Polycythaemia Neonatal Management Clinical Guideline

V1.0

June 2021

Summary

Infant identified as at risk of Capillary Hct Infants at risk polycythaemia with identified >65% and Capillary  Infant of one sign or one sign or Haematocrit symptom diabetic mother symptom identified (Hct) >70%  Prolonged delayed cord clamping >2 mins  Twin to Twin transfusion Venous Full Blood Count  Intrauterine sample growth restriction (IUGR)  Cord pH <7.0  Large for gestational age Asymptomatic Symptomatic >98th centile  Chromosomal anomalies such as Trisomy 21, 18 and 13 Venous Hct Venous Hct Venous Hct Venous Hct  Severe 65-70% >70% 65-70% >70% Preeclampsia

Observations 4 Admit to NNU hourly for 12 hours Neonatal Unit 4 hours NEWS Observations 4 hourly 2 x pre feed blood IV dextrose observations. for 12 hours. sugars ECG Monitoring Ensure 2 x pre feed blood Ensure adequate High risk feeding adequate sugars. hydration, increase regime hydration, urine Ensure adequate fluid intake to one Fluid balance output. Observe hydration, urine day ahead if Monitor for signs of output. tolerated. electrolytes and Signs or jaundice. Observe for signs of Observing bilirubin Symptoms jaundice. adequate urine 12 hourly FBC  Hypoglycaemia Repeat FBC after 12 output until requiring hours. Observe for signs asymptomatic treatment with of jaundice and Hct <70% IV Dextrose  Jaundice requiring Symptomatic or phototherapy worsening FBC  CNS symptoms with unknown Venous cause - Hct 75% Irritability, If worsening lethargy, symptoms seizure  Poor feed absorption - not Consider Partial Exchange transfusion via UVC/UAC tolerating and peripheral venous line hourly feeds, Nil by Mouth until Hct < 65% then as per high risk bilious feeding regime aspirates Monitor urine output and fluid balance and investigate  Urine output < further causes 1ml/kg/hr Regular electrolytes and bilirubin and plot on appropriate chart 6-12 hourly FBC depending on severity of symptoms

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 2 of 14

1. Aim/Purpose of this Guideline

1.1. To assist staff with identifying possible causes for, and infants at risk of Neonatal Polycythaemia. This guidance is for the management of these infants in order to prevent further complications.

Data Protection Act 2018 (General Data Protection Regulation – GDPR) Legislation The Trust has a duty under the DPA18 to ensure that there is a valid legal basis to process personal and sensitive data. The legal basis for processing must be identified and documented before the processing begins. In many cases we may need consent; this must be explicit, informed and documented. We cannot rely on opt out, it must be opt in.

DPA18 is applicable to all staff; this includes those working as contractors and providers of services.

For more information about your obligations under the DPA18 please see the Information Use Framework Policy or contact the Information Governance Team [email protected]

2. The Guidance

Polycythaemia is defined as a venous packed cell volume/haematocrit (Hct) of over 65%. This condition affects approximately 1-5% of newborns in the postnatal period. Many affected infants are asymptomatic and the characteristic clinical features and related complications are thought to occur as a result of hyperviscosity. It is however important to note that not all polycythaemic infants suffer from hyperviscosity. Hyperviscosity can result in serious clinical consequences due to reduced blood flow to organs leading to poor tissue perfusion and possible microthrombus formation.

Sampling variability can occur when measuring haematocrit and therefore a venous, free flowing sample is gold standard and must always be obtained to confirm diagnosis. The treatment of polycythaemia is also variable because it is uncertain whether intervention affects long-term outcome and may be associated with some gastrointestinal morbidity.

Dehydration is also a common case of polycythaemia when identified into the 2nd -3rd day of life. This should be corrected and treated accordingly then haematocrit reviewed subsequently.

2.1. Infants at risk of Polycythaemia

 Infant of diabetic mother

 Delayed Cord clamping with other risk factors

 Twin to Twin transfusion

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 3 of 14

 Intrauterine growth restriction (IUGR)

 Intrapartum Hypoxia

 Large for gestational age

 Chromosomal anomalies such as Trisomy 21, 18 and 13

 Maternal Preeclampsia

 Endocrine abnormalities such as hypothyroidism, congenital adrenal hyperplasia

2.2. Signs and Symptoms

Many polycythaemic infants will be asymptomatic and only appear plethoric on examination. Polycythaemic infants who are developing hyperviscocity may present with these signs and symptoms over the first 24 hours;

 Respiratory distress +/- Apnoea

 Cyanosis

 Cardiopulmonary complications such as tachycardia, Persistent Pulmonary Hypertension of the Newborn (PPHN), cardiomegaly

