Essential strategies and tactics for managing sickle cell disease

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doi:doi: 10.12788/jfp.0441

Applied Evidence

Essential strategies and tactics for managing sickle cell disease

J Fam Pract. 2022 July;71(6):254-261 | doi: 10.12788/jfp.0441

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References

1. Lubeck D, Agodoa I, Bhakta N, et al. Estimated life expectancy and income of patients with sickle cell disease compared with those without sickle cell disease. JAMA Netw Open. 2019;2:e1915374. doi:10.1001/jamanetworkopen.2019.15374

2. Swanson ME, Grosse SD, Kulkarni R. Disability among individuals with sickle cell disease: literature review from a public health perspective. Am J Prev Med. 2011;41(6 suppl 4):S390-S397. doi: 10.1016/j.amepre.2011.09.006

3. Moskowitz JT, Butensky E, Harmatz P, et al. Caregiving time in sickle cell disease: psychological effects in maternal caregivers. Pediatr Blood Cancer. 2007;48:64-71. doi:10.1002/pbc.20792

4. Mehta SR, Afenyi-Annan A, Lottenberg R. Opportunities to improve outcomes in sickle cell disease. Am Fam Phys. 2006;74:303-310.

5. Yawn BP, John-Sowah J. Management of sickle cell disease: recommendations from the 2014 Expert Panel Report. Am Fam Phys. 2015;92:1069-1077.

6. National Heart, Lung, and Blood Institute, National Institutes of Health. Evidence-based management of sickle cell disease. Expert Panel Report, 2014. Accessed June 9, 2022. www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines

7. Committee Opinion No. 690: Carrier screening in the age of genomic medicine. Obstet Gynecol. 2017;129:e35-e40. doi: 10.1097/AOG.0000000000001951

8. Jordan LB, Smith-Whitley K, Treadwell MJ, et al. Screening U.S. college athletes for their sickle cell disease carrier status. Am J Prev Med. 2011;41:S406-S412. doi: 10.1016/j.amepre.2011.09.014

9. Adams RJ, McKie VC, Brambilla D, et al. Stroke prevention trial in sickle cell anemia. Control Clin Trials. 1998;19:110-129. doi: 10.1016/s0197-2456(97)00099-8

10. Alzahrani F, Alaidarous K, Alqarni S, et al. Incidence and predictors of bacterial infections in febrile children with sickle cell disease. Int J Pediatr Adolesc Med. 2021;8:236-238 doi: 10.1016/j.ijpam.2020.12.005

11. Committee on Infectious Diseases. Recommendations for prevention and control of influenza in children, 2021-2022. Pediatrics. 2021;148: e2021053745. doi: 10.1542/peds.2021-053745

12. Centers for Disease Control and Prevention. Study finds people with sickle cell disease who developed coronavirus disease have high rates of hospitalization, intensive care unit admission, and death. October 20, 2020. Accessed June 9, 2022. www.cdc.gov/ncbddd/sicklecell/features/scd-and-covid-19.html

13. James PA, Oparil S, Carter BL, et al. 2014 Evidence-based guideline for the management of high blood pressure in adults: report from the panel members appointed to the Eighth Joint National Committee (JNC 8). JAMA. 2014;311:507-520. doi:10.1001/jama.2013.284427

14. Baskin MN, Goh XL, Heeney MM, et al. Bacteremia risk and outpatient management of febrile patients with sickle cell disease. Pediatrics. 2013;131:1035-1041. doi: 10.1542/peds.2012-2139

15. Osunkwo I, Veeramreddy P, Arnall J, et al. Use of buprenorphine/naloxone in amelio rating acute care utilization and chronic opioid use in adults with sickle cell disease. Blood. 2019;134 (suppl 1):790. doi: 10.1182/blood-2019-126589

16. Vichinsky E, Hoppe CC, Ataga KI, et al; HOPE Trial Investigators. A phase 3 randomized trial of voxelotor in sickle cell disease. N Engl J Med. 2019;381:509-519. doi: 10.1056/NEJMoa1903212

17. Ataga KI, Kutlar A, Kanter J, et al. Crizanlizumab for the prevention of pain crises in sickle cell disease. N Engl J Med. 2017;376:429-439. doi: 10.1056/NEJMoa1611770

18. Dixit R, Nettem S, Madan SS, et al. Folate supplementation in people with sickle cell disease. Cochrane Database Syst Rev. 2016;2:CD011130. doi: 10.1002/14651858.CD011130.pub2

19. Brennan-Cook J, Bonnabeau E, Aponte R, et al. Barriers to care for persons with sickle cell disease: the case manager’s opportunity to improve patient outcomes. Prof Case Manag. 2018;23:213-219. doi: 10.1097/NCM.0000000000000260

COVID-19, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is especially problematic in patients with SCD¹²; infection causes mortality at a rate as high as 7%.⁵ The SARS-CoV-2 mRNA vaccine series is potentially lifesaving for these patients.¹² In addition, at times of high community prevalence, make an effort to minimize patients’ exposure to SARS-CoV-2, by providing telemedicine visits.

Follow the immunization schedule. Patients with SCD should receive all standard recommended vaccinations (ie, those recommended by the Advisory Committee on Immunization Practices.^a) Inactivated virus vaccines are preferred in SCD, when available. For patients who are behind on vaccinations, use a standard vaccine catch-up schedule.

Screening and prevention of complications such as stroke

Determining the risk of stroke. Patients with SCA who are not monitored have a 10% to 11% lifetime prevalence of stroke.^5,6,10 An abnormal transcranial Doppler (TCD) study (defined as a time-averaged mean maximum velocity ≥ 200 cm/s in the distal internal carotid artery or proximal middle cerebral artery) is predictive of a 40% risk of stroke in patients with SCA. With chronic transfusion therapy, a 92% reduction in the risk of stroke is achievable.¹⁰

All patients with SCA should undergo annual screening with TCD ultrasonography from ages 2 to 16 years.⁶ Those who have an abnormal TCD study should receive chronic transfusion therapy. Screening is not recommended for patients with SCD or SCT.

Other complications. Screen and manage as follows:

Proteinuria. Left untreated, SCD can affect the kidneys and lead to renal failure. Annual screening for proteinuria is recommended beginning at age 10 years, with referral when the test is positive and reproducible.
Lung disease and cardiovascular disease. Screening for progression of lung disease and for cardiovascular disease is not recommended in asymptomatic patients with SCD, except through the history.
Blood pressure screening and management of hypertension are based on Joint National Committee (JNC 8) guidelines.¹³
Screening for ocular complications by an eye care provider is recommended beginning at age 10 years.

TABLE 2^6,9 summarizes recommendations on the prevention and early detection of complications of SCD.

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