Obstetric and gynecology

1. SICKLE CELL ANEMIA/SICKLE CELL
CRISES MANAGEMENT GUIDELINES
HAEMATOLOGY UNIT –GAFAR IBN AUF
HOSPITAL
AUGUDT 2018

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SICKLE CELL ANEMIA/SICKLE CELL CRISES MANAGEMENT
GUIDELINES
INTRODUCTION:
.It must be emphasized that these guidelines do not replace on-going care by knowledgeable
providers.
When patients with sickle cell disease come to the emergency department they should be
triaged rapidly, and the physician responsible should be notified of their presence as
soon as possible.
Terminology
Sickle cell disease refers to all clinically relevant homozygous or doubly heterozygous sickle
hemoglobinopathies (HbSS, HbSC, HbSb0
–Thalassemia, etc); sickle cell anaemia refers
specifically to the homozygous disorder, HbSS.
Definitions of pain severity can be found in Table, with the accompanying descriptions of the
OUCHER test and the Verbal Report Scale.
PAIN
SEVERITY
VERBAL REPORT
SCALE (0-10)
OUCHER
SCORE
COMMENTS
Mild 1-3 10-30 − may not appear uncomfortable
− complains of pain
− +/- crying, neutral expression, at rest
or shifting in chair
Moderate 4-6 40-60 − may show facial grimacing, unhappiness,
irritability, poor appetite
− may be unable to carry out normal daily
activities, uninterested in social
interaction
Severe >7 >70 − facial grimacing, unhappiness, irritability,
poor appetite
− crying very uncomfortable to point of
agony
Seriously ill patients are defined as those:
• In shock
• With sepsis, meningitis, stroke, aplastic crises, or acute splenic sequestration crisis
• That are decompensating (e.g., progressing into chest crisis)
• With O2 saturation <90% while breathing room air

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1. ACUTE PAINFUL EPISODES (VASO-OCCLUSIVE CRISES)
Clinical/Laboratory Features
Painful VOC is the most frequent complication of sickle cell disease.
pain should be treated as early as possible, as persistent pain can debilitate the patient both
physically and psychologically. No laboratory features are pathognomonic of VOC; diagnosis
is based strictly on the history and physical examination.
Emergency Department Management
• Place patients with concurrent fever of 38.3°C oral (37.8° axilla) or higher immediately into
a room, and follow the Fever Protocol. After antibiotics have been administered, assess the
patient thoroughly
• Place children without fever in a room as soon as possible, and conduct a brief history and
physical concurrently with other measures
• Physical vital signs including O2 saturation, cardiopulmonary and hydration status, spleen
size, neurology exam, presence of jaundice, and localizing signs of infection
• Tests: Request blood counts (CBC, differential and reticulocyte count). Consider blood
typing and cross-matching if the child is in severe pain of his/her hemoglobin is 15 g/L or
more below baseline and reticulocyte count is suggestive of bone marrow suppression. If
clinically indicated, request a urine analysis, electrolytes, LFTs and amylase. In the
presence of chest pain, fever, or respiratory symptoms, request a chest x-ray and monitor
the child's O2 saturation. If the patient has severe respiratory distress, test arterial blood gas
(ABG) levels as well
• Insert a IV if the patient is febrile, dehydrated or in moderate to severe pain
Medications
• Mild to moderate pain: acetaminophen (15 mg/kg/dose) with codeine
(1 mg/kg/dose) PO q4h PRN (acetaminophen max, 65 mg/kg/day, and codeine max. 60
mg/dose and 6 mg/kg/day). These can be given separately, or together. Ibuprofen may
be helpful (6 mos-12 yrs: 5-10 mg/kg/dose PO q6-8H, max 40 mg/kg/day or 2400
mg/day). Encourage patients to drink. If within 30-60 minutes pain relief is inadequate
and there are no other acute complications, the patient can be discharged on oral
analgesics.
• Moderate to severe pain: IV bolus of morphine 0.1-0.15 mg/kg/dose (max. 7.5
mg/dose). It can be repeated once, 60 minutes later, if pain is inadequate controlled.
• Severe pain: morphine continuous IV infusion 40 micrograms/kg/hour. Administer IV
fluid to all patients in moderate to severe pain: a 10mL/kg bolus of saline, followed by1.5
times the maintenance as dextrose 5% in sodium chloride 0.45% solution. Additional
boluses of morphine, 0.05 mg/kg, can be given q1-2h PRN. If adequate pain relief is
established for 2 hours with 1 or 2 doses of intermittent morphine, then consider
administering acetaminophen with codeine (see above). These patients can then be
discharged home, if they are able to take oral analgesics. Any patient requiring more than
2 doses of morphine should be hospitalized.
• Pethidine 1mg/kg/dose 4-6 hours if morphine is not available.

