MANAGEMENT OF HEREDITARY SPHEROCYTOSIS IN THE NEONATAL PERIOD CLINICAL GUIDELINE 1. Aim/Purpose of this Guideline 1.1 This guideline aimed at all clinical staff responsible for the management of infants diagnosed with hereditary spherocytosis in the neonatal period. 2. The Guidance 2.1 Background Hereditary Spherocytosis (HS) is the commonest form of haemolytic anaemia in northern Europe, incidence 1:5000 births, or if milder forms are included 1:2000 births 1. HS is a red cell membrane disorder affecting the structural protein spectrin. Aplastic crises can occur with infections such as Parvovirus B19 1 The usual biconcave shaped red blood cells become spherical with a shortened life span as they are readily destroyed by the spleen 2. Inheritance is usually autosomal dominant (75%), although 30% of people inherit the recessive form and in 25% of cases can present in homozygous form with no family history 3 Resulting haemolysis can result in severe jaundice, anaemia, reticulocytosis and splenomegaly. 2.2 Presentation in neonates Neonates with HS usually present soon after birth, commonly first day, with rising unconjugated hyperbilirubinaemia. This can be severe, may not be controlled by phototherapy and may require echange transfusion. Cross reference neonatal jaundice guideline, Echange Transfusion Guideline It is characterised by a NEGATIVE Coombs/ DAGT test (unlike ABO/Rhesus incompatibility haemolytic disease) Blood film can show the presence of spherocytes but this is not a definitive diagnosis in the neonatal period 3 Splenomegaly is a good clinical sign and should be sought and documented 2.3 Anaemia Anaemia can be severe and can result in the need for repeated blood transfusions in the first year of life 1 Until the infant is on a mied diet it is beneficial to give 1mg of folic acid daily 3 2.4 Parents Parents will need reassurance that HS is not usually a serious disorder. Even if a family member with HS had a splenectomy in the past this is not indicative of inherited severity as surgery used to be performed more often than current eligibility criteria 4 Page 1 of 8
2.5 Management of Neonatal Hereditary Spherocytosis Family history of HS Within 12 hours of birth Early rising jaundice levels with Negative DAGT Group and DAGT (if not yet tested) SBR, FBC with film and reticulocyte count LFTs, U&Es, CRP Consider sepsis risks Ensure adequate hydration Double phototherapy 4-6 hourly SBR whilst on phototherapy (risk of rapid rise) Minimum of 2 SBR and FBC checks at 8 and 24 hours post phototherapy before discharge Rapidly rising SBR (over 10micromol/l per hour) contact Blood Bank early to organise blood for possible echange transfusion NB. Take blood spot for any baby transfused before day 5 of life TTOs and follow up: Folic acid 1mg once daily for 12 weeks minimum FBC + SBR at 1 week, 3 weeks 6 and 9 weeks as NNU OPA Monitor Hb until over 9g/dl with rising trend Transfuse if Hb <7g/dl for asymptomatic infants Discuss with Haematology Consultant re. abnormal film Follow up at 4 months with Paediatric Consultant Arrange future follow up with Consultant Paediatrician Dr Sian Harris from 6 months for positive infants Page 2 of 8
2.6 Auditable Standards To monitor compliance with this guideline it will be included in the neonatal clinical audit programme with findings presented at the directorate audit meeting. Any deficiencies/action plan will be presented at the Clinical Governance meeting. Any clinical incident reports relating to this guideline will be monitored against it. Page 3 of 8
3. Monitoring compliance and effectiveness This part must provide information on the processes and methodology for monitoring compliance with, and effectiveness of, the policy using the table below. Element to be monitored Lead Tool Frequency Reporting arrangements Acting on recommendations and Lead(s) Change in practice and lessons to be shared Key Changes to practice Dr. Paul Munyard Audit As dictated by audit findings Child Health Directorate Audit and Consultant led Neonatal clinical Guidelines Group Dr. Paul Munyard. Consultant Paediatrician and Neonatologist. Required changes to practice will be identified and actioned within 3 months. A lead member of the team will be identified to take each change forward where appropriate. Lessons will be shared with all the relevant 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, Diversity & Human Rights Policy' or the Equality and Diversity website. 4.2. Equality Impact Assessment The Initial Equality Impact Assessment Screening Form is at Appendi 2. Page 4 of 8
