MANAGEMENT OF SICKLE CELL PAIN CRISIS – Outpatient department Version
Full history and clinical examination:
Should include possible precipitating/ associated symptoms (e.g. dehydration, fever, abdominal pain, jaundice), any focus of
infection, pallor, and weight loss. Clinical examination must include assessment of liver & spleen size (cm) and chest
symptoms. Consider other causes of pain e.g. appendicitis
Baseline Observations: Temp, pulse rate,
respiratory rate, O2 saturations, BP & Blantyre scores.
O2 saturations monitored every 20-30 minutes until
pain controlled or patient stable.
Admit those with the following
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Investigations:
 CBC, Hb estimate
 Remember you might need a blood compatibility
screen
 Malaria Parasite
 Blood cultures & Urinalysis
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Sickle Cell Crisis - Pain Management Regimen
Stage 1: Mild Pain
Paracetamol (15mg/kg). PO/PR - Re-assess
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Pain not controlled
Not drinking / tolerating oral fluid
Significant anaemia (Hb <5g/dl)
Increased pallor, breathlessness, exhaustion
Abdominal pain or distension, diarrhoea,
Vomiting
Jaundice or evidence of haemolysis
Pyrexia, tachycardia or tachypnoea,
Chest pain
Headaches, drowsiness, stroke or any
abnormal neurological signs
Priapism (> 2 hours)
Stage 2: Moderate Pain
Paracetamol (15mg/kg) & ibuprofen (10mg/kg)
Stage 3: Moderate to Severe Pain
Paracetamol (15mg/kg) & ibuprofen (10mg/kg) plus oral morphine. If
pain improved but not completely – consider increasing dose of
paracetamol to 20mg/kg for 24 hours – patient must be admitted if this
dose is given.
(See dosing chart)
If no ibuprofen use diclofenac 1mg/kg IM if greater than 1 year old
Management
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Maintenance fluids orally, or IV Ringers lactate if not able to drink (See fluid calculation chart)
Analgesia according to pain management regime
Oxygen if Sa02 <92%
Treat Malaria using the Malaria protocol
Ceftriaxone(100mg/kg od) and Gentamicin(7.5mg/kg od)
Change to intravenous Ciprofloxacin (10 mg/kg every 8 hours) if no improvement on the above after 48hours
Prophylactic penicillin (or erythromycin if allergy to penicillin)
Folic acid, antihistamine & laxatives (if on opioids)
BLOOD TRANSFUSION- not always indicated, unless in severe anaemia, acute chest syndrome, cerebrovascular
events, multi-organ failure. Aim to keep HB closer to 10g/dl in these cases
Discharge when:
 Pain controlled on oral analgesia
 No other symptoms are present
 Parents happy to manage at home
 Maintenance fluid taken & tolerated (Calculate 24-hour fluid requirement & inform parents)
 Antibiotics prescribed if any indications of infection
 Remember prophylaxis penicillin, folic acid and Fansidar malaria prophylaxis
1
MANAGEMENT OF SICKLE CELL PAIN CRISIS – Inpatient Version
Full history and clinical examination:
Should include possible precipitating/ associated symptoms (e.g. dehydration, fever, abdominal pain, jaundice), any focus of
infection, pallor, and weight loss. Clinical examination must include assessment of liver & spleen size (cm) and chest
symptoms. Consider other causes of pain e.g. appendicitis
Investigations:
 CBC, Hb estimate
 Remember you might need a cross match
 Malaria Parasite

Urinalysis
Baseline Observations: Temp, pulse rate,
respiratory rate, O2 saturations, BP & Blantyre scores.
O2 saturations monitored every 20-30 minutes until
pain controlled or patient stable.
Sickle Cell Crisis - Pain Management Regimen
Stage 1: Mild Pain
Paracetamol (15mg/kg). PO/PR - Re-assess
Stage 2: Moderate Pain
Paracetamol (15mg/kg) & ibuprofen (10mg/kg)
Stage 3: Moderate to Severe Pain
Paracetamol (15mg/kg) & ibuprofen (10mg/kg) plus oral morphine. If pain improved
but not completely – consider increasing dose of paracetamol to 20mg/kg for 24
hours – patient must be admitted if this dose is given.
