flexible and tailored
CBT should only be offered to manage symptoms, improve functioning and reduce distress.
Talks about “energy management” – includes emotional, social, cognitive.
“Care and support plan” – physical activity including mobility but also activities of daily living. Plan periods of rest and activity, and incorporate the need for pre-emptive rest. Management of relapses and flares.
Main thrust of update is that CFS/ME is a complex, chronic medical condition affecting multiple body systems and its pathophysiology is still being investigated. It affects everyone differently and its impact varies widely – for some people symptoms still allow them to carry out some activities, whereas for others they cause substantial incapacity. It is a fluctuating condition in which a person’s symptoms can change unpredictably in nature and severity over a day, week or longer.
Often it profoundly affects different aspects of the lives of both people with ME/CFS and their families/carers including social life, emotional wellbeing and education.
Another big theme is prejudice, disbelief and stigma experienced by patients.
US IOM expert panel have rejected the name “chronic fatigue syndrome”, as patients hate it! Myalgic encephalitis (ME) also rejected on basis of insufficient evidence that this is the pathological process. They suggest “Systemic exertion intolerance”, which is probably even more rubbish, in my opinion.
Diagnostic criteria: all of the following 3 [BMJ2015; 350]
Substantial reduction/impairment in pre-illness levels of activity, that persists for more than six months [NICE 2007 says 3/12 for children], and accompanied by fatigue (often profound, new or definite onset, not the result of ongoing excessive exertion and not substantially alleviated by rest)
Worsening of symptoms after any type of exertion (including cognitive and emotional stress) – “post-exertional malaise“
Unrefreshing sleep, and/or sleep disturbance.
In addition, should have at least one of:
Cognitive impairment
Orthostatic intolerance.
Doesn’t mention chronic pain?! NICE says reconsider diagnosis in absence of cognitive difficulties or chronic pain.
Causes
Evidence (reproducible) implicating certain infections as a trigger. Co-existing mood disorder in substantial proportion of patients, sometimes sleep-wake disorder – likely to perpetuate/exacerbate.
Brain imaging has identified alternations suggesting that it is a brain problem.
Cochrane review of graded exercise therapy – may benefit sleep, physical function, self-perceived general health, and no evidence that it worsens outcomes. Curiously, no evidence for loss of aerobic fitness! Perhaps graded exercise tackles a hyper-reactive CNS response to exercise-related physiological signals. Note that fear of physical activity becomes conditioned when it commonly exacerbates symptoms.
Warn that exercise programmes can make things worse rather than better. Exercise should only be done as part of supervised programme, with physiotherapist – don’t just tell them to go the gym more! Start below baseline activity level.
Other
Relaxation techniques recommended by NICE.
CBT – should only be offered to manage symptoms, improve functioning and reduce distress. Again, not a “cure”. Analysis of both CBT and graded exercise suggests that benefit comes from reducing inactivity.
Sleep hygiene important. Include rest periods in plan but avoid day time naps, especially since sleep doesn’t usually help anyway!
Many people find exclusion diets useful, esp bowel symptoms, not recommended but involve dietician if attempted anyway.
Equipment to maintain independence can improve quality of life and should be part of overall management.
Beware boom-bust! Many patients over do it when they have a period of relative wellness. Flares and relapses are to be expected. Trigger? New medical problem? Adjust plan as necessary.
Severe CFS can increase risk of pressure ulcers, DVT, vitamin D deficiency and contractures.
Prognosis
Important to be honest at time of diagnosis. More optimistic in young people. Most adults improve, some are able to return to usual activities but others experience long term symptoms or relapse.
PACE trial aimed for less than full restoration of health as “recovery”, future trials should use clinically relevant improvement and patient self-perception.
Binge Eating Disorder (without the compensatory behaviours of bulimia eg vomiting, exercise)
Selective/Restrictive Eating
DSM-IV Criteria for Anorexia Nervosa
Body weight at or below 85% of that expected
Fear of gaining weight or becoming fat, even though underweight
Disturbed perception of body weight or shape
Amenorrhea, at least 3 consecutive cycles
Disturbance in way one’s body shape is experienced (?)
