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MRCOG - Part 2 > Sickle Cell Disease in Pregnancy > Flashcards

Sickle Cell Disease in Pregnancy Flashcards

1
Q

Definition of SCD

A

a group of genetic single-gene autosomal recessive disorders caused by the “sickle” gene, which affects haemoglobin structure.

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2
Q

What are the hemoglobin types of Sickle cell trait

A

Hb A & Hb S

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3
Q

Ratio of HbA:HbS in Sickle cell trait

A

60:40

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4
Q

Type of Hb in Sickle Cell Anemia

A

HbSS

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5
Q

incidence of pregnancies with SCD in UK

A

100-200 Pregnancies per year

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6
Q

How many children born with SCD worldwide each year

A

300 K infants

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7
Q

How many children born with SCD in UK each year

A

300 infants

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8
Q

Average life expectancy of SCD

A

mid 50s

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9
Q

Is vomiting a red flag in SCD pregnant woman

A

Vomiting leads to Dehydration which leads Sickle cell Crisis

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10
Q

In SCD it’s importnat to check for End-organ Damage like:

A
  • Echo for PHTN
  • BP & Urinalysis for HTN &/or Proteinuria
  • Retinal Scan for Proliferative retinopathy
  • Cardiac MRI for Iron Overload (if woman has high ferritin or multiple transfusion)
  • RFTs & LFTs anually
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11
Q

importance of retinal screening in SCD

A

for Proliferative Retinopathy (most common in pts with SCD esp. in HbSD)

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12
Q

Medications to be stopped in pregnant women with SCD

A
  1. Hydroxycarbamide (hydroxyurea): stop before 3 months of conception - teratogenic
    If peregnancy happened before stopping-> stop it, US for malformations (termination isn’t indicated)
  2. ACEI
  3. ARB
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13
Q

SCD patient is susciptable for what infections

A

SCD patients are in risk of infection with encapsulated bacteria
1. Strept Pneumococci
2. Hemophilus Influenza
3. Niesseria Gonnorreah
4. Salmonella

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14
Q

Vaccinations that SCD patient shoud have before pregnancy

A
  1. H. Influenza vaccine (single dose)
  2. Conjugated Meningeococcal. C vaccine (single dose)
  3. Pneumococcal vaccine (every 5 years)
  4. Hepatitis B vaccine
  5. Ifluenza and Swine flu vaccine* (annualy)*
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15
Q

US schedule for Pregnant SCD patient

A

7-9 W: viability scan (in view of inc. risk of miscarriage)
11-14 W: Routine 1st trimester scan
20 W: Anomaly Scan
From 24 W: Serial Growth Scan (every 4W)

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16
Q

Medications to be given in SCD pregnant patient

A
  • Folic Acid 5mg/d
  • Penicillin Prophylaxis
  • Iron supplements if iron def. anemia
  • Low dose Aspirin 75mg/d from 12W -> dec. risk of PE
  • Prophylactic LMWH & TED socking in antenatal admissions
  • NSAIDs allowed only bet. 12-28 W
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17
Q

What to do if pregnancy happedned while in SCD patient is on Hydoxycarbamide?

A

Stop the medication -> US for malformations
Termination isn’t indicated

18
Q

Blood Transfusion to pregnant SCD patient

A
  1. Routine prophylactic transfusion isn’t recommended (except in twins pregnancy)
  2. Alloimunization is common in SCD (18-36%)
  3. Rhesus Matching
  4. Blood should be tested negative for CMV
19
Q

Indication for blood transfusion in SCD patient during pregnancy

A
  • Clinical findings
  • hemoglobin under 6 g/dl
  • Fall of over to 2g/dl from baseline
20
Q

When is exchange transfusion is indicated in pregnant SCD patient

A
  • Acute Coronary syn. ACS
  • Acute Stroke
21
Q

What is Acute Painful Crisis

A

Also named vaso-occlusive crisis, it is the main clincal feature of SCD, should be excluded in SCD pt. who become unwell

