Pregnancy in sickle cell disease

Risks associated with pregnancy for mothers with sickle cell disease and their infants have decreased markedly during the last decade. Among 79 women with sickle cell anemia (156 pregnancies), maternal death decreased from 4.1% before 1972 to 1.7% after 1972; their infants' fetal and perinatal death rates decreased from 52.7 to 22.7% (P less than .05), and from 33.3 to 27.3% among infants of women with sickle hemoglobin C disease. There has been a significant improvement in birth weight specific mortality and an increase in number of weeks' gestation from 34.7 to 37.4 (P less than .05). A higher percentage of sickle hemoglobin C disease mothers completed their pregnancies with no complications (43%) when compared with sickle cell anemia mothers (21%), thus identifying a subset of women for whom pregnancy does not represent an increased risk. These results are attributed to improvements in state-of-the-art medical, obstetric, and perinatal care.     

Pregnancy in sickle cell disease in the UK

Summary. The complications and outcome of 125 pregnancies in women with sickle cell disease between 1975 and 1981 are reviewed. There were no maternal deaths, but a perinatal mortality rate of 48 per 1000. The main maternal complications were sickling crises (38%), anaemia (65%), infections (61%) and severe pregnancy-induced hypertensive disease (5%). There were three patients with eclampsia. There was a 13% spontaneous preterm delivery rate, 25% of the babies were'light-for-dates', fetal distress in labour was seen in 24% and 15% were delivered by caesarean section. There was an increased incidence of involuntary infertility and spontaneous abortion. The treatment by prophylactic blood transfusions is discussed.     

Sickle cell disease and pregnancy in Bahrain.

OBJECTIVE: To determine the morbidity and outcome of pregnancies complicated by homozygous sickle cell disease (SCD) in Bahrain. METHODS: A controlled study was conducted of all the pregnancies of women with SCD who were delivered at Salmaniya Medical Complex and affiliate hospitals in Bahrain between January 1, 1998, and December 31, 2002. RESULTS: There were 351 pregnancies with SCD, giving an incidence of 0.67%. The mean+/-S.D. age of women with SCD was 28.8+/-5.7 years. These women, who were mostly from Shia villages, had more spontaneous abortions than the 351 controls (32% and 12%, respectively). Compared with the control group, the mean+/-S.D. number of pregnancies among women with SCD was 5.0+/-1.9 vs. 5.7+/-3.1; birth weight, 2,965+/-540 g vs. 3,457+/-497 g; rate of preterm deliveries, 25.9% vs. 12%; incidence of intrauterine growth restriction, 10% to 13% vs. 4% to 7%; Apgar scores at 1 and 10 min, 8.1+/-0.8 and 8.8+/-0.8; and cesarean section rate, 19.0% vs. 12.7%. There were no differences in the rates of pre-eclampsia, antepartum and postpartum hemorrhage, or stillbirth. There were 4 maternal deaths directly related to SCD complications. Hemoglobin analysis was performed in 86% of the women with SCD. Of these women, 60.6% had homozygous SCD with raised levels of fetal hemoglobin (more than 5% HbF); 9.6% had sickle cell hemoglobin with beta-thalassemia disease; and 1.4% had sickle cell hemoglobin only. Vaso-occlusive crises were the most common cause of hospital admissions during pregnancy (42.2%), while hemolytic and sequestration crises accounted for 28.0% and 0.6% of admissions. CONCLUSION: Mortality, morbidity, and perinatal loss are still considerable among women with SCD in Bahrain. To reduce mortality and morbidity, there is a need for a multidisciplinary team able to deal with pregnancy complications due to SCD.     

Pregnancy in Jamaican women with homozygous sickle cell disease. Fetal and maternal outcome

The outcome of 664 pregnancies in 297 Jamaican women with homozygous sickle cell (SS) disease over the period 1959-1984 was reviewed. Overall, the spontaneous abortion rate was 118 per 1000 pregnancies, the stillbirth rate 128 per 1000 births, and perinatal mortality 171 per 1000 births. There was a striking secular increase in both spontaneous abortions and stillbirths which was accounted for, in part, by a trend for abortions to increase with maternal age up to the age of 30 years, a greater proportion of patients in the high-risk 25-29 year age group occurring in the 1980-1984 period. Another factor contributing to the poor obstetric performance in the 1980-1984 period was probably an increased survival and pregnancy rate in high-risk patients. Seven women had pregnancy-related deaths giving a pregnancy mortality rate of 1.1%.     

