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Sickle cell crisis pregnancy

Cathi Phillips, Margaret Peggy Boyd
Sickle cell disease is the genetic disorder most commonly detected with state-mandated newborn screening. Women with sickle cell disease struggle with psychosocial, emotional, and physical challenges throughout their lives. Pregnancy for women with sickle cell disease brings greater risk for maternal and fetal morbidity and mortality and increased likelihood of hospitalization for complications, including sickle cell pain crisis. Chronic maternal opioid use for pain can place newborns at risk for neonatal abstinence syndrome...
December 2017: Nursing for Women's Health
Sanaa Rizk, Elizabeth D Pulte, David Axelrod, Samir K Ballas
As outcomes of patients with sickle cell anemia improve and survival into adulthood with good quality of life and expectation of long-term survival becomes more common, challenges have developed, including issues related to reproduction. Pregnancy is frequently complicated in patients with sickle cell anemia with mortality up to 4.0%. Here we report maternal perinatal mortality in two women with sickle cell anemia who died post-partum due to acute chest syndrome (ACS), caused by bone marrow fat embolism and review the literature pertinent to this subject...
July 2017: Hemoglobin
Alexis C Gimovsky, Kate Fritton, Eugene Viscusi, Amanda Roman
Sickle cell crises occur frequently during pregnancy and are difficult to treat, even with high-dose opioids. Analgesia with ketamine has been suggested as an alternative, but its use during pregnancy is underreported. Two pregnant patients with uncontrolled sickle cell pain were treated with ketamine. Patient A reported no decrease in her pain, but her opioid requirements decreased. Patient B's pain resolved during ketamine administration. No serious maternal or neonatal adverse effects occurred. Ketamine may be considered as an adjunct analgesic in pregnant patients with sickle cell pain, although prospective clinical data are needed to fully assess its efficacy...
January 1, 2018: A&A practice
Michelle Matthews, Rebecca Pollack
PURPOSE: Hematopoietic stem cell transplantation (HSCT) is a cure for sickle cell disease (SCD) but frequently results in permanent sterility. The complications associated with oocyte cryopreservation and risks of future pregnancy are increased in SCD patients. This case report discusses risk reduction strategies and includes a literature review of pregnancy after HSCT. CASE: A 23-year-old woman underwent ovarian stimulation for fertility preservation resulting in cancelation due to acute pain crisis...
November 2017: Journal of Assisted Reproduction and Genetics
Soyoung Choi, Adam M Bush, Matthew T Borzage, Anand A Joshi, William J Mack, Thomas D Coates, Richard M Leahy, John C Wood
Sickle cell disease (SCD) is a life-threatening genetic condition. Patients suffer from chronic systemic and cerebral vascular disease that leads to early and cumulative neurological damage. Few studies have quantified the effects of this disease on brain morphometry and even fewer efforts have been devoted to older patients despite the progressive nature of the disease. This study quantifies global and regional brain volumes in adolescent and young adult patients with SCD and racially matched controls with the aim of distinguishing between age related changes associated with normal brain maturation and damage from sickle cell disease...
2017: NeuroImage: Clinical
Vinod Patil, Gamunu Ratnayake, Galina Fastovets
PURPOSE OF REVIEW: The current review outlines the challenges in managing pregnant women with sickle-cell anemia, who are at risk of becoming critically ill during pregnancy. RECENT FINDINGS: Sickle obstetric patients pose unique challenges to the anesthetist and intensivist. We discuss the role of prophylactic transfusions for specific indications like acute anemia and twin pregnancies. The management and prevention of vaso-occlusive crises and chest crisis are also outlined...
June 2017: Current Opinion in Anaesthesiology
Russell E Ware, Mariane de Montalembert, Léon Tshilolo, Miguel R Abboud
Sickle cell disease is a common and life-threatening haematological disorder that affects millions of people worldwide. Abnormal sickle-shaped erythrocytes disrupt blood flow in small vessels, and this vaso-occlusion leads to distal tissue ischaemia and inflammation, with symptoms defining the acute painful sickle-cell crisis. Repeated sickling and ongoing haemolytic anaemia, even when subclinical, lead to parenchymal injury and chronic organ damage, causing substantial morbidity and early mortality. Currently available treatments are limited to transfusions and hydroxycarbamide, although stem cell transplantation might be a potentially curative therapy...
