Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease
A clinical guideline recommended for use
In:
Accident and Emergency , Children’s Assessment Unit ,
Buxton Ward.
By:
Nurses, SHOs, SpRs, Consultants
For:
Children < 16 years
Key words:
Sickle, pain, crisis, analgesia, guideline.
Written by:
Dr Jo Ponnampalam, Consultant
Paediatrician/Haem.Onc,NNUHFT
Supported by:
Dr Hamish Lyall,Consultant Haematologist, NNUHFT
Dr J Wimperis, Consultant Haematologist, NNUHFT
Dr Mike Gattens,Consultant Paediatric Haematologists,
Addenbrookes Hospital
Accepted by James Paget University Hospital under the TriHospital Clinical Guidelines Assessment Panel (THCGAP)
Approved by:
Chair’s action 29 September 2014 and reported to Clinical
Guidelines Assessment Panel (CGAP) 15 October 2014
Reported as approved Clinical Standards Group
Effectiveness Sub-Board
to the:
Date of approval
September 2014
Amended August 2014 (following MHRA alert not to use codeine
in children<12 years of age and restrictions to those<19 years of
To be reviewed before: age)
27 August 2017
To be reviewed by:
Dr J Ponnampalam, Consultant Paediatrician
Guideline supersedes: CA4057 v5
Guideline Reg. No:
JCG0023 v1
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 1 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease
Quick reference guideline/s
See page 5 and 6 of this guideline.
Objective/s
Pain in sickle cell disease is thought to be due to complex and poorly understood
interactions between biological and psychosocial factors. This guideline aims to
provide a basic minimum standard of care for patients with acute painful crisis, paying
particular attention to adequate analgesia based on results of randomised controlled
trials.
Rationale
Severe acute pain is the most common manifestation of sickle cell disease requiring
hospital admission. Inappropriate treatment leads to unnecessary suffering, potentially
fatal complications and repeated admissions 3.
Vaso-occlusion within the bone marrow vasculature leads to bone infarction and
release of inflammatory mediators that activate afferent nerve fibres causing pain.
Hospital admissions for acute pain in sickle disease typically last 4-10 days, but this
may vary widely3.
The guidelines are based on recommendations by British Society of Haematology
published in 2003, Cochrane review of pain management in sickle cell disease in
2006, guidelines from The Royal London Hospital and North Middlesex Hospital
Haemoglobinopathy service and recommendations from the recent NICE guidelines on
acute sickle cell pain, June 2012.
Conditions covered by this guideline
Homozygous sickle cell anaemia (HbSS) accounts for 70% of patients in UK while
compound heterozygote for HbS and HbC (HbSC) and HbSβthal contribute to majority
of the rest.
Broad recommendations
Codeine preparations are no longer to be used as per MHRA alert on the use of
codeine in children<18 years of age June 2013.
On presentation, children with sickle cell disease should be assessed for severity
of pain despite the dose and type of analgesia already administered at home. If pain is
not controlled on non - opioids (Paracetamol, Ibuprofen), a stronger opioid like
Morphine should be considered. Children should be assessed for complications and
treated promptly and appropriately. Adequate hydration status and oxygenation
(saturation>95%) play a key role in management. Oxygen saturations should be
measured off oxygen on admission and maintained at >95%.
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 2 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Initial assessment

Triage as high priority

Offer analgesia for pain within 30 minutes of arrival to hospital

Measure oxygen saturations in air. Maintain oxygen saturation >95%
Assess for

Infection

Dehydration

Acute chest syndrome (fever, tachypnoea, chest pain, hypoxia, chest signs),

Girdle syndrome (abdominal pain due to vaso occlusion, gut ischaemia)

Cholecystitis

Splenic enlargement

Neurological events

Priapism
Pain Management
Offer analgesia within 30 minutes of presentation to hospital with acute painful crisis 4
(ensure detailed pain history and whether caused by painful crisis or whether
alternative diagnosis should be considered especially if pain reported as atypical by
patient)

Accurate analgesic history: Usual analgesia taken, dose, timing and
effectiveness.

Assess severity of pain (See pain assessment tool – appendix-1 as well as
parental assessment).

Aim for relief of pain according to WHO pain relief ladder 5 (Page 4).

