s c i r t a d e a P i Newer anticonvulsants NICE reserves gabapentin, lamotrigine, oxcarbaz- epine, tiagabine, to piramate, and vigabatrin (as an adjunct for partial seizures) for children not bene \201 t ing from (or able to tolerate) older drugs (above) or older drugs have contraindications/in teractions (the Pill) \200\224or the child is of childbearing potential or is likely to nee d drugs into her childbearing years. 706 209 , e n p e z a m a b r a C e n p e z a m a b r a c 0 0 7 \200\223 0 8 2 L / l o m a g n i r e p a t 0 8 1 \200\223 0 6 M J E N e d o r B d n a a m s a l p n 0 8 \200\223 0 4 i r o i h g u o r t t e g r a T y r e g r u s l l u f p e h u f p e h i e h t l l i l e v e l . , t o N t o N e t a m a r i p o t f i y s p e s c i r t a i d e a P . r e t a e r g o n s n o i t a n b m o c L / g m 9 9 \200\223 0 4 0 2 \200\223 0 1 0 4 \200\223 5 1 2 1 \200\223 4 g u r d r o ; l i n o m o o S p e ) s e r u z i e s t n a t s i s e r - g u r d n I i l r o m a t e c a r i t e v e s i . e n , k o o b t x e t s s e c o r p n w o d g n i r e p a t y r t i l y a d r e p c i n o l c o y m e t a b r e c a x e s e s o D . d n o c e s s e r u z i e s 3 \200\223 2 3 \200\223 2 2 \200\223 1 2 \200\223 1 2 \200\223 1 n e h t 2 e v i s n e h e r p m o c . e n p e z a b r a c x o , e n g i r t o m a 2 3 7 . I R M d n a e r a e c n e s b a i l i , e s o d e v i t c e ff e m a z a b o l c h 4 2 / g k / g m e t a m a r i p o t 1 . 0 \200\223 5 2 0 0 y 0 4 \200\223 5 1 0 2 \200\223 0 1 l l a u s U 5 2 \200\223 0 1 5 1 \200\223 5 8 \200\223 4 r o f d e d n e m m o c e r l y r t e c i v d a e h t g n i r u d e c n e r r u c e r s e d u l c n . a , r o y a m k r o w , y s p e r o m a t e c a r i t e v e L e t a o r p a v m u d o s l a c i g r u s o r u e n ( l i i r e h t i e n e n g i r t o m a 3 3 7 . 3 4 \200\223 8 3 : ) 1 ( 8 3 8 0 0 2 p E t n e m t a e r t l a v r e t n I s y a d n i , l 7 0 0 2 0 1 0 1 t n e m e r c n 5 7 7 1 t o n f I i l e g n E J . e t a o r p a v m u d o s e r a i e v i t c n u d A r o f l y r t ; e r u z i e s h 4 2 / g k / g m n i r t a b a g v d n a i e s o D r e f e r \200\207 . n e h t e n e b a t i u s n u : e b a t l 5 2 0 0 l e z i S 0 0 1 j l i l 5 5 0 0 5 0 . 1 ; l . . . . o r u e N r t a d e P d n a 2 t s r \201 s i . ; . f o : s n o i t a n b m o c e n g i r t o m a . ) s e r u z i e s i r o f . ) s d o r e t s o c i t r o c k s i r i i e c r u o S i f I e h T e t a o r p a v m u d o S t n e m s s e s s a e s o d t e g r a T e n b a g a i t , i h 4 2 / g k / g m . i d n o c e s e t a o r p a v m u d o s l a i t i n t c e ff e f o i . 7 9 4 p M C H O e c n e s b a d n a 5 0 0 5 . l s h t n o m 9 0 2 5 1 . 6 7 r o l 2 1 . c i x o t i r o f l ; e d m x u s o h t e e n p e z a m a b r a c , E T I S O P P O e e S n o t y n e h p i i i e r o m r o ( n i r t a b a g V i i e e S s t n a s l u v n o c i t n a g n p p o t S h t i i h 4 2 / g k / g m w d e r a p m o c g n i t r a t S c i n o l c o y m e t a b r e c a x e r e v e : s t \201 c i t e n 5 2 0 0 e s o d y r t , 0 1 0 1 e n p e z a b r a c x o 5 5 3 e s u . i . , t s r i F p e h e c i o h c i l y r t t o n : s m s a p s y a m i : s t \201 c i t s r i F 4 3 7 . 8 1 k a s k e e w 6 o D 4 1 7 \200 ? g u r d h c i h W t s r i F e n p e z a m a b r a C r o t e d c i n e g o t e k l l i a f e d m x u s o h t E a t i b r a b o n e h P i , : s t \201 8 6 1 m a p e z a n o C i , n o c i : s e c n e s b A n i t n e p a b a g g u r D i n o t y n e h P e l i t n a f n I e t a o r p a V n o s g u r d f I l c \200\223 c i i i 4 3 3 s i l i a l c o y M d o i r e p i t r a P y a m ; 6 9 9 1 n o T l l ( Behavioral problems \200\207Only enter battles you can win. If the child can win, be more subtle, eg con- sistent rewards, not inconsistent punishments. Get a health visitor \200 \231s advice; ensure everyone is encouraging the same response from the child. Entrances Fo od refusal and food fads are common. Reducing pressure on the child, discouraging parental over-reaction, and gradual enlarging of tiny portions of attractive food are usually all th at is needed. Check Hb. Keep a watchful eye on growth and weight gain. Overeating: Eating c omforts, and if the child is short on comfort, or if mother feels inadequate, the scene is set for overeating and lifelong patterns are begun. Diets may fail until the child is hospi talized (p348). If obese (p226, p530), remember hypothalamic syndromes (eg Prader \200\223W illi, p652), but the treatment remains the same. Pica is eating things which are not food, eg plastic, or feces (coprophagia); if persistent, look for other disturbed behaviors, auti sm, or \200\201IQ. Causes: iron or other mineral de \201 ciency; obsessive-compulsive dis order. Complications: lead poisoning; worm infestations. 