Blood Transfusions in Children with Sickle Cell Disease€¦ · (iv) If PCV

Author: Dr Jenny Welch , Consultant Haematologist M3/13/1094 Updated February 2018 Next review: February 2021 (Do not use after this date) Version number 5 © SC(NHS)FT 2018. Not for use outside the Trust. Page 1 of 13

Blood Transfusions in Children with Sickle Cell Disease Reference: CG1094

Written by: Dr Jenny Welch

Peer reviewer Dr Jeanette Payne

Approved: February 2018

Approved by D&TC: Not required

Review Due: February 2021

Intended Audience These guidelines are to be read in conjunction with SC(NHS)FT guidelines for the administration of blood and blood components located in the Blood Transfusion Information file at the nurses station on M3 or on the hospital intranet. Table of contents

1. Introduction

2. Blood Bank/Laboratory Transfusion Requests

3. Top-Up Transfusions

4. Exchange Transfusions

5. Routine Elective Transfusion

6. Surgery and Transfusions

7. Jehovah’s Witnesses

8. References Appendix I – Exchange transfusion monitoring document

CG1094 Sheffield Children’s NHS Foundation Trust

Blood Transfusions in Children with Sickle Cell Disease


1. Introduction Blood transfusion is used for specific indications and may be either -

a) Simple additive or “top-up” transfusion

b) Exchange transfusion

c) Regular transfusion Blood Bank need 24 - 48 hours notice to order phenotyped blood for ‘routine’ transfusions. For emergency transfusions, blood can be ordered if Blood Bank has the patient’s phenotype. Red cell phenotyping (or genotyping – discuss with blood bank) should be done as soon as possible after the diagnosis of sickle cell disease has been made so if the child comes in as an emergency the information is already available. Check that Hepatitis B immunisation has been given. This is normally done through the GP starting at age 1 year. However, if this has not been done arrange as soon as possible. If it is not clear check Hepatitis B antibody status and ask GP to immunise accordingly. See Ward M3 Guidelines, section 11, Haematology – Non Malignant, 1418, Guideline for establishing a diagnosis of a haemoglobinopathy – the first out patient visit’ (M3/11/1418)

2. Blood Bank/Laboratory Transfusion Requests Blood Bank should have the extended phenotype of all Sickle cell disease children who are registered with our centre. New patients should have a 2.5mls EDTA cross match sample and request form containing diagnosis and full patient identification sent to Blood Bank for:

ABO RhD typing

Antibody screen

Extended red cell phenotyping: (Rh, Kell, Fy, Jk and MNS, SS patients should be typed for U). If genotyping is done this requires 6mls

For all patients the request form must contain:

Sickle negative blood request.

Volume required - see notes below.

When and where it will be needed.

For previously transfused planned ‘routine’ transfusions send the request as below

Time since last transfusion Cross match sample sent

3–14 days <24 hrs before transfusion

14–28 days <72 hrs before transfusion

29 days–3 months <1 week before transfusion




For emergency transfusions, blood can be ordered if Blood Bank has the patient’s phenotype. If there are problems eg known multiple antibodies/a history of hyperhaemolysis please discuss with a consultant first. Contact Blood Bank as soon as the need for emergency transfusion has been identified to arrange supplies - if blood has to be ordered in and then cross-matched there may be a delay of ~ 4-6 hours. Contact numbers: Ext

Blood bank 17478

Haematology Laboratory 17221

Transfusion Practitioner 17107 Haematology SpR or Consultant – via switchboard

3. Top-Up Transfusions (Additive transfusions) Possible Indications:

Splenic or hepatic sequestration

Aplastic crisis

Fall in Hb by 20 - 30g/L from steady state or to < 50g/L

A child with chest symptoms with a fall in Haemoglobin (Hb)- discuss with consultant

Pre-operatively (see surgery section below)

Multi Organ Failure The aim of transfusion is to restore Hb to target 80 -100g/L BUT ensure the final haematocrit is not >0.35 since this will cause an increase in the whole blood viscosity and risk of sickling. Please discuss with a consultant if a higher haematocrit is found at the end of the transfusion. The volume of blood required for “top-up” transfusion is based on the formula: Volume of blood (packed cells) = [Desired Hb - Actual Hb] x Weight (kg) x 0.4 Where possible use full units, but do not over transfuse to avoid raising the final haematocrit too high. Children <10kg weight can receive part units. Administer according to SC(NHS)FT guidelines for the administration of blood and blood components. The normal rate of red cell transfusion is 5ml/kg/hour. Do NOT give frusemide with transfusions in sickle cell disease because of the increase in viscosity that may result.




4. Exchange Transfusion in Sickle Cell Disease Indications:

Acute chest syndrome. Consider exchange blood transfusion for patients with acute chest syndrome and imminent respiratory failure as this may prevent the need for endotracheal intubation and mechanical ventilation

Stroke (or TIA) to reduce HbS to 30% or less

Severe infection (meningitis, pneumonia)

Retinal artery occlusion, hepatic failure

Pre-operatively in selected patients

Multi Organ Failure Some cases of priapism may also need exchange transfusion – see relevant guideline The aim of exchange transfusion is to achieve an HbS% of <30%, final Hb <125g/L and PCV of <0.35

This target usually requires two manual exchanges.

