Health Maintenance for Sickle Cell Disease!

September is Sickle Cell Awareness Month!

All patients with sickle cell disease (SCD) should have a PCP. We play an integral role in these patients’ lives and have a holistic overview of their care. We can provide continuity and coordination of care.

Here are some important points to remember about health maintenance for patients with SCD:

  • Prophylaxis: Give oral PCN twice daily until 5yo (to be continued if patient has splenectomy or an invasive pneumococcal infection)
  • Immunizations: People with SCD have functional asplenia and thus have special immunization requirements (remember those encapsulated organisms?!) with guidance available from the CDC
  • Stroke: For 2-16yo patients with HbSS or HbSB0 thalassemia, screen annually with transcranial doppler to identify children at risk for stroke
  • HTN: BP screening should be done at every visit. Early treatment of HTN is critical because mild elevations in BP are associated with increased risk of overt stroke and silent cerebral infarct
  • Proteinuria: Annual screening for proteinuria with UA starting at age 10
  • Retinopathy: Annual ophthalmology screening for retinopathy starting at age 10
  • Contraception: CDC and WHO consider all methods of combined (estrogen-progestin) and progestin-only hormonal contraception and the copper-releasing IUD safe and effective for women with SCD
  • Depression: At least annual screening for depression
  • My personal favorite: recommend avoidance of exposure to pet reptiles to decrease the risk of salmonellosis!