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ABSTRACT

BACKGROUND: Pregnant women with sickle cell disease are known to experience higher incidences of pregnancy-related complications compared to the general population. Conflicting findings on the relationship between sickle cell disease and pregnancy complications were found with distinct variations noted from differing characteristics such as genotypes, economic background, and midwife interventions.

OBJECTIVES: We carried out a meta-analysis and systemic review to evaluate perinatal outcomes among women with SCD and the role of midwives.

SEARCH STRATEGY: Various medical databases were used for the study using keywords such as SCD and maternal and fetal complications.

SELECTION CRITERIA: The review used full research articles with studies carried out in the UK. The studies were published in English and compared to pregnant women with SCD and those without SCD as the control group.

DATA COLLECTION AND ANALYSIS: Comprehensive meta-analysis strategies were used to abstract and analyse the data. Primary outcomes considered for the study included maternal and fetal mortality. Secondary outcomes included preterm delivery, caesarean section, low birth weight and small for gestational age, pre-eclampsia, and eclampsia, and bacterial infection. P-score and confidence intervals were used to calculate the effects.

MAIN RESULTS: Ten studies that met the requirements of the selection criteria were included in the systemic review. SCD was linked to increased cases of fetal mortality. Also, SCD was associated with high cases of pre-eclampsia and eclampsia cases. The studies showed a link between maternal mortality and SCD. Only one of the studies examined the role of midwives during the perinatal management of pregnancy among SCD mothers. The systemic review determined that there is a strong association between sickle cell disease and adverse outcomes in pregnant cases mother (pre-eclampsia and maternal mortality), and baby (small for gestational age, preterm births, and perinatal deaths). An analysis of the different genotypes shows that women with HbSS disease have worse perinatal outcomes of the complications studied compared to the HbSC genotype. Although the cases of pregnancy-related complications secondary to SCD have reduced in the UK, the cases are still high in low-income settings when compared with the high-income areas.

 

CONCLUSION: Pregnant women with SCD have a higher probability of developing pregnancy-related complications compared to women without SCD.

KEYWORDS: Perinatal outcome, Sickle-cell Disease, Pregnancy.

 

LIST OF CONTENTS

ACKNOWLEDGMENTS.. 2

LIST OF CONTENTS.. 5

1.0 INTRODUCTION.. 7

1.1 ETHICAL CONSIDERATIONS.. 8

2.0 LITERATURE REVIEW… 8

2.1 Antepartum.. 8

2.2 Intra-partum.. 11

2.3 Postpartum.. 12

3.0 METHODS.. 13

3.1 Data Sources. 13

3.2 Data Extraction.. 13

3.3 Quality Assessment 13

3.4 Study Selection.. 14

3.5 Data Analysis. 14

4.0 RESULTS.. 15

4.1 Study Characteristics. 15

4.2 Antenatal Outcomes. 15

4.2.1 Pre-Eclampsia. 16

4.2.2 Intrauterine Grown Restriction.. 16

4.3 Intra-Partum.. 16

4.3.1 Preterm Delivery. 16

4.3.2 Caesarean Section Delivery. 17

4.4 Postpartum.. 17

4.4.1 Maternal Mortality. 17

4.4.2 Fetal Mortality. 18

4.4.3 Low Birth Weight 19

4.4.4 Bacterial Infection.. 19

5.0 DISCUSSION.. 19

6.0 STRENGTHS AND LIMITATIONS OF THE STUDY.. 23

7.0 INTERPRETATION.. 23

8.0 RECOMMENDATIONS.. 24

9.0 CONCLUSION.. 24

APPENDICES.. 29

 

 

 

1.0 INTRODUCTION

According to a 2019 report by National Perinatal Epidemiology Unit (NPEU), 10 in every 10,000 women die annually in the United Kingdom due to pregnancy-related complications to include haemoglobinopathies (P. 10). Sickle cell disease (SCD), according to statistics, is the most prevalent haemoglobinopathy in the United Kingdom. It presents with chronic anaemia, the phenotype of haemolysis, and vaso-occlusive complications (Jain et al., 2019). Women diagnosed with SCD have a high probability of developing pregnancy- and sickle-related complications during the peripartum period. The co-occurrence of SCD and pregnancy leads to high cases of morbidity and mortality during the perinatal period. This systemic review aims to analyse the fetal and maternal outcomes while identifying the role of the midwife during the perinatal period.

A cohort study carried out in 2014 by Oteng-Ntim et al. to review fetal and maternal outcomes among pregnant women with SCD established that this population presented with high incidences of sickle-cell related complication with a high percentage having anaemia during their initial visit, many requiring blood transfusion, and acute chest syndrome (ACS). According to the study, 50% of the SCD pregnant women reported a vaso-occlusive crisis during the antenatal period. 20% of the participants required a high dependency unit (HDU) services during the perinatal period. The research also found that there were increased pregnancy-related complications in this population to include venous thromboembolism, hypertensive disease, renal insufficiency, and a high incidence of fetal complications with the most prevalent being small for gestation and premature babies. A meta-analysis of complications occurring during pregnancy among SCD mothers found out that there is an increase in complications such as small for gestation, pre-eclampsia, stillbirth, and preterm delivery among this population compared to the other mothers. Other studies show a higher rate of admissions during the antepartum period, premature rupture of membranes, and sepsis after delivery in this population. To the researcher’s knowledge, no study has addressed the role of midwives during the perinatal period in the management of SCD pregnant women.

This review showed high rates of health-related complications and fetal mortalities among pregnant women with SCD. The reviewed studies indicated high incidences of pregnancy-related complications among women with HbSS compared to those with the HbSC and Hgb A. Additionally, women from lower economic classes had more complications compared to SCD mothers in high economic regions. The studies also established a correlation between SCD and maternal mortality.

1.1 ETHICAL CONSIDERATIONS

  • The researcher ensured that the authors of the articles used in the reviews were acknowledged.
  • There were no conflicts of interest during the analysis.
  • The Cochrane handbook was used as a guiding tool during the systemic analysis.
  • The review has no plagiarised materials.

2.0 LITERATURE REVIEW

SCD is a group of inherited single-gene autosomal recessive disorders caused by the ‘sickle’ gene that affects the haemoglobin structure (Bramilla, 2005, p. 2770). SCD is thought to have its origins from the Middle East and sub-Saharan Africa. Notably, SCD has seen increased numbers worldwide, especially in Western and European countries. SCD comprises of sickle cell anaemia (HbSS), heterozygous conditions, and abnormal haemoglobins such as haemoglobin C (HbSC), beta thalassaemia (HbSB thalassaemia). Haemoglobin S, when combined with normal haemoglobin is referred to as sickle trait (AS) and is usually asymptomatic (De Franceschi et al., 2012, p. 755). However, individuals with this trait often have a risk of developing urinary tract infections and other cases, microscopic haematuria.

SCD is considered to have the highest number of inherited cases in the global scene. There are more than 15,000 affected people in the UK (Shah et al., 2019). It is estimated that 100-200 pregnancies among SCD mothers occur annually in the UK.

SCD pathophysiology is a result of polymerisation of abnormal haemoglobin when one experiences low-oxygen conditions resulting in the formation of fragile, rigid sickle-shaped red blood cells (DeBaun and Kirkham, 2016, p. 833). The sickled cells are more prone to breakdown, causing haemolytic anaemia leading to vaso-occlusion in micro-vessels. Effects of vaso-occlusion include leg ulcers, stroke, renal dysfunction, avascular necrosis, hypertension, and cholelithiasis (Forni et al., 2014, p. 91). The National Institute for Health and Clinical Excellence (NICE) has developed guidelines to be used in the perinatal care for SCD mothers.