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OUTPATIENT MANAGEMENT
Medications
• Oral analgesics: acetaminophen (15 mg/kg/dose) with codeine (1mg/kg/dose) PO q4h for
a period of 48 hours (acetaminophen, max. 65 mg/kg/day and codeine, max. 60 mg/dose
and 6mg/kg/day).
• Ibuprofen may also be used (6mos -12 yrs; 5-10 mg/kg/dose PO q6-8h, max 40 mg/kg/day
or 2400 mg/day).
• If pain persists after 48 hours, patient should be re-evaluated
INPATIENT MANAGEMENT
Hospitalization is mandatory if pain control with oral analgesics is not adequate, or if other
problems (such as fever and dehydration) exist.
• Patients to be admitted under Paediatric Haematology Service if available.
• Observe patients closely for signs of deterioration with continuous cardiac and O2
saturation monitoring, vital signs q4h, fluid input and output, and daily weight. Assess
the child's comfort level q4h, and before and after each pain medication and non-
pharmacological intervention, with a consistent pain tool (see Table 1). Encourage as
much ambulance and activity as the child can tolerate
• Incentive spirometry is indicated for older children with chest or back pain: 10 breaths
q1-2h while awake, or 5 breaths every 15 minutes .If not available encourage deep
breathing.
• Offer heating pads, massage, warm baths, and other comfort measures. Request
consultations with a physiotherapist as appropriate.
Medications
IV morphine: 40 micrograms/kg/hour; if pain relief is inadequate titrate the dose q8h by
increments of 10-20 micrograms/kg/hour to a maximum of 100 micrograms/kg/hr. Boluses
of morphine (0.05 mg/kg) can be administered q1-2h IV PRN for breakthrough pain. When
effective analgesia is maintained for 24 hours, the dose can be decreased stepwise in 20
micrograms/kg/hour decrements, q8h
• Oral Morphine: An equivalent dose of long acting oral morphine may be used as an
alternative to continuous IV morphine in stable inpatients
• Step-down therapy: If the child is comfortable and has graduated to demand dosing only,
switch to oral analgesics: acetaminophen (10-15 mg/kg/dose) with codeine (1 mg/kg/dose
and 6 mg/kg/day) given separately or together. Ibuprofen may be helpful (6 mos- 12 yrs:
5-10 mg/kg/dose PO q6-8h, max 40 mg/kg/day or 2400 mg/day)

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• Stool softener: lactulose 1 ml/kg/day as a single dose or in two divided doses ,unless the
child has diarrhea
• Antihistamines : PRN for pruritus
• Hydration: continue IV/PO fluids at 1-1.5 times the maintenance rate
• Oxygen: monitor O2 saturation regularly, and provide oxygen to patients exhibiting
hypoxemia,routine use of oxygen is not recommended.
Discharge Guidelines: Discharge if
• Tolerating fluids and medications by mouth
• Pain control is adequate with PO medications
• Concurrent problems are resolved
2. Fever
EMERGENCY DEPARTMENT MANAGEMENT
Patients with sickle cell disease who present with fever of 38.3°C oral (37.8°C axilla) or
higher should be treated immediately
• Place the child immediately into an examining room, take a history, and do a physical
exam, concurrently with other measures (see below). Note vital signs, with O2 saturation,
temperature, degree of pallor, spleen size, and any neurological deficits, jaundice or
respiratory distress
• Take blood for CBC, reticulocyte count, blood film for malaria and blood culture. Ensure
that sickle cell disease is written on laboratory requisitions, and other tests as clinically
indicated.
• Give antibiotics within 30 minutes of presentation and before test results are available;
see Medications below for details. Parenteral antibiotics should be given even if there is
an obvious focus of infection (e.g. Otitis Media, URTI, etc)
• Other investigations may be indicated
− Chest x-ray, if the child has cough, hypoxemia, chest pain, or fever >40°C
− Monitoring arterial blood gases if available.
− Blood typing and cross-matching (cross & type) if the child has pallor, respiratory or
neurological symptoms, or splenic enlargement
− Urine culture, throat culture, stool culture
− Lumbar puncture
Evaluation for osteomyelitis

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• Medications
− Ceftriaxone 80mg/kg/dose IV (max. 2g/dose), given through the same venipunture as
blood was taken from. Intramuscular injection may be used if IV injection is not
possible
− Clindamycin (40 mg/kg/day IV ÷ q6-8h, max 2.7 g/day) if the patient is significantly
allergic to beta-lactam antibiotics, (e.g. anaphylaxis or other immediate
hypersensitivity reaction or serum sickness); clindamycin is not to be used alone as
the treatment of suspected meningitis, as it does not cross the blood-brain barrier
− Vancomycin (60 mg/kg/day IV÷q6h, max. 4g/day) in the child who is seriously ill
− Acetaminophen (15 mg/kg/dose PO/PR q4 PRN, max 65 mg/kg/day)
− Ibuprofen (6 mos – 12 yrs: 5-10 mg/kg/dose PO q6-8h, max 40 mg/kg/day or 2400
mg/day)
• Criteria for Admission
Hospitalization is strongly recommended when the patient appears unwell, particularly in
the presence of systemic toxicity, cardiovascular instability, and/or the following:
− Temperature is >40°C
− Recent doses of prophylactic penicillin have been missed
− Age <1 year
− Respiratory distress
− Segmental/lobar infiltrate on chest x-ray
− WBC is >30 or <5 X 109
/L, or platelets <150 C 109
/L
− Follow-up is uncertain (distance, inconvenience, poor compliance) or the family's
ability to cope is uncertain
− Previous episodes of severe sepsis or meningitis
INPATIENT MANAGEMENT
• Patients to be admitted under Paediatric Haematology Service if available.
• Observe patients closely for any deterioration in clinical status, which may indicate
septicemia or development of chest crisis (see Monitoring below)
• If the microbiology laboratory reports the 48h cultures as negative, stop antibiotics unless
there is a focal infection or persistent fever. If the culture is positive and the organism is
penicillin susceptible, change to penicillin. If the culture is positive for penicillin non-
susceptible pneumococcus, ensure that the patient is on vancomycin, in addition to
ceftriaxone or cefotaxime
Monitoring
• Measure vital signs q4h
• Measure O2 saturation continuously if the patient's O2 saturation < 94%
• Administer O2 to keep pulse O2 >94%
• Administer IV fluids at 1.5 times maintenance rate for the first day (and while patients
are febrile); then reduce to maintenance levels (PO + IV)
• Request blood cultures and CBC daily, if fever persists

