Dawaa Reference

Clinical reference

Sickle Cell Disease

Treatment options, dosing, cautions and Egyptian brands from the shipped Dawaa Reference card.

Evidence status

Checked against the sources named below

Sources7 sources

ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class BD65.01 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care · Sickle Cell Disease - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK482384/ · BSH Guideline on Management of Sickle Cell Disease 2018 · DROXIA (hydroxyurea) capsules US prescribing information, section 2.1 Dosage for Sickle Cell Anemia (DailyMed SetID a9494409-3571-4a81-9ad3-15ef7cb636a2) · Phenoxymethylpenicillin 250 mg Film-coated Tablets SmPC section 4.2 (eMC product 10628) · Sickle Cell Anemia - disease-level clinical article (sickle-cell-disease-referral-full.txt)

Verified against7 documents
  • No dose - referral pathway, no medicine given in primary care
  • Sickle Cell Disease - disease-level clinical article (sickle-cell-disease-clinical.txt)
  • BSH Guideline on Management of Sickle Cell Disease 2018
  • DROXIA (hydroxyurea) capsules US prescribing information, section 2.1 Dosage for Sickle Cell Anemia (DailyMed SetID a9494409-3571-4a81-9ad3-15ef7cb636a2)
  • Sickle Cell Anemia - disease-level clinical article (sickle-cell-disease-referral-full.txt)
  • Sickle Cell Disease Primary Care Management & Referral - disease-level clinical article (sickle-cell-disease-referral-clinical.txt)
  • Phenoxymethylpenicillin 250 mg Film-coated Tablets SmPC section 4.2 (eMC product 10628)

Verified date2026-08

Presentation reference

Is it this?

Reference only, to read alongside your own examination.

Symptoms — what the patient reports (12)

  • Crises do not begin at birth, because fetal haemoglobin shields the infant until it fades over roughly 6-9 months
  • Everyone with the disease has vaso-occlusive crises, the earliest being dactylitis from six months of age
  • Presentation is by complication, acute or chronic, and vaso-occlusive crisis is the commonest acute one
  • Any organ can be hit, though limbs and chest lead; pain that sounds atypical means hunting another cause
  • Acute chest syndrome opens suddenly with cough and breathlessness; fever may be absent, and when present suggests infection [breathlessness · cough]
  • Splenic sequestration: the spleen swells fast with left upper quadrant pain, commonest aged 1 to 4 years
  • Children show acute anaemia and hypovolaemic shock, while in adults the onset is more insidious [anaemia · shock]
  • Red flag: severe headache, altered mental state, slurred speech, fits or paralysis signal stroke [headache · paralysis · seizures · slurred speech]
  • Aplastic crisis, usually from parvovirus B-19: severe fatigue, anaemia, breathlessness, even fainting [anaemia · breathlessness · fatigue · syncope]
  • Acute intrahepatic cholestasis: sudden right upper quadrant pain, deepening jaundice, a big tender liver, pale stools [abdominal pain · jaundice · pale stools · right upper quadrant pain]
  • Priapism means a painful unwanted erection going beyond 4 hours
  • Leg ulcers sit on the inner and outer ankle, and once deep to bone can bring osteomyelitis

Signs — what you find (11)

  • Examine broadly to judge the need for oxygen, analgesia and transfusion
  • Red flag: a liver or spleen enlarging quickly points to a sequestration crisis
  • Orbital infarction shows proptosis, local pain, and swelling of lid or orbit [ischaemia · local tenderness · proptosis]
  • Eye movement is then reduced and visual acuity falls
  • Orbital compression syndrome brings ophthalmoplegia and sight loss from the orbital apex [loss of vision · ophthalmoplegia]
  • Hyphaema is blood in the anterior chamber after a blunt injury
  • Raised eye pressure is badly tolerated here and can end in retinal artery occlusion
  • Urine cannot be concentrated, so passing is frequent, dehydration likelier, and children may wet the bed [dehydration]
  • Papillary necrosis shows as blood in the urine, more so in HbSC disease [blood in the urine · necrosis]
  • Avascular necrosis of the femoral head is a common source of lasting pain and disability [necrosis]
  • Pulmonary hypertension surfaces as breathlessness on exertion or swollen legs [breathlessness · hypertension · leg swelling]

