Sickle Cell Disease (Acute Management)
Title of Guideline (must include
the word “Guideline” (not
protocol, policy, procedure
etc.)
Guideline on the management of patients with
Sickle Cell Disease presenting with acute
problems.
Contact Name and Job Title
(author)
Date of submission
Dr Emma Astwood Paediatric Haematologist
Dr Caroline Henry ST in Paediatrics
Dr Simone Stokley Paediatric Haematologist
Directorate: Family Health – Children
Speciality: Haematology and Oncology
July 2015
Date when guideline reviewed
July 2020
Guideline Number
1955
Explicit definition of patient group
to which it applies (e.g. inclusion
and exclusion criteria, diagnosis)
Abstract
Patients with Sickle Cell Disease presenting with
acute problems
Directorate & Speciality
Key Words
1a
2a
2b
3
4
5
This guideline provides management plans for
patients with Sickle Cell Disease presenting with
acute problems.
Paediatrics. Children. Sickle cell disease
Statement of the evidence base of the guideline – has the guideline been peer reviewed
by colleagues?
meta-analysis of randomised controlled
X
trials
at least one well-designed controlled study
without randomisation
at least one other type of well-designed
quasi-experimental study
well –designed non-experimental
descriptive studies (i.e. comparative /
correlation and case studies)
expert committee reports or opinions and /
or clinical experiences of respected
authorities
recommended best practise based on the
clinical experience of the guideline
developer
Consultation Process
Target audience
Staff at Nottingham Children’s Hospital via the
Guidelines E-mail process.
Staff at Nottingham Children’s Hospital
This guideline has been registered with the trust. However, clinical guidelines are
guidelines only. The interpretation and application of clinical guidelines will
remain the responsibility of the individual clinician. If in doubt contact a senior
colleague or expert. Caution is advised when using guidelines after the review
date.
Emma Astwood
Page 1 of 9
October 2015
Document Control
Document Amendment Record
Version
V1
V2
Issue Date
Author
Dr Clare Samuelson
Dr Emma Astwood, Paediatric
Haematologist
Dr Caroline Henry ST in Paediatrics
Dr Simone Stokley, Paediatric
Haematologist
October 2015
Summary of changes for new version:
Addition of Pain Management Flow Chart
Updated section on the Identification and treatment of specific crises
Statement of Compliance with Child Health Guidelines SOP
This guideline has followed Child Health Guideline SOP. It has been circulated to all
Paediatric Senior staff and comments incorporated before uploading to the Trust Guideline
site.
Martin Hewitt
Clinical Guideline Lead
21 June 2016
Emma Astwood
Page 2 of 9
October 2015
Management of Sickle Cell Crises in Children
Background
Prevalence: Sickle cell disease is the most common genetic disorder in England,
affecting more than 1 in 2000 live births. It causes high levels of morbidity, and still
significant rates of mortality (1-2%), in childhood. It should now be detected in most
cases at routine neonatal screening.
Cause: Normal adult haemoglobin (Hb A) consists of 2x  + 2x  chains. Hb S
results from a single amino acid substitution on the beta chain and is less soluble.
When deoxygenated, Hb S undergoes polymerisation leading to sickle-shaped cells.
This results in endothelial damage and blockage of blood vessels causing vasoocclusive events. Children who are homozygous for Hb S (SS) have the most severe
disease; those with an S/C or S/-thalassaemia genotype are also affected.
Heterozygotes are almost always unaffected.
Hyposplenism: these children are also at higher risk of severe infection from
encapsulated bacteria (e.g. Pneumococcus, Streptococcus, HiB, Salmonella spp.).
They should therefore receive regular Pneumovax immunisation (at 2 years of age
and 5-yearly thereafter) alongside the normal course of vaccinations (including
Prevenar), and annual influenza vaccination. They should also start long-term
prophylactic penicillin before the age of 3 months.
Regional Network: Nottingham Children’s Hospital is a Specialist
Haemoglobinopathy Centre. It provides advice and expertise, accepts transfers of
serious or difficult cases, and offers annual review of all children in the area with
sickle cell disease.
Acute Presentations
Type
Pathogenesis
Symptoms + Signs
Bony crisis
-Most common
reason for
admission
Vaso-occlusion
within bone /
bone marrow
vasculature
Acute pain +/- swelling in any
area, mild fever may be present
Acute chest
syndrome
-Major cause of
mortality
Infarction,
infection
Chest pain, fever, cough +/wheeze, respiratory distress,
hypoxia, chest signs, CXR
abnormalities
Abdominal crisis
Ischaemia /
infarction leading
to ileus
Abdo pain, fever, vomiting,
distension, guarding, fluid levels in
bowel loops on AXR
Emma Astwood
Page 3 of 9
October 2015
Acute
sequestration usually spleen,
rarely liver
Blockage of
venous drainage
Rapid increase in spleen (or liver)
size, abdo pain, symptomatic
anaemia, hypovolaemic shock
CNS event - TIA,
stroke
Ischaemia /
infarction /
haemorrhage
Acute neurological deficit, seizure,
headache, raised intracranial
pressure.
Priapism
Blockage of
venous drainage
Pain
Aplastic crisis
Usually caused
by infection with
Parvovirus B19
Symptomatic anaemia - pallor,
shortness of breath, fatigue. No
increase in jaundice. Also, signs
of Parvovirus B19 infection may
be present (fever, red cheeks).
Low Hb with reduced reticulocytes
Other complications:
Eye complications - proliferative retinopathy, haemorrhage, orbital infarction
Anyone presenting with acute eye pain, red eye or visual loss needs urgent
ophthalmology review
Airway hyper-reactivity, and longer term risk of pulmonary hypertension
Osteomyelitis – causative organisms include salmonella
Cholecystitis - gallstones secondary to high rate of haemolysis
DVT (very rare)
Acute myocardial infarction (very rare)
Always take note if a patient tells you their pain is not typical - may require
further investigation. Also make sure you consider non-sickle related causes
of symptoms
Management
General Principles:
The aim of treatment is to break the ‘sickling’ cycle which is achieved by:
 Hydration
 Oxygenation
 Prompt and adequate analgesia
 Early and prompt treatment of infection
 Identification and treatment of any complications
Emma Astwood
Page 4 of 9
October 2015
Hydration:
 Children with sickle cell disease can become dehydrated easily due to
impairment of renal concentrating power.
 All children should be assessed for any degree of dehydration by the
history; duration of illness, clinical examination and weight loss (if known).
Hb and PCV may be elevated as compared to the child’s steady state
values.
 A strict input/output chart should be completed on every child.
 Hyper-hydration with 1.5 x maintenance should be started on every child.
This fluid can be given orally/NG if patient well, otherwise IV.
Oxygenation:



High-flow O2 if SaO2≤95% in air or >3% below baseline SaO2 recorded at
last outpatient review
Aim to keep SaO2>95%
The patient’s oxygen saturation should be monitored by pulse oximetry
with regular readings on room air (minimum 4 hourly).
Prompt and effective analgesia:





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Analgesia should be started within 30 minutes of arrival
If severe pain - usually require IV morphine / Intranasal diamorphine for
rapid control of pain.
Once pain controlled manage with oral morphine if possible, otherwise
PCA / NCA.
Be guided by the patient’s/parents’ assessment of their own pain severity
Prescribe paracetamol + NSAID as well as opioids
Prescribe regular laxative and PRN antiemetic
Emma Astwood
Page 5 of 9
October 2015
Painful Sickle Cell Crisis
Red flags
Pre-hospital analgesia with paracetamol and
ibuprofen?
Chest pain
Sats<95% or ↑O2
requirement
Fever>38̊̊̊˚C
↑RR or effort
(recession, tracheal
tug, nasal flare)
Atypical pain
No - do pain score, give
paracetamol and ibuprofen,
review in 30 minutes with
repeat pain score. Follow
algorithm depending on pain
score
Yes
Complications
Acute stroke
Aplastic crisis
Infection
Osteomyelitis
Splenic
sequestration
Priapism
Pain score
30 minutes:
Give
analgesia
1 hour:
Reassess
pain
score,
sedation,
BP, HR,
RR,
Sats,
Temp
Mild <4
Moderate 4-7
Severe >7
Consider low
dose oral
morphine
Give oral
morphine
Give intranasal
diamorphine and dose
of oral morphine and
cannulate
Pain
controlled monitor for 2
hours on ward
if had oral
morphine. If
not can be
discharged
Pain score >4
Give dose of
oral morphine.
Monitor on
ward for 2 h
Pain
controlled monitor for 2
hours on
ward, ensure
regular
analgesia
written up
Pain score >4
Consider
intranasal
diamorphine
and
cannulate.
Follow severe
guideline
Pain
controlled monitor for 4
hours on
ward, ensure
regular
analgesia
prescribed
Pain score >7
after 30
minutes commence
PCA or NCA
(see separate
guidance for
PCA / NCA)
Prescribe regular analgesia. Reassess pain score every 30 minutes until pain controlled, then hourly
Sedation score, BP, HR, RR, Sats and Temp reassessed hourly
After 6 hours of hourly observations and patient stable, can reduce observations to 4 hourly unless on a
PCA/NCA
Doses as per BNFc. Note: intranasal diamorphine can only be given to children > 10kg.
Emma Astwood
Page 6 of 9
October 2015
Blood Tests
Blood tests should be performed in all children with a pain score of 4-7 despite
paracetamol and a NSAID or in whom infection, a specific crisis or acute anaemia is
suspected. Blood tests should include a FBC, reticulocyte count, U+Es/creatinine
and LFTs. Consider additional blood tests if infection or a specific crisis is suspected
(see below).
Early and prompt treatment of infection:
All children with sickle cell disorders are at increased risk of infection due partly to
hyposplenism.
The risk is highest for the HbSS genotype, and in infants up to the age of 5. This is
the time of particularly high risk for infection with encapsulated organisms such as
Pneumococcus, Meningococcus, Haemophilus and Salmonella.
It is common for a child with a simple acute painful episode to present with fever and
no obvious evidence of infection. It may therefore be difficult to differentiate between
acute bone infarction due to sickling and an osteomyelitis or septic arthritis.