 Gastrointestinal problems such as poor feeding, vomiting, Necrotising enterocolitis (NEC)

 Neurological complications such as lethargy, irritability, jitteriness, seizures

 Persistent hypoglycaemia requiring high GIR

 Hypocalcaemia

 Jaundice requiring more than standard phototherapy

 Renal complications such as decreased urine output, renal vein thrombosis

 Coagulation difficulties such as thrombocytopenia

2.3. Investigations

Haematocrit in term infants, who appear clinically well, should not be measured routinely, including those with risk factors. However, if signs or symptoms occur then the following investigations should be undertaken:

 Full maternal and infant history to identify predisposing factors

 Systematic clinical examination including: respiratory status, capillary refill time, femorals and bowel sounds

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 4 of 14

 Identify any risk of dehydration such as significant weight loss in first 5 days

 In any infant identified to be at risk of polycythaemia and presenting with one of the stated signs or symptoms they must have a venous full blood count (FBC) sent to the laboratory with a full electrolyte sample (green bottle) and blood gas for blood glucose

The diagnosis of polycythaemia can only be made from a venous Hct. It is important to acknowledge that many infants may present with symptoms similar to those attributed to polycythaemia with hyperviscosity and that other aetiologies should also be considered. Symptoms of hyperviscosity are likely to be severe and persistent.

2.4. Management

2.4.1. Asymptomatic and Symptomatic Management

2.4.1.1. Asymptomatic Infants with Hct 65-70%

 Clinical polycythaemia management not required

 Ensure adequate hydration by monitoring oral intake and seeking prompt support from infant feeding team if required

 Observing adequate urine output

 Observe for signs of jaundice

2.4.1.2. Asymptomatic Infants with Hct 70-75%

 Observations 4 hourly for 12 hours, following flowchart if any symptoms occur

 Two pre-feed blood sugars and manage as per Hypoglycaemia guideline if required

 Ensure adequate hydration by monitoring oral intake and seeking prompt support from infant feeding team if required

 Observe adequate urine output

 Observe for signs of jaundice

 Repeat venous FBC after 12 hours

2.4.1.3. Symptomatic Infants with Hct 65-70%- see decision making flowchart

 Observations 4 hourly for 12 hours, following flowchart if any further symptoms occur Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 5 of 14

 Two pre-feed blood sugars and manage as per Hypoglycaemia guideline if required

 Ensure adequate hydration by monitoring oral intake and seeking prompt support from infant feeding team if required. Increase fluid intake to one day ahead, pass nasogastric tube if unable to maintain volumes orally

 Observing adequate urine output

 Observe for signs of jaundice

2.4.1.4. Symptomatic infants with Hct >70%- see decision making flowchart

 Consider admitting to Neonatal Unit and commence IV dextrose for 24 hours minimum (can be fed a day ahead if mild symptoms only- e.g. jaundice only requiring phototherapy, one off hypoglycaemia)

 ECG Monitoring

 High risk feeding regime

 Monitor fluid balance and urine output

 Monitor electrolytes and bilirubin and plot on appropriate chart

 12 hourly FBC and electrolytes until asymptomatic and HCT <70%

 If worsening symptoms such as persistent hypoglycaemia or respiratory distress despite intravenous fluids, to discuss with Neonatal Consultant about performing a partial exchange transfusion

2.4.1.5. Symptomatic or Asymptomatic infants with Hct >75%- see decision making flowchart

 Consider Partial Exchange transfusion via Umbilical Venous Catheter/Umbilical Arterial Catheter and peripheral venous line

 Consideration should be given to investigation of other causes of polycythaemia especially if persistent/worsening symptoms

 Nil by mouth until Hct < 65% then as per high risk feeding regime

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 6 of 14

 Monitor urine output and fluid balance

 Regular electrolytes and bilirubin, and plot on appropriate chart

 6-12 hourly FBC depending on severity of symptoms

2.4.2. Partial Exchange Transfusion

2.4.2.1. The accepted treatment of polycythaemia is partial exchange transfusion (PET), however it should be noted that PET has not been shown to have beneficial effects on long term outcomes and due to associated complications should therefore be discussed with the Neonatal Consultant before being undertaken(1). Partial Exchange Transfusion in preterm infants is higher risk and must always be discussed with the Neonatal Consultant; with a cranial ultrasound pre-procedure for all infants <34 weeks CGA.

2.4.2.2. PET involves slowly removing some of the blood volume and replacing the withdrawn blood with fluids to help dilute the red blood cell concentration. Partial exchange transfusion must take place in an intensive care setting with continuous monitoring, following informed parental consent.