(See dosing chart)
If no ibuprofen use diclofenac 1mg/kg IM if greater than 1 year old
Management
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Maintenance fluids orally, or IV Ringers lactate if not able to drink (See fluid calculation chart)
Analgesia according to pain management regime
Oxygen if Sa02 <92%
Treat Malaria using the Malaria protocol
Ceftriaxone (100mg/kg od) and Gentamicin(7.5mg/kg od)
Change to intravenous Ciprofloxacin (10 mg/kg every 8 hours) if no improvement on the above after 48hours
Prophylactic penicillin (or erythromycin if allergy to penicillin)
Folic acid, antihistamine & laxatives (if on opioids)
BLOOD TRANSFUSION- not always indicated, unless in severe anaemia, acute chest syndrome, cerebrovascular
events, multi-organ failure. Aim to keep HB closer to 10g/dl in these cases
Discharge when:
 Pain controlled on oral analgesia
 No other symptoms are present
 Parents happy to manage at home
 Maintenance fluid taken & tolerated (Calculate 24-hour fluid requirement & inform parents)
 Antibiotics prescribed if any indications of infection
 Remember prophylaxis penicillin, folic acid and Fansidar malaria prophylaxis
2
SICKLE CELL CRISIS – PAIN RELIEF QUICK DOSING REFERENCE
Drug
Paracetamol
(PO or PR)
Ibuprofen
(preferred NSAID
for children < 1 year)
Diclofenac
Oral Morphine
Dose (inpatient)
15 mg/kg 4-6 hourly – maximum 4
doses in 24 hours.
If pain not controlled and child
needing help – dose can be
increased to 20mg/kg for 24 hours
Maximum single dose = 1 g
10 mg/kg 6-8 hourly – maximum 3
doses in 24 hours.
Maximum single dose = 400mg
(8hrly)
1 mg/kg 8 hourly – maximum 3
doses in 24 hours.
Maximum single dose = 50 mg
6-12 months
0.2mg/kg *4 hourly – maximum 6
doses in 24 hours.
From 1 year – 50 kg
0.2-0.3mg/kg *4 hourly – maximum
6 doses in 24 hours.
Over 50kg
15 mg *4 hourly – maximum 6
doses in 24 hours.
3
Dose (Outpatient)
15 mg/kg 4-6 hourly – maximum 4
doses in 24 hours
Maximum single dose = 1 g
5 – 10 mg/kg 4-6 hourly –
maximum 4 doses in 24 hours.
>50 kg give 400 mg 8 hourly
1 mg/kg 8 hourly – maximum 3
doses in 24 hours.
Maximum single dose = 50 mg
Not indicated
SICKLE CELL ANAEMIA AND FEVER
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Full history and clinical examination:
Should include associated symptoms (e.g. any focus of infection, pallor, cough, abdominal pain,
vomiting, diarrhoea and weight loss.
Check immunisation status and penicillin prophylaxis
Check Malaria prophylaxis
Clinical examination must include assessment of liver & spleen size (cm) and chest symptoms.
Consider other causes of pain e.g. appendicitis
Assess hydration status -Evidence of dehydration or shock (poor capillary refill, skin turgor, sunken
eyes, tachycardia, rapid, weak radial pulse, hypotension)
Remember bones! Osteomyelitis
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Investigations:
 CBC, Hb estimate,
 Malaria Parasite
 Urinalysis
 Chest X-ray
 Cross Match
Baseline Observations: Temp, pulse rate,
Respiratory rate, O2 saturations, BP & GCS/
Blantyre scores
Management
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Give high flow oxygen
Correct dehydration or shock following guidelines
Treat Malaria- following the Malaria guidelines
Intravenous Ceftriaxone 100mg/kg OD + gentamicin 7.5mg/kg od
If pneumonia suspected give ceftriaxone and oral azithromycin (10mg/kg od for 3 days)
Review antibiotics after 48 hours
All children with fever > 38.5 degrees should be admitted
Obtain chest X-ray if respiratory signs or symptoms, or high fever with no focus of infection
Transfuse if Hb<5 g/dl or evidence of acute chest syndrome, cerebrovascular events, multiorgan failure or on going vaso-occlusive episode despite analgesia and fluid management.
