Management of Sickle Cell Disease in Pregnancy

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Sickle cell anemia is an autosome linked recessive trait that can be transmitted from parents to the offspring when
both the partners are carrier for the gene (or heterozygous). The disease is controlled by a single pair of allele, HbA
and HbS. Out of the three possible genotypes only homozygous individuals for HbS (HbS, HbS) show the diseased phenotype. The ability to predict the clinical course of SCD during pregnancy is difficult. It is mandatory to follow up the patient closely from the very beginning i.e. from preconception to antenatal till labor. SCD is associated with both maternal and fetal complications and is associated with an increased incidence of perinatal mortality, premature
labor, fetal growth restriction and acute painful crises during pregnancy.

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Management of Sickle Cell Disease in Pregnancy

  1. 1. Management of Sickle Cell Disease in Pregnancy
  2. 2. Apollo Medicine 2012 September Volume 9, Number 3; pp. 181e183 Review Article Management of sickle cell disease in pregnancy Rashmi Sharma ABSTRACT Sickle cell anemia is an autosome linked recessive trait that can be transmitted from parents to the offspring when both the partners are carrier for the gene (or heterozygous). The disease is controlled by a single pair of allele, HbA and HbS. Out of the three possible genotypes only homozygous individuals for HbS (HbS, HbS) show the diseased phenotype. The ability to predict the clinical course of SCD during pregnancy is difficult. It is mandatory to follow up the patient closely from the very beginning i.e. from preconception to antenatal till labor. SCD is associated with both maternal and fetal complications and is associated with an increased incidence of perinatal mortality, premature labor, fetal growth restriction and acute painful crises during pregnancy. Copyright © 2012, Indraprastha Medical Corporation Ltd. All rights reserved. Keywords: Sickle cell anemia, Acute anemia, Hemoglobin, Pregnancy INTRODUCTION The term Sickle cell disease encompasses several different sickle hemoglobinopathies. It includes sickle cell anemia (HbSS) and the heterozygous conditions of hemoglobin S and other clinically abnormal hemoglobin. These include combination with hemoglobin C (giving HbSC), combination with beta thalassemia (giving HbSB thalassemia) and combination with hemoglobin D, E or O-Arab. The pathophysiology of SCD is a consequence of polymerization of the abnormal hemoglobin in low-oxygen conditions, which leads to the formation of rigid and fragile sickle-shaped red cells. Pregnancy with sickle cell disease needs to be taken care of very closely; from preconception to labor.2 PRECONCEPTION CARE Women with SCD should be seen preconceptually by a sickle specialist to receive information about how SCD affects pregnancy and how pregnancy affects sickle cell disease, and how to improve outcomes for mother and baby. This consultation should include optimization of management and screening for end-organ damage. Women with SCD should be encouraged to have the hemoglobinopathy status of their partner determined before they embark on pregnancy and they should receive appropriate counseling regarding the risk of having affected offspring.7 Management & treatment Patients with SCD are hyposplenic and are at risk of infection, in particular from encapsulated bacteria such as Neisseria meningitides, Streptococcus pneumonia and Haemophilus influenzae. They should be given penicillin prophylaxis. In addition, women should be given H. influenzae type b and the conjugated meningococcal C vaccine as a single dose if they have not received it as part of primary vaccination. Hepatitis B vaccination is recommended and the woman’s immune status should be determined preconceptually. Women with SCD should be advised to receive the influenza and ‘swine flu’ vaccine annually. Folic acid (5 mg) should be given once daily both preconceptually and throughout pregnancy.1 Hydroxycarbamide (hydroxyurea) should be stopped at least 3 months before conception and angiotensin-converting enzyme inhibitors and angiotensin receptor blockers before conception. Sr. Consultant Obs & Gynae, Apollo Hospital, Bilaspur, India. Received: 14.6.2012; Accepted: 2.7.2012; Available online: 7.7.2012 Copyright Ó 2012, Indraprastha Medical Corporation Ltd. All rights reserved. http://dx.doi.org/10.1016/j.apme.2012.07.002
  3. 3. 182 Apollo Medicine 2012 September; Vol. 9, No. 3 ANTENATAL CARE Women with SCD should aim to avoid precipitating factors of sickle cell crises such as exposure to extreme temperatures, dehydration and overexertion. Persistent vomiting can lead to dehydration and sickle cell crisis. Women with HbSC experience fewer adverse outcomes, but there is still evidence of an increased incidence of painful crises during pregnancy, fetal growth restriction, antepartum hospital admission and postpartum infection. Although outcomes among women with HbSC are better than in women with HbSS, some do have serious, unpredictable complications, and women with HbSC should therefore be monitored in the same way as those with HbSS.3 Management & treatment Women should be offered a viability scan at 7e9 weeks of gestation and the routine first-trimester scan (11e14 weeks of gestation) followed by 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. 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. The influenza vaccine should be recommended if it has not been administered in the previous year. 