 \201\201Chronic childhood illness, and family support Diseases such as severe asthma, CNS disease, and neoplasia may cause disintegration of even the most apparently secure families : \200\207consequent strife and marital breakdown may be more severe and have more far- re aching consequences than the illness itself. s c i r t a i d e a P 6 3 \200\235 . e t o n Remember that illness makes families poor, and movement down the so- cial scale leads to un predictable consequences in housing and (un)employ- ment. Families experiencing housing ins tability and food insecurity (without homelessness or hunger) are known to miss out on heal th care. 37 n=12,746 e d i c i u s s \200\231 d n a b s u h Marital disharmony may seem to be beyond the scope of pediatrics, but any holistic view of child health must put the family at the center of all at- tempts to foster child health and wellbeing. y m d a e r We see many families coping well with severe, prolonged illness in a child. But don \200 \231t presume that because things are OK in clinic today, you can aff ord to neglect the fo stering of family life. Given a certain amount of stress al- most all families will show ps ychopathology, in time. Your job is to prevent this if possible. Counseling skills are freq uently needed (p380) \200\224but do they work? Various tools are helpful in answering this: 38 \200 Communication is vital in any family, and this can be measured by scales I n e h t \200 e m o h y m n e h t such as the Communication Skills Test (CST).1 The Dyadic Adjustment Scale (DAS) looks at the emotional interaction within a marriage or a ny other pairing. 39 \200 The Miller Social Intimacy Scale (MSIS) looks at intimacy in re lationships, and intimacy is, beyond doubt, a source of strength within a family expe- rien cing illness in a child. d n a b o j y m t s o l Speci \201 c therapies validated in randomized trials in families coping with severe chron ic illness in a child include Emotionally Focused Marital Therapy (EMT), with these bene \201 ts: 40 \200 Higher overall levels of marital adjustment (on the DAS scale). None of t he couples receiving the active therapy deteriorated by >7 points, whereas one-third of cou ples in the control group had such a deteriora- tion (which shows the stress these families were under, and supports the notion that family psychopathology in the face of chronic chi ldhood illness is an evolving phenomenon \200\224the stress is reversible if you take certa in steps). \200 Better levels of intimacy as measured on MSIS scale, with eff ects per- si sting for at least 5 months. \200 Lower rates of negative communication (on the CST score) . \200 NNT=2 (2 couples need treating for one to improve). I \200 k c i s t o g n o s y m n e h w g n i h t y r e v e t s o l I \200\234 1 Full instruments available in: K Corcoran Measures for clinical practice: A sourcebook. N ew York. \200 The ill and feverish child Temperature Rectal is most reliable. 41 \200\22343 In the axilla: use an electronic or chem- ical dot thermometer. In the ear: use an infra-red tympa nic thermometer. \200 2007 3 questions to ask: How severe are the symptoms? and How approp riate is the child \200\231s response to the illness? The symptom may be severe (eg \200 \234terrible diarrhea all over the cot \200\235) without being biologically serious (if the baby is alert, drinking, wetting many nappies, and behaving as usual), but ANY appar- entl y mild symptom should set your internal alarm bell ringing if: 44,45 \200 Less than half the usual amount of feed has been taken in the last day. \200 There is breathing diffi cu lty, or high-pitched continuous moans or cries. \200 There is a history of being pale, mo ttled, cyanosed, and hot. \200 Dull expression; apathetic; uninterested in you; drowsy; d ehydrated. \200 A signi \201 cant reduction in the number of wet nappies in the last 24h . \200 Blood in diarrhea, or seizures, or tachycardia not explained by pain or fever. The 3rd question is: Is neutropenia possible (eg on chemotherapy)? Assess the \200\230traffi c light \200\231 way, p103, paying especial attention to red signs (pale, mottled, ashen bl ue, not staying awake when roused, etc). Always listen to your sixth sense: act if you have a bad feeling about this. Then observe the limbs: pain causes pseudoparalysis, eg with leg s in a frog position (hips & knees semi \202 exed, feet rotated outward), eg in osteomyeli tis, or septic arthri- tis. 46 \200\22348 The above signs carry extra weight if immunocompr omised (eg neo- nates; post measles; AIDS; cystic \201 brosis; leukemia; chemotherapy/mal ignancy; absent spleen; B/T cell dysfunction); congenital anomaly or foreign body, eg an in dwelling IVI line (bacteria may be unusual and of low virulence). 