This presentation talks about Haematological disorder in pregnancy specifically sickle cell disease in pregnancy. It's epidemiology, clinical presentation, diagnosis, management and it's prognosis

1. PRESENTER: Dr. Asma Zain
FACILITATOR: Dr. Amani

2. OUTLINE
 Introduction
 Epidemiology
 Pathophysiology
 How SCD affects pregnancy
 How pregnancy affects SCD
 Preconception, Antenatal, Intrapartum and
Postpartum care
 Summary
 References
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3. INTRODUCTION
 SCD is a group of inherited single-gene
autosomal recessive disorders.
 It is caused by a point mutation in the β globin
gene on chromosome 11.
 This causes substitution of valine for glutamic
acid at position 7 of the β-chain of normal
haemoglobin.
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4. INTRODUCTION
 Inheritance of one abnormal and one normal allele
confers sickle cell trait (a carrier state without
clinical symptoms),
Inheritance of two mutated alleles causes sickle cell
disease, characterized by varying amounts of
chronic hemolytic anemia, recurrent debilitating
pain and an array of clinical sequelae.
If both parents carry one HbS gene, the fetus has a
25% chance of having sickle cell disease, 50%
chance of having sickle cell trait, and 25% chance of
being unaffected
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5. INTRODUCTION
 SCD encompasses Sickle cell Anemia (SCA)
which possesses SS genotype, some heterozygous
conditions of the S gene and other clinically
abnormal hemoglobins such as beta thalassemia,
hemoglobin C, D, E and others.
 The combination of Sickle gene with any of
these abnormal hemoglobins will lead to a similar
clinical picture of SCD although the severity of
the disease and prevalence differ.
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6. INTRODUCTION
 Complication rates are lower in HbSC than
HbSS
 All pregnancies in women with SCD should
be treated as high risk and require the same
level of monitoring and care.
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7. INTRODUCTION
 SCD includes;
 HbSS: Sickle cell anemia
 HbSC
 HbSB
 HbSD
 HbSE
 HbSO-Arab
 HbAS: Sickle cell trait
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heterozygous conditions of haemoglobin S

8. EPIDEMIOLOGY
 It is currently estimated that about 400,000
children with SCD are born each year globally,
75% of them live in Sub-Saharan Africa.
 Tanzania rank 4th in the world with the highest
estimated number of newborns with SCD a year
(Nigeria 85,000, Democratic Republic of Congo
42,000, India 38,000; Tanzania 11,000 and
Uganda 10,000)
 In Tanzania, the incidence of SCD in pregnancy
is increasing.
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9. PATHOPHYSIOLOGY
 SCD is a consequence of polymerisation of the
abnormal haemoglobin in low-oxygen
conditions, which leads to the formation of rigid
and fragile sickle-shaped red cells.
 These cells are prone to increased breakdown,
which causes the haemolytic anaemia, and to
vaso-occlusion in the small blood vessels.
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10. PATHOPHYSIOLOGY
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11. EFFECT OF SCD ON PREGNANCY
 There is increased incidence of abortion,
IUGR, fetal stress, preterm labor, delivery by
c/s and stillbirths; and hence increase perinatal
mortality.
 Incidence of infection, thromboembolic
events, pre-eclampsia, abruption placentae and
postpartum hemorrhage is increased.
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12. EFFECT OF SCD ON PREGNANCY
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14. HOW PREGNANCYAFFECTS SCD
 There is consistent evidence that anemia and vaso-
occlusive or acute painful episodes occur more often in
pregnancy.
 Painful episodes are more common with advancing
pregnancy and postpartum.