2.4.2.3. Vascular access will be required for the transfusion to take place ideally a UAC/UVC (blood out) and a peripheral venous line (saline in).

2.4.2.4. Volume to be exchanged is 15ml/kg of blood with an at least 15ml/kg 0.9% Sodium Chloride, this should be performed in 5ml/kg aliquots over 30 minutes (90 minutes total time). Ensure baby’s thermal care is maintained during the procedure (2,3).

2.4.2.5. Equipment

 Cardio-respiratory monitoring equipment, including saturation and blood pressure monitoring

 Volumetric pump

 Sterile gowns, gloves and pack

 Closed system urine bag/ paediatric waste urine bag and extension set

 Assorted lure lock syringes, 3 way taps and extension sets depending on access

 Exchange transfusion observation and in/out chart Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 7 of 14

 Clock

2.4.2.6. Procedure

 Use aliquots of 5 mL/kg; withdraw blood and infuse an equal amount of saline

 First remove aliquot of blood in volume as above. Always ensure blood is removed first

 This first aliquot withdrawn should be sent for FBC, U&E, LFT, phosphate, SBR

 Hct, Calcium, magnesium, glucose and clotting

 Stop partial exchange transfusion if infant’s condition suddenly deteriorates – but always

 Leave infant’s blood volume in balance

 Sudden deterioration maybe be due to underlying condition, the procedure or an adverse reaction to transfusion

 Blood glucose should be measured prior to and after procedure

2.4.2.7. Complications of PET

 Gastrointestinal disturbances (e.g. abdominal distention, vomiting)

 Necrotising enterocolitis

 Electrolyte disturbances e.g. Hypoglycaemia

 Infection

2.4.2.8. Procedure: Nursing roles and Responsibilities

 Do not interrupt the phototherapy during exchange.

 Ensure parents are fully informed and have consented to procedure

 Ensure baby is in the Intensive care room and begin monitoring heart rate, respirations, blood pressure, oxygen saturations and temperature. Record baseline observations prior to procedure and then every 15 mins throughout the exchange

 Maintain neutral thermal environment, ideally in open cot

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 8 of 14

 Baby must be nil by mouth prior to an exchange, leave NGT on free drainage

 Administer IV fluids as prescribed and observe all lines as per policy

 Record pre transfusion blood glucose and then following the procedure

 Ensure day 0 blood spot sample has been taken and labelled

 Use a chart to document in and out volumes

 All clinical staff should be ready to start simultaneously and should have no other workload ie 1:1 care

 All lines should be monitored for extravasation, blanching, erythema, leakage, and oedema. They should be recorded as per Trust policy

 On completion the lines should be flushed with saline to maintain patency

 Inform parents that procedure has been completed

 Documentation - contemporaneously documented, accurate fluid balances, sample taken, observations, and any untoward reactions and outcomes reported appropriately

2.4.2.9. Nursing care post procedure

 Monitor and record blood glucoses at 1,2 and 4 hours post transfusion

 Observe for any distension, vomiting, and blood in stools

 Urinalysis

 Repeat FBC 6 hours post PET

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 9 of 14

3. Monitoring compliance and effectiveness

Element to be Compliance with policy/ key changes to practice monitored Lead Neonatal Guidelines Lead and Author

Tool Adherence to guidelines will be monitored as part of the ongoing audit process on a Word or Excel template Frequency As dictated by audit findings Reporting Neonatal Guidelines Group arrangements Acting on Neonatal Guidelines Lead and Author recommendations and Lead(s) Change in Required changes to practice will be identified and actioned within practice and 3 months, immediately if required. A lead member of the team will lessons to be be identified to take each change forward where appropriate. shared Lessons will be shared with all the relevant staff/stakeholders

4. Equality and Diversity

4.1. This document complies with the Royal Cornwall Hospitals NHS Trust service Equality and Diversity statement which can be found in the 'Equality, Inclusion & Human Rights Policy' or the Equality and Diversity website.

4.2. Equality Impact Assessment

The Initial Equality Impact Assessment Screening Form is at Appendix 2.