49 Fever (meningitis, p20 2; pneumonia, p160; UTI, p174.) Fever is the major acute presentation in pediatrics, eg fro m viruses, otitis media, or pharyngitis (p564), and prognosis is good. Here, fever may help (aids neutrophil migration; \200\200bio- activity of cytokines, \200\200interferon and \200\200 T-cell proliferation). 49 The challenge is to treat serious bacterial infections p romptly, without medicalizing normal child- hood ills. Do MSU within 24h of fever onset. Is LP needed? see above & p206. Bacteraemia occurs in 4% of febrile children and, in most, a source of in- fection is found by a good history and examination, blood & urine cultures, FBC/ \201 lm, CXR LP (see above), etc. Rapid virology is becoming available \200\224eg PCR and direct \202 uorescent assay (for adenoviruses). 50 ESR >30mm/h and \200\200C- r eactive protein also indicate bacterial infection \200\224eg Strep pneumoniae, N. meningiti dis. Sometimes the child (esp. between 3 and 24 months of age) has T \200\20639 C and W CC >15 \211\210109/L, but despite being feverish, he does not look ill, and no obvious focu s of infection can be found. This can be due to occult bac- teraemia, which is usually tran sient and self-limiting (although a small but signi \201 cant number of these children may develop a serious bacterial infec- tion). Management: Management of the febrile child is v ery resistant to writ- ten guidelines. NICE guidance (2007) gives a framework for the under 5s, but a large amount of clinical acumen is still needed. If in doubt, ask for senior hel p. If they look toxic, do bloods and a blood culture. If you have not identi \201 ed a sou rce for the infection, then do a full septic screen. With a sick patient, however, there sh ould be no delay: give cefotaxime 50mg/kg/6h or ceftriax- one1 50mg/kg/24h (for weights <50 kg). Most patients <3 months with a fever should be admitted, those under 1 month will inva riably need at least 48h of IV antibiotics. There is some leeway if aged 1 \200\2233 months : those that look unwell, or have a WCC >15 or <5 will need an LP and antibiotics, but if i nvestigations are normal and they look well some infants may be suitable for observation on ly/ outpatient management \200\224this will depend on the experience of the practitioner, o bservational skills of the parent and ease of follow-up. 51,52 106 \200 \200 s c i r t a d e a P i 1 Concomitant \200\224 with calcium contraindicates ceftriaxone use in neonates (?older ch ildren too): it causes precipitation in the lungs. Other CI: neonatal jaundice, albumin \200\201, acidosis, \200\201bilirubin binding. \200 Neonatal life support (NLS) after delivery 53,54 90% of neonates are perfectly health y: the best plan is to return these babies to the mother with no interference, to augment b onding. 55 Mother-and-baby skin-to-skin contact is ideal, rather than swaddling or nursery cots, and is the best way to main- tain temperature. 56 A pediatrician or nurse trained in advanced neonatal resuscitation should attend the following births: emer- gency caesars, br eeches, twins, forceps for fetal distress, intrapartum bleeding, prema- turity, hydrops fet alis, eclampsia and thickly stained meconium liquor. Before birth Check the equipment. Get a warm blanket. Heat the crib. At birth If pulse <100, poor color or res- piratory eff ort, set a clock in motion, and see OPPOSITE. Be alert to: \200 Hypothermia (use heat lamp). \200 Hypoglycaemia: glucose 10%, 2.5mL/kg IV. \200 Pethidine toxicity: naloxone 200 g (60 g/kg stat) IM or 10 g/kg repeated every few min; contraindicated in maternal opioid a buse. \200 Anemia (heavy fetal blood loss?) \200\224give 10mL/kg of 0.9% NaCl over 10 \200\22320 seconds. \200 Is there lung disease or congenital cyanotic heart disease (p136 )? Transfer to NICU/SCBU for monitoring. 107 \200 Dry the baby (unless <28 wks); place under radiant heat. \200\201 Set a clock in motion; a ssess col- our, tone, breathing & pulse. If not breathing after 60sec: \200\201 Control t he airway (head in the neutral position) \200\201 Support breathing: 5 in \202 ation brea ths; aim for in \202 ation pres- sure of 20cm H2O (max 40cm H2O). Con \201 rm response: v isible chest movements or \200\200heart rate. \200\201 If no response, check head posi- t ion and try a jaw thrust; then 5 in \202 ation breaths. Con \201 rm re- sponse: visible c hest movements or \200\200 heart rate. s c i r t a i d e a P \200\201 If still no response, get 2nd person to help with airway control and in \202 ati on breaths. Any sucking out of the pharynx should be un- der direct vision.59 \200 Repeat 5 in \202 ation breaths \200 Insert oropharyngeal airway \200 Repeat in \202 ation brea ths Consider intubation. Con \201 rm re- sponse: visible chest movements or increased hear t rate. Suck out oropharynx (before stimulating breathing!) only if meconium aspiration likely and the baby is \202 at. \200 21% O2 (air) is better than 100% O2. 