Essential strategies and tactics for managing sickle cell disease
Key to patients’ well-being is the family physician’s watchfulness—through periodic lab testing and health checks and diligent application of preventive measures.
PRACTICE RECOMMENDATIONS
› Offer rapid access to narcotic analgesia for patients with sickle cell disease (SCD) who have recurrent vaso-occlusive crises, to prevent unnecessary hospitalization. A
› Provide oral penicillin prophylaxis against pneumococcal disease in patients < 5 years of age who have sickle cell anemia (SCA), but not in children whose SCD is less severe. A
› Screen all patients with SCA annually, beginning at age 2 years until age 16 years, for their risk of stroke, using a transcranial Doppler study. A
› Administer the COVID-19 mRNA vaccine series to all patients with SCD, unless contraindicated. A
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
Testing should also include a pregnancy test for postmenarchal females because hydroxyurea is in US Food and Drug Administration pregnancy risk category X.
Avoid hydroxyurea in lactating women; dose the drug renally in the setting of renal disease. Because hydroxyurea has a high rate of serious adverse effects and drug-drug interactions, it should be offered in conjunction with an individualized care plan.
Hydroxyurea can also be offered to patients with other forms of SCD who have recurrent vaso-occlusive symptoms.
Two newer medications improve oxygen delivery in patients with SCD. Voxelotor, approved in 2019, works to reduce Hb S polymerization by binding to the alpha chain of Hb S and, subsequently, increasing its oxygen affinity. The drug is generally well tolerated and can be used in patients with SCD who are ≥ 12 years.16 Crizanlizumab is a monoclonal antibody directed against P-selectin, an adhesion molecule located on endothelial cells and activated platelets. The efficacy of crizanlizumab was demonstrated in the SUSTAIN trial, in which it reduced vaso-occlusive pain in patients ≥ 16 years.17
All of these medications have a narrow toxic–therapeutic window. They should therefore be administered with the participation of a multidisciplinary care team.
Continue to: The need to coordinated, comprehensive care
