Chapter 5 Behavioural and developmental paediatrics
Long Cases
Anorexia nervosa
The eating disorders anorexia nervosa (AN) and bulimia nervosa (BN) are potentially lethal conditions, and two of the leading bio-psychosocial developmental disorders amongst adolescents, particularly females, in countries with Western culture. They occur predominantly in countries where there is abundant food, where the ultimate sought-after physique is a slim one and where society is orientated to achievement. Eating disorders are about 10 times more common in females than males. AN affects around 0.5–1% of adolescent females in the USA, while BN affects around 1–5%.
The aims of this long-case section are as follows:
• To give an overview of current management strategies employed in treating children and adolescents diagnosed as having AN (by correctly interpreted criteria of ICD-10 [World Health Organization: ICD-10 Classification of mental and behavioural disorders: clinical descriptions and diagnostic guidelines, 1992] or DSM-IV [American Psychiatric Association: Diagnostic and standard manual of mental disorders, 4th edn, 1994]). This section predominantly follows the DSM-IV manual.
• To suggest a directed examination technique for detecting salient physical findings, and to guide the ordering of appropriate investigations that will enable accurate diagnosis of AN and its common complications.
• To give guidelines regarding need for hospitalisation and a review of the mechanisms of the irreversible, life-threatening complications of AN.
This long case deals exclusively with AN. The long case can be challenging, but offers a wide discussion base regarding management. As AN is the third most common chronic illness in adolescent girls in Australia, the UK and the USA, there are always long-term patients available in hospitals where examinations are held.
Background information
Risk factors that create susceptibility for the development of eating disorders include:
• Occurrence of eating disorders, substance abuse or affective disorders, in other family members.
• Obsessional, perfectionist personalities with negative self-evaluation.
• Co-morbid clinical problems such as depression, anxiety, obsessive–compulsive disorder, post-traumatic stress disorder and substance use disorders.
Inadvertent positive reinforcement can occur if the patient was initially overweight, and the initial weight loss was greeted with support for dieting efforts. Parents’ accidental tacit agreement by complying with demands of the eating-disordered child (buying diet foods, allowing vigorous exercise schedules) can reinforce the evolving ‘anorexic identity’.
AN is very much a misnomer. These patients are not anorectic; they have normal appetites. They just exert their control, as mentioned above, by refusing to take notice of their (initially) ordered physiology. If a child or adolescent labelled as having AN does complain of anorexia, and does not have body image distortion, then that patient needs to be assessed for an organic problem such as inflammatory bowel disease or occult malignancy (for the differential diagnosis of AN, see the short-case approach to weight loss in Chapter 8, Gastroenterology).
History
Current status
Behavioural symptoms: the A to F of AN
1. Activity: exercise (compulsive, at unusual hours, solitary, not part of competitive sport, prolonged duration, long-distance running, sit-ups or stomach crunches a favourite), constant movement, standing rather than sitting. Ask ‘How long do you exercise each day?’
2. Body image: negative comments (unhappy with thighs, abdomen, hips), frequent weighing of self, not happy with new lower weight, chooses baggy clothes to hide weight loss. Ask ‘What do you think about your weight? Are you average, skinny or overweight? What would be a healthy weight for you?’ Document how large the patient judges his or her body to be. Body checking: repeated weighing, pinching, measuring body parts, checking protrusion of particular bones, checking certain clothes fit, mirror gazing, comparison with others’ bodies. Body avoidance: the opposite to body checking, avoiding above, refusal to weigh, avoidance of mirrors. Minimising symptom severity. Shape concerns. Overvaluation of weight and shape in determining self-worth. Fear of weight gain. Disturbance in how their own body is experienced. Note that many young people choose not to share these concerns with a candidate because they are shameful emotions. Younger adolescents do not commonly express such concerns even to health professionals they know well.
3. Cognitive aspects: rigid thought processes, impaired judgement, obsessive thoughts, rigid beliefs (e.g. cannot eat this food with that food; cannot eat after specified time, such as 6 p.m., as will not digest food), ritualised behaviour associated with purchase, preparation and consumption of food.
4. Drug aspects: use of laxatives, diet pills, diuretics, stimulants, thyroxine, cigarette smoking, diabetic (T1DM) patients withholding insulin. Ask: ‘What [medicines] do you have?’
5. Eating behaviour: missing meals, eating very slowly (e.g. cuts peas in half), hiding food, feeding her or his food to pets, feigning eating (e.g. pushing food around plate), avoiding social events involving eating, self-induced vomiting after meals, spitting, prolonged fasting (over eight waking hours), strict rules about eating, avoidance of social eating, little variety in foods (extreme vegan diets, avoidance of fat), secret eating, cooking for others but not eating themslves.
6. Food intake: types of eating—restrictive (eating less), selective (limiting intake to a range of preferred foods), restrained (controlling type and amount of foods); refusal to eat foods perceived as fattening (e.g. meat, dairy products, fat, carbohydrates); high intake of water, diet drinks, vegetarian foods; claims ‘allergy’ to various foods; immediate guilt after eating (may induce vomiting, or exercise excessively).
