ANAEMIA IN PREGNANCY_NPMCN UPDATE COURSE JULY 2023-1.pptx

ANAEMIA AND
HAEMOGLOBINOPATHIES IN
PREGNANCY
Abiodun S. ADENIRAN (FMCOG; FWACS; MD; MHPM)
READER
Obstetrics & Gynaecology Department,
University of Ilorin/ University of Ilorin Teaching Hospital,
Nigeria.
UPDATE COURSE JULY 2023 by NPMCN

Outline
• Introduction
• Epidemiology
• Physiological changes in pregnancy and Anaemia
• Classification
• Approach to Management
• Current Research Questions
• Haemoglobinopathies
• Management of Haemoglobinopathies in
Pregnancy
• Conclusion
2
Anaemia & Haemoglobinopathies in
Pregnancy UPDATE Course July 2023

Lecture Objectives
• Revise the epidemiology of anaemia in
pregnancy
• Outline a rational approach to management of
Anaemia in pregnancy
• Enumerate current Research Questions/ Issues
on Anaemia in Pregnancy
• Discuss the management of pregnant women
with Haemoglobinopathies
3

Introduction
• Anaemia: fewer circulating red blood cells ( RBC) or a
reduction in the concentration of haemoglobin with
reduction in the O2-carrying capacity of the blood
• May follow reduced production / increased RBC loss
• An important global maternal health problem and
commonest medical disorder in pregnancy
• Important indirect cause of severe Maternal
Outcome ( 61.2% of near-misses and 32.8% of
maternal deaths) in the Nigeria Near-Miss and
Maternal Death Survey.1
4

Epidemiology
• Worldwide, anaemia affects approximately 1.62 billion
individuals ≈24.8% of the total global population.2
• The highest prevalence of anaemia occur among pre-
school children (47.4%) and pregnant women (41.8%)2
• Anaemia in pregnancy is considerably high (≈30–40%)
even in high-income countries2 compared to 35-75% in
Africa, Asia and Latin America
• 90% Fe deficiency Anaemia, ≈5% Folate Deficiency
5

Epidemiology- 2
Important socio-demographic factors:
• Education, parity (low and high), low social class, age
(18-20years, >35years), poor nutrition.
Other important factors:3
• Infestation with intestinal parasite: ↑3.59 times
• No Iron and folic-acid supplementation: ↑1.82 times
• Women in third trimester of pregnancy: ↑2.37 times
• Women who had low dietary diversity score: ↑3.59
6

Physiological changes in pregnancy vs.
Anaemia
• Plasma volume: ↑50%
• Red Cell Mass: ↑15-30%
• Graph of Hb is ‘U-shaped’ not linear
• Erythropoiesis: ↑MCV (up to 60fl), MCHC↔
• ↑Fe utilization: ↓serum Fe & Ferritin, ↑ TIBC
• ↑Folate requirement
• Hb: ↓20g/L from pre-pregnany level
7

Etiologic classification4
Blood loss
• Acute: APH
• Chronic: Hookworm, Bleeding
Hemorrhoid or PUD
Nutritional
• Iron, Folic acid or Vit B12
deficiency
Bone marrow failure
• Aplastic anaemia
• Isolated secondary failure of
erythropoiesis
• Drugs: Chloramphenicol,
Zidovudine
Haemolytic
-Inherited
• Red cell membrane defects
• Enzyme deficiency:G6PD
-Acquired
• Infections: Malaria, HIV
• Immune haemolytic anaemia
• Non-immune haemolytic
anaemia
• Systemic diseases: renal, liver
8

Morphological classification4
Hypochromic Microcytic
• Fe deficiency
• Thalassemia
• Sideroblastic anaemia
• Anaemia of chronic disorders
• Lead poisoning
Macrocytic
• Folic acid deficiency
• Vit B12 deficiency
• Liver disease
• COPD
• Myelodysplastic syndromes
• Anaemia from blood loss
Normocytic Normochromic
• Autoimmune haemolytic
anaemia
• SLE
• Bone marrow failure
• Malignancies
• Anaemia from blood loss
• Anaemia of chronic disease
9

