Pdf sickle cell disease in pregnancy elizabeth anionwu. 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. However, with early prenatal care and careful monitoring throughout pregnancy, a woman with scd can have a healthy pregnancy. It has a variable course and prediction of disease severity on a clinical level makes counselling difficult when a fetus is at risk and prenatal diagnosis is contemplated. Many women with scd have questions about the effect pregnancy will have on their scd. With sickle cell disease, youll have a higher risk of certain pregnancy complications, including miscarriage, preterm delivery and having a lowbirthweight baby. The most common type is known as sickle cell anaemia sca. Sickle cell disease is a blood disorder passed down from parent to child.
Sickle cell disease scd is the most common hereditary disease worldwide, presenting with anemia and intermittent severe pain. Jun 20, 2017 managing sickle cell disease during pregnancy can be overwhelming. Sickle cell disease scd is an inherited group of disorders characterized by the presence of hemoglobin s hbs, either from homozygosity for the sickle mutation in the beta globin chain of hemoglobin hbss or from compound heterozygosity of a sickle beta globin mutation with another beta globin mutation eg, sicklebeta thalassemia. Sickle cell disease is a term used for a group of conditions in which the pathology is due to the presence of hemoglobin s. Pregnancy in women with sickle cell disease scd is a highrisk situation. Pregnancy in a patient with scd is associated with high levels of maternal and fetal morbidity and mortality. Sickle cell disease is a serious health condition that runs in families and can shorten life. In a healthy person, red blood cells are round and flexible. Articles were identified through a pubmed search, including studies of women with scd with known maternal andor perinatal outcomes, as well as any known characteristics of reproductive history.
However, life expectancy is only 42 years in women 38 years in men, 2 with pregnancy being a major factor for death in women. Sickle cell anemia, or homozygous sickle cell disease, results from the inheritance of a sickle cell gene from both parents. This means that rcog guidelines are unlike protocols or guidelines issued. Management of sickle cell disease in pregnancy clinical practice. Some women may need blood transfusions to replace the sickle cells with fresh blood.
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. The primary manifestations of scd are chronic hemolytic anemia and pain crises due to vasoocclusion 4, 5. The study of sickle cell disease in pregnancy maternal and fetal outcome in port harcourt in nigeria this study has done in 4500 mothers booked during prenatal care and the results indicated that 0. But the illness can be serious enough to have a significant effect on a persons life. It can also cause pregnancy problems and other serious health problems throughout life. What you should know about sickle cell disease and pregnancy. 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. Normal red blood cells are smooth, round, and flexible. Pregnancy in sickle cell disease is a very highrisk. Approximately 8% of the africanamerican population carries the sickle cell trait. It will include preconceptual screening and antenatal, intrapartum and postnatal management.
Discuss pregnancy and contraception at each sickle clinic. Sickle cell disease scd is a group of blood disorders typically inherited from a persons parents. It results in an abnormality in the oxygencarrying protein haemoglobin found in red blood cells. Mar 22, 2018 all of them mentioned that sickle cell diseases lead to complications during pregnancy, parturition and perinatal period. Pregnancy exacerbates the preexisting pathophysiological characteristics of scd. At around 1 year of age, these children begin to have anaemia, pain, stroke. Due to major progress in the management of scd, most women now survive to an age where they are able to become pregnant. Most women with scd have healthy pregnancies, though it may increase the risk for complications. Babycenter is committed to providing the most helpful and trustworthy pregnancy and parenting information in the world. Maternity guidelines care of the pregnant patient with sickle cell disease gl914. Normally, rbcs are shaped like discs, which gives them the flexibility to travel through even.
Sickle cell disease scd is a genetic condition that is present at birth. These may be done several times during the pregnancy. Sickle cell disease can be diagnosed in an unborn baby by sampling some of the fluid surrounding the baby in the mothers womb amniotic fluid. Sickle cell disease and pregnancy health encyclopedia.
In the united states, it affects more people who are black or africanamerican than other racial or ethnic groups. But there are some risks to be aware of because you could develop complications from either condition. Managing sickle cell disease during pregnancy babycenter. Care of the pregnant patient with sickle cell disease gl914. Repeated vasoocclusive crises can affect multiple organ systems, and individuals with scd. A person with scd can pass the disease on to his or her children. During pregnancy the disease can become more severe, and pain episodes can occur more often. Purpose and scope the purpose of this guideline is to describe the management of pregnant women with sickle cell disease scd. Guidelines for the management of the acute painful crisis in sickle cell. People with this disorder have atypical hemoglobin molecules called hemoglobin s, which can distort red blood cells into a sickle, or crescent, shape signs and symptoms of sickle cell disease usually begin in early childhood. These risks are due, at least in part, to the metabolic demands, hypercoagulable state, and vascular stasis associated with pregnancy.
Red blood cells carry oxygen to the rest of your body. Sickle cell disease and pregnancy article pdf available in mediterranean journal of hematology and infectious diseases 111. Sickle cell disease is a serious genetic disorder affecting 1235 births in french guiana. Sickle cell disease in pregnancy, management of greentop guideline no. Management of sickle cell disease in pregnancy clinical. If you have scd, there is a problem with your hemoglobin. Hemoglobin is a protein in red blood cells that carries oxygen throughout the body.
This consultation should include optimisation of management and screening for end organ damage. During pregnancy there is a test to find out if the baby will have scd, sct, or neither one. High blood pressure and preeclampsia a potentially dangerous pregnancy complication characterized by the sudden onset of high blood pressure also are more common in women. What causes sickle cell disease and sickle cell trait. Prevalence of sickle cell disease in pregnant women and. Adults with sickle cell disease who live in the united states have a decreased life expectancy with the odds of surviving beyond the 7th decade of life reported to be less than 30%. Problems in sickle cell disease typically begin around 5 to 6. Pregnancy in women with sickle cell disease scd is associated with increased maternal and fetal morbidity and mortality. Sickle cell disease refers to a group of genetic disorders in which a patients red blood cells undergo a change of shape known as sickling. Management of sickle cell disease in pregnancy rcog. Sickle cell disease in pregnancy, management of greentop. Learn about the tests youll need, what to do if you have a pain crisis during pregnancy, and how to prevent complications. Jun 02, 2019 with sickle cell disease, youll have a higher risk of certain pregnancy complications, including miscarriage, preterm delivery and having a lowbirthweight baby.
What you should know about sickle cell disease and. Sickle cell disease is caused by inherited mutations of the globin gene, and is a multisystem disorder characterised by distortion, stiffness, and adhesion of red blood cells. Sickle cell disease occurs in approximately one out of. Pregnancy in sickle cell disease can be complicated as both prospective. Mild sickle cell disease may have no impact on a persons daytoday life. Pregnancy in sickle cell disease is at very high risk. Sickle cell disease also called scd is a condition in which the red blood cells in your body are shaped like a sickle like the letter c.
A pregnant woman with scd is at a higher risk of preterm labor. Sickle cell disease, sickle cell anemia, pregnancy, pregnancy complications introduction 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 1 3. Sickle cell anemia, or sickle cell disease scd, is a genetic disease of the red blood cells rbcs. Having sickle cell disease scd doesnt mean you cant have a healthy pregnancy. All of them mentioned that sickle cell diseases lead to complications during pregnancy, parturition and perinatal period. Hemoglobin is a protein in red blood cells that carries oxygen to the body. Outcomes vary widely owing to methodological limitations of clinical studies, but overall, hypertensive disorders of pregnancy, venothromboembolism, poor fetal growth, and maternal and perinatal mortality are increased globally.
If you or your partner has sickle cell anemia or the sickle cell trait, ask your doctor about this screening. All states screen newborns for sickle cell disease. Sickle cell disease in pregnancy obstetrics and gynecology clinics. D from adolescence, the intentions of women with sickle cell disease scd. 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. This publication, which was developed by physicians, nurses, psychologists, and social workers who specialize in the care of children and adults with sickle cell disease, describes the current approach to counseling and also to management of many of the medical complications of sickle cell disease. With scd, the hemoglobin forms into stiff rods within the red blood cells. Sickle cell disease during pregnancy what to expect. Adhesion of normal and sickle erythrocytes to endothelial monolayer cultures. Sickle cell disease scd is a group of inherited red blood cell disorders. However, rcog guidelines royal college of obstetrician and. Thats why youll work with a team of specialists with experience managing scd during pregnancy to make sure you get the best possible care. Pregnancy outcomes in women with sickle cell disease in low and high income countries. Following the cuban guidelines for maternal care program which includes the.
Pdf with advances in management, the majority of women in the uk with sickle cell disease now survive to have children. 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. Pdf sickle cell disease and pregnancy researchgate. Management of sickle cell disease in pregnancy this is the first edition of this guideline. In the united states, sickle cell disease affects an estimated 70,000 to 100,000 people, the majority of whom are african.
Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin s, which can distort red blood cells into a sickle, or crescent, shape. Pregnancy in women with sickle cell disease uptodate. Scd is recessive, which means that both parents must pass on the sickle cell gene for a child to be born with scd. Sickle cell disease sickle cell anemia medlineplus. Scd affects pregnancy and how pregnancy affects sickle cell disease, and how to improve.
This leads to a rigid, sicklelike shape under certain circumstances. People with sickle cell disease have abnormal hemoglobin. Complications in pregnant women with sickle cell disease. Sickle cell anemia is one of a group of disorders known as sickle cell disease. With thousands of awardwinning articles and community groups, you can track your pregnancy and babys growth, get answers to your toughest questions, and. Sickle cell disease is an inherited disorder of haemoglobin synthesis characterised by painful sickling crises.
Pregnancy has been associated with exacerbation of sickle cell disease and may place women, especially those with sickle cell anemia hbss, at an additional risk for obstetric complications. Sickle cell disease varies between individuals from mild to serious, but most people with it lead happy and normal lives. These unusually shaped cells give the disease its name. Sickle cell anemia diagnosis and treatment mayo clinic. In sickle cell anemia, some red blood cells become deformed, so they look like sickles used to cut wheat. Our content is doctor approved and evidence based, and our community is moderated, lively, and welcoming. Sickle cell anemia symptoms and causes mayo clinic. May 21, 2015 pregnancy exacerbates the preexisting pathophysiological characteristics of scd.