Guideline for Sickle Cell
Disease Patients Admitted
to Nalufenya
TABLE OF CONTENTS
Topic
Page Number
Routine care of patients with Sickle cell
3
Inpatient Management of Painful Crisis
4
Pain Relief Formulary
5
Sickle Cell and Fever
6
Splenic Sequestration
7
Aplastic Anaemia
8
Sickle Cell Stroke
9
Acute Chest Crisis
10
Management of Priapism
11
Fluid calculation
12
2
Routine Care For Sickle Cell Patients
All patients with sickle cell disease should attend Sickle Cell Clinic each
month and be given the following medications:
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Folic acid 5 mg tablets once day
ORS – 3 – 6 packets depending on age, patients should drink plenty of
fluids daily
Paracetamol 15 mg/kg 4 – 6 hourly for pain or fever
Fansidar tablets once a month
Age
< 2years
2 – 5 years
5- 10 years
10 – 15 years
>15 years
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Dose of Fansidar
¼ tablet
½ tablet
1 tablet
2 tablets
3 tablets
Multivitamin tablets - 1 tablet od < 20 kg and 2 tablets od > 20 kg
Tablet Penicillin V < 3 years 125 mg bd daily, 3- 5 years 250 mg bd
Pneumococcal vaccine for those who can afford to buy – this should
be given at 2 years of age and then should be re-vaccinated every 5
years
Children 2- 18 years who have suffered from stroke; had > 3 episodes
of painful crisis in a year; had 2 episodes of acute chest syndrome or
been transfused on more than two different admissions in one year can
be started on hydroxyurea as directed by a paediatrician
 Dose initially 10 mg/kg once a day increased by 5 mg/kg every 3
months to a dose of 15-30 mg/kg once a day depending on response
 CBC should be checked monthly – if patient becomes
neutropenic treatment should be stopped (neutrophils < 1000/mm3
The following patients should ALWAYS be admitted:
 Pain not controlled after adequate analgesia
 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)
3
In-patient Management of Painful Sickle Cell Crisis
Investigations:
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CBC, Hb estimate
Group and Cross match for 10 ml/kg packed red cells if Hb
<10g/dL
Malaria Parasite
Blood cultures & Urinalysis
Management
 Give 150% of normal maintenance fluids orally as ORS, or IV Ringers lactate if not
able to drink (See fluid calculation chart)
 Analgesia
 Oxygen if Sa02 <90%
 Treat Malaria using the Malaria protocol
 Ceftriaxone (100mg/kg od) and gentamicin (7.5mg/kg od) if evidence of infection
 Change to intravenous Ciprofloxacin (10 mg/kg every 8 hours – maximum 400mg/d) if no
improvement after 48 hours
 Prophylactic penicillin (or erythromycin if allergy to penicillin)
 Folic acid, antihistamine & laxatives (if on opioids)
 Blood transfusion- not always indicated, unless severe anaemia, acute chest syndrome,
cerebrovascular events, multi-organ failure. Aim to keep HB closer to 10g/dl in these
cases
Analgesia
Stage 1: Mild Pain
Paracetamol (15mg/kg) 4- 6 hourly. PO/PR
Stage 2: Moderate Pain
Paracetamol (15mg/kg) 4 – 6 hourly & ibuprofen (10mg/kg) 8 hourly
Stage 3: Moderate to Severe Pain
Paracetamol (15mg/kg) 4 – 6 hourly & ibuprofen (10mg/kg) 8 hourly plus oral morphine.
If pain improved but not completely – consider increasing dose of paracetamol to 20mg/kg for 24
hours.
(See dosing chart)
If no ibuprofen use diclofenac 1mg/kg IM if greater than 1 year old
Discharge when:
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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
using fluid calculation guidance on page 13)
Antibiotics prescribed if indications of infection
Remember prophylactic penicillin, folic acid and Fansidar malaria prophylaxis
4
Sickle cell crisis – analgesia formulary
Drug
Dose (Inpatient)
Dose (Outpatient)
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
15 mg/kg 4-6 hourly –
maximum 4 doses in 24
hours
Ibuprofen
(preferred NSAID
for children < 1 year)
10 mg/kg 6-8 hourly –
maximum 3 doses in 24
hours.
Maximum single dose=
400mg (8hrly)
5 – 10 mg/kg 4-6 hourly –
maximum 4 doses in 24
hours.
>50 kg give 400 mg 8
hourly
Diclofenac
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.
1 mg/kg 8 hourly –
maximum 3 doses in 24
hours.
Maximum single dose =
50 mg
Not indicated
Paracetamol
(PO or PR)
Oral Morphine
5
Maximum single dose = 1
g
Sickle cell disease and fever
Patients with sickle cell disease are at increased risk of infection due to
hyposplenism.
Children may present with septicaemia, pneumonia, meningitis or
osteomyelitis and are susceptible to malaria.