Variations in Definition of Anaemia in
pregnancy
• Non-uniform definition of anaemia in pregnancy
WHO5: Antenatal Hb < 110 g/L and postnatal < 100 g/L.
British Committee for Standards in Haematology guidelines6
• Hb level < 110 g/L in the first trimester
• Hb < 105 g/L in the second trimester
• Hb < 100 g/L postpartum period.
Nigeria: for practical purposes 100g/L
Definition of the severity5
• Mild: Hb 100-109g/L (10.0-10.9g/dl)
• Moderate: Hb 70-99g/L (7.0-9.9g/dl)
• Severe: Hb <70g/L (<7.0g/dl)
10

Fe homeostasis7,8
• Fe required for fetal growth and development originates from
mother
Maternal Fe requirement
• Decreases in early pregnancy: cessation of menses
• Increases to up to 3-8mg/day in late pregnancy
• 0.8mg/day 1st trimester to7.5mg/d in 3rd trimester
• Average requirement: 4.4mg/d throughout pregnancy
• Total body iron requirement for uncomplicated pregnancy:
1000-1500mg : fetus/placenta: 350mg; increase in maternal
RBC mass 450mg; bleeding during/after delivery: 250mg.
• To accommodate these, woman should have 500mg store
before, and consume 20mg-48mg dietary iron per day
11

Fe deficiency Anaemia (IDA)
Refers to anaemia with
inadequate serum Iron
Causes of deficiency:
• Inadequate nutritional
intake:
-Malnutrition
-Low socioeconomic status
-Vegetarianism
-Chronic illness
-Malabsorption due to celiac
disease
• Chronic blood loss
-Esophageal varices
-Bleeding peptic ulcer
-Inflammatory bowel disease
-Hookworm infestation
-Hemorrhoids
• May be precipitated by
increased demand of
pregnancy or growth spurt
of adolescents
12

Folic Acid deficiency
• Folic acid is a cofactor in nucleic acid synthesis and
has important role in cell division.
• Stores are limited (6-10mg); Daily requirement of
300-500µg.
• Deficiency causes Megaloblastic anemia.
• Risk factor: Multigravida, twin pregnancy,
Hyperemesis gravidarum, alcohol consumption,
smoking, malabsorption, antiepileptic drugs.
• Effects on mother: miscarriage
• Effects on Fetus: Neural tube defects, Cleft palate,
Preterm Birth
13

Approach to management
• Often asymptomatic- incidental finding on routine
screening
• Evidence of Hypoxia: Tiredness, dizziness, fatigue and
decreasing capacity to perform daily tasks.
• There may be pallor, dyspnea, palpitation, headache,
lightheadedness (and episodes of fainting), and
irritability.
• General examination: Glossitis, Stomatitis, Koilonychia,
pedal edema
• Systemic examination: Tachycardia, Tachypnea, Basal
crepitation if in Heart Failure with third Heart sound
14

Screening for Antepartum Anaemia
Screening 9
• Assumption that normal Hb
implies normal Fe level
Aim: Determine aetiology
• Hb
• Serum ferritin
• Iron saturation
• Total Iron Binding Capacity
• Reticulocyte count
• Reticulocyte Hb content
• Folate level
• Vit B12 level
British Society of Haematology 6
recommends measurement of
serum Ferritin in women with-
• Haemoglobinopathy
• Previous parenteral Fe therapy
• Previous anaemia
• Multiparity
• Inter-pregnancy interval <1 year
• Teenage pregnancy
• Recent bleeding episode
• High risk of bleeding in index
pregnancy
• Jehovah witnesses or vegetarians
15

Diagnosis of Fe deficiency in
pregnancy 9
• Serum ferritin : most widely used laboratory test
• Ferritin is an intracellular protein found at a number of
sites (e.g. liver & spleen) which store and release iron in a
controlled fashion.
• Small quantities of ferritin present in human serum.
Serum ferritin level can assess body’s total iron storage
• Note: Ferritin is an acute phase protein and increases
during active inflammation, malaria.
• Most Physicians use: cut off value of <30 μg/L.
• The threshold has 90% sensitivity and 85% specificity for
detecting iron deficiency during pregnancy
16