An automated exchange using a cell separator allows the exchange to be completed as a single procedure and can be arranged via the NHSBT Apheresis Unit. Suitable vascular access will need to be placed – please discuss on an individual basis with consultant haematologist and see the Automated Exchange transfusion guideline.

To calculate the volume of packed cells (ml) for each manual exchange: Volume of packed cells (in ml) for each exchange = 0.6 x 80 x weight (kg) NB Although this volume exchange would need to be repeated nearly 3 times to achieve the total exchange in practice it is not usually necessary to perform more than 2 procedures. Procedure: (i) Order the blood urgently (Blood Bank Ext 17478) and send sample for cross-match as

above. Request in addition-

FBC

Clotting screen

Renal, liver & bone profiles and glucose

HbS% (Baseline – The procedure can be started without the result) (ii) Discuss with PCCU as soon as the decision to perform an exchange transfusion is made

as their help will be needed for the procedure, the child will require an arterial catheter or 2 large venous catheters or a double lumen vascath and a PCCU bed.

(iii) Insert venous and arterial catheter OR two large-bore venous catheters or double lumen

vascath. If the child requires mechanical ventilation because of respiratory deterioration consider siting access for an automated exchange.




(iv) If PCV <0.20: Transfuse packed cells (8 ml/kg body weight) over a period of 30 mins to 1 hour and then proceed with ml for ml exchange.

If PCV >0.20 Consider infusing 10 to 20 ml/kg of 0.9% sodium chloride or Hartmann’s solution over 30 minutes to 1 hour and then proceed with ml for ml exchange.

(v) Perform ml for ml exchange:

The aim is to maintain isovolaemia by withdrawing the patient’s blood in aliquots and replacing with an infusion of donor blood at the same overall rate. The rate of the exchange transfusion will depend on the age, size, and clinical condition of the patient and how well the procedure is tolerated. It is important to check that the volumes input and output have been equal every 15 minutes.

Initial withdrawal aliquot size: Aliquots should be 5% of blood volume which equates to 4 ml/kg in infants (Blood volume is 80 ml/kg in infants). The maximum aliquot size should be 50 ml regardless of the age of the patient. The aliquot size may need to be reduced to 2 ml/kg (max. 25 ml) if the patient is cardiovascularly unstable. Remember to adjust the replacement infusion rate when changing the aliquot size. Initial replacement infusion rate: The infusion should be commenced at a rate of 12 times the aliquot size in ml per hour. So if the aliquots are 40 ml the infusion should be at a rate of 480 ml/hr. To perform the exchange:

a. Commence a blood transfusion through a peripheral cannula at standard rate as detailed above.

b. Withdraw aliquots of blood from the arterial or large-bore venous line, each aliquot

being withdrawn over 5 minutes.

c. Check the volume infused matches the volume withdrawn every 15 minutes. If the aliquots are taking longer than 5 minutes each reduce the rate of the replacement infusion to match.

During the exchange transfusion monitor;

Heart rate and BP (At least every 5 minutes).

Hb and PCV half way through the procedure. Note that Hb > 120g/L (PCV > 0.35) during the exchange may be associated with increased blood viscosity and complications. If this occurs give a 50ml aliquot of 0.9% saline and recheck.

Electrolytes (serum potassium and calcium) half way through the procedure

Note that Hb and electrolytes may be checked using a blood gas analysis machine.

If there is any cardiovascular instability during the procedure reduce the aliquot size to 2.5% of blood volume (2ml/kg, maximum 25 ml).




(vi) Document the procedure in the patient notes, including volume exchanged, time taken, and cardiovascular status throughout.

(vii) At the end of the exchange send samples for:

FBC

Clotting screen

Renal, liver, bone profiles and glucose

HbS%

Depending on the patient’s clinical state, discuss the need to proceed to a second exchange with the haematology consultant and repeat the investigations after each exchange. Allow an interval of at least 6 hours between exchanges (or overnight). Ensure that at the end of the exchange the final Hb <125g/land PCV <0.35. Examples: 10kg child. Blood volume 800ml. 40 ml aliquots. 20 cycles. Each cycle approx 5 mins + mid procedure assessment. Total time 100+ mins 20kg child. Blood volume 1600ml. 50ml aliquots. 32 cycles. Each cycle 5 mins, total procedure time 160+ mins 25kg child. Blood volume 2000ml. 50ml aliquots. 40 cycles. Total procedure time 200mins+ 40kg child. Total blood volume 3200ml, 50 ml aliquots, 64 cycles at 5 mins total time = 320mins+

5. Routine Elective Transfusion in Sickle Cell Disease The primary indication is to prevent recurrence of CVA (stroke) or prevent TIA progressing to completed stroke. Other possible indications include

Recurrent sickle chest syndrome (but Hydroxycarbamide would be the 1st line intervention of choice)

>3 severe vaso-occlusive crises/year (but again Hydroxycarbamide would usually be preferable)

The aim is to achieve a pre-transfusion Hb between 90 - 100g/L and gain a post-transfusion Hb 120g/L, while maintaining HbS below 30% (or HbS + HbC below 30%). In some patients, a less intensive regimen maintaining the HbS below 50% may be sufficient for stroke prevention after 3 years of regular transfusion – discuss with consultant. Procedure:

(i) Plan to give transfusion as an elective admission to M3 every 4 weeks as a ‘top-up’: Calculate blood volume based on the formula:

Volume of blood (packed cells) = [Desired Hbg/l - Actual Hbg/l] x Weight (kg) x 0.4

Where possible use full units, but do not over transfuse to avoid raising the final haemocrit too high. Children <10kg weight can receive part units.