 The vast majority of the acute vaso-occlusive events (pain
and acute chest syndrome) occur during the third trimester
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15. PRECONCEPTION CARE
 Testing partner for hemoglobinopathy
 Measurement of baseline blood pressure
 Retinal evaluation
 Chemistry panel, urinalysis, and 24-hour
protein excretion
 Haemoglobin/haematocrit and ferritin level
 Baseline urine culture
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16. PRECONCEPTION CARE
 Hepatitis B and C screening
 Serologic red cell phenotyping and screening
for red cell alloimmunization
 ECHO (Pulmonary hypertension)
 Polyvalent pneumococcal, Haemophilus
influenza type B, meningococcal vaccines and
hepatitis B
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17. PRECONCEPTION CARE
 Folic acid supplementation 1mg/day
 Penicillin prophylaxis or the equivalent**
 Hydroxyurea should be stopped at least 3
months before conception.
 Iron chelators should be stopped soon after
conception.
 ACEIs and ARBs should be stopped before
conception.
 Genetic counselling
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18. ANTENATAL CARE
 Multidisciplinary plan for antenatal care
 Stop hydroxyurea, ACE inhibitors, ARBs
 Folic acid 5 mg daily
 Advised not to use NSAIDS in the first 12
weeks or after 28 weeks.
 Low dose Aspirin 75mg from 12weeks
 Assess the need for LMWH
 Advice re crises prevention measures - warmth,
rest, hydration.
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19. ANTENATAL CARE
 Persistent vomiting can lead to dehydration
and sickle cell crisis and women should be
advised to seek medical advice early.
 The influenza vaccine should be
recommended if it has not been administered
in the previous year
 Prophylactic antibiotics*
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20. ANTENATAL CARE
 Iron supplementation (laboratory evidence of
iron deficiency)
 Blood pressure and urinalysis should be
performed at each consultation, and midstream
urine for culture performed monthly.
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21. ANTENATAL CARE
 Alloimmunization should be assessed at the
first prenatal visit, repeat at 24-28weeks if
negative and when the patient is admitted at
the time of delivery.
 Malaria prevention (SP)
 Close attention to multiple gestation.
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22. ANTENATAL CARE
 Ultrasound scanning
 7-9weeks: Viability scan
 11-14weeks: Routine first trimester scan
 20weeks: Detailed anomaly scan
 Serial fetal biometry scans (growth scans)
every 4 weeks from 24 weeks of gestation.
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23. ANTENATAL CARE
 Prenatal diagnosis
 Chorionic villus biopsy at 10 to 13 weeks of
gestation.
 Amniocentesis as early as 15 to 16 weeks.
 Fetal blood sampling can be performed after 20
weeks of gestation
 Detection of fetal SCD by evaluation of cell-free
DNA in maternal plasma is under investigation.
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24. ANTENATAL CARE
 Prophylactic and selective (on-demand)
chronic transfusion therapy
 The use of chronic transfusion therapy
prophylactically throughout pregnancy is
controversial.
 Guidelines and meta-analyses support
continuation of prepregnancy chronic transfusion
therapy.
 In addition, they recommend selective (on-
demand) transfusions for particular indications.
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25. ANTENATAL CARE
‘Top-up’ transfusion is indicated for women with
acute anaemia.
Hb under 6 g/dl or a fall of over 2 g/dl from
baseline.
Exchange transfusion is indicated for acute chest
syndrome or acute stroke.
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26. SC CRISES IN PREGNANCY
 Chest pain: painful crisis (rib), chest infection,
pulmonary embolus, chest crisis
 Jaundice: haemolysis, gallstones, obstetric causes
(HELLP, fatty liver, obstetric cholestasis)
 Abdominal pain: sickle crisis, gallstones,
pyelonephritis
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27. SICKLE CELL CRISIS
 Full blood count, reticulocyte count and renal
function.
 Blood cultures, chest X-ray, urine culture and
liver function tests.
 Pain relief (give within 30 minutes, effective 1
hour)
Paracetamol, codeine, morphine, ± NSAIDs
(12-28/40)
Laxatives, anti-pruritic and antiemetic
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28. SICKLE CELL CRISIS
 Oxygenation and hydration (An initial IV bolus
of 0.5 to 1 L of normal saline over the first hour
then 125mls/hr OR 60mls/kg/24hrs)
 Therapeutic antibiotics (febrile or there is a high
clinical suspicion of infection)
 Thromboprophylaxis should be given to women
admitted to hospital with acute painful crisis.