July 15, 2017: Lancet
Babasola O Okusanya, Olufemi T Oladapo
BACKGROUND: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies...
December 22, 2016: Cochrane Database of Systematic Reviews
Manickam Niraimathi, Rakhee Kar, Sajini Elizabeth Jacob, Debdatta Basu
Vaso-occlusive crisis in sickle cell anaemia is one of the commonest presentations and a leading cause of death. Death can be sudden and unexpected. Herein we present three cases of sickle cell anaemia with sudden death within 3 days of hospitalisation. All the three cases presented with fever and jaundice. Two cases presented consecutively in the same year within a span of 5 months while the other case had presented 2 years prior to these two cases. Infection was the precipitating event in two cases and pregnancy with infection in one...
June 2016: Indian Journal of Hematology & Blood Transfusion
Narcisse Elenga, Aurélie Adeline, John Balcaen, Tania Vaz, Mélanie Calvez, Anne Terraz, Laetitia Accrombessi, Gabriel Carles
Sickle cell disease is a serious genetic disorder affecting 1/235 births in French Guiana. This study aimed to describe the follow-up of pregnancies among sickle cell disease patients in Cayenne Hospital, in order to highlight the most reported complications. 62 records of pregnancies were analyzed among 44 females with sickle cell disease, between 2007 and 2013. Our results were compared to those of studies conducted in Brazil and Guadeloupe. There were 61 monofetal pregnancies and 2 twin pregnancies, 27 pregnancies among women with SS phenotype, 30 SC pregnancies, and five S-beta pregnancies...
2016: Obstetrics and Gynecology International
Paolo Perseghin
Hematological diseases in pregnancy should be carefully managed with a multidisciplinary approach, which should include obstetrics, hematology and, in selected patients, apheresis professionals. Hematological malignancies in pregnant women are rare, but the attending physicians should be aware that the use of cytotoxic drugs, tyrosine-kinase inhibitors or differentiating agents such as all-trans retinoic acid (ATRA) during the first trimester of pregnancy might be teratogenic and, in turn, induce fetal abnormalities or abortion...
December 2015: Transfusion and Apheresis Science
Nicholas J Schott, Mark H Yazer, Robert Krohner, Jonathan H Waters
Cell salvage is a process whereby the bloodshed from the operative field is collected and returned to the patient. It can be especially useful when allogeneic red blood cell (RBC) units are not readily available such as when the recipient has multiple alloantibodies. We report on the anesthesia and transfusion strategies for managing a pregnant patient with sickle cell disease (SCD) with HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome. A pregnant patient with twins at 30 weeks of gestation was admitted in an SCD crisis...
December 2014: Journal of Extra-corporeal Technology
Craig D Seaman, Jonathan Yabes, Jie Li, Charity G Moore, Margaret V Ragni
INTRODUCTION: The risk of venous thromboembolism (VTE) is higher during pregnancy, with an incidence between 0.05 and 0.2%, and among persons with sickle cell disease (SCD), yet the rates and risk factors, such as pneumonia, vasooclusive crisis (VOC), and acute chest syndrome (ACS), associated with pregnancy-related VTE are not firmly established in SCD. METHODS: Inpatient hospital discharge data from 2007-2011 were obtained from the Pennsylvania Health Care Cost Containment Council to estimate the rate of VTE among African American delivery hospitalizations with SCD and to compare pregnancy complications and medical comorbidities among pregnant women with SCD...
