Despite recent advances in the management of sickle cell disease scd through improved care, 1,2 reinduction of fetal hemoglobin synthesis, 3,4 and bone marrow transplantation, 5 the condition nonetheless frequently causes major morbidity and early death. Sickle cell anemia is an inherited red blood cell disorder in which there arent enough healthy red blood cells to carry oxygen throughout your body. We also evaluated the impact of blood transfusion on sickle cell complications during pregnancy. What you should know about sickle cell disease and pregnancy. However, rcog guidelines royal college of obstetrician and. Women with sickle cell trait demonstrated a shorter mean duration of pregnancy, compared with women in the control group. Sickle cell disease scd is the most common inherited hemoglobinopathy with approximately 300,000 neonates born globally every year, predominantly in countries like nigeria, india and democratic republic of congo. Higher frequencies of pregnancy complications have been reported among women with sickle cell disease scd compared with those without scd. Sickle cell anemia, or sickle cell disease scd, is a genetic disease of the red blood cells rbcs. Prenatal diagnosis of sickle cell disease harvard university. Delayed hemolytic transfusion reaction in sickle cell disease. Sep 05, 2019 sickle cell disease often becomes more severe and pain episodes more frequent during pregnancy, particularly in the third trimester. You will need to have a blood test to find out if you carry the sickle cell gene, ideally before you are pregnant or early in your pregnancy nhs 2016b, rcog 2011, phe 2017.
Sep 23, 2016 pathophysiology of sickle cell crisis in pregnancy clinical features any woman with pregnancy, who is with refractory anaemia and who originally belongs to the eastern india, especially with good social class, presenting with signs and symptoms of anaemia, in such cases haemoglobinopathies should be excluded. Sickle cell disease is an inherited condition in which your body makes abnormal red blood cells that are sickle or c shaped instead of round. Providers usually recommend nsaids to help women with pain when nursing because only a small amount of these drugs pass into breast milk. Preconception care and sickle cell anemia in pregnancy 14 journal of basic and clinical reproductive sciences january december 2012 vol 1 issue 1 and 2 abdominal shielding while urinary tract infection referred to urinary symptoms with positive urine culture. A woman with scd is more likely to have problems during pregnancy that can affect her health and the health of the unborn baby than a woman without scd. Prophylactic red blood cell exchange may be beneficial in the management of sickle cell disease in pregnancy. Normal red blood cells are smooth, round, and flexible. What causes sickle cell disease and sickle cell trait.
Pregnancy in women with sickle cell disease uptodate. Prophylactic transfusion for pregnant women with sickle cell disease. Pregnant women with sickle cell disease, especially sstype. Discuss pregnancy and contraception at each sickle clinic. The sickle cell and obstetric pregnancy and childbirth teams at guys and st thomas look after women with sickle. Hemoglobin is a protein in red blood cells that carries oxygen to the body.
Pdf pregnancy in sickle cell disease is a very highrisk. Any pregnant woman with hbss can present with shortness of. Sickle cell disease in pregnancy study 0210 data collection form case please report women delivering on or after 1st february 2010 and before 1st february 2011 case definition. Krauss, posttransfusion crises in sickle cell anemia. Pdf introduction there are significant fetal and maternal complications in pregnancies experienced by mothers with sickle cell disease scd. Women with sickle cell disease have a high risk of complications during pregnancy, according to new research from french guiana. Sickle cell disease and pregnancy health encyclopedia. Describe nursing care of a pregnant woman with sickle cell disease. Mar 23, 2015 pregnancy complications in women with sickle cell disease date. All babies born in the united states are screened for sickle cell disease shortly after birth, but you can also have tests during your pregnancy if you want to find out before your baby is born. This study aimed to describe the followup of pregnancies among sickle cell disease patients in cayenne hospital, in order to highlight the most reported complications.
Sickle cell disease scd is the most common inherited blood disorder. The sickle cell trait and pregnancy abrams, jerome m. Pregnancy in sickle cell disease can be complicated as both prospective. Either homozygous sickle cell disease or compound heterozygous conditions of hbs with hbc, d. Sickle cell disease scd is an inherited hemoglobinopathy that affects approximately 80,000100,000 americans in the us, with the majority of cases among african americans. Can you find out if your baby has scd or sickle cell trait during pregnancy.
Preconception counseling with an mfm specialist is also recommended so that parents understand what to expect during pregnancy, including complications. This consultation should include optimisation of management and screening for end organ damage. Sickle cell in pregnancy acog sickle cell odor odor sickle cell anemia and pregnancy sickle cell swollen eye download here free healthcaremagic app to ask a doctor. D 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.
Management of sickle cell disease in pregnancy clinical. This is another reason to tell your child that they have sickle cell trait later in life. Jun 02, 2019 sickle cell disease is an inherited condition in which your body makes abnormal red blood cells that are sickle or c shaped instead of round. A pregnant woman with sickle cell disease is more likely to have a miscarriage, preterm labour, or a lowbirthweight baby, a type of high blood pressure called pregnancyinduced hypertension, or even. This information is for you if you want to know more about pregnancy and sickle cell disease scd. Ugboma and others published sickle cell disease in pregnancy. It will include preconceptual screening and antenatal, intrapartum and postnatal management. Pulmonary hypertension as risk factor for death in scd nejm 3509886 895, 2004. When both parents have sct, they have a 50% chance of having a child with sct with every pregnancy. However, with early prenatal care and careful monitoring throughout pregnancy, a woman with scd can have a healthy pregnancy. We describe below a case of sickle cell anaemia in pregnancy in an indian patient and to our knowledge, this is the first case of this condition in pregnancy described locally. Its best to have the test before youre 10 weeks pregnant. Pregnancy complications in women with sickle cell disease.
The use of prophylactic transfusions to reduce the risk of sickle complications is. When both parents have sct, they have a 25% chance of having a child. It is not something you catch or develop later in life. Sickle cell disease scd is a genetic condition that is present at birth. Management of sickle cell disease in pregnancy rcog. This diagram shows that for each pregnancy, there is a one in four chance of the baby having sickle cell disease, if both parents are carriers. Normally, rbcs are shaped like discs, which gives them the flexibility to travel through even. Repeated vasoocclusive crises can affect multiple organ systems, and individuals with scd. You develop the disease if you inherit two sickle cell genes, one from each biological parent.
Home july 1959 volume 14 issue 1 the sickle cell trait and pregnancy. Sickle cell disease texas department of state health. Sickle cell disease and pregnancy ucla health library. Pregnancy in sickle cell disease is a very highrisk situation. Purpose and scope the purpose of this guideline is to describe the management of pregnant women with sickle cell disease scd.
Oct 15, 2007 the american college of obstetricians and gynecologists acog has released recommendations for screening and clinical management of hemoglobinopathies during pregnancy. Momstobe with sickle cell disease are more likely to experience anemia and painful episodes during pregnancy than at other times. When compared to healthy pregnant women, pregnant women with a severe. People with sickle cell disease have abnormal hemoglobin. Although most pregnancies complicated by maternal sickle cell disease scd are likely to result in livebirth, these pregnancies are at increased risk of obstetrical and fetal complications, as well as medical complications of scd. Jul 18, 2016 the study, pregnancy in sickle cell disease is a very highrisk situation. An observational study, published in the journal obstetrics and gynecology international, underscores that pregnant women with sickle cell disease should be supervised by a multidisciplinary team that includes primary care physicians, obstetricians, and a. Normally, hemoglobin in red blood cells takes up oxygen in the lungs and carries it through the arteries to all the cells in the tissues of the body. Sickle cell disease is a serious genetic disorder affecting 1235 births in french guiana.
Sickle cell trait sct is not a disease, but means that a person has inherited the sickle cell gene from one of his or. Sickle cell disease is often detected during pregnancy or soon after birth. Sickle cell disease scd is the most common hereditary disease worldwide, presenting with anemia and intermittent severe pain. Methods a prospective cohort of 104 pregnant women registered in the blood center of belo horizonte hemominas foundation was followed up at highrisk prenatal units. Sickle cell disease national heart, lung, and blood. Have sickle cell trait, in every pregnancy there is a. Sickle cell disease often becomes more severe and pain episodes more frequent during pregnancy, particularly in the third trimester.
Sickle cell disease is a blood disorder passed down from parent to child. Sep 19, 2017 for patients who have sickle cell disease, experts suggest genetic counseling before conceiving a child to ensure the parents are aware of the risks of having a child born with sickle cell disease. Pregnant women with sickle cell disease, especially ss. Jun 20, 2017 all babies born in the united states are screened for sickle cell disease shortly after birth, but you can also have tests during your pregnancy if you want to find out before your baby is born.
Sickle cell disease scd is when your red blood cells are shaped like sickles. Pathophysiology of sickle cell crisis in pregnancy clinical features any woman with pregnancy, who is with refractory anaemia and who originally belongs to the eastern india, especially with good social class, presenting with signs and symptoms of anaemia, in such cases haemoglobinopathies should be excluded. A person with scd can pass the disease on to his or her children. This is because youll have the option of more tests to find out whether your baby will be affected if this first test shows youre a carrier of a blood disorder. Sickle cell disease in pregnancy carries a high risk of maternal and fetal adverse outcomes. If you or your partner has scd or sickle cell trait, you can have a prenatal test to find out if your baby has scd or sickle cell trait. Sickle cell anemia symptoms and causes mayo clinic. During pregnancy there is a test to find out if the baby will have scd, sct, or neither one. Screening for sickle cell disease in pregnancy is offered to all pregnant women in england to check if theres a risk of a child being born with the condition, and all babies are offered screening. Sickle cell disease and pregnancy dr sinou micyline epouse tchana kontchou gynecologue obstetricien postgraduate training in reproductive health research faculty of medicine, university of yaounde 2007. Preconception care and sickle cell anemia in pregnancy. You can also have sickle cell trait, which means you carry the gene for sickle cell but dont have the disease.
Acog publishes guidelines on hemoglobinopathies in pregnancy. All the information, content and live chat provided on the site is intended to be for informational purposes only, and not a substitute for professional or medical advice. National sickle cell awareness month was developed to raise awareness about this inherited disorder that alters red blood cells, ultimately leading to a range of serious health complications. Maternal and fetal outcome in port harcourt, nigeria find, read and cite all the research you need on. This means that rcog guidelines are unlike protocols or guidelines issued. What you should know about sickle cell disease and.
The american college of obstetricians and gynecologists acog has released recommendations for screening and clinical management of hemoglobinopathies during pregnancy. Management of sickle cell disease in pregnancy this is the first edition of this guideline. Rarely, an adult with sickle cell trait will have health problems when they go through extreme stress or do hard physical work. In the first trimester, you can have chorionic villus sampling cvs between 10 and weeks of pregnancy. We compared the prevalence of maternal complications among intrapartum and postpartum women with scd to those without scd in. Pregnancy complications in women with sickle cell disease date. Pregnancy in a patient with scd is associated with high levels of maternal and fetal morbidity and mortality. The sickle cell gene is passed down from parents to their children. Pregnancy in sickle cell disease is a very highrisk. The primary manifestations of scd are chronic hemolytic anemia and pain crises due to vasoocclusion 4, 5. This does not mean that one in four babies born to this couple will have sickle cell disease. These risks are due, at least in part, to the metabolic demands, hypercoagulable state, and vascular stasis associated with pregnancy. Sickle cell disease can cause serious health complications that can include pneumonia, organ damage, swelling of hands and feet, chest pains and trouble breathing, blood in urine, fever, stroke, leg ulcers, infections, jaundice, gallstones, anemia, as well as painful erections in men and complications during pregnancy. With a longer life span and improved quality of life, these patients are becoming more and more pregnant.
The sickle cell and obstetric pregnancy and childbirth teams at guys and st thomas look after women with sickle cell disease scd before, during and after pregnancy. A woman with scd is more likely to have problems during pregnancy that can affect her health and the health of the unborn baby. The increased metabolic demands, hypercoagulable state, and vascular stasis associated with pregnancy predispose to these complications. Depending on where you live, your midwife will offer you a blood test for sickle cell as part of your antenatal care nhs 2016b. Management of sickle cell crisis in pregnancy obgyn key. Sickle cell anemia is one of a group of disorders known as sickle cell disease. This happens when only one parent passes on the sickle cell gene. Sickle cell disease and pregnancy patient information leaflet. Pregnancy and sickle cell disease guys and st thomas.
Maternal complications in a medicaidenrolled population article pdf available in maternal and child health journal 172 january 20 with 274 reads. Most women with scd have healthy pregnancies, though it may increase the risk for complications. Jun 20, 2017 if you took narcotics throughout your pregnancy, breastfeeding can reduce the withdrawal symptoms your baby might have after birth. Sickle cell disease is an inherited disease caused by defects, called mutations, in the beta globin gene that helps make hemoglobin. A delayed hemolytic transfusion reaction after partial exchange transfusion for sickle cell disease in pregnancy. Ppt sickle cell disease powerpoint presentation free. Continuing nursing education cne credit a total of 1. For women, sickle cell and pregnancy, is deemed high risk. The selected green journal articles are free through the end of. During pregnancy the disease can become more severe, and pain.
Sickle cell disease scd is a group of inherited singlegene autosomal recessive disorders caused by the sickle gene, which affects haemoglobin structure. D from adolescence, the intentions of women with sickle cell disease scd. Under the care of maternalfetal medicine mfm specialists, many women are able to carry a child to term even with a sickle. Perinatal and neonatal implications of sickle cell disease. Scd has its origins in subsaharan africa and the middle east, hence it is most prevalent in individuals of african descent as well as in the caribbean, middle east, parts of india and. The findings also show that women with the more severe sstype sickle cell disease are more likely to have premature babies, and to suffer health problems themselves, than women with less severe disease types. Sickle cell disease in pregnancy, management of greentop. Scd affects pregnancy and how pregnancy affects sickle cell disease, and how to improve. Screening for sickle cell and thalassaemia involves a blood test. Preeclampsia was the development of hypertension and proteinuria in the.
Scd is recessive, which means that both parents must pass on the sickle cell gene for a child to be born with scd. Management of sickle cell disease in pregnancy clinical practice. To evaluate complications in pregnant women with sickle cell disease, especially those leading to maternal death or near miss severe obstetric complications. Clinical complications in pregnant women with sickle cell. Sickle cell disease during pregnancy what to expect.
122 1431 740 1011 479 187 909 1454 1135 1195 1151 794 550 1278 1144 1126 1286 319 506 662 515 292 402 513 964 579 810 526 654 1254 1384 475