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Table 4 Summary of key recommendations in the four CPGs from ACOG, NHLBI, NICE, and RCOG*

From: Quality assessment of evidence-based clinical practice guidelines for the management of pregnant women with sickle cell disease using the AGREE II instrument: a systematic review

CPGs/ Recommendations ACOG [22] NHLBI [25] NICE [23] RCOG [24]
Preconception care
Genetic screening Individuals of African, Southeast Asian, and
Mediterranean descent are at increased risk for being carriers of hemoglobinopathies and should be offered carrier screening and, if both parents are determined to be carriers, genetic counseling.
• If the partner of a man or woman with SCD has unknown SCD or thalassemia status, refer the partner for hemoglobinopathy screening.
• After testing, refer couples who are at risk for having a potentially affected fetus and neonate for genetic counseling.
Not Mentioned Women and men with SCD should be encouraged to have the hemoglobinopathy status of their partner determined before they embark on pregnancy. If identified as an “at risk couple,” as per National
Screening Committee guidance, they should receive counseling and advice about reproductive options.
Penicillin prophylaxis Not mentioned Mentioned for the pediatric but not for the pregnant women population. Not mentioned Penicillin prophylaxis or the equivalent should be prescribed
Vaccination status updated pre-pregnancy Not Mentioned Mentioned in general for the adult population but not specifically for pregnant women Not Mentioned Women should be given H. influenza type b and the conjugated meningococcal C
vaccine as a single dose, if they have not received it as part of primary vaccination. The pneumococcal vaccine should be given every 5 years. Hepatitis B vaccination is recommended and the woman’s immune status should be determined pre-conceptually. Women with SCD should be advised to receive influenza and “swine flu” vaccines, annually.
Vitamin supplementation Pregnant patients with SCD need increased prenatal folic acid supplementation. The standard 1 mg of folate in prenatal vitamins is not adequate for patients with hemoglobinopathies; 4 mg per day of folic acid should be prescribed because of the continual turnover of red blood cells. Folic acid supplementation should be used whenever considering or at risk of pregnancy to prevent neural tube defects. Not Mentioned Folic acid (5 mg) should be given once daily both pre-conceptually and throughout pregnancy.
Medication review Hydroxyurea has been shown to reduce the frequency of painful crises in non-pregnant patients with severe SCD. However, the use of hydroxyurea is not recommended during pregnancy because it is teratogenic. In females who are pregnant or breastfeeding, discontinue hydroxyurea therapy. Not Mentioned • Hydroxycarbamide (hydroxyurea) should be stopped at least 3 months before conception.
• Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers should be stopped before conception.
Antenatal care
Antenatal hemoglobinopathy screening Not mentioned Not mentioned Not Mentioned.
But a link to the “NHS Sickle Cell and Thalassaemia Screening Program” was provided.
If the woman has not been seen pre-conceptually, she should be offered partner testing. If the partner is a carrier, appropriate counseling should be offered as early as possible in pregnancy – ideally by 10 weeks of gestation – to allow the option of first-trimester diagnosis and termination, based on the woman’s choice.
Medications during pregnancy Not mentioned Not clearly mentioned for women during pregnancy. Mentioned under research recommendations • If women have not undergone a pre-conceptual review, they should be advised to take folic acid (daily) and prophylactic antibiotics (if not contraindicated). Drugs that are unsafe in pregnancy should be stopped immediately.
• Iron supplementation should be given only if there is laboratory evidence of iron deficiency.
• Women with SCD should be considered for low-dose aspirin 75 mg once daily from 12 weeks of gestation to reduce the risk of developing pre-eclampsia.
• Women with SCD should be advised to receive prophylactic low-molecular-weight heparin during antenatal hospital admissions.
Blood transfusion or prophylactic exchange transfusion for pregnancies Recommended just to keep of Hb S to approximately 40%
While simultaneously raising the total hemoglobin concentration to about 10 g/dL.
Not Mentioned Not Mentioned • Routine prophylactic transfusion is not recommended during pregnancy for women with SCD.
• If acute exchange transfusion is required for the treatment of a sickle complication, it may be appropriate to continue the transfusion regimen for the remainder of the pregnancy.
• Blood should be matched for an extended phenotype including full rhesus typing (C, D, and E) as well as Kell typing.
• Blood used for transfusion in pregnancy should be cytomegalovirus negative.
