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Question 1

What is the most inherited condition worldwide?

Answer 1

SCD

Question 2

Women with sickle cell trait AS
Are at increased risk of what?

Answer 2

UTI
Microscopic haematuria

Question 3

What is the pathophysiology of SCD?

Answer 3

Polymerization of abnormal haemoglobin in low O2 conditions 👉formation of rigid and fragile sickle-shaped red cells
🔴 these cells are prone to increased breakdown 👉hemolytic anaemia/ vaso - occlusion in small vessels.

Question 4

What are the major complications of SCD?

Answer 4

Strock - pulmonary hypertension- renal dysfunction- retinal disease- leg ulcers- cholelithiasis- avascular necrosis.

Question 5

What are the additional risks in women with SCD in pregnancy?

Answer 5

Perinatal mortality
Premature labour
FGR
Spontaneous miscarriage
————————————-
Acute painful crisis
Maternal mortality
CS
Preeclampsia
Infection
Thromboembolism events
Antepartum haemorrhage

Question 6

What are the additional risks in women with HbSC in pregnancy?

Answer 6

Painful crisis during pregnancy
FGR
Antepartum hospital admission
Postpartum infections

Question 7

What are the informations that are relevant for women with SCD who are planning to conceive?

Answer 7

1- dehydration- cold- hypoxia- overexertion- stress 👉crisis
2- nausea/vomiting 👉dehydration
3- in pregnancy: ⬆️risk of:
* crisis
* acute chest syndrome ACS
* infections ( UTI)
4- ⬆️ risk of FGR👉⬆️fetal distress 👉⬆️labour induction 👉⬆️CS
5- chance of their baby affected by SCD

Question 8

How to make an assessment for SCD complications preconceptually?

Answer 8

1- screening for pulmonary hypertension ( if it hasn’t been carried out in the last year)
2- BP / urine analysis
3- renal / liver function
4- retinal screening ( proliferative retinopathy)
5- iron overload ( ⬆️ ferritin)
6- red cells antibodies

Question 9

How screening for pulmonary hypertension should be performed preconceptually?

Answer 9

With echocardiography
❤ Tricuspid regurgitation jet velocity of > 2.5 m/s is associated with high risk of pulmonary hypertension

Question 10

Which subgroup of SCD is at increased risk of proliferative retinopathy?

Answer 10

HbSC

Question 11

What are the conditions in the partner that require counseling and offer prenatal diagnosis? ( when the mother is affected by SCD)

Answer 11

• HbS
• B thalassaemia
• O- Arab
• HbC
• D- punjab

Question 12

What are the conditions in the partner that require counseling?
( when the mother is affected by SCD)

Answer 12

• DB thalassaemia
• lepore
• HbE
• hereditary persistent of fetal haemoglobin ( HPFH)

Question 13

What is the importance of antibiotic prophylaxis/ immunization in women with SCD?

Answer 13

As for all hyposplenic patients:
1- penicillin prophylaxis: at high risk of encapsulated bacteria such as :
* Neisseria meningitis
* streptococcus pneumonia
* Haemophilus influenza
Vaccination: for
* H. Influenza type b ✅
* conjugated meningococcal C ✅
As single dose
* pneumococcal vaccine ✅
Every 5 years
* Hepatitis B ✅
* influenza/ swine flu annually ✅

Question 14

What vitamin supplements should be given preconceptually?

Answer 14

5 mg folic acid
■[ outside pregnancy 1 mg ]■

Question 15

What medications should be reviewed preconceptually in women with SCD?

Answer 15

🔴 Hydroxycarbamide ( used to decrease the incidence of acute pain crisis) ❤ for 3 months preconception
🔴 ACEI - ARB ( uses to reduce proteinuria/ microalbuminuria )

Question 16

If the woman became pregnant while taking Hydroxycarbamide( hydroxuurea), how to manage?

Answer 16

It should be stopped
Level 3 US (for structural anomalies)
Termination IS NOT indicated

Question 17

What are the general aspects of antenatal care for women with SCD?