Tests (12)

  • Diagnosis is usually made in childhood, and newborn screening catches it at birth where such a programme runs
  • Other haemolytic conditions are excluded by history, examination, haemoglobin electrophoresis and the blood film
  • Creatinine rises late, so the first sign of kidney disease is microalbuminuria, 30 to 300 mg of albumin in a 24-hour collection; a spot urine-creatinine ratio is not a valid substitute here
  • Chest syndrome can begin during an admission for something else, so watch every inpatient for it
  • For chest syndrome send a blood count with differential, liver and kidney chemistry, blood and sputum cultures
  • A new infiltrate on the chest film is the defining feature of acute chest syndrome
  • Sequestration drops haemoglobin by more than 2gm/dL, with reticulocytosis and nucleated red cells
  • Hepatic sequestration is fast liver enlargement with haemoglobin down more than 2gm/dL; enzymes may stay normal
  • Transcranial Doppler and prevention cut childhood stroke; without them ~10% have overt stroke and 20 to 35% silent infarcts
  • A stroke needs urgent neurology input, a CT, and then MRI with MRA
  • In aplastic crisis the haemoglobin is very low and reticulocytes near absent
  • Cholestasis labs: very high bilirubin, raised alkaline phosphatase, deranged clotting; haemolysis markers may read normal

If not this — what else fits (12)

  • Haemoglobin S-beta-0 thalassaemia, which behaves just as HbSS does
  • Haemoglobin SC, milder, yet able to look like sickle cell anaemia
  • Haemoglobin S-beta+ thalassaemia, a milder variant again
  • Other haemoglobin variants can mimic it when co-inherited alongside HbS
  • Haemoglobin D-Punjab, O-Arab or E
  • Autoimmune haemolytic anaemia, warm or cold antibody
  • Alpha or beta thalassaemia
  • Paroxysmal nocturnal haemoglobinuria
  • Red cell membrane defects: hereditary spherocytosis or elliptocytosis
  • Enzyme defects: pyruvate kinase or glucose-6-phosphate deficiency
  • Drug-induced haemolysis, or a transfusion reaction, immediate or delayed
  • Microangiopathic haemolysis, or an infection such as malaria

SourceStatPearls "Sickle Cell Anemia" - disease-level clinical article

Presentation findings are traced to the source above.

Rx: Main treatment | Pneumococcal prophylaxis

MAIN TREATMENT - choose one, plus any add-on marked below

1

FOLIC ACID

1st line

Strength5 mg

Formoral.solid

Adult dose and duration

5 mg once daily - long-term baseline support

Paediatric dose

(Children <1 year: 2.5 mg once daily; Children >=1 year: 5 mg once daily)

Dose by age
Under 1 year:2.5 mg once daily
1 year and over:5 mg once daily
Dose source

BSH Guideline on Management of Sickle Cell Disease 2018

Why

Daily folic acid supports high erythroid turn-over in chronic haemolytic anaemia

Cautions
  • The preventive measures are penicillin V prophylaxis in children and pneumococcal/meningococcal vaccination.
  • URGENT HOSPITAL ADMISSION: Admit for acute chest syndrome, fever >38.5C, severe vaso-occlusive pain unresponsive to oral analgesia, or a sudden drop in Hb (aplastic/sequestration crisis).
  • This is SPECIALIST HAEMATOLOGY OVERSEEN CARE.
Egyptian brands
Egyptian brandManufacturerIndicative price
FOLIC ACID (EL NILE) 5 MG 50 TAB.EL NILE.26.25 EGP (0.53/unit)
FOLIC ACID (MEMPHIS) 5 MG 30 TAB.MEMPHIS15.75 EGP (0.53/unit)
FOLICINAD 5 MG 50 TABS.INAD PHARMA50.00 EGP (1.00/unit)
FOLIC ACID (EIPICO) 5 MG 20 TAB.EIPICO24.00 EGP (1.20/unit)
FOLIC ACID (EL NILE) 5 MG 30 TAB.EL NILE.36.00 EGP (1.20/unit)
FOLIRAQ 5000 MCG 30 CAPSULESHP PHARMA45.00 EGP (1.50/unit)
FOLIDDOX 400 MCG / 5 ML LIQUID 150 ML? strength differs? different route - not oral solidMADDOX PHARMA150.00 EGP
2