Pregnancy in sickle cell,anemia, sickle cell-hemoglobin C disease,and variants thereof

A simple, inexpensive, and yet effective instrument for obtaining circular biopsies of the squamocolumnar portion of the cervix is described.     

Pregnancy in Jamaican women with homozygous sickle cell disease. Fetal and maternal outcome

Summary. The outcome of 664 pregnancies in 297 Jamaican women with homozygous sickle cell (SS) disease over the period 1959–1984 was reviewed. Overall, the spontaneous abortion rate was 118 per 1000 pregnancies, the stillbirth rate 128 per 1000 births, and perinatal mortality 171 per 1000 births. There was a striking secular increase in both spontaneous abortions and stillbirths which was accounted for, in part, by a trend for abortions to increase with maternal age up to the age of 30 years, a greater proportion of patients in the high-risk 25–29 year age group occurring in the 1980–1984 period. Another factor contributing to the poor obstetric performance in the 1980–1984 period was probably an increased survival and pregnancy rate in high-risk patients. Seven women had pregnancy-related deaths giving a pregnancy mortality rate of 1.1%.     

Pregnancy in Jamaican women with homozygous sickle cell disease. Fetal and maternal outcome

Summary. The outcome of 664 pregnancies in 297 Jamaican women with homozygous sickle cell (SS) disease over the period 1959–1984 was reviewed. Overall, the spontaneous abortion rate was 118 per 1000 pregnancies, the stillbirth rate 128 per 1000 births, and perinatal mortality 171 per 1000 births. There was a striking secular increase in both spontaneous abortions and stillbirths which was accounted for, in part, by a trend for abortions to increase with maternal age up to the age of 30 years, a greater proportion of patients in the high-risk 25–29 year age group occurring in the 1980–1984 period. Another factor contributing to the poor obstetric performance in the 1980–1984 period was probably an increased survival and pregnancy rate in high-risk patients. Seven women had pregnancy-related deaths giving a pregnancy mortality rate of 1·1%.     

Pregnancy in patients with sickle cell disease: maternal and perinatal outcomes

Objective: To compare obstetrical, hematological and neonatal outcomes of pregnant women with or without sickle cell disease (SCD).

Methods: A prospective study of 60 pregnancies of 58 women with SCD (29 SCD-SS and 29 SCD-SC) compared with 192 pregnancies in 187 healthy pregnant women was carried out from January 2009 to August 2011.

Results: Compared to controls, the SCD group had higher rate of preterm delivery (p?p?p?=?0.003), and urinary infection (p?=?0.001, OR?=?3.31, CI 1.63–6.73), higher prevalence of small for gestational age babies (p?=?0.019, OR?=?2.66, CI 1.15–6.17), and more frequent baby admissions to progressive care unit (p?p?=?0.056). All adverse events were more frequent in the SS subgroup. Babies from the SS subgroup had the lowest weight at birth (2080?g) compared to SC (2737?g; p?Conclusion: SCD pregnant women – especially those in the SS subgroup – are more prone to experience perinatal and maternal complications in comparison with pregnant women without SCD.     

Pregnancy in women with sickle cell trait

Summary. The complications and outcome of 334 pregnancies in women with sickle cell trait beween 1975 and 1981 are reviewed. The perinatal mortality rate was 6 per 1000. The main maternal complications were recurrent urinary tract infections (6%) and haematuria (16.5%). Fetal distress in labour was seen in 17% and 11.5% were delivered by emergency caesarean section. Compared with a comparable group of women without sickle cell trait, the mean birthweight of the babies (3202 g) was not reduced. The mean age of the mothers (25 years), their mean parity (1.46) and the percentage who were para 4 or more (13%) were all significantly increased, with important implications for the continuing transmission of the sickle cell gene.     

Pregnancy outcomes in systemic sclerosis, primary pulmonary hypertension, and sickle cell disease

