Neonatal jaundice. Vad menar folk när de Say Någon kan inte se skogen för alla träd?

Transcription

1 Neonatal jaundice Vad menar folk när de Say Någon kan inte se skogen för alla träd?

2 Document title: Neonatal jaundice Publication date: November 2012 Document number: Replaces document: MN12.7-V4-R17 MN09.7-V3-R12 Neonatal jaundice: prevention, assessment and management Author: Queensland Maternity and Neonatal Clinical Guidelines Program Audience: Health professionals in Queensland public and private maternity services Review date: November 2017 Endorsed by: Contact: Statewide Maternity and Neonatal Clinical Network Queensland Maternity and Neonatal Clinical Guidelines Program URL: Disclaimer These guidelines have been prepared to promote and facilitate standardisation and consistency of practice, using a multidisciplinary approach. Information in this guideline is current at time of publication. Queensland Health does not accept liability to any person for loss or damage incurred as a result of reliance upon the material contained in this guideline. Clinical material offered in this guideline does not replace or remove clinical judgement or the professional care and duty necessary for each specific patient case. Clinical care carried out in accordance with this guideline should be provided within the context of locally available resources and expertise. This Guideline does not address all elements of standard practice and assumes that individual clinicians are responsible to: Discuss care with consumers in an environment that is culturally appropriate and which enables respectful confidential discussion. This includes the use of interpreter services where necessary Advise consumers of their choice and ensure informed consent is obtained. Provide care within scope of practice, meet all legislative requirements and maintain standards of professional conduct Apply standard precautions and additional precautions as necessary, when delivering care Document all care in accordance with mandatory and local requirements This work is licensed under a Creative Commons Attribution Non-Commercial No Derivatives 2.5 Australia licence. To view a copy of this licence, visit State of Queensland (Queensland Health) 2010 In essence you are free to copy and communicate the work in its current form for non-commercial purposes, as long as you attribute the authors and abide by the licence terms. You may not alter or adapt the work in any way. For permissions beyond the scope of this licence contact: Intellectual Property Officer, Queensland Health, GPO Box 48, Brisbane Qld 4001, ip_officer@health.qld.gov.au, phone (07) For further information contact Queensland Maternity and Neonatal Clinical Guidelines Program, RBWH Post Office, Herston Qld 4029, MN-Guidelines@health.qld.gov.au phone (07) Refer to online version, destroy printed copies after use Page 2 of 35

3 Flow chart: Assessment and investigation of jaundice Refer to online version, destroy printed copies after use Page 3 of 35

4 Abbreviations ALP ALT AST CMV DAT EBM FBC GGT G6PD IVIg LED LFT MC&S Alkaline phosphatase Alanine aminotransferase Aspartate aminotransferase Cytomegalovirus Direct antiglobulin test (Coombs test) Expressed breast milk Full blood count Gamma glutamyl transpeptidase Glucose-6-phosphate dehydrogenase Intravenous immunoglobulin Light emitting diode Liver function test µw microwatts NBST NNT nm Microscopy, culture and sensitivity test Newborn screening test Number needed to treat Nanometre ± Plus or minus PCR QCC QCMB RFDS Rh Rh D RSQ TcB T 4 TFT TORCH TSB TSH Polymerase chain reaction Queensland Emergency Systems Coordination Centre Queensland Centre for Mothers and Babies Royal Flying Doctor Service Rhesus Rhesus antibody type D Retrieval Services Queensland Transcutaneous bilirubin Thyroxine Thyroid function test Toxoplasmosis, rubella, cytomegalovirus, herpes simplex, human immunodeficiency virus Total serum bilirubin Thyroid stimulating hormone Refer to online version, destroy printed copies after use Page 4 of 35

5 Definition of terms ABO incompatibility Conjugated bilirubin Conventional phototherapy G6PD deficiency Haemolysis Hyperbilirubinaemia Intensive phototherapy ABO incompatibility describes an antibody reaction that occurs when mother and baby have different blood groups, typically maternal blood group O, and baby blood group A or B. Some mothers have naturally occurring anti-a and anti-b antibodies present in the circulation, which can pass across the placenta and bind to antigenic sites on fetal red cell[s]. Some mothers are sensitised by feto-maternal transfusion of ABO incompatible blood. 1 Also known as direct bilirubin. The form of bilirubin after being processed by the liver. It is then transported by the biliary system to the intestine and excreted, although it can be broken down (de-conjugated) and reabsorbed from the gut. Bacterial breakdown of bilirubin in the intestine contributes to the colour of the faeces. 1 Where the light source, either fluorescent or halogen but not fibreoptic, is positioned above the baby. 1 When positioned 20 cm above the baby, spectral irradiance is should measure at 8-10 µw/cm 2 /nm over the waveband interval nm. 2 Is familial. At risk babies include those of Mediterranean, Middle Eastern, African or Southeast Asian origin. 3 It is of X linked recessive inheritance and is very rare in females. Red blood cell breakdown. Raised levels of bilirubin in the blood. 1 Single or multiple phototherapy with spectral irradiance measuring at least 30 µw/cm 2 /nm over the waveband interval nm. 4 Refers to the light intensity or energy output of the phototherapy unit and the number of photons (spectral energy) that are delivered per unit area (cm 2 ) of Irradiance exposed skin. Confounded by optical properties of skin and rates of bilirubin production and elimination. 5 mg/dl Convert mg/dl to micromol/litre by multiplying by Multiple Phototherapy that is administered by more than one light source phototherapy simultaneously. 1 Also known as intensive or double/triple phototherapy. Opisthotonos Backward arching of the back. 1 Sclera The white of the eye (plural sclerae). 1 Significant hyperbilirubinaemia An elevation of the serum bilirubin to a level requiring treatment. 1 Thermoneutral An environment that keeps the body temperature within a range that will minimise energy expenditure. 1 Unconjugated bilirubin Unconjugated hyperbilirubinaemia Also known as indirect bilirubin. The form of bilirubin which has not been processed by the liver. As unconjugated bilirubin can penetrate the blood-brain barrier, it is potentially toxic and may result in short and/or long term neurological dysfunction. 1 Occurs when the liver cannot process the amount of unconjugated bilirubin presented to it. This may occur due to: Excessive haemolysis and/or Immaturity of liver enzymes involved in conjugation Refer to online version, destroy printed copies after use Page 5 of 35