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• Request reticulocyte counts twice a week.
• If the patient has penicillin/cephalosporin-resistant pneumococcal meningitis, or is not
improving after 36-48h of therapy, do a repeat lumbar puncture .
Antibiotic Regimens:
Meningitis not suspected
• Cefotaxime (200 mg/kg/day IV ÷ q6-8h; max 8g/day) until cultures are negative and
clinical status improves (minimum of 48h)
• Clindamycin (40 mg/kg/day IV ÷ q6-8h; max 2.7 g/day) in patients with significant
allergy to beta-lactam antibiotics
• Erythromycin (40 mg/kg/day IV ÷ q6-8h; max 4 g/day. Or 40 mg/kg/day PO ÷ q6-12h as
erythromycin estolate, max 2 g/day) for children 5-years of age or older with respiratory
symptoms and in children younger than 5-years of age, if high suspicion of Mycoplasma
• Vancomycin (60 mg/kg/day IV ÷ q6h; max 4g/day) is indicated in patients who are
severely ill (septic) or who deteriorate on cefotaxime/Ceftriaxone
• Penicillin 250,000 units/kg/day IV ÷ q4-6h (max 20 million units/day) where culture &
sensitivity results indicate the organism is penicillin susceptible
Presumed pneumococcal meningitis
• Ceftriaxone 80 mg/kg/dose IV given q12h X 3 doses then q24h (max. 2g/dose: 4g/day;
therefore in patients >25 kg, give 2g IV q12h x 3 doses, then give 2g IV q24h plus IV
Vancomycin (60 mg/kg/day ÷ q6h, max 4 g/day)
• Significant allergy to beta lactam antibiotics: Vancomycin (60 mg/kg/day IV ÷ q6h, max
4g/day) plus rifampin (20 mg/kg/day PO ÷ q12h max 1.2 g/day)
Discharge Guidelines
• Tolerating fluids and medications by mouth
• Afebrile for at least 24 h, with negative culture at 48 h
• Pulmonary symptoms, if any, have resolved
OUTPATIENT MANAGEMENT
Only a small fraction of these patients are potentially suitable of this form of management.
Following the initial dose of Ceftriaxone (in ED), if evaluation suggests that outpatient
management is possible, a short period of observation (2-4h) is advised, followed by re-
evaluation (assessment of vital signs, level of consciousness, and ability to take oral
fluids/meds) prior to discharge

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Outpatient management should be considered on a case by case option only if the
following criteria are met:
1. Temperature is <40°C
2. Recent doses of prophylactic penicillin have not been missed
3. Age >1 year
4. WBCs 5-20 X 109
/L; platelets >100 X 109
/L
5. No systemic toxicity; no other sickle cell complications
6. No respiratory distress
7. Ceftriaxone given
8. Follow-up can be ensures
Oral Medications
The patient should be given a prescription for a 3-day supply of an oral antibiotic:
• Cefixime 8mg/kg/day, given once daily (max. 400 mg/day)
or
• Cefaclor 40 mg/kg/day ÷ TID (max 1.5 g/day)
or
• Cefuroxime axetil 30mg/kg/day po ÷ bid (max 1 g/day) as suspension or 250 mg po bid
as tablets
Note: Patients with significant allergy to beta-lactam antibiotic may be treated with
clarithromycin or clindamycin (see below)
Acceptable alternatives include:
• Cefprozil: 6 mo to 12 years of age: 30 mg/kg/day ÷ bid (max. 1 g/day); > 12 years of
age: 250-500 mg bid
• Clarithromycin 15 mg/kg/day ÷ bid (max 1g/day)
• Clindamycin 30 mg/kg/day po ÷ q6-8h (max. 2 g/day)
3. Acute Chest Syndrome (ACS) or Pneumonia
EMERGENCY DEPARTMENT MANAGEMENT
Patients present to ED in various degrees of distress
• Place patients identified with a fever of 38.3°C oral (37.8°C axilla) or higher or who are
in respiratory distress immediately into a room.
• Children without fever and breathing difficulties should be seen as soon as possible