2.1 Antepartum

Antenatal care ought to be undertaken by a multi-disciplinary team that includes an obstetrician, midwife who has experience with high risk cases, and a haematologist (DeBaun et al., 2014, p.706). Also, pregnant women with SCD should undertake a medical review from a haematologist for the screening of any organ damage if they had not undergone this testing pre-conceptually. Further, the women should be taught on the risk factors to avoid that can cause sickle cell crises such as exposure to the extreme temperatures, overexertion, and dehydration. SCD pregnant women should be advised on instances to seek medical care such as severe vomiting, which can cause dehydration, a risk factor for vaso-occlusion. Also, the influenza vaccine must be administered if it had not been given within the past year (Vichinsky et al., 2011, p. 396).

The multi-disciplinary approach is aimed at improving maternal and fetal outcomes. A study done in the US showed that a multi-disciplinary approach improved the outcomes of SCD pregnant women (Giambona et al., 2015, p. 1134). In the UK, SCD pregnant women are advised to visit facilities that can provide care in emergency cases. Studies show that HbSC cases have fewer incidences of complications However, HbSC experiences complications associated with SCD and be given the same antenatal care as HbSS cases.

Additionally, partner testing should be done for the partner to find if they are a carrier and counselling offered during early pregnancy. It is essential for woman who has the potential of having an affected infant undergo prenatal diagnosis in early pregnancy. (Vitrano et al., 2016, p.367) The main objective of screening is to ensure that SCD pregnant receive appropriate maternity services.

The NICE guidelines outline the medications to be given to pregnant SCD mothers. It is recommended that the mother should be given a daily dose of folic acid throughout pregnancy (Hulihan et al., 2014, p.127). Also, prophylactic antibiotics should be given to prevent infections that precipitate a crisis. Drugs considered unsafe for pregnancy should be stopped. Iron supplementation should only be administered if there is evidence of iron deficiency.

The NICE guidelines also recommend a daily dosage of 75mg of low-dose aspirin from the second trimester to prevent pre-eclampsia. Furthermore, prophylactic heparin should be administered in the event of hospital admissions. Studies show that there is an increased probability of developing thromboembolism among SCD pregnant women (Matte et al., 2019). It is reported that 5 out of 12 deaths among SCD pregnant women occurs due to pulmonary embolism. To prevent this, women should be encouraged to use graduated compression stockings if they are deemed to be at risk. Notably, routine pregnancy care accorded to women without SCD should be accorded to this population.

During the antenatal period, SCD pregnant mothers can be placed on bed rest, hydrated, and given analgesics. The high probability of fetal loss requires regular fetal heart rate and biophysical profile monitoring. Corticosteroids should be administered for patients at risk of developing a crisis for the benefit of the foetus as they are at risk of pre-eclampsia, preterm premature rupture of membranes, and preterm labour.

The initial medical assessment of mothers with SCD focuses on the detection of medical conditions that require interventions such as abdominal crisis, infection, acute chest syndrome, dehydration, and severe anaemia (Aguilar Martinez et al., 2014, p.97). Typically, the management revolves around vigorous hydration, especially among patients presenting with fever, administration of analgesics as the patient can be in severe pain. Opiates are preferred to non-steroidal medications. Studies indicate that there is no risk for teratogenicity when opioids are used during pregnancy. However, long-term use is contraindicated in these cases, secondary to the risk of neonatal abstinence syndrome immediately after birth (Kaufmann et al., 2011, p. 1260). Monitoring of fetal well-being is vital during the prenatal period as SCD mothers are at risk of small for gestational age babies.

Blood transfusion is given prophylactically and therapeutically during the prenatal period. In prophylactic cases, blood transfusion is given to prevent adverse events such as stroke or pain crisis. Studies indicate that prophylactic blood transfusion among SCD mothers is attributed to improvements. A randomized trial carried out to establish the impact of prophylactic transfusion found a decrease in the number of painful crises but no significant improvement in pregnancy outcomes among SCD mothers. However, some arguments suggest that prophylactic blood transfusion is beneficial to SCD mothers at a high risk of developing pregnancy-related complications. Therapeutic transfusions, on the other hand, are indicated for symptomatic cases that are not responsive to conservative treatment such as severe anaemia, septicaemia, haemorrhage, and refractory painful crisis (Tsianakas et al., 2010, p.824). Also, SCD mothers scheduled for caesarean section, and women with persistent symptoms of pre-eclampsia are given blood transfusions.

The main aim of transfusion in SCD pregnant women in crisis is to reduce the number of HbSS while increasing the Hgb A concentration.  Limiting transfusion in SCD cases increases the risk for alloimmunization, hyper viscosity volume, septicaemia, iron overload, and volume overload (Brown et al., 2009, p. 375). The type of transfusion, whether simple or exchange, is based on the urgency of the situation. Exchange transfusion (erythrocytapheresis) allows for the removal of the sickled cells through centrifugation, where the patient’s clotting factors, platelets, plasma, and platelets alongside the donor’s Hgb A red blood cells are reversed back to the patient.

2.2 Intra-partum

NICE guidelines recommend that SCD pregnant women who had an uneventful prenatal period should be provided elective birth through induction or caesarean section after 38 weeks gestation (Piel et al., 2014, p. 85). SCD should not be a contraindication of vaginal delivery or undertaking vaginal birth after previous caesarean section. It is recommended for blood to be cross matched before delivery to ensure that there is availability of blood for transfusion in the event of any complication. SCD women have a higher probability of developing avascular necrosis which requires hip replacement. During the intra-partum period, the most suitable position for delivery should be discussed.

Notably, increased perinatal mortality are witnessed during the later stages during pregnancy; hence, there is no appropriate timing for delivery (Quinn, 2013, p. 1370). However, it is recommended that delivery among SCD mothers should be conducted between 38 to 40 weeks to prevent complications and adverse perinatal outcomes. On the other hand, caesarean section is recommended if there is any indication. Further, SCD mothers should deliver in hospital settings where complications can be managed. After confirmation, the multi-disciplinary team to include an experienced midwife, obstetrician, haematologist, and anaesthetist should be informed.

The woman should be kept warm throughout labour and provided with adequate fluid to prevent dehydration during labour. A continuous intra-partum electronic fetal heart rate monitoring should be done to assess for the foetus condition and assess for any need for caesarean delivery. In the UK, continuous monitoring is encouraged secondary to the risk of placenta abruption, stillbirth, and compromised placental reserve (Rees, Williams and Gladwin, 2010, p. 2021). Vital signs should be observed hourly with great importance to the temperature that will show any signs of infection that will require the administration of antibiotics. During caesarean section, the use of anaesthesia is not contraindicated. However, pethidine should not be used. Other opiates are not contraindicated. Regional anaesthesia is recommended for use among SCD women.

During labour, SCD mothers are at a high risk of developing a vaso-occlusive crisis. A diagnosis of a vaso-occlusive crisis is difficult during labour secondary to the presence of contractions. SCD mothers should remain in a lateral recumbent position and receive oxygen via a mask. The monitoring of the mother’s and foetus condition should be monitored closely. Studies indicate that an infusion of blood products during labour reduces the risk of a vaso-occlusive crisis. Maternal blood gas monitoring should be done continually, but invasive haemodynamic monitoring should be reserved only when there is an indication such as cardio-respiratory failure or severe pre-eclampsia (Locock and Kai, 2008). To avoid any infection, intrauterine catheters and urinary catheters should be used if need be.