Laboratory testing
• Hb: booking, 28weeks
• Red cell indices
-Low Hb, MCV, MCH, MCHC: suggest Fe deficiency
note: MCV rise in pregnancy (6fl)
-Serum Fe reduces in pregnancy: 12µmol/L and
TIBC<50µmol/L indicate Fe deficiency
Another study10 reported that MCHC most sensitive
in early prediction of IDA: this needs further
validation
17

Laboratory testing- 2
• Peripheral blood smear – microcytosis, hypochromia,
anisocytosis, poikilocytosis and target cells
• RBC indices:↓MCV, ↓MCH, ↓MCHC, MCV is the most
sensitive indicator
• ↓ Serum ferritin – first abnormal laboratory test
• ↓ Transferrin saturation – second to be affected
• ↑ Serum transferrin receptor – best indicator
• Bone marrow examination – no response to treatment
after 4 weeks of therapy
• Stool examination – for three consecutive days
18

Complications of anaemia in
pregnancy
Foetal
• Stillbirth, Placenta Abruption
• Fe status compromised with Maternal Hb <85g/L, Ferritin < 13.4
µg/L
Maternal
• Preterm labour,↑ intervention during labour including CS, ↑Risk
for PPH, maternal death, postpartum depression, altered maternal-
infant bonding, ↑blood transfusion
Neonates:
• LBW & increased NICU admission
• Anaemia, neurodevelopmental disorders- low IQ score, poor school
performance, visual & motor coordination defects, subnormal
language development.
19

General Approach12 - RCOG
• Screen at booking, then 28 weeks
• Normocytic or microcytic anaemia: oral iron,
check for rise at 2 weeks (assess compliance).
• Parenteral iron is indicated with no response
• Provide information on dietary advice
• Encourage hospital delivery
• Active management of third stage of labour to
minimize blood loss
20

Oral Fe
• Oral Fe is the first‐line treatment for IDA in pregnancy
• Oral dose of iron is 100–200 mg of elemental iron daily.
• Ferrous salts can be taken on an empty stomach to increase absorption
• Iron polymaltose preparations can be taken with food.
• Challenges of compliance: GI side effects (nausea, diarrhoea, constipation.
• Expected rise in Hb is 10 g/L over a two‐week period.
• If response is adequate, continue maintenance therapy until delivery.
• Take with water or a source of Vit C, preferably in the morning (lowest
hepcidin level), avoid concomitatnt use with antacid or multivitamin.
• >80mg elemental iron per day increases GI side effect
21

Parenteral Fe
• Poor / absent response to oral iron (Hb rise <10 g/L within 14–
28 days)
• Lack of compliance or intolerance to oral iron
• Severe anaemia (Hb <80 g/L) with no symptoms or need for
immediate transfusion
• Need for timely and rapid treatment in the 3rd trimester
• Women at high risk for major bleeding (e.g. placenta previa).
• IV iron offers earlier replenishment of total body iron stores/
timely increase in Hb
• Available IV iron preparations: Iron sucrose, Iron gluconate,
low molecular weight iron dextran, ferric carboxymaltose, iron
polymaltose complex, iron isomaltoside, and ferumoxytol
22

Safety profile of IV Fe
• Acute severe reaction-uncommon
• Doses administered are insufficient for parenchymal damage
• Increased risk for infection and CVS diseases- unproven
• Initially bioactive free iron, now Fe-CHO complexes reduced
toxicity
• Commonest formulations: Ferrous Carboxymaltose, Fe
Isomaltoside, Ferumoxytol: Rapid infusion, no premedication,
adverse effects are uncommon
• Contraindication: Previous anaphylaxis to parenteral Fe,
decompensated liver disease 6
• Hypophosphatemia after IV Fe: (especially FCM)- ongoing
research in Nigeria (IVON Trial- Prof Afolabi et al)
23

