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.

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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

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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

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Pregnancy planning

The Centers for Disease Control and Prevention recommends that a “reproductive life plan” be part of every person’s health journey (TABLE 2).^6,9The plan is especially relevant for female patients who have a known heritable concern, such as SCD, in which a pregnancy is more likely to be complicated by growth restriction, preterm delivery, and fetal demise. These risks are reduced—but not eliminated—with intensive surveillance of the pregnancy. Pregnancy in patients with SCD is also more likely to be complicated by preeclampsia, venous thromboembolism, infection, and maternal death.

Other recommendations:

Every patient with SCD should receive genetic counselling before conceiving, when possible.
Pregnancy should be considered high risk in women who have SCD, and monitored as such.
Women with SCD can use any method of contraception—none of which put them at increased risk of complications, compared to the general population. Rather, it is pregnancy that puts them at greater risk of morbidity and mortality in every age group.

Ambulatory management of acute complications

Vaso-occlusive pain crisis. The hallmark of SCD is the acute pain crisis. Almost all patients with SCD (and the occasional patient with SCT) will experience a pain crisis. In more affluent countries, management of an acute pain crisis almost always includes opioid analgesia.⁶

For the most part, pain crises manifest in a predictable pattern. Although patients with SCD might have acute pain, other causes of acute pain, such as an acute intra-abdominal process or (in older patients) a cardiac process, should be considered as well.

Prophylactic penicillin dosing has proved beneficial in patients with sickle cell disease, demonstrating a decrease in the risk of pneumococcal infection.

For patients having a vaso-occlusive pain crisis, achieving rapid analgesia is key to management. Ready availability of narcotics, at home or under observation, prevents subsequent hospitalization; nonsteroidal anti-inflammatory drugs can be used as adjuvant treatment in patients without contraindications.^5,6 An individualized treatment plan, including access to analgesia at an appropriate dosage, should be negotiated, and adhered to, by the patient and the care team.

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