735 Behavior modi \201 cation may help. 736 Exits Constipation is diffi culty in defecation; it may comprise of <3 stools pe r week; large hard stool; \200\230rabbit dropping \200\231 stool; distress/straining/bleed ing with passage of stool. It may lead to abdominal pain, abdominal masses, over \202 ow s oiling \200\230lavatory-blocking \200\231 enormous stools (megarectum), and anorexia. C aus- es: diet, poor \202 uid, or \201 bre intake \200\224or fear, eg as a result of a \201 ssure. Rarely Hirschprung \200\231s disease (p130). Failure to pass meconium in \201 rst 48h? Ask about onset of constipation and precipitants ( \201 ssure/change in diet /timing of potty training/fears and phobias/moving house/acute infections/family upheavals) . Red \202 ags include. \200 737 210 \200 s c i r t a d e a P i Constipation from birth or \201 rst few weeks. Failure to pass meconium within 48h. Falte ring growth (an amber \202 ag really; consider coeliac disease/hypothyroid). New weakness /abnormal re \202 exes in legs, delayed locomotion. Abnormal appearance of anus / skin in sacral/gluteal region (look for sacral dimples/hairy patches/ \202 attening of gluteal mus cles/multiple \201 ssures). Gross abdominal distension with vomiting. Action: \200 Find out about pot refusal \200 Does defecation hurt? \200 Is there pare ntal coercion? Break the vicious cycle of: large feces \200\203 pain/ \201 ssure \200 \203 fear of the pot \200\203 rectum overstretched \200\203 call-to-stool sensation dulled \200\203 soiling \200\203 parental exasperation \200\203 coercion. \200\207Exonerate t he child to boost con \201 dence for the main task of obeying calls-to-stool to keep the r ectum empty. Treat fecal impaction with escalating dose regimen of polyethylene glycol 3350 + electrolytes eg \200\230Movi- col Pediatric Plain \200\231 as \201 rst line interve ntion (NICE \200 737 suggests doses, which ex- ceed those in the BNFc, as follows: if <1y then \200\2231 sachet daily; if aged 2 \200\2235y then 2 sachets on day 1; increase by 2 sachets every 2 days to max. of 8 sachets daily; if 5 \200\22312 start on 4 sachets and in crease in steps of 2 to a maximum of 12 per day. If >12y use Movicol (lacks electrolytes and contains a higher dose of polyethylene glycol 3350) at 4 sachets on day 1, escalating b y 2 sachets/day to a maximum of 8. Follow this with maintenance \200\230Movicol Pediatri c Plain \200\231 (<12y)/Movicol (>12y) lactulose prolonged alternate-day senna, adj usting dose to produce regular soft stools. NB: behavior therapy in combination with laxati ves are eff ective, 738 but biofeed- back methods are not. 739 Clinics run by nurse special ists can be more eff ective than those run by consultants. 740 Dietary modi \201 cations t o ensure a balanced diet, with suffi cient \202 uids and \201 bre are necessary but not suffi cient by them- selves. Encourage daily physical activity. Treat perianal cellulitis with co-amox- iclav (for example). Digital rectal examination and abdominal x-rays are rare ly required. Do not use enemas until all attempts are oral medication have failed. Soiling is the escape of stool into the underclothing. Fecal incontinence is fecal soiling in the context of a physical/anatomical lesion (Hirschsprung \200\231s disease, anal malfor mation, anal trauma, meningomye- locele, muscle diseases). 741 Encopresis is the repeated p assage of solid feces in the wrong place in those >4yrs old. \200 : \200 \211\210 5 : 1. It may be voluntary or non-voluntary. Is this due to chronic constipation, and a rectum wh ich has lost its memory of what full and empty feels like? Treat retentive encopresis (80% of cases) with enemas, extra dietary \201 bre, stool softeners, and \200\230mandatory \200\231 daily toilet sittings 15 \200\22330mins after eating). Try behavior therapy for n on-retentive soiling. 742 NB: this demar- cation is not absolute: behavioral techniques suc h as diff erential attention, contingency management, and contracting are relevant to both forms of en- copresis. 743 Rarely, encopresis signi \201 es autoeroticism1 or a defecation disorder or learning2 diffi culty. 744 Whatever the cause, \201\201adopt a holistic stanc e and set the symptom in the family context. This is important because we know that encopre sis is associated with anxiety, depression, attention diffi culties, and environments with less expressiveness and poor organization, and social prob- lems (disruptive behavior, poor er school performance etc). 745 Aim to give the family time to air feelings that encopresis engenders (anger, shame, ridicule). Enuresis: \200 746 Infrequent bedwetting (<2 nights/w eek) occurs in 20% at 4 yrs and 8% at 9 . 