If severe pain: reassessment hourly for the first 3 hours (Flow chart - page 5).

Ensure adequate hydration especially with NSAIDs.

Consider non drug methods in pain management such as distraction play
therapy.
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 3 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
WHO pain relief ladder 5
Paracetamol 15 – 20 mg/kg/dose every 6 hrs (Appendix-2A)
Ibuprofen 10 mg/kg/dose every 8 hrs (Appendix-2B)
Reduced dose morphine 100-150 micrograms/kg every 6 hours (Appendix-2C)
If pain is mild or moderate and pain relief is acceptable with oral analgesia as above,
patient can be managed at home with appropriate follow up arranged in the Paediatric
Haematology clinic. If pain is severe and/or failed treatment with paracetamol,
ibuprofen and reduced dose oral morphine, the patient should be admitted and started
on the pain protocol for severe pain (Appendix-2).
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 4 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Pharmacological management of acute sickle pain:
Exclusions:
Severe Acute Chest Syndrome
Girdle Syndrome
Vomiting
(In these conditions, consider morphine PCA. Prompt transfer to a regional Paediatric
Intensive Care Unit for exchange transfusion is necessary in acute chest syndrome.
This can be discuss with the on call Paediatric Consultant/Pain team)
Child attends A&E/CAU with sickle pain
Assessment by Medical Team
Start Protocol if Paracetamol, Ibuprofen and reduced dose
morphine (100-150 micrograms/kg) already administered
0 hour
Give:
Morphine Sulphate 200 microgram/kg (max 10 mg) 10 mg/5mL liquid strength (Oramorph) start dose.
Or IV Morphine sulphate 100 microgram/kg Single Dose
AND Regular Paracetamol (15 – 20 mg/kg) 6 hourly and Regular Ibuprofen 10 mg/kg 8 hourly
Consider PR or IV Paracetamol if the patient is vomiting (Appendix-2A)
If pain is controlled at any stage → refer to the last box of this algorithm
1 hour
pain persists
Morphine Sulphate 200 microgram/kg (max 10 mg) 10 mg/5mL liquid strength (Oramorph) (2 nd dose).
2 hours
pain persists
Morphine Sulphate 200 microgram/kg (max 10 mg) 10 mg/5mL liquid strength (Oramorph) (3 rd dose).
3 hours
Analgesia unacceptable
Morphine PCA (Bleep Pain team
(day time)/ Anaesthesia SpR on call
(out of hours)
Analgesia unacceptable
pain persists
Analgesia acceptable
Morphine Sulphate 200 microgram/kg
(max 10 mg) 10 mg/5mL liquid strength
(Oramorph)(4th Dose)
Analgesia acceptable
Continue Morphine Sulphate 200
microgram/kg (max 10 mg) 10 mg/5mL
liquid strength (Oramorph) PRN 4 hourly
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 5 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Refer to Appendix-2 (A - D) for doses
Observation:
Monitor the following:
1. Temperature/ pulse rate/blood pressure/respiratory rate/oxygen saturation
2. Pain
3. Sedation (AVPU)
4. Strict fluid balance chart/urine output
5. Vital signs to include respiratory rate and oxygen saturation every 20 min until
pain controlled and stable; then every 2 hrs (Appendix- 4C).
If respiratory rate<10/min, omit maintenance dose of opioid analgesia and seek
medical assistance immediately - attention to ABC.
Reversal of Opiate induced respiratory depression:
Child 1month -12 years: Naloxone 5-10 microgram/kg, if response inadequate, give
subsequent dose of 100 micrograms/kg (max 2 mg).
Child 12-18 years: Naloxone 1.5-3 micrograms/kg, if response inadequate gives
subsequent dose of 100 micrograms every 2 mins.
Other medication:

Laxative(C): PO Lactulose/Movicol .(Appendix-3A)

Antipruritic: Chlorphenamine PRN (Appendix-3B)

Antiemetic: Metoclopramide Hydrochloride PRN (Appendix-3C)
Further investigation and monitoring:
FBC (C), U&Es, Urea and Creatinine, Group and save on admission
Other investigations should be dictated by clinical presentation

Blood, urine culture (febrile, rigors, hypotensive), throat swab (cough) and CRP
(febrile).

