Lothe S*, Hatkar P**, Satia MN***
(* Second Year Resident, **Associate Professor, *** Professor, Department of Obstetrics and Gynecology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India.)
Pregnancy in a woman with sickle cell disease (SCD) is associated with marked medical as well as obstetrics risk to both the mother and the baby. Combined efforts of multidisciplinary team including good obstetrician, expert perinatal team, hematologist and attentive physician, with a well devised therapeutic plan, in a setting of comprehensive tertiary care hospital can significantly decrease maternal and perinatal morbidity and mortality. This is an illustration of a case of a 27 year old primigravida with 29 weeks gestation who presented to us in sickle cell crisis, managed successfully with good maternal and fetal outcome.
Sickle cell disease (SCD) is an abnormality of red blood cells that may result in circulatory impairment, tissue damage, severe anemia, infarction and life threatening infection. Some patients with SCD have no change in disease activity while some have worsening disease during pregnancy. Changes in pregnancy like higher metabolic demand, hyper-coagulable state and vascular stasis may predispose to complications. Factors like infection, fever, dehydration, cold, overexertion, stress, acidosis, prolonged labor, operative delivery may precipitate a sickle cell crisis. A woman with sickle cell disease has higher chances of maternal and fetal complications as compared to one without sickle cell disease. Pre-conceptional screening, early registration, regular prenatal care, good intrapartum and postpartum management may help to successfully decrease the morbidity and mortality.
27 years old primigravida with 29 weeks by dates, corresponding by scan, came to casualty with complaints of severe pain in knee and shoulder joints associated with backache and fever with chills since four days. There were no complaints of bleeding from any site. She had similar complaints on and off since childhood. She was not in labor. She gave history of pneumonia at 10 years of age for which she was hospitalized and two units of blood were transfused. Presently patient was admitted in view of febrile illness with severe generalized body pain. On general examination she was markedly pale, there was no lymphadenopathy, JVP was not raised and vital parameters were stable. On respiratory system examination bilateral basal crepitations were present. On per abdominal examination uterus was 28 weeks, relaxed, cephalic presentation and fetal heart sounds were regular. On per vaginal examination os was 1.5 cm dilated, poorly effaced.
Blood investigations revealed hemoglobin (Hb) of 5.1 gm%, total white blood count 46900/mm3 suggestive of leukemoid reaction, and platelet count of 60000/mm3. Peripheral smear examination showed sickle cells, tear drop cells, fragmented red blood cells, Howell Jolly bodies and macrocytes with basophilic stippling from which inference of sickle cell disease was drawn. Provisional diagnosis of sickle cell disease with vaso-occlusive crisis was made. Hemoglobin electrophoresis was done which was suggestive of sickle cell anemia (HbSS). Conservative and symptomatic management was started. Adequate hydration, analgesia with injectable opioids and therapy to prevent acidosis was given, and intravenous antibiotics were started. TSH was normal. In view of Hb 5.1 gm% three units of blood were transfused. After 3 days, repeat investigations were done and Hb was 7.5 gm%, total blood count was 24000/ mm3 and platelet count was 120000/ mm3.
On day 5 of admission patient went in spontaneous labor. Adequate analgesia, oxygenation and hydration were given during labor. Patient delivered uneventfully, a female baby of 1.090 kg with Apgar score of 9/10. Baby was immediately shifted to neonatal intensive care unit due to prematurity and low birth weight. Post-delivery investigations showed raised total white blood counts, hence the patient was stared on oral antibiotics. Hemoglobin was 8.5 gm%. Patient had fever on day 3 after delivery thus was switched over to injectable antibiotics and symptomatic treatment was given. Husband’s sickling test was done which suggested normal study. The patient was symptomatically better, fever subsided thus she was discharged on day 10 post delivery. Baby was kept in neonatal intensive care unit, evaluated for sickle cell disease, gained weight and discharged on day 21 post delivery. Baby was found to have sickle cell trait during evaluation. Patient was counseled regarding future prognosis of baby and was advised to follow up with the pediatrician.
Sickle cell disease is a group of autosomal recessive disorders which affect the hemoglobin structure; it is inherited as a single gene defect. It is the most common inherited condition worldwide. Most of the pregnancies with sickle cell disease result in live birth but they are at increased risk of fetomaternal complications as well as medical complications of sickle cell disease.[2,3] Vaso-occlusive crisis is the most common complication that may be encountered in a pregnant woman with sickle cell disease. The term SCD comprises of sickle cell anemia (HbSS) and other heterozygous conditions of hemoglobin including combination with hemoglobin C (HbSC), combination with beta thalassemia (Hb Thalassemia) and other combinations with D, E or O-Arab. All these give similar phenotype but severity differs according to the condition. The combination of hemoglobin S with hemoglobin A, known as sickle cell trait (SA) is asymptomatic except for increased chances of urinary tract infections in this condition. SCD has its origin in Sub Saharan Africa and Middle East, hence it is prevalent in persons of African descent, southern Europeans, Middle Eastern and Asian Indians. Due to migrating population, SCD is increasing in importance worldwide. About 3,00,000 children are born with SCD each year.