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 in an effort to reduce the risk of developing preeclampsia. Women with SCD should be advised to receive prophylactic low-molecular-weight heparin during antenatal hospital admissions. Non-steroidal anti-inflammatory drugs (NSAIDs) should be prescribed only between 12 and 28 weeks of gestation owing to concerns regarding adverse effects on fetal development blood pressure and urinalysis should be performed at each consultation, and midstream urine for culture performed monthly.4 EMERGENCY MANAGEMENT Pregnant women presenting with acute painful crisis should be rapidly assessed by the multidisciplinary team and appropriate analgesia should be administered. The Sharma requirement for fluids and oxygen should be assessed. Thromboprophylaxis can be given to women admitted to hospital with acute painful crisis but Pethidine should be avoided because of the associated risk of seizures. SCD is associated with other acute complications including ACS, stroke and acute anemia. After acute pain, ACS is the most common complication, reported in 7e20% of pregnancies. Top-up blood transfusion may be required if the hemoglobin is falling, and certainly if the hemoglobin is less than 6.5 g/dl, but in severe hypoxia, and if the hemoglobin level is maintained, exchange transfusion will be required.5,6 Acute anemia in women with SCD may be attributable to erythrovirus infection. Infection with erythrovirus in SCD causes a red cell maturation arrest and an aplastic crisis characterized by a reticulocytopenia. Therefore, a reticulocyte count should be requested in any woman presenting with an acute anemia and, if low, may indicate infection with erythrovirus. Other investigations will depend on the clinical scenario but may include blood cultures, chest X-ray, urine culture and liver function tests. In case of low levels of hemoglobin and erythrovirus infection blood transfusion may be required. With erythrovirus infection there is the added risk of vertical transmission to the fetus, which can result in hydrops fetalis, hence a review by a fetal medicine specialist is indicated. Women with SCD can develop anemia owing to bleeding or any other causes of anemia incidental to the SCD. Rare causes of anemia in SCD include malaria and, occasionally, splenic sequestration in women with a mild phenotype. LABOR CARE Pregnant women with SCD who have a normally growing fetus should be offered elective birth through induction of labor, or by elective caesarean section if indicated, after 38þ0 weeks of gestation. SCD should not in itself be considered a contraindication to attempting vaginal delivery or vaginal birth after caesarean 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. It is recommended that, like most ‘high-risk’ conditions, delivery of the baby at 38e40 weeks of gestation will prevent late pregnancy complications and associated adverse perinatal events. Women should be kept warm and given adequate fluid during labor. Continuous intrapartum electronic fetal heart rate monitoring is recommended owing to the increased
  4. 4. Management of sickle cell disease in pregnancy risk of fetal distress which may necessitate operative delivery. There is an increased risk of painful crisis with protracted labor (more than 12 h), but this is often secondary to dehydration. In this situation, if the woman is well hydrated and labor is progressing, the labor should be carefully supervised; caesarean section should be considered if labor is not progressing well and delivery is not imminent. The demand for oxygen is increased during the intrapartum period and the use of pulse oximetry to detect hypoxia in the mother is appropriate during labor. Arterial blood gas analysis should be performed and oxygen therapy instituted if oxygen saturation is 94% or less. The clinician should have a low threshold to commence broad-spectrum antibiotics.8,9 Postpartum contraceptive advice should be given to all of them Prostoge containing contraceptive like progesterone only pills, cerazette injectable contraceptives (Depo-Provera) levenorgestrel intrauterine system (Mirena IUS) 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. DISCUSSION SCD is associated with both maternal and fetal complications and is associated with an increased incidence of perinatal mortality, premature labor, fetal growth restriction and acute painful crises during pregnancy. Some studies describe an increase in spontaneous miscarriage, antenatal hospitalization, maternal mortality, delivery by caesarean section, infection, thromboembolic events and antepartum hemorrhage. An increased risk of pre-eclampsia and pregnancy-induced hypertension has been described in other studies but not in all. In HbSC there are fewer reported Review Article 183 adverse outcomes, but there is evidence of an increased incidence of painful crises during pregnancy, fetal growth restriction, antepartum hospital admission and postpartum infection. CONFLICTS OF INTEREST The author has none to declare. REFERENCES 1. Green top guidelines. Royal College of Obstetricians and Gynecologists. 2. Pauling L, Itano HA, Singer SJ, Wells IC. Sickle cell anemia a molecular disease. Science. 1949;110:543e548. 3. Stuart MJ, Nagel RL. Sickle cell disease. Lancet. 2004;364: 1343e1360. 4. Disease Control Priorities in Developing Countries. 2nd ed. Washington, DC/New York, NY: The World Bank/Oxford University Press; 2006: 663e80. 5. Chakravarti A, Li CC, Buetow KH. Estimation of the marker gene frequency and linkage disequilibrium from conditional marker data. Am J Hum Genet. 1984;36:177e186. 6. Davies SC, Brozovi M. The presentation, management and prophylaxis of sickle cell disease. Blood Rev. 1989;3:29e44. 7. Serjeant GR. The emerging understanding of sickle cell disease. Br J Haematol. 2001;112:3e18. 8. Streetley A, Latinovic R, Hall K, Henthorn J. Implementation of universal newborn bloodspot screening for sickle cell disease and other clinically significant haemoglobinopathies in England: screening results for 2005e7. J Clin Pathol. 2009;62:26e30. 9. Angastiniotis M, Modell B, Englezos P, Boulyjenkou V. Prevention and control of haemoglobinopathies. Bull World Health Organ. 1995;73:375e386.
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