1 \200\2232% of >15y continue to wet the bed, usually from delayed maturation of bladder control (family history often +ve). Tests for di abetes, UTI and GU abnormality (p174) can occasionally yield surprises but are by no means compulsory unless there are clinical suspicions. The term \200\230secondary enuresis \200 \231 implies wetness after >6 months \200\231 dryness, and raises concerns about worries, i llness, or abuse. History: Ask about nights per week he wets the bed? Does it happen more t han once per night? Severe bedwetting is less likely to resolve spontaneously. Are there an y daytime symptoms? Frequency/ urgency may indicate an overactive bladder. How much does he drink dur- ing the day? Is there constipation/soiling; history of recurrent UTI (underlyin g urological abnormality)? If the child was dry and recently started bedwet- ting consider systemic illness and the possibility of child abuse. Treatment: Start with advice and reass ure parents than many children continue to wet the bed after achieving day-time dryness. En sure that caff eine based drinks are avoided and the toilet is used regularly during the da y (4 \200\2237 times is typi- cal). Reassure that he is neither infantile nor dirty. A syst em of rewards for agreed behaviors (eg drinking recommended levels of \202 uid, using the toi- let before bedtime, taking medicines, or helping change the sheets (not for dry night s which the child can t control) may be eff ective. Alarms ( vibra- tions) triggered by u rine in the bed can make 56% dry at 1yr; relapses are preventable by continuing use after d ryness. They are cheap or loanable from Child Guidance Services (or equivalent) \200\224eg Drinite . 747 www.bedwetting.co.uk. Desmopressin sublingual dose (if >5yrs): 120 g at bed time (max 240 g); \202 uid overload (Na+ \200\201) is rare, eg after gulping in swimming pool water, or going to rave parties. Have 1 week 3-monthly with no drugs. CI: cystic \201 brosis, uraemia, BP \200\200. 211 s c i r t a i d e a P 1 Anal erotic feelings reported by some encopretic children are intense, and some workers h old that the encopretic symptom, soiling, in these children is the result of a conscious fo rm of anal masturbation in which the fecal mass is used for stimulation. Encopresis interve ntions may be over-simplistic unless they take into account what encopretic children know a nd say about their soiling.748 2 Behavioral treatment of non-retentive, nocturnal encopresi s in those with learning disability involves waking prior to soiling, time-limited use of s uppositories, and rewards for appropriate evacuation.749 Attention de \201 cit disorder (ADD) & hyperactivity Attention de \201 cit hyperactivity disorder (ADHD) is the most common neurobehavioral disorder of childhood. It has prevalence of 3 \200\2235% in Western nations. 750 ADHD is commoner in learning-disabled children, an d if prenatal cannabis exposure. 751,752 The core diagnostic criteria are: impulsivity, in- attention and hyperactivity. Not all those with ADD are hyperactive. There is no diagnosti c test (but positron emission tomography may show \200\201function of frontal lobes and ne arby connec- tions). Most parents \201 rst note hyperactivity at the toddler stage, but m ost locomotor hyperac- tivity at this stage abates with time, so the di- agnosis is usually delayed until school entry or later. Signs often attenuate during adolescence, but may per sist into mid-adulthood. There is a familial tendency, and families with ADD are more likel y to have other diagnoses too, eg learn- ing diffi culties, mood, anxiety, and psychosocial disorders (DSM IV; p312). Associations: conduct disorder or other disruptive behavior diso rders (oppositional de \201 ant disorder). 753 Children and adolescents with ADD are at ri sk of being victims of assaults, as well as suicide and self-harm. 754 Treatment: \200 755 Diagnosis and drug treatment ought to be initiated by a special- ist (eg pyschiatrist/pedi atrician). Following diagnosis, time for explanation is required; off er a booklet to paren ts, give advice on positive parenting and behavioral techniques. 1st line treatment for pre -school children and school age children with moderate ADHD/moderate impairment is parent t raining/ed- ucation programs. Older children may bene \201 t from cognitive behavioral the rapy. Drugs may be useful in school age children if non-drug treatments fail (eg methylphen idate (Ritalin ): if 4 \200\2236y start at 2.5mg/12h, increasing weekly if needed by 2.5mg /day to a max of 1.4mg/kg/day; if >6 start at 5mg/12h in- creasing to a max of 2.1mg/kg/day (max 90mg daily); atomoxetine: 0.5mg/ kg/day (if >6yrs old and <70kg) increasing after 7 d ays to 1.5mg/kg/day). Se- vere ADHD in school age children methylphenidate and atomoxetine are 1st line treatments so ensure referral. Evidence for long-term use is uncertain. 756,75 7 Parental training and behavioral modi \201 cation techniques, eg prolonged eye- contact between parent and child should also be considered. 