54,57 Endotracheal intub ation is a key skill: use 3.5mm uncuff ed, unshouldered tubes on term infants; 3mm if 1.25 \200\223245kg (2.5mm if smaller). Learn from experts. Have many sizes to hand. Practice on models. Prognosis Mortality for prems is 315/1000 if 5min Apgar score 0 \200\2233, vs 5/100 0 if score is \211 7. Corresponding \201 gures at term are 244/1000 & 0.2/1000. If a ter m infant with Apgar score \211 3 has a low arterial pH ( \211 7), risk of neona- tal death increases 8-fold. 58 Survival in those needing CPR (cardiac resus) is 63% for infants of 0 .5 \200\2231.5kg, compared with 88% in these weight groups if CPR is not needed. Severe in- traventricular hemorrhage is seen in 15% of those needing CPR vs 5% in those who don t. \200\201 When chest is moving, continue with ventilation breaths if no spontaneous breathi ng. \200\201 Check heart rate; if absent or <60 start chest compressions. Do 3 chest compr essions to 1 breath, for 30sec \200\201 Reassess pulse: if improving, stop chest compressions. If not breathing, go on ventilating. If heart rate still \200\201 continue ventila- tion & chest compressions. \200\201 Consider IV or umbilical access & drugs, eg adrenaline (epinephrine): 10 g/kg (0 .1mL 1 : 10,000/kg) IV. At all stages ask Do I need help? Apgar Pulse Respirations Muscle tone Color 2 1 0 On suction Coughs well Depressed cough >100 <100 0 Strong cry Slow, irregular Limb \202 exion Nil Active Pink Blue limbs All blue or white No response Absent The neonatal intensive care unit (NICU) Neonatal intensive care is a technological developm ent of the basic creed of \201 rst aid \200\224 ABC; airway, breathing, and circulation. There is also an E. Epithelial cells determine whether low birthweight babies survive outsi de the uterus. They manage all interactions with the ex utero world: \200 Lung mechanics/ gas exchange \200 Renal tubular balance of \202 uid and electrolytes \200 Barrier fun ctions of the gut and skin for keeping bacteria out and water in, plus enabling diges- tion \200 Intact neuroepithelium lining of the ventricles of the brain and retina. 60 Monitor T , pulse, BP (intra-arterial if critical), respirations, blood gasses (SAO2 intra-art erial electrode), U&E, bilirubin, FBC, weight, weekly head circumference. The patient is us ually a premature baby. His mortal enemies are: cold, hypox- ia, hypoglycaemia (p112), resp iratory distress syndrome (p118), infection (p112); intraventricular hemorrhage; apnoea; ne crotizing enterocolitis (p120).1 You may become the problem: overzealous investigation/hand ling is damaging, 61 as is under-intervention. 62 Getting the balance right is vital. Cold With their small volume and relatively large surface area, this is a big problem for small babies. Incubators allow temperature (as well as humidity and FiO2) to be controlled, and a lso aff ord some protection against infection. FiO2 is the fraction of O2 in inspired air, ie 0.6=60% O2. Apnoeic attacks Prevalence: 25% of neonates <2.5kg; 90% if <1kg. Causes: Res piratory center immaturity; aspiration; heart failure; infection; PaO2 \200\201; glucose \200\201; Ca2+ \200\201; seizures; patent ductus (PDA); T \200\200 or \200\201; exhausti on; airway ob- struction. If stimulating the baby doesn \200\231t restore breathing, suck o ut the phar- ynx and use bag-and-mask ventilation. Avoid wild PaO2 \202 uctuations to pre vent ROP (below). Tests: CXR; U&E; infection screen; glucose; Ca2+; Mg2+. \201\227: If asp ira- tion is the problem, give small frequent feeds, or continuous tube feeds. Moni- tor SA O2 continuously; if hypoxic despite an ambient O2 of 40%, consider CPAP or IPPV. Caff eine citrate 20mg/kg PO/IVI stat, then 5mg/kg once daily starting 24h after stat dose \200\224so me neonates may need 10mg/kg; neonates >44 weeks postmenstrual age may need 10mg/kg/12h (fe wer SE than theophyllines). 63 108 \200 s c i r t a d e a P i Nasal CPAP doxapram may prevent hypoventilation. If apnoea is seizure-re- lated, see p11 2. Stopping ventilation: Try 4 \200\2235 days after apnoea has stopped. Prevention: Betamet hasone intrauterine maturation (p51). 64 RoP (retinopathy of prematurity) A disorder of the developing retina. Major risk factors are low birth weight and prematurity. Exposure to su pplemental oxygen is a cause, in particular large \202 uctuations in PaO2 , so careful ti tration of O2 levels has led to a decrease in the incidence of RoP. Abnormal \201 brovasc ular proliferation or retinal vessels may lead to retinal detachment and visual loss. Preva lence (lower limits): <1000g: 53%; \211 1250g: 43%; \211 1500g: 35%. 