Parenteral Fe (contd)
Calculating Iron deficit
• Elemental iron needed (mg) = [(Desired Hb –
Patient’s Hb)g/L x Weight (kg) x 0.24] +50% (to
replenish the store)
• Fe Carboxymaltose: given IV, comes in
50mg/ml formulation, dilute in 200ml N/S, no
need for test dose, no premedication, can be
given over 15-20minutes, maximum dose in
1000mg. See manufacturer’s brochure
24

Assessing response to therapy
• Sense of well being
• Improved outlook of patient
• Increased appetite
• ↑ Hb: 2 weeks after commencement
• Reticulocytosis within 5-10 days
• If no significant clinical or haematological
improvement in 3 weeks, Re-evaluate
25

Indications for blood transfusion
• Severe anaemia with severe symptoms
(Hb<7g/dl)
• Acute blood loss with continuing bleeding
• Women at risk for additional bleeding
• Imminent cardiac compromise
• Severe anaemia beyond 36 weeks
• Refractory anaemia
• Non-response to Iron therapy
26

Blood transfusion cases
• General recommendations: Compatible,
screened, cross-matched, no TTI
• Packed cells preferred over 4-6 hours and given
alternate days
• For acute blood loss, replacement may be faster
• End point: Hb 9g/L before 34 weeks and 11g/L
after 36 weeks
• Prophylaxis against infection in severe anaemia:
↓low resistance to infection or actual infection
27

Peripartum management
First stage
• Make Comfortable, Avoid
maternal stress, Analgesia
• Partograph
• Adequate oxygenation
• Avoid sympathetic
stimulation &
hyperventilation: rightward
shift of ODC
• Improve uterine blood flow
Second stage:
• Shorten (forceps)
Third stage
• Active management of third
stage, Prophylaxis for PPH
Puerperium
• Adequate rest
• Iron & folate therapy for 3/12
• Sepsis: Prophylaxis, watch
out and treat early
• Others: failure of lactation
Uterine sub involution
Thromboembolism
28

Postpartum anaemia
• There is lack of consensus on the definition of
postpartum anaemia
• WHO: Hb <100g/L
• USA (CDC): Hb <118 g/L 13
• The RCOG and the British Committee for
Standards in Haematology6: Hb <100 g/L
• The Swiss Society of Gynaecologists and
Obstetrics15: Hb <120 g/L.
29

Screening for PP Anaemia 9
• Universal versus selective screening
• Selective testing: no reliable, validated risk assessment screening tools and the
estimation of blood loss associated with delivery is often inaccurate.
• The optimal time point for testing is controversial (6-48 hours)
• There are complex hormonal, hemodynamic and haematinic changes that
occur in postpartum period and after a normal delivery it may take 5–7 days
for the maternal extracellular and intravascular changes to reach equilibrium.
• Earlier testing: significant PPH and/or uncorrected antenatal anaemia.
• If anaemia is detected, assess body iron status to confirm Fe deficiency.
• Note: There is oxidative stress/ inflammatory response; elevated ferritin levels
may be present for up to one week postpartum.
• Expert opinion: suggests use of Ferritin after the first week postpartum
30

Screening & Management of
Postpartum Anaemia
Definition Swiss Society
for Gynae &
Obs 15
Expert Committee for
Asia-Pacific Region 16
Network for Advancement of
Patient Blood Management,
Haemostasis &Thrombosis
(NATA) Guideline 17
Definition (Hb) <120 g/L <100g/L <100g/L within 24-48 hours
Oral Fe Rx Treat when Hb
95-120 g/L
Hb 95-99g/L
start 24-48hr PP
Asymptomatic/ mild symptom
Mild-moderate anaemia
IV Fe Rx Hb 85-95g/L Hb 65-95g/L
Start 24-48hr PP
No response to oral Fe (2-4wks)
Intolerant of Oral Fe
Blood
transfusion
Hb <60-65g/L Hb <65 g/L
Unstable: cardiovascular
Poor response to IV Fe
At risk from IV iron
Hb <60 g/L (non-bleeding
patient) taking clinical
signs and symptoms into
consideration.
31