6 Table of Contents 1 Introduction Bilirubin encephalopathy Prevention Assessment All babies Bilirubin measurement All jaundiced babies Additional assessment jaundice visible at less than 24 hours of age Additional assessment jaundice first visible at 24 hours to 10 days of age Additional assessment onset of jaundice after 10 days of age and prolonged jaundice Additional assessment conjugated hyperbilirubinaemia Treatment Inter-hospital transfer Phototherapy Bilirubin monitoring Type of phototherapy equipment Efficacy Phototherapy care considerations Phototherapy observations and hydration Side-effects of phototherapy Cessation of phototherapy Exchange transfusion Adjunct therapy Discharge planning and follow-up Follow-up assessment References Appendix A: Kramer s rule Appendix B: Neonatal jaundice treatment graphs Acknowledgements List of Tables Table 1. Types of jaundice... 7 Table 2. Adverse jaundice sequelae... 8 Table 3. Prevention of jaundice... 9 Table 4. Care for all babies - clinical practice points Table 5. Types of bilirubin measurement Table 6. Assessment for all babies with jaundice Table 7. Differential diagnosis and investigations for babies less than 24 hours of age Table 8. Differential diagnosis and investigations for babies 24 hours to 10 days of age Table 9. Differential diagnosis and investigations for after 10 days of age Table 10. Differential diagnosis and investigations for conjugated hyperbilirubinaemia Table 11. Serum bilirubin measuring and monitoring Table 12. Type of phototherapy equipment depending on gestation and serum bilirubin Table 13. Elements affecting phototherapy efficacy Table 14. Phototherapy care Table 15. Observations and hydration Table 16. Side-effects of phototherapy Table 17. Serum bilirubin measuring and monitoring ceasing phototherapy Table 18. Exchange transfusion Table 19. Adjunct therapy Table 20. Elements of discharge planning Table 21. Follow-up assessment Refer to online version, destroy printed copies after use Page 6 of 35

7 1 Introduction During the first week of life all newborns have increased bilirubin levels by adult standards, with approximately 50-60% of term 1,6 and 80% of preterm 1 babies developing jaundice. Neonatal jaundice refers to the yellow discolouration of the skin and sclera caused by the accumulation of bilirubin in the skin and mucous membranes. This is associated with hyperbilirubinaemia. Although most jaundice is physiologically normal [refer to Table 1], it is important to detect pathological causes of jaundice and those babies at risk of significant hyperbilirubinaemia with the aim of preventing bilirubin encephalopathy [refer to Table 2]. Table 1. Types of jaundice Jaundice Physiological Breast milk jaundice Pathological Explanation Physiological jaundice occurs due to the 1 : o Higher concentration of red blood cells in newborns o Shorter life span of newborn red blood cells o Slower metabolism, circulation and excretion of bilirubin in newborns Usually appears 2 to 4 days after birth, resolving after 1 to 2 weeks 6 (3 weeks if preterm) Frequently exacerbated by inadequate milk intake Is not associated with underlying disease and is usually benign Develops 5 to 7 days after birth and peaks at 14 days 7 At one month of age, approximately 10% of breastfed babies remain jaundiced 1 A suggested cause is an increased concentration of β-glucuronidase in breast milk and the associated increase in deconjugation and reabsorption of bilirubin 7 Is benign Refer to Sections for causes and investigations associated with time of onset of jaundice Refer to Section 1.1 Bilirubin encephalopathy Refer to online version, destroy printed copies after use Page 7 of 35

8 1.1 Bilirubin encephalopathy Unbound unconjugated bilirubin can penetrate the blood-brain barrier. It is potentially toxic and may result in short and/or long term neurological dysfunction 1 [refer to Table 2]. There is a poor correlation between circulating bilirubin levels and severity of encephalopathy. Table 2. Adverse jaundice sequelae Adverse sequelae Risk factors Acute bilirubin encephalopathy Chronic bilirubin encephalopathy Kernicterus Explanation Potential risk factors for neurotoxicity include: o Preterm birth 1 o Rapid rate of rise of serum bilirubin 1 o Hypoalbuminaemia o Other co-morbidities (sepsis, asphyxia and acidosis) Refers to the clinical manifestations of bilirubin toxicity 1 seen in the first few weeks after birth 5 Initial signs include: o Lethargy 5 o Irritability 1 o Apnoea 1 o Hypotonia and poor suck progressing to 3 : Hypertonia (opisthotonos) High pitched cry, and Eventually seizures and coma Refers to the pathogenic diagnosis of persistent brain dysfunction arising from hyperbilirubinaemia 1 Clinical findings of chronic bilirubin encephalopathy include 3 : o Athetoid cerebral palsy with or without seizures o Developmental delay o Hearing deficit o Oculomotor disturbances including paralysis of upward gaze (Parinaud s sign) o Dental dysplasia o Intellectual impairment A pathology term, referring to the yellow staining of the basal nuclei of the brain 1 The term is frequently used to refer to the clinical syndrome and sequelae, including acute and [more commonly the] chronic brain effects, of bilirubin encephalopathy 1 Refer to online version, destroy printed copies after use Page 8 of 35

