Monitoring

Patients require regular monitoring for complications of sickle cell disease.[151] The frequency at which the physician will see the patient is dependent on the age of the patient, and the frequency and severity of the patient's complications.

From birth to 12 months, sickle cell disease healthcare appointments should be scheduled every 2-4 months, with appointments at least every 6-12 months thereafter until transition to adult care.[151] At each appointment from age 1 year, take a complete blood count and reticulocyte count, and establish blood pressure, respiratory rate, pulse oximetry, and heart rate.[151]

Specific monitoring should include the following:

  • Patients treated with hydroxyurea require complete blood count with differential and reticulocyte count monitored on a monthly basis.

  • Transcranial Doppler (TCD) screening of children with HbSS or HbSB0 thalassemia is recommended, starting at 2 years of age and continued annually if TCD is normal (mean flow velocity <170 cm/second) or more frequently if TCD is marginal.[66][67] Children with abnormal results (mean flow velocity ≥200 cm/second) are tested again within 1-2 weeks.[67] TCD screening may be considered for children with sickle cell variants (other than HbSC), who have evidence of hemolysis in the same range as those with HbSS.[66]

  • MRI brain without sedation should be performed as soon as possible, in addition to TCD, in children and young adults with HbSS and Hb0 thalassemia to evaluate for silent cerebral infarct.[67] The American Society of Hematology recommends considering MRI screening at least once in early-school-age children and at least once in adults.[66]

  • Surveillance for cognitive impairment using simplified signaling questions is recommended for children and adults.[66]

  • Symptomatic adults should undergo an echocardiogram to evaluate for pulmonary hypertension.[111]​ ​ In the absence of symptoms, the American Society of Hematology recommends against screening echocardiography or pulmonary function tests, or screening for sleep disordered breathing (e.g., polysomnography).[111]

  • Patients require annual retinal exams to screen for proliferative retinopathy. Screening for retinopathy with a dilated eye exam begins at age 10 years.[38]​

  • Annual screening for the presence of increased urinary albumin excretion, and annual assessment of liver, pulmonary, and renal function, is recommended.[151]

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