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• History: ascertain breathing difficulties; fever; nature, duration and severity of pain;
medications already used; associated symptoms; previous successful experience with
analgesics; and previous episodes of ACS or pneumonia
• Physical: vital signs, with O2 saturation, cardiopulmonary and hydration status, spleen
size, neurologic exam, presence of jaundice and signs of infection
• Tests: request CBC, differential and reticulocyte count; blood culture, if the child is
febrile; and continuous O2 saturation monitoring, if he or she is in moderate to severe
respiratory distress. Request blood type and cross-matching (for possible exchange
transfusion), if in respiratory distress
• Commence IV fluids at maintenance flow rate
• Further labs: Request chest x-ray if the child has fever, chest pain, tachypnea or
respiratory symptoms
• Administer oxygen to maintain O2 saturation at >94% (this value is empirical; some use
90-94%)
• Place all patients admitted with ACS on cefotaxime 200 mg/kg/day IV ÷ q6-8h (max
8g/day), starting 24 hrs after the initial dose of Ceftriaxone (if Ceftriaxone was given).
Treat all patients with appropriate hydration, analgesics and antipyretics in addition to
other necessary investigation and treatment as per other protocols
INPATIENT MANAGEMENT
All patients with ACS should be hospitalized
• Patients to be admitted under Paediatric Haematology Service if available.
• Give antibiotics as indicated. See antibiotics, below, for more information
• Administer hydration, analgesics and antipyretics as necessary. Continue IV and PO
fluids at maintenance flow rates. Increase fluids as needed, If the child is dehydrated or
insensible losses are increased (e.g. persistent fever) excessive fluids, however may
precipitate or exacerbate ACS
• Diuretics: If signs (clinical or radiological) or fluid overload are present, administer IV
furosemide 0.5-1 mg/kg/dose (max 60 mg/dose)
• Bronchodilators: If the child has a history of reactive airway disease or wheezing,
consider bronchodilators e.g. salbutamol
• The role of corticosteroids in preventing clinical deterioration and reducing the need for
transfusions is unclear and requires further study
• For children older than 4 years, encourage deep breathing(10 deep breaths every 1-2
hours)
• Encourage ambulation and activity
• Request a CBC daily with reticulocyte counts twice a week) during the hospital stay; and
arterial/venous blood gases (ABG/VGB) daily, if not improving
Antibiotics
• Give Ceftriaxone (in Emergency Dept) then Cefotaxime ± Erythromycin (children >
5years with respiratory symptoms and children <5 years, if evidence of Mycoplasma) for
the first 72 hours
• Beyond 72 hrs, patients with mild pneumonia who are stable may be switched from
Cefotaxime to Cefuroxime

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− Patients who are seriously ill or unstable should receive Vancomycin plus Cefotaxime.
• Patients with a significant allergy to beta-lactam antibiotics may receive Clindamycin
• Doses:
− Cefotaxime 200 mg/kg/day IV ÷ q6-8h (max 8 g/day) start 24 hours after Ceftriaxone,
if given
− Cefuroxime 75 mg/kg/day IV ÷ q8h (max 4.5 g/day)
− Clindamycin 40 mg/kg/day IV ÷ q6-8h (max 2.7 g/day) or 30 mg/kg/day po qid (max 2
g/day)
− Erythromycin 40 mg/kg/day IV ÷ q6h (max 4g/day) or 40 mg/kg/day PO ÷ q6-12h as
erythromycin estolate (max 2g/day)
− Vancomycin (60 mg/kg/day IV ÷ q6h; max 4g/day)
−
Transfusion
• Patients with mild to moderate disease and Hemoglobin (Hb) at baseline do not generally
need a transfusion
• Patients with moderately severe disease and Hb 15 g/L less than baseline should be
transfused with packed RBCs, 10 mL/kg (simple transfusion). Patients should not be
transfused to a Hb of greater than 100 g/L (HCT >30%)
• Indications for exchange transfusion:
• Patients with severe disease,extensive infiltrates on chest x-ray; worsening ABGs;
increasing need for oxygen (>40% O2 ) and decreasing O2 saturation; need for ICU
admission.
Discharge Guidelines
• Does not require supplemental oxygen
• Afebrile for at least 24 hours
• Taking fluids and medications by mouth
• Pain control is adequate with PO medications
• Concurrent problems are resolve
4. APLASTIC CRISIS
Background
The condition is characterized by a rapid fall in hemoglobin (the retic count falls to <0.1%; Hb
falls often to 30-50 g/L) from a direct effect of parvovirus B19 or (rarely) other infectious
agents on erythroid progenitors in the marrow.
Management
1. In ED, conduct a history and physical exam. Note vital signs and any presence of postural
BP instability, a cardiac gallop, enlarged liver, or other signs of congestive heart failure.
Where possible, determine the patient's baseline Hb concentration.