Look for signs of dehydration (slow skin pinch, sunken eyes, lethargy)
and shock (tachycardia, cool peripheries, weak pulse, prolonged
capillary refill time)
Remember to examine bones for evidence of osteomyelitis – local
tenderness or swelling, ask if patient has been limping/refusing to
weight bear
Investigations
Baseline Observations: Temp, pulse
rate,
Respiratory rate, O2 saturations, BP &
GCS/ Blantyre scores
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CBC, Hb estimate
Malaria Parasite
Urinalysis
Chest X-ray
Cross Match
Management
 Correct dehydration or shock following guidelines
 Treat Malaria- following the Malaria guidelines
 For sepsis treat with Ceftriaxone 100mg/kg OD +
gentamicin 7.5mg/kg od
 If pneumonia suspected give ceftriaxone 100 mg/kg od
and oral azithromycin 10mg/kg od for 3 days (or
erythromycin:
< 2years 125 mg qds, 2-8 years 250 mg qds, 8-18 years
500 mg qds)
 Review antibiotics after 48 hours
 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
6
Acute anaemia due to splenic sequestration
Acute anaemia in SCD is most commonly due to splenic sequestration or
temporary red cell aplasia (usually due to parvovirus B19 infection)
In splenic sequestration the patient will present with an acute fall in
haemoglobin and acute enlargement of their spleen. It is a serious
complication of SCD and has high mortality.
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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 spleen size (document
measurement) and chest symptoms
Assess hydration status -Evidence of dehydration or shock (poor capillary
refill, skin turgor, sunken eyes, tachycardia, hypotension)
Management
 Insert cannula and take blood for Hb estimation and grouping and
cross matching – 10 ml/kg of packed red cells
 Give 10 ml/kg fluid bolus of Ringers Lactate or Normal Saline over
one hour or until blood is available
 Aim to raise Hb to 10 g/dL
 Assess for signs of infection and treat as appropriate
 Treat pain as per analgesia guidelines
7
Anaemia due to aplastic crisis
Patient may have a drop in their Hb over a week and may have associated
headache, abdominal pain and fever. It is difficult to differentiate between
splenic sequestration and aplastic crisis when reticulocyte count and IgM for
parvovirus testing are not available.
Investigations:
 CBC & reticulocytes,
 Oxygen saturation
 Malaria Parasite
 Cross Match
Baseline Observations: Temp, pulse
rate,
Respiratory rate, O2 saturations, BP
&Blantyre score
Management
 Insert IV cannula and take blood for CBC and reticulocytes, blood
slide and cross matching
 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 (10ml/kg
of packed red cells).
 Patient may need more than one transfusion.
 Treat fever and pain if present (see fever and pain advice)
8
Stroke
Acute neurological events occur in about 10% of patients with HbSS.
These can present as
 Hemiparesis or hemiplegia
 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. Always
consider other causes 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, premorbid 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
 Cross Match
Baseline Observations: Temp, pulse
rate,
Respiratory rate, O2 saturations, BP &
GCS/ Blantyre score
Management
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Give oxygen if O2 sats <90%
Maintain and treat airway, breathing and circulation problems first
If unconscious, pass an NGT to give ORS/milk feeds or IV Ringers
Lactate at the maintenance volume for weight (See fluid calculation
charts) to avoid aspiration
 Never forget to give dextrose (5ml/kg bolus of 10% dextrose). Then
maintenance fluid
 Arrange for transfusion if low HB to maintain HB at >10g/dl
 Regular observations monitoring - blood pressure, pulse, saturations,
level of consciousness
 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.
9
Acute Chest Syndrome
Patient will have pleuritic chest pain, fever 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. Patients can deteriorate quickly so treat urgently.
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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.
Clinical examination must include full respiratory assessment - oxygen
saturation, respiratory rate – there may be hypoventilation due to pain
Assess hydration status -Evidence of shock or dehydration (poor capillary
refill, skin turgor, sunken eyes, tachycardia, hypotension)
Baseline Observations: Temp, pulse
rate,
Respiratory rate, O2 saturations, BP
&Blantyre
Investigations:
 CBC & reticulocytes, Hb
estimate
 Oxygen saturation
 Malaria Parasite
 Urinalysis
 Chest X-ray – when stable
 Cross Match
Management
1. Admit to the emergency room and give oxygen via face mask
2. Maintain hydration with IV Ringers lactate (See fluid calculation chart) + oral
intake at maintenance rate
3. Prescribe regular morphine
4. Give IV antibiotics –IV Ceftriaxone and oral erythromycin
5. Consider chest x-ray – can patient be safely transferred to main hospital on
oxygen?
6. Cross match and transfuse 10 ml/kg of packed red cells if Hb is less than
10g/dL
10
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 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 (150% usual maintenance rate for weight) or give
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
11
Fluid Calculation in Children greater than 2months 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 kg)
Subsequent kilos (>20kg)
100ml /Kg
50ml/kg
25ml/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 * 25
1000
500
125
1625 In 24hours
135 mls every 2 hours
For a 14 kg child
First 10kg
Next 4kg
Total
1200ml/12
10* 100
4* 50
1000
200
1200 In 24hours
100 mls every 2 hours
For a 9 kg baby
First 10kg
Total
900ml/12
9* 100
900
900 In 24hours
75 mls every 2 hours
12