Acute complications in mother include acute chest pain syndrome, ischemic vaso-occlusive crisis, splenic sequestration, stroke, cholecystitis, acute renal failure. Chronic complications like chronic pain, cholelithiasis, retinal problems, pulmonary hypertension, renal dysfunction, venous thromboembolism can occur. Due to renal dysfunction, hypertension and placental ischemia, preeclampsia and eclampsia may occur. Fetal manifestations are due to uteroplacental insufficiency, opioid exposure for analgesia and alloimmunization. Studies have documented high risk of intrauterine growth restriction, preterm birth, low birth weight baby and stillbirth in cases of SCD. 50-70% of women with sickle cell disease require hospitalization at least once in their life and 30-40% of them require blood transfusion. Due to decreased oxygen carrying capacity of the sickle red blood cells, restricted fetal growth and maternal organ damage may result. Couple planning for a pregnancy should be aware of the increased risk to the woman’s health with SCD during pregnancy. Pre- conception counseling regarding the risks, mandatory investigations to be done, care during pregnancy and possible maternal and fetal outcome should be offered to each and every couple. If the patient is on hydroxyurea it should be stopped before 3 months of pregnancy or as soon as pregnancy is confirmed. Pregnancy is contraindicated or if occurs, should be terminated if the patient has pulmonary hypertension. During pregnancy, complete blood count, renal and liver function tests, 24 hours urine protein, retinal examination, 2D Echocardiography to rule out pulmonary hypertension, BP monitoring should be regularly done. Immunization should be given to the patient with pneumococcal, influenza type B and meningococcal vaccines as women with SCD have more susceptibility to these infections, especially if they have functional asplenia. Iron levels should be done before giving iron therapy to the patient. Fetal surveillance should include routine USG in 1st and 2nd trimester, serial USG for growth at interval of 3-4 weeks starting from 24 weeks and fetal testing at 32 weeks or sooner if indicated.
On presentation rapid assessment of the patient should be done for medical complications like sepsis, dehydration, pain crisis or acute chest syndrome requiring urgent intervention. History should be revealed if there are any precipitating factors. All mandatory investigations should be done. Some patients may require blood transfusion and in some patients prophylactic blood transfusion may be given to prevent the crisis. If the patient is in pain crisis aggressive management of pain with opioids should be done. Adequate hydration, oxygenation and prevention of acidosis should be done. Various guidelines such as Greentop guidelines by Royal College of Obstetrics And Gynecology, National Guideline Clearinghouse guidelines have been given for the management protocol for SCD in pregnancy. Mode of delivery should be decided according to the obstetric indications while recognizing that the cesarean rates are higher in cases with SCD. [2,7,8] Allogenic hematopoietic stem cell transplantation is the only definitive therapy with reported overall survival rate of 92 - 94% and event -free survival rate of 82 - 86%, but it should not be done in pregnancy. In conclusion, maternal and fetal morbidity secondary to this serious obstetric problem can be significantly reduced with careful attention to obstetric and medical details with an expert perinatal team and well devised plan in a setting of tertiary care.
Sickle Cell Disease In Pregnancy, Management of (Green Top Guidelines No 61), Royal College Of Obstetricians and Gynaecologists published on 26/08/2011 available from http://www.rcog.org.uk/en/guidelines-research-services/guidelines/gtg 61/
Villers MS, Jamison MG, De Castro LM, James AH. Morbidity associated with sickle cell disease in pregnancy. Am J Obstet Gynecol 2008; 199(2):125.e1-5.
Pregnancy, contraception and fertility. In: Standards for the Clinical Care of Adults with Sickle Cell Disease in the UK, Pub: Sickle Cell Society UK 2008; 59-68.
Martin JN Jr, Martin RW, Morrison JC. Acute management of sickle cell crisis in pregnancy. Clin Perinatol. Dec 1986;13(4): 853-68.
Angastiniotis M, Modell B, Englezos P, Boulyjenkov V. Prevention and control of hemoglobinopathies. Bull World Health Organ 1995;73(3): 375-86.
Yawn BP, Buchanan GR, Afenyi-Annan AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA Sep 2014; 312(10):1033-1048.
Ngo C, Kayem G, Habibi A, et al. Pregnancy in sickle cell disease: maternal and fetal outcomes in a population receiving prophylactic partial exchange transfusions. Eur J Obstet Gynecol Reprod Biol. Oct 2010; 152(2):138-142.
Barfield WD, Barradas DT, Manning SE, Kotelchuck M, Shapiro-Mendoza CK. Sickle cell disease and pregnancy outcomes: women of African descent. Am J Prev Med. Apr 2010; 38(4 Suppl): S542–9.
Locatelli F, Pagliara D. Allogenic hematopoitic stem cell transplantation in children with sickle cell disease. Pediatr Blood cancer. Aug 2012; 59(2): 372-6.
Lothe S, Hatkar P, Satia MN. JPGO 2015. Successful Pregnancy Outcome In A Case Of Sickle Cell Crisis Volume 3 No. 4. Available from: http://www.jpgo.org/2016/04/successful-pregnancy-outcome-in-case-of.html