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 10 of 14

Appendix 1. Governance Information Polycythaemia Neonatal Management Clinical Document Title Guideline V1.0 This document replaces (exact New Document title of previous version): Date Issued/Approved: January 2021

Date Valid From: June 2021

Date Valid To: June 2024

Directorate / Department Jenna Julian; Neonatal Nurse responsible (author/owner):

Contact details: 01872 252667

Guideline on the management of neonates with Brief summary of contents suspected or confirmed polycythaemia

Suggested Keywords: Neonatal Polycythaemia, Exchange Transfusion RCHT CFT KCCG Target Audience  Executive Director responsible Medical Director for Policy: Approval route for consultation Neonatal Guidelines Group and ratification:

General Manager confirming Mary Baulch approval processes Name of Governance Lead confirming approval by specialty Caroline Amukusana and care group management meetings Links to key external standards None Required 1. Dempsey, E. M., Barrington, K. (2006) Short and long term outcomes following partial exchange transfusion in the polycythaemic newborn: a systematic review. Arch Dis Child Fetal Neonatal Ed. 91(1). Related Documents: 2. Ghoshal, S., Matthews, Y. Y. (2011) Polycythaemia and Hyperviscosity in Newborn. 3. ADHD (2012) Newborn Clinical services guideline- Partial Exchange Transfusion. 4. Garcia- Prat, J.A. (2019) Neonatal Polycythemia.

Training Need Identified? No Publication Location (refer to Policy on Policies – Approvals Internet & Intranet  Intranet Only and Ratification):

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 11 of 14

Document Library Folder/Sub Clinical/ Neonatal Folder

Version Control Table

Version Changes Made by Date Summary of Changes No (Name and Job Title) January Jenna Julian; V1.0 Initial issue 2021 Neonatal Nurse

All or part of this document can be released under the Freedom of Information Act 2000

This document is to be retained for 10 years from the date of expiry. This document is only valid on the day of printing

Controlled Document This document has been created following the Royal Cornwall Hospitals NHS Trust Policy for the Development and Management of Knowledge, Procedural and Web Documents (The Policy on Policies). It should not be altered in any way without the express permission of the author or their Line Manager.

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 12 of 14

Appendix 2. Equality Impact Assessment

Section 1: Equality Impact Assessment Form Name of the strategy / policy /proposal / service function to be assessed Polycythaemia Neonatal Management Clinical Guideline V1.0 Directorate and service area: Is this a new or existing Policy? Neonatal New Name of individual/group completing EIA Contact details: Neonatal Guidelines Group 01872 252667 1. Policy Aim Who is the strategy / policy / Medical and Nursing staff caring for Neonates within proposal / service Maternity and Neonates function aimed at?

2. Policy Objectives Provide a framework for assessment and management of infants with symptomatic and asymptomatic polycythaemia.

3. Policy Intended Outcomes To improve the well-being of patients by offering the appropriate management.

4. How will you measure Audit/ Multidisciplinary team weekly discussion/ incidents/ risk the outcome? management

5. Who is intended to benefit from the Patients and their families. policy? 6a). Who did you Local External Workforce Patients Other consult with? groups organisations x

b). Please list any Please record specific names of groups: groups who have been consulted Neonatal Guidelines Group about this procedure. c). What was the outcome of the consultation?

Approved- 20th January 2021

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 13 of 14

7. The Impact Please complete the following table. If you are unsure/don’t know if there is a negative impact you need to repeat the consultation step. Are there concerns that the policy could have a positive/negative impact on: Protected Yes No Unsure Rationale for Assessment / Existing Evidence Characteristic Age X Sex (male, female non-binary, asexual X etc.)

Gender reassignment X Race/ethnic Any information provided should be in an communities accessible format for the parent/carer needs – i.e. X /groups available in different languages if required/access to an interpreter if required Disability (learning disability, Those parent/carers with any identified additional physical disability, needs will be referred for additional support as sensory impairment, appropriate- i.e. to the Liaison team or for X mental health specialised equipment. problems and some Written information will be provided in a format to long term health meet the family’s needs e.g. easy read, audio etc conditions) Religion/ All staff should be aware of any beliefs that may X other beliefs impact on the decision to treat Marriage and civil partnership X Pregnancy and maternity X

Sexual orientation (bisexual, gay, X heterosexual, lesbian) If all characteristics are ticked ‘no’, and this is not a major working or service change, you can end the assessment here as long as you have a robust rationale in place. I am confident that section 2 of this EIA does not need completing as there are no highlighted risks of negative impact occurring because of this policy.

Name of person confirming result of initial Neonatal Guidelines Group impact assessment: If you have ticked ‘yes’ to any characteristic above OR this is a major working or service change, you will need to complete section 2 of the EIA form available here: Section 2. Full Equality Analysis

For guidance please refer to the Equality Impact Assessments Policy (available from the document library) or contact the Human Rights, Equality and Inclusion Lead [email protected]

Polycythaemia Neonatal Management Clinical Guideline V1.0 Page 14 of 14