OBJECTIVE: Systemic sclerosis, primary pulmonary hypertension, and sickle cell disease are uncommon vasculopathic diseases affecting women. We estimated the nationwide occurrence of pregnancies in women with these conditions and compared pregnancy outcomes to the general obstetric population. METHODS: We studied the 2002-2004 Nationwide Inpatient Sample, of the Healthcare Cost and Utilization Project to estimate the number of obstetric hospitalizations and deliveries among women with systemic sclerosis, primary pulmonary hypertension, sickle cell disease, and women in the general population. Pregnancy outcomes included length of hospital stay, hypertensive disorders including preeclampsia, intrauterine growth restriction (IUGR), and cesarean delivery. Multivariable regression analyses were performed using maternal age, race or ethnicity, antiphospholipid antibody syndrome, diabetes mellitus, and renal failure as covariates. RESULTS: Of an estimated 11.2 million deliveries, 504 occurred in women with systemic sclerosis, 182 with primary pulmonary hypertension, and 4,352 with sickle cell disease. Systemic sclerosis, was associated with an increased risk of hypertensive disorders including preeclampsia (odds ratio [OR] 3.71, 95% confidence interval [CI] 2.25-6.15), IUGR (OR 3.74, 95% CI 1.51-9.28), and increased length of hospital stay. Primary pulmonary hypertension was associated with an increase in the odds of antenatal hospitalization (OR 4.67, 95% CI 2.88-7.57), hypertensive disorders including preeclampsia (OR 5.62, 95% CI 2.60-12.15) and a substantial increase in length of hospital stay. Sickle cell disease was associated with an increased odds of antenatal hospitalization (OR 5.56 95% CI 5.08-6.09), hypertensive disorders including preeclampsia (OR 1.78, 95% CI 1.48-2.14), and IUGR (OR 2.91, 95% CI 2.16-3.93), with a modest increase in length of hospital stay. CONCLUSION: Women with systemic sclerosis, primary pulmonary hypertension, and sickle cell disease have significantly increased rates of adverse pregnancy outcomes, requiring extensive preconceptional counseling about the risks of pregnancy.     

Pregnancy associated with both insulin-dependent diabetes mellitus and sickle cell disease. A report of two cases

Two pregnant women were treated for both insulin-dependent diabetes and sickle cell disease. Careful application of treatments developed for each of these conditions allowed both pregnancies to be successfully carried to term.     

Prophylactic transfusion in sickle cell disease

This prospective study was designed to compare the relative efficacy of two antibiotic regimens for the treatment of operative site infections subsequent to pelvic operations. Patients with endomyoparametritis after delivery or pelvic cellulitis subsequent to hysterectomy were randomized to treatment with the combination of penicillin-gentamicin or the single agent cefoxitin. Seventeen of the 26 patients (65%) with endomyoparametritis who were treated with penicillin-gentamicin were cured by antibiotic therapy alone, in comparison to 15 of 23 (65%) patients treated with cefoxitin. Fifty-eight percent of the patients with pelvic cellulitis who were treated with penicillin-gentamicin responded favorably, in comparison to 50% of the patients treated with cefoxitin. None of these differences was statistically significant. In this study, neither antibiotic regimen provided satisfactory initial treatment for surgically induced soft tissue pelvic infection. Moreover, 11 of the 28 patients with treatment failures (40%) developed serious sequelae of their primary infection.     

Third-trimester erythrocytapheresis in pregnant patients with sickle cell disease.

OBJECTIVE: To evaluate the effects of prophylactic transfusion by means of erythrocytapheresis at the beginning of the third trimester of pregnancy in women with sickle cell disease (SCD). METHODS: A cohort of 14 pregnant women with SCD who received prophylactic erythrocytapheresis transfusions at the beginning of the third trimester was retrospectively compared with a cohort of 17 pregnant women who received simple prophylactic transfusions for no indication other than SCD severity. RESULTS: Prophylactic erythrocytapheresis transfusions were associated with a lower risk of intrauterine growth restriction (OR, 0.11; 95% confidence interval, 0.01-1.00) and oligohydramnios (OR, 0.65; 95% confidence interval, 0.45-0.92) in pregnant women with SCD. CONCLUSION: These results suggest that erythrocytapheresis transfusions are beneficial in women with SCD who are in the third trimester of pregnancy. Given the decrease in transfusion risks, this therapy deserves further evaluation in future trials.     

Pregnancy in hemoglobin sickle cell patients at the University College Hospital, Ibadan.

A retrospective review of pregnancy outcome in hemoglobin (Hbsc) patients managed at the University College Hospital, Ibadan over a 5-year period (1984-1988) was carried out. The main antenatal complications included anemia (51.2%), bacterial infection (22.0%), bone pain crisis (7.3%) and preeclampsia (2.4%). Intrapartum complications included anemia (29.2%), bone pain crisis (12.2%) and pseudotoxemia (4.9%). The maternal and perinatal mortality rate were 48 and 195 per 1000, respectively. The duration of labor and operative delivery rate were not different from the general population.     

Pregnancy outcome in patients with homozygous sickle cell disease in a university hospital,Eastern Saudi Arabia

Objective  

To evaluate the maternal and fetal outcome in pregnant women with sickle cell disease and to highlight the complications encountered during pregnancy and delivery at a university hospital in the Eastern Saudi Arabia.