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2. Test CBC, diff, retics count, blood type and cross-match, and parvovirus and Epstein-Barr
virus serology
3. Transfusion is required if the patient is symptomatic and has no signs of bone marrow
recovery (no nucleated RBCs in a peripheral blood smear, and/or retic count <1%).
Transfuse 10-15 mL of packed RBCs per kilogram of body weight; transfuse slowly, by
dividing packed RBCs into aliquots of 5-7 mL/kg and serially transfusing each over 4
hours. Furosemide (0.5 mg/ kg, midway through the transfusion) may be required. Watch
for fluid overload. Patients in cardiac failure may benefit from a partial (isovolumic) RBC
exchange for very low Hb levels; but because the time needed to organize this, it may be
best to start with a simple transfusion. Transfuse slowly, with a diuretic.
4. Start transfusion immediately if the patient is unstable (e.g., has postural BP instability,
enlarged liver, cardiac gallop). If the patient is stable, transfusion can be delayed a few
hours if necessary.
5. The child should be hospitalized for 24 h unless other reasons justify longer hospitalization.
Following transfusion and observation for 24 h, patients can be discharged with follow-up.
6. Hemoglobin and retic count should be followed every 2-3 days as an outpatient until retic
count rises and Hb increases, whether a transfusion was given or not.
7. Place inpatients in contact isolation. As parvovirus can cause miscarriage (hydrops fetalis),
patients should have no contact with pregnant hospital personnel.
8. Fever during an aplastic crisis is most likely due to parvovirus infection, but patients should
still have a blood culture and receive parenteral antibiotics according to the Fever protocol.
5. ACUTE SPLENIC SEQUESTRATION CRISIS
Clinical / Laboratory Features
A child with an acute splenic sequestration crisis presents with symptoms of
▪ Acute anaemia (pallor, tachycardia, frank cardiovascular collapse)
▪ Splenomegaly (pain in the left upper quadrant, thrombocytopenia); and
▪ Evidence of an active bone marrow response (increased reticulocytes)
▪ On physical examination, patients show signs of anaemia, hypovolemia, and an
enlarged spleen (larger than baseline), sometimes massively so. Mild cases may be
asymptomatic.
▪ Hemoglobin concentration is at least 20 g/L below the baseline steady state. In severe
cases, hemoglobin may decline to life-threatening levels. Retic counts are usually

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elevated, which distinguishes this condition from aplastic crisis. The platelet count
often declines to <50 X 109
/L.
▪ The mainstay of ASSC management is transfusion, to provide circulating erythrocytes
and volume. Risk of recurrence is approximately 40-50%, usually within 3-years.
Because it is not possible to predict which children will have recurrent attacks, most
experts recommend splenectomy after the first major attack (for patients >2-years old),
or chronic transfusion to maintain a hemoglobin S level under 30% (for patients <2
years), which may allow postponement of splenectomy.
Emergency Department Treatment
1. Conduct a history and physical exam with emphasis on signs and symptoms of
cardiovascular collapse (shock), anemia, and hypovolemia. If the spleen is palpable, verify
with the parent, and chart that it is larger than normal.
2. Tests: CBC, diff, retic count, blood type and cross-match, O2 saturation and ABG.
Establish IV access.
3. In mild cases (which are uncommon) of ASSC (i.e., spleen only slightly larger than usual,
Hb <15 g/L below baseline, patient haemodynamically stable), the child may be closely
followed as an outpatient. Reinforce with parents how to palpate their child's spleen and
indicate to them that if the spleen is enlarged or if the child's condition deteriorates in any
way that they are to return immediately to the Emergency Department. It would be
reasonable to admit all patients with ASSC if follow-up is in doubt (on or just before a
weekend, where there are questions of patient / family compliance, etc).
4. Patients with more severe ASSC (most patients) should be admitted to the General
Paediatric Ward or to APICU if unstable.
Inpatient Management
1. While the child is an inpatient, take vitals (q2-4h), careful repeated physical assessments
(q4-6h) for spleen size (measure with tape and record), and Hb measurements (q8-12h).
The patient's heart and respiratory rates and O2 saturation should be recorded on a monitor.
Patients should be on a cardiac or O2 monitor.
2. To raise the hemoglobin and maintain cardiovascular stability, transfuse the patient with
10-15 mL/kg of packed RBCs if there are any signs of cardiovascular collapse or if Hb
levels are less than 40-50 g/L (repeat if necessary. Time permitting, use available
phenotypically matched (sickle-negative) blood. Aim for a Hb of 80-90 g/L.
3. In hospital, continue regularly scheduled medications.
4. Administer oxygen to keep O2 saturation ≥ 94%.

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5. An acute sequestration episode usually resolves within 2-5 days. When there is evidence
of rising hemoglobin and diminishing spleen size, the patient can be discharged, with close
outpatient follow-up.
6. For weeks-months following and episode of ASSC, some patients have persistent
splenomegaly and hypersplenism, with lower-than-usual- Hb and platelet values.
7. Recurrent episodes requiring transfusion should be treated with splenectomy if the child is
over 2-years of age, or with chronic transfusion if 2-years old or less. Any patient who
presents to the hospital with a recurrent episode of ASSC should be seen by the Paediatric
Surgeon. The patient should receive vaccinations for encapsulated organisms
(pneumococcus, meningococcus and Haemophilus type B).
5. STROKE
Clinical Features
Ischaemic stroke typically presents with signs and symptoms of hemiparesis or hemi
anaesthesia, visual field deficits, aphasia, cranial nerve palsies, or acute change in behaviour.
Hemorrhagic strokes present with more generalized phenomena such as coma, headaches, and
seizures. Transient ischaemic attacks (TIA) are defined by neurological signs that resolve
within 24-28 hours; they often occur before an infarctive stroke, but may go unnoticed in young
children.
Laboratory Features
A computed tomographic scan (without contrast) requested in the emergency room may appear
normal, whereas a CT done 2-7 days post-CVA usually shows areas of infarction. MRI/MRA
is very sensitive in detecting intracranial hemorrhage or infarction (2-6 hours after the attack).
Emergency Department Treatment
1. If CVA or meningitis is suspected, place patient immediately into a room, and assess as
soon as possible.
2. Stabilize vital signs and life support as necessary.
3. Treat seizure and increased intracranial pressure, if present.
4. Conduct a history and physical exam concurrently with other measures. History: quality,
timing, severity, and duration of headaches, previous headaches; nausea or vomiting,
drooling; visual changes; paresis; loss of coordination; parasthesias; fever; syncope;

