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Gaby’s Green Top Guidelines > 2011 Sickle Cell Disease: Swap BSH > Flashcards

2011 Sickle Cell Disease: Swap BSH Flashcards

1
Q

What are the significant maternal haemoglobinopathies that can lead to sickle cell disease?

A
  1. HbSS (homozygous, worst)
  2. HbSC
  3. HbSD
  4. HbSE
  5. HbSO
  6. HbS/Lepore
  7. HbS/β-thalassemia
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2
Q

What is the patholophsiilogy of sickle cell disease?

A
  1. Abnormal haemoglobin
  2. Rigid, fragile, sickle-shaped RBCs
  3. Haemolytic anaemia
  4. Vaso-occlusion
  5. Complications & crises related to 4
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3
Q

What are the complications of sickle cell disease?

A
  1. Acute chest syndrome
  2. Pulmonary hypertension
  3. Stroke
  4. Renal dysfunction
  5. Retinal disease
  6. Leg ulcers
  7. Cholelithiasis
  8. Avascular necrosis
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4
Q

What obstetric complications are at increased risk in sickle cell disease?

A
  1. Hypertension & PET
  2. VTE
  3. UTIs
  4. Preterm birth
  5. Low birth weight
  6. Stillbirth
  7. Maternal mortality
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5
Q

What aspects of preconceptual care should be discussed at the annual sickle cell review?

A
  1. Partner testing
  2. Reproductive options: non-intervention, prenatal diagnosis, PGD
  3. Review of complications
  4. Medication review
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6
Q

What medications should be recommended preconceptionally/antenatally in SCD?

A
  1. Folic acid 5mg OD preconceptually & throughout
  2. Vitamin D
  3. Daily antibiotic prophylaxis
  4. Vaccinations: annual flu, 5-yearly pneumococcal
  5. Aspirin from 12-36/40
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7
Q

Which medications should be stopped prior to conceiving in SCD?

A
  1. ARBs & plan for ACEis
  2. Hydroxycarbamide
  3. Iron chelators
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8
Q

What haemoglobinopathy screening should be offered antenatally in SCD?

A
  1. Partner screening if not preconceptual
  2. Prenatal diagnosis: FFD, CVS, amnio; with option of termination
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9
Q

What schedule of ultrasound scanning should be offered in SCD?

A

Serial growth scans 4-weekly from 24/40

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10
Q

Which women with SCD should blood transfusions be considered for?

A
  1. Medical, obstetric or fetal problems
  2. Previously on hydroxycarbamide
  3. Multiple pregnancy
  4. On long-term transfusions for stroke prevention or severe sickle complications
  5. Worsening anaemia
  6. Acute complications eg stroke, acute chest syndrome
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11
Q

Why should NSAIDs be avoided from 31/40?

A

Risk of premature closure of PDA

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12
Q

What recommendations are made for pain management in SCD?

A
  1. Agreed pain Mx plan
  2. Standard except avoid NSAIDs
  3. If admitted, MDT, thromboprophylaxis, careful fluid & oxygen balance
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13
Q

What proportion of women with SCD have acute pain a) in pregnancy, b) postnatal?

A

a) 57%
b) 22%

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14
Q

What proportion of pregnant women with SCD develop acute chest syndrome?

A

10%

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15
Q

What are the characteristics of acute chest syndrome?

A
  1. Fever
  2. Respiratory symptoms
  3. New pulmonary infiltrate on CXR
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16
Q

How should acute chest syndrome in SCD be managed?

A
  1. Admit
  2. Specific treatment pathway
  3. CXR, FBC
  4. Rule out infective causes & consider PE
  5. Antibiotics covering for atypically even if cultures negative
  6. Analgesia
  7. Incentive spirometry
  8. Top-up or exchange transfusion
17
Q

What are the main differentials for acute neuro impairment in SCD?

A
  1. Stroke
  2. PET/eclampsia
18
Q

Which infections should be considered in acute anaemia in SCD?

A
  1. Parvovirus B19 = erythrovirus
  2. Malaria
19
Q

How does parvovirus infection manifest in SCD?

A
  1. Red cell maturation arrest
  2. Aplastic crisis
  3. Retuculocytopenia
20
Q

How much is the risk of pregnancy-related VTE increased in SCD?

A

1.5-5x

21
Q

How should VTE risk be managed in SCD?

A
  1. Risk assess early pregnancy, hospital admission, intrapartum, postpartum
  2. Consider 28/40 to 6/52 PN
  3. Consider throughout if additional RFs
  4. Give during hospital admission
22
Q

At what gestation is birth recommended in SCD?

A

38-40/40

23
Q

What mode of birth is recommended in SCD?

A
  1. Vaginal birth inc VBAC unless contraindications
  2. Plan positions if hip replacement following AVN
  3. CS if obstetric indication
24
Q

Which intrapartum analgesic should be avoided in SCD & why?

A

Pethidine
Risk of seizures

25
Q

What are the UKMEC ratings in SCD?

A
  1. Progesterone-only: 1
    Also may reduce sickle pain
  2. Copper IUCD: 2, due to blood loss
  3. Combined: 2, due to VTE/CV risk

Gaby’s Green Top Guidelines (51 decks)

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