Sickle Cell Disease - Initial Management for Family Physicians

Overview

Sickle cell disease (SCD) results from a mutation in the beta globin gene (HbSS genotype). It causes chronic hemolytic anemia and significant multi-organ complications. Sickle cell trait (HbAS) is usually asymptomatic.

All SCD patients should be followed by a Hematologist. The role of the family physician is to initiate education and preventive care while awaiting the referral.

Urgent Symptoms - Educate Patients to Seek Care Immediately

*     Fever

*     Acute severe pain anywhere in the body

*     Chest pain or breathing difficulty

*     Neurological symptoms (stroke, TIA)

*     Priapism

*     Abdominal pain

Families should have a thermometer at home.

Triggers of Vaso-Occlusive Episodes

Educate patients and families to avoid:

*     Dehydration

*     Cold exposure

*     Illness or infection

*     Physical or psychological stress

*     Surgery (flag to surgical teams)

Infection Prophylaxis

Children with SCD require antimicrobial prophylaxis:

*     Age 2 months to 3 years: Penicillin V 125 mg twice daily

*     Age 3 to 5 years: Penicillin V 250 mg twice daily

Vaccination

Refer for:

*     Pneumococcal: PCV13 and PPV23

*     Meningococcal: serogroups B and ACYW-135

*     All routine immunizations

Nutrition

*     Folic acid 1 mg daily for all SCD patients

*     Daily multivitamin WITHOUT iron. Avoid iron unless the patient is clearly iron deficient.

*     Monitor calcium and vitamin D; supplement if needed

*     Monitor growth at every visit in children

Organ Monitoring (Annually)

*     Blood pressure

*     Serum creatinine

*     Urine microscopy and microalbumin

Ophthalmology

Refer all newly diagnosed SCD patients for a baseline ophthalmologic examination (retinopathy screening). If normal, follow up every 1-2 years.

Calgary Referral

Refer to Hematology through the Alberta Health Services referral pathway. Do not wait for the referral before starting the above preventive measures.