65 Classi- \201 ca tion: There are 5 stages, depending on site involved, the degree of retinal detachment, and extent (measured as clock hours in each eye). Treatment: Diode laser therapy causes less m yopia than cryotherapy. Screening: see BOX. IVH (intraventricular hemorrhage)2 occurs in 25 % if birthweight \200\2051500g. 66 Preterm infants are at risk of IVH due to unsupported b lood vessels in the sub- ependymal germinal matrix and the instability of blood pressure as sociated with birth trauma and respiratory distress. Delayed cord clamping in prems may \200\201risk. 67 Suspect in neonates who deteriorate rapidly (esp. in week 1). Signs: Seizu res, bulging fontanelle and cerebral irritability but many will have no clinical symptoms. 68 Tests: Ultrasound; CT. Complications: IQ \200\201, cerebral pal- sy, hydrocephalus (see DRIFT3). Many survive unscathed. \201\227: Meticulous nursing; head elevation; circulatory support, seizure control (p112). 1 UK perinatal death trends: deaths from congenital defects and hypoxia are 50% of 1980s ra tes, but death rates for infection are similar. Rates of multiple births and their problems are rising.69 2 Other types of bleeding: subdural; subarachnoid; parenchymal. MRI shows th at 26% of \200\230normal \200\231 babies have small bleeds, probably from skull pressure a t delivery. Bleeds were not found in cesarean births.70 3 DRIFT aims to treat IVH-associate d hydrocephalus. 109 The \201 rst breath and the road to pulmonary hypertension With our \201 rst breath pul monary vascular resistance falls, and there is a rush of blood to our lungs. 71 This is par tly mediated by endogenous nitric oxide (NO). This breath initiates changes from fetal to a dult circulation \200\224a process which may be interrupted in various conditions, eg mecon ium aspiration, pneumonia, respiratory distress syndrome, diaphragmatic hernia, group B str ep infection, and pulmonary hypoplasia. 72 Pulmonary hypertension arises as a consequence of these adverse events. It may also be due to hypertrophy of the muscular layer in the pulmonary arter- ies (primary pulmonary hyperte nsion). The chief diagnostic features are a background (eg meconium aspiration) and persisting desa turation despite intensive O2 use. When it is suspected, ar- range immediate echocardiograp hy, and get help. Echo will show right-to-left shunting at the ductus arteriosus in the abs ence of structural heart disease. s c i r t a i d e a P Inhaled nitric oxide (iNO)1 helps promote adult circulation and improves pul- monary outcom es for prems (eg 1000 \200\2231250g) who are at risk from BPD (p119). 73,74 It also \200 \201need for extracorporeal membrane oxygenation (below). It may also \200\201risk of brai n injury. iNO relaxes smooth muscle by \200\200production cyclic guano- sine monophosphate . (NB: sildena \201 l inhibits its degradation \200\224and may also have a role). 75,76 iN O may be associated with \200\200risk of intraventricular haemor- rhage. 77 Alternatives: adenosine, tolazoline, and epoprostenol (prostacyclin). 78 Ventilate (p110) gently, and cor rect reversible contributory factors (hypo- thermia, polycythaemia, hypocalcaemia, hypoglyc aemia). Give surfactant as indicated (p118). Persistent pulmonary hypertension occurs in 0.2% of live births. 79 Mortality is 10 \200 \22320% despite high-frequency ventilation, surfactant, inhaled nitric oxide, and extracorp oreal membrane oxygenation \200\224but is much higher when these therapies are not availabl e. 80 Extracorporeal membrane oxygenation (ECMO) ECMO is a complex proce- dure (done in spe cialized units) providing lifesupport for respiratory failure, which obviates the need for lung gas exchange. One trial showed that ECMO done after referral to one of 5 UK ECMO cen- tres \200\201death s from respiratory failure (oxygenation index \211 40).2 The trial was stopped early becau se the scrutineers found the results so strongly favored ECMO over traditional measures: 32 % died in the ECMO group, compared with 59% in the control group. The advantage was upheld irrespective of the pri- mary diagnosis, or the type of referring center. The number of inf ants suff er- ing severe disability at follow-up at 1yr was 1 in each group. 81N=185 Criteria which may make ECMO cost-eff ective: weight >2kg; no major con- genital malformati ons or CNS abnormality on ultrasound; gestation >34 weeks; oxygen index (OI) >40 (unrespons ive to NO inhalation). NB: more premature babies may bene \201 t, eg when problems with circuitry and heparinizat ion are solved. Need for ECMO may be 1 : 4000 live births. It is thought to be economicall y worthwhile. 82,83 Screening for retinopathy of prematurity84 Screening is recommended if <1500g or <32 weeks \200\231 gestation. If \211 27 weeks, screen at 30 \200\22331 weeks postmenstrual age. If born at 27 \200\22332 weeks then screen at 4 \200\2235 weeks post natal age. Screening ough t to be repeated 1 \200\2232 weekly depending on severity of disease. It must be done by an experienced ophthalmologist. 1 In one good trial a 24-day course of iNO started at 20 parts per million (for 48 \200\223 96h, reducing at weekly intervals to 10, 5, and 2 ppm).85 NB: iNO is new, expensive, and un certain; await more data before using. 