At discharge do not forget to give Malaria prophylaxis – ( Fansidar!.)
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SPLENIC SEQUESTRATION IN SICKLE CELL DISEASE
This is defined as severe anaemia HB<5 g/dl with new onset acutely enlarged and
tender spleen. Mild/moderate thrombocytopenia (Low platelet) is often present.
Consider aplastic anaemia as well if all the cell lines are low as well. It has a high
mortality rate
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Full history and clinical examination:
Should include associated symptoms (e.g. any focus of infection, PALLOR,
increasing tiredness)
Clinical examination must include assessment of liver & spleen size (cm) and
chest symptoms
Assess hydration status -Evidence of dehydration or shock (poor capillary refill,
skin turgor, sunken eyes, tachycardia, hypotension)
Baseline Observations: Temp, pulse rate
(tachycardia), respiratory rate, O2
saturations, Blood Pressure & Blantyre
Investigations:
 CBC & reticulocytes, Hb estimate
 If febrile –
Malaria Parasite
Blood cultures & microbiology,
Urinalysis
Chest X-ray
 Cross Match
Management
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While waiting for blood, give 0.9% Saline to treat Hypovolaemia. (1020ml/kg). Not more than 10ml/kg in patients who have pre-existing cardiac
condition.
Suggest initial transfusion of 10 ml/kg of packed red cells for patients with
haemoglobin <5g/dl or signs of shock.
Do not raise Hb above 10g/dl
Treat with antibiotics if febrile (see fever and sickle cell disease), and
analgesics for pain (see vaso-occlusive crisis)
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APLASTIC CRISIS & SICKLE CELL DISEASE
Associated with Hb below 5g/dl and low cell counts across all cell lines.
Usually associated with acute viral infection in particular parvovirus. May be associated with enlarged
spleen as well (see splenic sequestration)
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Full history and clinical examination:
Should include associated symptoms (e.g. any focus of infection, PALLOR, increasing tiredness,
cough, abdominal pain, vomiting, diarrhoea and weight loss.
Check immunisation status and penicillin prophylaxis
Clinical examination must include assessment of liver & spleen size (cm) and chest symptoms
Assess hydration status -Evidence of shock or dehydration (poor capillary refill, skin turgor, sunken
eyes, tachycardia, hypotension)
Investigations:
 CBC & reticulocytes,
 Oxygen saturation
 Malaria Parasite
 Cross Match
Baseline Observations: Temp, pulse rate,
Respiratory rate, O2 saturations, BP & Blantyre
score
Management
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Give high flow oxygen
Maintain and treat airway, breathing and circulation problems first
IV Normal saline and 5% Dextrose at maintenance volume per weight (See fluid calculation chart or
oral fluids at the same maintenance rates - do not add potassium to IV. fluid or use fluids with
potassium in
Transfuse patient if symptomatic anaemia or Hb < 5 g/dl (usually 10ml/kg over 4 hrs of whole blood).
Close observation for fluid overload. Transfusion may need to be repeated.
Treat fever and pain as required (see fever and crisis guidelines)
6
SICKLE CELL STROKE
Acute neurological events occur in about 10% of patients with HbSS.
These can present as
 Hemiparesis or hemiplagia
 Monoparesis
 Aphasia or dysphasia- loss of speech in a sickle cell patient suggest a stroke
 Seizures
 Cranial nerve palsies
 Coma
Can occur suddenly or as a complication of acute chest syndrome or aplastic crisis. Consider other
causes as well such as cerebral malaria or Meningitis
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Full history and clinical examination:
History should include illness before event
Clinical examination must include thorough neurological examination, pre-morbid status and
current deficit.
Never forget Pallor!!!
Glucose Level
Assess hydration status -Evidence of shock or dehydration (poor capillary refill, skin turgor, sunken
eyes, tachycardia, hypotension)
Level of consciousness
Investigations:
 CBC & reticulocytes, Hb Estimate
 Oxygen saturation
 Malaria Parasite
Baseline Observations: Temp, pulse rate,
Respiratory rate, O2 saturations, BP & GCS/
Blantyre score
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Cross Match
Management
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Give oxygen if O2 sats <90%
Maintain and treat airway, breathing and circulation problems first
If Unconscious NG (ORS) or IV Ringers Lactate at the maintenance volume for weight (See fluid
calculation charts) to avoid aspiration
Never forget to give GLUCOSE (5ml/kg bolus of 10% dextrose). Then maintenance fluid
Arrange for transfusion if low HB to maintain HB at 8g/dl.