Debates still exist on the use of anaesthesia among pregnant women with SCD undergoing caesarean section. Studies show that the use of general anaesthesia in this population increases the probability of postpartum haemorrhage (Locock and Kai, 2008). Regional anaesthesia is the preferred option for emergency cases among SCD pregnant women. Also, regional anaesthesia is recommended for relief of pain during labour as intravenous analgesics yield little or no effects in SCD cases.

SCD pregnant women in labour should be monitored for hypothermia and hypoxia while in labour as they precipitate a crisis. Neonatal resuscitation tools and qualified personnel should be available during labour. Although the use of oxytocic agents used for augmentation or induction is not contraindicated, they should be used with caution. Prostaglandin use for the management of postpartum bleeding is recommended as SCD mothers are at risk of PPH.

2.3 Postpartum

For women, whose babies are at risk of SCD, early testing should be done by collecting capillary samples. For the mothers, their oxygen saturation should be above 94% and adequately hydrated until discharge (Russo et al., 2019). Low molecular weight heparin should be given up to seven days post-delivery in vaginal delivery cases and for six weeks after caesarean section. Observe for SCD complications as they are still at risk. The woman should continue with the use of anti-thrombotic stockings. Routine care accorded to mothers without SCD should be given to this population.

Interventions carried out during the prenatal period can be employed during the postnatal period in the monitoring and management of pregnancy-related complications among SCD mothers. Studies show that the risk of infections, acidosis, and hypothermia are higher among SCD mothers compared to the general population. Early detection of these complications is vital to prevent a vaso-occlusive crisis. Thromboembolic and pulmonary oedema are higher among SCD mothers (Martin et al., 2013). Prophylactic anticoagulation is recommended during the postpartum period. Also, compression devices should be used to prevent thrombosis. It is essential to maintain higher Hgb A levels compared to HbSS during the postnatal period to reduce the risk of vaso-occlusive crisis.

3.0 METHODS

3.1 Data Sources

The search was done in various databases to include PubMed, EMBASE, MEDLINE, Google Scholar, Cochrane Library, RCM library, RCOG and NICE guidelines.  A search was done on English publications appearing with the words SCD, pregnant women, complications, and midwife’s role. The literature considered for the study was between 2009 and 2019. Screening of the titles and abstracts was done to determine if the research was relevant for the review. Retrieval of the articles was done, and an inclusion and exclusion criteria used to assess the documents. Materials that met the inclusion criteria were kept for data extraction and critical appraisal. Research done on fetal outcomes were included.

3.2 Data Extraction

Data extraction was done independently by one author using a standard extraction form. Information extracted from the studies included the authors of the research, the year the study was published, the name of the study, the design used to carry out the study, the number of participants in the study and control groups, the genotype of SCD, and the findings of the research. The studies included were scrutinized for the complications assessed during the perinatal period to include pre-eclampsia and eclampsia, neonatal mortality, maternal mortality, small for gestational age, preterm delivery, and caesarean section.

 

3.3 Quality Assessment

The New Castle-Ottawa (NOS) scale was used to determine the quality of the studies. The maximum score given to a study was nine, and the minimum a four (Luchini et al., 2017, p. 80). Four stars were allocated to selection of the study group, selection of the control group, and how the study demonstrated the outcome; comparability of the groups was given 2 stars; and 3 stars to for the assessment of the outcome, adequate follow-up, and the duration of follow-up. The studies were categorised as high, medium, and low-grade quality, where the scores were 7-9, 4-6, and 1-3, respectively.

3.4 Study Selection

Studies that published randomised clinical trials with findings on maternal, fetal, or perinatal outcomes among participants with SCD were included in the study. Review articles, studies without a control group, researches done on sickle-cell trait, and case reports were excluded.

A comprehensive approach was employed for the evaluation of fetal and maternal outcomes in the studies deemed relevant for the review. Primary outcomes for the analysis included perinatal mortality and intrauterine fetal demise (IUFD). Secondary outcomes included postpartum haemorrhage, caesarean section, sepsis, pre-eclampsia, and eclampsia for maternal outcomes; and preterm, intrauterine growth retardation, and low birth weight for the fetal outcomes.

Critical appraisal for the studies included in the meta-analysis was done. Three factors were considered to assess for the quality of each study to ascertain the validity of the research findings (Greenland, 2001). The study designs used in the research were prospective or retrospective, primary or secondary data sources were used, and a comparison group included in the study. The review considered sources that conducted prospective studies to be of a higher quality compared to those that used retrospective methods. Research that relied on primary data collection whereby the researchers carried out field research was considered to be of higher quality compared to those that relied on secondary data sources such as administrative and population-based databases. Studies that had comparison groups with similar characteristics as the study group were considered to be of high quality. According to the criteria used, high-quality studies possessed all three criteria; moderate quality had two domains, while the low quality had at least one area.

3.5 Data Analysis

A data abstraction form was used to analyse the findings. Online research was done to resolve any discrepancies in regard to the quality assessment, data abstraction, and studies to be included. Assessment of heterogeneity was done by the use of Higgins’ AND Cochran’s Q tests (Higgins and Thompson, 2002). Heterogeneity was considered to be significant when P < 0.1. When two studies had similar outcomes, pooling was done. A combination of data was done using the DerSimonian-Liard random-effects model, even in studies that showed no heterogeneity. This model assesses for true effect while considering the limitations of the study. The Cochran’s Q test was used to evaluate the differences between perinatal outcomes between the normal pregnancies and SCD pregnancies.

4.0 RESULTS

4.1 Study Characteristics

The characteristics of the 10 studies used in the meta-analysis are shown in Appendix 1. The studies used a cohort design. All the studies were UK based. The systemic review included 3227 women 2433 with SCD genotype and 794 control group. The number of women who contributed more than one pregnancy is not known.

4.2 Antenatal Outcomes

The risk for maternal mortality during pregnancy was six times higher in women with SCD compared to those without SCD according to the analysis of the studies. Women with SCD had a higher probability of experiencing stillbirth, pre-eclampsia, and preterm deliveries according to the results in the researches.

Oteng-Ntim et al. notes that although pregnant women with SCD were at a high risk of developing pregnancy-related complications compared to healthy pregnancies, there is no tool to measure the likelihood of the patient experiencing these symptoms (2014). This concept prevents medics from proper counselling SCD patients who are considering pregnancy. Oteng-Ntim et al. notes that the heterogeneity of the various genotypes of SCD makes it impossible to define the risks an individual with SCD will develop pregnancy-related complications.

An analysis of the studies shows a significant increase in maternal mortality in women with HbSS genotype and non-specified SCD (95% Cl) compared to those without sickle cell disease. Only one maternal mortality case was reported among women presenting with HbSC genotype.

The incidences of pre-eclampsia were double among women with HbSC genotype and in non-specified SCD when compared to pregnant women without SCD (95% Cl). Pre-eclampsia and eclampsia cases were higher among women with HbSS genotype. Stillbirth cases were four times higher among women with HbSS genotype and twice higher in the HbSC genotype group. Also, preterm deliveries were higher in the HbSS group (95% Cl).

The risks of developing pre-eclampsia, small for gestational age infants, and preterm deliveries were higher in SCD pregnant women regardless of income background of the woman. However, maternal mortality risks and stillbirth cases were reduced in high-income regions.