REFERRAL & SAFETY-NETTING (NO DRUG THERAPY)

1st line
Adult dose and duration

Inherited haemoglobin disorder causing vaso-occlusive pain crises and chronic haemolysis. GP supports with folic acid, infection-prevention advice and initial analgesia, but hydroxyurea initiation, transfusion decisions and crisis admission are haematologist-led.

Dose source

Sickle Cell Anemia - disease-level clinical article (sickle-cell-disease-referral-full.txt)

Why

Carries the referral criteria and warning signs for this condition, which apply whichever treatment is chosen.

Cautions
  • Refer a pain crisis that is not settling on simple analgesia - the ceiling of what primary care can do for a crisis is reached quickly.
  • Sudden worsening pallor is an emergency in sickle cell disease and not a gradual anaemia to investigate at leisure.
  • RED FLAG - Fever in an SCA patient requires prompt empiric antibiotics and urgent evaluation for sepsis; ill-appearing patients need admission and IV antibiotics.
  • RED FLAG - Acute chest syndrome (sudden cough/dyspnea +/- fever) is a medical emergency requiring hospitalization regardless of SCA phenotype.
  • RED FLAG - Stroke signs (severe headache, altered mental status, slurred speech, seizures, paralysis) require urgent neurological consultation and imaging.
  • RED FLAG - Priapism lasting more than 4 hours needs early urologic involvement; delayed management can cause permanent impotence.
  • RED FLAG - Splenic sequestration (rapid spleen enlargement with left-upper-quadrant pain, Hb drop >2g/dL) is a significant cause of acute, potentially life-threatening anemia, especially in young children.
  • RED FLAG - Splenic sequestration crisis resulting in hypovolemic shock requires urgent aggressive management.
3

HYDROXYUREA

add-on - not a substitute

Strength500 mg

Formoral.solid

Adult dose and duration

15 mg/kg once daily, titrate by 5 mg/kg every 12 weeks to max 35 mg/kg daily under specialist supervision - long-term under haematologist care

Paediatric dose

20 mg/kg/day [child max 1500 mg]

(20 mg/kg once daily under pediatric haematologist supervision; requires monthly FBC)

Dose by weight
3kg60 mg/day
4kg80 mg/day
5kg100 mg/day
6kg120 mg/day
7kg140 mg/day
8kg160 mg/day
9kg180 mg/day
10kg200 mg/day
11kg220 mg/day
12kg240 mg/day
13kg260 mg/day
14kg280 mg/day
15kg300 mg/day
16kg320 mg/day
17kg340 mg/day
18kg360 mg/day
19kg380 mg/day
20kg400 mg/day
21kg420 mg/day
22kg440 mg/day
23kg460 mg/day
24kg480 mg/day
25kg500 mg/day
26kg520 mg/day
27kg540 mg/day
28kg560 mg/day
29kg580 mg/day
30kg600 mg/day
31kg620 mg/day
32kg640 mg/day
33kg660 mg/day
34kg680 mg/day
35kg700 mg/day
36kg720 mg/day
37kg740 mg/day
38kg760 mg/day
39kg780 mg/day
40kg800 mg/day
41kg820 mg/day
42kg840 mg/day
43kg860 mg/day
44kg880 mg/day
45kg900 mg/day
46kg920 mg/day
47kg940 mg/day
48kg960 mg/day
49kg980 mg/day
50kg1000 mg/day
Dose source

DROXIA (hydroxyurea) capsules US prescribing information, section 2.1 Dosage for Sickle Cell Anemia (DailyMed SetID a9494409-3571-4a81-9ad3-15ef7cb636a2)

Why

Disease-modifying therapy: increases HbF levels, reduces vaso-occlusive pain crises and acute chest syndrome