758 Hypoallergenic diets (eg no tartraz ine) are controversial (may cause parental obsessions). Liaise with teachers: 30% of ADHD b oys are excluded from school \200\224this may violate their human rights. 759 Other ideas: Yoga, 760 homeopathy (probably no eff ect). 761 School refusal Setting: Emotional overprote ction; high social class; neurotic parents; schoolwork of high standard. In truancy, the re verse is true. Treat- ment: Confer with headteacher, parents, and an educational psychologi st. Escort by an education welfare offi cer aids prompt return to school. Other methods: ed ucational-support therapy, cognitive behavior therapy, and parent-teacher interventions. 76 2 Hypnosis is a good alternative (the hypnotist maintains contact with the child on the way to school and during the stressful morning hours via a mobile phone). 763 Often anxiety (e g separation-anxiety or phobias) depression need treatment too. 764 212 \200 Attention de \201 cit signsDSM-IV Often unable to: \200 Listen/attend closely to detail ( \200\230 carelessness) \200 To sustain attention in play activities Follow instructions \200 Finish homework (when not due to de \201 ance) \200 Organize tasks needing sustained application Ignore extraneous stimuli \200 Remember simple tasks Hyperactivity signs \200 Squirming / \201 dgeting \200 \200\230On the go all the time \200\231 \200 \200\230As if drive n by a motor \200\231 \200 Talks incessantly \200 Climbs over everything \200 Restles s \200 No quiet hobbies \200 Impulsivity \200\224ie: \200 blurts out answers \200 i nterrupts others \200 too impatient to take turns or to queue s c i r t a d e a P i This page intentionally left blank Delay in walking Babies usually learn to walk at 14 months old. If this has not occurred b y 20 months, ask yourself 2 questions: Is the child physically normal? Is de- velopment del ayed in other areas too? The com- monest causes re \202 ect chronic illness, global delay, benign immaturity, and generalized joint hypermo- bility (BOX). In boys consider Duchenne muscular dystrophy early (genetic counseling before moth- er \200\231s next pregnancy). The major reason for identi- fying late walkers is to exclude cerebral palsy. Cerebral palsy ( CP) comprises chronic disor- ders of posture and movement caused by non- progressive CNS le sions sustained before 2yrs old, resulting in delayed motor development, evolving CNS signs , learning disability (35%), and epilepsy. Most are due to antenatal events unrelated to bi rth trauma. Prevalence: 9% if gestation 23 \200\223 27wks; 6% if 28 \200\22330wks; 0.1% if term. 765 Survival: 20yrs if quadriplegic (much longer if less aff ected). \200 : \200 \211\210 1 : 1. Signs: Weakness, paralysis, delayed milestones, seizures, language/ speech problems. Classi \201 cation: There are four main types: Spastic; dyskinetic; ataxic and m ixed CP. Spasticity suggests a pyramidal lesion; uncoordinated, in- voluntary movements and postures (dystonias) suggest basal ganglia involve- ment. Most have either a spastic hemip legia (arm>leg; early development of hand preference \200\224ie <12mth; delay in walking, i ncreased deep re \202 exes of aff ect- ed limb) or a spastic diplegia (both legs aff ected worse than the arms, so that the child looks normal until he is picked up, when the legs \200\230scissor \200\231 \200\224hip \202 exion, adduction and internal rotation; with kn ee extension and feet plantar- \202 exed). Spastic quadriplegia is the most severe form an d is associated with seizures and IQ \200\201. Swallowing diffi culties (2o to retrobulbar palsy) may lead to aspira- tion pneumonia. Dyskinetic (athetoid) cerebral palsy: Unwanted a ctions; poor movement \202 ow/posture control; spasticity; hypotonia; hearing \200\201; d ysarthria. Association: kernicterus. Ataxic palsies are uncommon. There may be hypo- or hyp ertonia. \201\201Management teams Children \200\231s views must be taken into account in a ll matters concerning them. 766 UN Convention on the Rights of the Child \200\207Assume th at all disabled chil- dren are entitled to a \200\230full and decent life \200\231. The ai m is quality of life and full in- tegration into society. Because children have grown up ha nd-in-hand with dis- ability and are often uncowed by it, they often score as high as anyon e else on quality of life, if pain is treated. 