4.2.1 Pre-Eclampsia

Of the ten sources, only three sources discussed on pre-eclampsia. According to Eissa et al. in Pregnancy outcome in Sickle Cell Disease in the UK: Have things changed over Three Decades? The study of 206 SCD pregnant women in 4 years found out that no reduction reported in severe pre-eclampsia cases.

4.2.2 Intrauterine Grown Restriction

Out of the 10 sources, several have discussed on intrauterine growth retardation. According to Ngô et al., Pregnancy in sickle cell disease: maternal and fetal outcomes in a population receiving prophylactic partial, Intrauterine growth retardation was reportedly indicated in SCD pregnant women, which was reduced by blood transfusion.

4.3 Intra-Partum

4.3.1 Preterm Delivery

Out of the 10 sources, only two sources discussed preterm delivery. Ngô et al. established that preterm delivery among women with HbSS was higher at a rate of 73.6% as compared to that of HbSC, who had 26.4% at a P score of 0.001. Also, in Meeks et al. (2016) found that of 192 pregnant women with SCD and 187 healthy women it was established that SCD pregnant women had a higher rate of preterm delivery as compared to the control group. (p<0.00)

4.3.2 Caesarean Section Delivery

Out of the 10 sources, 5 sources discussed on caesarean delivery. According to AA Eissa, K Rantell, O Ashokkumar, SM Tuck, Pregnancy Outcome in Sickle Cell Disease in the UK: Have Things Changed Over Three Decades, it established an increase in emergency caesarean section delivery out of the 206 pregnant women with SCD. Also, Dormandy et al. (2010) established that pregnant women with HbSS were found to undergo caesarean section delivery at 38% rate as compared to the rate of 30% of HbSC pregnant women(p=0.02)

However, 3 studies indicated that there was an increased rate of caesarean section delivery among SCD pregnant women compared to the control group. Meeks et al. (2016) in Pregnancy in patients with sickle cell disease: maternal and perinatal outcomes found out that there was an increased caesarean section rate out of the 192 pregnancies and 187 health women (p<0.001).Also, Serjeant, Hambleton, and Thame, (2005) found that out of 71 participants and 65 control group, 30 % of women were subjected to emergency caesarean section delivery. Similar in (4), 39% of women delivered through caesarean section rate.

4.4 Postpartum

4.4.1 Maternal Mortality

Of the ten sources, six sources discussed on maternal mortality. According to Eissa et al. in Pregnancy outcome in Sickle Cell Disease in the UK: Have things changed over Three Decades? Maternal mortality during the perinatal period among women with SCD has decreased from 3.3% to 0.48% (Chase et al., 2010). However, mortality cases among women with SCD are higher compared to those without during the perinatal period, according to the studies (Chase et al., 2010). A study was done by Meeks et al. (2016) found that SCD has a higher maternal death rate (P= 0.056) in a study done among 192 participants and a control group of 187 mothers.

However 4 studies indicated that there were no maternal mortality rates during the perinatal period when SCD pregnant women were compared to those without the condition Serjeant, Hambleton and Thame, 2005 in Outcome of Pregnancy in Sickle Cell Disease Patients Attending a Combines Obstetric and Haematological Clinic found that out of the 71 participants compared to 65 with SCD there were no maternal deaths (p<0.001). These findings are replicated in a retrospective case-control study done by Ngo et al. established that there were no maternal deaths where 65 SCD women among 71 participants (P<0.001). Also, Holtkamp et al., (2017) in Pregnancy in sickle cell disease: maternal and fetal outcomes in a population receiving prophylactic partial exchange transfusions found that in 128 women with sickle cell disease there were no maternal deaths. Similarly, to Yu et al. (2009) in Fecundity and Pregnancy Outcome in a Cohort with Sickle Cell Haemoglobin C Disease followed from Birth, who found no maternal mortality among SCD mothers. Also, Dormandy et al. (2010) compared 109 participants, where 51 participants had SCD and 44 had sickle cell trait. His findings indicated that the genetic composition did not have a direct effect on the probability of maternal mortality.

4.4.2 Fetal Mortality

Of the ten sources, only five discussed on fetal mortality rate. According to Yu et al. in Outcome of pregnancy in sickle cell disease patients attending a combined obstetric and haematology clinic Out of 71 participants compared to 65 pregnant women with Sickle cell disease, only one fetal demise occurred, and this was due to abruption (Meeks et al., 2016). A study done by Eissa et al. (2019) Pregnancy outcomes in sickle cell disease, a retrospective cohort study from two tertiary centres in the UK, found that three intrauterine deaths occurred between 24, 29, and 34 weeks. The deaths occurred due to various complications with two congenital defects and the other due to severe intrauterine growth retardation (IUGR) similarly to Yu et al. (2009)

In a study done by Graham Sergeant, Ian Hamleton and Minerva Thame on stillbirth rates in Fecundity and pregnancy outcome in a cohort with sickle cellhaemoglobin C disease followed from birth stillbirth maternal rate among SCD -SC pregnant women and the control group which was SCD –AA had no significant differences. However, this finding differed significantly with SCD –SS pregnant women (Yu et al., 2009).

In a study done by Oteng-Ntim et al. on a post-partum haemorrhage. In Pregnancy Outcome in Patients with Sickle Cell Disease a UK National Cohort Study Comparing Sickle Cell Anaemia (HbSS) with HbSC disease out of the 109 women diagnosed with SCD showed sickle cell trait with 51 participants having HbSS while 44 had HbSC. The occurrence of postpartum haemorrhage was frequent in HbSC women at a rate of 16.5% and HbSS at 7.5% (p=0.02)

4.4.3 Low Birth Weight

Out of the 10 sources, three sources discussed on low birth weight. According to Vanessa Costa Marcos Viana, and Regina Aguiar: Pregnancy in patients with Sickle Cell Disease: Maternal and Perinatal Outcomes: A study done out of  58 women with  29 having 29 SCD SS trait and 29 SCD SC found out that low birth weights were frequent to women with SCD –SS (p<0.001).These results were replicated in a retrospective case-control study done by Graham Ian Hamleton, Minerva Thame established that low birth weights were reported to SCD-SS women as compared to SCD-SC at (p<0.002). Also, Costa, Viana, and Aguiar (2014) found out that low birth weights were common to HbSS pregnancies at a rate of 35.4% and HbSC at 14% (p=0.025).

4.4.4 Bacterial Infection

Out of the 10 sources, two sources discussed on Bacteria infection. According to Oteng-Ntim et al. in A National Cohort Study Comparing Sickle Cell Anaemia (HbSS) with HbSC disease, an increased rate of urinary tract infection was reported on women with Sickle cell Disease that is 11% overall; 19.6% in HbSS caused by bacteria proliferation. Also, Chase et al. (2010) reported that there were cases of urinary infection. Similar Meeks et al. (2016) out of the 192 women and 187 healthy pregnancies an increased rate of urinary infection was noted among women with SCD (p=0.001)

5.0 DISCUSSION

The systemic review determined that there is a strong association between sickle cell disease and adverse outcomes in pregnant cases mother (pre-eclampsia and maternal mortality), and baby (small for gestational age, preterm births, and perinatal deaths). An analysis of the different genotypes shows that women with HbSS disease have worse perinatal outcomes of the complications studied compared to the HbSC genotype. Although the cases of pregnancy-related complications secondary to SCD have reduced in the UK, the cases are still high in the low-income settings when compared with the high-income areas.

The study used a comprehensive literature search written in the English language. Only studies carried out in the UK were included in the meta-analysis. The total of the pooled sample size comprised of 3227 participants combined with the control group. The outcome of the analysis showed that SCD increases the probability of a mother developing complications during the perinatal period. However, 6 out of the 10 failed to analyse the different genotypes among the SCD cases to determine if the type determines the severity of the complications. Additionally, several studies carried out their research in single institutions, which may not be a representation of the entire population.