Answer 17

1- multidisciplinary team
2- review by hematologist: to be screened for end organ damage (if hasn’t been undertaken preconceptually)
3- avoid precipitating factors of crisis: * exposure to extreme Temp.
* dehydration ( vomiting..)
* overexertion
4- influenza vaccine ( if it hasn’t been administered in the previous year)

Question 18

If the woman with SCD hasn’t been seen preconceptually, what should be offered?

Answer 18

1- partner testing.
2- if the partner is a carrier 👉option of first trimester diagnosis and termination ( ideally 8- 10 weeks)

Question 19

What medication should be given during pregnancy to women with SCD?

Answer 19

1- folic acid
2- antibiotics prophylaxis ( penicillin)
3- iron supplements ONLY if there is an evidence of iron deficiency.
4- 🔴 Aspirin 75 mg from 12 weeks ( to reduce the risk of preeclampsia)
5- LMWH during antenatal hospital admissions
6- if NSAIDS are needed: ONLY between 12- 28 weeks

Question 20

What additional care should be provided during the antenatal appointments for women with SCD?

Answer 20

1- BP + urine analysis ( looking for proteinuria ) at each consultation.
2- midstream urine for culture performed monthly.

Question 21

What is the recommended schedule of US scanning during pregnancy for women with SCD?

Answer 21

1- viability scan 7-9 weeks
2- routine 11-14 weeks
3- detailed anomaly scan 18-20 w
4- from 24w: every 4 weeks

Question 22

What are the indications for blood transfusion in pregnancy complicated by SCD?

Answer 22

🔴 women with previous serious ( maternal/ fetal/ medical) complications 👉top-up OR exchange
🔴 women who are on transfusion regimen before pregnancy FOR:
-Primary or secondary stroke
-Prevention of severe complications
👉 transfusion should be continued
🔴 Twin pregnancy 👉prophylactic transfusion
🔴acute anaemia 👉top up
🔴Acute chest syndrome👉Exchange
🔴acute strock 👉Exchange.

Question 23

What are the general aspects of blood transfusion during pregnancy?

Answer 23

❤Routine prophylactic transfusion IS NOT recommended
* if acute exchange transfusion is required 👉continue for the remainder of the pregnancy.
* blood should be matched for extended phenotype: - full rhesus ( C,D,E) +kell typing
* blood should be cytomegalovirus negative.

Question 24

What is the prevalence of allo immunity in individuals with SCD?

Answer 24

18-36%

Question 25

What is the most frequent complication of SCD during pregnancy?

Answer 25

Painful crisis 27- 50 % Most frequent cause of hospital admissions.

Question 26

What is the optimal management of acute painful crisis during pregnancy in women with SCD?

Answer 26

1- excluded as a matter of urgency 2- multidisciplinary team 3- appropriate analgesia 🛑pethidine SHOULD NOT be used ( assessment risk of seizures) 4- assess the requirement of : fluid and oxygen. 5- thromboprophylaxis should be given to women admitted to hospital

Question 27

When a woman with painful crisis should be referred to hospital?

Answer 27

1- pain which doesn't settle with simple analgesia. 2- who are febrile 3- have atypical pain 4- have chest pain 5- have symptoms of shortness of breath.

Question 28

How to manage mild pain in a painful crisis during pregnancy?

Answer 28

🗯 in the community With rest + oral fluids + paracetamol Or weak opioids NSAID should be used only between 12-28 weeks.

Question 29

What are the initial investigations for woman with SCD having a painful crisis?

Answer 29

1-FBC 2-Reticulocyte count 3-Renal function *Other investigations depend on clinical exam: Blood culture Chest X ray Urine culture Liver function

Question 30

Based on WHO analgesia ladder , how to deal with painful crisis in pregnancy in a woman with SCD?

Answer 30

Initial analgesia should be given within 30 minutes of arriving at hospital Effective analgesia should be achieved within 1 hour ❤ mild pain 👉 paracetamol ❤NSAIDS 👉 only between 12- 28 w ❤ moderate pain 👉weak opioids ( co-dydramol, co-codamol,dihydrocodeine) ❤severe pain 👉Morphine 🛑pethidine should be avoided 👉 - risk of toxicity - associated seizures

Question 31

How to monitor pregnant woman with painful crisis while she is receiving analgesia ?

