Sickle cell

Question 1

Definition + prevelance

Answer 1

• Autosomal recessive
• Mainly in Africa
• Most common inherited disorder

Question 2

Risks of SC in preg

Answer 2

• FGR
• PTB
• Inc mortality
• Acute paingul crises

Question 3

Pre-conception

Answer 3

• Contraception
• Counsel on impact of preg on SC
• Optimise mx and screen for end organ damage
• Partner screening - counsel for termination if +
• Folic acid 5mg

Question 4

Pre-conception screening

Answer 4

• ECHO - inc risk of pulmonary HTN
• BP and urine
• Retinal screening - to check for proliferative retinopathy
• Screen for iron overload- cardiac T2 MRI, liver US
• Check for red cell antibodies

Question 5

Vaccine and abx

Answer 5

• Prophylactic penicillin / erythromycin (if allergic) to all SC pt
• HiB + Men C vaccine
• Hep B vacc

Question 6

Medications

Answer 6

• Stop hydroxycarbamide for 3/12
• Stop ACEi/ARB

Question 7

AN care

Answer 7

• Joint obs heam care
• Avoid triggers- dehydration, over exertion, heat/cold
• Seek help early for N+V
• Vaccines and screening if not done pre-conception
• Folic acid 5mg
• Aspirin for PET
• VTE risk
• Iron if Hb low

Question 8

Scans

Answer 8

• Early scan 7-9w
• Dating scan 11-14 w
• Anomaly scan 18-20w
• Serial scans evrey 4w from 24w

Question 9

Blood transfusion

Answer 9

• If symptomatic, Hb <60 or drop of >20 from baseline.
• If acute tx needed, cont regimen for the preg
• Blood- CMV neg, ABO and Rh compatible, antigen matched (C, E, Kell)

Question 10

Acute painful crises

Answer 10

• Assess quickly
• Analgesia- AVOID PETHEDINE, inc seizure risk
• Fluid and O2
• LMWH- if IP
• Investigations- symptoms based.

Question 11

Acute chest syndrome

Answer 11

• Most common complication after acute pain
• Tachypnoea, chest pain, cough, SOB
• Rx- IV abx, O2, blood tx

Question 12

Other complications

Answer 12

• Stroke- infarct/ heamorrhage
• Acute anaemia- may be due to erythrovirus infection. Treat w blood tx.

Question 13

Intrapartum mx

Answer 13

• IOL/CS at 38w if no complications
• X-match blood if atypical antibodies
• Hip replacements- discuss positions for birth
• Delivery on LW
• Warmed fluids
• CEFM
• Fluid balance- avoid dehydration
• Routine abx not needed

Question 14

PN Care

Answer 14

• Testing for baby- early if high risk
  Maternal obs + fluid balance
• VTE prophylaxsis
• Contraception advise