Secondary to the significant progress in the health sector and the management of SCD, more women are now able to survive. Notably, the life expectancy of women with SCD is 42 years, with many succumbing to pregnancy-related complications. During pregnancy, there is an exacerbation of pre-existing pathological changes caused by SCD, such as anaemia, an increase in the risk for infection, increased coagulation, and vaso-occlusion (Kaufmann et al., 2011, p. 1260). Additionally, the plasma levels that often increase during pregnancy are elevated in SCD cases and are thought to cause increased incidences of antepartum bleeding, painful crises, thrombolytic complications, and pulmonary complications. Pregnancy in SCD cases is believed to cause these complications even in women who previously had few symptoms. Notably, SCD women in their childbearing age accumulate various comorbidities to include renal dysfunction, avascular necrosis of the hip, pulmonary hypertension, and retinal disease that complicate pregnancy outcomes. The severity of the complications varies from patient to patient, with the SCD genotype playing a major role in the outcome. HbSS cases have more severe complications compared to HbSC. Chronic fetal hypoxia can be related to impaired placental blood flow, with the result being many cases of perinatal complications.

The studies show that HbSS cases have a six-fold increase in maternal complications when compared to the control group that entailed mothers with HbAA genotype. Additionally, the SCD group had a marked increase for small for gestational age infants, preeclampsia, preterm deliveries, and stillbirths. Although nine of the studies outlined the adverse outcomes of pregnancy among women with SCD, the lack of heterogeneity of the populations under study and the sources of data made it difficult to ascertain the risk associated with SCD in pregnancy.

Interestingly, the analysis of the studies showed that the adverse effects of SCD during pregnancy, such as stillbirths and maternal deaths are higher when compared to less-adverse complications. These findings denote that there is inadequate management of acute complications leading to an increase in unfavourable outcomes among SCD pregnant women. Also, the prevention measures put in place for SCD mothers may be ineffective in preventing adverse complications.

The study used a comprehensive literature search written in English language. Only studies carried out in the UK were included in the meta-analysis. The total of the pooled sample size comprised of 3227 participants combined with the control group. The outcome of the analysis showed that SCD increases the probability of a mother developing complications during the perinatal period. However, 6 out of the 10 failed to analyse the different genotypes among the SCD cases to determine if the type determines the severity of the complications. Additionally, a number of the studies carried out their research in single institutions, which may not be a representation of the entire population.

Secondary to the significant progress in the health sector and the management of SCD, more women are now able to survive. Notably, the life expectancy of women with SCD is 42 years, with many succumbing to pregnancy-related complications. During pregnancy, there is an exacerbation of pre-existing pathological changes caused by SCD, such as anaemia, an increase in the risk for infection, increased coagulation, and vaso-occlusion. Additionally, the plasmic levels that often increase during pregnancy are elevated in SCD cases and are thought to cause increased incidences of antepartum bleeding, painful crises, thrombotic complications, and pulmonary complications. Pregnancy in SCD cases is believed to cause these complications even in women who previously had few symptoms. Notably, SCD women in their childbearing age accumulate various comorbidities to include renal dysfunction, avascular necrosis of the hip, pulmonary hypertension, and retinal disease that complicate pregnancy outcomes. The severity of the complications varies from patient to patient, with the SCD genotype playing a significant role in the outcome. HbSS cases have more severe complications compared to HbSC. Chronic fetal hypoxia can be related to impaired placental blood flow, with the result being many cases of perinatal complications.

The studies show that HbSS cases have a six-fold increase in maternal complications when compared to the control group that entailed mothers with HbAA genotype. Additionally, the SCD group had a marked improvement for small for gestational age infants, preeclampsia, preterm deliveries, and stillbirths. Although nine of the studies outlined the adverse outcomes of pregnancy among women with SCD, the lack of heterogeneity of the populations under study and the sources of data made it difficult to ascertain the risk associated with SCD in pregnancy.

Interestingly, the analysis of the studies showed that the adverse effects of SCD during pregnancy, such as stillbirths and maternal deaths are higher when compared to less-adverse complications. These findings denote that there is inadequate management of acute complications leading to an increase in unfavourable outcomes among SCD pregnant women. Also, the prevention measures put in place for SCD mothers may be ineffective in preventing adverse complications.

The meta-analysis established that there is an association between SCD and perinatal complications to the mother and baby. Women who had HbSS genotype exhibited worse perinatal outcomes compared to the other genotypes. Studies that had a high rating in the quality score had significant relative differences between the study and control group compared to the low-quality researches. This may have occurred due to a better analysis of the data among the high-quality scores.

To the best of the researcher’s knowledge, the study is the first meta-analysis and systemic review of perinatal outcomes and midwives’ role in SCD care done in the UK. The pooled sample of 794 mothers without SCD increases the reliability of the study. The study was limited by the use of some sources that utilised a single institution to collect data. Also, some studies failed to state the findings of pregnancies according to the genotype. However, the researcher mitigated this limitation through stratification of the study population into specific genotypes to include the non-specific group. The researcher was not able to adjust the confidence intervals of those women who were included in the study with different pregnancies as this information was not provided in the selected studies.

The systemic review limited the outcomes to pregnancy-related complications and outcomes such as congenital fetal abnormality, postpartum haemorrhage, placenta abruption, thromboembolism, pulmonary hypertension, and maternal jaundice which had inconsistent data were not included in the analysis. It should be a concern that women with SCD have a higher probability of maternal mortality. One study reported on multi-organ failure, sepsis, and acute chest syndrome being higher among women with SCD and a cause of maternal mortality in this population. The other studies did not highlight the exact cause of maternal mortality or the direct correlation between SCD and maternal mortality. The risk of maternal mortality is fourfold among women with HbSS genotype, while the HbSC group only had one maternal death. The risk of morbidity in the two groups is sizeable.

Additionally, there is a substantial difference between the risk of stillbirth and maternal death among mothers with high and low economic status. Although pregnancy-related complications are not significantly different between the two groups, these findings can be quantified with the availability of resources, expertise, and healthcare facilities. The situation may be aggravated by the lack of a tool that determines SCD mothers who are at a higher risk of developing pregnancy-related complications.

Vascular and physiological changes that are typically witnessed during pregnancy have been accused of causing the exacerbations of pregnancy-related complications among SCD mothers such as fetal growth restriction and pre-eclampsia. Regular screening in the third trimester for complications and fetal growth is one of the ways of mitigating the adverse outcomes. However, such measures may be limited to facilities without adequate monitoring equipment.

More studies should be carried out to understand the reasons behind the increase in pregnancy-related complications in the HbSS genotype. Researches have been done to establish the effectiveness of prophylactic transfusion as a preventative measure show no significant impact in the prevention of complications. More trials should be carried out on transfusion modalities among women with SCD considered high risk of developing complications as one of the choices of preventing perinatal complications.

6.0 STRENGTHS AND LIMITATIONS OF THE STUDY

The unique aspect of this systemic review is the inclusion of research done among pregnant women with SCD in the UK to include both the high and low-income regions. Comparing the SCD mothers to the healthy population facilitated the formulation of testable hypotheses where SCD is linked to high rates of perinatal complications. The odds ratio in maternal mortality where more cases are in low-income regions compared to high-income areas is suggestive of modifiable risk factors in the prevention of this complication.