9 2 Prevention Table 3. Prevention of jaundice Element Pregnancy, labour and birth Breastfeeding Clinical practice points Test all pregnant women for ABO, Rh (D) blood types and red cell antibodies 5,3 during pregnancy: o If maternal red blood cell antibodies noted antenatally, test cord blood: Blood group 1 including Rh type Direct antiglobulin test (DAT/Coombs test) 5,3 Full blood count (FBC) for haemoglobin and haematocrit Discuss with neonatologist 3 If the mother has not had antenatal blood tests send: o Maternal blood for blood group (ABO/Rh), and o Baby s cord blood for blood group 1, Rh type and DAT 5,3 Tests not predictive of severe hyperbilirubinaemia include 1 : o Umbilical cord blood total serum bilirubin o End-tidal carbon monoxide measurement (ETCOc) o Routine umbilical cord DAT Delayed cord clamping is recommended by the World Health Organization. 8 It is associated with an increased risk of jaundice requiring phototherapy, which is offset by improved iron status of the newborn for up to 6 months 9 Support all women who choose to breastfeed 1 Encourage demand feeding or at least 3-4 hourly or as age appropriate Consider referral to a midwife or Lactation Consultant (if available) to provide the mother with feeding support 3 Promote the ingestion of colostrum to increase stooling to prevent reabsorption of bilirubin o Use of suppositories and enemas to increase stooling is of no clinical benefit 3 Supplementation with water and/or dextrose water does not affect bilirubin levels and is not recommended 3 o If supplemental fluids are indicated, then maternal expressed breast milk (EBM) is the feed of choice Refer to Guideline: Breastfeeding initiation 10 Refer to online version, destroy printed copies after use Page 9 of 35

10 3 Assessment 3.1 All babies Table 4. Care for all babies - clinical practice points Element All babies Visual examination Clinical practice points Identify babies with risk factors associated with significant hyperbilirubinaemia 1 : o Gestational age less than 38 weeks o A previous sibling requiring phototherapy o Mother s intention to breastfeed exclusively [refer to Section 2 and Guideline: Breastfeeding initiation 10 ] o Visible jaundice within the first 24 hours following birth If risk factor identified: o For visible jaundice within the first 24 hours following birth an urgent medical review is required within 6 hours [refer to Section 3.4] o For other risk factors arrange an additional visual inspection, by a healthcare professional, within the first 48 hours of the baby s life Examine all babies for jaundice at every opportunity especially in the first 72 hours 1,3 Educate and encourage parents and carers to observe for signs of adequate hydration, feeding and jaundice [refer to Queensland Centre for Mothers and Babies (QCMB) Neonatal jaundice parent information sheet 11 ] Ensure appropriate discharge planning and follow-up [refer to Section 1] Always assess the baby in a bright and preferably natural light 1 (e.g. in daylight by a window 5 ) Assess the sclerae, gums and blanch the baby s skin with a finger to observe the underlying skin colour 1 Jaundice appears first in the face and progresses caudally to the trunk and extremities 1 [Refer to Appendix A for associated information] Visual estimation of bilirubin levels can lead to errors 2,3 particularly in babies 1 : o With darker skin tones o Who are preterm o Under 36 hours of age o Who are receiving phototherapy Visual examination is sufficient to exclude jaundice 1 Do not measure bilirubin levels routinely in babies who are not visibly jaundiced 1 Consider a bilirubin measurement in any baby who is jaundiced [refer to Table 5] Refer to online version, destroy printed copies after use Page 10 of 35

11 3.2 Bilirubin measurement Table 5. Types of bilirubin measurement Visible jaundice Transcutaneous bilirubin (TcB) Total serum bilirubin Clinical practice points Only for babies 1 : o Gestational age of 35 weeks or more o More than 24 hours of age May be particularly useful in health care settings where total serum bilirubin level results are not expected to be available within 6 hours Prior to commencing phototherapy, consider a photo-opaque patch applied to the baby s skin (e.g. the forehead) 12 o TcB measurements have been shown to be accurate when taken on skin that is not exposed to phototherapy 12 Base clinical decisions on a TcB trend rather than a single value 12 If TcB is greater than 250 micromol/l check serum bilirubin 1 Use according to the manufacturer s information (e.g. calibration requirements) TcB measurements may decrease the numbers of heel pricks and/or invasive blood tests required 13 If TcB is not indicated or available 1 For babies 1 : o Gestational age of less than 35 weeks o Less than 24 hours of age Preferable to TcB for babies within treatment thresholds and for subsequent measurements 1 Treat venous and capillary total serum bilirubin levels the same 5,3 If collected in the community: o Collect in heparinised, light protected blood tube Ensure sample is mixed well o Protect from light (e.g. by using brown paper bag) o Avoid excessive heat (e.g. carry in esky/cooler box in the car) o Enable analysis as soon as practical o Consider collection of full blood count at the same time Refer to online version, destroy printed copies after use Page 11 of 35

12 3.3 All jaundiced babies Table 6. Assessment for all babies with jaundice All jaundiced babies Clinical practice points Assessment Jaundice approaching exchange level Measure and record bilirubin level in all babies within 6 hours of identifying obvious or suspected significant jaundice o Interpret bilirubin levels according to the baby s postnatal age in hours and manage hyperbilirubinaemia according to the Neonatal jaundice treatment graphs 1 [refer to Appendix B] Do not use sunlight as a treatment for jaundice or hyperbilirubinaemia 1 Parents and carers: o Provide information on treatment for hyperbilirubinaemia if indicated o Breastfeeding babies [refer to Section 2]: Encourage mother to breastfeed frequently (e.g. every 3 hours) and to wake their baby for feeds if necessary Develop a feeding plan and provide lactation support Jaundice may be a sign of serious illness Review the history: o Family history of significant haemolysis (including G6PD deficiency) Clinical examination including: o Feeding since birth o Weight o Hydration including elimination (number of wet nappies and stools) o Risk factors: Preterm birth Onset of jaundice within 24 hours [refer to Table 7] Sepsis Asphyxia Acidosis Signs of illness (e.g. lethargy, poor feeding, fever/temperature instability, vomiting, significant weight loss, irritability) Dark urine and light stools [refer to Table 9 and Table 10] Bilirubin is rising more than 8.5 micromol/l per hour Jaundice reappears after an initial improvement Jaundice persisting after 2 weeks in a term baby and 3 weeks in a preterm baby [refer to Table 9] Investigate the cause of jaundice if it is not explained by the history and clinical examination 5 [refer to Sections ] Do not use the albumin/bilirubin ratio when making decisions about the management of hyperbilirubinaemia 1 Refer to neonatologist/paediatrician for management if results abnormal or jaundice persisting without cause Seek neonatologist/paediatrician advice Consider further investigations o Direct (conjugated) bilirubin abnormal if greater than 25 micromol/l 1 o Liver function test (LFT) o G6PD deficiency 1 o Screen for Gilbert Syndrome Refer to online version, destroy printed copies after use Page 12 of 35