December 2014: Thrombosis Research
Suheyl Asma, Ilknur Kozanoglu, Ebru Tarım, Cagla Sarıturk, Cigdem Gereklioglu, Aydan Akdeniz, Mutlu Kasar, Nurhilal H Turgut, Mahmut Yeral, Fatih Kandemir, Can Boga, Hakan Ozdogu
BACKGROUND: Sickle cell disease (SCD) is associated with chronic hemolysis and painful episodes. Pregnancy accelerates sickle cell complications, including prepartum and postpartum vasoocclusive crisis, pulmonary complications, and preeclampsia or eclampsia. Fetal complications include preterm birth and its associated risks, intrauterine growth restriction, and a high rate of perinatal mortality. The purpose of this study was to evaluate pregnancy outcomes in patients with SCD who underwent planned preventive red blood cell exchange (RBCX)...
January 2015: Transfusion
Khushnooma Italia, Dipti Upadhye, Pooja Dabke, Harshada Kangane, Stacy Colaco, Pratibha Sawant, Anita Nadkarni, Ajit Gorakshakar, Dipty Jain, Yazdi Italia, Kanjaksha Ghosh, Roshan Colah
BACKGROUND: Co-inheritance of structural hemoglobin variants like HbS, HbD(Punjab) and HbE can lead to a variable clinical presentation and only few cases have been described so far in the Indian population. METHODS: We present the varied clinical and hematological presentation of 22 cases (HbSD(Punjab) disease-15, HbSE disease-4, HbD(Punjab)E disease-3) referred to us for diagnosis. RESULTS: Two of the 15 HbSD(Punjab) disease patients had moderate crisis, one presented with mild hemolytic anemia; however, the other 12 patients had a severe clinical presentation with frequent blood transfusion requirements, vaso occlusive crisis, avascular necrosis of the femur and febrile illness...
April 20, 2014: Clinica Chimica Acta; International Journal of Clinical Chemistry
Nada Alayed, Abbas Kezouh, Lisa Oddy, Haim A Abenhaim
OBJECTIVE: To estimate the prevalence of sickle cell disease (SCD) in pregnancy, and to measure risk factors, morbidity, and mortality among women with SCD with and without crisis at the time of birth. METHODS: We conducted a population-based, retrospective cohort study on all births in the Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP-NIS) from 1999 to 2008. Births to SCD with and without crisis were identified using ICD-9 codes. Adjusted effects of risk factors and outcomes were estimated using logistic regression analyses...
July 2014: Journal of Perinatal Medicine
Babasola O Okusanya, Olufemi T Oladapo
BACKGROUND: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies...
December 3, 2013: Cochrane Database of Systematic Reviews
Mayoor M Daigavane, Rabindra K Jena, Tushar J Kar
Sickle cell anemia, the homozygous genotype of sickle cell disease is one of the most common heritable diseases in the world. The Arab-Asian haplotype present in India is one of the least severe of all haplotypes. Many sickle cell anemia patients are now leading a symptom-free productive life due to hydroxyurea (HU) and better supportive care. Although pregnancy in sickle cell anemia patients is considered a high-risk category, it perinatal outcome is least studied, particularly among carriers of the Arab-Asian haplotype...
2013: Hemoglobin
Marc R Parrish, John C Morrison
Pregnant women with sickle cell disease appear to be more likely to experience antepartum, intrapartum, and postpartum complications when compared with unaffected women. Access to high-risk obstetric care, patient education, and close follow-up is important to minimize maternal morbidity and mortality. A high index of suspicion and good diagnostic acumen is necessary to obtain optimal results in the pregnant patient affected by sickle cell crisis.
August 2013: Seminars in Perinatology
Shumaila Zia, Muhammad Rafique
OBJECTIVE: To compare the pregnancy and neonatal outcome in sickle cell disease and trait women. METHOD: The retrospective comparative study was conducted at Abha General Hospital, Abha, Saudi Arabia, from January 2009 to December 2011. The records of women having sickle cell disease and trait were reviewed for antenatal and postnatal complications. Pregnancy and foetal outcome was also compared in both the groups. Chi-square, Fisher's Exact and Student's t tests were used for statistical analysis...
June 2013: JPMA. the Journal of the Pakistan Medical Association
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