CXR (febrile, breathless, tachypnoea, chest pain, chest signs, reduced O2
Saturation)

Blood Gas (SaO2<95%, unexplained drowsiness)

Liver function test(abdominal pain, increased jaundice), LDH

Reticulocytes (Hb lower than normal/falling)
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 6 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.

Abdominal ultrasound scan (abnormal LFTs, abdominal pain, splenomegaly)

Parvovirus B19 serology (reticulocytopenia)

CT/MRI brain (Seizure, Transient Ischaemic Attack, Stroke, Severe Headache)

Limb radiograph should not be performed unless there is history of trauma,
persistent pain or unexplained swelling. Sickling can cause localised, painful
swelling, bone infarcts. The differential diagnosis of osteomyelitis needs to be
considered. MRI may help in diagnosis.
Further management:
Fluid Management:
Attention to strict fluid balance.
Ensure normal maintenance fluid intake orally if not dehydrated/no continued fluid
loss. If not tolerated orally, NG/IV fluid administration required (Appendix- 4C).
Hyperhydration (150% maintenance) tried in moderate/severe painful crisis-selected
cases discuss with on call Consultant.
Oxygen:
Administer oxygen to maintain saturation more than 95% (Appendix- 4C).
Beware of increasing oxygen requirement to maintain saturations >95% as this could
be a sign of impending acute sickle chest syndrome- a medical emergency.
Antibiotics:
Patients with sickle cell disease are more susceptible to Streptococcus Pneumoniae
Haemophilus Influenzae B, Meningococcus and Salmonella species. All children
should already be on Penicillin prophylaxis. Broad spectrum antibiotics should be
started if febrile (>38 degree C), generally unwell, has chest symptoms and signs,
infection suspected for some other reason (Appendix- 4C).
Start IV Ceftriaxone 80mg/kg; add PO Clarithromycin if chest symptoms or signs
present.
If patient is receiving iron chelation and presents with abdominal pain/diarrhoea:

Take blood and stool cultures.

Start Ciprofloxacin (to cover Yersinia).