Ultrasound Scanning and fetal surveillance during pregnancy Pregnancies in women with sickle cell disease are at increased risk for spontaneous abortion, preterm labor, IUGR, and stillbirth. For this reason, a plan for serial ultrasound examinations and antepartum fetal testing is reasonable. Fetal surveillance, which includes growth ultrasounds and antepartum testing (non-stress tests, biophysical profiles, and contraction stress tests), may lead to planned early delivery and can reduce but not eliminate risks (not mentioned as a recommendation). Not mentioned • Women should be offered a viability scan at 7–9 weeks of gestation. Women should be offered the routine first-trimester scan (11–14 weeks of gestation) and a detailed anomaly scan at 20 weeks of gestation. In addition, women should be offered serial fetal biometry scans (growth scans) every 4 weeks from 24 weeks of gestation.
Acute painful crisis Major complications (e.g., worsening anemia; intrapartum complications such as hemorrhage, septicemia, and cesarean delivery; painful crisis; and chest syndrome) may require intervention with an exchange transfusion (not mentioned as a recommendation).
Painful crises in pregnancy as well as in the non-pregnant patients are managed by rapid assessment of the level of pain and prompt administration of analgesia.
Not clearly mentioned for women during pregnancy. • For pregnant women with an acute painful sickle cell episode, seek advice from the obstetrics team and refer when indicated.
• Offer all patients regular paracetamol and NSAIDs (non-steroidal anti-inflammatory drugs) by a suitable administration route, in addition to an opioid, unless contraindicated (Not clearly mentioned for women during pregnancy).
• The use of NSAIDs should be avoided during pregnancy unless the potential benefits outweigh the risks. NSAIDs should be avoided for treating an acute painful sickle cell episode in women in the third trimester. See the “British National Formulary” for details of contraindications.
• Women with SCD who become unwell should have sickle cell crisis excluded as a matter of urgency.
• Pregnant women presenting with acute painful crisis should be rapidly assessed by the multidisciplinary team and appropriate analgesia should be administered. Pethidine should not be used because of the associated risk of seizures.
• Women admitted with sickle cell crisis should be looked after by the multidisciplinary team, involving obstetricians, midwives, hematologists, and anesthetists.
• The requirement for fluids and oxygen should be assessed, and fluids and oxygen administered if required.
• Thromboprophylaxis should be given to women admitted to the hospital with an acute painful crisis.
Intrapartum care
Timing and mode of delivery Not mentioned Not mentioned Not mentioned • Pregnant women with SCD who have a normally growing fetus should be offered elective birth through induction of labor, or by elective cesarean section if indicated, after 38 + 0 weeks of gestation.
• SCD should not in itself be considered a contraindication to attempting vaginal delivery or vaginal birth after cesarean section.
• Blood should be cross-matched for delivery if there are atypical antibodies present (since this may delay the availability of blood), otherwise a “group and save” will suffice.
• In women who have hip replacements (because of avascular necrosis) it is important to discuss suitable positions for delivery.
Optimal mode of analgesia and anesthesia Not mentioned (analgesia mentioned with painful crisis). Not clearly mentioned for women during pregnancy. Not mentioned • Women with SCD should be offered an anesthetic assessment in the third trimester of pregnancy.
• Avoid the use of pethidine, but other opiates can be used.
• Regional analgesia is recommended for cesarean section.
Postpartum care
Neonatal screening Not mentioned Not mentioned Not mentioned In pregnant women where the baby is at high risk of SCD (i.e. the partner is a carrier or affected), early testing for SCD should be offered. Capillary samples should be sent to laboratories experienced in the routine analysis of SCD in newborn samples. This will usually be at a regional center.
VTE prophylaxis Not Mentioned. Not clearly mentioned for women during pregnancy. Not mentioned Low-molecular-weight heparin should be administered while in hospital and 7 days post-discharge following vaginal delivery or for a period of 6 weeks following cesarean section.
Contraception Not Mentioned • Progestin-only contraceptives (pills, injections, and implants), levonorgestrel IUDs, and barrier methods have no restrictions or concerns for use in women with SCD.
• If the benefits are considered to outweigh the risks, combined hormonal contraceptives (pills, patches, and rings) may be used in women with SCD.
Not Mentioned • Progestogen-containing contraceptives such as the progesterone-only pill, injectable contraceptives, and the levonorgestrel intrauterine system are safe and effective in SCD.
• Estrogen-containing contraceptives should be used as second-line agents.
• Barrier methods are as safe and effective in women with SCD as in the general population.
  1. Abbreviations: ACOG American College of Obstetricians and Gynecologists; CPG clinical practice guideline or guidance; DCOI declaration of conflict of interests; NICE National Institute of Health and Care Excellence; NHLBI National Institutes of Health, National Heart, Lung, and Blood Institute; PGD Preimplantation genetic diagnosis; and RCOG Royal College of Obstetricians and Gynaecologists