(ii) Patients receiving regular transfusions should be reviewed by medical staff at each visit.

(iii) Check Hepatitis status and immunise. Repeat Hepatitis C antibodies and anti-HBsAb annually and give Hepatitis B booster when indicated.

(iv) Children on regular transfusions will need to start iron chelation once ferritin >1000ng/ml (after ~1 year of transfusion) - see Ward M3 guidelines, Section 11, Haematology – Non Malignant, 1315, Thalassaemia and other Transfusion Dependent Conditions (M3/11/1315). An alternative is automated regular exchanges for older children with good venous access, and no alloantibodies. (v) Children on regular transfusions should attend the transfusion clinic 3 times per year so

that correct monitoring is put in place and an annual review carried out and stroke patients will be seen periodically in the neurology clinic. Their imaging and follow up is individualised

6. Surgery and Transfusions in Sickle Cell Disease

This is the subject of a separate document. See Ward M3 Guidelines, section 13, Sickle Cell Disease, 1096, Anaesthesia in Children with Sickle Cell Disease (M3/13/1096). Please note: All cases of sickle cell disease requiring surgery must be brought to the attention of a consultant anaesthetist, consultant surgeon and consultant paediatric haematologist. All patients should be assessed on an individual basis and, discussed with a consultant paediatric haematologist.

7. Children of Jehovah’s Witnesses Parents who are Jehovah’s Witnesses may not give consent for the use of cellular blood products or plasma for their child. However, sensitive discussion of the situation may avoid the need for legal intervention in order to treat the child. It is very important to explain to the older child what is happening and why transfusion is necessary since they too may be distressed by the evident conflict between their parents’ beliefs and the need for transfusion. Some parents in this situation may sign a statement acknowledging that in a life-threatening situation the doctors caring for the child will want to give blood and that they understand the medical staff carry the responsibility for that decision although they do not agree. The Jehovah’s Witness Society has been helpful in providing support for individual children undergoing transfusion, with practical suggestions such as covering the bag and giving set to minimise the anxiety it may cause.




In the event of parents refusing to allow transfusion in a life-threatening situation, then legal advice must be sought to allow treatment to proceed in the interests of the child. Make sure the child’s consultant(s) are aware of the situation. For legal advice during normal working hours contact the Risk Management Department. Out of hours contact the Director on call through switchboard, who will be able to guide the team through the legal process. Occasionally in some situations, transfusion may be avoided by the use of recombinant EPO which is usually acceptable to Jehovah’s Witnesses. Always discuss with a consultant. Disclaimer This document is by no means a substitute for clinical judgement. The information contained in this document is especially for the use of qualified professionals with the appropriate training and experience

8. References Vichinsky et al, N Eng J Med (1995) 206-212




Appendix I

Exchange transfusion monitoring document.

Copies of this monitoring document can be found behind the guideline in the Ward M3 folder, or on Qpulse under M3/Form1/1094

Affix patient label or complete

SCH Hospital Number………………

Surname………………………………

Forename……………………………

D.O.B………………………………..

NHS Number…………………………



Date:

Time:


Bar

Code

Start Time

Actual time

Vol. removed

Total Vol. removed

Volume infused

Total volume infused

Fluid Balance

Heart Rate

Blood Pressure

Sats (%)

PCV Hb Na+ K

+ Ca2+

(ionised)

Glucose Actions

Start 0 0 0 0 0

+ 5 mins

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Surname………………………………


D.O.B………………………………..




Date:

Time:


Bar

Code

Start Time

Actual time

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Total Vol. removed

Volume infused


Fluid Balance

Heart Rate

Blood Pressure

Sats (%)

PCV Hb Na+ K

+ Ca2+

(ionised)

Glucose Actions

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Surname………………………………


D.O.B………………………………..




Date:

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Bar

Code

Start Time

Actual time

Vol. removed

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Volume infused


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Heart Rate

Blood Pressure

Sats (%)

PCV Hb Na+ K

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Ca2+

(ionised)

Glucose Actions

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Surname………………………………


D.O.B………………………………..




Date:

Time:


Bar

Code

Start Time

Actual time

Vol. removed

Total Vol. removed

Volume infused


Fluid Balance

Heart Rate

Blood Pressure

Sats (%)

PCV Hb Na+ K

+ Ca

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(ionised) Glucose Actions

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Documents

Blood Transfusions in Children with Sickle Cell Disease€¦ · (iv) If PCV