2 What is the oxygen index (OI)? In the equation, MA P is the mean airway pressure in cmH2O.86 FiO2 is the partial pressure of inspired O2. OI=( FiO2 \211\210 MAP) PaO2 Ventilatory support for neonates This is a skill to be learned at the cot side. Nurses and specialist respiratory therapists will help you. Needs of apparently similar babies vary, s o what fol- lows is only a guide to prepare your mind before teaching. Continuous re \201 ne- ment in the light of transcutaneous and blood gas analysis is needed. The aims are to i mprove gas exchange, decrease work of breathing, and enable ventila- tion for those with re spiratory depression or apnoea. Non-invasive ventilation HFNC (high- \202 ow nasal cannula ) A high \202 ow nasal cannula delivers a distend- ing positive pressure to the airways s imilar to CPAP. Humidifying the gas deliv- ered decreased side eff ects of mucosal dryness. Use of HFNC may reduce the number of ventilated days compared to CPAP, but it is not yet c onsidered to be standard practice. 87 CPAP (continuous positive airways pressure) Pressure is raised throughout the respiratory cycle, so assisting spontaneous inspiration. With skil l, this method has few complications, and is useful as a \201 rst stage in ventilating a baby before it is known whether he or she will need IMV. NIPPV (nasal intermittent positive pressure ventilation) This combines nasal CPAP with superimposed ventilator breathing at a set pressure \200\224it can be used as a bridge between invasive ventilation and nasal CPA P. Invasive ventilation Invasive ventilation is broadly composed of conventional mechanical ven- tilation (CMV) (a.k.a intermittent mandatory ventilation (IMV)) and high fre- quency ventilation (HFV). TCPL (time-cycled pressure limited ventilation) Continuous \202 ows of heated and humidi \201 ed gas pass via an endotracheal tube. The breath delivered is set by peak inspiratory pressure (PIP) and either the absolute inspiratory time (TI), or the in spiratory:expiratory ratio (I:E ratio). The delivered tidal volume is de- pendent on lung c ompliance and resistance. Nasotracheal siting is best (fewer tube displacements). 88 TCPL a llows the infant to make respiratory eff orts be- tween ventilator breaths which can lead t o dysynchrony between the ventila- tor and the baby. Initial settings: Choose to give good chest in \202 ation and air entry on auscultation and adequate transcutaneous O2 readings. Typical settings might be TI 0.32sec, 40 cycles/min, inspiratory pressure 14 \200\22318cmH 2O, and PEEP 5cmH2O. Adjust in the light of blood gas analysis. PTV (patient-triggered vent ilation), including SIMV and SIPPV. PTV combines TCPL ventilation with a sensor which detec ts spontaneous breaths. The ventila- tor then delivers a breath which is synchronized with the infant \200\231s own inspira- tory eff ort. In PTV, inspiratory and end-expiratory pres sure is set by the opera- tor, but the rate (within limits) set by the baby. PTV is associa ted with a shorter duration of ventilation. 89,90 Hiccups cause problems if abdominal movem ent is used to detect inspiration. HFV (high-frequency ventilation) delivers small volumes of gas at very rapid rates. Its aim is to reduce ventilator-associated lung injuries. There are several diff erent types \200\224high-frequency positive pressure ventilation (HFPPV), jet ven- tilation, \202 ow interrupted and oscillatory ventilation (HFOV). HFPPV may red uce incidence of air leak compared to CMV (see BOX 4). 91 Paralysis Pancuronium (eg 30 g/k g IV; then 10 g/kg every 1.5 \200\2234h to maintain paralysis) prevents pneumothorax IV H (p108) in asynchronous respiratory ef- forts (eg needing unexpectedly high PI). 92 110 s c i r t a d e a P i 111 Factors associated with a good outcome from ventilation \200\207Don \200\231t base decisio ns on when to ventilate solely on degree of prematu- rity. Factors associated with a better outcome: antenatal corticosteroids, singleton pregnancy, \200 sex, and higher birthweigh t (in 100g increments). Each of these equates with 1 extra week of gestation in determining progno- sis. 93 In this study of infants born at 22 to 25 weeks \200\231 gestation, 49% di ed, 61% died or had profound impairment (eg neurosensory disability, IQ \200\201, or cerebr al palsy), and 73% died or had some impairment. Some complications of mechanical ventilation of neonates Because of the positive pressure i t produces, positive pressure ventilation will cause some haemodynamic compromise (hypotens ion, \200\201cardiac output). Lung Pneumothorax; pulmonary hemorrhage; bronchopulmonary dy splasia (p119); interstitial pulmonary emphysema; pneumonia. Multidrug-resistant organisms are often the cause of late-onset ventilator-associated pneumonia (cefepime has a role here ). 94 Post extubation atelectasis may be more fre- quent after nasal intubation (esp. in ve ry-low-birthweight infants). 