Stop iron chelation.
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 7 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Blood Transfusion:
Note: Please discuss with on call Paediatric/Haematology Consultant if blood top up
/exchange transfusion is required(Paediatric exchange transfusions should take place
in a PICU setting at Addenbrookes).
Blood transfusion is required for symptomatic anaemia (unexplained tachycardia,
tachypnoea, fatigue) not as a treatment for pain (Appendix-4). Transfuse if Hb has
fallen more than 2 g/dL and is below 5 g/dL with a low reticulocyte count (<100x10 9/l)
with sickle cell negative blood transfusion. If blood transfusion is required, the
possibility of sequestration (hepatic, splenic) and parvovirus infection should be
considered.
Exchange Transfusion is indicated in cerebrovascular accident, severe chest crisis
and multiorgan failure.
When blood is requested, sickle negative blood should be ordered.
Liaison with duty Paediatric Haematologist Addenbrookes Hospital
Patients with sickle cell disease are largely managed at NNUHFT, however if
exchange transfusion is required, this will be performed by the Paediatric
Haematologists at Addenbrookes Hospital within the PICU setting. Patients with HBSS
are managed within the North Middlesex Haemoglobinopathy network and the point for
local contact for NNUHFT is to Addenbrookes Hospital sub-network group.
Inform Dr Ponnampalam or Dr Soman of child’s admission to Buxton ward.
It is important that Addenbrookes Hospital Paediatric Haematologists are
informed of all admissions where children with HBSS are unwell with an oxygen
requirement to maintain saturations >95% (or increasing oxygen requirement),
signs or symptoms of a pneumonia (can mimic acute chest crisis),
sequestration crisis or any patient requiring HDU care on Buxton. This
constitutes a medical emergency. Early recognition and transfer of sick children
known to have HBSS should be of high clinical priority and improves outcomes.
Possible acute complications
1. Acute Chest Syndrome
Beware of the possibility of acute chest syndrome in patients with acute painful sickle
cell episode if any of the following are present at any time from presentation to
discharge:
Abnormal respiratory signs/symptoms
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 8 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Chest pain
Fever
Oxygen saturations of <95% or a rising oxygen requirement
2. Acute Stroke
3. Aplastic crisis (check Parvovirus B19 serology)
4. Infections
5. Osteomyelitis
6. Splenic sequestration
Pre discharge check list
1. Check that the patient has follow up in Dr Ponnampalam/Dr Lyall joint Paediatric
Haematology OPD within 4 weeks ( liaise with Dr Ponnampalam’s secretary on
ext 3622)
2. Advise on oral fluid intake and analgesia including clear dosage and frequency
of administration. Note: oral dose morphine as TTO should reflect the
reduced dose regime only-prescribe as TTO for 3 days only
3. Check on prophylactic Penicillin BD and Folic acid OD
4. Check has sickle cell card (contact Dr Jo Ponnampalam’s secretary – Ext 3622 if
required)
5. Inform either Dr Ponnampalam/Dr Soman of child’s attendance/admission
Clinical audit standards
1. Aim to assess patient for pain within 10 minutes of admission.
2. Aim for patient to receive analgesia within 30 minutes of admission(C).
3. Aim for adequate oral or intravenous hydration to be established within 30
minutes.
4. Aim for pain to be controlled to an acceptable level within 60 minutes(C).
5. The patient should be kept warm and offered reassurance.
6. Maintain oxygen saturation ≥ 95%(C).
Summary of development and consultation process undertaken before
registration and dissemination
The authors listed above on behalf of the paediatric directorate have developed this
guideline after a comprehensive literature research and discussions with
paediatricians, anaesthetists, pain team, nursing staff and pharmacist. The final
version was endorsed by the Paediatric clinical guidelines group.
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 9 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Distribution list/ dissemination method
This guideline will be distributed to A&E, CAU, Buxton ward, CDW and placed on the
intranet.
References/ source documents
1. Jean L. Raphael, Krystal Harvey and Kent Stobart -The Cochrane Library and
sickle cell disease: an overview of reviews. Evid.-Based Child Health 2: 1094–
1101 (2007) http://www3.interscience.wiley.com/cgibin/fulltext/117914042/PDFSTART
2. Dunlop RJ, Bennett KCLB. Pain management for sickle cell disease (Review).
