BNF for Children 2011-2012

452 9.1.3 Drugs used in hypoplastic, haemolytic, renal anaemias BNFC 2011–20129 Nutrition and bloodmonthly; risk of gastro-intestinal ulceration andhaemorrhage; platelet count less than 50 10 9 /litre;consider treatment interruption if unexplained cytopeniaoccurs; not recommended in conditions whichmay reduce life expectancy (e.g. high-risk myelodysplasticsyndromes); history of liver cirrhosis; test liverfunction before treatment, then every 2 weeks duringthe first month, and then monthly; measure baselineserum-creatinine and monitor renal function weeklyduring the first month of treatment and monthlythereafter; test for proteinuria monthly; interactions:Appendix 1 (deferasirox)Hepatic impairment manufacturer advises caution—no information available; avoid in severe impairmentRenal impairment reduce dose by 10 mg/kg if serumcreatinineincreased above age-appropriate limits orestimated glomerular filtration rate less than 90 mL/minute/1.73 m 2 on 2 consecutive occasions—interrupttreatment if deterioration in renal function persistsafter dose reduction; avoid if estimated glomerularfiltration rate less than 60 mL/minute/1.73 m 2Pregnancy manufacturer advises avoid unless essential—toxicityin animal studiesBreast-feeding manufacturer advises avoid—presentin milk in animal studiesSide-effects gastro-intestinal disturbances (includingulceration and fatal haemorrhage); headache; proteinuria;pruritus, rash; less commonly oedema, hepatitis,cholelithiasis, fatigue, anxiety, sleep disorder, dizziness,pyrexia, pharyngitis, glucosuria, renal tubulopathy,disturbances of hearing and vision (includinglens opacity and maculopathy), and skin pigmentation;hepatic failure, acute renal failure, blood disorders(including agranulocytosis, neutropenia, pancytopenia,and thrombocytopenia), hypersensitivityreactions (including anaphylaxis and angioedema),and alopecia also reportedLicensed use see notes aboveIndication and doseChronic iron overload. By mouthChild 2–18 years initially 10–30 mg/kg once dailyaccording to serum-ferritin concentration andamount of transfused blood (consult product literature);maintenance, adjust dose every 3–6months in steps of 5–10 mg/kg according toserum-ferritin concentration; usual max. 30 mg/kgdaily, but may be increased to max. 40 mg/kg dailyand reduced in steps of 5–10 mg/kg once controlachievedNote Dose should be rounded to nearest whole tabletsizeExjade c (Novartis) TADispersible tablets, deferasirox 125 mg, net price 28-tab pack = £117.60; 250 mg, 28-tab pack = £235.20;500 mg, 28-tab pack = £470.40. Label: 13, 22, counselling,administrationCounselling Tablets should be dispersed in water, orangejuice, or apple juice; if necessary, resuspend residue andswallowDEFERIPRONECautions monitor neutrophil count weekly and discontinuetreatment if neutropenia developsBlood disorders Patients or their carers should be told howto recognise signs of neutropenia and advised to seekimmediate medical attention if symptoms such as fever orsore throat developContra-indications history of agranulocytosis orrecurrent neutropeniaHepatic impairment manufacturer advises monitorliver function—interrupt treatment if persistent elevationin serum alanine aminotransferaseRenal impairment manufacturer advises caution—noinformation availablePregnancy manufacturer advises avoid beforeintended conception and during pregnancy—teratogenicand embryotoxic in animal studies; contraceptionadvised in girls of child-bearing potentialBreast-feeding manufacturer advises avoid—noinformation availableSide-effects gastro-intestinal disturbances (reducingdose and increasing gradually may improve tolerance),increased appetite; headache; red-brown urinediscoloration; neutropenia, agranulocytosis; zincdeficiency; arthropathyLicensed use see notes aboveIndication and doseIron overload in thalassaemia major. By mouthChild 6–18 years 25 mg/kg 3 times daily (max.100 mg/kg daily)Ferriprox c (Swedish Orphan) ATablets, f/c, scored, deferiprone 500 mg, net price100-tab pack = £152.39. Label: 14, counselling, blooddisordersOral solution, red, deferiprone 100 mg/mL, net price500 mL = £152.39. Label: 14, counselling, blood disordersDESFERRIOXAMINE MESILATE(Deferoxamine Mesilate)Cautions eye and ear examinations before treatmentand at 3-month intervals during treatment; monitorbody-weight and height in children at 3-month intervals—riskof growth restriction with excessive doses;aluminium-related encephalopathy (may exacerbateneurological dysfunction); interactions: Appendix 1(desferrioxamine)Renal impairment use with cautionPregnancy teratogenic in animal studies, manufactureradvises use only if potential benefit outweighsriskBreast-feeding manufacturer advises use only ifpotential benefit outweighs risk—no informationavailableSide-effects nausea, vomiting, abdominal pain,headache, pyrexia, growth retardation and bone disorders(see Cautions), arthralgia, myalgia, hearingdisturbances, injection-site reactions; rarely diarrhoea,hepatic impairment, hypotension (especiallywhen given too rapidly by intravenous injection),anaphylaxis, Yersinia and mucormycosis infections,blood dyscrasias (including thrombocytopenia andleucopenia), leg cramps, bone pain, visual disturbances(including lens opacity and retinopathy), rash;very rarely acute respiratory distress, neurologicaldisturbances (including dizziness, neuropathy, con-