Many interventions including pneumococcal immunization, folic acid and zinc supplementation, penicillin prophylaxis and early identification of complications and their treatment, have now ensured that many children globally, with SCD who would hitherto have died during infancy, live to adulthood. However, in Africa with more than 75% of disease burden globally, and 1,000 children born with the disease daily, less than 10 per cent of children born with SCD live to adulthood.
Now, whereas penicillin prophylaxis prevents certain deadly infections and folate supplementation support the body with a vital ingredient to replace fragile blood cells lost to SCD effects, none of them actually is able to modify the disease to reduce the frequency of hospitalizations due to illness, transfusions, intensity and frequency of pain and acute chest syndrome (SCD starving the lungs of blood and ‘gas’ leading to sudden death in many instances) crises. The only medicine that has been found to do this and approved by the Food and Drugs Authority, FDA-USA, is HYDROXYUREA, HU.
• HU is safe for children from the age of 9 months
• Offer HU to all kids with SCD SS from the age of 9 months
• HU does not increase the risk of infections among children with SCD-SS
• Always regularly (every 2 to 3 months at least) monitor the blood counts of all patients on HU even if they are doing very well
• HU is very effective when administered once every 24 hours (by the clock, at the same time daily)
• Ensure continuity of care; be guided by a protocol
• In the event of an acute event or illness, you do not need to stop HU
• The KATH Pharmacy’s Manufacturing Unit prepares a stable suspension for kids who cannot take capsules
• For growing children always adjust their doses for their weights; hence the need for regularly scheduled visits
• For maximum benefit, dose escalate to maximum tolerated dose with monitoring and/or till haematological toxicity (as per blood counts)
• Patients on HU must continue their penicillin prophylaxis and folate (and zinc) supplementation
Dear Practitioner,
Today, inform a parent about HU and how s/he can get help for their kids or relatives with SCD (SS genotype) and do it right: by the protocol.
Story BY: Dr Lawrence Osei-Tutu