Recommendation: NATA in conjunction with
FIGO and EBCOG
• Screen for anaemia at booking, 28weeks, or any time if
symptoms of anaemia are present
• Microcytic or normocytic anaemia from ID: confirm by a trial
of oral iron (unless Haemoglobinopathies) or a serum ferritin
• Poor response to oral Fe: Serum ferritin plus other evaluation
• Anaemic women (Mediterranean, Middle/ Far East or Africa):
r/o Haemoglobinopathies
• Anaemia in known haemoglobinopathy: serum ferritin check
(give oral Fe only if <30 ng/mL).
• Areas with a high prevalence of anaemia in pregnancy:
Routine daily oral iron (30–60mg) and folic acid (400 𝜇g)
• Mild-moderate IDA (Hb≥80 g L) in 1st/2nd trimester: oral iron
+ folic acid 32

Recommendation: NATA in conjunction
with FIGO and EBCOG- 2
• Once the Hb is in the normal range, continue Fe supplementation for at
least 3months to replenish iron stores
• Consider IV Fe: severe IDA (Hb <80 g /L), IDA after 34 weeks of gestation
• Consider IV Fe: women with confirmed IDA who fail to respond to oral iron
(Hb increase <10 or 20 g/L in 2-4 weeks) or intolerant to oral Fe
• Give erythropoiesis stimulating agents (ESA): moderate-severe anaemia
not responding to IV Fe due to inappropriate synthesis of, and/or response
to, endogenous erythropoietin levels, in consultation with a haematologist
• Make every effort to correct anaemia prior to delivery + Hospital delivery
• Active management of the 3rd stage of labour to ↓blood loss
• Mild-Moderate PPA: Give 80–100mg elemental Fe daily for 3 months if
haemodynamically stable and asymptomatic or mildly symptomatic
33

Knowledge Gap/ Research
Opportunities
1. Methods of Diagnosis
(Hb measurement) 18
Detailed cost-analysis of
accurate tests
Method Sensitivity
(95% CI)
Specificity
(95%CI)
Clinical
Assessment
56% (19-92) 62% (30-93)
Haemoglobi
n colour
scale
67% (56-76) 67 %(48-82)
Cu sulphate
test
97% (88-
100)
71% (55-85)
Sahli 86% (75-
94%
83% (68-93)
Hemocue 85% (79-90) 80% (76-83)
Non-
invasive Hb
sensor
(HBM 2000)
34% (27-41) 92 (82-97)
34

2. Hb Cut-off to define Anaemia
• Universal vs. Locality cut-off: Ethnicity /geography
• GA-related cut-off
• Maternal age specific cut-off
GA Specific Cut-off
• Based on the non-linear relationship of Hb with GA
China19: 143,307 singleton pregnancies, 139 hospitals
• Mean Hb: 125.75g/L (T1), 118.71g/L (T3)
• Reference for anaemia: T1: 108g/L; T2: 103g/L; T3:
99g/L
35

3. Daily iron and folic acid
supplementation
WHO recommendation 20
• Fe: 30-60mg elemental Fe + Folic acid: 400µg (0.4mg)
Randomized double-blind, intention-to-treat study comparing
20mg, 40mg, 60mg, 80mg oral Fe fumarate (comparable groups)21
• Serial Evaluation with Fe status markers (Hb, serum ferritin,
soluble transferrin receptors) at 18/52, 32/52, 39/52 GA; 8/52 PP
• 20mg group- ↓parameters at 32/52, 39/52
• No significant difference between 40mg, 60mg & 80mg
• Side effects: not significant in all 4 groups
• 40mg is appropriate for supplementation
30 mg elemental Fe (150mg Fe SO4, 90mg Fe fumarate or 250mg Fe
gluconate)
36