766 Parents may \201 nd it a comfort to kn ow this. Physio- and occupational therapists, orthopedic surgeons, and orthoses ex- perts a id holistic assessment: can he roll over (both ways)? Sit? Grasp? Trans- fer objects from h and to hand? Good head righting? Ability to shift weight when prone with forearm support. I Q. Is toileting possible? Can he hold a pen or a spoon? Muscle strengthening can help. 767 Calipers may prevent deformity (equinovarus, equinovalgus, hip dislocation from excessive \202 exion/adduction). Attempts to show bene \201 ts of neurophysiotherapy (to help equil ibrium and righting) don \200\231t show bene \201 t over simple motor stimulation. Some pa rents try the Hungarian Pet approach: \201\201Here the \200\230conductor \200\231 devot es herself to the child, using interaction with peers to reinforce successes: eg manipulati on, art, writing, \201 ne movement, and social skills. 768 Treat co-morbidities such as e pilepsy (p208). Botulinum toxin (p460) has an uncertain role. 769 \200\223771 Epidural cord elec- trostimulation & intrathecal or oral baclofen (bene \201 ts uncertain). 772,773 Pro gnosis By 6yrs, 54% with quadriplegia (80% if hemiplegic or diplegic) gain urinary continen ce spontaneously. If IQ \200\201, 38% are dry at this age. 774n=601 214 \200 Typical causes: Prenatal factors \200 APH (with hypoxia) \200 X-rays \200 Alcohol \200 CMV; rubella; HIV \200 Toxoplasmosis \200 Rhesus disease Perinatal factors: \200 Birth trauma \200 Fetal distress \200 Hypoglycaemia \200 Hyperbilirubinaemia Postna tal factors: \200 Trauma/intraventricular hemorrhage Hypoxia \200 Meningoencephalitis \200 Cerebral vein thrombosis s c i r t a d e a P (from dehydration) i 215 Delay in talking (For autism, see p394.) \200\207When in doubt, ask the mother what she th inks is wrong. \200\207Always test the hearing. Ensure the result is as reliable as possib le. Speech development This dialog portrays the mystery of language learn- ing: Daughter: \200\234I don \200\231t want to eat my ice cream yet \200\235 Father: \200\234Don \200\231 t procrasti- nate! \200\235 \200\234Daddy, how can I understand you if you use words I don \200\231t understand? \200\235 \200\234If I only ever used words you understood we could never have started talk- ing. \200\235 \200\234Why? \200\235 \200\234Well, to start with you never understood anything \200 \200\235 \200\234Didn \200\231t I? \200\224 OK, wait a moment \200 I \200\231ll start my ice cream before it melts. \200\235 At 1 year a few wor ds may be used meaningfully. At 1 \201 yrs old 2-word utterances ( \200\234Daddy come \200 \235). At 2 years old \200 subject \200\223verb \200\223object sentences appear ( \200\234 I want a pudding \200\235). At 3 \201 yrs old \200 the child has mastered thought, languag e, abstraction, and the elements of reason, having a 1000-word vocabulary at his or her dis posal, ena- bling sentences such as: \200\234I give her cake \200\231 cos she \200\231s hu ngry \200\235. Words exist to give ideas currency, and so often that currency proves counte r- feit \200\224a process which so often starts with if, eg \200\234If I hadn \200\231t th rown the cup on the ground, I might have got a pudding \200\235. The uttering of \200\234I f \200\235, linked with an emotional response, is the most human of all constructions, opening up worlds divorced from reality, providing for the exercise of imagination, the va lidation of dreams, the understanding of motives, and the control of events. The rest of li fe holds nothing to match the intellectual and linguistic pace of these \201 rst years. F urther linguistic development is devoted to seemingly conceptually mi- nor tasks, such as e xpanding vocabulary. There is much variation in speech timing: what is \200\230clearly abn ormal \200\231? Vocabulary size: If <50 words at 3yrs old, suspect deafness \200\224or: Exp ressive dysphasia or speech dyspraxia (eg if there is a telegraphic quality to speech, poor clar- ity, and deteriorating behavior, eg frustration). \200 Audio-premotor syndrome (AP M). The child can- not re \202 ect sounds correctly heard into motor control of larynx and respiration. Instead of bab- bling, the child is quiet, unable to hum or sing. s c i r t a i d e a P Other causes of delayed talking: Congenital: \200 Klinefelter \200\231s syn. \200 Galac tosaemia \200 Histidinaemia \200 Auditory agnosia \200 Floating-harbor syn. Acquired with or after: \200 Meningoencephalitis \200 Head injury \200 Landau-Kleff ner syn. Respiro-laryngeal (RL) dysfunction (dysphonia from incorrect vocal fold vibration/air \202 ow regu- lation). The voice is loud and rough. Congenital aphonia (thin eff ortful voice; it \200\231s rare). Speech clarity: By 2 yrs, p arents should under- stand most speech. If not, suspect deafness \200\224or: \200 Articul atory dyspraxia (easy consonants are (epilepsy + progressive loss of language) b and m with the lips, and d with the tongue \200\224the phonetic components of babbling). \200 : \200 \211\210 3 : 1. Tongue-tie is a possible cause ( \200\230 poor sounds needin g tongue elevation \200\224 d and s) \200\224surgery to the frenum may be needed (+speech t herapy). Distinguish from phonological causes (disordered sound for speech processing \200 \224may present as sound awareness problems (diffi culty in analys- ing sound structure of words). Both are common. APM or RL dysfunction, as described above. Understanding: By 2 years a child should under stand \200\234Get your shoes \200\235 (if he has any), if not suspect: \200 Deafness \200\224if the hearing is impaired (eg 25 \200\22340dB loss) secretory otitis media is like ly to be the cause. Worse hearing loss is probably sensorineural \200 Cognitive impairmen t \200 Deprivation. Speech therapy Refer early, before school starts. NB: randomized tria ls have not shown any clear bene \201 ts from this strategy. 