The Cochrane Database of Systematic Reviews. Issue 2. Art No,:
CD003350.pub2. DOI: 10.1002/14651858.CD003350.pub2. (2006)
http://mrw.interscience.wiley.com/cochrane/clsysrev/articles/CD003350/pdf_fs.h
tml
3. David Rees, Denmark Hill Guidelines for management of the acute painful crisis
in sickle cell disease (2003) British Journal of Haematology, 2003, 120,744-752.
http://www.bcshguidelines.com/pdf/sicklecelldisease_0503.pdf
4. NICE clinical guideline-sickle cell acute painful episode
5. WHO pain ladder http://www.who.int/cancer/palliative/painladder/en
6. BNF for children 2013/2014 http://bnfc.org/bnfc
7. Royal London Hospital Guideline on ‘Management of painful crisis in sickle cell
disease’.
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 10 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Appendix-1
Pain Assessment Tool for Infants and Toddlers
(FLACC Behavioural Scale)
Category
Face
Legs
Activity
Cry
Scoring
0
1
No particular
Occasional grimace
expression or
or frown, withdrawn,
smile
disinterested
Normal position or
Uneasy, restless,
relaxed
tense
Lying quietly,
Squirming, shifting
normal position,
back and forth,
moves easily
tense
No cry (awake or Moans or whimpers;
asleep)
occasional
complaint
2
Frequent to
constant quivering
chin, clenched jaw
Kicking, or legs
drawn up
Arched, rigid or
jerking
Crying steadily,
screams or sobs,
frequent
complaints
Consolability Content, relaxed
Reassured by
Difficult to console
occasional touching, or comfort
hugging or being
talked to,
distractible
Each of the five categories (F) Face; (L) Legs; (A) Activity; (C) Cry; (C)
Consolability is scored from 0-2, which results in a total score between
zero and ten:
0 = No Pain (0) 1-3 = Mild Pain (1) 4-7 = Moderate Pain (2)
8-10 = Severe Pain (3)
Pain Assessment Tool for Children aged 3-7 Years
(Modified Wong and Baker)
0
1
2
Appendix-2
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 11 of 15
3
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
A.
Paracetamol
By mouth
Child 1–3 months
30–60 mg every 8 hours as necessary; for severe symptoms 20 mg/kg as a single dose then 15–20 mg/kg
every 6–8 hours; max. 60 mg/kg daily in divided doses
Child 3–12 months
60–120 mg every 4–6 hours (max. 4 doses in 24 hours); for severe symptoms 20 mg/kg every 6 hours
(max. 90 mg/kg daily in divided doses) for 48 hours (or longer if necessary and if adverse effects ruled out)
then 15 mg/kg every 6 hours
Child 1–5 years
120–250 mg every 4–6 hours (max. 4 doses in 24 hours); for severe symptoms 20 mg/kg every 6 hours
(max. 90 mg/kg daily in divided doses) for 48 hours (or longer if necessary and if adverse effects ruled out)
then 15 mg/kg every 6 hours
Child 6–12 years
250–500 mg every 4–6 hours (max. 4 doses in 24 hours); for severe symptoms 20 mg/kg (max. 1 g) every
6 hours (max. 90 mg/kg daily in divided doses, not to exceed 4 g) for 48 hours (or longer if necessary and
if adverse effects ruled out) then 15 mg/kg every 6 hours; max. 4 g daily
Child 12–18 years
500 mg every 4–6 hours; for severe symptoms 1 g every 4–6 hours (max. 4 doses in 24 hours)
By rectum
Child 1–3 months
30–60 mg every 8 hours as necessary; for severe symptoms 30 mg/kg as a single dose then 20 mg/kg
every 8 hours; max. 60 mg/kg daily in divided doses
Child 3–12 months
60–125 mg every 4–6 hours as necessary (max. 4 doses in 24 hours); for severe symptoms 40 mg/kg as a
single dose then 20 mg/kg every 4–6 hours (max. 90 mg/kg daily in divided doses) for 48 hours (or longer
if necessary and if adverse effects ruled out) then 15 mg/kg every 6 hours
Child 1–5 years
125–250 mg every 4–6 hours as necessary (max. 4 doses in 24 hours); for severe symptoms 40 mg/kg as
a single dose then 20 mg/kg every 4–6 hours (max. 90 mg/kg daily in divided doses) for 48 hours (or
longer if necessary and if adverse effects ruled out) then 15 mg/kg every 6 hours
Child 5–12 years
250–500 mg every 4–6 hours as necessary (max. 4 doses in 24 hours); for severe symptoms 40 mg/kg
(max. 1 g) as a single dose then 20 mg/kg every 6 hours (max. 90 mg/kg daily in divided doses) for 48
hours (or longer if necessary and if adverse effects ruled out) then 15 mg/kg every 6 hours
Child 12–18 years
500 mg every 4–6 hours; for severe symptoms 1 g every 4–6 hours; max. 4 g daily in divided doses
By intravenous infusion over 15 minutes
Child body-weight under 10 kg
7.5 mg/kg every 4–6 hours; max. 30 mg/kg daily
Child body-weight 10–50 kg
15 mg/kg every 4–6 hours; max. 60 mg/kg daily
Child body-weight over 50 kg
1 g every 4–6 hours; max. 4 g daily
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 12 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