4. Dosing regimen 22
• Non-inferiority study of dosing for oral iron
• Thrice weekly (TIW) vs. daily dosing (TID)
• Primary outcome: ↑Hb ≥3g/dl
• Secondary: Adverse effect, RBC indices, Fe profile,
compliance
• Recovery of TID more rapid but ALL participants had
recovered by 4 weeks of study
• No statistical difference in Biomarkers assessed
• TIW is not inferior to TID
• TIW fewer GI adverse effect, lower cost
37

Haemoglobinopathies
• Haemoglobinopathies are conditions in which
there is an inherent haemoglobin defect resulting
in abnormal (e.g. sickle cell) or reduced globin
formation (e.g. thalassemia)
• SCD is commoner: autosomal recessive disorder
characterised by abnormal Hb genotype with
occurrence of Sickle cell haemoglobin (HbS) in
combination with another abnormal Hb
• The genes for inheritance are transmitted in the
Mendelian fashion, so both homozygous and
heterozygous forms occur
38

Haemoglobinopathies-2
• Characterized by sickling of RBC during physiological stress
leading to vaso-occlusion with pain crises, but can cause
more serious complications.
• SCD leads to ↑red cell turnover and a chronic haemolytic
anaemia which is further affected by the physiological
changes of pregnancy.
• The most common forms of SCD are:
- HbSS; HbSC; HbS β-thalassaemia.
• More rarely there are other causes of sickle cell disease:
- HbSD-Punjab; HbSE; HbSO-Arab.
HbSC: ↓complications; but ↑Pain crises, IUGR, Antenatal
Hospitalization, PP Infection, requires same level of vigilance
as HbSS
39

Haemoglobin
• Hb S: Valine replaces Glutamic acid at position 6 of
the βglobin chain
• Hb C: lysine replaces glutamic acid at postion 6 of
beta chain
• Thalassaemias: reduction in the synthesis of either
alpha or beta chain
• Hb is a polypeptide with MW 64450, the oxygen
carrying pigment in the RBC
• Made up of 4 subunits, each subunit contains
heme moiety conjugated to a polypeptide
40

PATHOPHYSIOLOGY
• Deoxygenation causes valine to form hydrophobic
bonds with adjacent globin chains with insoluble
tetramas,which polymerises into long fragile and
rigid strands that deform the red cell membrane and
block small vessels causing pain crises.
• This phenomenon is known as sickling and it is
aggravated by an increased concentration of HbS
within the RBC, infection, acidosis, dehydration,
hypoxic state, extreme change of temprature and
stressful conditions including pregnancy.
• These cells are prone to increased breakdown, which
causes haemolytic anaemia
41

CLINICAL FEATURES
Associated with 2 major crises
• Anaemia: increased haemolysis, aplastic/ sequestration crises
• Pain crises: ischaemia from vaso-occlusion of micro
vasculature
Other clinical features
• Sickle cell facie
• Avascular necrosis of the head of femur (common in HbSC)
• Pelvic deformities
• Subfertility and Infertility; reproductive career may be marred
by high incidence of fetal loss.
42

INVESTIGATIONS
• Sickling test; Solubility test
• Haemoglobin electrophoresis.
• Full Blood Count and Blood Film
• Serum Ferritin, serum iron and TIBC
• Serum folate assay
• Urinalysis
43

Complications of SCD
Maternal Complications
• Worsening anaemia
• Increased risk of
infections, particularly UTI
and chest infection
• Increased sickle cell crises,
particularly in the third
trimester
• Acute Chest syndrome
• Hypertension and pre-
Eclampsia
• Thromboembolic disease
Foetal
• Inheritance of HbS gene
• Miscarriage
• IUGR
• IUFD
• Preterm delivery
• Stillbirth
• Opiate toxicity in neonate
44