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seizures, recreational or prescribed drug use. Physical vital signs, detailed neurological
exam, hydration status, spleen size, presence of jaundice, signs of infection, etc.
5. Tests: CBC, diff, retic count, electrolytes, blood typing and cross-matching, Hb
electrophoresis (for % HbS), blood and urine cultures if patient is febrile, lumbar puncture
if meningitis is suspected. Blood for coagulation screen (PT, aPTT). Send extra "coag
tube" for rest of coagulation work-up; antithrombin III, protein C, protein S, plasminogen,
APC resistance, nonspecific inhibitors, anticardiolipin antibody, etc. Establish an IV at
maintenance flow rates.
6. IF the child is febrile, immediately administer cefotaxime (200 mg/kg/day; divided q6-8H,
max 8 g/day) or (ceftriaxone 80 mg/kg/dose; max 2 g/dose). Add IV vancomycin (60
mg/kg/day; divided q6h, max 4 g/day), if the child is septic or meningitis is likely. Patients
with significant allergy to beta-lactam antibodies can be treated with IV clindamycin (40
mg/kg/day, divided q6-8h, max 2.78 g/day) [For fever only]. For sepsis or meningitis in
children with beta-lactam allergy refer to Fever Protocol.
7. Administer oxygen to maintain O2 saturation.
8. See other critical pathways (protocols) for fever, VOC, etc.
9. Begin preparations for an RBC exchange transfusion to be done. (See Transfusion
protocol). The transfusion will take place in the APICU, but preparations will begin in ED.
Inpatient Management – Critical Care Unit
1. Admit to APICU.
2. Request MRI and MRA. If these tests are not immediately available, arrange for a CT
without contrast, to exclude intracranial hemorrhage.
3. If the patient is febrile, administer cefotaxime (200 mg/kg/day IV, divided q6-8h; max 8
g/day). If the patient has a significant allergy to beta-lactam antibiotics, give IV
clindamycin (40 mg/kg/day, divided q6-8h; max. 2,7 g/day). Clindamycin is not to be
used alone in the treatment of suspected meningitis, as it does not cross the blood-brain
barrier, if meningitis is suspected, add IV vancomycin (60 mg/kg/day, divided q6h; max.
4 g/d). In patients with significant allergy to beta-lactam antibiotics , treat meningitis
with vancomycin and rifampin (see Fever Protocol).
4. Continue IV fluids at maintenance flow rates.
5. For diagnosed CVA, perform an RBC exchange transfusion to a hemoglobin of 100g/L
if possible and HbS level of <30% of total Hb (see Transfusion protocol). A Hb of 100
g/L may not be possible in patients in whom base Hb exceeds this value. Remove the
central venous line as soon as possible after the blood exchange, to reduce the risk of
thrombosis.
6. Consult with Neurology for baseline assessment and follow-up.
7. Consult with physical and occupation therapists.

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8. When the patient is ready for discharge, organize clinic follow-up and the next
transfusion.
Discharge Criteria
1. The patient has been clinically and neurologically stable for at least 24 hours post
transfusion(s).
2. The child has been afebrile for at least 24 hours.
3. The child is taking fluids and medications orally.
4. Hematology and physical therapy follow-up has been organized.
5. A chronic transfusion program has been initiated.
SURGERY AND ANESTHESIA
• Patients with sickle cell disease who undergo surgery have an increased risk of peri-
operative complications and ambulatory surgery is usually not advised. Careful
preoperative preparation, including CBC and reticulocyte count, urinalysis and PT and
PTT, of the patient by a team consisting of a surgeon, hematologist and anesthesiologist
will minimize or eliminate these complications. Patients with organ damage and/or
coexistent disease must be identified because they are at increased risk for peri-
operative complications.
• Surgical procedures that have an increased probability of ischemia or hypoxia deserve
special attention. These include cardiothoracic surgery, techniques associated with
hypotension, hypothermia, hyperventilation, and vascular surgery. Laparoscopic
surgery appears to lower the postoperative complications of sickle cell disease and
should be used in appropriate settings.
• All patients should be evaluated by the anesthesiologist the day before surgery.
• Patients requiring general anesthesia should receive maintenance fluids at least 12 hours
before surgery
• With strict attention paid to urinary output and weight. Transfusion is necessary only
to raise the hemoglobin to 10-11gm/dl. It is not necessary to lower the percentage of S
hemoglobin a fixed amount by exchange transfusion. Patients with a history of
pulmonary or CNS disease, recurrent hospitalization, and/or those who have been
previously heavily transfused are at high risk for perioperative complications,
especially ASC and vaso-occlusive events. A physical examination and chart review
should be supplemented by the following tests:
✓ Arterial oxygen pressure or oxygen saturation measured by pulse oximetry
✓ Pulmonary function tests with bronchodilator response analysis regardless of a history
of acute chest syndrome, asthma, or other pulmonary complications
✓ Echocardiogram
✓ Renal and liver function
Preoperative assessment of the patient should include signs of vaso-occlusion, fever, infection
and dehydration. The laboratory and physical examination results should be reviewed to
identify abnormalities in the heart, liver, kidneys, brain and lungs. The use of incentive

