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7-Sickle Cell Disease In Pregnancy > Nice > Flashcards

Nice Flashcards

1
Q

What is the most inherited condition worldwide?

A

SCD

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2
Q

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

A

UTI
Microscopic haematuria

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3
Q

What is the pathophysiology of SCD?

A

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.

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4
Q

What are the major complications of SCD?

A

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

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5
Q

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

A

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

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6
Q

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

A

Painful crisis during pregnancy
FGR
Antepartum hospital admission
Postpartum infections

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7
Q

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

A

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

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8
Q

How to make an assessment for SCD complications preconceptually?

A

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

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9
Q

How screening for pulmonary hypertension should be performed preconceptually?

A

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

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10
Q

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

A

HbSC

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11
Q

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

A
  • HbS
  • B thalassaemia
  • O- Arab
  • HbC
  • D- punjab
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12
Q

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

A
  • DB thalassaemia
  • lepore
  • HbE
  • hereditary persistent of fetal haemoglobin ( HPFH)
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13
Q

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

A

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 ✅

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14
Q

What vitamin supplements should be given preconceptually?

A

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

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15
Q

What medications should be reviewed preconceptually in women with SCD?

A

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

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16
Q

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

A

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

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17
Q

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

A

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)

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18
Q

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

A

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

19
Q

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

A

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

20
Q

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

A

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

21
Q

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

A

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

22
Q

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

A

🔴 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.

23
Q

What are the general aspects of blood transfusion during pregnancy?

A

❤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.

24
Q

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

A

18-36%

25
Q

What is the most frequent complication of SCD during pregnancy?

A

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

26
Q

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

A

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

27
Q

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

A

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.

28
Q

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

A

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

29
Q

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

A

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

30
Q

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

A

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

31
Q

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

A

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)

32
Q

What are the considerations about women receiving opiates?

A

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

33
Q

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

A

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

34
Q

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

A

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

35
Q

How should ACS be treated in pregnant women with SCD?

A

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

36
Q

What are the most important complications of SCD in pregnancy?

A

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

37
Q

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

A

🔴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)

38
Q

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

A

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

39
Q

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

A

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

40
Q

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

A

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

41
Q

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

A
  • 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
42
Q

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

A

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

43
Q

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

A

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

44
Q

What postpartum contraceptive advice should women with SCD be given?

A

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

7-Sickle Cell Disease In Pregnancy (1 decks)

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