95 Airways Upper airway obstruction (worse in inspiration and may cause stri- dor). Consider bronchoscopy (may show supraglottic lesions). Laryngomala- c ia gastro-oesophageal re \202 ux also occur, but more rarely. 96 Others Patent ductus arte riosus; \200\200intracranial pressure intraventricular hemorrhage (p108); retinopathy o f prematurity (p108); subcutaneous em- physema; pneumomediastinum; pneumopericardium; pneum operitoneum (transdiaphragmatic); air embolus. s c i r t a i d e a P Weaning from the ventilator Decrease the rate of IMV and lower PI by 2cmH2O at a time; try extubating if blood gas OK with 4cmH2O PEEP and a PIP of 12 \200\22314 with spontaneous br eaths over a backup rate of 5. Racemic adrenaline doesn \200\231t stop intubation-associated \200\200upper airways re- sistance. 97 Nasal intermittent positive pressure ventilation (NIPPV) delivers ventilator breaths via na sal prongs. If each breath is synchronized with the baby \200\231s own inspiratory eff orts , this may prevent the need for re-intubation. 98 Other factors Pain relief/sedation: Consider 5% glucose IVI with morphine 25 \200\223100 g /kg over \211 5min, then 5 \200\22340 g/kg/h. This is thought safe, and lowers catecholam ine concentrations (an objective correlate of pain and stress which helps form pain assessm ent tools \200\22599 ) \200\224only quasi-valid! 100 Air leak: Air ruptures alveoli tracki ng along vessels and bronchioles (pulmo- nary interstitial emphysema), and may extend intra pleurally (pneumothorax + lung collapse), or into the mediastinum or peritoneum. Associated with high PIP, it is less common with HFOV. Signs: Tachypnoea, cyanosis, chest asymmetry. The lateral decubitus CXR is often diagnostic if you have time. \200 Prompt \200\230blind \200\231 needle aspiration of a pneumothorax may be needed. Aspi- rate through the second intercostal space in the midclavicular line with a 25G \200\230butter \202 y \200\231 nee dle and a 50mL syringe on a 3-way tap. If the leak is continuous, use underwater seal drain age. \200 The ill neonate Sepsis Common (1 \200\22310/1000 births), and commonly overwhelming ( mortality 15 \200\22350%). Here, signs may be minimal, or as opposite (BOX). Action: ABC (p 108). Clear the airway; intubate and ventilate if necessary. This should correct acid- osis , so bicarbonate is rarely needed. Set up a colloid IVI (20mL/kg initially). Exclude hypogl ycaemia; do blood gasses. Infection screen: \200 Blood culture, virology, FBC, platelets, glucose, CXR. \200 Lumbar puncture: CSF (p202) for urgent Gram stain, cell count, protei n & glucose level, culture, and virology. \200 Stool: for virology. \200 Urine: microsc opy, culture, and virology. \200 ENT swabs: for culture. Antibiotics: 101 In early onset infection (ie neonates <48h old), group B streps and E. coli (+ any organism prevalent in y our NICU) are common. Treat with benzylpenicillin and gentamicin for 10 \200\22314 days. An alternative is amoxicil- lin and cefotaxime, but this may be less eff ective. 102 Doses: B enzylpenicillin: 50mg/kg/12h (increase to 8-hrly once >1wk old); gentamicin 4mg/kg/24h IV ( 36-hrly in prems <32 weeks \200\231 gestation). Note on dilutions Dilute 600mg of benzylpen icillin in 1.6mL of water; 600mg of the powder displaces 0.4mL, so \201 - nal concentrati on is 300mg/mL. In late-onset infection (ie neonates over 48h old), coagulase \200\223ve s taphs and listeria are possible, so regimens include \202 u- cloxacillin + gentamicin, am oxicillin + cefotaxime or amoxicillin + gentamicin. Vancomycin or teicoplanin (p195) may be used. If the CSF suggests meningi- tis, see p202. Neonatal seizures ( 4/1000 births) Cause s: Hypoxic-ischaemic encephalopa- thy (due to birth asphyxia/respiratory distress etc); inf ection (meningitis/en- cephalitis); intracranial hemorrhage/infarction; structural CNS lesi ons (focal cortical dysplasia/tuberous sclerosis); metabolic disturbance (hypoglycaemia; \200\201Ca2+; \200\200 \200\201Na+; \200\201Mg2+); metabolic disorders (urea cycle disord ers/amino acid metabolism). Diagnosis: Can be diffi cult as there may only be subtle clinic al signs of seizures (see BOX 2). EEG can con \201 rm seizure activity. Treatment: ABC. Tu rn on the side. Ask an experienced nurse to help. \200 Rule out or treat reversible cause s such as hypoglycaemia and electrolyte 112 \200 s c i r t a d e a P i disorders. Start on empirical antibiotics if possible sepsis. \200 Investigate to \201 nd cause of seizures. \200 If prolonged or repeated seizures consider anticonvulsants. First line Ph e- nobarbital Loading dose: 20mg/kg IV as slow injection. Second line: Phe- nytoin 18mg/kg IVI Other agents: clonazepam; midazolam; paraldehyde. If \201 ts continue: pyridoxine 50 \200\223100mg IV + 50 \200\223100mg/day PO (?for all <2yrs old with undiagnosed seizures: i n case of pyridoxine de \201 ciency).Autosomal recessive 103 3 \200\223 milk + calcium glu conate 10%, 4.4mL/kg/day Hypocalcaemia: Use low PO4 PO (IV: 0.5mL/kg diluted in 4.8mL/kg of saline over 5 \200\22310min). Monitor ECG. NB: American experts use higher IV emergency do ses: 2mL/kg of a 10% solution. Hypomagnesaemia: Give 100mg/kg of MgSO4 10% (=100mg/mL),[3] IV over >10min. 104 Shock Causes: Blood loss (placental hemorrhage, twin \200\223twin trans fusion; intraventricular hemorrhage; lung hemorrhage); capillary plasma leaks (sepsis, hypo xia, acidosis, necrotizing enterocolitis); \202 uid loss (D&V; \200\200insensible loss; inappropriate diuresis); cardiac causes (hypoxia, hypoglycaemia, L to R shunts, valve disea se, coarctation). Signs: Pulse \200\200; BP \200\201; urine output \200\201; coma. Manag ement: ABC. Ventilate as needed. Treat causes. Aim for a CVP of 5 \200\2238cm- H2O. Give co lloid 10 \200\22320mL/kg IV as needed. Inotropes may be used, eg dopa- mine 3 \200\22320 g /kg per min[4] dobutamine 5 \200\22320 g/kg/min as needed 105 (may act synergistically; detailed dosing: p203). There is scant experimental evidence for this regimen, and there m ay be side eff ects (blunting of respiratory drive; endocrine/immunological impairment; GI function \200\201). 106 Milrinone: see BNFc If the pH is <7.2, sodium bicarbonate, eg 1 \200\2232mmol/kg IV as a 4.2% solution, may be indicated (if adequate ventilation; don \200 \231t mix with inotropes). 113 Problems with neonatal sepsis Diagnosis is often hard, as signs may be non-speci \201 c, a nd cultures take time \200\224and time is never on your side. \200\207Sepsis is al- ways i n the diff erential diagnosis of any unwell neonate. 12 predictive signs for se- vere illness if <1 week old: \200 Seizures15 \200 Stiff lim bs15 \200 Cyanosis13 \200 Capillary re \201 ll \211 3sec10 \200 Diffi culty feeding 10 \200 Severe chest indrawing9 \200 T \211 35.5 9 \200 Movement only when stimu- Non-speci \201 c and probably unreliable tests: C-reactive protein, FBC and \201 lm: loo k- ing at the ratio of immature to total neutro- phils may help. NB: it \200\231s possible that looking for soluble immunological mediators may help: ideal ones may be interleukin-1 receptor antago- nist (IL-1ra), and interleukin-6 (IL-6). Speci \201 c- ity: 92% and 83%, respectively. Circulating intercellular adhesion molecule-1 (ciCA M-1) is less reliable.107 lated7 \200 Respiratory rate \211 603 \200 Lethargy3 \200 Grunting3 \200 T \211 37.5 3 A rule requiring any one sign has high sensitivity (87%) and speci- \201 city (7 4%). The number in blue refers to the odds ratio.108 s c i r t a i d e a P De \201 nitive cultures of bloodet al take 48 \200\22372h. Normal values are often hard t o de \201 ne, eg a CSF WCC of up to 10/mm3 with 60% polymorphs may be normal in neonates. Changing patterns of antibiotic resistance Ampicillin/amoxicillin and gentamicin is a safe combination for most neonates. This duo may reduce over-use of vancomycin.109 \200\223112 D rug pharmacokinetic problems These are only partly obviated by doing plasma levels. Creatin ine, sex, and birthweight in \202 uence what is an accept- able level in complex ways. Wit h aminoglycosides, adjust dose as indicated in the Data sheet if renal function is poor. If doing levels is problematic, ce- fotaxime is a good alternative (p202).113 Gentamicin: see p175. Subtle manifestations of neonatal seizures Unless you have a high index of suspicion, you m ay miss seizures referable to the brainstem, eg nystagmus, conjugate eye movements, posturi ng, sucking movements, lip smacking, etc. Grand mal is rare. EEGs are helpful in diagnos- i ng when unsure. Always look for metabolic abnormalities (these are treated with metabolic a pproaches; don \200\231t rely on conventional anticonvulsants). 114 Examination of the neonate1 The aim is to screen for abnormality, and to see if the mother has any ques- tions or diffi culties. The following is a recommended routine before the bab y leaves hospital \200\224or during the 1st week of life for home deliveries. Before the ex amination \201 nd out if the birthweight was normal. Was the birth and preg- nancy normal ? Is mother Rh \200\223ve? Find a quiet, warm, well-lit room. Enlist the mother \200\231s h elp. Explain your aims. Does she look angry or depressed? Listen if she talks. Examine syst ematically, eg head-to-toe. Wash your hands meticu- lously. Note observations (eg T ; rect al is more reliable than tympanic). 115,166 Head Circumference (50th centile=35cm, p224), s hape (odd shapes from a diffi - cult labor soon resolve), fontanelles (tense if crying or i ntracranial pressure \200\200; sunken if dehydrated). Eyes: Red re \202 ex (absent in cata ract & retinoblastoma); corneal opacities; conjunctivitis. Ears: Shape; position. Are they low set (ie below eyes)? The tip of the nose, when pressed, shows jaundice in white ba- bie s. Breathing out of the nose (shut the mouth) tests for choanal atresia. En- sure oto-acous tic screening is done (p548). Are follow-up brainstem evoked responses needed? 