PRE-CONCEPTION CARE
• Aim: To optimize the woman
• Should plan their pregnancies and offered pre-conception care by MDT
• Discuss risks related to pregnancy
-Drug review (potential teratogenicity)- D/C Hydroxycarbamide at least 3
months before pregnancy (not an indication for termination); ACE inhibitors,
Angiotensin II receptor blockers, Hydroxyurea and chelation therapy.
-Ensure Folic acid and Prophylaxis with Proguanil
-Pain management: PCM, Codeine, NSAID, Opioid
-Penicillin prophylaxis: encapsulated bacteria e.g. (N meningitidis, Strep
pneumonia, H influenza)
• Perform Genetic screening/ partner testing: appropriateness of PGD, NIPT
• Vitamin D deficiency is common- Regular monitoring and supplementation
• History & Physical examination
• Pre-conception review of chronic complications of SCD: Renal, HTN, CVA,
AVN
45

Antenatal care
• MDT: Obstetrician, Haematologist, Midwives, counsellor
• Revisit Prenatal care
• Genetic screening: prenatal test
• Appointment: Individualize, Monitor- Hb, BP, Urinalysis, etc.
• Fe supplementation: only for proven deficiency
• Report & treat Hyperemesis promptly
• Multiple gestation: higher risk, closer monitoring
• PIH: Higher risk Aspirin prophylaxis from 12 weeks
• Risk assessment for VTE +/- Prophylaxis
• Serial USS: 1st trimester, 20, 24 weeks then every 4 weeks
• 36 weeks: Review Birth plan
46

Blood transfusion24-26
• Consider in: worsening anaemia, acute SCD complications,
• Women on long-term transfusion for stroke prevention or to ameliorate severe SCD
complications should continue throughout pregnancy
• Standard care vs. Prophylactic
Meta-analysis on prophylactic transfusion25: ↓VOC, Preterm delivery, maternal/
perinatal mortality, neonatal death. No difference: UTI, PE, Acute chest syndrome,
SGA, LBW, IUFD
• When?- Poor Clinical status, Complications (ACS, Intractable pain), Hb<60g/L
• Optimal Hb before CS: Inconclusive, (Hb >90g/L ↓ post-op sickle complications
[ACS]) 26
• Give ABO-compatible, Rh, Kell and CMV Negative, Matched blood. If woman had
significant Red cell antibodies, give blood without the corresponding antigens
Consider prophylactic transfusion:
• Previous or current medical, obstetric or fetal problems related to SCD
• Women on hydroxycarbamide before pregnancy
• Multiple gestation
47

Acute Pain Crises
• Commonest complication in pregnancy
• ↑Antenatal and postpartum period for HbSS
• Why?
-Physical/Psychological stress, dehydration, worsening
anaemia, ↑Red Cell turnover, pro-coagulant state of
pregnancy, ↑Infection risk
• Mild: rest at home, oral fluids, PCM, weak opioids, NSAID.
• Severe: MDC, Admission, IVF (caution in Renal Disease, PE),
Opioid, (Avoid Pethidine- Associated seizure), Oxygen- keep
SPO2>95%, precipitating factor, Antibiotics- infections,
Thromboprophylaxis (LMW Heparin), +/-Blood transfusion
• Monitor with pain score; ICU care if no improvement.
48

Acute Chest Syndrome
• Life threatening complication, occurs in 10% of women
• May develop before or after admission for other reasons
• Fever and/or respiratory symptoms, hypoxia
• FBC, Chest x-ray (pulmonary infiltrates), Arterial blood
gases
• Precipitated usually by infection: search for the focus
• Pain relief, rehydration, Spirometry, treat infection (bacteria
or viral), Blood transfusion especially in hypoxic women
(simple or exchange transfusion)
• Critical team care: ICU care
• If blood transfusion is necessitated, may need prophylactic
transfusion for the rest of the pregnancy.
49

Venous Thromboembolism (VTE) and
Thromboprophylaxis
• SCD increases risk for VTE and DVT during
pregnancy and Puerperium
• VTE risk 3-5 in women with complications: VOC,
etc.
• Risk assessment: early pregnancy, if admitted,
Intrapartum, postpartum periods
• Thromboprophylaxis from 28weeks till 6weeks PP,
If there are additional risk factors, start from
beginning of pregnancy
• Offer Thromboprophylaxis for VOC or other pain
crises
50