B. Ibuprofen
By mouth
1–3 months 5 mg/kg 3–4 times daily
3–6 months 50 mg 3 times daily; max. 30 mg/kg daily in 3–4 divided doses
6 months–1 year 50 mg 3–4 times daily; max. 30 mg/kg daily in 3–4 divided doses
1–4 yrs 100 mg 3 times daily; max. 30 mg/kg daily in 3–4 divided doses
4–7 yrs 150 mg 3 times daily; max. 30 mg/kg daily in 3–4 divided doses
7–10 yrs 200 mg 3 times daily; max. 30 mg/kg (max. 2.4 g) daily in 3–4 divided doses
10–12 yrs 300 mg 3 times daily; max.30 mg/kg (max. 2.4 g) daily in 3–4 divide doses
12–18 yrs initially 300–400 mg 3–4 times daily; increased if necessary to max. 600 mg 4 times daily;
maintenance dose of 200–400 mg 3 times daily may be adequate
C. Morphine Sulphate
By mouth or by rectum
Reduced dose oral morphine all ages 100-150 micrograms/kg/dose 6 hourly
Otherwise:
Child 1–3months
initially 50–100 micrograms/kg every 4 hours, adjusted according to response
Child 3-6 months
100-150micrograms/kg every 4 hours, adjusted according to response
Child 6-12 months
200 micrograms/kg every 4 hours, adjusted according to response
Child 1–2 years
initially 200–300 micrograms/kg every 4 hours, adjusted according to response
Child 2–12 years
initially 200–300 micrograms/kg (max. 10 mg) every 4 hours, adjusted according to response
Child 12–18 years
initially 5–10 mg every 4 hours, adjusted according to response
By intravenous injection over at least 5 minutes
Neonate
initially 50 micrograms/kg every 6 hours, adjusted according to response
Child 1–6 months
initially 100 micrograms/kg every 6 hours, adjusted according to response
Child 6 months–12 years
initially 100 micrograms/kg every 4 hours, adjusted according to response
Child 12–18 years
initially 2.5 mg every 4 hours, adjusted according to response
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 13 of 15
Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Appendix-3
Movicol Paediatric Plain
By mouth
Child 1–6 yrs 1 sachet daily; adjust dose to produce regular soft stools (max. 4 sachets daily)
Child 6–12 yrs 2 sachets daily; adjust dose to produce regular soft stools (max. 4 sachets daily)
Administration
Mix content of each sachet in quarter of a glass (approx. 60–65 ml) of water
Movicol
By mouth
Child 12–18 years 1–3 sachets daily in divided doses usually for up to 2 weeks; maintenance, 1–2
sachets daily
Administration
Mix content of each sachet in half a glass (approx. 125 mL) of water
Lactulose
By mouth
1 month–1 yr 2.5 mL twice daily, adjusted according to response
1–5 yrs 5 mL twice daily, adjusted according to response
5–10 yrs 10 mL twice daily, adjusted according to response
10–18 yrs 15 mL twice daily, adjusted according to response
Chlorphenamine Maleate
By mouth
1 month–2 years 1 mg twice daily
2–6 years 1 mg every 4–6 hours, max. 6 mg daily
6–12 years 2 mg every 4–6 hours, max. 12 mg daily
12–18 years 4 mg every 4–6 hours, max. 24 mg daily
Metocopramide Hydrochloride
By mouth, or by intramuscular injection or by intravenous injection over 1–2 minutes
Neonate
100 micrograms/kg every 6–8 hours (by mouth or by intravenous injection only)
Child 1 month–1 year and body-weight up to 10 kg
100 micrograms/kg (max. 1 mg) twice daily
Child 1–3 years and body-weight 10–14 kg
1 mg 2–3 times daily
Child 3–5 years and body-weight 15–19 kg
2 mg 2–3 times daily
Child 5–9 years and body-weight 20–29 kg
2.5 mg 3 times daily
Child 9–18 years and body-weight 30–60 kg
5 mg 3 times daily
Child 15–18 years and body-weight over 60 kg
10 mg 3 times daily
Note Daily dose of metocolopramide should not normally exceed 500 micrograms/kg
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
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Trust Guideline for the Management of Painful Crisis in Children with
Sickle Cell Disease.
Appendix-4
Classification of grades of recommendations
A Requires at least one randomized controlled trial as a part of a body of literature of
overall good quality and consistency addressing specific recommendation
B Requires the availability of well-conducted clinical studies, but no randomized
clinical trials on the topic of recommendation
C Requires evidence obtained from expert committee reports or opinions and ⁄ or
clinical experiences of respected authorities. Indicates an absence of directly
applicable clinical studies of good quality.
Authors: Dr.A Bhaduri, Dr A Babiker, Dr.R Ramaswamy, Dr.J Ponnampalam, Dr.J Wimperis Date of issue: September 2014
Valid until: September 2017
Guideline Ref No JCG0023 v1
Document: Management of painful crisis in children with Sickle Cell Disease
Copy of complete document available from: Trust Intranet
Page 15 of 15