117 Complexi on: Cyanosed, pale, jaundiced, or ruddy (poly- cythaemia)? Mouth: Look inside; insert a \201 nger: is the palate intact? Is suck good? Does the baby \200\231s face look normal? Dy smorphism can be diffi cult to de- tect soon after birth as the baby may have some puffi ne ss in the face. Arms & hands Single palmar creases (normal or Down \200\231s). Waiter \200 \231s (porter \200\231s) tip sign of Erb \200\231s palsy of C5 & 6 trunks (p77; p766). Numb er of \201 ngers. Clinodac- tyly (5th \201 nger is curved towards the ring \201 nger, eg in Down \200\231s). Thorax Watch respirations; note grunting and intercostal recession (respira- tory distress). Palpate the precordium and apex beat. Listen to the heart and lun gs. Inspect the vertebral column for neural tube defects. Abdomen Expect to feel the liver. Any other masses? Inspect the umbilicus. Is it healthy? Flare suggests sepsis. Next, lift the skin to assess skin turgor. In- spect genitalia and anus. Are the ori \201 ces patent? Ensure in the 1st 24 hours the baby passes urine (consider posterior urethral valves in bo ys if not) and stool (consider Hirschprung \200\231s, cystic \201 brosis, hypothyroidism) . Is the urinary mea- tus misplaced (hypospadias), and are both testes descended? The neona tal clitoris often looks rather large, but if very large, consider CAH, p134. Bleeding PV m ay be a normal variant following maternal estrogen withdrawal. Legs Test for congenital dis location of the hip (p684). Avoid repeated tests as it hurts, and may induce dislocation. C an you feel femoral pulses (to \200\230exclude \200\231 coarctation)? Note talipes (p684). Toes: too many, too few, or too blue? Buttocks/sacrum Is there an anus? Are there \200 \230mongolian spots \200\231? (blue \200\224and harmless). Tufts of hair dimples suggest bi \201 da occulta? Any pilonidal sinus? CNS Assess posture and handle the baby. Intuitio n can be most helpful in decid- ing if the baby is ill or well. Is he jittery (hypoxia/isch aemia, encephalopathy, hypoglycaemia, infection, hypocalcaemia)? There should be some contr ol of the head. Do limbs move normally. Is the tone \202 oppy2 or spastic? Are responses absent on one side (hemiplegia)? The Moro re \202 ex rarely adds important infor- mation ( and is uncomfortable for the baby). It is done by sitting the baby at 45 , supporting the head. On momentarily removing the support the arms will ab- duct, the hands open and then t he arms adduct. Stroke the palm to elicit a grasp re \202 ex. Is the baby post-mature, lig ht-for-dates, or premature (p128)? \200\207Discuss any abnormality with the parents after liaising with a senior doctor. 114 \200 \200 s c i r t a d e a P i 1 The neonatal period is the 1st 28 days of life; if prem, 44 completed weeks of the infant \200\231s conceptional age (=the chronological age plus gestational age at birth).118 2 Ca uses of \202 oppiness: Sepsis; hypoglycaemia; dehydration; hypothermia; trauma/abuse; myo pathy; poor nutrition; botulism (may look like near sudden infant death syndrome)119; mater nal drugs (loraze- pam; clozapine); alcohol withdrawal; rickets; Down \200\231s, Ehlers \200\223Danlos, or Prader \200\223Willi syndromes; cerebral palsy; CNS syndromes \200\224eg muscular dystrophy, myasthenia, Zellweger leukodystrophy, Guillain \200\223Barr \200\224 or myotonic dystrophy (shake hands with the mother: she may have delayed release of grip). 120 Neonatal jaundice \200 121 Neonatal jaundice is common, occurring in 60% of neonates. \200 121 Most do not need any treatment. Jaundice is caused by raised bilirubin levels. Whe n severe, or not properly managed, kernicterus, a permanent form of brain damage, may occur . Transcutaneous bilirubin levels measured by midwives in homes may prevent kernicterus in babies discharged early by allowing early detec- tion of jaundice. In non-Caucasians, the d evice needs recalibration: don \200\231t rely on tests such as pressing the nose. 122 Manag ement of severe neonatal jaundice typically involves phototherapy, and exchange transfusion if very severe. The threshold levels for these treatments varies with gestational age, and post- natal age. (see NICE GUIDELINES at www.nice.org.uk/cg98) Hyperbilirubinaemia (<200 mol/L) after 24h is usually \200\230physiological \200\231: 1 Increased bilirubin producti on in neonates due to shorter RBC lifespan. 2 Decreased bilirubin conjugation due to hepati c immaturity. 3 Absence of gut \202 ora impedes elimination of bile pigment. 115 \200 s c i r t a i d e a P Exclusive breastfeeding (esp. if there are feeding diffi culties \200\203 \200\201intake \200\203 dehydration \200\203 \200\201bilirubin elimination + \200\200enterohepatic circul ation of biliru- bin \200\224not usually a reason to stop 123).