Physical symptoms of AN
1. Current weight, height, age-appropriate BMI centile, %BMI calculated from matching centiles on growth charts or 50% centile BMI for age, rate of weight loss, fluctuations in weight, lowest weight, highest weight.
2. Amenorrhoea, oligomenorrhoea. Absence of at least three consecutive menstrual cycles. Ask: ‘Are you having regular periods?’ ‘When was your last period?’
3. Pubertal delay—Tanner staging required.
4. Lethargy, weakness, fatigue. Ask: ‘How are your energy levels now compared to when you were at a healthier weight?’
5. Constipation (including pretending to have this to obtain laxatives). Ask: ‘Have you had any laxatives for this?’ ‘How often?’
6. Hair and skin: hair loss, dry skin, purplish skin peripherally, oedema.
7. Circulation: dizziness, lightheadedness, near-syncope, palpitations. Ask: ‘Have you had any dizziness, fainting, shortness of breath, palpitations/feeling your heart going really fast, or any chest pain?
8. Sensitivity to cold. Ask: ‘Does cold weather bother you?’ ‘Do you feel the cold more easily than other people?’
9. Symptoms in post-pubertal adolescents: reduced libido, reduced hair and beard growth, and reduced waking erections in adolescent males.
Complications of AN
1. Acute: refeeding syndrome (the key issue is hypophosphataemia, hence the need for daily bloods to check for low phosphate and to check electrolytes).
2. Chronic: growth deceleration, pubertal arrest, bone loss (osteopaenia or osteoporosis) or failure of normal bone accretion. Depression can be part of the biological effects on the brain from loss of weight, as well as part of the pre-morbid personality issues. Always ask about suicidal ideation; the two commonest causes of death from AN in adulthood are complications of malnutrition per se and suicide.
3. Potentially fatal: severe hypokalaemia, hypophosphataemia leading to arrhythmias, congestive heart failure.
Current management of AN for inpatients
1. Multidisciplinary list of team: members (e.g. paediatrician or physician specialising in adolescents, psychiatrist, psychologist, dietician, occupational therapist, physiotherapist, social worker, nurse, teacher, art therapist, music therapist) together, with clarity about case management role; degree of engagement with patient (against wishes, perhaps) and parents; degree of success so far; whether the team addresses issues adequately in the opinion of patient/parents.
2. Procedures of management program: usual measurements made (how patient is weighed—e.g. in underwear with gown; frequency of weighing), whether bladder scan is done (to subtract volume of urine from weight—note that this is inaccurate if patient drinks just prior to scan) or urine specific gravity checked (to detect water loading) unless salt loaded; whether there is a target weight known by patient; how height is checked; how often blood tests are performed; whether bone density is checked and how often; whether calcium supplements are given, whether the team has a target weight for the patient.
3. Approach to weight gain: eating meals and snacks provided; need for nasogastric tube, now or earlier in the admission; prescription of psychotropic medication during this admission.
Social history
Disease impact on siblings
Sibling rivalry, hostility, support, similar disordered thoughts developing.
Examination for anorexia nervosa
The approach given in Table 5.1 assesses patients with AN for disease severity and current status. It omits the various negative findings that would be relevant in a patient in whom the diagnosis was only suspected, but not yet proven. For the differential diagnosis for AN, see the short case on weight loss in Chapter 8 (Gastroenterology).
| 1. Introduce self |
| 2. General inspection |
| Parameters |
PCM = protein calorie malnutrition; SVC = superior vena cava.
Monitoring of disease
Not many routine tests are done in AN.
2 Documentation of disease progression
Progression is documented at intervals of 6 months to 1 year. The following may be done: bone density checked using DEXA (dual energy X-ray absorptiometry) machine (at 12 monthly intervals; Medicare only reimburses annually); gonadotropin levels (low); follicle-stimulating hormone (FSH); luteinising hormone (LH); oestradiol (low); pelvic ultrasound (ovarian shrinkage, few follicular cysts).
Management
Hospitalisation
Indications for admission to hospital are as follows:
(mnemonic: POLICE WT, police weight)
O. Obvious (over 5%) dehydration/Oxygen saturation < 85% (exceedingly rare)
Monitoring parameters
A target weight must be calculated for each patient, based on their height (measured with a stadiometer), their pre-morbid weight, their current weight (many units weigh patients in their underwear, wearing a gown, post-passing urine in the toilet, and before eating/drinking, checking for hidden weights and performing an ultrasound of the bladder to subtract the volume of urine), their previous maximum weight and their minimum weight, and their Tanner staging. The target weight may need ongoing revision, until epiphyseal fusion. Some patients, when they become aware of their target weight, have the uncanny ability to produce that number when on the scales. For this reason, many units do not specify the target weight, but merely indicate whether there has been a gain or loss, and the degree of that change.
Prognosis
In comparison to adults with established AN, adolescents with AN generally have a more optimistic course with about two thirds making a complete recovery. Those with a shorter duration of illness at the time of accessing clinical services (e.g. less than 1 year) generally have a better outcome.