Answer 31

Pain/ sedation / vital signs/ respirator rate / O2 saturation * every 20- 30 minutes Until pain is controlled and signs are stable * then every 2 hours ( hourly if receiving parenteral opiates)

Question 32

What are the considerations about women receiving opiates?

Answer 32

1-If respiratory rate < 10/min 👉 stop analgesia +consider Naloxone 2- Treat the adverse effects of opiates:antihistamines ( antipruritic) Laxatives / antiemetics 3- opiates are NOT associated with teratogenicity or congenital malformations 4- opiates may be associated with transient suppression of fetal movement + reduced baseline variability of FHR

Question 33

After acute pain what is the most common complication in women with SCD?

Answer 33

Acute chest syndrome ACS 7-20% of pregnancies Symptoms & signs: same as pneumonia: tachypnoea / chest pain Cough / shortness of breath Chest X-ray: new infiltrate

Question 34

What is the DD of acute chest syndrome in women with SCD?

Answer 34

*Acute severe infection with H1N1 virus in pregnancy * pneumonia * pulmonary embolism ( if the woman presented with acute hypoxia)

Question 35

How should ACS be treated in pregnant women with SCD?

Answer 35

ACS suspected 👉 urgently reviewed by hematology team Hypoxia 👉 by critical care team ❤ treatment: - IV antibiotics - O2 - if Hb< 6.5 👉 top up transfusion - hypoxia + maintained Hb 👉 exchange transfusion. - if pulmonary embolism is suspected 👉LMWH

Question 36

What are the most important complications of SCD in pregnancy?

Answer 36

Painful crisis 27- 50% ACS 7- 20 % Acute stroke Acute anaemia

Question 37

How to diagnose and treat acute stroke associated with SCD in pregnancy?

Answer 37

🔴Diagnosis: 1-considered in any woman with SCD + acute neurological impairment 2- urgent brain imaging 🔴 treatment: hematologist review 👉 urgent rapid exchange transfusion ( decrease long term neurological damage)

Question 38

What is the DD of acute anaemia in women with SCD?

Answer 38

1- erythrovirus infection 2- bleeding 3- malaria 4- splenic sequestration

Question 39

If a woman with SCD presented with acute anaemia, what lab test should be requested?

Answer 39

Reticulocyte number If ⬇️ 👉 erythrovirus infection [ causes red cell maturation arrest & aplastic crisis] 👉 1- blood transfusion 2- the woman must be isolated 🛑 added risk of vertical transmission to the fetus 👉hydrops fetalis

Question 40

What is the optimal timing and mode of delivery in women with SCD?

Answer 40

Mode : vaginal delivery Timing: after 38 weeks ( induction of labour or CS) * atypical antibodies 👉 blood should be cross matched Otherwise group and save

Question 41

What is the optimum care and place of birth for women with SCD?

Answer 41

- in hospitals ( because of increased risk of painful crisis with protracted labour ) - multidisciplinary team -kept warm - adequate fluid during labour - pulse oximetry to detect hypoxia - continuous CTG - hourly observation of vital signs 🛑 routine antibiotics prophylaxis ISN'T recommended

Question 42

What is the optimum mode of analgesia and anaesthesia in women with SCD?

Answer 42

Avoid pithidine Opiates can be used Regional analgesia is recommended for CS General anaesthesia should be avoided

Question 43

What should be the optimum care post delivery for women with SCD?

Answer 43

1- if the baby is at high risk of SCD 👉 early capillary sample testing for SCD should be offered. 2- adequate hydration 3- maintain maternal O2 saturation > 95% . 4- LMWH while in hospital and *7 days post discharge/ vaginal birth *6 weeks post discharge/ CS 5- early mobilization

Question 44

What postpartum contraceptive advice should women with SCD be given?

Answer 44

POP / injectable contraceptive/ mirena: are safe and effective 🛑 estrogen- containing contraceptive should be used as second line agents. ( because of the concern about increased risk of venous thromboembolism 🛑copper IUD also category 2