# Sickle Cell Disease - Category: chronic - Review status: reviewed (every claim checked against a document named on this page) - 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 date: 2026-08 ## Verified against - 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) ## Treatment metadata - Folic acid — 5 mg — oral.solid - Referral & safety-netting (no drug therapy) - Hydroxyurea — 500 mg — oral.solid - Phenoxymethyl penicillin — 250 mg — oral.solid ## Complete treatment card ```text SICKLE CELL DISEASE 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) Review status: REVIEWED against 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) (2026-08) 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 Source StatPearls "Sickle Cell Anemia" - disease-level clinical article Status 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] Adult 5 mg once daily - long-term baseline support Peds Under 1 year: 2.5 mg once daily 1 year and over: 5 mg once daily (Children <1 year: 2.5 mg once daily; Children >=1 year: 5 mg once daily) Source BSH Guideline on Management of Sickle Cell Disease 2018 Why Daily folic acid supports high erythroid turn-over in chronic haemolytic anaemia Caution 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. Egypt FOLIC ACID (EL NILE) 5 MG 50 TAB. EL NILE. 26.25 EGP (0.53/unit) FOLIC ACID (MEMPHIS) 5 MG 30 TAB. MEMPHIS 15.75 EGP (0.53/unit) FOLICINAD 5 MG 50 TABS. INAD PHARMA 50.00 EGP (1.00/unit) FOLIC ACID (EIPICO) 5 MG 20 TAB. EIPICO 24.00 EGP (1.20/unit) FOLIC ACID (EL NILE) 5 MG 30 TAB. EL NILE. 36.00 EGP (1.20/unit) FOLIRAQ 5000 MCG 30 CAPSULES HP PHARMA 45.00 EGP (1.50/unit) FOLIDDOX 400 MCG / 5 ML LIQUID 150 ML MADDOX PHARMA 150.00 EGP -> ? strength differs, ? different route - not oral solid 2. REFERRAL & SAFETY-NETTING (NO DRUG THERAPY) [1st line] Adult 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. 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. Caution 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] Adult 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 Peds 20 mg/kg/day [child max 1500 mg] (20 mg/kg once daily under pediatric haematologist supervision; requires monthly FBC) 3kg -> 60 mg/day 4kg -> 80 mg/day 5kg -> 100 mg/day 6kg -> 120 mg/day 7kg -> 140 mg/day 8kg -> 160 mg/day 9kg -> 180 mg/day 10kg -> 200 mg/day 11kg -> 220 mg/day 12kg -> 240 mg/day 13kg -> 260 mg/day 14kg -> 280 mg/day 15kg -> 300 mg/day 16kg -> 320 mg/day 17kg -> 340 mg/day 18kg -> 360 mg/day 19kg -> 380 mg/day 20kg -> 400 mg/day 21kg -> 420 mg/day 22kg -> 440 mg/day 23kg -> 460 mg/day 24kg -> 480 mg/day 25kg -> 500 mg/day 26kg -> 520 mg/day 27kg -> 540 mg/day 28kg -> 560 mg/day 29kg -> 580 mg/day 30kg -> 600 mg/day 31kg -> 620 mg/day 32kg -> 640 mg/day 33kg -> 660 mg/day 34kg -> 680 mg/day 35kg -> 700 mg/day 36kg -> 720 mg/day 37kg -> 740 mg/day 38kg -> 760 mg/day 39kg -> 780 mg/day 40kg -> 800 mg/day 41kg -> 820 mg/day 42kg -> 840 mg/day 43kg -> 860 mg/day 44kg -> 880 mg/day 45kg -> 900 mg/day 46kg -> 920 mg/day 47kg -> 940 mg/day 48kg -> 960 mg/day 49kg -> 980 mg/day 50kg -> 1000 mg/day 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 Caution 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. Egypt CYTODROX 500 MG 100 CAPS. CIPLA LTD. - ... 588.00 EGP (5.88/unit) HYDREA 500 MG 20 CAPS. BRISTOL-MYERS... 230.00 EGP (11.50/unit) HYDROXYUREA-MEDAC 500 MG 100 CAPS. SS MEDAC > EGYPTIAN PHAR... 2250.00 EGP (22.50/unit) PNEUMOCOCCAL PROPHYLAXIS - give alongside 4. PHENOXYMETHYL PENICILLIN [add-on - not a substitute] Adult 500 mg every 12 hours - Long-term - the disease article describes it as lifelong Peds 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. 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." Caution 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. Egypt OSPEN 1.0 MIU 12 F.C.TABS. SANDOZ 24.00 EGP (2.00/unit) -> ? strength differs OSPEN 1.5 MIU 12 F.C.TABS. SANDOZ 41.00 EGP (3.42/unit) -> ? strength differs Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy. ``` --- Dawaa Reference is a reference for prescribers, not a medical device, and does not replace clinical judgement. [Privacy policy](/privacy)