Regardless of the mothers’ income status, the continued adverse incomes in all economic groups highlight the need for more resources and training of midwives in the management of SCD complications during pregnancy.

The methodological approach employed in the systemic review has various strengths. The study used three main factors to determine the quality of the researches to be included in the meta-analysis. The studies were stratified according to the region they were conducted in, and only those done in the UK were selected.

7.0 INTERPRETATION

The lack of evidence-based guidelines on the monitoring and management of pregnant women with SCD necessitates a multidisciplinary (MDT) approach to include obstetricians, midwives, and haematologists. Some practitioners have used prophylactic transfusions, which have not been effective in reducing pregnancy-related complications among SCD mothers. The high rates of maternal and fetal complications among SCD mothers compared to the general population suggest an underlying biological effect and not inefficient healthcare or lack of resources. Efforts put in place to minimise pregnancy complications in the SCD population should target biological factors that have a direct impact on perinatal outcomes.

8.0 RECOMMENDATIONS

Based on the findings of this meta-analysis, prospective studies should be carried out to determine the biological factors that precipitate maternal and fetal complications among SCD mothers.

9.0 CONCLUSION

The appropriate management of perinatal management of pregnant women with SCD is still being debated in the UK.  Nonetheless, pregnancy outcomes in this population have significantly improved thanks to technological advancements in the healthcare industry. Most of the studies recommended a multi-disciplinary approach in the prevention of pregnancy-related complications among SCD mothers. Antenatal management should involve education and counselling on healthy diets, regular intake of prenatal vitamins, rehydration, and prevention of precipitants such as excessive exercises and cold environments that precipitate a vaso-occlusive crisis. Regular monitoring during the perinatal period to assess for pregnancy-related complications is vital for preventing fetal and maternal mortality that are high among SCD mothers.

The results from the analysis show some limitations of some of the studies included in the systemic review. Significant heterogeneity was found in most of the outcomes. Based on the study design incorporated in the meta-analysis, it is difficult to conduct a multivariable analysis. It is difficult to evaluate whether low economic status contributed directly to the high mortality rates of SCD mothers. However, the systemic review allows for the formulation of hypothesis for prospective studies. Also, none of the studies evaluated systemic interventions to improve pregnancy outcomes among SCD mothers.

 

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APPENDICES

Appendix 1: Literature Analysis

Author, Title, journal Purpose Data Collection Tools Approach

(Qualitative or Qualitative)

Number of Subjects Subject Characteristics Limitations
1. AA Eissa, K Rantell, O Ashokkumar, SM Tuck

Pregnancy Outcome in Sickle Cell Disease in the UK: Have Things Changed Over Three Decades

  Journal, UK

      (2019)

To assess trends in mortality rates and trends in complication rates. Survey of trends of mortality cases among pregnant women with SCD.

Chi-square to assess the trends of the rates of complication.

Quantitative approach was used to compare results from the survey done in 11 hospitals in the UK.

This was a retrospective study.

206 participants. Participants gave consent for the study. Pregnant women with complicated SCD.

 

The study did not evaluate the individual characteristic of the participants.
2. Daveena Meeks, Susan E Robinson, David Macleod, and Eugene Oteng-Ntim.

 

Birth Weights in Sickle Cell Disease Pregnancies: A Cohort Study

  Journal, UK

     (2016)

To establish whether children born to pregnant women with SCD are at risk of low birth weight or not. Electronic clinical databases were used for the retrospective study.

 

Quantitative study to establish the birth weight of children born of SCD mothers. 88 participants with SCD

176 controls

All participants gave consent for the study.

88 (50 participants with HbSS,

38, HbSC genotype)

 

Control group (176, HbAA).

The study did not evaluate maternal and gestation pathologies known to affect birth weight, such as hypertension.
3. Oteng-Ntim, Ayensah B, Marian Knight, and Jo Howard

Pregnancy Outcome in Patients with Sickle Cell Disease in the UK- A National Cohort Study Comparing Sickle Cell Anaemia (Hbss) with HbSC disease.

  Journal, UK

      (2014)

Maternal and Foetal outcomes in pregnant women with SCD. Questionnaires, Hospital statistics Cohort study.

Quantitative method.

Mixed methods: surveillance system.

A prospective method was used.

109 women with SCD

All participants gave consent.

51 Participants with HbSS

44, HbSC

The study lacked a control group with similar characteristics with the participant.

Lack of similar studies to compare results with.

4. Chase et al.

Pregnancy outcomes in sickle cell disease: a retrospective cohort study from two tertiary centres in the UK.

   Journal, UK

       (2010)

To establish the pregnancy outcomes among women with SCD. Survey Quantitative: a retrospective cohort study. 122 pregnant women with single pregnancy. 64, HbSS

45, HbSC

11, thalassemia

1, HbE

1, Sickle cell disease delta

The study failed to establish the link between participant characteristics with the development of SCD complications during the perinatal period.
5. Ngô et al.

Pregnancy in sickle cell disease: maternal and fetal outcomes in a population were receiving prophylactic partial exchange transfusions.

   Journal, UK

       (2010)

To establish the outcomes among pregnant women with SCD receiving prophylactic transfusion. Questionnaires and interviews Quantitative: retrospective case-control study 128, study group

128, control group. All participants gave consent for the study.

128, HbSS

128, HbAA

A small study group was used; therefore, the results could not give a true representation of the population.
6. Vanessa Costa, Marcos Viana, and Regina Aguiar

Pregnancy in patients with Sickle Cell Disease: Maternal and Perinatal Outcomes.

  Journal, UK

      (2014)

To compare neonatal, haematological, and obstetrical outcomes in gravid women with or without SCD. Hospital electronic databases were used. Quantitative: prospective study with a control group. 252 participants who consented for the study. 60, SCD pregnant women

192, healthy pregnant women.

The study did not look into individual women characteristics that are a risk factor to development of complications.
7. Dormandy et al.

Effectiveness of earlier antenatal screening for sickle cell disease and thalassemia in primary care: cluster randomised trial.

   Journal, UK

       (2010)

To establish the effectiveness of antenatal screening offered for sickle cell disease and thalassemia to facilitate utilisation of screening in primary care. Electronic databases from the hospital Partial factorial cluster randomised controlled trial 1708 participants who consented for the study. Pregnant women before 28 weeks gestation.

 

Midwives involved in the screening process.

Midwives’ knowledge of the screening process was not evaluated.

 

The number of midwives involved in the study is not known.

8. Holtkamp et al.

Experiences of a High-Risk Population with Prenatal Hemoglobinopathy Carrier Screening in a Primary Care Setting: a Qualitative Study. 

 

 

   Journal, UK

       (2017)

To assess the experiences of SCD pregnant women in hospitals and establish the midwives knowledge on their role in the management of SCD crisis during the perinatal period. Semi-structured interview was used to assess for the knowledge of the midwives role.

Interview method for pregnant women with SCD participating in the study.

Qualitative method was used.

A prospective method was used.

Pregnant women with SCD, 26.

 

Midwives, 24

Pregnant women who participate in the study were above 25 years of age, between 5-26 weeks gestation, and from different countries.

 

The midwives interviewed worked in the maternity unit during the study.

The interviews were conducted for a short period (7-10 minutes); thus, little information was obtained.

 

The close-ended questions used in the interview resulted in minimal information.

9. Yu et al.

Outcome of pregnancy in Sickle cell disease patients attending a combined obstetric and haematology clinic.

   Journal, UK

       (2010)

To establish the outcome of pregnancy in women with SCD attending obstetric clinic Hospital records were used to assess the outcome of pregnancy in women with sickle cell attending clinic at King’s college hospital Quantitative methods were used.