775 N=159 Impairment, disability, and handicap WHO de \201 nitions Impairment entails a pathological process, eg spina bi \201 da, which may cause certain disabilities, eg walking diffi cult y. Handicap is their social consequence (eg cannot walk to school). Learning delay (mental handicap) The mother often makes the \201 rst di- agnosis. An IQ <35 constitutes a severe learning disability (mental handicap, p314). \200\207Beware con \202 ating IQ with intel lect: the latter implies more than problem- solving and memory: intellect entails the abili ty to speculate, to learn from mistakes, to have a view of oneself and others, to see relat ionships between events in diff erent domains of experience \200\224as well as the ability to use lan- guage either to map the world, or to weave ironic webs of truth and deceit (and , on a good day, to do both simultaneously). Causes: Severe mental impairment usually has a de \201 nable cause, whereas mild intellectual disability is often familial, with no well -de \201 ned cause. Be pre- pared to refer to an expert. Congenital disorders are legion: chromosomal (eg Down \200\231s; fragile X, p648); metabolic (eg PKU p183). Acquired: Perina tal infection p34 \200\22337, birth injury and cerebral palsy, trauma, meningitis. Lead exp osure: This is a leading preventable cause of mildly impaired IQ. For example in 2-yr-olds for each 0.48 mol/L plasma increment there is an associ- ated 5 \200\2238 point fall in IQ as measured on the Wechsler Intelligence Scale for Children (revised). This defect is long -lasting. Chemical defects associated with intellectual disability \200\224eg: Homocyst- in uria: Paraplegia, \201 ts, friable hair, emboli, cataracts; homocystine is found in the u rine. Treat with a low-methionine, cystine-supplemented diet, with large doses of pyridoxin e. Maple syrup urine disease: Hypoglycaemia, acidosis, \201 ts, death. Urine smells of ma ple syrup, due to defective metabolism of branched chain keto acids. Treatment: high-calori e amino acid controlled diet. Thiamine has been tried. Tryptophanuria: Rough, pigmented ski n. Treat with nicotinic acid. Management: Refer to an expert, so that no treatable cause is missed. Would the family like help from group, such as MENCAP? Other members of the family may need special support (eg normal siblings, who now feel neglected). If the IQ is >35, l ife in the community is the aim. Physical handicap Sensory: Deafness, see p548. Blindness: congenital de- fects are described on p454. Principal acquired causes of blindness are: ret in- opathy of prematurity, vitamin A de \201 ciency, onchocerciasis (p450), eye injuries, cataract (eg Down \200\231s syndrome). CNS & musculoskeletal problems: (Congenital or acqui red) Causes: accidents (eg near-drowning), cerebral palsy (p214), spina bi \201 da (p140), after meningitis, polio, congenital infections (above), tumors, syndromes (p638). Wheelcha irs:1 For indoors or outdoors? Patient-operated, motorized or pushed? What sort of restrain ts to prevent falling out? If collapsible, how small must it be to get into the car? Are th e sides removable to aid transfer from chair to bed? Can the child control the brakes? Are there adjustable el- evated leg rests? Liaise with the physio and occupational therapist. C alipers will allow some patients to stand and walk. Long-leg calipers are required for thos e with complete leg paralysis. The top should be constructed so that it does not induce pre ssure sores. A knee lock supports the knee in the standing position. An internal coil sprin g prevents foot drop. 216 \200 s c i r t a d e a P i 1 Disabled Living Foundation, www.dlf.org.uk UK tel. 0845 130 9177 (local rate).776 217 Check-list to guide management of handicap In hospitals or the community, we should address each of these points: \200 Screening and its documentation on local handicap registers. \200 Communication with parents. \200 Referring to/liaising with district handicap team +community pediatrician. \200 Access to specialist services, including physiotherapy, ort hopedic surgery. \200 Assessing special needs for schooling and housing. \200 Co-ordina ting