16. 16
spirometry prior to surgery should be encouraged. Careful post operative monitoring and
management, including incentive spirometry, is essential to lessen the incidence of ACS, CVA
and acute pain episodes.
6. Priapism
*It occurs when sickle cells congest the corpora cavernosa and prevent the emptying of
blood from the penis.
*The acute presentation is a prolonged painful erection that persists beyond several
hours
*Chronic priapism consists of repetitive, reversible, painful erections called “stuttering”.
Management
At home
-Exercise –cycling, walking
-Urination
-Analgesia (paracetamol, mefenamic acid)
-Fluid intake
-Warm path
-If not resolves in 3-hours should report to hospital
At hospital
-Parentral hydration
- Analgesia
-Catheterization
-Blood transfusion
-Exchange transfusion- if no improvement to the above measures for 6-hours
-Surgical intervention
a. Aspiration and irrigation of the corpora with saline, pseudoephedrine or
adrenaline.(to be done by the urologist).
b. Cavernous-spongiosum shunts.

17. 17
TRANSFUSION THERAPY
Transfusion therapy is not without risks. The most common are iron overload,
alloimmunization and transmission of infections such as hepatitis or HIV. Since patients with
sickle cell disease may require multiple transfusions over the course of their lives, the risks
are multiplied, and except in acute emergencies, transfusions should only be given in
consultation with a hematologist. Transfusion is not indicated in chronic, steady-state anemia,
uncomplicated acute painful crisis, infection, uncomplicated pregnancy, or avascular
necrosis.
Initial characterization of a patient’s red blood cell antigen (phenotyping) is advisable at an
early age or when first red cell antibodies have developed. Select donor blood to closely
match the patient’s phenotype. Extended cross matching for minor antigens is desirable. The
patient’s transfusion history should be checked for previous evidence of alloimmunization, as
antibody
Titers may be undetectable months to years after the antibody challenge. Leukocyte-depleted
PRBCs, preferably prefiltered blood and blood product should be used. Fresh blood less than
5-7 days old must be used if available. If not available, use the freshest blood available. Use
sickle cell negative blood to facilitate proper monitoring of post transfusion level of
hemoglobin S through hemoglobin electrophoresis. Monitor outcome of transfusion therapy
by measuring hemoglobin/hematocrit.
Types of transfusion
• Simple transfusion: for severe anemia (<5g/dl); aplastic crisis, sequestration crisis,
though the decision depends on the patient’s clinical status, hemoglobin and hematocrit and
the reticulocyte count, or prior to surgery when general anesthesia is anticipated.
Volume of PRBC to be transfused should be adjusted to the pre-transfusion hemoglobin
level to avoid cardiac overload.
• Chronic transfusion:
➢ For stroke prevention, to achieve a S hemoglobin level < 30%;
➢ Chronic leg ulcers when local measures are unsuccessful; frequent priapism (for
prolonged cases lasting more than 6-12 hours, consider a single volume exchange).
➢ Exchange transfusion or partial exchange transfusion in specific cases, and, when
appropriate, for acute chest syndrome and cerebral vascular accident.
➢ Post transfusion (simple, chronic or exchange) hemoglobin level should never
exceed 11 grams.
➢ Periodic assessment of iron stores and prompt attention to any abnormalities noted are
imperative. Most of these patients will require chelation therapy.
➢ Formula for volume of red cells for transfusion-
➢ Whole blood donor unit average PCV= 35%
➢ Packed red cell unit average PCV=70%

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➢ Transfusion volume = (total blood volume x (PCV target –PCV pre
transfusion) / PCV of donor unit
➢ Example- for 30 kg child with pre-transfusion PCV 25%, goal PCV 32%, average
PRBC unit PCV 70%,
➢ {(75ml/kg x 30) (0.32- 0.25)} /0.70=225 ml (7.5 ml/kg)
❖ The Hb should never be raised acutely to > 10g/dl or haematocrit to > 0.35 since this
is likely to increase the blood viscosity.
❖ Frusemide is not given with transfusions in SCD because of the increase in viscosity
that may result.
❖ Do not use a sickle positive blood
❖ Packed red blood cells should be used except when blood volume expansion is
needed.
References:
1. Haberkern CM, Neumayr LD, et al. Cholecystectomy in sickle cell anemia patients:
2. perioperative outcome of 364 cases. National preoperative transfusion study group.
Blood.1997; 89(5): 1533-42.
3. Koshy M, Weiner SJ, et al. Surgery and anesthesia in sickle cell disease. Cooperative
study of sickle cell diseases. Blood. 1995; 86(10): 3676-84.
4. Vichinsky EP, Haberkern CM, et al. A comparison of conservative and aggressive
transfusion regimens in the perioperative management of sickle cell disease.
Preoperative transfusion in sickle cell disease study group. N Engl J Med. 1995; 333(4):
206-13.
5. Waldron P. Tonsillectomy, adeniodectomy, and myringotomy in sickle cell disease.
6. Preoperative transfusion in sickle cell disease study group. J Pediatr Hematol Oncol.
1999;21(2): 129-35.
Hydroxyurea Guidelines
Generic name: Hydroxycarbamide
Available preparations: 500 mg capsules