A randomised clinical trial was obtained.

71 participants.

Consent was obtained.

65 women were pregnant and had SCD The characteristics of the participant were not considered to know the risk factors that exposed them to SCD.
10. Graham Serjeant, Ian Hamleton, Minerva Thame.

Fecundity and pregnancy outcome in a cohort with sickle cell-haemoglobin C disease followed from birth

Journal, UK

     (2005)

To compare postnatal complications between women with SCD and those with normal HbAA Hospital records were used to follow the outcomes of the participants since birth. Quantitative method: Prospective cohort study. 189 participants. Consent was obtained from all participants. 43, SCD patients

 

52, HbSS

 

94, HbAA

The study did not assess individual characteristics that may predispose one to pregnancy complications during the postnatal period.

 

 

 

 

 

 

 

 

Appendix 2

New Castle-Ottawa Scale

Author (Year) Study Design Selection Comparability Exposure/ Outcome Total/9
Eissa et al. (2019) Historical cohort 4 2 1 7
Yu et al. (2009) Historical cohort 3 1 1 5
Chase et al. (2010) Historical cohort 4 1 2 7
Ngô et al. (2010) Historical cohort 3 2 1 6
Meeks et al. (2016) Historical cohort 4 1 1 6
Costa et al Prospective cohort 3 1 1 5
Daveena et al. (2016) Historical cohort 4 1 2 7
Dormandy et al. (2010) Historical cohort 3 2 1 6
Holtkamp et al. (2017) Historical cohort 2 1 1 4

 

Serjeant et al. (2005) Prospective cohort 4 1 2 7

Appendix 3

Search Strategy

 

 

2 sources identified through other searches.

 

28 sources identified when the databases were used.

 

30 sources screened in total
10 sources excluded

 

 

 

20 full-text sources assessed for their eligibility

 

10 articles were included in the meta-analysis
10 sources excluded

2 were systemic reviews

2- important for background and discussion, not for analysis

4- Full text was not available

2- The researchers carried out observational studies.

 

9 sources provided quantitative data

 

1 source provided qualitative data

 

 

 

 

 

 

 

Appendix 4

Databases

Database: PUBMED
Search no. Concept Search String No. Results
S1 SCD HbSS 140
S2  

HbSC

90
S3 AS 110
S4 Other Anomalies 240
S5 Pregnant women Antenatal 310
S6 Intra-partum 390
S7 Post-partum 260
S8 Foetal Outcome 410
S9 Complications Antenatal 260
S10 Intra-partum 59
S11 Post-partum 275
S12 Foetal outcome 410
S13 Midwife’s role (perspective or perception or opinion or experience or attitude) 155
S14 Final Search S4 AND S8 AND S12 AND S13 25

 

 

Database: MEDLINE
Search no. Concept Search String No. Results
S1 SCD HbSS 220
S2  

HbSC

190
S3 AS 160
S4 Other Anomalies 230
S5 Pregnant women Antenatal 320
S6 Intra-partum 330
S7 Post-partum 400
S8 Foetal Outcome 470
S9 Complications Antenatal 150
S10 Intra-partum 76
S11 Post-partum 110
S12 Foetal outcome 509
S13 Midwife’s role (perspective or perception or opinion or experience or attitude) 170
S14 Final Search S4 AND S8 AND S12 AND S13 50

 

 

Database: EMBRACE
Search no. Concept Search String No. Results
S1 SCD HbSS 300
S2  

HbSC

450
S3 AS 370
S4 Other Anomalies 150
S5 Pregnant women Antenatal 260
S6 Intra-partum 390
S7 Post-partum 370
S8 Foetal Outcome 660
S9 Complications Antenatal 170
S10 Intra-partum 90
S11 Post-partum 400
S12 Foetal outcome 200
S13 Midwife’s role (perspective or perception or opinion or experience or attitude) 530
S14 Final Search S4 AND S8 AND S12 AND S13 30

 

 

 

Database: COCHRANE
Search no. SCD Search String No. Results
S1  

Pregnant women

HbSS 200
S2  

HbSC

40
S3 AS 50
S4 Other Anomalies 130
S5  

Complications

Antenatal 205
S6 Intra-partum 330
S7 Post-partum 360
S8 Foetal Outcome 660
S9  

Midwife’s role

Antenatal 140
S10 Intra-partum 89
S11 Post-partum 430
S12 Foetal outcome 620
S13 Final Search (perspective or perception or opinion or experience or attitude) 430
S14 SCD S4 AND S8 AND S12 AND S13 55

 

 

 

Appendix 5

Proposal Form

MWY 3330 DISSERTATION MODULE

PROPOSAL FORM

 

NAME OF STUDENT:    Sandra Dandison                                    

COHORT:    September 2017                                                                                                          

NAME OF SUPERVISOR / S:    Lindsey Ahmet

Title of proposed dissertation:

A Literature Review on Pregnancy Outcomes Among Women with Sickle Cell Disease and The Role of Midwives in their Perinatal Management.

Aims and objectives of the study:

Aims

This study aims at assessing pregnancy outcomes among women with sickle cell disease (SCD), its impact and complication, and the current practice among midwives on perinatal care in this population.

Objectives

  1. To describe the experiences of pregnant women diagnosed with SCD during perinatal management using both quantitative and qualitative analysis methods.
  2. To identify the risks and complications associated with SCD, the screening tests undertaken and their impact in pregnancy and infant outcomes by analysing epidemiological studies.
  3. To explore the role midwives take in the detection and management of pregnant women with SCD by analysing the available sources.

Reasons for wishing to carry out the work:

The reasons for carrying out this research is based on the researcher’s clinical experience in the labour unit where two mothers developed complications associated with SCD post-delivery on two separate occasions. Also, review of literature by the researcher on the condition among pregnant women in the UK and the statistics on morbidity and mortality cases being higher compared to normal cases lead to the study.

Relevance of the Study to My Midwifery Practice:

The rising cases of SCD in the population and the increasing number of pregnant women with the condition necessitate a midwife’s understanding of the anticipated risks (Jain et al., 2019).The evidence obtained will ensure that a midwife manages the patient according to evidence-based research and acts promptly in the advent of a complication. Additionally, the study will help me to:

  1. To create knowledge on how to manage pregnancy and labour among women with SCD depending on the probable risks and presenting complications as a means of ensuring a healthy outcome for the mother and baby.
  2. To develop understanding on the role and responsibilities of the midwife during the perinatal period in the management of SCD.
  3. To understand the current management of SCD among pregnant women according to the updated guidelines.

Literature and sources of information reviewed and that I intend to use for the project:

SCD is still a severe complicating factor in pregnancy despite many advances in the medical field. Reports indicate that perinatal morbidity and mortality among pregnant women with SCD have continued to increase (Meeks et al., 2016). Various management strategies according to the set guidelines are being implemented to prevent the occurrence of complications from twenty-eight weeks gestation based on a woman’s haematological and obstetric histories (Oteng-Ntim et al., 2015, p. 3316). However, these interventions have not reduced the risks associated with complications in the third trimester of pregnancy nor improved the outcomes of labour among women with SCD though they are used widely in the UK. Sickle cell affects multiple organs and organ systems such as the liver and lungs (Elenga et al., 2016). Pregnant women with SCD still have a higher probability of developing complications during the perinatal period. A study carried out by e UK Obstetrics Surveillance System (UKOSS) showed that 50% of pregnant women with SCD developed complications that include anaemia, acute chest syndrome, and painful vaso-occlusive crisis (Jain et al., 2019). 20% of these cases required high dependency unit admission (HDU). women with SCD developed complications such as hypertensive diseases such as pre-eclampsia and pregnancy-induced hypertension compared to other women. SCD in pregnancy was associated with foetal complications such as small for gestational age, prematurity, and still births. ​87.5% of deliveries among SCD women are conducted through caesarean section (Elenga et al., 2016). These were complications of SCD in pregnancy. These statistics are significantly higher compared to the general population where majority of the caesarean sections are done among women with intrapartum complications. High caesarean cases among SCD women are secondary to labour complications and foetal sickle-related complications (Meeks et al., 2016). Foetal loss in SCD accounts for 18.6% of all foetal deaths in the country annually.