neuropsychological/neurodevelopmental assessments. \200 Co-ordinating measures of se verity (eg electrophysiology CT/MRI). \200 Liaison with dietician on special foods. \200 Promotion of long-term concordance with treatment/education programs. \200 Educatio n about the consequences of the illness. \200 Encourage contact with family support group s. \200 Off ering family planning before patients become unintentionally pregnant. \200 Pre-conception counseling (p2, with specialist in molecular genetics). \200 Co-ordinatin g prenatal diagnostic tests and fetal assessment. s c i r t a i d e a P Society, pediatrics in the community, and family-oriented care 99.9% of pediatric care goes on in the community, provided by mothers, fathers, GPs, nurses, physios, community pediatr icians, child-minders, spe- cial-needs teachers, sports/PE teachers, and their assistants. Inevitably if you are studying pediatrics within hospital you will have a biased view of wh at pediatrics is like \200\224nowhere more at odds with reality than in the spheres of impa irment, disability, and handicap. \200\207If you really want to make a difference to child ren \200\231s lives, get out into the community at every chance. Find out what is going on \200\224and then start contributing. . g n o r w s e o g l l a t i n e h w s m e l b o r p Increasingly, this is being advocated by pediatric training programs in the UK and abroad \200\224eg the Community Pediatrics Training Initiative. 777 Children \200\231s health and wellbeing are inextricably linked to their parents \200\231 physical, emotional and social health, social circumstances, and child-rear- ing practices. 778 These cannot be appreciate d or molded to the child \200\231s ad- vantage without at least one foot in the community. h t l a e h l a t n e m d o o h d l i h c No pediatrician can work well without understanding the multicultural demographics and marr iage statistics of the population from which her patients come. For example, in some areas the median income of families with married parents has increased by 146% since 1970, but fe male-headed households have had growth of 131% (less in inner city areas). In one study, th e median income of female-headed households was only 47% of that of married-couple families and only 65% of that of families with 2 married parents in which the wife was not employed . The proportion of children who live in poverty is 5-fold greater for female-headed famil ies than for married-couple families. 778 In the UK, 1.4 million children live in poverty d e- spite one parent having a job. 779 This has a greater eff ect on children \200\231s heal th than all the goings on in pediatric wards and hospitals. These problems seem resistant t o socialism, the minimum wage, tax credits and bene \201 ts. f o s e c e i p e h t p u k c i p e w d n a , m s i l a i r e t a m Most families with young children depend on child care, of varying quality. This causes \200\200 costs (only partly mitigated by government funds), long days for children, with st ress imposed by travel and exposure to infections. 778 f o s e u l a v More and more parents are devoting time once available to their children to the care of the ir own parents. They won \200\231t tell you of this in brief ward en- counters, but these f acts become clear when working in the community. 778 \201\201Pediatricians have a key role in fostering interdisciplinary collabo- ration between schools, hospitals, and other child -related institutions, and they must feel able to refer parents for physical, emotional, or social problems, or health risk behaviors that can adversely aff ect the health or emotion al or social wellbeing of their child. e s l a f e h t t i m s n a r t s e i l i m a F Developmental screening tests Developmental disorders are a group of conditions leading to impairment in at least one functional area \200\224eg cognition, motor skills (gross or \201 ne), social- emotional or communication (speech, language, hearing or vision). Around 20 \200\223 25% of children have at least one developmental delay, and as many as 50% of th ese will not be detected before starting primary school. 780 Conditions falling under the u mbrella of developmental disorders include autism spectrum dis- orders, speech-language imp airment, learning disabilities and psycho-social problems. Biological risk factors include: prematurity, low birth weight, birth asphyxia, chronic illness and hearing/vision impairme nt. Environmental risk factors include: poverty, low parental education, parental mental il lness and social isolation. 