19. 19
Recommendations:
In all infants 9 months of age and older, children and adolescent with SCA, regardless
of clinical severity, Hydroxyurea is strongly recommended to reduce SCA related
complications (pain, dactylitis, ACS, anaemia). It is beneficial for those with HbS B-
thalassaemia or HbSC who have recurrent sickle cell-associated pain that interferes with daily
activities or quality of life.
The followings are recommended before starting Hydroxyurea:
o CBC, reticulocyte count.
o Quantitative HB electrophoresis for HbF level.
o RFT, LFT.
➢ Children: The starting dose of Hydroxyurea is 15 - 20 mg/kg/day.
➢ Adult: 500 mg.
➢ Cases of CKD: 5 – 10mg/kg/day.
➢ Increase by 5 mg/kg/day increments every 8 weeks.
➢ Give till mild myelosuppression is achieved, up to maximum of 35 mg/kg/day.
Monitoring of therapy:
• CBC monthly until reaching a stable dose then every 2 – 3 months.
• Aim for a target of absolutr neutrophil count (ANC) > 1.250/ul, platelets count >
80.000/ul.
• If either gets below this level, withhold hydroxyurea.
• Monitor CBC weekly until it returns to normal (usually in 1-2 weeks).
• When CBC returns to normal reintroduce Hydroxyurea by a dose 5 mg/kg lower
than the dose before the onset of cytopenia.
• Hydroxyurea is hepatotoxic and LFT should be done every 3 – 6 months, and to be
Withheld if ALT level increases by 100%.
➢ Both males and females of reproductive age should be counseled regarding the need
for contraception while taking hydroxyurea.
VACCINATION GUIGELINES (SCA)

20. 20
Children with sickle cell anaemia should be immunized against:
➢ Streptococcus pneumonae
➢ Haemophilus influenza type B
➢ Neisseria meningitides
➢ Hepatitis B virus, influenza
PNEUMOCOCCAL VACCINE GUIGELINE:
▪ Pneumococcal conjugate (Prevenar 13) is recommended for sicklers under age of 2
years. In Sudan, it’s given at 6 weeks and two other doses one month apart.
▪ Pneumococcal polysaccharide (Pneumovax 23): Recommended at 2 years of age.
▪ Sicklers who come late also should receive the vaccine.
Age Previous doses Recommendations
< 2 years Received PSV-13 according
to schedule.
Presenting late.
Single dose of the PPSV-23
after their second birthday
2 doses (separated by at least
1 month) of the PSV-13
before the age of 12 months,
3rd
dose at 12–13 months.*
12 months - 5 years Not been vaccinated OR not
completed the primary
course.
2 doses of PSV-13 separated
by an interval of 2 months.*
> 5 years Single dose of the PPSV-23.
* All sicklers < 5 years should receive a single dose of the PPSV-23 after their second
birthday and at least 2 months after the final dose of the PSV-13.
MENINGOCOCCAL VACCINE GUIGELINES:

21. 21
Actually, the recommended vaccines against meningococci are not available in Sudan; a
single dose of meningococcal conjugate A is given.
UPTODATE® recommends the following series of vaccination for cases of SCA.
Ages Recommended
formulation
Primary
series
Revaccination
9 - 23
months
Menactra® 2 doses,
given at ages
9 and 12
months
Children who remain at increased risk
should receive a booster dose 3 years
after the primary series
2 - 10
years
Menactra® or
Menveo®
2 doses,
2 months
apart
Every 5 years; if a 1-dose primary series
was administered, revaccinate at the
earliest opportunity, then every 5 years
11 - 18
years
Menactra® or
Menveo®
2 doses,
2 months
apart
Every 5 years; if a 1-dose primary series
was administered, revaccinate at the
earliest opportunity, then every 5 years.
At age 16 if primary series at age 11 or
12 years
Menveo®: meningococcal qudrivalents A C Y W135 oligosaccharide diphtheria CRM197
conjugate vaccine.
Menactra®: meningococcal qudrivalents A C Y W135 polysaccharide diphtheria toxoid
conjugate vaccine.
Hib VACCINE GUIGELINES:

22. 22
Age Previous doses Recommendations
12 - 59
months
No OR only 1 dose of Hib vaccine
before 12 months of age.
2 or more doses of Hib vaccine before
12 months of age.
2 additional doses of Hib vaccine 8
weeks apart.
1 additional dose.
❖ 1 dose of Hib vaccine should be administered to sicklers aged 5 years or older.