 

Sources to be used:

  1. Cardosa, D., Ridout, A., Nanda, S., Howard, J., Robinson, S. and Oteng-Ntim, E. (2018). Maternal sickle cell disease and twin pregnancy: a case series and review of the literature. Hematology, 24(1), pp.148-158.
  2. Eissa, A., Rantell, K., Ashokkumar, O. and Tuck, S. (2012). Pregnancy outcome in sickle cell disease in the UK: have things changed over three decades? Archives of Disease in Childhood – Fetal and Neonatal Edition, 97(Suppl 1), pp. A34.2-A34.
  3. Jain, D., Lodha, P., Colah, R., Atmapoojya, P. and Atmapoojya, P. (2019). Sickle Cell Disease and Pregnancy. Mediterranean Journal of Haematology and Infectious Diseases, 11(1), p.e2019040.
  4. Kuo, K., Aviram, A., Merrill, M. and Caughey, A. (2016). 499: Optimal timing of delivery for women with sickle cell disease. American Journal of Obstetrics and Gynecology, 214(1), pp.S272-S273.
  5. Meeks, D., Robinson, S., Macleod, D. and Oteng-Ntim, E. (2016). Birth Weights in Sickle Cell Disease Pregnancies: A Cohort Study. PLOS ONE, 11(10), p.e0165238.
  6. Okusanya, B. and Oladapo, O. (2016). Prophylactic versus selective blood transfusion for sickle cell disease in pregnancy. Cochrane Database of Systematic Reviews.
  7. Oteng-Ntim, E., Ayensah, B., Knight, M. and Howard, J. (2014). Pregnancy outcome in patients with sickle cell disease in the UK – a national cohort study comparing sickle cell anaemia (HbSS) with HbSC disease. British Journal of Haematology, 169(1), pp.129-137.
  8. Yu, C., Stasiowska, E., Stephens, A., Awogbade, M. and Davies, A. (2009). Outcome of pregnancy in sickle cell disease patients attending a combined obstetric and haematology clinic. Journal of Obstetrics and Gynaecology, 29(6), pp.512-516.

Methods and Analysis

I will search the following databases for studies on SCD among pregnant women, implications and complications, and the role of midwives in perinatal care: PubMed, EMBASE, MEDLINE, Google Scholar, and Cochrane Library. RCM library, RCOG and NICE guidelines will be used in the study. After selecting the eligible sources from the search, review of the findings, extraction of data and synthesis will be done. The studies obtained will be reviewed and evaluated for quality and bias. The eligibility criteria will include observational studies indicating perinatal and maternal health outcomes among pregnant women with SCD against a comparative group of pregnant women with normal pregnancy. Ten studies are eligible for inclusion. Studies done outside the UK will be excluded in the study. Statistical methods will be used to obtain prevalence estimates in the studies obtained in the UK populations.

Timeline for Undertaking and Completing the Project.

The research project will be submitted as indicated below in the timetable after a formal notification to proceed is given. Analysing the sources will be done in the first two months after approval. Liaising with my supervisor will be done throughout the project to provide guidance, support and direction.

 

 

 

 

Table 1: Gantt chart for the Dissertation approach

 

Activities  

Sept

 

Oct

 

Nov

 

Dec

 

Jan

 

Feb

 

Mar

 

Apr

 

May

 

Jun

Identify research area
Formulate research question and planning
Write research proposal
Feedback from supervisor
Submission of dissertation proposal
Formulate research strategies and select methods
Write background, aims and methodology. Write Lit Crit Grid and feedback
Literature review and identifying sources
Data collection and analysis
Preparing dissertation findings
First draft
Second draft
Final draft
Final meeting with supervisor before deadline
Submission of dissertation

2019                                       2020

 

 

 

 

Reference List

Elenga, N., Adeline, A., Balcaen, J., Vaz, T., Calvez, M., Terraz, A., Accrombessi, L. and Carles, G. (2016). Pregnancy in Sickle Cell Disease Is a Very High-Risk Situation: An Observational Study.

Jain, D., Lodha, P., Colah, R., Atmapoojya, P. and Atmapoojya, P. (2019). Sickle Cell Disease and Pregnancy. Mediterranean Journal of Haematology and Infectious Diseases, 11(1), p.e2019040.

Meeks, D., Robinson, S., Macleod, D. and Oteng-Ntim, E. (2016). Birth Weights in Sickle Cell Disease Pregnancies: A Cohort Study. PLOS ONE, 11(10), p.e0165238.

Oteng-Ntim, E., Meeks, D., Seed, P., Webster, L., Howard, J., Doyle, P. and Chappell, L. (2015). Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis. Blood, 125(21), pp.3316-3325.

 

 

Date of submission of proposal to your supervisor:   28/10/19

Date of appointment with your supervisor to discuss proposal: tbc

 

Signature of student:    Sandra Dandison           Date:   08/11/19

 

 

 

 

 

 

 

 

 

 

 

 

 

PROJECT SUPERVISION

REVIEW OF DISSERTATION PROPOSAL

 

Feedback from project supervisor:

Overall a very sensible subject and relevant to many mothers in London. Not many midwifery perspectives have been presented on this topic, so this is a timely review of the literature. This could later be converted to a publication for other midwives after successful completion of the dissertation. The timeline needs a little more detail and include some methodological reading and preparation.

 

Negotiated strategies between student / supervisor for undertaking and completing project:

For student to keep teacher informed as to progress and to endeavour to meet deadlines and be honest and seek help /clarification if required.

Email feedback on sections will be provided with the 1st section to be seen in a few weeks a larger list of likely sources and a chapter outline with deadline dates. The 1st formal section to be done is the lit crit grid and then the chapter describing the search technique.  When face to face meetings are not possible communications will by email attachment or phone /skype discussion.

 

Action plan:

Student to review the comments above in REVIEW word edit these out make any changes before submitting this proposal to Dissertation Module leaser.

 

 

Signature of student:  Sandra Dandison        Date: 08/11/19

 

Signature of supervisor:      Lindsey Ahmet                Date: 2/11/19

 

Appendix 5

Abbreviations

 

  • ACS- Acute Chest Syndrome
  • HbAA-
  • HbE-
  • HbSC- Haemoglobin C
  • HbSS- Haemoglobin S
  • HDU- High Dependency Unit
  • Hgb A- Haemoglobin A
  • IUFD- Intrauterine Fetal Demise
  • LMWH- Low-molecular weight heparin
  • NPEU- National Perinatal Epidemiology Unit
  • RCM- Royal College of Midwives
  • RCOG- Royal College of Gynaecologists
  • PPH- Post-Partum Haemorrhage
  • SCD- Sickle Cell Disease

 

 

 

 

 

 

 

 

 

 

 

Appendix 6

Glossary

 

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