13 3.4 Additional assessment jaundice visible at less than 24 hours of age Table 7. Differential diagnosis and investigations for babies less than 24 hours of age Jaundice visible at less than 24 hours Clinical practice point Differential diagnosis Investigations A medical emergency: o Measure and record the serum bilirubin within 2 hours of identifying obvious or suspected jaundice 1 Commence phototherapy whilst awaiting serum bilirubin results o Urgent neonatology/paediatric/medical review required: Within 6 hours of identifying obvious or suspected jaundice To exclude pathological causes of jaundice Level 1-3 Neonatal services: o Organise transfer to nearest referral service [refer to Section 4.1] Almost always pathological Usually due to haemolysis: o Rhesus disease o ABO incompatibility o Red cell enzyme defects (e.g. G6PD deficiency) Sepsis (e.g. acute/intrauterine infection) Rarer causes may include: o Other blood group incompatibilities (Kell, Duffy, anti-e) o Red cell membrane defects (hereditary spherocytosis) Refer to Table 6. Assessment for all babies with jaundice Routine 1 : o TSB for baseline level to assess response to treatment o Full blood count. Consider ± blood film o Blood group (maternal and baby) 1 o DAT consider strength of reaction 1 (DAT may have a weak false positive for mothers who received Anti D during pregnancy) Consider 1 : o G6PD deficiency o Microbiological cultures (MC&S) of: Blood Urine Cerebrospinal fluid Refer to online version, destroy printed copies after use Page 13 of 35

14 3.5 Additional assessment jaundice first visible at 24 hours to 10 days of age Table 8. Differential diagnosis and investigations for babies 24 hours to 10 days of age Jaundice first visible at 24 hours to 10 days Clinical practice point Differential diagnosis Investigations Measure and record the transcutaneous or serum bilirubin level within 6 hours of identifying obvious or suspecting significant jaundice 1 Commence phototherapy if bilirubin higher than the Neonatal jaundice treatment graph threshold [refer to Appendix B] Medical review required Level 1-3 neonatal services, consider phototherapy if results are unavailable within 6 hours, and: o Baby has risk factors o TcB is greater than 250 micromol/l or above treatment threshold o Jaundice is below the nipple line 13 Consider transfer to a higher level facility if bilirubin not responding to treatment Most commonly benign physiological jaundice Dehydration Sepsis Haemolysis Polycythemia Breakdown of extravasated blood (e.g. bruising) Increased entero-hepatic circulation which may be due to gut obstruction Metabolic disease including galactosaemia Refer to Table 6. Assessment for all babies with jaundice Routine 1 : o TSB for baseline level to assess response to treatment o Full blood count. Consider ± blood film o Blood group (maternal and baby) 1 o DAT consider strength of reaction 1 (DAT may have a weak false positive for mothers who received Anti D during pregnancy) o Newborn Screening Test (NBST) if not already taken Consider: o G6PD deficiency (at risk newborns include Mediterranean, Middle Eastern, African or Southeast Asian origin) 1,3 o MC&S 1 : Blood Urine Cerebrospinal fluid o TORCH screen Refer to online version, destroy printed copies after use Page 14 of 35

15 3.6 Additional assessment onset of jaundice after 10 days of age and prolonged jaundice Table 9. Differential diagnosis and investigations for after 10 days of age Onset of jaundice after 10 days and prolonged jaundice Prolonged jaundice Differential diagnosis Investigations Obvious persisting clinical jaundice at greater than 2 weeks of age in term babies and greater than 3 weeks of age in preterm babies Sepsis Hypothyroidism Hypopituitarism Hypoadrenalism Haemolytic anaemia Hereditary Spherocytosis Pyloric stenosis or gastrointestinal obstruction Breast milk jaundice Requires investigation due to the risk of serious disease Medical review including: o Examination/enquiry regarding stool colour Pale stools and dark urine require urgent discussion with a neonatologist/paediatrician/gastro-enterologist o Review of previous pathology results Routine: o Total and conjugated bilirubin If conjugated bilirubin greater than 25 micromol/l refer to Table 10 o FBC + blood film o Reticulocyte count o Blood group o DAT consider strength of reaction 1 (DAT may have a weak false positive for mothers who received Anti D during pregnancy) o Thyroid function test (TFT) (including TSH and T 4 ) o Review NBST results* If the baby is unwell: o Discuss with a paediatrician/neonatologist o Consider further investigations Septic screen If above test results are normal, the baby is well and breastfeeding, it is likely to be breast milk jaundice * Health Services Support Agency, Pathology Queensland, Central Laboratory, Chemical Pathology, Newborn screening: neonatal_screening@health.qld.gov.au; phone or Refer to online version, destroy printed copies after use Page 15 of 35