Cautions
  • Causes bone marrow suppression: monitor FBC every 4 weeks.
  • Teratogenic: contraception required during treatment and for 6 months after stopping.
  • Hydroxyurea is SPECIALIST HAEMATOLOGIST PRESCRIBED ONLY.
Egyptian brands
Egyptian brandManufacturerIndicative price
CYTODROX 500 MG 100 CAPS.CIPLA LTD. - INDIA > STAR INTERNATIONAL COMPANY588.00 EGP (5.88/unit)
HYDREA 500 MG 20 CAPS.BRISTOL-MYERS SQUIBB > ONCO PHARM230.00 EGP (11.50/unit)
HYDROXYUREA-MEDAC 500 MG 100 CAPS. SSMEDAC > EGYPTIAN PHARMACEUTICAL TRADING COMPANY2250.00 EGP (22.50/unit)

PNEUMOCOCCAL PROPHYLAXIS - give alongside

4

PHENOXYMETHYL PENICILLIN

Pneumococcal prophylaxis

add-on - not a substitute

Strength250 mg

Formoral.solid

Adult dose and duration

500 mg every 12 hours - Long-term - the disease article describes it as lifelong

Paediatric dose

The label's dosing table for pneumococcal prophylaxis (section 4.2) - which covers asplenia and sickle cell disease - gives adults and children over 12 years 500 mg every 12 hours, children 6 to 12 years 250 mg every 12 hours, and children under 5 years 125 mg every 12 hours. It leaves no separate band for age 5 to 6, jumping straight from under-5 to 6-12, and it adds that tablets are not usually given under 5 years or to a child who cannot swallow them - use the oral suspension there instead.

Dose source

Phenoxymethylpenicillin 250 mg Film-coated Tablets SmPC section 4.2 (eMC product 10628)

Why

A5 chunk 5 refused this because MSF states the indication and attaches no prophylactic dose - its dose table is for streptococcal pharyngitis, scarlet fever and diphtheria, and borrowing that would have been inventing a regimen. The dose was hunted instead. The Egyptian formulary's phenoxymethylpenicillin monograph has no sickle-cell entry either, so a UK label was fetched and cached: the Phenoxymethylpenicillin 250 mg Film-coated Tablets SmPC carries "Prophylaxis of pneumococcal infection (e.g. in asplenia and in patients with sickle cell disease)." in section 4.1 and the prophylactic regimen in section 4.2. The indication is also in this card's own article - "Besides lifelong administration of penicillin V, vaccinations against meningococcus, pneumococcus, and hemophilus influenza B are available to prevent infection with encapsulated organisms."

Cautions
  • CONTRAINDICATED, per section 4.3 of the label: phenoxymethylpenicillin is not for the patient known to be hypersensitive to penicillin or to any excipient in the product, and it is used cautiously in anyone with a history of allergy. Attention should be paid to possible cross-sensitivity with other beta-lactams such as cephalosporins.
  • This is prophylaxis, not treatment. The label adds that severe acute infections should not be treated with phenoxymethylpenicillin - a febrile sickle-cell patient needs assessment and parenteral cover, not this tablet.
  • MSF names the indication and gives no dose for it - it lists phenoxymethylpenicillin as one given to children to head off pneumococcal infection in sickle cell disease, and to stop acute rheumatic fever returning. The dose here is the UK label's, not MSF's, and not the pharyngitis dose.
  • Egyptian products are sold in international units - OSPEN 1.0 MIU and 1.5 MIU tablets. The label's own conversion puts phenoxymethylpenicillin 250 mg at roughly 400,000 units, so 500 mg is about 800,000 units.
  • Take at least 30 minutes before or 2 hours after food; food reduces absorption.
  • Prophylaxis does not replace pneumococcal, meningococcal and Hib vaccination - the article names them in the same sentence.
Egyptian brands
Egyptian brandManufacturerIndicative price
OSPEN 1.0 MIU 12 F.C.TABS.? strength differsSANDOZ24.00 EGP (2.00/unit)
OSPEN 1.5 MIU 12 F.C.TABS.? strength differsSANDOZ41.00 EGP (3.42/unit)

Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy.