Table of Contents
Patients, Families and Friends
Health Care Providers
|Specific Problems: Pregnancy|
|Health Care Providers - Problem Oriented Clinical Guidelines|
Management of pregnancy in the patient with a sickle syndrome requires coordinated care by obstetricians and hematologists knowledgeable in the disease. There are increased risks for the pregnant patient and spontaneous abortion is more likely. With careful management, there is no reason that women cannot have children. All female patients should receive accurate information about risks of pregnancy, genetic transmission of sickle syndromes, methods of contraception, prenatal diagnosis, prevention of sexually transmitted disease and the increased responsibility of raising children at puberty and periodically throughout their reproductive lives.
by Hiba Tamim, MD
Edited by James Eckman, M.D. and Allan Platt, PA-C
Clinical Findings Subjective Data
Present Illness. Patient usually present with amenorrhea and associated symptoms of breast enlargement and tenderness, morning sickness, change in pigmentation, fatigue, and quickening. Menstrual irregularity is common in patients with sickle syndromes.
Past Medical History. Characterization of menstrual history and sexual activity is important. Occurrence, outcome, and management of previous pregnancies should be determined. Past episodes of sexually transmitted diseases is sought. Previous transfusions, occurrence of alloantibodies, and transfusion reactions are documented.
Minimum Lab. Serum beta HCG, CBC with reticulocyte and platelet count, Chem profile including electrolytes, BUN, Creat, glucose, AST, bilirubin, LDH and alk ptase, urinalysis, VDRL, HIV, alloantibody screen. Hemoglobin electrophoresis of the father.
Additional Lab. Consider red cell antigen phenotype for common alloantibodies.
Interuterine Pregnancy. Presence of enlarged uterus and positive beta HCG provide a presumptive diagnosis. Presence of fetal heart tones, fatal movement, or ultrasound confirm the diagnosis.
Tubal Pregnancy. Often presents with abdominal pain that may be confused with sickle pain. Beta HCG, physical exam, and ultrasound confirm the diagnosis.
Pseudocyesis. Spurious pregnancy is occasionally observed in young patients who want to be pregnant. A negative Beta HCG excludes pregnancy..
Myomatous Uterus. May be mistaken for pregnancy. A negative Beta HCG excludes pregnancy.
All patients should be under the care of an obstetrician with interest and expertise in management of pregnancy in patients with sickle syndromes. The primary care physician and hematologist should be involved as members of the management team..
All patients should be on folic acid, prenatal vitamins, and standard iron supplementation unless iron overload is present. Follow-up visits are usually scheduled every two weeks with weekly visits for complications and during the last four to six weeks.
Delivery is managed based on obstetrical considerations.
Spontaneous abortions, pyelonephritis, thrombophlebitis, and toxemia may be more common but management is the same as in patients without sickle syndromes.
Transfusions should be reserved for symptomatic anemia, increase in the frequency of pain episodes, and chest syndrome with hypoxia. Transfusion to a hemoglobin of 10 g/dl should also be done in patients receiving general anesthesia.
Preeclampsia and acute anemic episodes in HbSS women.are risk factors for infants who are small for gestational age.
Appropriate nutrition and avoidance of alcohol, drugs, smoking and sexually transmitted diseases should also be explained.
Contraception is controversial in sickle syndromes, however, there is no compelling evidence which precludes the use of any available method. The combination of a condom and spermicidal foam provides safe, effective contraception and may reduce the transmission of AIDS and other sexually transmitted diseases. Oral contraception is probably the most effective and is probably safe if low-dose estrogen preparations are used. The IUD is also effective. however, many avoid this form of contraception because of the potential increased risk of infection. Diaphragms are usually less effective, however, may be satisfactory if combined with a spermicidal preparation. Progesterone based prevention by pill or depo preparations is also effective.
Fathers should always be tested and the risk of having an affected child determined. It is very important to do both a CBC with indices and hemoglobin electrophoresis to detect structural hemoglobin variants and thalassemias. Non-directive counseling should be provided with information about prenatal diagnosis.
Rh(D) immune globulin should be given to Rh negative mothers after delivery, spontaneous abortion, or termination using standard guidelines.
Termination of pregnancy is accomplished by suction curettage in the first thirteen weeks. Hypertonic urea with prostaglandin F2 is safe in later pregnancies. Hypertonic saline induction should be avoided because of the potential for causing increased sickling that can theoretically cause complications.
All preadolescent children should receive information about prevention of pregnancy and sexually transmitted diseases. This should be periodically reinforced during the reproductive years. Patients who want children should be provided with accurate information about the potential for increased problems during pregnancy, the increased rate of spontaneous abortions, the risk for have affected children, and the physical and psychological stress associated with parenthood.
Accurate information about birth control options should be provided by experts. The availability of screening for the father and prenatal testing should be outlined.
During pregnancy, it is very important to educate the pregnant patient about avoiding medications, drugs, alcohol, smoking, and sexually transmitted disease. Appropriate information about an adequate, balance diet should be stressed.
Serjeant GR, Loy LL, Crowther M, Hambleton IR, Thame M. Outcome of Pregnancy in Homozygous Sickle Cell Disease. Obstet Gynecol. 2004 Jun;103(6):1278-1285. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15172865
Freeman MG, Ruth GJ. SS disease, SC disease, and CC disease - Obstetric considerations and treatment. Clin. Obstet. Gynecol. 12:134-1155, 1969.
Milner PF, Jones BR, Dobler J. Outcome of pregnancy in sickle cell anemia and sickle cell-hemoglobin C disease. Amer.J Obstet. Gynecol. 138:239-245, 1980.
Koshy M, Ashenhurst J. Management of pregnancy in sickle cell anemia. Texas Reports Reproductive Biol. Med. 40:273-282, 1980-1981.
De Ceulaer K, Hayes R, Gruber C, Serjeant GR. Medroxyprogesterone acetate in homozygous sickle cell disease. Lancet ii:229-231, 1982.
Sergeant GR. Sickle haemoglobin and pregnancy. Brit. Med. J 287:628-630, 1983.
Charache S, Niebyl JR. Pregnancy in sickle cell disease. Clin. Haematol. 14:729-745, 1985.
Powars DR, Sandhu M, Niland-Weiss, et al. Pregnancy in sickle cell disease. Obstet. Gynecol. 67:217-228, 1986.
Tuck SM, James CE, Brewster EM, et al. Prophylactic blood transfusion in maternal sickle cell syndromes. Brit. J.Obstet. Gynaecol. 94:121-125, 1987.
Koshy M, Burd L, Wallace D, et al. Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. New Engl. J. Med. 319:1447-1452, 1988.
Breckwoldt M, Wieacker P, Geisthovel F. Oral contraception in disease states. Amer. J. Obstet. Gynecol. 163:2213-2216, 1990.
Guillebaud J. Oral contraceptives in risk groups: Exclusion of monitoring. Amer. J. Obstet. Gynecol. 163:443-446, 1990.
Danzer BI, Birnbach DJ, Thys DM. Anesthesia for the parturient with sickle cell disease. J. Clin. Anesth. 8:598-602, 1996.
Howard R. Management of sickling conditions in pregnancy. Br. J. Hosp. Medicine. 56:7-10, 1996.
Smith JA, Espeland M, et al. Pregnancy in sickle cell disease: Experience of the cooperative study of sickle cell disease. Obstet. Gynecol. 87:199-204, 1996.