ICD-10 adds a couple of things:
BMI < 15
Weight loss caused by food avoidance, self induced vomiting, purging, excessive exercise (not in DSM)
loss of libido in men
Pubertal delay if early onset
For children, these strict criteria can overlook significant disordered eating – wide variation in weight and height gain through puberty; menses not present else irregular. Levels of cognitive development obviously vary. Abnormal food behaviours eg slow eating, hiding may have been present from a young age. One type of eating disorder can change into another.
Interest in healthy eating and exercise may initially give impression that child is well. Dieting is miserable for most people, but for some it appears to relieve anxiety, which can lead to a vicious cycle. Sometimes it is specific foods that are feared.
BMI <2nd centile prob more useful for kids. GOSH Criteria (Nicoles, Chater & Lask 2000) talks about determined wt loss, abnormal cognitions about weight/shape but also morbid preoccupation. Similarly, Bulimia Nervosa defined as Recurrent binges and purges, Sense of lack of control, Morbid preoccupation with weight or shape. Purging is any behaviour to prevent weight gain including self induced vomiting, fasting, dieting, excessive exercise, misuse of medicines such as diuretics or laxatives.
Questions:
What did you eat yesterday? Are there foods you don’t eat any more?
Are you more interested in food and cooking?
Are you trying to cut back?
Does it ever feel like your eating gets out of control?
What happens if you can’t exercise?
Have you been making yourself sick? Do you drink water to prevent hunger?
What do you see when you look in the mirror? (Body dysmorphia = perception of shape, size that is unrelated to reality). Is there a weight you would like to be? What sort of things do you look at online?
Explore mood and risk of self harm. Anhedonia (inability to enjoy anything)common. Ask direct questions about abuse or neglect (explain this is routine).
Early intervention associated with better outcomes. Poor outcome in anorexia if patient does not receive effective treatment in first 3 years.
Principles –
Give diagnosis – may not be appreciated. Anosognosia = inability to see weight loss or failing health (and therefore others with that viewpoint are irrational or unkind)
Strengthening family relationships away from food (in many families tends to be around meals, snacks, eating out, and many conversations around favourite foods etc)
Conceptualising the eating problem as being separate from the young person. Eating problem as “bullying voice”. Avoid discussions of weight or body image (“you look healthier” can be misinterpreted as “you look fat” by eating problem).
Not a choice, not rational. Alexithymia (inability to express feelings) common. Genetic heritability accounts for approximately 50–80% of the risk of developing an eating disorder, often pre-existing tendencies towards anxiety, inflexibility, difficulties with emotional regulation, enhanced sensitivity towards punishment. [Proposed mechanism here]
Families are not to blame!
Food is medicine. Enforcing regular, balanced meals and snacks (3 of each daily) as a family improves mood, behaviour as well as physical symptoms. Terrifying at first, of course. Metabolism often ramps up once refeeding begins, so a huge increase in intake is often required to achieve restoration of healthy weight (and catch up growth) in anorexia. For bulimia, regular pattern of eating more important.
Don’t allow meal choices or negotiation, discourage involvement in or observation of food preparation, which reinforces disordered thinking. Reduces anxiety when not required to make “difficult” choices about problem foods.
Avoid regular weighing and other forms of body checking
Full recovery is possible, especially when detected early eg months rather than years.
Can be helpful to offer option not to be told weight. Beware concealing weights on body, water loading before weighing day. Praise honesty, highlight confidentiality, agree sharing of information with parents.
Parents can become used to “new normal” of disordered eating, and might not appreciate risks. Alternatively, young person might feel threatened by alliance between doctor and parents.
Target weight is tricky – what is required for normal bodily function? What was growth/puberty trajectory before eating restricted? So healthy thoughts about food, normal periods (often 9 months or more), return of premorbid personality etc. Fluctuating weight gain may be due to metabolism, fluid shifts, concealed weights or water loading, concealed purging (silent exercise eg crunches in bed).