16 3.7 Additional assessment conjugated hyperbilirubinaemia Table 10. Differential diagnosis and investigations for conjugated hyperbilirubinaemia Conjugated hyperbilirubinaemia Differential diagnosis Investigations Congenital obstruction and malformations of the biliary system (e.g. biliary atresia, choledochal cyst, bile duct stenosis) Idiopathic neonatal hepatitis Infections (Hepatitis B, TORCH, sepsis, intrauterine) Metabolic disorders (galactosaemia, hereditary fructose intolerance, Alpha- 1 antitrypsin deficiency, tyrosinaemia, glycogen storage disease type IV, hypothyroidism) Prolonged parenteral nutrition Conjugated hyperbilirubinaemia requires urgent discussion with a neonatologist/paediatrician/gastro-enterologist Consider initiating investigations by requesting: o Total serum bilirubin and conjugated bilirubin levels o LFT (including: AST, ALT, GGT, ALP and albumin) o Coagulation screen o Blood gas (with blood glucose) o Liver ultrasound o Ferritin o TFTs (including TSH and T 4 ) o Alpha-1-antitrypsin phenotype o Urine: Cytomegalovirus (CMV) PCR Culture and sensitivity Reducing substances Additional investigations to consider include: o Urine: Organic acids Amino acids o Serum amino acids o Plasma: Ammonia Pyruvate Lactate 4 Treatment Hyperbilirubinaemia can be treated with phototherapy, exchange transfusion and pharmacological agents. 1, 3 Adequate hydration is also an important consideration in the baby with moderate to high bilirubin levels. It is important to also treat the underlying illnesses that may be causing jaundice (e.g. infection). 4.1 Inter-hospital transfer Management options will depend on the services available at each facility [refer to the Queensland Health Clinical Services Capability Framework 14 ]. For management advice, contact the Queensland Emergency Medical Systems Coordination Centre (QCC) by calling Transfer or referral to a higher level facility for management and treatment options may be appropriate. This will be coordinated by the QCC via Retrieval Services Queensland (RSQ) and a Neonatal medical coordinator (phone: ) or the Royal Flying Doctor Service ((RFDS) as per local procedures). Refer to Guideline: Neonatal stabilisation for retrieval. Babies requiring inter-hospital transfer for management of jaundice by phototherapy or exchange transfusion, require phototherapy (at least a biliblanket) and IV fluids en-route (this can be provided by the retrieval team). Refer to online version, destroy printed copies after use Page 16 of 35

17 4.2 Phototherapy Bilirubin monitoring Table 11. Serum bilirubin measuring and monitoring Phototherapy Commencing During Continuous multiple phototherapy Clinical practice point When assessing the need for phototherapy, do not subtract the direct reacting or conjugated bilirubin level from the total serum bilirubin level 5 Interpret bilirubin levels according to the baby s postnatal age in hours and manage hyperbilirubinaemia according to the Neonatal jaundice treatment graphs 1 [refer to Appendix B] o For the first 14 days after birth, use the graph that reflects the baby s gestational age at birth Do not commence phototherapy unless the bilirubin level exceeds the threshold For babies greater than 12 hours old with a serum bilirubin level 1-50 micromol/l below the phototherapy threshold: o Repeat the serum bilirubin in 6-24 hours as clinically indicated Document time and type of phototherapy commenced Refer to Section Phototherapy care considerations If the serum bilirubin is decreasing or unlikely to be significantly higher in the next hours, then measure the serum bilirubin hourly If after 24 hours, the serum bilirubin is stable or falling: o Measure the serum bilirubin every 24 hours until bilirubin is below treatment threshold [refer to Table 17] Increase frequency of serum bilirubin measurements as clinically indicated o Consider measuring the bilirubin 4-6 hourly until the rise of serum bilirubin has stabilised Switch off phototherapy light during blood collection Initiate if the serum bilirubin level 1 : o Is rising rapidly (greater than 8.5 micromol/l/hour) o Is less than 50 micromol/l below the exchange transfusion threshold o Does not fall in response to single phototherapy Reduce to single phototherapy when the serum bilirubin level: o Is at minimum 50 micromol/l below the threshold for which exchange transfusion is indicated 1 Ceasing Refer to Section Cessation of phototherapy Refer to online version, destroy printed copies after use Page 17 of 35

18 4.2.2 Type of phototherapy equipment As well as effectiveness, also consider the impact on maternal and baby bonding when choosing phototherapy equipment to treat neonatal jaundice. Table 12. Type of phototherapy equipment depending on gestation and serum bilirubin Element Term babies ( 37 weeks) Preterm babies (< 37 weeks) Continuous multiple phototherapy Clinical practice point Conventional blue light 1, overhead light emitting diodes (LED) 15 and other modes of phototherapy are preferred to fibreoptic phototherapy as first line treatment Conventional compared to fibreoptic phototherapy is associated with: o A greater decrease in serum bilirubin o Shorter duration of phototherapy 1,6 Use either fibreoptic, conventional blue light or LED phototherapy Conventional compared to fibreoptic phototherapy is associated with 1 : o A longer duration of phototherapy Early phototherapy is associated with a lower mean peak in serum bilirubin level 1 Usually requires at least 2 banks of phototherapy lights 3 or the use of a combination of methods (e.g. conventional phototherapy plus biliblanket) o Single LED devices may deliver the same spectral irradiance as multiple phototherapy 4 Use special blue fluorescent tubes or specially designed LEDs 5 if available Efficacy Table 13. Elements affecting phototherapy efficacy Element Clinical practice point Equipment Maintain and use according to manufacturers instructions Wavelengths in the blue-green spectrum (~ nm) are effective with Light source special blue the most effective (~460 nm) 4 Light (spectral) irradiance Energy output is usually measured in microwatts per cm 2 (µw/cm 2 ) and is normally marked on each phototherapy unit Conventional phototherapy spectral irradiance should measure 8-10 µw/cm 2 /nm over the waveband interval nm 2 Continuous multiple (intensive) phototherapy spectral irradiance should measure least 30 µw/cm 2 /nm over the waveband interval nm 4 Confirm spectral irradiance with an appropriate meter calibrated over the appropriate wavelength range 4 Check phototherapy units regularly using the method recommended by the manufacturer 1, to ensure adequate irradiance is delivered: o Do not exceed recommended hours of use prior to technical service as irradiance of all lamps decreases with use 4 Maintain distance from baby and orientation of light source according to manufacturer s instructions 4 Light rays should be perpendicular to the surface of the incubator to minimise reflectance 4 Refer to online version, destroy printed copies after use Page 18 of 35