22
Q

Management of Acute painful crisis in pregnancy

A
  1. Rapid Assessment and appropriate analgesia given
  2. Pethidine shouldn’t be used (risk of seizures)
  3. Fluids & O2 when needed
  4. Thromboprophylaxis should be given
23
Q

Most frequent complication of SCD in pregnancy

A

Acute painful crisis (most common cause of admission)

24
Q

Indication for referral to hospital in women with painful crisis

A
  1. Pain doesn’t relieved by simple analgesics
  2. Fever
  3. Atypical pain or chest pain or shortness of breath
25
Q

Investigations of Painful Crisis

A
  • FBC
  • Reticulocytes count
  • RFT
  • Others depending on clincal scenario
26
Q

Management of painful crisis

A
  1. Mild pain: Paracetamol
  2. mild to moderate: NSAIDs (if 12-28w)
  3. Moderate: Weak opioids (co-dydramol- dihydrocodiene)
  4. Severe pain: Strong Opiates (morphine)

Monitor pain severity, RR, O2 saturation every 20 minutes
If RR<10/min omit maintainanace analgesia (naloxone)

27
Q

When treating painful crisis with strong opioids, how to follow up

A

if a mother recieved opiates in late preg. for ong time: neonate should be observed for signs of opioid withdrawal

28
Q

Assesing infection in painful crisis

A
  1. offer antibiotics if febrile
  2. WBCs are high in SCD, doesn’t mean infection
  3. Thromboprophylaxis to women w/ SCD and Painful crisis
29
Q

Fluid intake in management of a case of painful crisis

A

at least 60 ml/kg/24 hours

30
Q

Management of Acute stroke d.t. SCD in pregnant patient

A

Rapid exchange blood transfusion, can dec. long term neurological damage
Thrombolysis isn’t indicated

31
Q

Woman with SCD presented with acute hypoxia, what to suspect?

A

suspect Pulmonary embolism which has a raised risk with SCD.
There should be a low threshold for considering pulmonary embolism

32
Q

Causes of Acute anemia in patient with SCD

A

Due to Erythovirus infection:
- Causes aplastic anemia
- risk of vertical transmission to fetus causin hydrops fetalis

Other causes:
malaria & splenic sequestration

33
Q

Delivery of SCD patient

A
  1. It isn’t a CI of Vaginal delivery or VBAC
  2. Eletive birth by IOL or elective CS if indicated, after 38w with no FGR
  3. Blood Crossmatch if there are atypical anitbodies
  4. if there are no antibodies just “group and save”
33
Q

Delivery of SCD patient

A
  1. It isn’t a CI of Vaginal delivery or VBAC
  2. Eletive birth by IOL or elective CS if indicated, after 38w with no FGR
  3. Blood Crossmatch if there are atypical anitbodies
  4. if there are no antibodies just “group and save”
34
Q

Optimal mode of anaesthesia

A
  1. regional is recommended in CS
  2. General should be avoided
35
Q

Postpartum care of aptient with SCD

A
  1. Early testing of baby for SCD
  2. maintain maternal O2 saturation >94%
  3. Adeguate hydration
  4. LMWH while in hospital for 7 days after vaginal delivery and 6 weeks after CS
    5.
36
Q

Postpartum contraceptive safe with SCD

A
  1. POP, Depo-provera & Mirena are safe and effective.
  2. Estrogen containing pills - Cu IUD(2nd line)
  3. DMPA: less painful episodes
37
Q

What is Acute Chest Syndrome

A

Acute chest syndrome affects the lungs and is diagnosed by a new pulmonary infiltrate, dyspnea, hypoxia after ruling out pulmonary embolism or pneumonia.

38
Q

What is Acute Chest Syndrome

A

Acute chest syndrome affects the lungs and is diagnosed by a new pulmonary infiltrate, dyspnea, hypoxia after ruling out pulmonary embolism or pneumonia.

39
Q

Management of acute chest syndrome

A

Acute chest syndrome, a complication of sickle cell disease, when severe is best treated with partial exchange transfusion.

MRCOG - Part 2 (44 decks)

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