781 Developmental surveillance refers to an ongoing process of following a child over time with a view to contextualizing a child s development. It involv es discussing concerns with parents, eliciting a perinatal history, identifying risk factor s, as well as observing the child attempting diff erent skills at diff erent times and refe rring the child when appropriate to other health professionals (eg physiotherapists, speech therapists or audiometrists). Developmental sur- veillance can be incorporated into well-c hild checks, general physical examina- tion and routine immunization visits. Developmental screening refers to a brief assessment aimed at identifying those children who require furt her investigation and assessment. It is typi- cally carried out using a developmental scree ning tool. Screening should be carried out within the broader undertaking of developmental surveillance. Repeating screening at diff erent ages increases the accuracy of the test. Pa - rental concern about a child s development may in itself constitute a reliable screening test. 782 The Denver Developmental Screening Test (DDST) and its successor the Denver II ha ve largely been replaced with more sensitive tests. Screening may be based on parent report alone (parent completed tests) or through direct observation together with parent report ( directly administered tests). Some of the most commonly used tests are discussed below \200 \224all are copyrighted products: The Parents Evaluation of Developmental Status (PEDS) to ol is a parent completed test consisting of 10 questions (8 yes/no, 2 open ended). It can b e given to parents to complete prior to attending health visits and takes less than 5 minut es to complete. It has a sensitivity of 74 \200\22380% and is suitable for children up to 8 years old. 780 It identi \201 es children as low, moderate or high risk for various disab ilities and identi \201 es an optimal course of action. Available at www.pedtest.com The A ges and Stages Questionnaire (ASQ), another parent completed test, consists of 21 age speci \201 c questionnaires. It can be used to evaluate children aged 1 month to 5 years old. It has a sensitivity of 85% and takes 10 \200\22320 min- utes to complete. 780 It has a si ngle cut-off score indicating which children need further referral. Available at: agesandst ages.com The Modi \201 ed Checklist for Autism in Toddlers (M-CHAT) is a second-stage pare nt completed test that is more speci \201 c for autism spectrum disorders. It has a sensit ivity of 90%. Available free online at: www. \201 rstsigns.org. The Brigance Screens-II is a directly administered test, combining parental observation and the eliciting of skills f rom children. It covers multiple domains of development including speech-language, motor, g eneral knowledge as well as reading and math at the older age groups. Available at: www.cur riculu- massociates.com Whilst the American Association of Pediatrics recommends both devel op- mental surveillance and screening, 783 the National Screening Committee (UK) doesn t re commend developmental screening. 784 218 s c i r t a d e a P i Developmental milestones 219 Average age Milestone Red \202 ags 6 weeks smiles, \200 follow eyes past midline 4 \200\2236 months At 6 months if \200 no smile \200 no grasp \200 not rolling \200 poor head control sits with support \200 rolls \200 reaches out for objects starts babbling \200 crawls \200 sits without support \200 pulls to stand \200 give s toy on request \200 turns head to name \200 responds to bye-bye \200 gestures wit h 6 \200\2239 months At 9 months if s c i r t a i d e a P no response to words \200 lack of eye contact or facial expression no gestures \200 no passing of toys from hand to hand \200 not sitting without support or crawling babbling \200 \201 rst tooth 7 \200\22312 months At 12 months if develops pincer grasp \200 plays peek-a-boo \200 walks with a hand unable to pick up small items not crawling / bottom held shuffl ing waves goodbye not standing holding on to furniture no babbled phrases 12 \200\22315 months single words \200 listens to stories \200 drinks from cup 18 months uninterested in playing speaks 6 words \200 able to walk up steps \200 names pictures \200 walks independentl y \200 scribbles \200 builds with blocks with others \200 no clear words \200 not walking without support not able to hold crayon \200 unable to stack 2 blocks At 2 years if 1.5 \200\2232 years kicks/throws a ball \200 runs \200 2-word sentences \200 follows a 2-step has <50 words \200 diffi culty handling small objects unable to climb stairs \200 no interest in feeding command stacks 5 \200\2236 blocks \200 turns pages \200 uses a spoon \200 helps with dressing or dressing If there is regression, or loss of a previously developed skill, this should be considered a red \202 ag requiring immediate investigation. Other red \202 ags at any age include poor interaction with others, diff erence in strength between right and left sides of body, abnormal tone and strong parental concern.