Where food refusal is an issue, energy dense food is required – increase fat content, avoid water or diet drinks, leave fruit/veg till after other foods eaten. Smoothies or milkshakes often better tolerated. Bloating and nausea with refeeding common initially but should improve.
Family based treatment is recommended by NICE as first line. Emphasises that parents initially take back responsibility for feeding, then gradually handing it back to the young person. Minimisation of blame. If ineffective then CBT.
Psychotropic medication not recommended – metanalysis found no benefit from antidepressants in anorexia.
No evidence based guidelines for re-introduction of nutrition/energy in adolescents!
For the majority of patients, 40kcal/kg/day (1200kcal/day) appears to be safe – don’t start a meal plan with less calorific content than they were receiving prior to admission, although difficult when history of the amount taken is unclear.
The meal plan should comply with normal macronutrient guidelines (10-15% protein, 30-35% fat, 50-60% carbohydrates).
Increase the meal plan by 200kcal/day until 2000kcal/day is achieved.
Fluid 50ml/kg/d for 15yr+, 55ml/kg/d 11-14yr (Shaw et al). GOSH use standard paediatric fluid requirement calculations.
Promote weight gain 0.5kg/week (NICE 2004, Junior Marsipan 2012). Ignores malnutrition, of course. Percentage weight for height used, but easiest to divide BMI by median BMI for age/sex – 85% is underweight, 90% is satisfactory.
Check electrolytes, calcium, phosphate, magnesium, liver function, vit D on admission. QTc must be calculated MANUALLY (to find end of T, draw tangent through steepest part of curve).
All patients should be prescribed prophylactic dose of Vitamin D at a dose of 800IU/day whilst waiting for Vitamin D levels to be reported.
Prophylactic phosphate should not be routinely prescribed, however it should be considered where:
There has been a previous history of re-feeding syndrome.
Multiple risk factors
Consider Thiamine where starvation has been very prolonged (e.g. greater than one year at very low weight and poor intake) or there is a concern about vitamin deficiency.
A low phosphate (<1.1 mmol/L) before initiating feeds is unusual (see below) and should be corrected as soon as is possible on the day of admission:
Low phosphates should be discussed with the responsible consultant.
Give two sandoz-phosphate tablets and commence TDS regular phosphate regime.
Recheck U&E in 12 hours and monitor clinically (see below).
Do not make any increases on the feeding regime until phosphate has been corrected.
If phosphate is still low at 12 hours then consider repeated double dose, or IV correction. This is unusual.
Other causes of low phosphate should be excluded – in particular Vitamin D deficiency and hypoparathyroidism: check PTH and Vitamin D with next set of bloods (if hasn’t already been checked). These bloods should not hold up commencing of feeding once phosphate is normalised.
If phosphate is significantly low (<0.5) consider IV replacement – this will generally mean transfer to a medical ward.
Phosphates that are potentially dangerously low (<0.3) should be managed on a medical ward/PICU and discussions should occur with the consultant and CSPs about transfer before commencing feeding.
Refeeding syndrome
Biochemical abnormalities AND cardiovascular and neurological findings. Onset is in first 48 hours, up to first five days of initiating feeding (cases up to 18 days later described -consider at risk for up to 20 days). Cardiac arrest has occurred.
Early finding is drop in phosphate (increased requirement as body switches back to carbohydrate metabolism, plus chronic phosphate depletion due to starvation). Potassium, Mg also fall.
All patients considered at risk of re-feeding syndrome should be monitored for clinical signs of the re-feeding syndrome:
Resting tachycardia (differential for this includes anxiety).
Oedema or swelling, especially in the legs.
Confusion or altered conscious state (always check glucose in this case).
Patients should have:
Daily inspection for any signs of oedema (in particular peripheral oedema) for first five days.
Three times/day resting pulse and lying and standing blood pressure for first five days.
Monitor for biochemical/blood parameters of the re-feeding syndrome:
Daily urea, creatinine, sodium, potassium, phosphate, magnesium daily for five days. The drop in phosphate seen when re-feeding will normally occur within 48-72 hours.
