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
Simple transfusion can be used in an acute setting to restore and maintain Hb at a safe level. Iron overload and formation of RBC alloantigen are associated with multiple transfusions; once either of these conditions is established, subsequent transfusion therapy can be harmful. Care must be taken to prescribe transfusion appropriately; leukocyte-depleted RBCs should be used when available.
It is important to define specific goals of transfusion to optimize its use. Patients who have received multiple transfusions should have enhanced monitoring for bloodborne infection, such as hepatitis C virus. Acute aplastic crises are caused by parvovirus B19; when other members of the household who have SCD are present, they should be monitored for this viral infection with serial measurement of Hb and white blood cell count.6
Other acute problems. Should stroke, acute renal failure, priapism, or hepatobiliary complications develop, evaluate the patient rapidly and refer them to the appropriate care team for management.
Management of chronic complications
Chronic pain is a problem for many patients with SCD. The etiology of this symptom should be investigated fully because a vaso-occlusive crisis is characterized by acute pain. Avascular necrosis or ulcers due to chronic vaso-occlusion should be managed definitively when possible. Adjuvant therapy for chronic pain, such as heat or massage, should be encouraged.
In some patients, chronic pain without objective findings develops over time and becomes unresponsive to nonopioid pharmacotherapy. Such patients might require chronic opioid therapy, the need for which is dictated by the ability of the patient to perform their activities of daily living. For patients who require long-term daily narcotic drugs, best practices—obtaining informed consent, using registries and contracts, random drug testing, and providing naloxone [Narcan] for overdose emergency use—should be employed.15
Continue to: Chronic anemia