19 4.2.4 Phototherapy care considerations Table 14. Phototherapy care Element Clinical practice point Equipment Conventional Incubator or open cot according to clinical need 1 and unit policy Fibreoptic pad Incubator or open cot according to clinical need (e.g. biliblanket) LED Incubator or open cot according to clinical need and unit policy Phototherapy bed (e.g. bilibed) Open cot or as per manufacturer s recommendation Consider adding/changing to bank of lights if total serum bilirubin level continues to rise Maximise exposed body surface area 1 o Total body surface area exposed to phototherapy is clinically more important than the number of devices used Clothing Remove all clothing (except folded down disposable nappy) o Remove nappy when serum bilirubin levels continue to rise or continuous multiple phototherapy is required Place fibreoptic pads 15 or a LED mattress below the baby Fibreoptic pad Place fibreoptic pad between skin and singlet o Writing side facing away from baby, illuminating side facing baby o Cable at baby s feet, tip of the pad at the baby s shoulders o Turn variable intensity knob to fully on (e.g. represented by sun) Parents are able to handle baby Phototherapy bed Dress only in manufacturer s jumpsuit to maximise exposure to light Supine recommended 1 Baby s position In intensive and special care nurseries, babies who are nursed in a prone position (for clinical reasons) require continuous cardio respiratory monitoring 16 Required to protect immature retina 3,17 Remove with feeds/cares 18 o Perform eye care as indicated Eye patches o Replace before recommencing phototherapy Refer to manufacturers recommendations for fibreoptic pads and phototherapy beds, as eye patches are recommended by some manufacturers Discuss and provide parents with information including: o Why phototherapy is being considered o The possible adverse effects of phototherapy o The need for eye protection and routine eye care o Reassurance that short breaks for feeding, nappy changing and cuddles will be encouraged o What might happen if phototherapy fails o Rebound jaundice o Potential long-term adverse effects of phototherapy Parents & carers o Potential impact on breastfeeding and how to minimise this o Refer to QCMB Neonatal jaundice Parent information sheet 11 Where possible, enable mother and baby to remain together while baby receives phototherapy Provide support and encourage parental interaction with their baby 1 Consider home phototherapy for low risk babies o Refer to local guidelines o Regular monitoring of serum bilirubin is essential 5 o Refer to Section 5 Discharge planning and follow-up Refer to online version, destroy printed copies after use Page 19 of 35

20 4.2.5 Phototherapy observations and hydration Table 15. Observations and hydration Element Clinical practice point Ensure the baby is kept in a thermoneutral environment 1 to minimise the side effects of temperature instability 15 Temperature Monitor the baby s temperature 1 : o Hourly for the first 4 hours 18, then 4-6 hourly (e.g. with feeds) Incubator Conventional phototherapy may lead to an elevated incubator temperature: o Do not turn the incubator off It is not safe to nurse a baby in an incubator that has been turned off as air no longer circulates LED devices produce minimal heat 15 Cover temperature probe with reflective disc if servo control method is used to monitor temperature o Servo control should only be used by experienced clinicians Other observations Hydration Conventional blue light and LED phototherapy As per clinical condition and/or maturity With conventional blue light therapy it is difficult to distinguish cyanotic colour changes in the baby ensure an apnoea or saturation monitor is also used Check for skin rashes [refer to Photosensitivity row in Table 16] Report dark urine and/or light (pale) stools Provide lactation and feeding support 1 o Monitor attachment, milk transfer (sucking and swallowing) and maternal milk supply o Breastfeeding on demand at least 3-4 hourly or as age appropriate Wake baby if necessary o Phototherapy often starts at the same time the breast milk supply is increasing. Therefore additional feeds may not be necessary Assess wet nappies 1 Document input and output Daily weigh 1 if required Additional feeds may be indicated with: o Excessive weight loss (e.g. greater than 12 percent of birth weight in breastfed babies 19 ) o Evidence of hypovolaemia or dehydration (e.g. decreased urine output) o Stool output is less than three small stools a day 20 If additional feeds are indicated: o Maternal EBM is the preferred choice 1 o Do not supplement with water or dextrose water 1,2 o Intragastric feeds with EBM and/or artificial formula (if parents/carers are agreeable) may be required 2 Discuss risk and benefits and obtain consent for the use of formula o Intravenous therapy may be indicated in severe cases or preferred by some mothers to protect breastfeeding May be discontinued for shorts breaks (e.g. up to 30 minutes) for breastfeeding, nappy changing and cuddles 1 Does not routinely require additional fluids or feeds 1 Fibreoptic Remains in-situ during feeds Continuous multiple phototherapy Do not interrupt for feeding 1 consider intragastric feeding Continue lactation and feeding support to assist with breastfeeding when treatment ceases 1 Refer to online version, destroy printed copies after use Page 20 of 35

21 4.2.6 Side-effects of phototherapy Table 16. Side-effects of phototherapy Side effect Insensible water loss Intestinal hypermotility Photosensitivity Bronze baby syndrome Parental and carer anxiety Considerations Changes in the baby s thermal environment leads to increased peripheral blood flow and insensible water loss 17 Likely to be more of an issue with conventional rather than LED phototherapy Associated with distended abdomen, loose stools and diarrhoea 3 Congenital porphyria or a family history of porphyria is a contraindication to the use of phototherapy. 5,17 Babies with congenital erythropoietic porphyria can develop severe blistering and photosensitivity during phototherapy Concomitant use of photosensitising drugs or agents is a contraindication to phototherapy 5,17 Babies with cholestatic jaundice may development bronze baby syndrome 15 and rarely purpura and bullous eruptions 5 Potential separation may cause interference in mother baby interaction 5,3 Some parents find eye patching disturbing 5 Refer to Table 14. Phototherapy care Cessation of phototherapy Table 17. Serum bilirubin measuring and monitoring ceasing phototherapy Phototherapy Ceasing Clinical practice points Cease phototherapy when serum bilirubin is at least 50 micromol/l below the phototherapy threshold 1 o Inform the parents/carers about the possibility of rebound jaundice and the potential need for follow-up serum bilirubin Check for rebound with repeat serum bilirubin hours after ceasing phototherapy o Assess the need for the baby to stay in hospital during this time o Babies at increased risk of clinically significant rebound are those 7 : Born at less than 37 weeks gestation With haemolytic disease Treated with phototherapy during the birth hospitalisation Refer to online version, destroy printed copies after use Page 21 of 35