Appendi 1. Governance Information Document Title Date Issued/Approved: 30 September 2015 Management of Hereditary Spherocytosis in the Neonatal period Clinical Guideline Date Valid From: November 2015 Date Valid To: November 2018 Directorate / Department responsible (author/owner): Dr Munyard. Consultant Paediatrician and Neonatologist Contact details: (01872) 252667 Brief summary of contents This guideline outlines the diagnosis, management of hereditary spherocytosis in the neonatal period. Suggested Keywords: Target Audience Eecutive Director responsible for Policy: Neonatal. Hereditary Spherocytosis. RCHT PCH CFT KCCG Eecutive Director Date revised: 30:09:2015 This document replaces (eact title of previous version): Approval route (names of committees)/consultation: Guideline for the management of Hereditary Spherocytosis in the neonatal period Consultant approval. Child Health Directorate Audit. Neonatal Clinical Guidelines Group Divisional Manager confirming approval processes Name and Post Title of additional signatories Signature of Eecutive Director giving approval Publication Location (refer to Policy on Policies Approvals and Ratification): Document Library Folder/Sub Folder Links to key eternal standards Sheena Wallace Not Required {Original Copy Signed} Internet & Intranet Intranet Only Neonatal. Midwifery. Child Health. Clinical None Page 5 of 8
Related Documents: 1. Bolton-Maggs PHB (2004) Hereditary Spherocytosis; new guidelines. Archives of Disease in Childhood; 89:809-812 2. Eber,S.W(1990) Variable clinical severity of hereditary Spherocytosis: relation to erythrocyte spectrin concentration Journal of Pediatrics 177;409-11 3. Murray,N.,Roberts,I.(2007) Haemolytic disease of the newborn Archives of Disease in Childhood Fetal Neonatal Ed. 92;83-88 4. Delhommeau F, Cynober,T., Schischmanoff,P et al (2000) Natural history of hereditary spherocytosis during the first year of life Blood; 95:393-7 Training Need Identified? No Version Control Table Date Versio n No Summary of Changes Changes Made by (Name and Job Title) Oct 2013 V!.0 Initial Issue Author: Judith Clegg. Neonatal Nurse Practitioner 30:09:15 V2.0 Reformatted, reviewed and approved at Consultant led Neonatal Guidelines Group meeting. Formatter: Kim Smith. Staff Nurse. [Please complete all boes and delete help notes in blue italics including this note] 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 epiry. 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 on Document Production. It should not be altered in any way without the epress permission of the author or their Line Manager. Page 6 of 8
Appendi 2. Initial Equality Impact Assessment Form Name of the strategy / policy /proposal / service function to be assessed (hereafter referred to as policy) (Provide brief description): Management of Hereditary Spherocytosis in the Neonatal period Clinical Guideline Directorate and service area: Child and Is this a new or eisting Policy? Eisting Women s Health. Neonatal Name of individual completing Telephone: (01872) 252667 assessment: Dr. Paul Munyard. 1. Policy Aim* Who is the strategy / policy / proposal / service function aimed at? 2. Policy Objectives* As above This guideline is aimed at clinical staff responsible for the management of infants diagnosed with hereditary spherocytosis 3. Policy intended Outcomes* 4. *How will you measure the outcome? 5. Who is intended to benefit from the policy? 6a) Is consultation required with the workforce, equality groups, local interest groups etc. around this policy? b) If yes, have these *groups been consulted? C). Please list any groups who have been consulted about this procedure. Audit Audit Patients and staff. No. Consultant led Neonatal Guidelines group approved. N/A N/A 7. The Impact Please complete the following table. Are there concerns that the policy could have differential impact on: Equality Strands: Yes No Rationale for Assessment / Eisting Evidence Age Page 7 of 8
Se (male, female, transgender / gender reassignment) Race / Ethnic communities /groups Disability - learning disability, physical disability, sensory impairment and mental health problems Religion / other beliefs Marriage and civil partnership Pregnancy and maternity Seual Orientation, Biseual, Gay, heteroseual, Lesbian You will need to continue to a full Equality Impact Assessment if the following have been highlighted: You have ticked Yes in any column above and No consultation or evidence of there being consultation- this ecludes any policies which have been identified as not requiring consultation. or Major service redesign or development 8. Please indicate if a full equality analysis is recommended. No 9. If you are not recommending a Full Impact assessment please eplain why. No area indicated Signature of policy developer / lead manager / director Dr Paul Munyard Date of completion and submission 10:11:2015 Names and signatures of members carrying out the Screening Assessment 1. 2. Keep one copy and send a copy to the Human Rights, Equality and Inclusion Lead, c/o Royal Cornwall Hospitals NHS Trust, Human Resources Department, Knowledge Spa, Truro, Cornwall, TR1 3HD A summary of the results will be published on the Trust s web site. Signed Kim Smith Date 10:11:2015 Page 8 of 8