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29. INTRAPARTUM
 Induction of labour, or by elective caesarean
section if indicated, after 38+0 weeks of
gestation.
 Keep warm and given adequate fluid during
labour.
 Continuous Intrapartum electronic FHR
monitoring is recommended.
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30. INTRAPARTUM
 Oxygen therapy instituted if oxygen saturation
is 94% or less.
 Sickle cell crisis in labour should be treated as
per the guidance for antepartum crisis.
 Epidural anaesthesia has been used
particularly for acute pain episodes around
labor and delivery.
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31. INTRAPARTUM
 Preoperatively, the patient should be well-
hydrated and oxygen saturation should be
maintained at ≥95%.
 Transfusion with target haemoglobin 10 to 11
g/dL is reasonable.
 Regional anaesthesia is generally safer and
preferable.
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32. INTRAPARTUM
 Fluid balance is important because these
patients are at risk for fluid retention from
subclinical cardiomyopathy.
 A cord blood specimen.
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33. POSTPARTUM
 High risk babies of SCD, Early testing for SCD
should be offered.
 LMWH should be administered while in hospital
7 days post-discharge following vaginal delivery
6 weeks following caesarean section.
 Mechanical thromboprophylaxis is recommended.
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34. POSTPARTUM
 Breastfeeding should be encouraged
(hydroxyurea+iron chelators are
contraindicated)
 Contraception
POP, DMPA, LNG/ETG, LNGIUD: MEC 1
CHC: MEC 2
 There is limited evidence that the use of
DMPA reduces the frequency of acute painful
episodes
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35. REPRODUCTIVE HEALTH PROMOTION
AND PRECONCEPTION COUNSELLING
 Should be offered to all adolescents and young
adults with SCD as well as women with SCD
postpartum.
 For women who wish to become pregnant,
offer preconception counselling, and for those
who do not wish to become pregnant, offer
contraception counselling.
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36. SUMMARY
 Pregnant women with SCD are at increased
risk for adverse outcomes.
 Preconceptional evaluation and counselling
should be thorough.
 Adequate hydration and oxygenation, keeping
warm, and measures to reduce infection are
important to reduce the risk of vaso-occlusion.
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37. SUMMARY
 Access to a multidisciplinary care team
knowledgeable about SCD and high-risk
obstetrics can significantly decrease morbidity
and mortality.
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38. REFERENCES
1. Manchikanti A, Grimes DA, Lopez LM, Schulz KF. Steroid hormones for contraception in
women with sickle cell disease. Cochrane Database Syst Rev. 2007;(2). doi:
10.1002/14651858.CD006261.pub2.
2. Bellina JH, Bickers JN. Modern management of sickle cell disease in pregnancy. South Med
J. 1974;67(4):426–30. doi: 10.1097/00007611-197404000-00013
3. Muganyizi PS, Kidanto H. Sickle Cell Disease in Pregnancy: Trend and Pregnancy
Outcomes at a Tertiary Hospital in Tanzania. PLoS One. 2013;8(2):0–4. doi:
10.1371/journal.pone.0056541
4. Smith-Whitley K. Complications in pregnant women with sickle cell disease. Am Soc
Hematol (United States). 2019;2019(1):359–66. doi: 10.1182/hematology.2019000039
5. Lemercier D, Habibi A, Albinni S. Transfusion-related adverse events are decreased in
pregnant women with sickle cell disease by a change in policy from systematic transfusion
to prophylactic oxygen therapy at home: a retrospective survey by the international sickle
cell disease observato. Am J Hematol. 2018; doi: 10.1002/ajh.25097
6. MINISTRY OF HEALTH, COMMUNITY DEVELOPMENT G, CHILDREN EA. UNITED
REPUBLIC OF TANZANIA, SICKLE CELL DISEASE CLINICAL MANAGEMENT
GUIDELINES. 2020;First Edit.
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