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

Cultures of blood, urine and other possible sites of infection should be
routinely taken in any child presenting with acute pain and fever.
If there is a high level of suspicion imaging by ultrasound to look for a
subperiostial fluid collection and surgical drainage should be considered
before starting antibiotics.
Prophylactic penicillin should be continued if a different antibiotic is not
prescribed for a specific infection.
The first line IV antibiotic for infection is Ceftriaxone. If the child requires oral
antibiotics the first line is Co-Amoxiclav.
If the patient has chest signs or an abnormal CXR add in clarithromycin
Consider malaria films, particularly if travelled to at risk region <1 year ago.
Identification and treatment of specific crises:
*Discuss with paediatric haematology/oncology consultant on call urgently
Bony crisis - Consider osteomyelitis (including salmonella) if persistent pyrexia,
positive blood cultures, high CRP (may be secondary to infarction, similar symptoms)
Acute chest syndrome - Urgent CXR, blood gas, broad spectrum antibiotics (IV
ceftriaxone + IV clarithromycin), consider trial of bronchodilators, incentive
spirometry. If severe will require urgent exchange transfusion. If persistent pain
consider checking cardiac enzymes. [See Sickle Cell (Chest Crisis guideline)]
Abdominal crisis - Check LFTs, abdo USS to look for gallstones + splenic size,
consider checking amylase. Surgical review unless mild symptoms without guarding.
* (if severe)
If on iron chelation therapy, and presenting with abdominal pain and diarrhoea,
consider Yersinia infection. Stop chelation whilst investigating.
Emma Astwood
Page 7 of 9
October 2015
Splenic (or rarely hepatic) Sequestration – Urgent transfusion (not dependent on Hb
level), careful monitoring. Avoid surgery during acute episode but consider
splenectomy at a later date. * [See Sickle Cell (Sequestration guideline)]
CNS event - Urgent CT brain, urgent exchange transfusion if confirmed.* Involve
neurosurgeons if haemorrhagic event. [See Sickle Cell Disease (Neurological
complications) guideline]
Priapism - Early surgical review. May require aspiration and irrigation with etilefrine. *
[See Sickle Cell (Priapism guideline)]
Aplastic crisis - Check Parvovirus B19 serology. See 'Transfusion' below. [See Sickle
Cell (Acute Anaemia) guideline]
Transfusion - Consider if: symptomatic anaemia, fall in Hb of 20g/L or more below
baseline particularly if absent reticulocyte response. In sequestration transfusion is
required urgently even with a normal Hb. Blood must be Rh and Kell antigen
matched and leucocyte depleted. Aim for baseline Hb in most situations. In splenic
sequestration aim for a Hb of 100g/L.
Other Considerations:
 Continue regular folic acid.
 Check vaccination status.
 If on hydroxycarbamide, stop during acute crisis if neutropenic (<1x109/l)/
thrombocytopenic (<100x109/l)/ low reticulocyte count (<100x109/l).
 Haemoglobinopathy Service Information leaflet ‘Pain relief for your child at
home’
 Notify community sickle cell nurse counsellors of admission:
 Nottingham Sickle Cell and Thalassaemia Service, Tel: 0115 8838424 Fax:
0115 8838425. Lead Specialist Manager: Joanne Bloomfield
Emma Astwood
Page 8 of 9
October 2015
Summary of Management
Child presents with sickle cell crisis
Generic management:






ABC + call help if needed
O2
Warmth
Analgesia (initial pain control +
ongoing needs)
Hyperhydration
Send baseline bloods
What type of crisis is this? (see above)
Specific management and
investigation for type of crisis (see
above) + haematology advice as
needed
References
1. ‘Sickle cell disease in childhood, standards and guidelines for clinical care’.
Published by the NHS Sickle Cell and Thalassaemia Screening Programme
2nd Edition October 2010. Full guideline available at: http://www.kclphs.org.uk/haemscreening/Documents/DETAILED_CLIN_Oct19.pdf
2. National confidential enquiry into patient outcome and death – May 2008 – ‘a
sickle crisis?’. Full report available via NCEPOD website:
http://www.ncepod.org.uk/newsletters.htm
3. Dick MC. Standards for the management of sickle cell disease in children.
Arch Dis Child Educ Pract Ed, 2008;93:169-176.
4. Shord SS et al. Evaluation of opioid induced nausea and vomiting in sickle
cell disease. Am J Hematol, 2008 Mar;83(3):196-9
5. Guidelines for the management of the acute painful crisis in sickle cell
disease. Prepared on behalf of the British Committee for Standards in
Haematology General Haematology Task Force by the Sickle Cell Working
Party. British Journal of Haematology, 2003;120:744-52
6. Sickle cell disease in childhood – management Whittington Health NHS,
March 2015
Emma Astwood
Page 9 of 9
October 2015