Labour and Delivery
• When to deliver: If pregnancy is uncomplicated, delivery should be planned for 38 to 40 weeks
• Mode of delivery: will be determined by obstetric factors, no contraindication to VBAC
• Delivery at a facility with MDT and can manage probable complications
• Optimal Intrapartum care:
-Avoid hypothermia- Keep warm
-Avoid hypoxia: Oxygen supplementation
-Adequate hydration
-Adequate analgesia: Epidural is preferred
-Avoid prolonged labour (>12 hours)- Partograph
-Antibiotics- low threshold
-Available grouped/crossmatched blood: 2 units of compatible Hb AA blood
-Continuous electronic fetal monitoring
-Serial Hb and Urinalysis
-Shorten second stage of labour
• In unplanned delivery/emergency, reverse heparinisation using protamine sulphate when the
second stage of labour is imminent or immediately before an operative delivery
51

Postpartum care
• Remain vigilant: (20-25% crises are postnatal)
• Maintain hydration, oxygenation, analgesia
• Early ambulation
• Other routine care including breastfeeding
• If baby is at higher risk of SCD, send samples to laboratory
with facilities for early diagnosis
• Antithrombotic stockings
• Thromboprophylaxis- up to six weeks
• Contraception: Individualize, Progestagen-only methods
reduce risk of sickle pain crises. Barrier method, Sterilization,
IUS can be used.
52

Thalassemia
• Group of inherited blood disorders with abnormal formation of RBC
• Women may be transfusion dependent or non-transfusion dependent
• Transfusion dependent women need their medical care optimized before
pregnancy where possible as this can be associated with organ damage
due to iron overload (cardiac disease, diabetes). This can lead to increased
risks to the mother and safety of pregnancy should be considered. Iron
chelation should be reviewed, and where possible, stopped 3 months pre-
conception.
• Non-transfusion dependent women may require transfusion support in
pregnancy due to the physiological changes which occur and so should be
monitored by a specialist team.
• Most other care similar to SCD
53

CONCLUSION
• In view of the common nature of anaemia in
pregnancy, facilities should have protocols for the
management.
• Since most Haemoglobinopathies are inherited as
autosomal recessive disorders, screening counselling
and prenatal diagnosis are important
• Social support for these women is mandatory, as the
diseases is a major drain on their emotional, physical
and financial reserves.
54

References
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System, 1993-2005. Public Health Nutr. 2009;12(4): 444-454.
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sub-Saharan Africa: A systematic review and Meta-Analysis. Arch Public Health. 2021;79(1):219.
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References- 2
9. Butwik AJ, McDonnell N. Antepartum and Postpartum Anaemia: A Narrative review. Int J Obstset
Anaestet 2021;102985
10. Rabindrakumar MSK, Pujitha WV, Gooneratne L, Arambepola C, Senanayake H, Thoradeniya T. The
role of haematological indices in predicting early iron deficiency among pregnant women in an urban
area of Sri Lanka. BMC Hematology. 2018;18(1):37.
11. Wiesenack C, Meybohm P, Neef V, Kranke P. Current concepts in preoperative anaemia management
in obstetrics. Curr Opinion Anaes. 2023;36:255-262.
12. RCOG. Blood transfusion in obstetrics. Green Top Guideline No. 47. May 2015.
13. Centers for Disease Control and Prevention. Recommendations to prevent and control iron
deficiency in the United States. MMWR Recomm Rep. 1998;47(1):1–29.
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pregnancy and postpartum. Arch Gynecol Obstet. 2017;296(54):1229–1234.
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for the diagnosis and treatment of iron-deficiency anemia during pregnancy and the postpartum period
in the Asia-Pacific region. J Perinat Med. 2011;39:113–121.
56

References- 3
16. Munoz M, Pena-Rosas JP, Robinson S, Milman N, Holzgreve W, Breymann C, et al.
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deficiencies in pregnancy and in the post-partum period: NATA consensus statement.
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57

References- 4
23. NHS. Haemoglobinopathies in Pregnancy: Guideline for
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58

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