Regular observations monitoring
Give broad-spectrum antibiotics for meningitis (see fever and sickle cell guideline)
Treat Malaria if positive BS or history of partially treated Malaria
Physiotherapy if neurological deficit present
Ensure follow up is arranged prior to discharge.
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ACUTE CHEST SYNDROME IN SICKLE CELL ANAEMIA
An acute illness related to infarction of the lung tissue. Usually associated with lower respiratory
symptoms, and hypoxia. Chest pain and hypoxia may be the only signs. Chest pain should be treated
as an acute chest syndrome and not simply as a vaso-occlusive disorder.
Note- this is a life threatening illness and patients may deteriorate quickly. ACT QUICKLY!
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Full history and clinical examination:
Focused pain history, chest pain, breathlessness
include associated symptoms (e.g. any other focus of infection, pallor, cough, ability to eat or
drink.
Check immunisation status and penicillin prophylaxis
Clinical examination must include assessment of respiration- Hypoventilation due to pain, oxygen
saturation, respiratory rate and temperature.
Never forget Pallor!!!
Assess hydration status -Evidence of shock or dehydration (poor capillary refill, skin turgor, sunken
eyes, tachycardia, hypotension)
Investigations:
 CBC & reticulocytes, Hb estimate
 Oxygen saturation
 Malaria Parasite
 Urinalysis
 Chest X-ray – when stable
Baseline Observations: Temp, pulse rate,
Respiratory rate, O2 saturations, BP & Blantyre
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Cross Match
Management
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Give oxygen if saturations <90%
Maintain and treat airway, breathing and circulation problems first
Maintain hydration with IV Ringers lactate (See fluid calculation chart) + oral intake at maintenance
rate
Prescribe regular pain relief (using Vaso- Occlusive guidelines)
Broad-spectrum antibiotics -Ceftriaxone and azithromycin
Review antibiotics in 48 hours and change to Ciprofloxacin if no improvement
Obtain chest x-ray
All sickle cell patients with chest pain should be admitted,
Cross match and transfusion if required, maintaining HB> 8g/dl
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Management of priapism in sickle cell disease
Mean age of first episode is 15 years
SCD disease causes ischaemic or stuttering priapism
Nocturnal erections, sexual activity, dehydration, fever, and exposure to cold are the
most common precipitants of priapism in children with SCD
Aims are to
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Relieve pain
Abort erection and preserve sexual function
>36 hours of priapism – most will become impotent
Management steps
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Pain relief - Opiate analgesia should be given – morphine 0.4mg/kg every 4
hours orally
Drink extra fluids/IV fluids if not able to drink
Try to urinate
Walking
Warm bath (not cold water!!!!!)
If the above simple measures are not successful then need to consider surgical
options such as aspiration and irrigation. Should be referred to surgeons.
Aspiration – ONLY IF NO SURGEONS ARE AVAILABLE
Aspirate up 20-50 mls of blood from cavernosum- can be repeated 3 times
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Fluid Calculation in Children greater than 2 months old
For normal maintenance fluid without dehydration i.e. normal fluid requirement in
24hours (day) use this formula.
Fluid Calculation
First 10kg
Next 10kg (11-20)
Remain amount (>20kg)
–100ml /Kg
–50ml/kg
-20ml/kg
Total divided by 12 gives volume to be given every 2 hours
This is different from fluid management in a dehydrated child or in children with
diarrhea and vomiting who will require management using Plan A, B or C.
E.g. 25 kg Child
First 10kg
Next 10kg
Next 5kg
Total
1600ml/12
10* 100
10* 50
5 * 20
1000
500
100
1600 In 24hours
133 mls every 2 hours
10* 100
4* 50
1000
200
1200 In 24hours
100 mls every 2 hours
9* 100
900
900 In 24hours
75 mls every 2 hours
For a 14 kg child
First 10kg
Next 4kg
Total
1200ml/12
For a 9 kg baby
First 10kg
Total
900ml/12
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