22 4.3 Exchange transfusion A total serum bilirubin level at or above the exchange transfusion level should be considered a medical emergency. Commence continuous multiple phototherapy immediately 1,2 and discuss further care with a neonatologist. Table 18. Exchange transfusion Elements Indications 1 Preparing for exchange transfusion Possible adverse effects Considerations The serum bilirubin level is above the exchange transfusion threshold and is not expected to be below the threshold after 6 hours of continuous multiple phototherapy 1 Immediate exchange transfusion is recommended if: o There are signs of bilirubin encephalopathy 1 Exchange transfusion should only be performed by trained personnel in a Level 6 or Level 5 (in consultation with a Level 6) neonatal intensive care service 14 o If immediate exchange transfusion is required discuss the management of this situation with a neonatologist o Arrange transfer to an appropriate higher level facility as required [refer to Section 4.1 and Guideline: Neonatal stabilisation for retrieval 21 ] Facilities undertaking exchange transfusions should ensure local guidelines are in place Initiate/maintain continuous multiple phototherapy 1 Consider Intravenous Immunoglobulin (IVIg) 1 : o Refer to Table 19 for IVIg indications and clinical practice points Discuss with parents or carers ensuring information is provided on 1 : o Why the treatment is being considered o Anticipated duration of treatment o Possible adverse effects o When it would be possible for parents to see and hold their baby o The need to admit their baby to an intensive care nursery Consider collecting blood for investigation of the rare causes of severe hyperbilirubinaemia 3 : o Discuss with a neonatologist o There is no value in collecting blood for investigation of causes of jaundice after an exchange transfusion Thrombocytopenia 1 Hypocalcaemia 1 Catheter malfunction Hypotension 1 Venous thrombosis 1,22 Hypokalaemia Hypoglycaemia 1 Vasospasm 22 Apnoea 22 Bradycardia 22 Cyanosis 22 Necrotising enterocolitis 22 Refer to online version, destroy printed copies after use Page 22 of 35

23 4.4 Adjunct therapy Table 19. Adjunct therapy Therapy Intravenous Immunoglobulin Other Consideration and clinical practice points Maintain continuous multiple phototherapy Consult with a neonatologist prior to treatment Consider IVIg (500 mg/kg over 4 hours) in a baby with the following exceptional circumstances 1 : o Rhesus haemolytic disease or ABO haemolytic disease, and o The serum bilirubin is rising by more than 8.5 micromol/l/hour despite continuous multiple phototherapy Refer to the National Blood Authority Australia and current product information sheet 7 for further administration information Reduces the use of and number of exchange transfusions per baby in Rhesus haemolytic disease (NNT = 2-3) or ABO haemolytic disease (NNT = 5-13) 1 The use of IVIg may be recommended in special circumstances such as 23 : o Parental refusal for exchange transfusion o Where appropriate blood components for exchange transfusion are unavailable Offer parents or carers information on IVIg including: o Why IVIg is being considered o Why IVIg may be needed to treat significant hyperbilirubinaemia o The possible adverse effects of IVIg o When it will be possible for parents or carers to see and hold the baby Evidence does not support the use of: 1 : o Agar o Albumin o Barbiturates o Charcoal o Cholestyramine o Clofibrate o D-penicillamine o Glycerin o Manna o Metalloporphyrins o Riboflavin o Traditional Chinese medicine o Acupuncture o Homeopathy o Sunlight Refer to online version, destroy printed copies after use Page 23 of 35

24 5 Discharge planning and follow-up Table 20. Elements of discharge planning Elements Parents and carers Consideration and clinical practice points Discuss and provide written information on the signs of jaundice, adequate hydration and feeding 1 Reassure that neonatal jaundice is common and usually transient o Refer to QCMB Neonatal jaundice Parent information sheet 11 Advise parents against exposure to sunlight as a treatment for jaundice If the cause of jaundice is G6PD deficiency advise parents: o Against the use of naphthalene o To wash all clothing/ bedding that may have been exposed to Naphthalene Advise parents and carers to seek urgent advice from a healthcare professional if their baby becomes jaundiced within the first 24 hours of life Parents and carers should also seek advice from a healthcare professional if their baby: o Becomes jaundice o Has worsening jaundice o Has jaundice persisting beyond 14 days o Is passing pale chalky stools or dark urine o Is not feeding well o Shows signs of dehydration Organise and/or advise parent(s) to have their baby examined by a qualified health care professional in the first few days after discharge to assess if the baby is well and for the presence of jaundice 2,3 Timing and location of this assessment is determined by 2,3 : o Length of hospital stay o Presence of associated risk factors for significant hyperbilirubinaemia [refer to Table 4] o Risk of other neonatal problems Recommended follow-up Baby discharged Should be seen by Number of visits Before 24 hours of age Between 24 and 48 hours of age Between 48 and 72 hours of age 72 hours of age 5 If risk factor(s) present, baby well and not visibly jaundiced 48 hours 1 96 hours of age hours of age 5 2 visits 5 : hours hours Increase frequency if risk factors present or as indicated 5 Within the first 24 hours of life do not discharge a baby with visible jaundice investigate as per Sections 3.3 and 3.4 Never discharge a baby with conjugated hyperbilirubinaemia without attempting to find the cause investigate as per Sections 3.3 and 3.7 If appropriate follow-up cannot be arranged and there are risk factors it may be necessary to delay discharge until 5 : o Follow-up can be arranged o The greatest risk has passed (72-96 hours) Ensure discharge documentation includes a summary for parents and clinicians including any follow-up blood tests (e.g. repeat FBC and serum bilirubin levels) that are required Refer to online version, destroy printed copies after use Page 24 of 35

