{ "schema_version": 1, "kind": "condition", "id": "sickle-cell-disease", "name": "Sickle Cell Disease", "category": "chronic", "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", "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)", "verified_date": "2026-08", "treatments": [ { "id": 1637, "generic": "Folic acid", "line": 1, "is_adjunct": false, "form": "oral.solid", "strength_mg": 5.0, "adult_dose": "5 mg once daily", "adult_duration": "long-term baseline support", "dose_source": "BSH Guideline on Management of Sickle Cell Disease 2018", "rationale": "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." ], "peds_mgkg_low": null, "peds_mgkg_high": null, "peds_max_mg": null, "peds_basis": null, "peds_unit": null, "peds_note": "Children <1 year: 2.5 mg once daily; Children >=1 year: 5 mg once daily", "peds_min_weight_kg": null, "peds_max_weight_kg": null, "peds_age_min_months": null, "peds_age_max_months": null, "peds_age_bands": [ { "label": "Under 1 year", "age_min_months": 0, "age_max_months": 11, "dose_text": "2.5 mg once daily" }, { "label": "1 year and over", "age_min_months": 12, "age_max_months": 216, "dose_text": "5 mg once daily" } ], "peds_doses": null, "brands": [ { "trade_name": "FOLIC ACID (EL NILE) 5 MG 50 TAB.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "EL NILE.", "price_egp": 26.25, "pack_count": 50, "unit_price": 0.53, "strength_mg": 5.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "FOLIC ACID (MEMPHIS) 5 MG 30 TAB.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "MEMPHIS", "price_egp": 15.75, "pack_count": 30, "unit_price": 0.53, "strength_mg": 5.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "FOLICINAD 5 MG 50 TABS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "INAD PHARMA", "price_egp": 50.0, "pack_count": 50, "unit_price": 1.0, "strength_mg": 5.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "FOLIC ACID (EIPICO) 5 MG 20 TAB.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "EIPICO", "price_egp": 24.0, "pack_count": 20, "unit_price": 1.2, "strength_mg": 5.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "FOLIC ACID (EL NILE) 5 MG 30 TAB.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "EL NILE.", "price_egp": 36.0, "pack_count": 30, "unit_price": 1.2, "strength_mg": 5.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "FOLIRAQ 5000 MCG 30 CAPSULES", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "HP PHARMA", "price_egp": 45.0, "pack_count": 30, "unit_price": 1.5, "strength_mg": 5.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "FOLIDDOX 400 MCG / 5 ML LIQUID 150 ML", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "MADDOX PHARMA", "price_egp": 150.0, "pack_count": null, "unit_price": null, "strength_mg": 0.4, "exact_strength": false, "exact_form": false, "discontinued": false, "also_contains": null } ], "condition_id": "sickle-cell-disease" }, { "id": 1639, "generic": "Referral & safety-netting (no drug therapy)", "line": 1, "is_adjunct": false, "form": null, "strength_mg": null, "adult_dose": "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.", "adult_duration": null, "dose_source": "Sickle Cell Anemia - disease-level clinical article (sickle-cell-disease-referral-full.txt)", "rationale": "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." ], "peds_mgkg_low": null, "peds_mgkg_high": null, "peds_max_mg": null, "peds_basis": null, "peds_unit": null, "peds_note": null, "peds_min_weight_kg": null, "peds_max_weight_kg": null, "peds_age_min_months": null, "peds_age_max_months": null, "peds_age_bands": null, "peds_doses": null, "brands": [], "condition_id": "sickle-cell-disease" }, { "id": 1638, "generic": "Hydroxyurea", "line": 1, "is_adjunct": true, "form": "oral.solid", "strength_mg": 500.0, "adult_dose": "15 mg/kg once daily, titrate by 5 mg/kg every 12 weeks to max 35 mg/kg daily under specialist supervision", "adult_duration": "long-term under haematologist care", "dose_source": "DROXIA (hydroxyurea) capsules US prescribing information, section 2.1 Dosage for Sickle Cell Anemia (DailyMed SetID a9494409-3571-4a81-9ad3-15ef7cb636a2)", "rationale": "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." ], "peds_mgkg_low": 20.0, "peds_mgkg_high": 20.0, "peds_max_mg": 1500.0, "peds_basis": "day", "peds_unit": "mg", "peds_note": "20 mg/kg once daily under pediatric haematologist supervision; requires monthly FBC", "peds_min_weight_kg": null, "peds_max_weight_kg": null, "peds_age_min_months": null, "peds_age_max_months": null, "peds_age_bands": null, "peds_doses": [ { "weight_kg": 10, "low_mg": 200, "high_mg": 200, "capped": false }, { "weight_kg": 20, "low_mg": 400, "high_mg": 400, "capped": false }, { "weight_kg": 30, "low_mg": 600, "high_mg": 600, "capped": false }, { "weight_kg": 40, "low_mg": 800, "high_mg": 800, "capped": false } ], "brands": [ { "trade_name": "CYTODROX 500 MG 100 CAPS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "CIPLA LTD. - INDIA > STAR INTERNATIONAL COMPANY", "price_egp": 588.0, "pack_count": 100, "unit_price": 5.88, "strength_mg": 500.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "HYDREA 500 MG 20 CAPS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "BRISTOL-MYERS SQUIBB > ONCO PHARM", "price_egp": 230.0, "pack_count": 20, "unit_price": 11.5, "strength_mg": 500.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "HYDROXYUREA-MEDAC 500 MG 100 CAPS. SS", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "MEDAC > EGYPTIAN PHARMACEUTICAL TRADING COMPANY", "price_egp": 2250.0, "pack_count": 100, "unit_price": 22.5, "strength_mg": 500.0, "exact_strength": true, "exact_form": true, "discontinued": false, "also_contains": null } ], "condition_id": "sickle-cell-disease" }, { "id": 1640, "generic": "Phenoxymethyl penicillin", "line": 1, "is_adjunct": true, "form": "oral.solid", "strength_mg": 250.0, "adult_dose": "500 mg every 12 hours", "adult_duration": "Long-term - the disease article describes it as lifelong", "dose_source": "Phenoxymethylpenicillin 250 mg Film-coated Tablets SmPC section 4.2 (eMC product 10628)", "rationale": "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." ], "peds_mgkg_low": null, "peds_mgkg_high": null, "peds_max_mg": null, "peds_basis": null, "peds_unit": null, "peds_note": "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.", "peds_min_weight_kg": null, "peds_max_weight_kg": null, "peds_age_min_months": null, "peds_age_max_months": null, "peds_age_bands": null, "peds_doses": null, "brands": [ { "trade_name": "OSPEN 1.0 MIU 12 F.C.TABS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "SANDOZ", "price_egp": 24.0, "pack_count": 12, "unit_price": 2.0, "strength_mg": null, "exact_strength": false, "exact_form": true, "discontinued": false, "also_contains": null }, { "trade_name": "OSPEN 1.5 MIU 12 F.C.TABS.", "scientific_name": null, "normalized_ingredient": null, "manufacturer": "SANDOZ", "price_egp": 41.0, "pack_count": 12, "unit_price": 3.42, "strength_mg": null, "exact_strength": false, "exact_form": true, "discontinued": false, "also_contains": null } ], "condition_id": "sickle-cell-disease" } ] }