Applied Evidence

Essential strategies and tactics for managing sickle cell disease

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References

In addition, keep in mind that preventive recommendations made by the US Preventive Services Task Force (Exhibit 5 in the Expert Panel Report)6 apply to all patients with SCD and SCT.

Prevention of invasive pneumococcal disease

All patients with SCD are assumed to have lifelong splenic dysfunction that begins in childhood. This is particularly true for those with SCA. In the absence of vaccination, the lifetime incidence of pneumococcal bacteremia resulting in serious complications is as high as 16% in SCD.10 In multiple randomized clinical trials, prophylactic penicillin dosing has proved beneficial in these patients, demonstrating a decrease in the risk of (1) pneumococcal infection and (2) early death during the study period, with minimal adverse effects.5

Prophylactic penicillin dosing should be initiated during infancy in patients with SCA. From ages 3 months to 3 years, the dosage of penicillin V is 125 mg twice daily; from 3 to 5 years, 250 mg twice daily. After age 5 years, the decision to continue penicillin is individualized, with consideration of prior severe pneumococcal infection and general preventive health maintenance. Penicillin-allergic patients can be given erythromycin. All patients with SCD who have had surgical splenectomy should be placed on antibiotic prophylaxis (ie, penicillin as dosed above).5

The polyvalent pneumococcal vaccine has resulted in significant protection against invasive pneumococcal disease; mortality from pneumococcal disease among patients with SCD who are younger than 14 years has decreased dramatically since the vaccine was introduced.6 For all patients with SCD, the standard PCV13 series should be administered beginning at age 6 weeks. A 2-dose series of the PPSV23 vaccine, which includes more Streptococcus pneumoniae serotypes than the PCV13 vaccine, should be administered beginning at age 2 years or 8 weeks after completion of the PCV13 series, whichever comes first.

Prevention of flu, COVID-19, and other vaccine-preventable illness

Influenza. Beginning at age 6 months, all patients with SCD should receive inactivated influenza vaccine annually at the beginning of the influenza season. Avoid using the live attenuated vaccine (Flumist) because of an associated increased risk of severe or complicated infection.11

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