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MRCOG Maternal Medicine > Sickle Cell Disease in Pregnancy GTG > Flashcards

Sickle Cell Disease in Pregnancy GTG Flashcards

1
Q

What is the inheritance of sickle cell?

A

AR

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2
Q

Number ore pregnancies per year in women with SCD?

How many babies born with the condition?

A

100-200/year

300 born/year

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3
Q

What is sickle cell Disease/Trait?

A

Disease: Abnormality of the haemoglobin HbSS or HBS + other clinically abnormal haemoglobin HbSC, HbSB

Trait: 1 Abnormal gene HbAS carrier, - mostly asymptomatic, risk UTI, microscopic haematuria

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4
Q

Common clinical presentation of SCD

A

Painful crisis
* Avascular necrosis
* Stroke
* Pulmonary hypertension
* Retinal disease
* Leg ulcers
* Acute chest syndrome (ACS)
* Renal dysfunction
* Cholelithiasis

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5
Q

What should be offered in pre-conception care for women with sickle cell disease?

A

Review meds - stop ACEi/ARB/hydroxycarbamine, iron chelators

Vaccines: Influenza/pneumococaal

Start meds - 5mg Folic acid, ensure daily penicillin (hyposplenic), aspirin 12 weeks, consider LMWH in 1st trimester depending VTE, vitmain D

Assess chronic disease
-ECHO (PAH)
- BP/urine/O2 sats
- U+E/LFT
- Retinal screen

  • Screen red cell antibodies
  • Test partner
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6
Q

Which partner haemoglinopathies which conditions should be referred for PGD/counselling

A

HbS
B thalassaemia
O-Arab
HbC
D-punjab

Consider
DB thalassaemia, Lepore, HbE, hereditary persistance fetal haeomgloin

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7
Q

Vaccines for patients with SCD or thalassaemia + splenectomy

A

Vaccine:
* Pneumococcal vaccine
* Hepatitis B
* H. influenzae type b
* Influenza and swine flu
* Conjugated meningococcal C
* Meningitis B and ACWY

+ daily penicillin

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8
Q

What gestation can NSAIDS be given?

A

12-28 weeks

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9
Q

When should USS be offered?

A

Early 7-9 weeks (increased risk misc)
11-14 weeks
Detailed 20 weeks
Every 4 weeks from 24 weeks

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10
Q

When should be seen antenatally?

A

Review MDT Obs and Haemophilia @ 16 + 20 weeks
From 24 weeks MDT every 4 weeks, and MW every 4 weeks - seen every 2 weeks

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11
Q

What should be carefully checked at each appointment

A

BP and urine - high risk PET, carefully monitor for rise in BP

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12
Q

When should FBC be checked?

A

20, 28 and 32 weeks

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13
Q

How common is alloimmunisation in SCD?

A

18-36% - risk delayed transfusion reaction, haemolytic disease of newborn

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14
Q

When should blood transfusion be offered fort SCD?

A

Do not offer routinely, MDT discussion

Previous serious medical/obstetric/fetal complication - Exchange/top-up

Transfusion regime pre-pregnancy for stroke prevention - continue in pregnancy

Twin preg - prophylactic transfusion

Acute anaemia - top-up

Acute chest syndrome/actute stroke - exchange

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15
Q

How common is acute pain crisis in pregnancy?

A

57% of pregnancies
17% required hospital admission

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16
Q

How to investigate actue pain crisis

A

A-E assessment
Observations, <95% give oxygen
Hydration status
Consider - ACS, acute stroke, aplastic crisis, infection, osteomyelitis, splenic sequestration
Bloods - looks at FBC, reticulocytes, renal function

17
Q

What treatment to offer in acute pain crisis?

A

MDT (Obs, MW, haem,anaesthetics)
Offer analgesia within 30 mins
Consider Laxatives, antipruritic, antiseptic
Montior pain/sedation/vitals - O2 sats every 20-30 mins until pain controlled
VTE prophylaxis
Consider IVI

> 28 weeks CTG, consider USS

18
Q

What analgesia to give in pain crisis

A

Do not give pethidine
Start PO - opioids
NSAIDS if 12-28

Consider opioid toxicity if <RR, consider naloxone

Should have plan for pain crisis

19
Q

How common is acute chest syndrome in pregnancy? How does it present?

A

10% SCD in pregnancy
Fever +/- resp symptoms/ new pulmonary infiltrated on CXR.

DD PE/pneumonia

20
Q

How to manage acute chest syndrome

A

Analgesia
Oxygen
Incentive spirometry
Tx bacterial/viral infection - give Abx that cover atypical Abx

Blood transfusion - if falling or <65, severe exchange

Consider critical care for non-invasive/invasive ventilation if deteriorating

If requires transfusion should be offer blood transufiouns for the rest of pregnancy.

21
Q

If SCD women present with neurological Sx, what to consider and mange?

A

Acute stroke - infarctive or haemorrhage.

Urgent CT/MRI

Discuss with stroke physician and haem.

DD PET/eclamasia

Required exchange transfusion, discuss thromboylsis

22
Q

What is an important cause of acute anaemia to consider? What management?

A

Consider Parvovirus B19
Reticulocyte - will be low, red cell maturation arrest
Test serology
Blood transfusion & isolate
Refer to MDU - asses fetal anaemia

Consider malaria, splenic sequestration

23
Q

VTE assessment in SCD

A
  • Calculate score in early pregnancy
  • If SCD alone for LMWH from 28 weeks and 6 weeks PP, if additional RF, from beginning of pregnancy
  • LMWH during admission
  • Higher risk if complications such as vast-occlusive disease
24
Q

When should pregnancy women with SCD and a normally grown baby be delivered?

A

38-40 weeks

Can offer SVD/IOL or ELCS

Can offer VBAC

25
Q

Optical intrapartum care for SCD

A

Deliver in hospital able to manage complication of SCD/high risk pregnancy
MDT (haem, Obs, anaesthetic, senior MW)

Good analgesia
Avoid dehydration - fluid balance
Regular O2 Sats <94% ABG & oxygen
Avoid protracted labour
Avoid pethidine
Hourly obs including temp
Cont CTG
X match if atypical antibodies
Good positioning - especially if hip replacement (avascular nerosis of hip)
Keep patient warm

If EMCS - regional avoid GA

26
Q

What is the risk of sickle cell crisis in the postpartum period?

A

21-25%, more common post GA

27
Q

How to avoid sickle cell crisis post partum?

A

Same as intrapartum, good analgesia, fluid balance, mobilise.

28
Q

Can women with SCD breastfeed? Any precautions?

A

Yes, encourage BF
Avoid prescribing codeine to mother, can have dihydrocodiene and tramadol at lowest dose

29
Q

How long PN LMWH?

A

6 weeks

30
Q

What are the preferred options of contraception with women with SCD? What contraceptives have cautions?

A

Lowest rate of failure - LNG-IUS and IM DMPA - can reduced painful crisis

CuIUD - Category 2 - increased risk blood loss

CHCs Category 2 - risk of thrombosis and SC crisis maybe increased

31
Q

When should fetal testing of SCD be performed?

A

If partner is a carrier or partner affect or unknown status

MRCOG Maternal Medicine (41 decks)

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