25 5.1 Follow-up assessment Table 21. Follow-up assessment Elements Follow-up assessment Consideration and clinical practice points Follow-up assessment must include 2 : o Baby s weight and percentage change from birth weight o Review of feeding history to determine adequacy of intake o Voiding and stooling pattern o Presence or absence of jaundice o Clinical judgement to determine the need for total serum bilirubin level measurement If there is any doubt measure the total serum bilirubin level 2 Other follow-up considerations include: o Healthy hearing o Encephalopathy long term medical follow-up recommended o Haemolysis follow-up for 4-6 weeks, due to potential for anaemia: Advise weekly FBC and reticulocyte count Folic acid supplementation if continued haemolysis is suspected Refer to online version, destroy printed copies after use Page 25 of 35

26 References 1. National Institute for Health and Clinical Excellence. Neonatal jaundice. CG98. London: National Insititute for Health and Clinical Excellence; Gregory MLP, Martin CR, Cloherty JP. Neonatal hyperbilirubinemia. In: Cloherty JP, Eichenwald EC, Hansen AR, Stark AR, editors. Manual of neonatal care. 7th ed. Philadelphia: Lippincott Williams & Wilkins Canadian Paediatric Society. Position statement: (FN ). Guidelines for detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants (35 or more weeks' gestation). Paediatric Child Health. 2007; 12 Supplement B(May/June):1B-12B. 4. American Academy of Pediatrics. Technical report: Phototherapy to prevent severe neonatal hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2011; 128:e1046-e American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Clinical Practice Guideline: Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 2004; 114: DOI: /peds Woodgate P, Jardine LA. Neonatal jaundice. BMJ: Clinical Evidence. 2011; 09: National Blood Authority Australia, Standing Council on Health. Criteria for the clinical use of intravenous immunoglobulin in Australia. Second edition [cited 2012 August 25]. Available from: 8. World Health Organization, Department of Making Pregnancy Safer. WHO recommendations for the prevention of postpartum haemorrhage McDonald S, Middleton P. Effect of timing of umbilical cord clamping of term infants on maternal and neonatal outcomes. Cochrane Database of Systematic Reviews. 2008; Issue 2. Art. No.: CD DOI: / CD pub Queensland Maternity and Neonatal Clinical Guidelines Program. Breastfeeding initiation. Guideline No. MN10.19-V2-R15. Queensland Health [cited 2012 September 4]. Available from: Queensland Centre for Mothers and Babies, Queensland Maternity and Neonatal Clinical Guidelines Program. Parent information sheet. Neonatal jaundice [cited 2012 August 25]. Available from: Zecca E, Barone G, Daniele DL, Marra R, Tiberi E, Romagnoli C, et al. Skin bilirubin measurement during phototherapy in preterm and term newborn infants. Early Human Development. 2009; doi: /j.earlhumdev Maisels MJ. Noninvasive measurements of bilirubin. Pediatrics. 2012; 129(4): Queensland Health. Clinical Services Capability Framework for Public and Licensed Private Health Facilities v3.0. Brisbane: Queensland Government Department of Health Kumar P, Chawla D, Deorari A. Light-emitting diode phototherapy for unconjugated hyperbilirubinaemia in neonates. Cochrane Database of Systematic Reviews. 2011; Issue 12. Art. No.: CD DOI: / CD pub Queensland Health. Safe infant care to reduce the risk of sudden unexpected deaths in infancy policy statement and guidelines. Queensland Government; Maisels MJ, McDonagh AF. Phototherapy for neonatal jaundice. The New England Journal of Medicine. 2008; 358(9): Truman P. Jaundice in the preterm infant: effective management. Journal of Neonatal Nursing. 2003; 9(1): Wong R, Bhutani V. Treatment of unconjugated hyperbilirubinemia in term and late preterm infants. UpToDate [cited 2012 April 27]. Available from: Refer to online version, destroy printed copies after use Page 26 of 35

27 20. Wong R, Bhutani V. Pathogenesis and etiology of unconjugated hyperbilirubinemia in the newborn. UpToDate [cited 2012 April 27]. Available from: Queensland Maternity and Neonatal Clinical Guidelines Program. Neonatal stabilisation for retrieval. Guideline No. MN11.18-V1-R16. Queensland Health [cited 2012 September 4]. Available from: US Preventive Services Task Force (USPSTF). Screening of infants for hyperbilirubinemia to prevent chronic bilirubin encephalopathy: US Preventive Services Task Force recommendation statement. Rockville (MD): Agency for Healthcare Research and Quality (AHRQ); Horn A, Kirsten G, Kroon S, Henning P, Moller G, Pieper C, et al. Phototherapy and exchange transfusion for neonatal hyperbilirubinaemia: neonatal academic hospitals' consensus guidelines for South African hospitals and primary care facilities. South African Medical Journal. 2006; 96(9): Kramer LI. Advancement of dermal icterus in the jaundiced newborn. American Journal of Diseases of Children. 1969; 118(September): Refer to online version, destroy printed copies after use Page 27 of 35

28 Appendix A: Kramer s rule Please note: Visual estimation of bilirubin levels can lead to errors particularly in babies 1 : o With darker skin tones o Who are preterm o Under 36 hours of age o Who have already commenced phototherapy Visual estimation should not be relied on to estimate the bilirubin level in a baby with jaundice 1 Kramer recognised the cephalocaudal progression of jaundice with increasing total serum bilirubin levels and divided the baby into 5 zones, with a total serum bilirubin level measurement associated with each zone. This is known as Kramer s rule (see Figure 1) and has traditionally been used to visually assess the severity of jaundice. 24 Zone Definition TSB (micromol/l) 1 Head and neck Upper trunk Lower trunk and thighs Arms and lower legs Palms and soles >250 Figure 1. Kramer s Rule Refer to online version, destroy printed copies after use Page 28 of 35

29 Appendix B: Neonatal jaundice treatment graphs These example forms require approval for use by local health service. Refer to online version, destroy printed copies after use Page 29 of 35

30 Refer to online version, destroy printed copies after use Page 30 of 35

31 Refer to online version, destroy printed copies after use Page 31 of 35

32 Refer to online version, destroy printed copies after use Page 32 of 35

33 Refer to online version, destroy printed copies after use Page 33 of 35

34 Refer to online version, destroy printed copies after use Page 34 of 35