Attention deficit hyperactivity disorder (ADHD)
Introduction
The aims of this long-case section, as well as of the subsequent short-case section, are as follows:
• To give a practical guide to the management of children diagnosed as having ADHD (by correctly interpreted criteria of ICD-10 [World Health Organization: ICD-10 Classification of mental and behavioural disorders: clinical descriptions and diagnostic guidelines, 1992] or DSM-IV [American Psychiatric Association: Diagnostic and standard manual of mental disorders, 4th edn, 1994]). This section predominantly follows the DSM-IV manual.
• To enable diagnosis of conditions that can be misinterpreted as ADHD.
• To enable the candidate to define not only that symptoms are present (to fulfil criteria) and to exclude relevant differential diagnoses, but also to define the developmental predicament—which areas of development are ‘handicapped’ because of ADHD self-control problems.
• To provide clear guidelines regarding the use of stimulants, including dosage and timing recommendations, side effects, contraindications and appropriate follow-up.
• To enable the candidate to articulate a long-term management program based on routine regular visits to the paediatrician, developmental and mental health optimisation, and a plan to eventually get the child off medication (akin to epilepsy management). The candidate needs to understand that ADHD is usually lifelong neuropathology; hence there needs to be a long-term management structure such as we would regard as routine for other chronic conditions such as diabetes or CF.
Background information
Few diagnostic labels have caused as much media controversy and divergence of opinion as ADHD. Despite different rates of diagnosis in different countries, similar prevalence rates are reported across different cultures when standard diagnostic criteria are applied rigorously. Traditionally, ADHD is very commonly diagnosed in the USA but remains less frequently diagnosed in the UK. Diagnosis rates within Australia are somewhere in between. There is still uncertainty as to whether ADHD represents the dysfunctional end of a continuum of normal temperamental characteristics or whether it represents a discrete qualitatively different biological or psychological condition. The name for this behavioural syndrome has changed over the years, from labels that were unpopular with parents (‘minimal brain damage’) to ‘minimal brain dysfunction’, ‘hyperkinetic child syndrome’, ‘minimal cerebral dysfunction’, ‘psycho-organic syndrome of childhood’, to attention deficit disorder (ADD) and finally ADHD, which has been the most widely accepted term.
• Symptoms of inattention that are maladaptive and inconsistent with developmental level.
• Symptoms of hyperactivity–impulsivity that are maladaptive and inconsistent with developmental level.
• Age of onset criteria that specify that symptoms need to be present before age 7 (school age in the USA).
• Some impairment from the symptoms present in at least two settings (e.g. at school and at home).
• Clear evidence of clinically significant impairment in social or academic functioning.
• The symptoms must not occur exclusively during, or be better accounted for by, a pervasive developmental disorder, a psychosis or another mental disorder.
The aetiology of ADHD is likely to be multifactorial, a combination of genetic, biological and environmental aspects. Twin studies suggest that 75% of the cause of ADHD is genetic. Parental ADHD increases the risk of ADHD in the child eightfold. Researchers have been trying to find a candidate gene for ADHD. The most recognised gene association is the 7-repeat allele of the D4 dopamine receptor gene (DRD4∗7); agonists at the D4 receptor site include dopamine, adrenaline and noradrenaline. Independent of parental ADHD, exposure to cigarettes and alcohol in utero increases the risk of ADHD.
Co-morbid diagnoses include the following:
• Specific learning difficulties (SLD).
• Conduct disorder (repetitive, persistent pattern of behaviour, where the basic rights of others or major age-appropriate societal norms or rules are violated; see DSM-IV).
• Oppositional defiant disorder (ODD, a pattern of negativistic, hostile, defiant behaviour; see DSM-IV).
The frequency of diagnosis varies between countries. In some states of the USA, over 25% of boys are taking stimulant medication for ADHD. In Australia, it has been estimated to affect between 1 and 5% of the population. Difficulty arises in diagnosis because the condition is considered as a delay in maturation of normal learning processes. Poor short-term memory, lack of concentration, impulsiveness and hyperactivity are all normal in a child at 3 years of age. By age 5–7, however, these characteristics are usually controlled. This is the reason why many kindergarten and primary school teachers describe these children as immature. Approximately 95% of children will be capable of controlling these characteristics by 5–7 years of age. Although parents, teachers and health professionals seem quite comfortable with the concept of a spectrum of developmental motor function (some normal children walk at 10 months, others at 18 months), any apparent delay within the spectrum of the (normal) development of learning engenders great concern. It should help to remember that the brain is continually growing, doubling in size in the first 12 months of life, with neuronal growth occurring throughout adolescence and adult life. Obviously, this growth and development will not occur at the same rate in all children.
Recent developments in ADHD management
Recent evidence-based data has emerged that is challenging the way this condition has been managed previously. Important studies have been released, which have called into question several aspects of typical stimulant prescribing and use. There has been debate for years as to whether short-term effects of stimulants lead to any long-term benefits; so far there has been a paucity of well-designed studies to answer this issue. Some studies show academic improvement in the short term, but benefits gone by 3 years; some show no effect of stimulant use on academic outcomes, and some have found long-term academic gains, particularly in mathematics.
